WorldWideScience

Sample records for chronic idiopathic thrombocytopenic

  1. 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.

  2. 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

  3. 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...

  4. 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

  5. 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.

  6. 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

  7. 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.

  8. Validity of a procedure to identify patients with chronic idiopathic thrombocytopenic purpura in the Danish National Registry of Patients

    Directory of Open Access Journals (Sweden)

    Katrine Edith Klith Heden

    2009-02-01

    Full Text Available Katrine Edith Klith Heden, Annette Østergaard Jensen, Dora Körmendiné Farkas, Mette NørgaardDepartment of Clinical Epidemiology, Aarhus University Hospital, DenmarkBackground: Administrative data may be useful for epidemiological studies of chronic idiopathic thrombocytopenic purpura (ITP. However, the quality of the recorded diagnoses needs evaluation.Aim: We evaluated the validity in predicting chronic ITP of the International Classifi cation of Diseases (ICD-10 diagnoses of ITP in the Danish National Registry of Patients (NRP.Methods: We used the NRP to identify patients with ITP, according to code D69.3, from January 1, 1996 to December 31, 2007. We defined chronic ITP as lasting longer than 6 months by including only patients with 2 or more hospital ITP diagnoses over longer than 6 months. We confirmed diagnoses by evaluating each candidate chronic ITP patient’s medical chart and estimating the positive predictive value (PPV and 95% confidence interval (CI of the recorded NRP diagnostic code.Results: We identified 513 patients with chronic ITP in the NRP. We were able to retrieve the charts of 439. After evaluation of the charts, 410 patients were deemed to have a valid diagnosis of chronic ITP, yielding a PPV of 0.93 (95% CI: 0.91–0.96.Conclusion: The validity of this procedure to identify chronic ITP patients was high. The NRP is valid for epidemiological studies of patients with chronic ITP.Keywords: validity, idiopathic thrombocytopenic purpura, National Registry of Patients

  9. Perioperative Care of a Patient with Refractory Idiopathic Thrombocytopenic Purpura Undergoing Total Knee Arthroplasty

    OpenAIRE

    Singhal, Rohit; Gudimetla, Veera; Stewart, Andrew; Luscombe, Karen L; Charalambous, Charalambos P.

    2012-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder leading to low platelet count and an increased risk of bleeding. Major joint replacement surgery in a patient with ITP can be associated with severe postoperative bleeding. We present our experience of perioperative management in a patient with severe refractory chronic idiopathic thrombocytopenic purpura who successfully underwent a cemented total knee replacement.

  10. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.

  13. Idiopathic thrombocytopenic purpura and MMR vaccine

    OpenAIRE

    Miller, E; Waight, P; Farrington, C.; Andrews, N.; Stowe, J; Taylor, B

    2001-01-01

    A CAUSAL ASSOCIATION BETWEEN MEASLES—mumps-rubella (MMR) vaccine and idiopathic thrombocytopenic purpura (ITP) was confirmed using immunisation/hospital admission record linkage. The absolute risk within six weeks of immunisation was 1 in 22 300 doses, with two of every three cases occurring in the six week post-immunisation period being caused by MMR. Children with ITP before MMR had no vaccine associated recurrences.



  14. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    Science.gov (United States)

    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 however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting. PMID:26819784

  15. 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...... however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of...... hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

  18. [The dynamic detection of cytokins under aplastic anemia and idiopathic thrombocytopenic purpura in the process of impact of hypoxic hypoxia].

    Science.gov (United States)

    Éralieva, M O

    2012-05-01

    The natural hypoxic hypoxia enhances the synthesis processes and decrease the concentration balance in cytokine rets in patients with depression of hematopoiesis. It is established that the patients with aplastic anemia and idiopathic thrombocytopenic purpura, in contrast with patients with acute leucosis, chronic myelolecosis and erythroid myeloma, have quite high values of IL-2, which can be involved in the organization of full-fledgee response to antigen stimuli. The application of hypoxic hypoxia as an additional method in treatment of aplastic anemia and idiopathic thrombocytopenic purpura has no side effects and contraindications. PMID:22834158

  19. Pregnancy outcomes in women with idiopathic thrombocytopenic purpura

    OpenAIRE

    Yassaee, Fakhrolmolouk; Eskandari, Roghieh; Amiri, Zohreh

    2012-01-01

    Background: Idiopathic thrombocytopenic purpura (ITP) is a disease that commonly affects women of reproductive age and is associated with maternal and fetal complications. Objective: The aim of the present study was to report the perinatal outcome in pregnant women with ITP. Materials and Methods: Twenty one pregnant women with ITP admitted in a teaching hospital in Tehran, from October 2008 to February 2010, were enrolled in this prospective historical cohort study; course and perinatal outc...

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

    Directory of Open Access Journals (Sweden)

    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.

  1. [Bronchiolitis obliterans with organizing pneumonia associated with idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    Presas, J L; Piriz, J; Serra, S L; Paz, E D; Allievi, A; Kartin, D; Olmedo, G

    1998-01-01

    We report a case of a 35 year-old woman with idiopathic thrombocytopenic purpura (ITP) who, under treatment with immunosuppressive drugs, developed bilateral interstitial pulmonary disease. Previously she had been splenectomized and treated with corticosteroids and cyclosporin. During the clinical course, the patient developed alterations of the hepatogram and presented a positive serology for Epstein-Barr virus. The lung biopsy showed the histologic pattern of obliterative bronchiolitis, interstitial inflammatory infiltration and intraalveolar pneumonia (BOOP). We could not find in the literature a previous report in which ITP was associated with BOOP. Of interest was the spontaneous remission of the pulmonary disease after suppression of cyclosporin and positive serology for Epstein-Barr virus. PMID:9674210

  2. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

    Directory of Open Access Journals (Sweden)

    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.

  3. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    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.

  4. 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

  5. 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

  6. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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...

  9. 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

  10. 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

  11. 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.

  12. Prognostic Factors of Response to Laparoscopic Splenectomy in Patients with Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Kwon, Hyuk-Chan; Moon, Chang Hoon; Cho, Young Rak; Kim, Min Chan; Kim, Kyeong Hee; Han, Jin Yeong; Lee, Young Ho; Oh, Sung Yong; Kim, Sung-Hyun; Kim, Jae-Seok; Kim, Hyo-Jin

    2005-01-01

    Laparoscopic splenectomy (LS) has become the treatment of choice for patients with idiopathic thrombocytopenic purpura (ITP) who do not respond to medical treatment. The aim of this study was to identify factors predictive of outcome after LS for ITP. From May 1997 to December 2002, we performed 30 LS on patients with ITP. A positive response was defined as a postoperative platelet count greater than 50,000/µL and no requirement for maintenance therapy. Chi-square testing was performed to det...

  13. 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.

  14. Spontaneous bilateral peripapillary, subhyaloid and vitreous hemorrhage with only minor platelet deficit in idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Wan-Wei L

    2014-01-01

    Full Text Available Loo Wan-Wei,1,2 Tuan-Jaffar Tengku-Norina,1 Ahmad-Alwi Azma-Azalina,1 Abdul-Ghani Zulkifli,1 Embong Zunaina21Department of Ophthalmology, Hospital Raja Perempuan Zainab II, Kota Bharu, 2Department of Ophthalmology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, MalaysiaAbstract: A 45-year-old female with underlying idiopathic thrombocytopenic purpura (ITP complained of acute onset of reduced vision and floaters, in both eyes, for 3 weeks. Visual acuity was 6/36 and 6/60 in the right eye and left eye, respectively. Ophthalmoscopy showed bilateral peripapillary, subhyaloid and vitreous hemorrhage. Hematological evaluation revealed moderate anemia (hemoglobin: 93 g/L and mild thrombocytopenia (platelets: 120×109/L. She was co-managed by a hematologist and ophthalmologists; she was treated medically. Follow-up care during the next 6 weeks revealed spontaneous, partially resolving hemorrhage, with improvement of visual acuity. The purpose of this case report is to highlight ophthalmic involvement of ITP in this patient, despite her only-mild thrombocytopenia, and her spontaneous recovery, despite her receiving only medical treatment.Keywords: idiopathic thrombocytopenic purpura, subhyaloid hemorrhage, vitreous hemorrhage

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

    Directory of Open Access Journals (Sweden)

    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

  16. 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

  17. Subcutaneous anti-D treatment of idiopathic thrombocytopenic purpura in children.

    Science.gov (United States)

    Kjaersgaard, Mimi; Edslev, Pernille Wendtland; Hasle, Henrik

    2009-12-15

    We investigated the effect of subcutaneous anti-D IgG as platelet enhancing therapy in children with idiopathic thrombocytopenic purpura (ITP). Twenty-three children were treated with subcutaneous anti-D 50 microg/kg. The median platelet count increased from 7 x 10(9) to 31 x 10(9)/L on day 3 (P < 0.01). The median decline in hemoglobin was 1.3 g/dl. Two children experienced minor fever and chills within 24 hr of treatment. Pain at the injection site was common but self-limiting with no effect on activity level. These results suggest subcutaneous anti-D IgG 50 microg/kg as an effective and well-tolerated treatment option in childhood ITP. PMID:19722275

  18. Using decision analysis techniques to deal with "unanswerable" questions in idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Klaassen, Robert

    2003-12-01

    Idiopathic thrombocytopenic purpura (ITP) is a common disorder with rare adverse outcomes. This makes it a particularly difficult area in which to undertake conventional studies. An alternative method for solving clinical questions is decision analysis, which is in essence a computer-assisted synthesis of the literature. Using the example of a newly diagnosed ITP patient, the author attempts to answer the question of whether a bone marrow aspirate (BMA) is required prior to starting steroids. Using decision analysis methodology, the author determines that BMA is not essential prior to starting steroids. More importantly, three variables critical to the decision-making process are determined: the risk of death from the BMA procedure, the altered chance of survival for a patient with acute lymphoblastic leukemia (ALL) inappropriately given steroids, and how sensitive the complete blood count is at determining the risk of ALL. This scenario demonstrates the value of decision analysis and lays the groundwork for future endeavors. PMID:14668643

  19. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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

  2. A Case of Non-Hodgkin's Lymphoma in Patient with Coombs' Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Park, So Yeon; Kim, Soyon; Kim, Eun Sil; Choi, Soon Uk; Hyun, Hee Jae; Ahn, Ju Young; Lee, Ju Hyoung; Ryu, Seo Hee; Park, Jae Hyun; Lee, Gyeong In; Lee, Hyo Jin

    2012-01-01

    Coombs' negative autoimmune hemolytic anemia (AIHA) is a rare disease which shares similar clinical and hematological features with Coombs' positive AIHA, but its exact frequency remains unknown. There have been few reports of idiopathic thrombocytopenic purpura (ITP) and Coombs' negative AIHA associated with other lymphoproliferative disorders (LPDs). Since there is a well known association between LPDs and autoimmune phenomena, it is important to investigate the possibility of an underlying...

  3. 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...

  4. Antidepressants in chronic idiopathic urticaria.

    Science.gov (United States)

    Yasharpour, Michelle R; Randhawa, Inderpal

    2011-01-01

    Chronic idiopathic urticaria (CIU) is a common disease estimated to affect 0.1% of the population and can be very difficult to treat. Many psychotropic medications have been reported to be successful in treating refractory CIU. The purpose of this article was to discuss the pathophysiology of chronic urticaria and provide practicing allergists and dermatologists alternative treatment options in the management of refractory CIU, especially in those who have concurrent psychiatric comorbidity. A review was performed of pertinent literature pertaining to the pathophysiology of CIU and the many psychotropic medications reportedly successful in disease management. Although more research is needed, this article serves to broaden the mind of the physician treating CIU. PMID:22221435

  5. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  7. Two cases of Vici syndrome associated with Idiopathic Thrombocytopenic Purpura (ITP) with a review of the literature.

    Science.gov (United States)

    Huenerberg, Katherine; Hudspeth, Michelle; Bergmann, Shayla; Pai, Shashidhar; Singh, Balvir; Duong, Angie

    2016-05-01

    Vici syndrome is a rare congenital disorder first described in 1988. To date, 31 cases have been reported in the literature. The characteristic features of this syndrome include: agenesis of the corpus callosum, albinism, cardiomyopathy, variable immunodeficiency, cataracts, and myopathy. We report two Hispanic sisters with genetically confirmed Vici syndrome who both developed Idiopathic Thrombocytopenic Purpura. To our knowledge, this is an immunologic process that has been previously undescribed within the phenotype of Vici syndrome and should be added to the spectrum of variable immune dysregulation that can be found in these patients. © 2016 Wiley Periodicals, Inc. PMID:26854214

  8. 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

  9. An infantile case of cytomegalovirus induced idiopathic thrombocytopenic purpura with predominant proliferation of CD10 positive lymphoblast in bone marrow.

    Science.gov (United States)

    Mizutani, K; Azuma, E; Komada, Y; Ito, M; Sakurai, M; Hironaka, T; Hirai, K

    1995-02-01

    An infant with cytomegalovirus infection (CMV) developed idiopathic thrombocytopenic purpura (ITP) at 4 months of age. A bone marrow (BM) aspiration showed a remarkable increase of immature megakaryocytes and prominent proliferation of lymphoblasts. Flow cytometric analysis of the bone marrow cells showed that the predominant cells in the lymphocyte cluster were of B-lineage (CD19) with CD10 (common acute lymphoblastic leukemia antigen) positive. Virus study showed a higher titer of CMV antibody. Cytomegalovirus DNA was detected by the polymerase chain reaction (PCR) method in urine, peripheral cells and marrow cells. Low-grade fever, diarrhea and petechiae were accompanied by mild liver dysfunction. Complete remission was made with intravenous high-dose immunoglobulin (IVIg) without progression to overt acute leukemia. The percentage of CD10+/CD19+ lymphocytes in bone marrow also diminished. We postulated that the proliferation of immature lymphocytes and megakaryocytes in bone marrow was caused by maturation arrest that might result from CMV infection. PMID:7754772

  10. Histochemical and biochemical observations of the spleen in atypical Niemann-Pick disease and in idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Safanda, J; Fakan, F

    1981-01-01

    In a case of adolescent Niemann-Pick disease (NP) and in a case of idiopathic thrombocytopenic purpura (ITP), the histologic picture of the spleen showed appreciable similarity in localization of sparing cells and in a number of histochemical tests. The sphingomyelin, which was the main organ phospholipid in both conditions, contained substantially elevated content of C24 fatty acids. Detailed analysis of spleen lipids showed great relative increase of lysobisphosphatidic acid and of cholesterol which was in NP mainly in free form but in ITP surprisingly mainly esterified, mostly to oleic and palmitic acid. Possible molecular mechanism of sphingomyelin storage was enzymologically followed in model conditions using separated lipid fractions from NP's spleen. The activity of sphingomyelinase (Cl. perfringens exotoxin) was in comparison to phospholipase C relatively specifically inhibited by lysobisphosphatidic acid. PMID:6168156

  11. Low-dose vincristine in the treatment of corticosteroid-refractory idiopathic thrombocytopenic purpura (ITP) in non-splenectomized patients.

