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Sample records for plasmapheresis

  1. [Plasmapheresis in haematology].

    Science.gov (United States)

    Woźniak, Krzysztof; Urbanowska, Elżbieta; Snarski, Emilian

    2015-01-01

    Plasmapheresis also known as a therapeutic plasma exchange (TPE) is a procedure of plasma removal with it's ineligible plasma's component. Usually it is a supportive measure used simultaneously with the treatment, but in a few diseases, e.g. in trombotictrombocytopenic purpura (TTP), it is a first-choice treatment. During the plasmapheresis plasma is mostly replaced by 20% solution of albumin or combination of 20% solution of albumin and 0.9% solution of NaCl, however in some diseases fresh frozen plasma (FFP) is used. Plasmaphereses have found a wide application in different branches of medicine: hematology, neurology, nephrology, reumatology. Plasmapheresis is an invasive procedure, but when performed by qualified staff it is rather safe and serious complications are very rare.The most common complications of plasmapheresis are mild, usually caused by electrolyte disturbances (hypokalemia, hypokalcemia) or anticoagulation. More serious complication can be associated with FFP transfusion, extracorporeal circulation or presence of intravenous catheter. The latter one is usually necessary to perform the plasmapheresis. In haematology the most common indication for plasmapheresis is the supportive treatment of multiple myeloma. The procedure is performed in patients with high protein levels endangered with hyperviscosity syndrome. Less frequent indications to plasmapheresis in haematology are: Waldenström's macroblobulynaemia, idiopathic thrombocytopenic purpura (ITP), pure red cell aplasia (PRCA), polyneuropaties connected with haematological disorders. Supportive treatment of haemofagocytic syndrome (HLH--hemophagocytic lymphohistiocytosis) is one of the new indications. Plasmaphereses are used in treatment of about 150 different diseases and more and more new needs for this method are identified.

  2. Management of plasmapheresis in Germany.

    Science.gov (United States)

    Müller, N

    1996-05-01

    In Germany, plasmapheresis is carried out for many years in a "mixed" system by private and non-profit organisation. Most of the institutions and blood transfusion service, active in the field of plasmapheresis are members of the "Association for the Establishment, Co-ordination and Working Methods of the Plasmapheresis Centres". All centres are authorized by governmental bodies for manufacturing of pharmaceutical products with regulatory controls regarding licensing and accreditation of blood collection establishment. All parties concerned agree that self-sufficiency of plasma has to be achieved. For this purpose there is a shortage of about 400,000 1 of plasma. In 1994, about 240,000 1 of plasma were collected by plasmapheresis, 91% for fractionation and 8.7% for direct clinical uses. In addition about 970,000 1 of plasma were derived from whole blood donations. The dualism of non-profit making and commercial organisations should be regarded as a chance for diversification in the collection and processing of plasma. A long term increase in plasmapheresis plasma can be achieved by initiating plasmapheresis programmes and co-operation using the existing infrastructures and supports the aim for self-sufficiency.

  3. Value of plasmapheresis in hepatic encephalopathy.

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    Riviello, J J; Halligan, G E; Dunn, S P; Widzer, S J; Foley, C M; Breningstall, G N; Grover, W D

    1990-01-01

    Plasmapheresis is used for treating the complications of liver failure. We performed plasmapheresis on 6 children with hepatic encephalopathy resulting from acute hepatic failure and prospectively assessed its effects on neurologic and electrophysiologic (electroencephalography and evoked potentials) function. Clinical improvement was observed in 3 of 6 patients; changes in the serum ammonia value or the results of initial electrophysiologic tests did not predict the patient response. Two patients underwent transplantation after neurologic improvement was produced by plasmapheresis; however, despite plasmapheresis, 4 patients progressed to brain death. Our data demonstrate that plasmapheresis may transiently improve the encephalopathy of acute hepatic failure but is not curative alone. Therefore, plasmapheresis may be a useful adjunct in the treatment of liver failure, potentially improving the pretransplantation status of the patient.

  4. Plasmapheresis in the management of severe hypertriglyceridemia.

    Science.gov (United States)

    Seda, Gilbert; Meyer, Jill M; Amundson, Dennis E; Daheshia, Massoud

    2013-08-01

    Plasmapheresis can benefit a variety of critically ill patients. A woman with diabetic ketoacidosis and severe hypertriglyceridemia was treated with plasmapheresis when conventional treatments did not markedly reduce her triglyceridemia. The patient was admitted to a medical intensive care unit because of diabetic ketoacidosis with severe lipemia. The lipemia-associated interference in laboratory studies made treatment of electrolyte abnormalities extremely difficult. The hypertriglyceridemia was initially treated with insulin, antilipidemic medications, and heparin, but the levels of triglycerides remained elevated, delaying results of needed laboratory studies for hours. After plasmapheresis, the serum level of triglycerides decreased by 77% in less than 24 hours. Severe lipemia interferes with photometric laboratory studies, yielding an underestimation of serum levels of electrolytes. Plasmapheresis is safe, rapid, and effective for emergent management of severe hypertriglyceridemia in critically ill patients. The impact of the procedure on critical care nursing is growing as nurses become involved in the treatment and follow-up care of patients who have plasmapheresis.

  5. [Clinical experience of automated double filtration plasmapheresis].

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    Lee, C T; Chuang, F R; Hsu, K T; Lam, K K; Liao, S C; Liu, C C; Chen, J B; Jang, S W; Chien, Y S; Pan, H H

    1996-12-01

    Double filtration plasmapheresis, one kind of fractionation plasmapheresis, was developed from membrane type plasmapheresis to remove only the pathogen and return the normal protein back to the patient. We started our automated double filtration plasmapheresis since December 1993. There were 13 patients who received one hundred treatments totally during one year period. And they are myasthenia gravis (8 patients); acute inflammatory demyelinating polyneuropathy (1 patient), multiple myeloma (1 patient); acquired factor VIII inhibitor (1 patient); autoimmune hemolytic anemia (1 patient); systemic lupus erythematous (1 patient). Technically double filtration plasmapheresis is easy to perform and time-saving. It also makes necessity of replacement fluid less frequent. Incidence of complication is rare, and this includes hypotension 2%, palpitation 1%, headache 1%, hemolysis 4%, air emboli 1%, high secondary pressure 2%, and no motality during our treatment. Clinical response is documented in cases of myasthenia gravis; acute inflammatory demyelinating polyneuropathy and acquired factor VIII inhibitor in our study. In conclusion, double filtration plasmapheresis is a time-saving, convenient, and safe therapeutic modality with rare complication. Because its effectiveness on limited kinds of diseases and costs relatively high price, thus plasmapheresis should be used in selected cases and treat aggressively if indicated.

  6. Treatment of plasmapheresis refractory thrombotic thrombocytopenic purpura with double-filtration membrane plasmapheresis.

    Science.gov (United States)

    Karakus, Volkan; Deveci, Burak; Kurtoğlu, Erdal

    2013-06-01

    Thrombotic thrombocytic purpura (TTP) is a life-threatening disorder. Without plasma exchange treatment (PET) the mortality rate is quite high. Double-filtration plasmapheresis is an alternative opportunity for TTP patients refractory to PET. Here we report our experience in a refractory TTP patient who was successfully treated by means of double-filtration plasmapheresis therapy.

  7. Removal of 10-hydroxycarbazepine by plasmapheresis

    DEFF Research Database (Denmark)

    Christensen, J; Balslev, T; Villadsen, J;

    2001-01-01

    with studies on other anticonvulsant medications (carbamazepine, valproic acid, phenobarbital, and phenytoin), indicating that minor fractions (2% to 10%) of body stores of these drugs are depleted during plasmapheresis. The authors conclude that it is unnecessary to adjust the oxcarbazepine dosage when...

  8. Removal of 10-hydroxycarbazepine by plasmapheresis

    DEFF Research Database (Denmark)

    Christensen, J; Balslev, T; Villadsen, J

    2001-01-01

    with studies on other anticonvulsant medications (carbamazepine, valproic acid, phenobarbital, and phenytoin), indicating that minor fractions (2% to 10%) of body stores of these drugs are depleted during plasmapheresis. The authors conclude that it is unnecessary to adjust the oxcarbazepine dosage when...

  9. Plasmapheresis/immunoadsorption in patients with pemphigus

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    Rıfkiye Küçükoğlu

    2015-03-01

    Full Text Available Background and Design: Plasmapheresis or immunoadsorption which is a more advanced method are among the treatment options that induce a rapid remission in severe and resistant pemphigus. The aim of this study is to evaluate the efficacy and safety of plasmapheresis or immunoadsorption therapies via the clinical experience gained from our patients with pemphigus vulgaris and paraneoplastic pemphigus. Materials and Methods: Among patients who were diagnosed with pemphigus according to the histopathology and/or immunofluorescence methods and followed-up at our bullous diseases outpatient clinic between the years 1987 and 2013, 7 patients (5 PV, 2 PNP, who were treated with plasmapheresis or immunoadsorption, were retrospectively analyzed. Among these patients, 3 were treated with classical plasmapheresis, whereas 2 with double-filtration plasmapheresis, 1 with immunoadsorption, and 1 initially with classical plasmapheresis followed by immunoadsorption due to impairment of the hemodynamic parameters. The clinical findings, antibody titers, and immunological responses before and after the therapy, the therapy dosages and adverse effects were evaluated according to the data gained from the patient files. Results: In 4 patients (1., 3., 4. and 5. patients with pemphigus vulgaris, complete clinical response was achieved, while 1 patient (2. patient showed partial regression in skin lesions. In both of the patients (6.,7. patients with paraneoplastic pemphigus the therapy discontinued due to complications. In all of the evaluated patients, the analysis of the antibody titers before and after the therapy revealed lower levels in comparison to those detected at the initiation of the therapy. In 2 patients hypoalbuminemia and in 2 patients catheter infection were developed as the side effects of the therapy. Conclusion: Even though plasmapheresis or immunoadsorption may be chosen as a good mid-treatment method to decrease the severity of the disease

  10. Therapeutic plasmapheresis using membrane plasma separation.

    Science.gov (United States)

    Sinha, Aditi; Tiwari, Anand Narain; Chanchlani, Rahul; Seetharamanjaneyulu, V; Hari, Pankaj; Bagga, Arvind

    2012-08-01

    The authors present their experience with therapeutic plasmapheresis (TPE) using membrane filters at the pediatric dialysis unit of a referral center. Between January 2006 and December 2010, 486 sessions of TPE were performed in 39 patients (range 6-17 y), chiefly for atypical hemolytic uremic syndrome (HUS, n = 22), crescentic glomerulonephritis (n = 8) and focal segmental glomerulosclerosis (n = 5). Satisfactory response was noted in 32 patients, particularly with HUS (n = 22) or crescentic glomerulonephritis (n = 6). Adverse effects included chills or urticaria (n = 8 sessions), hypocalcemia (n = 6) and hypotension (n = 5). The present findings highlight the safety, efficacy and feasibility of TPE using membrane filtration.

  11. USE OF THERAPEUTIC PLASMAPHERESIS IN ETHYLENE DIBRO MIDE INTOXICATION

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    Dolly

    2013-02-01

    Full Text Available ABSTRACT: In view of high incidence of mortality with Ethylen e dibromide poisoning, a retrospective study was conducted, with objectives t o compare demographic, toxicological & clinical outcome in patients treated conservatively and those who underwent therapeutic plasmapheresis

  12. Plasmapheresis and other extracorporeal filtration techniques in critical patients.

    Science.gov (United States)

    Daga Ruiz, D; Fonseca San Miguel, F; González de Molina, F J; Úbeda-Iglesias, A; Navas Pérez, A; Jannone Forés, R

    2017-04-01

    Plasmapheresis is an extracorporeal technique that eliminates macromolecules involved in pathological processes from plasma. A review is made of the technical aspects, main indications in critical care and potential complications of plasmapheresis, as well as of other extracorporeal filtration techniques such as endotoxin-removal columns and other devices designed to eliminate cytokines or modulate the inflammatory immune response in critical patients. Copyright © 2016 Elsevier España, S.L.U. y SEMICYUC. All rights reserved.

  13. 21 CFR 606.110 - Plateletpheresis, leukapheresis, and plasmapheresis.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 7 2010-04-01 2010-04-01 false Plateletpheresis, leukapheresis, and plasmapheresis. 606.110 Section 606.110 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) BIOLOGICS CURRENT GOOD MANUFACTURING PRACTICE FOR BLOOD AND BLOOD COMPONENTS Production and Process Controls §...

  14. A plasmapheresis protocol for refractory pulmonary alveolar proteinosis.

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    Garber, Bryan; Albores, Jeffrey; Wang, Tisha; Neville, Thanh H

    2015-04-01

    Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of proteinaceous material within the lungs. While typically managed with whole lung lavage (WLL), more recent PAP therapies aimed at reducing granulocyte-macrophage colony stimulating factor autoantibodies (anti-GM-CSF) have reduced symptoms and improved lung function. We present a patient with PAP refractory to WLL, exogenous GM-CSF and rituximab who underwent a novel plasmapheresis protocol as a therapeutic trial. While previously reported regimens have utilized plasmapheresis sessions distributed over months, our patient underwent five consecutive days of plasmapheresis, followed by rituximab. Anti-GM-CSF levels decreased from 24.8 to 2.7 mcg/mL post-plasmapheresis. This reduction of autoantibody correlated with reduction in WLL frequency, increase in diffusing capacity for carbon monoxide, and subjective improvement in dyspnea. Our case suggests that five consecutive days of plasmapharesis results in increased clearance of anti-GM-CSF and may be potentially efficacious in cases of refractory PAP.

  15. Severe childhood amitriptyline intoxication and plasmapheresis: a case report.

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    Karacı, Mehmet; Özçetin, Mustafa; Dilsiz, Günter; Güçlü-Songür, Yaşar Gözde

    2013-01-01

    Tricyclic antidepressant intoxication is one of the most frequently encountered and life-threatening causes of intoxication among referrals to emergency departments due to drug intoxication. There is no known antidote against any of the tricyclic antidepressants. The American Society for Apheresis (ASFA) recommends plasmapheresis to support primary treatment in this type of drug poisoning, which does not respond to certain and traditional treatments. We present a 15-year-old girl who ingested amitriptyline with suicidal intent. On admission, she was in a comatose state (Glasgow Coma Scale score: 5), with no spontaneous respiration and presence of pathological reflexes. Due to the intake history of lethal doses and the severe clinical picture, plasmapheresis was performed. She was discharged on her fifth day of hospitalization.Due to the high plasma protein binding property of amitriptyline, plasma exchange therapy should be considered in cases of severe amitriptyline intoxication as a life-saving therapeutic modality.

  16. A memantin HCL intoxication responsive to plasmapheresis therapy.

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    Cekmen, N; Bedel, P; Erdemli, O

    2011-01-01

    Memantin HCL (Ebixa) is a drug which antagonizes the effects of N-methyl-D-aspartat receptors and which is used for the treatment of acute Alzheimer patients. Plasmapheresis is a method of cleaning nonspecific extracorporeal blood and it is applied in many immunologic and toxicologic diseases. Female patient at the age of 35 was admitted to the emergency department with complaints of tendency to sleep and sensory loss. About 12 hours before her history she had taken 200 tablets of 10 mg memantin HCL (Ebixa) (2000 mg) and she was transferred to an intensive care department with the diagnosis of drug toxicity (400 mg toxic dose). Her memantin HCL (Ebixa) level in blood was 12,000 ng/mL. It was reported in her physical examination that she was unconscious, her general condition was bad, there were no cooperation and orientation, ahe hadmydriasis and reflexes of light, cornea and eyelash were bilaterally positive and she had horizontal nystagmus. Glascow Coma Scale of the patient was 6, body temperature was 37.5 degrees C and she had tachycardia (130/min) and hypertension (160/90 mmHg). Intravenous Diazepam was effective aginst recurring convulsions. Sinusoidal tachycardia was detected with electrocardiography (EKG) and respiratory alkalosis in arterial blood gases. Six cysles of plasmapheresis were aplied and in the sixth cycle the memantin HCL (Ebixa) level turned to normal. As a result of the sixth plasmapheresis the findings were normal and that is why she was discharged from the hospital. Plasmapheresis should be taken into consideration in case of drug overdose or high doses of plasmatic proteinous drug toxicities (Ref. 11).

  17. Adult-onset Satoyoshi syndrome and response to plasmapheresis

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    Rajeshwari Aghoram

    2016-01-01

    Full Text Available Satoyoshi syndrome is a rare disease characterized by alopecia, recurrent muscle spasms, diarrhea, and skeletal abnormalities Adult-onset disease is reported only in five patients. Most of the reports have not characterized the nature of muscle spasm in the disease. In this paper, we report the first case of adult-onset Satoyoshi syndrome from India and the clinical and electrophysiological response to plasmapheresis.

  18. Long-term experience of plasmapheresis in antibody-mediated rejection in renal transplantation.

    LENUS (Irish Health Repository)

    Brown, C M

    2009-11-01

    Antibody-mediated rejection (AMR) continues to pose a serious challenge in renal transplantation with potentially devastating consequences. Treatment options for this condition include plasmapheresis, high-dose intravenous immunoglobulin (IVIG), plasmapheresis with low-dose IVIG, and the use of rituximab (anti-CD20 chimeric antibody). We previously reported on the short-term outcome of plasmapheresis as a rescue therapy for AMR in our centre. We now report on the long-term follow up.

  19. Protective Antigen (PA) and Toxin Neutralization (TNA) Antibody Patterns in Anthrax Vaccinees Undergoing Serial Plasmapheresis

    Science.gov (United States)

    2005-03-28

    Undergoing Serial Plasmapheresis Phillip R. Pittman,1 Susan F. Leitman,2 Julio G. Barrera Oro,3 Sarah L. Norris,4 Nina M. Marano,5 Manmohan V. Ranadive...plasmapheresis on serum proteins and immunoglobulins. Transfusion 15:467–472. 12. Gold, H. 1936 . Studies on anthrax. Clinical report of ten human cases

  20. Reducing the economic burden in management of Guillain–Barre syndrome using modified plasmapheresis

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    Rekha Ramaswamy Iyer

    2016-01-01

    Full Text Available Background: Guillain–Barre syndrome (GBS is an autoimmune acute inflammatory demyelinating polyneuropathy affecting the peripheral nervous system treated with high-dose immunoglobulin, physical therapy, or plasmapheresis. Immunoglobulins are expensive and even plasmapheresis might not be affordable to patients visiting government set-ups.Aims: This study was undertaken to emphasize the efficacy of plasmapheresis in treatment of adult GBS patients and to narrate methods of reducing the economic burden in the treatment of these patients using modified plasmapheresis. Methods: A study was conducted on 12 adult GBS patients at Sir Takhtasinhji General Hospital, Bhavnagar from July 2012 to July 2014. Patients were assessed on a 6-point disability scale. They were treated with plasmapheresis over 10 days with REF627 kit from Haemonetics Corporation Limited on MCS+ machine. Improvement was noted by the change in the disability scale score and expenses of various modes of treatment were also considered. Results: Seventy-five percent showed improvement at the end of the treatment. The cost of modified plasmapheresis was Rs. 8000/cycle, i.e., Rs. 40,000/patient. Conclusion: Plasmapheresis along with proper supportive measures is a more cost-effective efficacious mode of therapy in adult patients of GBS. Further, modified plasmapheresis using REF627 kit and 6% hexastarch as replacement fluid on MCS+ apheresis machine reduces the cost of therapy for poor patients visiting government set-ups.

  1. Effect of plasmapheresis on the immune system in endotoxin-induced sepsis

    DEFF Research Database (Denmark)

    Toft, P; Schmidt, R; Broechner, A C

    2008-01-01

    BACKGROUND: It has been proposed that plasmapheresis is most effective when applied early in Gram-negative sepsis. We therefore studied the effect of early plasmapheresis on immunity in experimental Escherichia coli endotoxin-induced sepsis. METHODS: 20 pigs received 30 microg/kg of E. coli...

  2. Obilježja bolesnika liječenih plazmaferezom [Characteristics of patients treated with plasmapheresis

    OpenAIRE

    Brunetta Gavranić, Bruna

    2015-01-01

    Frequent use of therapeutic plasmapheresis and advances in medicine and technology generated significant changes in indications, complications, method and epidemiological characteristics of patients treated with this procedure. Nevertheless many questions still remain open. Database of the National Referral Centre for therapeutic plasmapheresis and apheresis was retrospectively analyzed for changes in indications, complications, method and epidemiological characteristics of patients treate...

  3. Improvement of advanced postvaccinal demyelinating encephalitis due to plasmapheresis

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    Andreas Rogalewski

    2007-01-01

    Full Text Available Andreas Rogalewski1, Jörg Kraus3, Martin Hasselblatt2, Christoffer Kraemer1, Wolf-Rüdiger Schäbitz11Department of Neurology; 2Institute of Neuropathology, University of Muenster, Germany, 3Paracelsus Private Medical University and Salzburger Landesklinken, Christian-Doppler-Klinik, Department of Neurology, Salzburg, AustriaAbstract: We report a case of acute demyelinating encephalitis that occurred after viral vaccination against hepatitis A-, hepatitis B-, and poliovirus and vaccination against bacterial toxins of diphtheria and tetanus. After different diagnosis had been excluded, we diagnosed postvaccinal demyelinating encephalitis and started treatment with high dose intravenous methylprednisolone, followed by peroral application in decreasing dosages for three weeks. A few days after the treatment with methylprednisolone had been finished, the patient’s medical condition deteriorated again. Thus, we initiated plasma exchange at an advanced state of illness, which led to significant continuous improvement. The role of plasma exchange is discussed controversially, in particular the issue of timing. We report a case that shows improvement due to plasmapheresis several weeks after symptom onset.Keywords: ADEM, vaccination, encephalitis, plasmapheresis, demyelination, plasma exchange

  4. [Goodpasture syndrome: treatment initiation with plasmapheresis before histologic diagnostic verification].

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    Gartenschläger, M; Lenci, G; Schinzel, H; Schweden, F; Dienes, H P; Weilemann, L S

    1996-11-01

    A 28 years old male patient presented, after a history of previous recurrent hemoptysis, with diffuse bilateral air space consolidation at chest radiography (CXR). Within 48 hours, partial respiratory insufficiency developed and required intubation. On a clinical and roentgenographic basis, the diagnosis of a Goodpasture syndrome was suspected. Plasmapheresis and immunosuppressive therapy with prednisone and cyclophosphamide were started immediately. Three days after admission, macrohematuria developed and serum creatinine began to rise to a maximum of 3.9 mg/dl. Totally, 13 plasmaphereses were performed within 27 days. Clinical, laboratory and radiological findings improved markedly. 30 days after admission, the patient was discharged and followed on an outpatient basis. Serum creatinine eventually decreased to 1.1 mg/dl. Initially, circulating antibodies against glomerular basement membrane (GBM) were positive, controls remained negative. Renal biopsy was performed after the acute phase and showed glomerulonephritis and linear immunoglobulin deposition along the GBM. Radiologic findings at CXR and high resolution computed tomography are demonstrated.

  5. Electroconvulsive therapy and/or plasmapheresis in autoimmune encephalitis?

    Science.gov (United States)

    Gough, Jessica L; Coebergh, Jan; Chandra, Brunda; Nilforooshan, Ramin

    2016-01-01

    Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes - anti-N-Methyl-D-Aspartate receptor (NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment (and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment. We discuss several previously published case reports and one new case. These reports examined the effects of electroconvulsive therapy (ECT) on patients with autoimmune encephalitis, particularly those in whom psychiatric symptoms are especially debilitating and refractory to standard treatment. We also discuss factors predicting good outcome and possible mechanisms by which ECT may be effective. Numerous cases, such as those presented by Wingfield, Tsutsui, Florance, Sansing, Braakman and Matsumoto, demonstrate effective use of ECT in anti-NMDAR encephalitis patients with severe psychiatric symptoms such as catatonia, psychosis, narcolepsy and stupor who had failed to respond to standard treatments alone. We also present a new case of a 71-year-old female who presented to a psychiatric unit initially with depression, which escalated to catatonia, delusions, nihilism and auditory hallucinations. After anti-NMDAR antibodies were isolated, she was treated by the neurology team with plasmapheresis and steroids, with a partial response. She received multiple sessions of ECT and her psychiatric symptoms completely resolved and she returned to her premorbid state. For this reason, we suggest that ECT should be considered, particularly in those patients who are non-responders to standard therapies. PMID

  6. Utility of Double Filtration Plasmapheresis in Acute Antibody Mediated Renal Allograft Rejection: Report of Three Cases

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    Yalçın SOLAK

    2011-09-01

    Full Text Available Plasmapheresis is an extracorporeal procedure, which is often employed to rapidly lower circulating titers of autoantibodies, immune complexes or toxins. There are two types of plasmapheresis namely, regular plasmapheresis (RPP by centrifugation and membrane filtration, and double filtration plasmapheresis (DFPP which is a special form of membrane filtration in which two membranes called as plasma separator and plasma fractionator are employed to filter macromolecules more selectively. DFPP have several advantages over RP. Despite widespread utilization of DFPP in the setting of ABO blood group incompatible kidney transplantation, there is no report regarding DFPP in patients with antibody mediated acute renal allograft rejection who are good candidates for beneficial effects of DFPP. Here we report three renal transplant recipients in whom DFPP was applied as a component of anti-rejection treatment regimen.

  7. Development of recurrent facial palsy during plasmapheresis in Guillain-Barré syndrome: a case report

    OpenAIRE

    Weimer Louis H; Stevenson Mary L; Bogorad Ilya V

    2010-01-01

    Abstract Introduction Guillain-Barré syndrome is an immune-mediated polyneuropathy that is routinely initially treated with either intravenous immunoglobulin or plasmapheresis. To the best of our knowledge, no association between plasmapheresis treatment and acute onset of facial neuropathy has been reported. Case presentation A 35-year-old Caucasian man with no significant prior medical history developed ascending motor weakness and laboratory findings consistent with a diagnosis of Guillain...

  8. Development of recurrent facial palsy during plasmapheresis in Guillain-Barré syndrome: a case report

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    Weimer Louis H

    2010-08-01

    Full Text Available Abstract Introduction Guillain-Barré syndrome is an immune-mediated polyneuropathy that is routinely initially treated with either intravenous immunoglobulin or plasmapheresis. To the best of our knowledge, no association between plasmapheresis treatment and acute onset of facial neuropathy has been reported. Case presentation A 35-year-old Caucasian man with no significant prior medical history developed ascending motor weakness and laboratory findings consistent with a diagnosis of Guillain-Barré syndrome. Plasmapheresis was initiated. Acute facial palsy developed during the plasma exchange that subsequently resolved and then acutely recurred during the subsequent plasma exchange. Conclusion To the best of our knowledge, no prior cases of acute facial palsy developing during plasmapheresis treatment are known. Although facial nerve involvement is common in typical Guillain-Barré syndrome, the temporal association with treatment, near-complete resolution and later recurrence support the association. The possible mechanism of plasmapheresis-induced worsening of peripheral nerve function in Guillain-Barré syndrome is unknown.

  9. The steroid-sparing effect of long-term plasmapheresis in pemphigus

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Carstens, Jan; Jørgensen, Jan

    1995-01-01

    Glucocorticoids and immunosuppressive agents can induce remission in most patients with pemphigus, but mortality remains at 5 to 15% due to complications from these drugs. We reviewed the adjunctive effect of long-term plasmapheresis in 8 patients with pemphigus. Four cases had been resistant to ...... where cyclosporine was introduced. This first report of long-term plasmapheresis demonstrates clinical efficacy in pemphigus and a considerable steroid-sparing effect.......Glucocorticoids and immunosuppressive agents can induce remission in most patients with pemphigus, but mortality remains at 5 to 15% due to complications from these drugs. We reviewed the adjunctive effect of long-term plasmapheresis in 8 patients with pemphigus. Four cases had been resistant...

  10. Double filtration plasmapheresis in a dog with multiple myeloma and hyperviscosity syndrome

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    I. Lippi

    2015-08-01

    Full Text Available A 12 year old, 38 kg, mix-breed, intact male dog presented with a 20 day history of clinical signs consistent with hyperviscosity syndrome secondary to multiple myeloma. The dog received three double filtration plasmapheresis treatments on day 0, 7 and 22 after presentation. A significant (p<0.05 reduction in serum total protein, alpha-2 and gamma globulins was found following each treatment. These reductions were accompanied by a complete resolution, although temporary, of the clinical signs of hyperviscosity syndrome. The present study reported for the first time the use of double filtration plasmapheresis to reduce clinical signs of hyperviscosity syndrome in a dog with multiple myeloma.

  11. Successful treatment of malaria tropica with acute renal failure and cerebral involvement by plasmapheresis and hemodialysis.

    Science.gov (United States)

    Stuby, U; Kaiser, W; Biesenbach, G; Zazgornik, J

    1988-01-01

    A non-immune, 31-year-old woman developed an acute infection with Plasmodium falciparum after travelling to Kenia. The parasites proved resistant to chloroquine and sulfadoxine/pyrimethamine. The course of the disease was complicated by acute renal failure, hepatocellular damage, disorders of blood coagulation, thrombocytopenia, hemolysis and cerebral involvement. Despite a very high level of parasitemia (50% parasitized erythrocytes) a rapid clinical improvement was achieved by plasmapheresis and hemodialysis. Our experience shows that plasmapheresis and hemodialysis are excellent additive methods which rapidly improve clinical symptoms and may reduce morbidity and mortality in severe malaria tropica.

  12. Early plasmapheresis and rituximab for acute humoral rejection after ABO-compatible liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Nassim Kamar; Laurence Lavayssière; Fabrice Muscari; Janick Selves; Céline Guilbeau-Frugier; Isabelle Cardeau; Laure Esposito; Olivier Cointault; Marie Béatrice Nogier; Jean Marie Peron; Philippe Otal; Marylise Fort; Lionel Rostaing

    2009-01-01

    Acute humoral rejection (AHR) is uncommon after ABOcompatible liver transplantation. Herein, we report two cases of AHR treated with plasmapheresis and rituximab in two ABO-compatible liver-transplant patients with preformed anti-human leukocyte antigen donor-specific antibodies. Patient 1 experienced a biopsy-proven AHR at day 10 post-transplant. She was treated by steroid pulses, and OKT3. Because of persisting signs of biopsy-proven AHR at day 26, she was treated by plasmapheresis and rituximab. Liver enzyme levels did not improve, and she died on day 41. Patient 2 experienced a biopsy-proven AHR on day 10 post-transplant. She was treated by steroid pulses, plasmapheresis, and rituximab.Liver enzymes returned to within normal range 18 dafter diagnosis. Liver biopsies, at 3 and 9 mo post-transplant,showed complete resolution of AHR. We conclude that plasmapheresis should be started as soon as AHR is diagnosed, and be associated with a B-cell depleting agent. Rituximab may be considered as a first-line therapy.

  13. Possible role for exchange plasmapheresis with fresh frozen plasma for maternal indications in selected cases of preeclampsia and eclampsia.

    Science.gov (United States)

    Schwartz, M L

    1986-07-01

    A new case of a preeclamptic who developed the diagnostic criteria for the hemolytic uremic syndrome and who was treated with plasmapheresis is presented. Fourteen case reports of gravidas with preeclampsia treated by exchange plasmapheresis with fresh frozen plasma for maternal indications are reviewed. Only four cases had been reported as eclampsia or preeclampsia. The other cases had been reported as thrombotic thrombocytopenic purpura, the postpartum hemolytic uremic syndrome, or fatty liver of pregnancy. The possible role of plasmapheresis in treating very carefully selected cases of preeclampsia is discussed.

  14. Plasmapheresis Rescue Therapy in Progressive Systemic ANCA-Associated Vasculitis : Single-Center Results of Stepwise Escalation of Immunosuppression

    NARCIS (Netherlands)

    de Joode, Anoek A. E.; Sanders, Jan Stephan; Smid, W. Martin; Stegeman, Coen A.

    2014-01-01

    Objective: We evaluated 26 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) with progressive disease despite treatment with cyclophosphamide and steroids treated with additional plasmapheresis and compared outcome with 50 matched-disease controls. Methods: Pat

  15. Fulminant Wilson's Disease Managed with Plasmapheresis as a Bridge to Liver Transplant.

    Science.gov (United States)

    Hilal, Talal; Morehead, R Scott

    2014-01-01

    New-onset jaundice can be a manifestation of multiple pathologic processes including hemolysis, parenchymal liver disease, and cholestasis; the differential diagnosis is broad and requires a systematic approach. We report a case of a patient who presented with jaundice after starting minocycline for the treatment of acne vulgaris and rapidly developed fulminant liver failure found to be due to Wilson's disease. She also manifested severe Coomb's negative hemolytic anemia and renal failure secondary to hepatorenal syndrome. As a bridge to liver transplant, she was successfully treated with plasmapheresis to decrease serum copper in addition to hemodialysis for acidosis and hyperkalemia. She was able to receive a liver and made a full recovery. The case highlights the use of plasmapheresis as an adjunctive treatment modality in cases of fulminant liver failure due to Wilson's disease.

  16. Fulminant Wilson’s Disease Managed with Plasmapheresis as a Bridge to Liver Transplant

    Directory of Open Access Journals (Sweden)

    Talal Hilal

    2014-01-01

    Full Text Available New-onset jaundice can be a manifestation of multiple pathologic processes including hemolysis, parenchymal liver disease, and cholestasis; the differential diagnosis is broad and requires a systematic approach. We report a case of a patient who presented with jaundice after starting minocycline for the treatment of acne vulgaris and rapidly developed fulminant liver failure found to be due to Wilson’s disease. She also manifested severe Coomb’s negative hemolytic anemia and renal failure secondary to hepatorenal syndrome. As a bridge to liver transplant, she was successfully treated with plasmapheresis to decrease serum copper in addition to hemodialysis for acidosis and hyperkalemia. She was able to receive a liver and made a full recovery. The case highlights the use of plasmapheresis as an adjunctive treatment modality in cases of fulminant liver failure due to Wilson’s disease.

  17. An alternative treatment in hypertriglyceridemia-induced acute pancreatitis in pregnancy: Plasmapheresis

    OpenAIRE

    2012-01-01

    Hormonal influences during pregnancy can compromise otherwise controlled lipid levels in women with familial hypertriglyceridemia and predispose to pancreatitis leading to increased morbidity in both mother and fetus. Both cholesterol and triglyceride levels in serum increase progressively during pregnancy. The mainstay of treatment includes dietary restriction of fatty meal and lipid-lowering medications. Experiences with plasmapheresis are limited. We report two cases of hypertriglyceridemi...

  18. Acute Respiratory Failure due to Neuromyelitis Optica Treated Successfully with Plasmapheresis

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    Massa Zantah

    2016-01-01

    Full Text Available Neuromyelitis Optica (NMO is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.

  19. Plasmapheresis for Preventing Complication of Hypertriglyceridemia: A Case Report and Review of Literature.

    Science.gov (United States)

    Costantini, Nicoletta; Mameli, Antonella; Marongiu, Francesco

    2016-01-01

    Severe hypertriglyceridemia is a common indication for the need of plasma exchange in treatment of hypertriglyceridemic-induced pancreatitis when normal therapies fail to garner a response. Application of plasmapheresis to prevent complication of hypertriglyceridemia is limited because of its cost and availability. We present a case of a 44-year-old man with metabolic syndrome and a medical history of secondary polycythemia in obesity hypoventilation syndrome, whose laboratory tests revealed a triglycerides value of 3965 mg/dL. To prevent the complication of pancreatitis due to hypertriglyceridemia, we performed plasma exchange 3 times when conventional treatments did not sufficiently reduce the high level of triglycerides. A review of the current available literature was therefore conducted to provide an overview of the present data on apheretic treatment for patients with severe hypertriglyceridemia. Several case reports and case series have used plasmapheresis in acute treatment of hypertriglyceridemia pancreatitis related. In our case, the choice of plasmapheresis was applied in prevention of possible complications of hypertriglyceridemia.

  20. Response to Plasmapheresis Measured by Angiogenic Factors in a Woman with Antiphospholipid Syndrome in Pregnancy

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    Karoline Mayer-Pickel

    2015-01-01

    Full Text Available An imbalance of angiogenic and antiangiogenic placental factors such as endoglin and soluble fms-like tyrosine kinase 1 has been implicated in the pathophysiology of preeclampsia. Extraction of these substances by plasmapheresis might be a therapeutical approach in cases of severe early-onset preeclampsia. Case Report. A 21-year-old primigravida with antiphospholipid syndrome developed early-onset preeclampsia at 18 weeks’ gestation. She was treated successfully with plasmapheresis in order to prolong pregnancy. Endoglin and sflt-1-levels were measured by ELISA before and after treatment. Endoglin levels decreased significantly after treatment (p < 0.05 and showed a significant decrease throughout pregnancy. A rerise of endoglin and sflt-1 preceded placental abruption 4 weeks before onset of incident. Conclusion. Due to the limited long-term therapeutical possibilities for pregnancies complicated by PE, plasmapheresis seems to be a therapeutical option. This consideration refers especially to pregnancies with early-onset preeclampsia, in which, after first conventional treatment of PE, prolongation of pregnancy should be above all.

  1. TRADITIONAL AND CASCADE PLASMAPHERESIS IN ANTIBODY TITERS’ REDUCTION IN RENAL TRANSPLANT RECIPIENTS

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    A.V. Vatazin

    2014-01-01

    Full Text Available Introduction. One of the current tasks of transplantology is to overcome «graft-host» immune confl ict. Partially this confl ict is caused by the presence of circulating pre-existing antibodies. Highly sensitized patients have a greater risk of rejection and subsequent graft loss. There are several methods to remove the antibodies, one of which is a double fi ltration plasmapheresis (DFPF. This report presents our experience of DFPF in recipients of high immunologic risk.Aim: to compare the effectiveness of traditional and double filtration plasmapheresis in desensitization of patients with high risk of immunological complications.Methods. The study included 30 patients after kidney transplantation. All patients were classifi ed as high-immunologic risk group. In 15 patients of study group we performed DFPF, in 15 patients of comparison group – traditional plasmapheresis. We monitored the immune status: markers of humoral immunity activation – IgG, IgM, IgA before and after the procedures. DFPF procedure was performed on OctoNova (MeSys, Germany with a plasmafi lter and plasma components separator. Protocol biopsies were performed on days 30 and 90.Results. The concentration of antibodies may be effectively reduced with DFPF. Total IgM and IgG antibodies were reduced by 30–55% of the original level. There was a less albumin loss in case of DFPF application. There is 1 patient with antibody-mediated rejection with graft dysfunction in study group. There are no signs of rejection in 30- and 90-day biopsy in study group. But there were three patients with subclinical antibody-mediated rejection in the comparison group.Conclusion. DFPF can safely and effectively reduce the high titers of antibodies that are responsible for humoral rejection of renal allograft. Reduction of antibodies in sensitized patients immediately after transplantation may improve graft function.

  2. FEATURES OF PLASMAPHERESIS IN THE TREATMENT OF GRAFT REJECTION AFTER KIDNEY TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    A. V. Vatazin

    2015-01-01

    Full Text Available Introduction. The development of immunological confl ict in the form of host-versus-graft reaction has always been main problem in transplantation. The worst case is the development of humoral rejection with the presence of circulating immune complexes and antibodies. There are several methods for quick removal of antibodies; among those are traditional plasmapheresis (PA and double fi ltration plasmapheresis (DFPF. In this paper we present our experience with these two methods and give a comparative evaluation of the effectiveness in the treatment of acute humoral rejection in renal allograft. Aim: to compare the effectiveness of traditional and double fi ltration plasmapheresis while processing different volumes of plasma in the treatment of host-versus-graft disease after kidney transplantation.Methods. The study included 58 patients after kidney transplantation. All patients had increased activity of humoral immunity, which was confi rmed by immunofl uorescence with luminescence C4d complement component. In 26 patients we performed DFPF, in 32 patients – traditional PA. We divided the DFPF patients into 4 subgroups depending on the amount of processed plasma: > 50% (5 patients, 50–100% (8 patients, 100–150% (7 patients, 150–200% (6 patients of circulating plasma volume. We also divided PA patients into four subgroups depending on the volume of plasma removed: >50% (8 patients, 50–70% (12 patients, 70–90% (7 patients, 90–110% (5 patients of the volume of circulating plasma. We monitored the immune status with markers of humoral immunity activation IgM, IgG before and after each of the procedures.Results. Each procedure of traditional PA and DFPF was accompanied by a marked decrease in blood concentrations of IgM and IgG antibodies. Their level decreased by an average of 30–55% of the original. However, some patients in both groups showed an increase in the concentration of these immunoglobulins in 1–2 days

  3. [Myocardiopathy diagnosed in utero in a mother with SS-A antibodies treated with plasmapheresis].

    Science.gov (United States)

    Arroyave, C M; Puente Ledezma, F; Montiel Amoroso, G; Martínez García, A C

    1995-03-01

    We report a 36 years old patient with Sjogren's syndrome, who during her second pregnancy, the product developed a miocardiopathy with complete heart block that was diagnosed in utero at 26 weeks of pregnancy. Simultaneously, laboratory data reported a SS-A/Ro titer of 1:50,000 with positive antiphospholipids antibodies. Patient was subjected three times to plasmapheresis with three blood volume exchange each time. During the procedures, we had monitor the product and no hemodinamic changes were observed. Unfortunately, 25 days later the patient reported absence of fetal movement and by ecosonography and Doppler was not observed fetal movement or cardiac function. This pregnancy ends in cesarea. The patient is in perfect clinical conditions under control using prednisone and methotrexate.

  4. The beneficial effect of plasmapheresis in mixed connective tissue disease with coexisting antiphospholipid syndrome.

    Science.gov (United States)

    Szodoray, P; Hajas, A; Toth, L; Szakall, S; Nakken, B; Soltesz, P; Bodolay, E

    2014-09-01

    The authors report a rare case of a female patient with mixed connective tissue disease (MCTD) with coexisting antiphospholipid syndrome (APS). Five years after the diagnosis of MCTD high concentrations of anticardiolipin (anti-CL) and anti-β2-glycoprotein (anti-β2GPI) autoantibodies were present in the patient's serum without thrombotic events. Epstein-Barr virus (EBV) reactivation provoked APS, with the clinical manifestations of livedo reticularis, digital gangrene and leg ulcers. Skin biopsy from the necrotic area showed multiple fibrin microthrombi in the superficial vessels. Corticosteroid pulse therapy, and plasma exchange in combination with synchronized cyclophosphamide was administered, which led to improvement of the digital gangrenes, while no new lesions developed. The number of CD27high plasma cells decreased, and the previous high levels of autoantibodies also normalized in the peripheral blood. In the case of MCTD with coexisting APS combination therapy, including plasmapheresis has beneficial effects.

  5. Modeling hepatitis C virus therapies combining drugs and lectin affinity plasmapheresis.

    Science.gov (United States)

    Tullis, Richard H; Duffin, R Paul; Ichim, Thomas E; Joyce, James A; Levin, Nathan W

    2010-01-01

    Hepatitis C virus (HCV) infection can be cured by standard pegylated interferon (IFN) + ribavirin drug therapy in 30-50% of treatment-naïve genotype 1 HCV patients. Cure rate is defined as a sustained viral response measured 6 months after the end of treatment. Recently, Fujiwara et al. [Hepatol Res 2007;37:701-710], using a double-filtration plasmapheresis (DFPP) technique, showed that simple physical reduction in circulating HCV using a 1-week pretreatment increased the cure rate for treatment-naïve type 1 HCV patients from 50 (controls) to 78% (treated). For previous nonresponders, the cure rate increased from 30 to 71%. This effect occurs even though the DFPP per treatment HCV viral load reduction averaged 26%. In clinical studies discussed here, a lectin affinity plasmapheresis (LAP) device caused an estimated 41% decrease in viral load as previously reported. A more detailed analysis using normalized data to correct for any variations in initial viral load gave an average 29% per treatment viral load reduction in 5 HCV-positive dialysis patients. The latter data indicate that continuous application of LAP could bring HCV viral load to undetectable levels in 4.1 days. Compared to DFPP, the LAP approach has the advantage that no plasma losses are incurred. In addition hemopurification can be carried out for extended periods of time analogous to continuous renal replacement therapy for the treatment of acute kidney failure, making the process much more effective. Calculations based on these data predict that continuous hemopurification would substantially increase the rate of viral load reduction (approx. 14-fold) and therefore increase the cure rate for HCV standard-of-care drug therapies without adding additional drugs and their associated side effects.

  6. Acute pancreatitis induced by hypertriglyceridemia and treatment with plasmapheresis: case report = Pancreatitis aguda inducida por hipertrigliceridemia y tratamiento con plasmaféresis: reporte de un caso

    Directory of Open Access Journals (Sweden)

    Muñoz Ortiz, Édison

    2012-10-01

    Full Text Available Hypertriglyceridemia (HTG is a potential cause of acute pancreatitis (AP, especially when its values are greater than 1.000 mg/dL. Different therapeutic measures have been proposed for patients with AP secondary to HTG, including the one that seems to be more effective: plasmapheresis. We report the case of a patient with severe HTG (triglycerides 6.480 mg/dL that suffered from AP, and had favorable evolution with plasmapheresis.

  7. Life Saving Plasmapheresis for the Management of Hemolytic Crisis and Acute Liver Failure in Wilson’s Disease

    Directory of Open Access Journals (Sweden)

    Mohammad Reza Pashaei

    2009-06-01

    Full Text Available Wilson's disease, caused by a deficient cellular copper export system, is transmitted as an autosomal recessive inherited disorder and results in copper accumulation in liver and other organs, particularly in brain. Acute hepatic failure and severe Coombs' negative hemolysis may occur in the course of the disease which has a poor prognosis and most patients do not survive the crisis. Only liver transplantation has been recommended as an effective medical intervention. Herein, we presented a 25-year-old woman with impaired consciousness, acute hepatic failure and hemolysis who was treated with plasmapheresis and albumin replacement. Beside improvement in medical condition, serum copper and hemolysis decreased significantly and renal function was preserved. We concluded that plasmapheresis may be a life saving intervention during fulminant hepatic failure of Wilson's disease.

  8. Part of plasmapheresis with plasma filtration adsorption combined with continuous hemodiafiltration in the treatment of severe acute liver failure

    Science.gov (United States)

    Li, Maoqin; Wang, Zhidong; Wang, Yining; Du, Changhong; Li, Songhai; Shi, Zaixiang; Lu, Bo

    2016-01-01

    The present study is a retrospective analysis of 11 cases with severe acute liver failure combined with multiple organ dysfunction syndrome (MODS) performed during the period June, 2012 to December, 2014. After part of plasmapheresis with plasma filtration adsorption combined with continuous hemodiafiltration treatment, good curative effects were obtained and the main clinical symptoms and biochemical index were significantly improved. Following treatment, 8 of the 11 patients survived at a survival rate of 72.7%, and 3 patients succumbed with a mortality of 27.3%. The results suggested that part of plasmapheresis with plasma filtration adsorption combined with continuous venovenous hemodiafiltration (CVVHDF) treatment is beneficial in the removal of metabolites and toxins. Additonally, it can effectively improve liver function and clinical symptoms, improve hepatic encephalopathy, correct the disorder of internal environment, and improve the prognosis of patients. PMID:27698760

  9. Goodpasture syndrome: selective removal of anti-alpha 3 (IV) collagen autoantibodies. A potential therapeutic alternative to plasmapheresis.

    Science.gov (United States)

    Boutaud, A A; Kalluri, R; Kahsai, T Z; Noelken, M E; Hudson, B G

    1996-01-01

    Anti-alpha 3(IV) collagen autoantibodies have been implicated in the pathogenesis of Goodpasture syndrome, an autoimmune disorder causing glomerulonephritis and pulmonary hemorrhage. Currently treatment involves removal of the entire IgG fraction of plasma by plasmapheresis or adsorption to protein A. The present study shows that the anti-alpha 3(IV)NC1 autoantibodies can be removed from plasma specifically and quantitatively by affinity chromatography utilizing either alpha 3 NC1 domain of bovine type IV collagen or recombinant alpha 3 NC1 domain of human type IV collagen immobilized to agarose beads. This study shows the feasibility of using affinity chromatography, as an alternative to plasmapheresis, to exclusively remove the pathogenic autoantibodies from the plasma of patients with Goodpasture syndrome.

  10. A successful case of anti-NMDAR encephalitis without tumor treated with a prolonged regimen of plasmapheresis

    Directory of Open Access Journals (Sweden)

    Mateus Mistieri Simabukuro

    Full Text Available ABSTRACT Anti-NMDA receptor encephalitis is a severe but treatable autoimmune disease of the CNS. However, the use of immunotherapy and long-term outcomes have yet to be defined for this disease. We describe a case of an 18-year-old male diagnosed with anti-NMDAR encephalitis not associated with tumor, which did not respond to initial treatment with immunoglobulin, followed by corticosteroids, cyclophosphamide and evolved with significant clinical improvement after a prolonged course of plasmapheresis. Although it is not possible to affirm the good outcome was due solely to the prolonged plasmapheresis regimen, recently published data shows that improvement may take weeks or months to occur. This case discloses another therapeutic possibility for patients with refractory disease who fail to respond to recommended first-line and second-line therapy.

  11. Plasmapheresis for refractory status epilepticus, part I: A scoping systematic review of the adult literature.

    Science.gov (United States)

    Zeiler, F A; Matuszczak, M; Teitelbaum, J; Kazina, C J; Gillman, L M

    2016-12-01

    Our goal was to perform a scoping systematic review of the literature on the use of plasmapheresis or plasma exchange (PE) for refractory status epilepticus (RSE) in adults. Articles from MEDLINE, BIOSIS, EMBASE, Global Health, Healthstar, Scopus, Cochrane Library, the International Clinical Trials Registry Platform, clinicaltrials.gov (inception to May 2016), reference lists of relevant articles, and gray literature were searched. The strength of evidence was adjudicated using both the Oxford and GRADE methodology by two independent reviewers. Twenty-two original articles were identified. Twenty-seven adult patients were described in these articles, with a variety of autoimmune conditions leading to RSE. Seizure response with the application of PE therapy occurred in 14 of the 27 patients (51.9%), with 1 (3.7%) and 13 (48.1%) displaying partial and complete responses respectively. Generalized RSE was the most likely seizure subtype to respond to PE therapy. One patient had recorded an adverse events related to PE therapy. Oxford level 4, GRADE D evidence exists to suggest an uncertain response of adult autoimmune RSE to PE therapy. Thus, the routine application of PE therapy for adult autoimmune RSE cannot be recommended at this time. Copyright © 2016 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  12. Evidence of relative iron deficiency in platelet- and plasma-pheresis donors correlates with donation frequency.

    Science.gov (United States)

    Li, Huihui; Condon, Frances; Kessler, Debra; Nandi, Vijay; Rebosa, Mark; Westerman, Mark; Shaz, Beth H; Ginzburg, Yelena

    2016-12-01

    The loss of iron stores and resulting iron deficiency is well documented in whole blood or red blood cell donors. We hypothesized that relative iron deficiency also occurs as a result of more frequent platelet- and plasma-pheresis (apheresis) donation. To test this hypothesis, we proposed a pilot cross-sectional study to analyze erythropoiesis- and iron-related parameters in white male apheresis donors: (1) relative to controls, (2) in correlation with apheresis donation frequency, and (3) in correlation with pre-donation platelet count. Fifty eligible apheresis donors and eight controls were enrolled in the study. Apheresis donors were found to have a lower serum ferritin and serum hepcidin and exhibited evidence of iron restricted erythropoiesis relative to controls. Furthermore, among donors, lower MCV, CH(r) , hepcidin concentration, and serum ferritin were observed in more frequent apheresis donors. Correlations between donation frequency and hepcidin and ferritin were noted in apheresis donors. This pilot study demonstrates that apheresis donors are relatively iron deficient compared to controls and supports the premise that frequent apheresis donation correlates with relatively iron restricted erythropoiesis. An analysis of iron- and erythropoiesis-related parameters in a broader population of frequent apheresis donors (i.e., female and non-white donors) may demonstrate larger deficits and an even greater potential benefit of iron replacement. J. Clin. Apheresis 31:551-558, 2016. © 2015 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  13. Emergency Plasmapheresis in a case of ThromboticThrombocytopenic Purpura (TTP

    Directory of Open Access Journals (Sweden)

    Mariaserena Pioli Di Marco

    2013-12-01

    Full Text Available An 84 year-old female was admitted to our Department of Vascular Internal Medicine after a sudden onset of weakness on her right side and aphasia along with signs of myocardial ischemia from Electrocardiogram (EKG. Clinical and blood exams led to a suspicion of Moschcowitz syndrome, which was reinforced by the presence of numerous schistocytes on a peripheral blood smear.Due to a rapid deterioration of vital signs as well as alertness, the patient underwent an emergency transfusion and plasmapheresis treatment as recommended by American Society of Apheresis (ASFA guidelines: one plasma volume was replaced with fresh frozen plasma (FFP every 24 hours, for the first eight days, in order to reach at least a level of 150,000 platelets/mm3 over three consecutive days accompanied by a decrease in LDH until to 670 UI/l.After this therapy, the clinical picture significantly improved with a complete recovery of consciousness and the disappearance of neurological defects.Examinations to determine the etiology made us hypothesize a secondary status of thrombotic thrombocytopenic purpura due to an autoimmune disorder compatible with Sjogren’s syndrome. The patient was discharged and prescribed prednisone.Currently the patient is in good clinical condition and continues the therapy with prednisone (5 mg/die.

  14. Steroid unresponsive anti-NMDA receptor encephalitis during pregnancy successfully treated with plasmapheresis.

    Science.gov (United States)

    Shahani, Lokesh

    2015-04-29

    Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is an autoimmune disorder resulting in neurological and psychiatric symptoms. It is rare during pregnancy and treatment is extremely challenging as little data exist to guide management. A 26-year-old woman presented at 22 weeks of gestation with intermittent headache and an acute episode of bizarre behaviour and grandiose delusions resulting in hospitalisation. The patient was worked up for encephalitis and was found to have anti-NMDA receptor antibody in cerebrospinal fluid as well as in serum. She was initially treated with high-dose steroids but failed to improve clinically and serologically. She was then treated with plasmapheresis and showed clinical and serological response. She had a successful delivery at 37 weeks and the baby did not show serological evidence of disease. This case adds to the sparse literature of anti-NMDA receptor encephalitis during pregnancy and adds to the differential diagnosis of new onset psychiatric symptoms during pregnancy.

  15. Plasmapheresis reverses all side-effects of a cisplatin overdose – a case report and treatment recommendation

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    Ploner Ferdinand

    2006-01-01

    Full Text Available Abstract Background Cisplatin is widely used as an antineoplastic agent since it is effective against a broad spectrum of different tumours. Nevertheless, it has several potential side effects affecting different organ systems and an overdose may lead to life-threatening complications and even death. Case presentation We report on a 46-year old woman with non-small cell lung cancer who accidentally received 225 mg/m2 of cisplatin, which was threefold the dose as scheduled, within a 3-day period. Two days later, the patient presented with hearing loss, severe nausea and vomiting, acute renal failure as well as elevated liver enzymes. In addition, she developed a severe myelodepression. After plasmapheresis on two consecutive days and vigorous supportive treatment, the toxicity-related symptoms improved and the patient recovered without any sequelae. Conclusion To date, no general accepted guidelines for the treatment of cisplatin overdoses are available. Along with the experience from other published cases, our report shows that plasmapheresis is capable of lowering cisplatin plasma and serum levels efficiently. Therefore, plasma exchange performed as soon as possible can ameliorate all side effects of a cisplatin overdose and be a potential tool for clinicians for treatment. However, additional intensive supportive treatment-modalities are necessary to control all occurring side effects.

  16. Therapeutic Plasmapheresis. A Gate to an Effective Treatment of Severe Pathological Conditions

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    Claudia Stefanutti

    2014-07-01

    pregnancy do not exist (6. A further complication of the apheresis issue is represented by the indication of sepsis with multi organ dysfunction syndrome (MODS as the most common cause of death in patients in non-coronary intensive care units. Presently, an effective treatment to reduce mortality in sepsis and MODS patients is still not available. Therapeutic PEX in the management of sepsis and MODS was suggested several years ago and more recently (7, 8. On the other side, even a selective LA technique such as Heparin induced Extracorporeal Lipoprotein Precipitation (H.E.L.P. was used in the treatment of sepsis and it is still on use (9. In conclusion, extracorporeal therapeutic techniques can be clinically helpful in emergency, in deferred urgency and in chronic, long-term use. As these techniques are invasive and relatively complex, appropriate equipment and highly skillful medical and non-medical staff personnel is a necessary complement.   Key words: Therapeutic Plasmapheresis, Plasma Exchange, Lipoprotein Apheresis, Hyperlipidemic Pancreatitis, Acute Pancreatitis, Homozygous- Heterozygous Familial Hypercholesterolemia, Hyper Lp(a lipoproteinemia

  17. Early primary graft failure after a pediatric heart transplant and successful rescue with plasmapheresis, immunoglobulins, and alemtuzumab

    Directory of Open Access Journals (Sweden)

    Shashi Raj

    2017-01-01

    Full Text Available Early primary graft failure after pediatric orthotopic heart transplantation (OHT has a high mortality rate and can occur due to several causes including but not limited to prolonged graft ischemia time, suboptimal preimplant myocardial preservation, hyperacute rejection, and maladaptation of the graft to the host's hemodynamic status. Mechanical circulatory support with either extracorporeal membrane oxygenation (ECMO or ventricular assist device has been used for the rescue of primary graft failure in pediatric patients after heart transplant. Cardiac arrest before ECMO initiation in these patients is associated with adverse neurologic outcome although those surviving to hospital discharge generally have excellent long-term outcome. We report a case of early primary graft failure after OHT who required ECMO support and successful rescue with plasmapheresis, immunoglobulins, and alemtuzumab.

  18. Double Filtration Plasmapheresis in Treatment of Patients With Co-Infection of Hepatitis C and Human Immunodeficiency Virus.

    Science.gov (United States)

    Mednikov, Roman V; Rabinovich, Vladislav I; Kizlo, Svetlana N; Belyakov, Nikolai A; Sokolov, Alexey A

    2016-08-01

    One of the main causes of mortality of human immunodeficiency virus (HIV)-infected patients are complications of chronic hepatitis C virus (HCV). Combining drug therapy for HCV with double filtration plasmapheresis (DFPP) has significantly increased the effectiveness of treatment for these patients. However, there are no data on the use of this method for the treatment of patients co-infected with HIV and HCV. We demonstrated that positive clinical effect in the treatment of HCV patients by DFPP (previously demonstrated) is also achieved in the treatment of HIV infected patients, co-infected with HCV. The obtained efficiency of 62.5% is almost two times higher than the predicted treatment efficiency. We can conclude that the complex therapy of hepatitis C, including DFPP and medication by PEG-IFN + RBV is an effective and safe approach for the treatment of HCV in patients co-infected with HCV and HIV.

  19. Plasmapheresis and corticosteroid treatment for persistent jaundice after successful drainage of common bile duct stones by endoscopic retrograde cholangiography

    Institute of Scientific and Technical Information of China (English)

    Ulku Saritas; Bunyamin Aydin; Yucel Ustundag

    2007-01-01

    Prolonged cholestasis is a very rare complication of endoscopic retrograde cholangiography (ERC). Only few cases with this complication are reported in the English literature. We report persisting cholestatic jaundice in a 73-year old man after successful therapeutic ERC for choledocholithiasis. Serologic tests for viral and autoimmune hepatitis were all negative. A second-look ERC was normal also. He denied any medication except for prophylaxis given intravenous 1 g ceftriaxon prior to the ERC procedure.After an unsuccessful trial with ursodeoxycholic acid and cholestyramine for 2 wk, this case was efficiently treated with corticosteroids and plasmapheresis. His cholestatic enzymes became normal and intense pruritis quickly resolved after this treatment which lasted during his follow-up period. We discussed the possible mechanisms and treatment alternatives of intrahepatic cholestasis associated with the ERC procedure.

  20. Recurrent Proliferative Glomerulonephritis With Monoclonal IgG Deposits After a Renal Transplant Which Was Insensitive to Pulse Therapy Remitted by Double Filtration Plasmapheresis.

    Science.gov (United States)

    Wu, Di; Chen, Jin-Song; Cheng, Dong-Rui; Chen, Hao; Li, Xue; Ji, Shu-Ming; Xie, Ke-Nan; Ni, Xue-Feng; Liu, Zhi-Hong; Wen, Ji-Qiu

    2015-10-01

    Proliferative glomerulonephritis with monoclonal IgG deposits manifesting as a nephrotic syndrome recently has been described as a renal disease with the pathological features of mesangial and subendothelial deposits of monoclonal IgG. Eight cases of recurrent proliferative glomerulonephritis with monoclonal IgG deposits after a renal transplant have been reported. Almost all of these patients had a certain remission of proteinuria by steroids alone or with cyclophosphamide, and had further remission through other special treatments (ie, rituximab and plasmapheresis). We present a case of recurrent proliferative glomerulonephritis with monoclonal IgG deposits of the IgG3? subtype after a renal transplant, which was insensitive to pulse intravenous methyl-prednisolone and cyclophosphamide remitted by double filtration plasmapheresis. This case report reveals that recurrent proliferative glomerulo-nephritis with monoclonal IgG deposits may be insensitive to intravenous pulse therapy of methylprednisolone and cyclophosphamide. We advocate double filtration plasmapheresis as an effective treatment of proliferative glomerulo-nephritis with monoclonal IgG deposits on remission of proteinuria.

  1. Plasmapheresis as preparatory method for thymectomy in myasthenia gravis Plasmaferese como método preparatório de timectomia em miastenia grave

    Directory of Open Access Journals (Sweden)

    José Carlos Brant Seggia

    1995-09-01

    Full Text Available To study the effects of plasmapheresis in preparation for thymectomy, two groups of 40 patients were selected from a sample of 286 patients with myasthenia gravis examined by the first author Group 1 included patients (15 male and 25 female; age range 8-64 yrs who underwent thymectomy without previous plasmapheresis, whereas patients in group 2 (17 male and 23 female; age range 11-61 yrs were thymectomized after plasmapheresis. We required patients to have a minimum follow-up period of 12 months to be included in the study. A clinical evaluation protocol composed of 76 items was developed for the study. We found significant improvement in respiratory function and muscular strength in patients thymetomized after plasmapheresis. Furthermore, the combined treatment reduced cost and length of hospital stay. Therefore, we conclude that plasmapheresis should be considered as a coadjuvant to thymectomy in the treatment of myasthenia gravis.No intuito de estudar as repercussões de um curso de plasmaferese como método preparatório para a timectomia, tomamos duas amostras de 40 pacientes selecionados de um universo de 286 casos de miastenia gravis, em que a investigação clínica, eletrofisiológica, laboratorial, o seguimento e o protocolo foram completamente realizados. Consideramos como grupo 1 aquele em que a timectomia foi realizada sem preparação pre-operatória e grupo 2 aquele em que a plasmaferese foi executada como coadjuvante a timectomia. O grupo 1 era composto de 15 pacientes do sexo masculino e 25 do feminino; a idade variou de 8 a 64 anos com mediana de 30 anos. No segundo grupo, 17 eram masculinos e 23 femininos; a idade variou de 11 a 61 anos com mediana de 33 anos. Foi preenchida ficha de avaliação clínica sequencial de 76 itens e traçamos um curso de plasmaferese de 5 sessões seguido da timectomia, mantendo a mesma técnica operatória, realizada pela mesma equipe cirúrgica, tentando assim minimizar as distorções na an

  2. The natural course of hepatitis C virus infection 18 years after an epidemic outbreak of non-A, non-B hepatitis in a plasmapheresis centre.

    Science.gov (United States)

    Datz, C; Cramp, M; Haas, T; Dietze, O; Nitschko, H; Froesner, G; Muss, N; Sandhofer, F; Vogel, W

    1999-04-01

    The natural history of hepatitis C virus (HCV) infection is variable and factors determining the course of the illness are unclear. To determine the natural course of HCV infection in a well characterised group of patients 18 years after an epidemic outbreak of non-A, non-B hepatitis at a plasmapheresis centre. Between 1994 and 1996, 20 of 30 affected individuals were studied. HCV infection was confirmed using second and third generation ELISA test kits. HCV RNA was detected by a polymerase chain reaction (PCR) method and HCV genotyping was performed by analysing amplicons from the conserved 5'-non-translated region generated by nested PCR. Thirty two liver biopsies were carried out in 14 patients. HCV antibodies were detected in all subjects. Eighteen patients had abnormal liver enzymes and 17 were HCV RNA positive, all of whom were infected with genotype 1a. Ninety per cent of this cohort showed evidence of chronic HCV infection with 50% having progressive liver disease and 20% cirrhosis 18 years after acute onset of non-A, non-B hepatitis. Considerable variation in disease outcome occurred between individuals and no correlation with clinical features of the acute illness was found. Variability in the consequences of HCV infection in cases infected with the same virus suggests that host factors are important in determining disease outcome. The factors which determine differences in the natural history of the disease still remain to be elucidated.

  3. Desensitization with plasmapheresis and anti-Cd20 for ABO incompatible kidney transplantation from living donor: experience of a single center in Italy.

    Science.gov (United States)

    Silvestre, C; Furian, L; Marson, P; Tison, T; Valente, M; Marchini, F; Rossi, B; Bonfante, L; Valerio, F; Cozzi, E; Rigotti, P

    2014-09-01

    Blood group incompatibility in kidney transplants from a living donor can be successfully overcome by using various desensitization protocols: intravenous immunoglobulin, plasmapheresis (PP), immunoadsorption, and double filtration PP. From July 2010 to October 2013, we performed 10 ABO incompatible kidney transplantation (KT) procedures from a living donor. The desensitization protocol was based on rituximab and PP+cytomegalovirus immune globulin. All patients received induction with basiliximab, except 1 case treated with Thymoglobuline® (ATG) for the simultaneous presence of donor-specific antibody. Tacrolimus and mycophenolate mofetil were initiated at the time of desensitization and continued after the transplant. After a mean follow-up of 11.6±10.4 months, all patients are alive with a functioning graft. The mean serum creatinine concentration at 1 month, 3 months, 6 months, and 1 year was 1.48±0.29, 1.47±0.18, 1.47±0.27, and 1.5±0.27 mg/dl. Three episodes of acute cellular rejection occurred in 2 patients. There was only 1 case of BK virus infection, treated with reduction of immunosuppressive therapy. The protocol biopsy specimens at 1, 3, and 6 months were C4d positive in the absence of acute rejection. Desensitization with rituximab, PP, and anti-cytomegalovirus immune globulin allowed us to perform transplants from living donors to ABO incompatible recipients with excellent results and reduced costs. Copyright © 2014 Elsevier Inc. All rights reserved.

  4. A combination therapy protocol of plasmapheresis, intravenous immunoglobulins and betamethasone to treat anti-Ro/La-related congenital atrioventricular block. A case series and review of the literature.

    Science.gov (United States)

    Ruffatti, Amelia; Marson, Piero; Svaluto-Moreolo, Giorgio; Marozio, Luca; Tibaldi, Maria; Favaro, Maria; Calligaro, Antonia; Grava, Chiara; Hoxha, Ariela; Pengo, Vittorio; Punzi, Leonardo

    2013-05-01

    The aim of this report was to evaluate the efficacy and safety of a combined treatment protocol used to treat 2nd and 3rd degree anti-Ro/La-related congenital atrioventricular block (CAVB). Six consecutive women diagnosed with 2nd degree (three cases) or 3rd degree block (three cases) between 2009 and 2011 referred to our outpatient clinic underwent a combination therapy protocol composed of weekly plasmapheresis, fortnightly 1g/kg intravenous immunoglobulins (IVIG) and daily betamethasone (4mg/day) throughout pregnancy. IVIG (1g/kg) treatment in the neonates was begun at birth and administered every fifteen days until passive maternal antibodies became undetectable. The fetuses affected with 2nd degree block (cases 1, 2 and 3) reverted to a normal atrioventricular conduction after combined therapy, while those with a 3rd degree block remained stable (case 4), showed an increase in the ventricular rate (case 5) or an improvement in cardiac function (case 6). None of the fetuses with 3rd degree CAVB had other cardiac complications such as cardiomyopathy or fetal hydrops. The follow-up of the children affected with 2nd degree CAVB revealed a complete regression of the block in cases 1 and 3, and no signs of relevant worsening in case 2. The infants affected with 3rd degree block (cases 4, 5, and 6) remained stable and until now only one has required a pacemaker at the age of 10months. If these results are confirmed by large-scale studies, this protocol could lead to improved outcomes in the treatment of this devastating disease. Copyright © 2013 Elsevier B.V. All rights reserved.

  5. Study on infection of human immunodeficiency virus among cases of plasmapheresis-borne malaria%单采血浆相关性疟疾合并艾滋病病毒感染的研究

    Institute of Scientific and Technical Information of China (English)

    陈素良; 赵宏儒; 张玉琪; 赵翠英; 李保军; 白广义; 梁良; 陈志强

    2012-01-01

    目的 了解单采血浆相关性疟疾病例中,艾滋病病毒(HIV)感染情况.方法 对1993-2005年间单采血浆相关性疟疾病例和非疟疾既往单采血浆供血者进行调查和采集血标本,采用快速蛋白印迹试验或酶联免疫吸附试验检测HIV抗体,初筛阳性者再用蛋白印迹试验进行确认;对现症疟疾病例的血标本用吉氏液染色-光学显微镜(油镜)检查疟原虫.结果 220例单采血浆相关性疟疾病例的HIV感染率为30.0%(66/220),3 008例非疟疾单采血浆供血者的HIV感染率为2.4% (72/3 008).1993年12月和1994年1月采集并检测的上述两人群,HIV抗体均为阴性;而1995年3月至2005年采集并检测的上述两人群,HIV阳性率则分别为43.4%(66/152)和2.4%(72/2 958).单采血浆相关性疟疾为间日疟,感染的HIV为HIV-1型.结论 单采血浆相关性疟疾病例的HIV感染率较高.%Objective To understand characteristics of human immunodeficiency virus (HIV) infection among cases of plasmapheresis -borne malaria. Methods Two hundred and twenty cases of plasmapheresis-borne malaria occurred between 1993 and March 1995, and 3 008 former plamapheresis donors were investigated and their blood specimens were collected. Staining of giemsa-optical microscope (oil lens) inspection method was used to detect plasmodium. HIV antibodies were detected by the screening assay of ELISA and confirming assay of Western blot. Results The rates of HIV infection was 30. 0%(66/220)for cases of plasmapheresis-borne malaria and 2. 4%(72/3 008) for plasmapheresis donors without history of malaria. Sera samples taken from the above two population groups in December 1993 and January 1994 were negative for HIV antibody. Moreover, positive rate of HIV antibody of sera taken in March 1995 to 2005 were 43. 42% (66/152)and 2. 43%(72/2 958) respectively. The type of plasmapheresis-borne malaria was tertian malaria and that of the infected virus was HIV-1. Conclusion The infection

  6. 双重滤过血浆置换抢救吉兰-巴雷综合征一例%Double filtration plasmapheresis rescues a case of Guillain-Barré syndrome

    Institute of Scientific and Technical Information of China (English)

    蒋欣欣; 冯剑; 胡卫民; 陆明晰; 屠春艳; 王本勇; 叶有新

    2010-01-01

    @@ 体液免疫在吉兰-巴雷综合征(Guillain-Barré syndrome,GBS)中起着重要作用[1].肾上腺皮质激素无法阻止其病情进展,但是清除患者体内的致病物质可以达到治疗目的[2].我们试用双重滤过血浆置换(double-filtration plasmapheresis,DFPP)抢救吉兰.巴雷综合征1例,效果良好,报道如下.

  7. Asparaginase-associated concurrence of hyperlipidemia, hyperglobulinemia, and thrombocytosis was successfully treated by centrifuge/membrane hybrid double-filtration plasmapheresis.

    Science.gov (United States)

    Wang, Taina; Xu, Bin; Fan, Rong; Liu, Zhihong; Gong, Dehua

    2016-01-01

    Asparaginase-associated concurrence of hyperlipidemia, hyperglobulinemia, and thrombocytosis is a rare complication requiring aggressive lipoprotein apheresis, but no one of currently available lipoprotein apheresis methods can simultaneously resolve the 3 abnormalities. Herein, we reported a construction of double-filtration plasmapheresis (DFPP) using a combination of centrifugal/membranous plasma separation techniques to successfully treat a patient with hyperlipidemia, hyperglobulinemia, and thrombocytosis. A male presented with severe hyperlipidemia, hyperglobulinemia, and thrombocytosis during asparaginase treatment for NK/T-cell lymphoblastic lymphoma and was scheduled to receive lipoprotein apheresis. To simultaneously remove lipoproteins, immunoglobulin, and deplete platelets from blood, a centrifuge/membrane hybrid DFPP was constructed as following steps: plasma and part of platelets were separated first from whole blood by centrifugal technique and then divided by a fraction plasma separator into 2 parts: platelets and plasma components with large size, which were discarded; and those containing albumin, which were returned to blood with a supplement of extrinsic albumin solution. DFPP lasted 240 minutes uneventfully, processing 5450-mL plasma. The concentrations of plasma components before DFPP were as follows: triglycerides 38.22 mmol/L, total cholesterols 22.98 mmol/L, immunoglobulin A (IgA) 15.7 g/L, IgG 12.7 g/L, and IgM 14.3 g/L; whereas after treatment were 5.69 mmol/L, 2.38 mmol/L, 2.5 g/L, 7.7 g/L, and 0.4 g/L, respectively. The respective reduction ratio was 85.1%, 89.6%, 83.9%, 39.4%, and 96.9%. Platelet count decreased by 40.4% (from 612 × 10(9)/L to 365 × 10(9)/L). Centrifuge/membrane hybrid DFPP can simultaneously remove lipoproteins, immunoglobulin, and deplete platelets, with a success in treatment of asparaginase treatment-induced hyperlipidemia, hyperglobulinemia, and thrombocytosis, and may be useful for patients

  8. Effects of Different Plasmapheresis Supplement Timing on Therapeutic Efficacy of Toxic Liver Injury%血浆置换不同补充时机对药物性肝损伤疗效的影响

    Institute of Scientific and Technical Information of China (English)

    秦海珍; 陈绪军

    2015-01-01

    目的:探讨血浆置换不同补充时机对药物性肝损伤疗效的影响.方法:96例药物性肝损伤患者,按照随机数字表法分为A、B、C组,各32例.所有患者在常规治疗基础上进行血浆置换治疗.其中,A组患者与血浆置换同步进行的替补液为100%新鲜血浆;B组患者与血浆置换同步进行的替补液为:先补充40%生理盐水,再补充60%新鲜血浆;C组患者在血浆置换至离体血液达1个体循环量的12%时,再开始补充替补液,补充顺序为先补充40%生理盐水,再补充60%新鲜血浆.观察各组患者治疗前后的临床症状及体征、肝脏功能和凝血酶原活动度.结果:采用血浆置换治疗药物性肝损伤均可获得较好的疗效,但C组患者临床症状及体征消失的时间显著短于A组和B组,B组又显著短于A组,组间比较差异均具有统计学意义(P0.05).结论:在不同时机进行血浆置换对药物性肝损伤有着不同的疗效,在血浆置换至离体血液达1个体循环量的12%时,先补充40%生理盐水,再补充60%新鲜血浆的效果较为显著.%OBJECTIVE:To explore the effect of different plasmapheresis supplement timing on therapeutic efficacy of toxic liver injury. METHODS:96 patients with toxic liver injury and divided into group A,B ,C and D with 32 cases in each group ac-cording to different plasmapheresis supplement timing. All patients received plasmapheresis supplement based on routine treatment. In group A,synchronized fluid replacement was 100% fresh plasma;in group B,synchronized fluid replacement was 40% normal saline firstly,and then 60%fresh plasma;in group C,substitute liquid was given till the in vitro blood reached 12%of circulation amount,supplement order as 40% normal saline for the first supplement,and then add 60% fresh plasma. The clinical symptoms and signs,liver function,prothrombin activity and blood biochemical indicators were observed in each group before and after treat-ment. RESULTS:Plasmapheresis

  9. 连续多次单采血浆者低密度脂蛋白及相关复合物的氧化易感性分析%The Plasma Oxidative Susceptibility of LDL, LDL Level in Long-term Successive Plasmapheresis Donors

    Institute of Scientific and Technical Information of China (English)

    牛冬梅; 吴嘉; 于瑞杰; 孙晓君; 汪俊军

    2013-01-01

    目的 探讨连续多次单采血浆者氧化低密度脂蛋白(ox-LDL)、β2-糖蛋白Ⅰ/氧化低密度脂蛋白(β2-GPI/ox-LDL)复合物的水平,并分析其体内低密度脂蛋白(LDL)的氧化易感性.方法 分别检测60例连续多次单采血浆者和60例初次血浆单采者的ox-LDL、β2-GPI/ox-LDL复合物的水平,同时分析其LDL氧化易感性的变化.结果 与初次血浆单采者相比,连续多次单采血浆者的血浆ox-LDL、β2-GPI/ox-LDL复合物水平的差异均无统计学意义,其LDL氧化延滞时间延长、氧化速率及总氧化值下降,但差异均无统计学意义.结论 连续多次的血浆单采对体内ox-LDL、β2-GPI/ox-LDL复合物的水平以及LDL氧化易感性均无明显影响,不会增加其心血管疾病的风险.%Objectives To investigate the changes of plasma oxidized LDL (ox-LDL), β2-glycoprotein I with ox-LDL (β2-GPI/ox-LDL) levels and low density lipoprotein (LDL) oxidative susceptibility in long-term intensive plasmapheresis donors. Methods The plasma levels of ox-LDL and β2-GPI/ox-LDL were determined in 60 intensive plasmapheresis donors and 60 first-time donors, and their plasma susceptibility of LDL to in vitro oxidation was also measured. Results No significant differences were found in plasma ox-LDL and β2-GPI/ox-LDL levels between the individuals undergoing long-term intensive plasmapheresis and the first-time donors. The lag time, maximal oxidation rate and total amount of conjugated dienes of LDL oxidizability were lower in intensive plasmapheresis donors, but the changes were not significant statistically. Conclusions Long-term intensive donor plasmapheresis has no statistcal effect on the plasma LDL oxidative susceptibility, ox-LDL and β2-GPI/ox-LDL levels, meanwhile successive plasma demation will not result in increasing of cardiovascular risk.

