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Sample records for chronic idiopathic thrombocytopenic

  1. Chronic idiopathic thrombocytopenic purpura: present strategy, guidelines and new insights

    NARCIS (Netherlands)

    W. Stevens; H. Koene; J.J. Zwaginga; G. Vreugdenhil

    2006-01-01

    Idiopathic thrombocytopenic purpura. (ITP) is an immune-mediated thrombocytopenia. The diagnosis is made after exclusion of other secondary causes of thrombocytopenic disorders. The primary treatment goal is to prevent severe bleeding rather than achieve normal platelet counts. In adults ITP usually

  2. Long-term outcomes of combined chemotherapy in chronic refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    TAO Jie; HUANG Ying; LI Hong-qiang; WANG Ting-ting; WANG Xiao-yan; JI Lin-xiang; YANG Ren-chi

    2007-01-01

    @@ Adult idiopathic thrombocytopenic purpura (ITP) is a chronic acquired organ-specific autoimmune hemorrhagic disease characterized by the production of auto-antibodies against antigens on the membranes of platelet, resulting in enhanced Fc-mediated destruction of the platelets by macrophages in the reticuloendothelial system.

  3. Treatment options for chronic idiopathic (immune) thrombocytopenic purpura.

    Science.gov (United States)

    George, J N

    2000-01-01

    The goal of treatment for idiopathic (immune) thrombocytopenic purpura (ITP) is to prevent serious bleeding. Traditionally, corticosteroids have been used as first-line therapy followed by splenectomy. Experience with splenectomy over 60 years shows that approximately two thirds of patients achieve normal platelet counts during the initial observation, but that thrombocytopenia often recurs with longer follow-up. We know that long-term use of corticosteroids can lead to significant morbidities; there is no consensus regarding the appropriate timing or indications for splenectomy. To address the Issue of appropriate use of splenectomy, we designed a multicenter clinical trial that will randomize patients to either standard care, involving prednisone followed by splenectomy, or to a novel regimen of limited prednisone treatment followed by WinRho SDF (Nabi, Boca Raton, FL) (anti-D) therapy to maintain the platelet count in a safe range for 1 year. Anti-D can be administered easily in an outpatient setting with few side effects and can provide predictable, transient increases in platelet count. The hypothesis is that prolonged maintenance therapy with a nontoxic regimen may increase the percentage of patients who will experience a spontaneous remission from thrombocytopenia, thereby avoiding an invasive and permanent surgical procedure, splenectomy, and its potentially life-threatening sequelae. PMID:10676922

  4. Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

    OpenAIRE

    Maryam Maghbool; Masood Maghbool; Mehdi Shahriari; Mehran Karimi

    2009-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori) infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was...

  5. Idiopathic thrombocytopenic purpura

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    L Kayal

    2014-01-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is defined as a hematologic disorder, characterized by isolated thrombocytopenia without a clinically apparent cause. The major causes of accelerated platelet consumption include immune thrombocytopenia, decreased bone marrow production, and increased splenic sequestration. The clinical presentation may be acute with severe bleeding, or insidious with slow development with mild or no symptoms. The initial laboratory tests useful at the first visit to predict future diagnosis were erythrocyte count, leukocyte count, anti-glycoprotein IIb/IIIa antibodies, reticulated platelets, plasma thrombopoietin level. Treatment should be restricted to those patients with moderate or severe thrombocytopenia who are bleeding or at risk of bleeding. We present a case report on ITP with clinical presentation, diagnosis and management.

  6. Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

    Science.gov (United States)

    Maghbool, Maryam; Maghbool, Masood; Shahriari, Mehdi; Karimi, Mehran

    2009-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori) infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was ten years). A specially-designed data sheet was used to record information on age, sex, duration of disease, family history of bleeding disorders, previous treatments and median platelet count. In patients with H. pylori infection, antimicrobial treatment consisted of amoxicillin, metronidazol and omeprazol. Response was assessed every month for one year and defined as complete (platelet count >150×10(9)/L) or partial (platelet count between 50 and 150×10(9)/L). We detected H. pylori infection in 5 patients. In 4 of them increased platelet count was seen during one year of follow-up and in one patient the platelet count was acceptable during six months. Although the pathological mechanism of H. pylori-induced thrombocytopenia was unclear in our patient sample, the assessment of H. pylori infection and use of eradication therapy should be attempted in chronic and resistant ITP patients. PMID:21589818

  7. Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

    Directory of Open Access Journals (Sweden)

    Maryam Maghbool

    2009-07-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was ten years. A specially-designed data sheet was used to record information on age, sex, duration of disease, family history of bleeding disorders, previous treatments and median platelet count. In patients with H. pylori infection, antimicrobial treatment consisted of amoxicillin, metronidazol and omeprazol. Response was assessed every month for one year and defined as complete (platelet count >150x109/L or partial (platelet count between 50 and 150x109/L. We detected H. pylori infection in 5 patients. In 4 of them increased platelet count was seen during one year of follow-up and in one patient the platelet count was acceptable during six months. Although the pathological mechanism of H. pylori-induced thrombocytopenia was unclear in our patient sample, the assessment of H. pylori infection and use of eradication therapy should be attempted in chronic and resistant ITP patients.

  8. Treatment of Infantile Chronic Idiopathic Thrombocytopenic Purpura by Auxiliary Use of Auriculo-Acupoint Pressing

    Institute of Scientific and Technical Information of China (English)

    卢燕

    2001-01-01

    @@According to the TCM theories of Meridian and Pulse-Picture, the author probed in the treatment of idiopathic thrombocytopenic purpura (ITP) with auriculo-acupoint pressing (AAP) and obtained good result. The study was reported as follows. METHODS General Materials Forty-five patients with chronic refractory of ITP selected from the 269 ITP in-patients, hospitalized from January 1991 to January 1998, were observed. They were diagnosed according to the clinical manifestations, peripheral blood picture and bone marrow examination, as well as platelet antibody test in some of them, which were all in accordance with the unified diagnostic standard of ITP in China(1). All of the patients had course of disease over half a year and their disease treated with hormone for 2-3 months ineffectively, and had hemorrhagic symptoms, such as dermatorrhagia and rhinorrhagia, with no hepatosplenomegaly, and platelet count within 8-72×109/L. The hormone therapy was withdrawn or stopped gradually in the observation period.

  9. Impact of Helicobacter pylori Eradication Therapy on Platelet Counts in Patients With Chronic Idiopathic Thrombocytopenic Purpura.

    Science.gov (United States)

    Amiri, Mohamadreza

    2016-01-01

    This study was a before and after clinical evaluation of Helicobacter pylori eradication on platelet counts in a group of 23 patients with chronic Idiopathic (Autoimmune) thrombocytopenic purpura (CITP). H. pylori infection was identified in patients by a (13)C-urea breath test and confirmed by an H. pylori stool antigen test. Eradication was conducted in patients testing positive. Infected (n = 10) and uninfected (n = 13) patient groups did not differ with respect to age, gender, history of previous splenectomy, treatment with anti-D, current treatment with corticosteroids, or initial platelet counts. H pylori eradication was successful in eight infected CITP patients, with two patients not responsive to treatment. Compared to the uninfected group, patients in the infected group who responded to eradication therapy had significantly increased platelet counts after six months (56.2 ± 22.2 vs. 233 ± 85.6 ×10(3) million cells/L; P < 0.01), whereas platelet counts in the non-responding patients and uninfected group did not differ after this period of time. H. pylori eradication promotes significant platelet count improvement in patients with CITP. Thus, all patients with CITP should be tested and treated for H. pylori infections. PMID:26925898

  10. The Effect of Costimulatory Factors in the Pathogenesis of Chronic Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    崔国惠; 刘筱萍; 姚军霞

    2003-01-01

    To investigate the effect of costimulatory factors in the pathogenesis of chronic idiopathic thrombocytopenic purpura (CITP), we examined the expression of CD80 on platelets and megakaryocytes in patients with CITP and the controls by FACS. By using CD80 monoclonal antibody (McAb) to inhibit interaction among cells which is mediated by costimulatory factors, we observed the effect of CD80 McAb on the growth and maturation of megakaryocytic progenitors of patients with CITP in vitro. The results showed the expression of CD80 on platelets and megakaryocytes in CITP group was significantly higher than that in controls (P<0.01). There was a significantly positive correlation between the expression of CD80 on platelets and serum PAIgG in CITP (r =0.86, P<0. 05). The mean of various clone numbers (CFU-MK, BFU-MK and mCFU-MK) in CITP were all lower than those in controls (P<0. 05). In megakaryoeytes co-cultured with CD80 McAb, there was an increasing tendency of the number of CFU-MK and big CFU-MK (the number of megakaryocyte with GPⅢa positive was more than 20) and mediate CFU-MK (the number of megakaryocyte with GPⅢa positive was 11- 20). When the concentration of CD80 McAb was 10 μg/L, there was a significant difference in the number of megakaryocytic colony formation (CFUMK, BFU-MK and mCFU-MK) between the group with CD80 McAb and that without it (P<0.05). These showed the abnormality of costimulatory factors had important effect in the pathogenesis of CITP.

  11. Pulmonary hyalinizing granuloma. Bilateral pulmonary nodules associated with chronic idiopathic thrombocytopenic purpura.

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    Satti, Mohamed B; Batouk, Abdelnasir A; Abdelaziz, Muntasir M; Ahmad, Mohamed F; Abdelaal, Mohamed A

    2005-09-01

    We report a case of a 30-year-old female who had been treated periodically with steroids for idiopathic thrombocytopenic purpura ITP over the last 10 years. Recently, during the course of investigation, she was found to have incidental asymptomatic multiple pulmonary nodules on chest CT. Following a needle biopsy to exclude malignancy, 2 nodules were excised and were histologically confirmed as pulmonary hyalinizing granuloma PHG. The remaining 2 nodules regressed on increasing her dose of steroids. The case is discussed with emphasis on the histological and radiological differential diagnosis, in addition to including ITP among the spectrum of immunologic conditions associated with PHG. PMID:16155671

  12. Chronic idiopathic thrombocytopenic purpura in adult Chinese patients: a retrospective single-centered analysis of 1791 cases

    Institute of Scientific and Technical Information of China (English)

    LI Hong-qiang; ZHANG Lei; ZHAO Hui; JI Lin-xiang; YANG Ren-chi

    2005-01-01

    Background Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with chronic ITP. This study aimed at defining the treatment outcome and prognostic factors for chronic ITP based on a large cohort of Chinese patients followed up for over 25 years at a single center.Methods The medical records of 1791 patients aged 14 years or older who were diagnosed as having chronic ITP at our hospital from 1974 to 1999 were retrospectively analyzed.Conclusions Adult Chinese chronic ITP patients can have long-term remission after steroid therapy and splenectomies. Primary steroid refractoriness is a prognostic factor predicting poor subsequent response to a splenectomy.

  13. Eltrombopag for the treatment of chronic immune or idiopathic thrombocytopenic purpura: a NICE single technology appraisal.

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    Boyers, Dwayne; Jia, Xueli; Jenkinson, David; Mowatt, Graham

    2012-06-01

    The National Institute for Health and Clinical Excellence (NICE) invited the manufacturer of eltrombopag (GlaxoSmithKline) to submit evidence for the clinical and cost effectiveness of this drug for the treatment of patients with chronic immune or idiopathic thrombocytopenic purpura (ITP), as part of the their Single Technology Appraisal (STA) process. The Aberdeen Technology Assessment Review (TAR) Group, commissioned to act as the evidence review group (ERG), critically reviewed and supplemented the submitted evidence. This paper describes the company submission, the ERG review and NICE's subsequent decisions. The ERG critically appraised the clinical and cost-effectiveness evidence submitted by the manufacturer, independently searched for relevant literature, conducted a critical appraisal of the submitted economic models and explored the impact of altering some of the key model assumptions as well as combining relevant sensitivity analyses. Three trials were used to inform the safety and efficacy aspects of this submission; however, one high-quality randomized controlled trial (RAISE study) was the principal source of evidence and was used to inform the economic model. Eltrombopag had greater odds of achieving the primary outcome of a platelet count between 50 × 10^⁹/L and 400 × 10^⁹/L during the 6-month treatment period than placebo (odds ratio [OR] 8.2, 99% CI 3.6, 18.7). In the eltrombopag group, 50/83 (60%) of non-splenectomized patients and 18/49 (37%) of splenectomized patients achieved this outcome. The median duration of response was 10.9 weeks for eltrombopag (splenectomized 6 and non-splenectomized 13.4) compared with 0 for placebo. Eltrombopag patients required less rescue medication and had lower odds of bleeding events for both the splenectomized and the non-splenectomized patients. For a watch-and-rescue strategy of care, the comparator was placebo and the ERG found that substantial reductions in the cost of eltrombopag are needed

  14. Crohn's colitis and idiopathic thrombocytopenic purpura

    OpenAIRE

    Boyne, M.; Dye, K.

    2000-01-01

    A 17 year old girl with active Crohn's colitis developed idiopathic thrombocytopenic purpura that was managed with intravenous immune globulins and cyclosporin A. The possible association between Crohn's disease and immune thrombocytopenia is explored.


Keywords: Crohn's disease; colitis; thrombocytopenia

  15. Hematologic case: Idiopathic thrombocytopenic purpura

    OpenAIRE

    São Simão, T.; Salgado, M.; Costa, E.; Barbot, J.

    2012-01-01

    The immune thrombocytopenic purpura (ITP) is a controversial disease. The generality of the literature argues that a historical objective clinical examination and a blood count with careful observation of the peripheral blood smear is sufficient for diagnosis. Some cases contradict this belief.

  16. Evaluation of humoral immune function in patients with chronic idiopathic thrombocytopenic purpura.

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    Mohammad Saeid Rahiminejad

    2013-03-01

    Full Text Available Coincidence of autoimmune diseases such as immune thrombocytopenic purpura (ITP with  immunodeficiencies has  been  reported  previously in  patients  who  suffered  from primary antibody deficiency (PAD. But there is no original study on immunological profiles of ITP patients to find out their probable immune deficiency.In this case-control study, ITP patients’ humoral immunity was investigated for diagnosis of PAD in comparison with normal population. To evaluate the humoral immune system against polysaccharide antigens, patients’ serum immunoglobulin levels were measured and a 23-valent pneumococcal  capsular polysaccharide vaccine (PPV23 was administrated  to evaluate the antibody response to vaccination.In  this  study, 14 out  of  36 patients  (39% were diagnosed with antibody mediated immune deficiency including 2 patients (5.5% with immunoglobulin class deficiency and 4 (11% with IgG subclass deficiency. The remaining patients suffered from specific antibody deficiency. The most frequent deficiency in ITP patients was specific antibody deficiency.Therefore, immunological survey on ITP patients may be important especially for those who have undergone splenectomy.

  17. Initial management of adults with idiopathic (immune) thrombocytopenic purpura.

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    George, J N

    2002-03-01

    Since idiopathic (immune) thrombocytopenic purpura (ITP) in adults is usually a chronic condition with few spontaneous remissions, the goal of treatment is not cure, but to maintain a hemostatically safe platelet level. The indication for treatment should be based not merely on platelet counts, but also clinical indices of bleeding. Although most patients show good initial response to prednisone, the side effects of steroids limit this treatment. Currently, long-term management usually involves splenectomy. Since splenectomy has surgical risks and may also predispose the patient to sepsis, a clinical trial using anti-D (WinRho-SDR) has been performed to determine whether this treatment can safely delay or avoid the need for surgery. The use of WinRho may also reveal the occurrence of spontaneous remissions, a previously unrecognized subgroup of adults with chronic ITP. PMID:11913992

  18. Dieulafoy Lesion in the Ascending Colon Presenting with Gastrointestinal Bleeding and Severe Anemia Complicated by a Coexisting Severe Resistant Chronic Idiopathic Thrombocytopenic Purpura

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    Sherif Ali Eltawansy

    2014-01-01

    Full Text Available Background. GI (gastrointestinal bleeding can be due to a variety of etiologies ranging from being common like bleeding peptic ulcer disease or esophageal varices. One of the rarely documented causes is the Dieulafoy lesion which is known as an abnormally large ectatic artery that penetrates the gut wall, occasionally eroding through the mucosa causing massive bleeding. In addition to that, we refer to the uncommon presentation of Dieulafoy lesion itself as it is well known to be found in the stomach, esophagus, duodenum, and jejunum but not the ascending colon as in our case. The patient had a coexisting ITP (idiopathic thrombocytopenic purpura that was resistant to different therapies. Case Report. We report a case of a 48-year-old Egyptian female known for chronic ITP resistant to treatment. The patient presented with bright red bleeding per rectum and severe life threatening anemia. Endoscopic study showed a Dieulafoy lesion. Endoscopic clipping was successful in controlling the bleeding. Conclusion. Dieulafoy lesion is a rare reason for GI bleeding and can present in common or unexpected places. Also extreme caution should be used in patients with bleeding tendency due to different reasons, like ITP in our case.

  19. Evaluation of the effects of and earliest response rate to anti-D treatment in children with chronic idiopathic thrombocytopenic purpura: a pilot study.

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    Yetgin, Sevgi; Aytaç, Selin; Olcay, Lale; Tunç, Bahattin; Ozbek, Namik; Aydinok, Yeşim

    2010-01-01

    In this pilot study, 30 (14 male, 16 female; median age: 8 years, range: 2-18) chronic non-splenectomized idiopathic thrombocytopenic purpura (ITP) patients with Rh+ blood group and their 49 attacks were evaluated after intravenous (i.v.) anti-D (WinRho SDF, Cangene Corporation, Winnipeg, MB, Canada) treatment at a dose of 50 microg/kg x 3 days (n = 21 cases; 35 attacks) or a single dose of 75 microg/kg (n = 9 cases; 14 attacks) to define the hemostatic dose of anti-D. Five of 30 patients (22/49 attacks) were resistant to steroid, intravenous immunoglobulin (IVIG) and vincristine treatment. Hemoglobin (Hb), white blood cells (WBC), platelets (plt) and reticulocytes (ret) were evaluated before and after treatment during the follow-up in sequences on the 1st, 7th, 14th and 21st days after anti-D treatment if the patients had no symptom. All patients, even the resistant ones, experienced an increase in plt count to provide protection from bleeding (> or = 20 x 10(9)/L in patients with symptoms, > or = 10 x 10(9)/L in patients without symptoms). The plt responses of one resistant and five non-resistant patients treated with a single 75 microg/kg dose of i.v. anti-D in 8 attacks were monitored at the 2nd, 4th, 8th, 24th and 48th hours of the treatment. A protective plt level was attained within 2 hours in 6 attacks of five non-resistant cases and in 24 hours in the remaining 2 attacks of one resistant case. This pilot study suggests that anti-D treatment in ITP patients is effective and can increase plt to a level adequate enough to protect from hemorrhage within 2 hours, when given in a 75 microg/kg dose. A few adverse events (i.e. chills, hemolysis and hemoglobinuria) resolved without intervention. PMID:20560246

  20. The European Medicines Agency review of eltrombopag (Revolade) for the treatment of adult chronic immune (idiopathic) thrombocytopenic purpura: summary of the scientific assessment of the Committee for Medicinal Products for Human Use.

    Science.gov (United States)

    Nieto, Maria; Calvo, Gonzalo; Hudson, Ian; Feldschreiber, Peter; Brown, David; Lee, Ching Cheng; Lay, Geoffrey; Valeri, Anna; Abadie, Eric; Thomas, Angela; Pignatti, Francesco

    2011-09-01

    On 11(th) March 2010, the European Commission issued a marketing authorization valid throughout the European Union for Revolade for the treatment of adult chronic immune (idiopathic) thrombocytopenic purpura. Revolade is an orphan medicinal product indicated for splenectomized patients with immune (idiopathic) thrombocytopenic purpura who are refractory to other treatments (e.g. corticosteroids, immunoglobulins) and as second-line treatment for non-splenectomized patients where surgery is contraindicated. The active substance of Revolade is eltrombopag (ATC code B02BX05). Eltrombopag increases platelet production through activation of the thrombopoietin receptor. The recommended oral dose is 50 mg once daily to achieve and maintain a platelet count of the 50×10(9)/L or more necessary to reduce or prevent the risk of bleeding. The benefit of Revolade is a durable response in maintaining platelet levels. The most common side effects include headache, nausea, hepatobiliary toxicity, diarrhea, fatigue, paresthesia, constipation, rash, pruritus, cataract, arthralgia and myalgia. The decision to grant the marketing authorization was based on the favorable recommendation of the Committee for Medicinal Products for Human Use of the European Medicines Agency. The objective of this paper is to describe the data submitted to the European Medicines Agency and to summarize the scientific review of the application. The detailed scientific assessment report and product information, including the summary of product characteristics, are available on the European Medicines Agency website (www.ema.europa.eu). PMID:21712542

  1. Pregnancy and Birth Outcomes among Women with Idiopathic Thrombocytopenic Purpura

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    Diego F. Wyszynski

    2016-01-01

    Full Text Available Objective. To examine pregnancy and birth outcomes among women with idiopathic thrombocytopenic purpura (ITP or chronic ITP (cITP diagnosed before or during pregnancy. Methods. A linkage of mothers and babies within a large US health insurance database that combines enrollment data, pharmacy claims, and medical claims was carried out to identify pregnancies in women with ITP or cITP. Outcomes included preterm birth, elective and spontaneous loss, and major congenital anomalies. Results. Results suggest that women diagnosed with ITP or cITP prior to their estimated date of conception may be at higher risk for stillbirth, fetal loss, and premature delivery. Among 446 pregnancies in women with ITP, 346 resulted in live births. Women with cITP experienced more adverse outcomes than those with a pregnancy-related diagnosis of ITP. Although 7.8% of all live births had major congenital anomalies, the majority were isolated heart defects. Among deliveries in women with cITP, 15.2% of live births were preterm. Conclusions. The results of this study provide further evidence that cause and duration of maternal ITP are important determinants of the outcomes of pregnancy.

  2. Dameshek W, Miller EB. The megakaryocytes in idiopathic thrombocytopenic purpura, a form of hypersplenism. 1946.

    Science.gov (United States)

    2016-01-01

    This paper, by one of the legends of hematology, William Dameshek, and his colleague Edward Miller, is from the inaugural issue of Blood. By studying bone marrow specimens from controls, patients with acute or chronic immune thrombocytopenia, or patients with other thrombocytopenic disorders, the authors concluded that, in idiopathic thrombocytopenic purpura (ITP), production of platelets from megakaryocytes is defective, even while marrow megakaryocytes are greatly increased in number. This defect resolved after splenectomy. The authors appropriately credit E. Frank with having proposed defective platelet production from megakaryocytes in ITP in 1915. The idea that platelet production was defective in ITP was superseded or ignored for decades, but it has now been validated by the therapeutic effectiveness of the thrombopoietin mimetics in ITP.

  3. Studies on megakaryopoiesis in patients with myelodysplasia and idiopathic thrombocytopenic purpura

    NARCIS (Netherlands)

    Houwerzijl, Ewout Johan

    2008-01-01

    In this thesis mechanisms of thrombocytopenia (a low number of platelets) in patients with myelodysplastic syndromes (MDS) or idiopathic thrombocytopenic purpura (ITP) were investigated. Thrombocytopenia can cause serious bleeding complications and elucidation of the underlying pathophysiology of th

  4. Current Concept for the Diagnosis and Treatment of Adult Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    侯明

    2005-01-01

    @@ Idiopathic thrombocytopenic purpura (ITP) is a common hematologic disorder manifested by immunemediated thrombocytopenia. The estimated incidence ranges from 50 ~ 100 per million each year in western countries, roughly divided between adults and children.

  5. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

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    Joji Nagasaki

    2016-01-01

    Full Text Available The etiologies of secondary idiopathic thrombocytopenic purpura (ITP include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP eradication or corticosteroid treatment, all of the patients’ platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations.

  6. Flow cytometric analysis of anti-platelet antibodies in idiopathic thrombocytopenic purpura.

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    Latorraca, A; Lanza, F; Moretti, S; Ferrari, L; Reverberi, R; Galluccio, L; Castoldi, G

    1994-01-01

    Anti-platelet antibody measurement may be important in defining the pathogenesis of thrombocytopenic states. In this paper we compared three anti-human immunoglobulin reagents by using them to detect anti-platelet antibodies on the platelet surface and in the serum of 14 patients with chronic idiopathic thrombocytopenic purpura (ITP) and 22 thrombocytopenic disorders. Samples were analyzed by both flow cytometry and a fluorescence microscope. In ITP patients, the direct test was positive in 50% of the cases, while the indirect technique proved to be positive in a slightly higher number of those tested (56%). Furthermore, the number of positive cases was similar for the three reagents used in this study, although the mean percentage of positive platelets was higher for the kappa/lambda monoclonal reagent. These data further support the sensitivity and reproducibility of flow cytometry analysis, which was capable of detecting antiplatelet antibodies in all patients with transfused Cooley's disease (regarded as positive control), as well as in a significant number of patients with ITP or related diseases. On the basis of the data presented here, definitive proof regarding the presence of anti-platelet antibodies in patients with thrombocytopenia still has to be found, and further studies are needed in order to ascertain the autoimmune nature of these disorders. PMID:7926978

  7. [Wilson's disease associated with olfactory paranoid syndrome and idiopathic thrombocytopenic purpura].

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    Sagawa, Morihiko; Takao, Masaki; Nogawa, Shigeru; Mizuno, Masafumi; Murata, Mitsuru; Amano, Takahiro; Koto, Atsuo

    2003-10-01

    In this study we report an individual of Wilson's disease associated with olfactory paranoid syndrome and idiopathic thrombocytopenic purpura. The initial symptom of this female patient was olfactory paranoia at age 17. Although that psychiatric symptom was well controlled under pharmacological treatment for two years, she developed olfactory paranoia as well as sialorrhea, dysarthria and finger tremor at age 20. A year later rigidity was also present in the extremities. At age 23, idiopathic thrombocytopenic purpura was found based on hematological examinations. Because her extrapyramidal symptoms were progressive, she was referred to our department to evaluate her neurologic condition. She was diagnosed as having Wilson's disease based on (1) the presence of Kayser-Fleischer rings, (2) extrapyramidal signs, and (3) a decreased level of serum copper and ceruloplasmin. T2 and FLAIR images of brain MRI showed hyperintense lesions in the putamen, thalamus and pontine tegmentum. Diffusion-weighted images also showed hyperintense lesions in the thalamus and pontine tegmentum. The biopsy specimen of the liver revealed chronic hepatitis with copper accumulation. Since D-penicillamine treatment was initiated, she has shown no olfactory paranoia and exacerbation of ITP. Her gait disturbance has also improved. Olfactory paranoia and ITP are rare clinical complications of Wilson's disease. Further analysis may warrant consideration of the pathophysiological mechanism of the psychiatric, hematological and neuroradiological condition seen in Wilson's disease.

  8. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

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    Christopher Coleman

    2014-01-01

    Full Text Available Pulmonary hyalinizing granuloma (PHG is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP in our patient, may also favor HG. In this case report we find an association between PHG and ITP.

  9. Pulmonary hyalinizing granuloma associated with idiopathic thrombocytopenic purpura.

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    Coleman, Christopher; Nassar, Aziza; McComb, Barbara

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP) in our patient, may also favor HG. In this case report we find an association between PHG and ITP. PMID:24744965

  10. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    Science.gov (United States)

    Coleman, Christopher; Nassar, Aziza; McComb, Barbara

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP) in our patient, may also favor HG. In this case report we find an association between PHG and ITP. PMID:24744965

  11. Peliosis hepatis presenting with massive hepatomegaly in a patient with idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Kim, Sun Bean; Kim, Do Kyung; Byun, Sun Jeong; Park, Ji Hye; Choi, Jin Young; Park, Young Nyun; Kim, Do Young

    2015-12-01

    Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months. PMID:26770928

  12. Rapid encephalopathy associated with anti-D immune globulin treatment for idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Golla, Sunitha; Horkan, Clare; Dogaru, Grigore; Teske, Thomas E; Christopher, Kenneth

    2008-01-01

    Rho (D) immune globulin intravenous (IV RhIG, WinRho SDF) has been shown to be a safe treatment for idiopathic thrombocytopenic purpura. Common side effects of IV RhIG include mild hemolysis, febrile reaction and headache. Significant hemolysis with renal impairment is infrequently noted. A single case of irreversible encephalopathy following IV RhIG has been reported. We report a second case of encephalopathy following an infusion of IV RhIG for treatment of idiopathic thrombocytopenic purpura. PMID:18957844

  13. Idiopathic chronic eosinophilic pneumonia

    OpenAIRE

    Cordier Jean-François; Marchand Eric

    2006-01-01

    Abstract Idiopathic chronic eosinophilic pneumonia (ICEP) is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or blood eosinophilia, and peripheral pulmonary infiltrates on chest imaging. Eosinophilia is present in most cases, usually in excess of 1000/mm3. In absence of significant blood eosinophilia, a diagnosis of ICEP is supported by the demonstration of bronchoalveolar lavage eosinophilia. ICEP is typically associated with eosinophil counts higher than ...

  14. Ultrastructural study shows morphologic features of apoptosis and para-apoptosis in megakaryocytes from patients with idiopathic thrombocytopenic purpura

    NARCIS (Netherlands)

    Houwerzijl, EJ; Blom, NR; van der Want, JJL; Esselink, MT; Koornstra, JJ; Smit, JW; Louwes, H; Vellenga, E; de Wolf, JTM

    2004-01-01

    To investigate whether altered megakaryocyte morphology contributes to reduced platelet production in idiopathic thrombocytopenic purpura (ITP), ultrastructural analysis of megakaryocytes was performed in 11 ITP patients. Ultrastructural abnormalities compatible with (para-)apoptosis were present in

  15. Splenectomy in children with idiopathic thrombocytopenic purpura : A prospective study of 134 children from the Intercontinental Childhood ITP Study Group

    NARCIS (Netherlands)

    Kuehne, Thomas; Blanchette, Victor; Buchanan, George R.; Ramenghi, Ugo; Donato, Hugo; Tamminga, Rienk Y. J.; Rischewski, Johannes; Berchtold, Willi; Imbach, Paul

    2007-01-01

    Background. Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited. Procedure. Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in

  16. A Case Associated with Comorbidities Among Cerebral Infarction, Idiopathic Thrombocytopenic Purpura, and Triple X Syndrome

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    Hanjun Kim

    2014-06-01

    Full Text Available A 46-year-old female presented to the emergency room due to the chief complaint of left-sided weakness. By imaging study, she was diagnosed with cerebral infarction. Thrombolytic and antiplatelet agents were not considered due to the “golden hour” for treatment having passed and a low platelet count. The peripheral blood smear, bone marrow biopsy, and aspirate findings were consistent with immune thrombocytopenic purpura. The chromosome analysis revealed the 47,XXX karyotype. To the best of our knowledge, this is the first case report associated with the comorbidities of cerebral infarction, idiopathic thrombocytopenic purpura, and triple X syndrome.

  17. The Relationship between Self-esteem and Quality of Life of Patients with Idiopathic Thrombocytopenic Purpura at Isfahan's Sayed Al-Shohada Hospital, Iran, in 2013

    OpenAIRE

    Hemati, Zeinab; Kiani, Davood

    2016-01-01

    Background: Idiopathic thrombocytopenic purpura (ITP) is a chronic disease which is accompanied with hopelessness and loss of the sense of well-being due to its symptoms and treatment. It also affects patients' sense of social and spiritual well-being. This disorder decreases patients' self-esteem and their quality of life by changing their mental image and self-confidence. This study was performed to find the relationship between self-esteem and quality of life of patients with ITP. Subjects...

  18. Rapid irreversible encephalopathy associated with anti-D immune globulin treatment for idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Christopher, Kenneth; Horkan, Clare; Barb, Ilie T; Arbelaez, Christian; Hodgdon, Travis A; Yodice, Paul C

    2004-11-01

    Intravenous Rho (D) immune globulin (IV RhIG, WinRho SDF) has been shown to be a safe treatment for idiopathic thrombocytopenic purpura (ITP). Common side effects of IV RhIG include mild hemolysis, febrile reaction, and headache. Significant hemolysis with renal impairment following IV RhIG has been reported. We report a case of irreversible encephalopathy 48 hr following an infusion of IV RhIG for treatment of ITP. PMID:15495245

  19. The spleen and splenectomy in immune (idiopathic) thrombocytopenic purpura.

    Science.gov (United States)

    Sandler, S G

    2000-01-01

    The benefits of surgical splenectomy in patients with immune (Idiopathic) thrombocytopenia purpura (ITP) probably reflect the combined effects of eliminating a source of antiplatelet antibody synthesis as well as the primary site of platelet destruction. The recent availability of intravenous Rho(D) Immune globulin (WinRho SDF; Nabi, Boca Raton, FL) presents an opportunity to extend the duration of nonsurgical (spleen-sparing) management of chronic ITP by inducing reversible Fc blockade. While new methods for laparoscopic splenectomy may offer improved surgical outcomes and reduced costs for ITP patients in the near-term, the long-term consequences of splenectomy remain to be determined. Partial splenectomy has been shown to be effective in the management of anemia in hereditary spherocytosis and elliptocytosis, while preserving vital splenic phagocytic and immune functions. The concept that cell destruction occurs in reticuloendothelial cells has been updated with recognition that the mononuclear phagocyte is neither a reticular nor an endothelial cell. Immune phagocytosis is now understood to be mediated by macrophage IgG Fc and complement receptors. A key factor for devising a strategy for selecting medical or surgical splenectomy, or postponing splenectomy, is an assessment of the relative importance of splenic immune versus phagocytic function in the pathogenesis of ITP. PMID:10676918

  20. An Ischemic Stroke Related to Eltrombopag Use in a Patient with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Demet Arslan

    2016-03-01

    Full Text Available Thrombopoetin receptor agonists like eltrombopag, used for Idiopathic Thrombocytopenic Purpura treatment rarely cause to the ischemic cerebrovascular disease. In this re­port, a patient, have been followed by ITP diagnosis for 5 years, admitted to the emergency service for right hemi­paresis and aphasia. It was diagnosed as ischemic cere­brovascular disease. Etiology of patient’s cerebrovascu­lar event was associated with eltrombopag. Because of this rare seen situation, it was argued by in the light of the literature.

  1. Crohn’s Disease and Idiopathic Thrombocytopenic Purpura in a Patient with Ectodermal Dysplasia and Immunodeficiency

    Directory of Open Access Journals (Sweden)

    Farzaneh Motamed

    2006-09-01

    Full Text Available In this case report we will describe a rare association between anhyrotic ectodermal dysplasia (AED and immunodeficiency and autoimmunity [in our case: Idiopathic Thrombocytopenic Purpura (ITP and Crohn disease]. AED is a rare congenital disorder characterized by sparse hair, abnormal teeth and anhidrosis due to lack of eccrine glands. The survey of 87 cases with (AED revealed only one Irritable Bowel Disease (IBD.  AED has only two relevancies with immunodeficiency: (EDA-ID: Ectodermal Dysplasia Anhyrotic with Immunodeficiency and APE-CED (Autoimmune polyendocrinopathy, Candidiasis and Ectodermal Dysplasia that in our case EDA-ID is strongly suspected.

  2. Idiopathic chronic eosinophilic pneumonia

    Directory of Open Access Journals (Sweden)

    Cordier Jean-François

    2006-04-01

    Full Text Available Abstract Idiopathic chronic eosinophilic pneumonia (ICEP is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or blood eosinophilia, and peripheral pulmonary infiltrates on chest imaging. Eosinophilia is present in most cases, usually in excess of 1000/mm3. In absence of significant blood eosinophilia, a diagnosis of ICEP is supported by the demonstration of bronchoalveolar lavage eosinophilia. ICEP is typically associated with eosinophil counts higher than lymphocyte counts in the bronchoalveolar lavage. ICEP is a rare disorder of unknown cause. Its exact prevalence remains unknown. ICEP may affect every age group but is rare in childhood. It is twice as frequent in women as in men. One third to one half of the ICEP patients have a history of asthma. The mainstay of treatment of ICEP is systemic corticosteroids. Response to oral corticosteroid therapy is dramatic and has led to the consideration of corticosteroid challenge as a diagnostic test for ICEP. Nevertheless, relapses or development of severe asthma are frequent when tapering or withdrawing treatment. Long-term oral corticosteroid therapy is necessary in up to half of the patients.

  3. Regulatory T Cells in Patients with Idiopathic Thrombocytopenic Purpura

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    Alev Akyol Erikçi

    2016-05-01

    Full Text Available Objective: Immune thrombocytopenic purpura (ITP is an immunemediated bleeding disorder in which platelets are opsonized by autoantibodies and destroyed by an Fc receptor-mediated phagocytosis by the reticuloendothelial system within the spleen. Autoimmune processes are also considered in the pathogenesis of this disorder. CD4+CD25+FoxP3+ regulatory T (Treg cells and CD8+CD28- Treg cells have roles in autoimmune diseases. We investigated these regulatory cells in ITP patients. Materials and Methods: We included 22 ITP patients and 16 age-matched healthy subjects. CD4+CD25+FoxP3+ Treg cells and CD8+CD28- cells were investigated by three-color flow cytometry. The ratios of these cell populations to total lymphocytes were calculated. Statistical analysis was carried out with the Mann-Whitney U test. Results: CD4+CD25+ Treg cells were 9.69±3.70% and 12.99±5.58% in patients with ITP and controls, respectively. CD4+CD25highFoxP3+ cells were 27.72±19.74% and 27.55±23.98% in ITP patients and controls, respectively. The percentages of both of these cell types were not statistically significant when compared to the control group. Conclusion: We did not find any differences in ratios of CD4+CD25+FoxP3+ Treg cells or CD8+CD28- T cells in lymphocytes between patients and healthy subjects. We conclude that these circulatory cells are not different in ITP, but further studies are needed to explore the putative roles of these regulatory cells.

  4. Squamous cell carcinoma larynx presenting as idiopathic thrombocytopenic purpura

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    Bekur R

    2015-01-01

    Full Text Available Association of immune thrombocytpenic purpura with solid malignancy as paraneoplastic manifestation has been reported earlier mainly with lymphoma and breast cancer. We report the case of a patient with squamous cell carcinoma of the larynx presenting with idiopathic thombocytopenic purpura (ITP. A 67-year-old lady presented with multiple ecchymotic patches and petechiae all over the body and bleeding from oral cavity was found to have severe thrombocytopenia diagnosed as ITP with bone marrow evidence of peripheral destruction without infiltration of bone marrow. Five months later she was diagnosed to have squamous cell carcinoma of larynx. Platelet count improved after splenectomy.

  5. Splenectomy in patients with idiopathic thrombocytopenic purpura: Analysis of 109 cases

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    Enver Ay

    2012-03-01

    Full Text Available Objectives: Splenectomy is performed in order to provide the treatment in the patients with severe idiopathic thrombocytopenic purpura, refractory to medical treatment. In this study, we aimed to investigate the postoperatif and longterm outcomes in the patients who underwent splenectomy with the diagnosis of idiopathic thrombocytopenic purpura.Materials and Methods: Between 2001-2010 at Dicle University Medical Faculty, General Surgery Department, a retrospective review of the 109 patients who had undergone splenectomy for ITP was reviewed. Age, gender, presence of accessory spleens and location, duration of the operation, number of preoperative platelet tranfusion, number of preoperative and postoperative blood transfusion, length of hospital stay, long-term outcomes, morbidity and mortality were recorded.Results: The mean age was 37.10 ± 16.62 (16-72, and there were 88 (80.7% female and 21 (19.3% male patients. The mean operation time was 44.87 ± 10:32 (30-120 minutes. The average postoperative blood and preoperative platelet transfusion were 1.63 ± 0.85 (0-3 and 2.01 ± 0.71 (1-3 units, respectively. The accessory spleens were encountered in 20 (18.3% patients at the ultrasonographic examination. And also the accessory spleens were encountered in 23 (21.1% patients during operation and confirmed with histopathologic examination. The most common localization of accessory spleens were splenic hilus. The postoperative complications were occurred in 16 patients (14.7% and the most complication was atelectasia. The mean length of hospital stay was 4:56 ± 2:45 (2-12 days. Patients were followed for an average of 28 (9-48 months. At the follow-up period, 1 (0.9 % patient had died.Conclusion: Splenectomy can be performed safely in the treatment of the patients with idiopathic thrombocytopenic purpura unresponsive to medical treatment. Long-term good results can be obtained with splenectomy in these patients. The accessory spleens should not be

  6. Cytokine-induced killer cell therapy-associated idiopathic thrombocytopenic purpura: rare but noteworthy.

    Science.gov (United States)

    Fu, Xiaomin; Zhang, Yong; Gao, Quanli; Lin, Jizhen; Zhang, Qinxian; Xu, Benling; Song, Yongping

    2016-09-01

    Idiopathic thrombocytopenic purpura (ITP) is characterized by a diminished platelet count, an autoimmune condition with antibodies against platelets and an increased tendency to bleed. The association between ITP and solid tumors is uncommon. Cytokine-induced killer (CIK) cell therapy is a well tolerated and promising cancer treatment with minimal toxicity. For the first time, CIK cell therapy was reported to be followed by ITP. The mechanism through which CIK induces ITP remains unclear. Imbalanced ratio of Th cells, decreased numbers or impaired function of Treg cells and excessive secretion of cytokines inducing abnormal activation of B cells may be among the possible reasons. Therefore, a better understanding of this rare condition will require further investigation of these cases. PMID:27485074

  7. Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura

    Science.gov (United States)

    García-Méndez, Jorge; Carrillo-Casas, Erika M.; Rangel-Cordero, Andrea; Leyva-Leyva, Margarita; Xicohtencatl-Cortes, Juan; Arenas, Roberto; Hernández-Castro, Rigoberto

    2016-01-01

    Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis. PMID:27313917

  8. Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura

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    Jorge García-Méndez

    2016-01-01

    Full Text Available Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis.

  9. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura

    Science.gov (United States)

    Zheng, X. Long; Wu, Haifeng M.; Shang, Dezhi; Falls, Erica; Skipwith, Christopher G.; Cataland, Spero R.; Bennett, Charles L.; Kwaan, Hau C.

    2010-01-01

    Background Type G immunoglobulins against ADAMTS13 are the primary cause of acquired (idiopathic) thrombotic thrombocytopenic purpura. However, the domains of ADAMTS13 which the type G anti-ADAMT13 immunoglobulins target have not been investigated in a large cohort of patients with thrombotic thrombocytopenic purpura. Design and Methods Sixty-seven patients with acquired idiopathic thrombotic thrombocytopenic purpura were prospectively collected from three major U.S. centers. An enzyme-linked immunosorbent assay determined plasma concentrations of anti-ADAMTS13 type G immunoglobulins, whereas immunoprecipitation plus western blotting determined the binding domains of these type G immunoglobulins. Results Plasma anti-ADAMTS13 type G immunoglobulins from 67 patients all bound full-length ADAMTS13 and a variant truncated after the eighth TSP1 repeat (delCUB). Approximately 97% (65/67) of patients harbored type G immunoglobulins targeted against a variant truncated after the spacer domain (MDTCS). However, only 12% of patients’ samples reacted with a variant lacking the Cys-rich and spacer domains (MDT). In addition, approximately 37%, 31%, and 46% of patients’ type G immunoglobulins interacted with the ADAMTS13 fragment containing TSP1 2-8 repeats (T2-8), CUB domains, and TSP1 5-8 repeats plus CUB domains (T5-8CUB), respectively. The presence of type G immunoglobulins targeted against the T2-8 and/or CUB domains was inversely correlated with the patients’ platelet counts on admission. Conclusions This multicenter study further demonstrated that the multiple domains of ADAMTS13, particularly the Cys-rich and spacer domains, are frequently targeted by anti-ADAMTS13 type G immunoglobulins in patients with acquired (idiopathic) thrombotic thrombocytopenic purpura. Our data shed more light on the pathogenesis of acquired thrombotic thrombocytopenic purpura and provide further rationales for adjunctive immunotherapy. PMID:20378566

  10. Life-threatening autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura: successful seletive splenic artery embolization

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    matteo molica

    2016-04-01

    Full Text Available Selective splenic artery embolization (SSAE is a nonsurgical intervention characterized by the transcatheter occlusion of the splenic artery and/or its branch vessels using metallic coils or other embolic devices. It has been applied for the management of splenic trauma, hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia and splenic hemangioma. We hereby describe a case of a patient affected by idiopathic thrombocytopenic purpura (ITP and warm auto-immune hemolytic anemia (AIHA both resistant to immunosuppressive and biological therapies, not eligible for a surgical intervention because of her critical conditions. She underwent SSAE and achieved a hematologic complete response within a few days without complications. SSAE is a minimally invasive procedure to date not considered a standard option in the management of AIHA and ITP. However, following the progressive improvement of the techniques, its indications have been extended, with a reduction in morbidity and mortality compared to splenectomy in patients with critical clinical conditions. SSAE was a lifesaving therapeutic approach for our patient and it may represent a real alternative for the treatment of resistant AIHA and ITP patients not eligible for splenectomy.

  11. Acquired Idiopathic ADAMTS13 Activity Deficient Thrombotic Thrombocytopenic Purpura in a Population from Japan

    Science.gov (United States)

    Matsumoto, Masanori; Bennett, Charles L.; Isonishi, Ayami; Qureshi, Zaina; Hori, Yuji; Hayakawa, Masaki; Yoshida, Yoko; Yagi, Hideo; Fujimura, Yoshihiro

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity <5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01), while older patients were more likely to die during the TTP hospitalization (p<0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed. PMID:22427934

  12. Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.

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    Masanori Matsumoto

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a type of thrombotic microangiopathy (TMA. Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai ADAMTS13-deficient TTP (ADAMTS13 activity <5% were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01, while older patients were more likely to die during the TTP hospitalization (p<0.05. Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort and gender composition (60% to 100% female in the other cohort. We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.

  13. [Clinical significance of Helicobacter pylori in children with idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    Tang, Ying; Wang, Shu-Chun; Wang, Lu-Juan; Liu, Yong; Wang, Hai-Ying; Wang, Zhan-Ju

    2013-04-01

    This study was aimed to investigate the clinic significance of helicobacter pylori (HP) in children with idiopathic thrombocytopenic purpura (ITP). The infection of HP in 92 ITP children was determined by (13) C-Urea Breath Test, the same test was also performed on 66 healthy children. The 68 children infected with HP were randomly divided into 2 groups: single drug group treated only with corticosteroid and; combined drug group treated with corticosteroid and anti-helicobacter pylori treatment. The results showed that 68 patients infected with HP were found in 92 ITP children (74.7%), 26 patients infected with HP were observed in 66 healthy children (39.4%), which was lower than that in ITP children (74.7%, P helicobacter pylori therapy, the total effective rate and cure rate of ITP patients increased respectively from 73.5% to 94.1%, and the total recurrence rate (17.0%) was much lower than single drug group (47.1%, P helicobacter pylori group was higher than that in the single drug group (P helicobacter pylori therapy would help to improve the therapeutic efficacy and reduce the recurrence of ITP children.

  14. Vitamin D deficiency in chronic idiopathic urticaria.

    OpenAIRE

    2015-01-01

    Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urt...

  15. Vitamin D deficiency in chronic idiopathic urticaria.

    Science.gov (United States)

    Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

    2015-04-01

    Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

  16. CD16 and CD32 Gene Polymorphisms May Contribute to Risk of Idiopathic Thrombocytopenic Purpura.

    Science.gov (United States)

    Xu, Jiannan; Zhao, Liyun; Zhang, Yan; Guo, Qingxu; Chen, Hui

    2016-01-01

    BACKGROUND Epidemiological studies have evaluated the associations of CD16 158F>V and CD32 131H>R gene polymorphisms with the risk of idiopathic thrombocytopenic purpura (ITP). MATERIAL AND METHODS Published studies on CD16 158F>V and CD32 131H>R polymorphisms with susceptibility to ITP were systematically reviewed until April 1, 2014. The Cochrane Library Database, Medline, CINAHL, EMBASE, Web of Science, and Chinese Biomedical Database (CBM) were used to search for relevant studies and then a meta-analysis was conducted by using Stata 12.0 software in order to produce consistent statistical results. RESULTS In total, 10 clinical case-control studies with 741 ITP patients and 1092 healthy controls were enrolled for quantitative data analysis. Results of this meta-analysis suggest that CD16 158F>V polymorphism had strong correlations with the susceptibility to ITP under 5 genetic models (all PR polymorphism and the susceptibility to ITP (all P>0.05). Subgroup analysis by ethnicity revealed that CD16 158F>V polymorphism was associated with the increased risk of ITP among both Caucasian and non-Caucasian populations. Nevertheless, no statistically significant correlations between CD32 131H>R polymorphism and the risk of ITP were observed among Caucasians and non-Caucasians (all P>0.05). CONCLUSIONS Our findings indicate that CD16 158F>V polymorphism may contribute to the increased risk of ITP, whereas CD32 131H>R polymorphism may not be an important risk factor for ITP. PMID:27315784

  17. IL-23及其相关细胞因子在慢性特发性血小板减少性紫癜中的表达与作用探讨%Expression and Significance of Interleukin-23 and Its Related Cytokines in Chronic Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    黄颖; 李永志; 魏彩霞; 黎承平; 李维佳; 杨弘

    2011-01-01

    The aim of this study was to investigate the expression and immunologic regulation function of interleukin23 and its related cytokines in chronic idiopathic thrombocytopenic purpura(ITP) patients. Levels of cytokines in peripheral blood mononuclear cells(PBMNC) were detected by reverse-transcription real-time polymerase chain reaction in 30 patients with chronic ITP and 15 healthy volunteers. The quantity of IL-23, IL-12, IL-17 in serum was detected by enzyme-linked immunosorbent assay(ELISA). The results showed that low detectable mRNA levels of IL-23p19 ,IL-12p35,IL-27 and IL-12p40 were found in all patients and healthy persons. Trace of IL-17 mRNA were expressed in PBMNC of part of patients and normal controls. Levels of IL-12p35, IL-27, IL-17 mRNA between healthy persons and chronic ITP patients were not statistically different. Compared with normal controls, patients showed the lower expression levels of IL23p19 and IL-12p40 mRNA (p <0.01 ). The IL-12 levels of chronic ITP patients were significantly higher than that of normal controls (p<0.01). The IL-23 and IL-17 levels of chronic ITP patients were same to that of normal controls. It is concluded that the imbalance of T cell subsets in ITP patients mainly associated with IL-12, but not with IL-23 and IL-17.%本研究旨在探讨IL-23及其它的IL-12家族成员在慢性特发性血小板减少性萦癜(ITP)的表达及其免疫调节功能.运用反转录实时PCR方法检测30例慢性ITP患者和15例正常对照者外周血单个核细胞(PBMNC)中IL-23p19、IL-12p35、IL-12p40、IL-27、IL-17 mRNA的表达水平,用ELISA方法检测血浆IL-23、IL-12、IL-17含量并分析其在慢性ITP中的表达规律及与T亚群的关系.结果表明,慢性ITP患者及正常人PBMNC均低水平表达IL-23p19、IL-12p35、IL-27、IL-12p40 mRNA;部分患者及正常人PBMNC微量表达IL-17 mRNA.IL-12p35、IL-27、1L-17mRNA的表达水平与正常对照相比无显著差异,IL-23p19、IL-12p40 mRNA的表达水

  18. A Parturient with Chronic Immune Thrombocytopenic Purpura: Anaesthetic Management for Caesarean Section

    Directory of Open Access Journals (Sweden)

    Sushma KS

    2015-08-01

    Full Text Available Immune Thrombocytopenic Purpura (ITP accounts for 4-5% of cases of pregnancy with thrombocytopenia. Their clinical condition may deteriorate during pregnancy subjecting these patients at high risk of bleeding. We report anaesthetic management of a parturient with chronic ITP for caesarean section.

  19. Efficacy, safety, and dose response of intravenous anti-D immune globulin (WinRho SDF) for the treatment of idiopathic thrombocytopenic purpura in children.

    Science.gov (United States)

    Freiberg, A; Mauger, D

    1998-01-01

    We analyzed data from 20 children treated for acute or chronic idiopathic (immune) thrombocytopenic purpura (ITP) at a single institution to determine the relationship between dose of intravenous anti-D immune globulin (WinRho SDF; Nabi, Boca Raton, FL), increase in platelet count, and decrease in hemoglobin in the therapy of ITP. Higher doses of anti-D were clearly associated with a greater therapeutic response in the platelet count, with no increase in hemolysis for both acute and chronic ITP. A significant correlation was found between dose and peak increase in platelet count measured in the 14 days following administration. This effect was present for both acute ITP (17 infusions, P = .0001) and chronic ITP (30 infusions, P = .038). Although hemolysis was seen in nearly all infusions, with a median hemoglobin fall of 1.9 g/dL (range, 0 to 4.2), the decrease in hemoglobin was greater than 2.5 for only three infusions, and the largest fall in hemoglobin (4.2) was in a child with an underlying hemolytic anemia. Furthermore, for both acute and chronic ITP there was no relationship between the decrease in hemoglobin and the dose given (P = .22), nor between the increase in platelet count and fall in hemoglobin (P = .27). This analysis supports the use of higher doses of anti-D for the treatment of ITP, and demonstrates the need for a trial of high-dose anti-D (>100 microg/kg) in acute and chronic ITP. PMID:9523746

  20. Expression of peripheral Blood lymphocyte subpopulation in patients with acute and chronic idiopathic thrombocytopenic purpura%急慢性特发性血小板减少性紫癜患者外周血淋巴细胞亚群的表达

    Institute of Scientific and Technical Information of China (English)

    张红; 刘庆华; 田芳

    2014-01-01

    目的:检测急慢性特发性血小板减少性紫癜(ITP)患者外周血淋巴细胞亚群,并探讨其在发病机制中的作用。方法选择74例ITP患者及30例健康查体人员的外周血,采用流式细胞术检测淋巴细胞亚群(CD3+, CD3+CD4+, CD3+CD8+, CD4+/CD8+, CD19+)。结果①ITP患者淋巴细胞亚群CD3+CD4+为(34.15±8.55)%,正常对照组为(36.74±3.65)%, t=2.166, P=0.033;ITP患者CD4+/CD8+为(1.24±0.44)%,正常对照组为(1.48±0.25)%, t=3.582, P=0.001;ITP患者CD3+CD8+为(30.63±7.72)%, CD19+为(18.23±7.67)%,均显著高于正常对照组(P=0.000)。②急性ITP患者外周血CD19+细胞比例为(22.6±7.25)%,慢性ITP患者为(14.14±5.57)%,两者差异有统计学意义(t=5.677, P=0.000);而慢性ITP患者的CD3+细胞的比例为(76.02±11.00)%,急性ITP患者为(66.82±10.95)%, t=3.583, P=0.001。结论 ITP的发生不仅存在B淋巴细胞异常,也存在T淋巴细胞的异常。急性ITP患者以体液免疫功能亢进为主,慢性患者则表现为细胞免疫功能亢进为主。%Objective To detect the expression of peripheral blood lymphocyte subpopulation in patients with acute and chronic idiopathic thrombocytopenic purpura (ITP), and to investigate its influence on pathogenesis. Methods The expression of lymphocyte subpopulation (CD3+, CD3+CD4+, CD3+CD8+, CD4+/CD8+, CD19+) in the peripheral blood was analyzed using flow cytometry in 74 patients with ITP and 30 healthy people. Results ①CD3+CD4+of lymphocyte subpopulation in patients with ITP was (34.15±8.55)%, while that of the control group was (36.74±3.65)%(t=2.166, P=0.033). CD4+/CD8+of lymphocyte subpopulation in patients with ITP was (1.24±0.44)%, and that of the control group was (1.48±0.25)% (t=3.582, P=0.001). CD3+CD8+ and CD19+of lymphocyte subpopulation in patients with ITP were (30.63±7.72)%and (18.23±7.67)%, and they were all obviously higher than the control group (P=0.000).②The proportion of CD19+B cells in peripheral Blood of acute

  1. Partial splenic embolization combined with vincristine infusion for the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome: observation of its long-term efficacy

    International Nuclear Information System (INIS)

    Objective: To observe the long-term efficacy of partial spleen embolization combined with vincristine infusion in treating refractory idiopathic thrombocytopenic purpura (ITP) and Evans syndrome. Methods: During the period of 2000-2007, partial spleen embolization together with vincristine infusion was carried out in 30 patients with refractory idiopathic thrombocytopenic purpura (n=24) or Evans syndrome (n=6). Vincristine infusion (2 mg) via splenic artery was performed before partial spleen embolization procedure. The long-term effectiveness was observed and analyzed. Results: One week after the treatment, the platelet count was increased from preoperative (10.23±8.28) × 109/L to (140.28±85.45) × 109/L in patients with ITP, while the platelet count was increased from preoperative (12±8) × 109/L to (210±60) × 109/L in patients with Evans syndrome. Meanwhile, the hemoglobin level showed an increase in different degrees, from preoperative (63.00±13.62) g/L to postoperative (123.00±13.14) g/L. The therapeutic effectiveness was 100%. During the follow-up time lasting for 3-5 years, recurrence was seen in 11 patients (36.7%) and the overall efficacy rate was 63.3%. Conclusion: For the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome, partial spleen embolization combined with vincristine infusion carries reliable long-term efficacy. (author)

  2. Chronic Idiopathic Urticaria (CIU) is no longer idiopathic

    DEFF Research Database (Denmark)

    Maurer, M; Bindslev-Jensen, C; Gimenez-Arnau, A;

    2013-01-01

    for the same type of urticaria, which has implications for comparing study outcomes and drug licensing. Consequently, there is an urgent need for a harmonized and universally accepted nomenclature and classification of urticaria and to avoid the routine use of the outdated aetiological term, chronic idiopathic......During recent years our knowledge of the aetiology and pathogenesis of urticaria has advanced considerably allowing us to better characterize urticaria subtypes. However, although the classification of urticaria has undergone some revisions during this time (1), authors still use different names...... urticaria (CIU)....

  3. Mathematical models for the study of the dynamics of indium-111-labelled platelets in idiopathic thrombocytopenic purpura

    Energy Technology Data Exchange (ETDEWEB)

    Savolainen, S.

    1992-01-01

    Platelet kinetics in patients with idiopathic thrombocytopenic purpura (ITP) was investigated by applying various models (compartmental and open models, and functional and uptake analyses) to data on indium-111 labelled platelets monitored with a gamma camera following intravenous injection of labelled platelets. The usefulness of the selected models was tested by relating kinetic data to pathophysiological phenomena. A comparison of the results of platelet and colloid kinetics showed that the splenic platelet kinetics in ITP patients does not seem to be primarily dependent on the reticuloendothelial system. Although closed three-compartmental analysis seemed to be superior to the other models applied, none of the methods of analysis tested in this study appears to provide a complete description of short-lived platelet dynamics, as for every model certain assumptions that are not entirely relevant have to be made; this stresses the importance of combining various methods for a comprehensive analysis of a complex phenomenon. Conclusions concerning the function of biological systems should be based on more than one dynamic model or calculation method, since applying only one model (or calculation method) may give artifactual results due to poor statistics of observed data or to inexactness of the assumptions concerning the model.