    Science.gov (United States)

    Cervantes, F.; Montserrat, E.; Rozman, C.; Diumenjo, C.; Feliu, E.; Grañena, A.

    1980-01-01

    Eight non-splenectomized patients with corticosteroid-refractory idiopathic thrombocytopenic purpura (ITP) were treated with low-dose vincristine (1 mg/week up to a total dose of 4 mg). Complete remission was achieved in 2 cases and partial remission in 3. Bleeding stopped in one patient who failed to remit. No statistical relationship was found between the response to vincristine and the duration of the disease or the corticosteroid-therapy. Side effects were only observed in one patient. By comparing these results with those reported in the literature, it can be inferred that low-dose vincristine may be useful in the management of corticosteroid-refractory ITP. PMID:7194478

  12. 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

  13. Chronic idiopathic polyneuropathy treated with azathioprine.

    OpenAIRE

    Pentland, B; Adams, G G; Mawdsley, C

    1982-01-01

    The results of azathioprine therapy in five patients with chronic progressive or relapsing idiopathic inflammatory polyneuropathy are described. In four patients a sustained improvement followed treatment and in the other patient azathioprine successfully replaced corticosteroid therapy. The improvement was often delayed for up to three months. The literature on the use of azathioprine in chronic polyneuropathy is reviewed. We suggest that there is a place for azathioprine treatment in patien...

  14. 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)

  15. Levocetirizine for chronic idiopathic urticaria: a review.

    Science.gov (United States)

    Ducharme, Erin E; Weinberg, Jeffrey M

    2009-03-01

    Only recently available on the US market, levocetirizine has emerged as an effective new option in the treatment of chronic idiopathic urticaria (CIU). Levocetirizine is the active isomer of cetirizine and demonstrates twice the affinity for the H1 receptor compared with the parent compound. In the treatment of CIU, levocetirizine has consistently demonstrated high response rates, fast onset, and a favorable side effect profile. PMID:19271371

  16. Clinical significance of detecting soluble glycocalicin and thrombopoietin in the differential diagnosis of idiopathic thrombocytopenic purpura and aplstic anemia

    International Nuclear Information System (INIS)

    Objective: To investigate the clinical significance of detecting soluble platelet glycocalicin (sGC) and thrombopoietin (TPO) in the differential diagnosis of idiopathic thrombocytopenic purpura (ITP) and aplastic anemia (AA). Methods: Plasma sGC and serum TPO in 83 patients with ITP, 47 patients with AA and 50 normal individuals were detected by iminunoradiometric assay (IRMA) and enzymelinked immunosorbent assay (ELISA), respectively. Statistical analysis was performed using Q test and P value of 0.05). But serum TPO level in AA group was significantly higher than that in ITP and normal groups: (857.43 ± 228.43) ng/L vs (90.32 ± 39.43) ng/L and (70.29 ± 25.16) ng/L, and they were considered statistically significant (Q=24.45 and 18.25, both P < 0.01). Conclusion: Detecting plasma sGC and serum TPO might be helpful for differentiating ITP and AA and for understanding the pathophysiology of thrombocytopenia. (authors)

  17. Refractory chronic immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia.

    Science.gov (United States)

    Horino, Satoshi; Rikiishi, Takeshi; Niizuma, Hidetaka; Abe, Hiroshi; Watanabe, Yuko; Onuma, Masaei; Hoshi, Yoshiyuki; Sasahara, Yoji; Yoshinari, Miyako; Kazama, Takuro; Hayashi, Yutaka; Kumaki, Satoru; Tsuchiya, Shigeru

    2009-11-01

    Immune thrombocytopenic purpura (ITP) has been associated with several hematologic malignancies such as Hodgkin and non-Hodgkin lymphomas and chronic lymphocytic leukemia, but it is rare in children with acute lymphoblastic leukemia (ALL). Here, we report a 7-year-old girl with chronic ITP during early intensive phase of chemotherapy for ALL. She underwent splenectomy because thrombocytopenia had persisted even after treatment with intravenous immunoglobulin (IVIG), steroids, vincristine, rituximab, and anti-D antibody. After splenectomy, her platelet count had recovered, and maintenance therapy could be resumed with a support of IVIG. To our knowledge, this is the first child case of chronic ITP during chemotherapy for ALL and splenectomy was effective in this patient. PMID:19816666

  18. Chronic idiopathic urticaria: treatment with omalizumab.

    Science.gov (United States)

    Naaman, Sandra; Sussman, Gordon

    2014-01-01

    Chronic idiopathic urticaria (CIU) is a common autoimmune skin condition characterized by spontaneously recurring hives for 6 weeks or longer. The new terminology used for CIU in most countries including Canada is chronic spontaneous urticaria (CSU). CSU is associated with significant psychosocial morbidity with a markedly negative impact on overall quality of life. Conventional approaches with antihistamines, even at high doses, is effective in about 50% of patients suffering from CSU. A new treatment option, omalizumab, a humanized monoclonal antibody against the Fc domain of IgE, has undergone the scrutiny of randomized research studies evaluating the efficacy in CSU. This editorial reviews mechanisms of action of omalizumab, efficacy, cost and potential side effect profile. Omalizumab has emerged as a very promising treatment option for patients with CSU. Future research is necessary to establish standardized protocols related to dosing as well as monitoring possible adverse effects of long-term treatment. PMID:25807072

  19. 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)

  20. Chronic idiopathic urticaria and anxiety symptoms.

    Science.gov (United States)

    Barbosa, Filipe; Freitas, João; Barbosa, António

    2011-10-01

    Chronic idiopathic urticaria (CIU) is a frequently disabling disease with a negative influence on the quality of life, and can cause psychopathological symptoms, such as anxiety. Our aim is to study further anxiety symptoms on CIU patients. Both CIU patients and the control group were studied by means of validated scales for psychopathology symptoms, psychological variables and quality of life. In this study, we reported high levels of anxiety symptoms. We found statistically significant correlations between anxiety symptoms, some personality dimensions, insecure attachment styles, alexithymia and with some quality of life dimensions. CIU patients exhibit high levels of psychological distress that could potentiate difficulties at several domains, namely social, emotional, general health perception and interpersonal relationships. PMID:21459916

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

    DEFF Research Database (Denmark)

    Maurer, M; Bindslev-Jensen, C; Gimenez-Arnau, A; Godse, K; Grattan, C E M; Hide, M; Kaplan, A P; Makris, M; Simons, F E R; Zhao, Z; Zuberbier, T; Church, M K

    2013-01-01

    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 ...... idiopathic urticaria (CIU)....

  2. Chronic idiopathic urticaria and Graves' disease.

    Science.gov (United States)

    Ruggeri, R M; Imbesi, S; Saitta, S; Campennì, A; Cannavò, S; Trimarchi, F; Gangemi, S

    2013-01-01

    Chronic urticaria is a common condition characterized by recurrent episodes of mast cell-driven wheal and flare-type skin reactions lasting for more than 6 weeks. In about 75% of cases, the underlying causes remain unknown, and the term chronic idiopathic urticaria (CIU) is used to emphasize that wheals develop independently of identified external stimuli. Although CIU affects about 1.0% of the general population, its etiopathogenesis is not yet well understood. It is now widely accepted that in many cases CIU should be regarded as an autoimmune disorder caused by circulating and functionally active IgG autoantibodies specific for the IgE receptor (FceRI) present on mast cells and basophils or for IgE itself. The well-known association of CIU with other autoimmune processes/diseases represents further indirect evidence of its autoimmune origin. Autoimmune thyroid diseases, especially autoimmune thyroiditis, represent the most frequently investigated diseases in association with CIU. Here we review this topic with particular regard to the association between Graves' disease and CIU. The possible pathogenetic mechanisms and the clinical implications of such an association are discussed. PMID:23609949

  3. 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

  4. 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

  5. Chronic idiopathic urticaria: prevalence and clinical course.

    Science.gov (United States)

    Kulthanan, Kanokvalai; Jiamton, Sukhum; Thumpimukvatana, Narumol; Pinkaew, Sumruay

    2007-05-01

    The purpose of our study was to assess the prevalence and clinical course of patients with chronic idiopathic urticaria (CIU), as well as possible causes or associated findings, laboratory findings and the duration of the disease in patients with chronic urticaria (CU). We retrospectively reviewed the 450 case record forms of patients with CU and/or angioedema who attended the Department of Dermatology, Siriraj Hospital, during the period 2000-2004. Of 450 patients with CU, 337 patients (75%) were diagnosed as CIU. Forty-three patients (9.5%) had physical urticaria, while 17 patients (3.8%) had infectious causes. Other possible causes were food, thyroid diseases, atopy, drugs, dyspepsia and collagen vascular diseases. In eighty-nine percent of patients, no abnormalities were detected at the time of physical examination. The most common abnormal laboratory finding was minimal elevation of the erythrocyte sedimentary rate (42%). In 61 patients, autologous serum skin tests had been done. Fifteen patients (24.5%) had positive results i.e. autoimmune urticaria. Anti-thyroglobulin and anti-microsomal antibodies were positive in 16 % and 12% of CIU patients respectively. After 1 year from the onset of the symptoms, 34.5% of CIU patients were free of symptoms and after 1.2 years from the onset of the symptoms, 56.5% of autoimmune urticaria patients were free of symptoms. The median disease duration of CIU and autoimmune urticaria were 390 days and 450 days respectively. Our study provided an overview of CU and CIU in a large series of Thai patients, based on etiological aspects and clinical courses. PMID:17408437

  6. Azathioprine in chronic relapsing idiopathic polyneuropathy.

    OpenAIRE

    Pentland, B

    1980-01-01

    A 33-year-old housewife with a 14-year history of relapsing polyneuropathy of unknown cause who has apparently responded to azathioprine therapy is described. The place of this form of treatment in idiopathic polyneuropathy is discussed.

  7. 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.

  8. 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.

  9. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  11. 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

  12. [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

  13. Graves disease associated with chronic idiopathic urticaria: 2 case reports.

    Science.gov (United States)

    Bansal, A S; Hayman, G R

    2009-01-01

    Chronic idiopathic urticaria (CIU) is well known to be associated with antithyroid peroxidase antibodies and autoimmune thyroiditis. Coexisting Graves disease has only rarely been observed. We describe 2 patients with CIU who developed autoimmune hyperthyroidism with antithyrotropin receptor antibodies. Antithyroid peroxidase antibodies were also present in 1 of the patients, but both responded poorly to high-dose antihistamine therapy. Both patients improved significantly, and their thyroid function recovered with carbimazole. We advise clinicians to be alert to the symptoms of hyperthyroidism when patients with CIU respond poorly to antihistamine therapy, as prompt treatment of hyperthyroidism significantly improves urticaria. PMID:19274930

  14. New advances in the treatment of adult chronic immune thrombocytopenic purpura: role of thrombopoietin receptor-stimulating agents

    Directory of Open Access Journals (Sweden)

    Ara Metjian

    2009-12-01

    Full Text Available Ara Metjian1, Charles S Abrams21Department of Medicine, Duke University Medical Center, Durham, NC, USA; 2Department of Medicine, University of Pennsylvania, Philadelphia, PA, USAAbstract: Decades of basic science and clinical research have led to an increased understanding of the pathophysiology of immune thrombocytopenic purpura (ITP, the processes underlying thrombopoiesis, and the treatment of chronic ITP. Now, new agents are available to treat ITP in a nonimmunosuppressive fashion. Lessons learned from the clinical trials of recombinant human thrombopoietin (TPO have led to the development of a novel class of compounds: nonimmunogenic agonists of the thrombopoietin receptor. Representing the first nonimmunosuppressive agents to treat chronic refractory ITP in decades, medications such as romiplostim and eltrombopag were recently approved by the US Food and Drug Administration. These new agents offer physicians a new tool for treating difficult cases of ITP in their medical armamentarium. Additional TPO mimetics are also being developed that show promise in vitro, and await future development.Keywords: thrombocytopenia, immune-mediated thrombocytopenia, eltrombopag, romiplostim, thrombopoietin, thrombopoietin receptor

  15. 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.

  16. 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.

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

    OpenAIRE

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

    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).

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Cyclosporin-A efficacy in chronic idiopathic urticaria.

    Science.gov (United States)

    Di Leo, E; Nettis, E; Aloia, A M; Moschetta, M; Carbonara, M; Dammacco, F; Vacca, A

    2011-01-01

    Common drugs in the therapy of chronic idiopathic urticaria (CIU) include antihistamines alone or combined with corticosteroids, but severe unresponsive patients require alternative treatments. This retrospective study aims to evaluate clinical response and safety of low-dose and long-term oral Cyclosporin-A (CyA) in unresponsive patients. One hundred and ten CIU patients, unresponsive to a previous treatment (antihistamines plus prednisone 0.2 mg/kg/day), received additional oral CyA 1–3 mg/kg/day for 6 months. The patients were subdivided into three groups (A, B, C) according to the different CyA doses. Parameters of clinical efficacy including pruritus, and size and number of wheals were evaluated at baseline, after three and six months. All adverse events were recorded. The mean total symptom severity score decreased by 63% in Group A, 76% in Group B, and 85% in Group C after 6 months. Total disappearance of the symptoms was recorded in 43 patients (39.1%): 7 (28%) of Group A; 12 (37.5%) of Group B and 24 (45%) of Group C. After a mean of 2 months from CyA suspension, 14 patients (11%) had recurrence of symptoms. Minor side effects were noted in 8 patients (7%). Our study indicates that low-dose, long-term CyA therapy is efficacious and safe in severe unresponsive CIU. PMID:21496403

  20. Chronic idiopathic urticaria with functional autoantibodies: 12 years on.

    Science.gov (United States)

    Sabroe, R A; Greaves, M W

    2006-05-01

    It is now recognized that approximately one-third of patients with chronic idiopathic urticaria (CIU) have histamine-releasing autoantibodies directed against either the high-affinity IgE receptor or, less frequently, against IgE. However, there are several unsolved problems relating to the role of such autoantibodies in the disease. Additionally, it is not clear whether CIU with autoantibodies can be classified as an autoimmune disease. The detection of patients with autoantibodies also poses challenges. Firstly, the only in vivo method, the autologous serum skin test, is at best 80% sensitive and specific using in vitro basophil histamine release assays as the verum. Secondly, in vitro tests are only done in a small number of research laboratories, and are not widely commercially available, and thirdly, there is some divergence between results obtained by different methods (functional and immunoassays) used to detect patients with autoantibodies. The presence of autoantibodies may be important clinically in a small group of severely affected, treatment-resistant patients, where immunomodulatory treatments may be helpful. PMID:16634880

  1. The effect of Helicobacter pylori eradication on idiopathic chronic urticaria

    Directory of Open Access Journals (Sweden)

    Ali Reza Khalilian4

    2009-01-01

    Full Text Available (Received 14 April, 2009 ; Accepted 9 November, 2009AbstractBackground and purpose: Chronic idiopathic urticaria (CIU is a recurrent disorder that is characterized by itching wheels lasting more than 6 weeks. Etiology of CIU is unknown. The aim of this study is to assess the efficacy of Helicobacter pylori (Hp infection eradication in improvement of CIU.Materials and methods: In this double blind placebo-controlled clinical trial, 60 CIU patients were enrolled in the study. Urea breath test (UBT was performed for indicating the Hp infection. Hp positive patients were divided in 2 groups: cases that were treated with quadruple therapy for eradication of Hp(Amoxicillin, Omeprazole, Bismuth and Metronidazole and controls (placebo. The gastrointestinal (GI and dermatologic symptoms and signs were recorded and compared in the beginning of the study, and 1 and 3 months after the treatment.Results: From 60 CIU patients, 32 (53% were UBT positive. 15 cases and 13 controls completed the study. There were no significant statistical differences between the two groups for their GI and dermatologic complaints before intervention, and 1 and 3 months after eradication, except for pyrosis that was improved in cases after 3 months (P= 0.029Conclusion: Eradication of Hp does not have any effect on improving CIU symptoms or signs.J Mazand Univ Med Sci 2009; 19(72: 2-8 (Persian.