  10. 血浆置换-血液滤过序贯救治重症毒蘑菇中毒的疗效观察与护理%Observation of the curative effect and nursing of the plasmapheresis-hemofiltration sequential treatment in curing severe poisonous mushroom poisoning

    Institute of Scientific and Technical Information of China (English)

    李鑫; 肖向莉; 张欣; 王丽; 佘兴红

    2016-01-01

    目的::了解血浆置换-血液滤过序贯用于救治重症毒蘑菇中毒及肝、肾等多脏器功能损伤的临床疗效与护理措施。方法:2010年1月~2014年10月对我院收治的11例重症毒蘑菇中毒患儿在常规治疗的同时,采取血浆置换-血液滤过序贯治疗并实施护理。结果:1例死亡,2例因经济原因自动出院,其余8例均治愈。结论:使用血浆置换-血液滤过序贯对救治重症毒蘑菇中毒及多系统功能损伤患者疗效显著。%Objective:To know the curative effect and nursing of the plasmapheresis-hemofiltration sequential treatment in curing severe poisonous mush-room poisoning and the functional injuries of the liver,the kidney and other viscera. Methods:Take the plasma exchange we adopted the plasmapheresis-hemofiltration sequential treatment for 11 children patient of severe poisonous mushroom poisoning received in our hospital from January 2010 to October 2014 while applying conventional therapies to them. Results:One child died,two left the hospital automatically for economic reasons; and the other eight children were all cured. Conclusion:There will be significant effect in curing severe poisonous mushroom poisoning and functional injuries of multi-systems with the plasmapheresis-hemofiltration sequential treatment.

  11. 21 CFR 640.65 - Plasmapheresis.

    Science.gov (United States)

    2010-04-01

    ... not interfere with or jeopardize the treatment of the syphilitic donor. (3) A donor identification..., blood is removed from a donor, the plasma separated from the formed elements, and at least the red blood cells returned to the donor. This procedure shall be described in detail in the biologics...

  12. Clinical effect of combination of hemoperfusion and plasmapheresis in the treatment of elderly patients with severe organophosphate poisoning%血液灌流与血浆置换联合抢救老年重度有机磷中毒的疗效

    Institute of Scientific and Technical Information of China (English)

    白刃

    2014-01-01

    目的:探究老年有机磷中毒重度型应用血浆置换联合血液灌流疗法的临床效果。方法有机磷中毒老年患者100例,按治疗方式的不同分为对照组和治疗组,每组50例。对照组进行血液灌流疗法,治疗组进行血液灌流联合血浆置换疗法,观察两组患者各项生化指标、临床疗效和并发症的情况。结果两组治疗后各项生化指标均有改善,但治疗组的指标水平明显优于对照组;治疗组的阿托品中毒、中间综合征、病死和反跳等并发症的发生率显著低于对照组,差异具有统计学意义(P<0.05)。结论血液灌流联合血浆置换抢救老年重度有机磷中毒,有效的减少了患者并发症的发生,降低了患者各项生化指标的数值,提高了患者预后的生活水平,值得在临床上推广。%ObjectiveTo discuss the clinical effect of combination of hemoperfusion and plasmapheresis in the treatment of elderly patients with severe organophosphate poisoning.MethodsDivided 100 elderly patients with severe organophosphate poisoning into control group and treatment group according to different treatment methods, each with 50 cases. Control group was given hemoperfusion therapy while treatment group was given combined therapy of hemoperfusion and plasmapheresis. Biochemical indicators, clinical effects and complications of two groups were observed.ResultsBiochemical indicators of both groups improved after treatment, but indicator levels of treatment group were obviously higher than control group; occurrence rates of atropinismus, intermediate syndrome, fatality and rebound were obviously lower than control group(P<0.05). ConclusionCombination of hemoperfusion and plasmapheresis for rescue of elderly patients with severe organophosphate poisoning effectively reduces occurrences of complications, decreases values of biochemical indicators and improves prognostic life levels of patients, so it is worthy of clinical

  13. 血浆净化治疗格林-巴利综合征临床疗效分析%The clinical effect of plasmapheresis for Guillain-Barre syndrome

    Institute of Scientific and Technical Information of China (English)

    傅芳婷; 张凌; 卞维静; 焦劲松

    2006-01-01

    目的观察血浆净化(plasmapheresis,PP)在格林-巴利综合征(Guillain-Barre syndrome)(GBS)患者中的临床疗效,初步探讨不同净化方法疗效的比较.方法回顾1998~2004年6年间在中日友好医院神经内科住院的GBS患者38例的临床资料,所有病例均符合GBS诊断标准.PP治疗组23例,男18例,女5例,年龄15~76岁,平均(39.95±16.66)岁;非PP治疗组15例,男11例,女4例,年龄20~70岁,平均(44.07±14.70)岁.两组患者按Hughes分类进行分级.每次处理血浆量为1~2个血浆容量.观察治疗前后临床症状及肌力的改善程度,减少1个Hughes等级为GBS临床改善.结果疗效及转归:PP组患者进步1级的日数平均为(10.83±9.38)天,而非PP组为(32.13±11.30)天,P<0.01.呼吸机辅助呼吸者,PP组持续7天,而非PP组则持续18天.PP组死亡1例,非PP组无死亡.PP组23例中,单重血浆置换(PE)16例,双重血浆置换(DFPP)4例,血浆吸附(PA)3例;16例PE者进步1级的平均日数为11.44天; 4例DFPP者进步1级的平均日数为7.25天;3例PA者进步1级的平均日数为12.33天.并发症:PP治疗84例次,发生不良反应13例次,发生率15.5%.PE治疗57例次,9例次出现不同程度的口周及四肢麻木、荨麻疹、寒战、发热和低血压等;DFPP治疗16例次,4例次发生血压下降;PA治疗11例次,无并发症发生.结论PP治疗能够迅速缓解GBS患者的临床症状,可显著缩短病程以及使用人工呼吸的间期,改善患者预后.不同方法并发症的发生明显不同,PE治疗并发症较多,DFPP和PA治疗并发症少.治疗方法上尽量寻求效果相当、并发症少而且费用较低者.

  14. Combination therapy of double filtration plasmapheresis and glucocorticoid in severe lupus nephritis%双重血浆置换联合激素治疗重症狼疮性肾炎的临床观察

    Institute of Scientific and Technical Information of China (English)

    章海涛; 刘正钊; 胡伟新; 陈樱花; 龚德华; 季大玺; 刘志红

    2013-01-01

    Objective:To investigate the clinical efficacy and safety of double filtration plasmapheresis (DFPP)accompanied with corticosteroid in patients with severe lupus nephritis (LN).Methodology:Thirty one patients (19 females and 12 males,average age of 24.4 ± 10.9 y) with severe LN including class Ⅳ (n =19) 、Ⅲ(n =1) 、Ⅴ + Ⅳ(n =8) 、Ⅴ + Ⅲ(n =2) and Ⅴ (n =1) were enrolled in this study.Among them,22 cases showed rapidly progressive glomerulonephritis(RPGN) with elevated serum creatinine(SCr) (300.6 ± 185.6 mmol/L),and 11 of them needed renal replacement therapy.DFPP was performed with two-fold plasma volume on each session using membrane type plasma component separator (EC50W and EC20W,Asahi Kasei Kuraray,Japan).Results:(1)Clinical efficacy:After DFPP treating 2.6 times(1 ~3)for each patient,their SLE-DAI was reduced significantly from (16.0 ± 3.5) to (9.7 ± 1.5) (P < 0.01) and SCr was decreased from (300.6 ± 185.6) mmol/L to (247.5 ±168.0) mmol/L (P <0.05).Six patients got off dialysis respectively.Gross hematuria disappeared in 4 patients and there was no change in proteinuria.(2) Immunologic parameters:After DFPP,the levels of serum IgG were decreased from (10.9 ±5.2) g/L to (4.6 2.0) g/L (P <0.01),and the titers of anti-dsDNA antibody were significantly declined,while the levels of complements and lymphcytes had no change.(3)Follow up:31 patients were followed up for 1 ~ 29 months (10.1 ±6.4).6 patients achieved complete remission,18 had partial remission and 6 developed to ESRD with maintenance hemodialysis.(4)Adverse effect:one had catheter infection and one had hypotension episodes during DFPP treatment.Conclusion:DFPP could decrease activity of SLE and improve renal function through rapidly and effectively clearance of antibodies in severe LN.So DFPP accompanied with corticosteroid is an effective therapeutic method for severe LN.However,the right indication,suitable course and dosage of DFPP will need to deeply investigate.%

  15. Anestesia em paciente obstétrica portadora de anemia falciforme e traço talassêmico após plasmaféresis: relato de caso Anesthesia in obstetric patient with sickle cell anemia and thalassemic trait after plasmapheresis: case report

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    Eduardo Barbosa Leão

    2005-06-01

    encaminada a la UTI, bajo intubación orotraqueal, y en uso de drogas vasoactivas, habiendo sido extubada después de 3 horas. CONCLUSIONES: Este caso se mostró un desafío para el equipo, ya que la paciente presentaba inestabilidad hemodinámica y alteración del coagulograma, condiciones que contraindican la anestesia regional, además de esto, la plasmaféresis potencialmente depleta las existencias de colinesterasas plasmáticas, lo que interfiere en la anestesia. Mientras, el arsenal medicamentoso disponible, permitió el manoseo seguro de esta situación.BACKGROUND AND OBJECTIVES: Plasmapheresis is the technique of choice for severe hemolytic anemia patients. A consequence is plasma cholinesterase depletion, which interferes with metabolism of some neuromuscular blockers currently used in anesthesiology. CASE REPORT: Pregnant patient, 26 years old, physical status ASA IV, 30 weeks and 3 days gestational age, with sickle cell anemia, thalassemic trait and allo-immunization for high frequency antigens. Patient presented sickling crisis being transfused with incompatible blood. Patient evolved with massive hemolysis being admitted with 3 g/dL hemoglobin and 10% hematocrit, severe jaundice, tachycardia, apathic and pale. Hematological evaluation has concluded for the inexistence of compatible blood for transfusion. Patient was treated with steroids, immunoglobulins and plasmapheresis. In the second admission day patient evolved with acute renal failure and pulmonary edema, general state worsening and hemodynamic instability. Gestation resolution was indicated due to patient's clinical conditions and consequent acute fetal suffering. Patient was admitted to the operating room conscious, pale, with dyspnea, jaundice, 91% SpO2 in room air, heart rate of 110 bpm and blood pressure of 110 x 70 mmHg, under dopamine (1 µg.kg-1.min-1 and dobutamine (10 µg.kg-1.min-1. We decided for balanced general anesthesia with alfentanil (2.5 mg, etomidate (14 mg, atracurium (35 mg and

  16. A preliminary study of double filtration plasmapheresis in ANCA associated vasculitis with severe renal involvement%双重血浆置换治疗抗中性粒细胞胞质抗体相关血管炎严重肾损害的临床疗效

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    陈樱花; 龚德华; 刘正钊; 章海涛; 刘志红; 胡伟新

    2012-01-01

    Objective: To investigate the clinical efficacy of double filtration plasmapheresis ( DFPP) in antineutrophil cytoplasmic autoantibody ( ANCA) associated vasculitis ( AAV) with severe renal involvement. Methodology: Twelve patients ( female 5, male 7, age 43 ~ 72 years ) with new onset and active AAV were included this study. BVAS scores were (14. 7 ±3. 2). 10 patients had serum positive P/MPO-ANCA,the others had serum positive C/ PR3-ANCA,and the average levels of ANCA were (275 ± 33. 9) RU/ml. On admission, they had a mean SCr of (549 ± 197)μmol/L and all of them needed renal replacement therapy. Every case had renal biopsy,and histological patterns were classified into focal ( ≥50% normal glomeruli, n = 1),crescentic (≥50% glomeruli with cellular crescents,n =5),mixed ( <50% crescentic and < 50% globally sclerotic glomeruli,n = 3 ) and sclerotic (≥50% globally sclerotic glomeruli,n = 3 ). All of them were received DFPP treatment as well as methylprednisolone pulse therapy followed by oral prednisone and mycophenloate mofetil ( MMF) or intravenous cyclophosphamide pulse therapy. Double volumes of plasma were processedduring each DFPP session every other day. EC50W and EC20W ( Asahi Kasei Corporation, Japan) were used as first filter for plasma separation and second filter for plasma fractionation, respectively. The changes of ANCA and renal function were investigated. Results; All patients were treated with DFPP for 3 ~5 times. At the end of DFPP,the serum level of MPO-ANCA from [ (275 ±33. 9) to (1 112 ±55. 1) RU/ml,P <0. 01 ] was lower significantly, with a decrement of (60. 0 ± 18. 1)%. Ten patients (83. 3% ) were free from dialysis, while one patient (the pathologic classification was crescentic) was followed only one month have not been free from dialysis, the other one without improvement of renal function received maintenance hemodialysis. Ten patients who free from dialysis after DFPP were followed 6. 20 ± 4. 80 (1 ~ 15 months ). Among

  17. 前置胎盘患者自体血浆分离联合术中自体血回收的可行性%Feasibility of autologous plasmapheresis combined with intraoperative blood salvage during cesarean section in patients with placenta previa

    Institute of Scientific and Technical Information of China (English)

    卢子会; 周春波; 黄长顺; 严海雅

    2014-01-01

    Objective To evaluate the efficacy and safety of autologous plasmapheresis combined with intraoperative blood salvage during cesarean section in patients with placenta previa. Methods Sixty patients with placenta previa scheduled for elective cesarean section, were randomly divided into three groups with 20 cases in each group. In group I, blood was with-drawn before anesthesia, to sequester the autologous plasma for re- infusion. An autotransfusion device was used to col ect and re- infuse autologous RBC during the course of the operation in both groups I and II, while an al ogenic blood transfusion was conducted in group III as necessary. Hemoglobin(Hb), hematocrit (Hct), platelet count (Plt), prothrombin time (PT), activated par-tial thromboplastin (aPTT), fibrinogen (FIB) were detected at time points of entering operation room (T1), 10min after col ecting blood(T2), 10 min before autologous re- infusing(T3), 10 min after autologous blood re- infusing(T4), 6h after operation(T5) and 24h after operation (T6). Hemodynamic changes, fetal heart rate, Apger score, umbilical cord artery blood gas analysis, volumes of blood loss and blood transfusion of three groups were documented and compared. Results There were no significant differ-ences in hemodynamic changes, fetal heart rate, Apger score and umbilical cord artery blood gas analysis among three groups. The increase of Plt level of Group I was significantly different from Group II and Group III at T4 and T5 (P<0.05);PT level change in Group I had significant differences compared with Group II and Group III at T4 (P<0.05). The volumes of plasma transfusion in Group II and Group III had significant differences compared with Group I (P<0.05). The volumes of packed red blood cells (RBCs) transfusion in Group III had significant differences compared with Group I and Group II (P<0.05). Conclusion Autolo-gous plasmapheresis combined with intraoperative blood salvage is safe and feasible, which can reduce the al ogeneic

  18. Successful Treatment Of Homozygous Familial Hypercholesterolemia Using Cascade Filtration Plasmapheresis

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    Fatih Kardas

    2012-12-01

    Full Text Available OBJECTIVE: The aim of our study is to discuss the efficacy of low-density lipoprotein-cholesterol (LDL-C apheresis procedure using the cascade filtration system for pediatric patients with homozygous familial hypercholesterolemia (FH, and to clarify the adverse effects and difficulties. METHODS: LDL apheresis using the cascade filtration system was performed in 3 pediatric patients with homozygous FH. In total, 120 apheresis sessions were performed for all patients. RESULTS: Cascade filtration therapy significantly reduced the mean LDL-C values from 418 ± 62 mg/dl to 145 ± 43 mg/dl (p<0.05. We determined an acute mean reduction in the plasma levels of total cholesterol (57.9%, LDL cholesterol (70.8%, and high-density lipoprotein (HDL cholesterol (40.7%. Treatments were well tolerated. The most frequent clinical adverse effects were hypotension in 3 sessions (2.5%, chills/feeling cold (1.7% in 2 sessions, and nausea and vomiting in 3 sessions (2.5%. CONCLUSION: Our experience with three patients using the cascade filtration system were, good clinical outcomes, laboratory findings, safety of usage, minor adverse effects and technical problems.

  19. Plasmapheresis in hypertriglyceridemia-related pancreatitis: a case report

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    Andrea Tampieri

    2012-07-01

    Full Text Available Hypertriglyceridemia (HTG is the third most common cause of acute pancreatitis (AP, accounting for up to 7% of cases. The clinical manifestations are similar to those of AP from other causes, but it may be difficult to recognize because of confounding laboratory investigations induced by HTG, such as a falsely normal serum amylase. Prompt recognition is important to provide adequate treatment. The maintenance of blood triglyceride (TG levels below 500 mg/dl has been shown to accelerate the clinical improvement in patients with hypertriglyceridemic pancreatitis (HTGP. In many cases series apheresis was effective in reducing HTG and an early initiation is likely to be beneficial in order to prevent recurrence of AP and the development of necrotizing pancreatitis. Definitive guidelines for the treatment of HTGP and randomized trials that compare the effectiveness of apheresis with the medical therapy alone are still lacking.

  20. Plasmapheresis for the treatment of kidney diseases.

    Science.gov (United States)

    Clark, William F; Huang, Shih-Han S; Walsh, Michael W; Farah, Myriam; Hildebrand, Ainslie M; Sontrop, Jessica M

    2016-11-01

    The purpose of this review is to examine the evidence supporting the application of plasma exchange in renal disease. Our review focuses on the following 6 most common renal indications for plasma exchange based on 2014 registry data from the Canadian Apheresis Group: (i) thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome; (ii) renal transplantation, (iii) anti-neutrophil cytoplasm antibodies-associated vasculitis, (iv) cryoglobulinemia, (v) focal segmental glomerulosclerosis, and (vi) Goodpasture syndrome. The rarity of these diseases and their rapid, often fatal course mean that randomized controlled studies of plasma exchange are rarely conducted. Although evidence from an adequately powered randomized controlled trial supports the use of plasma exchange to treat thrombotic thrombocytopenic purpura, the use of plasma exchange to treat other renal diseases is only supported by observational and mechanistic studies. Larger well-designed trials are needed to clarify the potential role of plasma exchange in renal disease. Growing international collaboration will improve the quality of future studies in this area. Copyright © 2016 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  1. Remove MPO-ANCA from circulation of patients with vasculitides by a novel double-filtration plasmapheresis therapy using selective plasma separators combination%选择性血浆分离器行双重血浆置换对抗中性粒细胞胞质抗体清除的研究

    Institute of Scientific and Technical Information of China (English)

    龚德华; 季大玺; 朱冬冬; 徐斌; 崔俊; 刘志红

    2011-01-01

    Objective; To investigate the ability to remove different plasma proteins by double-filtration plasmapheresis (DFPP) using various primary separator and secondary separator combinations; based on it, to propose a novel DFPP therapy, and test its ability to eliminate MPO-ANCA from circulation of patients with vasculitides. Methodology;Fifteen patients with serum MPO-ANCA positive diagnosed as vaseulitides were enrolled and received 44 sessions DFPP. Three kinds of plasma filters were used to serve as primary and secondary filter, one plasma separator MPS07 and two plasma fraction separators EC50W, EC20W. There were three combinations; MPS07/EC50W, MPS07/ EC20W, and EC50W/EC20W combination; in each combination, the former was used as primary filter, and the latter was used as secondary filter. Double volume of plasma was processed, and 30 ~40g human albumin was supplemented during each session. One received 3 sessions DFPP using MPS07/EC50W, 9 received 27 sessions DFPP using MPS07/EC20W, and the other 5 patients received 14 sessions DFPP using EC50W/EC20W. During DFPP, waste plasma was discarded in intermittent way.When the pressure pre-secondary filter reached the threshold value to discard plasma, 800ml normalsaline was flushing the secondary filter to force filtration of plasma proteins accumulated in filter, before waste plasma was discarded. Sieving coefficients (SC) of albumin, IgA, IgG and IgM were measured for 3 filters, as well as reduction percentage of plasma proteins concentrations, MPO-ANCA titer by single session DFPP. Results: MPS07 filter was well permeable for all above-mentioned plasma proteins ( SC > 0. 6 ) ; while EC50W filter was well permeable for albumin and IgG, less permeable for IgA, and little permeable for IgM ( SC 0. 06) ; EC20W filter was permeable for only small proportion of albumin and IgG, much less for IgA, and impermeable for IgM ( SC 0. 03 ). Single session of DFPP had no effect on serum albumin level, but had diverse effects on

  2. 双重血浆置换联合他汀治疗肾病综合征严重高胆固醇血症的疗效%Treatment of severe hypercholesterolemia in resistant nephrotic syndrome: the effect of combined therapy using double filtration plasmapheresis and statins

    Institute of Scientific and Technical Information of China (English)

    龚德华; 徐斌; 张丽华; 朱冬冬; 季大玺; 刘志红

    2013-01-01

    by a case-controlled design, to investigate the effect of combined therapy using double filtration plasmapheresis (DFPP) and oral statins on severe hypercholesterolemia in resistant nephrotic syndrome (NS). Methodology; Twenty two patients with severe hypercholesterolemia caused by resistant NS were enrolled in this study. They were divided into three groups as follow: 2 of them in the group receiving a single session of DFPP alone ( DFPP group) , 8 in the group receiving a single session of DFPP combined with oral Atorvastatin (Combination group) , and 12 in the group receiving oral Atorvastatin alone (Statins group). Other treatments for primary disease and symptom-control remained unchanged. All patients were followed-up to 4 weeks. Results: The baseline levels of serum total cholesterol (TC) were (12. 7 ±1.40) mmol/L in DFPP group, (17. 1 ±4. 70) mmol/L in combination group,and (15. 4 ±5. 10) mmol/L in statins group ( P > 0. 05 ). Single session of DFPP decreased serum TC by (85. 8% ±7. 20% ) ( P < 0. 01) , accompanied with a decline of plasma immunoglobulin A (IgA) , M and fibrinogen (P < 0. 01) but not albumin or IgG. No remission was found in the all groups during 4 weeks follow-up. During the follow-up period, compared with baselinedata,serum TC was 85. 9% at the 1 week, 100% at the 2 week, and 134% at the end in DFPP group, serum TC was below 50% in the first 2 weeks,and 70. 2% at the end in combination group,and serum TC was around 81. 1% through the whole period in the statins group. The levels of serum TC were significantly lower than that in the other groups at the first two weeks ( P < 0. 01). Conclusion; Oral statin alone can only decline lipids slightly in patients with severe hypercholesterolemia due to resistant nephrosis, while combined therapy using DFPP plus oral statin can decrease lipids to a larger extent in these patients.%目的:观察双重血浆置换(DFPP)联合他汀类降脂药治疗肾病综合征(NS)严重高胆固醇血症的

  3. Therapeutic Plasmapheresis Enabling Radioactive Iodine Treatment in a Patient with Thyrotoxicosis.

    Science.gov (United States)

    Min, Se Hee; Phung, Anita; Oh, Tae Jung; Han, Kyou-Sup; Kim, Man Jin; Kim, Jee Min; Lee, Ji Hyun; Park, Young Joo

    2015-10-01

    Therapeutic plasma exchange (TPE) is one possible treatment for patients resistant to conventional antithyroid drugs or requiring urgent attention for thyrotoxicosis. We report a 35-yr-old man with thyrotoxicosis, ultimately attributed to Graves' disease in whom antithyroid drug used initially was soon discontinued, due to abnormal liver function, and replaced by Lugol's solution. Three weeks later, an escape phenomenon (to Lugol's solution) was apparent, so we performed TPE to control the thyrotoxicosis. Two courses of TPE by a centrifugal type machine resulted in diminished levels of thyroid hormone levels, which then rebounded after another two courses of membrane filtration type TPE. However, the patient could be treated with radioactive iodine therapy without any complications at present.

  4. Feasibility of using DNA-immobilized nanocellulose-based immunoadsorbent for systemic lupus erythematosus plasmapheresis.

    Science.gov (United States)

    Xu, Changgang; Carlsson, Daniel O; Mihranyan, Albert

    2016-07-01

    The goal of this project was to study the feasibility of using a DNA-immobilized nanocellulose-based immunoadsorbent for possible application in medical apheresis such as systemic lupus erythematosus (SLE) treatment. Calf thymus DNA was bound to high surface area nanocellulose membrane at varying concentrations using UV-irradiation. The DNA-immobilized samples were characterized with scanning electron microscopy, atomic force microscopy, and phosphorus elemental analysis. The anti-ds-DNA IgG binding was tested in vitro using ELISA. The produced sample showed high affinity in vitro to bind anti-ds-DNA-antibodies from mice, as much as 80% of added IgG was bound by the membrane. Furthermore, the binding efficiency was quantitatively dependent on the amount of immobilized DNA onto nanocellulose membrane. The described nanocellulose membranes are interesting immunoadsorbents for continued clinical studies.

  5. A follow-up ¹⁸F-FDG brain PET study in a case of Hashimoto's encephalopathy causing drug-resistant status epilepticus treated with plasmapheresis.

    Science.gov (United States)

    Pari, Elisa; Rinaldi, Fabrizio; Premi, Enrico; Codella, Maria; Rao, Renata; Paghera, Barbara; Panarotto, Maria Beatrice; De Maria, Giovanni; Padovani, Alessandro

    2014-04-01

    Hashimoto's encephalopathy (HE) is a rare neuropsychiatric syndrome associated with antithyroid antibodies. It may have an acute onset (episodes of cerebral ischemia, seizure, and psychosis) or it may present as an indolent form (depression, cognitive decline, myoclonus, tremors, and fluctuations in level of consciousness). We here describe a case of encephalopathy presenting as non-convulsive status epilepticus associated with Hashimoto's thyroiditis (HT), unresponsive to corticosteroid therapy, with improvement after plasma exchange treatment. A previously healthy 19-year-old woman, presented generalized tonic-clonic seizures. About a month later, she manifested a speech disorder characterized by difficulties in the production and comprehension of language. Within a few days she also developed confusion and difficulties in recognizing familiar places, with gradual worsening over time. EEG revealed a non-convulsive status epilepticus (NCSE). CSF examination showed slightly elevated cell count and four oligoclonal bands. MRI was unremarkable, and (18)F-FDG brain PET showed widespread hypometabolism, mostly in posterior regions bilaterally. Laboratory and ultrasound findings showed signs of HT. Treatment with steroid was introduced without any improvement. After five sessions of plasma exchange there was a decrease of antithyroid antibodies, as well as EEG and clinical improvement. Three months after discharge (18)F-FDG brain PET showed a complete normalization of the picture, and the patient was asymptomatic. This report emphasizes the successful treatment of HE with plasma exchange in a patient who presented with NCSE. Based on the actual evidence, the term "Encephalopathy associated with Hashimoto's thyroiditis" may be the most proper. Furthermore, to our knowledge, this is the first case of an adult patient studied twice with an (18)F-FDG brain PET: prior to treatment with plasma exchange, and at 3 months follow-up when the patient was clinically completely asymptomatic. Studies in more patients are needed to clarify the relevance of (18)F-FDG brain PET as a possible diagnostic tool for HE.

  6. 离心式机械单采血浆管路、贮存袋的研究%Study On Mechanical Plasmapheresis Plasma Tubing And Plasma Bag

    Institute of Scientific and Technical Information of China (English)

    姜跃琴; 姚蓝; 王养敬; 许斌; 张磊

    2002-01-01

    目的:研究单采血浆中两种耗材-管路和贮存袋.材料和方法:以压力监测器接头(DPM)、导管流量和贮存袋耐寒性为研究关键,并与国内外同类产品或材料作比较.结果:DPM必须具有适宜的透气性(感应时间≤2s)、阻血性(能耐受40Kpa,40s)、滤除率(0.5um粒子,≥90%)、导管流量的准确性(与机器设定值误差<5%)、稳定性(采集过程中流量误差<5%)可通过控制导管内径(3.08~3.24mm)和选择弹性PVC材料来解决,贮存袋选用耐寒PVC料.扫描电镜证实韧性断裂.结论:研制成的管路和PCS2机器适配能取代进口同类产品,研制的贮存袋能低温保存血浆,破损率小于2‰.

  7. A Case of Subacute Cutaneous Lupus Erythematosus in a Patient with Mixed Connective Tissue Disease: Successful Treatment with Plasmapheresis and Rituximab

    Directory of Open Access Journals (Sweden)

    M. Fantò

    2013-01-01

    Full Text Available A 30-year-old woman affected by Mixed Connective Tissue Disease with scleroderma spectrum developed a facial eruption, a clinical and histological characteristic of subacute cutaneous lupus erythematosus (SCLE. Speckled anti-nuclear antibodies, high-titer anti-ribonucleoprotein1, anti-Sm, anti-Cardiolipin (aCL IgG/IgM, and anti-Ro/SSA antibodies were positive. SCLE was resistant to Azathioprine, Hydroxychloroquine, and Methotrexate while Mycophenolate Mofetil was suspended due to side effects. Subsequently, the patient was treated with three cycles of therapeutic plasma exchange (TPE followed, one month after the last TPE, by the anti-CD20 antibody Rituximab (RTX (375 mg/m2 weekly for 4 weeks. Eight and 16 months later the patient received other two TPE and RTX cycles, respectively. This therapeutic approach has allowed to obtain a complete skin healing persistent even after 8-month follow-up. Moreover, mitigation of Raynaud's phenomenon, resolution of alopecia, and a decline of aCL IgG/IgM and anti-Ro/SSA antibodies were observed.

  8. Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients Plasmaférese no tratamento da miastenia grave: estudo retrospectivo de 26 pacientes

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    Rosana Carandina-Maffeis

    2004-06-01

    Full Text Available We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE therapy in myasthenia gravis (MG. About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G or IIIa (MGFA scale. One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD ± 1.4 each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD ± 35.2; median 100.5 (extremes of 55 and 215. The mean volume of plasma exchanged in each session was 2396 ml (SD ± 561; median 2225 (extremes 1512 and 4500. Side effects occurred: reversible hypotension (seven cases, mild tremor or paresthesias (seven cases. Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.Analisamos a experiência do Hospital das Clínicas da Unicamp com plasmaferese: (PF na miastania grave (MG. 17,8 % do total dos casos de MG submeteu-se a PF, 26 casos, 19 mulheres e sete homens. A idade média de início da MG foi 28 anos (extremos 11 e 69. O menor déficit clínico foi IIb (O & G e IIIa (MGFA. A PF foi indicada no pré-operatório de timectomia em um caso e em sete devido a crise miastênica. Em 18 casos, com MG generalizada e sintomas bulbares ou com exacerbação de déficit prévio, a PF foi indicada como intervenção aguda. Em dois pacientes desse grupo ela foi indicada também em regime crônico de ciclos mensais. Os 26 pacientes submeteram-se a 44 ciclos e a 171 sessões de PF. O número médio de sessões em cada ciclo foi 3,9 (DP ± 1,4; mediana de 5, extremos 2 e 6. O tempo médio de cada sessão foi 106,5 minutos (DP ± 35,2; mediana de 100,5 (extremos 55 e 215.O volume médio de plasma trocado em cada sessão foi 2396 ml (DP ± 561; mediana 2225 (extremos 1512 e 4500. Efeitos colaterais foram reversíveis: hipotensão (sete casos, tremor ou parestesias leves (sete casos. Taxas de infecção e mortalidade devido a PF foram zero. A totalidade dos pacientes teve benefícios imediatos a cada ciclo de PF e usualmente receberam prednisona ou outro imunossupressor. Houve boa aceitação ao procedimento em 80,7% dos pacientes.

  9. Successful treatment of anti-NMDA receptor encephalitis with a prompt ovarian tumour removal and prolonged course of plasmapheresis: A case report

    Science.gov (United States)

    Rypulak, Elzbieta; Borys, Michal; Piwowarczyk, Pawel; Fijalkowska, Magdalena; Potrec, Beata; Sysiak, Justyna; Spustek, Janusz; Bartkowska-Sniatkowska, Alicja; Kotarski, Jan; Turski, Waldemar A.; Rejdak, Konrad; Czuczwar, Miroslaw

    2016-01-01

    Anti-N-methyl-d-aspartate-receptor (NMDAR) encephalitis is an uncommon autoimmune disorder with a wide spectrum of neuropsychiatric symptoms. There is a great requirement to emphasize the importance of a multidisciplinary team approach in the process of diagnosis and treatment of the potentially fatal condition, including psychiatrists, neurologists, gynaecologists and intensivists. Physicians must be aware that psychiatric and neurological disorders, which are typical features for NMDAR encephalitis in young women with ovarian tumours, may progress into status epilepticus and respiratory insufficiency. This disease can only be successfully treated with prompt surgical intervention and an early implementation of a wide array of immunosuppressive therapies. Optimal timing of initiation of therapeutic plasma exchange, as well as duration of treatment necessary to achieve desirable outcomes in patients with NMDAR remains unknown. The present case report aims to raise awareness about the importance of early implementation of this potentially life-saving therapy and continuing the treatment courses until full subsidence of symptoms. PMID:28101360

  10. ANTIOXIDANT DEFENSE SYSTEM CORRECTION IN TREATMENT OF PAYIENTS WITH INFERTILITY AND PERITONEAL ENDOMETRIOSIS

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    Dubinskaya, E.D.

    2016-09-01

    The findings suggest that the efficiency of the proposed comprehensive treatment techniques (laparoscopy and subsequent course of therapeutic plasmapheresis of patients with peritoneal endometriosis and infertility and with point mutations in the gene NAT2. The use of plasmapheresis  is pathogenetically justified in patients of the studied group.

  11. 血浆置换及静脉注射免疫球蛋白治疗格林巴利综合征临床研究%Clinical study on plasmapheresis and intravenous immunoglobulin in treating Guillain-Barre syndrome

    Institute of Scientific and Technical Information of China (English)

    王丹; 郭珍立; 任敏; 曾可斌

    2015-01-01

    Objective To compare the clinical effects of small dose of plasma exchange (SVP),high-dose plasma ex-change (LVP) and intravenous immunoglobulin(IVIG) in treating Guillain-Barre syndrome(GBS). Methods 75 GBS inpatients in the First Affiliated Hospital of Chongqing Medical University from June 2008 to June 2014 were randomly assigned to the SVP group(41 cases),LVP group(8 cases) and IVIG group(26 cases) according to the voluntary choice and plasma sources. Then the clinical data,efficacy and adverse reactions in 3 groups were analyzed. Results The total effective rates in the SVP group,LVP group and IVIG group were 90.2%,87.5%and 92.3%respectively,the differences were not statistically significant(χ2=0.91,P=0.186),but the SVP group had longer hospitalization duration and the LVP group had higher occurrence rate of adverse reactions , the hospitalization duration and the occurrence rate of adverse reactions had statistical difference among 3 groups (P<0.05). Con-clusion SVP,LVP and IVIG all are the effective way for treating GBS at present. Early treatment can significantly improve the prognosis. The three kinds of therapy have no significant difference in the curative effects.%目的:对比研究小剂量血浆置换(SVP)、大剂量血浆置换(LVP)及静脉注射免疫球蛋白(IVIG)治疗格林巴利综合征(GBS)的临床效应。方法对重庆医科大学附属第一医院2008年6月至2014年6月收治的75例GBS住院患者,根据患者自愿选择及血浆来源分为SVP组(41例)、LVP组(8例)和IVIG(26例)进行治疗,并对患者的临床资料、疗效及不良反应进行分析比较。结果 SVP、LVP、IVIG组总有效率分别为90.2%、87.5%、92.3%,三组比较,差异无统计学意义(χ2=0.91,P=0.186),但SVP组住院时间较长,LVP组不良反应发生率较高,三组患者住院时间、不良反应发生率比较,差异均有统计学意义(P<0.05)。结论 SVP、LVP和IVIG都是目前治疗GBS的有效方法,早期治疗可以明显改善预后。三者疗效无显著差异。

  12. Recurrent Goodpasture's syndrome.

    OpenAIRE

    Burke, B. R.; Bear, R. A.

    1983-01-01

    Goodpasture's syndrome was diagnosed in a 17-year-old boy with glomerulonephritis and hemoptysis. He was successfully treated with cyclophosphamide, prednisone and courses of plasmapheresis. The syndrome recurred 3 1/2 years later and was again successfully treated.

  13. Pathogenesis of rheumatoid arthritis and the immune response

    Energy Technology Data Exchange (ETDEWEB)

    Scheinberg, M.A.

    1983-08-01

    The interrelationship among lymphocytes, macrophages, and neutrophils appears to be an important aspect of the synovial inflammation that is characteristic of rheumatoid arthritis. In a study comparing gold sodium aurothiomalate (GST) with auranofin (Au), an orally absorbed compound, both appeared to inhibit the disease process and no difference between parenteral and oral administration was observed. Another study involved two groups of nine patients with severe rheumatoid arthritis. One group underwent plasmapheresis. The second group underwent total lymphoid irradiation. Both agents appeared to inhibit the disease process. Plasmapheresis was better tolerated that irradiation.

  14. [Hyperthyroid and acute tonsillitis in a 23-year-old woman].

    Science.gov (United States)

    Beyer, G; Küster, I; Budde, C; Wilhelm, E; Hoene, A; Evert, K; Stracke, S; Friesecke, S; Mayerle, J; Steveling, A

    2016-07-01

    A 23-year-old woman with preexisting Graves' disease who received thiamazole treatment presented with fever, dysphagia, hyperthyroidism and leukopenia. With suspicion of thyreotoxicosis accompanied by drug-induced agranulocytosis she was successfully managed by plasmapheresis, G‑CSF administration and inhibition of periphereal conversion of thyroid hormones. In due course she underwent thyroidectomy. Thiamazole is frequently associated with drug-induced agranulocytosis. Long-term therapy with thiamazole requires critical evaluation and alternatives should be considered early. Plasmapheresis is an adequate treatment option to achieve normal thyroid hormonal status.

  15. Immune mediated crescentic MPGN secondary to HBV infection: A rare presentation for a common infection.

    Science.gov (United States)

    Mareddy, Aswani Srinivas; Rangaswamy, Dharshan; Vankalakunti, Mahesha; Attur, Ravindra Prabhu; Nagaraju, Shankar Prasad; Koti, Neeraja

    2016-01-01

    Hepatitis B virus (HBV) infection presenting as crescentic glomerulonephritis in the absence of cryoglobulinemia is an extremely rare phenomenon. We report a case of a 44-year-old male with HBV infection, who underwent kidney biopsy for rapidly progressive renal failure and nephrotic range proteinuria. Histopathological evaluation of the kidney biopsy was consistent with immune complex mediated crescentic membranoproliferative glomerulonephritis (MPGN). The patient achieved complete renal and virological remission with steroids, plasmapheresis and antiviral therapy. This case report summarises the importance of early initiation of immunosuppression and plasmapheresis under antiviral coverage for improved clinical outcomes.

  16. Immune mediated crescentic MPGN secondary to HBV infection: A rare presentation for a common infection

    Directory of Open Access Journals (Sweden)

    Aswani Srinivas Mareddy

    2016-01-01

    Full Text Available Hepatitis B virus (HBV infection presenting as crescentic glomerulonephritis in the absence of cryoglobulinemia is an extremely rare phenomenon. We report a case of a 44-year-old male with HBV infection, who underwent kidney biopsy for rapidly progressive renal failure and nephrotic range proteinuria. Histopathological evaluation of the kidney biopsy was consistent with immune complex mediated crescentic membranoproliferative glomerulonephritis (MPGN. The patient achieved complete renal and virological remission with steroids, plasmapheresis and antiviral therapy. This case report summarises the importance of early initiation of immunosuppression and plasmapheresis under antiviral coverage for improved clinical outcomes.

  17. Impact of Detoxification Techniques on Pulmonary Gas Exchange Function in Patients with Generalized Peritonitis

    Directory of Open Access Journals (Sweden)

    R. A. Mlinnik

    2012-01-01

    Full Text Available Objective: to analyze the impact of different detoxification techniques on pulmonary gas exchange function in patients with generalized peritonitis complicated by multiple organ failure. Subjects and methods. One hundred and thirty patients with generalized peritonitis were examined. According to the used detoxification techniques, the patients were divided into 5 groups. All the patients underwent a comprehensive examination, the key element of which was the evaluation of gas exchange parameters. Results. Membrane plasmapheresis and plasmapheresis with sodium hypochlorite infusion to the plasma filter in patients with peritonitis are shown to improve pulmonary blood oxygenation.

  18. The selective therapeutic apheresis procedures.

    Science.gov (United States)

    Sanchez, Amber P; Cunard, Robyn; Ward, David M

    2013-02-01

    Selective apheresis procedures have been developed to target specific molecules, antibodies, or cellular elements in a variety of diseases. The advantage of the selective apheresis procedures over conventional therapeutic plasmapheresis is preservation of other essential plasma components such as albumin, immunoglobulins, and clotting factors. These procedures are more commonly employed in Europe and Japan, and few are available in the USA. Apheresis procedures discussed in this review include the various technologies available for low-density lipoprotein (LDL) apheresis, double filtration plasmapheresis (DFPP), cryofiltration, immunoadsorption procedures, adsorption resins that process plasma, extracorporeal photopheresis, and leukocyte apheresis.

  19. Comparison of nutritional parameters after abo incompatible living donor renal transplantation

    Directory of Open Access Journals (Sweden)

    Joon Seok Oh

    2012-06-01

    By the end of the first year, serum hemoglobin, calcium, albumin, HDL, bilirubin, AST, ALT were increased statistically. But serum phosphate, globulin were decreased statistically. In conclusion, successful ABO incompatible living donor kidney transplantation would restore a normal nutritional status even though the patients were performed plasmapheresis during the pre-transplant period.

  20. Hypertriglyceridemia Induced Pancreatitis (Chylomicronemia Syndrome Treated with Supportive Care

    Directory of Open Access Journals (Sweden)

    Emin Uysal

    2014-01-01

    Full Text Available Hypertriglyceridemia is a rare cause of pancreatitis. In treatment pancreatic rest, lifestyle changes, medications (fibrates, n-3 polyunsaturated fatty acids, and nicotinic acid are essential. Many experimental treatment modalities have been reported as insulin and heparin infusion and plasmapheresis. In this study we present the hypertriglyceridemia-induced pancreatitis treated with supportive care.

  1. Non-Invasive Evaluation of Raynaud’s Phenomenon,

    Science.gov (United States)

    1984-02-28

    phenomenon and includes thromboangiitis obliterans (most frequently seen in young males), arteriosclerosis I __1 (elderly males), and thoracic outlet...presence of an abnorman flow signal or absence of flow is evidnece of a disease artery. Ultrasound is also useful in differential diagnosis when thoracic ...antagonist ketanserin, 23 30 calcium-channel antagonist nifedipine, and intraarterial reserpine; thyroid hormone; plasmapheresis; 2 7 and sympathectomy

  2. Magnetisk resonans billeddannelse af dissemineret sklerose

    DEFF Research Database (Denmark)

    Christiansen, A P; Wanscher, B; Larsson, H B

    1994-01-01

    Gadolinium-DTPA, it is possible to distinguish a newly developed plaque from an older one. Therefore, MRI has become an important examination in therapeutic trials. Just now, MRI with Gadolinium-DTPA is being used to evaluate the efficacy of plasmapheresis and immunoglobulin treatment in a joint study...

  3. Relapsing-Remitting MS (RRMS)

    Medline Plus

    Full Text Available ... Treating MS d Comprehensive Care Developing a Healthcare Team Make the Most of Your Doctor Visits Advance Medical Directives d Find an MS Care Provider Partners in MS Care d Managing Relapses Plasmapheresis d Rehabilitation Functional Electrical Stimulation (FES) ...

  4. Postoperative repeated respiratory insufficiency and thyrotoxicosis in molar pregnancy.

    Science.gov (United States)

    Cekic, B; Geze, S; Ulusoy, H; Coskun, I; Erturk, E

    2012-06-01

    Following the removal of a hydatiform mole in a 34-year-old, 14-week pregnant patient, thyrotoxicosis and respiratory insufficiency attacks were twice unexpectedly repeated. The symptoms were resolved with the administration of plasmapheresis, antithyroid and β-blocker drugs and non-invasive mechanical ventilation; however, she was again operated due to prolonged elevated β-hCG.

  5. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy

    NARCIS (Netherlands)

    P.A. van Doorn (Pieter)

    1990-01-01

    textabstractPatients with a chronic inflammatory demyelinating polyneuropathy (CIDP) may respond to treatment with corticosteroids and to plasmapheresis, which was demonstrated in controlled clinical studies. In an uncontrolled study it was found that 13/17 CIDP patients had a rapid and clinical imp

  6. COMBINED PLASMA FILTRATION, SELECTIVE ADSORPTION OF CYTOKINES WITH HEMOFILTRATION AND PLASMA EXCHANGE IN THE CORRECTION OF PLATELET AND COAGULATION HEMOSTASIS IN PATIENTS WITH THE SYNDROME OF DISSEMINATED INTRAVASCULAR COAGULATION

    Directory of Open Access Journals (Sweden)

    L. V. Belskikh

    2015-01-01

    Full Text Available Aim. To improve the efficiency of treatment of the patients with pancreatic necrosis through correcting the platelet-coagulation potential by methods of combined plasma filtration, selective sorption of cytokines with hemofiltration and plasmapheresis. Methods. The effects of selective sorption of cytokines with hemofiltration and plasmapheresis upon coagulation potential were studied in 70 patients with acute pancreatitis. Plasmapheresis was performed on PCS 2 (Haemonetics, USA; selective sorption of cytokines with hemofiltration – on Lynda ® machine (Bellco, Italy. The method of computer laser phasemeter was used for vital assessing of platelet morphofunctional status. Results. In patients with pancreatic necrosis in the fermentation phase the percentage of resting platelets was 51.3%, which is 12% below the control numbers. The number of activated platelets increased: 25.1% of the cells were represented by platelets with low activation; 15.5% – highly activated cells. The number of degenerative-modified platelets increased by two times – up to 8.1%. In the phase of sequestration and suppurative complications the percentage of resting platelets was only 42.3%, which is 1.5 times lower than the control numbers. 34.2% of the cells were presented by platelets with low activation; 11.0% – highly activated cells. The number of degenerative-modified platelets exceeded 12.5%. The analysis of the morphological structure of the platelets population revealed that due to plasmapheresis inclusion into therapeutic complex in the phase of intoxication of pancreatic necrosis the normalization of platelet activation status of hemostasis was observed. In 5 (20% patients the normalization of average platelets morphometric parameters was shown after selective sorption of cytokines with hemofiltration and plasmapheresis. These patients demonstrated positive clinical dynamics. In 15 patients morphometric values of platelets either remained at the same

  7. INTRAVENOUS IMMUNOGLOBULIN ADMINISTRATION FOR DESENSITIZATION BEFORE RENAL TRANSPLANTATION AND MANAGING ANTIBODY-MEDIATED REJECTION

    Directory of Open Access Journals (Sweden)

    A. I. Sushkov

    2011-01-01

    Full Text Available Much attention has been placed recently in transplantation in highly HLA-sensitized patients. In attempts to remove these antibodies and enable successful renal transplantation, several approaches have been developed. Intravenous immunoglobulin (IVIG was found to be effective in the treatment of autoimmune and inflammatory disorders (e. g. Kawasaki disease, Guillain-Barre syndrome. Recently, a beneficial effect of IVIG on the reduc- tion of anti-HLA antibodies was described. The anti-inflammatory effect of IVIG provides hopeful opportunities in antibody-mediated rejection (AMR management. There are several protocols of IVIG administration for pre-transplant desensitization and AMR treatment: high-dose IVIG, low-dose IVIG + plasmapheresis, IVIG + plasmapheresis + rituximab. These advancements have enabled transplantation in patients previously considered untransplantable and in concert with new diagnostic techniques has resulted in new approaches to management of AMR. 

  8. Acute Hemorrhagic Leukoencephalitis in Children: A Case Report

    Science.gov (United States)

    Khademi, Gholam Reza; Aelami, Mohammad Hasan

    2016-01-01

    Acute hemorrhagic leukoencephalitis (AHLE) is a rare demyelinating disease characterized by an acute rapidly progressive fulminant inflammation of the white matter. In this case report, we introduce a case of AHLE in children with an interesting and lengthy process and successful treatment. A previously healthy 13-year-old girl was admitted to the hospital because of fever and loss of consciousness. After 4 days, she was referred to our pediatric intensive care unit in Mashhad, Iran. On admission, she had right-sided parotiditis. With a diagnosis of AHLE, our patient was treated with methylprednisolone, intravenous immunoglobulin, acyclovir, and plasmapheresis. AHLE is a rare and severe demyelinating disease, the mortality and morbidity of which can be decreased by early detection and treatment with steroid therapy, intravenous immunoglobulin, acyclovir, and plasmapheresis. PMID:27217610

  9. Acute Hemorrhagic Leukoencephalitis in Children: A Case Report

    Directory of Open Access Journals (Sweden)

    Gholam Reza Khademi

    2016-05-01

    Full Text Available Acute hemorrhagic leukoencephalitis (AHLE is a rare demyelinating disease characterized by an acute rapidly progressive fulminant inflammation of the white matter. In this case report, we introduce a case of AHLE in children with an interesting and lengthy process and successful treatment. A previously healthy 13-year-old girl was admitted to the hospital because of fever and loss of consciousness. After 4 days, she was referred to our pediatric intensive care unit in Mashhad, Iran. On admission, she had right-sided parotiditis. With a diagnosis of AHLE, our patient was treated with methylprednisolone, intravenous immunoglobulin, acyclovir, and plasmapheresis. AHLE is a rare and severe demyelinating disease, the mortality and morbidity of which can be decreased by early detection and treatment with steroid therapy, intravenous immunoglobulin, acyclovir, and plasmapheresis.

  10. Progressive multifocal leukoencephalopathy secondary to rituximab-induced immunosuppression and the presence of John Cunningham virus: a case report and literature review.

    Science.gov (United States)

    Kelly, Deirdre; Monaghan, Bernadette; McMahon, Eileen; Watson, Geoffrey; Kavanagh, Eoin; O'Rourke, Killian; McCaffrey, John; Carney, Desmond

    2016-09-01

    We present the case of a 60-year-old man who developed subacute neurologic changes, in the setting of stage III non-Hodgkin's follicular lymphoma, and was treated with induction chemotherapy, followed by a year of maintenance rituximab. Magnetic resonance imaging of the brain with gadolinium was pathognomonic for progressive multifocal leukoencephalopathy (PML). He was treated with sequential plasmapheresis and intravenous immunoglobulin with clinical improvement. A literature review of the diagnostic workup of rituximab-induced PML was undertaken. This case and the literature review demonstrate the important role of magnetic resonance imaging of the brain in diagnosis and follow-up of rituximab-induced PML. Specific radiologic features in combination with cerebrospinal fluid can be diagnostic and avoid the morbidity and mortality of a diagnostic brain biopsy. Plasmapheresis and intravenous immunoglobulin have a therapeutic role and demonstrate symptom improvement and disease control. Follow-up imaging in combination with clinical response is important in demonstrating a treatment response.

  11. Rituximab for the treatment of refractory simultaneous anti-glomerular basement membrane (anti-GBM) and membranous nephropathy.

    Science.gov (United States)

    Bandak, Ghassan; Jones, Bruce A; Li, Jian; Yee, Jerry; Umanath, Kausik

    2014-02-01

    Antibody-mediated anti-glomerular basement membrane (anti-GBM) disease occurs rarely in the presence of another B-cell disorder, membranous nephropathy. The coexistence of these two autoimmune disorders would be anticipated to require differing, specific therapies targeted to each disease process. We describe a case of concomitant membranous nephropathy and anti-GBM disease in which conventional therapy, including steroids, plasmapheresis and cyclophosphamide, failed to attenuate the anti-GBM disease, yet responded to an alternative treatment of rituximab. This B-cell directed, monoclonal, chimeric antibody treatment substantially reduced anti-GBM antibody titers and led to discontinuation of plasmapheresis, while maintaining the remission of membranous nephropathy and anti-GBM disease.

  12. Apheresis to treat systemic vasculitis.

    Science.gov (United States)

    Moussi-Frances, Julie; Sallée, Marion; Jourde-Chiche, Noémie

    2017-06-07

    Apheresis has been used in the treatment of severe systemic vasculitides, in conjunction with immunosuppressive therapies, for over 40 years. The aim is to rapidly remove autoantibodies or circulating immune complexes from the plasma. The two main indications at present are vasculitis associated with Antineutrophil Cytoplasmic Antibodies (ANCAs) manifesting as severe renal involvement and/or intraalveolar hemorrhage and antiglomerular basement membrane disease (Goodpasture syndrome). The ongoing PEXIVAS randomized controlled trial is assessing plasmapheresis to treat ANCA-associated vasculitis with or without severe renal involvement or intraalveolar hemorrhage. The two main apheresis techniques used to treat systemic vasculitis are plasmapheresis (by filtration, centrifugation, or double filtration) and immunoadsorption. The advantages and drawbacks of each technique are discussed here. Whether one technique is superior over the other in the current indications has not been proven. Copyright © 2017 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  13. Acute liver failure associated with occupational exposure to tetrachloroethylene.

    Science.gov (United States)

    Shen, Chuan; Zhao, Cai-Yan; Liu, Fang; Wang, Ya-Dong; Wang, Wei

    2011-01-01

    Tetrachloroethylene is a chlorinated solvent that is primarily used in dry cleaning and degreasing operations. Although the hepatotoxicity caused by tetrachloroethylene has been well documented in literature, it is rarely considered as a cause of acute liver failure. We report a case of a 39-yr-old man who was admitted to our hospital for acute liver failure due to tetrachloroethylene exposure. Histological examination of the liver revealed massive hepatic necrosis, prominently, in zone 3 of the hepatic lobules. The patient underwent supportive treatment along with 3 sessions of plasmapheresis, and consequently, he presented a favorable outcome. Repeat liver biopsy performed 6 months after the patient's discharge showed architectural distortion with postnecrotic cirrhosis. Physicians should be aware of the possibility of acute liver failure induced by tetrachloroethylene. Early plasmapheresis can be effective for individuals with sufficient capacity for hepatocyte regeneration.

  14. Hypertriglyceridemia: Is there a role for prophylactic apheresis? A case report

    Directory of Open Access Journals (Sweden)

    Ana Rita Francisco

    Full Text Available Abstract Severe hypertriglyceridemia has been consistently associated with an increased risk of cardiovascular disease and other complications, namely acute pancreatitis. We report a case of a 64 year-old woman with hypertrophic cardiomyopathy and metabolic syndrome with triglyceride level of 3260 mg/dL. Plasma exchange was performed with simultaneous medical treatment to achieve a rapid and effective lowering of triglycerides in order to prevent clinical complications. After three plasmapheresis sessions a marked reduction in triglyceride and total cholesterol levels was observed. Several cases have shown the importance of plasmapheresis in the treatment of acute pancreatitis. We intend to demonstrate the applicability of this technique as primary prophylaxis in the presence of extremely high serum triglyceridemia levels.

  15. Hemolytic uremic syndrome as a primary manifestation of acute human immunodeficiency virus infection.

    Science.gov (United States)

    Gomes, A M; Ventura, A; Almeida, C; Correia, M; Tavares, V; Mota, M; Seabra, J

    2009-05-01

    Hemolytic uremic syndrome may be associated with human immunodeficiency virus infection but it occurs in advanced stages of human immunodeficiency virus disease. As in other forms of hemolytic uremic syndrome plasmapheresis seems to be the treatment of choice. The authors present an unusual case of hemolytic uremic syndrome associated with acute human immunodeficiency virus infection in a 38 year-old black male. The patient was admitted with fever, asthenia, nausea, diarrhea, and reduced urinary output. He was found to have anemia, thrombocytopenia and severe renal failure. Hemolytic uremic syndrome was diagnosed and he was started on plasmapheresis and hemodialysis. Serological tests were consistent with acute human immunodeficiency virus infection: the enzyme linked immunosorbent assay for human immunodeficiency virus was weakly positive, Western Blot test was negative and human immunodeficiency virus RNA quantification was positive, with > 1,000,000 copies/microl. After 4 daily treatment sessions, patient's clinical condition improved and hemoglobin, platelets, lactic dehydrogenase and renal function normalized.

  16. Association of acute cerebellar ataxia and human papilloma virus vaccination: a case report.

    Science.gov (United States)

    Yonee, Chihiro; Toyoshima, Mitsuo; Maegaki, Yoshihiro; Kodama, Yuichi; Hayami, Hiroshi; Takahashi, Yukitoshi; Kusunoki, Susumu; Uchibori, Ayumi; Chiba, Atsuro; Kawano, Yoshifumi

    2013-10-01

    We report the case of a patient who developed symptoms of acute cerebellar ataxia (ACA) after administration of the human papilloma virus (HPV)-16/18 vaccine. This patient developed symptoms of ACA, including nausea, vertigo, severe limb and truncal ataxia, and bilateral spontaneous continuous horizontal nystagmus with irregular rhythm, 12 days after administration of the HPV-16/18 AS04-adjuvanted cervical cancer vaccine. After this, the patient received methylprednisolone pulse and intravenous immunoglobulin (IVIG) therapies as well as immunoadsorption plasmapheresis. Severe ACA symptoms did not improve after methylprednisolone pulse and IVIG therapies, but the patient recovered completely after immunoadsorption plasmapheresis. This temporal association strongly suggests that ACA was induced by the vaccination. Georg Thieme Verlag KG Stuttgart · New York.

  17. Neuromyelitis optica in an adolescent after bone marrow transplantation.

    Science.gov (United States)

    Baumer, Fiona M; Kamihara, Junne; Gorman, Mark P

    2015-01-01

    Central nervous system complications of bone marrow transplant are a common occurrence and the differential diagnosis is quite broad, including opportunistic infections, medications toxicities, graft versus host disease, and other autoimmune processes. We summarize previously reported cases of autoimmune myelitis in post-transplant patients and discuss a 17-year-old boy who presented with seronegative neuromyelitis optica after a bone marrow transplant for acute myeloid leukemia. Our patient had a marked improvement in symptoms after plasmapheresis. Including our patient, there have been at least eight cases of post-transplant autoimmune myelitis presented in the literature, and at least three of these are suspicious for neuromyelitis optica. Several of these patients had poor outcomes with persistent symptoms after the myelitis. Autoimmune processes such as neuromyelitis optica should be carefully considered in patients after transplant as aggressive treatment like early plasmapheresis may improve outcomes. Copyright © 2015 Elsevier Inc. All rights reserved.

  18. Treatment of extrahepatic manifestations of chronic hepatitis C viral infection--a challenge.

    Science.gov (United States)

    Tănăsescu, C; Ionescu, R

    2010-01-01

    Often, chronic hepatitis C infection is clinically manifested as extra hepatic disease. Therapy of the extra hepatic manifestations (EHM) is always difficult and based on the optimal and individual association of antiviral treatment, immunosuppressant and plasma cleaning techniques. We observed for 4 years 246 patients admitted to "Colentina" Internal Medicine Clinic, of whom 168 were diagnosed as chronic C hepatitis. 130 of those patients have had at least one EHM. In our experience, the presence of an EHM is significantly correlated with lack of early viral response and sustained viral response, as well. Cryoglobulinemic vasculitis needs to be treated with oral or pulse corticotherapy associated to plasmapheresis. When present, peripheral neuropathy and cryocrit greater than 10% are indicators of need of more than 3 plasmapheresis sessions.

  19. Efficacies of treatments for anti-NMDA receptor encephalitis.

    Science.gov (United States)

    Wang, Hsiuying

    2016-01-01

    Treatments for anti-N-methyl-D-aspartate (NMDA) receptor encephalitis include immunotherapy with steroids, intravenous immunoglobulin, plasma exchange, or plasmapheresis as first-line treatments, immunotherapy with rituximab or cyclophosphamide as second-line treatments, and tumor removal. In this systematic review, we evaluated previous studies and examined the association between certain microRNAs and anti-NMDA receptor encephalitis to investigate the performance of different treatment combinations. The efficacies of different combinations of treatments classified into the following four categories were compared: (I) intravenous immunoglobulin administration, (II) plasmapheresis or plasma exchange, (III) treatment with rituximab or cyclophosphamide and (IV) tumor removal. Statistical analyses showed that treatment combinations including at least two of these categories resulted in higher efficacy rates than treatment with a single form of therapy. These findings suggest that if a patient is not recovering, converting to other therapies is more likely to result in early recovery than continuing on the original therapy.

  20. [Successful treatment of enterohemorrhagic Escherichia coli O111-induced hemolytic-uremic syndrome and acute encephalopathy].

    Science.gov (United States)

    Mizuno, Hideki; Minouchi, Keiji; Aoyama, Shou; Hinoue, Yoshinobu

    2015-07-01

    We report a case of a woman in her twenties with enterohemorrhagic Escherichia coli O111-induced hemolytic-uremic syndrome who developed acute encephalopathy on day 5 of admission. She recovered after treatment with steroid pulse therapy, plasmapheresis, and recombinant thrombomodulin, without any adverse sequelae. We report this interesting case and provide a summary of the recent outbreak of enterohemorrhagic Escherichia coli O111.

  1. Treatment of intractable interstitial lung injury with alemtuzumab after lung transplantation

    DEFF Research Database (Denmark)

    Kohno, M; Perch, M; Andersen, E;

    2011-01-01

    of acute rejection, worsened within 2 weeks, despite high-dose steroids, change of calcineurin inhibitor, and plasmapheresis. Within a few days after a single, 10-mg, intravenous dose of alemtuzumab, the patient's health improved markedly. She has remained stable for 4 months on a standard, ambulatory......, posttransplant antirejection drug regimen. We have since successfully treated with alemtuzumab three additional patients who developed interstitial lung injury after lung transplantation, who are also summarized in this report....

  2. [Guillain-Barre syndrome with an especially severe course].

    Science.gov (United States)

    Kuliński, W; Cholewa, M; Rybak, T; Wołoszczak, J; Szulecka, I

    1997-01-01

    A case of polyradiculoneuritis in a 51-year-old man is reported. Symptoms occurred suddenly. Our serologic tests failed to explain the cause of the illness. Physical examination showed tetraplegia and respiratory muscles paralysis which required continuous use of respirator during six weeks. In the treatment were used: steroid hormones, antibiotics, anabolics, plasmapheresis. In physical treatment: kinesitherapy, massage, electrotherapy, hydrotherapy. After three years of intensive physical treatment patient was cured.

  3. Thrombotic Thrombocytopenic Purpura Associated with Pneumococcal Sepsis

    Directory of Open Access Journals (Sweden)

    Jeffrey R Schriber

    1993-01-01

    Full Text Available The first documented case of thrombotic thrombocytopenic purpura (TTP associated with pneumococcal septicemia is reported. This association has been previously demonstrated with hemolytic uremic syndrome. The patient presented with recurrent seizures, oliguric renal failure, fever, thrombocytopenia and microangiopathic hemolytic anemia; coagulation studies were normal. Blood and sputum cultures were positive for Streptococcus pneumoniae. The patient responded to therapy with plasmapheresis and antiplatelet agents as well as antibiotics. Coincident infection should be searched for in all cases of TTP.

  4. [Otolaryngologic manifestations of Churg-Strauss sindrome. Report of a case and review of the literature].

    Science.gov (United States)

    Pino Rivero, V; González Palomino, A; Pantoja Hernández, C G; Trinidad Ruíz, G; Pardo Romero, G; Montero García, C; Blasco Huelva, A

    2006-01-01

    Churg-Strauss Sindrome or allergic granulomatosis is a small vessel systemic vasculitis characterized by asthma, hypereosinophilia and necrotizing vasculitis with extravascular eosinophil granulomas. We describe a case assisted in our hospital who presented pulmonary infiltrates, fever, peripherical neuropathy, weight loss, myalgia, rhinosinusitis, with antecedents of nasal polyposis, and facial edema. Our aim is to review the main otolaryngologic manifestations of this rare illness that is treated with oral corticosteroids, immunosupresor drugs like ciclofosfamide and plasmapheresis.

  5. 重症肌无力的治疗现状%Circumstance of the therapy of myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    于逢春; 余华锋

    2004-01-01

    Myasthenia gravis is a most common neurological disease with autoimmune disorder. Most of the research on myasthenia gravis has focused on treatment . we will assess the effect of the treatments of myasthenia gravis, which including corticosteroids, immunosuppressive , IV immunoglobulin , plasmapheresis and thymectomy. The treatment for patients with myasthenia gravis usually depends on age, clinical characteristics and thymoma or nonthymoma-related MG and so on.

  6. Fatalt forløb ved in vitro-fertilisation

    DEFF Research Database (Denmark)

    Sørensen, Martin Kryspin; Møller-Sørensen, Hasse; Svane, Christian;

    2010-01-01

    Chylomicronaemia syndrome is a rare disorder primarily caused by a genetic defect which increases triglycerides, combined with a secondary inducing factor. We describe the fatal course of a 33-year-old, pregnant woman with known dyslipidaemia who had been treated with in vitro fertilisation...... and developed chylomicronaemia syndrome with severe hypertriglyceridaemia, hypertriglyceridaemia-induced acute pancreatitis and septic shock. Appropriate treatment including close monitoring, severe restriction of dietary fat intake and early plasmapheresis is emphasized - especially during pregnancy....

  7. Central venous line complications and tip detection

    OpenAIRE

    Ameneh Rezaee Gheshlaghi; Hamid Zamani Moghadam Dolu; Elham Pishbin; Maryam Salehi

    2015-01-01

    Central venous line is one of a creative instrument that saves human’s life in critical medical situation. Central venous line access is frequently involved in the disease management. It is used for rapid fluid therapy, transvenous pacemakers, infusion of some medications, hemodialysis or plasmapheresis and etc. Most of the emergency departments have some staffs that are trained for central venous line insertion but related complications occur during central venous line placement.Central veno...

  8. Priporočila za uporabo in zdravljenje s svežo zmrznjeno plazmo: Recommendations for the usage and treatment with fresh frozen plasma:

    OpenAIRE

    Domanovič, Dragoslav; Stecher, Adela; Zver, Samo

    2012-01-01

    Fresh frozen plasma (FFP) is a blood component obtained from processed whole blood or collected by plasmapheresis and afterwards stored in frozen state. Indications for the clinical use of FFP are single coagulation factor deficiencies (such as F V and F XI), multiple coagulation factors deficiency (most frequent as a consequence of massive hemorrhage), coagulopathy caused byadvanced liver disease, warfarin mediated coagulopathy and thrombotic thrombocytopenic purpura. Standard FFP treatment ...

  9. Plasma rich in growth factors: preliminary results of use in the preparation of future sites for implants.

    Science.gov (United States)

    Anitua, E

    1999-01-01

    This article presents preliminary clinical evidence of the beneficial effect of the use of plasma rich in growth factors of autologous origin. The plasma is obtained from the individual patient by plasmapheresis. The macroscopic and microscopic results obtained with bone regeneration using this technique, which uses no membrane or barrier, can be observed. The incorporation of these concepts can introduce several advantages, including the enhancement and acceleration of bone regeneration and more rapid and predictable soft tissue healing.

  10. Thrombocytopenic purpura: Importance of early diagnosis

    OpenAIRE

    Linklater, David M.; Voth, Arnold

    1996-01-01

    Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are important diagnostic considerations for family physicians because therapy can be lifesaving. Treatment with plasmapheresis is usually successful. Therefore, early diagnosis is essential. A patient with microangiopathic hemolytic anemia is described. Diagnosis of Hodgkin's disease was confirmed after the initial episode. The case highlights the important role of family physicians in the diagnosis and treatment of uncommon he...

  11. Prevention of pure red cell aplasia after major or bidirectional ABO blood group incompatible hematopoietic stem cell transplantation by pretransplant reduction of host anti-donor isoagglutinins

    OpenAIRE

    Stussi, G.; Halter, J; Bucheli, E; Valli, P V; Seebach, L; Gmür, J; Gratwohl, A; Passweg, J. R.; Seebach, J.D.

    2009-01-01

    BACKGROUND: Persistent anti-donor isoagglutinins after major ABO blood group incompatible hematopoietic stem cell transplantation may cause delayed red blood cell engraftment and post-transplant pure red cell aplasia. DESIGN AND METHODS: We investigated the effect of pretransplant anti-donor isoagglutinin reduction by in vivo absorption and/or plasmapheresis on the incidence of pure red cell aplasia and the time to red blood cell engraftment in 153 hematopoietic stem cell transplant recipient...

  12. The clinical efficacy of extracorporeal and intravascular hemocorrection methods in psoriasis

    Directory of Open Access Journals (Sweden)

    Baityakov V.V.

    2012-03-01

    Full Text Available Purpose. The study of the influence of extracorporeal and intravascular hemocorrection methods (plasmapheresis, ultraviolet blood radiation, ozonetherapy and its combination on skin process and life quality in patients with extensive psoriasis. Methods. 253 patients with extensive psoriasis from the age of 18 to 72 have been investigated. PASI (Psoriasis Area and Severity Index and dermatological index of life quality (DILQ have been used for treatment assessment. Results. The inclusion of efferent quantum and ozone therapy methods in the complex treatment of psoriasis has promoted to the more rapid and complete positive dynamics of skin process and the improvement of life quality. The use of plasmapheresis and its variations with photomodification or the ozone treatment of returning erythrocyte suspension has been the most effective. Plasmapheresis and its modifications are appropriateto be used in the complex treatment of the patients with severe forms of psoriasis. Conclusion: Further study of efferent quantum and ozone therapy methods and their wider use in psoriasis therapy and other chronic dermatosis seem to be promising.

  13. [Topics of chronic inflammatory demyelinating polyradiculoneuropathy--diagnosis and treatment situation in Japan].

    Science.gov (United States)

    Iijima, Masahiro

    2013-05-01

    CIDP is a motor and sensory neuropathy characterized by chronic, step-wised, or relapsing progression. Both cellular and humoral autoimmunity targeting the myelin sheath is assumed as the main mechanism of CIDP pathogenesis. While the AAN diagnostic criteria have been the main method in Japan, the EFNS/PNS criteria recently replaced them because of their good diagnostic sensitivity and clinical superiority. The first-line therapy for CIDP patients in Japan is intravenous immunoglobulin (IVIg), corticosteroids, and phasmapheresis, the same as in other countries. Regarding therapeutics, two major differences between Japan and other countries exist. Firstly, while half-dose IVIg (1 g/kg body weight) every three weeks was established as maintenance therapy as a result of the ICE study in 2008, full-dose IVIg (2 g/kg body weight over five days) once a month is still accepted in Japan for highly recurrent patients. Secondly, Japanese clinicians prefer immune adsorption plasmapheresis (IAPP) instead of plasma exchange (PE) among three types of plasmapheresis (IAPP, PE, and double-filtered plasmapheresis [DFPP]). These differences could be due to the characteristic and independent health insurance system in Japan. Using recent knowledge and diagnostic criteria, clinical trials have been based on these global platforms. Recently, efforts have been made to share these platforms with a worldwide vision.

  14. Panel-reactive antibody levels and renal transplantation rates in sensitized patients after desensitization and human leucocyte antigen amino acid residue matching.

    Science.gov (United States)

    Shang, Wenjun; Dong, Laidong; Feng, Guiwen; Wang, Yue; Pang, Xinlu; Li, Jinfeng; Liu, Lei; Zhang, Weihong

    2013-08-01

    To determine whether a new desensitization protocol (mycophenolate mofetil [MMF], plasmapheresis and antithymocyte globulin [ATG], complemented with human leucocyte antigen [HLA] amino acid residue matching) could reduce panel-reactive antibody (PRA) levels in sensitized patients, to facilitate successful renal transplantation. Patients awaiting transplantation with PRA levels >10% received treatment with MMF; those with PRA levels >30% were also treated with plasmapheresis. Patients whose PRA level was desensitization were eligible for transplantation. When a donor became available, traditional HLA matching and HLA amino acid residue matching were performed. All patients received ATG induction therapy postoperatively. Thirty-two sensitized patients were enrolled. Desensitization produced a significant decrease in PRA levels; 27 patients (84.4%) became eligible for transplantation and 26 (81.2%) subsequently underwent successful transplantation. Residue matching improved the proportion with a mismatch number of 0-1 from 7.7% to 65.4%, compared with traditional HLA matching. Postoperatively, all patients showed immediate graft function. Acute rejection occurred in three patients (11.5%) and infections in seven patients (25.9%); all were treated successfully. The combination of a desensitization protocol (MMF, plasmapheresis and ATG) and residue matching appears to be an effective strategy for sensitized patients awaiting renal transplantation.

  15. Antibody-Mediated Rejection of the Heart in the Setting of Autoimmune Demyelinating Polyneuropathy: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Kathryn J. Lindley

    2012-01-01

    Full Text Available Background. Antibody-mediated rejection (AMR is caused by the production of donor-specific antibodies (DSA which lead to allograft injury in part via complement activation. The inflammatory demyelinating polyneuropathies (IDP are inflammatory disorders of the nervous system, involving both cellular and humoral immune mechanisms directed against myelin. Case Report. A 58-year-old man five years after heart transplant presented with progressive dyspnea, imbalance, dysphagia, and weakness. Nerve conduction studies and electromyogram were consistent with IDP. Plasmapheresis and high-dose steroids resulted in improvement in neurologic symptoms. Within two weeks, he was readmitted with anasarca and acute renal failure, requiring intravenous furosemide and inotropic support. Echocardiogram and right heart catheterization revealed reduced cardiac function and elevated filling pressures. DSA was positive against HLA DR53, and endomyocardial biopsy revealed grade 1R chronic inflammation, with strong capillary endothelial immunostaining for C4d. Plasmapheresis and intravenous immunoglobulin (IVIG were initiated. His anasarca and renal failure subsequently resolved, echocardiogram showed improved function off inotropes, and anti-DR53 MFI was reduced by 57%. Conclusions. This is an example of a single immune-mediated process causing concurrent IDP and AMR. The improvement in cardiac function and neurologic symptoms with plasmapheresis, IVIG, and high-dose steroids argues for a unifying antibody-mediated mechanism.

  16. Massive thyroxine intoxication: evaluation of plasma extraction.

    Science.gov (United States)

    Binimelis, J; Bassas, L; Marruecos, L; Rodriguez, J; Domingo, M L; Madoz, P; Armengol, S; Mangues, M A; de Leiva, A

    1987-01-01

    Six patients were admitted after erroneous massive intake of levothyroxine (70-1200 mg over an interval of 2-12 days). All patients developed classical symptoms of thyrotoxicosis within 3 days of the first dose; five patients presented grade II-III coma and one became stuporous (days 7-10). Two patients developed left ventricular failure and three had arrhythmias (days 8-11). Total thyroid hormone levels in serum on admission ranged 935-7728 nmol/l for T4 (TT4) and 23-399 nmol/l for T3 (TT3). All patients received treatment with hydrocortisone and Propranolol. Propylthiouracil was also given in 3 cases. Extractive techniques (charcoal haemoperfusion and/or plasmapheresis) were initiated 8-14 days after the first dose of L-T4. The plasma disappearance rate (K) of TT4 with plasmapheresis was 30 times higher, on average, than under standard medical treatment (M). Also, K of TT4 under haemoperfusion was about five times higher than K under M. K changes for TT3 were higher under haemoperfusion than under plasmapheresis. Furthermore, extractive procedures shortened the average half life of TT4, (from 106.5 +/- 44.6 to 59.7 +/- 20.2 h, p less than 0.05).

  17. Coronary artery bypass grafting in a patient with active idiopathic cryoglobulinemia: revisiting the issue

    Directory of Open Access Journals (Sweden)

    Hafiz Abdul Moiz Fakih

    2016-02-01

    Full Text Available Background: Cryoglobulinemia is a cold-reactive autoimmune disease. It is of distinctive importance in cardiac surgery because of the use of hypothermic cardiopulmonary bypass (CPB. Cryoglobulins, which activate at variable levels of hypothermia, can cause precipitation during surgery leading to possibly severe leukocytoclastic or necrotizing vasculitis, clinically manifested as ischemic events, such as cutaneous ulcerations, glomerulonephritis, arthritis, or peripheral neuropathies among the most reported associated comorbidities. Management of CPB and systemic protection in this rare but unique scenario requires individualized planning. We report the case of a patient with active cryoglobulinemia who was preoperatively managed with plasmapheresis. He underwent hypothermic coronary bypass with no precipitation and flare during and after surgery. Case presentation: We describe the case of a 59-year-old Caucasian male with clinically significant idiopathic cryoglobulinemia and history of recurrent skin lesions and toe amputations secondary to cold exposure. He presented with 2-h duration of chest pain and new onset atrial fibrillation. After cardiac catheterization, a diagnosis of three-vessel coronary artery disease was established and coronary artery bypass grafting (CABG was scheduled. Because of a high risk of flare-up during surgery, the patient was preemptively treated with two sessions of plasmapheresis before bypass. He then underwent hypothermic CABG. The pre- and perioperative course was unremarkable without any clinical evidence of precipitation. The patient was discharged on day 6 postoperatively without any complications. Conclusion: Preoperative plasmapheresis before hypothermic coronary bypass can prevent fatal cryoglobulinemia-related complications in patients with active disease.

  18. Effects of hypoproteinemia on renal hemodynamics, arterial pressure, and fluid volume

    Energy Technology Data Exchange (ETDEWEB)

    Manning, R.D. Jr.