  4. Idiopathic thrombocytopenic purpura treatment of 80 cases%特发性血小板减少性紫癜80例治疗体会

    Institute of Scientific and Technical Information of China (English)

    王秋红

    2011-01-01

    目的 总结成人特发性血小板减少性紫癜(Idiopathic thromb0cytopeniC purpura,ITP)患者的临床特点.方法 对80例ITP患者的临床资料进行回顾性分析.结果 80例ITP患者经治疗后全部有效(100%),其中显效68例(85%),良效6例(7.5%),进步7例(7.5%).结论 ITP好发于中青年女性,多数对PRED治疗有反应,无反应者改用VCR多数仍可有反应,IVIG治疗疗效显著.%Objective To summarize the adult idiopathic thrombocytopenic purpura (idiopathic thrombocytopenic purpura, ITP) in patients with clinical features. Methods Totally 80 cases of ITP patients were retrospectively analyzed. Results Totally 80 ITP patients were all effective after treatment (100% ) , excellent in 68 cases( 85% ) ,good in 6 cases(7. 5% ) ,progress in 7 cases(7. 5% ) . Conclusions ITP occurs in young women, most of the PRED response to treatment, the majority of non - responders to switch to VCR can respond, IVIG therapy significantly.

  5. Randomized trial comparing intravenous immunoglobulin with methylprednisolone pulse therapy in acute idiopathic thrombocytopenic purpura. Danish I.T.P. Study Group.

    Science.gov (United States)

    Rosthøj, S; Nielsen, S; Pedersen, F K

    1996-08-01

    Forty-three children with newly diagnosed idiopathic thrombocytopenic purpura (ITP), platelet count (PC) below 20 x 10(9)l-1, and either continued bleeding or failure to show a spontaneous rise in the PC after a 3 day observation period were randomized to treatment with either intravenous immunoglobulin (IVIG) infusions 1 g kg-1 (n = 23) or intravenous methylprednisolone pulse therapy (MPPT) 30 mg kg-1 (n = 20) on two consecutive days. After 72 h, IVIG had induced greater platelet responses (mean PC 188 x 10(9) versus 77 x 10(9)l-1, 2p MPPT as the initial treatment for ITP. PMID:8863869

  6. TXRF analysis of low Z elements in serum of patients with idiopathic thrombocytopenic purpura using X-ray fluorescence

    Energy Technology Data Exchange (ETDEWEB)

    Canellas, Catarine G.L.; Leitao, Roberta G.; Lopes, Ricardo T., E-mail: catarine@lin.ufrj.b, E-mail: ricardo@lin.ufrj.b [Universidade Federal do Rio de Janeiro (PEN/COPPE/UFRJ), RJ (Brazil). Coordenacao dos Programas de Pos-Graduacao de Engenharia. Lab. de Instrumentacao Nuclear; Carvalho, Silvia M.F., E-mail: silvia@hemorio.rj.gov.b [State Institute of Hematology Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ (Brazil); Bellido, Alfredo Victor B., E-mail: alfredo@ien.gov.b [Federal Fluminense University (UFF), Niteroi, RJ (Brazil). Chemistry Inst.; Anjos, Marcelino J., E-mail: marcelin@lin.ufrj.b [State University of Rio de Janeiro (UERJ), RJ (Brazil). Physics Inst.

    2011-07-01

    Idiopathic thrombocytopenic purpura (ITP) is a blood disorder characterized by an abnormal decrease in the number of platelets in the blood. ITP results from development of an antibody directed against a structural platelet antigen (an autoantibody). Platelets are also called thrombocytes, meaning cells that form clots. The cause of ITP is not known and their diagnosis requires that other disorders be excluded through selective tests. In this work, forty patients suffering from ITP and sixty healthy volunteers (Control Group) were analyzed. All the serum samples had been collected from people who live in the urban area of Rio de Janeiro City/Brazil. Blood was collected into vacutainers without additives. The measurements were performed at the X-ray fluorescence beamline at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a monochromatic beam with maximum energy of 20 keV for the excitation and an Ultra-LEGe detector with resolution of 148 eV at 5.9 keV. Standard solutions with Vanadium as internal standard were prepared for calibration system. It was possible to determine the elemental concentrations of the following six elements: Na, P, S, Cl, K and Ca. The Student's t-test was used to analyze significant differences ({alpha} = 0.05) between group of patients with ITP and control group. The elements that presented significant differences for the mean of their concentrations between each one of the ITP group and control group in {mu}g.g-1 were: phosphorous (136{+-}12 and 92{+-}12), Sulphur (1077{+-}97 and 847{+-}80), Chlorine (2905{+-}385 and 2266{+-}378), Potassium (137{+-}118 and 82{+-}15) and Calcium (64{+-}7 and 44{+-}6) respectively. These results will help the biomedical field with regard to early diagnosis and improved medical treatment. Thus, our findings indicate that these elements can be related to the important biochemical processes in ITP. (author)

  7. Quantifying the reduction in immunoglobulin use over time in patients with chronic immune thrombocytopenic purpura receiving romiplostim (AMG 531)

    NARCIS (Netherlands)

    Pullarkat, Vinod A.; Gernsheirner, Terry B.; Wasser, Jeffrey S.; Newland, Adrian; Guthrie, Troy H.; de Wolf, Joost Th. M.; Stewart, Ron; Berger, Dietmar

    2009-01-01

    Patients with Immune thrombocytopenic purpura (ITP) often require immunoglobulin (Ig) therapy with intravenous 19 (IVIG) or anti-D to prevent or treat the serious bleeding events. Because the thrombopoietin (TPO) mimetic romiplostim (AMG 531; Nplate) elevates platelet counts in patients with chronic

  8. Retrospective analysis of rituximab therapy and splenectomy in childhood chronic and refractory immune thrombocytopenic purpura.

    Science.gov (United States)

    Ay, Yilmaz; Karapinar, Tuba H; Oymak, Yesim; Toret, Ersin; Demirag, Bengu; Ince, Dilek; Ozcan, Esin; Moueminoglou, Nergial; Koker, Sultan A; Vergin, Canan

    2016-06-01

    Immune thrombocytopenic purpura (ITP) results from accelerated platelet destruction mediated by autoantibodies to platelet glycoproteins. Some patients with chronic ITP are refractory to all therapies [steroids, intravenous immunoglobulin (IVIG), anti-D and immunosuppresive drugs] and have chronic low platelet counts and episodic bleeding. We retrospectively evaluated the efficacy and safety of rituximab treatment and splenectomy in paediatric patients diagnosed with chronic and refractory ITP who were unresponsive to steroids, IVIG, cyclosporine and mycophenolate mofetil. Records of patients with chronic and refractory ITP in 459 patients with primary ITP who were followed up in our hospital from January 2005 to December 2014 were reviewed. Fifteen of patients received rituximab and/or applied splenectomy. Fifteen chronic ITP patients (10 boys, five girls) with a mean age of 10 years were enrolled in the study. Two of these patients were suffering from Evans syndrome. The median time since diagnosis of ITP was 10 years. The median follow-up duration after starting Rituximab and splenectomy were 13 and 9.5 months, respectively.None of the seven patients who were treated with rituximab achieved a response. A splenectomy was performed in six of the seven patients who had been treated with rituximab. Complete and partial responses were achieved in 67 and 33% of the patients, respectively. We evaluated the clinical characteristics and responses of chronic ITP patients who did not receive rituximab therapy and underwent a splenectomy. The success rate was 100% in the eight patients with chronic and refractory ITP. Rituximab therapy might not be beneficial for some children with severe chronic ITP who are refractory to standard agents. A splenectomy might be useful and preferable to rituximab. PMID:26656905

  9. 儿童特发性血小板减少性紫癜202例临床分析%Clinical characteristics of 202 cases with children idiopathic thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    周登余

    2011-01-01

    Objective To analyze the characteristics and therapy efficiency of children idiopathic thrombocytopenic purpura (ITP), and compare the differences between acute and chronic ITP. Methods 202 cases of idiopathic thrombo-cytopenic purpura who were diagnosed and treated in the First Affiliated Hospital of Anhui Medical University from 2008 to 2010 were reviewed. Results Among 202 cases (106 male and 96 female), acute ITP was 177 cases (87.62% ), chronic ITP (CITP) and refractory ITP (RITP) were 25 cases (12.38% ). Most of acute ITP in children were less than 6 year old (77.23%). 174 cases (86.14%) of ITP had severe thrombocytopenia and 81.68% of them showed mild bleeding. For acute ITP, the efficiency of different therapy had no significant difference in Pred group (89.66%), IVIG group (73.33%) and combination group (88.98% ), respectively. But it was lower in combination group in chronic/re-fractory ITP (54.55% ). Conclusions Childhood ITP mostly occur at the age of 1 to 6 year, the incidence of ITP is e-qual between men and women, the majority of the children's ITP cases are acute course with mild bleeding and recover fast.%目的 分析儿童特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的临床表现及治疗情况,比较急、慢性ITP的不同点.方法 对安徽医科大学第一附属医院2008年1月~2010年12月期间,住院的202例ITP病例临床资料进行回顾性分析.结果 202例ITP患儿,男106例(52.48%),女96例(47.52%),急性ITP 177例(87.62%),慢性、难治性ITP 25例(12.38%),6岁及以下患儿156例(77.23%),6岁以上46例(22.77%);有174例(86.14%)ITP患儿发病时血小板呈重度、极重度减少,但临床多为轻度出血(81.68%);急性组单用激素治疗有效率89.66%,单用丙种球蛋白治疗有效率73.33%,两者联合治疗有效率为88.98%;慢性、难治性ITP激素联合丙种球蛋白治疗有效率54.55%.结论 儿童ITP多见于6岁及以下,男女发病机会均等,该病临床发病急

  10. Intravenous immunoglobulin therapy of idiopathic thrombocytopenic purpura in childhood and adolescence.

    Science.gov (United States)

    Bussel, J B; Hilgartner, M W

    1987-09-01

    Intravenous immunoglobulin is not only a dramatic clinical therapy, but it is also extremely interesting in regard to mechanism of action. The high cost of therapy limits its application, yet it appears to be equal to or perhaps slightly more effective than corticosteroids as a treatment of ITP and is far less toxic with prolonged use. The appropriate place for its exact use remains to be determined but probably includes patients urgently requiring rapid platelet increases (in conjunction with steroids), treatment of immunocompromised patients, and treatment of chronic patients, either children to avoid splenectomy or adults with severe disease after splenectomy. Controlled trials to resolve these clinical questions are urgently needed. Existing studies on its mechanisms of actions are very interesting and have furthered our understanding of the pathophysiology of ITP. Although future work may lead to further applications, initial enthusiasm for the use of IVGG in the treatment of other autoimmune diseases with the exception of myasthenia gravis has been limited by subsequent clinical experience. PMID:2452151

  11. Successful treatment of sepsis-induced disseminated intravascular coagulation in a patient with idiopathic thrombocytopenic purpura using recombinant human soluble thrombomodulin.

    Science.gov (United States)

    Koga, Tomohiro; Inoue, Daisuke; Okada, Akitomo; Aramaki, Toshiyuki; Yamasaki, Satoshi; Nakashima, Munetoshi; Kawakami, Atsushi; Eguchi, Katsumi

    2011-12-01

    Disseminated intravascular coagulation (DIC) may complicate a variety of disorders that contribute to mortality, particularly those related to bleeding. It is therefore very difficult to manage DIC in patients with known bleeding disorders. We treated a 62-year-old woman with idiopathic thrombocytopenic purpura (ITP) complicated with sepsis-induced DIC. She had been diagnosed with ITP 8 months prior to admission. Laboratory tests showed an elevation of d-dimer and endotoxin, while pyelonephritis was shown by abdominal computed tomography. Escherichia coli was detected by blood culture. Based on these findings, the patient was diagnosed with sepsis-induced DIC due to urinary tract infection. Thrombocytopenia was refractory despite the use of antibiotics and platelet transfusion, but it was promptly improved in response to recombinant human soluble thrombomodulin (rTM). We suggest that rTM provides a new therapeutic strategy for DIC patients with high hemorrhagic risk.

  12. The relationship between T CD4+ cells count and IL-17, IL-11 serum level in idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Nayereh Alizadeh

    2014-04-01

    Conclusion: In summary, our study indicated a role of IL-11 in ITP patients, also showed that ITP may not be associated with changes of plasma IL-17 levels and T CD4+ cells count relative to control population. Therefore, measurement of plasma IL-11 levels may be important criteria in development of ITP. In addition, it is concluded that determination of IL-11 can be a diagnostic marker to recognize thrombocytopenic purpura patients.

  13. Is Tc-99m Sulfur Colloid Scintigraphy Necessary in Chronic Immune Thrombocytopenic Purpura Before Splenectomy?

    Directory of Open Access Journals (Sweden)

    Ilçe HT et al.

    2011-05-01

    Full Text Available One of the most common reasons for elective splenectomy on adults ischronic immune thrombocytopenic purpura. It is characterized by thrombocyte destruction in spleen, so, management of splenectomy is the gold standard. However if there is remnant spleen tissue postoperatively it cause to going on thrombocytopenia. The principal reason of remnant spleen tissue is accessory spleen. So it is important to detect this tissue pre or postoperatively. Thirty years old, male patient underwent splenectomy four years ago because of chronic immunethrombocytopenic purpura. When thrombocytopenia recurrence occurredabdominal ultrasonography was performed and there was no abnormal sign. Then, Tc–99 m Sulfur Colloid Spleen Scintigraphy was performed and spleen tissue was detected in left hypochondriac region. Tc-99mSulfur Colloid Spleen Scintigraphy is one of the imaging method for accessory spleen. Especially if it is performed preoperatively the surgeon can be careful during the operation and at the same time detected accessory spleen was removed and recurrence can be prevented.

  14. The role of tumor necrosis factor-alpha -308 G/A and transforming growth factor-beta 1 -915 G/C polymorphisms in childhood idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Emel Okulu

    2011-09-01

    Full Text Available Objective: To increase our understanding of the etiology of idiopathic thrombocytopenic purpura (ITP some cytokine gene polymorphisms were analyzed for susceptibility to the disease. The aim of this study was to investigate the role of tumor necrosis factor-alpha (TNF-α -308 G/A and transforming growth factor-beta 1 (TGF-β1 –915 G/C polymorphisms in the development and clinical progression of childhood ITP.Materials and Methods: In all, 50 pediatric patients with ITP (25 with acute ITP and 25 with chronic ITP and 48 healthy controls were investigated via LightCycler® PCR analysis for TNF-α -308 G/A and TGF-β1 -915 G/C polymorphisms.Results: The frequency of TNF-α -308 G/A polymorphism was 20%, 16%, and 22.9% in the acute ITP patients, chronic ITP patients, and controls, respectively (p>0.05. The frequency of TGF-β1 -915 G/C polymorphism was 16%, 8%, and 8.3% in the acute ITP patients, chronic ITP patients, and controls, respectively (p>0.05. The risk of developing ITP and clinical progression were not associated with TNF-α -308 G/A (OR: 0.738, 95% CI: 0.275-1.981, and OR: 0.762, 95% CI: 0.179-3.249 or TGF-β1 -915 G/C (OR: 1.5, 95% CI: 0.396-5.685, and OR: 0.457, 95% CI: 0.076-2.755 polymorphisms. Conclusion: The frequency of TNF-α -308 G/A and TGF-β1 -915 G/C polymorphisms did not differ between pediatric ITP patients and healthy controls, and these polymorphisms were not associated with susceptibility to the development and clinical progression of the disease.

  15. 特发性血小板减少性紫癜患儿病原感染的临床分析%Clinical analysis of pathogen infection in children with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王晓格; 胡姬婷

    2014-01-01

    目的:探讨特发性血小板减少性紫癜(ITP)患儿与病原感染的关系。方法:回顾性分析89例ITP患儿发病的诱因,应用ELISA法检测巨细胞病毒、EB病毒、幽门螺杆菌(Hp)、支原体,并分析与ITP的关系。结果:89例ITP患儿中,46.1%的患儿发病前1~3周有病毒感染,17.9%的患儿在发病前曾有预防接种史。其中 CMV 阳性10例(11.24%),EB 病毒阳性7例(7.86%),Hp 阳性12例(13.48%),MP 阳性2例(2.25%),总阳性率30.34%(27/89)。发病时血小板计数及治疗后血小板升至正常的时间在病原阳性组与阴性组比较,差异无统计学意义(P>0.5)。经过随访,急性转为慢性 ITP 4例(4.49%),各病原阳性组总的转慢率7.40%,病原阴性组转慢率3.22%,两组比较差异有统计学意义(P<0.05)。结论:病原感染是特发性血小板减少性紫癜发病的一个重要原因,其中以HP感染所占比例较高,Hp感染组ITP患儿易迁延不愈转为慢性。%Objective:To explore the relationship between pathogen infection and children with idiopathic thrombocytopenic purpura(ITP).Methods:Cause of the disease of 89 children with ITP were retrospectively analyzed.Cytomegalovirus,EB virus, helicobacter pylori,mycoplasma were detected using ELISA method.We analyzed the relationship between them and ITP.Results:In the 89 children with ITP,46.1% children were infected by virus in 1 to 3 weeks before the onset of disease,17.9% children had vaccination history before the onset of disease.The CMV was positive in 10 cases(11.24%),EB was positive in 7 cases(7.86%),HP was positive in 12 cases(13.48%),MP was positive in 2 cases(2.25%).The total positive rate was 30.34%(27/89).The platelet count at the time of onset and platelet rose to normal time after treatment between pathogen positive group and negative group were compared.There were no statistical significance(P>0.5).At follow-up,4 patients with acute ITP turned to chronic ITP(4.49%).In

  16. AUTOLOGOUS SERUM SKIN TESTING (ASST IN CHRONIC IDIOPATHIC URTICARIA

    Directory of Open Access Journals (Sweden)

    Arun

    2014-01-01

    Full Text Available Chronic idiopathic urticaria (CIU is a form of urticaria , in which there appears to be persistent activation of mast cells , but the mechanism of mast cell triggering is unknown. The Autologous serum skin test (ASST is an in vivo test which assesses auto reactivity. ASST could be good screening test for Autoreactive urticaria a subset of chronic idiopathic urticaria. AIMS : To study the clinical profile of chronic idiopathic urticaria and pattern of ASST among p atients of chronic idiopathic urticaria. METHODS : Study included 200 patients of CIU that were recruited from outpatient department (OPD. Patient s with a history of urticaria for more than 6 weeks were included in the study. The detailed history , cutaneou s and systemic examination was done. ASST was performed under strict aseptic precaution. RESULTS : Out of 200 patients 100 were male and 100 were female. Mean age of patients was 31.9 yrs. Most common age group was 25 - 34yrs. ASST was found positive in 36(18 % patients. In most of the patients duration of disease was less than 11months (42% with the mean duration of disease 21.74 months. Mean duration of wheal was 64.7 minutes , in which most of the patients (112 having duration of wheal less than 59 min. Hi story of angioedema was positive in 109(54.5% patients. Aggravating factors were positive in 50(25% patients in which drugs and cold were the most common. Family history of urticaria and angioedema was present in 35 patients. None of patient complained a ny adverse reactions during and after ASST. CONCLUSION : ASST is the easily available bed side test for the diagnosis of autoreactive urticaria. It is a simple , inexpensive , semi invasive and easy - to - perform test which can be done and recorded by the dermat ologist himself. ASST may help in diagnosis and management of chronic idiopathic urticaria.

  17. Clinical efficiency of cyclosporine in chronic idiopathic urticaria in adults

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    V.I. Petrov

    2010-06-01

    Full Text Available The purpose of the research is to evaluate the clinical effectiveness of cyclosporine and other antihistamines in patients with chronic forms of urticaria resistant to basic first-line therapy. Open randomized controlled study has been performed in parallel groups. 53 patients with chronic idiopathic urticaria ages 18-50 years have been examined. In case of ineffectiveness of previous therapy, patients have been randomized into 2 groups: group I receiving cyclosporine (Sandimmune Neoral ® 2,5 mg/kg/day, group II receiving cetirizine (Zyrtec ® 10 mg/day and ranitidine (Zantac ® 300 mg/day orally. It has been found that the administration of cyclosporine in patients with severe chronic idiopathic urticaria provides a more rapid achievement of clinical effect than the therapy with H1/H2 histamine antagonists. It is confirmed by a significant decrease of total index of severity of illness and major symptoms of skin lesions. This tendency towards normalization of quality of life of patients taking cyclosporine remains during 8 weeks after the medication. Thus administration of cyclosporine can be considered as therapy of choice in patients with chronic idio-pathic urticaria with a severe course and ineffective long-term therapy with antihistamines / systemic corticosteroids

  18. Evaluation of H2 receptor antagonists in chronic idiopathic urticaria

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    Minocha Y

    1995-01-01

    Full Text Available H1-antagonist (hydroxyzine hydrochloride in dosage of 10 mg-25 mg thrice a day failed to elicit satisfactory response in 60 out of 170 patients of chronic idiopathic urticaria. Additional administration of H2-antagonist (cimetidine in dosage of 200 mg four times a day, in patients not responding earlier to H1-antagonist alones exhibited moderate to good improvement of various parameters of urticaria in approximately 85% patients

  19. Comparison Of Levocetirizine And Cetirizine In Chronic Idiopathic Urticaria

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    Rathi Sanjay K

    2004-01-01

    Full Text Available The aim of the study was to compare efficacy, onset of action, and sedative effects of cetirizine (CTZ and levocetirizine (levoCTZ in chronic idiopathic urtcaria with fifteen patients in each group. They were found to be equipotent and the onset of action was almost same. Twenty percent in CTZ and 13.33% in levoCTZ exhibited mild sedation.

  20. The efficacy of tonsillectomy in treatment idiopathic thrombocytopenic purpura%扁桃体切除术治疗血小板减少性紫癜的临床观察

    Institute of Scientific and Technical Information of China (English)

    唐林甫; 简树财; 唐奕; 罗小莉; 唐萍; 范颖; 李晞晨

    2012-01-01

    To study the causality between chronic tonsillitis and idiopathic thrombocytopenic purpura(ITP), and evaluate the therapeutic effect of tonsillectomy. Methods There were 31 patients who were sick of chronic tonsillitis complicated ITP , all of them were treated by tonsillectomy. To observe the clinical symptom and platelet count in 2-year follow-up. Results 24 cases'symptom were disappeared, and the platelet count rised to normal level on the 1nt week ,3nd month, 1nt year and 2nd year,there was statistical significance compared with preoperative (P <0.01), 7 cases' symptom of chronic tonsillitis were disappeared,but the symptom of ITP still existed and the platelet count had no significant increased compared with preoperative (p< 0. 01). Conclusion Chronic tonsillitis is an important cause of ITP, and tonsillectomy may be an effective treatment for ITP.%目的 探讨慢性扁桃体炎与血小板减少紫癜(ITP)的因果关系及治疗方法.方法 31例慢性扁桃体炎合并ITP的患者行扁桃体切除术,术后随访观察血小板计数及临床症状,随访2年.结果 24例慢性扁桃体炎及ITP症状消失,术后1周、3个月、1年、2年血小板计数达到正常水平与术前比较差异有统计学意义(P<0.01),随访期内未见ITP复发.7例患者扁桃体炎症状消失,但皮肤、粘膜出血未见明显改善,血小板计数较术前差异无统计学意义(P>0.05).结论 慢性扁桃体炎是引起ITP的重要原因之一,扁桃体切除术是治疗ITP的一种有效方法.

  1. Intracranial hemorrhage in acute and chronic childhood immune thrombocytopenic purpura over a ten-year period: an Egyptian multicenter study.

    Science.gov (United States)

    Elalfy, Mohsen; Elbarbary, Nancy; Khaddah, Normine; Abdelwahab, Magy; El Rashidy, Farida; Hassab, Hoda; Al-Tonbary, Youssef

    2010-01-01

    Intracranial hemorrhage (ICH) is a rare but major cause of death in immune thrombocytopenic purpura (ITP). The authors reviewed data of 1,840 patient with ITP, from 5 pediatric hematology centers in Egypt from 1997 to 2007, to study the incidence and risk factors of ICH. Ten cases of ICH were identified with a median age at presentation of 7.5 years; 4 patients had acute ITP, 2 persistent and 4 chronic. The platelet count was late referral to a specialized center. Our results suggest that treatment does not prevent ICH and that it can occur at any time during the course of the disease. Delayed referral can be considered a risk factor for unfavorable outcome of ICH, highlighting the importance of teaching sessions for patients and their parents to minimize subsequent morbidity and mortality of ICH in children with ITP. PMID:19955713

  2. [Surgical treatment of chronic idiopathic constipation].

    Science.gov (United States)

    Menguy, R; Chey, W

    We review current experience with surgical treatment of severe constipation due to primary inertia of the colon. Over the last 10 years, we have operated 18 patients (14 females and 4 males) with severe constipation. The surgical procedure was either nearly total colonectomy with ascending colon/rectum anastomosis (8 cases) or total colonectomy with ileorectal anastomosis (9 cases). In one patient, coloproctectomy was performed with an ileoanal anastomosi. Indications for surgery were based on results of barium emena and functional evaluation of defecation. Results were satisfactory in all patients. In several patients however, we noted that the motility of other levels of the digestive tract was also impaired. Colonectomy was introduced as a treatment for chronic constipation nearly a century ago and although very few indications have been retained in the recent this procedure has now become an acceptable surgical approach in a limited number of well-though-out cases. PMID:7729199

  3. Oral iron therapy and chronic idiopathic urticaria: sideropenic urticaria?

    Science.gov (United States)

    Guarneri, Fabrizio; Guarneri, Claudio; Cannavò, Serafinella Patrizia

    2014-01-01

    Chronic urticaria (CU) is frequent, remains often idiopathic despite diagnostic efforts, and sometimes poorly responds to oral antihistamines and/or corticosteroids. We noticed that hyposideremia is often found in patients with chronic idiopathic urticaria poorly responsive to usual treatments (prCIU), and oral iron therapy is frequently associated to improvement or resolution of urticaria. Between 2003 and 2012, we observed 122 patients with prCIU, of which 81 had moderate hyposideremia at our first visit. They continued the antihistamines already practiced and received oral iron therapy for 30 or 45 days. Two months after our first visit, all had normal serum iron levels; 64 reported complete remission of urticaria and 17 reported improvement superior to 80%. No adverse reactions to treatment were observed. Follow-up visits confirmed stability of results over 6 months. Our preliminary data show that hyposideremia is the only abnormality in many patients with prCIU, and restoration of normal iron serum levels is associated to remission or remarkable clinical improvement of urticaria. In consideration of low cost and potential benefits for some patients, determination of serum levels of iron could be introduced in the diagnostic workup of chronic urticaria, maybe as a second-level exam in patients without other relevant clinical or laboratory abnormalities.

  4. Clinical Observation on the Treatment of Childhood Refractory Idiopathic Thrombocytopenic Purpura with Dihuang Zhixue Capsule(地黄止血胶囊)

    Institute of Scientific and Technical Information of China (English)

    LIU Qing-chi; WU Wei-hai; WU Da-yong; FENG Xin-wang; MA Ya-hui; LI Jian-ying; PANG Yu-hui; SONG Shu-hua

    2008-01-01

    Objective:To observe the clinical effect of Dihuang Zhixue Capsule(地黄止血胶囊,DZC,a Chinese preparation for cooling blood and dispelling toxic substances) in the treatment of childhood refractory idiopathic thrombocytopenic purpura(RITP),with cyclosporin A(CsA)used as the control.Methods:Fody-one children of RITP were randomized into the treated group and the control group.The 21 patients in the treated group were orally given 2 to 3 DZC capsules each time,thrice a day and the 20 in the control group were given 3 mg/kg CsA per day,with 3 months as one therapeutic course.The therapeutic efficacy,platelet count and adverse reaction in the two groups were compared at the end of the course.Results:(1)In the treated group,1 (4.8%)patient was evaluated as cured,3(14.3%)as markedly effective,5(23.8%)as effective,5(23.8%)as improved,7(33.3%)as ineffective,with the total effective rate being 66.7%:while in the control group,the corresponding numbers were 0,2(10.0%),2(10.0%),3(15.0%),13(65.0%)and 35.0%,respectively,showing statistical significance in difference between the total effective rates of the two groups(X2=4.11,P=0.0426).(2)As compared with the baseline,the platelet count increased in both groups after 2 months'treatment(P<0.05).After 3 months'treatment,the platelet count was higher in the treated group than in the control group(P<0.05).(3)The improvement of hemorrhage in the treated group after 8 weeks'treatment was better than that in the control group (P<0.05).(4)No apparent adverse reaction was observed in fhe treated group,while in the control group,hirsutism was shown in 15 cases;gingival hyperplasia in 10;digestive reaction in 5,liver function impairment in 5.hypedension in 2 and renal impairment in 2.Conclusion:The therapeutic efficacy of DZC is better than that of CsA.and DZC shows good compliance but brings no obvious adverse reaction.

  5. Púrpura trombocitopênica idiopática e linfoma não-Hodgkin de células T na infância Idiopathic thrombocytopenic purpura and T-cell non-Hodgkin's lymphoma in childhood

    Directory of Open Access Journals (Sweden)

    Alessandra C. Borges

    2006-03-01

    Full Text Available Os linfomas representam 10% de todos os tumores malignos da infância e, destes, os linfomas não-Hodgkin são os mais freqüentes. Crianças com doenças auto-imunes apresentam maior probabilidade de desenvolver doenças linfoproliferativas, podendo ocorrer antes, durante ou após o aparecimento da neoplasia. A associação de púrpura trombocitopênica idiopática e linfomas é infreqüente (3%, principalmente na faixa etária pediátrica. Duas teorias tentam explicar a origem desta associação. Na primeira, a trombocitopenia seria decorrente da produção de auto-anticorpos antiplaquetas pelo clone tumoral. Na segunda, a PTI seria resultado de um estímulo antigênico persistente, secundário a uma desordem na proliferação linfóide. O objetivo do presente trabalho foi relatar a associação infreqüente na infância entre púrpura trombo-citopênica idiopática e linfoma não-Hodgkin de células T.Lymphomas represent 10% of all malignant tumors in childhood and from these non-Hodgkin's lymphomas are the most frequent. Children who have autoimmune diseases have a higher probability of developing lymphoproliferative diseases, which can happen before, during or after the appearance of the neoplasia. The association between idiopathic thrombocytopenic purpura and lymphomas is not common (3% especially in children. Two theories try to explain the origin of this association. In the first one, the thrombocytopenia would be a result of an autoantibody anti-blood platelet production by the tumoral clone. In the second one, the idiopathic thrombocytopenic purpura would be a result of a persistent antigenic stimulus subordinate to a disorder in the lymphoid proliferation. The aim of this work is to report the unusual association between idiopathic thrombocytopenic purpura and T-cell non-Hodgkin's lymphoma in childhood.

  6. Rituximab treatment for symptomatic chronic ITP

    NARCIS (Netherlands)

    Tamminga, Rienk Y. J.; Bruin, Marrie C. A.

    2006-01-01

    About 20% of the children diagnosed with acute idiopathic thrombocytopenic purpura (ITP) will run a chronic course. Only in a minority of these, platelet-count-enhancing treatments are indicated. Most treatment options are directed at decreasing platelet destruction including corticosteroids, intrav

  7. A Study of Anorectal Manometry in Patients with Chronic Idiopathic Constipation

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    To study the changes of anorectal motility in patients with chronic idiopathic constipation, anorectal motility was investigated by water-perfused manometric system in 30 patients with chronic idiopathic constipation and 18 healthy subjects. Our results showed that there was no significant difference between the constipation group and the control group in anal sphincteric resting pressure and anal maximal squeezing pressure. The minimum relaxation volume, the rectal defecatory threshold, the rectal maximal tolerable volume and the rectal compliance in the patients were significantly higher than those in the controls (P< 0. 01 or P< 0. 05). It is concluded that patients with chronic idiopathic constipation have anorectal motility disturbances.

  8. Management of chronic immune thrombocytopenic purpura: targeting insufficient megakaryopoiesis as a novel therapeutic principle

    OpenAIRE

    Rank, Andreas

    2010-01-01

    Andreas Rank, Oliver Weigert, Helmut OstermannMedizinische Klinik III – Grosshadern, Klinikum der Ludwig Maximilians-Universitaet Munich, Munich, GermanyAbstract: Traditionally, anti-platelet autoantibodies accelerating platelet clearance from the peripheral circulation have been recognized as the primary pathopysiological mechanism in chronic immune thrombocytopenia (ITP). Recently, increasing evidence supports the co-existence of insufficient megakaryopoiesis. Inadequate low throm...

  9. The Role of Dehydroepiandrosterone Sulfate In Chronic Idiopathic Urticaria Pathogenesis

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    Filiz Canpolat

    2009-03-01

    Full Text Available Objective: Adrenal androgens, dehydroepiandrosterone (DHEA and dehydroepiandrosterone sulfate (DHEA-S seem implicated in the pathophysiology of autoimmune disorders, as natural immunosuppressors. The relationship of chronic idiopathic urticaria (CIU with circulating concentration of DHEA-S has been reported previously. However, this link and underlying mechanisms remain unknown. The aim of this study was to evaluate circulating DHEA-S concentration in larger patients group suffering from CIU.Materials and Methods: Serum concentration of DHEA-S was measured in 65 patients (34 women, 31 men with CIU and compared with 40 age and sex matched, non atopic healthy volunteers. Disease duration and extent of involvement were recorded.Results: The patients with CIU presented significantly decreased values of DHEA-S levels (102.66±28.95 µg/dl with respect to control group (174.22±39.58 µg/dl (p<0.01. No significant correlation was found between DHEA-S concentration and the duration of the disease, disease activity and gender.Conclusion: Our results support the hypothesis that DHEA-S may be a cause for the development of CIU, regardless of their gender. For definitive role of DHEA-S in CIU pathogenesis, further studies are needed.

  10. 特发性血小板减少性紫癜患儿HLA-DRB1基因多态性%HLA-DRB1 polymorphism of idiopathic thrombocytopenic purpura in children

    Institute of Scientific and Technical Information of China (English)

    李晓红; 盛光耀

    2011-01-01

    Aim: To estimate the relation between human leukocyte antigen-DRBl ( HLA-DRB1 ) and idiopathic thrombocytopenic purpura (ITP) in children. Methods: Peripheral blood samples and stools were collected from 200 children with ITP and 200 normal children. GICA and ABC-ELISA were used to detect the Helicobacter pylori (Hp) antibody in blood and Hp antigen in stool. PCR specific sequence primers( PCR-SSP) was used to identify HLA-DRB1 gene. Results: The frequency of HLA-DRB1 * 07 significantly decreased in ITP children compared with controls(x2 = 5.444,P =0.020). Compared with acute ITP( AITP) , the frequency of HLA-DRB1 * 11 significantly increased in children with chronic ITP (CITP)(x2 = 6.224,P=0.013).The frequencies of HLA-DRB1 * 14 and HLA-DRB1 * 11 in ITP children with Hp infection were higher than those without Hp infection(x2 =8.602 and 8.794,all P were 0.003). Conclusion:HLA-DRB1 *07 seems to be aprotective factor to against ITP. HLA DRB1 * 11 may play an important role in CITP. HLA-DRB1 * 11 and HLA-DRB1 * 14 seems to predict susceptibility to Hp infection in ITP children.%目的:探讨人类白细胞抗原DRB1(HLA-DRB1)基因多态性与儿童特发性血小板减少性紫癜(ITP)的关系.方法:分别采用胶体金法和ABC-ELISA法检测200例ITP患儿和200例对照全血幽门螺杆菌(Hp)-1gG抗体和粪便Hp抗原;序列特异性引物PCR(PCR-SSP)法对HLA-DRB1基因进行分型.结果:①ITP组与对照组HLA-DRB1*07基因频率分别为6.5%和13.5%,差异有统计学意义(χ2=5.444,P=0.020).②急性ITP(AITP)与慢性ITP(CITP)患儿HLA-DRB1*11基因频率分别为9.3%和22.0%,差异有统计学意义(χ2=6.224,P均为0.003).③Hp阳性与Hp阴性ITP患儿HLA-DRB1*14和HLA-DRB1*11基因频率分别为16.0%和3.8%及22.3%和7.5%,差异有统计学意义(χ2=8.602和8.794,P均=0.003).结论:①HLA-DRB1*07可能对儿童ITP的发病具有保护作用.②具有HLA-DRB1*11基因型的患儿易发展为CITP.③HLA-DRB1*14和HLA-DRB1*11基因可能是儿童ITP患儿Hp感染的易感因素.

  11. Management of chronic immune thrombocytopenic purpura: targeting insufficient megakaryopoiesis as a novel therapeutic principle

    Directory of Open Access Journals (Sweden)

    Andreas Rank

    2010-05-01

    Full Text Available Andreas Rank, Oliver Weigert, Helmut OstermannMedizinische Klinik III – Grosshadern, Klinikum der Ludwig Maximilians-Universitaet Munich, Munich, GermanyAbstract: Traditionally, anti-platelet autoantibodies accelerating platelet clearance from the peripheral circulation have been recognized as the primary pathopysiological mechanism in chronic immune thrombocytopenia (ITP. Recently, increasing evidence supports the co-existence of insufficient megakaryopoiesis. Inadequate low thrombopoietin (TPO levels are associated with insufficient proliferation and differentiation of megakaryocytes, decreased proplatelet formation, and subsequent platelet release. Recently two novel activators of TPO receptors have been made available: romiplostim and eltrombopag. In several phase III studies, both agents demonstrated increase of platelet counts in about 80% of chronic ITP patients within 2 to 3 weeks. These agents substantially broaden the therapeutic options for patients with chronic ITP although long-term results are still pending. This review will provide an update on the current conception of underlying mechanisms in ITP and novel, pathophysiologically based treatment options.Keywords: immune thrombocytopenia, romiplostim, eltrombopag, megakaryopoiesis

  12. Helicobacter pylori infection and idiopathic thrombocytopenic purpura in children%幽门螺杆菌感染与儿童特发性血小板减少性紫癜

    Institute of Scientific and Technical Information of China (English)

    刘巧

    2010-01-01

    Idiopathic thrombocytopenic purpura(ITP) is characterized by platelet destruction which is mediated by auto-antibodies. Recent researches show that helicobacter pylori infection is correlated with part of FTP in adult. Eradication of helicobacter pylori infection has brought dramatically platelet elevation. It was reported that cross reaction played a very important part in the helicobacter related FTP, but the exact mechanism is still unknown, whether the FTP in children needs helicobacter pylori eradication is under debate.%特发性血小板减少性紫癜(ITP)是以自身抗体介导的血小板破坏为特征的一组疾病,近年来许多研究显示部分成年人ITP的发生与幽门螺杆菌(Hp)感染密切相关,清除Hp后ITP患者的血小板数量有明显提高.研究认为Hp相关的ITP与抗原交叉反应有关,但其确切机制目前尚不明确;儿童ITP是否需要清除Hp治疗,国内外也存在争议.

  13. Idiopathic portal hypertension and chronic arsenic poisoning. Report of a case.

    Science.gov (United States)

    Chainuvati, T; Viranuvatti, V

    1979-01-01

    We report a case of idiopathic portal hypertension which is related to chronic arsenic poisoning. Only 7 cases have been reported previously. The patient presented with bleeding esophageal varices. Splenomegaly and hyperkeratosis of palms and soles were later noted and led to the discovery of chronic arsenic poisoning. The hemodynamic studies revealed a gradient between the splenic pulp pressure and hepatic wedge pressure which is consistent with presinusoidal hypertension. The liver histology revealed only mild portal fibrosis. Arsenic poisoning is one cause of idiopathic protal hypertension.

  14. Relationship between idiopathic thrombocytopenic purpura and helicobacter pylori CagA protein in adults%成人特发性血小板减少性紫癜与幽门螺杆菌CagA蛋白的关系

    Institute of Scientific and Technical Information of China (English)

    张晋琳; 李慧; 王晓冬; 王春森; 吴良华

    2011-01-01

    目的了解成人特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)与幽门螺杆菌(helicobacter pylori,Hp)CagA蛋白的关系.方法 采用14C尿素呼吸试验对正常成人和ITP患者进行Hp检测后,对Hp阳性的ITP患者进行CagA蛋白抗体检测.依据急性ITP,慢性ITP,难治性ITP对ITP患者进行分类.结果 223例正常患者中123例Hp阳性,感染率为55.2%,而CagA蛋白抗体阳性为42例,占34.1%.278例ITP患者中Hp阳性176例,感染率63.3%,其中CagA蛋白抗体阳性83例,占47.2%,ITP患者的Hp感染率与正常人相似,但CagA蛋白抗体阳性率显著高于正常人(P0.05).结论 纳入研究的ITP患者的Hp感染率与正常人相似,但CagA蛋白抗体阳性率明显高于正常人,提示CagA蛋白在成人ITP的发生发展中具有一定作用.进一步分析也显示不同类型的ITP患者Hp感染率及CagA蛋白阳性率差异无统计学意义.

  15. Anti-D (WinRho SD) treatment of children with chronic autoimmune thrombocytopenic purpura stimulates transient cytokine/chemokine production.

    Science.gov (United States)

    Semple, J W; Allen, D; Rutherford, M; Woloski, M; David, M; Wakefield, C; Butchart, S; Freedman, J; Blanchette, V

    2002-03-01

    Intravenous anti-D is often used in the treatment of autoimmune thrombocytopenic purpura (AITP), but little is known about its mechanisms of action. To investigate anti-D's potential in vivo mechanism(s) of action, a small group (N = 7) of children with chronic AITP was studied. The children initially received either 25 or 50 microg/kg of WinRho-SD in a four-cycle cross-over trial, and peripheral blood samples from the first and third cycles were assessed for cytokine levels at pre-treatment, 3 hr, 1 day, and 8 days post-treatment. Results showed that platelet counts significantly increased in all the children by day 8 post-treatment. Analysis of serum by ELISA showed that there was a significant but transient rise in both pro- and anti-inflammatory cytokine/chemokine levels (e.g., IL1RA, IL6, GM-CSF, MCP-1 alpha, TNF-alpha and MCP-1) by 3 hr post-treatment in both cycles which returned to baseline levels by 8 days post-treatment. These results suggest that anti-D administration may initially activate the RES in the form of cytokine/chemokine secretion, which is subsequently followed by an increase in platelet counts. It is possible that the induced cytokine/chemokine storm may have an effect on several physiological processes such as those mediating either adverse effects or potentially RES phagocytic activity. PMID:11891813

  16. Veltuzumab, an anti-CD20 mAb for the treatment of non-Hodgkin's lymphoma, chronic lymphocytic leukemia and immune thrombocytopenic purpura.

    Science.gov (United States)

    Milani, Cannon; Castillo, Jorge

    2009-04-01

    Veltuzumab is a humanized, second-generation anti-CD20 mAb currently under development by Immunomedics Inc for the potential treatment of B-cell non-Hodgkin's lymphoma (NHL) and chronic lymphocytic leukemia (CLL). Licensee Nycomed is developing veltuzumab for the potential treatment of rheumatoid arthritis and immune thrombocytopenic purpura (ITP). Veltuzumab contains 90 to 95% human antibody sequences with identical antigen framework regions to epratuzumab (a humanized anti-CD22 mAb) and similar antigen-binding determinants to rituximab (chimeric, anti-CD20 mAb and the first-line treatment of aggressive and indolent NHL). In vitro studies have demonstrated that veltuzumab has enhanced binding avidities and a stronger effect on complement-dependent cytotoxicity compared with rituximab in selected cell lines. In dose-finding phase I/II clinical trials in patients with low-grade NHL, intravenous veltuzumab demonstrated a substantial rate of complete responses in concurrence with shorter and more tolerable infusions compared with rituximab. Currently there has been no evidence of an immune response to repeated administrations, and no serious adverse events related to veltuzumab treatment in patients with NHL. Veltuzumab is undergoing clinical trials using a low-dose subcutaneous formulation in patients with NHL, CLL and ITP. Prospective, randomized clinical trials are needed to clarify the role veltuzumab will play in a market where the therapy of B-cell lymphoproliferative disorders is dominated by rituximab. PMID:19330725

  17. Simultaneous Manifestation of Chronic Myelomonocytic Leukemia and Multiple Myeloma during Treatment by Prednisolone and Eltrombopag for Immune-Mediated Thrombocytopenic Purpura.

    Science.gov (United States)

    Hagihara, Masao; Inoue, Morihiro; Kodama, Kenichiro; Uchida, Tomoyuki; Hua, Jian

    2016-01-01

    An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM). The patient received lenalidomide plus dexamethasone therapy but died due to exacerbation of the disorder. It was supposed that thrombocytopenia was secondarily caused by CMML and MM developed at a later period. PMID:27597907

  18. Epstein -barr virus infection in children with acute idiopathic thrombocytopenic purpura%儿童急性特发性血小板减少性紫癜与EB病毒感染的关系

    Institute of Scientific and Technical Information of China (English)

    白松婷; 赵晓明; 盛光耀; 方营旗

    2011-01-01

    Objective Acute idiopathic thrombocytopenic purpura (AITP) is the most common hemorrhagenic disease in children. It often appears to be related to the sensitization by some viral infections, such as Epstein - Barr virus ( EBV ), cytomegalovirus (CMV), Human parvovirus B19 ( HPVB19 ) and so on. The present study is to elucidate EBV infection in children with AITP. Methods The study cohorts consisted of 87 children with AITP and 40 healthy children. Specific viral antibody - EBV - IgM was detected by ELISA. The laboratory characteristics were analyzed in patients with and without EBV infection. Results Twenty - one (24. 14% ) of 87 children with AITP associated with EBV infection. Which was markedly higher than that of the controls (P <0. 01 ). In children with AITP,the platelet and magakaryocyte counts were similar in patients with and without EBV infection (P > 0. 05 ). However, in the group of EBV infection, the positive rate of antiplatelet antibodies (GPIIb/Ⅲa)in plasma and the numbers of variant lymphocytes in peripheral smear were both significantly increased compared with the group of non -EBV infection( P < 0. 01 ). Conclusions EBV should be the causative viral agent in some children with AITP. Its pathogenesis may initiate the autoimmune reaction.%目的 探讨儿童急性特发性血小板减少性紫癜(AITP)与EBV感染的关系.方法 用酶联免疫吸附法(ELISA)测定87例AITP患儿病毒特异性抗体EBV-IgM,做骨髓巨核细胞、外周血小板以及外周血异形淋巴细胞计数,采用改良MAIPA法检测血小板特异性抗体(GPⅡb/Ⅲa).对照组为40例健康体检的儿童.用统计学分析软件包SPSS13.0进行统计学分析.结果 87例AITP患儿中,EBV-IgM阳性者21例,阳性率24.14%,明显高于对照组(P0.05).结论 EBV感染确实与部分儿童AITP的发病有关.EBV感染引起ITP的机制可能与引发机体自身免疫反应有关.

  19. The intervention of amifostine in idiopathic thrombocytopenic purpura mouse%氨磷汀对免疫性血小板减少性紫癜动物模型小鼠的干预作用

    Institute of Scientific and Technical Information of China (English)

    周国坚; 王桂明; 蔡小连

    2013-01-01

      目的探讨氨磷汀对免疫性血小板减少性紫癜(ITP)动物模型小鼠血小板、脾脏系数、骨髓巨噬细胞的影响。方法通过获取BALB/C 小鼠血小板抗体,接种到豚鼠身上,获取豚鼠抗小鼠血小板抗体(APS)。将APS腹腔注射BALB/C 小鼠建立免疫性血小板减少性紫癜动物模型,使用氨磷汀腹腔注射进行治疗,观察氨磷汀对模型小鼠的血小板、脾脏系数、骨髓巨噬细胞的影响。结果氨磷汀给药后均使ITP模型小鼠血小板计数上升、脾脏系数减少、骨髓成熟巨核细胞增多,与模型组比较差异有统计学意义(P<0.01)。结论氨磷汀对免疫性血小板减少性紫癜有较为明显的治疗效果。%Objective To study the effect of amifostine on mice models of idiopathic thrombocytopenic purpura(ITP). Methods Guinea pig anti mice platelet serum(GP-APS) was diluted with saline(1:4),and intravitreal injected into mice once in 2 days for 10 days.Two dosage of amifostine was intravitreal injected into mice in 10 days since mice models of ITP were established. Results Amifostine can enhance the amount of platelets,promote the mature of megakaryocyte of mice significantly and improve the histological pathology of spleen. Conclusion Amifostine is an effective and safety medicine in treating ITP, and can be used widely in clinic.

  20. Preliminary Screening of PRO Scale Items of Idiopathic Thrombocytopenic Purpura%特发性血小板减少性紫癜PRO量表条目的初步筛选

    Institute of Scientific and Technical Information of China (English)

    龙远雄; 马利; 何丽云; 闫世艳; 王彬; 王双连

    2015-01-01

    目的:筛选特发性血小板减少性紫癜(ITP)PRO量表条目,形成适合我国ITP患者疗效评价的PRO量表。方法:采用专家评分法、因子分析法、离散程度法、克朗巴赫系数(Cronbach Alpha)法、相关系数法(包括2种),从条目的重要性大、敏感性高、独立性强、代表性好、确定性等方面进行筛选。结果:终选量表共54个条目,分为生理、心理和社会3个领域,其中生理领域包括出血、疼痛与不适、睡眠与休息三个维度,最终保留条目数为36条,心理领域保留12条,社会保留6条。结论:筛选后的量表离推广运用还有一定差距,还需在其他人群中进行信度和效度检验。%Objective:To screen PRO scale items of idiopathic thrombocytopenic purpura and form the PRO scale suitable for the evaluation of therapeutic efficiency of ITP patients in China. Methods: The expert grading method, factor analysis method, discrete degree method, Cronbach Alpha method and correlation coefficient method (including two methods) are used to screen from the important, high-sensitivity, strong-independence, representative and certain entries. Results:The ultimate scale has a total of 54 items, divided into physiological, psychological and social area. Among them, the physical area includes three dimensions, which are bleeding, pain and discomfort, and sleep and rest. There are a total of 36 final reserved entries, 12 reserved entries in the psychological area and 6 social reserved entries. Conclusion: After the screening the scale still cannot be popularized and applied. We still need to do the reliability and validity test in other groups.

  1. Diminished Cellular Immune Response to Carbonic Anhydrase II in Patients with Sjogren's Syndrome and Idiopathic Chronic Pancreatitis

    Directory of Open Access Journals (Sweden)

    Onishi S

    2004-07-01

    Full Text Available CONTEXT: A serum antibody to carbonic anhydrase II has been reported in patients with Sjögren’s syndrome and idiopathic chronic pancreatitis. OBJECTIVE: To evaluate cellular immune response to carbonic anhydrase II in patients with Sjögren’s syndrome and idiopathic chronic pancreatitis. PATIENTS: Idiopathic chronic pancreatitis (n=23, Sjögren’s syndrome (n=12, alcoholic chronic pancreatitis (n=3 and normal controls (n=13. MAIN OUTCOME MEASURES: Proliferation assay of peripheral blood mononuclear cells. RESULTS: Notable increased proliferation of the mononuclear cells upon stimulation with carbonic anhydrase II was observed in 2 patients with idiopathic chronic pancreatitis (9% and 2 patients with Sjögren’s syndrome (17% but not in patients with alcoholic chronic pancreatitis nor in normal controls. Among the four study groups, there was no significant difference in the prevalence rate of the positive proliferative responses (P=0.444. CONCLUSION: Carbonic anhydrase II may not be a major target antigen for the immunological process in the pathogenesis of Sjögren’s syndrome and idiopathic chronic pancreatitis. Serum antibody to carbonic anhydrase II may be detected in these patients as a consequence of the immune reaction against other antigens which mimic carbonic anhydrase II.

  2. Chronic idiopathic axonal neuropathy and pain, treated with the endogenous lipid mediator palmitoylethanolamide: a case collection

    Directory of Open Access Journals (Sweden)

    Keppel Hesselink JM

    2013-09-01

    Full Text Available J M Keppel Hesselink Faculty of Medicine, University Witten/Herdecke, Germany Abstract: Chronic idiopathic axonal polyneuropathy is a frequent diagnosis in patients suffering from idiopathic polyneuropathy and neuropathic pain. No guidelines exist on how to treat these patients. To date, there are no results available from randomized clinical trials, and mostly classical neuropathic analgesics are prescribed, such as amitriptyline and gabapentine. However, the usefulness of these drugs is limited, as many patients remain in pain despite treatment, or suffer debilitating side effects. Palmitoylethanolamide (PEA is a new analgesic compound, tested in more than 4,000 patients in various clinical trials in a variety of patients suffering from various neuropathic pain states. It is available in Europe and the USA as a food supplement under the brand name PeaPure, and it is available for medical purposes in Italy and Spain under brand names Normast and Pelvilen. We present a case series of seven patients with an electrophysiological confirmed diagnosis of chronic idiopathic axonal polyneuropathy, suffering from neuropathic pains, mostly refractory to previous analgesics. In all these patients, PEA reduced pain significantly, without side effects. PEA can be administered in addition to other analgesics, without negative drug–drug interactions, or can be used as a stand-alone analgesic. Due to a favorable ratio between efficacy and safety, PEA should be considered more often as a treatment for neuropathic pain. Keywords: CIAP, polyneuropathy, treatment, neuropathic

  3. 重组人白细胞介素-11联合长春新碱治疗激素无效特发性血小板减少性紫癜临床观察%Investigation of the Efficacy of Recombinant Human Interleukin-11 in Combination with Vincristine in Hormone Invalid Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    滕志

    2012-01-01

    目的 探讨重组人白细胞介素-Ⅱ (rhIL-Ⅱ)联合长春新碱治疗激素无效特发性血小板减少性紫癜(ITP)患者的临床疗效及安全性.方法 选取2008年12月~2011年12月在我院血液内科住院治疗的112例激素无效ITP患者为研究对象,应用随机数字表法将患者分为对照组和观察组.对照组患者给予长春新碱,而观察组患者则加用rhIL-Ⅱ进行治疗,比较对照组和观察组患者临床疗效及药物不良反应发生情况.结果 观察组患者治疗总有效率(85.71%)明显高于对照组的(69.64%),比较差异有统计学意义(P<0.05).两组患者不良反应发生率比较差异无统计学意义(P>0.05).结论 rhIL-Ⅱ联合长春新碱治疗激素无效ITP具有较好的疗效和安全性,值得在经济条件落后地区及基层医院推广.%Objective The purpose of this study is To investigate the Clinical efficacy and safety of recombinant human interleukin-II combined with vincristine in hormone invalid idiopathic thrombocytopenic purpura. Methods 112 patients with hormone invalid idiopathic thrombocytopenic purpura in our hospital from December 2008 to December 2011 were randomly divided into control group and observation group. Patients in the control group were treated with vincristine. Patients in the observation group were treated with recombinant human interleukin- II combined with vincristine. The efficacy and side effect were analyzed in the control group and observation group. Results The total efficient rate was higher in the observation group than control group (85.71% vs 69.64%,P0.05). Conclusion Recombinant human interleukin- II combined with vincristine obtained satisfactory efficacy and safety for hormone invalid idiopathic thrombocytopenic purpura. It is worth expanding in hospitals of regional basic-level economic conditions

  4. Acceptance and Commitment Therapy in the Rehabilitation of a Girl with Chronic Idiopathic Pain: Are We Breaking New Ground?

    Science.gov (United States)

    Asmundson, Gordon J. G.; Hadjistavropolous, Heather D.

    2006-01-01

    Wicksell and colleagues (2005) addressed the important and currently understudied realm of chronic idiopathic musculoskeletal pain treatment in children. They presented a brief overview of empirically supported treatments for children with chronic pain, detailed their case and intervention strategy, and offered conclusions regarding the potential…

  5. Linaclotide in Chronic Idiopathic Constipation Patients with Moderate to Severe Abdominal Bloating: A Randomized, Controlled Trial.

    Directory of Open Access Journals (Sweden)

    Brian E Lacy

    Full Text Available Abdominal bloating is a common and bothersome symptom of chronic idiopathic constipation. The objective of this trial was to evaluate the efficacy and safety of linaclotide in patients with chronic idiopathic constipation and concomitant moderate-to-severe abdominal bloating.This Phase 3b, randomized, double-blind, placebo-controlled clinical trial randomized patients to oral linaclotide (145 or 290 μg or placebo once daily for 12 weeks. Eligible patients met Rome II criteria for chronic constipation upon entry with an average abdominal bloating score ≥5 (self-assessment: 0 10-point numerical rating scale during the 14-day baseline period. Patients reported abdominal symptoms (including bloating and bowel symptoms daily; adverse events were monitored. The primary responder endpoint required patients to have ≥3 complete spontaneous bowel movements/week with an increase of ≥1 from baseline, for ≥9 of 12 weeks. The primary endpoint compared linaclotide 145 μg vs. placebo.The intent-to-treat population included 483 patients (mean age=47.3 years, female=91.5%, white=67.7%. The primary endpoint was met by 15.7% of linaclotide 145 μg patients vs. 7.6% of placebo patients (P<0.05. Both linaclotide doses significantly improved abdominal bloating vs. placebo (P<0.05 for all secondary endpoints, controlling for multiplicity. Approximately one-third of linaclotide patients (each group had ≥50% mean decrease from baseline in abdominal bloating vs. 18% of placebo patients (P<0.01. Diarrhea was reported in 6% and 17% of linaclotide 145 and 290 μg patients, respectively, and 2% of placebo patients. AEs resulted in premature discontinuation of 5% and 9% of linaclotide 145 μg and 290 μg patients, respectively, and 6% of placebo patients.Once-daily linaclotide (145 and 290 μg significantly improved bowel and abdominal symptoms in chronic idiopathic constipation patients with moderate-to-severe baseline abdominal bloating; in particular

  6. Neuroendoscopic treatment of idiopathic occlusion of unilateral foramen of Monro presenting as chronic headache

    Directory of Open Access Journals (Sweden)

    Dhaval Shukla

    2016-01-01

    Full Text Available Asymmetric ventriculomegly due to idiopathic occlusion of the foramen of Monro is rare. Such patients present with clinical features of raised intracranial pressure (ICP. Presentation as chronic headache has not been previously described. In the absence of raised ICP, pursuing surgical treatment raises a clinical dilemma as the headache may be a primary headache with no improvement after surgery. A 21-year-old woman presented with chronic headache. She was found to have asymmetric ventriculomegaly due to the occlusion of the foramen of Monro. She underwent endoscopic septostomy and widening of the foramen of Monro. Her headache subsided after surgery. At 15 months of follow-up, she was free from headache without medications. Unilateral occlusion of the foramen of Monro can present with asymmetric ventriculomegaly resulting in chronic headache. Though the symptoms of raised ICP may not be present, still endoscopic relief of ventriculomegaly leads to cure of headache.

  7. The Role of Food Additives and Natural Foods Containing Vasoactive Amines in Chronic Idiopathic Urticaria

    OpenAIRE

    Sami Ozturk; Fuat Erel; A.Zafer Caliskaner; Mehmet Karaayvaz; Mustafa Gulec; Ozgur Kartal

    2007-01-01

    Most patients with chronic idiopathic urticaria (CIU) need long-term treatment but there is little known about the prognosis of CIU. The aim of this study was to evaluate the natural course of CIU and to find out if there are risk factors that predict the prognosis. In this prospective study, we obtained data from patients first diagnosed and treated for CIU between September 2003 and September 2005. This study was included 157 patients with CIU. We observed duration of the disease, effects o...