  2. 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...

  3. 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.

  4. 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

  5. Desloratadine for chronic idiopathic urticaria: a review of clinical efficacy.

    Science.gov (United States)

    DuBuske, Lawrence

    2007-01-01

    Chronic idiopathic urticaria (CIU) is a disabling affliction that considerably limits patients' daily activities and interferes with sleep. Clinical studies have shown that histamine H1-receptor antagonists (antihistamines) are highly effective for inhibiting the hives/wheals and pruritus associated with CIU, as well as improving patients' quality of life. Desloratadine is a rapid-acting, once-daily, nonsedating selective H1-receptor antagonist/inverse receptor agonist with proven clinical efficacy in patients with CIU. It has 10-20 times the in vivo H1 receptor-binding affinity of loratadine, its parent compound, and 52-194 times the H1 receptor-binding affinity of cetirizine, ebastine, loratadine, and fexofenadine. Desloratadine displays linear pharmacokinetics after oral administration. Age and sex have no apparent effect on the drug's metabolism and elimination, and food does not affect its bioavailability or absorption. Desloratadine also exerts anti-inflammatory effects via mechanisms that are independent of H1-receptor antagonism. Results from randomized, double-blind, placebo-controlled studies of 6 weeks' duration in adults and adolescents with moderate-to-severe CIU indicate that desloratadine significantly minimizes the severity of pruritus, reduces the number and size of hives, and improves disease-impaired sleep and daily activities. Improvements were noted after a single dose of desloratadine and were maintained over 6 weeks of treatment. Desloratadine was safe and well tolerated in clinical trials of patients with CIU. The adverse effect profile of desloratadine in adults, as well as in children aged from 6 months to 11 years, is comparable to that of placebo. Evaluations of cognitive and psychomotor performance in adults indicate no impairment of function with dosages of desloratadine 5 mg/day. In conclusion, desloratadine is an important therapeutic option for prompt and enduring symptom relief in patients with moderate-to-severe CIU. In addition

  6. Comparison of endogenous and radiolabeled bile acid excretion in patients with idiopathic chronic diarrhea

    Energy Technology Data Exchange (ETDEWEB)

    Schiller, L.R.; Bilhartz, L.E.; Santa Ana, C.A. (Baylor Univ. Medical Center, Dallas, TX (USA))

    1990-04-01

    Fecal recovery of radioactivity after ingestion of a bolus of radiolabeled bile acid is abnormally high in most patients with idiopathic chronic diarrhea. To evaluate the significance of this malabsorption, concurrent fecal excretion of both exogenous radiolabeled bile acid and endogenous (unlabeled) bile acid were measured in patients with idiopathic chronic diarrhea. Subjects received a 2.5-microCi oral dose of taurocholic acid labeled with 14C in the 24th position of the steroid moiety. Endogenous bile acid excretion was measured by a hydroxysteroid dehydrogenase assay on a concurrent 72-h stool collection. Both radiolabeled and endogenous bile acid excretion were abnormally high in most patients with chronic diarrhea compared with normal subjects, even when equivoluminous diarrhea was induced in normal subjects by ingestion of osmotically active solutions. The correlation between radiolabeled and endogenous bile acid excretion was good. However, neither radiolabeled nor endogenous bile acid excretion was as abnormal as is typically seen in patients with ileal resection, and none of these diarrhea patients responded to treatment with cholestyramine with stool weights less than 200 g. These results suggest (a) that this radiolabeled bile acid excretion test accurately reflects excess endogenous bile acid excretion; (b) that excess endogenous bile acid excretion is not caused by diarrhea per se; (c) that spontaneously occurring idiopathic chronic diarrhea is often associated with increased endogenous bile acid excretion; and (d) that bile acid malabsorption is not likely to be the primary cause of diarrhea in most of these patients.

  7. Comparison of endogenous and radiolabeled bile acid excretion in patients with idiopathic chronic diarrhea

    International Nuclear Information System (INIS)

    Fecal recovery of radioactivity after ingestion of a bolus of radiolabeled bile acid is abnormally high in most patients with idiopathic chronic diarrhea. To evaluate the significance of this malabsorption, concurrent fecal excretion of both exogenous radiolabeled bile acid and endogenous (unlabeled) bile acid were measured in patients with idiopathic chronic diarrhea. Subjects received a 2.5-microCi oral dose of taurocholic acid labeled with 14C in the 24th position of the steroid moiety. Endogenous bile acid excretion was measured by a hydroxysteroid dehydrogenase assay on a concurrent 72-h stool collection. Both radiolabeled and endogenous bile acid excretion were abnormally high in most patients with chronic diarrhea compared with normal subjects, even when equivoluminous diarrhea was induced in normal subjects by ingestion of osmotically active solutions. The correlation between radiolabeled and endogenous bile acid excretion was good. However, neither radiolabeled nor endogenous bile acid excretion was as abnormal as is typically seen in patients with ileal resection, and none of these diarrhea patients responded to treatment with cholestyramine with stool weights less than 200 g. These results suggest (a) that this radiolabeled bile acid excretion test accurately reflects excess endogenous bile acid excretion; (b) that excess endogenous bile acid excretion is not caused by diarrhea per se; (c) that spontaneously occurring idiopathic chronic diarrhea is often associated with increased endogenous bile acid excretion; and (d) that bile acid malabsorption is not likely to be the primary cause of diarrhea in most of these patients

  8. The development of a decision support system for the diagnosis of chronic idiopathic facial pain.

    OpenAIRE

    Chalidapongse, P.

    2004-01-01

    The aim of the research was to develop (a) a well structured electronic medical record for a decision support system, and (b) logical algorithms for the diagnosis of Chronic Idiopathic Facial Pain (CIFP) and for educating trainees. This project started by validating the paper-based Facial Pain Proforma (FPP) with a panel of 3 experts. The FPP received a top grade consensus for history and examination. However, family relationships were considered too intrusive by one pain specialist and one c...

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

    OpenAIRE

    Tuğçe Bulakbaşı Balcı; Meltem Yüksel; Zerrin Yılmaz; Feride İffet Şahin

    2010-01-01

    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. H...

  10. Clinical experience with low-dose itraconazole in chronic idiopathic cough

    OpenAIRE

    Ogawa, Haruhiko; Fujimura, Masaki; Takeuchi, Yasuo; Makimura, Koichi

    2013-01-01

    Background The presence of basidiomycetous (BM) fungi in induced sputum is an important clinical finding in chronic idiopathic cough (CIC). However, the efficacy of anti-fungal therapy for CIC has not been evaluated. Methods We selected 10 patients with CIC and carried out allergological examinations for Bjerkandera adusta, a BM fungus that has been shown to enhance cough severity. The efficacy of low-dose itraconazole (ITCZ) therapy (50 mg/day) for 14 days as an adjunctive therapy was estima...

  11. High prevalence of myocardial monoclonal antimyosin antibody uptake in patients with chronic idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Monoclonal antimyosin antibody studies were undertaken to assess the presence of myocardial uptake in patients with chronic idiopathic dilated cardiomyopathy. Three groups were studied: 17 patients with chronic (greater than 12 months) idiopathic dilated cardiomyopathy, 12 patients with a large, poorly contracting left ventricle not due to dilated cardiomyopathy (control patients) and 8 normal individuals. The patients in the cardiomyopathy and control groups showed a similar degree of clinical and functional impairment. Imaging was undertaken 48 h after antimyosin injection. The heart/lung ratio of antimyosin uptake was used to assess the results. The mean ratio in the cardiomyopathy group was 1.83 +/- 0.36 (range 1.40 to 2.80), a value significantly higher than that obtained in the control patients without cardiomyopathy (mean 1.46 +/- 0.04, range 1.38 to 1.50) or normal subjects (mean 1.46 +/- 0.13, range 1.31 to 1.6) (p less than 0.01). No difference in the ratio was noted between the normal subjects and control patients. Abnormal antimyosin uptake was seen in 12 (70%) of the 17 patients with cardiomyopathy and in only 1 (8%) of the 12 control patients. Positive monoclonal antimyosin antibody studies are highly prevalent in chronic idiopathic dilated cardiomyopathy

  12. Comparison of the efficacy of olopatadine and fexofenadine in chronic idiopathic urticaria patients: a crossover study.

    Science.gov (United States)

    Tanizaki, Hideaki; Yamamoto, Yosuke; Nakamizo, Satoshi; Otsuka, Atsushi; Miyachi, Yoshiki; Kabashima, Kenji

    2015-01-01

    Olopatadine is one of the second-generation H1 antihistamines that were used for treating allergic disorders initially in Asia, and now also in Western countries. Whereas several trials compared the efficacy on chronic urticaria among second-generation H1 antihistamines, no study has directly compared the clinical efficacy between olopatadine and other H1 antihistamines in patients with chronic idiopathic urticaria (CIU). In this study, we address this issue for the first time and conclude that olopatadine is a good candidate for the treatment of CIU. PMID:25592003

  13. 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

  14. 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

  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. Micromegakaryocytes in a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and chronic thrombocytopenic purpura

    Energy Technology Data Exchange (ETDEWEB)

    Gangarossa, S.; Mattina, T.; Romano, V.; Milana, G.; Mollica, F.; Schiliro, G. [Universita di Catania (Italy)

    1996-03-15

    Thrombocytopenia or pancytopenia is frequently reported in patients with partial 11q deletion but there are no reports on bone marrow morphology of these patients. We report on a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and its classical clinical manifestations including chronic thrombocytopenic purpura in whom micromegakaryocytes were found in the bone marrow aspirate. This is the first report of the presence of micromegakaryocytes in the bone marrow of a patient with 11q deletion. Accurate examination of the bone marrow of other patients with the 11q deletion may clarify whether the observation of micromegakaryocytes is common in these patients. Micromegakaryocytes may indicate a defect of development. Two genes for two DNA binding proteins that are likely to be involved in hematopoiesis map in the 11q region: Ets-1, that maps to 11q24, close to D11S912, and the nuclear-factor-related-kB gene that maps to 11q24-q25. It is possible that these genes, when present in only one copy, result in thrombocytopenia or pancytopenia as observed in this patient. 23 refs., 2 figs., 1 tab.

  17. Micromegakaryocytes in a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and chronic thrombocytopenic purpura.

    Science.gov (United States)

    Gangarossa, S; Mattina, T; Romano, V; Milana, G; Mollica, F; Schilirò, G

    1996-03-15

    Thrombocytopenia or pancytopenia is frequently reported in patients with partial 11q deletion but there are no reports on bone marrow morphology of these patients. We report on a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and its classical clinical manifestations including chronic thrombocytopenic purpura in whom micromegakaryocytes were found in the bone marrow aspirate. This is the first report of the presence of micromegakaryocytes in the bone marrow of a patient with 11q deletion. Accurate examination of the bone marrow of other patients with the 11q deletion may clarify whether the observation of micromegakaryocytes is common in these patients. Micromegakaryocytes may indicate a defect of development. Two genes for two DNA binding proteins that are likely to be involved in hematopoiesis map in the 11q region: Ets-1, that maps to 11q24, close to D11S912, and the nuclear-factor-related-kB gene that maps to 11q24-q25. It is possible that these genes, when present in only one copy, result in thrombocytopenia or pancytopenia as observed in this patient. PMID:8882392

  18. 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

  19. 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

    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.%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。

  20. 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.

  1. 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

  2. A biopsychosocial investigation of pediatric chronic pain with special focus on juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Lomholt, Johanne Jeppesen

    as a chronic pain diagnosis, and the pain is caused by the inflammations in the joint. The amount of pain experienced by a child with JIA may be modulated by psychological, social, and biological factors. The general purpose of the present thesis is twofold. First, the purpose is to investigate biopsychosocial...... associations between pain threshold and age, current pain experience, disease duration, or disease activity. Children with JIA had a substantially lower pain threshold even in areas usually unaffected by arthritis. Our findings suggest that JIA alters the pain perception and causes decreased pain threshold......Our understanding and management of pediatric chronic pain have advanced markedly over the last half century. Chronic pain is pain that persists for a usually more than three months and is highly prevalent in children and adolescents. Juvenile idiopathic arthritis (JIA) can be characterized...

  3. 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.

  4. Chronic idiopathic urticaria: Comparison of clinical features with positive autologous serum skin test

    Directory of Open Access Journals (Sweden)

    George Mamatha

    2008-01-01

    Full Text Available Background: Chronic idiopathic urticaria (CIU, in its extremely severe form, can pose a therapeutic challenge to the treating physician. It has been noted that in one third of such patients, autoantibodies against the IgE receptor are seen and such patients have more severe and unremitting urticaria. Aim: To compare clinical features of autoimmune urticaria with those of other CIU patients. Methods: We conducted a prospective study in an attempt to correlate the clinical features with autoantibodies, indirectly detected via the autologous serum skin test (ASST, which is the simplest and the best in vivo clinical test for detection of basophil histamine-releasing activity. Discussion: Out of 100 patients with chronic idiopathic urticaria, 34 showed a positive reaction to the autologous serum skin test and it was found that the frequency and severity of attacks was higher in these patients. Conclusion: ASST may be used as a simple and cost-effective test for the classification of chronic urticaria, which has proven to be a therapeutic challenge to the treating physician.