    1987-01-01

    The effects of long-term hypoproteinemia on renal hemodynamics, arterial pressure, and fluid volume were studied in eight conscious dogs over a 34-day period. Plasma protein concentration (PPC) was decreased by daily plasmapheresis, and the effects of decreasing and increasing sodium intake were measured. By the 12th day of plasmapheresis PPC had decreased to 2.5 g/dl from a control value of 7.2 g/dl, mean arterial pressure had decreased to 78% of control, glomerular filtration rate (GFR) was 75.2% of control, and urinary sodium excretion was decreased. By day 18 of plasmapheresis, estimated renal plasma flow (ERPF) was decreased to 60% of control due to the decreased arterial pressure and an increase in renal vascular resistance. GFR and ERPF were determined from the total clearance of (/sup 125/I)iothalamate and (/sup 131/I)iodohippurate. Also, plasma renin activity and plasma aldosterone concentration were both increased, and the relationship between mean arterial pressure and urinary sodium excretion was distinctly shifted to the left along the arterial pressure axis. In contradistinction to acute experiments, chronic hypoproteinemia results in decreases in GFR, ERPF, and urinary sodium excretion and has marked effects on both fluid volume and arterial pressure regulation.

  19. Interesting case of G6PD deficiency anemia with severe hemolysis

    Directory of Open Access Journals (Sweden)

    Anupam Chhabra

    2013-01-01

    Full Text Available Severe hemolysis was observed in a critically ill patient with G6Pd deficiency where the causative trigger could not be identified. We describe one young patient with severe hemolysis treated with two cycles of plasmapheresis which proved to be an effective tool in the treatment. The patient presented with diffuse pain abdomen, vomiting, yellowish discoloration of sclera and skin and acute breathlessness. Hemoglobin 5.4 mg/dl and total (T serum bilirubin 17.08 mg/dl: Direct (D 4.10 mg/dl and Indirect (I 12.98 mg/dl. Subsequently patient started passing black color urine. As the patient developed severe hemolysis and the trigger agent of hemolysis was unknown, two cycles of plasmapheresis were performed with the aim to remove unknown causative agent. Consequently no trace of hemolysis was found and patient stabilized. Plasmapheresis can be used to treat G6PD deficient patients with severe hemolysis due to unidentified trigger agent.

  20. Desensitization protocol in highly HLA-sensitized and ABO-incompatible high titer kidney transplantation.

    Science.gov (United States)

    Uchida, J; Machida, Y; Iwai, T; Naganuma, T; Kitamoto, K; Iguchi, T; Maeda, S; Kamada, Y; Kuwabara, N; Kim, T; Nakatani, T

    2010-12-01

    A positive crossmatch indicates the presence of donor-specific alloantibodies and is associated with a graft loss rate of >80%; anti-ABO blood group antibodies develop in response to exposure to foreign blood groups, resulting in immediate graft loss. However, a desensitization protocol for highly HLA-sensitized and ABO-incompatible high-titer kidney transplantation has not yet been established. We treated 6 patients with high (≥1:512) anti-A/B antibody titers and 2 highly HLA-sensitized patients. Our immunosuppression protocol was initiated 1 month before surgery and included mycophenolate mofetil (1 g/d) and/or low-dose steroid (methylprednisolone 8 mg/d). Two doses of the anti-CD20 antibody rituximab (150 mg/m(2)) were administered 2 weeks before and on the day of transplantation. We performed antibody removal with 6-12 sessions of plasmapheresis (plasma exchange or double-filtration plasmapheresis) before transplantation. Splenectomy was also performed on the day of transplantation. Postoperative immunosuppression followed the same regimen as ABO-compatible cases, in which calcineurin inhibitors were initiated 3 days before transplantation, combined with 2 doses of basiliximab. Of the 8 patients, 7 subsequently underwent successful living-donor kidney transplantation. Follow-up of our recipients showed that the patient and graft survival rates were 100%. Acute cellular rejection and antibody-mediated rejection episodes occurred in 1 of the 7 recipients. These findings suggest that our immunosuppression regimen consisting of rituximab infusions, splenectomy, plasmapheresis, and pharmacologic immunosuppression may prove to be effective as a desensitization protocol for highly HLA-sensitized and ABO-incompatible high-titer kidney transplantation. Copyright © 2010 Elsevier Inc. All rights reserved.

  1. Rituximab in anti-GBM disease: A retrospective study of 8 patients.

    Science.gov (United States)

    Touzot, Maxime; Poisson, Johanne; Faguer, Stanislas; Ribes, David; Cohen, Pascal; Geffray, Loic; Anguel, Nadia; François, Helene; Karras, Alexandre; Cacoub, Patrice; Durrbach, Antoine; Saadoun, David

    2015-06-01

    Anti-glomerular basement membrane (GBM) disease is a rare autoantibody-mediated disorder presenting as rapidly progressive glomerulonephritis, and often with pulmonary hemorrhage. Antibody removal with plasmapheresis and immunosuppressive drugs are the cornerstones of the treatment. Data regarding the use of specific B-cell depleting therapy such as rituximab are lacking. We conducted a retrospective observational study of 8 patients with severe and/or refractory GBM disease that received rituximab therapy. Eight patients (2 men, 6 women) with a mean age of 26 ± 13.1 years old were included. Seven had severe renal involvement [median creatinin level was 282 μmol/l, range (65-423)] requiring high immunosuppressive or plasmapheresis dependent, and two had relapse of pulmonary hemorrhage including one with renal failure. Patients received an initial immunosuppressive treatment including steroid and cyclosphosphamide (n = 8) and plasmapheresis (n = 5). Except one late relapse, rituximab therapy was started within two months after diagnosis. All patients except one received 4 weekly dose of rituximab (375 mg(2)). Anti-GBM antibodies were still present in 6/8 patients, at rituximab initiation. Complete remission was observed in 7 out of 8 patients, mostly 3 months after rituximab therapy. After a mean follow-up of 25.6 months (range 4-93), patient and renal survival were 100% and 75% respectively, but rituximab use did not improve GFR. Anti-GBM antibodies remained negative for all patients during follow-up. Only one patient developed a severe bacterial infection but no opportunistic or viral infections were reported. Rituximab may represent an additional and/or alternative therapy in the induction treatment of anti-GBM disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. Current therapy of systemic sclerosis (scleroderma).

    Science.gov (United States)

    Müller-Ladner, U; Benning, K; Lang, B

    1993-04-01

    Treatment of systemic sclerosis (scleroderma) presents a challenge to both the patient and the physician. Established approaches include long-term physiotherapy, disease-modifying agents such as D-penicillamine, and treatment of organ involvement. These efforts are often unsatisfactory since the results are poor. However, recent advances include treatment of Raynaud's phenomenon (plasmapheresis, stanozolol, and prostacyclin analogues), scleroderma renal crisis (angiotensin-converting enzyme inhibitors), and gastric hypomotility (cisapride). This article covers the current approaches to the disease-modifying therapy including those related to the function of collagen-producing fibroblasts, vascular alterations, and the cellular and humoral immune system, as well as treatment of involved organs.

  3. Crescentic Glomerulonephritis Associated with Pulmonary Tuberculosis.

    Science.gov (United States)

    Kanodia, K V; Vanikar, A V; Patel, R D; Suthar, K S; Trivedi, H L

    2016-01-01

    Tuberculosis of kidney and urinary tract is caused by members of the Mycobacterium tuberculosis complex. Kidney is usually infected by haematogenous spread of bacilli from focus of infection in the lungs. Glomerular involvement in tuberculosis presenting as a rapidly progressive glomerulonephritis is a rare entity. We report a rare case of crescentic glomerulonephritis associated with pulmonary tuberculosis in a 26-year-old man. Patient was treated with corticosteroids, haemodialysis, intravenous immunoglobulin and four cycles of plasmapheresis. He did not respond to 4-drug anti-tuberculosis treatment for renal pathology and was switched over to maintenance haemodialysis. However, he responded to pulmonary TB.

  4. Successful Pregnancy Outcome in Women with Recurrent IVF Failure and Anti-hCG Autoimmunity: A Report of Three Cases

    Directory of Open Access Journals (Sweden)

    Valeria Muller

    2016-01-01

    Full Text Available We report three cases of effective management of infertility in women with a history of repeated unsuccessful IVF attempts, who have developed antibodies to hCG. A novel approach to conservative treatment of immunologic reproductive failure, suggested for selected patients, included membrane plasmapheresis, combined prednisolone, and intravenous immunoglobulin therapy. No adverse side effects were observed; all cases resulted in pregnancy and subsequent life births. In order to be given an adequate efficient treatment, women with recurrent implantation failure should be suspected for autoimmune factor of infertility and its possible association with anti-hCG autoimmunity.

  5. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant

    Directory of Open Access Journals (Sweden)

    Pedro García Cosmes

    2016-03-01

    Full Text Available Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimes. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+ who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease.

  6. Anesthetic management of a parturient with Guillain-Barre syndrome posted for emergency caesarian section

    Directory of Open Access Journals (Sweden)

    Abhijit Paul

    2012-01-01

    Full Text Available We report the anesthetic management of a case of Guillain-Barre syndrome in the 34 th week of gestation coming for an emergency Cesarean section. The perioperative anesthetic challenges have been discussed with emphasis on the medical and anesthetic management which includes the use of plasma-pheresis, intravenous gamma-globulin, and the safety of preservative free 0.75% isobaric ropivacaine, which was administered intrathecally in this difficult medical condition with excellent hemodynamic, maternal, and fetal outcome. The sensory and motor blocks achieved were well suited to the clinical risks and conditions.

  7. [Sudden deafness as the initial manifestation of chronic myelogenous leukemia: case report].

    Science.gov (United States)

    Chen, Yandi; An, Lifeng; Jin, Guanghua

    2015-05-01

    To study the pathogenesis of hearing loss in chronic myelogenous leukemia (CML). To report one case with CML whose first sign was sudden unilateral hearing loss. Sudden hearing loss in CML was presented with dramatic high white blood cell count in peripheral blood. Some cases of sudden hearing loss in CML may be improved or even cured by leukapheresis and intrathecal chemotherapy. The proposed pathogenesis for deafness in leukemia is due to hyperleukocytosis, hyperviscosity syndrome, leukemic infiltration and the inner ear hemorrhage. In treatment, clinicians should quickly reduce the number of white blood cells to lighten the tumor burden. Intrathecal injection of MTX and plasmapheresis is commonly used.

  8. PTU-associated cutaneous vasculitis with ANCA anti-MPO and anti-PR3 antibodies.

    Science.gov (United States)

    Jacobs, E M G; Hartkamp, A; Kaasjager, H A H

    2003-09-01

    A 36-year-old woman presented at our clinic with symmetrical, tender, palpable purpuric lesions on her lower legs and buttocks after restarting PTU therapy for relapsing Graves' disease. PTU-induced vasculitis was diagnosed with remarkable ANCA anti-MPO and anti-PR3 antibody positivity. The purpuric skin lesions resolved immediately after discontinuation of the drug and the ANCA titres lowered. In the presence of activated neutrophils, PTU could induce a high cytotoxity and injure the vessel walls. Treatment of choice is discontinuation of the drug. Sometimes more aggressive therapy as cyclophosphamide or plasmapheresis is warranted.

  9. Olanzapine-Induced Hypertriglyceridemia Resulting in Necrotizing Pancreatitis

    Science.gov (United States)

    Roy-Chaudhury, Prabir; Yadlapalli, Ganesh

    2016-01-01

    Olanzapine is an atypical antipsychotic agent that was approved by the Food and Drug Administration in 1996 for treatment of psychotic disorders, bipolar disorder, and schizophrenia. Since that time, numerous case reports have been published that describe the association of olanzapine and the development of pancreatitis. Furthermore, 3 reports suggest the mechanism of olanzapine-induced hypertriglyceridemia as the etiology of this progression. We report a case of a 36-year-old man who developed necrotizing pancreatitis secondary to olanzapine-induced hypertriglyceridemia. This case, to our knowledge, is the most severe case of this progression and the first case requiring plasmapheresis for acute management.

  10. The role of chemotherapy in gastric cancer-related microangiopathic haemolytic anaemia

    Science.gov (United States)

    Goldstein, David

    2017-01-01

    We report a case of a previously well 46-year-old man who presented with microangiopathic haemolytic anaemia (MAHA) of unknown origin. After extensive investigations, he was diagnosed with cancer-related microangiopathic haemolytic anaemia (CR-MAHA) secondary to gastric adenocarcinoma. Initial treatment with plasmapheresis was ineffective, but the patient’s haematological abnormalities improved markedly with chemotherapy directed against his gastric cancer. Our case amplifies previous experience of gastric cancer-associated MAHA which responded to treatment with chemotherapy. We review current understanding of the proposed pathophysiology of CR-MAHA and conclude that this condition is ideally treated with chemotherapy.

  11. Catastrophic antiphospholipid syndrome presenting as fever of unknown origin

    Directory of Open Access Journals (Sweden)

    Fatma I Al-Beladi

    2012-01-01

    Full Text Available Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies with characteristic clinical manifestation, which include venous, arterial thrombosis, thrombotic microangiopathy, and recurrent fetal loss. The syndrome can be secondary to many causes including systemic lupus erythematosus (SLE or "primary" antiphospholipid syndrome (APLS. We report a case of a man with catastrophic antiphospholipid syndrome (CAPS, which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis.

  12. SEVERE IMMUNE HEMOLYTIC ANEMIA AFTER LIVER TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    A. I. Sushkov

    2013-01-01

    Full Text Available Clinical case of successful treatment of severe immune hemolytic anemia after liver transplantation is represen- ted in this article. The cause of complication was so-called passenger lymphocyte syndrome (a type of graft- versus-host disease. Two plasmapheresis sessions and Ig (0.5 g/kg in combination with increased maintenance immunosuppression with a short course of oral methylprednisolone in a total dose of 150 mg during 12 days were effective. The patient was discharged from hospital 34 days after transplantation in a satisfactory condition with a stable hemoglobin level. 

  13. Chronic Inflammatory Demyelinating Polyneuropathy Following Anti-TNF-α Therapy With Infliximab for Crohn's Disease

    Science.gov (United States)

    Concepcion, Orestes; Schlachterman, Alexander; Glover, Sarah; Forsmark, Christopher Y.

    2016-01-01

    We present a 29-year-old male with Crohn's disease who developed chronic inflammatory demyelinating polyneuropathy (CIDP) related to infliximab therapy. He developed lower extremity weakness and dysesthesia 3 weeks after a fourth infliximab dose. Laboratory examination revealed an elevated cerebrospinal fluid protein without pleocytosis. The patient initially responded to plasmapheresis therapy with marked symptomatic improvement, but relapsed and was refractory to subsequent treatments with plasmaphereisis, intravenous immunoglobulin, and glucocorticoids. While a causal relationship between infliximab and CIDP cannot be proven, clinicians should monitor Crohn's disease patients who are receiving TNF-α antagonists for neurologic symptoms suggestive of demyelinating disease. PMID:27144200

  14. [Extracorporeal detoxication in the treatment of peritonitis complicated with multiple organ insufficiency].

    Science.gov (United States)

    Lobakov, A I; Vatazin, A V; Fomin, A M; Andrianova, E Iu; Abakumova, L Ia

    1994-07-01

    The article deals with experience in the use of filtration and combined methods of extracorporeal detoxification in 201 patients with peritonitis in the phase of polyorganic insufficiency. The methods were developed in the department of abdominal surgery of the Moscow Regional Scientific Research Clinical Institute. The authors show the indications for and the advantages, the mechanisms of the effect, and the results of bifiltration cascade exchange plasmapheresis, improved hemofiltration method, and selective detoxification of portal blood, and the combination of these methods with continuous membrane blood oxygenation. The mortality reduced from 46.6% in the control group to 26.5% in the main group.

  15. CHALLENGES IN TREATMENT OF RENAL GRAFT ACUTE ANTIBODY-MEDIATED REJECTION

    Directory of Open Access Journals (Sweden)

    A. I. Sushkov

    2016-01-01

    Full Text Available Diagnostic criteria and treatment protocols for acute antibody-mediated rejection (AMR of kidney allograft remain controversial. We report the case of early severe AMR after primary kidney transplantation. The graft removal was considered in the absence of treatment efficacy and in the presence of systemic infl ammatory response syndrome. However, at surgery the graft looked normal and it was not removed. The repeated treatment course (plasmapheresis, antithymocyte globulin, intravenous immunoglobulin and rituximab was effective. The patient has good and stable graft function in 1 year after transplantation. 

  16. Thrombotic microangiopathy: An unusual cause of renal failure in rheumatoid arthritis

    Science.gov (United States)

    Sakthirajan, R.; Dhanapriya, J.; Dineshkumar, T.; Gopalakrishnan, N.; Murugan, S.; Balasubramaniyan, T.

    2017-01-01

    Rheumatoid arthritis (RA) is one of the commonest rheumatological diseases. Renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. Only a few cases of TMA in patients with RA were reported to date. We describe a 45-year-old female patient with RA who presented with oliguria and edema. Renal biopsy showed TMA with patchy cortical necrosis. She improved with hemodialysis and plasmapheresis. PMID:28182045

  17. Retreatment with intravenous immunoglobulin (IVIG of refractory Guillain Barre syndrome in children

    Directory of Open Access Journals (Sweden)

    Rivas Larrauri Francisco

    2014-07-01

    Full Text Available Guillain-Barré syndrome (GBS is an acute symmetrical pa- ralyzing disease due to a demyelinating polyrradiculoneuropathy, often induced by a preceding infection 1. The main modalities for the treatment of GBS include plasmapheresis and intrave- nous immune globulin. Reports of the use of IVIG in children with GBS are limited: 1 g/kg for two days or 400 mg/kg for five days. While these studies in children are not definitive because of design limitations, their results are consistent with the larger randomized trials in adults 2,3.

  18. Anti-glomerular basement membrane: A rare cause of renal failure in children

    Directory of Open Access Journals (Sweden)

    Indira Agarwal

    2017-01-01

    Full Text Available Anti-glomerular basement membrane (GBM disease is a rare cause of acute renal failure and known to have bad prognosis regarding renal functions recovery and patient survival specially when diagnosed late and presents with severe renal failure that requires dialysis. We report a case of 11-year-old child with acute renal failure secondary to anti-GBM disease and associated with antineutrophil cytoplasmic antibody-positive vasculitis. He was treated with plasmapheresis, steroids, and cyclophosphamide with recovery of his kidney functions.

  19. Role of therapeutic apheresis in infectious and inflammatory diseases: Current knowledge and unanswered questions.

    Science.gov (United States)

    Sloan, Steven R; Andrzejewski, Chester; Aqui, Nicole A; Kiss, Joseph E; Krause, Peter J; Park, Yara A

    2015-10-01

    Apheresis can remove pathogens and mediators that contribute to pathogenic inflammatory responses in diseases not generally considered to be "Hematologic." Erythrocytapheresis can remove intracellular pathogens such as Babesiosis. Plasmapheresis can remove mediators of the inflammatory response in conditions such as sepsis, chronic autoimmune urticaria and malignant pertussis. Leukapheresis can remove potentially harmful leukocytes in Crohn's Disease and malignant pertussis. While apheresis can remove all of these substances, the clinical efficacy and pathophysiologic changes that occur during apheresis in these conditions are largely unknown. Hence, the clinical utility of apheresis in these conditions is largely unknown and research in these areas has the potential to benefit many patients with a variety of diseases.

  20. Successful treatment of life-threatening Evans syndrome due to antiphospholipid antibody syndrome by rituximab-based regimen: a case with long-term follow-up.

    Science.gov (United States)

    Rückert, A; Glimm, H; Lübbert, M; Grüllich, C

    2008-08-01

    An association of antiphospholipid antibody syndrome with antibodies directed against either phospholipids or plasma proteins strongly suggest that B-cell dysfunction may be involved in its pathogenesis. Antiphospholipid antibody syndrome with autoimmune cytopenias shows a poor response rate to conventional treatment with anticoagulants, glucocorticosteroids, immunosuppressive agents, intravenous immunoglobulin or plasmapheresis. We report a case of life-threatening antiphospholipid antibody syndrome with Evans syndrome receiving successful multimodal treatment including anti-CD20 monoclonal antibody rituximab with long-term follow-up.

  1. Treatment of Subepidermal Autoimmune Bullous Diseases Sürekli Eğitim

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    Ekin Şavk

    2011-06-01

    Full Text Available The common therapeutic goal for all subepidermal bullous diseases is to prevent inflammation and production of pathogenic autoantibodies and/or to facilitate the elimination of these antibodies. Diseases included in this group are the pemphigoids, linear IgA dermatosis, epidermolysis bullosa acquisita, dermatitis herpetiformis and bullous systemic erythematosus. Corticosteroids are the most commonly used systemic medication. Other alternatives include dapsone, various antibiotics and nicotinamide, azathioprine, cyclophosphamide, methotrexate, cycloporine, mycophenolate mofetil, IV immunoglobulins and plasmapheresis. Rapid reepithelialization and prevention of secondary infections are provided by local hygenic measures including antiseptic baths and wound care. (Turkderm 2011; 45 Suppl 1: 54-8

  2. Treatment of Subepidermal Autoimmune Bullous Diseases

    Directory of Open Access Journals (Sweden)

    Ekin Şavk

    2011-06-01

    Full Text Available The common therapeutic goal for all subepidermal bullous diseases is to prevent inflammation and production of pathogenic autoantibodies and/or to facilitate the elimination of these antibodies. Diseases included in this group are the pemphigoids, linear IgA dermatosis, epidermolysis bullosa acquisita, dermatitis herpetiformis and bullous systemic erythematosus. Corticosteroids are the most commonly used systemic medication. Other alternatives include dapsone, various antibiotics and nicotinamide, azathioprine, cyclophosphamide, methotrexate, cycloporine, mycophenolate mofetil, IV immunoglobulins and plasmapheresis. Rapid reepithelialization and prevention of secondary infections are provided by local hygenic measures including antiseptic baths and wound care.

  3. Catastrophic antiphospholipid syndrome presenting as fever of unknown origin.

    Science.gov (United States)

    Al-Beladi, Fatma I

    2012-01-01

    Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies with characteristic clinical manifestation, which include venous, arterial thrombosis, thrombotic microangiopathy, and recurrent fetal loss. The syndrome can be secondary to many causes including systemic lupus erythematosus (SLE) or "primary" antiphospholipid syndrome (APLS). We report a case of a man with catastrophic antiphospholipid syndrome (CAPS), which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis.

  4. Emergency caesarean delivery in a patient with cerebral malaria-leptospira co infection: Anaesthetic and critical care considerations

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    Sukhen Samanta

    2014-01-01

    Full Text Available Malaria-leptospira co-infection is rarely detected. Emergency surgery in such patients has not been reported. We describe such a case of a 24-year-old primigravida at term pregnancy posted for emergency caesarean delivery who developed pulmonary haemorrhage, acute respiratory distress syndrome, acute kidney injury, and cerebral oedema. Here, we discuss the perioperative management, pain management (with transverse abdominis plane block, intensive care management (special reference to management of pulmonary haemorrhage with intra pulmonary factor VIIa and the role of plasmapheresis in leptospira related jaundice with renal failure.

  5. [Pulmonary-renal syndrome].

    Science.gov (United States)

    Risso, Jorge A; Mazzocchi, Octavio; De All, Jorge; Gnocchi, César A

    2009-01-01

    The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbidity-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  6. Goodpasture syndrome in a pregnant woman.

    Science.gov (United States)

    Vasiliou, D Marie; Maxwell, Cynthia; Shah, Prakeshkumar; Sermer, Mathew

    2005-11-01

    Goodpasture syndrome, an immunologic disorder characterized by glomerulonephritis and pulmonary hemorrhage, rarely presents in pregnancy. We describe a patient who was diagnosed with Goodpasture syndrome in her second trimester. She required daily hemodialysis, intermittent plasmapheresis, and immunosuppressive therapy. Her pregnancy was complicated by hypertension, and she delivered a low birth weight neonate prematurely at 26 4/7 weeks of gestation by cesarean due to nonreassuring fetal status. Deterioration in the fetal status may have been secondary to complications of hypertension, in addition to prematurity. Goodpasture syndrome in pregnancy may be associated with significant maternal and fetal morbidity.

  7. Bortezomib for antibody mediated rejection treatment: experience at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán in Mexico City.

    Science.gov (United States)

    Leyva, Sergio; Marino-Vázquez, Lluvia A; Reyes-Loaeza, Jorge A; Vega, Olynka; Uribe-Uribe, Norma; Alberú, Josefina; Morales-Buenrostro, Luis E

    2009-01-01

    The use of bortezomib as a treatment modality of AHR improved and stabilized graft function (clinical response) in the majority of patients. Its use in single dose, even combined with rituximab, does not seem to be useful to obtain a sustained clinical response neither to reduce HLAabs level. The use of 4 doses of bortezomib in days 1, 4, 7, and 10 (1.3 mg/m2 BSA each) plus plasmapheresis produced both a good clinical response and a reduction in DSA. Moving forward, it will necessary to define the long-term effectiveness of bortezomib and whether rituximab administration is indispensable to achieve this goal.

  8. Significantly improved survival time in pigs with complete liver ischemia treated with a novel bioartificial liver.

    Science.gov (United States)

    Flendrig, L M; Calise, F; Di Florio, E; Mancini, A; Ceriello, A; Santaniello, W; Mezza, E; Sicoli, F; Belleza, G; Bracco, A; Cozzolino, S; Scala, D; Mazzone, M; Fattore, M; Gonzales, E; Chamuleau, R A

    1999-10-01

    Aim of the study was to evaluate treatment efficacy and safety of a scaled-up version of our porcine hepatocytes based BAL system in pigs with complete liver ischemia (LIS). Thirty-one pigs underwent total devascularization of the liver (LIS) by termino-lateral porta-caval shunts and sutures around the bile duct, the common hepatic and gastroduodenal arteries and their accessory branches. The hepato-duodenal ligament was completely transected. Four experimental groups were studied: the first control group (LIS Control, n = 10) received glucose infusion only, the second control group (LIS Plasmapheresis, n = 8) was connected to a centrifugal plasma-separator with a bottle representing the bioreactor volume, the third control group (LIS Empty-BAL, n = 5) received BAL treatment without cells, and the treated group (LIS Cell-BAL, n = 8) was connected for a maximum period of 24 hours to our scaled-up BAL seeded with around 14 billion viable primary porcine hepatocytes. BAL treatment significantly prolonged life in large animals (approximately 35 kg) with complete LIS (Controls, mean +/- SEM: 33.1 +/- 3 h, Cell-BAL: 51.1 +/- 3.4 h; p = 0.001; longest survivor 63 h). In addition, blood ammonia and total bilirubin levels decreased significantly, indicating metabolic activity of porcine hepatocytes in the bioreactor. No significant differences were noticed among the three control groups, indicating that there was no device effect and that the plasmapheresis procedure was well tolerated. No important adverse effects were observed.

  9. Systematic review of hypertriglyceridemia-induced acute pancreatitis: A more virulent etiology?

    Science.gov (United States)

    Carr, Rosalie A; Rejowski, Benjamin J; Cote, Gregory A; Pitt, Henry A; Zyromski, Nicholas J

    2016-01-01

    We sought to define the severity and natural history of hypertriglyceridemia induced acute pancreatitis (HTG-AP), specifically whether HTG-AP causes more severe AP than that caused by other etiologies. Systematic review of the English literature. Thirty-four studies (15 countries; 1972-2015) included 1340 HTG-AP patients (weighted mean prevalence of 9%). The median admission triglyceride concentration was 2622 mg/dl (range 1160-9769). Patients with HTG have a 14% weighted mean prevalence of AP. Plasmapheresis decreased circulating triglycerides, but did not conclusively affect AP mortality. Only 7 reports (n = 392 patients) compared severity of HTG-AP to that of AP from other etiologies. Of these, 2 studies found no difference in severity, while 5 suggested that HTG-AP patients may have increased severity compared to AP of other etiology. 1) hypertriglyceridemia is a relatively uncommon (9%) cause of acute pancreatitis; however, patients with hypertriglyceridemia have a high (14%) incidence of acute pancreatitis; 2) plasmapheresis may offer specific therapy unique to this patient population; and 3) data specifically comparing the severity of HTG-AP with AP caused by other etiologies are heterogeneous and scarce. Copyright © 2016. Published by Elsevier B.V.

  10. Progressive multifocal leukoencephalopathy secondary to rituximab-induced immunosuppression and the presence of John Cunningham virus: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Deirdre Kelly, MBBCH

    2016-09-01

    Full Text Available We present the case of a 60-year-old man who developed subacute neurologic changes, in the setting of stage III non-Hodgkin's follicular lymphoma, and was treated with induction chemotherapy, followed by a year of maintenance rituximab. Magnetic resonance imaging of the brain with gadolinium was pathognomonic for progressive multifocal leukoencephalopathy (PML. He was treated with sequential plasmapheresis and intravenous immunoglobulin with clinical improvement. A literature review of the diagnostic workup of rituximab-induced PML was undertaken. This case and the literature review demonstrate the important role of magnetic resonance imaging of the brain in diagnosis and follow-up of rituximab-induced PML. Specific radiologic features in combination with cerebrospinal fluid can be diagnostic and avoid the morbidity and mortality of a diagnostic brain biopsy. Plasmapheresis and intravenous immunoglobulin have a therapeutic role and demonstrate symptom improvement and disease control. Follow-up imaging in combination with clinical response is important in demonstrating a treatment response.

  11. [New therapy schemes for acute, subacute and chronic variants of extrinsic allergic alveolitis].

    Science.gov (United States)

    Makar'iants, N N; Shmelev, E I

    2012-01-01

    In order to improve treatment of patients with exogenous allergic alveolitis morphologically different variants of the disease, i.e. acute, subacute and chronic were identified and confirmed. For each variant of exogenous allergic alveolitis new therapy schemes were proposed. The study included 74 patients who were divided into 5 groups. In the first group with acute exogenous allergic alveolitis inhalation glycocorticosteroids in high doses in combination with plasmapheresis were prescribed, in the second group standard therapy with systemic glycocorticosteroids was prescribed. The third and the fourth group consisted of patients with subacute exogenous allergic alveolitis. The protracted ambroxol inhalation using nebulizers and the reduced dose of systemic glycocorticosteroids were used in the third group; and the standard dose of systemic glycocorticosteroids was used in the fourth. The fifth group consisted of patients with chronic exogenous allergic alveolitis, who received the standard dose of glycocorticosteroids and cytostatic drugs. After one month of therapy, it was ascertained that the use of high doses of inhalation glycocorticosteroids in combination with plasmapheresis in patients with acute exogenous allergic alveolitis led to significant improvements in clinical and CT presentation, physical activity tolerance, as well as the use of systemic glycocorticosteroids. The use of ambroxol inhalation in patients with subacute exogenous allergic alveolitis led to a significant improvement in clinical symptomatology, functional parameters and CT presentation, thus enabling to reduce the dose of glycocorticosteroids used and to avoid unwanted side effects.

  12. Rasmussen's encephalitis: the relevance of neuropsychological assessment in patient's treatment and follow up

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    Guimarães Catarina Abraão

    2002-01-01

    Full Text Available Rasmussen's encephalitis is characterized by refractory epilepsy, neurological deterioration and progressive atrophy of one cerebral hemisphere. The objective of this study is to describe the importance of neuropsychological evaluation in the treatment decision and follow-up of patients with Rasmussen´s encephalitis. Neuropsychological assessment was performed in two steps. Firstly, the clinical history was obtained and the Vineland adaptative behavior scale (VABS was applied. After this first step, the patients with social maturity level equal or higher than the inferior limit underwent a battery of neuropsychological assessment. We evaluated three patients before any specific treatment was started, and six months after the intervention (surgery or plasmapheresis. Patient 1 underwent left hemispherectomy and had global improvement on second neuropsychological assessment. This suggests that the decision of performing surgery was adequate. Patients 2 and 3 underwent plasmapheresis. They did not present cognitive decline between both evaluations which suggest that our decision of postponing surgery was adequate as well. We conclude that neuropsychological assessment is important when evaluating patients with Rasmussen´s encephalitis. That is especially true for patients in whom disease progression is slow, and surgery timing has to be carefully planned.

  13. D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease.

    Science.gov (United States)

    Lee, Yeonhee; Lee, Sang Taek; Cho, Heeyeon

    2016-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with systemic vasculitis. The pathophysiology of ANCA-associated vasculitis (AAV) has not been clearly proven, and drug-induced ANCA-associated vasculitis has been reported. Wilson's disease is an inborn error of copper metabolism caused by a mutation in the copper transporting gene ATP7B, and traditional treatment is based on copper chelation with agents such as D-penicillamine. There have been rare reports that prolonged D-penicillamine therapy might cause adverse renal events such as membranous nephropathy and minimal change disease, but it is questionable if D-penicillamine induces ANCA-associated vasculitis. We describe 2 patients with Wilson's disease treated with D-penicillamine who presented with ANCA (+) vasculitis and renal involvement. The 2 patients also showed positive results for antinuclear antibody (ANA). Their kidney biopsy findings were compatible with crescentic/necrotizing glomerulonephritis, pauci-immune type. After diagnosis of AAV, D-penicillamine was stopped. Patients were then treated with plasmapheresis and immunosuppressants, including methylprednisolone pulse therapy and intravenous cyclophosphamide. One patient progressed to end-stage renal disease and the other showed persistent proteinuria. These cases suggest that D-penicillamine may induce ANA (+) ANCA (+) vasculitis with severe renal involvement in pediatric patients, and plasmapheresis combined with immunosuppressant should be considered.

  14. Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report [v1; ref status: indexed, http://f1000r.es/24q

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    Dino Mijatovic

    2014-03-01

    Full Text Available Introduction: Hemolytic-uremic syndrome (HUS is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS, toxic megacolon with ileus, pancreatitis, central nervous system (CNS disorders and multiple organ failure (MOF. Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. In the next week of, what initially appeared as typical HUS, she developed MOF, including ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.

  15. Current and emerging therapies for the treatment of myasthenia gravis

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    Renato Mantegazza

    2011-03-01

    Full Text Available Renato Mantegazza, Silvia Bonanno, Giorgia Camera, Carlo AntozziDepartment of Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico Carlo Besta, Milan, ItalyAbstract: Myasthenia gravis (MG is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.Keywords: myasthenia gravis, therapy, immunosuppression, thymectomy, plasmapheresis

  16. Refsum's Disease—Use of the Intestinal Lipase Inhibitor, Orlistat, as a Novel Therapeutic Approach to a Complex Disorder

    Directory of Open Access Journals (Sweden)

    Nimalie J. Perera

    2011-01-01

    Full Text Available Refsum's Disease is an inherited metabolic disorder in which a metabolite of branched chain fatty acids accumulates due to lack of appropriate oxidative enzymes. Patients have elevated plasma phytanic acid levels and high concentrations of phytanic acid in a variety of tissues leading to progressive tissue damage. Besides retinal degeneration or retinal dystrophy associated with adult onset retinitis pigmentosa, additional symptoms include chronic polyneuropathy, cerebellar ataxia, sensorineural hearing loss, anosmia, ichthyosis, as well as skeletal, cardiac, hepatic, and renal abnormalities. Current management includes avoidance of dietary sources of branched chain fatty acids and regular plasmapheresis to prevent accumulation of these compounds to ameliorate progressive neurological deficits. Two brothers with Refsum's disease who experienced progressive symptoms despite optimal diet and plasmapheresis were commenced on a novel therapy. We report the effect of the intestinal lipase inhibitor, Orlistat, which led to significant reduction (P-value <0.001 on 2-sample unpaired t-test of mean preplasmapheresis phytanic acid levels with retardation of the progression of most of their dermatological and neurological symptoms.

  17. Perioperative Desensitization Improves Outcomes Among Crossmatch Positive Recipients of Deceased Donor Renal Transplants.

    Science.gov (United States)

    Sharma, Amit; King, Anne; Kumar, Dhiren; Behnke, Martha; McDougan, Felecia; Kimball, Pamela M

    2016-06-01

    Graft failure due to chronic rejection is greater among renal transplant patients with donor-specific antibody (DSA) than among DSA-free patients. For patients dependent on deceased donor transplantation, preoperative desensitization to eliminate DSAs may be impractical. We speculated that perioperative desensitization might eliminate preexisting DSAs and prevent de novo DSAs and improve graft outcomes. We report that brief perioperative desensitization using either intravenous immunoglobulin (IVIG) or plasmapheresis/IVIG (PP/IVIG) treatment improves clinical outcomes among patients with positive crossmatches. Immediately following deceased donor transplantation, 235 renal recipients were assigned points for PRA and flow crossmatches (FCXM): delayed graft function (DGF) ≤ 1 point received standard therapy; 2 points received high-dose IVIG; and ≥3 points received PP/IVIG. The DSAs were serially monitored by single antigen bead luminex for 1 year. Five-year clinical outcomes were determined from the chart review. All desensitized patients had preoperatively positive FCXM with DSA. Rejection was more common (P desensitized than nonsensitized groups. However, overall graft survivals were similar between the groups (P = not significant) and superior to historic untreated patients (P 90% in all desensitizated patients with DSA elimination as well as PP/IVIG patients with residual DSA. In contrast, IVIG patients with persistent DSA had poorer graft survival (45%, P desensitization improved overall graft survival of sensitized patients compared to historic untreated patients. Plasmapheresis/IVIG had greater impact on DSA eradication and graft survival than IVIG alone. © 2016, NATCO.

  18. Tailor-made therapy for viral hepatitis: recent advances.

    Science.gov (United States)

    Kudo, Masatoshi

    2011-01-01

    Combination therapy of pegylated interferon-α with ribavirin (PEG-IFN/RBV) is a standard of care for chronic hepatitis C (CHC). The majority of CHC patients are infected with HCV genotype I. The recent discovery revealed by a genome-wide association study technology provides the important role of interleukin-28B (IL28B) and inosine triphosphatase (ITPA) in HCV infection. In addition, response to PEG-IFN/RBV therapy is correlated with insulin resistance, hepatic steatosis, and hepatic fibrosis in CHC patients. Double-filtration plasmapheresis together with IFN therapy has proved to be effective in the reduction of viral load during the early stage of treatment. In CHC patients, not only IL28B status, but also the treatment period of PEG-IFN/RBV is important. Even when new polymerase/protease inhibitors are introduced in the treatment of CHC, tailor-made treatment for CHC using IL28B, inosine triphosphatase testing or double-filtration plasmapheresis treatment prolonged treatment strategy is highly recommended. The relative etiologic role of prior hepatitis B virus infection in the development of non-B non-C hepatocellular carcinoma is also known in hepatitis B-endemic areas.

  19. Pulmonary Renal Syndrome After Streptococcal Pharyngitis

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    Gopi Mara-Koosham PhD

    2016-04-01

    Full Text Available Pulmonary renal syndrome is a class of small vessel vasculitides that are characterized by the dual presentation of diffuse alveolar hemorrhage (DAH and glomerulonephritis. Pulmonary renal syndrome has multiple etiologies, but its development has been rarely reported following infection with group A streptococcus. We present the case of a 36-year-old Native American male who was transferred to our facility due to refractory hypoxic respiratory failure. He had been diagnosed with streptococcal pharyngitis 2 weeks prior to admission. Given the presence of hemoptysis, bronchoscopy was performed and was consistent with DAH. Urinalysis demonstrated hematuria and proteinuria, in the setting of elevated creatinine and blood urea nitrogen. Additionally, antistreptolysin O titer was positive. Given the constellation of laboratory findings and history of streptococcal pharyngitis, the patient was diagnosed with PRS secondary to streptococcal infection. High-dose methylprednisolone was initiated with concomitant plasmapheresis. He was extubated successfully after his respiratory status improved and was eventually discharged home after making a full recovery within 2 weeks after admission. This case illustrates the importance of clinically relevant sequelae of streptococcal infection as well as the appropriate treatment of PRS secondary to streptococcal pharyngitis with plasmapheresis and intravenous corticosteroids.

  20. IVIg in other autoimmune neurological disorders: current status and future prospects.

    Science.gov (United States)

    Dalakas, Marinos

    2008-07-01

    A number of autoimmune disorders have been identified in which IVIg treatment may be beneficial. Evidence for the use of IVIg in inflammatory myopathies comes from controlled trials in dermatomyositis (DM) and sporadic-inclusion body myositis (s-IBM). In DM, muscle strength was increased and neuromuscular scores and skin rashes improved. Results for s-IBM have not been as encouraging as those observed for DM. Subsequently, IVIg should be recommended as a second-line therapy in DM and used for life-threatening dysphagia in s-IBM. Using an animal model of experimental autoimmune myasthenia gravis (MG), studies also indicate that IVIg can significantly improve clinical symptoms and affect pathogenic idiotypic antibodies. In human MG, studies indicate that IVIg exhibited equal efficacy compared to plasmapheresis. IVIg can therefore be recommended for use in an MG crisis or in lieu of plasmapheresis. The role of IVIg in the chronic management of MG has not been studied. IVIg has also been investigated in autoimmune CNS disorders. In a controlled study in patients with stiff person syndrome IVIg was effective, with improvements in the distribution of stiffness index and heightened sensitivity scores. For neurodegenerative diseases such as Alzheimer's disease, post-polio syndrome, pain, fibrosis, and autoimmune sleep disorders, some early promising results for the use of IVIg are emerging, but remain to be fully investigated. In conclusion, IVIg appears to be an effective treatment for a number of autoimmune disorders, however, optimal dosing and pharmacogenetic studies are necessary.

  1. Alleviating liver failure conditions using an integrated hybrid cryogel based cellular bioreactor as a bioartificial liver support

    Science.gov (United States)

    Damania, Apeksha; Hassan, Mohsin; Shirakigawa, Nana; Mizumoto, Hiroshi; Kumar, Anupam; Sarin, Shiv K.; Ijima, Hiroyuki; Kamihira, Masamichi; Kumar, Ashok

    2017-01-01

    Conventionally, some bioartificial liver devices are used with separate plasmapheresis unit to separate out plasma from whole blood and adsorbent column to detoxify plasma before it passes through a hepatocytes-laden bioreactor. We aim to develop a hybrid bioreactor that integrates the separate modules in one compact design improving the efficacy of the cryogel based bioreactor as a bioartificial liver support. A plasma separation membrane and an activated carbon cloth are placed over a HepG2-loaded cryogel scaffold in a three-chambered bioreactor design. This bioreactor is consequently connected extracorporeally to a rat model of acute liver failure for 3 h and major biochemical parameters studied. Bilirubin and aspartate transaminase showed a percentage decrease of 20–60% in the integrated bioreactor as opposed to 5–15% in the conventional setup. Urea and ammonia levels which showed negligible change in the conventional setup increase (40%) and decrease (18%), respectively in the integrated system. Also, an overall increase of 5% in human albumin in rat plasma indicated bioreactor functionality in terms of synthetic functions. These results were corroborated by offline evaluation of patient plasma. Hence, integrating the plasmapheresis and adsorbent units with the bioreactor module in one compact design improves the efficacy of the bioartificial liver device. PMID:28079174

  2. Late effects of childhood cancer treatment: severe hypertriglyceridaemia, central obesity, non alcoholic fatty liver disease and diabetes as complications of childhood total body irradiation.

    Science.gov (United States)

    Rajendran, R; Abu, E; Fadl, A; Byrne, C D

    2013-08-01

    Childhood cancer survivors may develop a number of endocrine complications linked to organ failure, such as hypogonadism, diabetes and growth hormone deficiency. However, increasing evidence now suggests that total body irradiation treatment, specifically, is linked with future risk of insulin resistance, hepatic steatosis and dyslipidaemia, possibly because total body irradiation affects adipocyte differentiation and impairs subcutaneous adipose tissue depot expansion during times of positive energy balance. We describe a 20-year-old woman who developed pancreatitis with severe hypertriglyceridaemia (serum triglycerides > 300 mmol/l) that required plasmapheresis. She had received total body irradiation prior to her bone marrow transplant at age 6 years for relapsed acute lymphoblastic leukaemia. She developed ovarian failure at age 12 years. At age 15 years she was noted to have hyperglycaemia, increased blood pressure, hepatic steatosis and mild hypertriglyceridaemia. She presented with severe hypertriglyceridaemia and eruptive xanthoma, and developed pancreatitis 12 h after admission. She was treated with plasmapheresis and intravenous insulin and made an excellent recovery. We implicate and discuss total body irradiation as the major contributing factor to her severe hypertriglyceridaemia, compounded by worsening glycaemic control, oestrogen deficiency and a changing adult lifestyle. Children who have received total body irradiation are at risk of diabetes and an exaggerated form of the metabolic syndrome with hypertriglyceridaemia, which can be life-threatening. We suggest that survivors of total body irradiation treatment require careful lifelong monitoring of their metabolic status. © 2013 The Authors. Diabetic Medicine © 2013 Diabetes UK.

  3. Role of proteasome inhibition in sensitized transplant candidates

    Institute of Scientific and Technical Information of China (English)

    Matthew J. Everly; Jason J. Everly; Paul I. Terasaki

    2011-01-01

    s from medical meetings related to transplant were also used.Study selection Articles were selected if they were trial results or case studies for the use of bortezomib in the sensitized patient population.Results The early data using bortezomib as a part of desensitization regimens has shown success. Although one cycle (4 doses) of bortezomib seems to have affect on many patients, it also seems likely that to provide complete desensitization multiple cycles will be required. Regarding safety, bortezomib has been shown to have minimal side effects. The most common side effects reported are those of thrombocytopenia and anemia. These side effects are dose related and self limiting upon discontinuation of the treatment.Conclusions Bortezomib with plasmapheresis is a promising new alternative to desensitization protocols that use either high dose intravenous immune globulin (IVIG) or Iow dose IVIG and plasmapheresis. The efficacy on antibody reduction looks to be batter that that of the IVIG based regimens without significant addition toxicity. The results of ongoing prospective trials are positive and their complete results are greatly anticipated.

  4. Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

    Directory of Open Access Journals (Sweden)

    Parissis Haralabos

    2010-09-01

    Full Text Available Abstract Background To report our experience of a rather uncommon drug interaction, resulting in hemolytic uremic syndrome (HUS. Methods Two consecutive cases of hemolytic uremic syndrome were diagnosed in our service. In both patients the use of macrolides in patients taking Tacrolimus, resulted in high levels of Tacrolimus. Results The first patient was a 48 years old female with Bilateral emphysema. She underwent Single Sequential Lung Transplantation. She developed reperfusion injury requiring prolonged stay. Tacrolimus introduced (Day 51. The patient remained well up till 5 months later; Erythromycin commenced for chest infection. High Tacrolimus levels and a clinical diagnosis of HUS were made. She was treated with plasmapheresis successfully. The second case was a 57 years old female with Emphysema & A1 Antithrypsin deficiency. She underwent Right Single Lung Transplantation. A2 rejection with mild Obliterative Bronchiolitis diagnosed 1 year later and she switched to Tacrolimus. She was admitted to her local Hospital two and a half years later with right middle lobe consolidation. The patient commenced on amoxicillin and clarithromycin. Worsening renal indices, high Tacrolimus levels, hemolytic anemia & low Platelets were detected. HUS diagnosed & treated with plasmapheresis. Conclusions There are 21 cases of HUS following lung transplantation in the literature that may have been induced by high tacrolimus levels. Macrolides in patients taking Cyclosporin or Tacrolimus lead to high levels. Mechanism of action could be glomeruloconstrictor effect with reduced GFR increased production of Endothelin-1 and increased Platelet aggregation.

  5. An unusual case of altered mental status in a young woman

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    Rama Mohana Rao Challapalli Sri

    2011-01-01

    Full Text Available Context : We describe a case of paraneoplastic neurologic syndrome, namely N-Methyl-D-Aspartic acid receptor antibody associated limbic encephalitis, a rare cause of altered mental status in the young. Case Report : A 28 year old Caucasian female nurse presented with acute onset difficulty with word finding and increasing confusion and agitation. She also had visual hallucinations, transient episodes of unresponsiveness, and lingual dyskinesias. Workup including blood, imaging and regular cerebrospinal fluid (CSF studies was unremarkable. She subsequently developed complex partial seizures. Computerized Tomography scan of chest/abdomen/pelvis revealed a dermoid cyst of the left ovary and CSF N-Methyl-D-Aspartic acid receptor antibody returned positive confirming the diagnosis of paraneoplastic NMDA receptor antibody associated limbic encephalitis. She was treated with methylprednisolone therapy along with plasmapheresis and a left salpingo-opherectomy was performed. The patient showed significant improvement with respect to her cognitive function and had no more seizures. Conclusion : N-Methyl-D-Aspartic acid receptor antibody associated limbic encephalitis is a rare paraneoplastic neurologic syndrome with symptoms including psychiatric manifestations, seizures, language disturbances and autonomic instability. It develops due to antibody induced decrease in N-Methyl-D-Aspartic acid receptors. There is a significant association with ovarian teratoma in >50% female cases. Treatment includes resection of tumor, glucocorticoids, plasmapheresis and Intravenous Immunoglobulin therapy.

  6. Necrotizing RPGN with linear anti IgG deposits in a patient with history of granulomatosis with polyangiitis: a case report

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    Parekh N

    2014-11-01

    Full Text Available Ninad Parekh, Edward Epstein, Suzanne El-Sayegh Department of Medicine, Division of Nephrology, Staten Island University Hospital, Staten Island, NY, USA Introduction: Diagnosing the etiology of a rapidly progressive glomerulonephritis is of vital importance to guide appropriate therapeutic management. This case highlights the complexity involved in establishing diagnosis when presentation is atypical. In certain cases diagnosis cannot be established based on clinical presentation or biopsy findings alone, and critical analysis of biopsy findings in context of clinical presentation is crucial to guide the clinical decision-making process.Case presentation: A 47-year-old Hispanic male with history of granulomatosis with polyangiitis (GPA in remission on azathioprine, presented with fatigue and lethargy. Physical examination was unremarkable. Laboratory data revealed elevated creatinine and otherwise normal electrolytes. Urinalysis showed numerous dysmorphic red blood cells with few red cell casts. His serologic results were all negative except anti-proteinase-3 antibody at very low titers. Kidney biopsy showed necrotizing crescentic glomerulonephritis with linear immunoglobulin G staining along the basement membrane.Conclusion: This case presented conflicting serologic and histopathologic findings. The presence of anti-proteinase-3 antibody supported diagnosis of recurrence of GPA. However, linear staining of immunoglobulin G (IgG on immunofluorescence (IF staining of renal biopsy supported anti-glomerular basement membrane (GBM disease. The treatment of anti-GBM disease and GPA both involve immunosuppression with prednisone and cyclophosphamide. However, patients with anti-GBM disease are also treated with plasmapheresis early in the disease presentation to prevent further damage. The patient with GPA, on the other hand, was shown to benefit from plasmapheresis only in the case of severe renal disease (serum creatinine level more than 5 mg/dL or

  7. Treatment of Severe Hypertriglyceridemia with Continuous Insulin Infusion

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    Yesica Rodríguez Santana

    2011-01-01

    Full Text Available Severe hypertriglyceridemia (SH represents a therapeutic emergency because of the possibility of developing cardiovascular events and hyperlipemic acute pancreatitis (PA. Most patients with SH suffer primary or genetic abnormality in lipid metabolism in combination with a precipitating factor such as uncontrolled diabetes mellitus, alcoholism, and drug intake. The standard treatment of hypertriglyceridemia (HTG with omega 3 fatty acids and fibrates, along with dietary changes, has no effect on an emergency situation. There are no clinical guidelines to SH, but therapy with insulin, heparin, a combination of both, plasmapheresis, or octreotide have been tested succesfully. We report the case of a 10-year-old girl with clinical acute pancreatitis and diabetic ketoacidosis debut, along with incidental finding of an SH, who had a good outcome after treatment with insulin intravenous infusion.

  8. [Acute hypertrygliceridemic pancreatitis].

    Science.gov (United States)

    Senosiain Lalastra, Carla; Tavío Hernández, Eduardo; Moreira Vicente, Victor; Maroto Castellanos, Maite; García Sánchez, Maria Concepción; Aicart Ramos, Marta; Téllez Vivajos, Luis; Cuño Roldán, José Luis

    2013-04-01

    Acute hypertriglyceridemic pancreatitis is the third cause of acute pancreatitis in the Western population. There is usually an underlying alteration in lipid metabolism and a secondary factor. Clinical presentation is similar to that of pancreatitis of other etiologies, but the course of acute hypertriglyceridemic pancreatitis seems to be worse and more recurrent. Some laboratory data can be artefacts, leading to diagnostic errors. This is the case of amylase, which can show false low levels. Treatment is based on intense fluidotherapy and analgesia. When there is no response to conservative management, other methods to lower triglyceride levels should be used. Several options are available, such as plasmapheresis, insulin, and heparin. The present article provides a review of the current literature on this entity.

  9. Recent Advances and Review on Treatment of Stiff Person Syndrome in Adults and Pediatric Patients.

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    Bhatti, Adnan Bashir; Gazali, Zarine Anwar

    2015-12-22

    Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Studies have shown that the paraneoplastic variant of SPS is more common in patients with breast cancer who harbour amphiphysin antibodies, followed by colon cancer, lung cancer, Hodgkin's disease, and malignant thymoma.  Currently, the treatment for SPS revolves around improving the quality of life by reducing the symptoms as far as possible with the use of GABAergic agonists, such as diazepam or other benzodiazepines, steroids, plasmapheresis, and intravenous immunoglobulin (IVIG). There have been random clinical trials with Rituximab, but nothing concrete has been suggested. A treatment approach with standard drugs and cognitive behavioral therapy (CBT) seems to be promising.

  10. Fulminant Hepatic Failure Secondary to Herpes Hepatitis in a Patient With Myasthenia Crisis: An Elusive Diagnosis.

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    Patel, Harish; Tariq, Hassan; Gaduputi, Vinaya; Vakde, Trupti; Makker, Jasbir; Daniel, Myrta

    2016-12-01

    Herpes hepatitis is a rare cause of fulminant hepatic failure contributing to less than 1% of all cases. It is most often seen in persons who are immunosuppressed and in pregnant women. The presentation is usually non-specific and rapidly progressive, thus making antemortem diagnosis of this condition rare. We present a patient who was on chronic immunosuppressive therapy for systemic lupus erythematosus and subsequently developed multi-organ failure with anicteric transaminitis as a result of disseminated herpes infection. The diagnosis was only made post-mortem. A confounding factor in this case was the fact that the patient underwent plasmapheresis, which skewed the interpretation of liver function tests in the setting of acute liver failure.

  11. INVESTIGATION OF A ROLE OF THE IMMUNOGENICITY OF INFLIXIMAB IN THE THERAPY OF ANKYLOSING SPONDYLITIS

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    O. A. Rumyantseva

    2016-01-01

    Full Text Available At present, there are a number of unsolved problems associated with unawareness of the causes and ways to prevent the inefficacy of tumor necrosis factor-α  inhibitors.Objective: to study the causes of secondary inefficacy of infliximab (INF, by analyzing its concentrations and antidrug antibody levels in the serum of ankylosing spondylitis (AS patients receiving long INF,  as well as a possibility to overcome its secondary inefficacy through plasmapheresis.Subjects and methods. 54 patients with active AS (BASDAI > 4 underwent regular long-term  (1-to-10-year treatment with INF 5 mg/kg according to the standard scheme. During the therapy blood samples were taken before a regular INF infusion to quantify the levels of antibodies to the drug and its concentration. According to the efficiency of the drug, two groups were formed: 1 27 (50% patients with INF inefficacy (an exacerbation occurred 2–4 weeks after infusion; 2 27 patients with drug efficacy. The levels of anti-double stranded DNA antibodies and antinuclear factor were estimated in 27 patients to investigate a relationship between the immunogenicity of INF and the presence of autoantibodies in its secondary inefficacy. A plasmapheresis session was carried out in 5 patients before a regular IFN infusion.Results and discussion. Anti-INF antibodies were found in 28 (52% patients, these being more common  in the patients with drug inefficacy than in the others (67 and 37%, respectively; p < 0.05. In the patients with INF inefficacy, anti-INF antibody levels were significantly higher than in those with preserved drug effect (18.33 and 4.67 U/ml, respectively; р < 0.05. Moreover, the serum concentration of INF was not significantly different in these groups (1.6 and 2.96 μg/ml. There was an inverse correlation  between INF concentrations and anti-INF antibodies (r = -0.7; p < 0.05. The level of autoantibodies  did not correlate with that of anti-INF antibodies. Following

  12. Neurological manifestations of Chikungunya and Zika infections

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    Talys J. Pinheiro

    Full Text Available ABSTRACT The epidemics of Chikungunya virus (CHIKV and Zika virus (ZIKV infections have been considered the most important epidemiological occurrences in the Americas. The clinical picture of CHIKV infection is characterized by high fever, exanthema, myalgia, headaches, and arthralgia. Besides the typical clinical picture of CHIKV, atypical manifestations of neurological complications have been reported: meningo-encephalitis, meningoencephalo-myeloradiculitis, myeloradiculitis, myelitis, myeloneuropathy, Guillain-Barré syndrome and others. The diagnosis is based on clinical, epidemiological, and laboratory criteria. The most common symptoms of ZIKV infection are skin rash (mostly maculopapular, fever, arthralgia, myalgia, headache, and conjunctivitis. Some epidemics that have recently occurred in French Polynesia and Brazil, reported the most severe conditions, with involvement of the nervous system (Guillain-Barré syndrome, transverse myelitis, microcephaly and meningitis. The treatment for ZIKV and CHIKV infections are symptomatic and the management for neurological complications depends on the type of affliction. Intravenous immunoglobulin, plasmapheresis, and corticosteroid pulse therapy are options.

  13. Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy.

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    Gharbi, Chems; Bourry, Edward; Rouvier, Philippe; Hacini, Sabria; Letaief, Ahmed; Baumelou, Alain; Izzedine, Hassane

    2010-10-01

    Although uncommon, thrombotic microangiopathy (TMA) is one of the most serious complications in patients with systemic lupus erythematosus. A 30-year-old black woman admitted to our hospital because of fever, fatigue, 'dark' urine and rapidly progressive renal failure was found to have systemic lupus erythematous and atypical hemolytic uremic syndrome. Kidney biopsy showed WHO class IV lupus nephritis with crescents and TMA. Hemodialysis was initiated for worsening renal failure. The patient was treated with corticosteroids, monthly pulse intravenous Cyclophosphamide, plasmapheresis and Rituximab on a weekly basis for 4 weeks. The patient's blood pressure was aggressively controlled using antihypertensive agents. Despite this extensive therapy, she remained dialysis dependent although hematological parameters returned to normal values.

  14. Immunoadsorption in patients with autoimmune ion channel disorders of the peripheral nervous system.

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    Antozzi, Carlo

    2013-01-01

    Autoimmune ion channel disorders of the peripheral nervous system include myasthenia gravis, the Lambert-Eaton myasthenic syndrome, acquired neuromyotonia and autoimmune autonomic ganglionopathies. These disorders are characterized by the common feature of being mediated by IgG autoantibodies against identified target antigens, i.e. the acetylcholine receptor, the voltage-gated calcium and potassium channels, and the neuronal acetylcholine receptor. Moreover, experimental animal models have been identified for these diseases that respond to immunotherapy and are improved by plasmapheresis. On this basis, autoimmune ion channel disorders represent the ideal candidate for therapeutic apheresis. Immunoadsorption can be the treatment of choice when intensive apheretic protocols or long-term treatments must be performed, in patients needing frequent apheresis to keep a stable clinical condition, in case of unresponsiveness to corticosteroids and immunosuppressive treatments, or failure with TPE or intravenous immunoglobulins, and in patients with severe contraindications to long-term corticosteroids.

  15. Severe gestational hypertriglyceridemia: A practical approach for clinicians

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    Wong, Bertha; Ooi, Teik C

    2015-01-01

    Severe gestational hypertriglyceridemia is a potentially life threatening and complex condition to manage, requiring attention to a delicate balance between maternal and fetal needs. During pregnancy, significant alterations to lipid homeostasis occur to ensure transfer of nutrients to the fetus. In women with an underlying genetic predisposition or a secondary exacerbating factor, severe gestational hypertriglyceridemia can arise, leading to devastating complications, including acute pancreatitis. Multidisciplinary care, implementation of a low-fat diet with nutritional support, and institution of a hierarchical therapeutic approach are all crucial to reduce maternal and fetal morbidity. To avoid maternal pancreatitis, close surveillance of triglycerides throughout pregnancy with elective hospitalization for refractory cases is recommended. Careful dietary planning is required to prevent neural and retinal complications from fetal essential fatty acid deficiency. Questions remain about the safety of fibrates and plasmapheresis in pregnancy as well as the optimal timing for induction and delivery of these women. PMID:27512474

  16. An unusual cause of fulminant Guillain-Barré syndrome: angel's trumpet.

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    Sevketoglu, Esra; Tatlı, Burak; Tuğcu, Betul; Demirelli, Yasar; Hatipoglu, Sami

    2010-11-01

    A 5-year-old previously healthy boy presented with typical findings of Guillain-Barré syndrome and unilateral tonic pupil. He was placed on mechanical ventilation for 35 days for respiratory failure. Plasmapheresis and two courses of intravenous immunoglobulin therapy were given to the patient, and he experienced stepwise recovery from his illness. This case of acute motor axonal neuropathy type Guillain-Barré syndrome is novel in that the cause was established as ingestion of a toxic solanaceous plant, angel's trumpet (Brugmansia suaveolens; syn. Datura suaveolens). Understanding the signs and symptoms of angel's trumpet toxicity can allow for early diagnosis and proper case management. Copyright © 2010 Elsevier Inc. All rights reserved.

  17. Crioglobulinemia mixta asociada a infección por hepatitis C y gangrena de miembros inferiores

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    Mariel Del Rio

    1997-10-01

    Full Text Available Cryoglobulinemia is a systemic disorder in which the blood contains inmunoglobulins that reversibly precipitate in the cold, giving rise to immune complex deposition that may cause small vessel vasculitis. A strong association with Hepatitis C has been made in the latest years. We describe a patient with hepatitis C and Cryoglobulinemia who wazzu developed one of the most severe crises with clinical manifestations of gangrene in feet and upper extremities. Patient ended with amputation of the lower limbs. His life was saved with plasmapheresis, use of frozen plasma, steroids, heparin and alfa interferon. In this article we report the case and we do a review of the literature on cryoglobulinemia. (Rev Med Hered 1997;8:183-188.

  18. Eyelid Necrosis and Secondary Cicatrical Ectropion Secondary to Levamisole-Associated Vasculitis.

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    Ramesh, Sathyadeepak; Sobti, Deepak; Mancini, Ronald

    2015-09-21

    A 56-year-old female presented with eyelid necrosis secondary to systemic levamisole-induced vasculitis. Skin biopsy revealed necrotic epidermis with small-vessel thrombosis, fibrinoid reaction, and neutrophilic infiltration of vessel walls in the dermis with +pANCA. She was treated with plasmapheresis and steroids. Six months later, she developed severe, symptomatic cicatrical ectropion with marked anterior lamellar shortage and middle lamellar contracture. Scar release in the middle lamellar plane with lateral tarsal strip procedures was performed, with full-thickness skin grafts from the upper eyelids. She remained fully epithelialized postoperatively with improvement in symptoms, although she incomplete graft take due to her eyelid necrosis and compromised dermal blood supply.

  19. Hashimoto's encephalopathy: four cases and review of literature.

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    Gul Mert, Gülen; Horoz, Ozden Ozgur; Herguner, M Ozlem; Incecik, Faruk; Yildizdas, R Dincer; Onenli Mungan, Neslihan; Yuksel, Bilgin; Altunbasak, Sakir

    2014-04-01

    Hashimoto's encephalopathy is a rare clinically heterogenous condition consisting of encephalopathy, seizures and variable neurological and psychiatric manifestations, accompanied by high titres of serum antithyroid antibodies. We described the clinical and laboratory findings of four children (aged 8-17 years) with Hashimoto's encephalopathy. The clinical features of three patients at presentation included refractory epilepsy, and confusion, and one patient presented with behavioral and cognitive changes. During their presentation, two of them were in euthyroid, and the others were in hypothyroid status. All patients manifested increased antithyroid antibodies. Two patients improved with steroid treatment. The others responded to plasmapheresis instead of corticosteroid treatment. Physicians' awareness of this complication is of great importance because most patients respond dramatically to the treatment.

  20. [TOTAL PARENTERAL NUTRITION IN A PREGNANT PATIENT WITH ACUTE PANCREATITIS AND LIPOPROTEIN LIPASE DEFICIENCY].

    Science.gov (United States)

    Contreras-Bolívar, Victoria; González-Molero, Inmaculada; Valdivieso, Pedro; Olveira, Gabriel

    2015-10-01

    We present a case of severe acute pancreatitis induced by hypertriglyceridemia secondary to lipoprotein lipase (LPL) deficiency in a pregnant patient with gestational diabetes, initially maneged with diet but it was later necessary to carry out artificial nutricional support measures: total parenteral nutrition. LPL deficiency might cause severe hypertriglyceridemia, repetition acute pancreatitis which is an unwieldy and severe situation during pregnancy. Acute familial hypertriglyceridemia pancreatitis accounts for 5% of cases, including LPL deficiency. The goal of treatment is to reach triglycerides levels below 500 mg/dl, being very low fat diet the treatment of choice, drugs or plasmapheresis techniques can also be associated. TPN enriched in ω3 fatty acids and glutamine was safe and effective in our patient with significant decrease in triglyceride levels.

  1. Successful simultaneous pancreas kidney transplantation from living-related donor against positive cross-match.

    Science.gov (United States)

    Sammartino, Cinzia; Pham, Thuy; Panaro, Fabrizio; Bogetti, Diego; Jarzembowski, Tomasz; Sankary, Howard; Morelli, Nicola; Testa, Giuliano; Benedetti, Enrico

    2004-01-01

    A positive pretransplant flow cytometry cross-match (FC-XM) allows precise identification of high-risk recipients vulnerable to hyperacute or accelerated rejection after transplantation. Living donor kidney transplant recipient candidates with positive cross-match have been successfully treated with a combination of plasmapheresis (therapeutic plasma exchange, TPEX) and intravenous immunoglobulin (IVIG), achieving conversion to negative cross-match and successful transplant. We report the first successful case of simultaneous pancreas kidney transplant (SPKT) from a living donor (LD) performed against an initially positive FC-XM, converted to negative using a protocol based on TPEX and IVIG in combination with antiCD20 monoclonal antibody. This strategy of overcoming the cross-match barriers in living donation may offer a chance of successful transplantation to highly sensitized candidates for SPKT, for whom cadaveric transplant is difficult to achieve.

  2. Gemcitabine-associated thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Zupancic, Melanie; Shah, Prabodh C; Shah-Khan, Farheen

    2007-07-01

    Gemcitabine-associated thrombotic thrombocytopenic purpura (TTP) is a rare complication of gemcitabine treatment with a incidence ranging from 0.015% to 1.4%. Clinically, this disease manifests as haemolytic anaemia, thrombocytopenia, and renal insufficiency; hypertension and neurological and pulmonary symptoms are also known complications. The risk of TTP increases as the cumulative dose of gemcitabine approaches 20,000 mg/m(2). The pathophysiology of this disease entity is unknown although several theories, involving both immune and non-immune mechanisms, have been proposed. The most effective treatment is discontinuation of gemcitabine, the provision of antihypertensive medications as needed, and consideration of plasmapheresis or use of immunoadsorption column in severe cases.

  3. A case of nearly mistaken AB para-Bombay blood group donor transplanted to a group 'O' recipient.

    Science.gov (United States)

    Townamchai, Natavudh; Watanaboonyongcharoen, Phandee; Chancharoenthana, Wiwat; Avihingsanon, Yingyos

    2014-10-31

    Unintentional ABO mismatch kidney transplantation can cause detrimental hyperacute rejection. We report the first successful ABO incompatible kidney transplantation from an AB para-Bombay donor to O recipient. At the initial evaluation, the donor's ABO type was discordance on the cell typing and serum typing, which typed to be 'O' as cell typing and 'AB' as serum typing. At the second investigation, it was confirmed that the donor had a unique, rare but not uncommon blood type AB para-Bombay which was incompatible with the recipient's blood group. The kidney transplantation was successfully performed by an ABO incompatible preconditioning, double filtration plasmapheresis (DFPP) and rituximab. The serum creatinine at 12 months post-transplantation was 1.3 mg/dL. The pathology of the kidney biopsy showed no signs of rejection.

  4. [Therapeutic plasma exchange: a review of the literature].

    Science.gov (United States)

    Andrulli, Simeone; Bigi, Maria Carla; Granata, Antonio; Cossu, Maria; Logias, Francesco

    2012-01-01

    Therapeutic plasma exchange is an extracorporeal plasmapheresis method for removing high-molecular-weight pathogens and toxins from the circulation. It can be indicated in many clinical conditions, both kidney-related and non-kidney-related. This review focuses on clinical trials related to Goodpasture syndrome, thrombotic thrombocytopenic purpura, and acute renal insufficiency due to multiple myeloma. It also discusses the difficulties and opportunities associated with the development of a randomized controlled multicenter study and of a web-based database. Finally, we report a summary of the risks and complications of therapeutic plasma exchange and how we can update the information on their frequency and seriousness by means of a longitudinal prospective multicenter study open to all centers performing the procedure.

  5. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant.

    Science.gov (United States)

    García Cosmes, Pedro; Fraile Gómez, Pilar; Lewczuk, Kamil; Rodríguez González, Marta; Ruiz Ferreras, Elena; Tabernero Fernández, Guadalupe

    2016-01-01

    Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimens. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+) who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease. Copyright © 2015 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  6. [Cryoglobulinemic vasculitis].

    Science.gov (United States)

    Blank, N; Lorenz, H-M

    2016-04-01

    Cryoglobulinemic vasculitis is a disease of the small arteries and affects the skin, joints, peripheral nerves, kidneys and other organs. Even small amounts of cryoglobulins can lead to severe impairment for the patient but the detection of such low amounts of cryoglobulins can be difficult. The causes of cryoglobulinemic vasculitis include monoclonal hematological diseases, autoimmune diseases and chronic infections. Therapy involves treatment of the underlying disease and glucocorticoids, rituximab and plasmapheresis depending on the severity of the vasculitis. Recognition of typical clinical symptoms is essential in order to initiate appropriate laboratory diagnostic procedures. Sometimes many investigations are necessary. In the absence of clinical trials treatment recommendations can often only be derived from registry data.

  7. A review of the use of biological agents for chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Stübgen, Joerg-Patrick

    2013-03-15

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is a group of idiopathic, acquired, immune-mediated inflammatory demyelinating diseases of the peripheral nervous system. A majority of patients with CIDP respond to "first-line" treatment with IVIG, plasmapheresis and/or corticosteroids. There exists insufficient evidence to ascertain the benefit of treatment with "conventional" immunosuppressive drugs. The inconsistent efficacy, long-term financial burden and health risks of non-specific immune altering therapy have drawn recurrent attention to the possible usefulness of a variety of biological agents that target key aspects in the CIDP immunopathogenic pathways. This review aims to give an updated account of the scientific rationale and potential use of biological therapeutics in patients with CIDP. No specific treatment recommendations are given. The discovery, development and application of biological markers by modern molecular diagnostic techniques may help identify drug-naïve or treatment-resistant CIDP patients most likely to respond to targeted immunotherapy.

  8. Acute Demyelinating Polyneuropathy after Lung Transplantation: Guillain-Barré Syndrome or Tacrolimus Toxicity?

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    Nirmal S. Sharma

    2014-01-01

    Full Text Available Guillain-Barré syndrome (GBS has been described after solid organ and bone marrow transplantation mostly due to viral infections and possibly calcineurin inhibitors. Incidence after bone marrow transplant is 0.3–0.7%, though incidence in other transplants is not well known. We present the first description of tacrolimus associated GBS in lung transplant recipients in the English language literature. The pathophysiology of tacrolimus-induced polyneuropathy is not known, but some have hypothesized that tacrolimus induces an inflammatory phenomenon by differential effects on T cell subsets. Diagnosis of association may be challenging and requires high index of suspicion. The optimal treatment of GBS-associated with tacrolimus after lung transplantation is unknown, although drug discontinuation may result in improvement in some patients, while some reports suggest that the use of IVIG and/or plasmapheresis may be helpful and safe in organ transplant recipients with severe symptoms.

  9. A wolf in another wolf's clothing.

    Science.gov (United States)

    Lamba, Amrit; Kapp, Meghan; Paueksakon, Paisit; Parekh, Parth J

    2017-01-01

    This case of infective endocarditis masquerading as mixed cryoglobulinemia in a man with a history of intravenous drug use (IVDU) and hepatitis C virus (HCV) highlights the importance of maintaining a broad differential and continually re-evaluating the working diagnosis as new information presents itself. The patient presented to an outside hospital and was treated for presumptive mixed cryoglobulinemia with corticosteroid therapy. When the patient did not improve, he was transferred to a tertiary care center for possible Rituximab and/or plasmapheresis. Further investigation revealed Enterococcus bacteremia with subsequent workup consistent with infective endocarditis (IE). This case highlights a diagnostic dilemma and demonstrates the importance of a thorough evaluation as it pertains to overlapping features of IE and mixed cryoglobulinemia.

  10. Strategies for building reference standards for autoantibodies

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    Joanna eSheldon

    2015-04-01

    Full Text Available Producing robust, certified, traceable reference material for autoantibody testing is a vital element in maintaining the validity of results that are generated in the daily clinical laboratory routine. This is a huge challenge because of the high number of variables involved in the detection and measurement of the autoantibodies. The production of such materials is time consuming and needs rigorous attention to detail; this is best achieved by an overarching independent body who will oversee the process in a not for profit manner.Much effort has been made to build international standards for quantitative and qualitative assays based on monoclonal antibodies, obtained from affinity purification and plasmapheresis. The big challenge is to respect individual differences in immune response to the same antigen. A promising ongoing initiative is the construction of pools with monospecific samples from different individuals.

  11. Progressive multifocal leucoencephalopathy in an immunocompetent patient with favourable outcome. A case report

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    Mørk Sverre J

    2010-05-01

    Full Text Available Abstract Background To report the clinical course of PML in an apparently immunocompetent patient treated with cidofovir. Case Presentation A 35-year-old immunocompetent man who developed progressive hemianopsia, aphasia, and limb weakness underwent repeated MRI scans of the brain, spinal fluid analyses, and brain biopsy. Before diagnosis was established based on brain biopsy, he was consecutively treated with methylprednisolone, acyclovir, ceftriaxone and plasmapheresis, but he deteriorated rapidly suggestive of the immune reconstitution inflammatory syndrome (IRIS. He started to recover two weeks after the initiation of treatment with cidofovir and has had no relapse at 3 1/2 years of follow-up. MRI has shown marked improvement. Conclusions PML should be considered in immunocompetent patients with a typical clinical course and MRI findings compatible with PML. Treatment with cidofovir should be considered as early as possible in the disease course.

  12. Moxifloxacin (Avelox Induced Thrombotic Thrombocytopenic Purpura

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    Sikander P. Surana

    2012-01-01

    Full Text Available We report a case of a 66-year-old African-American female who presented with complaints of progressively worsening weakness, shortness of breath on minimal exertion, lethargy for the last few days, and short episodes of aphasia lasting 20–30 seconds. Prior to presentation, she was treated with two courses of moxifloxacin for sinusitis. Laboratory examination was remarkable for anemia and thrombocytopenia with elevated lactate dehydrogenase and no evidence of renal failure. Peripheral smear showed numerous schistocytes and she was diagnosed with thrombotic thrombocytopenic purpura. Moxifloxacin was identified as the offending agent. The patient was treated with prednisone and plasmapheresis. To the best of our knowledge, this is the first reported case of thrombotic thrombocytopenic purpura associated with the use of moxifloxacin. Although rare, physicians should be aware of this serious complication associated with its use.

  13. Diffuse Alveolar Hemorrhage: A Rare Life-Threatening Condition in Systemic Lupus Erythematosus

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    Ravi Paul Singh Virdi

    2012-01-01

    Full Text Available Diffuse alveolar hemorrhage (DAH is a rare life-threatening complication in systemic lupus erythematosus (SLE associated with high mortality rates. DAH is more common in women, and mean age of onset is around 30 years. It mostly occurs in patients with established diagnosis of SLE but can be the initial presentation of lupus in approximately 20%. DAH should be suspected in lupus patient presenting with new pulmonary infiltrates, decline in hemoglobin, hemoptysis, dyspnea, hypoxemia, and increase in carbon monoxide diffusion capacity. Radiographic evidence of bilateral pulmonary alveolar infiltrates that are usually perihilar or basilar with sparing of apices is seen. DAH can often mimic clinically and radiologically severe pneumonia or ARDS. Treatment includes high-dose corticosteroids, cyclophosphamide, and plasmapheresis. We report a case of diffuse alveolar hemorrhage complicating SLE flare-up in a male patient.