  8. [Keratoderma palmoplantaris varians (striata et areata), a form of chronic idiopathic acrokeratosis described by Degos].

    Science.gov (United States)

    Padial, A; Morales, V; Armario-Hita, J C; Ingunza, J L; Fernández-Vozmediano, J M

    2008-03-01

    We report a case of a 15-year-old boy with hyperkeratotic lesions that were linear or striated on the palms and nummular on the soles. He was the only family member known to be affected, suggesting that the condition could be attributed to a de novo mutation or the recessive form of keratoderma palmoplantaris striata, described by Degos as chronic idiopathic acrokeratosis. The lesions did not improve with topical treatments (keratolytic agents, emollients, or corticosteroids) or oral retinoids. We observed that scratching of the affected areas was the main reason for deterioration of the lesions. PMID:18346435

  9. 成人特发性血小板减少性紫癜淋巴细胞亚群研究%Study on Changes and Clinical Significance of Lymphocyte Subtypes in Adult Patients with Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    孙雄飞; 陈卫布; 林海清; 伍娟

    2012-01-01

    目的 研究淋巴细胞亚群在成人特发性血小板减少性紫癜(ITP)治疗前后及正常对照人群中的表达变化,探讨其在ITP发病机制中的作用及临床意义.方法 应用流式细胞术检测23例ITP患者治疗前、后及23例正常对照者外周血淋巴细胞亚群的表达水平,包括CD 3+、CD3+CD4+、CD 3+CD 8+、CD 19+淋巴细胞百分比及CD 3+CD4+/CD 3+CD 8+比值.结果 ITP患者治疗前CD3+T淋巴细胞百分比、CD3+CD4+T淋巴细胞百分比及CD 3+CD4+/CD 3+CD 8+比值均较正常对照组及ITP治疗后组减低(P<0.05),CD 3+ CD 8+T淋巴细胞百分比及CD 19+B淋巴细胞百分比较正常对照组及ITP治疗后组升高(P<0.05),正常对照组与治疗后ITP组四项指标无明显统计学差异(P<0.05).结论 T、B淋巴细胞亚群异常参与了ITP的发病,对其检测可能对ITP的诊断及治疗有帮助.%Objective To study the changes in lymphocyte subsets in patients with idiopathic thrombocytopenic purpura (1TP) before and after therapy and healthy controls ,and then to explore the clinical significance of the related factors envolved in pathlgenesis of ITP. Methods The percentages of lymphocyte subsets in the perpheral blood of 23 patients with ITP and 23 heaithy controls were detected by flow cytometry, including CD 3+ , CD 3+ CD 4+, CD 3 + CD 8 VCD 19+ lymphocytes and CD 3 + CD 4+/ VCD 3 + CD 8+. Results The percentages of CD 3 + T lymphocyte, CD 4 + T lymphocyte and the ratio of CD 4 VCD 8 + in patients with ITP were obviously lower than those in healthy controls and ITP after immunosuppressive therapy ( P < 0. 05 ) , but the percentages of CD 3 + CD 8 + T lymphocyte and CD 19 + B lymphocyte was much higher ( P < 0.05) than those in heaithy controls and ITP afrer immunosuppressive therapy. There were no significant difference between the healthy controls and ITP afrer therapy (P <0.05) . Conclusions The aberrant changes in T and B lymphocyte subsets are involved in the pathogenesis of ITP

  10. 幽门螺杆菌感染与特发性血小板减少性紫癜关系的荟萃分析%Relationship between Helicobacter pylori infection and idiopathic thrombocytopenic purpura: a meta-analysis

    Institute of Scientific and Technical Information of China (English)

    谭勇; 施瑞华; 赵福军

    2009-01-01

    目的:评价特发性血小板减少性紫癜(ITP)与幽门螺杆菌(H pylori )感染的相关性.方法:检索PubMed、EMBase和CNKI数据库,获取ITP与H pylori 感染的病例-对照研究, 将入选的病例分成4组:H pylori 阳性并成功清除组; H pylori 阳性但清除不成功组; H pylori 阳性未清除治疗组; H pylori 阴性组. 每组均记录进入实验时各患者的基础血小板计数和随访时的血小板计数, 并在组内行荟萃分析.结果:检索文献中纳入21篇病例-对照研究, 其中国外17篇, 国内4篇. 在随访前后, H pylori阳性并成功清除组P LT计数有统计学意义(WMD 61.70, 95%CI:47.58-75.81); H pylori阳性但清除不成功组PLT计数无统计学意义(WMD 11.41, 95%CI:-0.07-22.88); H pylori阳性未清除治疗组P LT计数无统计学意义(WMD 15.77, 95%CI:-7.99-39.54); H pylori阴性组PLT计数有统计学意义(WMD 24.24,95%CI:8.54-39.93).结论:H pylor i 阳性的ITP患者在成功清除H pylori 后PLT计数上升, 但H pylori 感染并不是ITP发生的唯一因素.%AIM: To investigate the relationship between Helicobacter pylori (H pylori) infection and idiopathic thrombocytopenic purpura (ITP). METHODS: The databases of PubMed, EMBase and CNKI were searched for published case-control studies on association between H pylori infection and ITP. We focused on the difference in the platelet count between the experimental arm (H pylori-infected patients who responded to eradication therapy) and each control arm (H pylori-infected patients who failed to respond to eradication therapy; H pylori-infected patients without eradication therapy and H pylori-negative patients). Data were extracted using a standardized form and the meta-analysis was performed. RESULTS: Twenty-one eligible studies, including 17 studies carried by foreigners, and 4 by Chinese researchers, were included in the metaanalysis. There was a statistically significant difference in platelet count between patients who responded

  11. 儿童急性特发性血小板减少性紫癜125例临床分析%Clinical analysis of 125 cases of children with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    刘德新; 廖宁

    2012-01-01

    Objective To discuss the clinical features of children with idiopathic thrombocytopenic purpura (FTP). Methods The clinical data of 125 children with ITP and 3 different strategies of therapy were retrospectively analyzed. Results (1)lt occurred more commonly in small children, accounting for 60% of all cases, of which 37 cases (29.6% ) ranged from one month to one year old, 38 cases (30.4% ) ranged from one year to 3 years old. (2) Among all cases, 67 (53.6% ) had unclear causes, 58 (46.4% ) had clear causes, and 12 cases had vaccination histories within one month. (3)The bleeding symptoms could be effectively controlled by ACTH. The PLT counts increased dramatically after 3-day and 7-day treatment( P <0.01). Conclusions (1 )Childhood FTP occurred commonly in younger children. (2) More than half cases had prodromal infection histories. The incidence rate of FTP caused by vaccine injection was higher than previous reports. (3)The treatment of ACTH was effective for ITP, including oral prednisone, oral prednisone plus intravenous IVIG and intravenous DXM plus IYIG.%目的 探讨儿童特发性血小板减少性紫癜(ITP)的临床特征.方法 回顾性分析125例ITP住院患儿的临床资料,并对其发病年龄、诱因、出血情况及3种不同治疗方案的治疗效果进行分析.结果 (1)婴幼儿发病多见,其中年龄1个月~1岁者37例(29.6%),~3岁者38例(30.4%),≤3岁占所有病例数的60%;(2)起病诱因不明者67例(53.6%),诱因明确58例(46.4%),12例(9.6%)发病前1个月内有预防接种史;(3)以肾上腺糖皮质激素治疗为主的综合治疗可使出血症状很快消失;治疗后3、7d的血小板值与治疗前比较差异有显著性(P<0.01).结论 (1)儿童ITP发病年龄以婴幼儿期多见;(2)约半教患儿有前驱感染史,疫苗接种后ITP发生率高于以往报道;(3)泼尼松、泼尼松+静脉注射用人免疫球蛋白治疗以及地塞米松+静脉注射用人免疫球蛋白3

  12. 老年特发性血小板减少性紫癜患者的临床观察%Clinical study on the elderly patients with idiopathic/immune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    范芸; 常乃柏; 邢宝利; 裴蕾; 李元明; 顾惜春; 许晓东

    2008-01-01

    目的 总结老年特发性血小板减少性紫癜(ITP)患者的发病特点、治疗及临床转归.方法 回顾分析1992-2007年我院住院治疗的老年ITP患者的临床资料,并与同期住院的非老年患者的临床资料进行对照.结果 老年患者(老年组)43例,男性16例,女性27例;随访时间1个月~15年,存活35例.43例患者中,7例血小板持续(30~50)×109/L,出血不显著,未予以治疗;36例首选泼尼松治疗,敏感型25例(69.4%),以完全反应或部分反应健康存活;脾切除或栓塞4例,3例血小板恢复正常;对于泼尼松治疗不敏感者分别使用免疫抑制剂,其中硫唑嘌呤21例,环孢A23例,长春新碱3例及环磷酰胺9例,硫唑嘌呤、环孢A疗效优于长春新碱及环磷酰胺.进展为难治性ITP5例,难治率为13.9%;进展为未定性单克隆免疫球蛋白增多症(MGUS)和淋巴瘤各1例.死亡8例,死于外伤感染引发的心肺功能衰竭4例,肿瘤3例,脑出血1例.结论 老年ITP患者临床表现不典型,致命性出血的风险低,对免疫抑制剂的反应与非老年组近似,治疗宜个体化.%Objective To explore the clinical characteristics,therapy reactions and prognosis of the elderly patients with idiopathic thrombocytopenic purpura(ITP). Methods A total of 43elderly ITP patients(age≥60 years old)including 16 men and 27 women were reviewed and further followed up for 1 month to 15 years. Results Until June 2007,35 elderly ITP patients survived,platelet counts were sustained(30-50)×109/L in 7 cases,but no significant bleeding was found.Thirty-six patients had adrenocorticosteroid therapy first, 25 patients were sensitive to adrenocorticosteroid therapy,4 patients underwent splenectomy,and 3 patients achieved a normal platelet count. Immunosuppressive agents(vinscristine,cyclophosphamide, azathioprine and Cyclosporin A)treatments were held in 5 6 case-times,Cyclosporin A and azathioprine were more effective than vinscristine and cyclophosphamide

  13. Does chronic idiopathic dizziness reflect an impairment of sensory predictions of self-motion?

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    Joern K Pomper

    2013-11-01

    Full Text Available Most patients suffering from chronic idiopathic dizziness do not present signs of vestibular dysfunction or organic failures of other kinds. Hence, this kind of dizziness is commonly seen as psychogenic in nature, sharing commonalities with specific phobias, panic disorder and generalized anxiety. A more specific concept put forward by Brandt and Dieterich (1986 states that these patients suffer from dizziness because of an inadequate compensation of self-induced sensory stimulation. According to this hypothesis self-motion-induced reafferent visual stimulation is interpreted as motion in the world since a predictive signal reflecting the consequences of self-motion, needed to compensate the reafferent stimulus, is inadequate. While conceptually intriguing, experimental evidence supporting the idea of an inadequate prediction of the sensory consequences of own movements has as yet been lacking. Here we tested this hypothesis by applying it to the perception of background motion induced by smooth-pursuit eye movements. As a matter of fact, we found the same mildly undercompensating prediction, responsible for the perception of slight illusory world motion („Filehne illusion in the 15 patients tested and their age-matched controls. Likewise, the ability to adapt this prediction to the needs of the visual context was not deteriorated in patients. Finally, we could not find any correlation between measures of the individual severity of dizziness and the ability to predict. In sum, our results do not support the concept of a deviant prediction of self-induced sensory stimulation as cause of chronic idiopathic dizziness.

  14. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does...... of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

  15. Treatment of Idiopathic Chronic Orchialgia with Transcutaneous Electrical Nerve Stimulation (TENS:A Preliminary Result

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    Ekrem Akdeniz

    2016-01-01

    Full Text Available Purpose: Unilateral or bilateral testicular pain lasting more than 3 months is called as chronic orchialgia. Aproximately 25-50% of chronic orchialgia is idiopatic origin. This study aimed the effectiveness of Transcutaneous Electrical Nerve Stimulation (TENS therapy due to Idiopathic Chronic Orchialgia (ICO. Methods: Five patients were included into this study with ICO that diagnosed with physical examination, urine analyses, urinary system x-ray film, and scrotal doppler ultrasound. Medical history revealed that multiple conservative therapy attempts failed to alleviate the pain. Two of the patients had right sided ICO. Traditional TENS device is placed to the most painful points. TENS applied 3 times in a week with duration 30 minutes for 4 weeks. Before and after TENS application, patients were evaluated by using Visual Analog Scale (VAS at first and third months. Results: Median age of patients was 26.20±2.38 (22-30. Mean VAS value was 6.52 ± 0.89 before the procedure. After 1 month VAS value was 3.82 ± 0.83 (p0.05. None of the patients needed any analgesics after during the one month. No complications, hyperemia or hypoesthesia of the scrotal or penile skin, occurred after the procedure. Conclusion: TENS reduces pain by increasing endorphin release in the spinal cord dorsal horn. TENS is very effective method for first 1 month in patients with ICO but its effect reduces by the time. There is no standard therapeutic protocol for idiopathic chronic orchialgia. Therefore TENS may be an alternative for patients who do not benefit from medical therapy and do not want invasive procedures. Short-term use of TENS and low number of the patients are the limitations of this study. Randomized, placebo-controlled, and longer follow-up period studies are needed to better assess the efficacy of TENS for ICO.

  16. A comparative study of efficacy of cetirizine versus astemizole in chronic idiopathic urticaria

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    Vijay M

    1994-01-01

    Full Text Available Efficacy of Cetirizine and Astemizole was compared in 50 cases of chronic Idiopathic urticaria. Patients were divided into 2 groups viz A and B. Before starting the therapy, routine investigations were carried out to rule out any septic focus. Prior to treatment, 1 week before all medications were withdrawn. Cetirizine and Astemizole, 10mg each, were given as a single daily morning dose for 1 month and patients were followed up for 1 month. 40% excellent response was observed with Cetirizine while 20% excellent response with Astemizole. Good response was observed in 32% patients with Cetirizine and 28% with Astemizole. No side effects were observed except sedation, 8% in Cetirizine group and 20% with Astemizole group.

  17. Prospective study of biofeedback retraining in patients with chronic idiopathic functional constipation

    Institute of Scientific and Technical Information of China (English)

    Jun Wang; Mao-Hong Luo; Qing-Hui Qi; Zuo-Liang Dong

    2003-01-01

    AIM: To determine the efficacy and long-term outcome of biofeedback treatment for chronic idiopathic constipation and to compare the efficacy of two modes of biofeedback (EMG-based and manometry-based biofeedback).METHODS: Fifty consecutive contactable patients included 8 cases of slow transit constipation, 36 cases of anorectic outlet obstruction and 6 cases of mixed constipation. Two modes of biofeedback were used for these 50 patients, 30 of whom had EMG-based biofeedback, and 20 had manometrybased biofeedback. Before treatment, a consultation and physical examination were done for all the patients, related information such as bowel function and gut transit time was documented, psychological test (symptom checkJist 90, SCL90)and anorectic physiological test and defecography were applied. After biofeedback management, all the patients were followed up. The Student′s t-test, chi-squared test and Logistic regression were used for statistical analysis.RESULTS: The period of following up ranged from 12 to 24months (Median 18 months). 70% of patients felt that biofeedback was helpful, and 62.5% of patients with constipation were improved. Clinical manifestations including straining, abdominal pain, bloating, were relieved, and less oral laxative was used. Spontaneous bowel frequency and psychological state were improved significantly after treatment. Patients with slow and normal transit, and those with and without paradoxical contraction of the anal sphincter on straining, benefited equally from the treatment. The psychological status rather than anorectal test could predict outcome. The efficacy of the two modes of biofeedback was similar without side effects.CONCLUSION: This study suggests that biofeedback has a long-term effect with no side effects, for the majority of patients with chronic idiopathic constipation unresponsive to traditional treatment. Pelvic floor abnormalities and transit time should not be the selection criteria for treatment.

  18. Clinical observation and nursing of Chinese medicine combined with recombinant interleukin-11 in treating refractory idiopathic thrombocytopenic purpura%中药联合重组白介素-11治疗难治性特发性血小板减少性紫癜的临床观察和护理

    Institute of Scientific and Technical Information of China (English)

    李洪琴; 陈波; 江桂林; 张静静; 石焕玉; 霍长亮

    2014-01-01

    目的:探讨中药联合重组白介素-11治疗难治性特发性血小板减少性紫癜的临床疗效和护理措施。方法将52例肾性贫血难治性特发性血小板减少性紫癜患者随机分成2组,治疗组26例给予重组人白介素-11(巨和粒)治疗,皮下注射,每天1次,连用14 d,每个月为1个疗程,并口服中药汤剂(由黄芪、生地、仙鹤草、水牛角、丹皮、紫珠草等组成),日1剂,早晚分服;对照组26例只给予重组人白介素-11(巨和粒)治疗。2组均配合专科护理干预措施,均治疗2个月。结果治疗组总有效率为88.5%,对照组为65.4%,2组比较差异有统计学意义(P <0.05)。结论中药联合重组白介素-Ⅱ对难治性特发性血小板减少性紫癜有显著疗效。%ABSTRACT:Objective To explore the clinical curative effect and nursing of Chinese medicine combined with recombinant interleukin-2 in treating refractory idiopathic thrombocytopenic purpu-ra.Methods 52 renal anemia refractory idiopathic thrombocytopenic purpura patients were ran-domly divided into treatment group and control group.The treatment group with 26 cases was given subcutaneous injection of recombinant human interleukin-2(Juhe Granule)for 1 time daily,con-tinuous treatment lasted for 14 days per month and 1 months was a course of treatment.Besides, Chinese medicine decoction(composed of radix astragali,radix rehmanniae,herba agrimoniae,buf-falo horn,cortex moutan,callicarpa macrophylla vahl,etc)was orally taken for 1 agent daily for 2 times in the morning and evening.The control group with 26 cases was given only recombinant hu-man interleukin-2 (Juhe Granule).The course of the treatment in the two groups was lasted for 2 months and nursing intervention measures were conducted during the treatment.Results The ef-fective rate was 88.5% in the treatment group and 65.4% in the control group.Comparison of the two groups showed that the

  19. Study of Autophagy and Microangiopathy in Sural Nerves of Patients with Chronic Idiopathic Axonal Polyneuropathy

    Science.gov (United States)

    Samuelsson, Kristin; Osman, Ayman A. M.; Angeria, Maria; Risling, Mårten; Mohseni, Simin; Press, Rayomand

    2016-01-01

    Twenty-five percent of polyneuropathies are idiopathic. Microangiopathy has been suggested to be a possible pathogenic cause of chronic idiopathic axonal polyneuropathy (CIAP). Dysfunction of the autophagy pathway has been implicated as a marker of neurodegeneration in the central nervous system, but the autophagy process is not explored in the peripheral nervous system. In the current study, we examined the presence of microangiopathy and autophagy-related structures in sural nerve biopsies of 10 patients with CIAP, 11 controls with inflammatory neuropathy and 10 controls without sensory polyneuropathy. We did not find any significant difference in endoneurial microangiopathic markers in patients with CIAP compared to normal controls, though we did find a correlation between basal lamina area thickness and age. Unexpectedly, we found a significantly larger basal lamina area thickness in patients with vasculitic neuropathy. Furthermore, we found a significantly higher density of endoneurial autophagy-related structures, particularly in patients with CIAP but also in patients with inflammatory neuropathy, compared to normal controls. It is unclear if the alteration in the autophagy pathway is a consequence or a cause of the neuropathy. Our results do not support the hypothesis that CIAP is primarily caused by a microangiopathic process in endoneurial blood vessels in peripheral nerves. The significantly higher density of autophagy structures in sural nerves obtained from patients with CIAP and inflammatory neuropathy vs. controls indicates the involvement of this pathway in neuropathy, particularly in CIAP, since the increase in density of autophagy-related structures was more pronounced in patients with CIAP than those with inflammatory neuropathy. To our knowledge this is the first report investigating signs of autophagy process in peripheral nerves in patients with CIAP and inflammatory neuropathy. PMID:27662650

  20. Complex cytogenetic findings in the bone marrow of a chronic idiopathic myelofibrosis patient

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    Tuğçe Bulakbaşı Balcı

    2010-06-01

    Full Text Available Chronic idiopathic myelofibrosis is a myeloproliferative disorder characterized by splenomegaly, myeloid metaplasia and reactive bone marrow fibrosis. Karyotype analysis of the bone marrow is an integral part of the diagnosis, especially as a discriminative tool in ruling out reactive conditions. The frequency of clonal cytogenetic anomalies in this disease is the highest among its group, varying between 30 and 75%. Among these, trisomy 1q, 20q-, 13q- and +8 are the most common aberrations. Here we report a 66-year-old male patient whose bone marrow biopsy revealed signs of chronic myeloproliferative changes and dysmegakaryopoiesis. He was administered hydroxyurea treatment, splenic radiotherapy and multiple transfusions. The patient worsened in the following months and the second bone marrow biopsy revealed myelofibrosis. Cytogenetic analysis of this bone marrow sample revealed a complex karyotype reported to be 46,XY,del(9(q22q34,t(8;17;21(q22;q21;q22[23]/46,XY[2], with a previously undefined three-way translocation and deletion in chromosome 9. The patient died shortly thereafter.

  1. Idiopathic Chronic Eosinophilic Pneumonia: Retrospective Analysis of 17 Cases from a Single Center in Turkey

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    Sibel Arınç

    2016-08-01

    Full Text Available Objective: Idiopathic chronic eosinophilic pneumonia (ICEP is a rare eosinophilic lung disorder with an unknown etiology and is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or peripheral eosinophilia, and the accumulation of eosinophils in the lungs. We aimed to present diagnostic test results and follow-up outcomes of 17 patients who were diagnosed with ICEP in our hospital in light of literature. Methods: Between 2008 and 2013, we examined 17 cases of ICEP. We evaluated clinical and laboratory findings together with the long-term follow-up data. Results: The patients had a mean age of 40.8 years at presentation, and the female/male ratio was 0.8. The most common symptoms were cough (94%, shortness of breath (76%, and high fever (35%. Bronchoalveolar lavage eosinophil percentages of the patients ranged from 3% to 80%. Nine (53% patients experienced recurrence. Six patients were maintained on low dose steroid due to repeating relapses. Among these patients, 7 (77.7% had a total IgE level of above 500/IU/mL. Conclusion: Relapses are common in ICEP after the withdrawal of corticosteroid treatment or during dose reduction. We point out the importance of the close monitoring of patients for identifying relapse. A higher total IgE level during diagnosis may serve as a predictor of recurrence.

  2. Extensive Molecular Analysis Suggested the Strong Genetic Heterogeneity of Idiopathic Chronic Pancreatitis

    Science.gov (United States)

    Sofia, Valentina Maria; Da Sacco, Letizia; Surace, Cecilia; Tomaiuolo, Anna Cristina; Genovese, Silvia; Grotta, Simona; Gnazzo, Maria; Petrocchi, Stefano; Ciocca, Laura; Alghisi, Federico; Montemitro, Enza; Martemucci, Luigi; Elce, Ausilia; Lucidi, Vincenzina; Castaldo, Giuseppe; Angioni, Adriano

    2016-01-01

    Genetic features of chronic pancreatitis (CP) have been investigated extensively, mainly by testing genes associated to the trypsinogen activation pathway. However, different molecular pathways involving other genes may be implicated in CP pathogenesis. A total of 80 patients with idiopathic chronic pancreatitis (ICP) were investigated using a Next-Generation Sequencing (NGS) approach with a panel of 70 genes related to six different pancreatic pathways: premature activation of trypsinogen, modifier genes of cystic fibrosis phenotype, pancreatic secretion and ion homeostasis, calcium signaling and zymogen granules (ZG) exocytosis, autophagy and autoimmune pancreatitis-related genes. We detected mutations in 34 out of 70 genes examined; of the 80 patients, 64 (80.0%) were positive for mutations in one or more genes and 16 (20.0%) had no mutations. Mutations in CFTR were detected in 32 of the 80 patients (40.0%) and 22 of them exhibited at least one mutation in genes of other pancreatic pathways. Of the remaining 48 patients, 13/80 (16.3%) had mutations in genes involved in premature activation of trypsinogen and 19/80 (23.8%) had mutations only in genes of the other pathways: 38 (59.3%) of the 64 patients positive for mutations showed variants in two or more genes. Our data, although to be extended with functional analysis of novel mutations, suggest a high rate of genetic heterogeneity in CP and that trans-heterozygosity may predispose to the ICP phenotype. PMID:27264265

  3. Recurrent Acute Myocardial Infarction in Patients with Immune Thrombocytopenic Purpura

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    Fengyi Shen

    2014-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP, also known as idiopathic thrombocytopenic purpura, is an acquired immune-mediated disease of adults and children characterized by a transient or persistent decrease of platelets and, depending upon the degree of thrombocytopenia, an increased risk of bleeding. The use of standard treatments for acute myocardial infarction (AMI, such as antiplatelet agents and anticoagulants, pose serious problems in patients with ITP due to the potential higher risk of bleeding complications. There are no current guidelines available for management of ITP patients with AMI. In this brief review of the limited available literature, we discuss the proposed pathophysiological link between ITP and arterial thrombosis and the challenging medical and interventional treatment of these patients.

  4. PrimiOtic and PrimiOtic Plus: novel probiotic for primates suffering from idiopathic chronic diarrhea.

    Science.gov (United States)

    Lecker, Jaime L; Froberg-Fejko, Karen

    2015-10-01

    Idiopathic chronic diarrhea of nonhuman primates is a major gastrointestinal disorder and a leading cause of serious morbidity in nonhuman primates kept in captivity. Many animals are not responsive to traditional treatments. Millions of dollars are spent annually on diagnosis and supportive care of these animals. Probiotics like Bio-Serv's PrimiOtic and PrimiOtic Plus can help to reduce the incidence of diarrhea in captive nonhuman primates by supporting the natural microflora in the gut. PMID:26398619

  5. Chronic Recurrent Multifocal Osteomyelitis with Concomitant Features of Juvenile Idiopathic Arthritis

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    Elena Tsitsami

    2011-01-01

    Full Text Available We report a case of a 13-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO who developed severe arthritis in four different joints within the first year from the onset of the disease. Her multiple vertebrae lesions showed significant amelioration after a 2-month treatment with prednisolone. In parallel, the initial severe symmetrical arthritis of both knees showing overt synovitis and joint effusion, in the absence of lesions in the metaphyses of the femur or the tibia, responded remarkably well in intra-articular triamcinolone hexacetonide injections. However, upon discontinuation of prednisolone, the patient developed severe arthritis of her right ankle and the proximal interphalangeal joint of her right middle finger. Thus, prednisolone was reinitiated combined with methotrexate, and the patient went into remission, which persists one year after prednisolone tapering. The appearance of arthritis in both knees in the absence of bone lesions and the emergence of severe arthritis of the ankle after remission of spinal bone lesions suggest that CRMO and juvenile idiopathic arthritis may coexist and be causally related.

  6. Loratadine versus levocetirizine in chronic idiopathic urticaria: A comparative study of efficacy and safety

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    Anuradha P

    2010-01-01

    Full Text Available Background : Treatment of chronic idiopathic urticaria (CIU is challenging because of its unpredictable course and negative influence on the quality of life. New treatments are being developed, but antihistaminics remain the cornerstone of the therapeutic approach. Newer generation antihistaminics such as loratadine and levocetirizine have already proved to be safe and efficacious for CIU. Objective : To choose the better drug between loratadine and levocetirizine for CIU, by comparing their efficacy and safety. Methods : A randomized, open, outdoor-based clinical study was conducted on 60 patients of CIU, to compare the two drugs. After initial clinical assessment and baseline investigations, loratadine was prescribed to 30 patients and levocetirizine to another 30 patients for four weeks. At follow-up, the patients were re-evaluated and then compared using different statistical tools. Result : The comparative study showed that the changes in differential eosinophil count (P = 0.006 and absolute eosinophil count (P = 0.003 in the levocetirizine group was statistically significant. The results of the Total Symptom Score showed better symptomatic improvement of CIU with levocetirizine as compared to loratadine. The overall incidence of adverse drug reactions was also found to be less in the levocetirizine group. Conclusion : An analysis of the results of all the parameters of safety and efficacy proves the superiority of levocetirizine over loratadine for CIU.

  7. Chronic idiopathic urticaria, psychological co-morbidity and posttraumatic stress: the impact of alexithymia and repression.

    Science.gov (United States)

    Hunkin, Victoria; Chung, Man Cheung

    2012-12-01

    The objective of this study was to investigate the interrelationship between chronic idiopathic urticaria (CIU), psychological co-morbidity, posttraumatic stress, repression and alexithymia. 89 participants with CIU and 105 without CIU responded to an online questionnaire. Both groups completed the general health questionnaire-12, the perceived stress scale, the posttraumatic stress diagnostic scale and the Toronto alexithymia scale-20 and were categorised into four defence mechanism groups (repressive, defensive, high-anxious, low-anxious). CIU participants also completed the Skindex-17 and a self-report severity measure. CIU participants reported higher levels of alexithymia than the control group and their defence mechanism was most likely to be categorised as defensive, with conscious self-image management reported alongside high manifest anxiety. Partial least squares analysis revealed significant paths between posttraumatic stress and CIU severity and psychological co-morbidity. Posttraumatic stress was associated with alexithymia and type of defence mechanism. Only being in the high-anxious group partially mediated the relationship between posttraumatic stress and CIU severity. In conclusion, there is evidence for a relationship between CIU and trauma. The severity of posttraumatic symptoms varies depending upon alexithymic traits and defence mechanisms used. Disease severity and psychological co-morbidity are differentially influenced by the relationships between trauma, alexithymic traits and defence mechanisms. PMID:22362490

  8. The Role of Food Additives and Natural Foods Containing Vasoactive Amines in Chronic Idiopathic Urticaria

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    Fuat Erel

    2007-10-01

    Full Text Available Most patients with chronic idiopathic urticaria (CIU need long-term treatment but there is little known about the prognosis of CIU. The aim of this study was to evaluate the natural course of CIU and to find out if there are risk factors that predict the prognosis. In this prospective study, we obtained data from patients first diagnosed and treated for CIU between September 2003 and September 2005. This study was included 157 patients with CIU. We observed duration of the disease, effects of food additives and preservatives in CIU. As possible prognostic factors we observed sex, age, atopy, intolerance to food additives and preservatives. Allergic reactions were seen to appear in 37% (n=50 cases due to natural foods, in 36% (n=49 cases due to foods containing additives, and in 27% (n=37 cases due to both natural foods and foods containing additives. For patients with CIU, food colors, sweeteners and preservatives that are added into foods are an important etiological factor. Moreover, histamine and histamine-like endogen pharmacological agents can cause allergic reactions. Hence, these foods should be taken into consideration in etiology especially in patients with CIU, and due to potential etiology, elimination of patients from these foods for a while is a significant step in treatment. [TAF Prev Med Bull. 2007; 6(5: 351-356

  9. Living with Thrombotic Thrombocytopenic Purpura

    Science.gov (United States)

    ... Some people fully recover from thrombotic thrombocytopenic purpura (TTP). However, relapses (flareups) can occur in many people who have acquired and inherited TTP. If you've had TTP, call your doctor ...

  10. What Causes Thrombotic Thrombocytopenic Purpura?

    Science.gov (United States)

    ... protein in the blood) causes thrombotic thrombocytopenic purpura (TTP). The ADAMTS13 gene controls the enzyme, which is ... enough enzyme activity causes overactive blood clotting. In TTP, blood clots form in small blood vessels throughout ...

  11. 特发性血小板减少性紫癜患儿血小板膜糖蛋白表达及临床意义%Expression of platelet membrane glycoproteins in pediatric idiopathic thrombocytopenic purpura and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    王娜; 柴忆欢; 何海龙; 李建琴; 曹岚; 曹丽娟

    2013-01-01

    Objective To explore the relationship between the expression of platelet membrane glycoprotein in pediatric idiopathic thrombocytopenic purpura (ITP) and its clinical significance.Methods A modified monoclonal antibody immobilization of platelet antigen (MAIPA) method was used to detect the positive expression rates of 4 platelet membrane glycoproteins (GP Ⅰ b/Ⅸ,GP Ⅰ b,GP Ⅲ a,and GP Ⅰ b) in 80 pediatric patients with ITP.The correlation was explored between the GP positive rate and the clinical efficacy in pediatric ITP.Trying to observe the correlationship between the GP positive rate of pediatric ITP in the total,the different gender,the acute and chronic and the treatment response rate in pediatric ITP respectively.Results There was a significant difference in curative rate statistically between the GP positive group and the GP negative group(x2 =8.535,P < 0.01) in 80 pediatric ITP patients,but no statistic difference in curative rate existed between the 36 female and 44 male(x2 =0.013,P >0.05).Markedly statistic difference was found in the female(x2 =4.433,P < 0.05),the same to the male (x2 =4.156,P < 0.05).Meanwhile,there was an extremely statistic difference between 67 acute and 13 chronic patients(x2 =23.513,P < 0.001).Apparently statistic difference also occurred in the acute (x2 =4.157,P < 0.05),but not in the chronic cases (x2 =0.410,P > 0.05).Conclusions The clinical response rate is significantly correlated with the GP positive rate in pediatric ITP,but not correlated with gender.The GP positive rate can reflect the disease status of pediatric ITP to a certain extent and be used as an indicator for judging the efficacy and monitor prognosis of pediatric ITP.%目的 检测儿童特发性血小板减少性紫癜(ITP)血小板膜糖蛋白(GP)的表达并探讨其临床意义.方法 采用改良单克隆抗体特异性俘获血小板抗原(MAIPA)法检测80例ITP患儿GP Ⅰ b/Ⅸ、GPⅡb、GPⅢa、GPⅠ b的阳性表达率.分别

  12. Correlation of serum antithyroid microsomal antibody and autologous serum skin test in patients with chronic idiopathic urticaria

    Directory of Open Access Journals (Sweden)

    Snehal Balvant Lunge

    2015-01-01

    Full Text Available Background: About 25-45% of patients of chronic urticaria (CU have been stated to have histamine releasing autoantibodies in their blood. The term autoimmune urticaria is increasingly being accepted for this subgroup of patients. Review of the literature suggests high autologous serum skin test (ASST positivity and presence of antithyroid microsomal antibodies in patients with autoimmune urticaria. Aims: To study prevalence of ASST positivity and antithyroid microsomal antibodies in chronic "idiopathic" urticaria and to study the correlation between the two parameters. Methods: All patients of chronic idiopathic urticaria satisfying inclusion/exclusion criteria were enrolled in the study after written informed consent. Patients of CU secondary to infections and infestations, physical urticaria including dermatographism, mastocytosis, urticarial vasculitis and those on treatment with immunosuppressive drugs for urticaria were excluded from the study. In all of these patients, complete blood count; ASST, serum T3/T4/thyroid stimulating hormone levels, antithyroid microsomal antibody (AMA levels were done. Statistical analysis was done by Chi-square test, Fisher exact test and Kappa statistics. Results: Study included 24 males and 26 females with mean age of 39.54 years. Majority of patients belonged to 20-40 years of age. Females showed more ASST positivity. A total of 12 out of 50 (24% patients showed positive ASST. A total of four out of 12 (33.33% had positive ASST and raised AMA levels. Conclusion: Only 25% of patients of chronic idiopathic urticaria had positive ASST. ASST and AMA levels were positively correlated in our study. Further studies are required to authenticate this association.

  13. How Is Thrombotic Thrombocytopenic Purpura Treated?

    Science.gov (United States)

    ... Is Thrombotic Thrombocytopenic Purpura Treated? Thrombotic thrombocytopenic purpura (TTP) can be fatal or cause lasting damage, such ... it's not treated right away. In most cases, TTP occurs suddenly and lasts for days or weeks, ...

  14. Autoantibody production in chronic idiopathic urticaria is not associated with Helicobacter pylori infection

    Directory of Open Access Journals (Sweden)

    Atta A.M.

    2004-01-01

    Full Text Available Chronic idiopathic urticaria (CIU is a dermatological syndrome, characterized by raised erythematous skin lesions, that affects 20% of the general population and has been associated with autoimmunity. However, some reports have also suggested a close relationship between CIU and Helicobacter pylori infection, which is endemic in developing countries and associated with chronic gastritis, peptic ulcer disease, and gastric carcinoma. In the present study, we investigated the occurrence of autoantibodies in sera from 23 CIU subjects infected with H. pylori and from 23 CIU subjects without this infection. The presence of anti-thyroid antibodies was determined by indirect hemagglutination assay and the presence of autoantibodies to IgE and C1INH was determined by ELISA. Antibodies to thyroid antigens were detected at low titers from 100 to 400 in three of 23 (13% CIU-infected subjects and in four of 23 (17% CIU-noninfected subjects. The titers of anti-IgE autoantibodies were similar in these CIU groups, presenting absorbances of 1.16 ± 0.09 and 1.07 ± 0.16, respectively, while a titer of 1.14 ± 0.15 was detected in the healthy control group. The concentration of anti-C1INH autoantibodies was the same in the CIU-infected and -noninfected subjects (7.28 ± 1.31 and 7.91 ± 2.45 ng/ml, respectively, and was 7.20 ± 2.25 ng/ml in the healthy control group. However, the serum levels of complexed anti-C1INH antibodies were increased in CIU-infected subjects compared to CIU-noninfected subjects and healthy controls with an absorbance of 1.51 ± 0.21 vs 1.36 ± 0.16 and 1.26 ± 0.23, respectively (P < 0.05, indicating an impaired clearance of immune complexes in CIU-infected patients. In conclusion, no correlation was observed between H. pylori infection and autoantibody production in CIU patients consistent with reports of clinical studies.

  15. Efficacy and safety of omalizumab in patients with chronic idiopathic/spontaneous urticaria who remain symptomatic on h1 antihistamines

    DEFF Research Database (Denmark)

    Saini, Sarbjit S; Bindslev-Jensen, Carsten; Maurer, Marcus;

    2015-01-01

    ASTERIA I was a 40-week, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of subcutaneous omalizumab as add-on therapy for 24 weeks in patients with chronic idiopathic urticaria/spontaneous urticaria (CIU/CSU) who remained symptomatic despite H1 antihistamine....... The omalizumab 300-mg group met all nine secondary end points, including a significant decrease in the duration of time to reach minimally important difference response (⩾5-point decrease) in weekly ISS (Purticaria activity score over 7...

  16. Impact of chronic Immune Thrombocytopenic Purpura (ITP on health-related quality of life: a conceptual model starting with the patient perspective

    Directory of Open Access Journals (Sweden)

    George James N

    2008-02-01

    Full Text Available Abstract Background Immune thrombocytopenic purpura (ITP, a condition characterized by autoimmune-mediated platelet destruction and suboptimal platelet production, is associated with symptoms such as bruising, epistaxis, menorrhagia, mucosal bleeding from the gastrointestinal and urinary tracts and, rarely central nervous system bleeding. The aim of this research is to develop a conceptual model to describe the impact of ITP and its treatment on patients' health-related quality of life (HRQoL. Methods A literature search and focus groups with adult ITP patients were conducted to identify areas of HRQoL affected by ITP. Published literature was reviewed to identify key HRQoL issues and existing questionnaires used to assess HRQoL. Focus group transcripts were reviewed, and common themes were extracted by grouping conceptual categories that described the impact on HRQoL. Results The literature synthesis and themes from the focus group data suggest that decreased platelet counts, disease symptoms, and treatment side effects influence multiple domains of HRQoL for ITP patients. Key areas affected by ITP and its treatments include emotional and functional health, work life, social and leisure activities, and reproductive health. Conclusion ITP affects various areas of HRQoL. This conceptual model will help inform the evaluation of therapeutic strategies for ITP.

  17. Progress of CD4 + CD25 + regulatory T cells in pathogenesis of idiopathic thrombocytopenic purpura%CD4+CD25+调节性T细胞在特发性血小板减少性紫癜发病机制中的作用

    Institute of Scientific and Technical Information of China (English)

    刘小群

    2011-01-01

    CD4 + CD25 + regulatory T cells(Treg) are thought to be a subgroup of mature CD4 + T cells.Forkhead winged helix transcription factor-3 (Foxp3)is specifically expressed on them and plays a key role in their development and function. CD4 + CD25 + Treg cells can maintain the stabilization of internal environment by two principal pathways to suppress the immunological function: the direct suppression of the target cells by cell-contact and the secretion of suppressor cytokines. At present, it has been considered that decreased number and dysfunction of CD4+ CD25+ Treg cells are closely related to pathogenesis of autoimmune disease. Recent findings show that CD4+ CD25+ Treg cells play an important role in pathogenesis of idiopathic thrombocytopenic purpura.%CD4+ CD25+调节性T细胞(regulatory T cell,Treg)是一种成熟的CD4+T细胞亚群,而叉头翼状螺旋转录因子3(forkhead winged helix transcription factor-3,Foxp3)特异性表达于该细胞上,对其发育和功能起关键作用。CD4+ CD25+ Treg细胞主要通过直接接触和分泌抑制性细胞因子两大途径发挥免疫抑制功能,维持机体内环境的稳定。目前认为其数目减少和功能障碍与自身免疫性疾病的发生密切相关。近年来研究表明CD4+ CD25+ Treg细胞在特发性血小板减少性紫癜的发病中起重要作用。

  18. Idiopathic fibrosing pancreatitis: a rare cause of chronic obstructive jaundice in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hye Seong; Kim, Hyun Sook; Kim, Hack Hee; Kim, Ok Hwa; Kim, Choon Yul; Bahk, Yong Whee [Catholic Medical College, Seoul (Korea, Republic of)

    1992-01-15

    We report a 14-months-old infant who had obstructive jaundice caused by idiopathic fibrosing pancreatitis. Ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic ducts, common bile duct, and the pancreatic duct. Diffuse swelling of the pancreas was also noted on CT. At laparotomy, the head portion of the pancreas revealed a stony hard consistency, and proliferation of fibrotic tissue was confirmed pathologically. Idiopathic fibrosing pancreatitis is a very rare disease entity in childhood, but should be considered in the differential diagnosis of obstructive jaundice in children who demonstrate bile duct and pancreatic duct dilatation and/or diffuse pancreas swelling.

  19. ADAMTS13 Deficiency and Thrombotic Thrombocytopenic Purpura Associated with Trimethoprim-Sulfamethoxazole

    OpenAIRE

    Bapani, Sowjanya; Epperla, Narendranath; Kasirye, Yusuf; Mercier, Richard; Garcia-Montilla, Romel

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a hematological disease characterized by microangiopathic hemolytic anemia and thrombocytopenia. Although the link between ADAMTS13 deficiency and idiopathic TTP has been well-established, the role of trimethoprim-sulfamethoxazole (TMP-SMX) in the pathogenesis of TTP is not yet well elucidated. To the best of our knowledge, there have been only two previous reports linking this medication with the development of TTP. We present the case of a health...

  20. Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

    OpenAIRE

    Niaz Faraz; Aleem Aamer

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE) and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to ant...

  1. Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

    OpenAIRE

    Aline Maria Yamaguti Rios Paes da Silva; Fernanda Alves Barbosa; Philipe Vianna de Barros; João Tadeu Damian Souto Filho; Gustavo Fernandes Ribas

    2011-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD) is ext...

  2. Thrombotic thrombocytopenic purpura in childhood

    NARCIS (Netherlands)

    M.C. Bouw; N. Dors; H. van Ommen; N.L. Ramakers-van Woerden

    2009-01-01

    Thrombotic thrombocytopenic puripura (TTP) is a rare disease, especially in childhood, and has a high mortality rate in the absence of appropriate treatment. it is characterised by microangiopathic haemolytic anaemia and consumptive thrombocytopenia. TTP may be difficult to distinguish from haemolyt

  3. The Role of Amifostine in Treating Idiopathic Thrombocytopenic Purpura%氨磷汀在治疗特发性血小板减少性紫癜中的作用

    Institute of Scientific and Technical Information of China (English)

    靳英; 李明阳; 刘波; 张伟; 朱宏丽

    2012-01-01

    特发性血小板减少性紫癜(ITP)并不少见,该病诊断不难,治疗方法多.但本例患者高龄、既往治疗出现副作用,用药受限,常规药物效果不佳,经氨磷汀治疗而使患者得以痊愈,目前已随访观察5个月无复发.结合文献,氨磷汀对高龄、难治性病例安全有效,提示氨磷汀可能是ITP治疗中一种较有潜力的药物.%This is to discuss Amifostine mechanism by analyzing one case with Idiopathic Thromhocytopenic Purpura (ITP). ITP is not difficult to diagnozed. Many drugs are used to treat it. One 92 —year—old male patient with 1TP was not ameliorated by routine treatment. Finally he was cured by Amifostine . Then he was followed —up for 5 months without recurrence. We can say that Amifostine is safe and effective, further more it is possible a potentional medicine to cure elderly serious ITP.

  4. Juvenile idiopathic arthritis

    Science.gov (United States)

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It is thought to be an autoimmune illness . This means the body attacks ...

  5. Linaclotide-a novel secretagogue in the treatment of irritable bowel syndrome with constipation and chronic idiopathic constipation.

    Science.gov (United States)

    Sharma, Shivani; Sharma, Tina; Dhingra, Richa; Tomar, Prince; Singh, Sukhminder; Malhotra, Manav; Bhardwaj, T R

    2013-10-01

    Irritable bowel syndrome with constipation (IBS-C) and chronic idiopathic constipation (CIC) are highly prevalent gastrointestinal disorders. Traditional symptoms based therapies had somewhat limited success and efficacy in addressing the disorders. Recently, linaclotide emerged as novel peptide capable of improving abdominal symptoms in patients suffering from IBS-C and CIC. Guanylate cyclase C (GC-C) receptor a multi domain protein, found to be molecular target for linaclotide which acts by activating GC-C receptor on the apical surface of intestinal epithelial cells. Binding of linaclotide to GC-C receptor triggers the elevation of second messenger cGMP that elicits fluid secretion into intestinal cells which play a critical role in maintaining homeostasis through cystic fibrosis transmembrane conductance regulator (CFTR). Data from Phase II and III clinical trials demonstrated that linaclotide seems to produce a statistically significant increase in stool frequency, improved straining, decreased abdominal pain and discomfort. PMID:24001336

  6. Association of human parvovirus B19 infection and childhood idiopathic thrombocy-topenic purpura: a meta analysis of Chinese literatures%人微小病毒B19感染与儿童特发性血小板减少性紫癜关系的meta分析

    Institute of Scientific and Technical Information of China (English)

    张耀东; 胡群; 刘双又; 刘爱国; 王冠玲; 熊昊; 孙燕

    2009-01-01

    Objective To study the relationship between human parvovirus B19 infection and childhood idiopathic thrombocytopenic purpura ( FTP) by the principle of evidence based medicine. Methods Papers related to the relationship between human parvovirus B19 infection and childhood ITP published between 1994 and 2008 were retrieved electronically from the Chinese Journals Full-text Database and the Wanfang Data. These relevant papers on case-control trials were statistically studied by meta analysis. Results Eight papers that met the inclusion criteria were included for this meta analysis. Five hundred and sixteen cases of childhood ITP and 246 healthy controls were enrolled. The meta analysis showed that the incidence of human parvovirus B19 infection in the ITP group was significantly higher than that in the control group ( OR = 13. 71, 95% CI = 7. 07-26. 59, Z = 7. 75, P < 0. 01). Conclusions Human parvovirus B19 infection is closely associated with childhood ITP.%目的 综合分析人微小病毒B19感染与儿童特发性血小板减少性紫癜(ITp)的关系.方法 计算机检索中国期刊全文数据库、万方数据库,检索时间从1994~2008年,纳入人微小病毒B19感染与儿童ITP关系的病例对照研究,对文献数据进行meta分析.结果 共纳入8个研究,其中病例组516例,对照组246例.meta分析显示人微小病毒B19感染在病例组的表达高于对照组(OR=13.71,95% CI=7.07~26.59,Z=7.75,P<0.01).结论 人微小病毒B19感染与儿童ITP的发病有密切相关性.

  7. Serum Cobalamin (Vitamin B12) Concentrations in Rhesus Macaques (Macaca mulatta) and Pigtailed Macaques (Macaca nemestrina) with Chronic Idiopathic Diarrhea.

    Science.gov (United States)

    Izzi, Jessica M; Beck, Sarah E; Adams, Robert J; Metcalf Pate, Kelly A; Hutchinson, Eric K

    2016-01-01

    Chronic diarrhea poses a significant threat to the health of NHP research colonies, and its primary etiology remains unclear. In macaques, the clinical presentation of intractable diarrhea and weight loss that are accompanied by inflammatory infiltrates within the gastrointestinal tract closely resembles inflammatory bowel disease of humans, dogs, and cats, in which low serum and tissue cobalamin (vitamin B12) levels are due to intestinal malabsorption. We therefore hypothesized that macaques with chronic idiopathic diarrhea (CID) have lower serum cobalamin concentrations than do healthy macaques. Here we measured serum cobalamin concentrations in both rhesus and pigtailed macaques with CID and compared them with those of healthy controls. Serum cobalamin levels were 2.5-fold lower in pigtailed macaques with CID than control animals but did not differ between rhesus macaques with CID and their controls. This finding supports the use of serum cobalamin concentration as an adjunct diagnostic tool in pigtailed macaques that present with clinical symptoms of chronic gastrointestinal disease. This use of serum vitamin B12 levels has implications for the future use of parenteral cobalamin supplementation to improve clinical outcomes in this species.

  8. Haemolytic uraemic syndrome and thrombocytopenic thrombotic purpura

    NARCIS (Netherlands)

    Zijlstra, JG

    1997-01-01

    Haemolytic uraemic syndrome thrombocytopenic thrombotic purpura (HUS/TTP) remains an incompletely understood complex disease process that involves many organs. It was first described, as thrombocytopenic purpura, by Moschcowitz in 1924 (1). Since that time the prognosis of this disease has improved

  9. Changes of serum IL-6, IL-18 and IFN-γ in children with idiopathic thrombocytopenic purura%IL-6、IL-18和IFN-γ在特发性血小板减少性紫癜中的水平变化

    Institute of Scientific and Technical Information of China (English)

    路桂云

    2013-01-01

    目的 通过测定特发性血小板减少性紫癜(ITP)患儿血清中白细胞介素-6(IL-6)、白细胞介素-18(IL-18)和γ-干扰素(IFN-γ)的含量变化,探讨IL-6、IL-18和IFN-γ在ITP的发病机制中的作用.方法 选择ITP急性发作期患儿40例(急性组),经治疗后处于缓解期的患儿35例(缓解组),另选择30名健康儿童作为对照(对照组),采用酶联免疫法(ELISA)检测血清IL-6、IL-18和IFN-γ水平变化,并进行统计学分析比较.结果 ITP患儿血清IL-6、IL-18和IFN-γ水平急性期组明显高于缓解期组和对照组,缓解期组亦高于对照组,差异均有统计学意义(P均<0.05).结论 IL-6、IL-18和IFN-γ可能均参与了ITP的发病机制,通过测定血清IL-6、IL-18和IFN-γ水平可以用来判断ITP的病情和预后.%Objective To study the content changes of serum IL-6,IL-18 and IFN-γ in children with idiopathic thrombocytopenic purpura (ITP),and to investigate the role in the pathogenesis of IL-6,IL-18 and IFN-γin ITP.Methods Active group (n =40) and remission group (n =35) with ITR were picked up,and 30 healthy children served as normal controls.IL-6,IL-18 and IFN-γ levels were measured by ELISA,and the results were compared.Results The serum levels of IL-6,IL-18 and IFN-γ in children with active group were markedly higher than those in remission group and healthy controls,and the serum levels of IL-6,IL-18 and IFN-γin remission group were markedly higher than those in healthy controls (P < 0.05).Conclusions IL-6,IL-18 and IFN-γmaybe involved in the pathogenesis of ITP.Determination of IL-6,IL-18 and INF-γ levels can be used to judge the ITP' s condition and prognosis.

  10. 特发性血小板减少性紫癜患者血清白细胞介素11水平、淋巴细胞亚群及NK细胞变化的临床意义%Clinical significance of the serum level interleukin-11, lymphocyte subsets and NK cells in idiopathic thrombocytopenic purpura patients

    Institute of Scientific and Technical Information of China (English)

    张红梅; 李艳敏

    2011-01-01

    目的 检测特发性血小板减少性紫癜(ITP)患者血清白细胞介素11(IL-11)水平、淋巴细胞亚群及NK细胞的变化,探讨相关因素在ITP发病中的作用.方法 应用酶联免疫吸附法(ELISA)、流式细胞术分别检测50例ITP(ITP组)和30例健康体检者(对照组)血清IL-11水平、淋巴细胞亚群及NK细胞的变化.结果 ITP组的血小板计数[(30.21±19.40)×109/L]明显低于对照组[(207.21±31.55)×109/L](P<0.05),ITP组患者血清IL-11水平[(255.72±163.43)ng/L]明显高于对照组[(40.60±5.57)ng/L](P<0.05),相关分析表明ITP患者血清IL-11水平与血小板计数呈负相关(r=-0.557,P<0.05);ITP组患者CD3+、CD4+T淋巴细胞百分比及CD4+/CD8+明显低于对照组(P<0.05),CD8+T淋巴细胞百分比明显高于对照组(P<0.05);CD3-CD(16+56)+NK细胞百分比明显低于对照组(P<0.05).结论 IL-11水平、淋巴细胞亚群及NK细胞变化与ITP的发病密切相关,且IL-11水平与血小板计数可能存在负反馈调节作用.%Objective To detect the serum level of interleukin (IL)-1 1, lymphocyte subsets and NK cells in patients with idiopathic thrombocytopenic purpura (ITP), and explore the related factors in the pathogenesis of ITP. Methods The serum level of IL-11, lymphocyte subsets and NK cells were detected by double antibody sandwich enzyme linked immunosorbent assay (ELISA) and flow cytometry in 50 ITP patients (ITP group) and 30 controls (control group). Results The platelet in ITP group [ (30.21 ± 19.40) ×109/L] was lower than that in control group [ (207.21 ± 31.55 ) × 109/L] obviously (P < 0.05 ); the serum level of IL-11 in ITP group [(255.72 ± 163.43) ng/L] was significantly higher than that in control group [ (40.60 ± 5.57 ) ng/L ] (P < 0.05 ). The correlation analysis indicated that the blood serum levels of IL- 11 had negative relationship with the platelet (r = -0.557 ,P < 0.05). The percentage of CD3+ and CD4+ T lymphocyte percentage, CD4+/CD8+ in ITP

  11. Minimal Important Difference (MID of the Dermatology Life Quality Index (DLQI: Results from patients with chronic idiopathic urticaria

    Directory of Open Access Journals (Sweden)

    Leahy Michael

    2005-05-01

    Full Text Available Abstract Background The Dermatology Quality Life Index (DLQI has seen widespread use as a health-related quality of life measure for a variety of dermatological diseases. The purpose of this study was to estimate the minimal important difference (MID on the DLQI for patients with chronic idiopathic urticaria (CIU. Methods Data from 2 Phase III clinical trials of patients (N = 476 for Study A; N = 468 for Study B with CIU were analyzed separately to estimate the MID for the DLQI for these populations. Both distributional based and anchor based approaches were used for deriving estimates. The anchor based approach relied upon patient self assessments of pruritus severity; the distributional based approaches relied upon estimating the standard error of measurement, as well as one-half the standard deviation of the DLQI from each study. Results The distributional approaches resulted in estimates of MID ranging from 2.24 to 3.10 for the two studies. The anchor based approach resulted in estimates of 3.21 and 2.97 for the two studies. Conclusion An MID for the DLQI in the range of 2.24 to 3.10 is recommended in interpreting results for patients with CIU.

  12. How Is Thrombotic Thrombocytopenic Purpura Diagnosed?

    Science.gov (United States)

    ... Diagnosed? Your doctor will diagnosis thrombotic thrombocytopenic purpura (TTP) based on your medical history, a physical exam, and test results. If TTP is suspected or diagnosed, a hematologist will be ...

  13. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Christopher Coleman; Aziza Nassar; Barbara McComb

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two n...

  14. Successful rituximab treatment in an elderly patient with recurrent thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Matsubara, Etsuko; Yamanouchi, Jun; Hato, Takaaki; Takeuchi, Kazuto; Niiya, Toshiyuki; Yasukawa, Masaki

    2016-07-01

    An 81-year-old man presenting with fever, neurological symptoms, thrombocytopenia, and hemolytic anemia was diagnosed with acquired idiopathic thrombotic thrombocytopenic purpura (TTP). His disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity was TTP recurrence based on ADAMTS13 activity TTP in Japan, we report the efficacy and safety of rituximab in an elderly patient with recurrent TTP. We suggest that rituximab therapy should be started as soon as possible for recurrent TTP in patients with high titers of ADAMTS13 inhibitor. PMID:27498731

  15. COMPARATIVE EFFICACY OF CHRONIC HEART FAILURE TREATMENT WITH PERINDOPRIL OR CARVEDILOL IN PATIENTS WITH ALCOHOLIC OR IDIOPATHIC DILATED CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Zhirov

    2015-09-01

    Full Text Available Aim. To study the clinical and hemodynamic efficacy of monotherapy with ACE inhibitor perindopril or beta-blocker carvedilol in patients with chronic heart failure (CHF due to dilated cardiomyopathy (DCMP of various etiology.Material and methods. Patients (n=69 with DCMP of different etiology were included into the open randomized study. Idiopathic DCMP (IDCMP was diagnosed in 26 patients and alcoholic cardiomyopathy (ACMP - in 43 patients. Patients of IDCMP and ACMP groups were randomized for treatment with perindopril (groups 1 and 3, respectively or carvedilol (groups 2 and 4, respectively. Follow-up was 6 months. End-diastolic and end-systolic left ventricular volume, stroke volume index, ejection fraction (EF and exercise capacity were determined at baseline and in 2 and 6 months of treatment. Safety of the treatments was also assessed.Results. Group 1: the average CHF class (NYHA decreased by 20.7% (p<0.01, EF increased by 18.2% (p<0.05. Group 2: the average CHF class decreased by 29.6% (p<0.01, EF increased by 18.2% (p<0.05. Group 3: the average CHF class decreased by 14.3% (p<0.01, EF increased by 19.6% (p<0.05. Group 4: the average CHF class decreased by 41.4% (p<0.001, EF increased by 32.8% (p<0.001.Conclusion. Monotherapy with carvedilol in patients with ACMP was more effective than this with perindopril. Long-term monotherapy with perindopril or carvedilol in patients with DCMP was well tolerated and safety.

  16. Therapeutic helminth infection of macaques with idiopathic chronic diarrhea alters the inflammatory signature and mucosal microbiota of the colon.