  5. Stress, psychiatric co-morbidity and coping in patients with chronic idiopathic urticaria.

    Science.gov (United States)

    Chung, Man Cheung; Symons, Christine; Gilliam, Jane; Kaminski, Edward R

    2010-04-01

    This study examined life event stress, perceived stress and psychiatric co-morbidity among patients with Chronic Idiopathic Urticaria (CIU). It also investigated the relationship between coping, stress, the severity of CIU and psychiatric co-morbidity. Total of 100 CIU patients and 60 allergy patients participated in the study. They completed the General Health Questionnaire, the Social Readjustment Rating Scale, the Perceived Stress Scale, and the Ways of Coping Checklist. Compared with allergy patients, CIU patients had worse co-morbidity and higher levels of life event stress and perceived stress. Emotion-focussed coping was associated with the severity of CIU; perceived stress was associated with co-morbidity. PMID:20204926

  6. 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...

  7. [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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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

  11. 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

  12. Helicobacter pylori (H.p. Infection: A possible cause of Chronic Idiopathic Urticaria

    Directory of Open Access Journals (Sweden)

    Amer Abu El ­ Enin*, Abd El-Wahab Fathe** Mohamed Khedr

    2008-03-01

    Full Text Available Chronic idiopathic urticaria (CIU is one of the most frequent skin diseases with an incidence ranging from 1.2% to 23% , however its causes remains unknown in the vast majority of cases. A possible relationship between chronic urticaria and Helicobacter pylori (H.p. has been recently suggested. The aim of this study was to determine the prevalence of Helicobacter pylori(H.P. infection in patients with C.I.U. and to investigates the effectiveness of eradication therapy of (H.p. infection on this skin disease. 100 patients (40 males and 60 females with C.I.U as well as 45 apparently healthy (age, sex, matched controls were enrolled in this study underwent serological testing for H.pylori infection with the 13C ­urea test as well as by specific IgG antibodies against the H. pylori (ELISA technique. Positive patients were treated with, Omeprazole ­ Clarithromycin + Amoxicillin for 10 days. Eradication and clinical course were followed up two months after the treatment in comparison to the negative cases as a control group. The clinical evaluation and follow up were demonstrated as worse,. same, less and disappearance of flares. In our results there was significant difference in the sero prevalence of H.pylori infection between idiopathic chronic urticaria patients and healthy subjects. H.pylori was detected in 75% of patients and 20% of controls. Out of 75 patients treated , only 24 showed complete remission of their urticaria after successfully eradicating H.pylori infection ,the others only having some improvement in their symptoms. The mean lgG titer was 86.8 + 42.4 among patients group, where it was 16.+ 42.8 among the control group (p< 0.05 . After treating the positive patients with appropriate antibiotics for 10 days there are a significant decrease of H.P. specific lgG as compared to the negative cases and also bacterium eradication is associated with a remission of urticarial symptoms. as compared to the negative cases. In conclusion

  13. Novel Helicobacter species isolated from rhesus monkeys with chronic idiopathic colitis.

    Science.gov (United States)

    Fox, J G; Handt, L; Xu, S; Shen, Z; Dewhirst, F E; Paster, B J; Dangler, C A; Lodge, K; Motzel, S; Klein, H

    2001-05-01

    Chronic, idiopathic diffuse colitis is a well recognised clinical and pathological entity in captive rhesus monkeys. Six rhesus monkeys were diagnosed with clinically debilitating, chronic diarrhoea. Histologically, colonic tissues were characterised as chronic, moderate to severe colitis and typhlitis, with diffuse mononuclear inflammation of lamina propria, reactive lymphoid hyperplasia and multifocal micro-abscesses. Colonic tissues were cultured for Salmonella spp. and Shigella spp.; all results were negative. Samples were negative for Clostridium difficile A and B toxins, and special stains of colonic tissue for acid-fast bacteria were also negative. The six diarrhoeic monkeys tested gave negative results for serum IgG antibodies to herpes B virus, STLV, SRV and SIV. Colonic tissue from the six diarrhoeic and two clinically normal monkeys with histologically confirmed colitis from the same colony were also subjected to micro-aerobic culture. Micro-aerobic cultures from all eight monkeys incubated at 37 degrees C and 42 degrees C revealed pinpoint or spreading colonies on antibiotic-containing media. Bacteria were identified as gram-negative, oxidase positive and urease negative. Of the nine strains characterised biochemically, two separate biotypes (corresponding to different species by 16S rRNA analysis) were identified. One biotype (type 1), from non-diarrhoeic monkeys and the second biotype (type 2) from diarrhoeic animals with subclinical chronic colonic inflammation, differed by catalase activity, ability to reduce nitrate to nitrite and sensitivity to cephalothin. Complete 16S rRNA analysis of five of the nine strains characterised biochemically indicated that the organisms isolated were two novel Helicobacter spp. By electron microscopy, these novel helicobacters had spiral morphology with bipolar sheathed flagella. This is the first report describing the isolation of novel Helicobacter spp. from inflamed colons of rhesus monkeys. Studies are needed to

  14. Immunophenotype characteristics of peripheral blood mononuclear leukocytes of chronic idiopathic urticaria patients.

    Science.gov (United States)

    Garmendia, Jenny V; Zabaleta, Mercedes; Aldrey, Oscar; Rivera, Henry; De Sanctis, Juan B; Bianco, Nicolás E; Blanca, Isaac

    2006-12-01

    The pathogenesis of chronic idiopathic urticaria (CIU) is not completely understood although autoimmunity has been proposed. The aim of the study was to assess the expression of different leukocyte antigens, by flow cytometry, assaying total blood of 29 patients with CIU and of 20 sex and age matched controls. Moreover, we assessed soluble CD154 a marker of immune cell activation, predominantly memory T cells. When patients were divided depending an their response to the autologous serum skin test (ASST), three different groups were encountered: group 1 (n=11): with negative ASST-, group 2 (n=11): positive ASST (ASST+) with normal lymphocyte counts and group 3 (n=7): ASST+ with low lymphocyte counts (CIU pathogenesis. PMID:17176904

  15. Treatment of chronic idiopathic urticaria with levamisole: a multicentre, randomized, double-blind, controlled trial.

    Science.gov (United States)

    Zhang, H; Shan, C; Hua, Z; Zhao, P; Zhang, H

    2009-01-01

    The objective of this study was to evaluate the efficacy of treating chronic idiopathic urticaria (CIU) with levamisole in combination with levocetirizine. This was a multicentre, randomized, double-blind, controlled trial that included 132 patients with active CIU who were treated for 6 weeks with either levocetirizine alone (control group; n = 65) or levamisole plus levocetirizine (treatment group; n = 67). Response to therapy was evaluated by measuring the efficacy rate. After 2 weeks of treatment, there was no significant difference in the efficacy rate between the treatment and control groups (54.84% and 42.37%, respectively). After 6 weeks of treatment, a statistically significant difference in the efficacy rate was observed between the groups (76.27% and 54.39% for the treatment and control groups, respectively). This study demonstrated that a combination of levamisole plus levocetirizine is more effective than levocetirizine alone and potentially provides a new, promising approach to the treatment of CIU. PMID:19761700

  16. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  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. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Autologous serum skin test in chronic idiopathic urticaria: prevalence, correlation and clinical implications.

    Science.gov (United States)

    Kulthanan, Kanokvalai; Jiamton, Sukhum; Gorvanich, Taniya; Pinkaew, Sumruay

    2006-12-01

    Some cases of chronic idiopathic urticaria (CIU) have histamine-releasing IgG autoantibodies in their blood. This disease subgroup is called "autoimmune urticaria". To date, the autologous serum skin test (ASST) is the best in vivo clinical test for the detection of basophil histamine-releasing activity in vitro. This study aimed to find the prevalence of ASST positive cases in Thai patients with CIU, to identify factors related to the positivity of ASST and to find the clinical implications of ASST in CIU. A retrospective study was performed among 85 CIU patients who attended the Urticaria Clinic at the Department of Dermatology, Siriraj Hospital and were willing to perform ASST, from January 2002 to December 2003. Twenty-one (24.7%) patients had a positive ASST. There was no significant difference between patients with positive ASST and negative ASST as to the severity of the disease (wheal numbers, wheal size, itching scores and the extent of body involvement) as well as the duration of the disease. PMID:17348242

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Sami Ozturk

    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(5.000: 351-356

  4. Second-generation antihistamines for the treatment of chronic idiopathic urticaria.

    Science.gov (United States)

    Belsito, Donald V

    2010-05-01

    Chronic idiopathic urticaria (CIU) is a serious disorder that can greatly compromise quality of life. While H1 antihistamines are the accepted first-line treatment for CIU, older generations of these agents (e.g., hydroxyzine, diphenhydramine) are associated with anticholinergic and CNS effects, such as drowsiness and sedation, that can pose risks to patients, especially when driving. Second-generation agents available in the United States (U.S.) (e.g., cetirizine, desloratadine, fexofenadine, levocetirizine, loratadine) has greatly reduced these CNS effects, making them the current treatments of choice in CIU, but their potency and tolerability profiles vary. Differences in duration of in vivo receptor occupancy may affect the potency of H1 antihistamines. Levocetirizine appears to have greater in vivo H1 receptor occupancy compared with later generation H1 antihistamines, which may confer an advantageous efficacy/safety profile. This has been confirmed in a recent head-to-head study showing that levocetirizine was more effective than desloratadine in improving pruritus in CIU patients. Fexofenadine has been shown to have a low occupancy of H1 antihistamine receptors in the brain, which reduces the likelihood of sedation. More studies are required to further assess receptor occupancy and other factors that may differentiate the second-generation H1 antihistamines. PMID:20480793

  5. Levocetirizine: The latest treatment option for allergic rhinitis and chronic idiopathic urticaria.

    Science.gov (United States)

    Dubuske, Lawrence M

    2007-01-01

    Levocetirizine is the most recent antihistamine available in the United States and is indicated for the symptomatic treatment of allergic rhinitis (AR; seasonal [SAR] and perennial [PAR]) and chronic idiopathic urticaria (CIU). The purpose of this study was to review the current literature on pharmacologic properties of levocetirizine, its safety, tolerability, and effectiveness in AR and CIU. Relevant articles in English or with English abstracts were identified from systematic Medline searches using combinations of the terms antihistamine/s, CIU, H(1)-receptor antagonist/s, levocetirizine, PAR and persistent AR (PER), pharmacodynamic, pharmacokinetic, and SAR. Levocetirizine is the active enantiomer of cetirizine. Pharmacologic and clinical studies indicate that levocetirizine has a fast onset and long duration of action, with a well-tolerated adverse effect profile. These favorable features may be caused by levocetirizine's pharmacokinetic and pharmacodynamic properties including high bioavailability, low apparent volume of distribution, low degree of metabolism, and high in vivo potency and H(1)-receptor occupancy. Several large well-controlled clinical trials in adults and children aged 6-12 years have shown levocetirizine to be consistently efficacious and well tolerated in relieving the symptoms of SAR, PAR, and PER and CIU. Levocetirizine is a welcome new treatment option in the United States for symptomatic treatment of AR and CIU. PMID:18201439

  6. Lack of a role for cross-reacting anti-thyroid antibodies in chronic idiopathic urticaria.

    Science.gov (United States)

    Mozena, Jonathan D; Tiñana, Adrienne; Negri, Julie; Steinke, John W; Borish, Larry

    2010-07-01

    The etiology of chronic idiopathic urticaria (CIU) is attributed to autoantibodies directed against the alpha-chain of the high-affinity IgE receptor (FcepsilonRIalpha) or IgE on mast cells in 30-60% of patients. Approximately 30% of CIU patients have Hashimoto's thyroiditis (HT). We investigated the pathophysiologic relationship of anti-thyroid and anti-FcepsilonRIalpha antibodies. Nine individuals with both CIU and HT underwent autologous serum skin testing (ASST) and sera were assayed for thyroid autoantibodies, thyroid-stimulating hormone, and anti-FcepsilonRIalpha antibodies. Serum samples were studied for their ability to activate a human mast cell line (LUVA) as determined by cysteinyl leukotriene (CysLT) production. Experiments were performed to determine whether epitope cross-reactivity could explain the high incidence of HT found in CIU patients. A significant proportion of CIU patients had a positive ASST (nine of six) and anti-FcepsilonRIalpha antibodies (six of nine). Incubation of patient sera with FcepsilonRIalpha, but not thyroglobulin or thyroid peroxidase, resulted in the decreased ability to detect anti-FcepsilonRIalpha antibodies. Incubation with thyroid antigens did not inhibit CysLT production by mast cells. Epitopic cross-reactivity does not explain the increased prevalence of HT found in CIU patients. The frequent concurrence of HT and CIU likely reflects a genetic tendency toward autoimmune diseases. PMID:20182447

  7. Association of interleukin 10 and transforming growth factor β gene polymorphisms with chronic idiopathic urticaria.

    Science.gov (United States)

    Tavakol, Marzieh; Movahedi, Masoud; Amirzargar, Ali Akbar; Aryan, Zahra; Bidoki, Alireza Zare; Heidari, Kimia; Soltani, Samaneh; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Nabavi, Mohammad; Nasiri, Rasoul; Ahmadvand, Alireza; Rezaei, Nima

    2014-01-01

    Transforming growth factor β (TGF-β) and interleukin 10 (IL-10) are two anti-inflammatory cytokines that are implicated in the pathogenesis of urticaria. The goal of this study was to examine the possible association of polymorphisms of TGF-β and IL-10 genes with susceptibility to chronic idiopathic urticaria (CIU). This study was conducted on 90 patients with CIU. Polymerase chain reaction (PCR) was done to determine the genotype at 5 polymorphic sites; TGF-β (codon10C/T and codon25G/C) and IL-10 (-1082G/A, -819C/T, and -592C/A). The C allele at codon 25 of TGF-β was more prevalent in CIU patients compared to controls (OR = 9.5, 95% CI = 5.4-16.8, PCIU patients (PCIU and controls; CC haplotype was overrepresented, while CG and TG haplotypes were underrepresented (PCIU. Additionally, patients with CIU seem to have genotypes leading to high production of TGF-β and IL-10. PMID:25580782

  8. Clinical and laboratory features of antihistamine-resistant chronic idiopathic urticaria.

    Science.gov (United States)

    Magen, Eli; Mishal, Joseph; Zeldin, Yuri; Schlesinger, Menachem

    2011-01-01

    Chronic idiopathic (spontaneous) urticaria (CIU) is sometimes resistant to the conventional and high doses of antihistamines (AHs). This study compares the clinical and laboratory characteristics of AH responsive and AH-resistant CIU subjects. Clinical and laboratory data were retrospectively collected from 385 CIU patients. Urticaria activity score (UAS), concomitant angioedema, dermatographism, positive autologous serum skin (ASST), and laboratory data were collected. The control group consisted of 44 sex- and age-matched healthy individuals. Two hundred forty-five CIU patients controlled with AH medications were included in the CIU group. Forty-six patients failed to show clinical improvement during 8 weeks of treatment with fourfold AH doses and were included in the resistant CIU (R-CIU) group. The R-CIU group was characterized with a higher incidence (58.7%) of angioedema than the CIU group (28.5%; p CIU versus 12.2% in CIU; p = 0.014), more positive ASST (73.9% in R-CIU versus 45.4% in CIU; p CIU versus 3.32 ± 1.25 in CIU; CIU was characterized with more severe basopenia (0.04 ± 0.07 cell/mm(3) versus 0.16 ± 0.13 cell/mm(3); p CIU is a clinically more severe disease with laboratory features of low-grade inflammation and platelet activation. PMID:22221441

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

    Directory of Open Access Journals (Sweden)

    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

  10. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  12. The effect of L-thyroxine treatment on chronic idiopathic urticaria and autoimmune thyroiditis.