  14. Acute transverse myelitis and subacute thyroiditis associated with dengue viral infection: A case report and literature review

    Science.gov (United States)

    Mo, Zhiming; Dong, Yaxian; Chen, Xiaolian; Yao, Huiyan; Zhang, Bin

    2016-01-01

    Acute transverse myelitis is a rare manifestation of dengue infection. To the best of our knowledge, only 6 cases of acute transverse myelitis as a manifestation of dengue infection have been reported thus far. The present study described a case of acute transverse myelitis complicated with subacute thyroiditis 6 days after the onset of dengue viral infection. In addition, the available literature was searched to identify similar previous cases. Treatment with intravenous pulse methylprednisolone immunoglobulin plasmapheresis and physiotherapy resulted in partial recovery at 3 months post-infection. In conclusion, the involvement of dengue infection should be considered in patients who develop central nervous system manifestations during or after the recovery period of dengue infection. Furthermore, since methylprednisolone and immunoglobulin are effective during the active phase of the infection, prompt diagnosis and initiation of treatment are crucial. PMID:27703498

  15. Efficacy of Puls-therapy with Glucocorticoids in Patients with Autoimmune Ophtalmopatay

    Directory of Open Access Journals (Sweden)

    E V Bogomazova

    2008-09-01

    Full Text Available The article is presents the results of estimation efficiency of various schemes pathogenetic glucocorticoids treatment of autoimmune ophthalmopathy. Research of clinical parameters dynamics including subjective and objective attributes, proptosis, dynamics of the soluble form of intercellular molecule adhesion-1 levels was made before treatment and at the end of research (in 6 month follow up after complete course of pathogenetic treatment. The study has demonstrated that combination of glucocorticoids pulse-therapy and mean volume plasmapheresis is the most effective pathogenetic method of autoimmune ophthalmopathy treatment; absence of advantages of the used schemes of treatment for reduction proptosis. Advantages of use of the combined therapy are marked in comparison with monotherapy by glucocorticoids at estimation of subjective attributes.

  16. A combination therapy to treat second-degree anti-Ro/La-related congenital heart block: a strategy to avoid stable third-degree heart block?

    Science.gov (United States)

    Ruffatti, A; Milanesi, O; Chiandetti, L; Cerutti, A; Gervasi, M T; De Silvestro, G; Pengo, V; Punzi, L

    2012-05-01

    While mainly based on the use of fluorinated steroids, there is no standard management of anti-Ro/La-related congenital heart block (CHB). This is a report concerning two consecutive cases of anti-Ro/La-related second-degree block treated with betamethasone (4 mg/day), weekly plasmapheresis, and intravenous immunoglobulins (IVIGs; 1 g/kg) administered every 15 days, a therapy that was begun shortly after CHB was detected and continued until delivery. The newborns were also treated with IVIG (1 g/kg) soon after birth and continued fortnightly until the anti-Ro/La antibody levels became undetectable. In both cases second-degree AV block reverted to a stable sinus rhythm with a first-degree atrioventricular (AV) block. Moreover, there was no recurrence of CHB when therapy was suspended, as confirmed by a 29 month and an eight month follow-up, respectively.

  17. Pathogenetic substantiation of extracorporeal detoxication in surgery.

    Science.gov (United States)

    Ender, L A; Lekhtman, A M; Lobakov, A I

    1990-01-01

    The report is based on the results of treatment of 116 patients with severe purulent-destructive abdominal cavity diseases and 320 patients with liver failure due to bile ducts obturation. Along with surgical operations, methods of organism extracorporeal detoxication-haemosorption, exchange plasmapheresis, autoblood ultraviolet irradiation--were used in all patients either in isolation or in combination with each other. Toxical metabolites concentration, blood integral toxicity, peptids with mean molecular weight concentration, liver profile enzimes activity, lipid metabolism indices, general and liver haemodynamics and electroencephalogram were studied. On the basis of correlations between indices obtained by the method of extreme grouping factors-syndromes were formed, and then in accordance with a special program summary evaluation of detoxication was calculated in marks. A drop in lethality by 28.3% was ensured, as well as a drop in postoperatyonal liver failure by 51.4%.

  18. Potential biomarkers for monitoring therapeutic response in patients with CIDP.

    Science.gov (United States)

    Dalakas, Marinos C

    2011-06-01

    Although the majority of patients with CIDP variably respond to intravenous immunoglobulin (IVIg), steroids, or plasmapheresis, 30% of them are unresponsive or insufficiently responsive to these therapies. The heterogeneity in therapeutic responses necessitates the need to search for biomarkers to determine the most suitable therapy from the outset and explore the best means for monitoring disease activity. The ICE study, which led to the first FDA-approved indication for IVIg in CIDP, has shown that maintenance therapy prevents relapses and axonal loss. In this paper, the multiple actions exerted by IVIg on the immunoregulatory network of CIDP are discussed as potential predictors of response to therapies. Emerging molecular markers, promising in identifying responders to IVIg from non-responders, include modulation of FcγRIIB receptors on monocytes and genome-wide transcription studies related to inflammatory mediators, demyelination, or axonal degeneration. Skin biopsies, Peripheral Blood Lymhocytes, CSF, and sera are accessible surrogate tissues for further exploring these molecules during therapies.

  19. Treatment of myasthenia gravis with dropped head: a report of 2 cases and review of the literature.

    Science.gov (United States)

    Tamai, Michihiro; Hashimoto, Takao; Isobe, Takashi; Sato, Hiromasa; Doden, Tadashi; Nakano, Takeshi

    2015-05-01

    We report two patients with myasthenia gravis (MG) who showed dropped head as an early myasthenic manifestation. They had elevated anti-acetylcholine receptor antibody and showed improvement of the symptoms after intravenous injection of edorophonium chloride. One patient had thymoma and developed myasthenic crisis two weeks after thymectomy. The patient recovered from the crisis after a combination of immunoadsorption plasmapheresis (IAPP) and initiation of steroid and tacrolimus. The other patient without thymoma initiated treatment with steroid, tacrolimus and IAPP and showed complete recovery one month later. Dropped head in MG can recover well with immunosuppression therapy using steroid, and IAPP is helpful in getting a rapid improvement of dropped head as well as recovery from myasthenic crisis. When we consider treatment for MG with dropped head, we should take into account that MG of this type can develop myasthenic crisis and use the same treatment strategy as that for generalized MG.

  20. Effect of intravenous immunoglobulin in Guilain-Barre syndrome, myasthenia gravis and chronic idiopathic demyelinative polyneuropathy, A survey in Imam Khomeini Hospital

    Directory of Open Access Journals (Sweden)

    Qaffarpoor M

    1999-09-01

    Full Text Available With retrospective evaluation of 44 patients suffering from Guilan-Barre Syndrome (GBS, Chronic Idiopathic Demtyelinative Polyradiculoneuropathy (CIDP and Myasthenia Gravis (MG treated with intravenous immunoglobulin, we found following results: 1 Initial symptoms of improvement on forth or fifth days. 2 Maximum recovery for CIDP and MG were after 16-24 and 3-11 days, respectively. 3 No major complication, but mild side effects in 32% of patients. 4 In patients with GBS one grade improvement achieved after 8-30 days. 5 Intravenous immunoglobulin (IVIG plus plasmapheresis had no advantages over IVIG alone. 6 No reasonable conclusion about relapsing rate and duration of response due to follow up restrictions.

  1. The Forgotten Guide Wire: A Rare Complication of Hemodialysis Catheter Insertion

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    Said Abuhasna

    2011-01-01

    Full Text Available A rare complication of a hemodialysis catheter insertion is the loss of the complete guide wire into the circulation. A complete guide wire in the circulation may not necessarily produce symptoms, and it may remain unnoticed for a significant period of time. We present a rare case where a complete guide wire was lost into the circulation during insertion of a hemodialysis catheter into the right femoral vein in a 19-year-old female with systemic lupus erythromatosis. The patient remained asymptomatic through two plasmapheresis treatments over a period of 2 days. The guide wire was eventually retrieved without complications. The factors leading to the wire being forgotten will be reviewed, and the measures initiated to prevent any future occurrence will be addressed.

  2. Bone marrow processing on the Haemonetics V50 cell separator.

    Science.gov (United States)

    Anderson, N A; Cornish, J M; Godwin, V; Gunstone, M J; Oakhill, A; Pamphilon, D H

    1990-01-01

    We have processed 27 bone marrow (BM) harvests using the Haemonetics V50 cell separator with a paediatric plasmapheresis set and programmed for lymphocyte collection. The mean starting volume of 843 mL was processed in 6-8 cycles to a buffy coat (BC) with a mean volume of 230 mL. The mean starting mononuclear cell (MNC) count was 1.22 x 10 8/kg recipient weight, and recovery was 92%. Clonogenic potential of the BC was assessed using CFU-GM assays and recovery was measured after cryopreservation or purging. On 4 occasions where major ABO incompatibility existed between donor and recipient, both BM and BC were consecutively diluted in compatible blood and processed twice. This achieved a calculated reduction in donor erythrocytes of 98%. The procedure was efficient and yielded a BC fraction suitable for cryopreservation and purging. Adequate stem-cells were retained as verified by CFU-GM assays and documentation of stable engraftment.

  3. Guillain-Barré syndrome and ethylene diamine tetraacetic acid-dependent pseudothrombocytopenia associated with mumps.

    Science.gov (United States)

    Sawazaki, A; Nakamura, N; Jyokaji, H; Minami, S; Nakamura, S; Matsuda, T

    1996-12-01

    A 35-year-old man with Guillain-Barré syndrome and ethylene diamine tetraacetic acid (EDTA)-dependent pseudothrombocytopenia associated with serologically-confirmed mumps is presented. A polyneuropathy developed 18 days after the onset of mumps, that improved with plasmapheresis. A decreased platelet count was observed 25 days after the onset of mumps attributed to platelet agglutination in blood anticoagulated with EDTA, and the agglutination was prevented by other anticoagulants. Guillain-Barré syndrome associated with mumps is rare and EDTA-dependent pseudothrombocytopenia following mumps has never been documented. This represents the first report of Guillain-Barré syndrome and EDTA-dependent pseudothrombocytopenia following mumps. Furthermore, our findings suggest activation of the humoral immune response as a potential pathogenesis.

  4. Miller-Fisher syndrome associated with chronic lymphocytic leukemia

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    Aki Zeynep

    2008-01-01

    Full Text Available Chronic lymphocytic leukemia (CLL is a frequent hematological malignancy, with meningeal or peripheral nerve infiltrations being the most commonly encountered neurological complications. In this report, we describe a CLL patient with Miller-Fisher syndrome (MFS who responded to immune modulation with plasmapheresis. A 47-year-old man diagnosed as B-cell CLL admitted with neutropenic fever. He complained of diplopia and numbness of both arms. Neurological examination revealed a bilateral external ophthalmoplegia, dysphagia, dysarthria, mild shoulder girdle muscle weakness and gait ataxia, accompanied by absent tendon reflexes. Nerve conduction studies were indicative of a predominantly axonal sensori-motor peripheral neuropathy. This association of CLL with MFS had not been previously reported in the literature.

  5. Myelodysplastic Syndrome Clinically Presenting with the “Classic TTP Pentad”

    Science.gov (United States)

    Polanco Jácome, Evelyn Carolina; Guevara, Elizabeth; Mattoo, Vijay

    2017-01-01

    The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving. The following case illustrates an unusual presentation of MDS in a patient who came in to the emergency room with the classic TTP “pentad” of fever, renal involvement, MAHA, mental status changes, and thrombocytopenia. We will focus our discussion in the clinical presentation of this case. PMID:28255478

  6. Antiphospholipid syndrome:a survey of clinical characters in ten cases

    Institute of Scientific and Technical Information of China (English)

    陈李华; 姜玲玲; 厉有名; 彭清璧

    2003-01-01

    Objective: To gain further understanding of the antiphospholipid syndrome(APS) . Methods : Analysing clinical and laboratory data on ten cases of APS. Results: Thrombocytopenia appeared in all cases.Venous thrombi of limbs appeared in five eases and neurological abnormalities in two cases. Renal impairments were found in three cases. One case manifested left renal venous thrombi and the other two cases thrombotic microangiopathy. Budd-Chiari syndrome was found in one case. One of the ten cases was catastrophic APS(CAPS) presented as acute diffuse swelling, cyanosis, pain, ischemia and necrosis in fingers and limbs,recurrem shock, ascites, hepatic and respiratory dysfunction. Anticoagulams and corticosteroids could be effective for dealing with APS. It was critical to treat catastrophic APS with anticoagulants or plasmapheresis as early as possible. Conclusions: APS shows variable manifestations for good prognosis, but catastrophic APS has fatal risk. The main treatment for APS is the use of anticoagulams and immunosuppressives.

  7. Antiphospholipid syndrome:a survey of clinical characters in ten cases

    Institute of Scientific and Technical Information of China (English)

    陈李华; 姜玲玲; 厉有名; 彭清璧

    2003-01-01

    Objective: To gain further understanding of the antiphospholipid syndrome(APS). Methods: Analysing clinical and laboratory data on ten cases of APS. Results: Thrombocytopenia appeared in all cases. Venous thrombi of limbs appeared in five cases and neurological abnormalities in two cases. Renal impairments were found in three cases. One case manifested left renal venous thrombi and the other two cases thrombotic microangiopathy. Budd-Chiari syndrome was found in one case. One of the ten cases was catastrophic APS(CAPS) presented as acute diffuse swelling, cyanosis, pain, ischemia and necrosis in fingers and limbs, recurrent shock, ascites, hepatic and respiratory dysfunction. Anticoagulants and corticosteroids could be effective for dealing with APS. It was critical to treat catastrophic APS with anticoagulants or plasmapheresis as early as possible. Conclusions: APS shows variable manifestations for good prognosis, but catastrophic APS has fatal risk. The main treatment for APS is the use of anticoagulants and immunosuppressives.

  8. Successful isolated liver transplantation in a child with atypical hemolytic uremic syndrome and a mutation in complement factor H.

    Science.gov (United States)

    Haller, W; Milford, D V; Goodship, T H J; Sharif, K; Mirza, D F; McKiernan, P J

    2010-09-01

    A male infant was diagnosed with atypical hemolytic uremic syndrome (aHUS) at the age of 5.5 months. Sequencing of the gene (CFH) encoding complement factor H revealed a heterozygous mutation (c.3644G>A, p.Arg1215Gln). Despite maintenance plasmapheresis he developed recurrent episodes of aHUS and vascular access complications while maintaining stable renal function. At the age of 5 years he received an isolated split liver graft following a previously established protocol using pretransplant plasma exchange (PE) and intratransplant plasma infusion. Graft function, renal function and disease remission are preserved 2 years after transplantation. Preemptive liver transplantation prior to the development of end stage renal disease is a valuable option in the management of aHUS associated with CFH mutations.

  9. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Zahra Habibagahi

    2015-07-01

    Full Text Available Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.

  10. Neuropsychiatric Systemic Lupus Erythematosus

    Science.gov (United States)

    Popescu, Alexandra; Kao, Amy H

    2011-01-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) is the least understood, yet perhaps the most prevalent manifestation of lupus. The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic and autoantibody-mediated neuronal injury. The management of NPSLE is multimodal and has not been subjected to rigorous study. Different treatment regimens include nonsteroidal anti-inflammatory drugs, anticoagulation, and immunosuppressives such as cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate. For refractory NPSLE, intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab have been used. Adjunctive symptomatic treatment complements these therapies by targeting mood disorders, psychosis, cognitive impairment, seizures or headaches. Several new biological agents are being tested including Belimumab, a human monoclonal antibody that targets B lymphocyte stimulator. This review focuses on the pathophysiology, treatment, and new potential therapies for neuropsychiatric manifestations of systemic lupus erythematosus. PMID:22379459

  11. The use of electroconvulsive therapy in a patient with juvenile systemic lupus erythematosus and catatonia.

    Science.gov (United States)

    Mon, T; L'ecuyer, S; Farber, N B; White, A J; Baszis, K W; Hearn, J K; Spiegel, T E; French, A R; Kitcharoensakkul, M

    2012-12-01

    Catatonia is a rare manifestation in patients with systemic lupus erythematosus (SLE). As catatonia can be associated with both psychiatric and organic conditions, this could create a diagnostic dilemma once this occurs in SLE patients. The report describes a 15-year-old female with SLE who developed catatonia three days after the diagnosis of SLE was made. Her catatonia was refractory to the treatment with immunosuppressive therapy, which included pulse methylprednisolone, intravenous cyclophosphamide, rituximab, intravenous immunoglobulin (IVIG) and plasmapheresis. Given her persistent catatonia, electroconvulsive therapy (ECT) was initiated three months after the onset of her symptoms. After the third ECT treatment, her mental status dramatically improved and returned nearly to baseline while she was continued on the immunosuppression. This is the first report of a successful ECT therapy in catatonic lupus in children.

  12. [10 years' of production and use of human rabies immunoglobulin in Yugoslavia].

    Science.gov (United States)

    Romić, M; Tomović, O; Medić, P; Pelević, S; Sindić, M; Popović, M; Gligorović, V; Bogdanović, G; Mitrović, M; Petrović, M; Stankov, S; Lazarević-Ivanc, L; Lalosević, V; Lalosević, D

    2001-01-01

    Application of the rabies immunoglobuline is a compulsory part of the prophylaxis of rabies in all severe, transdermal lesions caused by rabies infected animals. Sylvatic rabies has spread in the past few years throughout the whole Yugoslavia, and human cases of rabies have also been reported in other East European countries. In order to achieve the highest level of rabies prophylaxis, apart from postinfective rabies vaccination, it is necessary to provide passive immunization using specific antibodies against rabies. After successful immunization of the young, healthy volunteers in 1990, National Blood Transfusion Institute, in cooperation with the Pasteur Institute from Novi Sad, prepared the first quantities of immunized plasma by plasmapheresis procedure and human rabies immunoglobuline. Without national production, sufficient quantities of human rabies immunoglobuline could not be provided, since the price on the world market is rather high (over $1000 per patient).

  13. Relapse of HCV Genotype 1b Infection After Sofosbuvir/Ledipasvir Treatment Presenting as De Novo Cryoglobulinemic Vasculitis

    Science.gov (United States)

    Moreno, Alan D.; Joseph, Nora; Kim, George; Fimmel, Claus J.

    2017-01-01

    Relapse of hepatitis C virus (HCV) genotype 1 infection after combination therapy with sofosbuvir and ledipasvir is unusual. We report a treatment-naïve, non-cirrhotic patient in whom the relapse of genotype 1b HCV infection was accompanied by de novo cryoglobulinemic vasculitis and glomerulonephritis, requiring hemodialysis for acute renal failure. Sequence analysis revealed several resistance-associated variants in the HCV NS5a gene but not in NS3/4A. The patient’s vasculitis was successfully treated with immunosuppression and plasmapheresis, followed by retreatment of HCV with a combination of sofosbuvir, simeprevir, and ribavirin. The patient achieved sustained virological response, recovered his renal function, and remains in remission from cryoglobulinemia. PMID:28184378

  14. [Immune-mediated neuropathies].

    Science.gov (United States)

    Stoll, G; Reiners, K

    2016-08-01

    The Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are the most common immune-mediated polyneuropathies, which can show variable clinical and electrophysiological manifestations. Rarer immune-mediated neuropathies encompass paraproteinemic neuropathies (PPN), multifocal motor neuropathy (MMN) and vasculitic neuropathies. The diagnosis usually relies on the history of symptom evolution, distribution of nerve dysfunction and particularly on characteristic features in nerve conduction studies, aided by cerebrospinal fluid (CSF) examination and nerve biopsy findings. The therapeutic toolbox encompasses corticosteroids, immunoglobulins and plasmapheresis often accompanied by long-term immunosuppression. It is important to note that immune-mediated neuropathies selectively respond to treatment and contraindications need to be considered. Despite treatment a considerable number of patients suffer from permanent neurological deficits.

  15. A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

    Directory of Open Access Journals (Sweden)

    Helen Chioma Okoh

    2015-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS variant of Guillain-Barré syndrome (GBS as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG but with remarkable and complete recovery after the second 5-day course of IVIG.

  16. [Treatment of multiple sclerosis symptoms and exacerbations].

    Science.gov (United States)

    Prieto González, José María

    2014-12-01

    In the last few years, there has been an explosion of new drugs acting on the clinical course of multiple sclerosis (MS) but less attention has been paid to better knowledge of the symptoms of this disease and their pathogenesis and treatment, which is essential to improve patients' quality of life. Because many patients have numerous concurrent symptoms during their clinical course, their management is complex and consequently it is important to know which symptoms are a direct result of the degenerative lesions of MS. The present article describes all the therapeutic options available for spasticity and its associated pain, paroxystic symptoms, fatigue, genitourinary disorders and sexual dysfunction, tremor, ataxia, gait disorder and cognitive impairment, with special emphasis on novel treatments. The article also defines exacerbations, how to recognize them and the available treatments, mainly oral administration of high-dose methylprednisolone and plasmapheresis. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  17. [Autoimmune channelopathies].

    Science.gov (United States)

    Michaud, M; Delrieu, J; Astudillo, L

    2011-12-01

    Autoimmune channelopathies are rare neuromuscular diseases that have been characterized clinically for several decades but for which the evidence of associated antibodies has only been recently demonstrated. Ion channels have an important role of activation, inhibition and regulation in neuromuscular transmission. Myasthenia gravis, generally associated with the presence of anti-acetylcholine receptor antibody, is the best-known channelopathy. Other anti-channel antibodies, including voltage-dependent, are associated with several neurological diseases, as illustrated by anti-voltage-gated calcium channels found in Lambert-Eaton myasthenic syndrome and paraneoplastic cerebellar ataxia, and anti-voltage-gated potassium channels found in neuromyotonia, Morvan's syndrome and limbic encephalitis. The treatment of autoimmune channelopathies is logically based on corticosteroids, immunosuppressant drugs, intravenous immunoglobulins and plasmapheresis. Copyright © 2011 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  18. Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

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    Kim Ma

    2016-01-01

    Full Text Available Warm autoimmune hemolytic anemia (wAIHA is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60–80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA.

  19. Síndromes hemorrágicas pulmonares Pulmonary hemorrhage syndromes

    Directory of Open Access Journals (Sweden)

    Eduardo da Rosa Borges

    2005-07-01

    Full Text Available As síndromes hemorrágicas pulmonares caracterizam-se por infiltrado pulmonar bilateral, queda dos níveis de hemoglobina e hipoxemia. Dentre as causas de sangramento estão as infecções, vasculites, coagulopatias e doenças do colágeno. A terapêutica consiste do tratamento da doença causal e suporte ventilatório, podendo ser associada a plasmaferese.Pulmonary hemorrhage syndromes are characterized by bilateral pulmonary infiltrates, decreased serum levels of hemoglobin, and hypoxemia. The causes of pulmonary hemorrhage include: infections, vasculitis, coagulopathies and collagen diseases. The therapy consists of treating the underlying disease and providing ventilatory support. In some cases, performing plasmapheresis can be beneficial.

  20. An unusual cause of type 2 respiratory failure

    Directory of Open Access Journals (Sweden)

    Srinivas Rajagopala

    2015-01-01

    Full Text Available We present a female patient who was referred for management of respiratory failure. She was being evaluated and managed as worsening chronic inflammatory demyelinating polyneuropathy with type 2 respiratory failure. Initial examination showed hypertrichosis, clubbing and papilledema along with severe distal and proximal motor-predominant weakness with impending respiratory failure. She was managed with noninvasive ventilation (NIV and plasmapheresis awaiting diagnostic investigations. Immunofixation showed an "M band" and free lambda chain levels were elevated. Radiographs showed the classic osteosclerotic lesions of POEMS (polyradiculoneuropathy, organomegaly, endocrinopathy, M-protein and Skin abnormalities syndrome. Six weeks after commencing radiotherapy to the osteosclerotic lesions, the patient responded favorably and remains off nocturnal NIV support.

  1. Treatment of pediatric chronic inflammatory demyelinating polyneuropathy: Challenges, controversies and questions.

    Science.gov (United States)

    Desai, Jay; Ramos-Platt, Leigh; Mitchell, Wendy G

    2015-01-01

    Pediatric chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon acquired disorder of unknown cause, presumed to have an immunological basis. We report 20 patients seen at Children's Hospital Los Angeles over a period of 10 years. The outcome of our patients was favorable in a vast majority with good response to various treatments instituted. However, residual neurologic deficit was common. The choice of treatment modality was empirical and selected by the treating neurologist. Intravenous immunoglobulin (IVIG) and corticosteroids were most commonly utilized for treatment. Plasmapheresis, mycophenolate mofetil, rituximab, cyclophosphamide, azathioprine, and abatacept were added if the patients were refractory to IVIG or became corticosteroid dependent. The spectrum of disease severity ranged from a single monophasic episode, to multiphasic with infrequent relapses with good response to IVIG, to progressive disease refractory to multiple therapies.

  2. Specific features of chronic inflammatory demyelinating polyneuropathy in children

    Directory of Open Access Journals (Sweden)

    A. L. Kurenkov

    2012-01-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is an autoimmune peripheral neuropathy that affects both adults and children. The basis for the paper is the analysis of 5 cases of CIDP in children (3 girls and 2 boys aged 5 to 17 years, followed up for 3 to 6 years. The types of its clinical picture and electromyographic changes at different disease stages are considered in detail. The course of the disease is traced during therapy with corticosteroids and intravenous human immunoglobulin and plasmapheresis. The results of the authors’ observations are compared with those of investigations conducted by other authors. The consideration of the diagnosis of CIDP and its treatment options focuses on that the international standards must be necessarily met to minimize errors in its differential diagnosis and management of these patients, and to make the prognosis for the disease.

  3. Treatment of pediatric chronic inflammatory demyelinating polyneuropathy: Challenges, controversies, and questions

    Directory of Open Access Journals (Sweden)

    Jay Desai

    2015-01-01

    Full Text Available Pediatric chronic inflammatory demyelinating polyneuropathy (CIDP is an uncommon acquired disorder of unknown cause, presumed to have an immunological basis. We report 20 patients seen at Children′s Hospital Los Angeles over a period of 10 years. The outcome of our patients was favorable in a vast majority with good response to various treatments instituted. However, residual neurologic deficit was common. The choice of treatment modality was empirical and selected by the treating neurologist. Intravenous immunoglobulin (IVIG and corticosteroids were most commonly utilized for treatment. Plasmapheresis, mycophenolate mofetil, rituximab, cyclophosphamide, azathioprine, and abatacept were added if the patients were refractory to IVIG or became corticosteroid dependent. The spectrum of disease severity ranged from a single monophasic episode, to multiphasic with infrequent relapses with good response to IVIG, to progressive disease refractory to multiple therapies.

  4. Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

    Directory of Open Access Journals (Sweden)

    Narges Karimi

    2015-07-01

    Full Text Available Context: Chronic inflammatory demyelinating polyradiculopathy (CIDP is an acquired and autoimmune neuropathy, characterized by a chronic, rapidly progressive, symmetric weakness. In children, abnormal gait is as a first symptom of muscle weakness. Evidence Acquisition: The diagnosis of CIDP is on the basis of clinical characteristics, electrodiagnostic that shows the severity of the disease, lumbar puncture and spine magnetic resonance imaging (MRI. Results: The first-line treatments in childhood CIDP are intravenous immunoglobulin (IVIG, corticosteroids, and plasmapheresis. Response to first-line therapies is usually satisfactory; nevertheless, recommendations regarding the choice of second-line therapy can only be prepared on the basis of the existing practice described in some of the case reports. Conclusions: This review demonstrated the clinical presentation, diagnosis, and treatment of childhood CIDP.

  5. THE ROLE OF PRE-TRANSPLANT AND DE NOVO ALLOANTIBODIES IN KIDNEY TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    A. I. Sushkov

    2011-01-01

    Full Text Available Kidney transplant recipients may have pre-transplant alloantibodies or develop de novo anti-HLA and non- HLA antibodies after transplantation. Although these antibodies may be donor-specific or non-donor-specific, their presence may increase the risk for acute and chronic rejection, thereby decreasing allograft survival. The introduction of high sensitive and specific methods to detect anti-HLA antibodies, both before and after trans- plantation, will help to define transplant recipients who might be at increased risk for early or late allograft failure. Moreover, knowledge of alloantibody status before transplantation may help to guide the appropriate use of immunomodulatory agents and plasmapheresis to remove anti-HLA antibodies or downregulate their production. The review focuses on the associations between renal graft outcome and pre-transplant and de novo alloantibodies. 

  6. Pancytopenia with severe thrombocytopenia in a patient treated with twice-weekly LDL-apheresis by polyacrylate adsorption from whole blood.

    Science.gov (United States)

    Nowack, Rainer; Wiedemann, Günther

    2010-01-01

    Pancytopenia with severe thrombocytopenia occurred in a patient treated with low-density lipoprotein (LDL)-apheresis by polyacrylate adsorption from whole blood, after treatment frequency had been increased from once to twice a week. Cell counts recovered with discontinuation of LDL-apheresis, but thrombocytopenia recurred after resumption of twice-weekly treatments. Thrombocyte counts remained stable following the replacement of polyacrylate adsorption from whole blood by double-filtration plasmapheresis. The complications' close coincidence with twice-weekly polyacrylate adsorption from whole blood suggests a causal relationship, although by a still unknown mechanism. Monitoring of thrombocytes should be advised in patients treated with LDL-apheresis by polyacrylate adsorption from whole blood.

  7. [Sporadic Late-Onset Nemaline Myopathy Associated with MGUS].

    Science.gov (United States)

    Nagai, Taiji; Sunada, Yoshihide

    2015-12-01

    Sporadic late-onset nemaline myopathy is an uncommon disease. Clinically, it is characterized by progressive muscle weakness that can develop in limbs or axial muscles. Asymmetrical distal weakness, facial weakness, dropped head, and dysphagia can also occur. Since the serum creatine kinase level usually remains within the normal range, patients can be misdiagnosed with motor neuron disease. Recognition of nemaline rods on muscle biopsy is crucial for accurate diagnosis. If it is associated with monoclonal gammopathy of undetermined significance, the outcome is known to be unfavorable. In spite of various immunotherapies such as corticosteroids, immunosuppressants, and plasmapheresis, most patients die of respiratory failure within 5 years. Since the efficacy of autologous stem cell transplantation following high-dose melphalan was first reported in 2008, there have been accumulating reports that showed the positive effect of this therapy for the disease.

  8. Different Outcome of Goodpasture Syndrome

    Directory of Open Access Journals (Sweden)

    Ristovska Vesna

    2016-06-01

    Full Text Available Goodpasture syndrome is a rare autoimmune disease, with significant morbidity and mortality in young people and otherwise healthy population. Complete disease remission is possible with prompt diagnosis and treatment. We report 3 cases with Goodpasture syndrome treated at the Department of Nephrology, University Clinic of Nephrology, with different outcome. All of the patients were with similar clinical feature, with renal failure that needed treatment with hemodialysis. But results of the treatment with plasmapheresis indicate that this procedure reduces morbidity in patients with Goodpasture syndrome. The clinical course and the outcome of the disease were different. The disease is unpredictable, and the early diagnosis and start with the treatment is important for the remission.

  9. Fatal Cytomegalovirus Disease after Combination Therapy with Corticosteroids and Rituximab for Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Talal Hilal

    2015-01-01

    Full Text Available The association of cytomegalovirus (CMV with autoimmune disease is poorly understood with suggested causality and reported viral reactivation coinciding with active inflammation. We report a case of a patient who presented with diffuse alveolar hemorrhage and acute renal failure from rapidly progressive glomerulonephritis ultimately diagnosed with granulomatosis with polyangiitis (GPA. She was acutely managed with plasmapheresis to reduce antibody-mediated end-organ damage, hemodialysis for worsening hyperkalemia and acidosis, and high-dose intravenous methylprednisolone. She was transitioned to oral prednisone and started on weekly rituximab with resultant remission induction over a three-week period at which point she developed reactivation of CMV causing severe fatal lung disease and viremia. The case highlights the multiple factors associated with CMV reactivation in cases of severe systemic inflammatory states and the need for further research to help establish practice guidelines regarding antimicrobial prophylaxis in patients with autoimmune diseases on prolonged courses of corticosteroids and biologic agents.

  10. Chikungunya Fever Presenting as Life Threatening Thrombotic Thrombocytopenic Purpura.

    Science.gov (United States)

    Kumar, Vimal; Jain, Rujul; Kumar, Arvind; Nischal, Neeraj; Jorwal, Pankaj; Soneja, Manish; Arava, Sudheer; Wig, Naveet

    2017-07-01

    It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic thrombocytopenic purpura leading to multiple areas of skin necrosis, peripheral digital gangrene, haemolytic anemia, renal failure and severe thrombocytopenia with bleeding. This complication was most likely due to inhibitor autoantibody formation against ADAMTS13 triggered by chikungunya virus leading to thrombotic thrombocytopenic purpura. Patient was treated with plasmapheresis and other supportive careto which she responded. Her symptoms subsided, and she is symptom free and leading normal life in her follow up visits. © Journal of the Association of Physicians of India 2011.

  11. Evaluation of the Organon Teknika Plasmapur system with new software and two types of filter.

    Science.gov (United States)

    Eijkhout, H W; Van Driessche, P; Schade, J H; Hack, C E; De Wit, H J

    1991-01-01

    A convenient plasmapheresis apparatus is the Plasmapur system (Organon Teknika). Recently the software of the Plasmapur monitor has been changed. We evaluated the modified Plasmapur monitor and two types of Plasmapur separators containing polypropylene membranes with a mean maximum pore size of 0.5 mum and 0.6 mum respectively. 50 plasmaphereses with each separator were performed; during 10 procedures donor blood samples and samples from the plasma obtained were drawn. No hypersensitivity reactions were observed, the operator "hands on" time was less than 5 min, the mean procedure time was 45 min to collect 650 mL of plasma with both types of filters. Biochemical analysis of the samples indicated that with both separators the plasma obtained was of good quality with respect to Factor VIII and other proteins and that no significant activation of the complement or clotting cascades occurred.

  12. Pemphigus vulgaris: approach to treatment.

    Science.gov (United States)

    Sinha, Animesh A; Hoffman, Melissa B; Janicke, Elise C

    2015-04-01

    The therapeutic management of pemphigus vulgaris (PV) is centered around immunosuppression, which can be generalized, as in the use of corticosteroids or steroid sparing agents, or specific, as in therapeutic blockage of autoantibody production, certain cytokines, or signaling pathways. Currently, the backbone of treatment for PV, particularly, first line therapy, remains systemic corticosteroids. Although very effective, the significant side effects of long-term corticosteroid usage are well documented. Adjunctive therapies aim to eliminate, or at least decrease, the necessary dose of corticosteroids. Specifically, azathioprine, cyclophosphamide, methotrexate, cyclosporine, mycophenolate mofetil and dapsone are now widely used in PV. Intravenous immunoglobulin (IVIG), plasmapheresis, immunoadsorption, and most recently, rituximab, are other members of the therapeutic armamentarium. However, despite the widening range of treatment options in PV, well-controlled clinical trials and consensus guidelines are lacking.

  13. Four cases of recalcitrant pemphigus vulgaris salvaged with rituximab

    Directory of Open Access Journals (Sweden)

    Samyak Ganjre

    2017-01-01

    Full Text Available Although the long-term use of immunosuppressives – supplemented with more aggressive treatments such as immunoadsorption, intravenous immunoglobulins, or plasmapheresis in recalcitrant cases has dramatically improved the prognosis of pemphigus vulgaris, opportunistic infections secondary to immunosuppression continue to cause significant mortality. We report four cases– three old ones, who had accumulated significant morbidities over their disease duration ranging from 5 to 10 years, and the fourth, a teenage female intolerant to corticosteroids and idiosyncratic to methotrexate– who achieved complete remission on administration of rituximab by the lymphoma protocol. One of the old cases who had recalcitrant mucositis experienced its complete subsidence without any adjuvant whatsoever. All continue to remain asymptomatic for 11–20 months. None had infusion reactions or any delayed side effects.

  14. Bortezomib for acute humoral rejection treatment at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán in Mexico City: an update.

    Science.gov (United States)

    Leyva, Sergio; Marino, Lluvia A; Alberú, Josefina; Morales-Buenrostro, Luis E

    2010-01-01

    The use of bortezomib as a treatment modality of AHR improved and stabilized graft function (clinical response) in the majority of patients. Its use in single dose, even combined with rituximab, does not seem to be useful to obtain a sustained clinical response, or to reduce HLAabs level. The use of 4 doses of bortezomib in days 1, 4, 7, and 10 (1.3 mg/m2 BSA each) plus plasmapheresis produced both a good clinical response and a reduction in DSA. Moving forward, it will be necessary to define the long-term effectiveness of bortezomib and whether rituximab administration is indispensable to achieve this goal. Until now, it is evident that many patients needed retreatment and they were well tolerated.

  15. S-100b and neuron-specific enolase in patients with fulminant hepatic failure

    DEFF Research Database (Denmark)

    Strauss, Gitte Irene; Christiansen, Michael; Møller, Kirsten

    2001-01-01

    Patients with fulminant hepatic failure (FHF) frequently develop cerebral edema and intracranial hypertension. The aim of this study was to evaluate circulating S-100b and neuron-specific enolase (NSE) levels as markers of neurological outcome in patients with FHF. In a subgroup of patients......, the cerebral flux of S-100b and NSE was measured. We included 35 patients with FHF, 6 patients with acute on chronic liver disease (AOCLD), 13 patients with cirrhosis of the liver without hepatic encephalopathy, and 8 healthy subjects. Blood samples were obtained from catheters placed in the radial artery...... and internal jugular bulb. The net cerebral flux of S-100b and NSE was measured, and the effect of short-term hyperventilation, as well as the effect of high-volume plasmapheresis, on circulating levels of these two biomarkers was determined. Blood levels of S-100b were greater in patients with FHF and AOCLD...

  16. Comparison between the Traditional and a Rapid Screening Test for Cryoimmunoglobulins Detection

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    Federica Romitelli

    2015-01-01

    Full Text Available Objectives. A new rapid, automatic, and sensitive screening test useful to detect cryoglobulins in serum samples is proposed. Design and Methods. The increase of turbidity during the cryoglobulin aggregation was monitored spectrophotometrically in sera from 400 patients with clinical evidence of cryoglobulinemia related disorders and 100 controls. Results were correlated to those obtained by the traditional method. Results. Kinetics of the aggregation curves were described by their maximum turbidity increase, lag time, and slope. Despite a partial correspondence between the traditional and the rapid test, patients with symptomatic cryoglobulinemia showed turbidity values significantly higher than the determined cutoff. Moreover, a functional classification of cryoglobulins is proposed. Conclusions. Due to its high reproducibility, operator independence, low cost, and results obtained within 2 hours, the rapid test can be used as a “real time” monitoring of cryoglobulinemia related diseases and for the evaluation of plasmapheresis efficacy.

  17. A case of primary renal allograft dysfunction due to myeloma cast nephropathy

    Directory of Open Access Journals (Sweden)

    Umesh Lingaraj

    2015-01-01

    Full Text Available We report a rare case of primary renal allograft dysfunction due to myeloma cast nephropathy in a patient with no overt clinical features of multiple myeloma preceding his transplantation. A 45-year-old man on hemodialysis for six months for end-stage kidney disease due to presumed chronic glomerulonephritis developed immediate graft dysfunction post-transplantation. The graft biopsy was diagnostic of myeloma cast nephropathy. Other criteria for lambda light chain multiple myeloma were fulfilled with immunofixation electrophoresis and bone marrow biopsy. He was treated with plasmapheresis, bortezomib and high-dose dexamethasone. However, the patient succumbed to septicemia on the 37 th post-operative day. This is probably the first report of primary renal allograft dysfunction due to myeloma cast nephropathy diagnosed within the first week posttransplanation in a patient with unrecognized multiple myeloma.

  18. Treatment of Focal Segmental Glomerulosclerosis Recurrence in the Renal Allograft: A Report of Two Cases.

    Science.gov (United States)

    Tran, Minh-Ha; Chan, Cynthia; Pasch, Whitney; Carpenter, Philip; Ichii, Hirohito; Foster, Clarence

    2016-01-01

    Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients. Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved. In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair. In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

  19. A case of primary renal allograft dysfunction due to myeloma cast nephropathy.

    Science.gov (United States)

    Lingaraj, Umesh; Vankalakunti, Mahesha; Radhakrishnan, Hemachandar; Sreedhara, C G; Rajanna, Sunil

    2015-09-01

    We report a rare case of primary renal allograft dysfunction due to myeloma cast nephropathy in a patient with no overt clinical features of multiple myeloma preceding his transplantation. A 45-year-old man on hemodialysis for six months for end-stage kidney disease due to presumed chronic glomerulonephritis developed immediate graft dysfunction post-transplantation. The graft biopsy was diagnostic of myeloma cast nephropathy. Other criteria for lambda light chain multiple myeloma were fulfilled with immunofixation electrophoresis and bone marrow biopsy. He was treated with plasmapheresis, bortezomib and high-dose dexamethasone. However, the patient succumbed to septicemia on the 37 th post-operative day. This is probably the first report of primary renal allograft dysfunction due to myeloma cast nephropathy diagnosed within the first week post-transplanation in a patient with unrecognized multiple myeloma.

  20. Belatacept for Maintenance Immunosuppression in Lung Transplantation

    Directory of Open Access Journals (Sweden)

    Christine Hui PharmD

    2014-06-01

    Full Text Available Belatacept is a novel immunosuppressant that blocks a T-cell costimulation pathway and is approved for use in adult kidney transplant recipients. Its safety and efficacy have not been established after lung transplantation. We present a case of a lung transplant recipient treated with belatacept. A 56-year-old man underwent bilateral lung retransplantation for bronchiolitis obliterans syndrome (BOS. In the third year posttransplant, he developed hemolytic uremic syndrome (HUS attributed to tacrolimus. Tacrolimus was changed to sirolimus. One month later, he presented with worsening renal function and HUS attributed to sirolimus. Plasmapheresis and steroid pulse were initiated with clinical improvement, and sirolimus was switched to belatacept. He experienced no episodes of cellular rejection but developed recurrent BOS. Complications during treatment included anemia and recurrent pneumonias. The safety and efficacy of belatacept in lung transplantation remains unclear; further studies are needed.

  1. Ketamine Infusion Associated with Improved Neurology in a Patient with NMDA Receptor Encephalitis

    Directory of Open Access Journals (Sweden)

    Michael MacMahon

    2013-01-01

    Full Text Available A young lady was ventilated on intensive care for a prolonged period with NMDA receptor encephalitis. She had undergone steroid, immunoglobulin, and plasmapheresis with no evidence of recovery. Her main management issue was the control of severe orofacial and limb dyskinesia. Large doses of sedating agents had been used to control the dystonia but were ineffective, unless she was fully anaesthetised. The introduction of a ketamine infusion was associated with a dramatic improvement in her symptoms such that it was possible to remove her tracheostomy two days after commencement. She was discharged shortly after that and is making a good recovery. The successful use of ketamine has not previously been described in this context, and we hope this case report will provide some insight into the management of this rare but serious condition.

  2. Rhythmic delta activity represents a form of nonconvulsive status epilepticus in anti-NMDA receptor antibody encephalitis.

    Science.gov (United States)

    Kirkpatrick, McNeill P; Clarke, Charles D; Sonmezturk, Hasan H; Abou-Khalil, Bassel

    2011-02-01

    Anti-NMDA receptor antibody encephalitis is a limbic encephalitis with psychiatric manifestations, abnormal movements, coma, and seizures. The coma and abnormal movements are not typically attributed to seizure activity, and slow activity is the most common EEG finding. We report drug-resistant nonconvulsive status epilepticus as the basis for coma in a 19-year-old woman with anti-NMDA receptor antibodies and a mediastinal teratoma. The EEG showed generalized rhythmic delta activity, with evolution in morphology, frequency, and field typical of nonconvulsive status epilepticus. The status was refractory to antiepileptic drugs, repeated drug-induced coma, resection of the tumor, intravenous steroids, rituximab, and plasmapheresis. She awoke after the addition of felbamate, and the rhythmic delta activity ceased. The rhythmic delta activity described with coma in anti-NMDA receptor antibody encephalitis may represent a pattern of status epilepticus in some patients. Felbamate, which has NMDA receptor antagonist activity, should be studied as a therapeutic agent in this condition.

  3. Optimum Conditions for the Efficacy and Safety of Cryofiltration Apheresis: An Analysis of Circuit Temperatures Depending on Plasma Flow Rate and Cooling Coil Lengths/Turns.

    Science.gov (United States)

    Nakajima, Hirofumi; Kaneko, Shuzo; Sato, Yukihiro; Takano, Tomoo; Hosino, Toshihisa

    2015-08-01

    A system providing both appropriate cooling and warming are needed for the efficacy and safety of cryofiltration (CF) plasmapheresis. We measured some points of CF circuit temperatures with varying plasma flow rates (QP  = 10-40 mL/min) and the numbers of connecting cooling coils (one or two) under the conditions of blood flow rate (QB ) 100 mL/min with 7700-mm coil length, 19 turns, and 50-mL priming volume. We measured the respective temperatures of each point of starting/returning for an extracorporeal circuit (TA /TV ), intracooling coil (TC ), and post-plasma fractionator (PF) (TPF ). The subtraction of TV from TA (ΔT) was used as an indicator of safe return. There were no significant differences in TC , TPF , or ΔT in accordance with each QP between that of one and two coils. All of the Tc values under the condition QP  ≤ 20 mL/min achieved <4°C. The TPF under the condition QP  ≥ 20 mL/min was not significantly different compared to that of QP 30 mL/min (the lowest condition). Although the ΔT increased depending on the QP increase, the ΔT under the condition QP  ≤ 15 mL/min was not significantly different from that of the control (one-way double-filtration plasmapheresis [DFPP]) group. We conclude that (i) one coil is enough for effective cooling in CF, and (ii) an ideal QP that fulfills the required conditions for both effective cooling and sufficient warming of returning fluid does not exist, but QP from 15 to 20 mL/min may be a relevant range.

  4. Evidence-based evaluation of treatment strategy for multiple sclerosis

    Directory of Open Access Journals (Sweden)

    LI Meng-qiu

    2012-04-01

    Full Text Available Objective To formulate the best treatment plan for multiple sclerosis (MS patients by evaluating the therapeutic efficacy and side effect of various evidence-based programs. Methods Key words were defined as multiple sclerosis, immunomodulatory therapy and therapy, etc. We searched MEDLINE, Cochrane Library, Wanfang data bases for Scientific Journals in China and National Knowledge Infrastructure for Chinese Scientific Journals Database. Additionally, we applied manual searching and screened out conference paper and academic dissertation, etc, from various references. After that we obtained and evaluated by Jadad scales on systematic reviews, randomized controlled trials, controlled clinical trials and observational study cases about glucocorticoids, plasmapheresis, intravenous immunoglobulin, IFN-β, glatiramer acetate, mitoxantrone, natalizumab, fingolimod. Results After screening, all seventeen selected resources included systematic reviews 6 articles, randomized controlled trials 7 articles, controlled clinical trials 2 articles, observational study cases 2 articles, among which fifteen articles were proved to be high quality (according to Jadad scoring system, five score 4, six score 5, four score 7, two chapters were judged to be low quality scoring 3. Finally, we summerize that: 1 The first choice of treatment for acute relapses is glucocorticoids and we suggest that plasmapheresis or intravenous immunoglobulin may be tried as an alternative therapy in acute MS relapse, especially in case of contraindications to intravenous methylprednisolone. 2 Immunomodulatory or immunosuppressive treatment (IFN-β, glatiramer acetate, mitoxantrone, natalizumab can be an option to prevent new relapses and progression of disability. 3 Fingolimod is an oral treatment for multiple sclerosis to improve treatment adherence. Conclusion Using evidence-based medicine methods can provide us best clinical evidence on MS treatment.

  5. Pseudo-thrombotic thrombocytopenic purpura: A rare presentation of pernicious anemia

    Directory of Open Access Journals (Sweden)

    Ashvin K Tadakamalla

    2011-01-01

    Full Text Available Context: Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of pernicious anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura. Case Report : A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B 12 was found to be low. Treatment with intra-muscular vitamin B 12 led to symptomatic and hematologic improvement. Pernicious anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis. Conclusion : Clinicians must be aware of unusual clinical presentation of vitamin B 12 deficiency with schistocytes as the management is simple and effective.

  6. Late-Onset Drug-Induced Cholestasis in a Living-Related Liver Transplant Donor With Progressive Familial Intrahepatic Cholestasis.

    Science.gov (United States)

    Harmancı, Özgür; Ensaroğlu, Fatih; Özçay, Figen; Öcal, Serkan; Korkmaz, Murat; Özdemir, B Handan; Selçuk, Haldun; Moray, Gökhan; Haberal, Mehmet

    2015-11-01

    We present a rare case of progressive familial intrahepatic cholestasis within a family. A 34-yearold female became a living-related liver transplant donor for her son, who had the disease. Nine years after the transplant, the mother developed severe intrahepatic cholestasis, for which she was evaluated after using an oral contraceptive drug. She presented with jaundice, pruritus, and increased bilirubin levels, together with elevated gamma glutamyl transferase and alkaline phosphatase levels. A liver biopsy revealed findings consistent with intrahepatic cholestasis. However, despite follow-up management and cessation of the insulting drug, her total bilirubin count continuously increased to 20 mg/dL and was accompanied by intractable pruritus. A total of 9 plasmapheresis sessions were performed, and she was started on a regimen of ursodeoxycholic acid (13 mg/kg/d) and cholestyramine (4 g, 3 times daily). The clinical and laboratory picture dramatically improved following cessation of the oral contraceptive, plasmapheresis sessions, and drug treatment. The patient's cholestasis normalized within 3 months, and she recovered uneventfully. A genetic analysis of the whole family revealed that both parents were heterozygous for the mutation c.124G>A in ABCB11, and the son was homozygous for this mutation. These findings supported varying degrees of bile salt export pump deficiency in the family members. Defective bile salt excretory system function can result in a wide spectrum of clinical presentations, ranging from progressive familial intrahepatic cholestasis requiring liver transplant to late-onset drug-induced cholestasis. Our findings suggest that, in a heterozygous carrier of a progressive familial intrahepatic cholestasis mutation, drug-induced cholestasis is responsive to treatment, after which the clinical picture can normalize within 3 months.

  7. Hypertriglyceridemia: Is there a role for prophylactic apheresis? A case report.

    Science.gov (United States)

    Francisco, Ana Rita; Gonçalves, Inês; Veiga, Fátima; Pedro, Mónica Mendes; Pinto, Fausto J; Brito, Dulce

    2016-01-01

    Severe hypertriglyceridemia has been consistently associated with an increased risk of cardiovascular disease and other complications, namely acute pancreatitis. We report a case of a 64 year-old woman with hypertrophic cardiomyopathy and metabolic syndrome with triglyceride level of 3260 mg/dL. Plasma exchange was performed with simultaneous medical treatment to achieve a rapid and effective lowering of triglycerides in order to prevent clinical complications. After three plasmapheresis sessions a marked reduction in triglyceride and total cholesterol levels was observed. Several cases have shown the importance of plasmapheresis in the treatment of acute pancreatitis. We intend to demonstrate the applicability of this technique as primary prophylaxis in the presence of extremely high serum triglyceridemia levels. Resumo A hipertrigliceridemia grave tem sido associada de forma consistente ao aumento do risco cardiovascular e a outras complicações, nomeadamente, pancreatite aguda. Descrevemos um caso de uma mulher de 64 anos, com miocardiopatia hipertrófica e síndrome metabólica com valor sérico de triglicerídeos de 3260 mg/dL. Foi efectuada plasmaferese e optimizado o tratamento médico para alcançar uma redução rápida e efectiva dos níveis dos triglicerídeos, prevenindo complicações clínicas. Após três sessões de plasmaferese, verificou-se uma redução marcada dos triglicerídeos e do colesterol total. Existem alguns casos descritos na literatura demonstrado a importância da plasmaferese no tratamento da pancreatite aguda em contexto de hipertrigliceridemia grave. Os autores pretendem com este caso demonstrar a aplicabilidade desta técnica em contexto de prevenção primária em doentes com níveis de triglicerídeos extremamente aumentados.

  8. Fibrinogen salvage during DF Thermo using Evaflux-5A plasma fractionators.

    Science.gov (United States)

    Nakashima, Momoko; Yasui, Masahide; Ihara, Akira

    2012-10-01

    DF Thermo, a modified form of double-filtration plasmapheresis (DFPP), has been used for the treatment of various indications such as arteriosclerosis obliterans (ASO). In case of ASO, fibrinogen is a substance to be removed by DFPP. On the other hand, plasmapheresis for chronic viral hepatitis C became an insurance covered treatment in Japan in April 2008. Since then DFPP has also become a treatment of chronic viral hepatitis C as an adjunctive therapy for the purpose of improving the effect of medication. Therefore, there has been a growing concern in recent years about patients' low fibrinogen levels due to DFPP treatment. With the aim of improving fibrinogen retention by DF Thermo, we examined by in vitro trial, the effects when recirculating the filtrate and elevating its temperature. The trial was conducted using bovine plasma, run through experimental circuits with the same configuration as the clinical setting of the One-Way method and DF Thermo method. The DF Thermo circuit contained a thermostat, on which the temperature was set to 40°C. Two One-Way method circuits were prepared with different temperature settings, i.e., 20°C and 40°C. With these three different conditions, variance of the fibrinogen retention under different temperatures and the implementation of recirculation were compared. Results show that the DF Thermo circuit tends to have enhanced the fibrinogen retention compared to the One-Way method 20°C and 40°C. The explanation is likely as follows: viscosity of plasma reduces when warmed, which in turn helps maintain the permeability of membrane, and the recirculation of the plasma helps prevent membrane fouling, thus more fibrinogen is retained in the DF Thermo method.

  9. Organizing the Donation of Convalescent Plasma for a Therapeutic Clinical Trial on Ebola Virus Disease: The Experience in Guinea

    Science.gov (United States)

    Delamou, Alexandre; Haba, Nyankoye Yves; Mari-Saez, Almudena; Gallian, Pierre; Ronse, Maya; Jacobs, Jan; Camara, Bienvenu Salim; Kadio, Kadio Jean-Jacques Olivier; Guemou, Achille; Kolie, Jean Pe; De Crop, Maaike; Chavarin, Patricia; Jacquot, Chantal; Lazaygues, Catherine; De Weggheleire, Anja; Lynen, Lutgarde; van Griensven, Johan

    2016-01-01

    Although convalescent plasma (CP) transfusion was prioritized among potential Ebola treatments by the World Health Organization, there were concerns on the feasibility of its implementation. We report on the successful organization of donor mobilization and plasma collection as part of the Ebola-Tx clinical trial from November 2014 to July 2015 in Conakry, Guinea. Project implementation registers, tools and reports, mission reports, and minutes of research team meetings were used to reconstruct the sequence of events on how donor mobilization was organized, plasmapheresis was set up, and how effective this approach was in collecting CP. An initial needs assessment of the Guinean National Blood Transfusion Center resulted in targeted training of staff on site, resulting in autonomy and independent production of CP within 3 months. The Conakry Ebola Survivors Association played a direct role in donor mobilization and organization of CP donations. A total of 98 Ebola survivors were screened for plasma donation, of which 84 were found eligible for plasmapheresis. Of these, 26 (30.9%) were excluded. The remaining 58 donors made a total of 90 donations, corresponding to 50.9 L of CP. This sufficed to treat the 99 eligible patients enrolled in the trial. Within a poor resource emergency context, transfusion capacity could be rapidly improved through the strengthening of local capacities and gradual transfer of skills coupled with active involvement of Ebola survivors. However, large-scale plasma collection or multisite studies may require further adaptations of both strategy and logistics. The Ebola-Tx trial was funded by the European Union and others. PMID:27430546

  10. Prospective iterative trial of proteasome inhibitor-based desensitization.

    Science.gov (United States)

    Woodle, E S; Shields, A R; Ejaz, N S; Sadaka, B; Girnita, A; Walsh, R C; Alloway, R R; Brailey, P; Cardi, M A; Abu Jawdeh, B G; Roy-Chaudhury, P; Govil, A; Mogilishetty, G

    2015-01-01

    A prospective iterative trial of proteasome inhibitor (PI)-based therapy for reducing HLA antibody (Ab) levels was conducted in five phases differing in bortezomib dosing density and plasmapheresis timing. Phases included 1 or 2 bortezomib cycles (1.3 mg/m(2) × 6-8 doses), one rituximab dose and plasmapheresis. HLA Abs were measured by solid phase and flow cytometry (FCM) assays. Immunodominant Ab (iAb) was defined as highest HLA Ab level. Forty-four patients received 52 desensitization courses (7 patients enrolled in multiple phases): Phase 1 (n = 20), Phase 2 (n = 12), Phase 3 (n = 10), Phase 4 (n = 5), Phase 5 (n = 5). iAb reductions were observed in 38 of 44 (86%) patients and persisted up to 10 months. In Phase 1, a 51.5% iAb reduction was observed at 28 days with bortezomib alone. iAb reductions increased with higher bortezomib dosing densities and included class I, II, and public antigens (HLA DRβ3, HLA DRβ4 and HLA DRβ5). FCM median channel shifts decreased in 11/11 (100%) patients by a mean of 103 ± 54 mean channel shifts (log scale). Nineteen out of 44 patients (43.2%) were transplanted with low acute rejection rates (18.8%) and de novo DSA formation (12.5%). In conclusion, PI-based desensitization consistently and durably reduces HLA Ab levels providing an alternative to intravenous immune globulin-based desensitization. © Copyright 2014 The American Society of Transplantation and the American Society of Transplant Surgeons.

  11. Ameliorating Role Exerted by Al-Hijamah in Autoimmune Diseases: Effect on Serum Autoantibodies and Inflammatory Mediators.

    Science.gov (United States)

    Baghdadi, Hussam; Abdel-Aziz, Nada; Ahmed, Nagwa Sayed; Mahmoud, Hany Salah; Barghash, Ayman; Nasrat, Abdullah; Nabo, Manal Mohamed Helmy; El Sayed, Salah Mohamed

    2015-04-01

    Autoimmune diseases have common properties characterized by abnormal blood chemistry with high serum autoimmune antibodies, and inflammatory mediators. Those causative pathological substances (CPS) cannot be excreted by physiological mechanisms. Current treatments for autoimmune diseases involve steroids, cytotoxic drugs, plasmapheresis and monoclonal antibodies. Wet cupping therapy (WCT) of prophetic medicine is called Al-hijamah that treats numerous diseases having different etiology and pathogenesis via a pressure-dependent and size-dependent non-specific filtration then excretion of CPS causing clearance of blood and interstitial fluids. Al-hijamah clears blood passing through the fenestrated skin capillaries. Medical bases of Al-hijamah were reported in the evidence-based Taibah mechanism (Taibah theory). Al-hijamah was reported to be an excellent treatment for rheumatoid arthritis that improved patients' blood chemistry and induced significant clinical improvement and pharmacological potentiation. Al-hijamah improved the natural immunity and suppressed the pathological immunity through decreasing the serum level of autoantibodies, inflammatory mediators, and serum ferritin (a key player in autoimmunity). Al-hijamah reduced significantly pain severity, number of swollen joints and disease activity with no significant side effects. Main steps of Al-hijamah are skin suction (cupping), scarification (sharatmihjam in Arabic) and second suction (triple S technique) that is better therapeutically than the traditional WCT (double S technique). Whenever an excess noxious substance is to be removed from patients' blood and interstitial fluids, Al-hijamah is indicated. Shartatmihjam is a curative treatment in prophetic teachings according to the prophetic hadeeth: "Cure is in three: in shartatmihjam, oral honey and cauterization. I do not recommend my nation to cauterize". Al-hijamah may have better therapeutic benefits than plasmapheresis. Al-hijamah may be promising

  12. De novo post-transplant thrombotic microangiopathy localized only to the graft in autosomal dominant polycystic kidney disease with thrombophilia

    Science.gov (United States)

    Rolla, Davide; Fontana, Iris; Ravetti, Jean Louis; Marsano, Luigina; Bellino, Diego; Panaro, Laura; Ansaldo, Francesca; Mathiasen, Lisa; Storace, Giulia; Trezzi, Matteo

    2015-01-01

    Introduction: Thrombotic microangiopathy (TMA) is a serious complication of renal transplantation and is mostly related to the prothrombotic effect of calcineurin inhibitors (CNIs). A subset of TMA (29%-38%) is localized only to the graft. Case 1: A young woman suffering from autosomal dominant polycystic kidney disease (ADPKD) underwent kidney transplant. After 2 months, she showed slow renal deterioration (serum creatinine from 1.9 to 3.1 mg/dl), without hematological signs of hemolytic-uremic syndrome (HUS); only LDH enzyme transient increase was detected. Renal biopsy showed TMA: temporary withdraw of tacrolimus and plasmapheresis was performed. The renal function recovered (serum creatinine 1.9 mg/dl). From screening for thrombophilia, we found a mutation of the Leiden factor V gene. Case 2: A man affected by ADPKD underwent kidney transplantation, with delay graft function; first biopsy showed acute tubular necrosis, but a second biopsy revealed TMA, while no altered hematological parameters of HUS was detected. We observed only a slight increase of lactate dehydrogenase (LDH) levels. The tacrolimus was halved and plasmapheresis was performed: LDH levels normalized within 10 days and renal function improved (serum creatinine from 9 to 2.9 mg/dl). We found a mutation of the prothrombin gene. Only a renal biopsy clarifies the diagnosis of TMA, but it is necessary to pay attention to light increasing level of LDH. Conclusion: Prothrombotic effect of CNIs and mTOR inhibitor, mutation of genes encoding factor H or I, anticardiolipin antibodies, vascular rejection, cytomegalovirus infection are proposed to trigger TMA; we detected mutations of factor II and Leiden factor V, as facilitating conditions for TMA in patients affected by ADPKD. PMID:26693501

  13. Double-positive Goodpasture′s syndrome with concomitant active pulmonary tuberculosis

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    Waqar Kashif

    2013-01-01

    Full Text Available Anti-glomerular basement membrane (anti-GBM disease usually presents as rapidly progressive glomerulonephritis, and, when accompanied with pulmonary hemorrhage, it is called Goodpasture′s syndrome. Anti-neutrophilic cytoplasmic antibodies (ANCA may co-exist with anti-GBM antibodies. In most of these "double positive" cases, ANCA is specific for myeloperoxidase (p-ANCA. We report a rare case of a critically ill patient c-ANCA-associated double-positive Goodpasture′s syndrome with concomitant tuberculosis that was successfully treated with immunosuppression, plasmapheresis and anti-tuberculous therapy (ATT. A 32-year-old gentleman with a 15 pack-year smoking history presented with massive hemoptysis, respiratory failure and oliguria. Laboratory investigation revealed anemia, elevated creatinine and active urinary sediment. Chest X-ray revealed bilateral pulmonary infiltrates. Broad-spectrum antibiotics and intravenous corticosteroids were started. Bronchoscopy showed alveolar hemorrhage and smears from bronchial lavage from both lungs were positive for acid fast bacillus (AFB. Vasculitis work-up revealed high titers of c-ANCA and anti-GBM antibodies. Kidney biopsy revealed crescents in >50% glomeruli on light microscopy. Immunofluorescence showed linear deposition of IgG and C3. The patient received pulse methylprednisone for three days followed by oral prednisone and ATT. In addition, he also underwent nine sessions of plasmapheresis. Oral Cyclophosphamide was added on Day 10. The patient showed remarkable recovery as his lung fields cleared and his kidney function got stabilized. Cyclophosphamide was continued for three months and then switched to azathioprine. At six months, the creatinine is 1.2 mg/dL, with minimal proteinuria and a normal chest X-ray. To the best of our knowledge, this is the only reported case of double-positive Goodpasture′s syndrome (c-ANCA and anti GBM with active tuberculosis treated successfully.

  14. Patient with antibody-negative relapse of Goodpasture syndrome.

    Science.gov (United States)

    Benz, K; Amann, K; Dittrich, K; Hugo, C; Schnur, K; Dötsch, J

    2007-04-01

    Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. We present a male adolescent in whom hemoptysis was mistaken to be a sign of airway infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and therapy-refractant hemoptysis and, later, acute macrohematuria with renal insufficiency necessitating hemodialysis (initial creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased anti-GBM antibodies were detected. Thus, anti-GBM glomerulonephritis was diagnosed. After treatment with prednisone, oral cyclophosphamide and plasmapheresis, chest X-ray and hemoptysis improved, but renal failure persisted. Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe hemoptysis and respiratory insufficiency after smoke exposition. Despite negative anti-GBM antibodies, he was treated similarly to a relapse and after the second course of plasmapheresis the patients' general condition improved and hemoptysis subsided. During the next 10 months the patient was stable with negative antibodies. He was under intermittent hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with antihypertensive medicaments, calcitriol, calciumacetate, natriumhydrogencarbonate and allopurinol. The prognosis of anti-GBM glomerulonephritis depends on serum creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of

  15. Organizing the Donation of Convalescent Plasma for a Therapeutic Clinical Trial on Ebola Virus Disease: The Experience in Guinea.

    Science.gov (United States)

    Delamou, Alexandre; Haba, Nyankoye Yves; Mari-Saez, Almudena; Gallian, Pierre; Ronse, Maya; Jacobs, Jan; Camara, Bienvenu Salim; Kadio, Kadio Jean-Jacques Olivier; Guemou, Achille; Kolie, Jean Pe; Crop, Maaike De; Chavarin, Patricia; Jacquot, Chantal; Lazaygues, Catherine; Weggheleire, Anja De; Lynen, Lutgarde; van Griensven, Johan

    2016-09-01

    Although convalescent plasma (CP) transfusion was prioritized among potential Ebola treatments by the World Health Organization, there were concerns on the feasibility of its implementation. We report on the successful organization of donor mobilization and plasma collection as part of the Ebola-Tx clinical trial from November 2014 to July 2015 in Conakry, Guinea. Project implementation registers, tools and reports, mission reports, and minutes of research team meetings were used to reconstruct the sequence of events on how donor mobilization was organized, plasmapheresis was set up, and how effective this approach was in collecting CP. An initial needs assessment of the Guinean National Blood Transfusion Center resulted in targeted training of staff on site, resulting in autonomy and independent production of CP within 3 months. The Conakry Ebola Survivors Association played a direct role in donor mobilization and organization of CP donations. A total of 98 Ebola survivors were screened for plasma donation, of which 84 were found eligible for plasmapheresis. Of these, 26 (30.9%) were excluded. The remaining 58 donors made a total of 90 donations, corresponding to 50.9 L of CP. This sufficed to treat the 99 eligible patients enrolled in the trial. Within a poor resource emergency context, transfusion capacity could be rapidly improved through the strengthening of local capacities and gradual transfer of skills coupled with active involvement of Ebola survivors. However, large-scale plasma collection or multisite studies may require further adaptations of both strategy and logistics. The Ebola-Tx trial was funded by the European Union and others.

  16. Síndrome de Guillain Barré asociado a Brucelosis Guillain Barre syndrome in association with Brucellosis

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    Raúl Montalvo

    2010-06-01

    Full Text Available Se describe el caso de un varón de 47 años, con tiempo de enfermedad de dos días, caracterizado por pérdida de fuerza progresiva, simétrica y ascendente de miembros inferiores, se realizó punción lumbar luego de la tomografía cerebral y electromiografía lo cual evidenció polirradiculopatía motora pura con patrón axonal, compatible con el síndrome de Guillain Barré. Posteriormente, recibió cuatro sesiones de plasmaféresis, con mejoría clínica significativa desde la segunda sesión. Debido al antecedente epidemiológico se solicitó set para Brucellas, con rosa de Bengala positivo, se inició tratamiento antibiótico con rifampicina y doxiciclina, además de rehabilitación. Tres meses después el paciente mejoró completamente. La importancia del tratamiento temprano con plasmaféresis y determinar su diagnóstico etiológico hacen que el pronóstico del síndrome de Guillain Barré sea favorable.We describe a case of a 47 years old male, with a history of 2 days of progressive, ascendant, symmetrical weakness in the lower extremities; a lumbar puncture was performed after the brain CT scan, as well as an electromyography, evidencing pure motor polyradiculopathy with axonal pattern, compatible with Guillain Barre syndrome. Afterwards, he received four plasmapheresis sessions, with clinical improvement from the second session. Due to his epidemiological background, Brucella set testing was done. Rose Bengal was positive, antibiotic treatment with rifampin and doxicicline was initiated, as well as rehabilitation. Three months later the patient recovered completely. The relevance of early treatment with plasmapheresis and the definition of the etiologic diagnosis determine that the prognosis of the Guillain Barre syndrome is favorable.

  17. Oral and parenteral pyridostigmine in preparing Myasthenia Gravis patients for thymectomy;a randomized Clinical trial

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    Tadjeddein A

    2007-06-01

    Full Text Available Background: Respiratory failure and crisis is one of major complications of thymectomy in myasthenia gravis patients. There are different medication regimes for preparing these patients for surgery and reducing post-operative side effects. The goal of this study is to compare respiratory complications of oral vs. Parenteral preoperative administration of anticholinesterase agents for thymectomy in myasthenia gravis patients. Methods: This randomized controlled trial included 101 patients in class IIA or IIB of myasthenia gravis according to the Osserman classification system. The control group fasted for eight hours before surgery and oral anticholines-terase agents were replaced with parenteral ones. The case group also fasted for 8 hours before surgery, but pyridostigmine was continued at its usual dose until the time of operation and the last dose was given to patients with a small amount of water in the operating room on the operating bed. Results: There was no statistically meaningful difference between the two groups in terms of age, sex and pathologic findings. In comparison, the mean hospital stay for the case group was 3.98 days and 6.34 for the control group (p value = 0.003. There were eight cases of respiratory crisis or failure (16% in the control group but only 1 case (2% was observed in case group (p value = 0.014. Only one patient in the case group required re-intubation after the surgery; however, six patients in control group were re-intubated (p value = 0.053. Plasmapheresis was required for five patients in the control group and one patient in the case group (p value = 0.098. Tracheostomy was performed on two patients in the control group to accommodate prolonged intubation, but none of the case group required this procedure. Conclusion: This study shows that continuing oral anticholinesterase agents up to the time of operation, with the last dose at the operative theater, lowers the incidence of post-operative myasthenia

  18. Kidney transplantation from HLA-incompatible live donors: Efficiency and outcome of 32 patients after desensitisation.

    Science.gov (United States)

    Fernández, Constantino; Calvo, María; Leite, Natacha; López, Andrés; Ferreiro, Tamara; Ribera, Roi; Seijo, Rocío; Alonso, Ángel

    2017-07-19

    Desensitisation is a procedure undergone by the recipient of a kidney transplant from a donor who is cross-match positive. The aim of this study was to present the outcomes from our hospital of kidney transplant recipients from HLA-incompatible live donors after desensitisation. We studied 32 patients aged 46±14 years with a mean fluorescence intensity (MFI) versus class I HLA of 7979±4089 and 6825±4182 MFI versus class II and relative intensity scale (RIS) of 8.9±7.6. The complement-dependent cytotoxicity (CDC) cross-matching test was positive in 18 patients, flow cytometry was positive in 7 patients and donor-specific antibodies (DEA) were detected in 7. The protocol used was rituximab, plasmapheresis/immunoadsorption, immunoglobulins, tacrolimus, mycophenolic acid derivatives and prednisone. After 8±3 sessions of plasmapheresis/immunoadsorption, 23 patients were trasplanted (71.9%) and desensitisation was ineffective in 9. There were baseline differences in MFI class I (P<.001), RIS (P=.008), and CDC cross-matching, DSA and flow cytometry (P=.05). MFI class I and RIS were predictors of inefficiency in ROC curves. After follow-up of 43±30 months, 13 patients (56%) presented postoperative bleeding, 3 (13%) delayed graft function, 4 (17.4%) acute rejection, 6 (26%) CMV viraemia and 1 (4%) BK viraemia. Five-year patient survival was 90%, with 86% allograft survival. Five-year creatinine was 1.5±0.4 and proteinuria was 0.5±0.7. Kidney transplantation from HLA-incompatible live donors after desensitisation was possible in 71.9% of patients. MFI class I and RIS predict the inefficiency of desensitisation. Five-year allograft survival (86%) was acceptable with a low incidence of acute rejection (17.4%), although with a greater trend towards postoperative bleeding. Copyright © 2017 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  19. Cerebral perfusion reserve in carotid stenosis: Prognostic role in surgical and rheological therapy

    Energy Technology Data Exchange (ETDEWEB)

    Ussov, W.; Shipulin, V.; Shvera, I. [and others

    1994-05-01

    The study was aimed to evaluate the usefulness of the quantitative cerebral perfusion reserve SPECT for the prognosis in patients with critical stenoses of the internal carotid arteries treated surgically or rheotherapeutically. 23 patients with angiographically verified mono (n=12) or bilateral (n=11) stenosis of the internal carotid artery for more than 75% and with recent (>30 days) minor stroke or TIA events in anamnesis were referred for the study. 6 age-matched healthy persons served as controls. In everybody the cerebral perfusion reserve was studied by using of the local cerebral blood volume to cerebral blood flow (rCBV/rCBF, sec) index using a double isotope technique which combined Tl-199 diethyidithiocarbamate (Tl-199-DDC) SPECT with Tc-99m red blood cells (Tc-99m-RBC) SPECT. 12 patients underwent carotid endartherectomy and 11 were treated rheotherapeutically by nonselective plasma substitution (5 times x 400 ml, >1800 nil in total). Patients were studied twicely: before and 10 days after the treatment, and then followed-up neurologically in the course of 1 year. Patients who had regional rCBV/rCBF index increased in the stenosed a.carotis int. - dependent region before treatment (normal value 5.2, sd 0.2 sec) have got it significantly decreased after intervention both in surgical group (from 7.1, sd 0.4 sec, down to 5.3, sd 0.3 sec, p<0.01 n=7) and in rheotherapeutical one (from 7.3, sd 0.5 sec, to 5.9, sd 0.4 sec, p<0.02 n=8). In everybody who expressed the rCBV/rCBF decreased below 5.6 sec after intervention there were no cerebrovascular ischemic events observed in the course of 12 months after carotid surgery and during 5 months in patients treated by plasmapheresis. We conclude that rCBV/rCBF SPECT index gives an independent prognostic information useful for both rheologic and surgical treatment of cerebrovascular disease; and that plasmapheresis suppress the short-term rise of cerebral ischemic events, which effect is predictable by SPECT.

  20. Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus.

    Science.gov (United States)

    Aytaç, Selin; Batu, Ezgi Deniz; Ünal, Şule; Bilginer, Yelda; Çetin, Mualla; Tuncer, Murat; Gümrük, Fatma; Özen, Seza

    2016-10-01

    Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years. More SJIA patients had MAS at disease onset than SLE patients (53.6 vs. 16.7 %). Fever, high C-reactive protein and hyperferritinemia were present in all MAS episodes. Rash was less (p = 0.03), and fatigue was more frequent (p = 0.042) in SLE than SJIA patients. All received corticosteroids. Cyclosporine was given in 74.2 % of SJIA-MAS; 66.7 % of SLE-MAS episodes. Intravenous immunoglobulin, anakinra, or etoposide was administered during 67.7; 41.9; 32.3 % of SJIA-MAS and 33.3; 33.3; 50 % of SLE-MAS episodes, respectively. Plasmapheresis was performed during 41.9 % of SJIA-MAS and 33.3 % of SLE-MAS episodes. The mortality rate was 11.8 % (n = 4;3 SJIA, 1 SLE). Hepatosplenomegaly was more frequent (p = 0.005), and plasmapheresis was performed more frequently (p = 0.021) in the patients who died compared to the cured patients. The median duration between symptom onset and admission to our hospital was longer among the patients who died (16.5 vs. 7 days; p = 0.049). Our patients' characteristics were similar to the reported cases, but our mortality rate is slightly higher probably due to late referral to our center. Early diagnosis and effective treatment are crucial to prevent mortality.

  1. A case of minimal change nephrotic syndrome with acute renal failure complicating Hashimotoâs disease.

    Science.gov (United States)

    Iwazu, Y; Nemoto, J; Okuda, K; Nakazawa, E; Hashimoto, A; Fujio, Y; Sakamoto, M; Ando, Y; Muto, S; Kusano, E

    2008-01-01

    A 63-year-old man was admitted to our hospital for evaluation of generalized edema. Coexistence of severe hypothyroidism and nephrotic syndrome was detected by laboratory examination. High titer of both antimicrosomal antibody and antithyroid peroxidase antibody indicated Hashimotoâs disease. Renal biopsy showed minimal change glomerular abnormality, but no findings of membranous nephropathy. A series of medical treatments, including steroid therapy, thyroid hormone and human albumin replacement therapy, were administered. However, acute renal failure accompanied by hypotension, was not sufficiently prevented. After 9 sessions of plasmapheresis therapy, the severe proteinuria and low serum albumin levels were improved. Even after resting hypotension was normalized, neither renal function nor thyroid function were fully recovered. After discharge, renal function gradually returned to normal, and the blood pressure developed into a hypertensive state concomitant with the normalization of thyroid function. This report is a rare case of autoimmune thyroid disease complicated with minimal change nephrotic syndrome. In most cases of nephritic syndrome, acute renal failure (ARF) has been reported to coexist with hypertension. Although pseudohypothyroidism is well-known in nephrotic pathophysiology, complications of actual hypothyroidism are uncommon. It is suggested that the development of hypotension and ARF could be enhanced not only by hypoproteinemia, but also by severe hypothyroidism.

  2. Treatment of Wegener’s granulomatosis

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    G. Valesini

    2011-09-01

    Full Text Available Treatment of Wegener’s granulomatosis, often a life-threatening disease, has greatly improved, considering that before corticosteroids and immunosuppressives were available, the average survival time of patients amounted to no more than 5 months. The management of Wegener’s granulomatosis can be divided in two stages: induction of remission and maintenance of remission. The standard regimen for the induction of remission consists of cyclophosphamide, 2 mg/kg/day orally, in combination with prednisone, 1mg/Kg/day orally, with a gradual tapering once remission has been obtained. To lower the overall cumulative dose, monthly intravenous pulses of cyclophosphamide have been evaluated. Other alternative treatments as high doses of corticosteroids, methotrexate, or plasmapheresis have been proposed, together as prophylaxis with trimethoprim-sulfamethoxazole. To minimize toxicity, for maintenance therapy other drugs are also used such as methotrexate, azathioprine, cyclosporine. Frequent therapeutic changes are needed due to the great variability of the disease; while important aspects are the recognition and treatment of relapse, and include not only the management of resistant disease, but also some particular aspects such as disease in chronic dialysis, renal transplant, pregnancy. Other cytotoxic drugs like leflunomide or mycofenolic mofetil appear to be promising, while new efforts to identify more effective and less toxic therapies include biologic products, such as high-dose immunoglobulin, TNF antagonists and other monoclonal antibodies. Many different kind of clinical trials are going on to better evaluate the real efficacy and safety of these treatments in Wegener’s granulomatosis.