    Directory of Open Access Journals (Sweden)

    Mara Jana Broadhurst

    Full Text Available Idiopathic chronic diarrhea (ICD is a leading cause of morbidity amongst rhesus monkeys kept in captivity. Here, we show that exposure of affected animals to the whipworm Trichuris trichiura led to clinical improvement in fecal consistency, accompanied by weight gain, in four out of the five treated monkeys. By flow cytometry analysis of pinch biopsies collected during colonoscopies before and after treatment, we found an induction of a mucosal T(H2 response following helminth treatment that was associated with a decrease in activated CD4(+ Ki67+ cells. In parallel, expression profiling with oligonucleotide microarrays and real-time PCR analysis revealed reductions in T(H1-type inflammatory gene expression and increased expression of genes associated with IgE signaling, mast cell activation, eosinophil recruitment, alternative activation of macrophages, and worm expulsion. By quantifying bacterial 16S rRNA in pinch biopsies using real-time PCR analysis, we found reduced bacterial attachment to the intestinal mucosa post-treatment. Finally, deep sequencing of bacterial 16S rRNA revealed changes to the composition of microbial communities attached to the intestinal mucosa following helminth treatment. Thus, the genus Streptophyta of the phylum Cyanobacteria was vastly increased in abundance in three out of five ICD monkeys relative to healthy controls, but was reduced to control levels post-treatment; by contrast, the phylum Tenericutes was expanded post-treatment. These findings suggest that helminth treatment in primates can ameliorate colitis by restoring mucosal barrier functions and reducing overall bacterial attachment, and also by altering the communities of attached bacteria. These results also define ICD in monkeys as a tractable preclinical model for ulcerative colitis in which these effects can be further investigated.

  17. COMPARATIVE EFFICACY OF CHRONIC HEART FAILURE TREATMENT WITH PERINDOPRIL OR CARVEDILOL IN PATIENTS WITH ALCOHOLIC OR IDIOPATHIC DILATED CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Zhirov

    2013-01-01

    Full Text Available Aim. To study the clinical and hemodynamic efficacy of monotherapy with ACE inhibitor perindopril or beta-blocker carvedilol in patients with chronic heart failure (CHF due to dilated cardiomyopathy (DCMP of various etiology.Material and methods. Patients (n=69 with DCMP of different etiology were included into the open randomized study. Idiopathic DCMP (IDCMP was diagnosed in 26 patients and alcoholic cardiomyopathy (ACMP - in 43 patients. Patients of IDCMP and ACMP groups were randomized for treatment with perindopril (groups 1 and 3, respectively or carvedilol (groups 2 and 4, respectively. Follow-up was 6 months. End-diastolic and end-systolic left ventricular volume, stroke volume index, ejection fraction (EF and exercise capacity were determined at baseline and in 2 and 6 months of treatment. Safety of the treatments was also assessed.Results. Group 1: the average CHF class (NYHA decreased by 20.7% (p<0.01, EF increased by 18.2% (p<0.05. Group 2: the average CHF class decreased by 29.6% (p<0.01, EF increased by 18.2% (p<0.05. Group 3: the average CHF class decreased by 14.3% (p<0.01, EF increased by 19.6% (p<0.05. Group 4: the average CHF class decreased by 41.4% (p<0.001, EF increased by 32.8% (p<0.001.Conclusion. Monotherapy with carvedilol in patients with ACMP was more effective than this with perindopril. Long-term monotherapy with perindopril or carvedilol in patients with DCMP was well tolerated and safety.

  18. DNA methyltransferase 3B (DNMT3B -579G>T) promotor polymorphism and the susceptibility to pediatric immune thrombocytopenic purpura in Egypt.

    Science.gov (United States)

    Khorshied, Mervat Mamdooh; El-Ghamrawy, Mona Kamal

    2012-12-10

    Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by increased platelet destruction. Although the etiology of ITP remains unclear, it is accepted that both environmental and genetic factors play an important role in the development of the disease. The present study aimed at exploring a novel molecular determinant that may influence the susceptibility and course of ITP in Egyptian children. To achieve our aim, genotyping of DNMT3B -579G>T promotor polymorphism by polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) assay. The current study was conducted on 140 ITP patients and 150 age and gender matched healthy controls. The results obtained revealed that DNMT3B -579 TT homotype was significantly higher in ITP patients and conferred almost three fold increased risk of ITP (OR=3.16, 95%CI=1.73-5.79). There was no statistically significant difference between ITP patients with wild or mutant genotypes as regards their clinical or laboratory data. Furthermore, there was no statistical difference in the distribution of DNMT3B -579G>T genotypes between acute and chronic ITP patients. In conclusion, DNMT3B -579G>T promotor polymorphism represents a novel genetic risk factor for ITP but not a predictor for tendency to chronicity in pediatric ITP in Egypt.

  19. 特发性血小板减少性紫癜患儿血清IL-6,sIL-2R的变化及意义%Changes of serum IL-6 and sIL-2R levels in patients with idiopathic thrombocytopenic purpura and their clinical significance

    Institute of Scientific and Technical Information of China (English)

    张国利; 张剑白; 蒋丽鑫

    2009-01-01

    Objective To study the changes and the clinical significance of serum IL-6 and sIL-2R levels in children with idiopathic thrombocytopenic purpura( ITP). Methods The levels of serum IL-6 and sIL-2R were measured in 37 ITP children with sandwich ELSIA as well as in 20 healthy children. At the same time, PAIb' s qualitative determination was checked in 25 ITP children. And the relationships of PLT counts,IL-6 and sIL-2R's level in ITP children' s peripheral blood were analyzed. Results The levels of ITP children' s IL-6 and sIL-2R were significantly higher than those of control group(P <0. 01). The levels of IL-6 and sIL-2R were dramatically higher in the group of ITP children before treatment responsive to after treatment(P <0.01 ). The levels of severe hemorrhage group' s IL-6, sIL-2R were significantly higher than those of hyporrhea group' s( P < 0.05 ). Nine of 25 ITP children' s PAIb was completely positive(9/25) ,the levels of ITP children's IL-6, sIL-2R in completely positive group were significantly higher than non-positive group' s( P < 0. 05 ). The levels of ITP children' s IL-6, sIL-2R ( before treating) were negative correlation with PLT counts. The level between ITP children' s IL-6 and sIL- 2R was positive correlation. Conclusion PIL-6 and sIL-2R participate in ITP' s procedure. The levels change with the pathogenetic condition and the course of disease. To sum up, cell mediated immunity plays a role in ITP' s procedure as well as humoral-mediated immunity.%目的 探讨特发性血小板减少性紫癜(ITP)患儿血清中细胞因子白介素6(IL-6)、可溶性白介素2受体(sIL-2R)的变化及临床意义.方法 采用双抗体夹心酶联免疫(ELISA)定量测定方法检测37例ITP患儿和20例正常健康儿童血清中IL-6、sIL-2R水平,同时对其中25例ITP患儿进行血小板相关抗体PAIb(IgG、IgA、IgM)的定性测定,并对ITP患儿外周血中血小板计数、血清IL-6、sIL-2R水平进行相关分析.结论 ITP患儿血清IL-6

  20. Changes and Signiifcance of Peripheral BloodγδT Cells and IL-17 and IL-23 Levels in Patients With Idiopathic Thrombocytopenic Purpura%成人特发性血小板减少性紫癜患者外周血γT细胞及IL-17、IL-23水平的变化和意义

    Institute of Scientific and Technical Information of China (English)

    杨国雷; 张复华; 凌奕文; 牛国敏

    2015-01-01

    目的:通过检测ITP患者外周血γδT细胞、IL-17与IL-23水平的变化,进一步探讨ITP的发病机制,为ITP的防治新途径提供理论依据。方法采用流式细胞术检测外周血γδT细胞;酶联免疫吸附试验方法检测 IL-17、IL-23。实验组分正常对照组和ITP组;ITP组分为治疗前组与治疗后组(有效组、无效组);ITP组初始均予地塞米松10 mg/d,此后口服强的松维持并逐渐减量,总疗程1个月。结果正常对照组外周血γδT、IL-17、IL-23分别为(6.41±1.76)%,(43.04±6.10)pg/ml,(34.03±3.64)pg/ml;ITP治疗前组:γδT、IL-17、IL-23分别为(14.90±2.41)%,(76.67±11.86)pg/ml,(63.06±12.08)pg/ml;治疗有效组:γδT、IL-17、IL-23分别为(7.19±1.29)%,(53.95±11.97)pg/ml,(37.12±3.89)pg/ml;治疗无效组:(12.87±2.11)%,(62.51±10.11)pg/ml,(58.42±10.08)pg/ml。结论 ITP患者外周血γδT细胞、IL-17、IL-23水平较正常人高,γδT细胞、IL-17、IL-23参与了ITP的发生发展;ITP患者治疗有效者γδT细胞、IL-17、IL-23水平下降。%Objective To explore the pathogenesis of idiopathic thrombocytopenic purpura (ITP), and provide theoretical basis for new pathways to the prevention and control of ITP, through testing γδT cells, IL-17 and IL-23 in peripheral blood of ITP patients. Methods γδT cells in peripheral blood was testing by flow cytometry. IL-17 and IL-23 in peripheral blood was testing by enzyme-linked immunosorbent assay. Experiment group was divided into normal control group and ITP patient group. ITP patient group was divided into pre-treatment group and treatment group (effective and non-effective group). ITP patient group was initially given dexamethasone 10 mg/d, following oral prednisone (reduced gradually), total course lasting a month. ResultsγδT cells, IL-17 and IL-23 in peripheral blood of normal control group were (6.41±1.76)%, (43.04± 6

  1. Comparison of anti-D immunoglobulin and high-dose intravenous immunoglobulin therapy in childhood acute idiopathic thrombocytopenic purpura:a systematic review and meta-analysis%抗-D免疫球蛋白与大剂量静脉丙种球蛋白治疗儿童急性特发性血小板减少性紫癜的meta分析

    Institute of Scientific and Technical Information of China (English)

    泥永安; 刘壮; 廖必才

    2013-01-01

    Objectives To compare the effectiveness and safety of intravenous anti-D immunoglobulin (IV anti-D) with high-dose intravenous immunoglobulin (IVIG) as initial treatments of acute idiopathic thrombocytopenic purpura (ITP) in children. Methods Randomized controlled trials comparing anti-D with high-dose IVIG in the treatment of childhood acute ITP were systematically reviewed from PubMed, Embase and Cochrane Central Register of Controlled Trials and hand-searched reference lists. The number of patients with a platelet count>20 × 109/L at 72 hours after treatment initia-tion, and the decrease in hemoglobin were the primary outcomes. The meta-analysis was performed by RevMan 5.1. Results A total of 771 relevant articles were retrieved, and ifve studies were included. The RR (anti-D versus high dose IVIG) of achieving a platelet count>20×109/L at 72 hours was 0.90 (95%CI:0.82~0.98). However, subgroup analysis suggested no signiifcant difference between anti-D at a dose of 50μg/kg and high-dose IVIG (RR 0.98, 95% CI: 0.84~1.13), as well as between anti-D at a dose of 75μg/kg and high-dose IVIG (RR 0.88, 95%CI:0.75~1.03). Hemoglobin drop was greater in the anti-D group. No patients, however, required transfusions of erythrocyte suspensions. Conclusions IV anti-D may be as ef-fective as high-dose IVIG in the treatment of childhood acute ITP at 72 hours after therapy. The side effects of anti-D were tolerated and acceptable.%  目的比较静脉注射抗-D免疫球蛋白(anti-D immunoglobulin,anti-D)与大剂量静脉注射免疫球蛋白(IVIG)治疗儿童急性特发性血小板减少性紫癜(ITP)的有效性及安全性。方法计算机检索PubMed、Embase和Cochrane Central Register of Controlled Trials。手工查阅计算机检索到的文献的参考文献目录。选取治疗72 h后血小板计数>20×109/L的百分率和血红蛋白下降值作为主要测量指标。采用RevMan 5.1对纳入文献进行meta分析。

  2. 升血小板胶囊联合激素治疗特发性血小板减少性紫癜有效性的系统评价%Effectiveness of Platelet Increasing Capsule Combined with Hormone in Treating Idiopathic Thrombocytopenic Purpura: A Systematic Review

    Institute of Scientific and Technical Information of China (English)

    叶华觅; 郝建萍; 赵芳; 王欢; 郭新红

    2013-01-01

    Objective To systematically evaluate the effectiveness of platelet increasing capsule combined with hormone in treating idiopathic thrombocytopenic purpura (ITP).Methods Randomized controlled trials (RCTs) of ITP treated by platelet increasing capsule combined with hormone were electronically searched from PubMed (1966 to 2012),The Cochrane Library (CENTRAL,Issue 3,2012),CBM (1978 to 2012),CNKI (1979 to 2012),WanFang Data (1998 to 2012),and VIP (1991 to 2012).References of included studies were also retrieved.The literature was independently screened according to exclusion and inclusion criteria by two researchers independently and meta-analysis was conducted using RevMan 5.1 software after data extraction and quality assessment.Results 10 RCTs were included involving 588 patients.The results meta-analysis showed that,the group which was treated by platelet increasing capsule combined with hormone was superior to the hormone alone group in the aspects of overall effectiveness rate (RR=1.18,95%CI 1.06 to 1.32,P=0.003),the significant effectiveness rate (RR=1.57,95%CI 1.29 to 1.91,P<0.000 01),blood platelet count (MD=21.54,95%CI 13.85 to 29.23,P<0.000 01),and the recurrence rate (RR=0.49,95%CI 0.34 to 0.69,P<0.000 01) which was lower.Conclusion Current evidence has showed that platelet increasing capsule combined with hormone in treating ITP is better than hormone alone.However,more high quality RCTs are needed to verify the above conclusion in future.%目的 系统评价升血小板胶囊联合激素治疗特发性血小板减少性紫癜(ITP)的有效性.方法 计算机检索PubMed (1966~2012)、CENTRAL(2012年第3期)、CBM (1978~2012)、CNKI (1979~2012)、Wanfang Data (1998~2012)、VIP (1991~2012)等数据库,收集升血小板胶囊联合激素治疗特发性血小板减少性紫癜的随机对照试验(RCT),并追溯纳入研究的参考文献.由两位研究者按照纳入与排除标准独立筛选文献、提取资料和评价质

  3. 慢性特发性荨麻疹从腠理论治%Chronic Idiopathic Urticaria Treatment from Striae and Interstitial Space

    Institute of Scientific and Technical Information of China (English)

    王兴兰; 杨恩品

    2012-01-01

    Striae and interstitial space doctrine is an important part of the theory of traditional Chinese medicine, striae and interstitial space are microscopic gaps of the tissue and the cell, such as a place that defensive qi, body fluid perfuse and transform, and playing an important function of guarding outside. Guarding outside dose not firm, striae and interstitial space being loose is a key cause and pathogenesis of chronic idiopathic urticaria. Strengthen superficies and replenish striae is an effective measure to treat chronic idiopathic urticaria.%腠理学说是中医理论的重要组成部分,腠理属组织、细胞间的细微间隙,是卫气、津液等精微物质灌注、运行、转化的场所,发挥着重要的卫外功能.卫外不固、腠理不密是引起慢性特发性荨麻疹的病机关键,固卫实腠是治疗慢性特发性荨麻疹的有效措施.

  4. Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy

    Directory of Open Access Journals (Sweden)

    Alwin Tilanus

    2015-01-01

    Full Text Available We present a case of a male patient with chronic renal insufficiency, due to crescentic glomerulonephritis with IgA deposits, who successively developed (idiopathic thrombocytopenic purpura (ITP and MPO-ANCA microscopic polyangiitis (MPA with pulmonary fibrosis. The patient presented with cough, weight loss, and dyspnea on exertion. CT imaging and pulmonary function tests were compatible with interstitial pneumonitis with pulmonary fibrosis. Laboratory results showed high MPO-ANCA titers; the urinary sediment was bland. The patient was treated successfully with cyclophosphamide and methyl-prednisolone. This unique case illustrates the diagnostic and therapeutic challenges of an unusual presentation of microscopic polyangiitis presenting first as isolated kidney disease with recurrence in the form of pneumonitis without renal involvement, in association with renal IgA deposits and ITP as coexisting autoimmune conditions.

  5. Safety and Efficacy Study of Romiplostim to Treat ITP in Pediatric Subjects

    Science.gov (United States)

    2016-01-13

    Idiopathic Thrombocytopenic Purpura; Thrombocytopenia; Thrombocytopenia in Pediatric Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenia in Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenic Purpura; Immune Thrombocytopenia

  6. 婴幼儿与3岁以上儿童特发性血小板减少性紫癜的临床对比%Comparison study of idiopathic thrombocytopenic purpura in the children below 3 years old with above 3 years old

    Institute of Scientific and Technical Information of China (English)

    刘巧; 宪莹; 戴碧涛; 徐酉华; 肖剑文; 王世一; 陆玲玲; 于洁

    2009-01-01

    小板上升速度快于单用激素治疗.%Objective To study the clinical features of pediatric patients with idiopathic thrombocytopenic purpura (1TP) from infancy to adolescence, and compare their treatment response and the effects. Methods The data of the ITP children diagnosed and administrated between 2006~2008 were collected and analyzed by soft ware SPSS 12.0. The patients were divided into infant group ( 3 years old). The gender, demographic information, platelet count at the diagnosis, bleeding and anemia, virus infection and megakaryocytes, and most importantly, the treatment response were evaluated. Results (1 )The percentage of the male in infant group (66%) was statistically higher than that (50.6% ) in the elder group ( P = 0.031). (2) The incidence of epistaxis in the infant group (9.7% , n - 10) was statistically lower than that (43.7%) in the elder group (P < 0.001). The incidence of bleeding-caused anemia in the infant group (11.7%) was statistically lower than that (43.7%) in the elder children (P = 0.001). (3) The incidence of viral enteritis as a motivation in the infant group (14.56% ) was statistically higher than that (1.15%) in the elder group (P = 0.001); the incidence of vaccine inoculation as a motivation in the infant group (8.74%) was statistically higher than that (1.15%) in the elder group (P = 0.045). About 32.32% was cytomegalovi-rus-positive in the infant group while 2.35% in the elder group (P < 0.001). (4)The percentage of EB virus positive in infant group (4.04%) was statistically lower than that (14.12% ) in the elder patients(P - 0.016). (5) Both the median and the increase ratio of megakrocytes in infant group ( 144,81.7%) were statistically lower than that (280,96.6%) in the elder group (P = 0.005 ; P = 0.002). (6) Un-der the treatment of steroids only, the time for the platelet to become normal in infant group(7.1 ±2.6) was statistically shorter than that( 10.3±3.2) in the elder children(P< 0.001). Under the treatment

  7. Idiopathic anaphylaxis.

    Science.gov (United States)

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented. PMID:17493503

  8. 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.

  9. Efficacy of vincristine in combination with interleukin-11 treatment of refractory idiopathic thrombocytopenic purpura%长春新碱联合白介素11治疗难治性特发性血小板减少性紫癜疗效观察

    Institute of Scientific and Technical Information of China (English)

    刘国锋

    2013-01-01

    目的探讨长春新碱联合重组人白介素11治疗难治性血小板减少性紫癜的疗效方法。方法通过分析我院2006年3月~2011年2月间符合23例难治性血小板减少性紫癜的临床资料,采用长春新碱联合重组人白介素11进行治疗。结果显效8例,良9例,进步2例,无效4例,总有效率达73.9%(17/23)。结论长春新碱联合重组人白介素11治疗难治性ITP疗效切实可行,不良反应小。%Objective:Vincristine recombinant human interleukin 11 in the treatment of refractory thrombocytopenic purpura efficacy. Methods :By analyzing our hospital from March 2006 to February 2011 in line with the clinical data of 23 patients with refractory thrombocytopenic purpura, vincristine recombinant human interleukin-11 treatment. Results: Effective in 8 cases, good in 9 cases, progress in the two cases, 4 cases, the total effective rate of 73.9% (17/23). Conclusion: Vincristine recombinant human interleukin-11 treatment of refractory ITP efficacy practicable adverse reactions.

  10. Plasma exchange in thrombotic thrombocytopenic purpura.

    OpenAIRE

    Toffelmire, E B; Clark, W. F.; Cordy, P. E.; Linton, A. L.; Lohmann, R. C.

    1984-01-01

    Three patients were recently treated for thrombotic thrombocytopenic purpura (TTP). One presented with toxic shock syndrome; TTP developed but promptly responded to a regimen of antiplatelet agents, steroids and plasma exchange. In another the manifestations of TTP developed after presentation with hypertension and abdominal pain. This patient responded to a similar regimen but required extended treatment before remission could be maintained with medications alone. In the third patient the fu...

  11. The Kidney in Thrombotic Thrombocytopenic Purpura

    OpenAIRE

    Tsai, Han-Mou

    2007-01-01

    The kidney is commonly affected in thrombotic thrombocytopenic purpura (TTP), a multi-system disorder with microvascular thrombosis of the capillaries and arterioles. Nevertheless, due to difference in its diagnostic criteria, the frequency and severity of renal dysfunction in TTP remains controversial. With the recent studies indicating that severe deficiency of a VWF cleaving protease, ADAMTS13, is the main cause of platelet thrombosis in TTP, it is now possible to define TTP at the molecul...

  12. Idiopathic scoliosis.

    Science.gov (United States)

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit.

  13. The variation of immature platelet fraction in patients with thrombocytopenic diseases%未成熟血小板分数在血小板减少性疾病中的变化

    Institute of Scientific and Technical Information of China (English)

    蒋伟燕; 江明华; 吴义忠; 章赵华; 陈小剑

    2013-01-01

    目的 了解血小板减少性疾病患者外周血未成熟血小板分数(IPF)、高荧光强度未成熟血小板分数(H-IPF)、未成熟血小板绝对值(IPF#)和血小板平均侧向荧光强度(PLT-X)的变化,探讨其在血小板减少性疾病中的临床意义.方法 选取血小板减少性疾病86例[特发性血小板减少性紫癜(ITP)50例、再生障碍性贫血(AA)15例、急性白血病(AL)21例]、骨髓增生性疾病(MPD)32例[慢性粒细胞白血病(CML)11例、原发性血小板增多症(ET)16例、真性红细胞增多症(PV)5例]和健康对照者50名.应用SYSMEX XE-5000全自动血液分析仪检测各疾病组及健康对照组外周血血小板(PLT)、IPF、H-IPF、IPF#和PLT-X.将ITP组按PLT计数结果分为≤30×109/L、(>30~0.05).ITP各组间IPF差异无统计学意义(P>0.05).结论检测血小板相关参数(IPF、H-IPF和PLT-X)可能有助于血小板减少性疾病的鉴别诊断.%Objective To investigate the variation of immature platelet fraction ( IPF ), high-fluorescence intensity of immature platelet fraction ( H-IPF ), absolute value of immature platelet ( IPF#) and mean side fluorescence intensity of platelet( PLT-X ) in patients with thrombocytopenic diseases and their clinical significance in the thrombocytopenic diseases. Methods The platelet ( PLT), IPF, H-IPF, IPF# and PLT-X of peripheral blood in 86 patients with thrombocytopenic diseases [ 50 cases of idiopathic thrombocytopenic purpura ( ITP), 15 cases of aplastic anemia ( AA ) and 21 cases of acute leukemia ( AL ) ], 32 patients with myeloprolif erative disorders ( MPD ) [ 11 cases of chronic myelogenous leukemia ( CML), 16 cases of essential thrombocythemia( ET ) and 5 cases of polycythemia vera ( PV ) ] and 50 healthy subjects were determined by automatic hematology SYSMEX XE-5000 analyzer. According to the results of PLT, the 50 cases of ITP were classified into ≤30 × 109/L, ( >30- 0. 05 ). There was no statistical significance for IPF in the

  14. MRC chronic Dyspnea Scale: Relationships with cardiopulmonary exercise testing and 6-minute walk test in idiopathic pulmonary fibrosis patients: a prospective study

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    Roussos Charis

    2010-05-01

    Full Text Available Abstract Background Exertional dyspnea is the most prominent and disabling feature in idiopathic pulmonary fibrosis (IPF. The Medical Research Chronic (MRC chronic dyspnea score as well as physiological measurements obtained during cardiopulmonary exercise testing (CPET and the 6-minute walk test (6MWT are shown to provide information on the severity and survival of disease. Methods We prospectively recruited IPF patients and examined the relationship between the MRC score and either CPET or 6MWT parameters known to reflect physiologic derangements limiting exercise capacity in IPF patients Results Twenty-five patients with IPF were included in the study. Significant correlations were found between the MRC score and the distance (r = -.781, p 2 at the initiation and the end (r = -.542, p = 0.005 and r = -.713, p VO2 peak/kg (r = -.731, p 2 at peak exercise (r = -. 682, p 2 slope (r = .731, p 2 at AT (r = .630, p = 0.002 and the Borg scale at peak exercise (r = .50, p = 0.01 for the CPET. In multiple logistic regression analysis, the only variable independently related to the MRC is the distance walked at the 6MWT. Conclusion In this population of IPF patients a good correlation was found between the MRC chronic dyspnoea score and physiological parameters obtained during maximal and submaximal exercise testing known to reflect ventilatory impairment and exercise limitation as well as disease severity and survival. This finding is described for the first time in the literature in this group of patients as far as we know and could explain why a simple chronic dyspnea score provides reliable prognostic information on IPF.

  15. Serological evidence that activation of ubiquitous human herpesvirus-6 (HHV-6) plays a role in chronic idiopathic/spontaneous urticaria (CIU).

    Science.gov (United States)

    Dreyfus, D H

    2016-02-01

    Acute infection with viral pathogens in the herpesviridae family can trigger acute urticaria, and reactivation of herpesviridae is associated with cutaneous urticarial-like syndromes such as drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DRESS). Reactivation of latent herpesviridae has not been studied systematically in chronic idiopathic/spontaneous urticaria (CIU). This review proposes that CIU is an inflammatory disorder with autoimmune features (termed 'CVU' for chronic viral urticaria), based on serology consistent with the hypothesis that reactivation of a latent herpesvirus or -viruses may play a role in CIU. Serology obtained from a cohort of omalizumab (Xolair)-dependent patients with severe CIU was consistent with previous HHV-6 infection, persistent viral gene expression and replication. CIU patients also exhibited serological evidence of increased immune response to HHV-4 (Epstein-Barr virus, or EBV) but not all CIU patients were infected with EBV. These observations, combined with case reports of CIU response to anti-viral therapy, suggest that HHV-6, possibly interacting with HHV-4 in cutaneous tissues, is a candidate for further prospective study as a co-factor in CIU. PMID:26361716

  16. Serological evidence that activation of ubiquitous human herpesvirus-6 (HHV-6) plays a role in chronic idiopathic/spontaneous urticaria (CIU).

    Science.gov (United States)

    Dreyfus, D H

    2016-02-01

    Acute infection with viral pathogens in the herpesviridae family can trigger acute urticaria, and reactivation of herpesviridae is associated with cutaneous urticarial-like syndromes such as drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DRESS). Reactivation of latent herpesviridae has not been studied systematically in chronic idiopathic/spontaneous urticaria (CIU). This review proposes that CIU is an inflammatory disorder with autoimmune features (termed 'CVU' for chronic viral urticaria), based on serology consistent with the hypothesis that reactivation of a latent herpesvirus or -viruses may play a role in CIU. Serology obtained from a cohort of omalizumab (Xolair)-dependent patients with severe CIU was consistent with previous HHV-6 infection, persistent viral gene expression and replication. CIU patients also exhibited serological evidence of increased immune response to HHV-4 (Epstein-Barr virus, or EBV) but not all CIU patients were infected with EBV. These observations, combined with case reports of CIU response to anti-viral therapy, suggest that HHV-6, possibly interacting with HHV-4 in cutaneous tissues, is a candidate for further prospective study as a co-factor in CIU.

  17. Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Niaz Faraz

    2010-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued re-mission after eight months of follow-up. Rituximab appears to be an effective treatment in re-fractory cases of TTP associated with SLE.

  18. The bone marrow megakaryocyte change of acute and chronic idiopathic thrombocytopenic purpura%急慢性特发性血小板减少性紫癜骨髓巨核细胞改变

    Institute of Scientific and Technical Information of China (English)

    周建中

    2007-01-01

    目的 比较急慢性特发性血小板减少性紫癜(ITP)骨髓巨核细胞改变特点.方法 包括30例急性ITP及38例慢性ITP初诊患者,其骨髓片经瑞氏染色,显微镜下计数巨核细胞.结果 急慢性ITP的原巨核细胞、幼巨核细胞、颗粒型巨核细胞及裸核巨核细胞数量增多,尤其是急性ITP.急慢性ITP的产生,血小板型巨核细胞均减少,急性ITP更为显著.慢性ITP易见血小板型幼巨核细胞.结论 急慢性ITP巨核细胞增多,且显著成熟障碍,尤其是急性ITP.

  19. Physical activity recommendations for children with specific chronic health conditions: Juvenile idiopathic arthritis, hemophilia, asthma and cystic fibrosis

    OpenAIRE

    Philpott, J; Houghton, K.; Luke, A

    2010-01-01

    As a group, children with a chronic disease or disability are less active than their healthy peers. There are many reasons for suboptimal physical activity, including biological, psychological and social factors. Furthermore, the lack of specific guidelines for ‘safe’ physical activity participation poses a barrier to increasing activity. Physical activity provides significant general health benefits and may improve disease outcomes. Each child with a chronic illness should be evaluated by an...

  20. Correlation of serum antithyroid microsomal antibody and autologous serum skin test in patients with chronic idiopathic urticaria

    OpenAIRE

    Snehal Balvant Lunge; Milind Borkar; Sushil Pande

    2015-01-01

    Background: About 25–45% of patients of chronic urticaria (CU) have been stated to have histamine releasing autoantibodies in their blood. The term autoimmune urticaria is increasingly being accepted for this subgroup of patients. Review of the literature suggests high autologous serum skin test (ASST) positivity and presence of antithyroid microsomal antibodies in patients with autoimmune urticaria. Aims: To study prevalence of ASST positivity and antithyroid microsomal antibodies in chronic...

  1. [New marker in thrombotic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Hillarp, A.; Lindblom, A.; Bjork, P.;

    2008-01-01

    Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...... concentrations of high molecular weight von Willebrand factor forms and increased platelet aggregation. Measurement of ADAMTS-13 activity is useful for the diagnosis of TTP and may also be relevant as a prognostic test for recurrent TTP Udgivelsesdato: 2008/8/11...

  2. Behavioral Problems in Juvenile Idiopathic Arthritis: A Controlled Study to Examine the Risk of Psychopathology in a Chronic Pediatric Disorder

    Science.gov (United States)

    Chamanara, Elham; Raeeskarami, Seyed-Reza

    2016-01-01

    Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached “borderline clinical” range or “clinical” range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies. PMID:27656678

  3. Behavioral Problems in Juvenile Idiopathic Arthritis: A Controlled Study to Examine the Risk of Psychopathology in a Chronic Pediatric Disorder

    Science.gov (United States)

    Chamanara, Elham; Raeeskarami, Seyed-Reza

    2016-01-01

    Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached “borderline clinical” range or “clinical” range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies.

  4. Evaluation of the efficacy and safety of fexofenadine in the management of chronic idiopathic urticaria: A prospective study with 512 patients

    Directory of Open Access Journals (Sweden)

    Dhar Sandipan

    2002-01-01

    Full Text Available Five hundred and twelve patients with chronic idiopathic urticaria (CIU were treated with fexofenadine at a dose on 180mg/day. Maximum number of patients were between 20 to 40 years of age and female to male ratio was 1.45:1. The severity of itching was calculated on a scale of 0 to 4 and was recorded by the patients. The mean daily total symptom score (TSS was measured as sum of the patients′ pruritus and number of wheal scores (0 to 7. A mean TSS was determined for each week. Baseline TSS came down to ′0′ by 4 weeks in all groups except those with TSS 4. There was no correlation between the baseline TSS and degree of improvement. Of 512, 14 (2.73% patients did not complete the study. The commonest adverse effect was headache (9.04%. There was no report of drowsiness or cardiac arrythmia. In no patient fexofenadine had to be withdrawn because of its adverse effects.

  5. Translocation t(3;12(q26;q21 In Jak2V617F Point Mutation Negative Chronic Idiopathic Myelofibrosis: A Case Report

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    S. Mešanović

    2014-06-01

    Full Text Available The myeloproliferative diseases (MPDs or myelo-proliferative neoplasms (MPNs are a group of diseases of the bone marrow in which excess cells are produced. Chronic idiopathic myelofibrosis (CIMF is a stem cell defect characterized by splenomegaly with multiorgan extramedullary hematopoiesis, immature peripheral blood granulocytes and erythrocytes and progressive bone marrow fibrosis. The most common chromosomal abnormalities seen in CIMF patients include numerical changes of chromosomes 7, 8 and 9, and structural changes of 1q, 5q, 13q and 20q. At least 75.0% of patients with bone marrow abnormalities have one or more of these chromosomal anomalies. Detection of the Janus kinase 2 (JAK2 mutation may be a potential major breakthrough for understanding the pathobiology of MPNs, and is an essential part of the diagnostic algorithm. In this study, we describe a JAK2V617F mutation negative CIMF patient who has the chromosomal translocation t(3;12(q26;q21 in her karyotype.

  6. A review of immune thrombocytopenic purpura: focus on the novel thrombopoietin agonists

    Directory of Open Access Journals (Sweden)

    Meaghan Khan

    2010-03-01

    Full Text Available Meaghan Khan, Joseph MikhaelDivision of Hematology – Oncology, Scottsdale, AZ, USAAbstract: Immune thrombocytopenic purpura (ITP is an autoimmune disorder that is characterized by antibody-mediated platelet destruction and decreased platelet production. ITP and its treatments have been recognized to cause diminished quality of life in those afflicted with this illness on levels comparable to other chronic diseases. The disease can be self-limiting, but in adults it often is a chronic process requiring medical intervention to maintain appropriate platelet counts and to reduce bleeding events. Many patients go on to develop disease that is refractory to current interventions. Historically, the aim of treatment has been focused on reducing the amount of antibody-mediated destruction but newer therapies have centered on the decreased platelet production. Two new medications that target production of platelets have recently been USA, Food and Drug Administration (FDA approved for the treatment of chronic relapsing ITP. Here, we provide an overview of ITP and a comprehensive review of the newest therapies aimed at the stimulation of platelet production.Keywords: immune thrombocytopenic purpura, therapy, thrombopoietin, romiplostim, AMG 531, eltrombopag

  7. A case of immune thrombocytopenic purpura presenting with intracranial hemorrhage

    Institute of Scientific and Technical Information of China (English)

    Sinan Akbayram; Fesih Aktar; Cihangir Akgn; Mehmet Seluk Bekta; Hseyinaksen; Ahmet Faik Oner

    2013-01-01

    Immune thrombocytopenic purpura is an acute, generally considered a self-limiting benign disorder with a60%-80% change of spontaneous recovery occurring usually within a few months after onset.Intracranial hemorrhage is a rare but life-threatening complication of childhood immune thrombocytopenic purpura.We report a4-year-old girl who admitted with headache, vomiting, bleeding from noise and bruises on the extremities.Her neurological examination was normal.Based on laboratory finding she was diagnosed immune thrombocytopenic purpura and intracranial hemorrhage.We suggest that cranial imaging should be perform in patients with immune thrombocytopenic purpura admitted with bleeding symptoms, vomiting and headache even if they had no abnormal neurological signs.

  8. Physical activity recommendations for children with specific chronic health conditions: Juvenile idiopathic arthritis, hemophilia, asthma and cystic fibrosis.

    Science.gov (United States)

    Philpott, J; Houghton, K; Luke, A

    2010-04-01

    As a group, children with a chronic disease or disability are less active than their healthy peers. There are many reasons for suboptimal physical activity, including biological, psychological and social factors. Furthermore, the lack of specific guidelines for 'safe' physical activity participation poses a barrier to increasing activity. Physical activity provides significant general health benefits and may improve disease outcomes. Each child with a chronic illness should be evaluated by an experienced physician for activity counselling and for identifing any contraindications to participation. The present statement reviews the benefits and risks of participation in sport and exercise for children with juvenile arthritis, hemophilia, asthma and cystic fibrosis. Guidelines for participation are included. PMID:21455465

  9. Idiopathic scoliosis

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    Jandrić Slavica

    2012-01-01

    Full Text Available Introduction. Idiopathic scoliosis is a structural and lateral curvature of the spine for which a currently recognizable cause has not been found and there is no basic evidence for physical and radiographic pathology. Complications. Scoliosis could be a cause of the back pain, deformities, respiratory and cardiology problems. There is a higher risk for decreasing of bone mineral density. Diagnosis and Management. Physical examination, radiography and stereophotogrametry are used in diagnostics of idiopathic scoliosis. The management of idiopathic scoliosis can be conservative or operative. The main forms of conservative management are: therapy exercises, electrical stimulation and spinal orthosis. The primary aim of scoliosis management is to stop curvature progression. The improvement of pulmonary function (vital capacity and treatment of pain are also of major importance. The guidelines for Conservative Management of Scoliosis. The International Society on Scoliosis Orthopaedic and Rehabilitation Treatment gave the guidelines for conservative management of scoliosis. Conservative management of scoliosis includes: a Lyonaise, Side-Shift, Dobosiewicz, Schroth and other kinesitherapy methods, b scoliosis intensive rehabilitation which appears to be effective with respect to many signs and symptoms of scoliosis and with respect to impeding curvature progression and c brace treatment, which has been found to be effective in preventing curvature progression and thus in altering the natural history of idiopathic scoliosis. Conclusion. The International Society has given the standards for kinesitherapy, intensive rehabilitation and spinal orthosis in clinical practice and clinical investigations. Surgical procedures can improve curves in the frontal plane, but have important limitations in maintaining fixation and achieving correction in other planes.

  10. Idiopathic scoliosis.

    OpenAIRE

    Jens Ivar Brox

    1989-01-01

    Introduction. Idiopathic scoliosis is a structural and lateral curvature of the spine for which a currently recognizable cause has not been found and there is no basic evidence for physical and radiographic pathology. Complications. Scoliosis could be a cause of the back pain, deformities, respiratory and cardiology problems. There is a higher risk for decreasing of bone mineral density. Diagnosis and Management. Physical examination, radiography and stereophotogrametry are used in diag...

  11. Hip joint structures assessment in juvenile idiopathic (chronic arthritis with ultrasound TOC \\o "1-5" \\h \\z examination

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    G. R. Movsisyan

    2004-01-01

    Full Text Available Sonographic (SG examination of hip joints was performed in 100 pts with juvenile chronic arthritis (JCA with different forms, activity and duration. SG signs of hip joint inflammation in JCA were identified. SG features in pts with different disease duration, form and activity were revealed. SG changes of clinically unchanged hip joints were noted which may be of important significance for early diagnosis of coxitis. More wide use of arthrosonography in pediatric rheumatology was proved.

  12. Recombinant thrombomodulin for secondary thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Nakamura, Kensuke; Inokuchi, Ryota; Hiruma, Takahiro; Ohshima, Kazuma; Sonoo, Tomohiro; Tokunaga, Kurato; Doi, Kent; Nakajima, Susumu

    2016-06-01

    In the pathogenesis of thrombotic thrombocytopenic purpura (TTP), reductions in the enzyme activity of ADAMTS13, which cuts ultralarge von Willebrand multimers, generates shear stress on the microvascular endothelium, leading to platelet aggregation and the formation of a thrombus. ADAMTS13 activity is markedly decreased in typical TTP, but is only mildly reduced in secondary TTP, which concomitantly develops with primary disease. The latter develops with septic disseminated intravascular coagulation (DIC) and often causes organ failure. Recombinant thrombomodulin (rTM) is a drug that is used to treat DIC and may also remit TTP because it improves vascular endothelial dysfunction. Therefore, we herein investigated the efficacy of rTM in patients treated for the pathology of secondary TTP. Patients who were admitted to the Emergency and Critical Care Center of our hospital and met the following conditions were extracted and retrospectively analyzed: hemolytic anemia accompanied by fragmented red blood cells (Hb TTP, significantly increased in the rTM treatment group: 3.3 ± 2.6→11.3 ± 14.6 versus 3.5 ± 3.7→5.7 ± 3.9 (×1000/μL) (P = 0.034). Thrombotic thrombocytopenic purpura originally requires invasive treatments and its prognosis is not favorable. Blood thrombomodulin levels also markedly increase due to vascular endothelial dysfunction, whereas rTM alleviates vascular endothelial dysfunction in TTP patients with high blood TM levels, suggesting the importance of administering rTM. Thus, rTM may be effective for secondary TTP and may be adopted as adjuvant therapy. PMID:27310951

  13. Idiopathic Scoliosis

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    Jens Ivar Brox

    2014-07-01

    Full Text Available Idiopathic scoliosis (IS is a lifetime condition and is defined as a structural, lateral rotated curvature of the spine of >10° on standing coronal plane radiographs. It should be distinguished from other causes of scoliosis. It can be classified as infantile, juvenile, and adolescent according to age. As a rule of thumb, about 80% of all curves are idiopathic, right convex thoracic, and present in otherwise healthy girls at the beginning of puberty. A family member most commonly detects scoliosis. The structural asymmetry of the spine is best observed by asking the patient to bend forward. IS is often seen in more than one member of a family, but the aetiology remains unknown. Multiple genes are likely to be involved with incomplete penetrance and variable expressivity. Early detection by screening allows for monitoring curve progression and timely initiation of bracing, but school screening is controversial and practises vary worldwide. Most patients have minor scoliosis and treatment is generally not recommended for patients with curves 45°. Scoliosis surgery was not successful until the introduction of Harrington’s instrumentation in the 1960s. Modern instrumentation has evolved from the Cotrel-Dubousset system in the 1980s, and a variety of methods are available today. Although scoliosis may be a burden, long-term studies suggest that a good quality of life is maintained in most patients.

  14. Idiopathic short stature

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    Vlaški Jovan

    2013-01-01

    Full Text Available Growth is a complex process and the basic characteristic of child- hood growth monitoring provides insight into the physiological and pathological events in the body. Statistically, the short stature means departure from the values of height for age and sex (in a particular environment, which is below -2 standard deviation score, or less than -2 standard deviation, i.e. below the third percentile. Advances in molecular genetics have contributed to the improvement of diagnostics in endocrinology. Analysis of patients’ genotypes should not be performed before taking a classical history, detailed clinical examination and appropriate tests. In patients with idiopathic short stature specific causes are excluded, such as growth hormone deficiency, Turner syndrome, short stature due to low birth weight, intrauterine growth retardation, small for gestational age, dysmorphology syndromes and chronic childhood diseases. The exclusion of abovementioned conditions leaves a large number of children with short stature whose etiology includes patients with genetic short stature or familial short stature and those who are low in relation to genetic potential, and who could also have some unrecognized endocrine defect. Idiopathic short stature represents a short stature of unknown cause of heterogeneous etiology, and is characterized by a normal response of growth hormone during stimulation tests (>10 ng/ml or 20 mJ/l, without other disorders, of normal body mass and length at birth. In idiopathic short stature standard deviation score rates <-2.25 (-2 to -3 or <1.2 percentile. These are also criteria for the initiation of growth hormone therapy. In children with short stature there is also the presence of psychological and social suffering. Goals of treatment with growth hormone involve achieving normal height and normal growth rate during childhood.

  15. Inhibition and Role of let-7d in Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Kusum V Pandit; Corcoran, David; Yousef, Hanadie; Yarlagadda, Manohar; Tzouvelekis, Argyris; Gibson, Kevin F.; Konishi, Kazuhisa; Yousem, Samuel A.; Singh, Mandal; Handley, Daniel; Richards, Thomas; Selman, Moises; Watkins, Simon C.; Pardo, Annie; Ben-Yehudah, Ahmi

    2010-01-01

    Rationale: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and usually lethal fibrotic lung disease characterized by profound changes in epithelial cell phenotype and fibroblast proliferation.

  16. Melatonin improves health status and sleep in children with idiopathic chronic sleep-onset insomnia: A randomized placebo-controlled trial.

    NARCIS (Netherlands)

    M.G. Smits; H.F. van Stel; K. van der Heijden; A.M. Meijer; A,.M.L. Coenen; G.A. Kerkhof

    2003-01-01

    Objective: To investigate the effect of melatonin treatment on health status and sleep in children with idiopathic sleep-onset insomnia. Method: A randomized, double-blind, placebo-controlled trial was conducted in a Dutch sleep center, involving 62 children, 6 to 12 years of age, who suffered more

  17. Idiopathic sclerosing orbital inflammation

    NARCIS (Netherlands)

    J.D. Hsuan; D. Selva; A.A. McNab; T.J. Sullivan; P. Saeed; B.A. O'Donnell

    2006-01-01

    Objective: To perform a multicenter review of the clinical features and treatment of 31 patients with idiopathic sclerosing orbital inflammation. Methods: We included all patients with histologically confirmed idiopathic sclerosing orbital inflammation from 5 regional orbital centers. We reviewed th

  18. Thrombotic thrombocytopenic purpura (TTP or Moschowitz syndrome: a true hematologic emergency

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    Deborah Melis

    2012-01-01

    Full Text Available Introduction: Thrombotic thrombocytopenic purpura (TTP is a thrombotic microangiopathy caused by congenital or inherited disorders involving the processing of the ultra-large forms of von Willebrand factor. As a result, platelet-rich microthrombi form in the small arterial vessels of various organs, particularly those of the brain, heart, and kidneys. The idiopathic autoimmune form of TTP is the most common. There are various subgroups of acquired TTP associated with HIV infection, sepsis, pregnancy, autoimmune disease, various disseminated malignancies, and drugs. If not promptly treated, TTP is associated with high mortality, making it a true medical emergency. Materials and methods: The article is based on a review of the literature published between January and October of 2009. Its aim is to clarify the diagnosis, treatment, and follow-up of TTP. Results: Diagnostic criteria include the presence of microangiopathic hemolytic anemia associated with thrombocytopenia in the absence of other obvious causes. Assays of ADAMTS13 activity and titration of acquired antibodies against this enzyme are indicated in the follow-up of disease and as prognostic indicators. Treatment centers around daily plasma exchange associated with immunosuppressant drug therapy, particularly steroids and more recently the monoclonal anti-CD20 antibody rituximab. Discussion: Despite improved treatment, TTP is still associated with significant mortality (10—20%, particularly when plasma exchange is initiated late. Relapse also occurs in a substantial proportion of patients (10—40% although the frequency of this outcome may be reduced by rituximab therapy.

  19. Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    João Tadeu Damian Souto Filho

    2011-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD is extremely rare and has only been described in nine patients. We describe the case of a 42-year-old female with MCTD who developed thrombocytopenia, microangiopathic hemolytic anemia, fever, and neurological symptoms. The patient had a good clinical evolution with infusion of high volume of fresh frozen plasma, steroid therapy, and support in an intensive care unit. Although the occurrence of TTP is rare in MCTD patients, it is important to recognize TTP as a cause of thrombocytopenia and hemolytic anemia in any patient with autoimmune diseases. Prompt institution of treatment remains the cornerstone of treatment of TTP even if plasma exchange is not available like what frequently happens in developing countries.

  20. Pathophysiology of thrombotic thrombocytopenic purpura : the "two-hit" paradigm

    NARCIS (Netherlands)

    Lotta, Luca Andrea

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread thrombosis in capillaries and small arteries. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patie

  1. Splenectomy for the treatment of thrombotic thrombocytopenic purpura

    NARCIS (Netherlands)

    Kappers-Klunne, MC; Wijermans, P; Fijnheer, R; Croockewit, AJ; van der Holt, B; de Wolf, JTM; Lowenberg, B; Brand, A

    2005-01-01

    Plasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these

  2. Active von Willebrand factor in thrombotic thrombocytopenic purpura and malaria

    NARCIS (Netherlands)

    Groot, E.

    2009-01-01

    Thrombotic thrombocytopenic purpura (TTP) and malaria are two diseases of distinct origin. TTP is a rare disorder caused by a deficiency of the von Willebrand factor (VWF) cleaving protease ADAMTS13. Malaria is a poverty-related disease caused by protozoan parasites from the genus Plasmodium. TTP an

  3. Opana® ER induced thrombotic thrombocytopenic purpura

    OpenAIRE

    Kotbi, Nabil; Han,Bernadine; Cheng,Duncan; Odom, Anna E

    2015-01-01

    We present the case of a patient who developed thrombotic thrombocytopenic purpura (TTP) following intravenous injection of Opana® ER. TTP reemerged after three months of abstinence with Opana misuse. This case report brings awareness to the possibility of developing TTP in those who misuse Opana, which is a growing concern.

  4. Evaluation of Three Kinds of Antihistamines Curative Effect for the Treatment of Chronic Idiopathic Urticarial%评价3种抗组胺药治疗慢性特发性荨麻疹的疗效

    Institute of Scientific and Technical Information of China (English)

    陈海兵

    2015-01-01

    目的:评价3种抗组胺药治疗慢性特发性荨麻疹的疗效。方法选取于我院就诊的慢性特发性荨麻疹患者90例作为研究对象,随机分为三组,每组各30人,分别给予西替利嗪药物治疗、氯雷他定药物治疗、依巴斯丁药物治疗。结果第7天、14天、28天三组有效率为:氯雷他定组分别为70.00%、83.33%、93.33%;西替利嗪组分别为66.67%、86.67%、90.00%;依巴斯丁组分别为76.67%、86.67%、96.67%。结论三种抗组胺药物在临床治疗慢性特发荨麻疹上都具有较好的效果,具有临床应用价值。%Objective To evaluate three kinds of antihistamines curative effect for the treatment of chronic idiopathic urticaria. Methods 90 patients with chronic idiopathic urticaria were selected from our hospital as research object, randomly dividing into three groups (30 people a group). Cetirizine group was given cetirizine medication, loratadine group was given loratadine drug therapy, by buss butyl group was given by buss butyl drug therapy. Results 7 days, 14 days, the effective rate of 28 days three groups:loratadine group was 70.00%, 83.33%and 70.00%respectively. Cetirizine group was 66.67%, 86.67%and 66.67%respectively. By bass butyl group 76.67%, 86.67, 96.67, respectively. Conclusion The three kinds of antihistamines in clinical treatment of chronic idiopathic urticaria had good effect, had clinical application value.

  5. 慢性荨麻疹与幽门螺旋杆菌感染相关性的探讨%The investigation of the relationship of Chronic idiopathic urticaria and Helicobacter pylori infection

    Institute of Scientific and Technical Information of China (English)

    麻荣武; 胡鲜香

    2009-01-01

    Objective To investigate the relationship of chronic idiopathic urticaria(CIU) and Helicobacter pylori(Hp). Methods The Helicobacter pylori stool antigens(HpSA) from 59 Chronic idiopathic urticaria patients and 65 cases of the healthy control group were detected with monoclonal antibody. And besides antihistamine, the patients with positive HpSA were treated with the eradication of Hp. And the patients with negative HpSA were only treated with antihistamine. Results There were 46 cases among 59 Hp patients showed positive HPSA, the ratio was 77.96%. And the cured was 41 cases (As cure rate was 89.13%). After the treatment, all 13 cases of the CIU patients with negative HpSA recrudesced in different degree. Conclusions CIU partly occurs related to Hp infection. On CIU patients with unknown pathogeny, Hp infection should be examined. If the examination indicates Hp infection, the patients should be treated with antihistamine, and be carried out the eradication of the Hp.%目的 探讨慢性荨麻疹(chronic idiopathic urticaria,CIU)与幽门螺旋杆菌(Helicobacter pylori,HP)的关系.方法 采用单克隆抗体对59例慢性荨麻疹患者及65例健康对照组的粪便Hp抗原进行检测,对粪便Hp抗原检测阳性患者除给予抗组胺治疗外,同时给予根除HP治疗.对粪便HP抗原检测阴性患者单给予抗组胺治疗.结果 59例慢性荨麻疹患者有46例粪便Hp抗原阳性,阳性率77.96%.治愈41例(治愈率89.13%).13例HP粪便抗原阴性慢性荨麻疹患者治疗后全部不同程度复发.结论 部分慢性荨麻疹发病与HP感染有关,对于病因不明的慢性荨麻疹患者,要给予检测是否有HP感染,检测提示HP感染,在给予抗组织胺药的同时,应同时进行根除HP治疗.

  6. Idiopathic portal hypertension

    International Nuclear Information System (INIS)

    To describe the radiologic findings of idiopathic portal hypertension and to find the points of differentiation between idiopathic portal hypertension and liver cirrhosis. Four portograms in five patients who for four years had suffered from pathologically confirmed idiopathic portal hypertension were retrospectively analyzed and compared with a portogram obtained from a control subject with liver cirrhosis. Portographic finding s of idiopathic portal hypertension were paucity of medium-sized portal branches, irregular and obtuse-angled division of peripheral branches, abrupt interruption and an avascular area beneath the liver margin. A portogram of idiopathic portal hypertension may be useful in differentiation this and liver cirrhosis

  7. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Nelson, Andrew D; Fischer, Philip R; Reed, Ann M; Wylam, Mark E

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  8. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    OpenAIRE

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis.

  9. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Andrew D. Nelson

    2015-01-01

    Full Text Available We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis.

  10. [Randomized comparison of intravenous immunoglobulin and methylprednisolone pulse therapy in children with newly diagnosed idiopathic thrombocytic purpura. The Danish ITP Study Group].

    Science.gov (United States)

    Rosthøj, S; Nielsen, S M; Pedersen, F K

    1998-03-01

    Forty three children with newly diagnosed idiopathic thrombocytopenic purpura (ITP), platelet count (pl.c.) below 20 x 10(9)/l, and either clinically significant bleeding or failure to show a spontaneous platelet rise within three days of admission were randomly allocated to treatment with intravenous infusions of either immunoglobulin (IVIG) 1 g/kg or methylprednisolone (MPPT) 30 mg/kg on two consecutive days. Prompt induction of partial remission with pl.c. > 50 x 10(9)/l after 72 hours was seen in 21/23 given IVIG versus 10/20 given MPPT (exact p = 0.003); mean pl.c.s after 72 hours were 188 versus 77 x 10(9)/l (2p 150 x 10(9)/l was achieved in 16/23 versus 10/20 (p = 0.16). During six months follow-up, there were no significant differences regarding relapse rates or chronic course. Eleven children with relapse were crossed over to the alternative treatment arm: the estimated treatment effect in pl.c. after 72 hours was 134 x 10(9)/l in favour of IVIG. These results indicate that IVIG infusions may be preferable to high-dose corticosteroids as initial treatment for children with ITP. PMID:9522658

  11. Cough in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    van Manen, Mirjam J G; Birring, Surinder S; Vancheri, Carlo; Cottin, Vincent; Renzoni, Elisabetta A; Russell, Anne-Marie; Wijsenbeek, Marlies S

    2016-09-01

    Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably "multifactorial" and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF. PMID:27581827

  12. Cough in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Mirjam J.G. van Manen

    2016-09-01

    Full Text Available Many patients with idiopathic pulmonary fibrosis (IPF complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably “multifactorial” and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF.

  13. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    Science.gov (United States)

    ... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

  14. Glucocorticoids in juvenile idiopathic arthritis.

    Science.gov (United States)

    Malattia, Clara; Martini, Alberto

    2014-05-01

    Although the use of corticosteroids in juvenile idiopathic arthritis (JIA) is now much more limited owing to the availability of methotrexate and biological agents, there are clinical scenarios where it is still indicated. For example, corticosteroids may be indicated for intraarticular injections to prevent joint deformities, as a "bridge" drug to relieve symptoms in polyarticular disease while waiting for methotrexate and biologics to exert their full therapeutic effects, and in the treatment of chronic iridocyclitis, macrophage activation syndrome, and systemic JIA, although the advent of interleukin (IL)-1 and IL-6 blockers has greatly reduced the latter indication.

  15. Thrombocytopenic purpura as adverse reaction to recombinant hepatitis B vaccine

    OpenAIRE

    Ronchi, F; Cecchi, P; Falcioni, F.; Marsciani, A; Minak, G.; Muratori, G; Tazzari, P; Beverini, S

    1998-01-01

    Three cases of immune thrombocytopenic purpura after the first dose of recombinant hepatitis B vaccine occurred in infants under 6 months of age. Other possible causes of this condition were excluded. Antiplatelet antibodies were present. A defect in platelet production was excluded in two children. Corticosteroid treatment was effective. Subsequent administration of other vaccines (against polio, diphtheria, and tetanus) did not cause relapse of thrombocytopenia.



  16. Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

    OpenAIRE

    Roriz, Mélanie; Landais, Mickael; Desprez, Jonathan; Barbet, Christelle; Azoulay, Elie; Galicier, Lionel; Wynckel, Alain; Baudel, Jean-luc; Provôt, François; Pène, Frédéric; Mira, Jean-Paul; Presne, Claire; Poullin, Pascale; Delmas, Yahsou; Kanouni, Tarik

    2015-01-01

    Abstract Autoimmune thrombotic thrombocytopenic purpura (TTP) can be associated with other autoimmune disorders, but their prevalence following autoimmune TTP remains unknown. To assess the prevalence of autoimmune disorders associated with TTP and to determine risk factors for and the time course of the development of an autoimmune disorder after a TTP episode, we performed a cross sectional study. Two-hundred sixty-one cases of autoimmune TTP were included in the French Reference Center reg...

  17. Thrombotic thrombocytopenic purpura: The role of ADAMTS13.

    Science.gov (United States)

    Rogers, Heesun J; Allen, Charles; Lichtin, Alan E

    2016-08-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease requiring prompt diagnosis and initiation of therapeutic plasma exchange to improve patient survival. However, diagnosis is often difficult because of atypical presentations and signs and symptoms that resemble other conditions. Measurements of ADAMTS13 activity, ADAMTS13 inhibitor, and ADAMTS13 autoantibody are useful for diagnosing TTP, guiding therapy, and predicting relapse. PMID:27505881

  18. Pathophysiology of thrombotic thrombocytopenic purpura: the "two-hit" paradigm

    OpenAIRE

    Lotta, Luca Andrea

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread thrombosis in capillaries and small arteries. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patients with TTP represented a turning point in the understanding of the pathophysiology of the disease. In spite of recent advances, the clinical course of TTP is characterized by considerable heterog...

  19. Thrombotic thrombocytopenic purpura in the first trimester of pregnancy

    Directory of Open Access Journals (Sweden)

    Pooja Sikka

    2013-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP occurs more commonly in women and so can be associated with pregnancy. The time during pregnancy with greatest risk for development of TTP is near term and during the post partum period. TTP occurring in early trimester is uncommon and is also associated with great maternal and fetal mortality. We report a successful outcome of pregnancy in a woman with TTP in early first trimester who was treated with therapeutic plasma exchange.

  20. Evaluation of a prototype electronic personal health record for patients with idiopathic thrombocytopenic purpura

    OpenAIRE

    Chiche, Laurent

    2012-01-01

    Laurent Chiche,1 Alessandra Brescianini,1 Julien Mancini,2 Hervé Servy,3 Jean-Marc Durand11Service de Médecine Interne, Centre de Compétence pour la prise en charge des Cytopénies Auto-immunes, Hôpital de la Conception, Marseille, 2Service de Santé Publique, Hôpital de la Timone, Marseille, 3Association AIMSU, Maison des Associations, La Ciotat, FranceBackground: Patients with rare diseases often lack information about...