    Science.gov (United States)

    Magen, Eli; Mishal, Joseph

    2012-01-01

    Autoimmune thyroiditis (AT) is more prevalent in patients with chronic idiopathic urticaria CIU) than in the general population. Previous small studies without any controlled comparison reported that CIU remits in patients with CIU and AT treated with L-thyroxine. To determine whether l-thyroxine treatment can improve the clinical course of CIU in patients with the co-occurrence of AT and CIU. A total of 749 patients with CIU were retrospectively studied. Clinical and laboratory evaluation and classification of chronic urticaria were performed according to the EAACI/GA(2)LEN/EDF/WAO guidelines. After L-thyroxine treatment for 53 ± 19 days, euthyroidism was restored in all subjects. Urticaria activity score (UAS) was evaluated at baseline and after three and six months. The control group consisted of matched 44 euthyroid subjects with CIU. A total of 44 (5.9%) patients were diagnosed to have hypothyroidism related to AT. Autologous serum skin test (ASST) was found to be positive in 17 (38.6%) of them. There was no statistically significant difference in baseline UAS, between the ASST+ (3.94 ± 1.52) and the ASST- (3.63 ± 1.42; P = 0.27) hypothyroid subjects and the euthyroid CIU controls (3.73 ± 1.74). During the L-thyroxine treatment, a significant reduction of UAS was observed in both hypothyroid ASST+ and ASST- subjects. However, the mean UAS after three and six months of L-thyroxine treatment remained not significantly different from that in control euthyroid subjects with CIU. L-Thyroxine treatment has no effect on the course of CIU in patients with CIU and AT. PMID:22182385

  13. 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.

  14. Clinical-laboratory characteristics of ANA-positive chronic idiopathic urticaria.

    Science.gov (United States)

    Magen, Eli; Waitman, Dan-Andrei; Dickstein, Yoav; Davidovich, Valentina; Kahan, Natan R

    2015-01-01

    Despite the established association between chronic idiopathic/spontaneous urticaria (CIU) and presence of antinuclear antibodies (ANAs), the prevalence of autoimmune comorbidities in this population has not been analyzed. Here, we aim to identify clinical and laboratory manifestations associated with ANA-positive CIU. ANA-positive patients were identified via electronic data capture from the electronic patient record database of Leumit Health care Services (LHS) of Israel. Patient characteristics, medical histories, and details of diagnostic workup, medical treatment, and follow-up were retrieved by performing a chart review of electronic patient records (EPRs). The prevalence of target diseases among ANA(+) CIU(+), ANA(+) CIU(-), and ANA(-) CIU(+) patients was calculated. A total of 91 ANA(+) CIU(+), 3131 ANA(+) CIU(-), and 478 ANA(-) CIU(+) patients were identified. The ANA(+) CIU(+) group was characterized by higher prevalence of Sjögren's syndrome (SS)-A 52 antibodies (Ab) (7.7% versus 2.4%; p = 0.008), SS-A 60 Ab (11% versus 2.8%; p = CIU(+) group. Additionally, ANA(+) CIU(+) patients were more likely to be diagnosed with thyroid autoimmune diseases, higher C-reactive protein (6.4 ± 10.3 versus 4.1 ± 8.8 mg/L; p = 0.027), and more profound basopenia (0.04 ± 0.09 versus 0.15 ± 0.11 cell/mm(3); p CIU patients. More ANA(+) CIU(+) patients were resistant to four-fold standard licensed doses of antihistamines than ANA(-) CIU(+) patients [11 (12.1%) versus 29 (6.1%); p = 0.046]. ANA-positive CIU is characterized by higher prevalence of SS-A 52, SS-A 60, and SS-B antibodies and poorer clinical response to antihistamine medications. PMID:25715242

  15. Basophil phenotypes in chronic idiopathic urticaria in relation to disease activity and autoantibodies.

    Science.gov (United States)

    Eckman, John A; Hamilton, Robert G; Gober, Laura M; Sterba, Patricia M; Saini, Sarbjit S

    2008-08-01

    Potentially pathogenic IgG autoantibodies to IgE or its receptor, Fc epsilonRIalpha, have been detected in approximately 40% of chronic idiopathic urticaria (CIU) patients. CIU patients' basophils display distinct altered Fc epsilonRIalpha-mediated degranulation. CIU patients with basophil histamine release in response to polyclonal goat anti-human IgE > or = 10% are classified as CIU responders (CIU-R) and CIU non-responders (CIU-NR). We compared the presence of autoantibodies to basophil degranulation phenotypes and to disease status (active or inactive). Sera were collected from non-CIU subjects and CIU subjects who participated in a longitudinal study of disease severity and had defined basophil degranulation phenotypes. Immunoenzymetric assays (IEMA) quantified IgG anti-Fc epsilonRIalpha and anti-IgE. IgG anti-Fc epsilonRIalpha antibody was detected in 57% of CIU-R (n=35), 55% of CIU-NR (n=29), and 57% of non-CIU subjects (n=23), whereas IgG anti-IgE was present in 43% of CIU-R, 45% of CIU-NR, and 30% of non-CIU subjects. Both the autoantibody levels and the functional basophil phenotype remained stable in subjects with active disease (n=16), whereas there was an enhancement in basophil function as subjects evolved into a state of remission (n=6), which appears independent of the presence of autoantibody. IEMAs detected a similar frequency of autoantibodies in CIU-R, CIU-NR, and non-CIU subjects. Basophil function may be independent of IEMA-detected autoantibodies. PMID:18356810

  16. Serum thyroid autoantibodies in patients with idiopathic either acute or chronic urticaria.

    Science.gov (United States)

    Gangemi, S; Saitta, S; Lombardo, G; Patafi, M; Benvenga, S

    2009-02-01

    In Italy, only one study was conducted on the detection of serum thyroid autoantibodies (ATA) in patients with urticaria. This northern-Italy study reported a 23% rate of ATA positiveness in 52 patients with chronic idiopathic urticaria (CIU). During the years 1998-2006, 688 patients with urticaria were hospitalized at our Division of Allergy and Clinical Immunology. Thyroglobulin and thyroperoxidase autoantibodies (TgAb and TPOAb) were assayed at admission in 144/688 patients. Of the 144 patients (mean age: 42.3+/-15.8 yr, range 17-84), 95 (72 women and 23 men) had an history of CIU (CIU group) and 49 (44 women and 5 men) did not [acute urticaria group or (AU)]. Of the 144 patients, 37 (25.7%) tested positive for at least one ATA: 31 with CIU (32.6%) and 6 with AU (12.2%, chi2=7.037, p=0.008). Positiveness for TPOAb or TgAb was 30/37 (81.1%) or 17/37 (45.9%); 10/37 (27.0%). Pre-hospitalization duration of CIU was longer in the 31 ATA positive patients compared to the 64 ATA negative patients (207.2+/-273.4 vs 81.6+/-106.3 weeks, p=0.015). Pre-hospitalization duration of CIU correlated positively with the log10-transformed serum concentration of TPOAb in the 25 CIU patients who tested TPOAb positive (r=0.42, p=0.039). We conclude that our rate of 31/95 (32.6%) positiveness for at least one type of ATA in CIU is greater than that of 6 representative international studies published between the years 2000 and 2006 (111/488 or 22.7%, range 15-29%, chi2=4.884, p=0.027). PMID:19411805

  17. Statin effects on regulatory and proinflammatory factors in chronic idiopathic urticaria.

    Science.gov (United States)

    Azor, M H; dos Santos, J C; Futata, E A; de Brito, C A; Maruta, C W; Rivitti, E A; da Silva Duarte, A J; Sato, M N

    2011-11-01

    Immunological dysfunction has been described to occur in chronic idiopathic urticaria (CIU), most notably in association with an inflammatory process. Some pharmacological agents as statins--drugs used in hypercholesterolaemia--display a broad effect on the immune response and thus should be tested in vitro in CIU. Our main objectives were to evaluate the effects of statins on the innate and adaptive immune response in CIU. Simvastatin or lovastatin have markedly inhibited the peripheral blood mononuclear cells (PBMC) proliferative response induced by T and B cell mitogens, superantigen or recall antigen. Simvastatin arrested phytohaemaglutinin (PHA)-induced T cells at the G0/G1 phase, inhibiting T helper type 1 (Th1), Th2, interleukin (IL)-10 and IL-17A cytokine secretion in both patients and healthy control groups. Up-regulation of suppressor of cytokine signalling 3 (SOCS3) mRNA expression in PHA-stimulated PBMCs from CIU patients was not modified by simvastatin, in contrast to the enhancing effect in the control group. Statin exhibited a less efficient inhibition effect on cytokine production [IL-6 and macrophage inflammatory protein (MIP)-1α] induced by Toll-like receptor (TLR)-4, to which a statin preincubation step was required. Furthermore, statin did not affect the tumour necrosis factor (TNF)-α secretion by lipopolysaccharide (LPS)-stimulated PBMC or CD14+ cells in CIU patients. In addition, LPS-activated PBMC from CIU patients showed impaired indoleamine 2,3-dioxygenase (IDO) mRNA expression compared to healthy control, which remained at decreased levels with statin treatment. Statins exhibited a marked down-regulatory effect in T cell functions, but were not able to control TLR-4 activation in CIU patients. The unbalanced regulatory SOCS3 and IDO expressions in CIU may contribute to the pathogenesis of the disease. PMID:21985375

  18. 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 ...

  19. 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 ...

  20. 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.

  1. Studies of the prevalence and significance of radiolabeled bile acid malabsorption in a group of patients with idiopathic chronic diarrhea

    International Nuclear Information System (INIS)

    We studied radiolabeled fecal bile acid excretion in 11 normal subjects and 17 patients with idiopathic chronic diarrhea for three major purposes: to establish normal values for this test in the presence of increased stool volumes (induced in normal subjects by ingestion of poorly absorbable solutions); to test for bile acid malabsorption in the patients and to correlate this with an independent test of ileal function, the Schilling test; and to compare the results of the bile acid excretion test with the subsequent effect of a bile acid binding agent (cholestyramine) on stool weight. In normal subjects fecal excretion of the radiolabel was increased with increasing stool volumes. As a group, patients with idiopathic chronic diarrhea excreted radiolabeled bile acid more rapidly than normal subjects with induced diarrhea (t1/2 56 +/- 8 vs. 236 +/- 60 h, respectively, p less than 0.005). There was a statistically significant positive correlation between t1/2 of radiolabeled bile acid and Schilling test results in these patients. Although 14 of 17 patients absorbed labeled taurocholic acid less well than any of the normal subjects with comparable volumes of induced diarrhea, cholestyramine had no statistically significant effect on stool weight in the patient group, and in none of the patients was stool weight reduced to within the normal range. In summary, most patients with idiopathic chronic diarrhea have bile acid malabsorption (as measured by fecal excretion of labeled bile acid), but they do not respond to cholestyramine therapy with a significant reduction in stool weight. Although the significance of these findings was not clearly established, the most likely interpretation is that bile acid malabsorption is a manifestation of an underlying intestinal motility or absorptive defect rather than the primary cause of diarrhea

  2. Once-daily rupatadine improves the symptoms of chronic idiopathic urticaria: a randomised, double-blind, placebo-controlled study.

    Science.gov (United States)

    Dubertret, Louis; Zalupca, Lavinia; Cristodoulo, Tania; Benea, Vasile; Medina, Iris; Fantin, Sara; Lahfa, Morad; Pérez, Iñaki; Izquierdo, Iñaki; Arnaiz, Eva

    2007-01-01

    This randomised, double-blind, placebo-controlled, parallel-group, international, dose-ranging study investigated the effect of treatment with rupatadine 5, 10 and 20 mg once daily for 4 weeks on symptoms and interference with daily activities and sleep in 12-65 years-old patients with moderate-to-severe chronic idiopathic urticaria (CIU). Rupatadine 10 and 20 mg significantly reduced pruritus severity by 62.05% and 71.87% respectively, from baseline, over a period of 4 weeks compared to reduction with placebo by 46.59% (p CIU. Rupatadine decreased pruritus severity, in a dose- and time-dependent manner. PMID:17478385

  3. 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, ...