  3. Warfarin reversal emerging as the major indication for fresh frozen plasma use at a tertiary care hospital.

    Science.gov (United States)

    Ozgonenel, Bulent; O'Malley, Barbara; Krishen, Priya; Eisenbrey, A B

    2007-12-01

    Because of the increase in the use of warfarin in the population in recent years, reversal of warfarin-related coagulopathy has become common in daily hospital practice. Transfusion of fresh frozen plasma (FFP) is the preferred treatment method for urgent warfarin reversal in the USA. We have undertaken a 1-month audit of FFP usage to ascertain the impact of warfarin use on the consumption of FFP. Sixty percent of the 376 units of FFP that were transfused during the study month were used to reverse warfarin effects. The most common reason to reverse warfarin was bleeding. Thirty-three percent of the units were used for the treatment of other coagulopathies, 7% were used in therapeutic plasmapheresis, and banks of hospitals serving a predominantly elderly patient population should anticipate a higher consumption of FFP. Careful monitoring of warfarin therapy, stringent implementation of the warfarin reversal guidelines, and the introduction of newer products for warfarin reversal would help reduce the consumption of FFP.

  4. Anti-glomerular basement membrane glomerulonephritis in an HIV positive patient: case report

    Directory of Open Access Journals (Sweden)

    Eduardo José Bellotto Monteiro

    Full Text Available We report on a case of a patient with HIV infection, diagnosed 18 months prior to the development of an anti-glomerular basement membrane (anti-GBM rapidly progressive glomerulonephritis; this is probably the first report of such an association. A 30-year-old white man presented with elevation of serum creatinine (1.3 - 13.5 mg/dL within one month. At admission, the urinalysis showed proteinuria of 7.2 g/L and 8,000,000 erythrocytes/mL. Renal biopsy corresponded to a crescentic diffuse proliferative glomerulonephritis mediated by anti-GBM, and serum testing for anti-GBM antibodies was positive; antinuclear antibodies (ANA and anti-neutrophilic cytoplasmic antibodies (ANCA were also positive. The patient underwent hemodyalisis and was treated with plasmapheresis, cyclophosphamide and prednisone. The association described here is not casual, as crescentic glomerulonephritis is not common in HIV-positive patients, anti-GBM glomerulonephritis is rare and anti-GBM antibodies are frequently observed in HIV-positive subjects when compared to the overall population. Based on the current case and on the elevated frequency of the positivity for such antibodies in this group of patients, it is advisable to be aware of the eventual association between these two conditions and to promote an active search for anti-GBM antibodies and early diagnosis of eventual urinary abnormalities in HIV-positive subjects, considering the severity of anti-GBM glomerulonephritis.

  5. First Bahraini adolescent with anti-NMDAR-Ab encephalitis.

    Science.gov (United States)

    Almuslamani, Ahood; Mahmood, Fatima

    2015-01-01

    Anti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is a new autoimmune, often paraneoplastic disorder that presents with complex neuropsychiatric symptoms. It was first described in 2007 by Dalmau et al. Our patient presented with headache, behavioral changes and then seizures with hallucinations. She was initially misdiagnosed to have schizophrenia and was prescribed antipsychotics. She deteriorated and developed further seizures with hypoventilation and choreoathetosis. Her blood investigations were positive for mycoplasma IGM. Her CSF studies showed high white cell counts, predominantly lymphocytes, and high anti-NMDA-R titre. Her brain MRI scans showed high tbl2 and FLAIR intensities in the grey and white matter of the left cerebellar hemisphere suggestive of acute disseminated encephalomyelitis. She responded to treatment with antibiotics, multiple antiepileptics, steroids and needed five sessions of plasmapheresis. There was no underlying malignancy on repeated scanning of the abdomen. She needed around one year for full recovery with intensive rehabilitation. The objective of this paper was to highlight the occurrence of this fairly new, challenging, easily missed, not-so-rare form of encephalitis often occurring in the absence of fever.

  6. Oncological emergencies for the internist

    Directory of Open Access Journals (Sweden)

    Umesh Das

    2015-01-01

    Full Text Available An oncologic emergency is defined as any acute, potentially life-threatening event, either directly or indirectly related to a patient′s cancer (ca or its treatment. It requires rapid intervention to avoid death or severe permanent damage. Most oncologic emergencies can be classified as metabolic, hematologic, structural, or side effects from chemotherapy agents. Tumor lysis syndrome is a metabolic emergency that presents as severe electrolyte abnormalities. The condition is treated with aggressive hydration, allopurinol or urate oxidase to lower uric acid levels. Hypercalcemia of malignancy is treated with aggressive rehydration, furosemide, and intravenous (IV bisphosphonates. Syndrome of inappropriate antidiuretic hormone should be suspected if a patient with ca presents with normovolemic hyponatremia. This metabolic condition usually is treated with fluid restriction and furosemide. Febrile neutropenia is a hematologic emergency that usually requires inpatient therapy with broad-spectrum antibiotics, although outpatient therapy may be appropriate for low-risk patients. Hyperviscosity syndrome usually is associated with Waldenstrφm′s macroglobulinemia, which is treated with plasmapheresis and chemotherapy. Structural oncologic emergencies are caused by direct compression of surrounding structures or by metastatic disease. Superior vena cava syndrome is the most common structural oncological emergency. Treatment options include chemotherapy, radiation, and IV stenting. Epidural spinal cord compression can be treated with dexamethasone, radiation, or surgery. Malignant pericardial effusion, which often is undiagnosed in ca patients, can be treated with pericardiocentesis or a pericardial window procedure.

  7. Diagnostics And Treatment Of Patients With Blood Circulation Insufficiency In Vertebrobasilar Bed

    Directory of Open Access Journals (Sweden)

    A.M. Khachatryan

    2009-12-01

    Full Text Available For improvement of treatment results of patients with vertebrobasilar insufficiency we have analyzed data of medical examination and treatment of 182 patients stayed in clinics of Hospital Surgery and Nervous System Diseases from the 1st of January of 2005 till the 30th of June of 2009. Method of screening diagnostics, quantitative and qualitative blood flow estimation by means of Doppler ultrasonic investigation and angiography have been performed. The cause of blood circulation insufficiency in the vertebrobasilar bed at 87 patients was vertebral artery syndrome and in 95 cases there were the significant hemodynamical damages of blood flow in aorta arch branches and intracranial arteries. The neuroangiotropic therapy has been made for all patients, but the clinical improvement period was short in most cases, more prolonged effect was observed in addition of conservative therapy with plasmapheresis by indications. In the complex treatment of patients with vertebrobasilar insufficiency accompanied by degenerative diseases of spine chemical sympathectomy of vertebral artery in the III segment in the form of procaine and spirit-procaine blockades was made in 116 and 69 cases accordingly. The surgical sympathectomy was made in 15 patients. The surgical operations were made in 62 of 95 patients with significant hemodynamic pathology of blood flow in the vertebrobasilar region. In most cases isolative and combinative reconstructive surgery on carotid arteries was carried out. It played a significant role in blood flow correction. Favorable postoperative results were observed in most cases (93, 5%

  8. Successful long-term (22 year) treatment of limited scleroderma using therapeutic plasma exchange: Is blood rheology the key?

    Science.gov (United States)

    Harris, Edward S; Meiselman, Herbert J; Moriarty, Patrick M; Weiss, John

    2017-01-01

    While a number of studies have shown short-term beneficial effects of therapeutic plasma exchange (TPE) for treating systemic scleroderma (SSc), there have been no reports on the very long-term usage of TPE as the sole systemic treatment intervention. We report the case of a male patient, originally diagnosed with limited systemic scleroderma (lcSSc) in early 1990, who has been undergoing regular plasmapheresis treatments for more than 22 years, beginning in late 1993. Prior to commencing treatment, the patient exhibited symptoms including severe gastro esophageal reflux disease (GERD) with esophagitis, frequent Raynaud's attacks, reduced lung function, and chronic chilling. With the exception of mild residual Raynaud's, all of the patient's symptoms reversed after three years of regular TPE treatments and he remains in complete remission. While the typical explanation for the therapeutic benefits seen with TPE focuses on temporary reduction of circulating antibodies or other pathogenic factors, we propose instead an explanation based on abnormal blood rheology as a novel disease pathogenesis model for SSc.

  9. Donor HLA-specific Abs: to BMT or not to BMT?

    Science.gov (United States)

    Leffell, M S; Jones, R J; Gladstone, D E

    2015-06-01

    The engraftment failure associated with Abs to donor-specific HLA (DSA) limits options for sensitized BMT candidates. Fourteen of fifteen patients with no other viable donor options were desensitized and transplanted using a regimen of plasmapheresis and low-dose i.v. Ig modified to accommodate pre-BMT conditioning. DSA levels were assessed by solid-phase immunoassays and cell-based crossmatch tests. DSA levels were monitored throughout desensitization and on day -1 to determine if there was any DSA rebound that would require additional treatment. A mean reduction in DSA level of 64.4% was achieved at the end of desensitization, with a subsequent reduction of 85.5% after transplantation. DSA in 11 patients was reduced to levels considered negative post-BMT, whereas DSA in three patients remained at low levels. All 14 patients achieved donor engraftment by day +60; however, seven patients suffered disease relapses. Four patients experienced mild, grade 1 GVHD. Factors influencing the response to desensitization include initial DSA strength, number, specificity, DSA rebound and a mismatch repeated from a prior transplant. While desensitization should be reserved for patients with limited donor options, careful DSA assessment and monitoring can facilitate successful engraftment after BMT.

  10. A fatal case of acute pulmonary embolism caused by right ventricular masses of acute lymphoblastic lymphoma-leukemia in a 13 year old girl

    Directory of Open Access Journals (Sweden)

    Yu Mi Ko Ko

    2012-07-01

    Full Text Available We report a case of a 13-year-old girl with acute lymphoblastic lymphoma- leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.

  11. The evidence for immunotherapy in dermatomyositis and polymyositis: a systematic review.

    Science.gov (United States)

    Vermaak, Erin; Tansley, Sarah L; McHugh, Neil J

    2015-12-01

    Dermatomyositis and polymyositis are rare chronic inflammatory disorders with significant associated morbidity and mortality despite treatment. High-dose corticosteroids in addition to other interventions such as immunosuppressants, immunomodulators, and more recently, biologics are commonly used in clinical practice; however, there are no clear guidelines directing their use. Our objective was to systematically review the evidence for immunotherapy in the treatment of dermatomyositis and polymyositis. Relevant studies were identified through Embase and PubMed database searches. Trials were selected using pre-determined selection criteria and then assessed for quality. Randomized controlled trials and experimental studies without true randomization and including adult patients with definite or probable dermatomyositis or polymyositis were evaluated. Any type of immunotherapy was considered. Clinical improvement, judged by assessment of muscle strength after 6 months, was the primary outcome. Secondary outcomes included IMACS definition of improvement, improvements in patient and physician global scores, physical function, and muscle enzymes. Twelve studies met eligibility criteria. Differences in trial design, quality, and variable reporting of baseline characteristics and outcomes made direct comparison impossible. Although no treatment can be recommended on the basis of this review, improved outcomes were demonstrated with a number of agents including methotrexate, azathioprine, ciclosporin, rituximab, and intravenous immunoglobulin. Plasmapheresis and leukapheresis were of no apparent benefit. More high-quality randomized controlled trials are needed to establish the role of immunosuppressive agents in the treatment of these conditions and the clinical context in which they are most likely to be beneficial.

  12. Postulated Role of Vasoactive Neuropeptide-Related Immunopathology of the Blood Brain Barrier and Virchow-Robin Spaces in the Aetiology of Neurological-Related Conditions

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    D. R. Staines

    2008-01-01

    Full Text Available Vasoactive neuropeptides (VNs such as pituitary adenylate cyclase-activating polypeptide (PACAP and vasoactive intestinal peptide (VIP have critical roles as neurotransmitters, vasodilators including perfusion and hypoxia regulators, as well as immune and nociception modulators. They have key roles in blood vessels in the central nervous system (CNS including maintaining functional integrity of the blood brain barrier (BBB and blood spinal barrier (BSB. VNs are potent activators of adenylate cyclase and thus also have a key role in cyclic AMP production affecting regulatory T cell and other immune functions. Virchow-Robin spaces (VRSs are perivascular compartments surrounding small vessels within the CNS and contain VNs. Autoimmunity of VNs or VN receptors may affect BBB and VRS function and, therefore, may contribute to the aetiology of neurological-related conditions including multiple sclerosis, Parkinson's disease, and amyotrophic lateral sclerosis. VN autoimmunity will likely affect CNS and immunological homeostasis. Various pharmacological and immunological treatments including phosphodiesterase inhibitors and plasmapheresis may be indicated.

  13. Guillain-Barré syndrome and Crohn disease: a case report.

    Science.gov (United States)

    Yanagida, Hidehiko; Sugimoto, Keisuke; Izu, Akane; Wada, Norihisa; Sakata, Naoki; Takemura, Tsukasa

    2014-09-01

    Development of both Crohn disease and Guillain-Barré syndrome likely involves autoimmunity associated with excessive inflammatory cytokines. We treated a girl who developed Guillain-Barré syndrome during the course of Crohn disease. Although high-dose γ-globulin therapy administered initially for Guillain-Barré syndrome was ineffective, plasmapheresis ameliorated her acute neuropathic symptoms. Crohn disease was managed with Salazopyrin administration and enteral feeding. Chronic inflammation of the intestinal mucosa caused by Crohn disease can allow presentation of microbial intestinal antigens normally hidden from the immune system. Such presentation could incite an extraintestinal immune response on the basis of molecular mimicry, leading to activation of systemic autoimmunity against the nervous system. Accordingly, concurrence of Guillain-Barré syndrome and Crohn disease in our patient appeared to result from shared autoimmune mechanisms and systemic and local increases in cytokine concentrations. The patient also developed erythema nodosum and gall stones, relatively common complications of Crohn disease. However, Guillain-Barré syndrome is rare.

  14. Successful rescue of refractory, severe antibody mediated rejection with splenectomy.

    Science.gov (United States)

    Kaplan, Bruce; Gangemi, Antonio; Thielke, James; Oberholzer, José; Sankary, Howard; Benedetti, Enrico

    2007-01-15

    Antibody-mediated rejection (AMR) commonly occurs after transplantation of ABO-incompatible and sensitized renal transplant. Treatment regimens commonly include a combination of plasmapheresis (PL) and intravenous immunoglobulin (IVIG). However, some cases of AMR remain refractory to treatment. We report a case series of four patients with AMR refractory to standard therapy (ST) who resolved after splenectomy. Four living donor kidney transplant recipients were diagnosed with AMR. Two patients were ABO incompatible, one was cross-match positive and one had no obvious predisposing factors. After failure of therapy with corticosteroids, PL, IVIG, Thymoglobulin, and Rituximab (three patients) or Campath (one patient), AMR was treated with laparoscopic splenectomy. After an average of 11 days of ST, laparoscopic splenectomy was performed for rescue. The urinary output improved immediately in all patients, serum creatinine levels decreased within 48 hr, and ABO titers fell in the ABO-incompatible patient and the cross-match became negative in the two sensitized patients. Splenectomy may play a role in the treatment of AMR refractory to ST.

  15. Managing hypertriglyceridemia in children with systemic lupus erythematosus: Two sides of the same coin.

    Science.gov (United States)

    Basu, Biswanath; Babu, Binu George; Bhattacharyya, Suman

    2016-10-04

    Hypertriglyceridemia is common in children with systemic lupus erythematosus (SLE). A retrospective analysis of the baseline clinical-pathological presentation and treatment outcome (status of lipid profiles) was performed in two children with SLE, who presented with extreme hypertriglyceridemia over a follow-up period of four weeks. The children were treated with prednisolone, mycophenolate mofetil (MMF), hydroxychloroquine and hypolipidemic agents, depending on their disease status. On serial follow-up, the first child showed a significantly raised serum triglyceride level after receiving one week of oral prednisolone therapy. Anti-lipoprotein-lipase (LPL) autoantibody was absent. Lipid profile levels of this child gradually improved after replacing oral prednisolone with another immunosuppressant, namely MMF. The second child presented with extreme hypertriglyceridemia with positive anti-LPL autoantibody. She responded to plasmapheresis followed by increasing the dose of immunosuppressant. So, extreme hypertriglyceridemia in children with SLE may be steroid induced or due to presence of anti-LPL auto antibody. Management should be individualized depending on the etiology. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  16. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

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    Nathalie Foray

    2016-01-01

    Full Text Available Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA. Renal biopsy revealed crescentic glomerulonephritis (GN pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV. Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion. Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

  17. Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

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    Cesar A. Perez

    2011-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE. Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

  18. Primary Sjogren’s Syndrome Presented with Sensory Ataxia Associated with Bilateral Hearing Loss and Dementia

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    Madjdinasab Nastaran

    2009-10-01

    Full Text Available Primary Sjorgen syndrome is one of the commonest autoimmune diseases with characteristic of involvement of lachrymal and salivary glands, but other organ involvements as peripheral and central nervous system are also possible. The reported case is a 23 year old lady presented with progressive sensory ataxia and weakness of four limbs, bilateral sensory hearing loss and cognitive impairment with minimental score equal to 15/30 since one year prior to admission with associated bilateral central corneal opacity, dry mouth and dry eyes. Electro physiologic studies showed sensory motor axonal polyneuropathy . A biopsy of sural nerve and salivary glands of lower lip showed lymphocytic infiltration. Serologic evidence showed positive Anti Ro (SS-B, negative HCV and HIV antibody, thereafter the diagnosis was confirmed and according to this diagnosis she received high dose of intravenous methyl prednisolon then both hearing loss and cognitive impairment improved partially (minimental score 21/30 . At last, she underwent plasmapheresis and her sensory ataxia improved greatly.

  19. Adult Living Donor Liver Transplantation with ABO-Incompatible Grafts: A German Single Center Experience

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    Armin D. Goralczyk

    2009-01-01

    Full Text Available Adult living donor liver transplantations (ALDLTs across the ABO blood group barrier have been reported in Asia, North Americas, and Europe, but not yet in Germany. Several strategies have been established to overcome the detrimental effects that are attached with such a disparity between donor and host, but no gold standard has yet emerged. Here, we present the first experiences with three ABO-incompatible adult living donor liver transplantations in Germany applying different immunosuppressive strategies. Four patient-donor couples were considered for ABO-incompatible ALDLT. In these patients, resident ABO blood group antibodies (isoagglutinins were depleted by plasmapheresis or immunoadsorption and replenishment was inhibited by splenectomy and/or B-cell-targeted immunosuppression. Despite different treatments ALDLT could safely be performed in three patients and all patients had good initial graft function without signs for antibody-mediated rejection (AMR. Two patients had long-term graft survival with stable graft function. We thus propose the feasibility of ABO-incompatible ALDLT with these protocols and advocate further expansion of ABO incompatible ALDLT in multicenter trials to improve efficacy and safety.

  20. [Pathogenesis and morphology of hemophagocytic syndrome in the spleen].

    Science.gov (United States)

    Gattenlöhner, S; Müller-Hermelink, H K

    2008-03-01

    Hemophagocytic syndrome (macrophage activation syndrome) is a group of disorders characterized by clinical, histological and biological symptoms of T-lymphocytes/NK cell-driven macrophage stimulation with subsequent hemophagocytosis which can be observed in various organs, particularly in the bone marrow and spleen. The main clinical symptoms include cytopenia, multiple organ dysfunction, fever unresponsive to antibiotics, hepatosplenomegaly and rash. Besides rare inherited immune system anomalies (primary hemophagocytic syndrome), the most prevalent etiologies of hemophagocytic syndrome are infections (viral, bacterial and parasitic), cancers, lymphoproliferative disorders and systemic diseases. Hemophagocytic syndrome is treated with immunosuppressives, cytostatic drugs, plasmapheresis and intravenous immunoglobulins/anti-TNF antibodies and due to the multiorgan involvement and the associated high mortality, recognition of early serum changes, such as very high levels of ferritin is mandatory. The most relevant histological changes are the increase of histiocytes showing evidence of phagocytosis of erythroid cells, platelets and granulocytes as well as in the spleen a rarefaction of the white pulpa with lack of lymph follicles and abundant lymphocytes, plasma cells and plasma cell precursors in the pulpa cords.

  1. Antiphospholipid syndrome

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    Pavlović Dragan M.

    2010-01-01

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disease with recurrent thromboses and pregnancy complications (90% are female patients that can be primary and secondary (with concomitant autoimmune disease. Antiphospholipid antibodies are prothrombotic but also act directly with brain tissue. One clinical and one laboratory criterion is necessary for the diagnosis of APS. Positive serological tests have to be confirmed after at least 12 weeks. Clinical picture consists of thromboses in many organs and spontaneous miscarriages, sometimes thrombocytopaenia and haemolytic anaemia, but neurological cases are the most frequent: headaches, stroke, encephalopathy, seizures, visual disturbances, Sneddon syndrome, dementia, vertigo, chorea, balism, transitory global amnesia, psychosis, transversal myelopathy and Guillain-Barre syndrome. About 50% of strokes below 50 years of age are caused by APS. The first line of therapy in stroke is anticoagulation: intravenous heparin or low-weight heparins. In chronic treatment, oral anticoagulation and antiplatelet therapy are used, warfarin and aspirin, mostly for life. In resistant cases, corticosteroids, intravenous immunoglobulins and plasmapheresis are necessary. Prognosis is good in most patients but some are treatment-resistant with recurrent thrombotic events and eventually death.

  2. Acute flaccid paraplegia: neurological approach, diagnostic workup, and therapeutic options

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    Gentian Vyshka

    2015-03-01

    Full Text Available Acute flaccid paraplegia is a clinical occurrence with extreme importance, due to the dramatic presentation, the severity of the underlying disorder, and the generally poor prognosis that follows such a condition. Among etiological factors, the traumatic events are of particular interest, with the clinical treating dealing with a severely ill patient, following fall from height, motor vehicle collisions, and direct shocks applied over the vertebral column. The non-traumatic list is more numerous; however the severity of the acute paraplegia is not necessarily of a lesser degree. Viral infections, autoimmune disorders, and ischemic events involving feeding spinal arteries have been imputed. However, chemical and medications injected during procedures or accidentally intrathecal administration can produce acute flaccid paraplegia. A careful neurological assessment and complete electrophysiological and imaging studies must follow. In spite of the poor prognosis, different therapeutic options have been proposed and applied. Neurosurgical and orthopedic interventions are often necessary when trauma is present, with high dose glucocorticoids treatment preceding the intervention, in a hope to decrease edema-related compression over the spinal cord. Immunoglobulins and plasmapheresis are logical and helpful options when a polyradiculoneuritis produces such a clinical picture. The role of decompression, as neurosurgical exclusivity, has been considered as well.

  3. [A treatment of neuromyelitis optica (Devic's disease) during pregnancy].

    Science.gov (United States)

    Daouda, Moussa Toudou; Obenda, Norlin Samuel; Assadeck, Hamid; Camara, Diankanagbe; Djibo, Fatimata Hassane

    2016-01-01

    Neuromyelitis optica (Devic's disease) is an inflammatory demyelinating disease of the central nervous system that mainly affects spinal cord, optic nerve and brain regions with high aquaporin 4 antigen expression. This is a severe autoimmune disease caused by autoantibodies directed against aquaporin 4 and associated with high morbidity and mortality. Unlike other inflammatory conditions such as multiple sclerosis or rheumatoid polyarthritis, pregnancy does not seem to influence the activity of neuromyelitis optica, hence the need for a thorough treatment during pregnancy. Corticosteroid therapy is the treatment of choice for neuromyelitis optica during pregnancy. Other treatments may also be used including rituximab, some immunosuppressive agents and immunoglobulins. Immunosuppressive treatment or rituximab is recommended when the long-term corticosteroid treatment is contraindicated, in case of inefficiency or if side effects are intolerable. Immunoglobulins are administered to patients with serious outbreaks of neuromyelitis optica which do not respond to bolus methylprednisolone. Immunoglobulins alone can also be continued at a dose of 0.4 g/kg/day for 6-8 weeks until delivery. Plasmapheresis is also a good alternative to bolus methylprednisolone when outbreaks are extremely severe.

  4. [Controversial issues in the Giornale Italiano di Nefrologia: how to treat patients with focal segmental glomerular sclerosis].

    Science.gov (United States)

    Passerini, P; Scolari, F; Frasca', G M; Leoni, A; Venturelli, C; Dallera, N; Ravera, S; Balestra, E; Freddi, P; Fanciulli, E; D'Arezzo, M; Sagripanti, S

    2009-01-01

    Primary focal segmental glomerular sclerosis (FSGS) commonly presents with nephrotic syndrome. Spontaneous remission is rare and persistent nephrotic syndrome is a marker of poor prognosis. For this reason, obtaining remission using drugs with minimal side effects is desirable. The treatment of FSGS, however, represents a challenge. Not only is there a lack of prospective controlled trials, but FSGS is a syndrome of unknown pathophysiology, generally treated with drugs having a mechanism of action that is poorly understood in this setting, the use of which has often drawn criticism because it is based on empirical assumptions rather than pathogenetic evidence. At present, corticosteroids are the standard first-line approach in patients with idiopathic FSGS. Cytotoxic agents and cyclosporin A constitute a good therapeutic option for steroid-dependent patients or frequent relapsers. Mycophenolate mofetil, rituximab and plasmapheresis should be used as rescue treatment because further studies are required to determine their safety and efficacy. Clearly, real progress in FSGS treatment can only be obtained by research focused on the pathophysiology of this disease, so that a therapeutic approach can be defined that is based on reason rather than chance.

  5. Immunotherapy for anti-NMDAR encephalitis: A review of paraneoplastic, autoimmune encephalopathy

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    Ruu-Fen Tzang

    2017-03-01

    Full Text Available Paraneoplastic immune-mediated encephalopathy is a recently described disease. Dalmau et al first linked the presence of Anti-N-methyl-d-aspartate receptor (anti-NMDAR antibodies to paraneoplastic psychiatric and neurologic disease after encountering a case of anti-NMDAR encephalitis in a woman with ovarian teratoma in 2007. Nerve cells from a teratoma or previous viral infection could trigger autoantibodies, causing NMDA receptors to become dysfunctional in neurotransmission across synapses. Symptoms of anti-NMDAR encephalitis include prodromal symptoms, psychiatric symptoms, speech dysfunction, seizure, abnormal neurological movement, and autonomic dysfunction. This disease is reversible and treatable; however, early diagnosis and treatment are essential as they may prevent excess antibodies from causing severe or prolonged harm in the brain. First-line Immunotherapy includes intravenous high-dose steroids (methylprednisolone, intravenous immunoglobulin (IVIG, and/or plasmapheresis. Second-line immunotherapy includes targeted B-cell therapy with rituximab and cyclophosphamide. Taiwan is one of the countries where tests for detecting this disease, which are expensive, are not currently available. A cell-based indirect immunofluorescence test for the detection of IgG antibodies against the NMDA receptor should become more available for aiding diagnostics. Most importantly, early immune modulatory therapy including steroid, IVIG, and plasma exchange should become financially more feasible for use in treatment in Taiwan.

  6. [Refsum's disease. Epidemiologic, clinical and biological correlation. 6 cases].

    Science.gov (United States)

    Petit, H; Leys, D; Skjeldal, O H; Caron, J C; Lambert, P; Lehembre, P; Hache, J C

    1986-01-01

    Nine patients with symptoms and signs of Refsum's disease are reported. In 6 a systemic accumulation of phytanic acid was demonstrated, together with low phytanic acid oxidase activity in skin fibroblasts in 5 of them. In 3, no disorder of phytanic acid metabolism was demonstrated. In 3, the diagnosis was made during the pre-clinical period. The disease seems more frequent in Northern France, which agrees with the hypothesis of a genetic mutation which would have taken place in Scandinavia some centuries ago and was subsequently spread by the Vikings. The effects of a dietary treatment on serum phytanic acid levels and clinical disorders are reported. The general condition of the patients improved remarkably but only partially. The diet is unpalatable and in some patients the level of serum phytanic acid increased, due to the mobilization of body fat. Patients with very high levels of phytanic acid might be initially treated by plasmapheresis. For the same reason, the diet should supply enough calories to keep body weight unchanged, and body weight loss whatever its cause should be avoided.

  7. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Naóe; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia.

  8. Mesangial IgA deposits indicate pathogenesis of anti-glomerular basement membrane disease.

    Science.gov (United States)

    Wang, Aifeng; Wang, Yongping; Wang, Guobao; Zhou, Zhanmei; Xun, Zhang; Tan, Xiaohui

    2012-05-01

    Anti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic glomerulonephritis with immunoglobulin G (IgG) autoantibodies to the non-collagenous (NC1) domain of α3(IV) collagen presenting along the GBM. The patient clinically manifests with rapidly progressive glomerulonephritis (RPGN) with pulmonary hemorrhage (Goodpasture syndrome). In rare cases, other immunocomplexes of IgA or IgM are involved, but their specificities have not been determined. We report a rare case of a 31-year-old female who was diagnosed as having anti-GBM disease with extensive IgA deposits in the mesangium. This patient presented heavy hematuria, proteinuria with increasing creatinine, but no lung hemorrhage. Renal biopsy showed crescentic glomerulonephritis (type Ⅰ) with strong IgA (3+) as lump and branch shape. Therapies with pulse methylprednisolone, plasmapheresis and cyclophosphamide administration were less effective. This case is different from the present type Ⅰ crescentic glomerulonephritis and the specificity of IgA deposits may implicate the pathogenesis of anti-GBM disease.

  9. [Clinical consequence and significance of anti-neutrophil cytoplasmic antibody positivity in anti-glomerular basement membrane disease].

    Science.gov (United States)

    File, Ibolya; Pucsok, Klára; Trinn, Csilla; Ujhelyi, László; Balla, József; Mátyus, János

    2013-10-27

    Patients with renopulmonary syndrome who have both anti-neutrophil cytoplasmic and anti-glomerular basement membrane antibodies have been described since 1989. The aim of the authors was to analyse the data of "double positive" patients diagnosed in their department, and compare these with previous studies. During the last 16 years, 87 anti-neutrophil cytoplasmic antibody positive and 11 anti-glomerular basement membrane antibody positive patients were diagnosed. Four patients with anti-glomerular basement membrane antibodies (36%) had detectable anti-neutrophil cytoplasmic antibodies, 2 patients were positive for anti-myeloperoxidase and 2 patients for anti-proteinase 3. In comparison with patients having anti-glomerular basement membrane antibodies, the double-positive patients were characterized by older age (median of 46 vs. 24 years), lack of male dominance (50% vs. 71%), more frequent presence of previous extrarenal symptoms (50% vs. 0%), and lower anti-glomerular basement membrane antibody levels (<100EU/ml: 100% vs. 29%). The double-positive patients had more favourable 1-year survival (100% vs. 71%), despite their older age and similar treatment regimen (immunosuppression 100% in both groups, plasmapheresis in 75% vs. 86%), but 1-year renal survival was not different (25% vs. 14%). In agreement with literature data, about one third of patients with anti-glomerular basement membrane antibodies had detectable anti-neutrophil cytoplasmic antibodies, and the coexistence of the two antibodies may have clinical consequences.

  10. Rapidly Developing Toxic Epidermal Necrolysis

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    Viktoria Oline Barrios Poulsen

    2013-01-01

    Full Text Available Severe cutaneous reactions with potentially fatal outcomes can have many different causes. The Stevens-Johnson syndrome (SJS and toxic epidermal necrolysis (TEN are rare. They are characterized by a low incidence but high mortality, and drugs are most commonly implicated. Urgent active therapy is required. Prompt recognition and withdrawal of suspect drug and rapid intervention can result in favourable outcome. No further international guidelines for treatment exist, and much of the treatment relies on old or experimental concepts with no scientific evidence. We report on a 54-year-old man experiencing rapidly developing drug-induced severe TEN and presented multiorgan failure involving the respiratory and circulatory system, coagulopathy, and renal insufficiency. Detachment counted 30% of total body surface area (TBSA. SCORTEN = 5, indicating a mortality rate >90%. The patient was sedated and mechanically ventilated, supported with fluids and inotropes to maintain a stable circulation. Component therapy was guided by thromboelastography (TEG. The patient received plasmapheresis, and shock reversal treatment was initiated. He was transferred to a specialized intensive care burn unit within 24 hours from admittance. The initial care was continued, and hemodialysis was started. Pulmonary, circulatory, and renal sequelae resolved with intensive care, and re-epithelialization progressed slowly. The patient was discharged home on hospital day 19.

  11. Pulmonary alveolar proteinosis

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    B. Crestani

    2011-06-01

    Full Text Available Pulmonary alveolar proteinosis (PAP is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic “crazy paving” pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy or anti-GM-CSF antibodies (rituximab and plasmapheresis are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.

  12. [An experience of treatment of double positive myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) and anti-glomerular basement membrane antibodies in Goodpasture's syndrome onset of crescentic glomerulonephritis].

    Science.gov (United States)

    Takeda, T; Takeda, T; Naiki, Y; Yonekawa, S; Sakaguchi, M; Iwamoto, I; Tanaka, H; Hasegawa, H; Imada, A; Kanamaru, A; Hiruma, S; Maekura, S; Hashimoto, S; Yamazumi, T

    1998-11-01

    A 68-year-old woman was admitted to Kinki University Hospital because of progressive renal failure. She had been well until two months before admission. Laboratory data were as follows: serum creatinine 4.1 mg/dl, BUN 69 mg/dl, MPO-ANCA 33 EU, anti-glomerular basement membrane antibodies (AGBMA) 118 U. Histological findings showed cellular and fibrocellular crescents in many glomeruli. Therefore, we diagnosed rapidly progressive glomerulonephritis (RPGN) due to MPO-ANCA and anti-GBM associated renal disease. The patient was started on prednisolone and double filtration plasmapheresis (DFPP) therapy. Subsequently, the values of MPO-ANCA and AGBMA decreased. However, the patient's condition suddenly worsened and she died of interstitial pneumonia. Autopsy examination revealed crescentic glomerulonephritis and alveolar hemorrhage with linear deposition of IgG along the glomerular and alveolar capillary walls by immunofluorescence studies. We considered this to be a rare case of Goodpasture's syndrome associated with not only anti-GBM antibodies, but also MPO-ANCA.

  13. Outcome after Desensitization in HLA or ABO-Incompatible Kidney Transplant Recipients: A Single Center Experience.

    Science.gov (United States)

    Kauke, Teresa; Klimaschewski, Sandra; Schoenermarck, Ulf; Fischereder, Michael; Dick, Andrea; Guba, Markus; Stangl, Manfred; Werner, Jens; Meiser, Bruno; Habicht, Antje

    2016-01-01

    The shortage of deceased donors led to an increase of living donor kidney (LDK) transplantations performed in the presence of donor-specific antibodies (DSA) or ABO incompatibility (ABOi) using various desensitization protocols. We herein analyzed 26 ABOi and 8 Luminex positive DSA patients who were successfully desensitized by anti-CD20, antigen-specific immunoadsorption and/or plasmapheresis to receive an LDK transplant. Twenty LDK recipients with non-donor-specific HLA-antibodies (low risk) and 32 without anti-HLA antibodies (no risk) served as control groups. 1-year graft survival rate and renal function was similar in all 4 groups (creatinine: 1.63 ± 0.5 vs 1.78 ± 0.6 vs 1.64 ± 0.5 vs 1.6 ± 0.3 mg/dl in ABOi, DSA, low risk and no risk group). The incidence of acute T-cell mediated rejections did not differ between the 4 groups (15% vs 12, 5% vs 15% vs 22% in ABOi, DSA, low risk and no risk), while antibody-mediated rejections were only found in the DSA (25%) and ABOi (7.5%) groups. Incidence of BK nephropathy (BKVN) was significantly more frequent after desensitization as compared to controls (5/34 vs 0/52, p = 0.03). We demonstrate favorable short-term allograft outcome in LDK transplant recipients after desensitization. However, the desensitization was associated with an increased risk of BKVN.

  14. Successful Renal Transplantation with Desensitization in Highly Sensitized Patients: A Single Center Experience

    Science.gov (United States)

    Yoon, Hye Eun; Hyoung, Bok Jin; Hwang, Hyeon Seok; Lee, So Young; Jeon, Youn Joo; Song, Joon Chang; Oh, Eun-Jee; Park, Sun Cheol; Choi, Bum Soon; Moon, In Sung; Kim, Yong Soo

    2009-01-01

    Intravenous immunoglobulin (IVIG) and/or plasmapheresis (PP) are effective in preventing antibody-mediated rejection (AMR) of kidney allografts, but AMR is still a problem. This study reports our experience in living donor renal transplantation in highly sensitized patients. Ten patients with positive crossmatch tests or high levels of panel-reactive antibody (PRA) were included. Eight patients were desensitized with pretransplant PP and low dose IVIG, and two were additionally treated with rituximab. Allograft function, number of acute rejection (AR) episodes, protocol biopsy findings, and the presence of donor-specific antibody (DSA) were evaluated. With PP/IVIG, six out of eight patients showed good graft function without AR episodes. Protocol biopsies revealed no evidence of tissue injury or C4d deposits. Of two patients with AR, one was successfully treated with PP/IVIG, but the other lost graft function due to de novo production of DSA. Thereafter, rituximab was added to PP/IVIG in two cases. Rituximab gradually decreased PRA levels and the percentage of peripheral CD20+ cells. DSA was undetectable and protocol biopsy showed no C4d deposits. The graft function was stable and there were no AR episodes. Conclusively, desensitization using PP/IVIG with or without rituximab increases the likelihood of successful living donor renal transplantation in sensitized recipients. PMID:19194545

  15. Antibody-Mediated Rejection: Pathogenesis, Prevention, Treatment, and Outcomes

    Directory of Open Access Journals (Sweden)

    Olivia R. Blume

    2012-01-01

    Full Text Available Antibody-mediated rejection (AMR is a major cause of late kidney transplant failure. It is important to have an understanding of human-leukocyte antigen (HLA typing including well-designed studies to determine anti-MHC-class-I-related chain A (MICA and antibody rejection pathogenesis. This can allow for more specific diagnosis and treatment which may improve long-term graft function. HLA-specific antibody detection prior to transplantation allows one to help determine the risk for AMR while detection of DSA along with a biopsy confirms it. It is now appreciated that biopsy for AMR does not have to include diffuse C4d, but does require a closer look at peritubular capillary microvasculature. Although plasmapheresis (PP is effective in removing alloantibodies (DSAs from the circulation, rebound synthesis of alloantibodies can occur. Splenectomy is used in desensitization protocols for ABO incompatible transplants as well as being found to treat AMR refractory to conventional treatment. Also used are agents targeted for plasma cells, B cells, and the complement cascade which are bortezomib rituximab and eculizumab, respectively.

  16. Preoperative selective desensitization of live donor liver transplant recipients considering the degree of T lymphocyte cross-match titer, model for end-stage liver disease score, and graft liver volume.

    Science.gov (United States)

    Hong, Geun; Yi, Nam-Joon; Suh, Suk-won; Yoo, Tae; Kim, Hyeyoung; Park, Min-Su; Choi, YoungRok; Lee, Kyungbun; Lee, Kwang-Woong; Park, Myoung Hee; Suh, Kyung-Suk

    2014-05-01

    Several studies have suggested that a positive lymphocyte cross-matching (XM) is associated with low graft survival rates and a high prevalence of acute rejection after adult living donor liver transplantations (ALDLTs) using a small-for-size graft. However, there is still no consensus on preoperative desensitization. We adopted the desensitization protocol from ABO-incompatible LDLT. We performed desensitization for the selected patients according to the degree of T lymphocyte cross-match titer, model for end-stage liver disease (MELD) score, and graft liver volume. We retrospectively evaluated 230 consecutive ALDLT recipients for 5 yr. Eleven recipients (4.8%) showed a positive XM. Among them, five patients with the high titer (> 1:16) by antihuman globulin-augmented method (T-AHG) and one with a low titer but a high MELD score of 36 were selected for desensitization: rituximab injection and plasmapheresis before the transplantation. There were no major side effects of desensitization. Four of the patients showed successful depletion of the T-AHG titer. There was no mortality and hyperacute rejection in lymphocyte XM-positive patients, showing no significant difference in survival outcome between two groups (P=1.000). In conclusion, this desensitization protocol for the selected recipients considering the degree of T lymphocyte cross-match titer, MELD score, and graft liver volume is feasible and safe.

  17. Antibody desensitization therapy in highly sensitized lung transplant candidates.

    Science.gov (United States)

    Snyder, L D; Gray, A L; Reynolds, J M; Arepally, G M; Bedoya, A; Hartwig, M G; Davis, R D; Lopes, K E; Wegner, W E; Chen, D F; Palmer, S M

    2014-04-01

    As HLAs antibody detection technology has evolved, there is now detailed HLA antibody information available on prospective transplant recipients. Determining single antigen antibody specificity allows for a calculated panel reactive antibodies (cPRA) value, providing an estimate of the effective donor pool. For broadly sensitized lung transplant candidates (cPRA ≥ 80%), our center adopted a pretransplant multi-modal desensitization protocol in an effort to decrease the cPRA and expand the donor pool. This desensitization protocol included plasmapheresis, solumedrol, bortezomib and rituximab given in combination over 19 days followed by intravenous immunoglobulin. Eight of 18 candidates completed therapy with the primary reasons for early discontinuation being transplant (by avoiding unacceptable antigens) or thrombocytopenia. In a mixed-model analysis, there were no significant changes in PRA or cPRA changes over time with the protocol. A sub-analysis of the median fluorescence intensity (MFI) change indicated a small decline that was significant in antibodies with MFI 5000-10,000. Nine of 18 candidates subsequently had a transplant. Posttransplant survival in these nine recipients was comparable to other pretransplant-sensitized recipients who did not receive therapy. In summary, an aggressive multi-modal desensitization protocol does not significantly reduce pretransplant HLA antibodies in a broadly sensitized lung transplant candidate cohort. © Copyright 2014 The American Society of Transplantation and the American Society of Transplant Surgeons.

  18. Reducing sample complexity of polyclonal human autoantibodies by chromatofocusing.

    Science.gov (United States)

    Hagemann, Sascha; Faude, Alexander; Rabenstein, Monika; Balzer-Geldsetzer, Monika; Nölker, Carmen; Bacher, Michael; Dodel, Richard

    2010-08-15

    Chromatofocusing was performed in order to separate a polyclonal antigen-specific mixture of human immunoglobulins (IgGs) that would then allow for further analyses of as few different IgGs as possible. Because polyclonal IgGs only differ by amino acid sequence and possible post-translational modifications but not by molecular weight, we chose chromatofocusing for protein separation by different isoelectric points. We isolated antigen-specific IgGs from commercially available intravenous immunoglobulins (IVIG) using a combination of affinity- and size exclusion-chromatography and in order to reduce the complexity of the starting material IVIG was then replaced by single-donor plasmapheresis material. Using two-dimensional gel electrophoresis (2-DE), we observed a clear decrease in the number of different light and heavy chains in the chromatofocusing peak as compared to the starting material. In parallel, we monitored slight problems with the selected peak in isoelectric focusing as the first dimension of 2-DE, displayed in by the less proper focusing of the spots. When we tested whether IgGs were binding to their specific antigen after chromatofocusing, we were able to show that they were still in native conformation. In conclusion, we showed that chromatofocusing can be used as a first step in the analysis of mixtures of very similar proteins, e.g. polyclonal IgG preparations, in order to minimize the amount of different proteins in separated fractions in a reproducible way. Copyright 2010 Elsevier B.V. All rights reserved.

  19. The insulin autoimmune syndrome (IAS) as a cause of hypoglycaemia: an update on the pathophysiology, biochemical investigations and diagnosis.

    Science.gov (United States)

    Ismail, Adel A A

    2016-11-01

    Insulin autoimmune syndrome (IAS) is considered to be very rare in Caucasians. Understanding its pathophysiology is paramount in (a) appreciating its potential impact on analyses of pancreatic hormones and (b) explaining its highly variable clinical manifestations in non-diabetic, non-acutely ill patients with indeterminate hypoglycaemia. The underlying aetiology of IAS is the presence of variable affinity/avidity endogenous insulin antibodies in significant amounts. The two types of insulin antibodies namely antibodies which bind insulin and/or proinsulin(s) and receptor antibodies (insulin mimetic) will be discussed. Their biochemical and immunological roles in causing hypoglycaemia will be highlighted. Clinical manifestations of IAS can vary from mild and transient to spontaneous, severe and protracted hypoglycaemia necessitating in extreme cases plasmapheresis for glycaemic control. Antibodies of IAS can interfere in pancreatic immunoassay tests causing erroneous and potentially misleading results. Thorough testing for endogenous insulin antibodies must be considered in the investigations of non-diabetic, non-acutely ill patients with indeterminate and/or unexplained hypoglycaemia.

  20. Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

    Directory of Open Access Journals (Sweden)

    Kistler Thomas

    2011-06-01

    Full Text Available Abstract Background Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA. Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable. Case Presentation We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppresive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2, a novel autoantigen in patients with active small-vessel vasculitis (SVV. The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient. Conclusion We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.

  1. Morvan Syndrome: A Case Report With Patient Narrative and Video.

    Science.gov (United States)

    Maskery, Mark; Chhetri, Suresh K; Dayanandan, Rejith; Gall, Claire; Emsley, Hedley C A

    2016-01-01

    A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management.

  2. Thrombotic microangiopathy caused by oral contraceptives in a kidney transplant recipient.

    Science.gov (United States)

    Shirai, Hiroyuki; Yashima, Jun; Tojimbara, Tamotsu; Honda, Kazuho

    2016-07-01

    Thrombotic microangiopathy (TMA) after kidney transplantation has various aetiologies, including acute antibody-mediated rejection, bacterial or viral infection and immunosuppressive drugs, particularly calcineurin inhibitors. We present the case of a 28-year-old woman who developed TMA 30 months after the transplantation of an ABO-incompatible kidney from a living unrelated donor. The patient developed a sudden onset of allograft renal dysfunction and became uremic. She was transferred to our institution from a community hospital with strongly suspected acute allograft rejection. Intensive treatments for both T- and B-cell mediated acute rejection, including steroid pulse therapy, double-filtration plasmapheresis, antithymocyte globulin (1.5 mg/kg × 14 days) and rituximab (100 mg), were initiated during haemodialysis. However, her renal allograft function did not improve. Histopathological analysis 8 days after the treatment indicated TMA, despite the absence of apparent acute T-cell- or acute antibody-mediated rejection. There were no symptoms of infectious diseases, such as intestinal haemorrhagic colitis or viral infection. We concluded that the use of oral contraceptives, which had been initiated 3 weeks before TMA onset for the treatment of irregular vaginal bleeding, was the aetiologic agent.

  3. Anti-glomerular basement membrane disease superimposed on membranous nephropathy: a case report and review of the literature

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    Nivera Noel

    2010-08-01

    Full Text Available Abstract Introduction Anti-glomerular basement membrane disease is a rare autoimmune disorder characterized by pulmonary hemorrhage, crescentic glomerulonephritis and the presence of circulating anti-glomerular basement membrane antibodies. The simultaneous occurrence of both anti-glomerular basement membrane disease and membranous nephropathy is rare. Case presentation A 59-year-old Hispanic man presented with acute onset of nausea and vomiting and was found to have renal insufficiency. Work-up included a kidney biopsy, which revealed anti-glomerular basement membrane disease with underlying membranous nephropathy. He was treated with emergent hemodialysis, intravenous corticosteroids, plasmapheresis, and cyclophosphamide without improvement in his renal function. Conclusion Simultaneous anti-glomerular basement membrane disease and membranous nephropathy is very rare. There have been 16 previous case reports in the English language literature that have been associated with a high mortality and morbidity, and a very high rate of renal failure resulting in hemodialysis. Co-existence of membranous nephropathy and anti-glomerular basement membrane disease may be immune-mediated, although the exact mechanism is not clear.

  4. Neuro-ophthalmologic manifestations of systemic lupus erythematosus: a systematic review.

    Science.gov (United States)

    Man, Bik Ling; Mok, Chi Chiu; Fu, Yat Pang

    2014-06-01

    Herein we summarize the clinical presentation, treatment and outcome of neuro-ophthalmologic manifestations in patients with systemic lupus erythematosus (SLE). We performed a systematic review of the neuro-ophthalmologic manifestations of SLE reported in the English literature from 1970 to 2010 by a Medline search. The prevalence of neuro-ophthalmologic manifestations is 3.6% in adult and 1.6% in childhood SLE patients. Neuro-ophthalmologic manifestations of SLE are highly variable, with the commonest presentation being optic neuritis, followed by myasthenia gravis, visual field defects and pseudotumor cerebri. The underlying pathology was thought to be either SLE activity or its vascular complications. Most neuro-ophthalmologic manifestations of SLE are responsive to high-dose glucocorticoids. Anticoagulation is indicated when there is concomitant antiphospholipid syndrome. SLE-related neuromyelitis optica is often refractory to treatment and 92% of patients require multiple immunosuppressive protocols. Neuro-ophthalmologic manifestations of SLE are uncommon but heterogeneous. The prognosis of neuro-ophthalmologic manifestations in SLE is generally good because of their rapid response to glucocorticoids. Relapses of these manifestations may be reduced by the use of maintenance immunosuppression. Cyclophosphamide, azathioprine, plasmapheresis, intravenous immunoglobulin and rituximab can be considered in glucocorticoid-dependent or refractory cases. Anticoagulation is indicated when there is concomitant antiphospholipid syndrome.

  5. Significance of iron reduction for the therapy of chronic hepatitis C

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    Nožić Darko

    2005-01-01

    Full Text Available Background. It has been established that many patients with chronic hepatitis C have elevated serum iron, feritin levels and iron deposits in the liver. Therefore, the liver damage due to hepatitis C virus may be aggravated with iron overload. In many studies higher levels of iron in the blood and the liver were connected with the decreased response to interferon-alfa therapy for chronic viral hepatitis C. Recent introduction of pegylated interferons plus ribavirin has improved the therapeutic response, so it is now possible to cure more than 50% of the patients. Case report. Three patients with chronic hepatitis C and iron overload were presented. Iron reduction therapy using phlebotomy or eritrocytapheresis with plasmapheresis was done at different times in regard to specific antiviral therapy or as a sole therapy. Conclusion. It has been shown that iron reduction, sole or combined with antiviral therapy, led to the deacreased aminotransferase serum activity and might have slow down the evolution of chronic hepatitis C viral infection.

  6. The Evaluation of Albumin Use in an Iranian University Hospital

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    Elaheh Shafiee, Haleh Rezaee, Taher Entezari, Hadi Hamishehkar

    2016-09-01

    Full Text Available Background: Albumin is an expensive protein colloidal solution with various indications, especially in critically ill patients. The vast use of albumin in health care centers (particularly ICUs, the theoretical danger of contaminant transmission (as with any blood derivative, and the existence of more economical alternatives of equal efficacy evidence the importance of conducting a drug-utilization evaluation. The objective of this study was to assess the usage of albumin in patients at a hospital in Iran. Methods: Albumin administration was evaluated in 210 patients from different wards on randomly selected days during one year. Reasons for the prescription, the consumed dose, length of administration, and related laboratory tests were recorded. Results: Albumin was prescribed inappropriately in 76.2% and appropriately in 23.8% of inpatients. The most frequent inappropriate prescribing motives were hypoalbuminemia (35.6%, nutritional support (32.5%, and edema (24.4%, while the most appropriate prescriptions were edema (46%, nephrotic syndrome (18%, and plasmapheresis (16%. The total amount of albumin used for 210 patients was 68930 g, from which 51290 g costing $274607.1429 was administered for inappropriate indications. Conclusion: Despite the many valid guidelines defining the appropriate indications of albumin, this study demonstrated the extensive inappropriate use of this expensive preparation in one of the largest university-affiliated hospitals in northwestern Iran. It seems advisable to have the consumption of albumin continuously monitored.

  7. Thrombotic thrombocytopenic purpura following salvage chemotherapy with paclitaxel, ifosfamide and cisplatin in a patient with a refractory germ cell tumor: A case report and review of the literature

    Science.gov (United States)

    ULAS, ARIFE; SILAY, KAMILE; AKINCI, SEMA; AKINCI, MUHAMMED BULENT; SENDUR, MEHMET ALI; DEDE, DIDEM SENER; POLAT, YUNUS HALIL; YALCIN, BULENT

    2015-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy that is characterized by microvascular thrombosis, thrombocytopenia, hemolysis and end organ damage. An extensive variety of drugs, including certain chemotherapeutic agents, have been associated with TTP. However, paclitaxel, cisplatin and ifosfamide regimen (TIP)-induced TTP has not previously been described. The present study reports the case of a 43-year-old patient with a refractory testicular germ cell tumor who developed acute TTP during TIP chemotherapy. Following the third cycle of TIP chemotherapy, the patient developed fever, anemia, thrombocytopenia and confusion. A diagnosis of TTP was established. Plasmapheresis was initiated as daily treatment in the first week, then continued every other day for 4 weeks. TIP chemotherapy was discontinued. The patient's clinical and neurological symptoms improved markedly after a week. Renal function and hemolysis improved, and the patient was discharged in a stable condition. The patient did not develop any complications and has been in remission for 5 months. The Naranjo adverse drug reaction probability scale indicated a likely association between TTP and the TIP chemotherapy regimen in this patient. This case is also investigated with regard to the associated literature to increase the awareness of TTP following chemotherapy. PMID:26622823

  8. Use of interleukin-2 for management of natalizumab-associated progressive multifocal leukoencephalopathy: case report and review of literature.

    Science.gov (United States)

    Dubey, Divyanshu; Zhang, Yinan; Graves, Donna; DeSena, Allen D; Frohman, Elliot; Greenberg, Benjamin

    2016-05-01

    A 51-year-old woman with relapsing-remitting multiple sclerosis (RRMS) and 3-year history of natalizumab use developed expressive aphasia. A brain magnetic resonance image (MRI) showed left frontotemporal and right parietal lesion with mild contrast enhancement and cerebrospinal fluid (CSF) was positive for John Cunningham virus (JCV) by polymerase chain reaction (PCR). The patient received five cycles of plasmapheresis followed by intravenous immunoglobulin. Despite this intervention, her speech deteriorated and she developed right hemiparesis. Upon referral to our institution, CSF quantitative JCV PCR was notable for 834 copies/ml. The patient was given an initial dose of 50,000 units of interleukin-2 (IL-2) subcutaneously (SQ) followed by 1 million units IL-2 SQ daily. Due to concern for immune reconstitution inflammatory syndrome (IRIS), the patient also received intravenous methylprednisone weekly. The regimen was tolerated well by the patient with no severe adverse effects. Clinically, the patient showed some improvement, and became more responsive and regained right lower extremity antigravity strength. After 12 weeks of IL-2 therapy, JCV quantitative PCR was notable for 31 copies/ml and the patient was more responsive. Due to persistence of JCV, IL-2 therapy was changed to mefloquine. At follow up after 6 months, the patient showed no clinical deterioration.

  9. Advances in the diagnosis, pathogenesis and treatment of CIDP.

    Science.gov (United States)

    Dalakas, Marinos C

    2011-08-16

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. Despite clinical challenges in diagnosis-owing in part to the existence of disease variants, and different views on how many electrophysiological abnormalities are needed to document demyelination-consensus criteria seem to have been reached for research or clinical practice. Current standard of care involves corticosteroids, intravenous immunoglobulin (IVIg) and/or plasmapheresis, which provide short-term benefits. Maintenance therapy with IVIg can induce sustained remission, increase quality of life and prevent further axonal loss, but caution is needed to avoid overtreatment. Commonly used immunosuppressive drugs offer minimal benefit, necessitating the development of new therapies for treatment-refractory patients. Advances in our understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules. New biomarkers and scoring systems represent emerging tools with the potential to predict therapeutic responses and identify patients with active disease for enrollment into clinical trials. This Review highlights the recent advances in diagnosing CIDP, provides an update on the immunopathology including new target antigens, and discusses current treatments, ongoing challenges and future therapeutic directions.

  10. Clinical trials in CIDP and chronic autoimmune demyelinating polyneuropathies.

    Science.gov (United States)

    Dalakas, Marinos C

    2012-05-01

    The main chronic autoimmune neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and anti-myelin-associated glycoprotein (MAG) demyelinating neuropathy. On the basis of randomized controlled studies, corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis provide short-term benefits in CIDP. MMN responds only to IVIg. Because in MMN and CIDP, IVIg infusions are required every 3-6 weeks to sustain benefits or long-term remissions, there is a need for "IVIg-sparing" agents. In CIDP, immunosuppressive drugs, such as azathioprine, cyclosporine, methotrexate, mycophenolate, and cyclophosphamide, are used, but controlled trials have not shown that they are effective. Controlled trials have also not shown benefit to any agents in anti-MAG neuropathy. However, clinicians use many immunosuppressive drugs in both settings, but all have potentially serious side effects and are only effective in some patients. Thus, there is a need for new therapies in the inflammatory and paraproteinemic neuropathies. New agents targeting T cells, B cells, and transmigration and transduction molecules are discussed as potential treatment options for new trials. The need for biomarkers that predict therapeutic responses or identify patients with active disease is emphasized, and the search for better scoring tools that capture meaningful changes after response to therapies is highlighted.

  11. Autoimmune antigenic targets at the node of Ranvier in demyelinating disorders.

    Science.gov (United States)

    Stathopoulos, Panos; Alexopoulos, Harry; Dalakas, Marinos C

    2015-03-01

    Mounting evidence suggests that autoantibodies contribute to the pathogenesis of demyelination in the PNS and CNS. Rapid reversal of electrophysiological blockade after plasmapheresis or intravenous immunoglobulin treatment for acute or chronic inflammatory demyelinating polyneuropathy is more likely to result from removal or neutralization of an antibody that impairs saltatory conduction than from remyelination. Although up to 30% of patients with acute or chronic inflammatory demyelinating polyneuropathy harbour autoantibodies, specific antigens have been identified in no more than 13% of cases. To date, autoantigens identified at the node of Ranvier include neurofascin 186, gliomedin and possibly moesin in the nodal domain, and contactin-1, Caspr1 and neurofascin 155 in the paranodal domain. In some patients with multiple sclerosis, paranodal CNPase and juxtaparanodal contactin-2 trigger a humoral response. This Review explores the molecular anatomy of the node of Ranvier, focusing on proteins with extracellular domains that could serve as antigens. The clinical implications of node-specific antibody responses are addressed, and the best approaches to identify antibodies that target nodal proteins are highlighted. Also discussed are the roles of these antibodies as either secondary, disease-exacerbating responses, or as a primary effector mechanism that defines demyelination or axonal degeneration at the node, identifies disease subtypes or determines response to treatments.

  12. Myasthenia gravis: an update for the clinician.

    Science.gov (United States)

    Sieb, J P

    2014-03-01

    This paper provides a thorough overview of the current advances in diagnosis and therapy of myasthenia gravis (MG). Nowadays the term 'myasthenia gravis' includes heterogeneous autoimmune diseases, with a postsynaptic defect of neuromuscular transmission as the common feature. Myasthenia gravis should be classified according to the antibody specificity [acetylcholine, muscle-specific receptor tyrosine kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), seronegative], thymus histology (thymitis, thymoma, atrophy), age at onset (in children; aged less than or more than 50 years) and type of course (ocular or generalized). With optimal treatment, the prognosis is good in terms of daily functions, quality of life and survival. Symptomatic treatment with acetylcholine esterase inhibition is usually combined with immunosuppression. Azathioprine still remains the first choice for long-term immunosuppressive therapy. Alternative immunosuppressive options to azathioprine include cyclosporin, cyclophosphamide, methotrexate, mycophenolate mofetil and tacrolimus. Rituximab is a promising new drug for severe generalized MG. Emerging therapy options include belimumab, eculizumab and the granulocyte- macrophage colony-stimulating factor. One pilot study on etanercept has given disappointing results. For decades, thymectomy has been performed in younger adults to improve non-paraneoplastic MG. However, controlled prospective studies on the suspected benefit of this surgical procedure are still lacking. In acute exacerbations, including myasthenic crisis, intravenous immunoglobulin, plasmapheresis and immunoadsorption are similarly effective.

  13. One Case of Dilantin Leading to Drug Rash with Eosinophilia and Systemic Symptoms%苯妥英钠致药物超敏反应综合征1例

    Institute of Scientific and Technical Information of China (English)

    胡金华; 程书权; 全毅; 黄成军; 冼永超

    2012-01-01

    患者男,30岁,癫痫病史13年,因口服含苯妥英钠的抗癫痫中成药物1月诱发药物超敏综合征(DIHS),并以全身弥漫性猩红热样皮疹、肝功能衰竭为主要表现.经肾上腺糖皮质激素、人免疫球蛋白、胆红素吸附+血浆置换术、护肝解毒等综合治疗后好转出院.出院后1周因肢体抽搐再次服药后两次出现前述类似表现.经停药、对症治疗康复.随访24周,未复发.%A 30 - year - old male patient, had an Epilepsy history of 13 years, having undergone one - month oral administration of antiepileptic drugs containing dilantin, had a drug - induced Hypersensitivity Syndrome ( DIHS) , with main features including diffusive scarlatiniform rash in the whole body and liver failure. After a comprehensive treatments by adrenal cortical hormone, people immunoglobulin, bilirubin plasmapheresis and liver detoxification etc, he was improved and discharged. A week later, syndromes mentioned above re - occurred after having taken twice the medications for another body twitching. With drug withdrawal and corresponding symptomatic treatment, the patient then recovered. Following - up 24 weeks found no recurrence.

  14. Central venous line complications and tip detection

    Directory of Open Access Journals (Sweden)

    Ameneh Rezaee Gheshlaghi

    2015-06-01

    Full Text Available Central venous line is one of a creative instrument that saves human’s life in critical medical situation. Central venous line access is frequently involved in the disease management. It is used for rapid fluid therapy, transvenous pacemakers, infusion of some medications, hemodialysis or plasmapheresis and etc. Most of the emergency departments have some staffs that are trained for central venous line insertion but related complications occur during central venous line placement.Central venous line might have some complications and complication follow-up should be considered. Thromboembolism and infection are two important medical complications. Arterial puncture, hematoma, pneumothorax and hemothorax are mechanical Central venous line complications. Chest X-ray and some other techniques should be used for detecting these complications.Central venous line tip misplace is a considerable problem for emergency department staffs, previously chest X-ray has been used for central venous line misplace detection. In some recent studies, contrast-enhanced ultrasonography and intravascular electrocardiography have been used for central venous line misplace.

  15. Fresh frozen plasma use in a tertiary referral hospital.

    Science.gov (United States)

    Nicholls, M D; Au-Yeung, D; Iannella, M

    1994-06-01

    Hospital transfusion practice is directed towards the optimization of transfusion therapy. A fresh frozen plasma (FFP) usage audit was performed on 316 consecutive FFP units, transfused in 119 transfusion episodes to 75 patients (January-April 1992). All patients had coagulation tests before and after FFP administration; 76% of the coagulation tests were abnormal and of the 24% patients with normal coagulation tests 83% required a massive blood transfusion. Prophylactic to therapeutic and surgical (pre, intra, post) to medical use was 48%:52% and 56%:44%, respectively. From 1988 to 1992 there has been a 42% fall in the hospital's usage of FFP and the FFP to red cell usage is 1:10. In this audit four groups of patients received FFP appropriately for the following indications: coagulation factor deficiency (40%); acute reversal of anticoagulation therapy (19%); massive blood transfusion (27%) and complex medical problems including intensive plasmapheresis (12%). Only 2 patients received FFP (4 units) inappropriately. A total of 98.7% of the FFP units were considered appropriately transfused.

  16. IMPORTANCE OF RESEARCH HLA ANTIBODIES CLASS I AND II, AND MICA ANTIBODIES IN KIDNEY TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    M. Sh. Khubutia

    2011-01-01

    Full Text Available The purpose of this study was to investigate the occurrence of HLA and MICA antibodies in patients from the waiting list for kidney transplantation and their influence on the course of post-transplant period. Determination of HLA antibodies class I and II, and MICA antibodies was performed on a platform of Luminex (xMAP-tech- nology using sets LABScreen ONE LAMBDA (U.S.. A total of 156 patients from the waiting list for kidney transplantation. Revealed the presence of HLA and MICA antibodies in the serum of 31.4% of patients. Regraf- ted patients increased the content of antibodies to the antigens of HLA system was noted in 88.2% of cases, 47% met the combination of antibodies to the I, II classes and MICA. In patients awaiting first kidney transplantation, HLA and MICA antibodies were determined in 23.7% of cases. The presence of pretransplant HLA and MICA antibodies had a significant influence on the course of post-transplant period. Patients with the presence of HLA and MICA in 50% of cases delayed graft function. Sessions of plasmapheresis can reduce the concentration of HLA and MICA antibodies on average by 61.1%. 

  17. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.

    Science.gov (United States)

    Shah, Shivani; Geetha, Duvuru

    2015-01-01

    Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction) and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility.

  18. A case of passenger lymphocyte syndrome following minor ABO incompatible renal transplantation

    Directory of Open Access Journals (Sweden)

    Anju Dubey

    2014-01-01

    Full Text Available Immune hemolysis is one of the adverse effects that can occur following solid organ transplantation. Understanding the clinical settings and the various causes is necessary for prompt diagnosis and appropriate management. One such condition is passenger lymphocyte syndrome (PLS. This case report describes the case of a 27-year-old male renal allograft recipient of the B-positive blood group who received a kidney from an O-positive donor. Postoperatively, the patient showed declining hemoglobin (Hb level and was transfused with B-group packed RBCs (PRBCs, following which there was steep fall in Hb level. A request for PRBCs was sent to the blood bank and this time cross-match with B-group PRBCs showed incompatibility. The patient′s RBCs were found to be strongly DAT (direct anti-globulin test positive and the eluate showed the presence of anti-B with a titer of 32. Thus, diagnosis of probable PLS was made. The patient was managed with methylprednisolone, plasmapheresis and O-group PRBCs. Gradually his condition improved and was discharged in stable condition.

  19. A case of passenger lymphocyte syndrome following minor ABO incompatible renal transplantation.

    Science.gov (United States)

    Dubey, Anju; Pandey, Hemchandra; Sonker, Atul; Chaudhary, Rajendra K

    2014-01-01

    Immune hemolysis is one of the adverse effects that can occur following solid organ transplantation. Understanding the clinical settings and the various causes is necessary for prompt diagnosis and appropriate management. One such condition is passenger lymphocyte syndrome (PLS). This case report describes the case of a 27-year-old male renal allograft recipient of the B-positive blood group who received a kidney from an O-positive donor. Postoperatively, the patient showed declining hemoglobin (Hb) level and was transfused with B-group packed RBCs (PRBCs), following which there was steep fall in Hb level. A request for PRBCs was sent to the blood bank and this time cross-match with B-group PRBCs showed incompatibility. The patient's RBCs were found to be strongly DAT (direct anti-globulin test) positive and the eluate showed the presence of anti-B with a titer of 32. Thus, diagnosis of probable PLS was made. The patient was managed with methylprednisolone, plasmapheresis and O-group PRBCs. Gradually his condition improved and was discharged in stable condition.

  20. FSGS Recurrence in Adults after Renal Transplantation

    Directory of Open Access Journals (Sweden)

    Michael Rudnicki

    2016-01-01

    Full Text Available Recurrence of focal segmental glomerulosclerosis (FSGS in the allograft occurs in 30–50% of patients, and it is associated with poor renal allograft survival. Major risk factors for recurrence are younger age at diagnosis, rapid progression to end-stage renal disease, white race, and the loss of previous allografts due to recurrence. Recent data support the hypothesis that circulating permeability factors play a crucial role in podocyte injury and progression of FSGS. Due to lack of controlled trials, the management of recurrent FSGS is inconsistent and highly empirical. Prophylactic and perioperative treatment with plasmapheresis and high-dose (intravenous cyclosporine represent the main cornerstones of immunosuppressive therapy. In recent years, therapy with rituximab has shown promising results. Despite evidence of activation of the renin-angiotensin system (RAS in recurrent FSGS and its association with progression, only limited data exist on the renoprotective role of RAS blockade in this setting. Further well designed studies are needed on pathogenesis risk factors and therapeutical options in FSGS and its recurrence after transplantation.

  1. Treatment of Familial Hypercholesterolemia and Other Genetic Dyslipidemias.

    Science.gov (United States)

    Dujovne, Carlos A.

    2004-08-01

    Despite their inherited nature, familial dyslipidemias show large intra- and interfamilial variability in phenotypic expression, clinical presentations, and levels of abnormalities of serum lipid fractions. Once diagnosed, patients shall be considered at high cardiovascular risk and treated as per secondary prevention National Cholesterol Education Program III guidelines. Comorbidity treatments (ie, obesity, diabetes, and hypertension) are imperative. Lifestyle interventions shall soon be concomitantly followed by lipid-regulating drugs. The major aspects of the above interventions are the following: 1) therapeutic lifestyle change: regular aerobic exercises, conventional low-fat, low-cholesterol, low refined but high complex carbohydrates diet, avoidance of unproven fad diets (ie, Atkins); 2) plant stanols and sterol esters, 3) high-potency statins (eg, rosuvastatin, simvastatin, atorvastatin); 4) addition of nicotinic acid, bile acid binders, fibrates, or ezetimibe pending on the lipid fraction affected; 5) statins are the starting drug of choice with these exceptions: in isolated low-density lipoprotein cholesterol, niacin or fibrates may be preferable; in isolated severe hypertriglyceridemic conditions, fibrates or fish oil may be preferable; in children with isolated elevation of low-density lipoprotein cholesterol, ezetimibe or bile acid binders may be preferable; when serum lipoprotein (a) elevation is the most notable abnormality, niacin may be chosen as the initial drug for its unique effect on this fraction. Plasmapheresis, intestinal shunts, or liver transplantation are to be considered in that order as last resorts if the above fails to accomplish serum lipid level goals.

  2. Prevalence of Hepatitis G Virus in Liver Disease

    Directory of Open Access Journals (Sweden)

    Hitoshi Takagi

    1999-01-01

    Full Text Available The prevalence of hepatitis G virus (HGV in liver disease of non-A, -B, -C viral hepatitis, hepatitis B and hepatitis C was determined. Two of 44 patients (4.5% with liver injury without any hepatitis A, B or C marker were positive for HGV. One of five cases of hepatocellular carcinoma was positive for HGV. One of three cases with fulminant hepatitis was positive for HGV. This case was negative at the onset of fulminant hepatitis and became positive after plasmapheresis. No patient with acute (n=8 or chronic (n=5 hepatitis or liver cirrhosis (n=8 was positive for HGV in non-A, -B, -C liver disease. One of 30 patients with various HBV-positive liver diseases and nine (17.3 of 52 patients with type C liver disease were positive for HGV. In patients with hepatitis C, four (28.6% of 14 HGV-co-infected patients were complicated with diabetes mellitus compared with four (10.5% of 38 single hepatitis C virus (HCV-infected patients (not significant. In 12 HGV-positive patients, eight of 10 (80% had a history of blood transfusion. In HCV-positive patients, co-infection with HGV was not a risk factor in patients with diabetes mellitus as a complication. HGV appeared to cause non-A, -B, -C hepatitis rarely, and its main route of infection was blood transfusion.

  3. Spontaneous remission of post-transplant recurrent focal and segmental glomerulosclerosis

    Directory of Open Access Journals (Sweden)

    Bassam Saeed

    2011-01-01

    Full Text Available A 12-year-old girl with a history of steroid and cyclosporine (CsA resistant nephrotic syndrome owing to focal and segmental glomerulosclerosis (FSGS has progressed to end-stage renal disease (ESRD for which she underwent hemodialysis for 18 months before she successfully received a fully matched kidney transplant from her sister at the age of nine years. The post transplantation (Tx period was marked by an early and massive proteinuria indicating recurrent FSGS for which she received 12 sessions of plasmapheresis (PP; unfortunately, she did not appear to have any response to the PP therapy; thereafter, a conservative management comprising essentially enalapril and losartan has been initiated and was also not successful during the first four months, however, a very gradual response has been noticed to occur after five months of conservative therapy and ultimately, the patient attained complete remission after 21 months of treatment. Amazingly, 15 months after discontinuation of enalapril and losartan, she remained in a complete and sustained remission with a good renal allograft function. To the best of our knowledge, this is the first case ever reported in the literature of a "spontaneous" remission of post transplant recurrent FSGS.

  4. Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism.

    Science.gov (United States)

    Golbe, L I; Miller, D C; Duvoisin, R C

    1989-01-01

    A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide, methotrexate, and 5-flourouracil (5FU). Severe symmetric action and postural tremor with a myoclonic component developed, with minimal rest tremor, severe dysarthria and dysphagia, small-stepped and slightly ataxic gait progressing to a bedbound state, and severe widespread dystonic posturing. The latter began as a typical parkinsonian posture of trunk and upper extremities and progressed to a fixed and painful flexion of the elbows and wrists and extension of fingers and neck. Sinemet, anticholinergics, baclofen, diazepam, and plasmapheresis gave no benefit. The patient died of complications of immobility 5 months after neurologic symptom onset. Autopsy revealed many pigment-laden macrophages in substantia nigra and moderate loss of pigmented neurons. Inflammation, Lewy bodies, and tumor were absent. Cerebellar Purkinje cells were moderately depleted. Mild neuronal loss and gliosis were present in globus pallidus and cerebellar cortex. Stains for anti-human IgG, IgM, kappa, and lambda were negative. This, to our knowledge, is the first report of paraneoplastic degeneration of substantia nigra or paraneoplastic parkinsonism.

  5. An insidious presentation of thrombotic thrombocytopenic purpura:A case report and brief literature review

    Institute of Scientific and Technical Information of China (English)

    Shafeek Kiblawi; Elie Harmouche; Ralph Bou Chebl; Gilbert Abou Dagher

    2014-01-01

    Thrombotic thrombocytopenic purpura(TTP) is a rare thrombotic microangiopathy with an estimated incidence of11 cases/million population per year.Early treatment is essential and is curative in this disease where lack of treatment results in90% mortality.We describe an atypical case of a patient withTTP who presented to theEmergencyDepartment for headache, and was found to have thrombocytopenia but only mild anemia that was explained by another disease process.Case:A44-year-old female presented to theEmergencyDepartment for worsening headache and weakness over the last week.She had no fever and no focal neurological deficits but was pale and complained of severe headache.A blood test showed her to be anemic and thrombocytopenic.She explained that she had been having prolonged heavy menses over the last year.She was treated with blood and platelet transfusions, and seen by theGynecology service who treated her for uterine fibroids after which she was discharged.She returned1 week later with the same complaint, and was found to have a stable hemoglobin level but recurrence of thrombocytopenia.ATTP diagnosis was entertained and the workup confirmed it.The patient was treated with plasmapheresis and discharged home with no sequalae.Conclusion:Emergency physicians should keepTTP in mind when approaching cases of thrombocytopenia with mild anemia, even if an alternative diagnosis exists.

  6. Evaluation of a new microporous filtration membrane system for therapeutic plasma exchange.

    Science.gov (United States)

    Kurtz, S R; Carey, P M; McGill, M; Pineda, A A; Zaroulis, C G; Case, M T

    1987-01-01

    A new therapeutic plasma exchange device developed by Sarns Inc./3M was evaluated in plasmapheresis of 20 healthy volunteers and in a multicenter clinical study of therapeutic plasma exchange that included 49 patients. Safety and efficacy of plasma separation from whole blood were assessed for a module that contains Durapore microporous surfactant-free polyvinylidene fluoride membrane (Millipore Corp., Bedford, Mass., USA). The extra-corporeal volume was 80 ml. Citrate and heparin anticoagulants were utilized. Mean plasma separation efficiency was 62% with unhindered passage of plasma proteins through the membrane pores and no hemolysis or activation of complement as measured by total hemolytic complement (CH50) and C3 conversion. Mean decrease in platelet count after procedures was 10%. No severe reactions occurred, and citrate effects (13%) were comparable to values reported with centrifugal instruments. The Sarns Inc./3M Therapore device is a rapid, safe and efficient system for plasma exchange and potentially for source plasma collection. The principal benefits are small extracorporeal volume and cell-free filtrate.

  7. Studies on T-cell colony formation in chronic renal failure (CRF) patients.

    Science.gov (United States)

    Wakabayashi, Y; Sugimoto, M; Ishiyama, T; Horie, S; Abe, S; Hirose, S; Okuda, T

    1989-12-01

    In order to study the possibility of abnormal differentiation and proliferation of T-cell precursors in chronic renal failure (CRF), we studied T-cell colony formation in CRF patients. The two-step monolayer method, with phytohemagglutinin-P as the inducer, was used for T-cell colony formation. In our results, colony formation was markedly reduced in CRF patients in comparison with normal controls, with about half of the former showing no colony growth. All cases showed a significant increase in colony numbers with in vitro plasmapheresis (the replacement of autologous plasma in the culture system with normal AB plasma). A significant increase in colony numbers was also seen with the addition of exogenous interleukin-2 (IL-2). The addition of IL-2 in the presence of normal plasma, in particular, induced an increase in colony numbers to near the levels in normal subjects. These results suggest that T-cell precursors exist in near normal numbers in CRF patients and that there are uremic inhibitors in the plasma. A reduced production of IL-2 is also indicated. These factors may be involved in the pathogenesis of immunodeficiency in CRF patients.