  1. How we use WinRho in patients with idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Stotler, Brie A; Schwartz, Joseph

    2015-11-01

    Primary immune thrombocytopenia (ITP) is an autoimmune disease that affects children and adults. WinRho SDF is a D immune globulin product that is Food and Drug Administration approved for the treatment of ITP in D+ pediatric and adult patients. WinRho is a plasma-derived biologic product dispensed from blood banks. Transfusion medicine physicians serve as a resource to health care providers regarding blood component and derivative usage and, as such, should be familiar with the use of WinRho for ITP, including the dosage, administration, and contraindications. This report details the transfusion medicine consultation practice and guidelines at a tertiary care academic medical center for the usage of WinRho SDF in patients with ITP. PMID:26094894

  2. Rituximab chimeric anti-CD20 monoclonal antibody treatment for adult refractory idiopathic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Braendstrup, Peter; Bjerrum, Ole W; Nielsen, Ove J;

    2005-01-01

    . Recent studies have shown that rituximab, a chimeric anti-CD20 monoclonal antibody, is useful in the treatment of these patients, with overall response rates of about 50%. Most published reports have included a small number patients including case reports. The present study reports the results...... of a retrospective Danish multicenter study of rituximab in the treatment of adult patients with refractory ITP. Thirty-five patients (median age 52 years, range 17-82 years, 17 males) were included. One patient had immune thrombocytopenia and neutropenia. All patients had received prednisolone (Pred). Next to Pred...... of rituximab. The large majority of patients also received Pred and, in some cases, other concomitant immunosuppressive treatment during part of their rituximab treatment. A complete response (CR) was defined as a rise in the platelet count > 100 x 10(9)/L, a partial response (PR) as a rise in the platelet...

  3. Immune thrombocytopenic purpura-related hemotympanum presenting with hearing loss.

    Science.gov (United States)

    Fisgin, Tunc; Atmaca, Sinan; Duru, Feride; Ozyurek, Emel; Cetin, Recep; Albayrak, Davut

    2009-06-01

    A 5-year-old boy was admitted to our center with a major complaint of bilateral hearing loss for 2 days. He was diagnosed with acute immune thrombocytopenic purpura 3 months before the admission and treated with high-dose methylprednisolone 2 months ago. Physical examination revealed wet purpura in the oral mucosa, serous nasal discharge, multiple petechiae and ecchymosis of the lower lip. Otomicroscopic ear examination revealed the presence of bilateral hemotympanum. The patient denied head trauma, ear pain, fever, hypertension and medications, including salicylates. The patient received high-dose intravenous methylprednisolone because of low platelet count and wet purpura for 7 days and oral prophylactic amoxicillin-clavulanate for 14 days. The onset of the response to corticosteroids was rapid, and significant hematologic improvement was observed within a few days. The 2-week follow-up examination revealed intact tympanic membranes with normal color and mobility, and the patient restored normal hearing. In this patient, hemotympanum developed rapidly, and no predisposing cause other than immune thrombocytopenic purpura was found. However, presence of a serous nasal discharge may be a sign of viral upper respiratory tract infection. Therefore, it can be speculated that sneezing or coughing might have caused bilateral hemotympanum by increasing the middle ear pressure abruptly. We would like to emphasize that bleeding may occur in unusual sites and, unlike in healthy people, may cause bizarre symptoms in patients with bleeding diathesis. Hemotympanum can be considered among the indications to start treatment in patients with acute immune thrombocytopenic purpura. PMID:19530341

  4. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  5. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Meltzer, Eric B; Noble, Paul W

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median

  6. A conservative assessment of the major genetic causes of idiopathic chronic pancreatitis: data from a comprehensive analysis of PRSS1, SPINK1, CTRC and CFTR genes in 253 young French patients.

    Directory of Open Access Journals (Sweden)

    Emmanuelle Masson

    Full Text Available Idiopathic chronic pancreatitis (ICP has traditionally been defined as chronic pancreatitis in the absence of any obvious precipitating factors (e.g. alcohol abuse and family history of the disease. Studies over the past 15 years have revealed that ICP has a highly complex genetic architecture involving multiple gene loci. Here, we have attempted to provide a conservative assessment of the major genetic causes of ICP in a sample of 253 young French ICP patients. For the first time, conventional types of mutation (comprising coding sequence variants and variants at intron/exon boundaries and gross genomic rearrangements were screened for in all four major pancreatitis genes, PRSS1, SPINK1, CTRC and CFTR. For the purposes of the study, synonymous, intronic and 5'- or 3'-untranslated region variants were excluded from the analysis except where there was persuasive evidence of functional consequences. The remaining sequence variants/genotypes were classified into causative, contributory or neutral categories by consideration of (i their allele frequencies in patient and normal control populations, (ii their presumed or experimentally confirmed functional effects, (iii the relative importance of their associated genes in the pathogenesis of chronic pancreatitis and (iv gene-gene interactions wherever applicable. Adoption of this strategy allowed us to assess the pathogenic relevance of specific variants/genotypes to their respective carriers to an unprecedented degree. The genetic cause of ICP could be assigned in 23.7% of individuals in the study group. A strong genetic susceptibility factor was also present in an additional 24.5% of cases. Taken together, up to 48.2% of the studied ICP patients were found to display evidence of a genetic basis for their pancreatitis. Whereas these particular proportions may not be extrapolable to all ICP patients, the approach employed should serve as a useful framework for acquiring a better understanding of the

  7. Two Mechanistic Pathways for Thienopyridine-Associated Thrombotic Thrombocytopenic Purpura

    Science.gov (United States)

    Bennett, Charles L.; Kim, Benjamin; Zakarija, Anaadriana; Bandarenko, Nicholas; Pandey, Dilip K.; Buffie, Charlie G.; McKoy, June M.; Tevar, Amul D.; Cursio, John F.; Yarnold, Paul R.; Kwaan, Hau C.; De Masi, Davide; Sarode, Ravindra; Raife, Thomas J.; Kiss, Joseph E.; Raisch, Dennis W.; Davidson, Charles; Sadler, J. Evan; Ortel, Thomas L.; Zheng, X. Long; Kato, Seiji; Matsumoto, Masanori; Uemura, Masahito; Fujimura, Yoshihiro

    2011-01-01

    Objectives We sought to describe clinical and laboratory findings for a large cohort of patients with thienopyridine-associated thrombotic thrombocytopenic purpura (TTP). Background The thienopyridine derivatives, ticlopidine and clopidogrel, are the 2 most common drugs associated with TTP in databases maintained by the U.S. Food and Drug Administration (FDA). Methods Clinical reports of TTP associated with clopidogrel and ticlopidine were identified from medical records, published case reports, and FDA case reports (n = 128). Duration of thienopyridine exposure, clinical and laboratory findings, and survival were recorded. ADAMTS13 activity (n = 39) and inhibitor (n = 30) were measured for a subset of individuals. Results Compared with clopidogrel-associated TTP cases (n = 35), ticlopidine-associated TTP cases (n = 93) were more likely to have received more than 2 weeks of drug (90% vs. 26%), to be severely thrombocytopenic (84% vs. 60%), and to have normal renal function (72% vs. 45%) (p 15% (n = 13), TTP patients with severely deficient ADAMTS13 activity (n = 26) were more likely to have received ticlopidine (92.3% vs. 46.2%, p 2 weeks after thienopyridine, therapeutic plasma exchange (TPE) increased likelihood of survival (84% vs. 38%, p < 0.05). Among patients who developed TTP within 2 weeks of starting thienopyridines, survival was 77% with TPE and 78% without. Conclusions Thrombotic thrombocytopenic purpura is a rare complication of thienopyridine treatment. This drug toxicity appears to occur by 2 different mechanistic pathways, characterized primarily by time of onset before versus after 2 weeks of thienopyridine administration. If TTP occurs after 2 weeks of ticlopidine or clopidogrel therapy, therapeutic plasma exchange must be promptly instituted to enhance likelihood of survival. PMID:17868804

  8. Cardiac manifestations of idiopathic pulmonary fibrosis

    OpenAIRE

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14–43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart fa...

  9. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

    Full Text Available Scoliosis is called idiopathic when no other underlying disease can be identified. The etiology of adolescent idiopathic scoliosis (AIS is still unknown despite many years of research effort. Theories on AIS's etiology have included mechanical, hormonal, metabolic, neuromuscular, growth, and genetic abnormalities. Skeletally immature patients with adolescent idiopathic scoliosis are at risk of curve progression. The adolescent onset of severe idiopathic scoliosis has traditionally been evaluated using standing posteroanterior radiographs of the full spine to assess lateral curvature with the Cobb method. Scoliosis in children of school age and above primarily occurs in girls. The therapeutic goal in children is to prevent progression. In children, scoliosis of 20 and deg; or more should be treated with a brace, and scoliosis of 45 and deg; or more with surgery. [Arch Clin Exp Surg 2014; 3(3.000: 174-182

  10. Review of idiopathic pancreatitis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins,autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted.

  11. Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    ... Explore Idiopathic Pulmonary Fibrosis What Is... How the Lungs Work Other Names Causes Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics How the Lungs Work Lung Transplant Pulmonary Hypertension Pulmonary Rehabilitation Respiratory Failure ...

  12. Idiopathic Gingival Hyperplasia

    OpenAIRE

    Cekmez, Ferhat; Pirgon, Ozgur; Tanju, Ilhan Asya

    2009-01-01

    Gingival hyperplasia is a rare condition but it is important for cosmetic and mechanic reasons and because of its potential as an indicator of systemic disease. Gingival fibromatosis may exist as an isolated abnormality or as part of a syndrome. In this article a case that was diagnosed clinically and histologically as idiopathic gingival fibromatosis is presented. Patient with gingival hyperplasia should be examined to exclude other reasons to determine the idiopathic gingival fibromatosis o...

  13. Atherosclerosis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2012-01-01

    Full Text Available Atherosclerosis is a chronic inflammatory disease of the arteries. Clinical consequences of the atherosclerotic process occur in the adult population, however atherosclerotic process begins in childhood. The classic risk factors for atherosclerosis include obesity, dyslipidaemia, age, gender or family history. In recent years, attention has been drawn to the similarity between atherosclerotic inflammatory processes and inflammatory changes in the course of systemic connective tissue disease, in particular systemic lupus etythematosus (SLE or rheumatoid arthritis (RA. There is also observed the similarity of the pathogenetic background of development of atherosclerosis and juvenile idiopathic arthritis (JIA. Elevated levels of pro-inflammatory cytokines are observed in the course of juvenile idiopathic arthritis. Also homocysteine concentrations, which may play a significant role in the development of atherosclerotic lesions, are observed higher in patients with JIA. Some studies revealed higher carotid intima-media thickness (IMT index values in children with JIA. In view of the fact that atherosclerotic process begins as early as in childhood, the introduction of appropriate preventive measures in children is a matter of utmost importance.

  14. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989–2008)

    Science.gov (United States)

    Zakarija, Anaadriana; Kwaan, Hau C.; Moake, Joel L.; Bandarenko, Nicholas; Pandey, Dilip K.; McKoy, June M.; Yarnold, Paul R.; Raisch, Dennis W.; Winters, Jeffrey L.; Raife, Thomas J.; Cursio, John F.; Luu, Thanh Ha; Richey, Elizabeth A.; Fisher, Matthew J.; Ortel, Thomas L.; Tallman, Martin S.; Zheng, X. Long; Matsumoto, Masanori; Fujimura, Yoshihiro; Bennett, Charles L.

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a fulminant disease characterized by platelet aggregates, thrombocytopenia, renal insufficiency, neurologic changes, and mechanical injury to erythrocytes. Most idiopathic cases of TTP are characterized by a deficiency of ADAMTS13 (a disintegrin and metalloprotease, with thrombospondin-1-like domains) metalloprotease activity. Ironically, use of anti-platelet agents, the thienopyridine derivates clopidogrel and ticlopidine, is associated with drug induced TTP. Data were abstracted from a systematic review of English-language literature for thienopyridine-associated TTP identified in MEDLINE, EMBASE, the public website of the Food and Drug Administration, and abstracts from national scientific conferences from 1991 to April 2008. Ticlopidine and clopidogrel are the two most common drugs associated with TTP in FDA safety databases. Epidemiological studies identify recent initiation of anti-platelet agents as the most common risk factor associated with risks of developing TTP. Laboratory studies indicate that most cases of thienopyridine-associated TTP involve an antibody to ADAMTS13 metalloprotease, present with severe thrombocytopenia, and respond to therapeutic plasma exchange (TPE); a minority of thienopyridine-associated TTP presents with severe renal insufficiency, involves direct endothelial cell damage, and is less responsive to TPE. The evaluation of this potentially fatal drug toxicity can serve as a template for future efforts to comprehensively characterize other severe adverse drug reactions. PMID:19180126

  15. A report of disseminated adenocarcinoma presenting as thrombotic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Joaquín Valle Alonso

    2011-10-01

    Full Text Available Thrombotic microangiopathies (TMAs represent a heterogeneous group of diseases characterized by a microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. TMAs encompass thrombotic thrombocytopenic purpura (TTP, typically characterized by fever, central nervous system manifestations and hemolytic uremic syndrome (HUS, in which renal failure is the prominent abnormality. In patients with cancer TMAs may be related to various antineoplastic drugs or to the malignant disease itself. The reported series of patients with TMAs directly related to cancer are usually heterogeneous, retrospective, and encompass patients with hematologic malignancies with solid tumors or receiving chemotherapy, each of which may have distinct presentations and pathophysiological mechanisms. Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP. Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature. We present a case of a 34-year-old man with TTP syndrome secondary to metastatic small-bowel adenocarcinoma. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.

  16. A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis

    Directory of Open Access Journals (Sweden)

    Arimoto M

    2012-03-01

    Full Text Available Miyoko Arimoto1, Yutaka Komiyama2, Fumiko Okamae1, Akemi Ichibe1, Setsuko Teranishi1, Hirohiko Tokunaga1, Keiko Nakaya3, Michie Fujiwara3, Manabu Yamaoka4, Shuji Onishi4, Rie Miyamoto5, Naoto Nakamichi5, Shosaku Nomura51Blood Transfusion Unit, Kansai Medical University Takii Hospital, 2Department of Clinical Sciences and Laboratory Medicine, Kansai Medical University, 3Clinical Medical Technology Unit, Kansai Medical University Takii Hospital, 4Blood Transfusion Unit, Kansai Medical University Hirakata Hospital, 5First Department of Internal Medicine, Kansai Medical University, Moriguchi, JapanAbstract: Thrombotic thrombocytopenic purpura (TTP is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.Keywords: thrombotic thrombocytopenic purpura, ADAMTS13, acute pancreatitis, plasma exchange

  17. Bone marrow stroma in idiopathic myelofibrosis and other haematological diseases. An immunohistochemical study

    DEFF Research Database (Denmark)

    Lisse, I; Hasselbalch, H; Junker, P

    1991-01-01

    Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked...... and capillarization, with the development of continuous sheets of basement membrane material beneath endothelial cells....

  18. AMEGAKARYOCYTIC THROMBOCYTOPENIC PURPURA‎: A FIFTEEN YEAR EXPERIENCE

    Directory of Open Access Journals (Sweden)

    M. Bakhshi

    2006-07-01

    Full Text Available Totally implantable venous access devices (TIVAD or implantable catheter ports are devices which can be implanted subcutaneously. They enable prolonged and repeated access to the vascular system, into the peritoneal cavity or intravertebral space. This device is particularly useful for repeated medical injection, for blood sampling or transfusion of blood and blood derivatives and for total parenteral nutrition (TPN. Although many patients benefit from the insertion of TIVAD without any secondary effects, any surgical implantation can nevertheless lead to complications. ‎In this study, we investigated the advantages and disadvantages of TIVAD catheter in pediatric age group. A total of 94 cases, 2 to 14 years old, were included in our study. We implanted TIVAD in these patients for chemotherapy in 83 cases (88.29%, for prolonged TPN in 6 cases (6.38%, for corticosteroid and antibiotic therapy after ‎Kasai operation in 2 cases (2.12%, for intermittent IV therapy in 2 cases (2.12% and for need to partial parenteral nutrition in 1 case (1.06%. Out of 94 cases, 14 cases (15% had some kind of complications and 80 cases (85% had no complication. There was no mortality. Most patients and their parents (82 cases, 87.23% were satisfied from TIVAD. ‎It seems that TIVAD can be a useful device for many chronic patients who need an IV access for multiple injections.

  19. Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

    Directory of Open Access Journals (Sweden)

    Işın Yaprak

    2010-09-01

    Full Text Available Objective: Immune thrombocytopenic purpura (ITP is the most common cause of acquired thrombocytopenia in children. The objective of this study was to evaluate the presenting features, variation in the clinical courses, initial response rate to therapy, and long-term outcome in patients with ITP. Materials and Methods: Three hundred and fifty out of 491 newly diagnosed patients with ITP between the initial diagnosis ages of 6 months to 16 years were included in this retrospective, descriptive study. Patients with acute vs chronic ITP, acute vs recurrent ITP and chronic vs recurrent ITP were compared in terms of age at diagnosis, gender, initial platelet count, response rate to initial therapy, long-term outcome, and total duration of follow-up. Results: The clinical courses of the patients were determined as acute, chronic and recurrent in 63.8%, 29.1%, and 7.1%, respectively. Platelet count >20x109/L and initial diagnosis age >10 years were found to increase the probability of chronic outcome by at least two-fold. Conclusion: It is concluded that ITP in childhood is a common disease with low morbidity and mortality. In addition to the acute and chronic form, a rare recurrent form, which accounts for about 4-7% of all ITP patients, should be considered.

  20. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo;

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class...

  1. Immunologic effects of anti-D (WinRho-SD) in children with immune thrombocytopenic purpura.

    Science.gov (United States)

    Zimmerman, S A; Malinoski, F J; Ware, R E

    1998-02-01

    Intravenous immunoglobulin (IVIG) is an effective treatment for immune thrombocytopenic purpura (ITP) that induces transient blockade of the reticuloendothelial system (RES) with additional effects including alteration of T lymphocyte subsets and suppression of in vitro T lymphocyte proliferation. As anti-D also is an effective treatment for ITP, we investigated its in vitro and in vivo immunologic effects. The in vitro effects of various agents used in ITP therapy were compared using T lymphocyte proliferation assays. Anti-D caused significantly less inhibition than IVIG or dexamethasone, but non-specific protein was as suppressive as IVIG. Six children with chronic ITP were studied following anti-D administration. Patients received a single dose of anti-D (WinRho-SD, 50 microg/kg i.v. over 5 min) and were studied on day 0, day 7, and 1 month later. Anti-D did not affect T lymphocyte subsets including the T cell receptor variable beta repertoire, in vitro T lymphocyte proliferation to mitogens, recall antigens, or interleukin-2, in vitro IgG synthesis induced by pokeweed mitogen, or T lymphocyte cytokine mRNA levels. We conclude that anti-D has no demonstrable in vitro or in vivo effects on lymphocyte enumeration or function, and therefore likely is effective in the treatment of ITP primarily through RES blockade. PMID:9462545

  2. Optimal management of idiopathic scoliosis in adolescence

    Directory of Open Access Journals (Sweden)

    Kotwicki T

    2013-07-01

    the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical. Optimal management of idiopathic scoliosis requires cooperation within a professional team which includes the entire therapeutic spectrum, extending from simple watchful observation of nonprogressive mild deformities through to early surgery for rapidly deteriorating curvature. Probably most demanding is adequate management with regard to the individual course of the disease in a given patient, while avoiding overtreatment or undertreatment. Keywords: management, idiopathic scoliosis, adolescence

  3. Immune thrombocytopenic purpura secondary to cytomegalovirus infection: A case report.

    Directory of Open Access Journals (Sweden)

    Bessy S Flores Chang

    2015-11-01

    Full Text Available Immune Thrombocytopenic Purpura (ITP is defined as an acquired thrombocytopenia with antibodies detected against platelet surface antigens, and it is the most common form of thrombocytopenia in otherwise asymptomatic adults. ITP secondary to an underlying condition is a diagnosis of exclusion that is essential to establish for treatment efficacy. Secondary thrombocytopenia caused by Cytomegalovirus (CMV is common, however case reports associated with diagnosis in immunocompetent adults are rare, and to the best of our knowledge only 20 publications have been associated with this diagnosis. Our report is based on a clinical presentation of a 37 year old female complaining of petechiae, heavy menses, shortness of breath and a platelet count of 1 X 109 /L. Treatment with IVIG and steroids failed to improve platelet count. Subsequently, an infectious laboratory workup was performed, detecting CMV infection, and treatment with antiviral agents was initiated, causing platelet count to increase as viral load decreased.

  4. The clinical effect of clomipramine in chronic idiopathic pain disorder revisited using the Spielberger State Anxiety Symptom Scale (SSASS) as outcome scale

    DEFF Research Database (Denmark)

    Bech, Per; Gormsen, Lise; Loldrup, Dorte;

    2009-01-01

    only the negatively phrased items was used to measure the pure anxiolytic effect. The analgesic effect was measured by the Visual Analogue Scale (VAS) for bodily pain. The General Health Questionnaire (GHQ-12) was used to identify minor affective states. RESULTS: In total 171 patients with chronic non......-malignant pain were included (87 patients received placebo and 84 clomipramine). On the SSASS, clomipramine's (mean dose 125 mg daily) advantage over placebo in the planned 6-weeks' treatment period for all patients (intention-to-treat analysis) showed an effect size of 0.37. For completers only, the effect size...... was 0.45. In total 76 patients were GHQ-12 positive, and the effect size in favour of clomipramine was 0.50 (intention-to-treat approach) and 0.66 for completers. In general, the effect on the Bodily Pain VAS, i.e. the analgesic outcome, was low. Thus, even in completers who were GHQ-12 positive...

  5. 消风散联合地氯雷他定治疗小儿慢性特发性荨麻疹的疗效观察%Efficacy observation of Xiaofeng powder combined with loratadine in treatment of children with chronic idiopathic urticaria

    Institute of Scientific and Technical Information of China (English)

    李学燕; 于海凤

    2014-01-01

    目的:研究消风散联合地氯雷他定治疗小儿慢性特发性荨麻疹的临床疗效。方法选取120例小儿慢性荨麻疹患儿,随机分为治疗组与对照组各60例,其中对照组仅采用地氯雷他定治疗,而治疗组应用消风散与地氯雷他定联合治疗,比较两组疗效。结果两组治愈率、总有效率及复发率对比差异显著,具有统计学意义(P<0.05)。结论消风散联合地氯雷他定治疗小儿慢性特发性荨麻疹具有较好的疗效,值得临床推广。%Objective To study the clinical efficacy of Xiaofeng powder combined with loratadine in the treatment of children with chronic idiopathic urticaria. Methods 120 children with chronic idiopathic urticaria were selected and randomly divided into the treatment group and the control group,with 60 patients in each group. The control group received desloratadine treatment and the treatment group received Xiaofeng powder combined with desloratadine. The efficacy of the two groups was compared. Results The two groups were significantly different in the cure rate,total effective rate and recurrence rate,with statistical significance(P < 0.05). Conclusion Xiaofeng powder combined with loratadine shows good efficacy in the treatment of children with chronic idiopathic urticaria,thereby worthy of promotion.

  6. Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients.

    Science.gov (United States)

    Scaradavou, A; Woo, B; Woloski, B M; Cunningham-Rundles, S; Ettinger, L J; Aledort, L M; Bussel, J B

    1997-04-15

    We report the results of intravenous anti-D (WinRho, WinRho SD) therapy in 261 non-splenectomized patients treated at the New York Hospital-Cornell Medical Center over the period from 1987 to 1994. Children (n = 124) and adult patients (n = 137) with classic immune thrombocytopenic purpura (ITP; n = 156) or human immunodeficiency virus (HIV) related thrombocytopenia (n = 105) and acute (n = 75) or chronic (n = 186) disease at the time of the initial anti-D treatment were studied. In addition, 11 previously splenectomized patients were treated as a separate group. Our objectives were to evaluate the following. (1) Efficacy of anti-D: The response after the initial infusion was analyzed according to clinical parameters, such as patient's age, HIV status, gender, disease duration, pretreatment platelet count, and hemoglobin value, as well as treatment-related factors, including the dose of anti-D, the solvent detergent treatment of the preparation, and the type of administration. (2) Use of anti-D as maintenance therapy: The duration of response after the initial infusion and the results of subsequent treatments were evaluated. (3) Safety/toxicity of anti-D: Postinfusion reactions and hemoglobin decrease after treatment were studied. Anti-D is a safe treatment providing a hemostatic platelet increase in greater than 70% of the Rh+ non-splenectomized patients. The group with the best results is HIV- children, but all patient groups respond and the effect lasts more than 21 days in 50% of the responders. Duration of response is not influenced by HIV status; furthermore, HIV+ patients show no adverse effects on hemoglobin decrease or HIV disease progression. Patients with chronic ITP after splenectomy have minimal or no response to intravenous anti-D. PMID:9108386

  7. Juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Prakken, Berent; Albani, Salvatore; Martini, Alberto

    2011-01-01

    Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years. Pivotal studies in the past 5 years have led to substantial progress in various areas, ranging from disease classification to new treatments. Gene expres

  8. High-dose acetylcysteine in idiopathic pulmonary fibrosis.

    NARCIS (Netherlands)

    Demedts, M.; Behr, J.; Buhl, R.; Costabel, U.; Dekhuijzen, P.N.R.; Jansen, H.M.L.; MacNee, W.; Thomeer, M.; Wallaert, B.; Laurent, F.; Nicholson, A.G.; Verbeken, E.K.; Verschakelen, J.; Flower, C.D.; Capron, F.; Petruzzelli, S.; Vuyst, P. De; Bosch, J.M. van den; Rodriguez-Becerra, E.; Corvasce, G.; Lankhorst, I.L.M.; Sardina, M.; Montanari, M.

    2005-01-01

    BACKGROUND: Idiopathic pulmonary fibrosis is a chronic progressive disorder with a poor prognosis. METHODS: We conducted a double-blind, randomized, placebo-controlled multicenter study that assessed the effectiveness over one year of a high oral dose of acetylcysteine (600 mg three times daily) add

  9. Anti-neutrophil cytoplasmic antibodies in idiopathic inflammatory disorders

    NARCIS (Netherlands)

    Mulder, Alide Heleen Leontine

    1994-01-01

    This thesis studies the prevalence of anti-neutrophil cytoplasmic antibodies (ANCA) in idiopathic inflammatory disorders in which GS-ANA were described during the 60s: rheumatoid arthritis, autoimmune liver diseases, inflammatory bowel disease, and juvenile chronic arthritis. In all of these disorde

  10. Eosinofilia reacional, leucemia eosinofílica crônica e síndrome hipereosinofílica idiopática Reactive eosinophilia, chronic eosinophilic leukemia and idiopathic hypereosinophilic syndrome

    Directory of Open Access Journals (Sweden)

    Maria de Lourdes Lopes Ferrari Chauffaille

    2010-01-01

    conditions described as eosinophilia. Now these conditions are better understood due to the evolution of cellular and molecular biology. This knowledge has helped to characterize distinct disorders involving myeloid and lymphoid lineages. Hence, eosinophilia is categorized as reactive, clonal or idiopathic. With the introduction of anti-tyrosine kinase (imatinib mesylate therapy, which is effective for the FIP1L1/PDGFRa rearrangement, there is a possibility to control or cure chronic eosinophilic leukemia. For this reason, precise and fast diagnosis is necessary for ideal therapeutic decisions before organic lesions that are irreversible, such as heart injury, become established. The aim of this manuscript is to review eosinophilia and offer an update on diagnostic and therapeutic investigations.

  11. Idiopathic thromobocytopenic purpura in two mothers of children with DiGeorge sequence: A new component manifestation of deletion 22q11?

    Energy Technology Data Exchange (ETDEWEB)

    Levy, A.; Philip, N. [Hopital d`Enfants de la Timone, Marseilles (France); Michel, G. [Hopital d`Enfants de la Timone, Marseilles (France)] [and others

    1997-04-14

    The phenotypic spectrum caused by the microdeletion of chromosome 22q11 region is known to be variable. Nearly all patients with DiGeorge sequence (DGS) and approximately 60% of patients with velocardiofacial syndrome exhibit the deletion. Recent papers have reported various congenital defects in patients with 22q11 deletions. Conversely, some patients have minimal clinical expression. Ten to 25% of parents of patients with DGS exhibit the deletion and are nearly asymptomatic. Two female patients carrying a 22q11 microdeletion and presenting with idiopathic thrombocytopenic purpura are reported. Both had children with typical manifestations of DGS. 12 refs., 4 figs., 1 tab.

  12. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... Home Health Conditions juvenile idiopathic arthritis juvenile idiopathic arthritis Enable Javascript to view the expand/collapse boxes. ... All Open All Close All Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  13. Juvenile idiopathic arthritis: a clinical overview

    Energy Technology Data Exchange (ETDEWEB)

    Davidson, J

    2000-02-01

    The chronic arthritides in childhood remain a poorly understood group of conditions. Their classification has been a source of much confusion over the years with differences in terminology between Europe and North America. A significant step forward in paediatric rheumatology has been the recent development of an internationally agreed classification system which uses the overall term juvenile idiopathic arthritis (JIA). The various subtypes of JIA and their clinical features are described, together with an overview of their differential diagnosis, complications and outcomes. An outline of current management strategies is given and potential future developments highlighted.

  14. Phototesting In The Idiopathic Photodermatoses Among Indians

    Directory of Open Access Journals (Sweden)

    Bejoy P

    1998-01-01

    Full Text Available 50 patients with idiopathic photodermatoses were phototested using the solar simulator, with broad spectrum wavebands of the whole spectrum (WS and the whole spectrum â€" ultraviolet B (WS- UVB to determine the minimal erythema dose. Cases diagnosed as solar urticaria were further tested with visible light. Among the photodermatoses seen in this part of the country polymorphic light eruption (PLE was the commonest followed by chronic actinic dermatitis (CAD and solar urticaria. The UVB wavelengths appeared to be more responsible than UVA in PLE and CAD while the UVA and visible wavelengths were responsible for solar urticaria.

  15. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    Science.gov (United States)

    Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

    2015-01-01

    Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

  16. Idiopathic chronic eosinophilic pneumonia. A clinical and follow-up study of 62 cases. The Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).

    Science.gov (United States)

    Marchand, E; Reynaud-Gaubert, M; Lauque, D; Durieu, J; Tonnel, A B; Cordier, J F

    1998-09-01

    Idiopathic chronic eosinophilic pneumonia (CEP) is a rare disorder of unknown cause with nonspecific respiratory and systemic symptoms but rather characteristic peripheral alveolar infiltrates on imaging, developing mainly in women and in atopic subjects. The disorder is highly responsive to oral corticosteroid therapy, but relapses are frequent on reducing or stopping treatment. The long-term course of the disease and data regarding outcome, particularly the need for prolonged oral corticosteroid therapy and the development of severe asthma, are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features and, above all, the later course of CEP in a large homogeneous series of 62 stringently selected patients of whom 46 were followed for more than 1 year. The prevalence of smokers was low (6.5%) and about half of our patients (51.6%) had a previous, and often prolonged, history of asthma. The clinical and roentgenographic features were in keeping with previous studies, but we found that computed tomography could disclose ground glass opacities not detected by X-ray, and that migratory infiltrates before treatment were more frequent (25.5%) than reported previously. The bronchoalveolar lavage cellular count always showed a striking eosinophilic pattern, thus allowing distinction between CEP and cryptogenic organizing pneumonia, both syndromes sharing many common clinical and imaging features. About two-thirds of the patients (68%) showed a ventilatory defect in pulmonary function tests, with about one-half of these presenting with an obstructive pattern, sometimes without previous asthma. Along with the submucosal eosinophilic infiltration noted in 2 patients without ventilatory defect, this is strong evidence to confirm that CEP is not only an alveolointerstitial but also an airway disease. The dramatic response to oral corticosteroid therapy was observed in all treated patients. Although only 1

  17. Acute Idiopathic Scrotal Edema

    Directory of Open Access Journals (Sweden)

    Micheál Breen

    2013-01-01

    Full Text Available We report a case of acute idiopathic scrotal edema (AISE in a 4-year-old boy who presented with acute scrotal pain and erythema. The clinical features, ultrasound appearance, and natural history of this rare diagnosis are reviewed. In this report, we highlight the importance of good ultrasound technique in differentiating the etiology of the acute scrotum and demonstrate the color Doppler “Fountain Sign” that is highly suggestive of AISE.

  18. Idiopathic Brachial Neuritis

    OpenAIRE

    Gonzalez-Alegre, Pedro; Recober, Ana; Kelkar, Praful

    2002-01-01

    Idiopathic brachial neuritis is a well defined clinical condition that most commonly affects young adults, seen usually by primary care physicians, neurologists or orthopaedic surgeons. Its onset is characterized by acute, aching shoulder pain lasting a few days to weeks, followed by progressive shoulder girdle and upper extremity weakness and atrophy, with a slow but progressive recovery of motor function over 6 to 18 months. Its early recognition can help avoid unnecessary and potentially h...

  19. Idiopathic granulomatous mastitis

    International Nuclear Information System (INIS)

    Objective was to discuss the clinical and radiological features and treatment approaches in 14 patients diagnosed with idiopathic granulomatous mastitis (GM). We retrospectively evaluated the clinical features, radiological findings and treatment approaches in 14 patients with idiopathic GM in the General Surgery Department, Gulhane School of Medicine, Ankara, Turkey between April 2000 and June 2006. The mean age of the patients was 34.5 years (range 27-41 years). The complaints at admission were a mass in the breast in 7 (50%) patients, an abscess and a mass in 6 (42.8%) and a skin fisculain one (7.2%). Granulomatous mastitis was unilateral in all subjects (on the right in 5 patients and on the left in 9). All of the patients underwent ultrasonographic evaluation. Mammography was performed in 8 and magnetic resonance imaging in 5 patients. Seven patients (50%) were suspected to have breast carcinoma according radiological findings. We performed the large excision in 11, incisional biopsy plus abscess drainage in one, and incisional biopsy plus abscess drainage plus medical treatment (prednisolone, methotrexate) in 2 patients. Due to the development of abscess after 9 months, drainage and large excision were performed in one patient who received medical treatment. Idiopathic GM is a disease that generally affects young women of reproductive age and may be mistaken for breast carcinoma in clinical and radiological evaluations. The gold standard for the diagnosis is histopathologic evaluation. (author)

  20. FATAL OUTCOME OF INFECTION BY DENGUE 4 IN A PATIENT WITH THROMBOCYTOPENIC PURPURA AS A COMORBID CONDITION IN BRAZIL

    Directory of Open Access Journals (Sweden)

    Frederico Figueiredo Amâncio

    2014-06-01

    Full Text Available Dengue is currently a major public-health problem. Dengue virus (DENV is classified into four distinct serotypes, DENV 1-4. After 28 years of absence, DENV-4 was again detected in Brazil in 2010 in Roraima State, and one year later, the virus was identified in the northern Brazilian states of Amazonas and Pará, followed by Rio de Janeiro and São Paulo. In Minas Gerais, the first confirmed case of DENV-4 occurred in the municipality of Frutal in 2011 and has now been isolated from a growing number of patients. Although DENV-2 is associated with the highest risk of severe forms of the disease and death due to the infection, DENV-4 has also been associated with severe forms of the disease and an increasing risk of hemorrhagic manifestations. Herein, the first fatal case of confirmed DENV-4 in Brazil is reported. The patient was an 11-year-old girl from the municipality of Montes Claros in northern Minas Gerais State, Brazil. She had idiopathic thrombocytopenic purpura as a comorbid condition and presented with a fulminant course of infection, leading to death due to hemorrhagic complications. Diagnosis was confirmed by detection of Dengue-specific antibodies using IgM capture enzyme-linked immunosorbent assay and semi-nested RT-PCR. Primary care physicians and other health-care providers should bear in mind that DENV-4 can also result in severe forms of the disease and lead to hemorrhagic complications and death, mainly when dengue infection is associated with coexisting conditions.

  1. Two cases of thrombocytopenic purpura at onset of Zika virus infection.

    Science.gov (United States)

    Chraïbi, Samy; Najioullah, Fatiha; Bourdin, Carole; Pegliasco, Jean; Deligny, Christophe; Résière, Dabor; Meniane, Jean-Côme

    2016-10-01

    We report here two cases of thrombocytopenic purpura at onset of Zika virus infection. A 26-year-old woman and a 21-year-old man had thrombocytopenia above 5×10(9) platelets/L. Hemorrhagic symptoms were mucosal and subcutaneous bleeding and gross hematuria and they reported episode of conjunctivitis. In both cases blood and bone marrow analysis suggested thrombocytopenic purpura, blood PCR tests for Dengue (DENV), Chikungunya (CHIKV) and Zika virus (ZIKV) were negative. In both cases urinary PCR for ZIKV was positive, Prednisolone yielded early remission. Only three similar cases have been reported so far. In the Caribbean, DENV is also epidemic and responsible for severe thrombocytopenia. Coinfections can occur. Our report underlines the need to include a ZIKV assay in the diagnostic work-up of thrombocytopenic purpura in epidemic areas.

  2. Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy.

    Science.gov (United States)

    von Auer, Charis; von Krogh, Anne-Sophie; Kremer Hovinga, Johanna A; Lämmle, Bernhard

    2015-02-01

    The complex relation between thrombotic thrombocytopenic purpura (TTP) and pregnancy is concisely reviewed. Pregnancy is a very strong trigger for acute disease manifestation in patients with hereditary TTP caused by double heterozygous or homozygous mutations of ADAMTS13 (ADisintegrin And Metalloprotease with ThromboSpondin type 1 domains, no. 13). In several affected women disease onset during their first pregnancy leads to the diagnosis of hereditary TTP. Without plasma treatment mother and especially fetus are at high risk of dying. The relapse risk during a next pregnancy is almost 100% but regular plasma transfusion starting in early pregnancy will prevent acute TTP flare-up and may result in successful pregnancy outcome. Pregnancy may also constitute a mild risk factor for the onset of acute acquired TTP caused by autoantibody-mediated severe ADAMTS13 deficiency. Women having survived acute acquired TTP may not be at very high risk of TTP relapse during an ensuing next pregnancy but seem to have an elevated risk of preeclampsia. Monitoring of ADAMTS13 activity and inhibitor titre during pregnancy may help to guide management and to avoid disease recurrence. Finally, TTP needs to be distinguished from the much more frequent hypertensive pregnancy complications, preeclampsia and especially HELLP (Hemolysis, Elevated Liver Enzymes, Low Platelet count) syndrome. PMID:25903530

  3. Helicobacter pylori infection in patients with autoimmune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    Erdal Kurtoglu; Ertugrul Kayacetin; Aysegul Ugur

    2004-01-01

    AIM: To compare the prevalence of Helicobacter pylori (Hpylori) infection in autoimmune thrombocytopenic purpura (AITP) patients with that of nonthrombocytopenic controls,and to evaluate the efficacy of the treatment in H pylori(+)and H pylori(-) AITP patients.METHODS: The prevalence of gastric H pylori infection in 38 adult AITP patients (29 female and 9 male; median age 27 years; range 18-39 years) who consecutively admitted to our clinic was investagated.RESULTS: H pylori infection was found in 26 of 38 AITP patients (68.5%). H pylori infection was found in 15 of 23control subjects (65.2%). The difference in H pylori infection between the 2 groups was not significant. Thrombocyte count of H pylori-positive AITP patients was significantly lower than that of H pylori-negative AITP patients (P<0.05).Thrombocyte recovery of H pylori-positive group was less than that of H pylori-negative group (P<0.05).CONCLUSION: H pylori infection should be considerecd in the treatment of AITP patients with H pylori infection.

  4. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity.

    Science.gov (United States)

    Frankel, Stephen K; Cool, Carlyne D; Lynch, David A; Brown, Kevin K

    2004-12-01

    Between 1996 and 2001, we identified five cases of a unique idiopathic pleuroparenchymal lung disease characterized by a clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, marked pleural and parenchymal radiographic involvement with an upper lobe predominance, and surgical lung biopsy findings that did not fit with any of the currently defined interstitial pneumonias. The pathologic findings included the following: (1) intense fibrosis of the visceral pleura; (2) prominent, homogenous, subpleural fibroelastosis; (3) sparing of the parenchyma distant from the pleura; (4) mild, patchy lymphoplasmacytic infiltrates; and (5) small numbers of fibroblastic foci present at the leading edge of the fibrosis. In this report, we characterize the clinical, radiographic, physiologic, and pathologic findings of this entity, which we term idiopathic pleuroparenchymal fibroelastosis. PMID:15596706

  5. Idiopathic fistula-in-ano

    Institute of Scientific and Technical Information of China (English)

    Sherief Shawki; Steven D Wexner

    2011-01-01

    Fistula-in-ano is the most common form of perineal sep- sis. Typically, a fistula includes an internal opening, a track, and an external opening. The external opening might acutely appear following infection and/or an abs-cess, or more insiduously in a chronic manner. Mana-gement includes control of infection, assessment of the fistulous track in relation to the anal sphincter muscle, and finally, definitive treatment of the fistula. Fistulo-tomy was the most commonly used mode of manage-ment, but concerns about post-fistulotomy incontinence prompted the use of sphincter preserving techniques such as advancement flaps, fibrin glue, collagen fistula plug, ligation of the intersphincteric fistula track, and stem cells. Many descriptive and comparative studies have evaluated these different techniques with variable outcomes. The lack of consistent results, level I eviden-ce, or long-term follow-up, as well as the heterogeneity of fistula pathology has prevented a definitive treatment algorithm. This article will review the most commonly available modalities and techniques for managing idio-pathic fistula-in-ano.

  6. Premature subclinical atherosclerosis in children and young adults with juvenile idiopathic arthritis. A review considering preventive measures

    DEFF Research Database (Denmark)

    Bohr, Anna-Helene; Fuhlbrigge, Robert C; Pedersen, Freddy Karup;

    2016-01-01

    Many studies show that Juvenile Idiopathic Arthritis (JIA) is associated with early subclinical signs of atherosclerosis. Chronic inflammation per se may be an important driver but other known risk factors, such as dyslipidemia, hypertension, insulin insensitivity, a physically inactive lifestyle...

  7. [Idiopathic progressive subglottic stenosis].

    Science.gov (United States)

    Sittel, C

    2014-07-01

    Idiopathic subglottic stenosis is causing a narrowing of the central airway at the laryngotracheal junction. Etiology is remaining unclear at large. There is a marked preponderance for women in the fertile age, an association to estrogene or progesterone metabolism remains doubtful. Suggested treatment varies from repeated endoscopic interventions to primary open resection. Therapy selection in this heterogeneous condition should be based on the individual patient situation as well as surgeon's expertise. This complex entity is prone to complications and should preferably be managed in a referral center.

  8. Idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne M; Jensen, Rigmor H

    2015-01-01

    AIMS: The aims of this article are to characterize the headache in idiopathic intracranial hypertension (IIH) and to field-test the ICHD diagnostic criteria for headache attributed to IIH. MATERIALS AND METHODS: We included 44 patients with new-onset IIH. Thirty-four patients with suspected but u...... tinnitus may suggest intracranial hypertension. Based on data from a well-defined IIH cohort, we propose a revision of the ICDH-3 beta diagnostic criteria with improved clinical applicability and increased sensitivity and specificity....

  9. Nocardiose pulmonar em paciente usuário crônico de corticóides Pulmonary nocardiosis in a patient who was a chronic corticosteroid user

    Directory of Open Access Journals (Sweden)

    Renata Maria Ferreira Brasileiro

    2007-10-01

    Full Text Available Nocardiose pulmonar é uma infecção grave e incomum causada pela Nocardia ssp, microorganismos que podem se comportar tanto como oportunistas quanto patógenos primários e que pode se apresentar como desordem aguda ou crônica supurativa. Relata-se o caso de um paciente de 49 anos, portador de púrpura trombocitopênica idiopática em tratamento com corticóides que desenvolveu infecção pulmonar por Nocardia farcinica, isolada em hemocultura. Apesar de tratamento correto com sulfametoxazol-trimetoprim, o paciente morreu após três meses de instituída terapia.Pulmonary nocardiosis is an infrequent and severe infection caused by Nocardia spp. These are microorganisms that may behave both as opportunists and as primary pathogens, and they may present as either acute or suppurative chronic disorders. We report on the case of a 49-year-old man with idiopathic thrombocytopenic purpura that was being treated with corticosteroids. He developed pulmonary infection due to Nocardia farcinica, which was isolated from blood cultures. Despite correct treatment with trimethoprim-sulfamethoxazole, the patient died three months after this therapy was instituted.

  10. A retrospective study of 72 cases diagnosed with idiopathic trigeminal neuralgia in indian populace

    OpenAIRE

    Yadav, Sunil; Mittal, Hitesh-Chander; Sachdeva, Akash; Verma, Ajay; Dhupar, Vikas; Dhupar, Anita

    2015-01-01

    Context: Trigeminal neuralgia is as a chronic, debilitating condition, which can have a major impact on quality of life. There are few reports of trigeminal neuralgia in oriental populations. Objectives: To evaluate the retrospective data of the patients diagnosed with idiopathic trigeminal neuralgia and to understanding the disorder in the Indian populace. Methods: The retrospective data of 72 patients with typical idiopathic trigeminal neuralgia regarding age of onset, gender, site of invol...

  11. /sup 111/In-oxine platelet survivals in thrombocytopenic infants

    Energy Technology Data Exchange (ETDEWEB)

    Castle, V.; Coates, G.; Kelton, J.G.; Andrew, M.

    1987-09-01

    Thrombocytopenia is a common occurrence (20%) in sick neonates, but the causes have not been well studied. In this report we demonstrate that thrombocytopenia in the neonate is characterized by increased platelet destruction as shown by shortened homologous /sup 111/In-oxine-labeled platelet life spans. Thirty-one prospectively studied thrombocytopenic neonates were investigated by measuring the /sup 111/In-labeled platelet life span, platelet-associated IgG (PAIgG), and coagulation screening tests. In every infant, the thrombocytopenia was shown to have a destructive component since the mean platelet life span was significantly shortened to 65 +/- 6 (mean +/- SEM) hours with a range of one to 128 hours compared with adult values (212 +/- 8; range, 140 to 260; gamma function analysis). The platelet survival was directly related to the lowest platelet count and inversely related to both the highest mean platelet volume and duration of the thrombocytopenia. In 22 infants the percent recovery of the radiolabeled platelets was less than 50%, which suggested that increased sequestration also contributed to the thrombocytopenia. Infants with laboratory evidence of disseminated intravascular coagulation (n = 8) or immune platelet destruction evidenced by elevated levels of PAIgG (n = 13) had even shorter platelet survivals and a more severe thrombocytopenia compared with the ten infants in whom an underlying cause for the thrombocytopenia was not apparent. Full-body scintigraphic images obtained in 11 infants showed an increased uptake in the spleen and liver, with a spleen-to-liver ratio of 3:1. This study indicates that thrombocytopenia in sick neonates is primarily destructive, with a subgroup having evidence of increased platelet sequestration.

  12. Platelet kinetics and scintigraphic imaging in thrombocytopenic malaria patients.

    Science.gov (United States)

    Karanikas, Georgios; Zedwitz-Liebenstein, Konstantin; Eidherr, Harald; Schuetz, Matthias; Sauerman, Robert; Dudczak, Robert; Winkler, Stefan; Pabinger, Ingrid; Kletter, Kurt

    2004-03-01

    Thrombocytopenia is a common occurrence in acute malaria. It is attributed, among other factors, to excessive splenic platelet pooling and a shortened platelet lifespan. The aim of our study was to evaluate the platelet kinetics and sequestration site by isotopic studies in uncomplicated malaria-induced thrombocytopenia. Seven thrombocytopenic malaria patients (74,000+/-36,000 platelets/ micro l) were included in the study. Autologous (111)In-labeled platelet scintigraphy was performed up to 96 hours (h) post injection (p.i.) to evaluate the platelet sequestration site. Late sequestration for the spleen (S) and the liver (L) was analyzed according to the following activity ratios: S (spleen count on the last day of the platelet lifespan / spleen count at 30 min) and L (liver count on the last day of the platelet lifespan / liver count at 30 min). Additionally, platelet survival studies were performed. A normal late sequestration (S: 0.95+/-0.06 and L: 1.04+/-0.08; normal values, S and L: 1+/-0.2.) was observed in all of our patients. The platelet lifespan was reduced (1 to 4 days; normal range, 7-9 days), recovery was normal (mean, 63+/-6%; normal range, 55-75%), and the turnover rate was enhanced (mean, 95,000+/-80,000/ micro l/day; normal value, 35,000+/-4,500/ micro l/ day). According to the results of scintigraphy, the sequestration site by uncomplicated malaria-induced thrombocytopenia appears to be non-splenic and/or hepatic, yet diffuse. PMID:14983232

  13. Clinical Study on Chronic Idiopathic Urticaria with Mizolastine Combined with Compound Glycyrrhizin in the Treatment of%对慢性特发性荨麻疹采用咪唑斯汀联合复方甘草酸苷治疗的临床探讨

    Institute of Scientific and Technical Information of China (English)

    谭李萍

    2015-01-01

    Objective To observe the clinical effect of mizolastine combined with Compound Glycyrrhizin in the treatment of chronic idiopathic urticaria.Methods 66 patients with chronic idiopathic urticaria were treated in our hospital from February 2013 March to, and were randomly divided into control group and observation group, 33 cases in each group. Simple of mizolastine in the treatment was given to the control group, the observation group in the control group based on the combined with Compound Glycyrrhizin in the treatment, compared two groups of clinical curative effect.Results Observation of the treatment group total effective rate, recurrence rate, nettle rash, itching and erythema number integral were significantly better than the control group (P<0.05) difference has statistical significance.Conclusion in patients with chronic idiopathic urticaria with mizolastine combined with Compound Glycyrrhizin in the treatment of clinical curative effect, worthy of clinical application and promotion.%目的:观察分析咪唑斯汀联合复方甘草酸苷治疗慢性特发性荨麻疹的临床疗效。方法选取我院2013年2月至2015年3月收治的66例慢性特发性荨麻疹患者为研究对象,随机分为对照组与观察组,各33例。对照组给予单纯的咪唑斯汀治疗,观察组在对照组基础上联合复方甘草酸苷治疗,比较两组临床疗效。结果观察组治疗总有效率、疾病复发率、风团、瘙痒及红斑数目积分均显著优于对照组,(P<0.05)差异具有统计学意义。结论对慢性特发性荨麻疹患者采用咪唑斯汀联合复方甘草酸苷治疗的临床疗效显著,值得临床广泛应用及推广。

  14. 老年特发性便秘患者直肠肛门动力学变化及心理评价%CHANGES IN ANORECTAL DYNAMICS AND PSYCHOLOGICAL EVALUATION IN ELDERLY PATIENTS WITH CHRONIC IDIOPATHIC CONSTIPATION

    Institute of Scientific and Technical Information of China (English)

    高利利; 吴本俨; 邵勇; 李园

    2001-01-01

    To investigate what anorectal dynamic abnormality and psychological factors exits in elderly patients with chronic idiopathic constipation (CIC), study was performed in 59 elderly patients with CIC and 36 elderly healthy subjects(EHS).Anorectal pressure was measured with Medtronic PcPolygraf,which was a water perfused catheter system.All subjests completed a self-rated inventory assessing psychological distress including depression(SDS) and anxiety(SAS). Maximal squeezing pressure and the changes in anal pressure during defecation in elderly patients(EP) with CIC were lower than those in EHS(P<0.05).The rectal lowest volume of sensory threshold(RLSTV),the rectal threshold volume for desire to defecate and the rectal maximal volume of tolerance were higher in EP than those in EHS(P<0.05). There were 28(47.5%) of EP who had increasing anal pressure when defecation was stimulated.The scores of SDS and SAS were higher in EP than those in EHS(P<0.05). It suggested that elderly patients with CIC may be associated with lower sensitivity,higher tolerance and discoordination of annorectum during defecation.Both depression and anxiety were associated with abnormal dynamics in EP with CIC.%探讨老年慢性特发性便秘(CIC)患者直肠肛门运动功能变化及其与心理因素的关系。采用瑞典Medtronic公司生产的8通道水灌注式消化道压力检测系统、Zung抑郁自评量表(SDS)及焦虑自评量表(SAS)对59例患者及36例老年对照者进行直肠肛门压力测定及心理状况分析。结果发现,老年CIC患者最大缩榨压、模拟排便时肛管压力变化低于老年对照组(P<0.05),直肠初始感觉阈值、排便阈值和最大耐受容量均高于老年对照组(P<0.05),并有28例(47.5%)CIC患者模拟排便时出现肛管压力异常升高。老年CIC患者SDS、SAS标准总分均明显高于老年对照组(P<0.05)。说明老年CIC患者与直肠低敏感、高耐受及排便

  15. Recurrent Thrombotic Thrombocytopenic Purpura-Like Syndrome as a Paraneoplastic Phenomenon in Malignant Peritoneal Mesothelioma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Francisco Socola

    2012-01-01

    Full Text Available We report the case of an African American male with no significant past medical history presenting with recurrent, rapidly relapsing episodes of thrombotic thrombocytopenic purpura (TTP despite aggressive treatment with several lines of treatment. Incidentally, these episodes were associated with severe abdominal pain which eventually developed into acute abdomen and prompted exploratory laparotomy, revealing diffuse carcinomatosis with a tumor located on the left pelvis that was encasing the distal sigmoid colon. Pathology made a final diagnosis of peritoneal mesothelioma. TTP-like syndrome (TTP-LS has been described as a paraneoplastic phenomenon in several malignancies but never before in the setting of malignant mesothelioma. Paraneoplastic TTP-like syndrome has historically been associated with a dismal prognosis and particular clinical and laboratory abnormalities described in this paper. It is of utmost importance to make a prompt determination whether TTP is idiopathic or secondary to an underlying condition because of significant differences in their prognosis, treatment, and response. This paper also reviews the current literature regarding this challenging condition.

  16. Adolescent Idiopathic Scoliosis

    Science.gov (United States)

    Choudhry, Muhammad Naghman; Ahmad, Zafar; Verma, Rajat

    2016-01-01

    Background: Scoliosis refers to deviation of spine greater than 10 degrees in the coronal plane. Idiopathic Scoliosis is the most common spinal deformity that develops in otherwise healthy children. The sub types of scoliosis are based on the age of the child at presentation. Adolescent idiopathic scoliosis (AIS) by definition occurs in children over the age of 10 years until skeletal maturity. Objective: The objective of this review is to outline the features of AIS to allow the physician to recognise this condition and commence early treatment, thereby optimizing patient outcome. Method: A thorough literature search was performed using available databases, including Pubmed and Embase, to cover important research published covering AIS. Conclusion: AIS results in higher incidence of back pain and discontent with body image. Curves greater than 50 degrees in thoracic region and greater than 30 degrees in lumbar region progress at a rate of 0.5 to 1 degree per year into adulthood. Curves greater than 60 degrees can lead to pulmonary functional deficit. Therefore once the disease is recognized, effective treatment should be instituted to address the deformity and prevention of its long-term sequelae. PMID:27347243

  17. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders.

    NARCIS (Netherlands)

    Besbas, N.; Karpman, D.; Landau, D.; Loirat, C.; Proesmans, W.; Remuzzi, G.; Rizzoni, G.; Taylor, C.M.; Kar, N.C.A.J. van de; Zimmerhackl, L.B.

    2006-01-01

    The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increase

  18. An overview of platelet indices and methods for evaluating platelet function in thrombocytopenic patients

    DEFF Research Database (Denmark)

    Vinholt, Pernille Just; Hvas, Anne-Mette; Nybo, Mads

    2014-01-01

    in thrombocytopenia. Flow cytometry, platelet aggregometry and platelet secretion tests are used to diagnose specific platelet function defects. The flow cytometric activation marker P-selectin and surface coverage by the Cone and Plate[let] analyser™ predict bleeding in selected thrombocytopenic populations...

  19. A rare and successfully managed case of idiopathic thrombocytopenic purpura (ITP with previous caesarean with splenectomy with hepatitis C positive

    Directory of Open Access Journals (Sweden)

    Rajshree Dayanand Katke

    2014-08-01

    Full Text Available ITP occurs in approximately 2 of 1000 pregnant women. ITP may develop at any time during pregnancy, but is often initially recognized in the first trimester and is the most common cause of isolated thrombocytopenia in this time period. A 33 year old, married since 6 years, G2p1l1 with 33 weeks gestation referred to our tertiary centre. She was a known case of ITP with splenectomy done. She had a 5 year old male child, delivered by LSCS. She was diagnosed as having ITP at the age of 12 years. HCV antibody was weakly positive. ANA was positive. Emergency LSCS was done in view of scar tenderness. Post op she developed epistaxis, bleeding gums and per vaginum. Patient received multiple FFP, platelet transfusions and responded to treatment with methylprednisolone. The diagnosis and management of ITP in pregnancy is similar to that in the non-pregnant adult patient, but the risks to the developing fetus must be taken into account when choosing treatment and the maintenance of a safe platelet count, rather than prolonged remission, is the goal. Mode of delivery must be guided by obstetrical indications. [Int J Reprod Contracept Obstet Gynecol 2014; 3(4.000: 1119-1121

  20. Does the site of platelet sequestration predict the response to splenectomy in adult patients with immune thrombocytopenic purpura?

    Science.gov (United States)

    Navez, Julie; Hubert, Catherine; Gigot, Jean-François; Navez, Benoit; Lambert, Catherine; Jamar, François; Danse, Etienne; Lannoy, Valérie; Jabbour, Nicolas

    2015-01-01

    Splenectomy is the only potentially curative treatment for chronic immune thrombocytopenic purpura (ITP) in adults. However, one-third of the patients relapse without predictive factors identified. We evaluate the predictive value of the site of platelet sequestration on the response to splenectomy in patients with ITP. Eighty-two consecutive patients with ITP treated by splenectomy between 1992 and 2013 were retrospectively reviewed. Platelet sequestration site was studied by (111)Indium-oxinate-labeled platelets in 93% of patients. Response to splenectomy was defined at last follow-up as: complete response (CR) for platelet count (PC) ≥100 × 10(9)/L, response (R) for PC≥30 × 10(9)/L and 100 versus <=100, 95% CI [0.025-0.493], p = 0.004) were significant predictors of recurrence-free survival in multivariate analysis. Response to splenectomy was independent of the site of platelet sequestration in patients with ITP. Pre-operative platelet sequestration study in these patients cannot be recommended. PMID:25275667

  1. Differential Diagnosis in Idiopathic Granulomatous Mastitis and Tuberculous Mastitis

    OpenAIRE

    Seo, Hee Ri Na; Na, Kuk Young; Yim, Hyun Ee; Kim, Tae Hee; Kang, Doo Kyoung; Oh, Ki Keun; Kang, Seok Yun; An, Young-Sil; Chun, Mison; Kim, Woojae; Park, Rae Woong; Jung, Yong Sik; Kim, Ku Sang

    2012-01-01

    Purpose Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory disease of unknown etiology. The diagnosis of IGM requires that other granulomatous lesions in the breast be excluded. Tuberculous mastitis (TM) is also an uncommon disease that is often difficult to differentiate from IGM. The purpose of this study is to develop a new algorithm for the differential diagnosis and treatment of IGM and TM. Methods Medical records of 68 patients (58 with IGM and 10 with TM) between Ju...