  4. Chronic idiopathic urticaria and post-traumatic stress disorder (PTSD): an under-recognized comorbidity.

    Science.gov (United States)

    Gupta, Madhulika A; Gupta, Aditya K

    2012-01-01

    A large body of literature supports the role of psychologic stress in urticaria; however, the comorbidity between chronic idiopathic urticaria (CIU) and post-traumatic stress disorder (PTSD), a classic stress-mediated syndrome, has received little attention. The underlying etiology of urticaria is not identifiable in about 70% of patients, possibly because of difficulties with identification of a direct cause-and-effect relationship between a potential causative factor and the onset of urticaria. The core features of PTSD (Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision [DSMIV-TR]) that are important in urticaria include (1) autonomic nervous system reactivity and state of sympathetic hyperarousal that can manifest as CIU, and (2) the persistent re-experiencing of the traumatic events in PTSD, which can manifest as urticaria or angioedema, or both, affecting a previously traumatized body region (eg, urticarial wheals affecting the body region where the patient had been stabbed years earlier). The following features of PTSD make it difficult to use the cause-and-effect model for the determination of causation: (1) PTSD may first emerge years after the initial trauma and is classified as PTSD with Delayed Onset (DSMIV-TR); and (2) the traumatic triggers that precipitate the PTSD symptoms may be unique and idiosyncratic to the patient and not even qualify as stressful or traumatic by standard criteria (eg, precipitating events for the PTSD may include smell of a certain cologne that was used by the perpetrator or witnessing a scene in a movie that was reminiscent of the location where the abuse occurred). Finally, in PTSD with Delayed Onset, patients may not make a conscious association between their recurrent urticaria and their earlier traumas because they can develop classically conditioned associations between stimuli that are reminiscent of the original abuse situation and their somatic reactions such as urticaria. The clinician

  5. 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.

  6. 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。

  7. Idiopathic pulmonary hemosiderosis

    OpenAIRE

    Sunilkumar, B M; Sathishkumar, K M; Somashekhar, A R; P.P. Maiya

    2010-01-01

    Recurrent or chronic pulmonary hemorrhage is rare in children. Idiopathic pulmonary hemosiderosis (IPH) manifests as hemoptysis, diffuse parenchymal infiltrates on chest radiographs and microcytic hypochromic anemia. The hemoptysis present may be mistaken for more common diseases, delaying the diagnosis and further management. Idiopathic pulmonary hemosiderosis is a disorder of unknown etiology. Treatment of IPH includes immunosuppressive drugs along with supportive measures.

  8. 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...

  9. 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...

  10. 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...

  11. 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 ... illness . This means the body attacks and destroys healthy body ...

  12. 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

  13. mRNA profiles of cytokine receptors in unstimulated peripheral blood mononuclear cells from patients with chronic idiopathic urticaria.

    Science.gov (United States)

    Gao, Jianming; Yang, Aizhen; Chen, Min; Li, Ansheng; Yao, Xu; Li, Yumei; Xie, Shihai; Yang, Xueyuan; Zhong, Liansheng; Chen, Zhiqiang

    2011-03-01

    This present study was aimed to investigate the roles of the receptors of Th1/Th2 cytokines and chemokines in the pathogenesis of chronic idiopathic urticaria (CIU). Thirty patients with CIU, 30 patients with dermographism and 30 healthy controls were randomly enrolled. Reverse transcription-PCR (RT-PCR) was used to analyze the mRNA of cytokine receptors in peripheral blood mononuclear cells (PBMCs). The mRNA levels of tumor necrosis factor receptor (TNFR), interferon-γ receptor (IFN-γR), and interleukin-10 receptor (IL-10R) were statistically increased in the CIU group (P CIU and other groups. The mRNA levels of CCR3 and CCR6 were statistically increased in the CIU group (P CIU group than the healthy control group (P CIU. PMID:23554682

  14. Estimates of total costs for treatment of chronic id-iopathic urticaria in adults, depending on the scheme antiallergic therapy

    Directory of Open Access Journals (Sweden)

    Petrov V.I.

    2010-12-01

    Full Text Available Objective: Comparative economic evaluation of clinical application of various alternatives to anti-allergic therapy. The total budget for the treatment of severe chronic idiopathic urticaria for the 12 weeks of the study (4 weeks of therapy and 8 weeks of observation was assessed according to treatment: Group 1 -cyclosporine2.5 mg/kg/day, Group 2-cetirizine 20 mg and ranitidine 300 mg/day, Group 3 - cetirizine 20 mg. These groups were evaluated by direct medical, direct nonmedical and indirect costs. It was found that the lowest overall cost of treating 100 patients with severe CIU, within 12 weeks of the study were recorded in the group, where a combined therapy of cetirizine 10 mg ranitidine 300 mg. The greatest costs were associated with the appointment of therapy cetirizine 20 mg

  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...... treatment at licensed doses. Patients aged 12-75 years with CIU/CSU who remained symptomatic despite treatment with approved doses of H1 antihistamines were randomized (1:1:1:1) in a double-blind manner to subcutaneous omalizumab 75 mg, 150 mg, or 300 mg or placebo every 4 weeks for 24 weeks followed by 16...... event. Omalizumab 300 mg administered subcutaneously every 4 weeks reduced weekly ISS and other symptom scores versus placebo in CIU/CSU patients who remained symptomatic despite treatment with approved doses of H1 antihistamines....

  16. 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.

  17. 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.

  18. Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients?

    Science.gov (United States)

    Bois, Melanie C; Hu, Xiaowen; Ryu, Jay H; Yi, Eunhee S

    2016-07-01

    Chronic occult aspiration of small droplets (microaspiration) due to gastroesophageal reflux disease (GERD) and/or hiatal hernia is postulated to be a contributing factor in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Usual interstitial pneumonia (UIP) is the histopathologic correlate of IPF. We hypothesized that chronic microaspiration may manifest as prominent airway-centered fibroblastic foci (FFs) in IPF. UIP cases diagnosed by wedge biopsies over a 6-year period (2006-2011) were identified and scored (1-3) for the prominence of airway-centered FFs by 2 authors blinded for clinical history. Relevant clinical information was obtained. Thirty-seven patients (22 men) were diagnosed with IPF by multidisciplinary approach. Thirteen cases (35.1%) demonstrated high airway-centered FF score (score 3). Twenty (54.1%) patients carried a clinical diagnosis of GERD, and 3 patients (8.1%) had hiatal hernia. High airway-centered FF score was significantly associated with hiatal hernia diagnosis (P=.037) but not with a diagnosis of GERD or the use of proton pump inhibitors/histamine-2 receptor antagonists. High airway-centered FF score was associated with airway-centered acute inflammation (P=.028) and peribronchiolar granulomas (P=.042). In summary, IPF cases with hiatal hernia were more likely to have a prominent airway-centered FF. Given the strong association between hiatal hernia and GERD and their risk for developing chronic microaspiration, the prominent airway-centered FF in UIP might predict the presence of chronic microaspiration, acknowledging that GERD and proton pump inhibitor/histamine-2 receptor antagonist use failed to demonstrate a significant association. Larger studies are warranted for further investigation. PMID:26980038

  19. Atorvastatin-related thrombocytopenic purpura

    OpenAIRE

    Narayanan, Deepa; Kilpatrick, Eric S.

    2010-01-01

    A 44-year-old male patient with a single vessel ischaemic heart disease was referred to the lipid clinic for management of hypercholesterolaemia after an episode of admission with thrombocytopenic purpura secondary to atorvastatin. Atorvastatin was discontinued and his platelet counts improved gradually with steroids. He is now established on a different statin with no further episodes of thrombocytopenia. Though a drug challenge was never done, an idiosyncratic reaction to the initial statin...

  20. Eysenck's Two Big Personality Factors and Their Relationship to Depression in Patients with Chronic Idiopathic Pain Disorder: A Clinimetric Validation Analysis

    OpenAIRE

    Marianne Lunde; Per Bech; Stine Bjerrum Møller

    2012-01-01

    Aim. The clinimetric aspects of Eysenck’s two big personality factors (neuroticism and extraversion) were originally identified by principal component analysis but have been insufficiently analysed with item response theory models. Their relationship to states of melancholia and anxiety was subsequently analysed. Method. Patients with chronic idiopathic pain disorder were included in the study. The nonparametric item response model (Mokken) was compared to the coefficient alpha to validate th...

  1. 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

  2. Helicobacter pylori Eradication in Patients with Immune Thrombocytopenic Purpura: A Review and the Role of Biogeography.

    Science.gov (United States)

    Frydman, Galit H; Davis, Nick; Beck, Paul L; Fox, James G

    2015-08-01

    Idiopathic thrombocytopenic purpura (ITP) is typically a diagnosis of exclusion, assigned by clinicians after ruling out other identifiable etiologies. Since a report by Gasbarrini et al. in 1998, an accumulating body of evidence has proposed a pathophysiological link between ITP and chronic Helicobacter pylori (H. pylori) infection. Clinical reports have described a spontaneous resolution of ITP symptoms in about 50% of chronic ITP patients following empirical treatment of H. pylori infection, but response appears to be geography dependent. Studies have also documented that ITP patients in East Asian countries are more likely to express positive antibody titers against H. pylori-specific cytotoxic-associated gene A (CagA), a virulence factor that is associated with an increased risk for gastric diseases including carcinoma. While a definitive mechanism by which H. pylori may induce thrombocytopenia remains elusive, proposed pathways include molecular mimicry of CagA by host autoantibodies against platelet surface glycoproteins, as well as perturbations in the phagocytic activity of monocytes. Traditional treatments of ITP have been largely empirical, involving the use of immunosuppressive agents and immunoglobulin therapy. However, based on the findings of clinical reports emerging over the past 20 years, health organizations around the world increasingly suggest the detection and eradication of H. pylori as a treatment for ITP. Elucidating the exact molecular mechanisms of platelet activation in H. pylori-positive ITP patients, while considering biogeographical differences in response rates, could offer insight into how best to use clinical H. pylori eradication to treat ITP, but will require well-designed studies to confirm the suggested causative relationship between bacterial infection and an autoimmune disease state. PMID:25728540

  3. The effect of fexofenadine hydrochloride on productivity and quality of life in patients with chronic idiopathic urticaria.

    Science.gov (United States)

    Spector, Sheldon L; Shikiar, Richard; Harding, Gale; Meeves, Suzanne; Leahy, Michael J

    2007-02-01

    The present study examined the impact of once-daily fexofenadine hydrochloride (HCl) 180 mg on health-related quality of life (HRQL) in subjects with chronic idiopathic urticaria (CIU). This was a multicenter, randomized, double-blind. parallel-group, placebo-controlled study. Subjects completed the Dermatology Life Quality Index (DLQI) and the Work Productivity and Activity Impairment (WPAI) questionnaire at baseline and at weeks 2 and 4. The primary HRQL end point was mean change from baseline to week 4 in total DLQI score. Subjects in the fexofenadine HCl treatment group (n = 163) experienced significantly greater improvements in mean total DLQI score (P = .0219) and in the individual domains of symptoms and feelings (P = .0119) and personal relationships (P = .0091) compared with those in the placebo group (n = 91). Subjects who received fexofenadine HCl experienced less work productivity impairment, overall work impairment, and activity impairment than those who received placebo. The results indicated that once-daily fexofenadine HCl 180 mg improved the HRQL of subjects with CIU, as assessed by change in total DLQI score. PMID:17388220

  4. Low-dose and short-term cyclosporine treatment in patients with chronic idiopathic urticaria: a clinical and immunological evaluation.

    Science.gov (United States)

    Serhat Inaloz, H; Ozturk, Savas; Akcali, Cenk; Kirtak, Necmettin; Tarakcioglu, Mehmet

    2008-05-01

    The present study aimed to evaluate the effectiveness of 2.5 mg/kg/day cyclosporin (CsA) treatment in patients with severe chronic idiopathic urticaria (CIU) and the impact of CsA treatment on several cytokines involved in the etiopathogenesis of CIU. Twenty-seven CIU patients and 24 healthy control subjects were included in the study. The autologous serum skin test (ASST) for autoantibodies and urticaria activity scoring (UAS) were measured for the evaluation of the clinical severity and the response to therapy, and the serum levels of interleukin (IL)-6, IL-8, IL-2 receptor, IL-1beta, tumor necrosis factor (TNF)-alpha and IL-5 were measured. The mean UAS score was 32.07 +/- 7.05 and 6.22 +/- 3.84 before and after CsA treatment, respectively. The serum IL-2 receptor, TNF-alpha and IL-5 levels of patients before CsA treatment were statistically higher than those of the control group (P = 0.001), and after 4 weeks of CsA therapy the mean IL-2R, TNF-alpha and IL-5 levels were significantly decreased. The data from this study demonstrate that CsA therapy is efficient and safe for CIU patients. Increase in clinical efficacy and marked decreases in serum cytokine levels suggest that inhibition of cytokine generation is involved in the action of the drug in this clinical setting. PMID:18477227

  5. Markers of antioxidant defence system and lipid peroxidation in peripheral blood of female patients with chronic idiopathic urticaria.

    Science.gov (United States)

    Kasperska-Zajac, Alicja; Brzoza, Zenon; Polaniak, Renata; Rogala, Barbara; Birkner, Ewa

    2007-03-01

    Oxidative stress is an important event in lesional skin of patients with chronic idiopathic urticaria (CIU). In the present study, we assessed blood oxidant/antioxidant status of patients suffering from CIU with positive response to autologous serum skin test (ASST) and with negative ASST, to improve our understanding of biological processes and the part of oxidative stress in this disease. Activities of manganese superoxide dismutase (MnSOD), copper-zinc superoxide dismutase (Cu/ZnSOD), glutathione peroxidase (GSH-PX), and catalase (CAT) as indices of enzymatic antioxidant capacity, as well as malondialdehyde (MDA) level as a maker of lipid peroxidation were measured in plasma and erythrocytes from 14 CIU female patients showing positive ASST, 31 CIU female patients with negative ASST and in 19 sex- and age-matched healthy subjects. The antioxidant enzyme activity in plasma and in erythrocytes did not differ significantly among the three groups. Also, the plasma and erythrocytes MDA levels were similar in the three groups. Based on our results, it seems that systemic activity of the enzymatic antioxidants (CuZn/SOD, MnSOD, GSH-Px, and CAT) as well as level of lipid peroxidation determined by MDA may not be increased in the course of immune-inflammatory processes associated with CIU. We also suggest that the systemic oxidant/antioxidant status of CIU patients, showing positive response to ASST, may not be different from that of CIU patients with negative ASST. PMID:17171548

  6. Increased circulating pro-inflammatory cytokines and imbalanced regulatory T-cell cytokines production in chronic idiopathic urticaria.