  8. Artificial and bioartificial liver support: A review of perfusion treatment for hepatic failure patients

    Institute of Scientific and Technical Information of China (English)

    Katsutoshi Naruse; Wei Tang; Masatoshi Makuuchi

    2007-01-01

    Liver transplantation and blood purification therapy, including plasmapheresis, hemodiafiltration, and bioartificial liver support, are the available treatments for patients with severe hepatic failure. Bioartificial liver support, in which living liver tissue is used to support hepatic function, has been anticipated as an effective treatment for hepatic failure. The two mainstream systems developed for bioartificial liver support are extracorporeal whole liver perfusion (ECLP) and bioreactor systems. Comparing various types of bioartificial liver in view of function, safety, and operability, we concluded that the best efficacy can be provided by the ECLP system. Moreover, in our subsequent experiments comparing ECLP and apheresis therapy, ECLP offers more ammonia metabolism than HD and HF. In addition, ECLP can compensate amino acid imbalance and can secret bile. A controversial point with ECLP is the procedure is labor intensive, resulting in high costs. However, ECLP has the potential to reduce elevated serum ammonia levels of hepatic coma patients in a short duration. When these problems are solved, bioartificial liver support, especially ECLP, can be adopted as an option in ordinary clinical therapy to treat patients with hepatic failure.

  9. [LDL apheresis in the treatment of familial hypercholesterolemia].

    Science.gov (United States)

    Coker, Mahmut

    2014-10-01

    Low density lipoprotein (LDL) apheresis is one of the main therapeutic models for homozygous and severe heterozygous form of the familial hypercholesterolemia patients. Anti-atherogenic, anti-thrombogenic and anti-inflammatory effects of apheresis has positive effects on prevention of cardiovascular disease by improving of the tissue perfusion. Blood LDL cholesterol levels, response to medical therapy, presence or severity of the coronary heart disease are the main determinants of the apheresis indications. Except bleeding tendency and heparin sensitivity, there are no contraindications. In parallel with low body weight, pediatric practice could be more risky; however, there is also a 3.5-year-old apheresis application without problems. The first successful plasmapherisis for the removal of LDL cholesterol in circulation was performed in 1975. Plasmapheresis, today, is only emergency treatment model in cases of life-threatening hyperchylomicronemia. New apheresis techniques have rather high selectivity for atherogenic lipoproteins containing apolipoprotein-B100. If existing apheresis technique has low effectiveness (acute decrease of LDL cholesterol apheresis treatment significantly reduce the burden of cardiovascular disease in children and adult patients with homozygous or severe heterozygous familial hypercholesterolemia.

  10. The effect of different apheresis modalities on coagulation factor XIII level during antibody removal in ABO-blood type incompatible living related renal transplantation.

    Science.gov (United States)

    Hanafusa, Norio; Hamasaki, Yoshifumi; Kawarasaki, Hiroo; Kido, Ryo; Shibagaki, Yugo; Ishikawa, Akira; Enomoto, Yutaka; Fujita, Toshiro; Noiri, Eisei; Nangaku, Masaomi

    2013-10-01

    Apheresis therapy is used to remove pathogenic antibodies within the recipient blood during ABO-incompatible living related renal transplantation (LRRT). Factor XIII (FXIII) is a coagulating factor. Its deficiency reportedly engenders perioperative bleeding. This study compared apheresis modalities from the perspective of the FXIII level. Cases 1-3 were treated only with double-filtration plasmapheresis (DFPP) without (case 1) or with (cases 2 and 3) fresh frozen plasma (FFP) supplementation. Cases 4 and 5 were treated with simple plasma exchange (PEx) with FFP supplementation for the last session. Cases 1-3 showed a marked (case 1, 8.6%) or moderate (case 2, 26.2%; case 3, 28.4%) decrease in FXIII on the day before the procedure after the last apheresis session, although cases 4 (81.9%) and 5 (66.2%) did not. Case 1 experienced perioperative bleeding. The last session is usually performed the day before the surgical procedure. Therefore, FXIII elimination by DFPP might cause bleeding complications because of its slow recovery. The fact warrants that the last apheresis modality during the course might be PEx from the viewpoint of FXIII depletion.

  11. Plasma exchange in management of skin diseases%血浆置换疗法在皮肤病中的应用

    Institute of Scientific and Technical Information of China (English)

    林俊萍; 高兴华

    2000-01-01

    @@ 血浆置换疗法(plasma exchange,PE)是一种体外净化(apheresis)方法,通过血细胞分离机、分离膜等去除血浆中的致病因子,如特异性自身抗体、参与组织损伤的非特异性炎症介质(补体、C反应蛋白、纤维蛋白原等)、免疫复合物、胆固醇、巨球蛋白、毒性代谢产物、微生物及其代谢产物等,同时补充适量的正常人血浆、白蛋白或代血浆,从而发挥治疗作用.血浆去除术(plasmapheresis)系指通过在体外去除血浆来治疗疾病的方法.目前PE和血浆去除术已被作为同义词使用.

  12. [Results of QC vs QL study (quality of care vs quality of life) 2006-2009. The way of providing hemodialysis and a concomitant therapy].

    Science.gov (United States)

    Rutkowski, Bolesław; Nowaczyk, Ryszard; Rutkowski, Przemysław; Mierzicki, Piotr

    2011-01-01

    Healthcare development is the fact in the present world. Because of this the improvement of the quality of care and life of patients is of great importance. Since six years in our country, the study concerning quality of life and care of hemodialysed patients (QC vs QL) is performed annually. In three subsequent papers results of studies performed between 2006 and 2009 are summarised. Almost 7000 patients were studies in the analysed period. This was more than 10% of dialysed patients in every year. In the present paper we focused on the hemodialysis modalities and concomitant therapy. The increase of high-flux hemodialysis usage as well as plasmapheresis but not hemodiafiltration was noticed in the analysed period. Adequacy of the therapy was evaluated as Kt/V and was stable in the whole study. Treatment with erythropoesis stimulating agents (ESA) was provided in 100% of dialysis units, the dosage frequency was connected with long acting ESA. Because of changed trends and the payer requirements apart from frequency the route has also changed. The average haemoglobin level reflected European and county guidelines and changed during the observation. Additional help from psychologist and dietician is available in too small number of dialysis units. Summarizing, based on performed analysis the development of hemodialysis treatment and a high level of provided therapy comparable to other European countries was observed in Poland. Support for further development and improvement of renal replacement therapy is needed to achieve also better quality of life of our patients.

  13. Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines.

    Science.gov (United States)

    Ansell, Stephen M; Kyle, Robert A; Reeder, Craig B; Fonseca, Rafael; Mikhael, Joseph R; Morice, William G; Bergsagel, P Leif; Buadi, Francis K; Colgan, Joseph P; Dingli, David; Dispenzieri, Angela; Greipp, Philip R; Habermann, Thomas M; Hayman, Suzanne R; Inwards, David J; Johnston, Patrick B; Kumar, Shaji K; Lacy, Martha Q; Lust, John A; Markovic, Svetomir N; Micallef, Ivana N M; Nowakowski, Grzegorz S; Porrata, Luis F; Roy, Vivek; Russell, Stephen J; Short, Kristen E Detweiler; Stewart, A Keith; Thompson, Carrie A; Witzig, Thomas E; Zeldenrust, Steven R; Dalton, Robert J; Rajkumar, S Vincent; Gertz, Morie A

    2010-09-01

    Waldenström macroglobulinemia is a B-cell malignancy with lymphoplasmacytic infiltration in the bone marrow or lymphatic tissue and a monoclonal immunoglobulin M protein (IgM) in the serum. It is incurable with current therapy, and the decision to treat patients as well as the choice of treatment can be complex. Using a risk-adapted approach, we provide recommendations on timing and choice of therapy. Patients with smoldering or asymptomatic Waldenström macroglobulinemia and preserved hematologic function should be observed without therapy. Symptomatic patients with modest hematologic compromise, IgM-related neuropathy that requires therapy, or hemolytic anemia unresponsive to corticosteroids should receive standard doses of rituximab alone without maintenance therapy. Patients who have severe constitutional symptoms, profound hematologic compromise, symptomatic bulky disease, or hyperviscosity should be treated with the DRC (dexamethasone, rituximab, cyclophosphamide) regimen. Any patient with symptoms of hyperviscosity should first be treated with plasmapheresis. For patients who experience relapse after a response to initial therapy of more than 2 years' duration, the original therapy should be repeated. For patients who had an inadequate response to initial therapy or a response of less than 2 years' duration, an alternative agent or combination should be used. Autologous stem cell transplant should be considered in all eligible patients with relapsed disease.

  14. Primary therapy of Waldenstrom macroglobulinemia (WM) with weekly bortezomib, low-dose dexamethasone, and rituximab (BDR): long-term results of a phase 2 study of the European Myeloma Network (EMN).

    Science.gov (United States)

    Dimopoulos, Meletios A; García-Sanz, Ramón; Gavriatopoulou, Maria; Morel, Pierre; Kyrtsonis, Marie-Christine; Michalis, Eurydiki; Kartasis, Zafiris; Leleu, Xavier; Palladini, Giovanni; Tedeschi, Alessandra; Gika, Dimitra; Merlini, Giampaolo; Kastritis, Efstathios; Sonneveld, Pieter

    2013-11-07

    In this phase 2 multicenter trial, we evaluated the activity of bortezomib, dexamethasone, and rituximab (BDR) combination in previously untreated symptomatic patients with Waldenström macroglobulinemia (WM). To prevent immunoglobulin M (IgM) "flare," single agent bortezomib (1.3 mg/m(2) IV days 1, 4, 8, and 11; 21-day cycle), was followed by weekly IV bortezomib (1.6 mg/m(2) days 1, 8, 15, and 22) every 35 days for 4 additional cycles, followed by IV dexamethasone (40 mg) and IV rituximab (375 mg/m(2)) in cycles 2 and 5. Fifty-nine patients were treated; 45.5% and 40% were high and intermediate risk per the International Prognostic Scoring System for WM. On intent to treat, 85% responded (3% complete response, 7% very good partial response, 58% partial response [PR]). In 11% of patients, an increase of IgM ≥25% was observed after rituximab; no patient required plasmapheresis. After a minimum follow-up of 32 months, median progression-free survival was 42 months, 3-year duration of response for patients with ≥PR was 70%, and 3-year survival was 81%. Peripheral neuropathy occurred in 46% (grade ≥3 in 7%); only 8% discontinued bortezomib due to neuropathy. BDR is rapidly acting, well tolerated, and nonmyelotoxic, inducing durable responses in previously untreated WM.

  15. How I treat Waldenström macroglobulinemia.

    Science.gov (United States)

    Treon, Steven P

    2015-08-06

    Waldenström macroglobulinemia (WM) is a B-cell neoplasm manifested by the accumulation of clonal immunoglobulin (Ig)M-secreting lymphoplasmacytic cells. MYD88 and CXCR4 warts, hypogammaglobulinemia, infections, myelokathexis syndrome-like somatic mutations are present in >90% and 30% to 35% of WM patients, respectively, and impact disease presentation, treatment outcome, and overall survival. Familial predisposition is common in WM. Asymptomatic patients should be observed. Patients with disease-related hemoglobin <10 g/L, platelets <100 × 10(9)/L, bulky adenopathy and/or organomegaly, symptomatic hyperviscosity, peripheral neuropathy, amyloidosis, cryoglobulinemia, cold-agglutinin disease, or transformed disease should be considered for therapy. Plasmapheresis should be used for patients with symptomatic hyperviscosity and before rituximab for those with high serum IgM levels to preempt a symptomatic IgM flare. Treatment choice should take into account specific goals of therapy, necessity for rapid disease control, risk of treatment-related neuropathy, immunosuppression and secondary malignancies, and planning for future autologous stem cell transplantation. Frontline treatments include rituximab alone or rituximab combined with alkylators (bendamustine and cyclophosphamide), proteasome inhibitors (bortezomib and carfilzomib), nucleoside analogs (fludarabine and cladribine), and ibrutinib. In the salvage setting, an alternative frontline regimen, ibrutinib, everolimus, or stem cell transplantation can be considered. Investigational therapies under development for WM include agents that target MYD88, CXCR4, BCL2, and CD27/CD70 signaling, novel proteasome inhibitors, and chimeric antigen receptor-modified T-cell therapy.

  16. Waldenström Macroglobulinemia in Hepatitis C: Case Report and Review of the Current Literature

    Directory of Open Access Journals (Sweden)

    Ryan Nipp

    2014-01-01

    Full Text Available Background. Recent literature has associated hepatitis C virus with the development of non-Hodgkin lymphoma. Hepatitis C virus infection appears to promote lymphoproliferation, providing a plausible mechanism for a causative association; however, despite prior reports of patients with comorbid hepatitis C infection and Waldenström macroglobulinemia, the literature is in disagreement regarding whether there exists an association between these two conditions. Case Presentation. This case report describes a 57-year-old African-American male with chronic hepatitis C infection and cryoglobulinemia who presented with several episodes of transient confusion and paralysis and was found to have symptomatic hyperviscosity. The recognition of his condition was facilitated by characteristic findings on ophthalmologic examination. He was subsequently diagnosed with Waldenström macroglobulinemia on bone marrow biopsy. Conclusions. An up to date, comprehensive review of the literature suggests an association between hepatitis C and Waldenström macroglobulinemia. Data on optimal treatment of patients with comorbid hepatitis C infection and Waldenström macroglobulinemia is limited. We have provided a comprehensive review of previously explored treatment options to guide management of other similar patients. Our patient has since been treated with repeated plasmapheresis with a plan to pursue antiviral therapy.

  17. [Catastrophic antiphospholipid syndrome].

    Science.gov (United States)

    Wisłowska, Małgorzata

    2015-01-01

    Catastrophic antiphospholipid syndrome is the most dangerous form of the antiphospholipid syndrome, which is characterized by rapid onset of thrombosis in small vessels of many organs and intravascular coagulation, thrombocytopenia and hemolytic anemia. The syndrome develops over a short period of time with acute multi-organ failure, including kidney, respiratory, cardiovascular, central nervous system and adrenal glands, often associated with disseminated thrombotic microangiopathy. The catastrophic antiphospholipid syndrome involves three or more systems, organs and/or tissues, the development of symptoms must occur within less than one week, it is necessary to confirm the histopathological vascular occlusion in at least one organ or tissue, and laboratory confirmation of the presence of antiphospholipid antibodies in the serum on two occasions over an interval of 12 weeks. This syndrome is characterized by a high mortality despite the use of optimal treatment. Early diagnosis and aggressive treatment of patients with catastrophic antiphospholipid syndrome is essential to save the life of these patients. In the last 10 years, the mortality in this disease decreased from 50% to 30% with simultaneous treatment with anticoagulants, corticosteroids, plasmapheresis and immunoglobulins.

  18. Pure red cell aplasia accompanied by autoimmune hemolytic anemia in a patient with type A viral hepatitis.

    Science.gov (United States)

    Koiso, Hiromi; Kobayashi, Satsuki; Ueki, Kazue; Hamada, Tetsuya; Tsukamoto, Norifumi; Karasawa, Masamitsu; Murakami, Hirokazu; Nojima, Yoshihisa

    2009-05-01

    A rare case of acute hepatitis A associated with autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) is reported. A 55-year-old woman consulted a doctor because of common cold-like symptoms and she was referred to our hospital in January 2007. Laboratory findings showed a marked elevation of serum transaminase and total bilirubin levels (AST 9,605 IU/l, ALT 5,546 IU/l and T-bil 4.14 mg/dl), and prolonged prothrombin time, findings which suggested the risk of progression to fulminant hepatitis, and she was treated with plasmapheresis and hemodialysis filtration on the first and second hospital days. She was diagnosed with severe acute hepatitis A based on the elevation of serum IgM anti-hepatitis A virus. On the 20th hospital day, her hemoglobin level began to decrease in spite of improving transaminase levels without any signs of gastrointestinal bleeding. Bilirubin and LDH elevation, haptoglobin decline and a positive direct Coombs test were detected and these findings indicated AIHA complication; however, the reticulocyte count decreased and bone marrow showed marked erythroid hypoplasia so the co-existence of PRCA was diagnosed. After oral prednisolone administration (1 mg/kg/day), her hemolytic anemia rapidly improved.

  19. Atypical relapse of hemolytic uremic syndrome after transplantation.

    Science.gov (United States)

    Olie, Karolien H; Florquin, Sandrine; Groothoff, Jaap W; Verlaak, René; Strain, Lisa; Goodship, Timothy H J; Weening, Jan J; Davin, Jean-Claude

    2004-10-01

    Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of immediate recurrent HUS. Prophylactic plasma exchange before and after transplantation was used. Two months after transplantation, concomitant with a reduction in plasma exchange frequency, the plasma creatinine increased from 70 micro mol/l to 194 micro mol/l in 2 weeks without thrombocytopenia or signs of hemolytic anemia. The patient had minimal clinical symptoms and a presumptive diagnosis of graft rejection was made. Despite treatment with six daily pulses of methylprednisolone, plasma creatinine continued to increase and a graft biopsy was therefore undertaken. This showed the typical appearance of a thrombotic microangiopathy without any evidence of rejection. Despite daily plasmapheresis and replacement of cyclosporine with tacrolimus, there was no improvement and transplant nephrectomy was undertaken. This patient demonstrates that HUS can recur in a kidney transplant without the diagnostic hematological features and emphasizes the need for early transplant biopsy in such patients showing a decline in transplant function.

  20. Acute disseminated encephalomyelitis: a comprehensive review

    Directory of Open Access Journals (Sweden)

    LIU Zheng

    2013-09-01

    Full Text Available Acute disseminated encephalomyelitis (ADEM is a disease that is characterized by an immune-mediated inflammatory reaction and demyelination in the central nervous system, including optic nerve, brain and spinal cord, which is common in children, but also appears in adults. ADEM happens probably with a causative relationship to viral diseases or prior vaccinations. It can also occur without any cause. The clinical symptoms of ADEM can begin with fever, headache and meningeal signs, followed by abnormal mental status, seizures and focal neurological signs, such as limb pareses, visual decline and speech disturbances. And their clinical courses may be monophasic, recurrent or multiphasic, mild but also very aggressive. Neuroimaging may be characterized by large demyelinating lesions in the brain involving both the white and the grey matter, and spinal cord. Lesions in ADEM are typically large globular lesions, multiple, and asymmetric, partially with diffuse or ring-like gadolinium enhancement. The diagnosis of ADEM requires both multifocal involvement and encephalopathy by consensus criteria. The differential diagnoses of ADEM include a variety of disorders, such as viral encephalitis, multiple sclerosis, neuromyelitis optica spectrum disorders and primary central nervous system vasculitis, etc. Treatments of ADEM include corticosteroids, intravenous immunoglobulin, plasmapheresis and other immunosuppressive agents.

  1. Eculizumab prevents recurrent antiphospholipid antibody syndrome and enables successful renal transplantation.

    Science.gov (United States)

    Lonze, B E; Zachary, A A; Magro, C M; Desai, N M; Orandi, B J; Dagher, N N; Singer, A L; Carter-Monroe, N; Nazarian, S M; Segev, D L; Streiff, M B; Montgomery, R A

    2014-02-01

    Renal transplantation in patients with antiphospholipid antibodies has historically proven challenging due to increased risk for thrombosis and allograft failure. This is especially true for patients with antiphospholipid antibody syndrome (APS) and its rare subtype, the catastrophic antiphospholipid antibody syndrome (CAPS). Since a critical mechanism of thrombosis in APS/CAPS is one mediated by complement activation, we hypothesized that preemptive treatment with the terminal complement inhibitor, eculizumab, would reduce the extent of vascular injury and thrombosis, enabling renal transplantation for patients in whom it would otherwise be contraindicated. Three patients with APS, two with a history of CAPS, were treated with continuous systemic anticoagulation together with eculizumab prior to and following live donor renal transplantation. Two patients were also sensitized to human leukocyte antigens (HLA) and required plasmapheresis for reduction of donor-specific antibodies. After follow-up ranging from 4 months to 4 years, all patients have functioning renal allografts. No systemic thrombotic events or early graft losses were observed. While the appropriate duration of treatment remains to be determined, this case series suggests that complement inhibitors such as eculizumab may prove to be effective in preventing the recurrence of APS after renal transplantation.

  2. Serous macular detachment, yellow macular deposits, and prominent middle limiting membrane in multiple myeloma

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    Dogan B

    2015-04-01

    Full Text Available Berna Dogan,1 Muhammet Kazim Erol,1 Devrim Toslak,1 Deniz Turgut Coban,1 Mehmet Bulut,1 Ayse Cengiz,1 Esin Sogutlu Sari2 1Antalya Training and Research Hospital, Eye Clinic, Antalya, Turkey; 2Balikesir University Medicine Faculty, Eye Clinic, Balikesir, Turkey Abstract: Bone marrow-derived multiple myeloma is a type of plasma cell tumor that may be associated with ocular complications. A 52-year-old male patient was admitted to our eye clinic with the complaint of sudden visual loss and a visual acuity of 20/50 in the right eye and 20/800 in the left eye. Fundus examination revealed common flame-shaped hemorrhages, venous dilatation and tortuosity, Roth spots, serous macular detachment, and yellow macular deposits in both eyes. Evaluation with fundus fluorescein angiography, fundus autofluorescence, and spectral-domain optical coherence tomography resulted in suspicion of hyperviscosity retinopathy and referral to the hematology clinic. After hematology consultation confirmed a diagnosis of multiple myeloma, chemotherapy and plasmapheresis were initiated. Four months after presentation, best-corrected visual acuity was 20/20 in both eyes and improvement in hyperviscosity retinopathy, serous macular detachment, and yellow macular deposits was observed. Keywords: serous macular detachment, yellow macular deposit, prominent middle limiting membrane, multiple myeloma

  3. Treatment of neuromyelitis optica: an evidence based review

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    Douglas Sato

    2012-01-01

    Full Text Available Neuromyelitis optica (NMO is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. Aquaporin-4 antibody is positive in a high percentage of NMO patients and it is directed against this water channel richly expressed on foot processes of astrocytes. Due to the severity of NMO attacks and the high risk for disability, treatment should be instituted as soon as the diagnosis is confirmed. There is increasing evidence that NMO patients respond differently from patients with multiple sclerosis (MS, and, therefore, treatments for MS may not be suitable for NMO. Acute NMO attacks usually are treated with high dose intravenous corticosteroid pulse and plasmapheresis. Maintenance therapy is also required to avoid further attacks and it is based on low-dose oral corticosteroids and non-specific immunosuppressant drugs, like azathioprine and mycophenolate mofetil. New therapy strategies using monoclonal antibodies like rituximab have been tested in NMO, with positive results in open label studies. However, there is no controlled randomized trial to confirm the safety and efficacy for the drugs currently used in NMO.

  4. Application of flow cytometry to measure anti-A/B antibody in ABO-incompatible kidney transplantation.

    Science.gov (United States)

    Kim, H-J; Jung, H-Y; Choi, J-Y; Cho, J-H; Park, S-H; Kim, Y-L; Kim, H-K; Huh, S; Won, D-I; Kim, C-D

    2015-04-01

    The aim of this study was to compare anti-ABO antibody levels as measured by means of flow cytometry (FCM) with the levels measured with the use of the column agglutination test (CAT), and to evaluate the clinical outcome as it relates to the baseline mean fluorescence intensity (MFI) ratio obtained by FCM. We reviewed 21 cases of ABO-incompatible kidney transplantation (ABO-i KT). In these patients, baseline IgG titers were measured with the use of both FCM and CAT methods. We investigated the correlation between levels measured by FCM and those by CAT with the use of correlation coefficients. Patients were classified into a high MFI ratio group (≥200; n = 7) or low MFI ratio group (ABO antibody levels measured by means of the FCM-based method were highly correlated with the levels measured with the use of CAT in cases of ABO-i KT. The decreased level of anti-ABO antibody measured by means of FCM after plasmapheresis suggests its potential as an effective and objective method for assessment of anti-ABO antibody levels. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. Alveolar hemorrhage in systemic lupus erythematosus: a cohort review.

    Science.gov (United States)

    Andrade, C; Mendonça, T; Farinha, F; Correia, J; Marinho, A; Almeida, I; Vasconcelos, C

    2016-01-01

    Diffuse alveolar hemorrhage (DAH) is a rare but potentially catastrophic manifestation with a high mortality. Among rheumatologic diseases, it occurs most frequently in patients with systemic lupus erythematosus (SLE) and systemic vasculitis. Despite new diagnostic tools and therapies, it remains a diagnostic and therapeutic challenge. The aim of this work was to characterize the SLE patients with an episode of alveolar hemorrhage followed in our Clinical Immunology Unit (CIU). A retrospective chart review was carried out for all patients with SLE followed in CIU between 1984 and the end of 2013. We reviewed the following data: demographic characteristics, clinical and laboratory data, radiologic investigations, histologic studies, treatment, and outcome. We identified 10 episodes of DAH, corresponding to seven patients, all female. These represent 1.6% of SLE patients followed in our Unit. The age at DAH attack was 42.75 ± 18.9 years. The average time between diagnosis of SLE and the onset of DAH was 7.1 years. Three patients had the diagnosis of SLE and the DAH attack at the same time. Disease activity according to SLEDAI was high, ranging from 15 to 41. All patients were treated with methylprednisolone, 37.5% cyclophosphamide and 28.6% plasmapheresis. The overall mortality rate was 28.6%.

  6. A young patient with multisystem complications after cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Swaroopa Pulivarthi

    2014-01-01

    Full Text Available We are describing a case of an 18-year-old male patient with cytomegalovirus (CMV associated guillain-barre syndrome (GBS who presented with an acute onset of generalized weakness and numbness in the extremities, dysphagia, and facial diplegia, followed by respiratory failure, which led to mechanical ventilation. He had positive immunoglobulin G and immunoglobulin M antibodies against CMV, and CMV polymerase chain reaction was positive with <2000 copies of deoxyribonucleic acid. Human immunodeficiency virus test was negative. He received a course of ganciclovir, intravenous immunoglobulin, and plasmapheresis. After improving from acute episode, patient was transferred to a rehabilitation facility for physical and occupational therapy. At the rehabilitation facility, he exhibited signs of acute abdomen with pain in the left upper quadrant secondary to peritonitis from dislodged gastrostomy tube and underwent exploratory laparotomy. During the hospital course he was found to have splenic infarct and colitis on the computed tomography of abdomen. This case showed an immunocompetent young patient with multisystem complications including guillain-barre syndrome (GBS, splenic infarct, hepatitis, and colitis due to CMV.

  7. Hemophagocytic Lymphohistiocytosis Induced by Severe Pandemic Influenza A (H1N1 2009 Virus Infection: A Case Report

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    Xiang-Yan Zhang

    2011-01-01

    Full Text Available After early outbreaks in North America in April 2009, the pandemic influenza A (H1N1 virus spread rapidly around the world, and even some patients developed certain severe complications. We reported one case of hemophagocytic lymphohistiocytosis (HLH induced by severe pandemic influenza A (H1N1 virus infection. A 17-year-old girl had acute onset of fever, dry cough, rhinorrhea, and sore throat Her family members and close friends also had the similar symptoms. Anti-infection treatment with penicillin was given after 8 days of the onset of symptoms in the local hospital, and her chest radiograph showed consolidation of the left lung. Then, she was sent to the People's Hospital of Guizhou Province in China and endotracheal intubation were underwent on the ninth day for acute hypoxic respiratory failure. She was diagnosed with HLH induced by severe pandemic influenza A (H1N1 2009 virus. Oseltamivir, steroids, immunoglobulin, and plasmapheresis were given immediately after admission. After being treated in the People's Hospital of Guizhou Province for 16 days, she was discharged. This experience shows that HLH may be a life-threatening complication for severe pandemic influenza A (H1N1 2009 virus infection and responds well to therapy.

  8. [Snake bite injuries].

    Science.gov (United States)

    Turchányi, B; Szalontay, T; Zacher, G

    2000-05-14

    Authors treated five patients who suffered venomous snake-bite injury. Although these snakes are not native in Hungary, this kind of injury is estimated to be more frequent, because of the increasing number of the private collections and illegal import of these reptiles. The local and general symptoms, the therapeutic steps are summarised in this study considering the literature as well. Two patients did not show any systemic or local symptoms at the level of injury, they needed only short observation, and woundcare. The other three patients had serious transient systematic symptoms (vasolability, hypotension/shock, coagulopathy, confusion). Two of them were given specific antivenom. As the third patient did not agree with the serum therapy, plasmapheresis was the choice to treat him, and it seemed to be effective. Few hours later the patients needed surgery because of serious compartment syndrome of their affected upper extremity. Surgical decompression of all the compartments and different possibilities of the secondary skin closure technique are demonstrated. Two patient healed completely, but the right thumb of the third was lost. Authors summarise the effects of the poisons, the symptoms, and the basic therapeutic steps during the first aid and in the primary hospital phase, respectively. They point out the indications of the serum therapy and the correct surgical decompression of the injured extremity.

  9. Neuromyelitis optica in child: diagnostic and therapeutic challenges.

    Science.gov (United States)

    Dembinski, Karolina; Gieron-Korthals, Maria; Martinez, Carlos R; Rodriguez, Lisa

    2013-01-01

    Neuromyelitis optica (NMO) is a rare syndrome of severe inflammatory demyelination of the central nervous system, causing attacks of optic neuritis and transverse myelitis. Although uncommon, attention should be given to the proper identification and management of the affected patients. We present a case of a 13-year-old girl with severe neuromyelitis optica. The patient's initial presentation consisted of encephalopathy and optic neuritis. Approximately 2 months later, coinciding with the weaning of steroid treatment, she presented with ascending paralysis and respiratory failure. She was seropositive for NMO-IgG. Treatment included intravenous immune globulin, steroids, plasmapheresis, and rituximab and was complemented with proper nutrition, vitamins, minerals, and intense rehabilitation. Two years after the initial presentation and one short relapse, the patient has made a remarkable recovery without neurologic deficit. This report underscores the difficulty in making the initial diagnosis, choosing the best treatment, and the need for more streamlined pediatric guidelines for diagnosis, treatment, and prevention of relapses of pediatric NMO.

  10. Risk factors for poor renal prognosis in children with hemolytic uremic syndrome.

    Science.gov (United States)

    Gianviti, Alessandra; Tozzi, Alberto E; De Petris, Laura; Caprioli, Alfredo; Ravà, Lucilla; Edefonti, Alberto; Ardissino, Gianluigi; Montini, Giovanni; Zacchello, Graziella; Ferretti, Alfonso; Pecoraro, Carmine; De Palo, Tommaso; Caringella, Angela; Gaido, Maurizio; Coppo, Rosanna; Perfumo, Francesco; Miglietti, Nunzia; Ratsche, Ilse; Penza, Rosa; Capasso, Giovambattista; Maringhini, Silvio; Li Volti, Salvatore; Setzu, Carmen; Pennesi, Marco; Bettinelli, Alberto; Peratoner, Leopoldo; Pela, Ivana; Salvaggio, Elio; Lama, Giuliana; Maffei, Salvatore; Rizzoni, Gianfranco

    2003-12-01

    Many factors have been proposed as predictors of poor renal prognosis in children with hemolytic uremic syndrome (HUS), but their role is still controversial. Our aim was to detect the most reliable early predictors of poor renal prognosis to promptly identify children at major risk of bad outcome who could eventually benefit from early specific treatments, such as plasmapheresis. Prognostic factors identifiable at onset of HUS were evaluated by survival analysis and a proportional hazard model. These included age at onset, prodromal diarrhea (D), leukocyte count, central nervous system (CNS) involvement, and evidence of Shiga toxin-producing Escherichia coli (STEC) infection. Three hundred and eighty-seven HUS cases were reported; 276 were investigated for STEC infection and 189 (68%) proved positive. Age at onset, leukocyte count, and CNS involvement were not associated with the time to recovery. Absence of prodromal D and lack of evidence of STEC infection were independently associated with a poor renal prognosis; only 34% of patients D(-)STEC(- )recovered normal renal function compared with 65%-76% of D(+)STEC(+), D(+)STEC(-) and D(-)STEC(+ )patients. In conclusion, absence of both D and evidence of STEC infection are needed to identify patients with HUS and worst prognosis, while D(-) but STEC(+) patients have a significantly better prognosis.

  11. Exosome removal as a therapeutic adjuvant in cancer

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    Marleau Annette M

    2012-06-01

    Full Text Available Abstract Exosome secretion is a notable feature of malignancy owing to the roles of these nanoparticles in cancer growth, immune suppression, tumor angiogenesis and therapeutic resistance. Exosomes are 30–100 nm membrane vesicles released by many cells types during normal physiological processes. Tumors aberrantly secrete large quantities of exosomes that transport oncoproteins and immune suppressive molecules to support tumor growth and metastasis. The role of exosomes in intercellular signaling is exemplified by human epidermal growth factor receptor type 2 (HER2 over-expressing breast cancer, where exosomes with the HER2 oncoprotein stimulate tumor growth and interfere with the activity of the therapeutic antibody Herceptin®. Since numerous observations from experimental model systems point toward an important clinical impact of exosomes in cancer, several pharmacological strategies have been proposed for targeting their malignant activities. We also propose a novel device strategy involving extracorporeal hemofiltration of exosomes from the entire circulatory system using an affinity plasmapheresis platform known as the Aethlon ADAPT™ (adaptive dialysis-like affinity platform technology system, which would overcome the risks of toxicity and drug interactions posed by pharmacological approaches. This technology allows affinity agents, including exosome-binding lectins and antibodies, to be immobilized in the outer-capillary space of plasma filtration membranes that integrate into existing kidney dialysis systems. Device therapies that evolve from this platform allow rapid extracorporeal capture and selective retention of target particles 

  12. Acute flaccid paraplegia:neurological approach, diagnostic workup, and therapeutic options

    Institute of Scientific and Technical Information of China (English)

    Gentian Vyshka; Altin Kuqo; Serla Grabova; Eris Ranxha; Liro Buda; Jera Kruja

    2015-01-01

    Acute flaccid paraplegia is a clinical occurrence with extreme importance, due to the dramatic presentation, the severity of the underlying disorder, and the generally poor prognosis that follows such a condition. Among etiological factors, the traumatic events are of particular interest, with the clinical treating dealing with a severely ill patient, following fall from height, motor vehicle collisions, and direct shocks applied over the vertebral column. The non-traumatic list is more numerous;however the severity of the acute paraplegia is not necessarily of a lesser degree. Viral infections, autoimmune disorders, and ischemic events involving feeding spinal arteries have been imputed. However, chemical and medications injected during procedures or accidentally intrathecal administration can produce acute flaccid paraplegia. A careful neurological assessment and complete electrophysiological and imaging studies must follow. In spite of the poor prognosis, different therapeutic options have been proposed and applied. Neurosurgical and orthopedic interventions are often necessary when trauma is present, with high dose glucocorticoids treatment preceding the intervention, in a hope to decrease edema-related compression over the spinal cord. Immunoglobulins and plasmapheresis are logical and helpful options when a polyradiculoneuritis produces such a clinical picture. The role of decompression, as neurosurgical exclusivity, has been considered as well.

  13. [Anti-D isoimmunization severe in a twin pregnancy. Case report].

    Science.gov (United States)

    Calomarde Rees, María Consuelo; Iglesias Sánchez, Carlos; Martín Boado, Elena; Vegas, Gabriel; Omeñaca, Félix; González González, Antonio

    2012-03-01

    Perinatal hemolytic disease occurs secondary to a hemolytic phenomenon of immune origin resulting in fetal or neonatal anemia. A 38-year-old pregnant woman was referred to the Department of high risk Obstetrics, Hospital Universitario La Paz Madrid because of presenting a dichorionic diamniotic twin pregnancy spontaneously, pre-pregnancy diabetes poorly controlled and severe alloinmunization anti-D. Her first pregnancy ended in a normal delivery at term; in the period of 4 years, she has three newborn with 36, 34 and 40 weeks respectively, who die with a week of life. After that, two intrauterine fetal death occur at 26 weeks of gestation. The patient who is RhD negative, suffers anti-D inmunization with a antibody titration of 1/1024 with 14 weeks of gestation. Twelve plasmapheresis, eight doses of anti-D inmunoglobulins and intrauterine transfusions has been the treatment received. A severe anemia is found during the ultrasound control of the middLe cerebral artery peak systolic velocity in both twins since the 16th week. It remains stable thanks to the treatment. Finally at the 28th week of gestation, pregnancy is terminated with a cesarean section. The twins are born alive and premature, but with good general state. The measurement of the middle cerebral artery peak systolic velocity predicts moderate-severe fetal anemia cases, which are the most important in the clinical management because of the need of active treatment or finish the pregnancy.

  14. Does corticosteroid treatment cause prolonged recovery and increased total bilirubin level in severe ADAMTS-13-deficient TTP patient?

    Science.gov (United States)

    Sayiner, Zeynel Abidin; Acik, Didar Yanardag; Yilmaz, Mehmet; Subari, Salih; Mete, Ayse Ozlem; Dai, M Sinan

    2015-10-01

    A 41-year-old female patient complaining of fatigue, headache, mild confusion, and rush on her lower extremities was admitted to our emergency department. Laboratory tests revealed that he had anemia, thrombocytopenia, and increased levels of indirect bilirubin and lactic dehydrogenase (LDH) in blood tests. Direct and indirect Coombs tests were negative, and fragmented erythrocytes were observed in peripheral blood smears. The patient was diagnosed with thrombotic thrombocytopenic purpura (TTP). The best supportive care was provided. Therapeutic plasma exchange (TPE) and 1 mg/kg methylprednisolone treatments were administered. On the 10th day of treatment, LDH level and fragmented red blood cells in peripheral blood smear were decreased, but his direct and indirect bilirubin levels increased despite the fact that he was treated with 1 mg/kg methylprednisolone and TPE. The patient had severe ADAMTS-13 deficiency. After discontinued steroids treatment, his bilirubin level normalized within 4 days. On the 4th day after bilirubin level normalized, vincristine treatment was administered. TPE was also continued. There was no consensus about the optimal schedule for discontinuing plasmapheresis therapy, and also we observed total bilirubin level improvement with discontinued corticosteroid treatment. In this case, corticosteroid treatment was linked with the increase of total bilirubin level in severe ADAMTS-13-deficient TTP patient.

  15. An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome).

    Science.gov (United States)

    Esmaili, Haydarali; Mostafidi, Elmira; Mehramuz, Bahareh; Ardalan, Mohammadreza; Mohajel-Shoja, Mohammadali

    2016-01-01

    Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation.

  16. Bilateral Internuclear Ophthalmoplegia in a Patient with Devic’s Neuromyelitis Optica

    Directory of Open Access Journals (Sweden)

    E. Garcia-Martin

    2010-11-01

    Full Text Available An unusual presentation of Devic’s neuromyelitis optica (NMO disease associated with bilateral internuclear ophthalmoplegia (INO is described. A 32-year-old pregnant patient was diagnosed with NMO. First symptoms were headache and sudden visual loss in her right eye (RE. Eighteen months ago, she reported other neurologic symptoms such as paresthesia. Based on her visual field, fundoscopy and Ishihara test, she was diagnosed with retrobulbar neuritis of the RE. After delivery, new neurologic symptoms resembling transverse myelitis appeared. She was treated with methylprednisolone and plasmapheresis, which improved her visual acuity; however, a sudden bilateral INO appeared, with adduction defect and nystagmus with abduction in both eyes. No improvement was obtained after treatment with azathioprine and rituximab. Paresis of the legs and the right arm persisted, but double vision and OIN gradually disappeared. At the end, the patient had a residual exophoria in the RE and nystagmus with abduction in the left eye. Prevalence of NMO is lower than one case per one million inhabitants, and it is not likely to affect the encephalic trunk; furthermore, bilateral INO in NMO is rare. Two major criteria and at least two of the three minor ones are required to confirm a NMO diagnosis, and our patient fulfilled these diagnosis criteria.

  17. Albumin Utilization in a Teaching Hospital in Tehran: Time to Revise the Prescribing Strategies

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    Yokabed Kazemi

    2015-10-01

    Full Text Available Background: Since albumin imposes a relatively high cost to a healthcare system, drug use evaluation for this drug is much more important. This study wants to evaluate pattern of albumin use in a large university affiliated hospital in Tehran, Iran.Methods: A concurrent, cross-sectional study was performed in “Shaheed Rajaei” Cardiovascular, Medical and Research Center. All inpatient adults that were prescribed albumin during the study period were evaluated to register the indications for albumin usage according to the evidence-based guidelines.Results: Only for five patients (4% the albumin prescriptions were justifiable. Of these cases, intractable edema was the leading cause of albumin misuse (73 patients; 60.8%. The total 1468 vials of Albumin were prescribed for 120 patients during the study period. The most common reasons to prescribe albumin were acute normovolemic (34%, cardiac failure (0.83%, resistance edema with Albumin>2g/dL (61%, nephrotic syndrome (0.83%, plasmapheresis (1.67%, ascetic (1.67%.Conclusion: These data, together with previous national studies highly suggest a mandatory need for educational measures for practicing physicians along with strict regulations for prescription strategies regarding expensive drugs such as albumin.

  18. Cold Autoimmune Hemolytic Anemia due to High-grade non Hodgkin's B cell Lymphoma with Weak Response to Rituximab and Chemotherapy Regimens.

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    Nazel Khosroshahi, Behzad; Jafari, Mohammad; Vazini, Hossein; Ahmadi, Alireza; Shams, Keivan; Kholoujini, Mahdi

    2015-07-01

    Autoimmune hemolytic anemia (AIHA) is characterized by shortening of red blood cell (RBC) survival and the presence of autoantibodies directed against autologous RBCs. Approximately 20% of autoimmune hemolytic anemia cases are associated with cold-reactive antibody. About half of patients with AIHA have no underlying associated disease; these cases are termed primary or idiopathic. Secondary cases are associated with underlying diseases or with certain drugs. We report herein a rare case of cold autoimmiune hemolytic anemia due to high-grade non-Hodgkin's lymphoma of B-cell type with weak response to rituximab and chemotherapy regimens. For treatment B cell lymphoma, Due to lack of treatment response, we used chemotherapy regimens including R- CHOP for the first time, and then Hyper CVAD, R- ICE and ESHAP were administered, respectively. For treatment of autoimmune hemolytic anemia, we have used the corticosteroid, rituximab, plasmapheresis and blood transfusion and splenectomy. In spite of all attempts, the patient died of anemia and aggressive lymphoma nine months after diagnosis. To our knowledge, this is a rare report from cold autoimmune hemolytic anemia in combination with high-grade non-Hodgkin's lymphoma of B-cell type that is refractory to conventional therapies.

  19. [Successful treatment of HIV-associated chronic inflammatory demyelinating polyneuropathy by early initiation of highly active anti-retroviral therapy].

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    Kume, Kodai; Ikeda, Kazuyo; Kamada, Masaki; Touge, Tetsuo; Deguchi, Kazushi; Masaki, Tsutomu

    2013-01-01

    A 47-year-old man with HIV infection presented with lower leg dominant dysesthesia, muscle weakness and sensory ataxia of 3 month's duration. Nerve conduction studies (NCS) showed demyelination change in the median and tibial nerves and sensory nerve action potential (SNAP) in the sural nerve was not evoked. Somatosensory evoked potential (SEP) showed the delayed N9 latency. Diagnose of HIV-associated chronic inflammatory demyelinating polyneuropathy (CIDP) was made. Although the CD4 lymphocyte counts were relatively preserved (466/μl), highly active anti-retroviral therapy (HAART) was started according to a new guideline for the use of antiretroviral agents in HIV-1-infected adults and adolescents recommending early initiation of treatment. After six months, HIV1-RNA was not detected and the CD4 lymphocyte counts showed a recovering trend (585/μl). His symptoms had disappeared, except for dysesthesia in the tip of a toe. Repeated NCS demonstrated full recovery from the demyelination and appearance of SNAP in the sural nerve. The improvement of his symptoms and NCS findings has been maintained for two years. Although effectiveness of immunotherapies such as oral prednisone, high-dose immunoglobulins and plasmapheresis have been reported in HIV-associated CIDP, early initiation of HAART may be also important for favorable prognosis in HIV-associated CIDP.

  20. Treatment of chronic inflammatory demyelinating polyneuropathy.

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    Kleyman, Inna; Brannagan, Thomas H

    2015-07-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the acquired demyelinating neuropathies and is considered to be immune mediated. Diagnosis is typically based on clinical history, neurologic examination, electrophysiologic studies, CSF studies, and pathologic examination. Early diagnosis and treatment is important to prevent irreversible axonal loss and optimize improvement in function. The first-line agents for treatment are intravenous immunoglobulin (IVIg), corticosteroids, and plasmapheresis, which have all been demonstrated to be effective in controlled studies. Studies have not shown a significant difference between these three treatments, and the initial choice of therapy is often based on availability, cost, ease of administration, and side effect profile. If patients do not respond to one of these agents, they may respond to one of the others and sometimes in combination. If the first-line agents are not effective, chemotherapeutic or immunosuppressive agents may be considered. There are limited controlled studies of these modalities, and they are often used in conjunction with a first-line treatment. The majority of patients require long-term therapy to maintain a response and to prevent relapse.

  1. Diagnosis and treatment of chronic acquired demyelinating polyneuropathies.

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    Latov, Norman

    2014-08-01

    Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria. Demyelinating neuropathies are further classified as hereditary or acquired-this distinction is important, because the acquired neuropathies are immune-mediated and, thus, amenable to treatment. The acquired chronic demyelinating neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuropathy), multifocal motor neuropathy (MMN), and POEMS syndrome. They have characteristic--though overlapping--clinical presentations, are mediated by distinct immune mechanisms, and respond to different therapies. CIDP is the default diagnosis if the neuropathy is demyelinating and no other cause is found. Anti-MAG neuropathy is diagnosed on the basis of the presence of anti-MAG antibodies, MMN is characterized by multifocal weakness and motor conduction blocks, and POEMS syndrome is associated with IgG or IgA λ-type monoclonal gammopathy and osteosclerotic myeloma. The correct diagnosis, however, can be difficult to make in patients with atypical or overlapping presentations, or nondefinitive laboratory studies. First-line treatments include intravenous immunoglobulin (IVIg), corticosteroids or plasmapheresis for CIDP; IVIg for MMN; rituximab for anti-MAG neuropathy; and irradiation or chemotherapy for POEMS syndrome. A correct diagnosis is required for choosing the appropriate treatment, with the aim of preventing progressive neuropathy.

  2. Homozygous familial hypercholesterolaemia: update on management.

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    France, Michael

    2016-11-01

    Homozygous familial hypercholesterolaemia (HoFH) is an inherited disease causing an approximately fourfold increase in blood low-density lipoprotein cholesterol (LDLC) from birth compared with the age-matched normal population owing to reduced low-density lipoprotein receptor (LDLR) activity. Such elevated cholesterol is associated with accelerated atheromatous disease, particularly of the aortic root and coronary arteries. However, HoFH is clinically heterogeneous, reflecting residual low-density lipoprotein receptor (LDLR) activity. The main objective in treating children may be stated to be the avoidance of irreversible cardiac damage requiring heart transplantation by sufficient lowering of blood cholesterol. Lipoprotein apheresis or plasmapheresis are safe means of lowering cholesterol but may be insufficient on their own. Statin drugs, PCSK9 inhibitors ezetimibe and bile acid sequestrants are relatively ineffective if LDLR activity is lacking, but should be used if effective. Two new drugs, lomitapide and mipomersen, have been licensed specifically for HoFH by some regulatory authorities. They work by reducing LDL production rate. They have been associated with fatty liver in adults. Evidence of safety in children is lacking. An alternative is liver transplantation, which replaces the missing LDLR and normalises cholesterol. Clinicians are faced with a dilemma in choosing between these options or deferring such treatment associated with potential harm. Individual case descriptions are an important means of informing clinical judgement. Management of the two cases described in this issue is discussed in the light of modern developments in transplantation and pharmacotherapy.

  3. Management of myasthenic crisis in a child.

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    Rybojad, Beata; Lesiuk, Witold; Fijałkowska, Anna; Rybojad, Paweł; Sawicki, Marek; Lesiuk, Leszek

    2013-01-01

    Myasthenia gravis is an autoimmune disorder of peripheral nervous system, leading to fluctuating muscle weakness. It is caused by circulating antibodies that block acetylcholine nicotinic postsynaptic receptors at the postsynaptic neuromuscular junction. Myasthenic crisis is a life-threatening complication, which is defined as weakness from acquired myasthenia gravis. In this paper we described a 15-year-old boy who was admitted to the Paediatric Intensive Care Unit due to myasthenic crisis. He had suffered not only from myasthenia gravis but also hypothyroidism, cerebral palsy and epilepsy. The patient required mechanical ventilation and was successfully treated with both plasmapheresis and intravenous immunoglobulins. He recovered from the crisis and then thymectomy was performed. Perioperative period and anaesthesia passed uncomplicated. Discharged home from the hospital after 2.5 month-treatment, for the last 4 years, he has only come on scheduled outpatient medical appointments. This case reveals that myasthenic crisis, albeit rare, may occur in male adolescents. In such cases multidisciplinary care followed by surgery becomes a procedure of choice. Concomitant medical problems, if well controlled, do not affect the results of outcome of the underlying disease.

  4. Extracorporeal membrane oxygenation as a platform for recovery: a case report of a child with pulmonary hemorrhage, refractory hypoxemic respiratory failure, and new onset goodpasture syndrome.

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    Dalabih, Abdallah; Pietsch, John; Jabs, Kathy; Hardison, Daphne; Bridges, Brian C

    2012-06-01

    We report a case of a 9-year-old female with acute pulmonary hemorrhage and refractory hypoxemic respiratory failure secondary to Goodpasture syndrome (GS). After failing treatment with high frequency oscillatory ventilation and inhaled nitric oxide, she was successfully managed with venovenous extracorporeal membrane oxygenation (VV ECMO). The patient's weight at the time of cannulation was 31 kg. A 19 French 18 cm (arterial) Biomedicus cannula was inserted in the right internal jugular vein and used as the drain. A 17 French 50 cm (venous) Biomedicus cannula was inserted in the right femoral vein and used as the return. Then the patient was anticoagulated with 100 units/kg of intravenous heparin and the circuit was primed with one unit of packed red blood cells. VV ECMO was performed with an SIII Sorin roller head pump with integrated servo regulator and a Quadrox D Bioline coated oxygenator. Despite systemic anticoagulation with heparin, the patient's pulmonary hemorrhage resolved. Extracorporeal membrane oxygenation served as a platform through which we were able to provide renal replacement therapy and plasmapheresis. The patient was successfully discharged home with normal pulmonary function.

  5. Aggressive cutaneous vasculitis in a patient with chronic lymphatic leukemia following granulocyte colony stimulating factor injection: a case report.

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    El Husseiny, Noha M; Mattar, Mervat M

    2011-03-01

    Vasculitis has been reported in a few cases of chronic lymphatic leukemia and with granulocytic colony-stimulating factor therapy. Those with granulocytic colony-stimulating factor occurred after prolonged therapy and there was a rise in total leukocyte count unlike that in our patient who received just a single injection for the first time. We report the case of a 64-year-old Egyptian man with chronic lymphatic leukemia who developed progressive cutaneous vasculitic lesions following injection of a single dose of a granulocytic colony stimulating factor before a third cycle of chemotherapy to improve neutropenia. This is an unusual case and the pathogenesis is not fully understood. Our patient was not on any medical treatment except for bisoprolol for ischemic heart disease. Although aggressive management with steroids, anticoagulation and plasmapheresis had been carried out, the condition was aggressive and the patient's consciousness deteriorated. A magnetic resonance imaging scan of his brain revealed multiple ischemic foci that could be attributed to vasculitis of the brain. The aim of this case report is to highlight the importance of monitoring patients on granulocytic colony-stimulating factor therapy, especially in the context of other conditions (such as a hematological malignancy) that may lead to an adverse outcome.

  6. Aggressive cutaneous vasculitis in a patient with chronic lymphatic leukemia following granulocyte colony stimulating factor injection: a case report

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    El Husseiny Noha M

    2011-03-01

    Full Text Available Abstract Introduction Vasculitis has been reported in a few cases of chronic lymphatic leukemia and with granulocytic colony-stimulating factor therapy. Those with granulocytic colony-stimulating factor occurred after prolonged therapy and there was a rise in total leukocyte count unlike that in our patient who received just a single injection for the first time. Case presentation We report the case of a 64-year-old Egyptian man with chronic lymphatic leukemia who developed progressive cutaneous vasculitic lesions following injection of a single dose of a granulocytic colony stimulating factor before a third cycle of chemotherapy to improve neutropenia. This is an unusual case and the pathogenesis is not fully understood. Our patient was not on any medical treatment except for bisoprolol for ischemic heart disease. Although aggressive management with steroids, anticoagulation and plasmapheresis had been carried out, the condition was aggressive and the patient's consciousness deteriorated. A magnetic resonance imaging scan of his brain revealed multiple ischemic foci that could be attributed to vasculitis of the brain. Conclusion The aim of this case report is to highlight the importance of monitoring patients on granulocytic colony-stimulating factor therapy, especially in the context of other conditions (such as a hematological malignancy that may lead to an adverse outcome.

  7. [Extrahepatic manifestations of chronic hepatitis C virus infection].

    Science.gov (United States)

    Puchner, K P; Berg, T

    2009-05-01

    Current data suggest that HCV infection should be regarded as a systemic infectious disease with multiorgan involvement. More than 50 % of HCV-positive patients develop during the course of the disease at least one extrahepatic manifestation (EHM). The EHMs are often the first and only clinical signs of a chronic hepatitis C. Evidence of HCV infection should always be sought out in cases of unspecific chronic fatigue and/or rheumatic, haematological, endocrine or dermatological disorders. Key pathogenetic factors for the development of EHM are undisputably the HCV lymphotropism and cryoglobulinaemia. Nevertheless, the exact pathogenesis of many EHM still remains unclear. The therapeutic approach to EHM should concentrate on the eradication of HCV. Antiviral therapy in the form of peg-interferon and ribavirin should be regarded as the first-line therapy. Viral response leads mostly to a consecutive clinical response. However, in the case of HCV-related cytopaenias or neuropathies, antiviral therapy may trigger an aggravation of these conditions. Thus, organ-involvement, severity and course of the EHM should be always taken into account when choosing the appropriate therapeutic strategy. Immunosuppressive drugs, plasmapheresis and lately rituximab are counted among therapies that can be applied complementarly or alternatively to the antiviral therapy.

  8. Pemphigus: Our Clinical Experiences and Treatment Alternatives in the Resistant Cases

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    Soner Uzun

    2008-08-01

    Full Text Available Pemphigus is an autoimmune blistering disease affecting skin and mucous membranes which threatens the life. In our country it is the most common disease in this group. In our region, among the pemphigus cases, the most common variant is pemphigus vulgaris. Pemphigus vulgaris consists of the 80% of pemphigus cases and it occurs 10 times more than pemphigus foliaceus. Treatment of pemphigus is accepted as a miracle in clinical medicine. The disease, which had an almost always fatal outcome, had been turned to the disease which long-term remissions or “cure” can be achievable. However, in the past the cause of the death was the disease itself, nowadays, with decreasing frequency, all of the mortalities is due to the treatment side effects. In treatment of pemphigus which drug to use and when to use it has varieties according to the intended effect. Corticosteroids are the main treatment; besides IVIg, plasmapheresis or pulse steroid prefers to control the disease rapidly. Mainly immunosuppressive agents (azathioprine, methotrexate, cyclophosphamide, cyclosporine, and mycophenolate besides gold, dapsone, antibiotics or rituximab are using for late-term effect and to reduce the corticosteroid requirement.

  9. Anti-glomerular basement membrane glomerulonephritis in an HIV positive patient: case report

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    Eduardo José Bellotto Monteiro

    2006-02-01

    Full Text Available We report on a case of a patient with HIV infection, diagnosed 18 months prior to the development of an anti-glomerular basement membrane (anti-GBM rapidly progressive glomerulonephritis; this is probably the first report of such an association. A 30-year-old white man presented with elevation of serum creatinine (1.3 - 13.5 mg/dL within one month. At admission, the urinalysis showed proteinuria of 7.2 g/L and 8,000,000 erythrocytes/mL. Renal biopsy corresponded to a crescentic diffuse proliferative glomerulonephritis mediated by anti-GBM, and serum testing for anti-GBM antibodies was positive; antinuclear antibodies (ANA and anti-neutrophilic cytoplasmic antibodies (ANCA were also positive. The patient underwent hemodyalisis and was treated with plasmapheresis, cyclophosphamide and prednisone. The association described here is not casual, as crescentic glomerulonephritis is not common in HIV-positive patients, anti-GBM glomerulonephritis is rare and anti-GBM antibodies are frequently observed in HIV-positive subjects when compared to the overall population. Based on the current case and on the elevated frequency of the positivity for such antibodies in this group of patients, it is advisable to be aware of the eventual association between these two conditions and to promote an active search for anti-GBM antibodies and early diagnosis of eventual urinary abnormalities in HIV-positive subjects, considering the severity of anti-GBM glomerulonephritis.

  10. Elimination of islet cell antibodies and glutamic acid decarboxylase antibodies II in a patient with newly diagnosed insulin-dependent diabetes mellitus.

    Science.gov (United States)

    Richter, W O; Donner, M G; Schwandt, P

    1997-01-01

    Islet cell antibodies and glutamic acid decarboxylase II (GAD II) antibodies have been discussed in the autoimmune pathogenesis of insulin-dependent diabetes mellitus (IDDM). Hence, immunosuppressants, intravenous immunoglobulins, and plasmapheresis have been used in an effort to modulate autoimmune activity and thereby prevent the destruction of pancreatic beta-cells. We describe the autoantibody (islet cell antibody and GAD II) kinetics and clinical course in a patient with newly diagnosed IDDM treated with a specific immunoglobulin apheresis technique. Five days after the initial diagnosis a 37-year-old patient with IDDM underwent a series of seven immunoglobulin aphereses. Immunoglobulin (IgG, IgA, IgM), islet cell antibody, GAD II, and C-peptide concentrations were monitored for a time course of 74 days. Daily insulin requirements were recorded. One single immunoglobulin apheresis decreased IgG by 66.2 +/- 9.1%, IgA by 66.8 +/- 8.7%, and IgM by 57.7 +/- 12.9%. GAD II antibodies were reduced by 61.9 +/- 12.4%. The islet cell antibody titer declined from 1:32 to 1:4 after the treatment series. There were no relevant changes in the safety parameters determined nor were there any clinical side effects. The efficient decrease in islet cell antibodies and glutamic acid decarboxylase II antibodies in a patient with IDDM encourages further investigations into the impact of this treatment on the clinical course of this autoimmune disorder.

  11. Alemtuzumab Plus Cyclosporine Treatment of the Autoimmune Hemolytic Anemia in an Adult Bowel Transplant

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    A. Lauro

    2014-01-01

    Full Text Available An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA, anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG, a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA. Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft.

  12. Catastrophic Antiphospholipid Syndrome Presenting as Ischemic Pancreatitis in Systemic Lupus Erythematosus

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    Ajit Vyas

    2009-09-01

    Full Text Available Context Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week. Case report We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery. Conclusion Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients’ survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.

  13. Chronic urticaria

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    Sandeep Sachdeva

    2011-01-01

    Full Text Available Chronic urticaria (CU is a disturbing allergic condition of the skin. Although frequently benign, it may sometimes be a red flag sign of a serious internal disease. A multitude of etiologies have been implicated in the causation of CU, including physical, infective, vasculitic, psychological and idiopathic. An autoimmune basis of most of the ′idiopathic′ forms is now hypothesized. Histamine released from mast cells is the major effector in pathogenesis and it is clinically characterized by wheals that have a tendency to recur. Laboratory investigations aimed at a specific etiology are not always conclusive, though may be suggestive of an underlying condition. A clinical search for associated systemic disease is strongly advocated under appropriate circumstances. The mainstay of treatment remains H1 antihistaminics. These may be combined with complementary pharmacopeia in the form of H2 blockers, doxepin, nifedipine and leukotriene inhibitors. More radical therapy in the form of immunoglobulins, plasmapheresis and cyclophosphamide may be required for recalcitrant cases. Autologous transfusion and alternative remedies like acupuncture have prospects for future. A stepwise management results in favorable outcomes. An update on CU based on our experience with patients at a tertiary care centre is presented.

  14. Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis.

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    Sühs, Kurt-Wolfram; Wegner, Florian; Skripuletz, Thomas; Trebst, Corinna; Tayeb, Said Ben; Raab, Peter; Stangel, Martin

    2015-10-01

    Anti-N-methyl D-aspartate (NMDA) receptor encephalitis is the most common type of encephalitis in the spectrum of autoimmune encephalitis defined by antibodies targeting neuronal surface antigens. In the present study, the clinical spectrum of this disease is presented using instructive cases in correlation with the anti-NMDA receptor antibody titers in the cerebrospinal fluid (CSF) and serum. A total of 7 female patients admitted to the hospital of Hannover Medical School (Hannover, Germany) between 2008 and 2014 were diagnosed with anti-NMDA receptor encephalitis. Among these patients, 3 cases were selected to illustrate the range of similar and distinct clinical features across the spectrum of the disease and to compare anti-NMDA antibody levels throughout the disease course. All patients received immunosuppressive treatment with methylprednisolone, intravenous immunoglobulin and/or plasmapheresis, followed in the majority of patients by second-line therapy with rituximab and cyclophosphamide. The disease course correlated with NMDA receptor antibody titers, and to a greater extent with the ratio between antibody titer and protein concentration. A favorable clinical outcome with a modified Rankin Scale (mRS) score of ≤1 was achieved in 4 patients, 1 patient had an mRS score of 2 after 3 months of observation only, whereas 2 patients remained severely impaired (mRS score 4). Early and aggressive immunosuppressive treatment appears to support a good clinical outcome; however, the clinical signs and symptoms differ distinctively and treatment decisions have to be made on an individual basis.

  15. Acute psychosis due to non-paraneoplastic anti-NMDA-receptor encephalitis in a teenage girl: Case report.

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    Kramina, Sandra; Kevere, Laura; Bezborodovs, Nikita; Purvina, Santa; Rozentals, Guntis; Strautmanis, Jurgis; Viksna, Zane

    2015-12-01

    Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a disease occurring when antibodies produced by the body's own immune system attack NMDA-type glutamate receptors in the brain. Most anti-NMDAR encephalitis cases are associated with paraneoplastic syndrome. We analyze the case of a 15-year-old girl who was hospitalized in a child psychiatry clinic in Riga, Latvia, with de novo acute polymorphic psychotic disorder gradually progressing to a catatonic state. The patient received antipsychotic and electroconvulsive therapy with no beneficial effect. The council of doctors discussed differential diagnoses of schizophrenia-induced catatonia and the autoimmune limbic encephalitis-induced catatonic condition. When the diagnosis of anti-NMDAR autoimmune encephalitis was finally confirmed by repeated immunological assays (specific immunoglobulin [Ig] G and IgM in her blood serum and cerebrospinal fluid), and a paraneoplastic process was ruled out, she was started on immunomodulating therapy (methylprednisolone, Ig, plasmapheresis, rituximab), which changed the course of her disease. On immunomodulating treatment, her physical and mental health have gradually improved to almost complete reconvalescence. Psychiatrists should consider anti-NMDAR encephalitis as a differential diagnosis in first-episode psychosis patients presenting with disorientation, disturbed consciousness, pronounced cognitive deficits, movement disorder, dysautonomia, or rapid deterioration, and test for specific IgG NR1 autoantibodies, even if there are no specific findings on routine neuroimaging, electroencephalography (EEG), or cerebrospinal fluid tests.

  16. Anti-NMDA Receptor Encephalitis Presenting as an Acute Psychotic Episode in a Young Woman: An Underdiagnosed yet Treatable Disorder

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    Shikma Keller

    2014-01-01

    Full Text Available Anti-NMDA receptor (NMDAR encephalitis is a recently identified autoimmune disorder with prominent psychiatric symptoms. Patients usually present with acute behavioral change, psychosis, catatonic symptoms, memory deficits, seizures, dyskinesias, and autonomic instability. In female patients an ovarian teratoma is often identified. We describe a 32-year-old woman who presented with acute psychosis. Shortly after admission, she developed generalized seizures and deteriorated into a catatonic state. Although ancillary tests including MRI, electroencephalogram, and cerebrospinal fluid (CSF analysis were unremarkable, the presentation of acute psychosis in combination with recurrent seizures and a relentless course suggested autoimmune encephalitis. The patient underwent pelvic ultrasound which disclosed a dermoid cyst and which led to an urgent cystectomy. Plasmapheresis was then initiated, yielding partial response over the next two weeks. Following the detection of high titers of anti-NMDAR antibodies in the CSF, the patient ultimately received second line immunosuppressive treatment with rituximab. Over several months of cognitive rehabilitation a profound improvement was eventually noted, although minor anterograde memory deficits remained. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.

  17. Application of plasma exchange therapy and its adverse reactions observed in the clinical treatment of autoimmune dis -eases in neurology%血浆置换联合药物治疗在神经内科免疫疾病临床治疗中的应用及不良反应观察

    Institute of Scientific and Technical Information of China (English)

    刘世俊; 辜敏

    2015-01-01

    Objective To investigate the application of plasma exchange therapy and its adverse reactions in the clinical treatment of neurological autoimmune diseases .Methods A total of 90 patients of autoimmune diseases were selected from April 2012 to April 2014 in Department of Neurology of our hospital ,including 51 males and 39 females .All patients were randomly divided into plasma exchange group (n = 30) ,treatment group ( n = 30) and plasmapheresis combined treatment group ( n = 30) ,which were observed for each patient outcomes and ad‐verse events .Results The efficiency is 97% in plasma exchange combined with oral administration of globulin and prednisone tablets .It is higher than those of the patients in single drug treatment and plasmapheresis alone group . While the incidence of adverse reactions observed in patients showed that the combined incidence of adverse reac ‐tions in patients in drug treatment group was lower than that in plasma exchange group .It is of significant differ‐ence ( P < 0 .05) .Conclusion Plasma exchange treatment of autoimmune diseases and neurological diseases was of 3 significant effects .When treated through combination with the drug ,the effect were better and adverse reactions were reduced.%目的:探讨血浆置换联合药物治疗在神经内科免疫疾病临床治疗中的应用及不良反应发生情况。方法选取2012年4月至2014年4月在本院神经内科进行治疗的免疫疾病患者共90例,其中男性51例,女性39例,将所有患者随机分为血浆置换组、药物治疗组、血浆置换联合药物治疗组,每组30例患者,观察每组患者治疗效果及不良反应发生情况。结果通过血浆置换联合丙球球蛋白及泼尼松片口服,患者有效率97%,高于单独药物治疗和单独血浆置换患者,同时,观察患者不良反应发生情况发现,联合治疗组患者不良反应发生率低于药物治疗组和单独血浆置换组

  18. ALTERAÇÕES HEMATOLÓGICAS DURANTE A IMUNIZAÇÃO E APÓS A SANGRIA E PLASMAFERESE EM EQUINOS DE PRODUÇÃO DE SORO HIPERIMUNE ANTICROTÁLICO HAEMATOLOGY CHANGES DURING THE IMMUNIZATION AND AFTER THE BLEEDING AND PHASMAFERESE IN HORSES USED FOR HYPERIMUNE ANTI-CROTALIC SERUM PRODUCTION

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    Ronaldo Azevedo Ferreira

    2009-12-01

    Full Text Available

    Para o estabelecimento do quadro sanguíneo de equinos durante a imunização para produção de soro hiperimune anticrotálico e após as sangrias de produção e a plasmaferese, realizou-se avaliação hematológica para determinar a eficácia da plasmaferese na recuperação do quadro hematimétrico dos animais. Foram utilizados vinte animais, submetidos às normas do protocolo de imunização do Instituto Butantan, sendo  colhidas amostras antes das inoculações do antígeno (veneno crotálico, antes e depois das sangrias de produção, após as plasmafereses e quinze, trinta e quarenta e cinco dias após a última sangria, totalizando 340 colheitas, realizadas em dezessete momentos, para realização do hemograma. Significativas variações no quadro hematológico dos equinos foram observadas na fase de imunização, caracterizando anemia normocítica normocrômica, sem alterações significativas do leucograma. Nas fases de sangrias, observaram-se evidentes variações no quadro hematológico, demonstrando uma anemia normocítica normocrômica pós-sangrias, sem variações nos valores do leucograma. No período de repouso, foi evidente a eficácia da plasmaferese, com pronta, mas parcial, recuperação do hemograma, facilitando o rápido retorno para normalidade hematológica, tornando-os aptos à nova produção de soro hiperimune.

    PALAVRAS-CHAVES: Anticrotálico, equinos, hematologia, imunização, plasmaferese.

    Twenty animals were used to assess the blood profile of horses during immunization for anticrotalic hyperimmune serum production and after the bleedings and the plasmapherese, according to the protocol schedule of Instituto Butantan in order to evaluate efficacy of plasmapheresis. The samples were obtained before the antigen inoculations (crotalic venon, before and after the bleedings, right after the plasmapherese and 15, 30 and 45 days after the last one, making a

  19. Resultado da timectomia ampliada no tratamento de pacientes com Miastenia gravis Extended thymectomy for treating patients with Myasthenia gravis (MG

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    Raul Lopes Ruiz Jr

    2004-04-01

    outcome and prognosis will be enhanced by performing a more extensive resection of the gland and of the anterior mediastinum tissue. OBJECTIVE: To retrospectively evaluate response of MG patients to extended thymectomy. METHOD: From August 1992 to January 2003, forty-six MG patients were submitted to preoperative plasmapheresis and extended thymectomy. According to the time elapsed since onset of symptom they were divided into three groups; 25 months. RESULTS: There were 31 female and 15 male patients, mean age 30 years and average evolution time 26.3 months. Outpatient post-operative follow-up was on the average of 26.6 months. Level of response was good, 50% had full remission and a further 39% had good response. There was one death. The most common histopathology finding was thymic hyperplasia. Only 3 patients (6.5% had benign thymomas; 5 (10.8% had extra glandular thymic tissue: 2 in the perithymic fat and 3 in the pericardiac fat adjacent to the left phrenic nerve and aortal-pulmonary window. CONCLUSION: Treatment of MG by extended thymectomy is safe and efficient with high levels of complete remission. Extra glandular thymic tissue was found in some patients. As soon as diagnosis is completed, thymectomy is indicated together with plasmapheresis and medication, independent of age, onset of symptoms, and thymus pathology.

  20. Cascade filtration for TTP: an effective alternative to plasma exchange with cryodepleted plasma.

    Science.gov (United States)

    Bruni, R; Giannini, G; Lercari, G; Bo, A; Florio, G; De Luigi, M C; Marmont, A; Gobbi, A; Damasio, E; Valbonesi, M

    1999-12-01

    TTP remains enigmatic both in terms of etiology and management. The most recent approach is aggressive plasma exchange (PE) employing cryopoor plasma for replacement, based on the pathogenetic relevance given to exceedingly large Von Willebrand (VWF) multimers in the determination of the syndrome with normalization during remission. PE with fresh frozen plasma (FFP) is better than FFP infusion as shown by a recent Canadian study, supporting the theory that to treat TTP an offending circulating agent needs to be removed from the patient's plasma in contrast to the hypothesis that a missing factor is to be given along with FFP. A more recent hypothesis is supported by the results of studies published by the end of 1998 [Moake J, Chintagumpala M, Turner N et al. Blood 1994;84:490-97; Moake J, McPherson PD. Am J Med 1989;87: 3-9N] which would show that TTP is mediated by auto-antibodies to VWF-cleaving protease, or is the result of deficiency of the protease ascribed to abnormalities in its production, function or survival. Plasmapheresis without plasma infusion is relatively ineffective perhaps because it does not increase the protease activity. Cascade filtration (CF) is the autologous counterpart of plasmapheresis. It has been used by our group since 1980 to remove from patients plasma macromolecules such as VWF, fibrinogen, LDL and circulatory immune complexes (CIC). After secondary filtration, the autologous plasma has a composition which is very similar to that of allogeneic plasma after cryoprecipitation, a product which used in the management of TTP. Based on this knowledge, in 1994 we began to use CF in the treatment of TTP patients. In the beginning (7 patients) CF was combined with a decreasing number of conventional PEs using allogeneic plasma for substitution. Lately only CF with some plasma supplementation has been used in the last 9 cases. From a clinical point of view our 16 patients achieved remission after a number of treatments (11 +/- 7) that

  1. 血浆置换在神经系统疾病中的应用%Plasma exchange in the treatment of neurological diseases: analysis of 78 cases

    Institute of Scientific and Technical Information of China (English)

    母艳蕾; 张华

    2012-01-01

    目的 回顾分析血浆置换(plasma exchange,PE)治疗神经免疫性疾病患者的资料,了解其疗效和不良反应. 方法 对78例应用PE换治疗过的患者资料回顾性分析,其中包括73例重症肌无力(myasthenia gravis,MG),3例吉兰巴雷综合征(Guillain-Barre' syndrome,GBS),1 例慢性炎性脱髓鞘性多神经根神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)和1例多发性硬化(multiple sclerosis,MS).PE头3次为隔日1次,之后为隔周1次,偶有1周2次.应用临床评分观察疗效,根据临床记录了解有关副反应.结果 73例MG患者PE后第3d、第14d和第30d临床绝对评分明显降低,差异具有统计学意义.PE对3例GBS和1例CIDP患者疗效肯定,1例MS患者行双重置换(double filtration plasmapheresis,therapy,DFPP)后临床症状改善不明显,以上患者在PE治疗过程中未见严重不良反应. 结论 PE在多数神经系统免疫疾病如MG、GBS和CIDP疗效肯定,不良反应较少.在治疗中枢神经系统脱髓鞘病(例如MS等)的情况有待进一步观察.%Objectives To assess the efficacy and side effects of plasma exchange (PE) in the treatment of myasthenia gravis (MG), Guillain-Barre syndrome (GBS), chronic inflammatory demyelination polyneuropathy (CIDP) and multiple sclerosis (MS) patients. Methods We retrospectively reviewed 78 neurological patients treated with PE, including MG 73 cases, GBS 3 cases, CIDP one case and MS one case. PE was performed every other day for 3 times, and then once a week (or twice a week for some patients). Efficacy was evaluated by clinical scales assessed before and after PE, and the side effects were recorded. Results In the 73 MG cases, the scores of absolute scale for clinical signs after PE for 3, 14 and 30 days were statistically lower than the score before PE. In the 3 GBS and one CIDP cases, PE was effective in shortening recovery period and improving disability. In the MS case, no prominent improvement was detected after

  2. A “Mini-Epidemic” of anti-glomerular basement membrane disease: Clinical and epidemiological study

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    Umesh Lingaraj

    2017-01-01

    Full Text Available Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM antibody disease is rare, estimated to occur in fewer than one case per million population and accounts for less than 20% of rapidly progressive glomerulonephritis. The prevalence among patients evaluated for potential glomerular disease is lower. It accounts for fewer than 3% of all kidney biopsies done with crescentic glomerulonephritis. Cases of anti-GBM disease occurring in a cluster have rarely been reported. All biopsy proven anti-GBM disease cases were collected from January 2015 to March 2015 at our Institute. All cases were analyzed for demographic and clinical profile, pathological findings, treatment received and for any common environmental antigenic source. A total of 11 new biopsy proven anti-GBM cases were seen within a span of three months. Age group varied from 17–80 years. Seven were males and four were females. All were dialysis dependent at presentation. Seven had active cellular crescents, and four had fibrocellular. Only one patient was a smoker and none had a history of exposure to any forms of hydrocarbons. The peak seen from January 2015 to March 2015 does not correlate with any of seasonal occurrence of infections in southern India. Although there was clustering of cases to southern territories of Karnataka state, no common etiological agents could be identified. No patient had any previous urological surgeries. All patients received methylprednisolone with plasmapheresis 5–7 sessions and cyclophosphamide. All 11 patients were dialysis dependent at the end of three months. We conclude anti-GBM disease cannot be regarded as a rare cause of renal failure and lung hemorrhage. The occurrence of such epidemic within a short period suggests a possible unidentified environmental factor like infection or occupational agents as inciting agents. Identification of such inciting agents could help us in instituting appropriate preventing measures.

  3. Pathogenesis and Management of Pruritus in PBC and PSC.

    Science.gov (United States)

    Kremer, Andreas E; Namer, Barbara; Bolier, Ruth; Fischer, Michael J; Oude Elferink, Ronald P; Beuers, Ulrich

    2015-01-01

    , plasmapheresis, albumin dialysis or nasobiliary drainage. This review outlines the current knowledge on pathogenesis of cholestatic pruritus and summarizes evidence-based and experimental therapeutic interventions for cholestatic patients with itch.

  4. ADAMTS13 content in plasma-derived factor VIII/von Willebrand factor concentrates.

    Science.gov (United States)

    Peyvandi, Flora; Mannucci, Pier M; Valsecchi, Carla; Pontiggia, Silvia; Farina, Claudio; Retzios, Anastassios D

    2013-10-01

    Thrombotic thrombocytopenic purpura (TTP) is a microangiopathy syndrome caused by a congenital or acquired deficiency of ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor (VWF) and thus prevents the formation of platelet-rich thrombi in the microcirculation. TTP can be fatal if not appropriately and timely treated with the infusion of fresh frozen plasma (FFP) or exchange plasmapheresis, that reverse the process of microangiopathy by removing anti-ADAMTS13 autoantibodies and replacing functional ADAMTS13. The treatment of TTP with FFP is not free from risks and must be administered in hospitals or clinics, owing to the substantial amount of plasma volume infused or exchanged and the frequent need of catheter application. Moreover, most FFPs are not subjected to treatments to remove or inactivate blood-borne infectious agents. A number of recent reports indicate that certain plasma-derived VWF-factor VIII (FVIII) concentrates are clinically effective in the treatment of congenital TTP. In this study, we measured ADAMTS13 levels in various plasma-derived VWF-FVIII concentrates, showing that Koate(®) -DVI (Grifols), contained relatively high amounts of ADAMTS13 and that Alphanate(®) (Grifols) was the closest other product in terms of protease content. Koate(®) -DVI contains, on average (five lots tested), 0.091 ± 0.007 Units of ADAMTS13 activity per IU of FVIII. On the basis of this analysis and other reports of VWF-FVIII concentrate utilization in congenital TTP, potential dosing, and future clinical developments are discussed.