  2. Clinical case: Differential diagnosis of idiopathic pulmonary fibrosis

    OpenAIRE

    Cordeiro, Carlos Robalo; Alfaro, Tiago M.; Freitas, Sara

    2013-01-01

    Background The diagnosis of idiopathic pulmonary fibrosis can be quite challenging, even after careful clinical evaluation, imaging and pathological tests. This case report intends to demonstrate and discuss these difficulties, especially those concerning the differential diagnosis with chronic hypersensitivity pneumonitis. Case presentation A 58-year-old white male presented with shortness of breath, dry cough, fatigue and weight loss for two months. He was a former smoker and had regular ex...

  3. Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Basant R. Nassar BS

    2016-04-01

    Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH.

  4. Idiopathic Bilateral Bloody Tearing

    Directory of Open Access Journals (Sweden)

    Emrullah Beyazyıldız

    2015-01-01

    Full Text Available Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon.

  5. ADHD in idiopathic epilepsy

    Directory of Open Access Journals (Sweden)

    Marcos H. C. Duran

    2014-01-01

    Full Text Available Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners’ Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%, seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

  6. Idiopathic pulmonary fibrosis: treatment update.

    LENUS (Irish Health Repository)

    O'Connell, Oisin J

    2011-11-01

    Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Despite multiple recent clinical trials, there is no strong evidence supporting a survival advantage for any agent in the management of patients with IPF. The limited effectiveness of current treatment regimes has led to a search for novel therapies including antifibrotic strategies. This article reviews the evidence supporting the treatments currently used in the management of IPF.

  7. Managing juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Hawkins, Madeleine J; Dick, Andrew D; Lee, Richard J W; Ramanan, Athimalaipet V; Carreño, Ester; Guly, Catherine M; Ross, Adam H

    2016-01-01

    Bilateral chronic anterior uveitis is an extra-articular feature of juvenile idiopathic arthritis. Although figures vary, uveitis occurs in approximately 11%-13% of patients with this disease and is most commonly associated with the female gender, oligoarthritis, and presence of antinuclear antibodies. The disease has an insidious onset and is often asymptomatic. Managing patients with juvenile idiopathic arthritis-associated uveitis remains challenging as the disease may prove to be refractory to traditional treatment regimens. Stepwise immunomodulatory therapy is indicated, with new biologic drugs being used last in cases of refractory uveitis. Small scale studies and practice have provided the evidence to undertake randomized control trials to evaluate the efficacy, safety, and cost-effectiveness of anti-tumor necrosis factor-α therapies, such as infliximab and adalimumab. These have demonstrated promising results, with further data awaited from ongoing trials for adalimumab (as SYCAMORE and ADJUVITE trials). Lower grade evidence is supporting the use of newer biologics such as rituximab, daclizumab, tocilizumab, and abatacept in those cases refractory to anti-tumor necrosis factor-α therapy.

  8. Acute and subacute idiopathic interstitial pneumonias.

    Science.gov (United States)

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia. PMID:27123874

  9. Systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Cimaz, Rolando

    2016-09-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a systemic inflammatory disease which has up to now been classified as a category of juvenile idiopathic arthritis. However, in this context, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it may rather be part of the spectrum of autoinflammatory disorders. The disease is in fact unique with regard to the other JIA categories, in terms of clinical manifestations, prognosis, and response to conventional immunosuppressant therapies. It is characterized clinically by fever, lymphadenopathy, arthritis, rash, and serositis. IL-1 and IL-6 play a major role in the pathogenesis of SoJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SoJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation continue to be a major issue in patients' care. Recent advances on the pathogenesis and treatment have revolutionized the care and prognosis of this potentially life-threatening pediatric condition.

  10. Managing comorbidities in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Fulton BG

    2015-09-01

    Full Text Available Blair G Fulton,1 Christopher J Ryerson1,2 1Department of Medicine, 2Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada Abstract: Major risk factors for idiopathic pulmonary fibrosis (IPF include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature regarding the management of common pulmonary and extra-pulmonary comorbidities, including chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, venous thromboembolism, sleep-disordered breathing, gastroesophageal reflux disease, coronary artery disease, depression and anxiety, and deconditioning. Recent studies have provided some guidance on the management of these diseases in IPF; however, most treatment recommendations are extrapolated from studies of non-IPF patients. Additional studies are required to more accurately determine the clinical features of these comorbidities in patients with IPF and to evaluate conventional treatments and management strategies that are beneficial in non-IPF populations. Keywords: interstitial lung disease, management, idiopathic pulmonary fibrosis, comorbidities

  11. Plasma microRNA profiling of pediatric patients with immune thrombocytopenic purpura.

    Science.gov (United States)

    Bay, Ali; Coskun, Enes; Oztuzcu, Serdar; Ergun, Sercan; Yilmaz, Fatih; Aktekin, Elif

    2014-06-01

    Immune thrombocytopenic purpura (ITP) is a commonly acquired autoimmune bleeding disorder in children. MicroRNAs (miRNAs) are small RNAs which are found in cells and circulation, and play a role in protein synthesis and regulation. In this study, we aimed to determine a biomarker for childhood ITP comparing the plasma miRNA levels of children having ITP with healthy children. A total of 86 patients with ITP and 56 healthy children followed up by the Department of Pediatric Hematology and Oncology in University of Gaziantep since July 2011 were enrolled in the study. The 86 patients with ITP were evaluated in two groups as 43 acute ITP (aITP) and 43 chronic ITP (cITP) patients. Plasma expression levels of 379 miRNAs were investigated by RT-PCR (quantitative RT-PCR) technique and they were compared between aITP, cITP, and control groups. For all miRNAs, the average of raw quantification cycle values of three groups separately in the analysis chip was accepted as the reference gene value, and normalization was done according to this value. Statistically significant differences were detected in seven miRNAs (miR-302c-3p, miR-483-5p, miR-410, miR-544a, miR-302a-3p, miR-223-3p, and miR-597) investigated between the groups with respect to the expression levels. The expression rates were found to be over 95% in miR-302c-3p and miR-483-5p, over 75% in miR-410, and over 40% in miR-544, miR-302a-3p, and miR-223-3p in all three groups. The detection of significant differences between plasma miRNA levels of aITP and cITP patients and healthy children may provide useful information in the prediction of the course of disease, determination of disease etiopathogenesis, and the development of new therapeutic modalities.

  12. Cardiac manifestations of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14-43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  13. Idiopathic granulomatous mastitis: presentation, investigation and management.

    Science.gov (United States)

    Benson, John R; Dumitru, Dorin

    2016-06-01

    Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory condition of the breast which although benign can mimic carcinoma. Establishing a diagnosis can be challenging and requires a high index of suspicion with exclusion of infective and autoimmune breast diseases. IGM is characterized histologically by noncaseating granulomas which are of a lobulo-centric pattern and often associated with microabscess formation. Management of confirmed cases remains controversial with proponents of initial surgical or medical therapies - each has its associated problems which can be worse than the original symptoms of IGM. However, many patients require more than one modality of treatment to completely resolve IGM lesions and careful judgment is necessary to ensure optimal type and sequencing of treatments. PMID:27067146

  14. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... idiopathic pulmonary fibrosis. N Engl J Med. 2007 Mar 29;356(13):1317-26. Citation on PubMed ... PJ, Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational ...

  15. Adult idiopathic scoliosis: the tethered spine.

    Science.gov (United States)

    Whyte Ferguson, Lucy

    2014-01-01

    This article reports on an observational and treatment study using three case histories to describe common patterns of muscle and fascial asymmetry in adults with idiopathic scoliosis (IS) who have significant scoliotic curvatures that were not surgically corrected and who have chronic pain. Rather than being located in the paraspinal muscles, the myofascial trigger points (TrPs) apparently responsible for the pain were located at some distance from the spine, yet referred pain to locations throughout the thoracolumbar spine. Asymmetries in these muscles appear to tether the spine in such a way that they contribute to scoliotic curvatures. Evaluation also showed that each of these individuals had major ligamentous laxity and this may also have contributed to development of scoliotic curvatures. Treatment focused on release of TrPs found to refer pain into the spine, release of related fascia, and correction of related joint dysfunction. Treatment resulted in substantial relief of longstanding chronic pain. Treatment thus validated the diagnostic hypothesis that myofascial and fascial asymmetries were to some extent responsible for pain in adults with significant scoliotic curvatures. Treatment of these patterns of TrPs and muscle and fascial asymmetries and related joint dysfunction was also effective in relieving pain in each of these individuals after they were injured in auto accidents. Treatment of myofascial TrPs and asymmetrical fascial tension along with treatment of accompanying joint dysfunction is proposed as an effective approach to treating both chronic and acute pain in adults with scoliosis that has not been surgically corrected.

  16. Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions

    Directory of Open Access Journals (Sweden)

    Vivek Chowdhry

    2013-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP patients are at high-risk for bleeding complications during and after cardiac surgeries involving cardiopulmonary bypass. We report a patient with ITP with severe coronary artery disease and mitral valve regurgitation who underwent uncomplicated coronary artery bypass grafting and mitral valve replacement. Three weeks later, the patient was readmitted in a very low general condition with signs of pericardial tamponade. We describe our experience of managing the case.

  17. A case of refractory thrombotic thrombocytopenic purpura treated with plasmapheresis and rituximab.

    Science.gov (United States)

    Kirui, Nicholas; Sokwala, Ahmed

    2016-07-01

     Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder with no prevalence or incidence studies in sub-Saharan Africa. Acquired TTP has several causes, all of which lead to decreased activity of von Willebrand factor cleaving protease (ADAMTS13) due to autoantibodies that are directed towards ADAMTS13. We report a case of a 46-year-old man who presented with most of the classic clinical manifestations of TTP. PMID:27384362

  18. Peripheral digit ischemic syndrome can be a manifestation of postoperative thrombotic thrombocytopenic purpura

    OpenAIRE

    Chang, J. C.; Ikhlaque, N

    2004-01-01

    In addition to common dysfunction of the brain and kidney, thrombotic thrombocytopenic purpura (TTP) may present with atypical clinical features due to the involvement of other organs such as the lung, pancreas, heart, eye, and skin. We have also observed the unusual presentation of peripheral digit ischemic syndrome (PDIS) in some patients with postoperative TTP To clarify this relationship between TTP and PDIS, the hematologic data from the medical records of patients with known diagnoses o...

  19. DIFFERENT DISPARITIES OF GENDER AND RACE AMONG THE THROMBOTIC THROMBOCYTOPENIC PURPURA AND HEMOLYTIC-UREMIC SYNDROMES

    OpenAIRE

    Terrell, Deirdra R.; Vesely, Sara K.; Kremer Hovinga, Johanna A.; Lämmle, Bernhard; George, James N.

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) represent multiple disorders with diverse etiologies. We compared the gender and race of 335 patients enrolled in the Oklahoma TTP-HUS Registry across 21 years for their first episode of TTP or HUS to appropriate control groups. The relative frequency of women and white race among patients with TTP-HUS associated with a bloody diarrhea prodrome and the relative frequency of women with quinine-associated TTP-HUS were...

  20. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors

    OpenAIRE

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    French Reference Center for Thrombotic Microangiopathies International audience Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in Whi...

  1. Atypical presentations of thrombotic thrombocytopenic purpura: a diagnostic role for ADAMTS13.

    Science.gov (United States)

    Kalish, Yosef; Rottenstreich, Amihai; Rund, Deborah; Hochberg-Klein, Sarit

    2016-08-01

    Thrombotic thrombocytopenic purpura (TTP) is an acute, life threatening disease. Only a minority of patients expresses the complete clinical presentation and unusual manifestations can occur. Demonstration of low activity levels of ADAMTS13 (importance of having a high clinical suspicion of TTP in cases of thrombosis even without hematological abnormalities in patients with previous attacks of TTP. In this clinical scenario, measurement of ADAMTS13 activity is important for diagnosis and early administration of treatment. PMID:26867546

  2. Oral ezatiostat HCl (Telintra®, TLK199 and Idiopathic Chronic Neutropenia (ICN: a case report of complete response of a patient with G-CSF resistant ICN following treatment with ezatiostat, a glutathione S-transferase P1-1 (GSTP1-1 inhibitor

    Directory of Open Access Journals (Sweden)

    Lyons Roger M

    2011-11-01

    Full Text Available Abstract Idiopathic chronic neutropenia (ICN describes a heterogeneous group of hematologic diseases characterized by low circulating neutrophil levels often associated with recurrent fevers, chronic mucosal inflammation, and severe systemic infections. The severity and risk of complications, including serious infections, are inversely proportional to the absolute neutrophil count (ANC, with the greatest problems occurring in patients with an ANC of less than 0.5 × 109/L. This case report describes a 64-year-old female with longstanding rheumatoid arthritis who subsequently developed ICN with frequent episodes of sepsis requiring hospitalization and prolonged courses of antibiotics over a 4-year period. She was treated with granulocyte colony stimulating factors (G-CSF but had a delayed, highly variable, and volatile response. She was enrolled in a clinical trial evaluating the oral investigational agent ezatiostat. Ezatiostat, a glutathione S-transferase P1-1 inhibitor, activates Jun kinase, promoting the growth and maturation of hematopoietic progenitor stem cells. She responded by the end of the first month of treatment with stabilization of her ANC (despite tapering and then stopping G-CSF, clearing of fever, and healing of areas of infection. This ANC response to ezatiostat treatment has now been sustained for over 8 months and continues. These results suggest potential roles for ezatiostat in the treatment of patients with ICN who are not responsive to G-CSF, as an oral therapy alternative, or as an adjunct to G-CSF, and further studies are warranted.

  3. Male idiopathic oligoasthenoteratozoospermia

    Institute of Scientific and Technical Information of China (English)

    Giorgio Cavallini

    2006-01-01

    Idiopathic oligoasthenoteratozoospermia (iOAT) affects approximately 30% of all infertile men. This mini-review discussed recent data in this field. Age, non-inflammatory functional alterations in post-testicular organs, infective agents (Chlamydia trachomatis, herpes virus and adeno-associated viruses), alterations in gamete genome, mitochondrial alterations, environmental pollutants and "subtle" hormonal alterations are all considered possible causes of iOAT.Increase of reactive oxygen species in tubules and in seminal plasma and of apoptosis are reputed to affect sperm concentration, motility and morphology. iOAT is commonly diagnosed by exclusion, nevertheless spectral traces of the main testicular artery may be used as a diagnostic tool for iOAT. The following can be considered therapies for iOAT: 1) tamoxifen citrate (20 mg/d) + testosterone undecanoate (120 mg/d) (pregnancy rate per couple/month [prcm]:3.8%); 2) folic acid (66 mg/d) + zinc sulfate (5 mg/d); 3) L-carnitine (2 g/d) alone or in combination with acetyl-L-carnitine (1 g/d) (prcm: 2.3%); and 4) both carnitines + one 30 mg cinnoxicam suppository every 4 days (prcm: 8.5%).Alpha-blocking drugs improved sperm concentration but not morphology, motility or pregnancy rate. Tranilast (300 mg/d) increased sperm parameters and pregnancy rates in an initial uncontrolled study. Its efficacy on sperm concentration (but not on sperm motility, morphology or prcm) was confirmed in subsequent published reports. The efficacy of tamoxifen + testosterone undecanoate, tamoxifen alone, and recombinant follicle-stimulating hormone is still a matter for discussion.

  4. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant...

  5. Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

    LENUS (Irish Health Repository)

    Flanagan, Frances

    2013-03-01

    Idiopathic pulmonary hemosiderosis (IPH), a subtype of diffuse alveolar hemorrhage is a rare condition, first described by Virchow in 1864. Historically, it manifests in children in the first decade of life with the combination of hemoptysis, iron deficiency anemia, and alveolar infiltrates on chest radiograph. More recently, diffuse alveolar hemorrhage has been classified by the absence or presence of pulmonary capillaritis (PC), the latter carrying a potential for a poorer outcome. While systemic corticosteroids remain the first line treatment option, other immune modulators have been trailed including hydroxychloroquine, azathioprine, 6-mercaptopurine, and cyclophosphamide with varying results. Our case demonstrates for the first time, the successful use of intravenous cyclophosphamide in the management of chronic idiopathic PC.

  6. Chronic urticaria: new management options

    OpenAIRE

    Greenberger, Paul A.

    2014-01-01

    Chronic urticaria is defined as episodic or daily hives lasting for at least 6 weeks and impairs quality of life. Two main subtypes include chronic idiopathic (spontaneous) urticaria and inducible (physical) urticaria, but some patients have urticarial vasculitis. “Autoimmune chronic urticaria” implies the presence of histamine releasing or mast cell activating autoantibodies to IgE or FcϵRI, the high affinity receptor on mast cells and basophils. In patients not readily controlled with label...

  7. Thrombotic thrombocytopenic purpura-like syndrome associated with systemic lupus erythematosus--combined treatment with plasmapheresis and fresh frozen plasma infusion.

    OpenAIRE

    Lim, G. T.; Kim, S. S.; Park, S. H.; Choo, W. O.; Kang, D. H.; Park, I. S.; Chang, Y S; Y. S. YOON; Bang, B. K.

    1992-01-01

    We report on a patient with systemic lupus erythematosus, who, during the course of the illness, developed thrombotic thrombocytopenic purpura. In this case, the coexistence of these two conditions was confirmed by laboratory and pathologic findings. The infusion of fresh frozen plasma with plasmapheresis reversed the course of thrombotic thrombocytopenic purpura.

  8. Purine Bases in Blood Plasma of Patients with Chronic Pulmonary Diseases

    Directory of Open Access Journals (Sweden)

    Larissa E. Muravluyova

    2012-09-01

    Full Text Available The article is focused on the study of purine bases and intermediates of purine catabolism in plasma of patients with chronic obstructive bronchitis and idiopathic interstitial pneumonia. Decrease of adenine and hypoxantine in plasma of patients with idiopathic interstitial pneumonia was registered. Increase of guanine in plasma of patients with chronic obstructive pulmonary disease was established.

  9. Genetics in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Albers, Heleen Marion

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is a non-common disease in children that can persist into adulthood. JIA is considered to be an auto-immune disease. Genetic factors play a role in the pathogenesis. In a new cohort of JIA patients from North-West European descent genetic candidate gene associatio

  10. Analysis of Parallel and Transverse Visual Cues on the Gait of Individuals with Idiopathic Parkinson's Disease

    Science.gov (United States)

    de Melo Roiz, Roberta; Azevedo Cacho, Enio Walker; Cliquet, Alberto, Jr.; Barasnevicius Quagliato, Elizabeth Maria Aparecida

    2011-01-01

    Idiopathic Parkinson's disease (IPD) has been defined as a chronic progressive neurological disorder with characteristics that generate changes in gait pattern. Several studies have reported that appropriate external influences, such as visual or auditory cues may improve the gait pattern of patients with IPD. Therefore, the objective of this…

  11. Diagnosis and classification of juvenile idiopathic arthritis.

    Science.gov (United States)

    Eisenstein, Eli M; Berkun, Yackov

    2014-01-01

    In recent years, it has become increasingly clear that the term Juvenile Idiopathic Arthritis (JIA) comprises not one disease but several. Moreover, recent studies strongly suggest that some of these clinico-pathophysiologic entities appear to cross current diagnostic categories. The ultimate goal of the JIA classification is to facilitate development of better, more specific therapy for different forms of disease though improved understanding of pathophysiology. The past two decades have witnessed significant advances in treatment and improved outcomes for many children with chronic arthritis. However, understanding of the basic biologic processes underlying these diseases remains far from complete. As a result, even the best biologic agents of today represent "halfway technologies". Because they do not treat fundamental biologic processes, they are inherently expensive, need to be given for a long time in order to ameliorate the adverse effects of chronic inflammation, and do not cure the disease. Pediatric rheumatology is now entering an era in which diagnostic categories may need to change to keep up with discovery. A more precise, biologically based classification is likely to contribute to development of more specific and improved treatments for the various forms of childhood arthritis. In this review, we discuss how genetic, gene expression, and immunologic findings have begun to influence how these diseases are understood and classified.

  12. Idiopathic Inflammatory Myopathies: Clinical Approach and Management.

    Science.gov (United States)

    Malik, Asma; Hayat, Ghazala; Kalia, Junaid S; Guzman, Miguel A

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies. PMID:27242652

  13. Possible lower rate of chronic ITP after IVIG for acute childhood ITP an analysis from registry I of the Intercontinental Cooperative ITP Study Group (ICIS)

    NARCIS (Netherlands)

    Tamminga, Rienk; Berchtold, Willi; Bruin, Marrie; Buchanan, George R.; Kuehne, Thomas

    2009-01-01

    P>In children, one-third of immune thrombocytopenic purpura (ITP) patients follow a chronic course. The present study investigated whether treatment with intravenous immunoglobulin (IVIG) at the time of diagnosis of ITP is of prognostic significance, using data from 1984 children entered in Registry

  14. A cohort description and analysis of the effect of gabapentin on idiopathic cough

    OpenAIRE

    Van de Kerkhove, Charlotte; Goeminne, Pieter C; van Bleyenbergh, Pascal; Dupont, Lieven J.

    2012-01-01

    Background Chronic idiopathic cough (known as cough hypersensitivity syndrome) is defined by cough in the absence of an identifiable cause. Gabapentin has been suggested as a treatment but evidence is scarce. The aim of our study was to describe the clinical features of patients with unexplained chronic cough and to investigate the effect of gabapentin (600 mg twice a day for a minimal duration of 4 weeks) in reducing cough symptoms. Methods A patient cohort analysis was performed. Patients w...

  15. The absolute recommendation of chamber Neubauer method for platelets counting instead of indirect methods in severe thrombocytopenic patients

    Directory of Open Access Journals (Sweden)

    Oliveira Raimundo Antônio Gomes

    2003-01-01

    Full Text Available Accurate and precise platelet counting is crucial for recommending platelets transfusion for thrombocytopenic patients, principally when platelet counts are bellow 30,000/µl. As most laboratories still use the indirect methods for confirming low automated platelet counts, this work compared two indirect methods used in practice (Fonio and Nosanchunk et al. with the International Committee for Standardization in Hematology recommended direct method (Brecher and Cronkite. The obtained data show that the indirect methods present low precision and accuracy, and that the direct method should always be employed in severe thrombocytopenic samples thanks to its high precision.

  16. Thrombotic thrombocytopenic purpura and myoglobinuric acute renal failure following radiation therapy in a patient with polymyositis and cervical cancer

    International Nuclear Information System (INIS)

    A 73-year-old woman was admitted to receive radiation treatment for uterine cervical cancer, however a complex series of events ensued, leading to death. She developed an acute exacerbation of polymyositis complicated by thrombocytopenic purpura, rhabdomyolysis and acute renal failure. Radiation therapy may have produced an immune disturbance leading to the acute exacerbation of polymyositis. Auto-immune-mediated endothelial damage might have triggered a series of events leading to thrombotic thrombocytopenic purpura. Rhabdomyolysis seemed to be the main cause of acute renal failure. (author)

  17. Maternal anti-HLA class I antibodies are associated with reduced birth weight in thrombocytopenic neonates.

    Science.gov (United States)

    Dahl, J; Husebekk, A; Acharya, G; Flo, K; Stuge, T B; Skogen, B; Straume, B; Tiller, H

    2016-02-01

    In this comparative cross-sectional study, possible associations between maternal anti-HLA class I antibodies and birth weight in neonatal thrombocytopenia are explored. Although commonly detected in pregnancies and generally regarded as harmless, it has been suggested that such antibodies might be associated with fetal and neonatal alloimmune thrombocytopenia (FNAIT). As a link between FNAIT due to human platelet antigen 1a-specific antibodies and reduced birth weight in boys has previously been demonstrated, we wanted to explore whether maternal anti-HLA class I antibodies might also affect birth weight. To examine this, suspected cases of FNAIT referred to the Norwegian National Unit for Platelet Immunology during the period 1998-2009 were identified. Pregnancies where the only finding was maternal anti-HLA class I antibodies were included. An unselected group of pregnant women participating in a prospective study investigating maternal-fetal hemodynamics at the University Hospital North Norway during the years 2006-2010 served as controls. Twenty-nine percent of controls had anti-HLA class I antibodies. The thrombocytopenic neonates had a significantly lower adjusted birth weight (linear regression, P=0.036) and significantly higher odds of being small for gestational age (OR=6.72, P<0.001) compared with controls. Increasing anti-HLA class I antibody levels in the mother were significantly associated with lower birth weight and placental weight among thrombocytopenic neonates, but not among controls. These results indicate that maternal anti-HLA class I antibodies in thrombocytopenic neonates are associated with reduced fetal growth. Further studies are needed to test if placental function is affected.

  18. 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.

  19. Adult idiopathic scoliosis: the tethered spine.

    Science.gov (United States)

    Whyte Ferguson, Lucy

    2014-01-01

    This article reports on an observational and treatment study using three case histories to describe common patterns of muscle and fascial asymmetry in adults with idiopathic scoliosis (IS) who have significant scoliotic curvatures that were not surgically corrected and who have chronic pain. Rather than being located in the paraspinal muscles, the myofascial trigger points (TrPs) apparently responsible for the pain were located at some distance from the spine, yet referred pain to locations throughout the thoracolumbar spine. Asymmetries in these muscles appear to tether the spine in such a way that they contribute to scoliotic curvatures. Evaluation also showed that each of these individuals had major ligamentous laxity and this may also have contributed to development of scoliotic curvatures. Treatment focused on release of TrPs found to refer pain into the spine, release of related fascia, and correction of related joint dysfunction. Treatment resulted in substantial relief of longstanding chronic pain. Treatment thus validated the diagnostic hypothesis that myofascial and fascial asymmetries were to some extent responsible for pain in adults with significant scoliotic curvatures. Treatment of these patterns of TrPs and muscle and fascial asymmetries and related joint dysfunction was also effective in relieving pain in each of these individuals after they were injured in auto accidents. Treatment of myofascial TrPs and asymmetrical fascial tension along with treatment of accompanying joint dysfunction is proposed as an effective approach to treating both chronic and acute pain in adults with scoliosis that has not been surgically corrected. PMID:24411157

  20. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura:report of three cases

    Institute of Scientific and Technical Information of China (English)

    张文; 尤欣; 董怡

    2004-01-01

    @@ Systemic lupus erythematosus (SLE) is a multisystemic disease characterized by an autoimmune reaction.Thrombotic thrombocytopenic purpura (TTP) is a rare but severe syndrome with the manifestations of fever,thrombocytopenia, microangiopathic hemolysis,neurological symptoms, and renal involvement. The initial prognosis was reported to be dismal, 90% of patients dying within 3 months of onset. However, with the possibility of combined treatments, the survival rate has considerably improved. Data suggest a possible role of immune mechanisms in the development of TTP. 1,2We here report three cases of SLE complicated with TTP,and review the diagnosis, treatment, and outcome of SLE with TTP.

  1. CLINICAL FEATURES AND TREATMENT RESPONSE OF IMMUNE THROMBOCYTOPENIC PURPURA IN INFANTS

    Directory of Open Access Journals (Sweden)

    A. Ramyar N. Kalantari

    2007-09-01

    Full Text Available To determine the clinical features and treatment outcomes of infant with immune thrombo-cytopenic purpura (ITP. Retrospective analysis of 96 infant ITP patients treated from 1995 to 2005. The data abstracted comprised age, gender, clinical features, and treatment outcomes. The 56 male and 40 female infants had a median age of 3 months. Eighty presented with purpura, sixteen with active mucosal bleeding. The median platelet count was 13000 /l. Seventy-seven infants received intravenous immunoglobulin (IVIG, eighteen steroids and one patient was observed. Ninty-sex (96% responds to a single course of treatment. Infant with ITP respond favorably to treatment.

  2. Intravenous anti-D immunoglobulin in the treatment of resistant immune thrombocytopenic purpura in pregnancy.

    Science.gov (United States)

    Sieunarine, K; Shapiro, S; Al Obaidi, M J; Girling, J

    2007-04-01

    A 35-week pregnant 38-year-old woman presented with isolated thrombocytopenia (platelet count 4 x 10(9)/l). Investigations confirmed immune thrombocytopenic purpura, and she received treatment with prednisolone and intravenous immunoglobulins with no increment in the platelet count. At 37 and 38 weeks of the pregnancy, she received two doses of WinRho (anti-D immunoglobulin) at 50 microg/kg. Five days later, with a platelet count of 46 x 10(9)/l, she had an uncomplicated normal vaginal delivery. WinRho is a useful adjunct to other first-line treatment modalities for immune thrombocytopenia in pregnancy. PMID:17309547

  3. Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene

    DEFF Research Database (Denmark)

    Rank, Cecilie Utke; Kremer Hovinga, Johanna; Taleghani, Magnus Mansouri;

    2014-01-01

    , causing intravascular platelet clumping and thrombotic microangiopathy. Our patient, a 26-year-old man, had attacks of thrombotic thrombocytopenic purpura (TTP) with thrombocytopenia and a urine dipstick positive for hemoglobin (4+), often as the only sign of hemolytic activity. He had ADAMTS13 activity...... of A) leading to p.R1123H. This case report confirms the importance of the analysis of the ADAMTS13 activity and its inhibitor in patients who have episodes of TTP, with a very low platelet count and sometimes without the classic biochemical signs of hemolysis....

  4. Exudative Retinal Detachment Treatment in a Patient with Thrombotic Thrombocytopenic Purpura

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    Magali Sampo

    2016-02-01

    Full Text Available Purpose: We report a case of unilateral exudative retinal detachment in a patient with thrombotic thrombocytopenic purpura (TTP, without associated hypertension, successfully treated with plasmapheresis. Case Report: A 46-year-old woman with a medical history of TTP presented with unilateral exudative retinal detachment. Biological and radiological assessment eliminated other causes of exudative retinal detachment, including hypertension. Plasma exchange was performed, followed by a rapid improvement in visual acuity and total disappearance of serous detachment. Conclusion: Exudative unilateral retinal detachment is a rare complication of TTP and can be successfully treated by plasma exchange.

  5. Difficult Management of Coronary Artery Disease in a Patient with Thrombotic Thrombocytopenic Purpura

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    Fatemeh Jorfi

    2015-10-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a rare syndrome affecting multiple organs. There is no sufficient evidence regarding the clinical cardiac manifestations of TTP. Nonetheless, pathologic cardiac involvement is quite frequent in acute TTP, which is predominantly manifested as myocardial necrosis due to coronary arteriolar microthrombosis. The present case report describes a 43-year-old man with long-standing remitted TTP, who suffered from a sequence of refractory thrombotic epicardial coronary events. Aggressive medical and interventional therapies, including long-term dual antiplatelets and coronary angioplasty, were finally successful in remitting the thrombotic events. During his two-year follow up, he has been asymptomatic.

  6. ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura

    OpenAIRE

    Tsai, Han-Mou; Raoufi, Mojgan; Zhou, Wenhua; Guinto, Enriqueta; Grafos, Nickolas; Ranzurmal, Safi; Greenfield, Robert S.; Rand, Jacob H.

    2006-01-01

    Functional assays are commonly used to measure the antibodies of ADAMTS13 found in patients of thrombotic thrombocytopenic purpura (TTP). In this study we used an enzyme-linked immunoassay to analyze the ADAMTS13-binding IgG levels in six groups of individuals: normal, random hospitalized patients, acute TTP,TTP after receiving plasma therapy, TTP in remission, and other types of thrombotic microangiopathy (TMA). The results showed thatADAMTS13-binding IgG levels were elevated in 100% of the ...

  7. ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura

    OpenAIRE

    Manea, Minola; Kristoffersson, AnnCharlotte; Tsai, Han-Mou; Zhou, Wenhua; Winqvist, Ingemar; Oldaeus, Göran; Billström, Rolf; Björk, Peter; Holmberg, Lars; Karpman, Diana

    2006-01-01

    The activity of ADAMTS13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n = 20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS13 was not detected in the plasma fro...

  8. Immune thrombocytopenic purpura in ulcerative colitis: a case report and systematic review

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    Subhash Chandra

    2014-04-01

    Full Text Available Over 100 extraintestinal manifestations are reported in ulcerative colitis (UC. A commonly reported hematological manifestation is autoimmune hemolytic anemia. On rare occasions, immune thrombocytopenic purpura (ITP has been reported with UC. The presence of thrombocytopenia can complicate the clinical scenario as the number of bloody bowel movements is an important indicator of disease activity in UC. A proposed theory for this association is antigenic mimicry between a platelet surface antigen and bacterial glycoprotein. We are reporting a case of UC and associated ITP managed successfully with anti-TNF therapy. We also performed a systemic review of case reports and a case series reporting this association.

  9. [Juvenil idiopathic arthritis. Part 1: diagnosis, pathogenesis and clinical manifestations].

    Science.gov (United States)

    Espada, Graciela

    2009-10-01

    Juvenile idiopathic arthritis is not a single disease and constitutes an heterogeneous group of illnesses or inflammatory disorders. This new nomenclature encompasses different disease categories, each of which has different presentation, clinical signs, symptoms, and outcome. The cause of the disease is still unknown but both environmental and genetic factors seem to be related to its pathogenesis. Is the most common chronic rheumatic disease in children and an important cause of short-term and long-term disability. In this article, clinical manifestation, new classification and approach to diagnosis are reviewed.

  10. Sympathovagal balance analysis in idiopathic postural orthostatic tachycardia syndrome.

    Science.gov (United States)

    Russo, Vincenzo; De Crescenzo, Ilaria; Ammendola, Ernesto; Santangelo, Lucio; Calabrò, Raffaele

    2007-08-01

    The idiopathic postural tachycardia syndrome (POTS) is a complex disorder characterized by chronic orthostatic symptoms and an increase in heart rate within 10 minutes of standing on upright posture, without significant orthostatic hypotension. We describe a case of a 36 year-old patient with POTS, diagnosed by head-up tilt testing. Power spectral analysis of heart rate variability (HRV), performed during the tilt test, revealed the ratio of low and high frequency powers (LF/HF) that increased with the onset of orthostatic intolerance. The increase in LF/HF power ratio may represent sympathetic beta-receptors hyperactivity. Atenolol alleviated his clinical symptoms.

  11. The changing treatment landscape in idiopathic pulmonary fibrosis

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    Ulrich Costabel

    2015-03-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF is a chronic, progressive and irreversible fibrotic disease of the lung that has greatly frustrated clinicians for a long time. The prognosis of IPF (median survival 2–5 years following diagnosis is poorer than that of some cancers and for many years no significant advances were made in its management. However, between 2011 and 2014 a number of pivotal developments were made that have improved the outlook for patients with IPF. Herein, we review this rapidly changing landscape, discussing key events whilst still acknowledging that IPF remains a challenging disease to diagnose and manage.

  12. Resistin in idiopathic inflammatory myopathies

    OpenAIRE

    Filkova, Maria; Hulejova, Hana; Kuncova, Klara; Plestilova, Lenka; Andres Cerezo, Lucie; Mann, Herman; Klein, Martin; Zamecnik, Josef; Gay, Steffen; Vencovsky, Jiri; Senolt, Ladislav

    2012-01-01

    Introduction The purpose of this study was to evaluate and compare the serum levels and local expression of resistin in patients with idiopathic inflammatory myopathies to controls, and to determine the relationship between resistin levels, inflammation and disease activity. Methods Serum resistin levels were determined in 42 patients with inflammatory myopathies and 27 healthy controls. The association among resistin levels, inflammation, global disease activity and muscle strength was exami...

  13. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or a...... antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH....

  14. Idiopathic epilepsy and school achievement.

    OpenAIRE

    Sturniolo, M G; Galletti, F

    1994-01-01

    Forty one children (20 boys, 21 girls) aged 6-10.8 years (mean age 8.6 years) who were affected with idiopathic epilepsy underwent neuropsychological (Wechsler Intelligence Scale for Children, Bender test) and behavioural assessment (Personality Inventory for Children; this was also used in a matched control group). Further information was obtained by teachers' reports. School underachievement occurred in 25 children (61%). Statistical analysis showed no influence of sex, social background, a...

  15. [Chronic inflammatory demyelinating polyradiculoneuropathy].

    Science.gov (United States)

    Franques, J; Azulay, J-P; Pouget, J; Attarian, S

    2010-06-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a demyelinating chronic neuropathy of immune origin whose diagnosis is based upon clinical, biological and electrophysiological data; previously critical to the diagnosis the nerve biopsy is now restricted to the rare situations where accurate diagnosis cannot be reached using these data alone. CIDP are mainly idiopathic, but a few associated diseases must be sought for as they require specific attention. Such associated diseases must particularly be discussed when the manifestations are severe or resistant to immunomodulating or immunosuppressive agents. Indeed, idiopathic CIDP are usually responsive to these treatments. The effectiveness of these treatments is limited by the importance of the secondary axonal loss. The dependence or the resistance may sometimes justify the association of several immunomodulating treatments. A single randomized controlled trial support the use of cytotoxic drugs and none with rituximab.

  16. Gastaut type idiopathic occipital epilepsy

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    I. V. Volkov

    2015-01-01

    Full Text Available Idiopathic occipital epilepsy is a rare epileptic syndrome. Its incidence in a Novosibirsk cohort of patients with idiopathic focal epilepsy is 0.9%. Objective: to present a clinical description of new cases of Gastaut syndrome, the types of its course, and treatment options in these patients. Patients and methods. The study covers 17 cases of Gastaut type idiopathic occipital epilepsy in 13 women and 4 men aged 11–53 years. Results. Among 17 cases we present 4 family cases with the disease. Three generations in 2 families were observed to have epilepsy, including Gastaut syndrome concurrent with childhood absence epilepsy. The adolescent onset of the disease was seen in most cases. Its main symptoms were focal visual seizures (100%, focal sensory seizures (58.9%, cephalalgia (47.1%, speech disorders (41.2%, and secondarily generalized convulsive seizures (35.3%. According to the frequency of seizures, the investigators identified 5 types of the course: single focal seizures, rare focal seizures with or without convulsions, frequent focal seizures with or without convulsions. The identity of the course of epilepsy was found in familial cases. 76.5% of the patients had a good quality of life: 41.2% of them were untreated while 35.3% were treated; no seizures were noted. 

  17. Thrombotic thrombocytopenic purpura after allogeneic stem cell transplantation : a survey of the European Group for Blood and Marrow Transplantation (EBMT)

    NARCIS (Netherlands)

    Ruutu, T; Hermans, J; Niederwieser, D; Gratwohl, A; Kiehl, M; Volin, L; Bertz, H; Ljungman, P; Spence, D; Verdonck, LF; Prentice, HG; Bosi, A; du Toit, CE; Brinch, L; Apperley, JF

    2002-01-01

    A survey was carried out among the European Group for Blood and Marrow Transplantation (EBMT) centres to determine the incidence, risk factors, treatment and outcome of thrombotic thrombocytopenic purpura (TTP) following allogeneic haematopoietic stem cell transplantation. TTP was defined as the sim

  18. 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.

  19. Idiopathic inflammatory myopathies and the lung

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    Jean-Christophe Lega

    2015-06-01

    Full Text Available Idiopathic inflammatory myositis (IIM is a group of rare connective tissue diseases (CTDs characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD.

  20. [Optic neuritis in juvenile idiopathic arthritis patient].

    Science.gov (United States)

    Lourenço, Daniela M R; Buscatti, Izabel M; Lourenço, Benito; Monti, Fernanda C; Paz, José Albino; Silva, Clovis A

    2014-01-01

    Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5,793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required.

  1. Report - Recurrent hip arthritis diagnosed as juvenile idiopathic arthritis: A case report.

    Science.gov (United States)

    Chang, Tung-Ming; Yang, Kuender D; Yong, Su-Boon

    2016-05-01

    Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. It is a chronic inflammatory disease associated with arthritis of unknown etiology that begins before the age of 16 and persists for longer than 6 weeks. In this report, the case of a child who suffered recurrent alternative hip arthritis with bilateral hip arthritis is examined, in which he was finally diagnosed as suffering from Juvenile idiopathic arthritis. A 14-year-old boy of Taiwanese origin presented with a normal birth and developmental history. At the age of 10, right-side hip joint pain was experienced, which later migrated to the left side. On further inspection, synovium hypertrophy, cartilage erosion and hip turbid fluid accumulation were found and aseptic arthritis was presumed to be the primary cause. However, after re-examining both his clinical history and presentation, Juvenile idiopathic arthritis was the final diagnosis. Any child presenting with repeat joint swelling are at risk of Juvenile idiopathic arthritis. This is still to be the case if symptoms recede or heal and no initial diagnosis is made. Therefore, a better understanding of the risk of recurrent arthritis is needed. It cannot be emphasized strongly enough that Juvenile idiopathic arthritis should be suspected at all times when a child suffers from recurrent aseptic arthritis of the hip joint.

  2. Ultrastructural differences between diabetic and idiopathic gastroparesis

    NARCIS (Netherlands)

    Faussone-Pellegrini, Maria Simonetta; Grover, Madhusudan; Pasricha, Pankaj J.; Bernard, Cheryl E.; Lurken, Matthew S.; Smyrk, Thomas C.; Parkman, Henry P.; Abell, Thomas L.; Snape, William J.; Hasler, William L.; Uenalp-Arida, Aynur; Nguyen, Linda; Koch, Kenneth L.; Calles, Jorges; Lee, Linda; Tonascia, James; Hamilton, Frank A.; Farrugia, Gianrico

    2012-01-01

    The ultrastructural changes in diabetic and idiopathic gastroparesis are not well studied and it is not known whether there are different defects in the two disorders. As part of the Gastroparesis Clinical Research Consortium, full thickness gastric body biopsies from 20 diabetic and 20 idiopathic g

  3. Diagnostic dilemmas in chronic urticaria.

    Science.gov (United States)

    Toubi, E; Grattan, C; Zuberbier, T

    2015-06-01

    The European Academy of Allergy and Clinical Immunology (EAACI)/Global Allergy and Asthma European Network (GA(2) LEN)/European Dermatology Forum (EDF)/World Allergy Organization (WAO) recently published updated recommendations for the classification, diagnosis and management of chronic urticaria (CU). This article discusses several cases of CU that provide examples of how the recommendations in the guidelines can be implemented in the diagnosis of chronic spontaneous urticaria (CSU) (also called chronic idiopathic urticaria [CIU]), chronic inducible urticaria (CINDU) or CU with comorbidities.

  4. Practical management of Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Kishaba, Tomoo

    2015-01-01

    Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease. PMID:26278687

  5. Chronic urticaria: recent advances.

    Science.gov (United States)

    Greaves, Malcolm W; Tan, Kian Teo

    2007-10-01

    Chronic urticaria is an umbrella term, which encompasses physical urticarias, chronic "idiopathic" urticaria and urticarial vasculitis. It is important to recognize patients with physical urticarias as the investigation and treatment differs in important ways from patients with idiopathic chronic urticaria or urticarial vasculitis. Although relatively uncommon, urticarial vasculitis is an important diagnosis to make and requires histological confirmation by biopsy. Underlying systemic disease and systemic involvement, especially of the kidneys, should be sought. It is now recognized that chronic "idiopathic" urticaria includes a subset with an autoimmune basis caused by circulating autoantibodies against the high affinity IgE receptor (FceR1) and less commonly against IgE. Although the autologous serum skin test has been proven useful in prompting search for and characterization of circulating wheal-producing factors in chronic urticaria, its specificity as a screening test for presence of functional anti-FceR1 is low, and confirmation by demonstration of histamine-releasing activity in the patient's serum must be the benchmark test in establishing this diagnosis. Improved screening tests are being sought; for example, ability of the chronic urticaria patient's serum to evoke expression of CD 203c on donor human basophils is showing some promise. The strong association between autoimmune thyroid disease and autoimmune urticaria is also an area of ongoing research. Drug treatment continues to be centered on the H1 antihistamines, and the newer second-generation compounds appear to be safe and effective even in off-label dosage. Use of systemic steroids should be confined to special circumstances such as tapering regimens for acute flare-ups. Use of leukotriene antagonists is becoming popular, but the evidence for efficacy is conflicting. Cyclosporin is also effective and can be used in selected cases of autoimmune urticaria, and it is also effective in non

  6. Idiopathic Adulthood Ductopenia: ‘It Is Out There’

    Science.gov (United States)

    Bilal, Mohammad; Kazemi, Ali; Babich, Michael

    2016-01-01

    Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic entity of unknown origin characterized by loss of inter-lobular bile ducts that was first described two decades ago. Although the diagnostic criteria have been described in detail, IAD continues to be a rare diagnosis. Our thorough literature search revealed less than a hundred cases of IAD reported. Here we present a 34-year-old female with no significant past history who was evaluated for persistent elevation of serum alkaline phosphate levels. Serology was negative for all viral hepatitides, and a chronic liver disease workup was unremarkable. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography did not reveal any abnormalities in the biliary tree. Finally, a liver biopsy demonstrated ductopenia involving greater than 50% of the portal triads, making a diagnosis of IAD. Since the disease can progress rapidly, close follow-up is warranted, so liver transplantation can be pursued if deemed necessary. PMID:27403109

  7. Treatment of uveitis associated with juvenile idiopathic arthritis.

    Science.gov (United States)

    Bou, Rosa; Iglesias, Estíbaliz; Antón, Jordi

    2014-08-01

    Chronic anterior uveitis affects 10-30 % of patients with juvenile idiopathic arthritis (JIA) and is still a cause of blindness in childhood. In most patients it is asymptomatic, bilateral, and recurrent, so careful screening and early diagnosis are important to obtain the best long-term prognosis. The treatment of chronic uveitis associated with JIA is challenging. Initial treatment is based on topical steroids and mydriatic drops. Methotrexate is the most common first-line immunomodulatory drug used. For refractory patients, biologicals, mainly the anti-tumor-necrosis-factor (TNF) drugs adalimumab and infliximab, have been revealed to be effective and have changed the outcome for these patients. Collaboration between pediatric rheumatologists and ophthalmologists is important for the successful diagnosis and treatment of patients with uveitis associated with JIA. PMID:24938442

  8. Imaging of juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, Karl [Birmingham Children' s Hospital, Radiology Department, Birmingham (United Kingdom)

    2006-08-15

    Over the past decade there have been considerable changes in the classification and imaging of juvenile idiopathic arthritis (JIA). Radiology now has a considerable role in the management of JIA, the differential diagnosis, monitoring disease progression and detecting complications. The different imaging modalities available, their role and limitations are discussed in this article and the various disease features that the radiologist should be aware of are described. An approach to the imaging of the child with joint disease and in the monitoring of disease complications are also discussed. (orig.)

  9. [Chronic inflammatory bowel diseases in cats].

    Science.gov (United States)

    Ghermai, A K

    1989-01-01

    The aetiology of chronic idiopathic intestinal inflammation is unknown. It is characterized by a diffuse infiltration with inflammatory cells into the intestinal mucosa and sometimes submucosa. Cats with chronic intermittent vomiting and diarrhoea, later on accompanied by anorexia and weight loss, are presented. Definitive diagnosis can be obtained by intestinal biopsy only. An immune pathogenesis is suspected, which is supported by the fact, that chronic inflammatory bowel disease responds to steroid therapy.

  10. Qualitative Dermatoglyphics In Idiopathic Epilepsy

    Directory of Open Access Journals (Sweden)

    Ranganath Priya

    2004-01-01

    Full Text Available Genetic aetiology has been proposed for both idiopathic epilepsy and dermatoglyphics. Hence, the present study has been undertaken to find out the existence of any correlation between dermatoglyphics and idiopathic epilepsy. Material consisted of 100 patients (58 males and 42 females and 100 controls (52 males and 48 females. Patient′s age ranged from 5 to 40 years and controls were between 18 and 25 years. Dermatoglyphics were obtained by painting method. Qualitative parameters observed were percentage frequency of fingerprint patterns (loops, whorls and arches,, patterns in hypothenar area/ interdigital are and flexion creases (Simian crease, sydney line. On comparison with controls, in males, with hands combined, loops (52.24% and arches (7.93% were increased and whorls (39.83% were decreased (p<0.05. In females, with hands combined, arches (13.1% and whorls (36.43% were increased and loops (50.48% were decreased (p< 0.03. Significant differences have not been observed for the patterns in hypothenar area /interdigital area and flexion creases. These dermatoglyphics features could be used as additional markers to evaluate patients of epilepsy.

  11. Idiopathic epilepsy and school achievement.

    Science.gov (United States)

    Sturniolo, M G; Galletti, F

    1994-05-01

    Forty one children (20 boys, 21 girls) aged 6-10.8 years (mean age 8.6 years) who were affected with idiopathic epilepsy underwent neuropsychological (Wechsler Intelligence Scale for Children, Bender test) and behavioural assessment (Personality Inventory for Children; this was also used in a matched control group). Further information was obtained by teachers' reports. School underachievement occurred in 25 children (61%). Statistical analysis showed no influence of sex, social background, age of onset, seizure type, duration of illness, features seen on electroencephalography, and treatment. School failure was due to poor performance in almost all academic fields, and was associated with higher visuomotor impairment; children showing good school performance had a higher mean IQ and less visuomotor impairment. The behaviour of children with epilepsy who had a good academic performance did not differ from that of their healthy peers. Emotional maladjustment (social skill impairment, depression, poor motivation, and low self esteem) was associated with poor school performance. Such problems, that may complicate the course of idiopathic epilepsy and require an appropriate educational programme, should be carefully considered by the clinician. PMID:8017966

  12. Posterior asymmetry and idiopathic scoliosis

    CERN Document Server

    Rousie, D L; Berthoz, A

    2009-01-01

    Study design Are there neuro-anatomical abnormalities associated with idiopathic scoliosis (IS)? Posterior Basicranium (PBA) reflects cerebellum growth and contains vestibular organs, two structures suspected to be involved in scoliosis. Objective The aim of this study was to compare posterior basicranium asymmetry (PBA) in Idiopathic scoliosis (IS) and normal subjects. Method: To measure the shape of PBA in 3D, we defined an intra-cranial frame of reference based on CNS and guided by embryology of the neural tube. Measurements concerned three directions of space referred to a specific intra cranial referential. Data acquisition was performed with T2 MRI (G.E. Excite 1.5T, mode Fiesta). We explored a scoliosis group of 76 women and 20 men with a mean age of 17, 2 and a control group of 26 women and 16 men, with a mean age of 27, 7. Results: IS revealed a significant asymmetry of PBA (Pr>|t|<.0001) in 3 directions of space compared to the control group. This asymmetry was more pronounced in antero-posterior...

  13. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

    Science.gov (United States)

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population. PMID:27383202

  14. Neglect-induced pseudo-thrombotic thrombocytopenic purpura due to vitamin B12 deficiency.

    Science.gov (United States)

    Asano, Takeshi; Narazaki, Hidehiko; Kaizu, Kiyohiko; Matsukawa, Shouhei; Takema-Tochikubo, Yuki; Fujii, Shuichi; Saitoh, Nobuyuki; Mashiko, Kunihiko; Fujino, Osamu

    2015-10-01

    Although thrombotic thrombocytopenic purpura (TTP) is rare, early diagnosis and treatment are important for decreasing the mortality rate. Acquired vitamin B12 deficiency is frequently overlooked because of its rarity in developed countries, particularly in children and adolescents. The hematological changes in vitamin B12 deficiency present as megaloblastic anemia, increased lactate dehydrogenase, vasoconstriction, increased platelet aggregation, and abnormal activation of the coagulation followed by microangiopathy as well as neutropenia and thrombocytopenia. We report herein the case of a 15-year-old girl who had been neglected, which might have caused pseudo-TTP through malnutrition, particularly vitamin B12 deficiency. When we encounter cases of TTP in children, clinicians must be aware of the possibility of malnutrition, particularly with vitamin B12 deficiency, even in developed countries, and investigate the cause of malnutrition including neglect. PMID:26387768

  15. Clopidogrel-induced refractory thrombotic thrombocytopenic purpura successfully treated with rituximab.

    Science.gov (United States)

    Khodor, Sara; Castro, Miguel; McNamara, Colin; Chaulagain, Chakra P

    2016-06-01

    Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by microvascular aggregation of platelets and fibrin strands causing thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction. TTP can develop as a result of a deficiency in ADAMTS13 enzyme activity due to either a genetic defect or, more commonly, the development of anti-ADAMTS13 autoantibodies. TTP can also be associated with pregnancy, organ transplant, lupus, infections, and drugs. Here, we present a case of TTP that developed shortly after the start of clopidogrel treatment for acute ischemic stroke and acute myocardial infarction, and describe the clinical presentation, refractory course of the disease, and successful induction of remission through the use of rituximab in a setting of pre-existing autoimmune diseases. PMID:26684918

  16. Idiopathic subglottic stenosis: a familial predisposition.

    Science.gov (United States)

    Dumoulin, Elaine; Stather, David R; Gelfand, Gary; Maranda, Bruno; Maceachern, Paul; Tremblay, Alain

    2013-03-01

    Idiopathic subglottic stenosis is a narrowing of the trachea at the level of the cricoid cartilage of unknown etiology. It is a rare condition for which the real incidence has never been established owing to the difficulty of making the diagnosis. Although there is a female preponderance, no familial cases have been reported in the literature. We describe two pairs of sisters as well as a mother and daughter presenting with idiopathic subglottic stenosis. All known causes of tracheal stenosis were excluded, including prolonged intubation, surgery, autoimmune and inflammatory disorders, infection and gastroesophageal reflux disease. These are the first cases reported in the literature that suggest a genetic predisposition for idiopathic subglottic stenosis.

  17. Scintigraphic measurement of colonic transit in patients with idiopathic constipation

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, K. H.; Kim, C. K. [College of Medicine, Wonkwang Univ., Iksan (Korea, Republic of)

    1999-07-01

    To evaluate usefulness of scintigraphic measurement of total and regional colonic transit in patients with idiopathic constipation. 25 patients who were complained chronic constipation underwent scintigraphic measurement of the total and regional colon transit. Of them 10 patients were diagnosed as idiopathic constipation, none of whom had evidence of abnormal function of the pelvic floor. Ten healthy volunteers were also studied. 67 Gallium-labelled Amberlite resin particles were ingested in a coated capsule with methacrylate that dispersed in the ileocecal region. Images were obtained using a gamma camera at regular intervals for the 2, 4, 8, 24, and 48 hours after the initial counting of the radioactivity in the cecum. We determined the geometric center in four regions of interest in the colon (ascending, transverse, descending, and rectosigmoid). Ten patients with colonic inertia showed significant retention of solid residue in the ascending and transverse colon over a 48-hour period. The median values for the healthy subjects at 2, 4, 8, and 24, and 48 hours were 1.44{+-}0.2 (midway through ascending), 1.71{+-}0.45 (midway through transverse), 2.64{+-}0.95 (midway through descending), 3.94{+-}0.89 (midway through rectosigmoid), and 4.52{+-}0.76 (midway through the stool compartment). On the contrary, the values of ten patients with colonic inertia were 1.0{+-}0.0 (midway through ascending), 1.0{+-}0.0 (midway through ascending). 1.02 {+-}0.06 (midway through ascending), 1.70{+-}0.36 (midway through transverse), and 2.33{+-}0.31(midway through descending) at the same time (p<0.001). In patients with idiopathic constipation is characterized by exaggerated reservoir factions of the ascending and transverse colons. Scintigraphy using 67 Gallium-labelled pellets seems to be a useful tool to demonstrate the delayed colonic transit in patients with colonic inertia.

  18. Association of Celiac Disease With Idiopathic Pulmonary Hemosiderosis; Lane Hamilton Syndrome

    OpenAIRE

    Hikmet Tekin Nacaroglu; Ozlem Sarac Sandal; Ozlem Bag; Semiha Bahceci Erdem; Ozlem Bekem Soylu; Gulden Diniz; Aysel Ozturk; Demet Can

    2015-01-01

    Introduction: Idiopathic Pulmonary Hemosiderosis (IPH) is a rare cause of alveolar hemorrhage, which is seen primarily in childhood. Celiac disease is defined as a chronic, immune-mediated enteropathy of the small intestine, caused by exposure to dietary gluten in genetically pre-disposed individuals. Association of IPH and celiac disease is known as Lane Hamilton syndrome. There are limited number of case reports of this syndrome in literature. ...

  19. Mechanisms of neutrophil accumulation in the lungs of patients with idiopathic pulmonary fibrosis.

    OpenAIRE

    Hunninghake, G W; Gadek, J E; Lawley, T J; Crystal, R G

    1981-01-01

    Neutrophils are a characteristic feature of the alveolitis of idiopathic pulmonary fibrosis (IPF). a chronic disorder limited to lung. One mechanism by which neutrophils may be selectively attracted to lung and not other tissues is via the secretion of the neutrophil-specific chemotactic factor by alveolar macrophages. To evaluate the role of alveolar macrophages in modulating the migration of neutrophils to he lung in IPF, alveolar macrophages, obtained by bronchoalveolar lavage of patients ...

  20. Seroprevalence of parvovirus B19 IgG in children affected by juvenile idiopathic arthritis

    OpenAIRE

    Weissbrich, Benedikt; Süß-Fröhlich, Yvonne; Girschick, Hermann J.

    2007-01-01

    Parvovirus (PV) B19 is the causative agent of the childhood disease erythema infectiosum. An association of PV B19 with chronic arthropathies, sometimes resembling rheumatoid arthritis or juvenile idiopathic arthritis (JIA), has repeatedly been described. Other studies, however, have failed to identify any such relationship. In order to study further whether there is a link between PV B19 and JIA, we determined the prevalence of PV B19 specific IgG antibodies in serum samples from children wi...

  1. Raised serum levels of IGFBP-1 and IGFBP-2 in idiopathic pulmonary fibrosis

    OpenAIRE

    Guiot, Julien; Bondue, B.; HENKET, MONIQUE; CORHAY, JEAN-LOUIS; Louis, Renaud

    2016-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is a chronic lung disorder of unknown origin, which ultimately leads to death. Several growth factors such as IGFs (insulin-like-growth factor) and IGFBPs (insulin like growth factor binding proteins) seem to take part to the pathogenesis. We evaluated IGFs and IGFBPs in serum from patients with IPF and healthy subjects including 24 untreated IPF and 26 IPF receiving anti-fibrotic therapy and to compare them with healthy subjects. Methods Serum o...

  2. Complementary and alternative medicine use in adolescents with inflammatory bowel disease and juvenile idiopathic arthritis

    OpenAIRE

    Nousiainen, Pauliina; Merras-Salmio, Laura; Aalto, Kristiina; Kolho, Kaija-Leena

    2014-01-01

    Background The use of complementary alternative medicine (CAM) is potentially prevalent among paediatric patients with chronic diseases but with variable rates among different age groups, diseases and countries. There are no recent reports on CAM use among paediatric patients with inflammatory bowel disease (IBD) and juvenile idiopathic arthritis (JIA) in Europe. We hypothesized that CAM use associates with a more severe disease in paediatric IBD and JIA. Methods A cross-sectional questionnai...

  3. Lung and chest wall mechanics in ventilated patients with end stage idiopathic pulmonary fibrosis

    OpenAIRE

    Nava, S.; Rubini, F.