    Science.gov (United States)

    Dos Santos, Juliana Cristina; Azor, Mayce Helena; Nojima, Viviane Yoshimi; Lourenço, Francinelson Duarte; Prearo, Erica; Maruta, Celina Wakisaka; Rivitti, Evandro Ararigbóia; da Silva Duarte, Alberto José; Sato, Maria Notomi

    2008-10-01

    The immunologic characterization of chronic idiopathic urticaria (CIU), mainly regarding cytokine profile needs more investigation. We examined circulating inflammatory cytokine levels, T-cell induced secretion, and cytokine mRNA expression in patients with CIU subjected to the intradermal autologous serum skin test (ASST). Increased levels of circulating pro-inflammatory cytokines, such as TNF-alpha, IL-1beta, IL-12p70, and IL-6 have been observed in most of patients with CIU, together with an enhancement of IL-2 secretion following T-cell stimulation. Highlighting the inflammatory profile in CIU found in ASST positive, is the enhanced B-cell proliferative responsiveness and increased IL-17 secretion levels. ASST-positive patients also exhibited impaired IL-4 secretion associated with increased IL-10 production. Altered cytokine expression in patients with ASST-negative, was the down-modulation of spontaneous IL-10 mRNA expression levels in peripheral blood mononuclear cells. Our findings support the concept of immunologic dysregulation in CIU, revealing a systemic inflammatory profile associated with disturbed cytokine production by T cells, mainly related to IL-17 and IL-10 production. PMID:18586117

  7. 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.

  8. Role of IFN-γ and IL-6 cytokines and their association in determining susceptibility to chronic idiopathic urticaria.

    Science.gov (United States)

    Alasandagutti, Madhavi Latha; Ponnana, Meenakshi; Sivangala, Ramya; Thada, Shruthi; Joshi, Lavanya; Hussain, Hidayath; Ansari, Soheb Sadath; Valluri, Vijayalakshmi; Gaddam, Suman Latha

    2014-12-01

    Chronic Idiopathic Urticaria (CIU) is a common skin disorder, which may occur spontaneously. The aim of the present study was to assess the serum levels of interferon (IFN)-γ and interleukin (IL)-6 and to examine the association of IFN-γ+874 T/A and IL-6-174 G/C cytokine gene polymorphisms. To accomplish this, ELISA-based cytokine serum levels of IFN-γ (n=30) and IL-6 (n=30) in CIU patients (n=100) and Healthy Controls (HC) (n=200) were performed. Amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) was performed to verify the positional significance. A significant (pCIU patients compared to HC. The AT and TT genotypes of IFN-γ and GG genotype of IL-6 were found to be significantly associated with CIU. In conclusion, our findings show a significant increase in the cytokine levels of IFN-γ and IL-6, highlighting their regulatory role in the development of disease. In addition to this, association studies have revealed that TT genotype of IFN-γ +874 T/A and GG genotype of IL-6-174 G/C were susceptible towards the CIU. PMID:25409241

  9. Improvement of thrombocytopenia after treatment for Helicobacter pylori in a patient with immunologic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Aline Maia Rocha

    2014-04-01

    Full Text Available Immune thrombocytopenic purpura is the most common autoimmune hematologic disease, affecting individuals of different ages. Recently, the bacterium Helicobacter pylori entered the list of causes of immune thrombocytopenic purpura. Here we present the case of a 55-year-old female patient with low platelet counts initially attributed to chronic vaginal bleeding. As corticosteroid therapy was ineffective she was treated for H. pylori infection. Within four weeks the patient had a platelet count of 87.17 ×109/L accompanied by clinical improvement of the symptoms.

  10. Idiopathic infantile arterial calcification in a 12-year-old girl presenting as chronic mesenteric ischemia: imaging findings and angioplasty results

    International Nuclear Information System (INIS)

    We report an unusual case of chronic mesenteric ischemia presenting in a 12-year-old girl with idiopathic infantile arterial calcinosis (IIAC). This is the first reported case in the literature of chronic mesenteric ischemia in the setting of IIAC. The girl presented with a classical history of postprandial abdominal pain. Imaging demonstrated significant stenoses of the celiac axis, superior mesenteric artery (SMA) and inferior mesenteric artery (IMA). Angioplasty of the celiac axis and SMA was attempted, with successful dilation of the SMA only. At 3-, 6- and 12-month follow-ups, the child's symptoms had almost resolved. This case report has three important ramifications: chronic mesenteric ischemia is a possible clinical presentation in children with IACC, pre-angioplasty imaging is important in guiding treatment approach, and angioplasty was effective in this case of chronic mesenteric ischemia and offers hope for other similarly affected children. (orig.)

  11. T cell regulation in Juvenile Idiopathic Arthritis : Controlling chronic inflammation by pulling the right strings

    OpenAIRE

    Kleer, Ismé Mariëtte de

    2004-01-01

    Understanding the complex cellular and molecular mechanisms that regulate the immune response remains one of the major challenges in immunology. A key question is how the immune system is regulated in order to control a protective immune response and prevent chronic and destructive immunopathology. Within the adaptive immune system CD4+ T cells are keyplayers in the initiation and orchestration of immune responses. However, there is now increasing evidence that subsets of CD4+ T cells are als...

  12. Idiopathic venous thromboembolism and thrombophilia

    OpenAIRE

    Sinescu, C; Hostiuc, M; Bartos, D.

    2011-01-01

    During the past decade idiopathic venous thromboembolism has become a separate entity, a chronic illness which has required prolonged anticoagulation and other prevention strategies to avoid recurrences. This article reviews recent developments regarding unprovoked venous thromboembolism and its relation with thrombophilia. In the beginning, the latest definition of idiopathic venous thromboembolism is presented. The article continues with statistics about thrombophilia, related venous thromb...

  13. Idiopathic thrombocytopenic purpura following successful treatment of acute lymphoblastic leukemia.

    Science.gov (United States)

    Tannir, N M; Kantarjian, H

    2001-03-01

    Thrombocytopenia is common in patients with acute lymphocytic leukemia (ALL) at diagnosis. It is a universal side effect of dose-intensive regimens employed in the treatment of adult ALL. In patients with ALL who achieve remission, thrombocytopenia frequently indicates relapse. We report three adult patients successfully treated for ALL who developed thrombocytopenia and were found to have immune-mediated thrombocytopenia (ITP). Possible pathophysiologic mechanisms underlying the association of ALL and ITP are discussed. PMID:11342378

  14. Antihistamines do not inhibit the wheal induced by the intradermal injection of autologous serum in resistant chronic idiopathic urticaria.

    Science.gov (United States)

    Magen, Eli; Mishal, Joseph; Zeldin, Yuri; Schlesinger, Menachem

    2012-01-01

    Some patients with chronic idiopathic urticaria (CIU) are resistant to conventional doses of antihistamines (AHs). This study was designed to check whether the skin wheal and flare reaction produced by the intradermal injection of autologous serum (AS) and by histamine differs in AH-resistant and AH responder CIU patients. CIU patients with treatment failure under fexofenadine at 180 mg q.d. increased their daily dose of AH to 4 tablets daily. Those with significant improvement of urticaria activity score under fexofenadine at 180 mg were included in the CIU group. Subjects with treatment failure despite a full 8-week fourfold fexofenadine treatment were included in the resistant CIU (R-CIU group). The control group consisted of sex- and age-matched patents with allergic rhinitis. The AS skin test and intradermal histamine-induced wheal and flare reaction were performed at baseline (without AH), after 8 and 16 weeks (under AH treatment). Forty-six subjects were included in the CIU group, 21 were in the R-CIU group, and 44 were in the control group. Under AH therapy, the skin reaction to intradermal histamine injection was significantly diminished in all study groups. In the R-CIU group, fexofenadine at 180 mg did not suppress AS-induced wheal reaction (5.96 ± 2.25 mm; p = 0.85), and with a fourfold AH dose some reduction of AS-induced wheal (3.79 ± 1.74 mm; p = 0.008) was observed but remained larger than in the CIU (2.31 ± 1.12; p = 0.006) and control groups (2.52 ± 1.36; p = 0.037). AHs do not inhibit the wheal induced by the intradermal injection of AS in R-CIU. PMID:23394513

  15. 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.

  16. 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.

  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. 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 ...

  19. 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

  20. Focal ulcerative ileocolitis with terminal thrombocytopenic purpura in juvenile cotton top tamarins (Saguinus oedipus).

    Science.gov (United States)

    Snook, S S; Canfield, D R; Sehgal, P K; King, N W

    1989-03-01

    A newly recognized syndrome characterized by an acute focal ulcerative ileocolitis, anemia and thrombocytopenic purpura in five juvenile cotton-top tamarins is described. The presentation and morphology of this syndrome is distinct from any other reported gastrointestinal disease reported in tamarins. Traditional etiologies such as viruses, ingested toxins, Campylobacter, Salmonella and Yersinia and Clostridium difficile are not considered likely etiologic agents. Nontraditional etiologies such as anaerobes or pathologic strains of Escherichia coli are now being considered. This syndrome is of potential significance to ongoing research into the etiology of idiopathic tamarin colitis. PMID:2496272

  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. 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.

  3. 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

  4. 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.

  5. Acute Pancreatitis-Induced Thrombotic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Mohan Gurjar

    2012-01-01

    Full Text Available Context Acute pancreatitis due to thrombotic thrombocytopenic purpura is a well recognized condition. Here, we are reporting a rare converse phenomenon, in which thrombocytopenic purpura occurred secondary to acute pancreatitis. Case report A 19-year-old male referred to our intensive care unit with diagnosis of acute pancreatitis with multi-organ dysfunction. He had history of severe abdominal pain and recurrent vomiting about one month ago, requiring hospital admission. There, on diagnostic work-up at admission, abdominal ultrasonography was suggestive of pancreatitis. His serum amylase and lipase were 1,900 and 1,582 U/L, respectively. Other laboratory parameters were within normal limits. He was managed conservatively with intravenous fluids, antibiotics and analgesics; and discharged after about 2 weeks One week after discharge he was readmitted in same hospital with abdominal pain, multiple episodes of bilious vomiting and abdominal distention. Later on he was referred to our intensive care unit; having classical pentad of thrombocytopenic purpura, i.e., thrombocytopenia, micro-angiopathic hemolytic anemia, renal failure, encephalopathy, and fever. His condition improved with plasma exchange therapy and transferred out from our ICU to ward after 10 days of stay. Conclusion Thrombocytopenic purpura may be precipitate by acute pancreatitis due to multiple mechanisms. A high clinical suspicion is required to make an early diagnosis and allow early initiation of plasma exchange therapy, resulting in a goodprognosis.

  6. Análise de 39 casos de pneumonia intersticial crônica idiopática Analysis of 39 cases of idiopathic chronic interstitial pneumonia

    Directory of Open Access Journals (Sweden)

    Rogério Rufino

    2006-12-01

    Full Text Available OBJETIVO: Analisar de forma retrospectiva fragmentos de biópsias pulmonares que receberam o diagnóstico de pneumonia intersticial crônica idiopática, à luz da classificação da American Thoracic Society e European Respiratory Society, de 2000. MÉTODOS: A partir da revisão de 252 fragmentos de biópsias pulmonares a céu aberto de pacientes com doença intersticial pulmonar, no período de 1977 a 1999, 39 casos de doença pulmonar intersticial idiopática foram selecionados e reavaliados por dois patologistas, segundo a classificação da American Thoracic Society e European Respiratory Society, de 2000. RESULTADOS: Vinte e oito dos 39 diagnósticos foram mantidos (71,8%. Uma nova entidade patológica, a pneumonia intersticial não específica, foi incluída na reclassificação e houve superposição de padrões em seis casos. Mantiveram o mesmo diagnóstico 28 casos, 4 casos apresentaram associação entre fibrose pulmonar idiopática e organização pneumônica criptogênica, 1 entre organização pneumônica criptogênica e pneumonia intersticial não específica, e 1 entre pneumonia intersticial descamativa e pneumonia intersticial não específica. Todos os casos de fibrose pulmonar idiopática foram confirmados, embora 3 deles estivessem associados a organização pneumônica criptogênica. Os diagnósticos anteriores foram quase todos mantidos na revisão dos espécimes (p > 0,05. CONCLUSÃO: A classificação das doenças pulmonares intersticiais da American Thoracic Society e European Respiratory Society é uma ferramenta útil aos patologistas que lidam com biópsias pulmonares.OBJECTIVE: To make a retrospective analysis of lung biopsy samples obtained from patients diagnosed with chronic idiopathic interstitial pneumonia, as defined in the American Thoracic Society/European Respiratory Society classification system made public in 2000. METHODS: Samples from 252 open-lung biopsies of patients with interstitial lung disease, all

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  9. 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

  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. 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

  13. 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...

  14. 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...

  15. 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.

  16. Plasma concentration of interleukin 6 (IL-6), and its relationship with circulating concentration of dehydroepiandrosterone sulfate (DHEA-S) in patients with chronic idiopathic urticaria.

    Science.gov (United States)

    Kasperska-Zajac, Alicja; Brzoza, Zenon; Rogala, Barbara

    2007-08-01

    Decline in circulating DHEA-S concentration may be a phenomenon accompanying chronic idiopathic urticaria (CIU). IL-6 is a multifunctional proinflammatory cytokine which exerts a wide range of biological effects. A functional link between DHEA-S and IL-6 has been described. Therefore, the present study was performed to evaluate circulating concentration of IL-6 in patients with CIU and to study its relationship with DHEA-S and C-reactive protein (CRP) concentration. IL-6 plasma concentration was determined in 18 female non-atopic patients with CIU who had negative response to autologous serum skin test and 20 non-atopic healthy controls. Plasma concentration of IL-6 was statistically higher in CIU patients than in the control group, although all the values were found within the range of the normal subjects. CIU patients showed significantly lower DHEA-S concentration in serum than the controls. CRP concentration remained within the normal range and did not differ between the two groups. We did not find a significant correlation between concentration of IL-6 and DHEA-S, or CRP. It seems that the processes associated with CIU may be accompanied by slightly elevated plasma concentration of IL-6 and substantially decreased serum concentration of DHEA-S as compared with the healthy subjects. However, no association between IL-6 and DHEA-S concentration in the peripheral circulation of CIU patients was proved, suggesting that both phenomena may not be related to each other. PMID:17827029

  17. 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.

  18. Up-regulation of chemokine C-C ligand 2 (CCL2) and C-X-C chemokine 8 (CXCL8) expression by monocytes in chronic idiopathic urticaria.