  5. [Treatment of fulminant falciparum malaria with erythrapheresis].

    Science.gov (United States)

    Rouvier, B; Maudan, P; Debue, J F; Joussemet, M; Roué, R

    1988-01-01

    Ten days after his return from Cameroon, a twenty-six year old Frenchman, serving on voluntary service overseas, presented with fulminant falciparum malaria: shock, altered consciousness, haemolytic anaemia, threatening disseminated coagulation (platelets less than 150 X 10(-6).l-1; prothrombin time and Stuart factor less than 50%; fibrinogen less than 1.5 g.l-1). In spite of quinine therapy, parasitaemia increased from 4 to 35% within 24 h. Using an Haemonetics V50, the exchange of one and a half red blood cell masses was carried out with 17 red blood cell packs. Calcium gluconate was used to prevent the hypocalcaemia induced by the anticoagulant solution. The patient's platelets and plasma were completely reinjected. The result was very satisfactory. This kind of exchange, well tolerated clinically and biologically, would seem better than the classical exchange transfusion. When 10% of the red blood cells are infected by Plasmodium falciparum, it is necessary to exchange from one and a half to two blood masses. Lesser exchanges are always associated with important relapses and quinine therapy must be carried on during and after the exchange. Restricting this exchange only to red blood cells enabled the patient to benefit from his own coagulation factors, antibodies and platelets, and consequently to reduce the number of blood donors involved. However, metabolites (especially bilirubin and circulating immune complexes) were not eliminated. Partial plasmapheresis may be associated with erythropheresis using human albumin as plasma substitute. This technique needs to be assessed, in order to optimize immediate efficiency and post-transfusion infectious risk.

  6. Treatment of primary hypertriglyceridemia states--General approach and the role of extracorporeal methods.

    Science.gov (United States)

    Stefanutti, Claudia; Julius, Ulrich

    2015-05-01

    Hypertriglyceridemia (HTG) is a common metabolic disorder in which the concentration of very low density lipoproteins (VLDL) and of chylomicrons (CMs) is elevated in the plasma. HTG may be caused by primary and/or secondary causes and affected subjects may express HTG when children or in adulthood. In children and adults a genetic cause may underlie HTG which can be expressed as CMs a severe clinical picture known as Familial Hyperchylomicronemia due to lipoprotein lipase (LPL) or apolipoprotein (apo) CII deficiencies. Genetically determined HTG includes Familial Dysbetalipoproteinemia due to deficiency of apolipoprotein EIII of VLDL and Familial HTG. However, recent data suggest that classical Fredrickson phenotypes describing clinically HTG, which were once considered to be distinct based on biochemical features, have a shared genetic set up. The HTG has been classified according to a recent international paper: mild HTG: 2-10 mmol/L (176-882 mg/dL); severe HTG: > 10 mmol/L (>882 mg/dL) associated to CMs remnants, or Intermediate Density lipoprotein (IDL) like particles, and/or CMs. The treatment includes limitation of dietary content of saturated fat and alcohol, fibrates and omega3 fatty acids. When TG are severely elevated and associated to CMs the risk of acute pancreatitis suggests the use of more drastic therapeutic option such as therapeutic plasma exchange. This paper summarizes the experience with conventional plasmapheresis (Plasma-Exchange, PEX) and different Lipoprotein Apheresis methods with respect to acutely lowering TG levels in patients with normal TG, with mild and severe HTG. Upcoming promising therapies are gene therapy, novel apolipoprotein CIII inhibitors and lomitapide. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  7. Use of Permcath (Quinton) catheter in uraemic patients in whom the creation of conventional vascular access for haemodialysis is difficult.

    Science.gov (United States)

    Pourchez, T; Morinière, P; Fournier, A; Pietri, J

    1989-01-01

    During the last 4 years, the Permcath Quinton double-lumen silicone catheter was inserted into the internal jugular vein of 57 uraemic patients with difficulty for creating conventional vascular access for haemodialysis. In 4 patients, with definitive contraindication of conventional vascular access, this catheter still permits haemodialysis after a duration of 8-25 months. In 25 further patients with terminal uraemia, but poor vein system, it allowed the maturation of an arteriovenous fistula after 2-14 months of use. In 17 patients already on chronic haemodialysis, but who lost abruptly their vascular access (15 grafts and 2 arteriovenous fistulae), it allowed a new arteriovenous fistula to mature in 16 cases after a mean duration of 7.3 +/- months. In 5 patients with short life expectancy because of neoplasia, it allowed to dialyse them until their death which occurred after 6.5 +/- 2.2 months. In 6 patients with acute renal failure and haemostasis problems, it allowed to perform not only dialysis, but also plasmapheresis in 3 and parenteral nutrition in 3 other cases. The complications were the following: sepsis (n = 3); episodes of hypocoagulability due to inadvertent injection of heparin stored in the lumen (n = 2), thrombosis of the lumen (n = 3), and insufficient flow (n = 6). In no case these complications prevented continuation of haemodialysis. The catheter had to be removed in 2 cases because of septis and in 1 case because of insufficient flow. In 3 cases the catheter had to be replaced because of thrombosis and in 1 case because of laceration. These complication rates are, however, fewer than those reported in the literature for arteriovenous shunts or rigid subclavian and femoral catheters. The Permcath catheter seems, therefore to be the method of choice for immediated vascular access in patients in whom the creation of conventional vascular access is difficult.

  8. Neuropsychiatric involvement in systemic lupus erythematosus: current therapeutic approach.

    Science.gov (United States)

    Sanna, Giovanni; Bertolaccini, Maria Laura; Khamashta, Munther A

    2008-01-01

    The involvement of the central nervous system (CNS) is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE) patients and the less understood aspect of the disease. Its recognition and treatment continue to represent a major diagnostic and therapeutic challenge. Due to the lack of controlled randomized trials, current therapeutic approach is still empirical and based on clinical experience. The therapeutic choice depends on accurate diagnosis, identification of underlying pathogenic mechanism, severity of the presenting neuropsychiatric symptoms, and on prompt identification and management of contributing causes of CNS disease. Mild neuropsychiatric manifestations may need symptomatic treatment only. In more severe CNS disease it is important to distinguish between thrombotic and non-thrombotic mechanisms. Focal CNS manifestations, particularly TIA and stroke, are associated with the presence of antiphospholipid antibodies (aPL). Anticoagulation is warranted in patients with thrombotic disease, particularly in those with the antiphospholipid (Hughes) syndrome (APS). Other CNS manifestations, such as demyelinating syndrome, transverse myelitis, chorea, seizures, migraine and/or cognitive dysfunction, when associated with persistent positivity for aPL, may also benefit from anticoagulation in selected patients. Severe diffuse CNS manifestations, such as acute confusional state, generalised seizures, mood disorders and psychosis, generally require corticosteroids in the first instance. Pulse intravenous cyclophosphamide therapy may help when more severe manifestations are refractory to corticosteroids and other immunosuppressive agents, generally when response is not seen in 3-5 days. Plasmapheresis may also be added in severe cases of symptoms refractory to conventional treatment. Intravenous immunoglobulins, mycophenolate mofetil, rituximab, intratecal methotrexate and dexametasone deserve further studies to confirm their

  9. Treatment of Acute Antibody-Mediated Renal Allograft Rejection With Cyclophosphamide.

    Science.gov (United States)

    Waiser, Johannes; Duerr, Michael; Budde, Klemens; Rudolph, Birgit; Wu, Kaiyin; Bachmann, Friederike; Halleck, Fabian; Schönemann, Constanze; Lachmann, Nils

    2017-10-01

    Antibody-mediated rejection (AMR) is a major risk for renal allograft survival. Throughout decades, cyclophosphamide treatment has been proven to be effective in patients with antibody-associated autoimmune diseases. We investigated whether cyclophosphamide combined with plasmapheresis and intravenous immunoglobulins is an option for patients with AMR. Between March 2013 and November 2015, we initiated treatment of 13 consecutive patients with biopsy-proven acute AMR with intravenous cyclophosphamide pulses (15 mg/kg adapted to age and renal function) at 3-week intervals, PPH (6×), and high-dose intravenous immunoglobulin (1.5 g/kg). Treatment was completed after 6 cyclophosphamide pulses or in case of return to baseline serum creatinine together with reduction of donor-specific HLA antibodies (DSA) below 500 mean fluorescence intensity. Eleven of 13 patients completed treatment. Median follow-up was 18 (12-44) months. At the end of follow-up, graft survival was 77% (10/13). The 3 graft losses were caused at least in part by nonadherence and premature termination of treatment. Serum creatinine increased from 1.7±0.4 mg/dL at 3 months before diagnosis to 3.7±2.4 mg/dL at diagnosis (P = 0.01), and decreased to 2.1 ± 0.7 mg/dL at 3 months after diagnosis (P = 0.01). In 7 (64%) of 11 patients, who completed treatment, DSA decreased, in 4 (36%) of 11 DSA were below 500 mean fluorescence intensity after treatment. Dose reductions had to be performed in 3 of 13 patients for leukopenia. We observed 14 hospitalizations in 9 of 13 patients. To our knowledge, this is the first systematic report on cyclophosphamide-based treatment of acute AMR based on modern diagnostics. Treatment was effective and relatively safe. Future studies will show, whether cyclophosphamide proves to be a valuable alternative for the treatment of AMR.

  10. Pre-conception preparation at the antiphospholipid syndrome as way to improve reproductive health

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    Gulyash Tanysheva

    2014-01-01

    Full Text Available Introduction: Reproductive health is characterized by the condition of the woman in association with the course of pregnancy and childbirth. In this case, the absence of disease plays a fundamental role. Unfortunately, conditions that can negatively impact reproductive health and cause deterioration of pregnancy and delivery outcomes are frequent in women of reproductive age. Antiphospholipid syndrome (APS is one of the leading conditions that can negatively affect reproductive health and lead to various complications in pregnancy including fetal loss. Materials and methods: We assessed the effectiveness of pre-conception preparing, including traditional therapy of APS in conjunction with system enzyme therapy (SET and plasmapheresis sessions. We conducted a study in two groups: women with APS and pre-conception preparing (n = 49 and the control group were women without pre-conception preparing (n = 46. Results: The effect of pre-conception preparing in women with APS was assessed by the course and outcome of pregnancy. The total number of women with complications of pregnancy were 39.1% lower in the study group compared to the control group. Risk of miscarriage in the basic group observed 68.7 % less frequently compared to the  control group. The frequency of pre-eclampsia was 63.5 % less in the study group compared to the control group. We observed significantly lower rates of placental insufficiency in the study group and the difference in this parameter reached 65.2%. The risk of pre-term birth was 59.4 % lower in the study group compared to the control group. Conclusion: We concluded that pre-conception preparing in women with APS increases the possibility of physiological course pregnancy. Pre-conception preparing reduces the incidence of miscarriage, pre-term labor, and the development of pre-eclampsia, and placental insufficiency.

  11. [Catastrophic antiphospholipid syndrome during pregnancy].

    Science.gov (United States)

    Derks, Marloes; Oudijk, Martijn A; van der Made, Flip; van den Born, Bert-Jan H; van der Post, Joris A M

    2011-01-01

    Antiphospholipid syndrome (APS) had been previously diagnosed in three pregnant women aged 32, 27 and 36 years, respectively. All three of them were using low-molecular weight heparin for the prevention of thromboembolic complications. The first two women were admitted because of foetal growth retardation. In the first patient, either HELLP syndrome or exacerbation of APS was suspected. A caesarean section was performed due to foetal distress. The patient's condition deteriorated further postoperatively. Multiple infarctions in liver and placenta were identified. Catastrophic antiphospholipid syndrome (CAPS) was diagnosed. Despite intensive medical treatment including anticoagulation the patient died of massive pulmonary embolism. The second patient suffered from thrombocytopaenia, disturbances in hepatic function and epigastric pain. CAPS was diagnosed. The condition improved after treatment with glucocorticoids, but because of a poor foetal prognosis, delivery was induced and a lifeless son was born. The third woman was admitted due to pyelonephritis. Shortly thereafter, symptoms of HELLP syndrome developed and she was administered glucocorticoids. Hepatic infarcts and petechiae developed, indicating CAPS. Delivery was induced and a girl was born. Glucocorticoid treatment was resumed and combined with immunoglobulins and plasmapheresis. The patient recovered and was discharged together with her daughter. CAPS is a life-threatening variation of APS. It is characterised by multiple thromboses and rapidly progressive multi-organ failure. Mortality is high, but seems to diminish with treatment by immunosuppressive therapy. In pregnancy, clinical signs of CAPS are similar to those of HELLP syndrome. Since the treatment for HELLP syndrome is different from CAPS, a correct diagnosis is essential. Because of the rarity of this condition in combination with high rates of perinatal and maternal mortality, care for pregnant patients with APS should be centralised in

  12. Pregnancy Outcome in Women with Obstetric and Thrombotic Antiphospholipid Syndrome-A Retrospective Analysis and a Review of Additional Treatment in Pregnancy.

    Science.gov (United States)

    Mayer-Pickel, Karoline; Eberhard, Katharina; Lang, Uwe; Cervar-Zivkovic, Mila

    2016-07-09

    Antiphospholipid syndrome (APS) is associated with pregnancy complications such as recurrent early fetal loss (RFL), fetal death, preeclampsia (PE), and intrauterine growth restriction (obstetric APS/OAPS). Other clinical manifestations are venous and/or arterial thromboses (thrombotic APS/TAPS). The data of 37 pregnancies with OAPS and 37 pregnancies with TAPS were analyzed and compared. Overall, the most frequent APS antibodies (aPl) were LA as well as "triple-positivity"; LA antibodies were significantly more frequent in women with TAPS (67.6 % TAPS vs. 29.7 % OAPS, p pregnancies with TAPS, whereas in 18.9 % of pregnancies with OPS, no therapy has been given at all. One woman in OAPS and four women in TAPS were treated with plasmapheresis and immunoadsorption. There was no significant association between adverse obstetric outcome and therapy. The most frequent pregnancy complications were RFL in the OAPS group (32.4 vs. 13.5 % in TAPS) and PE in the TAPS group (18.9 % in OAPS and TAPS, respectively). The data of our study showed that pregnancies with OAPS and TAPS have a similar rate of pregnancy complications. However, pregnancies with OAPS tend to have rather RFL. Although we were not able to reveal a significant association with adverse obstetric outcome, it seems that the current adequate therapy for APS in pregnancy, consisting of LDA and LMWH, might rather prevent the development of RFL. Additionally, it might be considered to divide the obstetric APS into obstetric APS with early pregnancy complications and obstetric APS with late pregnancy complications. The division into two groups of obstetric APS might facilitate the choice of additional therapy in these women.

  13. Nephrogenic systemic fibrosis

    Directory of Open Access Journals (Sweden)

    Bhushan Madke

    2011-01-01

    Full Text Available Nephrogenic systemic fibrosis (NSF is a relatively new fibrosing disorder which has caught the attention of various specialities in the past decade. NSF is an extremely disabling and often painful condition, affecting up to 13% of the individuals with chronic kidney disease. The administration of a gadolinium chelate contrast agent has been reported to induce the development of NSF, particularly in patients who have acute or chronic renal disease with a glomerular filtration rate (GFR lower than 30-mL/min/1.73 m 2 and in those with acute renal insufficiency. Mass spectroscopy studies have demonstrated particles of gadolinium in the lesional tissue. The exact pathogenesis of this curious sclerosing condition is unknown. The role of the aberrant targeting of ′circulating fibrocytes′ to the peripheral tissues and viscera has been hypothesized. NSF has distinct clinicopathological features in the setting of renal failure and needs to be looked upon as a new entity on the block. The condition is characterized by irregular indurated plaques, with amoeba-like projections and islands of sparing, chiefly on the trunk and extremities. Flexion contractures of fingers, knees, and elbow joints are known to occur in advanced cases of NSF. The course is frequently associated with painful episodes and loss of ambulation. Histopathology shows haphazard arrangement of thickened bundles of collagen, varying amount of mucin, and increased population of fibroblast-like cells in the dermis. Immunohistochemistry shows increased deposition of type-I procollagen and CD 34+ cells having fibroblastic activity. The condition is refractory to treatment with corticosteroids and immunosuppressive agents. Various modalities of therapy such as UVA1 phototherapy, imatinib mesylate, photodynamic therapy, plasmapheresis, extracorporeal photochemotherapy, and high-dose intravenous immunoglobulin have shown a moderate degree of improvement in skin thickness scores. A prudent

  14. Chronic respiratory disfunction due to diffuse alveolar hemorrhage in patients with systemic lupus erythematosus and primary vasculitis.

    Science.gov (United States)

    Pérez Aceves, Eva; Pérez Cristóbal, Mario; Espinola Reyna, Gerardo A; Ariza Andraca, Raul; Xibille Fridmann, Daniel; Barile Fabris, Leonor A

    2013-01-01

    Pulmonary hemorrhage (PH) occurs in 2-5% of SLE patients, and is associated with a high mortality rate (79-90%). Diagnostic criteria for this complication include: 1) Pulmonary infiltrates, with at least ¾ of lung tissue involved in a chest x ray, 2) Acute respiratory failure, 3) A decrease of 3g/dL or more in hemoglobin levels. PH might lead to organized pneumonia, collagen deposition, and pulmonary fibrosis which in time might cause changes in pulmonary function tests with either restrictive or obstructive patterns. To evaluate the existence of abnormalities in pulmonary function tests after a PH episode. We included patients with SLE and primary vasculitis that developed PH. During the acute episode, we measured SLEDAI in SLE patients, five factor score in microscopic polyangiitis (MPA) and Birmingham Vasculitis Activity Store (BVAS) in granulomatosis with polyangiitis (GPA) (Wegener). We determined the number of PH events, treatment, and ventilator assistance requirements and correlated its association with abnormal pulmonary function tests. We included 10 patients, 7 with SLE, 2 with MPA and 1 with GPA (Wegener). The mean activity measures were: SLEDAI 20.4 ± 7.5, FFS 2, and BVAS 36. Treatment consisted in methylprednisolone (MPD) in 3 patients, MPD plus cyclophosphamide (CY) in 6 patients, and MPD, CY, IV immunoglobulin, and plasmapheresis in one patient. Five patients required ventilatory support. We found abnormalities in pulmonary function tests in 8 patients, three had an obstructive pattern and five a restrictive pattern; 2 patients did not show any change. We did not find a significant association with any of the studied variables. PH might cause abnormalities in pulmonary function tests and prolonged immunosuppressive treatment could be required. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  15. Kidney transplantation in immunologically high-risk patients.

    Science.gov (United States)

    Keven, K; Sengul, S; Celebi, Z K; Tuzuner, A; Yalcin, F; Duman, T; Tutkak, H

    2013-04-01

    An increased number of sensitized patients await kidney transplantation (KTx). Sensitization has a major impact on patient mortality and morbidity due to prolonged waiting time and may preclude live donor transplantation. However, recent reports have shown that KTx can be performed successfully using novel immunosuppressive protocols. This study presents our experience with patients displaying donor-specific antibody (DSA) (+). We enrolled 5 lymphocyte cross-match (LCM) negative (complement-dependent cytotoxicity) and panel-reactive antibody (PRA) plus DSA-positive patients mean fluorescein intensity [MFI] > 1000) who underwent living kidney donor procedures. All subjects were females and their mean age was 36.7 years. In our protocol, we started mycophenolate mofetil (2 g/d), tacrolimus (0.01 mg/kg) and prednisolone (0.5 mg/kg) on day -6. We performed 2 sessions of total plasma exchange (TPE) with albumin replacement and administered 2 doses of IVIG (5 g/d). On day -1, we added rituximab (200 mg). On the operation day and on day +4, the patients received doses of basiliximab. Serum samples were taken on days -6, 0, and 30 as well as at 1 year after transplantation. All patients displayed immediate graft function. Mean basal DSA titer was 5624 MFI. After desensitization, the MFI titers decreased at the time of transplantation to 2753 MFI, and were 2564 MFI at the 1st month and 802 MFI at 1st year. Three patients experienced acute rejection episodes (60%). After treatment for rejection, the average follow-up was 17 months and last creatinine levels were 0.6-0.8 mg/dL (minimum-maximum). In conclusion, KTx can be succesfully performed in sensitized patients displaying DSA. However, there seems to be a greater acute rejection risk. There is no consensus regarding adequate doses of IVIG or plasmapheresis treatments; furthermore, more studies are needed to clarify the safe MFI titer of the DSA.

  16. Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives.

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    Magro-Checa, César; Zirkzee, Elisabeth J; Huizinga, Tom W; Steup-Beekman, Gerda M

    2016-03-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both the central and peripheral nervous system. Due to the lack of a gold standard, the attribution of NP symptoms to SLE represents a clinical challenge that obligates the strict exclusion of any other potential cause. In the acute setting, management of these patients does not differ from other non-SLE subjects presenting with the same NP manifestation. Afterwards, an individualized therapeutic strategy, depending on the presenting manifestation and severity of symptoms, must be started. Clinical trials in NPSLE are scarce and most of the data are extracted from case series and case reports. High-dose glucocorticoids and intravenous cyclophosphamide remain the cornerstone for patients with severe symptoms that are thought to reflect inflammation or an underlying autoimmune process. Rituximab, intravenous immunoglobulins, or plasmapheresis may be used if response is not achieved. When patients present with mild to moderate NP manifestations, or when maintenance therapy is warranted, azathioprine and mycophenolate may be considered. When symptoms are thought to reflect a thrombotic underlying process, anticoagulation and antiplatelet agents are the mainstay of therapy, especially if antiphospholipid antibodies or antiphospholipid syndrome are present. Recent trials on SLE using new biologicals, based on newly understood SLE mechanisms, have shown promising results. Based on what we currently know about its pathogenesis, it is tempting to speculate how these new therapies may affect the management of NPSLE patients. This article provides a comprehensive and critical review of the literature on the epidemiology, pathophysiology, diagnosis, and management of NPSLE. We describe the most

  17. Cryoglobulinemia Vasculitis.

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    Cacoub, Patrice; Comarmond, Cloe; Domont, Fanny; Savey, Léa; Saadoun, David

    2015-09-01

    Cryoglobulinemic vasculitis (CryoVas) is a small-vessel vasculitis involving mainly the skin, the joints, the peripheral nervous system, and the kidneys. Type I CryoVas is single monoclonal immunoglobulins related to an underlying B-cell lymphoproliferative disorder. Type II and III cryoglobulins, often referred to as mixed cryoglobulinemia, consist of polyclonal immunoglobulin (Ig)G with or without monoclonal IgM with rheumatoid factor activity. Hepatitis C virus (HCV) infection represents the main cause of mixed CryoVas. The 10-year survival rates are 63%, 65%, and 87% in HCV-positive mixed CryoVas, HCV-negative mixed CryoVas, and type I CryoVas patients, respectively. In HCV-positive patients, baseline poor prognostic factors include the presence of severe liver fibrosis, and central nervous system, kidney, and heart involvement. Treatment with antivirals is associated with a good prognosis, whereas use of immunosuppressants (including corticosteroids) is associated with a poor outcome. In HCV-negative patients, pulmonary and gastrointestinal involvement, renal insufficiency, and age > 65 years are independently associated with death. Increased risk of lymphoma also should be underlined. Treatment of type I CryoVas is that of the hemopathy; specific treatment also includes plasma exchange, corticosteroids, rituximab, and ilomedine. In HCV-CryoVas with mild-to-moderate disease, an optimal antiviral treatment should be given. For HCV-CryoVas with severe vasculitis (ie, worsening of renal function, mononeuritis multiplex, extensive skin disease, intestinal ischemia…) control of disease with rituximab, with or without plasmapheresis, is required before initiation of antiviral therapy. Other immunosuppressants should be given only in case of refractory forms of CryoVas, frequently associated with underlying B-cell lymphoma.

  18. Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

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    Karolina Kędzierska

    2016-03-01

    Full Text Available Background: Pyoderma gangrenosum (PG is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG. The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment. Methods: Systematic review of the literature in PubMed using the search terms “Wegener granulomatosis AND Pyoderma gangrenosum” and case report. Results: The finding of 22 reports in the literature (PubMed suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide. Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

  19. Desensitization Protocol in Recipients of Deceased Kidney Donor With Donor-Specific Antibody-Low Titers.

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    Kanter Berga, J; Sancho Calabuig, A; Gavela Martinez, E; Puig Alcaraz, N; Avila Bernabeu, A; Crespo Albiach, J; Molina Vila, P; Beltrán Catalan, S; Pallardó Mateu, L

    2016-11-01

    Kidney transplantation is the better option for end-stage renal disease (ESRD), but for patients with human leukocyte antigen (HLA) sensitization, the wait times are significantly longer than for patients without antibodies. Many desensitization protocols have been described involving strong immunosuppression, the use of apheresis, and B-cell-modulating therapies. We have designed a desensitization protocol from day 0 for deceased donor kidney transplantation. Our aim was to present our initial experience with five kidney transplant patients. All patients had a negative complement-dependent cytotoxicity cross-match. The desensitization protocol included five to seven doses of thymoglobulin (1.25 mg/kg) and three sessions of plasmapheresis (PP) within the first week after transplantation, with intravenous immunoglobulin (500 mg/kg) after each PP session and one dose of rituximab on day 8. The presence of donor-specific antibodies (DSA) was analyzed by use of Luminex technology; levels between 1000 and 3000 mean fluorescence intensity were considered for desensitization. The median age was 44 years and median renal replacement therapy time was 9 years. All recipients presented 1 to 3 DSA specificities. There were no severe side effects related to PP, infusion of intravenous immunoglobulin, or rituximab. The median follow-up period was 19.3 months. Median serum creatinine level at last follow-up was 1.7 mg/dL. A kidney biopsy was performed in all patients. Graft and patient survival was 100%. Until now, few data are available concerning whether HLA-incompatible kidney transplantation after desensitization would benefit patients with ERSD. The desensitization strategy using the combination of PP, low doses of intravenous immunoglobulin, and rituximab at our center resulted in a satisfactory clinical outcome. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Kidney transplantation after desensitization in sensitized patients: a Korean National Audit.

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    Huh, Kyu Ha; Kim, Beom Seok; Yang, Jaeseok; Ahn, Jeongmyung; Kim, Myung-Gyu; Park, Jae Berm; Kim, Jong Man; Chung, Byung-Ha; Kim, Joong Kyung; Kong, Jin Min

    2012-10-01

    The number of end-stage renal disease (ESRD) patients with preformed antibodies waiting for a kidney transplant has been increasing lately. We conducted a nationwide study on the outcomes of kidney transplantation after desensitization in Korea. Six transplant centers have run desensitization programs. The patients who underwent living donor kidney transplantation after desensitization from 2002 to 2010 were retrospectively analyzed. A total of 86 cases were enrolled. Thirty-five of these were cases of re-transplantation (40.7 %). Indications of desensitization were positive complement-dependent cytotoxicity (CDC) cross-match responses (CDC(+), 36.0 %), positive flow-cytometric cross-match responses (FCX(+), 54.7 %), and positive donor-specific antibodies (DSA(+), 8.1 %). The desensitization protocols used pre-transplant plasmapheresis (95.3 %), intravenous immunoglobulin (62.8 %), and rituximab (67.4 %). Acute rejection occurred in 18 patients (20.9 %), graft failure occurred in 4 patients, and the 3-year graft survival rate was 93.8 %. The presence of DSA increased the acute rejection rate (P = 0.015) and decreased the 1-year post-transplant estimated glomerular filtration rate (P = 0.006). Although rejection-free survival rates did not differ significantly between the CDC(+) and FCX(+) groups, the 1-year estimated glomerular filtration rate was lower in the CDC(+) group (P = 0.010). Infectious and significant bleeding complications occurred in 15.5 % and 4.7 % of cases, respectively. Kidney transplantation after desensitization had good graft outcomes and tolerable complications in Korea, and therefore, this therapy can be recommended for sensitized ESRD patients.

  1. Infectious complications in living-donor kidney transplant recipients undergoing multi-modal desensitization.

    Science.gov (United States)

    Turza, Kristin C; Shafique, Michael; Lobo, Peter I; Sawyer, Robert G; Keith, Douglas S; Brayman, Kenneth L; Agarwal, Avinash

    2014-06-01

    Pre-existing humoral barriers challenge the transplantation of living donor kidneys (LDK) into highly sensitized ABO- and human leukocyte antigen (HLA)-incompatible recipients. Conditioning these LDK recipients' immune systems is required before they undergo transplantation. We hypothesized that medical desensitization would yield higher post-transplantation rates of infection. We conducted a study in which matched controls consisting of non-desensitized (NDS) LDK recipients were compared with desensitized (DS) receipients. Pre-transplantation desensitization included treatment with rituximab and mycophenolate mofetil followed by intravenous immunoglobulin (IVIg) and plasmapheresis. All participants in the study underwent induction therapy and maintenance immunosuppression. Primary outcomes included infection (opportunistic, local, systemic) within 12 mo after transplantation. Twenty-five patients underwent desensitization and LDK transplantation. Graft survival in the DS and NDS groups of patients was 96% and 98%, respectively. The mean 3- and 12-mo serum creatinine concentrations in the DS and NDS groups were 1.1±0.2 mg/dL and 1.2±0.3 mg/dL and 0.95±0.4 mg/dL and 0.73±0.8 mg/dL (p=0.3 and p=0.01), respectively. Thirty-six percent of the patients in the DS group had one or more infections, vs. 28% of those in the NDS group (p=0.1). No difference was observed in the frequency of opportunistic or systemic infections in the two groups. Local infections were statistically significantly more frequent in the DS group (60% vs. 30%, respectively; p=0.02). Pre-operative desensitization in highly sensitized LDK recipients is followed by a similar incidence of opportunistic and systemic infections as in NDS patients. Local infections were significantly more frequent in the DS than in the NDS patients in the study. With careful monitoring of infectious complications, pre-transplant desensitization permits LDK transplantation into highly sensitized patients.

  2. Pharmaceutical costs of desensitization therapy in patients awaiting lung transplantation in France.

    Science.gov (United States)

    Liu, Annaë; Bernard, Mélisande

    2014-02-01

    Based on solid experience in renal transplant, new treatments aiming to decrease anti-human leukocyte antigen (HLA) antibodies in patients awaiting lung transplant have recently been developed. The off-label use of high-dose intravenous polyvalent immunoglobulins (IVIg) and/or plasmapheresis changes the economical weight of pharmaceutical cost before lung transplantation. Our objective was to assess the budgetary impact of pharmaceutical costs of desensitization therapy. Two observational studies were conducted in 2009 and 2010 at the Bichat Claude Bernard (BCB) hospital in France. The first assessed the real pharmaceutical costs, and identified cost drivers, of desensitized (D+) patients awaiting lung transplantation. The second compared pharmaceutical and clinical data between D+ and non-treated (D-) patients. The major cost drivers were IVIg, representing 89.7 % of pharmaceutical costs. The real cost of drugs was €4,392 ± 647 per hospitalization. Mean hospitalization and annual pharmaceutical costs per patient were significantly higher for D+ than for D- patients (€6,972 vs. 2,925 and €13,074 vs. 399). D+ patients had a significantly higher average number of annual hospitalizations than did D- patients. Total IVIg costs represented 98 % of the pharmaceutical costs for desensitization stays. Pharmaceutical costs represented 40 % of total hospitalization costs for D+ versus only 7 % for D-. New desensitization protocols can help to manage the immunological hurdle of anti-donor antibodies in lung transplantation. They are expensive and not yet correctly covered by national health insurance, as they are supported by hospital budgets. A medico-economical evaluation of IVIg use in this indication seems necessary.

  3. Treatment Algorithms in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Muangchan, Chayawee; van Vollenhoven, Ronald F; Bernatsky, Sasha R; Smith, C Douglas; Hudson, Marie; Inanç, Murat; Rothfield, Naomi F; Nash, Peter T; Furie, Richard A; Senécal, Jean-Luc; Chandran, Vinod; Burgos-Vargas, Ruben; Ramsey-Goldman, Rosalind; Pope, Janet E

    2015-09-01

    then rituximab, IVIG, or plasmapheresis; and serious lupus nephritis: first-line therapy was glucocorticoids and mycophenolate, then cyclophosphamide then rituximab. We established variable agreement on treatment approaches. For some treatment decisions there was good agreement between experts even if no randomized controlled trial data were available. © 2015, American College of Rheumatology.

  4. Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis

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    Hohenforst-Schmidt W

    2013-07-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Arndt Petermann,2 Aikaterini Visouli,3 Paul Zarogoulidis,4 Kaid Darwiche,5 Ioanna Kougioumtzi,6 Kosmas Tsakiridis,3 Nikolaos Machairiotis,6 Markus Ketteler,2 Konstantinos Zarogoulidis,4 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 2Division of Nephrology, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 3Cardiothoracic Surgery Department, “Saint Luke” Private Hospital, Thessaloniki, Greece; 4Pulmonary Department, “G Papanikolaou” General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 5Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 6Surgery Department (National Health System, University General Hospital of Alexandroupolis, Alexandroupolis, GreeceAbstract: Extracorporeal membrane oxygenation (ECMO is increasingly applied in adults with acute refractory respiratory failure that is deemed reversible. Bleeding is the most frequent complication during ECMO support. Severe pre-existing bleeding has been considered a contraindication to ECMO application. Nevertheless, there are cases of successful ECMO application in patients with multiple trauma and hemorrhagic shock or head trauma and intracranial hemorrhage. ECMO has proved to be life-saving in several cases of life-threatening respiratory failure associated with pulmonary hemorrhage of various causes, including granulomatosis with polyangiitis (Wegener’s disease. We successfully applied ECMO in a 65-year-old woman with acute life-threatening respiratory failure due to diffuse massive pulmonary hemorrhage secondary to granulomatosis with polyangiitis, manifested as severe pulmonary-renal syndrome. ECMO sustained life and allowed disease control, together with plasmapheresis, cyclophosphamide, corticoids, and renal replacement therapy. The patient was successfully weaned from ECMO

  5. Morvan Syndrome

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    Maskery, Mark; Chhetri, Suresh K.; Dayanandan, Rejith; Gall, Claire

    2016-01-01

    A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management. PMID:26740856

  6. BIOPSY-PROVEN BK VIRUS NEPHROPATHY WITHOUT DETECTABLE BK VIREMIA IN A ONE-YEAR POST-KIDNEY TRANSPLANT RECIPIENT.

    Science.gov (United States)

    Ruangkanchanasetr, Prajej; Pumchandh, Norawee; Satirapoj, Bancha; Termmathurapoj, Sumeth; Pongthanapisith, Viroj

    2015-07-01

    BK virus nephropathy (BKVN) is an important clinical problem in kidney transplant (KT) recipients. The sequence of disease is usually viruria, viremia and then nephropathy. Diagnosis of BK virus (BKV) infection includes checking BKV DNA in the urine, in the plasma and histology on renal biopsy. This last method is used to diagnose BKVN. We describe a KT patient with BKVN without detectable BK viremia. A 62-year-old female with hypertensive nephropathy underwent renal transplant from a living relative donor in December 2011. Fourteen months after transplantation, her serum creatinine(SCr) rose up from 1.2 to 1.6 mg/dl with biopsy-proven acute antibody-mediated and cellular rejection. After pulse methylprednisolone, plasmapheresis and intravenous immunoglobulin, her SCr decreased to baseline but she subsequently developed cytomegalovirus infection with pancytopenia and transaminitis. The SCr rose to 1.9 mg/dl despite ganciclovir treatment. Renal ultrasound and antegrade pyelogram showed partial obstruction of the proximal ureter with moderate hydronephrosis. A quantitative polymerase chain reaction (PCR) assay for BKV DNA was negative (less than 10 copies/ml). A renal biopsy was performed and the pathology revealed viral cytopathic changes in the tubular epithelium with interstitial inflammation. The renal biopsy also showed BKV nucleic acid sequences by in-situ hybridization confirming BKVN. Immunosuppression regimen was changed to cyclosporine, low-dose prednisolone and leflunomide. A temporary percutaneous nephrostomy was performed. Her renal function improved within one week. The diagnosis of BKVN should be considered in a KT recipient with a rising SCr with or without BK viremia and should be made by renal biopsy.

  7. A Case Report of Adrenocorticotropic Hormone to Treat Recurrent Focal Segmental Glomerular Sclerosis Post-transplantation and Biomarker Monitoring

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    Anwar eSiddiq

    2015-03-01

    Full Text Available Background: Recurrent focal segmental glomerular sclerosis (rFSGS in renal transplant recipients (RTR is difficult to predict and treat. Early rFSGS is likely from circulating factors and preformed antibodies. Methods: We present the case of a 23-year-old white man who presented with rFSGS and acute renal failure requiring dialysis 9-months after a 1-haplotype matched living-related transplant. We retrospectively analyzed serum samples from various clinical stages for rFSGS biomarkers: serum glomerular albumin permeability (Palb, soluble urokinase-type plasminogen activator receptor (suPAR serum level with suPAR-β3 integrin signaling on human podocytes, and angiotensin II type I receptor-antibody (AT1R-Ab titer. Results: All biomarkers were abnormal at 1-year pre-transplant prior to initiation of dialysis and at the time of transplant. After initiation of hemodialysis, β3 integrin activity on human podocytes, in response to patient serum, as well as AT1R-Ab were further elevated. At the time of biopsy-proven recurrence, all biomarkers were abnormally high. One week after therapy with aborted plasmapheresis (secondary to intolerance, and high dose steroids, the Palb and suPAR- β3 integrin activity remained significantly positive. After 12-weeks of treatment with high-dose steroids, rituximab, and galactose, the patient remained hemodialysis-dependent. Three-months after his initial presentation we commenced adrenocorticotropic hormone (ACTH, Acthar® Gel, 80 units subcutaneously twice weekly. Four-weeks later he was able to discontinue dialysis. After 8-months of maintenance ACTH therapy, his serum creatinine stabilized at 1.79 mg/dL with less than 1 gram of proteinuria. Conclusion: ACTH therapy was associated with improvement in renal function within 4 weeks. The use of rFSGS biomarkers may aid in predicting development of rFSGS.

  8. [Comparative study on the effect of highly active antiretroviral therapy (HAART) in patients with HIV infection acquired by blood transfusion and paid plasma donation].

    Science.gov (United States)

    Chen, Su-Liang; Zhang, Yu-Qi; Zhao, Hong-Ru; Chen, Zhi-Qiang; Li, Bao-Jun; Zhao, Cui-Ying; Bai, Guang-Yi; Liang, Liang; Wang, Wei

    2012-08-01

    To study the effect of HAART in patients with AIDS acquire by blood transfusion and paid plasma donation. All AIDS patients whose disease was caused by blood transfusion and commercial plasma donation came from the domicile of Hebei Province. In the group of cases of blood transfusion in whom the infection was caused by one-time blood transfusion before and after 1995, there were 189 cases, of whom 105 cases on HAART were designated as observation group, and 84 cases who were not on HAART were designated as control group. The group of AIDS patients who were former commercial plasma donors (FCPDs) had 120 patients who were identified in the survey of 1995, of whom 63 cases on HAART were designated as observation group and 57 cases who were not on HAART were as control group. Onset dates were defined as the dates into the queue. Death due to AIDS was regarded as an outcome event. Subjects who were enrolled into the observation group and control group were epidemiologically followed up regularly. Observation was ended on December 31, 2010. Mortality of patients after HAART from groups of FCPDs and blood recipients were 4.42/100 person-years and 6.13/100 person-years, respectively. The survival rates of HAART groups were 80% in FCPDs for 110 months and 72% in blood recipients for 90 months, respectively. Meanwhile the mortality of no HAART groups were 182.05/100 person-years and 250.66/100 person-years, respectively. Mean survival of patients whose disease was caused by plasma donation and blood transfusion were 4 months and 3 months, respectively. Whether the HIV infection was caused by plasmapheresis or blood transfusion, the effects of HAART did not show difference. HAART cold reduce the death intensity and prolong survival.

  9. Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

    Science.gov (United States)

    Barut, Kenan; Yücel, Gözde; Sinoplu, Ada Bulut; Şahin, Sezgin; Adroviç, Amra; Kasapçopur, Özgür

    2015-01-01

    Aim: This study aimed to evaluate the demographic, clinical, laboratory properties of patients with macrophage activation syndrome and treatment outcomes. Material and Methods: The data of the patients who were diagnosed with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis between June 2013–May 2014 were evaluated by screening patient records. Results: Ten patients with macrophage activation syndrome were followed up in one year. The mean age at the time of diagnosis was found to be 7.6±4.5 years. The most common clinical finding at presentation (80%) was increased body temperature. Hepatosplenomegaly was found in half of the patients. The most common hematological finding (90%) was anemia. The mean erythrocyte sedimentation rate was found to be 71.8±36.2 mm/h, whereas it was measured to be lower (31.2±25.2 mm/h) at the time of the diagnosis of macrophage activation syndrome. Increased ferritin level was found in all of our patients (the mean ferritin level was found to be 23 957±15 525 ng/mL). Hypertriglyceridemia was found in nine patients (90%). The mean triglyceride level was found to be 397±332 mg/dL. Systemic steroid treatment was administered to all patients. Cyclosporine A was given to eight patients (80%), canakinumab was given to four patients (40%) and anakinra was given to five patients (50%). Plasmapheresis was performed in two patients. Improvement was found in all patients except for one patient. The patient in whom no improvement was observed showed a chronic course. Conclusions: The diagnosis of macrophage activation syndrome should be considered in presence of sudden disturbance in general condition, resistant high fever and systemic inflammation findings in children with active rheumatic disease. Complete recovery can be provided with early and efficient treatment in macrophage activation syndrome which develops secondary to systemic juvenil idiopathic arthritis. PMID:26884689

  10. Extracorporeal blood therapy in sepsis and acute respiratory distress syndrome: the "purifying dream".

    Science.gov (United States)

    Xu, Xuefeng; Dai, Huaping; Jia, Chun'e; Wang, Chen

    2014-01-01

    To discuss the rationale, hypothesis, modality of extracorporeal blood purification (EBP) techniques for the critically ill animal models or patients, and to summarize the experimental and clinical studies with inconsistent data which explored the EBP's efficacy in the areas of critical care medicine. Articles referred in this review were collected from the database of PubMed published in English up to June 2014. We had done a literature search by using the term "(sepsis OR acute lung injury OR acute respiratory distress syndrome) AND (extracorporeal blood purification OR hemofiltration OR hemoperfusion OR plasma exchange OR plasmapheresis OR adsorpiton)". Related original or review articles were included and carefully analyzed. Acute cellular and humoral immune disturbances occur in both sepsis and acute respiratory distress syndrome (ARDS). Treatments aimed at targeting one single pro-/anti-inflammatory mediator have largely failed with no proven clinical benefits. Such failure shifts the therapeutic rationale to the nonspecific, broad-spectrum methods for modulating the over-activated inflammatory and anti-inflammatory response. Therefore, EBP techniques have become the potential weapons with high promise for removing the circulating pro-/anti-inflammatory mediators and promoting immune reconstitution. Over the years, multiple extracorporeal techniques for the critically ill animal models or patients have been developed, including hemofiltration (HF), high-volume hemofiltration (HVHF), high-cutoff hemofiltration (HCO-HF), hemo-perfusion or -adsorption (HP/HA), coupled plasma filtration adsorption (CPFA), and plasma exchange (PE). These previous studies showed that EBP therapy was feasible and safe for the critically ill animal models or patients. However, data on their efficacy (especially on the clinical benefits, such as mortality) were inconsistent. It is not now to conclude that EBP intervention can purify septic or ARDS patients with high clinical efficacy

  11. Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment.

    Science.gov (United States)

    Duzova, Ali; Bakkaloglu, Aysin

    2008-01-01

    Central nervous system (CNS) manifestations are not rare in pediatric rheumatic diseases. They may be a relatively common feature of the disease, as in systemic lupus erythematosus (SLE) and Behçet's disease. Direct CNS involvement of a systemic rheumatic disease, primary CNS vasculitis, indirect involvement secondary to hypertension, hypoxia and metabolic changes, and drug associated adverse events may all result in CNS involvement. We have reviewed the CNS manifestations of SLE, Behçet's disease, Henoch-Schönlein purpura, polyarteritis nodosa, juvenile idiopathic arthritis, juvenile ankylosing spondylitis, familial Mediterranean fever, scleroderma, sarcoidosis, Wegener's granulomatosis, Takayasu's arteritis, CINCA syndrome, Kawasaki disease, and primary CNS vasculitis; and adverse CNS effects of anti-rheumatic drugs in pediatric patients. The manifestations are diverse; ranging from headache, seizures, chorea, changes in personality, depression, memory and concentration problems, cognitive impairment, cerebrovascular accidents to coma, and death. The value of cerebrospinal fluid (CSF) examination (pleocytosis, high level of protein), auto-antibodies in serum and CSF, electroencephalography, neuroimaging with computerized tomography, magnetic resonance imaging, SPECT, PET, and angiography depends on the disease. Brain biopsy is gold standard for the diagnosis of CNS vasculitis, however it may be inconclusive in 25% of cases. A thorough knowledge of the rheumatic diseases and therapy-related adverse events is mandatory for the management of a patient with rheumatic disease and CNS involvement. Severe CNS involvement is associated with poor prognosis, and high mortality rate. High dose steroid and cyclophosphamide (oral or intravenous) are first choice drugs in the treatment; plasmapheresis, IVIG, thalidomide, and intratechal treatment may be valuable in treatment-resistant, and serious cases.

  12. Clinical, immunopathologic, and therapeutic considerations of inflammatory myopathies.

    Science.gov (United States)

    Dalakas, M C

    1992-10-01

    The inflammatory myopathies encompass a group of heterogenous muscle diseases which have in common an acquired myopathy with histological signs of endomysial inflammation. We present evidence based on recently emerged clinical, histologic, immunopathologic, demographic and therapeutic observations that these myopathies comprise three major and distinct groups: polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis (IBM). Immune-mediated mechanisms characteristic for each group appear to play a primary role in the pathogenesis of these diseases. In DM there is an intramuscular microangiopathy mediated by the C5b-9 membranolytic attack complex, leading sequentially to loss of capillaries, muscle ischemia, muscle fiber necrosis and perifascicular atrophy. In contrast, in PM and IBM the muscle fiber injury is initiated by sensitized CD8+ cytotoxic T cells that recognize MHC-I restricted muscle antigens, leading to phagocytosis and fiber necrosis. Among the viruses implicated in the cause of inflammatory myopathies, only the retroviruses, HIV, HTLV-1 and simian retroviruses, have been convincingly associated with PM. Retroviruses, therefore, appear to be the leading group of viruses capable of triggering these diseases. The treatment of inflammatory myopathies has been largely empirical. A detailed therapeutic plan based on our experience with a large number of patients is presented. Patients with bona fide PM or DM respond to steroids to some degree and for some period of time. In contrast, patients with IBM do not respond to any therapy and the disease should be suspected when a patient with presumed PM has failed treatment. Methotrexate and cyclophosphamide are disappointing. Cyclosporine and Azathioprine are commonly used but they are of uncertain benefit. Plasmapheresis is ineffective. High-dose intravenous immunoglobulin is a promising new therapeutic modality.

  13. A review of toxic epidermal necrolysis management in Japan

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    Yuri Kinoshita

    2017-01-01

    Full Text Available Toxic epidermal necrolysis (TEN is a severe adverse drug reaction characterized by necrosis of the epidermis. Its incidence is approximately 1 per million a year and average mortality rate is high at 25–50%. TEN has a flu-like prodrome, followed by atypical, targetoid erythematous or purpuric macules on the skin. These macules coalesce to form flaccid blisters that slough off as areas of epidermal necrosis. Drugs such as allopurinol, sulfonamides, and carbamazepine are the most common causes. The human leukocyte antigen (HLA-B*15:02 in Asians being administered carbamazepine and the HLA-B*58:01 antigen in patients of all ethnicities being administered allopurinol are known to be high-risk factors. Rapid diagnosis, discontinuation of the causative drug, and supportive treatment are essential for better prognosis and improvement of sequelae. Till now, systemic corticosteroids and intravenous immunoglobulins have been used as the most common active interventions; however, no gold standard has been established. In Japan, physicians follow a unique diagnostic criteria and treatment guideline to improve the diagnosis rate and streamline treatments. This may be a contributing factor for the lower mortality rate (14.3%. The efficacy of systemic corticosteroids, immunoglobulins, and plasmapheresis may have been beneficial as well. In Japan, TEN is defined as an epidermal detachment of over 10% of the body surface area (BSA, while the globally accepted definition established by Bastuji-Garin describes it as an epidermal detachment of over 30% of the BSA. In Japanese individuals, HLA-A*02:06, HLA-A*02:07, HLA-A*31:01 and HLA-B*51:01 may be linked to higher risks of TEN.

  14. Síndrome de Guillain-Barré: patogenia, diagnóstico y cuidados críticos en pediatría

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    Deybis Sánchez Miranda

    2001-06-01

    Full Text Available El síndrome de Landry-Guillain-Barré-Strohl, se caracteriza por una parálisis motora ascendente o descendente bilateral con relativa simetría, hiporreflexia o arreflexia, toma bulbar, no presencia de trastornos sensitivos objetivos, síntomas disautonómicos y la parálisis respiratoria es la complicación más grave. El factor autoinmune y el fenómeno precedente tienen valor en la patogenia. El manejo de los pacientes debe ocurrir en una Unidad de Terapia Intensiva durante la fase de progresión, para el monitoreo ventilatorio y hemodinámico. Se debe garantizar nutrición, balance hidromineral neutro, control de la vía aérea y ventilación mecánica de acuerdo con los criterios clínicos, gasométricos y electrofisiológicos. La plasmaféresis, los esteroides y la gammaglobulina intravenosa parecen ser propuestas razonables de tratamiento si se utilizan en el momento adecuado.Landry-Guillain-Barré-Strohl syndrome is characterized by a bilateral ascending or descending motor paralysis with relative simmetry, hyporeflexia or areflexia, bulbar taking, no objective sensitive disorders, disautonomic symptoms and respiratory paralysis, which is the most severe complication. The autoimmune factor and the preceeding phenomenon have value in the pathogeny. Patients should be managed in an Intensive Care Unit during the progression stage for the ventilatory and hemodynamic monitoring. Nutrition, neutral hydromineral balance, control of the airways and mechanical ventilation should be guaranteed according to the clinical, gasometric and electrophysiological criteria. Plasmapheresis, steroids and intravenous gammaglobulins appear to be reasonable suggestions for treatment if they are used at the right time.

  15. Role of basic studies in expanding the donor pool for liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Hao Chen; Ying Zhang; Lin Zhou; Hai-Yang Xie; Shu-Sen Zheng

    2008-01-01

    BACKGROUND: Liver transplantation is an effective treatment for end-stage liver disease, but a huge gap remains between the number of people who need a liver transplant and the number of organs available. In order to maximize donor organ access for adult and pediatric recipients, novel surgical and liver replacement procedures have evolved. Newer surgical techniques include split cadaveric liver transplantation and living donor liver transplantation (LDLT). With marginal and abnormal donor livers, despite tremendous advances in surgical technology, individual surgical procedure can not be completely brought into play unless effective measurements and basal studies are undertaken. DATA SOURCES: A literature search of MEDLINE and the Web of Science database using "liver transplantation" and"expanding donor pool"was conducted and research articles were reviewed. RESULTS: Therapies directed toward scavenging O2-, inhibiting nicotinamide adenine dinucleotide phosphate oxidase, and/or immuno-neutralizing tumor necrosis factor-alpha may prove useful in limiting the liver injury induced by surgical procedures such as split liver transplantation or LDLT. Improved donor organ perfusion and preservation methods, modulation of inlfammatory cytokines, energy status enhancement, microcirculation amelioration, and antioxidant usage can improve non-heart beating donor liver transplantation. Effective measures have been taken to improve the local conditions of donor cells with steatosis, including usage of fat-derived hormone and inlfammatory mediators, ischemic preconditioning, depletion of Kupffer cells, and cytokine antibody and gene therapy. Double-ifltration plasmapheresis can effectively reduce HCV viremia and prevent HCV recurrence in patient with high HCV RNA levels after LDLT. CONCLUSIONS: Shortage of grafts and poor function of marginal and abnormal donor grafts put many patients at risk of death in waiting for liver transplantation. Advances in surgical

  16. Alveolar hemorrhage and kidney disease: characteristics and therapy.

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    Fatma, Lilia Ben; El Ati, Zohra; Lamia, Rais; Aich, Dorra Ben; Madiha, Krid; Wided, Smaoui; Maiz, Hedi Ben; Beji, Somaya; Karim, Zouaghi; Moussa, Fatma Ben

    2013-07-01

    Anti-neutrophil cytoplasmic antibody-associated vasculitis and Goodpasture's glomerular basement membrane disease are the most common causes of diffuse alveolar hemorrhage, a life-threatening disease. Systemic lupus erythematosus and the antiphospholipid syndrome are also causes of alveolar hemorrhage. We retrospectively reviewed 15 cases of diffuse alveolar hemorrhage (DAH) associated with renal diseases. Diagnosis of DAH was based on the presence of bloody bronchoalveolar lavage fluid. There were three men and 12 women, with a mean age of 50.5 years (extremes: 24-74 years). Proteinuria and hematuria were observed, respectively, in 15 and 14 cases. Six patients revealed arterial hypertension. Crescentic glomerulonephritis was diagnosed with kidney biopsies in ten cases. The etiology of renal disease was microscopic polyangiitis (MPA) in seven cases, Wegener disease in four cases, systemic lupus erythematous in one case, cryoglobulinemia in one case, myeloma in one case and propyl-thiouracil-induced MPA in one case. Hemoptysis occurred in 14 cases. The mean serum level of hemoglobin was 7.1 g/dL (5.1-10 g/dL). The mean serum creatinine concentration was 7.07 mg/dL (2.4-13.7 mg/dL). Gas exchange was severely compromised, with an oxygenation index <80 mmHg in 14 patients and <60 mmHg in seven patients. Bronchoalveolar lavage was performed in 11 cases, and had positive findings for hemorrhage in all. Methylprednisolone pulses and cyclophosphamide were used in 14 patients. Plasmapheresis was performed in three cases. One patient received cycles of Dexamethasome-Melphalan. Three patients died as a result of DAH. The mortality rate in our study was 20%.

  17. Complete congenital heart block in autoimmune hepatitis (SLA-positive).

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    Knolle, P; Mayet, W; Lohse, A W; Treichel, U; Meyer zum Büschenfelde, K H; Gerken, G

    1994-08-01

    Complete congenital heart block is a serious complication of neonatal lupus erythematosus which most often occurs in children of mothers suffering from connective tissue disease. We report the occurrence of complete congenital heart block associated with autoimmune hepatitis (SLA-positive). A 32-year-old woman was treated for more than 10 years for autoimmune hepatitis (SLA-/ANA-positive) and remained in clinical remission under immunosuppressive therapy. She showed an MHC-haplotype typical for autoimmune hepatitis (A1, B8, DR3). After a normal first pregnancy, an emergency caesarean section was performed in the 32nd week of her second pregnancy because of fetal bradycardia. The child died a few hours after delivery of complete congenital AV-block. Retrospective analysis of the maternal serum showed the emergence of SS-A/Ro-antibodies prior to the second pregnancy. The maternal serum antibodies were reactive with the 52 kD SS-A/Ro-antigen, as demonstrated by immunoblot employing recombinant SSA/Ro-antigen. The occurrence of complete congenital heart block has been shown to be associated with the presence of SS-A/Ro antibodies as well as the MHC-haplotype DR3. With respect to this genetic linkage, pregnant patients with autoimmune hepatitis and the MHC-haplotype DR3 should be examined for the presence of SS-A/Ro-antibodies. They should be closely followed during pregnancy to enable early detection of the development of congenital heart block, as prevention by plasmapheresis plus dexamethasone may be possible at an early stage.

  18. Eosinophilic Pustular Folliculitis: Case Report and Clinical Analysis%嗜酸性脓疱性毛囊炎1例及临床分析

    Institute of Scientific and Technical Information of China (English)

    高敏; 林达; 李卉; 王培光; 刘盛秀; 杨森

    2012-01-01

    目的 了解中国人群嗜酸性脓疱性毛囊炎(EPF)的临床特点.方法 报道1例男性嗜酸性脓疱性毛囊炎患者并对近10年来中国学者报道的20例患者做一总结分析.结果 20例患者中,男19例,女1例;发病年龄为20~ 50岁的中青年人群;发病部位以头面部为主,其次为躯干、四肢,掌跖部位受累少见;除2例艾滋病患者外,其他患者未伴发任何其他疾病;15例患者外周血嗜酸性粒细胞升高,5例不升高,比例为3:1.结论 EPF发病以男性为主,发病部位及临床表现存在一定差异,外周血嗜酸性粒细胞可升高或不升高.%A 30 - year - old male patient, had an Epilepsy history of 13 years, having undergone one - month oral administration of antiepileptic drugs containing dilantin, had a drug - induced Hypersensitivity Syndrome ( DIHS) , with main features including diffusive scarlatiniform rash in the whole body and liver failure. After a comprehensive treatments by adrenal cortical hormone, people immunoglobulin, bilirubin plasmapheresis and liver detoxification etc, he was improved and discharged. A week later, syndromes mentioned above re -occurred after having taken twice the medications for another body twitching. With drug withdrawal and corresponding symptomatic treatment, the patient then recovered. Following - up 24 weeks found no recurrence.

  19. Cerebral involvement in a patient with Goodpasture's disease due to shortened induction therapy: a case report

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    Preul Christoph

    2009-11-01

    Full Text Available Abstract Introduction Goodpasture's disease is a rare immunological disease with formation of pathognomonic antibodies against renal and pulmonary basement membranes. Cerebral involvement has been reported in several cases in the literature, yet the pathogenetic mechanism is not entirely clear. Case presentation A 21-year-old Caucasian man with Goodpasture's disease and end-stage renal disease presented with two generalized seizures after a period of mild cognitive disturbance. Blood pressure and routine laboratory tests did not exceed the patient's usual values, and examination of cerebrospinal fluid was unremarkable. Cerebral magnetic resonance imaging (MRI revealed multiple cortical and subcortical lesions on fluid-attenuated inversion recovery sequences. Since antiglomerular basement membrane antibodies were found to be positive with high titers, plasmapheresis was started. In addition, cyclophosphamide pulse therapy was given on day 13. Encephalopathy and MRI lesions disappeared during this therapy, and antiglomerular basement membrane antibodies were significantly reduced. Previous immunosuppressive therapy was performed without corticosteroids and terminated early after 3 months. The differential diagnostic considerations were cerebral vasculitis and posterior reversible encephalopathy syndrome. Vasculitis could be seen as an extrarenal manifestation of the underlying disease. Posterior reversible encephalopathy syndrome, on the other hand, can be triggered by immunosuppressive therapy and may appear without a hypertensive crisis. Conclusion A combination of central nervous system symptoms with a positive antiglomerular basement membrane test in a patient with Goodpasture's disease should immediately be treated as an acute exacerbation of the disease with likely cross-reactivity of antibodies with the choroid plexus. In our patient, a discontinuous strategy of immunosuppressive therapy may have favored recurrence of Goodpasture's disease.

  20. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis

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    Shah S

    2015-08-01

    Full Text Available Shivani Shah, Duvuru Geetha Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD, USA Abstract: Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility. Keywords: rituximab, ANCA-associated vasculitis, GPA, MPA, induction therapy, maintenance therapy

  1. Cryofibrinogenemia: a marker of severity of cryoglobulinemic vasculitis.

    Science.gov (United States)

    Michaud, Martin; Moulis, Guillaume; Puissant, Bénédicte; Balardy, Laurent; Huart, Antoine; Gaches, Francis; Cougoul, Pierre; Arlet, Philippe; Blancher, Antoine; Pourrat, Jacques; Sailler, Laurent

    2015-08-01

    Cryofibrinogenemia is frequently associated with cryoglobulinemia. The aim of this study was to determine the characteristics associated with the presence of cryofibrinogenemia in patients with cryoglobulinemic vasculitis. This was a single-center retrospective study that included patients with cryoglobulinemic vasculitis who were tested for cryofibrinogen at a tertiary referral center between January 1, 2011 and December 31, 2012. Twenty-nine patients fulfilled the CryoVas (cryoglobulinemic vasculitis) Survey criteria for cryoglobulinemic vasculitis. Eighteen patients had a detectable cryofibrinogen (CF-positive) and 11 had no detectable cryofibrinogen (CF-negative). Median cryoglobulin levels were 89 ± 129 mg/L in the CF-positive group and 68 ± 82 mg/L in the CF-negative group (P = .32). Median cryofibrinogen level was 70 ± 174 mg/L. Clinical manifestations were similar in both groups. Cancers and hematological disorders were more frequent among CF-positive patients (39% vs 0%, P = .026). Levels of alpha-1 and alpha-2 globulinemia were higher in the CF-positive group. Cryofibrinogenemia ≥ 100 mg/L was associated with cryoglobulinemic vasculitis (odds ratio [OR] 2.86; 95% confidence interval [CI], 1.06-7.73) in cryoglobulinemic patients. Presence of cryofibrinogenemia was associated with use of corticosteroids, immunosuppressants, or plasmapheresis in cryoglobulinemic vasculitis patients (OR 22.7; 95% CI, 2.02-256.44). Our results strongly suggest that presence of cryofibrinogenemia is associated with a more severe phenotype among patients with cryoglobulinemic vasculitis. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. [Congenital atrioventricular block and maternal autoimmune diseases].

    Science.gov (United States)

    Herreman, G; Sauvaget, F; Généreau, T; Galezowski, N

    1990-01-01

    Congenital heart block is rare; it is acquired in utero, definitive and, more often than not, complete. It can be diagnosed by the appearance of fetal bradycardia around the 23rd week of gestation, during ultrasonographic monitoring of pregnancy. Heart block is usually associated with the presence of anti-Ro and/or anti-La antibodies in the mother's serum. These maternal immunological abnormalities can be isolated or associated with an autoimmune disease, usually systemic lupus erythematosus, but also Sjögren's syndrome, or more rarely still, an as yet unclassified connective tissue disease. Anti-Ro and anti-La antibodies cross the placental barrier and react with a fetal heart, leading to acute fetal myocarditis by the 17th week of gestation. When severe, it is lethal, otherwise it can result in degeneration and endocardial fibroelastosis, disrupting conduction and leading to congenital heart block. The ideal treatment would be prevention with corticosteroids. When the mother is Ro or La antibody-positive before pregnancy, elimination of these circulating antibodies can be attempted by treatment with 0.5 mg/kg body wt/d of prednisolone for 3 months. If the treatment is successful, corticotherapy can be prescribed early in the pregnancy to try to protect the fetus. However, there is not always a relationship between maternal anti-Ro antibodies and fetal heart block. If the Ro/La antibody-positive woman is already pregnant, but before her 17th week, it is possible to prescribe dexamethasone, which crosses the placenta and remains active, sometimes in association with plasmapheresis.

  3. Green tea polyphenol (--epigallocatechin-3-gallate restores Nrf2 activity and ameliorates crescentic glomerulonephritis.

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    Ting Ye

    Full Text Available Crescentic glomerulonephritis (GN is the most severe form of GN and is associated with significant morbidity and mortality despite aggressive immunotherapy with steroids, cytotoxic drugs, and plasmapheresis. We examined the therapeutic efficacy of the green tea polyphenol (--epigallocatechin-3-gallate (EGCG, 50 mg/kg BW/day x3 weeks, a potent anti-inflammatory and anti-oxidant agent, on experimental crescentic GN induced in 129/svJ mice by administration of rabbit anti-mouse glomerular basement membrane sera. Routine histology and key molecules involved in inflammatory and redox signaling were studied. EGCG treatment significantly reduced mortality, decreased proteinuria and serum creatinine, and markedly improved renal histology when compared with vehicle-treated mice. The improvements in renal function and histology were accompanied by the restoration of Nrf2 signaling (which was impaired in vehicle-treated mice as shown by increased nuclear translocation of Nrf2 and cytoplasmic glutamate cysteine ligase catalytic subunit, glutamate cysteine ligase modifier subunit, and glutathione peroxidase. EGCG-treated mice also showed reduction in p-Akt, p-JNK, p-ERK1/2 and p-P38 as well as restoration of PPARγ and SIRT1 levels. Lower dose of EGCG (25 mg/kg BW/day x2 weeks treatment also significantly decreased proteinuria and serum creatinine, and markedly improved renal histology when compared with vehicle-treated mice. Thus, our data illustrate the efficacy of EGCG in reversing the progression of crescentic GN in mice by targeting multiple signaling and inflammatory pathways as well as countering oxidative stress.

  4. Green tea polyphenol (-)-epigallocatechin-3-gallate restores Nrf2 activity and ameliorates crescentic glomerulonephritis.

    Science.gov (United States)

    Ye, Ting; Zhen, Junhui; Du, Yong; Zhou, Jason K; Peng, Ai; Vaziri, Nosratola D; Mohan, Chandra; Xu, Yan; Zhou, Xin J

    2015-01-01

    Crescentic glomerulonephritis (GN) is the most severe form of GN and is associated with significant morbidity and mortality despite aggressive immunotherapy with steroids, cytotoxic drugs, and plasmapheresis. We examined the therapeutic efficacy of the green tea polyphenol (-)-epigallocatechin-3-gallate (EGCG, 50 mg/kg BW/day x3 weeks), a potent anti-inflammatory and anti-oxidant agent, on experimental crescentic GN induced in 129/svJ mice by administration of rabbit anti-mouse glomerular basement membrane sera. Routine histology and key molecules involved in inflammatory and redox signaling were studied. EGCG treatment significantly reduced mortality, decreased proteinuria and serum creatinine, and markedly improved renal histology when compared with vehicle-treated mice. The improvements in renal function and histology were accompanied by the restoration of Nrf2 signaling (which was impaired in vehicle-treated mice) as shown by increased nuclear translocation of Nrf2 and cytoplasmic glutamate cysteine ligase catalytic subunit, glutamate cysteine ligase modifier subunit, and glutathione peroxidase. EGCG-treated mice also showed reduction in p-Akt, p-JNK, p-ERK1/2 and p-P38 as well as restoration of PPARγ and SIRT1 levels. Lower dose of EGCG (25 mg/kg BW/day x2 weeks) treatment also significantly decreased proteinuria and serum creatinine, and markedly improved renal histology when compared with vehicle-treated mice. Thus, our data illustrate the efficacy of EGCG in reversing the progression of crescentic GN in mice by targeting multiple signaling and inflammatory pathways as well as countering oxidative stress.

  5. Apheresis activity in Spain: a survey of the Spanish Apheresis Group.

    Science.gov (United States)

    Lozano, Miguel; Cid, Joan; Areal, Carlos; Romon, Iñigo; Muncunill, Josep

    2013-12-01

    The Spanish Apheresis Group is a scientific association of physicians and nurses representing most of the medical centers in the country that are involved in apheresis. The group developed a survey in order to get information about the types and number of apheresis procedures performed in Spain. We received responses from 66 centers and we were able to collect data from at least one center of each autonomous region. There were 7 centers (11%) that did not perform any kind of apheresis procedures, 26 (39%) centers performed therapeutic apheresis procedures only, 18 (27%) centers performed apheresis donations only, and 15 (23%) centers performed both types of apheresis procedures. Regarding therapeutic apheresis in adult patients, plasma exchange (34%) and stem cell collections (30%) were the two therapeutic procedures most frequently reported, followed by erythrocytapheresis (13%) and extracorporeal photochemotherapy (11%). Regarding apheresis donation, our survey showed that the most frequent was multicomponent donation (45%) followed by plasmapheresis (28%) and single plateletapheresis (21%). When analyzing the current instrumentation for performing apheresis procedures, centers used the Spectra, Optia, and Trima devices (TerumoBCT) as the most frequent ones, followed by the MCS+(Haemonetics), Amicus (Fenwal), and Fresenius devices. In conclusion, we report here the first nationwide survey performed in Spain in order to get information about apheresis activities in our country. The survey is representative of Spain because we were able to collect data from at least one center from each of the different 17 autonomous regions, and we found a wide variety of therapeutic and donation procedures, as well as instrumentation used.

  6. A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI.

    Science.gov (United States)

    Rosenbaum, A N; Anavekar, N S; Ernste, F C; Mankad, S V; Le, R J; Manocha, K K; Barsness, G W

    2015-10-01

    This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the

  7. Apheresis and intravenous immunoglobulins used in addition to conventional therapy to treat high-risk pregnant antiphospholipid antibody syndrome patients. A prospective study.

    Science.gov (United States)

    Ruffatti, Amelia; Favaro, Maria; Hoxha, Ariela; Zambon, Alessandra; Marson, Piero; Del Ross, Teresa; Calligaro, Antonia; Tonello, Marta; Nardelli, Giovanni B

    2016-06-01

    Pregnant women with triple antibody positive antiphospholipid syndrome (APS) who have had thrombosis or a history of early, severe pregnancy complications are generally considered at high risk of pregnancy loss. The objectives of this study were to investigate the efficacy and safety of a relatively new treatment protocol used in addition to conventional therapy in high-risk pregnant patients affected with primary APS. The study's two inclusion criteria were: (1) the presence of triple antiphospholipid positivity, (2) previous thrombosis and/or a history of one or more early, severe pregnancy complications. Eighteen pregnancies occurring between 2002 and 2015 in 14 APS patients, (mean age 34.8±3.6 SD) were monitored. All 14 (100%) patients had triple antiphospholipid positivity. In addition, six of them (42.8%) had a history of thrombosis, four (28.6%) had one or more previous early and severe pregnancy complications, and four (30.8%) met both clinical study criteria. The study protocol included weekly plasmapheresis or immunoadsorption and fortnightly 1g/kg intravenous immunoglobulins. Seventeen of the pregnancies (94.4%) produced live neonates, all born between the 26th and 37th weeks of gestation (mean 33.1±3.5 SD). One female (5.5%), born prematurely at 24 weeks, died of sepsis a week after birth. There were two cases (11.1%) of severe pregnancy complications. No treatment side effects were registered. Given the high live birth rate and the safety associated to it, the study protocol described here could be taken into consideration by medical teams treating high-risk APS pregnant patients.

  8. Living donor renal transplantation in patients with antiphospholipid syndrome

    Science.gov (United States)

    Choi, Ji Yoon; Jung, Joo Hee; Shin, Sung; Kim, Young Hoon; Han, Duck Jong

    2016-01-01

    Abstract Introduction: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. Clinical Findings: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin. However, he frequently experienced recurrent DVT despite of anticoagulation therapy. Before the surgery, APS was confirmed based on positive results lupus anticoagulant in serological tests. A 40-year-old man with polycystic kidney disease and a history recurrent DVT tested positive for lupus anticoagulant and anticardiolipin antibodies. Lastly, a 42-year-old woman with ESRD was diagnosed with APS 7 years ago. She also developed DVT and tested positive for lupus anticoagulant and anti-B2-glycoprotein 1. The anticoagulation protocol was as follows in all cases: Warfarin was stopped 5 days before living donor renal transplantation and intravenous heparin therapy was started. During surgery, bolus heparin injections (3000 U) were administered to prevent arterial or venous thrombosis. Heparin was substituted with warfarin on postoperative day 4. The third patient (42/F) developed clinical rejection indicated by increased serum creatinine levels and donor-specific antibodies (DSA) and received steroid pulse therapy, plasmapheresis, and rituximab. This treatment restored graft function to within the normal range. The latest graft function in all patients was maintained at normal levels in the outpatient clinic. Conclusions: Living donor renal transplantation may be successful in patients with APS following perioperative anticoagulation therapy. However, because of the high risk of

  9. Outcome of Extended Thymectomy in Myasthenia Crisis Patient.

    Science.gov (United States)

    Aftabuddin, M; Bhandari, S

    2016-07-01

    Myasthenic crisis is a life-threatening condition. We studied the demographic, frequency, causes and clinical presentation of isolated Myasthenic crisis, steps of treatment and to review our experience of extended thymectomy on patients with at least one episode myasthenic crisis. A prospective and retrospective study was conducted on patients with at least one episode of myasthenic crisis, from March 2010 to September 2014, at the Department of Cardiac Surgery, BSMMU, Dhaka, Bangladesh who were referred for thymectomy. Eighteen patients (13.6% of the total 132 patients with myasthenia gravis were admitted with single to multiple episodes of myasthenic crisis, median crisis was 2.5 episodes. Mean age of the patient was 35.5 (18-72) years with male predominance. All eighteen patients had undergone extended thymectomy after completion of 5 cycle plasmapheresis, of which 2 had experienced postoperative respiratory crisis, required invasive ventilator support for median 14 days. One patient required invasive ventilator support after third post operative day. Six patients had thymoma and 12 had thymic hyperplasia. Three patients needed Intravenous immunoglobin. Nine patients needed post operative anti acetylcholinesterase inhibitor after median 2.5 post days. Post thymectomy remission and decreases the frequency of myasthenic crisis was seen in follow up and post operative medication requirement reduced significantly as compared to the preoperative requirement. This report highlights that the patients who had extended thymectomy after episodes of myasthenia crisis are benefitted even in the histhopathology report does not confirmed thymoma. After thymectomy, there was remission of myasthenic crisis. Patients with myasthenic crisis should have judicious drug adjustments under supervision and should be treated aggressively during impending myasthenic crisis. With modern management of myasthenia gravis, early surgery with myasthenic crisis is safe with good long

  10. Pediatric Guillain-Barré syndrome: Indicators for a severe course

    Directory of Open Access Journals (Sweden)

    Muhammet Ali Varkal

    2015-01-01

    Full Text Available Objectives: This study aims to retrospectively evaluate pediatric Guillain-Barré syndrome cases in a tertiary center in Istanbul, Turkey. Materials and Methods: The data of 40 patients with Guillain-Barré syndrome who had been admitted to the Department of Pediatrics at the Istanbul University Medical Faculty between 2005 and 2011 were collected. Mann-Whitney U, Kruskal-Wallis, chi-square, and Fisher′s exact tests were used for statistical analysis. Results: Mean patient age was 5.4 ± 3.0 years; 20 out of 40 patients (50% were female and 20 (50% were male. Preceding infection was detected in 32 cases (80%. Six patients had speech impairment. Out of eight patients with respiratory distress (20%, five required respiratory support (12.5% of which three of them had speech impairment as well. According to nerve conduction studies, 21 patients (52.5% had acute inflammatory demyelinating polyradiculoneuropathy, 14 (35% had acute motor axonal neuropathy, and five (12.5% had acute motor-sensory axonal neuropathy. Thirty-three patients (82.5% received intravenous immunglobulin, 3 (7.5% underwent plasmapheresis and 4 (10% received both. Time until recovery (P = 0.022 and time until aided (P = 0.036 and unaided (P = 0.027 walking were longer in patients with acute gastrointestinal infection than in those with upper respiratory tract infection (P < 0.05. Time until response to treatment (P = 0.001, time until aided (P = 0.001 and unaided (P = 0.002 walking, and time until complete recovery (P = 0.002 were longer in acute motor axonal neuropathy cases as compared to acute inflammatory demyelinating polyradiculoneuropathy cases. Conclusion: Recovery was longer with acute gastrointestinal infection and acute motor axonal neuropathy. Speech impairment could be a clinical clue for the need of mechanical ventilation.

  11. Late antibody-mediated rejection after heart transplantation: Mortality, graft function, and fulminant cardiac allograft vasculopathy.