    1999-01-01

    BACKGROUND—Idiopathic pulmonary fibrosis is an inflammatory disease which leads to chronic ventilatory insufficiency and is characterised by a reduction in pulmonary static and dynamic volumes. It has been suggested that lung elastance may also be abnormally increased, particularly in end stage disease, but this has not been systematically tested. The aim of this study was to assess the respiratory mechanics during mechanical ventilation in patients affected by end stage ...

  4. The Fecal Microbiome in Dogs with Acute Diarrhea and Idiopathic Inflammatory Bowel Disease

    OpenAIRE

    Jan S. Suchodolski; Melissa E Markel; Garcia-Mazcorro, Jose F.; Unterer, Stefan; Heilmann, Romy M.; Scot E Dowd; Kachroo, Priyanka; Ivanov, Ivan; Minamoto, Yasushi; Dillman, Enricka M.; Steiner, Jörg M.; Cook, Audrey K.; Toresson, Linda

    2012-01-01

    Background Recent molecular studies have revealed a highly complex bacterial assembly in the canine intestinal tract. There is mounting evidence that microbes play an important role in the pathogenesis of acute and chronic enteropathies of dogs, including idiopathic inflammatory bowel disease (IBD). The aim of this study was to characterize the bacterial microbiota in dogs with various gastrointestinal disorders. Methodology/Principal Findings Fecal samples from healthy dogs (n = 32), dogs wi...

  5. Lung cancer in idiopathic pulmonary fibrosis patients diagnosed during or after lung transplantation ☆

    OpenAIRE

    Hendriks, L.E.L.; Drent, M.; Haren, E.H.J. van; Verschakelen, J A; Verleden, G M

    2012-01-01

    Lung transplantation is an accepted therapy for patients with end-stage lung disease and offers a major survival benefit in selected patients. The most important indications are chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis besides cystic fibrosis and pulmonary arterial hypertension. The incidence of lung cancer in patients after Ltx is 20–25 times higher than in the general population. Diagnosis is often difficult in IPF patients because of the diffuse lung abnormal...

  6. The Etiology of Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Rigante, Donato; Bosco, Annalisa; Esposito, Susanna

    2015-10-01

    Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between environmental factors and multiple genes has been proposed as the most relevant working mechanism to the development of JIA. The concept that various microbes that colonize or infect not only the mucosal surfaces, like the oral cavity, but also the airways and gut might trigger autoimmune processes, resulting in chronic arthritides, and JIA was first drafted at the outset of last century. JIA development might be initiated and sustained by the exposure to environmental factors, including infectious agents which affect people at a young age, depending on the underlying genetic predisposition to synovial inflammation. Many data from patients with JIA suggest a scenario in which different external antigens incite multiple antigen-specific pathways, cytotoxic T cell responses, activation of classical complement cascade, and production of proinflammatory cytokines. In this review, emphasis is paid not only to the potential role of parvovirus B19 and Epstein-Barr virus in primis but also to the general involvement of different bacteria as Salmonella spp., Shigella spp., Campylobacter spp., Mycoplasma pneumoniae, Chlamydophila pneumoniae, Bartonella henselae, and Streptococcus pyogenes for the development of immune-mediated arthritides during childhood. No unequivocal evidence favoring or refuting these associations has been clearly proved, and today, the strict definition of JIA etiology remains unknown. The infection can represent a random event in a susceptible individual, or it can be a necessary factor in JIA development, always in combination with a peculiar genetic background. Further studies are needed in order to address the unsolved questions

  7. Nonchylous idiopathic pleural effusion in the newborn

    Directory of Open Access Journals (Sweden)

    Geeta Gathwala

    2011-01-01

    Full Text Available Congenital isolated pleural effusion is a rare cause of respiratory distress in neonates. It is usually chylous. Herein, we report a rare case of nonchylous congenital idiopathic pleural effusion.

  8. Idiopathic inflammatory myopathies: diagnosis, treatment and outcome

    NARCIS (Netherlands)

    J. van de Vlekkert

    2015-01-01

    The idiopathic inflammatory myopathies (IIM) in adults are a heterogenic group of disorders characterized by muscle inflammation and progressive muscle weakness. This group consists of five subacute-onset disorders: polymyositis (PM) which is extremely rare, (clinically amyopathic) dermatomyositis (

  9. Idiopathic Organ Transplant Chorioretinopathy after Liver Transplantation

    OpenAIRE

    Maria Fernanda Abalem; Pedro Carlos Carricondo; Sergio Luis Gianotti Pimentel; Walter Yukihiko Takahashi

    2015-01-01

    Idiopathic organ transplant chorioretinopathy is a rare disease associated with kidney and heart transplantation. We present a case secondary to liver transplantation including its multimodal imaging, differential diagnosis, and physiopathology discussion.

  10. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  11. Idiopathic Inflammatory Myopathies: An update

    Directory of Open Access Journals (Sweden)

    Bulent KURT

    2016-06-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are a heterogeneous group of disease with complex clinical features. It has been sub-classified as: (1 Dermatomyositis, (2 Polymyositis, and (3 Inclusion body myositis (IBM. Nowadays, there are some studies in literature suggest necrotizing autoimmune myopathy and immune-mediated necrotizing myopathy should also be added to this group of disease. There is a debate in the diagnosis of IIMs and up until now, about 12 criteria systems have been proposed. Some of the criteria systems have been used widely such as Griggs et al.'s proposal for IBM. Clinical findings, autoantibodies, enzymes, electrophysiological, and muscle biopsy findings are diagnostic tools. Because of diseases' complexity, none of the findings are diagnostic alone. In this study, we discussed the diagnostic criteria of IMMs and described detailed morphological features. [J Interdiscipl Histopathol 2016; 4(2.000: 41-45

  12. Idiopathic arterial calcification in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Patel, Maya [Department of Paediatric Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Red Cross Children' s Hospital, School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch, Cape Town (South Africa); Andronikou, Savvas; Solomon, Rustum; Sinclair, Paul; McCulloch, Mignon [Department of Paediatric Radiology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2004-08-01

    Idiopathic arterial calcification in infancy is usually fatal with death in early life and diagnosis at post mortem. This report describes a unique, late presentation with hypertension and cardiac failure in a child aged 33 months, found to have widespread arterial calcification at radiological imaging. The calcium-phosphate axis was normal and there was no other demonstrable cause for calcification. Additionally, the histological features of arterial calcification at renal biopsy paralleled the findings in infants with this disorder. The late presentation in this case is unusual and has not been previously reported. Ultrasound and CT are sensitive for calcification, and the disease should be suspected in children presenting with cardiac or respiratory manifestations and features of arterial calcification, where no metabolic cause is established. (orig.)

  13. Idiopathic Arterial Calcification of Infancy: Case Report

    OpenAIRE

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-01-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persi...

  14. Family history of idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Dauvilliers, Yves; Postuma, Ronald B; Ferini-Strambi, Luigi;

    2013-01-01

    To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort.......To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort....

  15. The classification, natural history and radiological/histological appearance of idiopathic pulmonary fibrosis and the other idiopathic interstitial pneumonias

    Directory of Open Access Journals (Sweden)

    G. Raghu

    2008-12-01

    Full Text Available The idiopathic interstitial pneumonias (IIPs are a heterogeneous group of rare interstitial lung diseases (ILDs or diffuse parenchymal lung diseases, which, as their name implies, are of unknown aetiology. The past 10 yrs have seen important advances in the classification of the IIPs into idiopathic pulmonary fibrosis (IPF and its corresponding histopathological pattern of usual interstitial pneumonia (UIP, plus six non-IPF IIP subtypes. The present article will look at the current classification of IIPs, arising from the Consensus Statement of the American Thoracic Society and European Respiratory Society, and discusses the importance of differential diagnosis of IPF from the non-IPF IIP subtypes, especially nonspecific interstitial pneumonia. Diagnosis of IIPs is a dynamic process involving close collaboration between pulmonologists, radiologists and pathologists. Increasingly accurate diagnosis of IPF has been made possible by the use of high-resolution computed tomography (HRCT and refinements in surgical lung biopsy. In IPF, a lung HRCT will typically reveal irregular reticular opacities, traction bronchiestasis and, most importantly, peripheral honeycombing. In contrast, histological examination shows evidence of UIP manifesting as typically subpleural and paraseptal established fibrosis, often with honeycomb changes, associated with mild chronic inflammation and varying numbers of fibroblastic foci in continuity with the edges of areas of established fibrosis. Despite these advances, obtaining a consistent and uniform diagnosis of idiopathic interstitial pneumonias is difficult, with studies showing significant disagreement in the diagnosis of interstitial lung diseases between academic centres of expertise and community-based clinicians. Greater interaction between academic and community clinicians, together with improved education, is needed to bridge this gap.

  16. Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

    Directory of Open Access Journals (Sweden)

    Bruno Crestani

    2014-06-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF is the most frequent chronic idiopathic interstitial pneumonia in adults. The management of rare diseases in France has been organised by a national plan for rare diseases, which endorsed a network of expert centres for rare diseases throughout France. This article is an overview of the executive summary of the French guidelines for the management of IPF, an initiative that emanated from the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases. This review aims at providing pulmonologists with a document that: 1 combines the current available evidence; 2 reviews practical modalities of diagnosis and management of IPF; and 3 is adapted to everyday medical practice. The French practical guidelines result from the combined efforts of a coordination committee, a writing committee and a multidisciplinary review panel, following recommendations from the Haute Autorité de Santé. All recommendations included in this article received at least 90% agreement by the reviewing panel. Herein, we summarise the main conclusions and practical recommendations of the French guidelines.

  17. [Retro-odontoid pseudotumor in diffuse idiopathic skeletal hyperostosis].

    Science.gov (United States)

    Castro-Castro, Julián; Castro-Bouzas, Daniel; Pinzón-Millán, Alfonso; Pastor-Zapata, Ana

    2014-01-01

    Retro-odontoid pseudotumors are lesions caused by inflammatory granulation or reactive soft tissue hypertrophy from chronic atlantoaxial subluxation. However, one-third of the cases reported in the medical literature did not show atlantoaxial instability clearly. The authors present the case of a 76-year-old man previously diagnosed with diffuse idiopathic skeletal hyperostosis who presented with severe progressive myelopathy. A magnetic resonance imaging of his cervical spine revealed a retro-odontoid predural mass, which caused a severe compression of the cervical spinal cord. The patient underwent a posterior laminectomy of the atlas and an occipitocervical fusion. After surgery, the pseudotumor was considerably smaller and the neurological symptoms improved. PMID:23465746

  18. Idiopathic Pulmonary Fibrosis and Myasthenia Gravis: An Unusual Association.

    Science.gov (United States)

    Chogtu, Bharti; Malik, Daliparty Vasudev; Magazine, Rahul

    2016-04-01

    Idiopathic Pulmonary Fibrosis (IPF) is a chronic fibrosing lung condition with high morbidity and mortality, accounting for about 25% of the cases of interstitial lung diseases. It usually has a progressive course resulting in death due to respiratory failure. Myasthenia Gravis (MG) is an autoimmune neuromuscular disease, caused by antibody mediated activity against acetylcholine receptor at the neuromuscular junction. It is characterized by fluctuating muscle weakness and fatigue. Extensive literature search did not reveal any case report of an association between these two conditions. Here we present a case of a patient with IPF who also developed MG. The diagnosis of IPF was based on High Resolution Computed Tomography (HRCT) of the lung and that of MG was based on clinical criteria and electrophysiological testing. The case was successfully managed. PMID:27190866

  19. A variety of gene polymorphisms associated with idiopathic granulomatous mastitis

    Science.gov (United States)

    Destek, Sebahattin; Gul, Vahit Onur; Ahioglu, Serkan

    2016-01-01

    Idiopathic granulomatous mastitis (IGM) is a rare and chronic inflammatory disorder. IGM mimics breast cancer regarding its clinical and radiological features. Etiology of IGM remains unclarified. Our patient was 37-year-old and 14 weeks pregnant. There was pain, redness and swelling in the right breast. The mass suggestive of malignancy was detected in sonography. Serum CA 125 and CA 15-3 levels were high. Genetic analysis was performed for the etiology. methylenetetrahydrofolate reductase (MTHFR) C 677 TT, β-fibrinogen-455 G>A, plasminogen activator inhibitor (PAI)-1 5 G/5 G, angiotensin-converting enzyme (ACE) I/D mutation was found. IGM was diagnosed by cor biopsy. An association was also reported between breast cancer and mutations in MTHFR-C 677 T, PAI-1, ACE genes. Genetic polymorphisms may involve in the development of IGM as it was seen in our case. Further studies should be conducted to better clarify this plausible association. PMID:27619324

  20. Idiopathic Lumbar Epidural Lipomatosis Mimicking Disc Herniation: A Case Report.

    Science.gov (United States)

    Duran, Efe; Ilik, Kemal; Acar, Turker; Yıldız, Melda

    2016-05-01

    Spinal epidural lipomatosis is a rare condition which is described as the accumulation of fat in the extradural territory and often causes dural impingement. Spinal epidural lipomatosis has been implicated in causing a variety of neurologic impairments ranging from back pain, radiculopathy, claudication, myelopathy or even cauda equina syndrome. We report a 46-year-old female with obesity and a history of chronic back pain and radiculopathy who developed idiopathic Spinal epidural lipomatosis diagnosed by magnetic resonance imaging. The purpose of this report is to present a case of spinal epidural lipomatosis presenting with symptomatic cord compression and also remind this rare condition as a the differential diagnosis of epidural lesions in patients with risk factors. PMID:27309484

  1. B-Cell Pathology in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    V. Wiegering

    2010-01-01

    Full Text Available Juvenile Idiopathic Arthritis (JIA is the most common cause of chronic arthritis in childhood and adolescents and encompasses a heterogeneous group of different diseases. Due to the promising results of B-cell depleting therapies in rheumatoid arthritis the role of B-cells in autoimmune diseases has to be discussed in a new context. Additionally, experiments in mouse models have shed new light on the antibody-independent role of B-cells in the development of autoimmune diseases. In this review we will discuss the importance of B-cells in the pathogenesis of JIA appraising the question for an immunological basis of B-cell targeted therapy in JIA.

  2. Idiopathic granulomatous hepatitis in an 11-year-old boy

    Directory of Open Access Journals (Sweden)

    Deepika Rustogi

    2016-01-01

    Full Text Available Hepatic granulomas are reported in 2-15% of all liver biopsies that are attributable to various causes. Owing to the diverse prognostic and therapeutic implications, a detailed workup of all liver biopsies with granulomatous lesions is mandatory. The cause of the disease may remain obscure in up to 50% cases of "idiopathic granulomatous hepatitis" (IGH. This disease runs a chronic relapsing course. Hepatocellular dysfunction may be an early predominant manifestation unlike that in the available literature. Timely recognition and diagnosis of IGH is imperative as the disease responds well to immunosuppressants that can prevent permanent liver damage. We report a case of a young boy with IGH who was successfully treated with immunosuppressive therapy.

  3. Chronic inflammatory demyelinating polyneuropathy associated with carcinoma.

    OpenAIRE

    Antoine, J C; Mosnier, J. F.; Lapras, J; Convers, P.; Absi, L; Laurent, B.; Michel, D

    1996-01-01

    The association of chronic inflammatory demyelinating polyneuropathy (CIDP) and carcinoma has rarely been reported and its relevance is debated. Thirty three consecutive patients with probable or definite CIDP (idiopathic or associated with M protein) were investigated. Three patients with definite CIDP had a concomitant carcinoma. One had an IgM paraprotein. Steroids and intravenous immunoglobulins were effective.

  4. Cancer risk after cyclophosphamide treatment in idiopathic membranous nephropathy

    NARCIS (Netherlands)

    Brand, J.A. van den; Dijk, P.R. van; Hofstra, J.M.; Wetzels, J.F.

    2014-01-01

    BACKGROUND AND OBJECTIVES: Cyclophosphamide treatment improves renal survival in patients with idiopathic membranous nephropathy. However, use of cyclophosphamide is associated with cancer. The incidence of malignancies in patients with idiopathic membranous nephropathy was evaluated, and the cancer

  5. Effects of increased von Willebrand factor levels on primary hemostasis in thrombocytopenic patients with liver cirrhosis.

    Directory of Open Access Journals (Sweden)

    Andreas Wannhoff

    Full Text Available In patients with liver cirrhosis procoagulant and anticoagulant changes occur simultaneously. During primary hemostasis, platelets adhere to subendothelial structures, via von Willebrand factor (vWF. We aimed to investigate the influence of vWF on primary hemostasis in patients with liver cirrhosis. Therefore we assessed in-vitro bleeding time as marker of primary hemostasis in cirrhotic patients, measuring the Platelet Function Analyzer (PFA-100 closure times with collagen and epinephrine (Col-Epi, upper limit of normal ≤ 165 s or collagen and ADP (Col-ADP, upper limit of normal ≤ 118 s. If Col-Epi and Col-ADP were prolonged, the PFA-100 was considered to be pathological. Effects of vWF on primary hemostasis in thrombocytopenic patients were analyzed and plasma vWF levels were modified by adding recombinant vWF or anti-vWF antibody. Of the 72 included cirrhotic patients, 32 (44.4% showed a pathological result for the PFA-100. They had mean closure times (± SD of 180 ± 62 s with Col-Epi and 160 ± 70 s with Col-ADP. Multivariate analysis revealed that hematocrit (P = 0.027 and vWF-antigen levels (P = 0.010 are the predictors of a pathological PFA-100 test in cirrhotic patients. In 21.4% of cirrhotic patients with platelet count ≥ 150/nL and hematocrit ≥ 27.0%, pathological PFA-100 results were found. In thrombocytopenic (< 150/nL patients with cirrhosis, normal PFA-100 results were associated with higher vWF-antigen levels (462.3 ± 235.9% vs. 338.7 ± 151.6%, P = 0.021. These results were confirmed by multivariate analysis in these patients as well as by adding recombinant vWF or polyclonal anti-vWF antibody that significantly shortened or prolonged closure times, respectively. In conclusion, primary hemostasis is impaired in cirrhotic patients. The effect of reduced platelet count in cirrhotic patients can at least be partly compensated by increased vWF levels. Recombinant vWF could be an alternative to platelet transfusions in the

  6. Idiopathic aneurysm of pulmonary artery

    Energy Technology Data Exchange (ETDEWEB)

    Pacheco, Julio B. Cota; Pimentel, Patricia N.; Knust, Beatriz S., E-mail: jcota@uol.com.br [Clinica de Cardiologia Cota Pacheco, Mogi das Cruzes, SP (Brazil)

    2015-07-15

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  7. Idiopathic aneurysm of pulmonary artery

    International Nuclear Information System (INIS)

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  8. Telltale teeth: Idiopathic Hypergonadotropic Hypogonadism

    Directory of Open Access Journals (Sweden)

    G S Lele

    2014-01-01

    Full Text Available The detection of any atypical extraoral or intraoral features warrants a thorough investigation, even if the patient is asymptomatic or unaware of these. At times, dental findings help in the diagnosis of an underlying systemic problem. These findings may or may not be associated with any syndrome. Thus, thorough examination and exhaustive investigations should be carried out for every atypical finding to ensure optimal oral and general health for the patient. Case Description: This is a case report of seventeen year old male who presented with peculiar/atypical dentition which ′told the tale′ and led to the diagnosis of underlying endocrinological problem about which the parents were totally unaware. The patient was short with central obesity and microcephaly. Intraorally, there was presence of thirty six microdonts. Consultation with pediatrician and endocrinologist, and thorough investigations confirmed the condition to be of ′Idiopathic Hypergonadotropic Hypogonadism′. The patient underwent not only oral rehabilitation, but also timely consultation and treatment from a pediatrician and an endocrinologist.

  9. Pneumothorax and idiopathic pulmonary fibrosis

    International Nuclear Information System (INIS)

    We evaluated the relation between the severity of idiopathic pulmonary fibrosis (IPF) and the incidence of pneumothorax on computed tomography (CT) images. In this retrospective study, we evaluated the presence of pneumothorax in 56 consecutive patients who died of IPF from the initial CT to death. We quantitatively analyzed a total of 207 CT images and measured the volume of the normal pattern (N-pattern) and each lesion pattern on the initial CT and their serial changes. The effects of pneumothorax and clinical and CT features on survival were evaluated using Cox regression analysis. Pneumothorax occurred in 17 of 56 patients. Comparison of the pneumothorax (+) and (-) groups showed the initial vital capacity (VC) was lower (P=0.005) and the follow-up period was shorter (P=0.03) in the former group. The decrease in the N-pattern volume in the pneumothorax (+) group was significantly faster than in the pneumothorax (-) group (P=0.013). Cox regression analyses identified a rapid decrease in N-pattern volume (P=0.008) and a rapid decrease in VC (P=0.002), but not pneumothorax, as significant predictors of poor survival. Pneumothorax in IPF patients is associated with lower VC and rapid deterioration of CT findings. The findings suggest that pneumothorax is a complication of advanced IPF. (author)

  10. Familial idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Huovinen, Joel; Kastinen, Sami; Komulainen, Simo; Oinas, Minna; Avellan, Cecilia; Frantzen, Janek; Rinne, Jaakko; Ronkainen, Antti; Kauppinen, Mikko; Lönnrot, Kimmo; Perola, Markus; Pyykkö, Okko T; Koivisto, Anne M; Remes, Anne M; Soininen, Hilkka; Hiltunen, Mikko; Helisalmi, Seppo; Kurki, Mitja; Jääskeläinen, Juha E; Leinonen, Ville

    2016-09-15

    Idiopathic normal pressure hydrocephalus (iNPH) is a late-onset surgically alleviated, progressive disease. We characterize a potential familial subgroup of iNPH in a nation-wide Finnish cohort of 375 shunt-operated iNPH-patients. The patients were questionnaired and phone-interviewed, whether they have relatives with either diagnosed iNPH or disease-related symptomatology. Then pedigrees of all families with more than one iNPH-case were drawn. Eighteen patients (4.8%) from 12 separate pedigrees had at least one shunt-operated relative whereas 42 patients (11%) had relatives with two or more triad symptoms. According to multivariate logistic regression analysis, familial iNPH-patients had up to 3-fold risk of clinical dementia compared to sporadic iNPH patients. This risk was independent from diagnosed Alzheimer's disease and APOE ε4 genotype. This study describes a familial entity of iNPH offering a novel approach to discover the potential genetic characteristics of iNPH. Discovered pedigrees offer an intriguing opportunity to conduct longitudinal studies targeting potential preclinical signs of iNPH. PMID:27538594

  11. HBV感染并发ITP病儿临床特征分析%A CLINICAL ANALYSIS BETWEEN HEPATITIS B VIRUS INFECTION AND IDIOPATHIC THROMBOCYTOPENIC PURPURA IN CHILDREN

    Institute of Scientific and Technical Information of China (English)

    宋爱琴; 董增义; 庞秀英; 卢愿; 李学荣; 孙立荣

    2006-01-01

    目的探讨乙型肝炎病毒(HBV)感染并发原发性血小板减少性紫癜(ITP)病儿临床特征、治疗反应和病程特点.方法将102例ITP病儿,分为两组,其中HBV-ITP组25例,非HBV-ITP组77例,病儿均采用大剂量丙种球蛋白、常规治疗量泼尼松、小剂量长春新碱等治疗,比较两组病儿的临床特征、治疗反应、病程特点等.结果 HBV-ITP组与非HBV-ITP组治疗前外周血血红蛋白水平、血小板数量及骨髓检查方面差异无显著意义(t=0.933、1.916,χ2=5.422, P>0.05);非HBV-ITP组外周血白细胞总数较HBV-ITP组高,差异有显著意义(t=5.050, P<0.01).采用大剂量丙种球蛋白、常规治疗量泼尼松、小剂量长春新碱治疗,HBV-ITP组疗效明显差于非HBV-ITP组(χ2=12.710、7.884, P<0.05,P=0.007).HBV-ITP病儿较非HBV-ITP病儿易于发展为慢性.结论 HBV-ITP病儿治疗反应差,病情易迁延、反复,容易向慢性转化.应进一步研究HBV感染对ITP发生发展的可能的作用机制.

  12. Correlation of Notch1/Hes1 Genes Expression Levels in Egyptian Paediatric Patients with Newly Diagnosed and Persistent Primary Immune(Idiopathic) Thrombocytopenic Purpura.

    Science.gov (United States)

    Gawdat, Rania Mohsen; Hammam, Amira Ahmed; Ezzat, Dina Ahmed

    2016-09-01

    Notch signalling is involved in the development of several autoimmune diseases, one of such diseases is ITP. The aim of this study was to investigate and compare the expression levels of Notch1 receptor and its target Hes1 gene in Egyptian paediatric ITP patients. Real-time quantitative reverse transcriptase polymerase chain reaction was used to analyse the expression levels of Notch1 and Hes1 in 42 children with primary ITP (22 newly diagnosed and 20 persistent) cases. Twenty age and sex matched non-ITP controls were included. The expression levels of Notch1 were higher in newly diagnosed and persistent cases than controls with high statistical significant difference (P value ITP patients but Hes1 was markedly elevated than Notch1 in few cases. High expression levels of Notch1/Hes1 indicated the important role of Notch signalling in both newly diagnosed and persistent ITP. High expression levels of Hes1 than Notch1 may shed light on its value as a therapeutic target for future research in ITP. PMID:27429531

  13. Idiopathic interstitial pneumonias: progress in classification, diagnosis, pathogenesis and management.

    OpenAIRE

    King, Talmadge E.

    2004-01-01

    The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopa...

  14. Genetics Home Reference: familial idiopathic basal ganglia calcification

    Science.gov (United States)

    ... Wang QK, Liu JY. Identification of a novel genetic locus on chromosome 8p21.1-q11.23 for idiopathic ... DH. Analysis of candidate genes at the IBGC1 locus associated with idiopathic basal ... DH. Genetic heterogeneity in familial idiopathic basal ganglia calcification (Fahr ...

  15. An update on pathophysiological mechanisms related to idiopathic oro-facial pain conditions with implications for management.

    Science.gov (United States)

    Forssell, H; Jääskeläinen, S; List, T; Svensson, P; Baad-Hansen, L

    2015-04-01

    Chronic oro-facial pain conditions such as persistent idiopathic facial pain (PIFP), atypical odontalgia (AO) and burning mouth syndrome (BMS), usually grouped together under the concept of idiopathic oro-facial pain, remain a diagnostic and therapeutic challenge. Lack of understanding of the underlying pathophysiological mechanisms of these pain conditions is one of the important reasons behind the problems in diagnostic and management. During the last two decades, neurophysiological, psychophysical, brain imaging and neuropathological methods have been systematically applied to study the trigeminal system in idiopathic oro-facial pain. The findings in these studies have provided evidence for neuropathic involvement in the pathophysiology of PIFP, AO and BMS. The present qualitative review is a joint effort of a group of oro-facial pain specialists and researchers to appraise the literature on idiopathic oro-facial pain with special focus on the currently available studies on their pathophysiological mechanisms. The implications of the findings of these studies for the clinical diagnosis and treatment of idiopathic oro-facial pain conditions are discussed. PMID:25483941

  16. Refractory Immune Thrombocytopenic Purpura and Cytomegalovirus Infection: A Call for a Change in the Current Guidelines

    Directory of Open Access Journals (Sweden)

    Alex Shimanovsky

    2016-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP is characterized by a decreased platelet count caused by excess destruction of platelets and inadequate platelet production. In many cases the etiology is not known, but viral illness is thought to play a role in the development of some cases of ITP. The current (2011 American Society of Hematology ITP guidelines recommend initial diagnostic studies to include testing for HIV and Hepatitis C. The guidelines suggest that initial treatment consist of observation, therapy with corticosteroids, IVIG or anti D. While most cases respond to the standard therapy such that the steroids may be tapered and the platelet counts remain at a hemostatically safe level. Some patients with ITP are dependent on long term steroid maintenance and the thrombocytopenia persists with the tapering of the steroids. Recent case reports demonstrate that ITP related to cytomegalovirus (CMV can persist in spite of standard therapy and that antiviral therapy maybe indicated. Herein we report a case of a 26-year-old female with persistent ITP that resolved after the delivery of a CMV infected infant and placenta. Furthermore we review the current literature on CMV-associated ITP and propose that the current ITP guidelines be amended to include assessment for CMV as part of the work-up for severe and refractory ITP prior to splenectomy.

  17. When the picture is fragmented: Vitamin B12 deficiency masquerading as thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Panchabhai, Tanmay S; Patil, Pradnya D; Riley, Elizabeth C; Mitchell, Charlene K

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) has high mortality and necessitates prompt recognition of microangiopathic hemolytic anemia (MAHA) and initiation of plasmapheresis. We present a challenging diagnostic workup and management of a 42-year-old man who presented with anemia, thrombocytopenia, and schistocytes on peripheral smear, all pointing to MAHA. Plasmapheresis and steroid therapy were promptly initiated, but hemolysis continued. Further workup showed megaloblastic anemia, severe Vitamin B12 deficiency, high iron saturation, and absent reticulocytosis, none of which could be explained by TTP. Severe Vitamin B12 deficiency can lead to hemolytic anemia from the destruction of red cells in the marrow that have failed the process of maturation. However, this should not cause thrombotic microangiopathy. Previous reports of B12 deficiency presenting with MAHA and a TTP-like manifestation have identified acute hyperhomocysteinemia as a missing link between B12 deficiency and MAHA, so this possibility was further explored. Our patient similarly had significantly elevated serum homocysteine levels, confirming this suspicion of Vitamin B12 deficiency. Vitamin B12 replacement led to normalization of the elevated levels of homocysteine, the disappearance of schistocytes on the peripheral smear, and resolution of the microangiopathic hemolysis, thereby confirming the diagnosis. It is pertinent that intensivists not only know the importance of early recognition and treatment of TTP but are also familiar with rare conditions that can present in a similar fashion. PMID:27308258

  18. Acute renal failure and severe rhabdomyolysis in a patient with resistant thrombotic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Al Qahtani S

    2011-10-01

    Full Text Available Saad Al Qahtani Intensive Care Department, Critical Care Response Team, King Abdulaziz Medical City (KAMC, National Guard Health Affairs; King Saud Bin Abdulaziz University for Health Sciences, College of Medicine, Riyadh, Kingdom of Saudi Arabia Abstract: Thrombotic thrombocytopenic purpura (TTP is a rare, life-threatening disorder. This paper describes the case of a 39-year-old Sudanese male who presented to the emergency room with fever, jaundice, decreased level of consciousness, and worsening kidney function for 7 days, a high lactate dehydrogenase level (1947, severe thrombocytopenia (platelets 8, and numerous schistocytes in the peripheral blood smear. The patient was admitted with a diagnosis of TTP for plasma exchange. Fourteen days later, his creatinine kinase (CK level rose to >50,000 IU; rhabdomyolysis was suggested. Continuous venovenous hemodialysis (CVVHD was started. The patient's CK level remained high, despite CVVHD, until the 6th day, after which this parameter gradually started to decrease. This report highlights a resistant case of TTP that presented with concomitant severe rhabdomyolysis, which demanded aggressive, continuous intervention. Keywords: TTP, CVVHD, continuous venovenous hemodialysis

  19. 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.

  20. [Antiphospholipid syndrome with autoimmune hemolytic anemia which mimics thrombotic thrombocytopenic purpura].

    Science.gov (United States)

    Karasawa, Naoki; Taniguchi, Yasuhiro; Hidaka, Tomonori; Katayose, Keiko; Kameda, Takuro; Side, Kotaro; Shimoda, Haruko; Nagata, Kenji; Kubuki, Yoko; Matsunaga, Takuya; Shimoda, Kazuya

    2010-04-01

    A 67-year-old woman was admitted to the hospital for lethargy, fever, hemolytic anemia, thrombocytopenia, and consciousness disturbance. Direct Coombs test was positive, and anti-cardiolipin beta2-glycoprotein I antibody was detected. She was diagnosed with antiphospholipid syndrome complicated with autoimmune hemolytic anemia (AIHA). She demonstrated variable consciousness disturbance, inability to distinguish right from left, dysgraphia and dyscalculia. Multiple cerebral infarctions, especially dominant cerebral hemisphere infarctions, were observed on magnetic resonance imaging. A ventilation-perfusion scan demonstrated the presence of a ventilation-perfusion mismatch in both lung fields, and multiple veinous embolisms in the right femoral, bilateral the great saphenous and popliteal veins. Therefore, pulmonary embolism and thrombophlebitis were diagnosed. Based on these findings, it was necessary to distinguish this diagnosis from thrombotic thrombocytopenic purpura (TTP). As ADAMTS-13 activity was within the normal range, TTP was denied. Thereafter, the patient was treated with 1 mg/kg of prednisolone for AIHA, 3 mg of warfarin, and 3500 units of low-molecular-weight heparin for thrombosis, and her condition improved. PMID:20467225

  1. Common variable immunodeficiency unmasked by treatment of immune thrombocytopenic purpura with Rituximab

    DEFF Research Database (Denmark)

    Mogensen, Trine H; Jensen, Jens Magnus Bernth; Petersen, Charlotte C;

    2013-01-01

    BACKGROUND: Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs of immunod......BACKGROUND: Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs...... of immunodeficiency, this is not always recognized. Despite novel insight into the molecular immunology of common variable immunodeficiency, several areas of uncertainty remain. In addition, the full spectrum of immunological effects of the B cell depleting anti-CD20 antibody Rituximab has not been fully explored....... To our knowledge this is the first report of development of CVID in a patient with normal immunoglobulin prior to Rituximab treatment. CASE PRESENTATION: Here we describe the highly unusual clinical presentation of a 34-year old Caucasian male with treatment refractory immune thrombocytopenic purpura...

  2. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

    Directory of Open Access Journals (Sweden)

    Suella Martino

    Full Text Available Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP. Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04. Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all. Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03. Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

  3. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors

    Science.gov (United States)

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all). Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03). Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population. PMID:27383202

  4. Genetic polymorphisms and idiopathic generalized epilepsies.

    Science.gov (United States)

    Lucarini, Nazzareno; Verrotti, Alberto; Napolioni, Valerio; Bosco, Guido; Curatolo, Paolo

    2007-09-01

    In recent years, progress in understanding the genetic basis of idiopathic generalized epilepsies has proven challenging because of their complex inheritance patterns and genetic heterogeneity. Genetic polymorphisms offer a convenient avenue for a better understanding of the genetic basis of idiopathic generalized epilepsy by providing evidence for the involvement of a given gene in these disorders, and by clarifying its pathogenetic mechanisms. Many of these genes encode for some important central nervous system ion channels (KCNJ10, KCNJ3, KCNQ2/KCNQ3, CLCN2, GABRG2, GABRA1, SCN1B, and SCN1A), while many others encode for ubiquitary enzymes that play crucial roles in various metabolic pathways (HP, ACP1, ME2, LGI4, OPRM1, GRIK1, BRD2, EFHC1, and EFHC2). We review the main genetic polymorphisms reported in idiopathic generalized epilepsy, and discusses their possible functional significance in the pathogenesis of seizures. PMID:17765802

  5. Nonsurgical Management of Adolescent Idiopathic Scoliosis.

    Science.gov (United States)

    Gomez, Jaime A; Hresko, M Timothy; Glotzbecker, Michael P

    2016-08-01

    Pediatric patient visits for spinal deformity are common. Most of these visits are for nonsurgical management of scoliosis, with approximately 600,000 visits for adolescent idiopathic scoliosis (AIS) annually. Appropriate management of scoliotic curves that do not meet surgical indication parameters is essential. Renewed enthusiasm for nonsurgical management of AIS (eg, bracing, physical therapy) exists in part because of the results of the Bracing in Adolescent Idiopathic Scoliosis Trial, which is the only randomized controlled trial available on the use of bracing for AIS. Bracing is appropriate for idiopathic curves between 20° and 40°, with successful control of these curves reported in >70% of patients. Patient adherence to the prescribed duration of wear is essential to maximize the effectiveness of the brace. The choice of brace type must be individualized according to the deformity and the patient's personality as well as the practice setting and brace availability. PMID:27388720

  6. Chronic pancreatitis: controversies in etiology, diagnosis and treatment Pancreatitis crónica: controversias respecto a la etiología, el diagnóstico y el tratamiento

    OpenAIRE

    P. Draganov; Toskes, P P

    2004-01-01

    The pathogenesis of idiopathic chronic pancreatitis remains poorly understood despite the high expectations for ascribing the pancreatic damage in affected patients to genetic defects. Mutations in the cationic trypsinogen gene, pancreatic secretory trypsin inhibitor, and the cystic fibrosis conductance regulator gene do not account for the chronic pancreatitis noted in most patients with idiopathic chronic pancreatitis. Small duct chronic pancreatitis can be best diagnosed with a hormone sti...

  7. Total fertilization failure and idiopathic subfertility

    Directory of Open Access Journals (Sweden)

    Goverde Angelique J

    2009-01-01

    Full Text Available Abstract Background To gain more insight in whether failure of intrauterine insemination (IUI treatment in patients with idiopathic subfertility could be related to diminished fertilization, the aim of this study is to compare the fertilization of an initial IVF procedure after six cycles of IUI and the fertilization of an initial IVF procedure without preceding IUI cycles in couples with idiopathic subfertility. Methods We performed a complimentary analysis of a randomized controlled trial, in which the number of total fertilization failure (TFF in the first IVF procedure after unsuccessful IUI was compared to those of IVF without preceding IUI in patients with idiopathic subfertility. These patients participated in a previous study that assessed the cost effectiveness of IUI versus IVF in idiopathic subfertility and were randomized to either IUI or IVF treatment. Results 45 patients underwent IVF after 6 cycles of unsuccessful IUI and 58 patients underwent IVF immediately without preceding IUI. In 7 patients the IVF treatment was cancelled before ovum pick. In the IVF after unsuccessful IUI group TFF was seen in 2 of the 39 patients (5% versus 7 of the 56 patients (13% in the immediate IVF group. After correction for confounding factors the TFF rate was not significantly different between the two groups (p = 0.08, OR 7.4; 95% CI: 0.5–14.9. Conclusion Our data showed that TFF and the fertilization rate in the first IVF treatment were not significantly different between couples with idiopathic subfertility undergoing IVF after failure of IUI versus those couples undergoing IVF immediately without prior IUI treatment. Apparently, impaired fertilization does not play a significant role in the success rate of IUI in patients with idiopathic subfertility.

  8. Chronic urticaria

    Directory of Open Access Journals (Sweden)

    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.

  9. Cauda Equina Neuritis: A Chronic Idiopathic Polyneuritis in Two Horses

    OpenAIRE

    Rousseaux, C. G.; Futcher, K. G.; Clark, E G; Naylor, J M

    1984-01-01

    Two cases of cauda equina neuritis are compared and contrasted. Neurological deficits of the tail and perineum were noted and functional deficits were seen in gait, urination, defecation and cranial nerve function. Lesions consisted of nonsuppurative inflammation of the nerve trunks and proliferation of the perineurium of the cauda equina. Cranial nerve involvement in one case supported a diagnosis of polyneuritis equi rather than cauda equina neuritis. The possible etiologies and pathogenesi...

  10. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    Science.gov (United States)

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

  11. An unusual case of idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Vikender S Yadav

    2013-01-01

    Full Text Available Idiopathic gingival fibromatosisis, a condition of undetermined cause can develop as an isolated disorder, but mostly it is associated with some syndrome. It usually begins at the time of eruption of permanent teeth but can develop with the eruption of deciduous dentition and rarely present at birth. This case report describes an unusual case of non-syndromic generalized idiopathic gingival fibromatosis in a 15-year-old male present since birth. Surgical treatment in the form of ledge and wedge procedure with internal bevel gingivectomy was performed. No recurrence of enlargement was seen after 2 years of follow-up.

  12. Meandering main pancreatic duct as a relevant factor to the onset of idiopathic recurrent acute pancreatitis.

    Directory of Open Access Journals (Sweden)

    Wataru Gonoi

    Full Text Available BACKGROUND: Meandering main pancreatic duct (MMPD, which comprises loop type and reverse-Z type main pancreatic duct (MPD, has long been discussed its relation to pancreatitis. However, no previous study has investigated its clinical significance. We aimed to determine the non-biased prevalence and the effect of MMPD on idiopathic pancreatitis using non-invasive magnetic resonance (MR technique. METHODS AND FINDINGS: A cross-sectional study performed in a tertiary referral center. The study enrolled 504 subjects from the community and 30 patients with idiopathic pancreatitis (7 acute, 13 chronic, and 10 recurrent acute. All subjects underwent MR scanning and medical examination. MMPD was diagnosed when the MPD in the head of pancreas formed two or more extrema in the horizontal direction on coronal images of MR cholangiopancreatography, making a loop or a reverse-Z shaped hairpin curves and not accompanied by other pancreatic ductal anomaly. Statistical comparison was made among groups on the rate of MMPD including loop and reverse-Z subtypes, MR findings, and clinical features. The rate of MMPD was significantly higher for all idiopathic pancreatitis/idiopathic recurrent acute pancreatitis (RAP (20%/40%; P<0.001/0.0001; odds ratio (OR, 11.1/29.0 than in the community (2.2% but was not higher for acute/chronic pancreatitis (14%/8%; P = 0.154/0.266. Multiple logistic regression analysis revealed MMPD to be a significant factor that induces pancreatitis/RAP (P<0.0001/0.0001; OR, 4.01/26.2. Loop/reverse-Z subtypes were found more frequently in idiopathic RAP subgroup (20%/20%; P = 0.009/0.007; OR, 20.2/24.2 than in the community (1.2%/1.0%. The other clinical and radiographic features were shown not associated with the onset of pancreatitis. CONCLUSIONS: MMPD is a common anatomical variant and might be a relevant factor to the onset of idiopathic RAP.

  13. Nodes of Ranvier and Paranodes in Chronic Acquired Neuropathies

    OpenAIRE

    Carmen Cifuentes-Diaz; Odile Dubourg; Theano Irinopoulou; Marc Vigny; Sylvie Lachkar; Laurence Decker; Patrick Charnay; Natalia Denisenko; Thierry Maisonobe; Jean-Marc Léger; Karine Viala; Jean-Jacques Hauw; Jean-Antoine Girault

    2011-01-01

    Chronic acquired neuropathies of unknown origin are classified as chronic inflammatory demyelinating polyneuropathies (CIDP) and chronic idiopathic axonal polyneuropathies (CIAP). The diagnosis can be very difficult, although it has important therapeutic implications since CIDP can be improved by immunomodulating treatment. The aim of this study was to examine the possible abnormalities of nodal and paranodal regions in these two types of neuropathies. Longitudinal sections of superficial per...

  14. Incidence of Thrombocytopenia in Idiopathic Hyperbilirubinemic Newborns

    Directory of Open Access Journals (Sweden)

    Hassan Boskabadi

    2014-06-01

    Conclusion: This study determines higher rate of thrombocytopenia among idiopathic hyperbilirubinemic neonates (36% and helps the practitioner to be aware of this association and avoid unnecessary investigations.We did not find a significant correlation between serum bilirubin values and thrombocytopenia.

  15. Idiopathic hepatic arterial malformation: a case report

    Institute of Scientific and Technical Information of China (English)

    郑蔚巍; 周康荣; 王佩芬; 陈祖望

    2003-01-01

    @@ Hepatic arterial malformation is a rare disorder which either origi nates idiopathically or may be associated with hereditary hemorrhagic telangiect asia (also known as Osler-Weber-Rendu disease). Although previous reports presented only descriptions of sonographic and angiographic findings,1-6 we present a case of splenic infarct caused by this disorder with CT and CTA findi ngs.

  16. Genetics Home Reference: idiopathic inflammatory myopathy

    Science.gov (United States)

    ... 1001/jama.2010.1977. Citation on PubMed or Free article on PubMed Central Shamim EA, Miller FW. Familial autoimmunity and the idiopathic inflammatory myopathies. Curr Rheumatol Rep. 2000 Jun;2(3):201-11. Review. Citation on PubMed Shamim EA, Rider LG, Miller FW. Update on the genetics of ...

  17. Jejunal atresia associated with idiopathic ileal perforation

    Directory of Open Access Journals (Sweden)

    Das P

    2008-01-01

    Full Text Available Jejunoileal atresia is one of the common causes of neonatal intestinal obstruction. Intestinal perforation with meconium peritonitis in the neonatal period, which carries a high mortality rate, is also common. The association of jejunal atresia with idiopathic ileal perforation is very rare.

  18. Idiopathic trigeminal neuropathy in a poodle

    Directory of Open Access Journals (Sweden)

    Carlos Eduardo Aparicio

    2010-12-01

    Full Text Available A seven years old, male poodle is examined presenting acute mandible paralysis (dropped jaw, drooling and difficulty for the apprehension and chewing; not evidence of an other alteration of cranial nerves. The muscular biopsy rules out a myositisof masticatory muscles. The disorder is resolved completely in 3 weeks confirming diagnosis of idiopathic trigeminal neuropathy.

  19. Selective fusion in adolescent idiopathic scoliosis

    Institute of Scientific and Technical Information of China (English)

    WANG Ting; XU Jian-guang; ZENG Bing-fang

    2008-01-01

    @@ Despite the continual evolution in the surgical treatment of adolescent idiopathic scoliosis (AIS), the goals of surgery remain to correct and stabilize the deformity in three dimensions, to maintain equilibrium of the shoulders and trunk, and to leave as many mobile spinal segments as possible.

  20. Idiopathic granulomatous mastitis: rare but important

    OpenAIRE

    Hwang, M J; Rogers, A; Vidya, R.

    2010-01-01

    A 61-year-old woman attended the breast clinic with unresolving mastitis and an associated mass, following failed treatment with antibiotics. Triple assessment confirmed idiopathic granulomatous mastitis. Unresponsive to further conservative management and steroid therapy, she underwent surgical excision and made uneventful recovery. No evidence of recurrence was detected at 18 months follow-up.

  1. Idiopathic Arterial Calcification of Infancy: Case Report.

    Science.gov (United States)

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-11-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding. PMID:27252793

  2. The lived experience with idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Kaldan, Gudrun; Marsaa, Kristoffer;

    2016-01-01

    The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family...

  3. Acute surgical management in idiopathic intracranial hypertension.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

    Idiopathic intracranial hypertension is a headache syndrome with progressive symptoms of raised intracranial pressure. Most commonly, it is a slow process where surveillance and medical management are the main treatment modalities. We describe herein an acute presentation with bilateral sixth nerve palsies, papilloedema and visual deterioration, where acute surgical intervention was a vision-saving operation.

  4. 胸腺五肽联合地氯雷他定治疗慢性特发性荨麻疹的临床观察%Clinical Observation of Thymopentin-5 Combined with Desloratadine in Treatment of Chronic Idiopathic Urticaria

    Institute of Scientific and Technical Information of China (English)

    晏勇; 余霞萍; 陈希林; 刘长山; 林应花

    2013-01-01

    目的:探讨胸腺五肽联合地氯雷他定治疗慢性特发性荨麻疹的临床疗效。方法:治疗慢性特发性荨麻疹患者180例,按照随机数字表法将其分为治疗组和对照组各90例。治疗组:口服地氯雷他定分散片5 mg,1次/d,同时胸腺五肽针1 mg加入0.9%氯化钠注射液250 mL,静脉滴注,1次/d;对照组:口服地氯雷他定片5 mg,1次/d。两组均连续用药4周后,随访观察12周,评定疗效并记录皮损变化和不良反应。结果:治疗组痊愈率为95.6%,总有效率为97.8%,对照组痊愈率为90%,总有效率为95.6%。两组短期症状控制效果都非常有效,差异无统计学意义(P>0.05)。两组痊愈患者复发情况观察结果显示,治疗组停药超过12周未复发(优等)病例52例(60.5%),对照组11例(13.6%);治疗组停药后4周以内复发(差等)病例9例(10.4%),对照组38例(46.9%),两组比较差异均有统计学意义(P0.05). The two group of patients relapse were observed. The results showed,the treatment group was more than 12 weeks in withdrawal recurrence(top)of 52 cases(60.5%),11 cases in the control group(13.6%),the treatment group 4 weeks after withdrawal recurrence within 9(low)cases(10.4%),38 cases in the control group(46.9%),and the difference of two indicates above between the two groups were no statistically significant(P<0.01).Conclusion:Thymopentin-5 combined with desloratadine in treatment of chronic idiopathic urticaria obvious curative effect,can effectively control the nettle rash and itching,little side effect,low recurrence rate,long-term effect is obviously higher than that of single desloratadine therapy.

  5. Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    高维强; 苏健; 白霞; 王兆钺; 阮长耿

    2004-01-01

    Background Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. In this study we investigated the von Willebrand factor-cleaving protease (vWF-cp) activity deficiency in patients with TTP.Methods The plasma or serum vWF-cp activity was measured using a sensitive enzyme-linked immunosorbent assay (ELISA) by detecting the residual collagen binding activity (R-CBA) of von Willebrand factor (vWF) before and after digestion by vWF-cp. Multimers of vWF in plasma of patients with TTP were also analyzed by SDS-agarose electrophoresis. Moreover, the serum vWF-cp activities were compared between the patients with TTP and those with tumors.Results The coefficient of variation for intra-batch and inter-batch of the assay were 3.60% and 8.35%. The plasma and serum vWF-cp activity in healthy individuals were (78.79±9.17)% (n=30) and (79.47±10.78)% (n=53), respectively, while the plasma vWF-cp activity in 5 patients with TTP was markedly decreased [(21.83±19.98)%, P<0.001]. The unusually large vWF multimers were observed in two plasma samples of the patients with TTP. Although the vWF-cp activities in patients with benign and malignant tumors were also decreased (P<0.03 and P<0.001, respectively), they were relatively high in comparison with that of TTP patients (P<0.001).Conclusion Measurement of the vWF-cp activity using R-CBA is a simple and rapid method for diagnosing TTP. The vWF-cp activity in patients with TTP was markedly lower than those of patients with tumors.

  6. Carboxiterminal pro-endothelin-1 as an endothelial cell biomarker in thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Mikes, Bálint; Sinkovits, György; Farkas, Péter; Csuka, Dorottya; Rázsó, Katalin; Réti, Marienn; Radványi, Gáspár; Demeter, Judit; Prohászka, Zoltán

    2016-05-01

    Thrombotic thrombocytopenic purpura (TTP) is characterised by the deficiency of the von Willebrand factor (VWF) cleaving protease (ADAMTS-13). Although several observations indicate an important role of endothelial activation in the pathogenesis of TTP, no reliable endothelial activation markers are available in the clinical management of TTP. Our aim was to investigate the presence of endothelial activation in TTP and to determine its connections with disease activity, therapy and complement activation. We enrolled 54 patients (median age 40.5; 44 females) and 57 healthy controls (median age 34; 30 females),VWF antigen, carboxiterminal-pro-endothelin-1 (CT-proET-1), complement Factor H and complement activation products (C3bBbP and SC5b-9) were measured. In both the acute and remission phase of TTP we found increased CT-proET-1 and VWF levels, while Factor H levels decreased compared with healthy controls. In remission, however, the elevated CT-proET-1 levels showed 22 % decrease when compared with the acute phase in paired samples (p=0.0031), whereas no changes for VWF and Factor H levels were observed. We also found positive correlations between CT-proET-1 levels and alternative pathway activation markers (C3bBbP; p=0.0360; r=0.4299). The data we present here demonstrate a role of endothelium activation in patients with acute TTP. The finding that CT-proET-1 levels decreased in remission compared with the acute phase further supports endothelial involvement. In addition, we show that endothelial activation also correlated with the activation of the alternative complement pathway. The data suggest that complement and endothelium activation jointly contribute to the development of TTP episodes in patients with predisposition to TTP. PMID:26763086

  7. Neonates born to mothers with immune thrombocytopenic purpura: a single-center experience of 20 years.

    Science.gov (United States)

    Bayhan, Turan; Tavil, Betül; Korkmaz, Ayşe; Ünal, Şule; Hanalioğlu, Damla; Yiğit, Şule; Gümrük, Fatma; Çetin, Mualla; Yurdakök, Murat

    2016-01-01

    Neonates born to mothers with immune thrombocytopenic purpura (ITP) have an increased risk of having thrombocytopenia and bleeding. The aim of our study was to determine maternal and fetal factors that can predict bleeding risk in neonates born to mothers with ITP, and effective treatment strategies by retrospective analysis of our single-center data. We performed a retrospective data review of neonates that were recorded as 'neonates born to mothers with ITP' in the Neonatal ICU of Hacettepe University, Ihsan Dogramacı Children's Hospital, Ankara, Turkey. Medical records of 36 neonates born from 35 mothers were analyzed. Among the 36 neonates born to mothers with ITP, thrombocytopenia (platelet count of less than 150 × 10/l) was detected in 20 (56.0%) neonates on the first day of life. Twelve of the 20 neonates with thrombocytopenia (60.0%) required treatment to increase the platelet counts. Clinical findings related to thrombocytopenia occurred in three (15.0%) neonates, but none of them presented with severe bleeding. There was no statistically significant association between neonatal lowest platelet count and maternal lowest platelet count, maternal platelet count at the time of delivery, and duration of thrombocytopenia, respectively. Neonates born to mothers with ITP have an increased tendency to develop thrombocytopenia, but severe bleeding is very rare in these neonates. Clinicians should pay special attention to follow these neonates. According to our results, both intravenous immunoglobulin and methyl prednisolone were found to be in equivalent efficacy for the treatment of neonatal thrombocytopenia due to maternal ITP. PMID:26258676

  8. Chronic pancreatitis

    Science.gov (United States)

    Chronic pancreatitis - chronic; Pancreatitis - chronic - discharge; Pancreatic insufficiency - chronic; Acute pancreatitis - chronic ... abuse over many years. Repeated episodes of acute pancreatitis can lead to chronic pancreatitis. Genetics may be ...

  9. Chronic urticaria: new management options.

    Science.gov (United States)

    Greenberger, Paul A

    2014-01-01

    Chronic urticaria is defined as episodic or daily hives lasting for at least 6 weeks and impairs quality of life. Two main subtypes include chronic idiopathic (spontaneous) urticaria and inducible (physical) urticaria, but some patients have urticarial vasculitis. "Autoimmune chronic urticaria" implies the presence of histamine releasing or mast cell activating autoantibodies to IgE or FcϵRI, the high affinity receptor on mast cells and basophils. In patients not readily controlled with labeled dosages of second generation H1 receptor antagonists (antihistamines), there is evidence for reduction of urticaria using up to 4 fold increases in labeled dosages. The biologic modifier, omalizumab, helps to reduce lesions of chronic urticaria within 1-2 weeks. PMID:25383135

  10. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?

    Directory of Open Access Journals (Sweden)

    Wim A. Wuyts

    2014-09-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF, the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP. However, UIP is not synonymous with IPF as other clinical conditions may be associated with UIP, including chronic hypersensitivity pneumonitis, collagen vascular disease, drug toxicity, asbestosis, familial IPF and Hermansky–Pudlak syndrome. Differentiating IPF (“idiopathic UIP” from conditions that mimic IPF (“secondary UIP” has substantial therapeutic and prognostic implications. A number of radiological and histological clues may help distinguish IPF from other conditions with a UIP pattern of fibrosis, but their appreciation requires extensive expertise in interstitial lung disease as well as an integrated multidisciplinary approach involving pulmonologists, radiologists and pathologists. In addition, multidisciplinary discussions may decrease the time to initial IPF diagnosis and, thus, enable more timely management. This concept was strongly emphasised by the 2011 ATS/ERS/JRS/ALAT guidelines. This article highlights, with the aid of a clinical case, the difficulties in making a diagnosis of IPF in clinical practice. Yet, an accurate diagnosis is critical, particularly given the availability of drugs that may reduce the pace of functional decline and disease progression in IPF.

  11. Dental manifestation of primary idiopathic hypoparathyroidism.

    Science.gov (United States)

    Srirangarajan, Sridharan; Satyanarayan, Aparna; Ravindra, Shivamurthy; Thakur, Srinath

    2014-07-01

    Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathic disorder, of idiopathic nature, characterized by a deficiency of parathyroid hormone causing low serum calcium (Ca) and high serum phosphorus concentrations. We present a case report ofa 40-year-old woman with complaint of bleeding gums and tingling sensation in fingers and toes reported to our department in March 2005 with no tangible diagnosis from previous medical examinations. Oral findings included abnormally short roots, cemental hyperplasia, widening of the periodontal ligament space and root resorption. Laboratory investigations revealed a low serum Ca level and along with the clinical findings, a diagnosis of IHP was reached. Oral prophylaxis was performed and Ca supplements were prescribed. We believe that this case report is a first of its kind reporting cemental hyperplasia, altered alveolar bone patterns and periodontal ligament widening in a patient with IHP.

  12. Dental manifestation of primary idiopathic hypoparathyroidism

    Directory of Open Access Journals (Sweden)

    Sridharan Srirangarajan

    2014-01-01

    Full Text Available Idiopathic hypoparathyroidism (IHP is a rare endocrinopathic disorder, of idiopathic nature, characterized by a deficiency of parathyroid hormone causing low serum calcium (Ca and high serum phosphorus concentrations. We present a case report ofa 40-year-old woman with complaint of bleeding gums and tingling sensation in fingers and toes reported to our department in March 2005 with no tangible diagnosis from previous medical examinations. Oral findings included abnormally short roots, cemental hyperplasia, widening of the periodontal ligament space and root resorption. Laboratory investigations revealed a low serum Ca level and along with the clinical findings, a diagnosis of IHP was reached. Oral prophylaxis was performed and Ca supplements were prescribed. We believe that this case report is a first of its kind reporting cemental hyperplasia, altered alveolar bone patterns and periodontal ligament widening in a patient with IHP.

  13. Idiopathic gingival enlargement and its management

    Directory of Open Access Journals (Sweden)

    Shetty Arvind

    2010-01-01

    Full Text Available Idiopathic gingival enlargement is a proliferative fibrous lesion of the gingival tissue that causes esthetic and functional problems. Both genetically and pharmacologically induced forms of gingival enlargement exist. This case report addresses the diagnosis and treatment of a case of idiopathic gingival enlargement in a 13-year-old female. The patient presented with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal / occlusal third of the tooth resulting in difficulty in speech and mastication since last three years. Patient also gave a history of surgical treatment being carried out four years back in upper anterior region suggesting of recurrence. Biopsy report confirmed the diagnosis of gingival hyperplasia. Gingivectomy was carried out in all four quadrants by using four different methods.

  14. Clinical characteristics of idiopathic portal hypertension

    Institute of Scientific and Technical Information of China (English)

    Ozgur Harmanci; Yusuf Bayraktar

    2007-01-01

    Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

  15. [Juvenile idiopathic arthritis: Definition and classification].