    Science.gov (United States)

    Coutance, Guillaume; Ouldamar, Salima; Rouvier, Philippe; Saheb, Samir; Suberbielle, Caroline; Bréchot, Nicolas; Hariri, Sarah; Lebreton, Guillaume; Leprince, Pascal; Varnous, Shaida

    2015-08-01

    Late antibody-mediated rejection (AMR) after heart transplantation is suspected to be associated with a poor short-term prognosis. A retrospective single-center observational study was performed. Late AMR was defined as AMR occurring at least 1 year after heart transplantation. The study included all consecutive patients with proven and treated late acute AMR at the authors' institution between November 2006 and February 2013. The aim was to analyze the prognosis after late AMR, including mortality, recurrence of AMR, left ventricular ejection fraction, and cardiac allograft vasculopathy (CAV). Selected endomyocardial biopsy specimens obtained before AMR were also blindly reviewed to identify early histologic signs of AMR. The study included 20 patients treated for late AMR. Despite aggressive immunosuppressive therapies (100% of patients received intravenous methylprednisolone, 90% received intravenous immunoglobulin [IVIg],85% received plasmapheresis, 45% received rituximab), the prognosis remained poor. Survival after late AMR was 80% at 1 month, 60% at 3 months, and 50% at 1 year. All early deaths (<3 months, n = 8) were directly attributable to graft dysfunction or to complication of the intense immunosuppressive regimen. Among survivors at 3 months (n = 12), histologic persistence or recurrence of AMR, persistent left ventricular dysfunction, and fulminant CAV were common (33%, 33%, and 17% of patients). Microvascular inflammation was detected in at least 1 biopsy specimen obtained before AMR in 13 patients (65%). Prognosis after late AMR is poor despite aggressive immunosuppressive therapies. Fulminant CAV is a common condition in these patients. Microvascular inflammation is frequent in endomyocardial biopsy specimens before manifestation of symptomatic AMR. Copyright © 2015 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

  12. Managing refractory cryoglobulinemic vasculitis: challenges and solutions

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    Ostojic P

    2017-05-01

    Full Text Available Predrag Ostojic, Ivan R Jeremic Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia Abstract: Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III, vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation – cryoglobulinemic vasculitis (CryoVas. Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers, weakness, peripheral neuropathy, Raynaud’s phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis. In patients with mixed cryoglobulinemia, prevalence of anti-hepatitis C virus (HCV antibodies and/or HCV RNA, detected by polymerase chain reaction (PCR, is reported to be up to 90%, indicating a significant role of HCV in the development of this condition. The goals of therapy for mixed cryoglobulinemia include immunoglobulin level reduction and antigen elimination. CryoVas not associated with HCV infection should be treated according to treatment recommendations for small-vessel vasculitides. CryoVas associated with chronic HCV infection should be treated with antivirals along with immunosuppressive drugs, with or without plasmapheresis, depending on disease severity and organ involvement. Patients who do not respond to first-line therapy may achieve remission when treatment with rituximab is started as second-line therapy. In HCV-related CryoVas, antiviral therapy should be given along with rituximab in order to achieve complete or partial remission. Moreover, rituximab has proven to be a glucocorticoid-sparing medication. Other potential therapies for refractory CryoVas include mycophenolate mofetil and belimumab

  13. L-Tryptophan depletion bioreactor, a possible cancer therapy

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    Rolf Bambauer

    2015-04-01

    Full Text Available The cancer therapeutic strategies knownto date are not adequate for all cancer patients. Most of them are followed by a high rate of side effects and complications. The L-tryptophan depletion bioreactor is described as a possible new method of cancer therapy. L-tryptophan is an essential amino acid which has been recognized as an important cancer nutrient and its removal can lead to destruction of the tumour. Normal human cells or tumor cells cannot synthesize L-tryptophan and therefore tumor resistance is unlikely to develop. L-tryptophan is also a constituent for different bio-molecules such as Serotonin, Melatonin, and is needed for other synthesis processes in the cell growth. L-tryptophan degrading enzymes with 3 iso-enzymes called tryptophan side chain oxydase (TSO I, II, III were isolated. The 3 iso-enzymes can be differentiated by tryptic digestion. They have different molecular weights with different effectivenesses. All the TSO enzymes have heme that can catalyze essentially similar reactions involving L-tryptophan as a substrate. The most effective TSO is the type TSO III. A column which contained TSO as a bioreactor was integrated in a plasmapheresis unit and tested it in different animals. In sheep and rabbits L-tryptophan depletion in plasma was shown at 95% and 100% rates respectively by a single pass through the bioreactor. The results in immune supprimized rats with tumors were impressive, too. In 20 different tumor cell lines there were different efficacies. Brest cancer and medulloblastoma showed the greatest efficacy of L-tryptophan degrading. The gene technology of TSO production from Pseudomonas is associated with formation of endotoxins. This disadvantage can be prevented by different washing procedures or by using fungal sources for the TSO production. TSO III is developed to treat cancer diseases successfully, and has low side effects. A combination of L-tryptophan depletion with all available cancer therapies is

  14. Exosome removal as a therapeutic adjuvant in cancer.

    Science.gov (United States)

    Marleau, Annette M; Chen, Chien-Shing; Joyce, James A; Tullis, Richard H

    2012-06-27

    Exosome secretion is a notable feature of malignancy owing to the roles of these nanoparticles in cancer growth, immune suppression, tumor angiogenesis and therapeutic resistance. Exosomes are 30-100 nm membrane vesicles released by many cells types during normal physiological processes. Tumors aberrantly secrete large quantities of exosomes that transport oncoproteins and immune suppressive molecules to support tumor growth and metastasis. The role of exosomes in intercellular signaling is exemplified by human epidermal growth factor receptor type 2 (HER2) over-expressing breast cancer, where exosomes with the HER2 oncoprotein stimulate tumor growth and interfere with the activity of the therapeutic antibody Herceptin®. Since numerous observations from experimental model systems point toward an important clinical impact of exosomes in cancer, several pharmacological strategies have been proposed for targeting their malignant activities. We also propose a novel device strategy involving extracorporeal hemofiltration of exosomes from the entire circulatory system using an affinity plasmapheresis platform known as the Aethlon ADAPT™ (adaptive dialysis-like affinity platform technology) system, which would overcome the risks of toxicity and drug interactions posed by pharmacological approaches. This technology allows affinity agents, including exosome-binding lectins and antibodies, to be immobilized in the outer-capillary space of plasma filtration membranes that integrate into existing kidney dialysis systems. Device therapies that evolve from this platform allow rapid extracorporeal capture and selective retention of target particles exosomes. This review discusses the possible therapeutic approaches for targeting immune suppressive exosomes in cancer patients, and the anticipated significance of these strategies for reversing immune dysfunction and improving responses to standard of care treatments.

  15. Hemocompatible polyethersulfone/polyurethane composite membrane for high-performance antifouling and antithrombotic dialyzer.

    Science.gov (United States)

    Yin, Zehua; Cheng, Chong; Qin, Hui; Nie, Chuanxiong; He, Chao; Zhao, Changsheng

    2015-01-01

    Researches on blood purification membranes are fuelled by diverse clinical needs, such as hemodialysis, hemodiafiltration, hemofiltration, plasmapheresis, and plasma collection. To approach high-performance dialyzer, the integrated antifouling and antithrombotic properties are highly necessary for the design/modification of advanced artificial membranes. In this study, we propose and demonstrate that the physical blend of triblock polyurethane (PU) and polyethersulfone (PES) may advance the performance of hemodialysis membranes with greatly enhanced blood compatibility. It was found that the triblock PU could be blended with PES at high ratio owing to their excellent miscibility. The surfaces of the PES/PU composite membranes were characterized using attenuated total reflectance-Fourier transform infrared spectroscopy, X-ray photoelectron spectroscopy, water contact angle measurement, and surface ζ-potentials. The results indicated that the membrane surfaces were assembled with hydrophilic segregation layer owing to the migration of amphiphilic PU segments during membrane preparation, which might confer the composite membranes with superior hemocompatibility. The cross-section scanning electron microscopy images of the composite membranes exhibited structure transformation from finger-like structure to sponge-like structure, which indicated that the composite membrane had tunable porosity and permeability. The further ultrafiltration experiments indicated that the composite membranes showed increased permeability and excellent antifouling ability. The blood compatibility observation indicated that PES/PU composite membranes owned decreased protein adsorption, suppressed platelet adhesion, and prolonged plasma recalcification time. These results indicated that the PES/PU composite membranes exhibited enhanced antifouling and antithrombotic properties than the pristine PES membrane. The strategy may forward the fabrication of blood compatible composite membranes for

  16. Refractory thrombotic thrombocytopenic purpura following acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Ebisa Bekele; Bethel Shiferaw; Alexandra Sokolova; Arpan Shah; Phillip Saunders; Alida Podrumar; Javed Iqbal

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13) activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6%after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  17. Refractor y thrombotic thrombocytopenic pur pura following acute pancreatitis

    Directory of Open Access Journals (Sweden)

    Ebisa Bekele

    2016-09-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13 activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6% after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  18. Anti-N-methyl-D-aspartate-receptor encephalitis: diagnosis, optimal management, and challenges

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    Mann AP

    2014-07-01

    Full Text Available Andrea P Mann,1 Elena Grebenciucova,2 Rimas V Lukas21Department of Psychiatry and Behavioral Neuroscience, 2Department of Neurology, University of Chicago, Chicago, IL, USAObjective: Anti-N-methyl-D-aspartate-receptor (NMDA-R encephalitis is a new autoimmune disorder, often paraneoplastic in nature, presenting with complex neuropsychiatric symptoms. Diagnosed serologically, this disorder is often responsive to immunosuppressant treatment. The objective of this review is to educate clinicians on the challenges of diagnosis and management of this disorder.Materials and methods: A review of the relevant literature on clinical presentation, pathophysiology, and recommended management was conducted using a PubMed search. Examination of the results identified articles published between 2007 and 2014.Results: The literature highlights the importance of recognizing early common signs and symptoms, which include hallucinations, seizures, altered mental status, and movement disorders, often in the absence of fever. Although the presence of blood and/or cerebrospinal fluid autoantibodies confirms diagnosis, approximately 15% of patients have only positive cerebrospinal fluid titers. Antibody detection should prompt a search for an underlying teratoma or other underlying neoplasm and the initiation of first-line immunosuppressant therapy: intravenous methylprednisolone, intravenous immunoglobulin, or plasmapheresis, or a combination thereof. Second-line treatment with rituximab or cyclophosphamide should be implemented if no improvement is noted after 10 days. Complications can include behavioral problems (eg, aggression and insomnia, hypoventilation, catatonia, and autonomic instability. Those patients who can be managed outside an intensive care unit and whose tumors are identified and removed typically have better rates of remission and functional outcomes.Conclusion: There is an increasing need for clinicians of different specialties, including

  19. Two cases of sporadic late onset nemaline myopathy effectively treated with immunotherapy.

    Science.gov (United States)

    Mizuno, Yukio; Mori-Yoshimura, Madoka; Okamoto, Tomoko; Oya, Yasushi; Nishino, Ichizo; Murata, Miho

    2016-09-29

    Sporadic late onset nemaline myopathy (SLONM) associated with monoclonal gammopathy of undetermined significance (MGUS) is an adult onset myopathy with poor clinical outcomes, requiring high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM-SCT). Here we report two cases of SLONM associated with MGUS in which improvements were achieved only with immunotherapy. A 39-year-old woman had a two-year history of dropped head syndrome and progressive proximal weakness. On admission, she was able to walk with assistance and had lordosis with camptocormia. Combination therapy with plasmapheresis and intravenous immunoglobulin in addition to intravenous methylprednisolone pulse therapy ameliorated camptocormia and proximal weakness after one year. A 51-year-old man had difficulty in raising his arms and required walking assistance prior to visiting our hospital. He had proximal weakness and atrophy, winged scapulae, and gait disturbance. After combination immunotherapy, no progression was observed for 13 years. In both cases, patients did not desire to undergo HDM-SCT, and IgG kappa monoclonal protein was positive, of which the levels were normalized after immunotherapy. Combination immunotherapy can be a possible alternative to HDM-SCT in patients with SLONM. Both patients showed myogenic changes with abundant fibrillation, and needle EMG revealed positive sharp waves. Case 1 showed high signal intensities in MRI STIR/T2WI in muscles showing weakness. These findings are commonly observed in patients with myositis, suggesting that, without muscle biopsy, SLONM may be misdiagnosed as myositis. Muscle biopsy revealed scattered fibers with nemaline bodies without type 2B deficiency, which are important pathological findings that differentiate SLONM from congenital nemaline myopathy.

  20. Alveolar hemorrhage and kidney disease: Characteristics and therapy

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    Lilia Ben Fatma

    2013-01-01

    Full Text Available Anti-neutrophil cytoplasmic antibody-associated vasculitis and Goodpasture′s glomerular basement membrane disease are the most common causes of diffuse alveolar hemorrhage, a life-threatening disease. Systemic lupus erythematosus and the antiphospholipid syndrome are also causes of alveolar hemorrhage. We retrospectively reviewed 15 cases of diffuse alveolar hemorrhage (DAH associated with renal diseases. Diagnosis of DAH was based on the presence of bloody bronchoalveolar lavage fluid. There were three men and 12 women, with a mean age of 50.5 years (extremes: 24-74 years. Proteinuria and hematuria were observed, respectively, in 15 and 14 cases. Six patients revealed arterial hypertension. Crescentic glomerulonephritis was diagnosed with kidney biopsies in ten cases. The etiology of renal disease was microscopic polyangiitis (MPA in seven cases, Wegener disease in four cases, systemic lupus erythematous in one case, cryoglobulinemia in one case, myeloma in one case and propyl-thiouracil-induced MPA in one case. Hemoptysis occurred in 14 cases. The mean serum level of hemoglobin was 7.1 g/dL (5.1-10 g/dL. The mean serum creatinine concentration was 7.07 mg/dL (2.4-13.7 mg/dL. Gas exchange was severely compromised, with an oxygenation index <80 mmHg in 14 patients and <60 mmHg in seven patients. Bronchoalveolar lavage was performed in 11 cases, and had positive findings for hemorrhage in all. Methylprednisolone pulses and cyclophosphamide were used in 14 patients. Plasmapheresis was performed in three cases. One patient received cycles of Dexamethasome-Melphalan. Three patients died as a result of DAH. The mortality rate in our study was 20%.

  1. Síndrome pulmón-riñón Pulmonary-renal syndrome

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    Jorge A. Risso

    2009-12-01

    Full Text Available El síndrome pulmón-riñón se define como una combinación de hemorragia alveolar difusa y glomerulonefritis. La coexistencia de estas dos afecciones clínicas se produce por enfermedades con distintos mecanismos patogénicos. Las vasculitis sistémicas primarias y el síndrome de Goodpasture son las etiologías más frecuentes. El lupus eritematoso sistémico, otras colagenopatías, las vasculitis con anticuerpos anticitoplasma de los neutrófilos negativos y las secundarias a drogas son causas mucho menos comunes. El diagnóstico temprano basado en criterios clínicos, radiológicos, de laboratorio e histológicos, permite iniciar el tratamiento disminuyendo su elevada morbi-mortalidad. La terapéutica se basa en altas dosis de corticoides, inmunosupresores, inhibidores del factor de necrosis tumoral y plasmaféresis.The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbility-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  2. Kinetic Targeting of pegylated liposomal Doxorubicin: a new Approach to Reduce Toxicity during Chemotherapy (CARL-trial

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    Jansen Martin

    2011-08-01

    Full Text Available Abstract Background The therapeutic success of chemotherapeutic agents is often limited by severe adverse effects. To reduce toxicity of these drugs, nanoscale particle-based drug delivery systems (DDS are used. DDS accumulate to some extent in tumor tissues, but only a very small portion of a given dose reaches this target. Accumulation of DDS in tumor tissues is supposed to be much faster than in certain other tissues in which side effects occur ("Kinetic Targeting". Once saturation in tumor tissue is achieved, most of the administered DDS still circulate in the plasma. The extracorporeal elimination of these circulating nanoparticles would probably reduce toxicity. Methods For the CARL-trial (Controlled Application and Removal of Liposomal chemotherapeutics, pegylated liposomal doxorubicin (PLD was used as chemotherapeutic agent and double filtration plasmapheresis (DFPP was performed for extracorporeal elimination of liposomes. PLD was given as 40 mg/m2 every 3 weeks in combination with vinorelbine 2 × 25 mg/m2 (neoadjuvant treatment of breast cancer, 12 patients, or as 40 mg/m2 every 4 weeks (recurrent ovarian cancer, 3 patients. Primary endpoints were the efficiency and safety profile of DFPP, and secondary endpoints were side effects and tumor response. Results DFPP eliminated ~62% of circulating PLD, corresponding to ~45% of the total dose (n = 57 cycles. AUC of doxorubicin was reduced by 50%. No leakage of doxorubicin was detected during elimination, and no relevant DFPP-related side effects occurred. Reduction in tumor size > 30% occurred in 10/12 (neoadjuvant and in 1/3 patients (recurrent. Only five grade 2 events and one grade 3 event (mucositis, neutropenia or leucopenia and a single palmar-plantar erythrodysesthesia grade 2 were reported. Conclusion Extracorporeal elimination of PLD by DFPP is safe and efficient. CARL can diminish the main dose-limiting side effects of PLD, and probably many different DDS alike. Trial

  3. Atypical hemolytic uremic syndrome diagnosed four years after ABO-incompatible kidney transplantation.

    Science.gov (United States)

    Kawaguchi, Keiko; Kawanishi, Kunio; Sato, Masayo; Itabashi, Mitsuyo; Fujii, Akiko; Kanetsuna, Yukiko; Huchinoue, Shouhei; Ohashi, Ryuji; Koike, Junki; Honda, Kazuho; Nagashima, Yoji; Nitta, Kosaku

    2015-07-01

    Atypical hemolytic uremic syndrome (aHUS) in allograft kidney transplantation is caused by various factors including rejection, infection, and immunosuppressive drugs. We present a case of a 32 year old woman with aHUS four years after an ABO-incompatible kidney transplantation from a living relative. The primary cause of end-stage renal disease was unknown; however, IgA nephropathy (IgAN) was suspected from her clinical course. She underwent pre-emptive kidney transplantation from her 60 year old mother. The allograft preserved good renal function [serum creatinine (sCr) level 110-130 μmol/L] until a sudden attack of abdominal pain four years after transplant, with acute renal failure (sCr level, 385.3 μmol/L), decreasing platelet count, and hemolytic anemia with schizocytes. On allograft biopsy, there was thrombotic microangiopathy in the glomeruli, with a cellular crescent formation and mesangial IgA and C3 deposition. Microvascular inflammation, such as glomerulitis, peritubular capillaritis, and arteriole endarteritis were also detected. A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) did not decrease and Shiga toxin was not detected. Donor-specific antibodies or autoantibodies, including anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane (anti-GBM) antibody, were negative. The patient was diagnosed with aHUS and received three sessions of plasmapheresis and methylprednisolone pulse therapy, followed by oral methylprednisolone (0.25-0.5 mg/kg) instead of tacrolimus. She temporarily required hemodialysis (sCr level, 658.3 μmol/L). Thereafter, her sCr level improved to 284.5 μmol/L without dialysis therapy. This case is clinically considered as aHUS after kidney transplantation, associated with various factors, including rejection, glomerulonephritis, and toxicity from drugs such as tacrolimus.

  4. 儿童抗N-甲基-D-天冬氨酸受体脑炎%Anti-N-methyl-D-aspartate receptor encephalitis in children

    Institute of Scientific and Technical Information of China (English)

    陈晨

    2013-01-01

    抗N-甲基-D-天冬氨酸受体(N-methyl-D-asparate receptor,NMDAR)脑炎是免疫介导的中枢神经系统综合征,主要临床表现有精神症状、运动障碍、抽搐发作等,常被误诊为病毒性脑炎,与肿瘤尤其卵巢畸胎瘤关系密切;儿童患病率高,抗NMDAR抗体是其较特异的检查;治疗主要为免疫抑制,一线治疗为激素、免疫球蛋白、血浆置换,二线治疗主要包括生物制剂、细胞毒药物;早期诊断及治疗对神经系统的恢复至关重要,总体预后良好.%Anti-N-methyl-D-aspartate receptor encephalitis is an autoimmune syndrome of central nervous system.The main clinical manifestations include psychiatric symptoms,movement disorders and seizures.It is often misdiagnosed as viral encephalitis.Tumors,especially the ovarian teratoma,play an important role in the disease.The prevalence in children is beyond imagination.Anti-NMDAR antibodies are specific diagnostic tests.Immune suppression is the main treatment.The first-line treatments include corticosteroids,intravenous immunoglobulin,and plasmapheresis.The second-line treatments include biological agents and cytotoxic drugs.Early diagnosis and treatment are essential for neurologic recovery.Overall prognosis is good.

  5. 移植肾破裂肾包膜切开止血法%Treatment of renal allograft rupture: renal capsulotomy and hemostatic satin hemostasis

    Institute of Scientific and Technical Information of China (English)

    陈江华; 王逸民; 寿张飞; 吴建永; 朱琮

    1998-01-01

    Renal allograft spontaneous rupture is a dangerous complication after cadaveric kidney transplantation at early stage. Six cases of critical renal allograft rupture with acute renal failure within 8-12 postoperative days were subjected to multipoint renal capsulotomy and hemostatic satin-sticky glue hemostasis in combination with double filtration plasmapheresis and antithymocyte globulin. The hemostatic effective rate was 100%. Renal allograft function in 4 patients were recovered. The remaining 2 cases had to undergo the removal of renal allograft due to other causes. It was considered that multipoint renal capsulotomy and hemostatic satin-sticky glue hemostasis graft was a safe, reliable, and simple procedure for the treatment of renal allograft rupture.%移植肾自发性破裂是同种异体肾移植术后早期的一个严重并发症,采用肾包膜多处切开结合止血绫-粘涂胶止血法保肾,并行双滤过法血浆分离术(DFPP)和应用抗胸腺细胞球蛋白(ATG)治疗6例严重移植肾破裂患者,止血效果达到100%,4例肾脏得以保存,2例切除移植肾.认为肾包膜多处切开结合止血绫-粘涂胶止血术是治疗严重移植肾破裂的一个安全、可靠和简便的止血保肾法.

  6. 合并Goodpasture's综合征孕妇所产新生儿伴发肺大疱一例并文献复习%One neonate with bullae of lung whose mother diagnosed with Goodpasture's syndrome during pregnancy:case report and review of the literatures

    Institute of Scientific and Technical Information of China (English)

    张乐嘉; 丁国芳; 王长燕; 沈菁; 李正红

    2015-01-01

    Objective To summarize the clinical features and possible impacts of Goodpasture's syndrome in pregnancy on the pulmonary and kidney of the newborn and the mothers. Methods One patient diagnosed Goodpasture's syndrome in pregnancy hospitalized in Peking Union Medical College Hospital on August 23 in 2011 delivered a neonate with bullae of lung. And literatures including 8 cases of pregnancy complicated by Goodpasture's syndrome worldwide through Medline were reviewed. Results (1) Case report:one 31-year-old women presented with acute renal failure at 30+6 weeks of gestation and delivered a male infant with birth weight 1 900 g by caesarean section at 31+1 weeks of gestation. Diagnosis was confirmed as Goodpasture's syndrome with anti-glomerular basement membrane(GBM) antibodies in serum and renal biopsy after delivery. Then she was treated with methylprednisolone, cyclophosphamide, plasmapheresis and dialysis. The neonate showed the lung bullae in the right middle lobe and bilateral intraventricular hemorrhage but renal function was transient normal with anti-GBM as 113.1 EU/ml. The baby was treated by glucocorticoid for two months and clinical symptoms were improved. Anti-GBM antibodies and chest CT showed normal. After been followed up for two years, the baby was normal. (2) Literatures review:the main manifestations of Goodpasture's syndrome in pregnancy were malignant hypertension and renal failure but respiratory symptoms were not obvious. Treated with plasmapheresis, hematodialysis and glucocorticoid maybe have good effects. Most cases had premature delivery. Neonatal anti-GBM antibodies coming from mothers could result to cerebral, renal and pulmonary injury which could be treated by glucocorticoid. Conclusions The Clinical features of pregnancy complicating the Goodpasture's syndrome are malignant hypertension and renal failure. Diagnosis was depended on positive anti-GBM antibodies and renal pathological changes and treatment were depended on

  7. HIGH AND LOW DOSE IVIG THERAPY IN GUILLAIN-BARRE SYNDROME CHILDREN: A COMPARISON

    Directory of Open Access Journals (Sweden)

    P. KARIMZADEH

    2006-06-01

    Full Text Available Introduction:Acute inflammatory demyelinating peripheral neuropathy (GuillainBarre-Syndrome is by far the most common cause of immune-medicatedperipheral nervous system disease in children; with the near disappearanceof poliomyelitis, GBS is responsible for the great majority of cases ofacute flaccid paralysis. So far, in several controlled studies, corticosteroids,plasmapheresis and IVIG have been utilized in pediatric patients, afflictedwith GBS. Regarding IVIG therapy, two methods have been used; thehigh dose (1 gr/kg/day for 2 days, and the low dose (400mg/kg/day for5 days. Review of literature shows that a faster rate of recovery can beaccomplished in patients who receive total dose of IVIG in 2 days ascompared to the dose being given over 5 days.Materials & Methods: In this study we have compared these two types of treatment in aninvestigation, conducted in the Mofid Children Hospital on pediatricpatients who had sudden onset of acute flaccid paralysis, and werediagnosed as having GBS. Based on histories, physical examination andelectrodiagnosis, subjects were divided in two groups, the high doseIVIG treatment, 1gr/kg/day for 2 days (experimental group, and the lowdose IVIG treatment, 400 mg/kg/day for 5 days (control group. Statisticalanalyses were then carried out using the appropriate software.Results: Result of this study showed a faster rate of recovery for patients in thehigh dose IVIG group; in this group duration of weakness of limbs wasshorter and returning of DTR was faster than in controls. In fact, in thistype of treatment, the relationship between high dose IVIG therapy anddrug side effects was not significant.Conclusion: Base upon the finding in the present study, we conclude that the highdose IVIG therapy is superior to low dose, in view of faster duration ofrecovery and shorter hospital stay.Also we may infer that shorter hospital stay could be a factor in reducingof more nasocomial infection.In conclusion, we suggest using

  8. Clinical and Laboratory Features, Treatment and Prognosis in Children with Guillian-Barre Syndrome

    Directory of Open Access Journals (Sweden)

    N. A. Suponeva

    2015-01-01

    Full Text Available A retrospective study of 42 cases of acute flaccid paralysis (AFP in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014, was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS. GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP (77%, 5 with acute motor axonal neuropathy (AMAN (16% and 2 with аcute motor-sensory axonal neuropathy (AMSAN in a total of cases (7%. Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51% compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per

  9. HIGH AND LOW DOSE IVIG THERAPY IN GUILLAIN-BARRE SYNDROME CHILDREN: A COMPARISON

    Directory of Open Access Journals (Sweden)

    P. KARIMZADEH MD

    2009-05-01

    Full Text Available Objective:Acute inflammatory demyelinating peripheral neuropathy (Guillain-Barre-Syndrome is by far the most common cause of immune-medicated peripheral nervous system disease in children; with the near disappearance of poliomyelitis, GBS is responsible for the great majority of cases of acute flaccid paralysis. So far, in several controlled studies, corticosteroids, plasmapheresis and IVIG have been utilized in pediatric patients, afflicted with GBS. Regarding IVIG therapy, two methods have been used; the high dose (1 gr/kg/day for 2 days, and the low dose (400mg/kg/day for 5 days. Review of literature shows that a faster rate of recovery can be accomplished in patients who receive total dose of IVIG in 2 days as compared to the dose being given over 5 days.Materials & Methods:In this study we have compared these two types of treatment in an investigation, conducted in the Mofid Children Hospital on pediatric patients who had sudden onset of acute flaccid  paralysis, and were diagnosed as having GBS. Based on histories, physical examination and electrodiagnosis, subjects were divided in two groups, the high dose IVIG treatment, 1gr/kg/day for 2 days (experimental group, and the low dose IVIG treatment, 400 mg/kg/day for 5 days (control group. Statistical analyses were then carried out using the appropriate software.Results:Result of this study showed a faster rate of recovery for patients in the high dose IVIG group; in this group duration of weakness of limbs was shorter and returning of DTR was faster than in controls. In fact, in this type of treatment, the relationship between high dose IVIG therapy and drug side effects was not significant.Conclusion:Base upon the finding in the present study, we conclude that the high dose IVIG therapy is superior to low dose, in view of faster duration of recovery and shorter hospital stay. Also we may infer that shorter hospital stay could be a factor in reducing of more nasocomial infection. In

  10. Guillain-Barré syndrome in the elderly: clinical, electrophysiological, therapeutic and outcome features Síndrome de Guillain-Barré no idoso: aspectos clínico-eletrofisiológicos, terapêutico e prognóstico

    Directory of Open Access Journals (Sweden)

    Marcondes C. França Jr

    2005-09-01

    Full Text Available There are few papers devoted to geriatric Guillain-Barré (GBS and many related issues remain unanswered. OBJECTIVE: To describe clinical, electrophysiological and therapeutic features in this age. METHOD: Clinico-epidemiological data and therapy of GBS patients older than 60 years were reviewed. Hughes scores were used to quantify neurological deficit and define outcome. RESULTS: Among 18 patients (mean age 64.8 years, 9 had evident prodrome and 80% noticed initially sensory-motor deficit. Demyelinating GBS was found in 8 and axonal in 6 subjects. There was one Miller-Fisher and 3 unclassified cases. Plasmapheresis (PFX was single therapy in 12 patients and intravenous immunoglobulin (IVIg in 2. Disability scores just before therapy were similar in both groups, so as short and long term outcome. CONCLUSION: Axonal GBS seems to be more frequent in the elderly and this may have prognostic implications. PFX and IVIg were suitable options, but complications were noticed with PFX. Prospective studies are needed to better understand and manage GBS in the elderly.Publicações sobre a síndrome de Guillain-Barré (SGB no idoso são escassas e várias questões sobre o tema estão abertas. OBJETIVO: Descrever aspectos clínico-eletrofisiológicos, terapêuticos e prognóstico no idoso. MÉTODO:Revisamos os prontuários de pacientes acima de 60 anos com SGB. A escala de Hughes foi usada para quantificar os déficits iniciais e finais. RESULTADOS: No total de 18 pacientes (média de idade 64,8 anos, 50% tiveram pródromo e 80% tiveram déficit sensitivo-motor no início. SGB desmielinizante foi encontrada em 8 pacientes, axonal em 6 e uma síndrome de Miller-Fisher. Três casos não puderam ser classificados. Plasmaférese (PFX foi empregada isoladamente em 12 pacientes e imunoglobulina endovenosa (IVIg em 2. A disfunção inicial nos dois grupos tratados era semelhante, assim como a evolução a curto e longo prazo. CONCLUSÃO: A forma axonal da SGB

  11. [The strategy for preventing HIV/AIDS transmission via the blood and its derivatives in Mexico].

    Science.gov (United States)

    Sepúlveda-Amor, J; del Río-Zolezzi, A; Valdespino-Gómez, J L; García-García, M de L; Velázquez-Velázquez, L; Volkow, P

    1995-01-01

    This study presents blood-associated AIDS epidemic trends in Mexico, including cases due to blood transfusions, cases in professional blood donors and hemophiliacs. We present also an overview of preventive measures--both legal and educative--undertaken to prevent this type of transmission and its effects on the epidemic. The first blood-associated AIDS cases in Mexico were reported in 1985, since then and up to July 1, 1994 a total of 1,728 adult cases and 116 pediatric cases have been reported (12.3% and 25% of the total cases, respectively). As in many other parts in the world, in Mexico women were markedly affected by this form of transmission; the women to men morbidity ratio is 1.35. Another group particularly affected by AIDS in Mexico are professional blood donors, who were infected because of improper management and recycling of blood transfusion centers bank materials such as plasmapheresis sets, in some blood transfusion centers in our country. Blood screening is mandatory for all blood donors in Mexico since May, 1986. A year later blood commercialization was banned because of the extremely high HIV infection prevalences found in some professional blood donors (7.2%). Since that time a whole set of preventive measures has been implemented in our country, including blood quality and safety control as well as educative interventions. Results of these measures began to become evident by the end of 1991, when newly reported blood associated AIDS cases started to decrease, as opposed to their continuous growth seen in previous years. Up to July 1, 1994 we estimate that a total of 2,750 AIDS cases have been prevented through these measures, recovering an average of 36 years of potential life for each of them. Although blood transmission preventive measures have rendered significant achievements, we still have to face some very complex challenges such as potential ruralization of the epidemic and its impact on HIV infection prevalences among potential blood

  12. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?

    Science.gov (United States)

    Prendiville, Julie S; Tucker, Lori B; Cabral, David A; Crawford, Richard I

    2004-01-01

    The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic

  13. Delayed hyperacute rejection in a patient who developed clostridium difficile infection after ABO-incompatible kidney transplantation

    Directory of Open Access Journals (Sweden)

    Gerald S Lipshutz

    2010-11-01

    Full Text Available Gerald S Lipshutz1, Elaine F Reed2, Phuong-Chi Pham3, Jeffrey M Miller4, Jennifer S Singer5, Gabriel M Danovitch6, Alan H Wilkinson6, Dean W Wallace7, Suzanne McGuire6, Phuong-Truc Pham8, Phuong-Thu Pham61Department of Surgery, Kidney and Pancreas Transplant Program, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 2Department of Pathology and Laboratory Medicine-Immunogenetics, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 3Department of Medicine, Nephrology Division, UCLA-Olive View Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 4Department of Medicine, Hematology Oncology Division, UCLA-Olive View Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 5Department of Surgery and Urology, Kidney and Pancreas Transplant Program, 6Department of Medicine, Nephrology Division, Kidney and Pancreas Transplant Program, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 7Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; 8Department of Science, Penn State University, Worthington-Scranton, Dunmore, PA, USAAbstract: Over the past decade ABO incompatible transplantation has emerged as an important potential source for increasing living kidney transplantation in selected transplant centers. Early reports suggest that patients who have elevated serum anti-blood group antibody titers (anti-A/B before transplantation and a rebound antibody production after antibody removal may be at high immunological risk. With currently available immune modulation protocols and immunosuppressive therapy, excellent short- and long-term patient and graft survival rates have been achieved even in those with high anti-A/B antibody titers before plasmapheresis or immunoadsorption. Nonetheless, acute infection with an organism possessing surface markers analogous to blood group antigens such as carbohydrate structures on

  14. Hashimoto's encephalopathy.%桥本脑病

    Institute of Scientific and Technical Information of China (English)

    赵伟秦

    2012-01-01

    Hashimoto's encephalopathy (HE) is a controversial neurological disorder that includes a group of heterogeneous neurological symptoms and the increased level of antithyroid antibodies. The clinical manifestations of HE may include encephalopathic features such as convulsion, behavioral and psychiatric abnormality, movement disorders and coma. Although HE has been believed to be correlated with Hashimoto's thyroiditis or thyroid dysfunction,the most common immuno-logical abnormality of HE is the presence of increased antibodies of antithyroglobulin or antimicrosomal. At present,it is still unclear as to whether the increased antithyroid antibodies is abiogenetic dysimmunity secondary to encephalopathy or it does cause the occurrence of disease. The effective reaction of HE to hormone or plasmapheresis also supported the hypothesis that this belonged to the immune pathogenic mechanism.%桥本脑病(HE)是一种目前尚存在争议的神经系统功能紊乱,包括患者出现的一组异质性的神经系统症状以及抗甲状腺抗体的水平增高.HE的临床表现可包括脑病的特点,如抽搐、精神行为异常、运动障碍和昏迷.尽管被认为与桥本甲状腺炎或甲状腺功能紊乱相关,HE最常见的免疫学异常是出现血清抗甲状腺球蛋白抗体(TG Ab)或抗甲状腺过氧化物酶(antimicrosomal)抗体(TMAb)的增高.目前尚不清楚抗甲状腺抗体的增高是继发于脑病的一种偶发免疫异常还是其确实导致了疾病的发生.HE对激素或者血浆置换等治疗的有效反应也支持这种属于免疫异常发病机制的推论.

  15. Extracorporeal blood therapy in sepsis and acute respiratory distress syndrome: the "purifying dream"

    Institute of Scientific and Technical Information of China (English)

    Xu Xuefeng; Dai Huaping; Jia Chun'e; Wang Chen

    2014-01-01

    Objective To discuss the rationale,hypothesis,modality of extracorporeal blood purification (EBP) techniques for the critically ill animal models or patients,and to summarize the experimental and clinical studies with inconsistent data which explored the EBP's efficacy in the areas of critical care medicine.Data sources Articles referred in this review were collected from the database of PubMed published in English up to June 2014.Study selection We had done a literature search by using the term "(sepsis OR acute lung injury OR acute respiratory distress syndrome) AND (extracorporeal blood purification OR hemofiltration OR hemoperfusion OR plasma exchange OR plasmapheresis OR adsorpiton)".Related original or review articles were included and carefully analyzed.Results Acute cellular and humoral immune disturbances occur in both sepsis and acute respiratory distress syndrome (ARDS).Treatments aimed at targeting one single pro-/anti-inflammatory mediator have largely failed with no proven clinical benefits.Such failure shifts the therapeutic rationale to the nonspecific,broad-spectrum methods for modulating the over-activated inflammatory and anti-inflammatory response.Therefore,EBP techniques have become the potential weapons with high promise for removing the circulating pro-/anti-inflammatory mediators and promoting immune reconstitution.Over the years,multiple extracorporeal techniques for the critically ill animal models or patients have been developed,including hemofiltration (HF),high-volume hemofiltration (HVHF),high-cutoff hemofiltration (HCO-HF),hemo-perfusion or-adsorption (HP/HA),coupled plasma filtration adsorption (CPFA),and plasma exchange (PE).These previous studies showed that EBP therapy was feasible and safe for the critically ill animal models or patients.However,data on their efficacy (especially on the clinical benefits,such as mortality) were inconsistent.Conclusions It is not now to conclude that EBP intervention can purify septic or ARDS

  16. Current experience with renal transplantation across the ABO barrier.

    Science.gov (United States)

    Nelson, P W; Helling, T S; Shield, C F; Beck, M; Bryan, C F

    1992-11-01

    Solid organ transplantation has traditionally been governed by the rules of blood group compatibility. Thus, it has been demonstrated that crossing the ABO blood group barrier generally results in hyperacute rejection. However, the A2 subtype of the blood group A is a weaker antigen. Under certain circumstances, organs from donors with blood group A2 can be transplanted across the ABO blood group barrier into recipients of O or B blood type. Since 1986, 33 patients including 24 blood group O and 9 blood group B patients received A2 (30) or A2B (3) donor kidneys. Both cadaver donor (31) and living-related grafts (2) have been undertaken. The mean follow-up since transplantation for the 21 patients with functioning grafts is 36 months, with a 67.2% current graft survival. Immunosuppression for these transplants consisted of azathioprine, prednisone, and cyclosporine, often in combination with prophylactic OKT3 or antilymphocyte globulin as protocol dictated. Special immunosuppressive protocols such as splenectomy or plasmapheresis were not used. The serum of the potential recipient was analyzed for immunoglobulin G (IgG) and immunoglobulin M (IgM) forms of antibody against A1 and A2 red blood cells. There is a strong correlation between a low (less than or equal to 1:8) anti-A1 IgG titer and both early and long-term graft function. Recipients with an IgG titer greater than 1:8 in the pretransplant serum had a much higher incidence of early graft failure. We no longer recommend transplantation of A2 kidneys into O or B recipients with a pretransplant titer of greater than 1:8 but found that recipients with low titers have graft function rates essentially equal to those of ABO-compatible patients. Patients with blood group B have, over time, lower anti-A IgG titers than do blood group O patients. In addition, the graft survival among blood group B patients is 89% compared with 58% among group O recipients. This may be due to the generally low titers found in blood

  17. Use of complement binding assays to assess the efficacy of antibody mediated rejection therapy and prediction of graft survival in kidney transplantation.

    Science.gov (United States)

    Ramon, Daniel S; Huang, Yihung; Zhao, Lili; Rendulic, TrisAnn; Park, Jeong M; Sung, Randall S; Samaniego, Milagros

    2017-02-01

    The Luminex® single antigen bead assay (SAB) is the method of choice for monitoring the treatment for antibody-mediated rejection (AMR). A ⩾50% reduction of the dominant donor-specific antibody (IgG-DSA) mean fluorescence intensity (MFI) has been associated with improved kidney allograft survival, and C1q-fixing DSA activity is associated with poor outcomes in patients with AMR. We aimed to investigate if C1q-DSA can be used as a reliable predictor of response to therapy and allograft survival in patients with biopsy-proven AMR. We tested pre- and post-treatment sera of 30 kidney transplant patients receiving plasmapheresis and low-dose IVIG for biopsy-proven AMR. IgG-DSA and C1q-DSA MFI were measured and correlated with graft loss or survival. Patients were classified as nonresponders (NR) when treatment resulted in <50% reduction in MFI of IgG-DSA and/or C1q-DSA was detectable following therapy. Differences in the percentage of patients deemed NR depended upon the end-point criterion (73% by reduction in IgG-DSA MFI vs. 50% by persistent C1q-DSA activity). None of the seven patients with <50% reduction of IgG-DSA but non-detectable C1q-DSA-fixing activity after therapy experienced graft loss, suggesting that C1q-DSA activity may better correlate with response. Reduction of C1q-DSA activity predicted graft survival better than IgG-DSA in the univariate Cox analysis (20.1% vs. 5.9% in NR; log-rank P-value=0.0147). A rapid reduction of DSA concentration below the threshold required for complement activation is associated with better graft survival, and C1q-DSA is a better predictor of outcomes than IgG-DSA MFI reduction. Copyright © 2016 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.

  18. Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries.

    Science.gov (United States)

    Dewan, Deepak; Gulati, Sanjeev; Sharma, Raj K; Prasad, Narayan; Jain, Manoj; Gupta, Amit; Kumar, Alok

    2008-03-01

    Crescentic glomerulonephritis (CsGN) is an uncommon entity in children. This prospective study was conducted to evaluate the aetiology, clinical spectrum and outcome in children with crescentic glomerulonephritis. The single-centre prospective study comprised of 22 children with biopsy proven CsGN who had been referred to our institute over the period January 2000 to December 2005. These patients were subjected to detailed clinical and biochemical examinations. The diagnosis of underlying renal disease was based on various criteria, including the clinical picture, serology and histopathology. The patients received intravenous methyl prednisolone, oral steroid treatment, and oral cyclophosphamide with or without plasmapheresis. All patients received supportive care, including control of hypertension and oedema and supportive management of renal insufficiency. During this 5-year period, CsGN accounted for 5.1% of all biopsies done in children. The mean age was 12.27 years (range 4 years to 18 years). There were eight girls and 14 boys. The mean duration of symptoms prior to referral was 2.47 months (range 5 days to 21 months). Aetiology was immune complex in 19 cases, anti-glomerular basement membrane (anti-GBM) antibody disease in two cases and pauci-immune (Wegener's granulomatosis) in one case. The percentage of crescents ranged from 50% to 100% (mean 70.6%). Twenty-one out of 22 (95.5%) children in our series had hypertension at presentation that required treatment with antihypertensive medications. The serum creatinine level at presentation ranged from 1.5 mg/dl to 11.4 mg/dl (mean 5.5 mg/dl). Of the 22 children, two were lost to follow-up, while the mean follow-up period of the rest of the 20 children was 8.13 months (range 1 month to 43 months). At the last follow-up of the 22 children, ten had stage 5 chronic kidney disease (CKD) and three had stage 4 CKD, while seven children had a calculated glomerular filtration rate (GFR) of >60 ml/min per 1.73 m(2) body

  19. Living-related liver transplantation for fulminant hepatic failure in children.

    Science.gov (United States)

    Tanaka, K; Uemoto, S; Inomata, Y; Tokunaga, Y; Ueda, M; Tokka, A; Sato, B; Yamaoka, Y

    1994-01-01

    Liver transplantation is increasingly accepted as a choice of treatment for fulminant hepatic failure (FHF) since it has been proved to significantly improve the survival rate in these patients compared with other therapeutic modalities. We have successfully performed a total of 76 living related liver transplantations (LRLT) three of which were for FHF. The first case was an 11-year-old boy with FHF due to an unidentified cause. He had required plasmapheresis a total of 24 times and haemofiltration to save his life before LRLT. He was transplanted with a left lobe (420 g) graft, calculated as 1.05% of his weight (40 kg). He recovered hepatic function uneventfully and was discharged from hospital after 7 weeks. The second case was a 13-year-old girl who developed FHF with grade III encephalopathy due to acute Wilson's disease, and was referred to us. She underwent LRLT with a left lobe graft (440 g), estimated as 0.95% of her weight (47 kg), which functioned well after surgery. The third case was a 13-year-old girl with grade II encephalopathy due to acute Wilson's disease. She was 27% obese with a body weight of 58 kg. She underwent LRLT with ABO blood group incompatibility with a left lobe (352 g), estimated as 0.80% of her weight (modified 44 kg). She was discharged with sensorimotor neuropathy due to vitamin B deficiency. The present results suggest that LRLT is feasible for FHF both clinically and ethically, and that a partial liver graft weighing around 1% of the recipient's weight can maintain the recipient's life. We limit the diagnostic indication for LRLT to chronic liver disease, since an urgent situation may affect a voluntary decision for the patient's parents to donate the partial liver. However, LRLT is thought to be an acceptable choice of treatment provided it is requested by the patient and family. Furthermore, it is a potential option for resolving the graft shortage in paediatric liver transplantation, being independent of cadaver donor

  20. Extracorporeal blood purification for acute liver failure in children%血液净化技术在小儿急性肝功能衰竭中的应用

    Institute of Scientific and Technical Information of China (English)

    崔云; 张育才

    2012-01-01

    小儿急性肝功能衰竭病死率高.目前公认的有效治疗方法是肝移植.人工肝为自体肝脏功能恢复创造条件或作为肝脏移植的“桥梁”.人工肝支持系统被分为生物型人工肝装置、非生物型人工肝装置和混合型人工肝装置3类.以往非生物型人工肝应用方法(如血浆置换、血液透析、血液滤过、血液/血浆灌流等)能改善血液生化指标,但不能影响预后.近年来,非生物型人工肝设备有了很大进步,出现了连续性肾替代治疗、组合式血液净化(普罗米修斯系统、分子吸附再循环系统等)新型血液净化模式.新型血液净化模式能提高肝功能衰竭患儿的生存率.%Pediatric acute liver failure has a high mortality.Liver transplantation is the only definite treatment.Extracorporeal liver support can be employed as a strategy for bridging to transplantation or recovery.There are three types of device for temporary support:biological,non-biological (also called artificial) and bio-artificial (hybrid techniques).Early non-biological device including hemodialysis,hemoperfusion/plasma perfusion and plasmapheresis have improved biochemical efficacy,but there are little data for clinical end points.Continuous blood purification and the combination of various non-biological methods such as the molecular adsorbent recirculatory system and Prometheus(R) are new extracorporeal blood purification therapy.The results of many experimental and clinical trials have improved the efficacy of the above mentioned methods.

  1. Macroglobulinemia de Waldenström com hemorragia intra-ocular Waldenström's macroglobulinaemia with intraocular hemorrhage

    Directory of Open Access Journals (Sweden)

    João J. Nassaralla Júnior

    2006-12-01

    accompanied by a severe IgM increase in serum viscosity. The most important ocular findings were reversible after plasmapheresis. Waldenström's macroglobulinaemia can involve both retinal and choroidal circulation. The prominent microvasculopathy is reversible after appropriate treatment.

  2. Pathogenesis and Treatment of Neonatal Lupus Erythematosus with Cardiac Involvement%新生儿红斑狼疮心脏受累

    Institute of Scientific and Technical Information of China (English)

    李霞; 张丽丽; 赵岩

    2015-01-01

    Neonatal lupus erythematosus (NLE)is a passively acquired autoimmune disease associated with maternal anti-Ro/SSA,anti-SSB/La and anti U1RNP antibodies,of which fetal heart involvement is the most serious clinical manifestations.The molecular mechanisms are not fully understood, but several targets for the maternal autoantibodies in the fetal heart have been suggested.Recent studies also indicate that environmental factors and fetal susceptibility genes determine whether an autoantibody-exposed fetus will develop congenital heart block (CHB)or not.Prenatal application of fluorinatedcorticosteroids,hydroxychloroquine,intravenous immunoglobulin (IVIG),and plasmapheresis have been under investigation,but the results are controversial.In this article,we reviewed the advances in the pathogenesis and treatment of NLE with cardiac involvement in order to pursue early interventions.%新生儿红斑狼疮(neonatal lupus erythematosus,NLE)是一种被动获得性自身免疫性疾病,发生在血清具有抗SSA/Ro、抗 SSB/La 或抗 U1核糖核蛋白抗体孕妇的后代中,可呈多系统受累,其中心脏受累为最严重的表现。其发病机制研究包括自身抗体、心肌细胞凋亡、分子模拟、炎性纤维化通路及环境、遗传因素等。治疗方案包括含氟糖皮质激素、静脉注射人免疫球蛋、羟氯喹以及血浆置换等,但疗效均存争议。总结 NLE 心脏受累的临床表现、发病机制及治疗进展,有助于提高该病早期诊断率并获得及早干预,从而获得良好的妊娠结局。

  3. Peculiarities of intensive care at destructive pneumonias in children

    Directory of Open Access Journals (Sweden)

    Марина Александровна Макарова

    2015-07-01

    Full Text Available In spite of success in treatment and diagnostics attained last years the problem of an acute pneumonia remains actual, this fact is caused by the growing rate of prolonged clinical course and unfavorable clinical outcomes. The growth of antibiotic resistance of microflora is a substantial problem. In addition even the most vigorous modern antibacterial preparations are not effective without an adequate surgical sanitation of the nidus of infection.The aim of the work is to improve the therapy of destructive pneumonia in children that need an intensive care.Methods. 12 patients 9 month – 12 years old who underwent the treatment of heavy community-acquired pneumonia were under observation. There was carried out an X-ray examination at admission and in dynamics, microbiological examination of lavages from the respiratory tract, pleural exudate and blood, determination of laboratory indexes of intoxication (by Kalf-Kalif, Dashtayants, Lubimova and the level of the middle molecules. The statistical analysis was done using the program package "Microsoft Excel" and "StatSoft 6".Results. In the course of microbiological examination it was demonstrated the prevalence of gram-negative flora and its associations with gram-positive one, the purely gram-positive flora was detected only in 25 % of children. So there was proved an inefficiency of antibiotics that effect mainly on the gram-positive microorganisms. In addition the cephalosporins of the third generation were found insufficiently effective. Such preparations as carbapenems or protected penicillins were chosen in vitro and according to its clinical efficiency. Plasmapheresis and bronchoscopy are useful in the complex therapy. Thoracoscopy must be carried out if the conservative treatment is not effectiveConclusions. At present the gram-negative flora and its associations with gram-positive one prevails in etiological structure of the heavy community-acquired pneumonias that must be taken into

  4. Immunopathogenesis of inflammatory myopathies.

    Science.gov (United States)

    Dalakas, M C

    1995-05-01

    Immune-mediated mechanisms appear to play a primary role in the pathogenesis of polymyositis (PM) and dermatomyositis (DM). The serum of patients with active DM has high levels of circulating complement fragments C3b, C4b, and C5b-9 membranolytic attack complex (MAC) and demonstrates a very high C3 uptake in an vitro assay system. The MAC and the immune complex-specific C3bNEO fragment are deposited on the endomysial capillaries early in the disease and lead sequentially to loss of capillaries, muscle ischemia, muscle fiber necrosis, and perifascicular atrophy. In contrast, in PM the muscle fiber injury is initiated by sensitized CD8+ cytotoxic T cells that recognize heretofore unknown and probably endogenous muscle antigens in the context of major histocompatibility complex (MHC) class I expression. A restricted (oligoclonal) pattern of T-cell receptor with prominence of Va1, Vb6, and Vb15 genes is noted within the endomysial infiltrates suggesting that the T-cell response is antigen driven. In both PM and DM, intercellular adhesion molecule (ICAM)-1 and vascular cell adhesion molecule (VCAM)-1 are upregulated in the endomysial endothelial cells and function as ligands for the leukocyte integrins leukocyte function-associated antigen (LFA)-1 and very late activating antigen (VLA)-4, allowing activated lymphocytes to adhere to the endothelial cells and migrate to the muscle fibers. Among viruses, only the retroviruses human immunodeficiency virus (HIV) and human T-cell lymphotropic virus (HTLV)-1 have been convincingly shown to trigger PM, which is mediated by nonviral-specific, cytotoxic CD8+ cells. The treatment of inflammatory myopathies remains empirical. Many patients respond to steroids to some degree and for some period of time. Azathioprine, methotrexate, cyclosporine, cyclophosphamide, and plasmapheresis can be of mild to moderate benefit. High-dose intravenous immunoglobulin (IVIg) is a promising therapeutic modality for some patients resistant to

  5. Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature

    Science.gov (United States)

    Abu-Hishmeh, Mohammad; Sattar, Alamgir; Zarlasht, Fnu; Ramadan, Mohamed; Abdel-Rahman, Aisha; Hinson, Shante; Hwang, Caroline

    2016-01-01

    Patient: Female, 34 Final Diagnosis: Refractory thrombotic thrombocytopenic purpura Symptoms: Fatigue Medication: — Clinical Procedure: Plasma exchange Specialty: Rheumatology • Hematology and Critical Care Objective: Rare co-existance of disease or pathology Background: Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE. In rare cases, TTP may precede the diagnosis of SLE or occur concurrently. Case Report: We present a case of a previously healthy 34-year-old female who presented with dizziness and flu-like symptoms and was found to have thrombocytopenia, hemolytic anemia, and schistocytes in the peripheral smear. She was subsequently diagnosed with TTP and started on plasmapheresis and high-dose steroids, but without a sustained response. A diagnosis of refractory TTP was made, and she was transferred to our facility for further management. Initially, the patient was started on rituximab, but her condition continued to deteriorate, with worsening thrombocytopenia. Later, she also fulfilled the Systemic Lupus International Collaborating Clinics (SLICC) criteria for diagnosis of SLE. Treatment of TTP in SLE patients is generally similar to that in the general population, but in refractory cases there are few reports in the literature that show the efficacy of cyclophosphamide. We started our patient on cyclophosphamide and noticed a sustained improvement in the platelet

  6. Antibody-Mediated Rejection: A Review

    Science.gov (United States)

    Garces, Jorge Carlos; Giusti, Sixto; Staffeld-Coit, Catherine; Bohorquez, Humberto; Cohen, Ari J.; Loss, George E.

    2017-01-01

    Background: Chronic antibody injury is a serious threat to allograft outcomes and is therefore the center of active research. In the continuum of allograft rejection, the development of antibodies plays a critical role. In recent years, an increased recognition of molecular and histologic changes has provided a better understanding of antibody-mediated rejection (AMR), as well as potential therapeutic interventions. However, several pathways are still unknown, which accounts for the lack of efficacy of some of the currently available agents that are used to treat rejection. Methods: We review the current diagnostic criteria for AMR; AMR paradigms; and desensitization, treatment, and prevention strategies. Results: Chronic antibody-mediated endothelial injury results in transplant glomerulopathy, manifested as glomerular basement membrane duplication, double contouring, or splitting. Clinical manifestations of AMR include proteinuria and a rise in serum creatinine. Current strategies for the treatment of AMR include antibody depletion with plasmapheresis (PLEX), immunoadsorption (IA), immunomodulation with intravenous immunoglobulin (IVIG), and T cell– or B cell–depleting agents. Some treatment benefits have been found in using PLEX and IA, and some small nonrandomized trials have identified some benefits in using rituximab and the proteasome inhibitor-based therapy bortezomib. More recent histologic follow-ups of patients treated with bortezomib have not shown significant benefits in terms of allograft outcomes. Furthermore, no specific treatment approaches have been approved by the US Food and Drug Administration. Other agents used for more difficult rejections include bortezomib and eculizumab (an anti-C5 monoclonal antibody). Conclusion: AMR is a fascinating field with ample opportunities for research and progress in the future. Despite the use of advanced techniques for the detection of human leukocyte antigen (HLA) or non-HLA donor-specific antibodies

  7. [Clinical characteristics and follow-up of pediatric patients with neuromyelitis optica and neuromyelitis optica spectrum disorders].

    Science.gov (United States)

    Yun, Wu; Weihua, Zhang; Xiaotun, Ren; Jiuwei, Li; Xinying, Yang; Junlan, Lyu; Changhong, Ding; Chunhong, Chen; Haitao, Ren; Liying, Cui; Fang, Fang

    2015-04-01

    gammaglobulin. The symptoms of 8 cases mitigated. Two cases whose symptoms showed no sign of improvement received plasmapheresis for acute attacks. Seven of the patients were followed up. The median duration of follow-up was 19 months (ranged from 13 months to 30 months). The median Expanded disability status (EDSS) score was 3 (range 1-7). Pediatric NMO and(or) NMOSD have a diverse clinical presentation which are more than just optic neuritis and transverse myelitis, including brain symptom. So it may be difficult to distinguish NMO and( or) NMOSD from acute disseminating encephalomyelitis and multiple sclerosis in the early stages of the disease. Antibodies to aquapoin-4 (AQP-Ab) testing is very important for differential diagnosis.

  8. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study.

    Science.gov (United States)

    Cabral, David A; Canter, Debra L; Muscal, Eyal; Nanda, Kabita; Wahezi, Dawn M; Spalding, Steven J; Twilt, Marinka; Benseler, Susanne M; Campillo, Sarah; Charuvanij, Sirirat; Dancey, Paul; Eberhard, Barbara A; Elder, Melissa E; Hersh, Aimee; Higgins, Gloria C; Huber, Adam M; Khubchandani, Raju; Kim, Susan; Klein-Gitelman, Marisa; Kostik, Mikhail M; Lawson, Erica F; Lee, Tzielan; Lubieniecka, Joanna M; McCurdy, Deborah; Moorthy, Lakshmi N; Morishita, Kimberly A; Nielsen, Susan M; O'Neil, Kathleen M; Reiff, Andreas; Ristic, Goran; Robinson, Angela B; Sarmiento, Angelyne; Shenoi, Susan; Toth, Mary B; Van Mater, Heather A; Wagner-Weiner, Linda; Weiss, Jennifer E; White, Andrew J; Yeung, Rae S M

    2016-10-01

    plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil. Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding. © 2016, American College of Rheumatology.

  9. Síndrome de Evans de aparición súbita en el embarazo Evans Syndrome of Sudden onset in Pregnancy

    Directory of Open Access Journals (Sweden)

    Jorge Barboza-Retana

    2011-12-01

    Full Text Available La trombocitopenia complica hasta el 10% de los embarazos. El síndrome de Evans es la coexistencia de la trombocitopenia inmune con anemia hemolítica autoinmune. Se presenta un caso de síndrome de Evans durante el embarazo, complicado por preclampsia y neutropenia fetal. Una mujer de 20 años de edad, previamente sana, primigesta, que presentó a las 34 semanas de gestación dolor epigástrico, trombocitopenia y pre eclampsia. Se le realizó parto por cesárea, por presentar síndrome de HELLP. A los 25 días de postparto, reingresó al Hospital con trombocitopenia severa, plaquetas 2000 x mm³, anemia, hemoglobina 5,3 g/dl y fiebre. El Coombs directo fue positivo y el análisis posterior demostró la presencia de anticuerpos anti-plaquetarios, y anticardiolipina. Recibió transfusiones de sangre y plaquetas. Se le trató con prednisona, plasmaferesis, azatioprina y finalmente, resolvió su cuadro clínico después de 10 días de internamiento en una unidad de cuidado intensivo.Thrombocytopenia complicates up to 10% of pregnancies. Evans syndrome is the coexistence of immune thrombocytopenia with autoimmune hemolytic anemia. We report a case of Evans syndrome during pregnancy complicated by preeclampsia and fetal neutropenia. A 20year-old primigravida, previously healthy, presented at 34 weeks of gestation epigastric pain, thrombocytopenia and preeclampsia. She underwent cesarean delivery for HELLP syndrome. In the 25th postpartum day, she was readmitted to the hospital with severe thrombocytopenia, platelet count in 2000x mm³, anemia, hemoglobin 5.3 g/dl and fever. The direct Coombs was positive and subsequent analysis showed the presence of platelet antibodies, and anticardiolipin. She received blood and platelet transfusions. She was treated with prednisone, plasmapheresis, azathioprine, and finally recovered 10 days in an intensive care unit.

  10. Aplasia pura de serie roja post-trasplante alogeneico de células progenitoras hematopoyeticas ABO incompatible Pure red cell aplasia after ABO incompatible bone marrow transplantation

    Directory of Open Access Journals (Sweden)

    E. Bulliorsky

    2002-12-01

    Full Text Available El trasplante alogeneico de células progenitoras hematopoyéticas (TCPH con incompatibilidad ABO entre el donante y el receptor puede en ocasiones asociarse a trastornos en la progenie eritroide desarrollada a partir de la médula ósea trasplantada, caracterizado por un funcionamiento tardío, inadecuado e incompleto de la misma. En este contexto, la aplasia pura de serie roja es la complicación más severa. Se han intentado tratamientos para la aplasia pura de serie roja post-TCPH con eritropoyetina o plasmaféresis, con relativo éxito. Algunos autores han informado también la utilización de globulina antilinfocitaria, asumiendo que dicha aplasia selectiva de la serie roja en la médula ósea trasplantada es mediada por un mecanismo inmune. En este trabajo se describe un paciente portador de una leucemia aguda en quien se realizó un TCPH alogeneico (ABO incompatible con su donante. Teniendo niveles bajos de aglutininas contra el grupo sanguíneo de la donante, desarrolló una aplasia pura de serie roja post - TCPH. La misma no mejoró con tratamiento con eritropoyetina o con un refuerzo de progenitores hematopoyéticos de sangre periférica de la misma donante (boost, resolviéndose totalmente luego de un tratamiento exitoso con globulina antilinfocitaria de origen equino.ABO incompatibility in allogeneic bone marrow transplantation may be associated with incomplete or delayed erythroid engraftment, being pure red cell aplasia (PRCA the most severe complication in this setting. Attempts for the treatment of PRCA have been made with erythropoietin or with plasmapheresis with relative success, and some authors have reported the reversibility of PRCA with antilymphocyte globulin (ALG or ATG, based on the assumption that PRCA might be immunologically mediated. We report herewith a patient with acute leukemia who developed post - BMT pure red cell aplasia. His sibling donor (sister was HLA identical and ABO incompatible, having low agglutinin

  11. Mixed cryoglobulinemia

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    Ferri Clodoveo

    2008-09-01

    , polyarthritis, and B-cell lymphomas. The first-line treatment of MC should focus on eradication of HCV by combined interferon-ribavirin treatment. Pathogenetic treatments (immunosuppressors, corticosteroids, and/or plasmapheresis should be tailored to each patient according to the progression and severity of the clinical manifestations. Long-term monitoring is recommended in all MC patients to assure timely diagnosis and treatment of the life-threatening complications. The overall prognosis is poorer in patients with renal disease, liver failure, lymphoproliferative disease and malignancies.

  12. Evidence-based evaluation of treatment strategy for multiple sclerosis%多发性硬化治疗措施的临床证据评价

    Institute of Scientific and Technical Information of China (English)

    李梦秋; 胡佳; 司洋; 李娟; 刘凌

    2012-01-01

    manual searching and screened out conference paper and academic dissertation, etc, from various references. After that we obtained and evaluated by Jadad scales on systematic reviews, randomized controlled trials, controlled clinical trials and observational study cases about glucocorticoids, plasmapheresis, intravenous immunoglobulin, IFN - P, glatiramer acetate, mitoxantrone, natalizumab, fingolimod. Results After screening, all seventeen selected resources included systematic reviews 6 articles, randomized controlled trials 7 articles, controlled clinical trials 2 articles, observational study cases 2 articles, among which fifteen articles were proved to be high quality (according to Jadad scoring system, five score 4, six score 5, four score 7), two chapters were judged to be low quality scoring 3. Finally, we summerize that: 1) The first choice of treatment for acute relapses is glucocorticoids and we suggest that plasmapheresis or intravenous immunoglobulin may be tried as an alternative therapy in acute MS relapse, especially in case of contraindications to intravenous methylprednisolone. 2) Immunomodulatory or immunosuppressive treatment (IFN - P, glatiramer acetate, mitoxantrone, natalizumab) can be an option to prevent new relapses and progression of disability. 3) Fingolimod is an oral treatment for multiple sclerosis to improve treatment adherence. Conclusion Using evidence-based medicine methods can provide us best clinical evidence on MS treatment.

  13. 血浆净化治疗在风湿免疫性疾病中的应用%Application of plasma purification in rheumatism and immune diseases

    Institute of Scientific and Technical Information of China (English)

    刘新宇; 王晓红; 李宾; 李永强; 黄敏; 刘爱群; 邹和群

    2012-01-01

    目的 探讨血浆净化技术在风湿免疫性疾病治疗中的应用. 方法 在南方医科大学第三附属医院血液净化中心行血浆净化治疗的风湿免疫性疾病患者63例,在药物治疗的基础上,根据病情选择双重滤过血浆净化技术(double-filtration plasmapheresis,DFPP)或血浆吸附技术(plasma absorption,PA)治疗.治疗过程中密切观察生命体征及不良反应.治疗前后检测血清免疫球蛋白、自身抗体、补体(C3、C4)、红细胞沉降率(ESR)、C反应蛋白(CRP)等指标,并观察治疗后的临床反应. 结果 16例系统性红斑狼疮患者在血浆净化治疗后SLEDAI评分、24h尿蛋白定量、抗ds-DNA抗体、IgG、C3均较治疗前显著改善,差异有统计学意义;10例类风湿性关节炎患者血浆净化治疗后ESR、IgG均较治疗前显著下降,其中8例(80%)患者晨僵时间缩短,乏力症状改善.7例(70%)患者关节肿胀和疼痛减轻.7例强直性脊柱炎患者治疗后关节疼痛、晨僵方面均有不同程度的减轻,有效率100%.在功能改善方面,有2例患者改善2个等级,4例患者改善1个等级,1例患者无改善,有效率86%;5例肌萎缩性脊髓侧索硬化症(“渐冻人”症)患者治疗后肌力均明显改善.单次DFPP治疗后IgA、IgG、IgM、C3、C4的下降率与单次PA治疗后IgA、IgG、IgM、C3、C4的下降率无显著的统计学差异. 结论 血浆净化技术可有效治疗多种风湿免疫性疾病,且安全性高,临床上值得广泛开展.%Objective To investigate the application of plasma purification in rheumatism and immune disease. Methods A total of 63 patients with rheumatism and immune diseases were treated with plasma purification in association with medications. Based on patient disease conditions, double-filtration plasma-pheresis (DFPP) or plasma absorption (PA) was selected for the therapy. Vital signs and adverse reactions were carefully monitored during the plasma purification processes

  14. SUCCESSFUL APPLICATION OF PERIPHERAL VENO-ARTERIAL EXTRACORPOREAL MEMBRANE OXYGENATION FOR CARDIAC ALLOGRAFT ANTIBODY-MEDIATED REJECTION WITH SEVERE HEMODYNAMIC COMPROMISE

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    V. N. Poptsov

    2015-01-01

    Full Text Available Introduction. Acute antibody-mediated rejection (AMR is one of the severe complications of early and late period after heart transplantation (HT. Only few case reports and studies presented of mechanical circulatory support (MCS application for refractory acute rejection causing hemodynamic compromise. Aim. We report the case of a woman with cardiogenic shock caused by severe AMR that was successfully treatment by peripheral venoarterial extracorporeal membrane oxygenation (VA ECMO. Material and methods. In december 2014, a 60-year-old woman with dilated cardiomyopathy was operated for HT. The patient had a good initial cardiac allograft function and no and was discharged from ICU on the 4th day after HT. 1st endomyocardial biopsy (EMB (the 7th day after HT showed absence of acute cellular and antibody-mediated rejection. On the 11th day after HT patient aggravated and presented clinical signs of life-threatening acute cardiac allograft dysfunction: arterial blood pressure 78/49/38 mm Hg, HR 111 in min, CVP 20 mm Hg, PAP 47/34/25 mm Hg, PCWP 25 mm Hg, CI 1.5 l/min/m2, adrenalin 110 ng/kg/min, dopamine 15 mcg/kg/min. ECG showed impairment of systolic left (LVEF 25% and right (RVEF 15% ventricle function, left and right ventricle diffuse hypokinesis, thickness of IVS, LV and RV wall 1.7, 1.4 and 0.8 cm, tricuspid and mitral valve regurgitation 2–3 degrees. EMB presented AMR. In conscience peripheral VA ECMO was installed. We used peripheral transcutaneous cannulation technique via femoral vessels – arterial cannula 15 F, venous cannula – 23 F, vascular catheter 14 G for anterograde leg’s perfusion. ACT 130–150 sec. AMR therapy included: methylprednisolon pulse-therapy (10 mg/kg for 5 day, IgG, plasmapheresis (No 7, rituximab. Results. Under MCS by VA ECMO we noted quick improvement of hemodynamic, metabolic homeostasis and organ functions. On the 6th day of VA ECMO (blood flow 1.8 l/min: arterial blood pressure 133/81/54 mm Hg, CVP 5 mm

  15. Urgencias en patología neuromuscular Emergencies in neuromuscular pathology

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    T. Ayuso

    2008-01-01

    . Within the broad group of neuromuscular diseases, those that most frequently provoke AMW and respiratory failure are Guillain-Barré syndrome (GBS and myasthenia gravis (MG. GBS is the most frequent cause of acute flaccid paralysis; it can cause respiratory failure in a third of cases, making mechanical ventilation necessary. Accurate diagnosis of this syndrome enables immunomodulatory treatment to be started, which has been shown to modify the course of the disease. Besides, clinical evaluation of the patients and knowledge of the simple tests of neurophysiology and respiratory function will guide the decision on mechanical ventilation, avoiding emergency intubation. The most frequent emergency caused by MG is myasthenic crisis, defined by the deterioration in the bulbar function with acute respiratory insufficiency and risk of respiratory stoppage. This occurs in 15-20% of myasthenic patients and can be triggered by numerous factors. Besides early identification of the crisis, it is important to suppress the triggering factors and to provide measure of ventilatory support. Amongst the pharmacological measures, the most useful instruments at present are plasmapheresis and intravenous immunoglobulins; these treatments do not cancel the need for intensive vigilance and of checking for imminent signs of respiratory failure that will involve invasive or non-invasive ventilatory support.

  16. The clinical progress of haemoperfusion in the treatment of paraquat poisoning%血液灌流治疗百草枯中毒的临床研究进展

    Institute of Scientific and Technical Information of China (English)

    李国强; 燕朋波; 李玉明

    2014-01-01

    The high mortality of paraquat (PQ) poisonings is mainly due to the lack of effective treatments. Most toxicologists recommend rapid initiation of charcoal haemoperfusion (CHP) to lower plasma PQ levels and to limit pulmonary and other organs uptake of PQ. Although there are considerable evidences of CHP efficacy in the reversion of the fatal outcome resulting from PQ poisonings, the usefulness of this therapy has been the subject of significant controversy with several evidences published in the literature showing a lack of clinical benefit in numerous cases. Considering that the blood concentration at a given time is directly related to clinical outcome, the rebound in plasma paraquat concentration after haemoperfusion may be partly relate to the poor clinical benefit, but may indicate the necessity for prolonged haemoperfusion. Continuous rather than intermittent haemoperfusion has been advocated for treatment of paraquat poisoning. However, 24h daily conventional pump driven haemoperfusion is usually impossible to carry out due to bleeding complications. Plasmapheresis is also a blood purification process. Unselective therapeutic plasma exchange is the first technology used for therapeutic apheresis (TA). However, limited efficacy by restricted plasma volume that can be exchanged in a single session is one of the severe limitations. To further increase the clearance of target molecules, plasma perfusion (PP) techniques was developed. modification of the conventional plasma perfusion in patients with paraquat poisoning has been reported and plasma levels of paraquat were reduced effectively.%百草枯中毒的高病死率主要与临床无有效治疗有关,临床多推荐早期活性炭灌流以降低血浆百草枯浓度水平,进而减少肺组织的摄取。虽然部分研究支持该方法,但也存在较大争论。百草枯中毒患者预后与血浆中的百草枯质量浓度直接相关,部分研究认为与全血灌流后血浆中百草枯浓

  17. Plasma from patients with systemic lupus erythematosus inhibits suppressive activity of mesenchymal stem cells against lupus B lymphocytes%系统性红斑狼疮患者血浆降低间充质干细胞对B淋巴细胞的抑制作用

    Institute of Scientific and Technical Information of China (English)

    聂瑛洁; 罗利梅; 查艳; 孙立; 骆辑; 潘润桑; 田晓滨

    2016-01-01

    Objective To investigate whether plasma from patients with systemic lupus erythematosus (SLE) inhibits the suppressive effects of mesenchymal stem cells (MSCs) on lupus B lymphocytes. Methods MSCs isolated and expanded from the bone marrow of healthy donors were co-cultured with B cells purified from the peripheral blood of SLE patients in the presence of fetal bovine serum or pooled plasma from SLE patients, and the proliferation and maturation of the B lymphocytes were analyzed. Results Co-culture with normal MSCs obviously inhibited the proliferation of lupus B cells and suppressed the maturation of B lymphocytes, which showed lowered expressions of CD27 and CD38. The pooled plasma from SLE patients significantly inhibited the suppressive effects of normal MSCs on B cell proliferation and maturation. Conclusion Plasma from SLE patients negatively modulates the effects of normal MSCs in suppressing lupus B cell proliferation and maturation to affect the therapeutic effect of MSC transplantation for treatment of SLE. Double filtration plasmapheresis may therefore prove beneficial to enhance the therapeutic effects of MSC transplantation for SLE.%目的:研究系统性红斑狼疮(SLE)患者血浆对健康人骨髓来源的间充质干细胞(MSCs)免疫抑制作用的影响。方法采用SLE患者富集血浆替代胎牛血清,共培养MSCs与SLE患者的B淋巴细胞,检测MSCs对B淋巴细胞增殖能力与成熟的影响。设置胎牛血清作为对照组。数据采用SPSS12.0软件进行学生t检验统计学分析,P<0.05认为差异有统计学意义。结果正常MSCs能抑制脂多糖刺激的SLE患者来源B细胞增殖,降低B细胞表面表达的CD27与CD38。SLE患者富集血浆能抵制MSC对B淋巴细胞增殖与成熟的抑制作用。结论正常MSCs能抑制SLE患者B淋巴细胞增殖与成熟,改变B淋巴细胞亚群所占比例。SLE患者血清能抑制MSCs对B淋巴细胞的免疫抑制作用,或将负向影响间充

  18. Extracorporeal adsorption therapy: A Method to improve targeted radiation delivered by radiometal-labeled monoclonal antibodies.

    Energy Technology Data Exchange (ETDEWEB)

    Nemecek, Eneida R.; Green, Damian J.; Fisher, Darrell R.; Pagal, John M.; Lin, Yukang; Gopal, A. K.; Durack, Lawrence D.; Rajendran, Joseph G.; Wilbur, D. S.; Nilsson, Rune; Sandberg, Bengt; Press, Oliver W.

    2008-04-01

    Many investigators have demonstrated the ability to treat hematologic malignancies with radiolabeled monoclonal antibodies targeting hematopoietic antigens such as anti-CD20 and anti-CD45. [1-5] Although the remission rates achieved with radioimmunotherapy (RIT) are relatively high, many patients subsequently relapse presumably due to suboptimal delivery of enough radiation to eradicate the malignancy. The dose-response of leukemia and lymphoma to radiation has been proven. Substantial amounts of radiation can be delivered by RIT if followed by hematopoietic cell transplantation to rescue the bone marrow from myeloablation.[ref] However, the maximum dose of RIT that can be used is still limited by toxicity to normal tissues affected by nonspecific delivery of radiation. Efforts to improve RIT focus on improving the therapeutic ratios of radiation in target versus non-target tissues by removing the fraction of radioisotope that fails to bind to target tissues and circulates freely in the bloodstream perfusing non-target tissues. Our group and others have explored several alternatives for removal of unbound circulating antibody. [refs] One such method, extracorporeal adsorption therapy (ECAT) consists of removing unbound antibody by a method similar to plasmapheresis after critical circulation time and distribution of antibody into target tissues have been achieved. Preclinical studies of ECAT in murine xenograft models demonstrated significant improvement in therapeutic ratios of radioactivity. Chen and colleagues demonstrated that a 2-hour ECAT procedure could remove 40 to 70% of the radioactivity from liver, lung and spleen. [ref] Although isotope concentration in the tumor was initially unaffected, a 50% decrease was noted approximately 36 hours after the procedure. This approach was also evaluated in a limited phase I pilot study of patients with refractory B-cell lymphoma. [ref] After radiographic confirmation of tumor localization of a test dose of anti-CD20

  19. Impact of human leukocyte antigen matching and recipients' panel reactive antibodies on two-year outcome in presensitized renal allograft recipients

    Institute of Scientific and Technical Information of China (English)

    MENG Hui-lin; JIN Xun-bo; LI Xiang-tie; WANG Hong-wei; L(U) Jia-ju

    2009-01-01

    group (P=0.001 and P=0.001), target antigen negative group (P=0.003 and P=0.001), and peak target antigen positive with negative at-transplant target antigen group (P=0.024 and P=0.002). Two-year graft survival rates of the target antigen positive group and HLA-Ⅰ target antigen positive group were significantly lower than the control group (P=0.012 and P=0.001). The two-year outcome of target antigen unknown group was similar to that of the target antigen positive group. Presensitizedrecipients with pre-transplant plasmapheresis or immunoadsorption (PRA prepared group) had a better but non-significant two-year outcome than the control group. However, the PRA unprepared presensitized recipients were different to the control group (P=0.004 for rejection rate and P=0.005 for survival rate). Hyperacute rejection (HR) occurred in three recipients with positive HLA-I target antigen and without mismatch according to Res M and in one case with positive PRA-Ⅱ (for an unknown target antigen). No HR occurred in eight cases with positive HLA-Ⅱ target antigens.Conclusions Pre-transplant PRA preparations might improve the access of presensitized patients to renal donors. Avoiding antigen-positive donors remains a fundamental measure in preventing HR and early rejections.

  20. Síndrome de Guillain Barré en pediatría Guillain-Barré syndrome in pediatrics

    Directory of Open Access Journals (Sweden)

    Ricardo Erazo Torricelli

    2009-01-01

    . AIDP is associated in 30-50% of cases with cranial nerve involvement, which is not observed in AMAN. MFS is characterized by ataxia, ophthalmoplegia and areflexia, but it may also present cranial nerve dysfunction. Recent data on the pathology and pathophysiology of GBS emphasize the important role of Campylobacter jejuni infection in generating anti-ganglioside antibodies (GM1 in AIDP, GQ1b in MFS and GD1a in AMAN, which damage myelin in AIDP and MFS and axons in AMAN. The differential diagnosis must rule out other disorders of the central nervous system (encephalitis, encephalomyelitis, myelitis, myasthenic syndromes, toxic neuropathies induced by heavy meals, drugs, chemical substances or animal toxins, and myopathic conditions, especially acute benign infectious myositis and neuromyopathy of the intensive care unit patient. It is important the treatment with immune globulin, at a total dose of 2 grams per kilogram administered over 48 hours. Plasmapheresis can be equally effective. GBS has a good prognosis in children with a total recovery in 85% of cases. Rehabilitation is crucial to attain a more rapid and global improvement.