    Science.gov (United States)

    Deslandre, C

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is a group of diseases defined by the presence of arthritis of more than 6 weeks duration in patients aged less than 16 years and with unknown etiology. The international classification based on clinical and biological criteria define each type of JIA: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, and psoriatic arthritis. However, some discussions persist concerning systemic-onset juvenile idiopathic arthritis, whose clinical symptoms and pathogenic mechanisms are quite similar to those observed in autoinflammatory diseases, arthritis with antinuclear factors (poly- and oligoarticular) that could be considered as a homogenous group, and a family history of psoriasis that frequently led to unclassified arthritis. Better knowledge of the pathogenic mechanisms should improve the initial clinical classification with more homogeneous groups of patients and reduce the number of unclassified cases of arthritis. PMID:26968301

  16. Spontaneous Idiopathic Unilateral Adrenal Haemorrhage (SIAH).

    Science.gov (United States)

    Naqvi, Syed Ali; Zaman, Shamas; Ahmed, Irfan

    2015-04-01

    Spontaneous Idiopathic Adrenal Haemorrhage (SIAH) is an unusual surgical emergency which can present with life threatening massive retroperitoneal bleeding. Most of the cases reported in the literature are associated with use of anticoagulation or underlying adrenal pathology such as tumors or cysts. Since this clinical entity is uncommon and clinical presentation is very indistinct, the diagnosis can be easily missed and can be challenging for the treating physicians. Nevertheless a raised clinical suspicion coupled with advances in radiological imaging have considerably improved the detection of SIAH in recent times. We report an unusual case of a 20 years old healthy female student who presented to our hospital with sudden onset of abdominal pain and shock. She was diagnosed as a case of massive spontaneous idiopathic unilateral adrenal haemorrhage, unaccompanied by any hematologic disorder, trauma or underlying pathology. Although patient was hemodynamically unstable at presentation, she was resuscitated promptly, investigated appropriately, hence recovered uneventfully with conservative management alone.

  17. Pattern of presentation of idiopathic polypoidal choroidal vasculopathy in Ibadan, Sub-Saharan Africa

    Directory of Open Access Journals (Sweden)

    Oluleye TS

    2013-07-01

    Full Text Available TS Oluleye, Y Babalola Retina and Vitreous Unit, Department of Ophthalmology, University of Ibadan and University College Hospital, Ibadan, Nigeria Background: Idiopathic polypoidal choroidal vasculopathy is an abnormal choroidal vascular pathology similar to age-related macular degeneration. It may present with sudden visual loss from hemorrhagic retinal pigment epithelial detachment and breakthrough vitreous hemorrhage or with chronic recurrent episodes. The condition is not uncommon in the retina clinic at the University College Hospital, Ibadan, Sub-Saharan Africa. This study presents the pattern of presentation in Ibadan. Methods: We review all cases of idiopathic polypoidal choroidal vasculopathy seen from 2007 to 2012 in the retina clinic at the University College Hospital, Ibadan, to determine the major pattern of presentations, available treatment modalities, and visual outcomes. Results: Ten cases were seen during the study period. Their mean age was 58 years, with a male to female ratio of 1:4. The most common presenting complaint was sudden visual loss. Major examination findings were retinal pigment epithelial detachment, orange subretinal lesions, and breakthrough vitreous hemorrhage. The modalities of treatment available included vitrectomy to clear vitreous hemorrhage. Intravitreal bevacizumab reduced the height of the pigment epithelial detachment and cleared vitreous hemorrhage. Thermal laser was applied for extrafoveal lesions. Two patients with subfoveal lesions were referred abroad for photodynamic therapy. Visual outcome showed significant improvement in vitrectomized patients who presented with vitreous hemorrhage. Presenting vision of hand motion and light perception improved to vision ranging from counting fingers to 6/12 after vitrectomy. Conclusion: Idiopathic polypoidal choroidal vasculopathy may not be uncommon in Sub-Saharan Africa. A high index of suspicion is warranted in the diagnosis so as to provide timely

  18. Capsaicin in Idiopathic Rhinitis A Hot Topic

    OpenAIRE

    van Rijswijk, J B

    2005-01-01

    textabstractIn chapter I the currently known causes for nonallergic noninfectious rhinitis and possible treatments are summarised. Also possible pathophysiological mechanisms underlying idiopathic rhinitis (IR) are discussed. In chapter II the aims of the studies are presented. This thesis comprises studies aimed at the therapeutic potential and safety of intranasal capsaicin in IR patients, developing a patient and physician friendly, effective intranasal capsaicin treatment regimen for IR a...

  19. Objective cough frequency in Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Key, Angela L; Holt, Kimberley; Hamilton, Andrew; Smith, Jaclyn A; Earis, John E

    2010-01-01

    Background Cough is a common presenting symptom in patients with Idiopathic Pulmonary Fibrosis (IPF). This study measured cough rates in IPF patients and investigated the association between cough and measures of health related quality of life and subjective cough assessments. In addition, IPF cough rates were related to measures of physiological disease severity and compared to cough rates in health and other respiratory conditions. Methods Nineteen IPF patients, mean age 70.8 years ± 8.6, f...

  20. Peripheral Blood Biomarkers in Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Vij, Rekha; Noth, Imre

    2012-01-01

    In this article, we review the evidence for peripheral blood biomarkers in idiopathic pulmonary fibrosis (IPF), a life-threatening fibrotic lung disease of unknown etiology. We focus on selected biomarkers present in peripheral blood, as they are easy to obtain, can be measured longitudinally, and have the greatest likelihood of achieving clinical utility. This article concentrates on biomarkers with mechanistic plausibility that may be directly involved in the development of IPF, including K...

  1. Relieving idiopathic dental pain without drugs

    OpenAIRE

    Haryono Utomo; M. Rulianto

    2011-01-01

    Background: Teeth are commonly obvious source of orofacial pain. Sometimes the pain source is undetectable, thus called as idiopathic dental pain. Since dentist wants to alleviate or eliminate the pains with every effort in their mind, a lot of drugs could be prescribed. Moreover, it is make sense that endodontic treatment or even tooth extraction will be done. Unfortunately, endodontic treatment may also initiate neuropathic tooth pain that is caused by nerve extirpation, thus worsen the pai...

  2. Current Treatments for Juvenile Idiopathic Arthritis

    OpenAIRE

    AlbertoMartini

    2011-01-01

    Juvenile idiopathic arthritis (JIA) differs markedly from adult rheumatoid arthritis (RA). It is not a single disease, but an exclusion diagnosis that gather together all forms of arthritis that begin before the age of 16 years, persist for more than 6 weeks, and are of unknown origin. The advent of the new biological treatments has dramatically changed both the observed responses to treatment and the expectations of therapies. The implementation of an adequate legislation as well as the ...

  3. Clomiphene Effects on Idiopathic Premature Ejaculation

    OpenAIRE

    Ketabchi

    2015-01-01

    Background Premature ejaculation (PE) is the inability to delay ejaculation, occurring sooner than they or their partner would like during sexual activities. PE is a challenging problem that can affect sexual enjoyment and may harm relationships of couples and affect their quality of life. In idiopathic PE, several helpful techniques and medicines are recommended, but none of them has yielded satisfactory results. Objectives Our o...

  4. The joints in juvenile idiopathic arthritis

    OpenAIRE

    Ording Muller, Lil-Sofie; Humphries, Paul; Rosendahl, Karen

    2015-01-01

    Abstract Juvenile idiopathic arthritis is the most common rheumatic entity in childhood. Imaging has become an important supplement to the clinical assessment of children with JIA. Radiographs still play an important role in the workup, and long-term follow-up in children with JIA, but are not sensitive to findings in the early disease stage. Both ultrasound and MRI are more sensitive to inflammatory changes than clinical assessment alone. However, the differentiation between normal findings ...

  5. Pulmonary Function Testing in Idiopathic Interstitial Pneumonias

    OpenAIRE

    Martinez, Fernando J; Flaherty, Kevin

    2006-01-01

    Diffuse parenchymal lung diseases are a group of disorders that involve the space between the epithelial and endothelial basement membranes and are generally segregated into four major categories. These include the idiopathic interstitial pneumonias, which are further categorized into seven clinical/radiologic/pathologic subsets. These disorders generally share a common pattern of physiologic abnormality characterized by a restrictive ventilatory defect and reduced diffusing capacity (DLCO). ...

  6. Renal involvement in idiopathic hypereosinophic syndrome

    OpenAIRE

    Shehwaro, Nathalie; Langlois, Anne Lyse; Gueutin, Victor; Izzedine, Hassane

    2013-01-01

    The hypereosinophilic syndromes (HESs) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. In idiopathic HES, the underlying cause of hypereosinophilia (HE) remains unknown despite thorough aetiological work-up. Kidney disease is thought to be rare in HES. Renal manifestations described include eosinophilic interstitial nephritis, various types of glomerulopathies, thrombotic m...

  7. Managing comorbidities in idiopathic pulmonary fibrosis

    OpenAIRE

    Fulton BG; Ryerson CJ

    2015-01-01

    Blair G Fulton,1 Christopher J Ryerson1,2 1Department of Medicine, 2Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada Abstract: Major risk factors for idiopathic pulmonary fibrosis (IPF) include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature rega...

  8. Pulmonary function in children with idiopathic scoliosis

    OpenAIRE

    Tsiligiannis Theofanis; Grivas Theodoros

    2012-01-01

    Abstract Idiopathic scoliosis, a common disorder of lateral displacement and rotation of vertebral bodies during periods of rapid somatic growth, has many effects on respiratory function. Scoliosis results in a restrictive lung disease with a multifactorial decrease in lung volumes, displaces the intrathoracic organs, impedes on the movement of ribs and affects the mechanics of the respiratory muscles. Scoliosis decreases the chest wall as well as the lung compliance and results in increased ...

  9. American Thoracic Society-European Respiratory Society Classification of the Idiopathic Interstitial Pneumonias: Advances in Knowledge since 2002.

    Science.gov (United States)

    Sverzellati, Nicola; Lynch, David A; Hansell, David M; Johkoh, Takeshi; King, Talmadge E; Travis, William D

    2015-01-01

    In the updated American Thoracic Society-European Respiratory Society classification of the idiopathic interstitial pneumonias (IIPs), the major entities have been preserved and grouped into (a) "chronic fibrosing IIPs" (idiopathic pulmonary fibrosis and idiopathic nonspecific interstitial pneumonia), (b) "smoking-related IIPs" (respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia), (c) "acute or subacute IIPs" (cryptogenic organizing pneumonia and acute interstitial pneumonia), and (d) "rare IIPs" (lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis). Furthermore, it has been acknowledged that a final diagnosis is not always achievable, and the category "unclassifiable IIP" has been proposed. The diagnostic interpretation of the IIPs is often challenging because other diseases with a known etiology (most notably, connective tissue disease and hypersensitivity pneumonitis) may show similar morphologic patterns. Indeed, more emphasis has been given to the integration of clinical, computed tomographic (CT), and pathologic findings for multidisciplinary diagnosis. Typical CT-based morphologic patterns are associated with the IIPs, and radiologists play an important role in diagnosis and characterization. Optimal CT quality and a systematic approach are both pivotal for evaluation of IIP. Interobserver variation for the various patterns encountered in the IIPs is an issue. It is important for radiologists to understand the longitudinal behavior of IIPs at serial CT examinations, especially for providing a framework for cases that are unclassifiable or in which a histologic diagnosis cannot be obtained.

  10. Idiopathic intracranial hypertension in pediatric patients

    Directory of Open Access Journals (Sweden)

    Nada Jirásková

    2008-11-01

    Full Text Available Nada Jirásková, Pavel RozsívalDepartment of Ophthalmology, University Hospital, Hradec Králové, Czech RepublicPurpose: To evaluate retrospectively the features, treatment, and outcome of idiopathic intracranial hypertension (IIH in children.Methods: Nine patients, 15 years and younger, diagnosed with IIH. Inclusion criteria were papilledema, normal brain computer tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 250 mm H2O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy.Results: Of the nine patients, eight were girls. Five girls were overweight and one boy was obese. The most common presenting symptom was headache (5 patients. Diplopia or strabismus did not occur in our group. Visual field abnormalities were present in all eyes, and severe visual loss resulting in light perception vision occurred in both eyes of one patient. Eight patients were treated medically with acetazolamide alone, and one girl needed a combination of acetazolamide and corticosteroids. This girl also required optic nerve sheath decompression surgery. Resolution of papilledema and recovery of visual function occurred in all patients.Conclusions: Idiopathic intracranial hypertension in prepubertal children is rather uncommon. Prompt diagnosis and management are important to prevent permanent visual loss.Keywords: idiopathic intracranial hypertension, pediatric, treatment

  11. Clomiphene Effects on Idiopathic Premature Ejaculation

    Directory of Open Access Journals (Sweden)

    Ketabchi

    2015-09-01

    Full Text Available Background Premature ejaculation (PE is the inability to delay ejaculation, occurring sooner than they or their partner would like during sexual activities. PE is a challenging problem that can affect sexual enjoyment and may harm relationships of couples and affect their quality of life. In idiopathic PE, several helpful techniques and medicines are recommended, but none of them has yielded satisfactory results. Objectives Our objective in this study was to evaluate the efficacy and safety of clomiphene as a selective estrogen receptor modulator on the treatment of idiopathic PE. Patients and Methods In a randomized clinical trial, 178 married men with idiopathic PE defined according to the Diagnostic and Statistical Manual of Mental Disorders Third Revised Version (DSM-III-R who referred to urology clinics over a 10-month period in 2012 were randomized into two groups, namely the study (clomiphene and control (placebo groups. They completed self-administered questionnaires that included intravaginal ejaculatory latency time (IELT, erectile dysfunction indexes, quality of life (QOL, sociodemographic characteristics, lifestyle, and medical illness. After 6 months of intervention, all data were compared with the baseline data and between the groups. Results Within the 10-month study course, 126 patients (70.8% completed this study. After intervention and comparison of the results between the two groups, IELT, sexual indexes, and QOL improved in the study group, but significant differences were observed only in the IELT and QOL findings. Conclusions Clomiphene seems to be useful in the pharmacological treatment of PE compared to the placebo.

  12. Pulmonary function in children with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Tsiligiannis Theofanis

    2012-03-01

    Full Text Available Abstract Idiopathic scoliosis, a common disorder of lateral displacement and rotation of vertebral bodies during periods of rapid somatic growth, has many effects on respiratory function. Scoliosis results in a restrictive lung disease with a multifactorial decrease in lung volumes, displaces the intrathoracic organs, impedes on the movement of ribs and affects the mechanics of the respiratory muscles. Scoliosis decreases the chest wall as well as the lung compliance and results in increased work of breathing at rest, during exercise and sleep. Pulmonary hypertension and respiratory failure may develop in severe disease. In this review the epidemiological and anatomical aspects of idiopathic scoliosis are noted, the pathophysiology and effects of idiopathic scoliosis on respiratory function are described, the pulmonary function testing including lung volumes, respiratory flow rates and airway resistance, chest wall movements, regional ventilation and perfusion, blood gases, response to exercise and sleep studies are presented. Preoperative pulmonary function testing required, as well as the effects of various surgical approaches on respiratory function are also discussed.

  13. Chronic Diarrhea

    Science.gov (United States)

    ... infections that cause chronic diarrhea be prevented? Chronic Diarrhea What is chronic diarrhea? Diarrhea that lasts for more than 2-4 ... represent a life-threatening illness. What causes chronic diarrhea? Chronic diarrhea has many different causes; these causes ...

  14. Hereditary chronic pancreatitis

    Directory of Open Access Journals (Sweden)

    Mössner Joachim

    2007-01-01

    Full Text Available Abstract Hereditary chronic pancreatitis (HCP is a very rare form of early onset chronic pancreatitis. With the exception of the young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic chronic pancreatitis. As well, diagnostic criteria and treatment of HCP resemble that of chronic pancreatitis of other causes. The clinical presentation is highly variable and includes chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, most patients have a mild disease. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in chronic pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes, such as the anionic trypsinogen (PRSS2, the serine protease inhibitor, Kazal type 1 (SPINK1 and the cystic fibrosis transmembrane conductance regulator (CFTR have been found to be associated with chronic pancreatitis (idiopathic and hereditary as well. Genetic testing should only be performed in carefully selected patients by direct DNA sequencing and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. Pancreatic cancer risk is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.

  15. Comparison of Efficacy and Dermatology Life Quality Index of Pharmacological Monotherapy and Combined Therapy in Patients with Chronic Idiopathic Urticaria%抗组胺药不同用药方法治疗慢性特发性荨麻疹疗效及患者生活质量

    Institute of Scientific and Technical Information of China (English)

    陈嫚轩; 刘玲玲

    2011-01-01

    目的:比较抗组胺药单一用药、联合用药2种方法治疗慢性特发性荨麻疹(CIU)的疗效及对患者生活质量改善的影响.方法:收集本院门诊确诊为CIU患者161例,对其临床资料进行回顾性分析.将患者分为抗组胺药单一用药组和联合用药组.疗程均为4周,治疗后第2、4周进行随访.应用荨麻疹活动性评分(UAS)标准按0-3级进行症状、体征评分,并根据症状、体征积分下降指数进行整体疗效评估;利用皮肤病生活质量指数(DLQI)调查问卷评价患者生活质量改善情况.结果:161例CIU患者中单一用药组107例,联合用药组54例;治疗4周结束时,联合用药组有效率为72.3%,单一用药组为72.9%,2组差异无统计学意义(X2=0.008,P=0.928);联合用药组治愈率为51.9%,高于单一用药组的40.2%,2组差异无统计学意义(X2=1.98,P=0.159);UAS评分,2组差异无统计学意义(P>0.05);治疗后2组DLQI评分均下降,联合用药组DLQI评分改善优于单一用药组,2组差异无统计学意义(P=0.052).结论:非镇静类H1受体拮抗剂单一用药及联合用药治疗慢性特发性荨麻疹的疗效相似,联合用药治愈率较高,控制及缓解症状的持久性相对更好,生活质量改善较为明显.%Objective To compare the clinical efficacy and the improvement of quality of life between the two treatment groups by using non-sedative Hl-antihistamine agents as monotherapy and combined therapy in chronic idiopathic urticaria (CIU) patients. Methods 161 CIU patients with comprehensive clinical data diagnosed in outpatient were collected. Clinical data of patients were retrospectively analyzed. The 161 patients were divided in two treatment groups, monotherapy group, only using one kind of non-sedative Hl-antihistamine agent (QD) and combined therapy group, combined using one kind of non-sedative Hl-antihistamine agent ( QD) with other medication. The duration of therapy was four weeks. Patients were evaluated before

  16. The role of helicobacter pylori infection in the pathogenesis of chronic urticaria

    International Nuclear Information System (INIS)

    Objective: To determine the prevalence of H. pylori infection in patients with idiopathic chronic urticaria (ICU) and to see if eradication of the bacterium affects the course of the urticaria. Patients and Methods: One hundred patients with idiopathic chronic urticaria and 43 healthy subjects (matched for age and sex) underwent serological testing for H. pylori infection. All patients with idiopathic chronic urticaria were examined for Helicobacter pylori infection with the /sup 13/C-urea test as well as the serological testing. Gastric biopsy was obtained from 36 patients. Patients with proven Helicobacter pylori infection were given treatment for 2 weeks. Six weeks afterwards they were tested again for Helicobacter pylori infection, and their urticaria was clinically assessed. Results: There was no significant difference in the seroprevalence of H. pylori infection between : idiopathic chronic urticaria patients and healthy subjects. Helicobacter pylori was detected in 76% of patients and 69.8% of controls. Out of the 76 patients treated, only 24 showed complete remission of their urticaria after successfully eradicating Helicobacter pylori infection, the others only having some improvement in their symptoms. Conclusion: Patients with idiopathic chronic urticaria have similar high rates of H. pylori infection as healthy subjects. Bacterium eradication is associated with improvement of urticaria symptoms, suggesting a possible role of Helicobacter pylori in the pathogenesis of this skin disorder. (author)

  17. Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

    Directory of Open Access Journals (Sweden)

    İlknur Tuğal-Tutkun

    2016-04-01

    Full Text Available Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual prognosis. The goal of treatment is to achieve enduring drug-free remission. The choice of therapeutic regimen needs to be tailored to each individual case. One must keep in mind that patients under immunomodulatory treatment should be monitored closely due to possible side effects. Local and systemic corticosteroids have long been the mainstay of therapy; however, long-term corticosteroid therapy should be avoided due to serious side effects. Steroid-sparing agents in the treatment of JIA-associated uveitis include antimetabolites and biologic agents in refractory cases. Among the various immunomodulatory agents, methotrexate is generally the first choice, as it has a well-established safety and efficacy profile in pediatric cases and does not appear to increase the risk of cancer. Other classic immunomodulators that may also be used in combination with methotrexate include azathioprine, mycophenolate mofetil, and cyclosporin A. Biologic agents, primarily tumor necrosis factor alpha inhibitors including infliximab or adalimumab, should be considered in cases of treatment failure with classic immunomodulatory agents.

  18. Current and novel drug therapies for idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Adamali HI

    2012-09-01

    Full Text Available Huzaifa I Adamali,1 Toby M Maher1–31Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK; 2National Heart and Lung Institute, Imperial College London, London, UK; 3Centre for Respiratory Research, University College London, London, UKAbstract: Over the past decade, there has been a cohesive effort from patients, physicians, clinical and basic scientists, and the pharmaceutical industry to find definitive treatments for idiopathic pulmonary fibrosis (IPF. As understanding of disease behavior and pathogenesis has improved, the aims of those treating IPF have shifted from reversing the disease to slowing or preventing progression of this chronic fibrotic illness. It is to be hoped that by slowing disease progression, survival will be improved from the current dismal median of 3.5 years following diagnosis. In Europe and Asia, a milestone has recently been reached with the licensing of the first IPF-specific drug, pirfenidone. This review assesses the current treatment modalities available for IPF, including pirfenidone. It also turns an eye to the future and discusses the growing number of promising compounds currently in development that it is hoped, in time, will make their way into the clinic as treatments for IPF.Keywords: interstitial lung disease, pirfenidone, clinical trials, usual interstitial pneumonia, acute exacerbations

  19. Idiopathic Pulmonary Fibrosis: Epidemiology, Clinical Features, Prognosis, and Management.

    Science.gov (United States)

    Lynch, Joseph P; Huynh, Richard H; Fishbein, Michael C; Saggar, Rajan; Belperio, John A; Weigt, S Sam

    2016-06-01

    Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic interstitial lung pneumonia associated with the histologic pattern of usual interstitial pneumonia (UIP). Although UIP is a distinct histologic lesion, this histologic pattern is not specific for IPF and can also be found in other diseases (e.g., connective tissue disease and asbestosis). Clinical features of IPF include progressive cough, dyspnea, restrictive ventilatory defect, and progressive fibrosis and destruction of the lung parenchyma. IPF is rare (13-42 cases/100,000), and primarily affects older adults (>50 years of age). The diagnosis of IPF often requires surgical lung biopsy, but the diagnosis can be affirmed with confidence in some patients provided the results of computed tomographic (CT) scans and clinical features are consistent. The clinical course is variable, but inexorable progression (typically over months to years) is typical. Mean survival from the onset of symptoms approximates 3 to 5 years. Medical treatment is only modestly effective, primarily by slowing the rate of disease progression. Lung transplantation is the best therapeutic option. PMID:27231859

  20. Unravelling the progressive pathophysiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    A. Günther

    2012-06-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF is a life-threatening condition, with a median survival of <3 yrs. The pathophysiology is not fully understood, but chronic injury of alveolar epithelial type II cells (AECII is considered key. In IPF, disturbed folding and processing of surfactant proteins and impaired DNA repair may represent underlying reasons for maladaptive endoplasmic reticulum stress responses, increased reactive oxygen species production and/or DNA damage. Excessive AECII apoptosis occurs, leading to permanently perturbed epithelial homeostasis. The role of secondary hits also becomes evident. These may aggravate the disease and result in increased epithelial turnover, exhausting the regenerative capacity of progenitors and disturbing epithelial–mesenchymal interactions. Fibroblast proliferation, transdifferentiation and matrix deposition may be mediated through various mechanisms including epithelial–mesenchymal transition, fibrocyte invasion or expansion of a local fibroblast population. Treatment modalities aiming to attenuate epithelial injury are currently in early pre-clinical development and may reach the clinical arena in only a few years. Meanwhile, novel drugs acting on highly activated fibroblasts such as pirfenidone, an anti-fibrotic drug authorised for IPF in the European Union, or BIBF 1120, a novel triple-kinase inhibitor (blocking vascular endothelial growth factor, platelet-derived growth factor and fibroblast growth factor currently under clinical investigation, seem to attenuate the progression of IPF.

  1. Abnormal glomerular basement membrane in idiopathic multicentric osteolysis

    NARCIS (Netherlands)

    Bakker, SJL; Vos, GD; Verschure, PDMM; Mulder, AH; Tiebosch, TMG

    1996-01-01

    The primary cause of nephropathy in idiopathic multicentric osteolysis is as yet unknown. We report a young girl with idiopathic multicentric osteolysis and nephropathy. An abnormal glomerular basement membrane was the only abnormality found in a renal biopsy taken 2 years before the development of

  2. Hyperoxaluria in idiopathic calcium nephrolithiasis--what are the limits?

    DEFF Research Database (Denmark)

    Osther, P J

    1999-01-01

    , and the syndrome of mild hyperoxaluric calcium nephrolithiasis could not be identified in our population of idiopathic stone formers. Hence, a limit of abnormal oxalate excretion that distinguishes an idiopathic stone former from a non-stone former could not be defined in our population. Therefore...

  3. Idiopathic scoliosis; a biomechanical and functional anatomical study.

    NARCIS (Netherlands)

    Veldhuizen, Albert Gerrit

    1985-01-01

    Although many types of causes of scoliosis are known, the idiopathic variety comprises the largest group and as its name indicates, its a etiology is unknown. Idiopathics coliosis develops in a previously normal vertebral column and has been recognized as an entity for more than a century.

  4. Idiopathic Sweet’s Syndrome Accompanying Bullous Lesions: Case report

    OpenAIRE

    Akı, Tuba; Karıncaoğlu, Yelda; Özcan, Hamdi; Seyhan, Muammer Eşrefoğlu; Kalaycı, Bülent; Karadağ, Neşe

    2004-01-01

    Sweet's syndrome is a rare dermatosis which is characterized by painful red plaques, pyrexia, elevated neutrophil count, and acellular infiltrate consisting predominantly of neutrophils that are diffusely distributed in the upper dermis. It may be either associated with upper respiratory tract infections, hematologic malignancies, drug hypersensitivities, sarcoidosis, autoimmune disorders or may be idiopathic. In this case an idiopathic SS that is associated with bullo...

  5. Perceived health status in self-reported adolescent idiopathic scoliosis

    DEFF Research Database (Denmark)

    Andersen, Mikkel Ø; Thomsen, Karsten; Kyvik, Kirsten O

    2010-01-01

    A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort.......A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort....

  6. What We Know About the Pathogenesis of Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Puglisi, S; Torrisi, S E; Giuliano, R; Vindigni, V; Vancheri, C

    2016-06-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease of unknown cause, occurring in adults, limited to the lungs and associated with the pathologic and radiologic pattern of usual interstitial pneumonia. Prognosis is poor, and most patients die of respiratory failure within 3 to 6 years from the onset of symptoms. Although our understanding of the pathogenesis of IPF has improved over the past two decades, the mechanisms responsible for this disorder have not been clearly defined. Aging is the single most important risk factor, but genetic, environmental, and diverse exogenous factors such as smoking, viral infections, chronic tissue injury (i.e., gastroesophageal reflux disease, traction injury) play contributory roles. In this review, we focus on pathogenetic mechanisms that we think are crucial for the initiation of the fibrotic process and for its progressive evolution. In the early stage of the disease, in the context of the permissive genetic background combined with the presence of specific risk factors, alveolar epithelial cells play a leading role. Subsequent evolution of the fibrotic process and its lethal progression is likely due to the abnormal tissue repair process that takes place in the lung and to the inability to counteract this process. In this phase of the disease, fibroblasts assume a crucial role. Current pharmacological treatment strategies for IPF have only modest value, principally by slowing the course of disease progression. Unfortunately, improvement or cure has not yet been achieved with pharmacological agents. The challenge for the future is to improve the comprehension of the mechanisms involved in the inception and evolution of IPF and their articulated interactions. This is fundamental not only to conceive and develop new drugs against this dreadful disease but also to apply different therapeutic approaches such as drug repositioning and personalized therapies in the management of IPF. PMID:27231860

  7. Idiopathic scoliosis: the tethered spine II: post-surgical pain.

    Science.gov (United States)

    Whyte Ferguson, Lucy

    2014-10-01

    The treatment of severe chronic pain in young people following surgery for the correction of curvatures of idiopathic scoliosis (IS) is presented through two case histories. Effective treatment involved release of myofascial trigger points (TrPs) known to refer pain into the spine, and treatment of related fascia and joint dysfunction. The TrPs found to be contributing to spinal area pain were located in muscles at some distance from the spine rather than in the paraspinal muscles. Referred pain from these TrPs apparently accounted for pain throughout the base of the neck and thoracolumbar spine. Exploratory surgery was considered for one patient to address pain following rod placement but the second surgery became unnecessary when the pain was controlled with treatment of the myofascial pain and joint dysfunction. The other individual had both scoliosis and hyperkyphosis, had undergone primary scoliosis surgery, and subsequently underwent a second surgery to remove hardware in an attempt to address her persistent pain following the initial surgery (and because of dislodged screws). The second surgery did not, however, reduce her pain. In both cases these individuals, with severe chronic pain following scoliosis corrective surgery, experienced a marked decrease of pain after myofascial treatment. As will be discussed below, despite the fact that a significant minority of individuals who have scoliosis corrective surgery are thought to require a second surgery, and despite the fact that pain is the most common reason leading to such revision surgery, myofascial pain syndrome (MPS) had apparently not previously been considered as a possible factor in their pain.

  8. Idiopathic scoliosis: the tethered spine II: post-surgical pain.

    Science.gov (United States)

    Whyte Ferguson, Lucy

    2014-10-01

    The treatment of severe chronic pain in young people following surgery for the correction of curvatures of idiopathic scoliosis (IS) is presented through two case histories. Effective treatment involved release of myofascial trigger points (TrPs) known to refer pain into the spine, and treatment of related fascia and joint dysfunction. The TrPs found to be contributing to spinal area pain were located in muscles at some distance from the spine rather than in the paraspinal muscles. Referred pain from these TrPs apparently accounted for pain throughout the base of the neck and thoracolumbar spine. Exploratory surgery was considered for one patient to address pain following rod placement but the second surgery became unnecessary when the pain was controlled with treatment of the myofascial pain and joint dysfunction. The other individual had both scoliosis and hyperkyphosis, had undergone primary scoliosis surgery, and subsequently underwent a second surgery to remove hardware in an attempt to address her persistent pain following the initial surgery (and because of dislodged screws). The second surgery did not, however, reduce her pain. In both cases these individuals, with severe chronic pain following scoliosis corrective surgery, experienced a marked decrease of pain after myofascial treatment. As will be discussed below, despite the fact that a significant minority of individuals who have scoliosis corrective surgery are thought to require a second surgery, and despite the fact that pain is the most common reason leading to such revision surgery, myofascial pain syndrome (MPS) had apparently not previously been considered as a possible factor in their pain. PMID:25440198

  9. The Role of Gender in Juvenile Idiopathic Arthritis-Associated Uveitis

    Directory of Open Access Journals (Sweden)

    Ahmadreza Moradi

    2014-01-01

    Full Text Available Uveitis is a common complication of juvenile idiopathic arthritis (JIA affecting up to 30% of patients with JIA. Although the typical bilateral chronic anterior uveitis associated with the persistent and extended oligoarticular and polyarticular, rheumatoid factor negative variants of JIA occurs predominantly in girls, boys may be more commonly affected in the HLA-B27 positive, enthesitis variant of JIA. While female gender has been associated with the development of the chronic anterior uveitis in children with JIA, the clinical course of JIA-associated uveitis may be worse in boys than in girls. The purpose of this paper is to review the available published literature to determine the role of gender in the clinical presentation and outcomes of patients with JIA-associated uveitis.

  10. Immunogenetics of juvenile idiopathic arthritis: A comprehensive review.

    Science.gov (United States)

    Hersh, Aimee O; Prahalad, Sampath

    2015-11-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthropathy of childhood. Juvenile idiopathic arthritis is believed to be a complex genetic trait influenced by both genetic and environmental factors. Twin and family studies suggest a substantial role for genetic factors in the predisposition to JIA. Describing the genetics is complicated by the heterogeneity of JIA; the International League of Associations for Rheumatology (ILAR) has defined seven categories of JIA based on distinct clinical and laboratory features. Utilizing a variety of techniques including candidate gene studies, the use of genotyping arrays such as Immunochip, and genome wide association studies (GWAS), both human leukocyte antigen (HLA) and non-HLA susceptibility loci associated with JIA have been described. Several of these polymorphisms (e.g. HLA class II, PTPN22, STAT4) are shared with other common autoimmune conditions; other novel polymorphisms that have been identified may be unique to JIA. Associations with oligoarticular and RF-negative polyarticular JIA are the best characterized. A strong association between HLA DRB1:11:03/04 and DRB1:08:01, and a protective effect of DRB1:15:01 have been described. HLA DPB1:02:01 has also been associated with oligoarticular and RF-negative polyarticular JIA. Besides PTPN22, STAT4 and PTPN2 variants, IL2, IL2RA, IL2RB, as well as IL6 and IL6R loci also harbor variants associated with oligoarticular and RF-negative polyarticular JIA. RF-positive polyarticular JIA is associated with many of the shared epitope encoding HLA DRB1 alleles, as well as PTPN22, STAT4 and TNFAIP3 variants. ERA is associated with HLA B27. Most other associations between JIA categories and HLA or non-HLA variants need confirmation. The formation of International Consortia to ascertain and analyze large cohorts of JIA categories, validation of reported findings in independent cohorts, and functional studies will enhance our understanding of the genetic

  11. Assessment of vascular function in systemic onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Sozeri, Betul; Atikan, Basak Yildiz; Ozdemir, Kadriye; Mir, Sevgi

    2016-07-01

    An increased incidence of cardiovascular disease has been found in rheumatic disorders. Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Prolonged immunological inflammatory process leads these patients to an early onset of atherosclerosis. We aimed to assess the presence of early vascular dysfunction in patients with systemic onset juvenile idiopathic arthritis (sJIA) and investigate the role of therapy sJIA in vascular health. Thirty-three patients (22 males, 11 females) diagnosed with sJIA according to the International League of Associations for Rheumatology criteria were compared to 72 age- and sex-matched controls. None of the participants was overweight, obese, or had a history of hypertension, dyslipidemia, diabetes mellitus, or cardiovascular disease. Arterial stiffness (As) was evaluated by measurement of carotid-femoral pulse wave velocity (PWV) and augmentation index (AIx) with a Vicorder. The mean age of patients in this study was 9.96 ± 3.71 years (range 4-16 years) and the mean age of controls was 10.9 ± 3.52 years (range 4-19 years). These two groups were well matched for age, sex, and BMI. The mean age of patients at the onset of disease was 7.06 ± 3.9 years (range 3-15 years). The mean duration of disease and active disease was 79 ± 45 months (range 6-162 months) and 58 ± 49 months (range 1-101 months), respectively. The highest levels of PWV and AIx were found in the patient group. Seven patients had had macrophage activation syndrome at presentation. In these patients, vascular changes were higher than other patients (6.30 ± 0.42 m/s vs 5.17 ± 0.55 m/s, p = 0.01, respectively). The corticosteroid therapy was found associated with higher PWV, (p < 0.05), while there was no difference between vascular parameters and use of non-steroid therapies (methotrexate (MTX), anti-TNF alfa agents). We also find statistically significant correlation between PWV

  12. Idiopathic Polyhydramnios: Severity and Perinatal Morbidity.

    Science.gov (United States)

    Wiegand, Samantha L; Beamon, Carmen J; Chescheir, Nancy C; Stamilio, David

    2016-06-01

    Objective To estimate the association between the severity of idiopathic polyhydramnios and adverse outcomes. Study Design Retrospective cohort study of deliveries at one hospital from 2000 to 2012 with an amniotic fluid index (AFI) measurement ≥24 + 0 weeks' gestation. Pregnancies complicated by diabetes, multiples, or fetal anomalies were excluded. Exposure was the degree of polyhydramnios: normal (AFI 5-24 cm), mild (≥ 24-30 cm), and moderate-severe (> 30 cm). Primary outcomes were perinatal mortality, neonatal intensive care unit (NICU) admission, and postpartum hemorrhage. Results There were 10,536 pregnancies: 10,188 with a normal AFI, 274 mild (78.74%), and 74 moderate-severe polyhydramnios (21.26%). Adverse outcomes were increased with idiopathic polyhydramnios: NICU admission (adjusted odds ratio [AOR] 3.71, 95% confidence interval [CI] 2.77-4.99), postpartum hemorrhage (AOR 15.81, 95% CI 7.82-31.96), macrosomia (AOR 3.41, 95% CI 2.61-4.47), low 5-minute Apgar score (AOR 2.60, 95% CI 1.57-4.30), and cesarean (AOR 2.16, 95% CI 1.74-2.69). There were increasing odds of macrosomia (mild: AOR 3.19, 95% CI 2.36-4.32; moderate-severe: AOR 4.44, 95% CI 2.53-7.79) and low 5-minute Apgar score (mild: AOR 2.24, 95% CI 1.23-4.08; moderate-severe: AOR 3.93, 95% CI 1.62-9.55) with increasing severity of polyhydramnios. Conclusion Idiopathic polyhydramnios is independently associated with increased risks of morbidity. There appears to be a dose-response relationship for neonatal macrosomia and low 5-minute Apgar score risks. PMID:26862725

  13. Idiopathic necrotizing sialometaplasia of parotid gland

    Directory of Open Access Journals (Sweden)

    Shailja Puri Wahal

    2013-01-01

    Full Text Available Necrotizing sialometaplasia (NS is an uncommon non-neoplastic, self-limiting inflammatory condition of the salivary glands. NS of major salivary glands is rare and simulates malignancy. If it is seen at this location, most of the cases are due to ischemia caused by vessel injury secondary to previous dental procedure or parotid gland surgery. We present a case of a parotid swelling that appeared as Warthin tumor on fine needle aspiration cytology (FNAC. On histology it turned out to be NS of parotid gland. The well known etiologies were absent in this case and hence it was labeled as idiopathic.

  14. Juvenile idiopathic arthritis: the paediatric perspective

    Energy Technology Data Exchange (ETDEWEB)

    Jordan, Alison [Birmingham Children' s Hospital, Department of Adolescent Rheumatology, Birmingham (United Kingdom); McDonagh, Janet E. [Birmingham Children' s Hospital, Institute of Child Health, Birmingham (United Kingdom)

    2006-08-15

    Paediatric rheumatology is a relatively new specialty that has developed rapidly over the last 30 years. There have been major advances, which have included improvements in the classification and management of juvenile idiopathic arthritis (JIA). The former has led to enhanced international collaboration with disease registries, multicentre research and the development of new therapeutic agents. This has resulted in improved disease control and remission induction in many. There is, however, still significant morbidity associated with JIA during childhood, adolescence and adulthood, and challenges for the future include early identification of those with a poorer prognosis, appropriate administration of safe therapies and optimizing outcomes as young people move through adolescence into adulthood. (orig.)

  15. Genetics of idiopathic generalized epilepsy: An overview

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    D. K. V. Prasad

    2013-01-01

    Full Text Available Idiopathic generalized epilepsy (IGE is a common type of epilepsy. Strong support for a genetic role in IGE comes from twin and family studies. Several subtypes of IGE have been reported but families often have members affected with different subtypes. Major advances have been made in the understanding of genetic basis of monogenic inherited epilepsies. However, most IGEs are complex genetic diseases and some susceptible IGE genes are shared across subtypes that determine subtypes in specific combinations. The high throughput technologies like deoxyribonucleic acid microarrays and sequencing technologies have the potential to identify causative genes or loci in non-familial cases.

  16. Idiopathic calcinosis cutis of nasal dorsum

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    Shrinath D.P Kamath

    2012-01-01

    Full Text Available A 23-year-old lady presented with a slowly progressing firm mass on the nasal dorsum since 8 months. Her biochemical, haematological and collagen vascular disease screening tests were normal. Radiographs of the nasal bones showed a subcutaneous calcifying lesion with no evidence of nasal bone erosion. A diagnosis of idiopathic calcinosis cutis (ICC was made. The mass was excised and soft tissue defect was augmented with silicone prosthesis. The histopathology with the haematoxylin and eosin staining and von Kossa stain confirmed the diagnosis of calcinosis cutis. This is an unusual presentation of ICC involving the nasal dorsum requiring surgery and nasal dorsal augmentation with silicone prosthesis.

  17. Idiopathic thoracic aortic aneurysm at pediatric age.

    Science.gov (United States)

    Marín-Manzano, E; González-de-Olano, D; Haurie-Girelli, J; Herráiz-Sarachaga, J I; Bermúdez-Cañete, R; Tamariz-Martel, A; Cuesta-Gimeno, C; Pérez-de-León, J

    2009-03-01

    A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

  18. Idiopathic lipoid pneumonia successfully treated with prednisolone.

    Science.gov (United States)

    Lococo, Filippo; Cesario, Alfredo; Porziella, Venanzio; Mulè, Antonino; Petrone, Gianluigi; Margaritora, Stefano; Granone, Pierluigi

    2012-01-01

    Lipoid pneumonia (LP) is a rare type of pneumonia that is radiologically characterized by lung infiltrates, although imaging alone may not be diagnostic. We describe an unusual 61-year-old patient with idiopathic LP presenting as a solitary pulmonary nodule mimicking lung cancer because of its rapid growth. After treatment with oral prednisone, a control chest radiogram indicated complete normalization of the radiologic features. This case shows that LP should be considered in the diagnostic assessment of any undefined pulmonary mass, after malignancy has been pathologically excluded. PMID:21419490

  19. MR imaging of idiopathic trigeminal neuralgia

    International Nuclear Information System (INIS)

    Thirty-four patients with idiopathic trigeminal neuralgia (ITN) were evaluated with MR imaging. Vascular contact at the proximal portion of the preganglionic segment (PGS) of the trigeminal nerve and deformity of the PGS was observed on the affected side in 97% and 62% of patients, respectively. Nonsurgical treatments were effective in 11 of the 13 patients without deformed PGS, but they failed to control trigeminal neuralgia in 8 of 21 patients with deformed PGS. This paper suggests that MR imaging could be used in the clinical assessment of trigeminal neuralgia before treatment

  20. Adolescent idiopathic scoliosis and back pain.

    Science.gov (United States)

    Balagué, Federico; Pellisé, Ferran

    2016-01-01

    This broad narrative review addresses the relationship between adolescent idiopathic scoliosis (AIS) and back pain. AIS can be responsible for low back pain, particularly major cases. However, a linear relationship between back pain and the magnitude of the deformity cannot be expected for any individual patient. A large number of juvenile patients can remain pain-free. The long-term prognosis is rather benign for many cases and thus a tailored approach to the individual patient seems mandatory. The level of evidence available does not allow stringent recommendations for any of the disorders included in this review. PMID:27648474

  1. Neocortical gamma oscillations in idiopathic generalized epilepsy

    DEFF Research Database (Denmark)

    Benedek, Krisztina; Berenyi, Antal; Gombkoto, Peter;

    2016-01-01

    Objective: Absence seizures in patients with idiopathic generalized epilepsy (IGE) may in part be explained by a decrease in phasic GABAA (type-A c-aminobutyric acid) receptor function, but the mechanisms are only partly understood. Here we studied the relation between ictal and interictal spike...... decrease in the 8-12 Hz frequency band. The rise in EEG gamma oscillations was short-lasting and decreased before activity declined at lower frequency ranges. Compared to control patients, patients with epilepsy also showed higher interictal values of mean coherence of gamma activity, but this interictal...

  2. Idiopathic esophagopleural fistula in the newborn

    Energy Technology Data Exchange (ETDEWEB)

    Iannaccone, G.; Cozzi, F.; Roggini, M.; Capocaccia, P.

    1982-07-01

    Idiopathic rupture of the esophagus in the neonate is a rare event, probably related to the same mechanism of ischemic necrosis responsible for other 'spontaneous' g.i. tract perforations in the newborn. The laceration is usually located on the right aspect of the distal esophagus and is complicated by esophagopleural fistula and hydropneumothorax. Plain chest film and esophagography are diagnostic. The condition is an emergency one and usually carries a bad prognosis without prompt surgical repair. A typical case is reported in a baby who survived without early surgery; a residual tiny blind pouch and a small hiatal hernia required surgery at 1 year of age.

  3. Renal involvement in idiopathic hypereosinophic syndrome

    Science.gov (United States)

    Shehwaro, Nathalie; Langlois, Anne Lyse; Gueutin, Victor; Izzedine, Hassane

    2013-01-01

    The hypereosinophilic syndromes (HESs) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. In idiopathic HES, the underlying cause of hypereosinophilia (HE) remains unknown despite thorough aetiological work-up. Kidney disease is thought to be rare in HES. Renal manifestations described include eosinophilic interstitial nephritis, various types of glomerulopathies, thrombotic microangiopathy (TMA) and electrolyte disturbances. The diagnosis must be made in time, because a recovery of renal function can be obtained if treatment is initiated promptly. PMID:26064485

  4. Autonomic symptoms in idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Ferini-Strambi, Luigi; Oertel, Wolfgang; Dauvilliers, Yves;

    2014-01-01

    Patients with idiopathic REM sleep behavior disorder (iRBD) are at very high risk of developing neurodegenerative synucleinopathies, which are disorders with prominent autonomic dysfunction. Several studies have documented autonomic dysfunction in iRBD, but large-scale assessment of autonomic...... symptoms has never been systematically performed. Patients with polysomnography-confirmed iRBD (318 cases) and controls (137 healthy volunteers and 181 sleep center controls with sleep diagnoses other than RBD) were recruited from 13 neurological centers in 10 countries from 2008 to 2011. A validated scale...

  5. Role of adalimumab in the management of children and adolescents with juvenile idiopathic arthritis and other rheumatic conditions

    Directory of Open Access Journals (Sweden)

    Marzan KA

    2012-06-01

    Full Text Available Katherine Anne B MarzanDivision of Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA, USAAbstract: Treatment of children and adolescents with juvenile idiopathic arthritis and other pediatric rheumatic diseases has evolved. Where once there was only a limited arsenal of medications, with significant side effects and inadequate efficacy, today, with an increased understanding of the pathogenesis of these diseases, there is a wider variety of more targeted and effective treatments. TNF-α is a cytokine involved in a number of inflammatory pathways in pediatric rheumatic diseases. The emergence of biologic modifiers that target TNF-α has been pivotal in providing the ability to deliver early and aggressive treatment. Adalimumab, a recombinant monoclonal antibody to TNF-α, is an important therapeutic option, which affords children and adolescents with chronic illnesses an improved quality of life.Keywords: adalimumab, juvenile idiopathic arthritis, TNF-α, pediatric, rheumatic diseases, treatment

  6. Staphylococcus aureus septicemia presenting as disseminated intravascular coagulation - thrombotic thrombocytopenic purpura overlap and thrombus in inferior vena cava, right atrium and right ventricle: a case report

    Directory of Open Access Journals (Sweden)

    Khwaja Saifullah Zafar

    2015-01-01

    Full Text Available Staphylococcal sepsis following furunculosis and complicated by suspected deep vein thrombosis and septic inferior vena caval, right atrium, right ventricle emboli accompanied by disseminated intravascular coagulation (DIC - thrombotic thrombocytopenic overlap in a 65 years old lady is presented. She was managed successfully with antibiotics and anticoagulation. The case is reported for its rarity and brings to light the vivid manifestations of septicemia specially staphylococcal. [Int J Res Med Sci 2015; 3(1.000: 368-372

  7. Refractory thrombotic thrombocytopenic purpura associated with oral contraceptives and factor V Leiden: a case report

    Science.gov (United States)

    Tsirakis, George; Mantadakis, Elpis; Xylouri, Irini; Foudoulakis, Andreas; Vardaki, Eleftheria; Katsipi, Irene; Daphnis, Eugene; Samonis, George

    2009-01-01

    Introduction Thrombotic microangiopathies constitute a heterogeneous group of diseases characterised by microangiopathic haemolytic anaemia and thrombocytopaenia associated with platelet aggregation in the microcirculation responsible for ischaemic manifestations. Classically, thrombotic microangiopathies are described as encompassing two main syndromes: thrombotic thrombocytopaenic purpura and the haemolytic-uraemic syndrome Many cases of idiopathic thrombotic thrombocytopaenic purpura have, to date, been associated with severe ADAMTS13 metalloprotease deficiency while haemolytic uraemic syndrome usually occurs in the context of normal protease activity. Oestrogens and factor V Leiden have rarely been implicated in the pathogenesis of thrombotic microangiopathy. Case presentation We describe the case of a 17-year-old female with refractory thrombotic thrombocytopaenic purpura. The patient was receiving a new generation of oral contraceptives for dysmenorrhoea and had factor V Leiden. After undergoing prolonged and intense plasma exchange therapy for 40 days and high dose oral corticosteroids therapy for 90 days, our patient recovered fully. Conclusion Patients with refractory thrombotic thrombocytopaenic purpura should likely be evaluated for congenital thrombophilic disorders and for ingestion of drugs that have been associated with this rare form of thrombotic microangiopathy. Identification of these and as yet other unknown genetic and/or acquired risk factors may lead to more judicious treatment approaches. PMID:19829833

  8. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon

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    Legakis Nikolaos

    2006-02-01

    Full Text Available Abstract Background Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. Case presentation A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Conclusion Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  9. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    Science.gov (United States)

    Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

    2016-01-01

    Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

  10. RADIOFREQUENCY ABLATION OF IDIOPATHIC RIGHT VENTRICULAR TACHYCARDIA

    Institute of Scientific and Technical Information of China (English)

    华伟; JituVohra

    1998-01-01

    This paper presents our experieaee with radioreqencey ablation (RFA) for idiopathic ventricular tschycardia (VT) arising from right ventricle in 12 patients(pts). The age range d patients was 21~50, with a mean of 38. 5 years. Ten out of 12 were females, 1 patient had eandia failure due to almost incessant VT while the rest had normal left ventricular function.Twelve pts had VT arising from the fight ventricle; of those, 9 were from the outflow truct, 2 from the RV apex, and l from the RV inflow. In all tats the diagnostic study and therapeutic RFA were combined in a single procedure, pacemapping and local aetlvition time were used to guide the site of RFA in Ors with VT arising froth the tight ventricle.RFA was successful in 11 of the 12 pts ( 91%). Ntmaher of RF applications were 1~27, mean 9. 6; fluoroscopy time were 4~75, mean 26. 9 minutes. RFA for idiopathic RV has a high success rate. This mode of treament should be considered as a nonphartaaeologieal curative treatment for symptomatic pts.

  11. Idiopathic spontaneous pneumomediastinum: radiologic and clinical features

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Young; Kim, Su Young; Kim, Yong Hoon; Hwang, Yoon Joon; Seo, Jung Wook; Han, Yoon Hee; Cha, Soon Joo; Hur, Gham [College of Medicine, Inje Univ., Goyang (Korea, Republic of)

    2004-07-01

    To evaluate the clinical presentations, radiological characteristics, and natural history of healthy adolescents presenting with idiopathic spontaneous pneumomediastinum. We retrospectively reviewed the simple radiographs of 14 consecutive patients (11 males) with spontaneous pneumomediastinum, who were examined over a period of 8 years, and analyzed their clinical history, radiographic findings including distribution, combined subcutaneous emphysema, mediastinal widening, pneumothorax, pleural effusion, and resolving period on follow up chest radiographs. We also obtained CT images of 7 patients for the assessment of additional information. The most common complaint at the time of presentation was chest pain and chest discomfort (8/14), followed by neck discomfort (6/14). The chest radiograph was of diagnostic value in alI cases. The main distribution of the pneumomediastinum was cervical (14/14), upper lung (13/14) and lower lung (6/14). Combined subcutaneous emphysema was observed in 6 patients. However, there were no cases of mediastinal widening, pneumothorax or pleural effusion. Complete resolution of the pneumomediastinum on the radiograph was observed after 10 days (mean 5.6), following purely conservative treatment. There was no additional information on the CT images, as compared with that on the radiographs. Idiopathic spontaneous pneumomediastinum is a benign entity that usually goes undiagnosed, but which responds very weIl to conservative treatment.

  12. Idiopathic spontaneous pneumomediastinum: radiologic and clinical features

    International Nuclear Information System (INIS)

    To evaluate the clinical presentations, radiological characteristics, and natural history of healthy adolescents presenting with idiopathic spontaneous pneumomediastinum. We retrospectively reviewed the simple radiographs of 14 consecutive patients (11 males) with spontaneous pneumomediastinum, who were examined over a period of 8 years, and analyzed their clinical history, radiographic findings including distribution, combined subcutaneous emphysema, mediastinal widening, pneumothorax, pleural effusion, and resolving period on follow up chest radiographs. We also obtained CT images of 7 patients for the assessment of additional information. The most common complaint at the time of presentation was chest pain and chest discomfort (8/14), followed by neck discomfort (6/14). The chest radiograph was of diagnostic value in alI cases. The main distribution of the pneumomediastinum was cervical (14/14), upper lung (13/14) and lower lung (6/14). Combined subcutaneous emphysema was observed in 6 patients. However, there were no cases of mediastinal widening, pneumothorax or pleural effusion. Complete resolution of the pneumomediastinum on the radiograph was observed after 10 days (mean 5.6), following purely conservative treatment. There was no additional information on the CT images, as compared with that on the radiographs. Idiopathic spontaneous pneumomediastinum is a benign entity that usually goes undiagnosed, but which responds very weIl to conservative treatment

  13. Idiopathic Adrenal Hematoma Masquerading as Neoplasm

    Directory of Open Access Journals (Sweden)

    Kazuki Sasaki

    2012-04-01

    Full Text Available We report herein a case of idiopathic adrenal hematoma. A 59-year-old Japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity. The tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland. His chief complaint was weight loss of 8 kg over the previous 6 months. He had no past medical history and took no medications, including no anticoagulants. Laboratory data were almost normal except for a slight elevation of PIVKA-II. The origin of the tumor was found to be the adrenal gland, as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries. A fine needle biopsy of the lesion was unable to confirm the diagnosis. Open right adrenalectomy was performed. The histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue. In the absence of any obvious etiology, the diagnosis was idiopathic adrenal hematoma.

  14. Idiopathic juxtafoveolar retinal telangiectasis: A current review

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    Nowilaty Sawsan

    2010-01-01

    Full Text Available Idiopathic juxtafoveolar retinal telangiectasis (IJFT, also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities.

  15. Pediatric Idiopathic Intracranial Hypertension: A Case Report

    Directory of Open Access Journals (Sweden)

    Divya Nandwani, OD

    2015-08-01

    Full Text Available Background: Idiopathic intracranial hypertension (IIH presents differently in prepubertal children as compared to postpubertal children and adults. In adults, IIH is most common in obese women of child-bearing age. However, when occurring in prepubertal children, IIH has been shown to have no predilection for gender and does not correlate with obesity. This case report exemplifies the rare occurrence of IIH in a pediatric patient. Case Report: A five-year-old Hispanic female patient was seen at the University Eye Center for a longstanding hypotropia secondary to a likely congenital fourth nerve palsy with an exotropia component. After a strabismus surgery and a series of follow-up visits, bilateral swollen optic nerves were observed, and a prompt referral to the emergency room was made. Consequently, she was diagnosed with IIH. A treatment regimen of acetazolamide was initiated with subsequent improvement of the clinical presentation of increased intracranial pressure, confirmed by the reduction of swelling of her optic nerves. Conclusions: Bilateral swelling of the optic nerves can be an emergency, especially in children. It is important urgently to rule out causes for increased intracranial pressure. If treatment is significantly delayed, or if no treatment is given to a patient with this condition, loss of visual function may occur. In addition to reporting a rare circumstance of pediatric idiopathic intracranial hypertension, this case report serves to remind eye care practitioners of the importance of monitoring the ocular health of patients closely, even in young, healthy children.

  16. Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

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    Mercier Pierre

    2009-08-01

    Full Text Available Abstract Background Adolescent idiopathic scoliosis is characterized by a three-dimensional deviation of the vertebral column and its etiopathogenesis is unknown. Various factors cause idiopathic scoliosis, and among these a prominent role has been attributed to the vestibular system. While the deficits in sensorimotor transformations have been documented in idiopathic scoliosis patients, little attention has been devoted to their capacity to integrate vestibular information for cognitive processing for space perception. Seated idiopathic scoliosis patients and control subjects experienced rotations of different directions and amplitudes in the dark and produced saccades that would reproduce their perceived spatial characteristics of the rotations (vestibular condition. We also controlled for possible alteration of the oculomotor and vestibular systems by measuring the subject's accuracy in producing saccades towards memorized peripheral targets in absence of body rotation and the gain of their vestibulo-ocular reflex. Results Compared to healthy controls, the idiopathic scoliosis patients underestimated the amplitude of their rotations. Moreover, the results revealed that idiopathic scoliosis patients produced accurate saccades to memorized peripheral targets in absence of body rotation and that their vestibulo-ocular reflex gain did not differ from that of control participants. Conclusion Overall, results of the present study demonstrate that idiopathic scoliosis patients have an alteration in cognitive integration of vestibular signals. It is possible that severe spine deformity developed partly due to impaired vestibular information travelling from the cerebellum to the vestibular cortical network or alteration in the cortical mechanisms processing the vestibular signals.

  17. Idiopathic Calcium Nephrolithiasis And Hypercalciuria: The Role Of Genes

    Science.gov (United States)

    Gambaro, Giovanni; Abaterusso, Cataldo

    2007-04-01

    Idiopathic calcium nephrolithiasis and hypercalciuria are multifactorial disease conditions, the pathogenesis of which involves the interaction of environmental and individual factors. Data support a strong role of genes in the pathogenesis of these two conditions. Findings obtained in monogenic disorders characterized by renal calcium stones, and/or hypercalciuria, and/or nephrocalcinosis have proposed a number of genes as candidate genes in the pathogenesis of the common idiopathic calcium nephrolithiasis and hypercalciuria. The physiological role of these genes, and findings in monogenic disorders and idiopathic, multifactorial disorders will be presented.

  18. Infliximab for Idiopathic Deep Cutaneous Vasculitis Refractory to Cyclophosphamide

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    Marcelo Derbli Schafranski

    2010-01-01

    Full Text Available Cutaneous vasculitis can be classified as primary or idiopathic; or secondary, when it presents as a manifestation of connective tissue diseases, infections, drug reactions or malignancies. Although most of the idiopathic cases are self-limited and responsive to supportive measures and nonsteroidal anti-inflammatory drugs, potent immunosuppressants are sometimes required for the management of the refractory situations. Here we describe a case of a 32-year-old Caucasian female patient with history of idiopathic cutaneous deep vasculitis unresponsive to methotrexate, dapsone, and cyclophosphamide who was effectively treated with infliximab.

  19. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    BACKGROUND: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term card

  20. PReS-FINAL-2148: Rheumates@work a cognitive behavioural internet based intervention promoting physical activity in children with juvenile idiopathic arthritis: Preliminary results of a randomized clinical trail

    NARCIS (Netherlands)

    Bos, J; Armbrust, W.; Geertzen, J.; Sauer, P.; Dijkstra, P.; Van Brussel, M.; Cappon, J.; Lelieveld, O.

    2013-01-01

    Introduction: Juvenile Idiopathic Arthritis (JIA) is a chronic disease in which periods of active inflammation alternate with periods of inactive disease in an unpredictable way. Although impairments are most pronounced in children with disease activity, deficits like fatigue, decreased physical act