    Science.gov (United States)

    Santos, J C; de Brito, C A; Futata, E A; Azor, M H; Orii, N M; Maruta, C W; Rivitti, E A; Duarte, A J S; Sato, M N

    2012-01-01

    The disturbed cytokine-chemokine network could play an important role in the onset of diseases with inflammatory processes such as chronic idiopathic urticaria (CIU). Our main objectives were to evaluate the relation between proinflammatory chemokine serum levels from CIU patients and their response to autologous skin test (ASST) and basophil histamine release (BHR). We also aimed to assess the chemokine secretion by peripheral blood mononuclear cells (PBMC) upon polyclonal stimulus and to evaluate chemokine C-C ligand 2/C-X-C chemokine 8 (CCL2/CXCL8) and Toll-like receptor-4 (TLR-4) expression in monocytes. We observed significantly higher serum levels of the CXCL8, CXCL9, CXCL10 and CCL2 in CIU patients compared to the healthy group, regardless of the BHR or ASST response. The basal secretion of CCL2 by PBMC or induced by Staphylococcus aureus enterotoxin A (SEA) was higher in CIU patients than in the control group, as well as for CXCL8 and CCL5 secretions upon phytohaemagglutinin stimulation. Also, up-regulation of CCL2 and CXCL8 mRNA expression was found in monocytes of patients upon SEA stimulation. The findings showed a high responsiveness of monocytes through CCL2/CXCL8 expression, contributing to the creation of a proinflammatory environment in CIU. PMID:22132892

  19. Cultured peripheral blood mast cells from chronic idiopathic urticaria patients spontaneously degranulate upon IgE sensitization: Relationship to expression of Syk and SHIP-2.

    Science.gov (United States)

    Saini, Sarbjit S; Paterniti, Miya; Vasagar, Kavitha; Gibbons, Scott P; Sterba, Patricia M; Vonakis, Becky M

    2009-09-01

    Recently, signaling changes in the FcvarepsilonRI pathway involving inositol lipid phosphatases have been identified in the basophils of chronic idiopathic urticaria (CIU) subjects. Based on the profile of basophil FcvarepsilonRI-mediated histamine degranulation, we have segregated CIU subjects into two groups, CIU Responder (CIU R) or CIU Nonresponder (CIU NR). In the present study, we compared expression of SHIP-1, SHIP-2, and Syk protein to histamine release (HR) from mast cells (MC) cultured from the peripheral blood of CIU R, CIU NR, and normal subjects. The MC of CIU R donors contained significantly increased Syk and decreased SHIP-2 as compared to CIU NR (Syk: p=0.038, SHIP-2: p=0.038) and normals (Syk: p=0.042, SHIP-2: p=0.027). Spontaneous HR from CIU donors was increased two-fold compared to normals (p=0.04). In summary, our results suggest a possible predilection for urticarial MC to spontaneously degranulate upon IgE sensitization contributing to the increased pruritus associated with CIU. PMID:19477690

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

    Directory of Open Access Journals (Sweden)

    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.

  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. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  4. Idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    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.

  5. 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...

  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. Efficacy and safety of omalizumab in patients with chronic idiopathic/spontaneous urticaria who remain symptomatic on H1 antihistamines: a randomized, placebo-controlled study.

    Science.gov (United States)

    Saini, Sarbjit S; Bindslev-Jensen, Carsten; Maurer, Marcus; Grob, Jean-Jacques; Bülbül Baskan, Emel; Bradley, Mary S; Canvin, Janice; Rahmaoui, Abdelkader; Georgiou, Panayiotis; Alpan, Oral; Spector, Sheldon; Rosén, Karin

    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 treatment at licensed doses. Patients aged 12-75 years with CIU/CSU who remained symptomatic despite treatment with approved doses of H1 antihistamines were randomized (1:1:1:1) in a double-blind manner to subcutaneous omalizumab 75 mg, 150 mg, or 300 mg or placebo every 4 weeks for 24 weeks followed by 16 weeks of follow-up. The primary end point was change from baseline in weekly itch severity score (ISS) at week 12. Among randomized patients (N=319: placebo n=80, omalizumab 75 mg n=78, 150 mg n=80, 300 mg n=81), 262 (82.1%) completed the study. Compared with placebo (n=80), mean weekly ISS was reduced from baseline to week 12 by an additional 2.96 points (95% confidence interval (CI): -4.71 to -1.21; P=0.0010), 2.95 points (95% CI: -4.72 to -1.18; P=0.0012), and 5.80 points (95% CI: -7.49 to -4.10; PCIU/CSU patients who remained symptomatic despite treatment with approved doses of H1 antihistamines. PMID:25046337

  8. Comprehensive screening for PRSS1, SPINK1, CFTR, CTRC and CLDN2 gene mutations in Chinese paediatric patients with idiopathic chronic pancreatitis: a cohort study

    Science.gov (United States)

    Wang, Wei; Sun, Xiao-Tian; Weng, Xiao-Ling; Zhou, Dai-Zhan; Sun, Chang; Xia, Tian; Hu, Liang-Hao; Lai, Xiao-Wei; Ye, Bo; Liu, Mu-Yun; Jiang, Fei; Gao, Jun; Bo, Lu-Min; Liu, Yun; Liao, Zhuan; Li, Zhao-Shen

    2013-01-01

    Objective Genetic alterations may contribute to chronic pancreatitis (CP) in Chinese young patients. This study was designed to investigate mutations of cationic trypsinogen (PRSS1), pancreatic secretory trypsin inhibitor or serine protease inhibitor Kazal type 1 (SPINK1), cystic fibrosis transmembrane conductance regulator (CFTR), chymotrypsin C (CTRC) and CLDN2 genes and the copy number variations (CNVs) of PRSS1 and asses associations with the development of idiopathic CP (ICP) in Chinese children. Design Retrospective. Setting A single center. Participants 75 ICP Chinese children (40 boys and 35 girls). Primary and secondary outcome measures Mutations of PRSS1, SPINK1, CFTR, CTRC and CLDN2 genes and CNVs. Results 7 patients had heterozygous mutations in PRSS1, that is, N29I (n=1), R122H or R122C (n=6). The CNVs of PRSS1 in five patients had abnormal copies (1 copy (n=4), five copies (n=1)). 43 patients had IVS3+2T>C (rs148954387) (10 homozygous and 33 heterozygous) in SPINK1. None of the PRSS1 mutation patients carried a SPINK1 mutation. Frequency of PRSS1 and SPINK1 mutations was 9.3% and 57.3%, respectively, with an overall frequency of 66.6% (50/75). In addition, one patient had a novel deletion of CFTR (GCTTCCTA from c.500 to c.508 leading to the shortened polypeptide molecule via a stop codon). Another patient had a novel missense in CLDN2 exon 2 (c.592A>C mutation). Clinically, patients with SPINK1 mutations had a higher rate of pancreatic duct stones, pancreatic pseudocyst and pancreatic calcification than those without SPINK1 mutations (pC mutation may play an important role in the pathogenesis of Chinese paediatric ICP. However, further study is needed to confirm and to investigate the role of these genes in the development of Chinese ICP. PMID:24002981

  9. 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

  10. Idiopathic short stature

    Directory of Open Access Journals (Sweden)

    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.

  11. Epidemiology of idiopathic pulmonary fibrosis

    OpenAIRE

    Ley, Brett

    2013-01-01

    Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of it...

  12. Epidemiology of idiopathic pulmonary fibrosis

    OpenAIRE

    Ley B; Collard HR

    2013-01-01

    Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its...

  13. 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

  14. 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.

  15. 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.

  16. 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.

  17. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

    Full Text Available Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence and public health impact (ie, health care costs and resource utilization. Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. Keywords: idiopathic pulmonary fibrosis, epidemiology, incidence, prevalence, mortality, risk factors

  18. 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

  19. 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

  20. 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.

  1. 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.

  2. [Acquired von Willebrand syndrome in a patient with immune thrombocytopenic purpura].

    Science.gov (United States)

    Ihara, Akihiro; Suzuki, Nobuaki; Matsushita, Tadashi; Ichinose, Akitada

    2015-07-01

    Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder similar to inherited von Willebrand disease. We describe a 78-year-old woman with coexistent idiopathic thrombocytopenic purpura (ITP) and AVWS. The patient had once been admitted to our hospital because of cerebral infarction. Her platelet count had been normal at that time. Ten years later, she showed a severe bleeding tendency (platelet count 3.2×10(4)/μl). Analysis of hemostatic parameters showed very low (IgG4) to VWF was detected by enzyme linked immunosorbent assay (ELISA). Factor XIII activity was 42%. Treatment with corticosteroids did not improve the thrombocytopenia, but did correct the bleeding diathesis. Also, VWF: Rco and VIII: C showed normalization. These findings indicated that the patient had ITP associated with AVWS. All reported cases of AVWS associated with systemic lupus erythematosus were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppression. This bleeding disorder occurs mainly in patients with lymphoproliferative, myeloproliferative, cardiovascular and immunologic disorders, but no patients with ITP have previously been reported. This patient had the rare presentation of AVWS complicated by ITP and factor XIII deficiency. PMID:26256928

  3. Refractory thrombotic thrombocytopenic purpura associated with primary Sjogren syndrome treated with rituximab: a case report.

    Science.gov (United States)

    Toumeh, Anis; Josh, Navpreet; Narwal, Rawan; Assaly, Ragheb

    2014-01-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, serious disease that involves multiple organs and is rapidly fatal if left untreated. TTP is associated with multisystem symptoms, such as thrombocytopenia, microangiopathic hemolytic anemia, renal impairment, central nervous system involvement, and fever. TTP is idiopathic in about 37% of the cases and can be associated with autoimmune diseases in 13% of the cases. Autoimmune disease-associated TTP can be refractory to plasma exchange and requires immunosuppressive therapy. We report a case of a previously healthy 55-year-old African American female who presented with shortness of breath, hemolytic anemia, renal impairment, and thrombocytopenia. The diagnosis of TTP was made, and plasmapheresis was initiated. However, recurrence happened 48 hours after plasmapheresis was stopped. Autoimmune workup for refractory TTP revealed positive antinuclear antibodies, Anti-SSA, and Anti-SSB. Lip biopsy revealed findings consistent with Sjogren syndrome. Treatment with Rituximab was started, and significant clinical and laboratory response was achieved. The patient remained asymptomatic thereafter. A high clinical suspicion of autoimmune diseases is important as TTP tends to be refractory to plasma exchange in these cases, and immunosuppressive therapy is a key. PMID:23011161

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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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.

  6. Life-Threatening Autoimmune Hemolytic Anemia and Idhiopatic Thrombocytopenic Purpura. Successful Selective Splenic Artery Embolization

    Science.gov (United States)

    Molica, Matteo; Massaro, Fulvio; Annechini, Giorgia; Baldacci, Erminia; D’Elia, Gianna Maria; Rosati, Riccardo; Trisolini, Silvia Maria; Volpicelli, Paola; Foà, Robin; Capria, Saveria

    2016-01-01

    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. PMID:27158433

  7. 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

  8. 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

  9. 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

  10. 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.

  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. 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 ...

  13. 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...

  14. 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...

  15. 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

  16. 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

  17. 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...

  18. 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

  19. 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...

  20. 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.



  1. 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

  2. Idiopathic pulmonary fibrosis: the need for early diagnosis

    OpenAIRE

    Cicchitto, Gaetano; Sanguinetti, Claudio M

    2013-01-01

    Idiopathic pulmonary fibrosis (IPF), a chronic fibrosing lung disease of a progressive nature and unknown etiology, has the largest epidemiological impact and the worst prognosis among the idiopathic interstitial pneumonias (IIP). Despite the progress in pathogenetic knowledge, many aspects are still dubious, in particular the biomolecular mechanisms activated in the early stages of the disease. Early diagnosis is desirable not only to better define aspects of the natural history of the disea...

  3. [Idiopathic Pulmonary Fibrosis].

    Science.gov (United States)

    Prasse, A

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF. PMID:26444136

  4. Comparison between sensitivity of autologous skin serum test and autologous plasma skin test in patients with Chronic Idiopathic Urticaria for detection of antibody against IgE or IgE receptor (FcεRIα).

    Science.gov (United States)

    Sajedi, Vahid; Movahedi, Masoud; Aghamohammadi, Asghar; Aghamohamadi, Asghar; Gharagozlou, Mohammad; Ghareguzlou, Mohammad; Shafiei, Alireza; Soheili, Habib; Sanajian, Nahal

    2011-06-01

    Intradermal injection of autologous serum and plasma elicit a cutaneous reactivity in almost 45-60% of patients with Chronic Idiopathic Urticaria (CIU). This reactivity is associated with the presence of auto antibodies against IgE or IgE receptors. This study was carried out to compare the cutaneous reactivity of autologous serum and plasma skin tests in a series of patients with CIU for diagnosis of auto antibodies against IgE or IgE receptor. Fifty eight patients with CIU were injected intradermally with autologous serum and plasma (anticoagulated by citrate). Histamine was used as positive control and normal saline as negative control. The study group was checked by routine laboratory tests (CBC, U/A etc), allergens with skin prick tests, and serum IgE level, and auto antibodies against thyroid as well. Duration of urticaria was another factor which was assessed.There was no significant difference between positive ASST and positive APST patients for the above mentioned tests. 77.6% of the patients were Positive for APST and 65.5% were ASST positive. Duration of urticaria was longer in patients with positive ASST and APST than ASST and APST negative patients, although the difference was not statistically significant.Autologus serum skin test (ASST) and autologous plasma skin test (APST) could be used for estimation of duration and severity of urticaria and planning for the treatment. PMID:21625019

  5. 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...

  6. 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.

  7. 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 ...

  8. 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

  9. Treatment of immune thrombocytopenic purpura: focus on eltrombopag

    Directory of Open Access Journals (Sweden)

    Lawrence Rice

    2009-03-01

    Full Text Available Lawrence RiceWeill Cornell Medical College, The Methodist Hospital, Houston, Texas, USAAbstract: Immune thrombocytopenic purpura (ITP is a relatively common autoimmune disorder in which antibodies are produced to circulating platelets. Symptoms can be mild, but for most patients the risk of severe bleeding is unacceptable and treatment is required. Glucocorticoids followed by splenectomy had been the mainstays of therapy. High dose intravenous immunoglobulin and anti-RhD therapy are available for patients with severe illness, but produce only temporary benefit. Rituximab may provide more durable responses, danazol may be underutilized, and immunosuppressants and cytotoxic agents are less often required. Recently the pathophysiology of ITP has been more clearly elucidated, particularly the importance of decreased production of platelets in most patients and the very blunted rise that occurs in serum thrombopoietin (TPO. The isolation of TPO and better understanding of its role in thrombopoiesis has led to the development of new highly effective treatments. TPO analogs had some successes in treating highly refractory ITP patients but were taken out of development due to TPO-antibody induction. Two second-generation TPO-mimetics, romiplostim and the orally available eltrombopag, have recently been licensed in some territories for the treatment of ITP. Approval of eltrombopag was based on results from Phase II and III placebo-controlled clinical trials and a long-term extension study. About 80% of patients achieve significant increases in platelet count (11% of placebo patients, with reduced bleeding and reduced use of concomitant medications; responses are often durable with no tachyphylaxis. The side effects of eltrombopag are generally mild and not worse than placebo, although there are concerns about hepatic dysfunction, and the potentials for thromboses, marrow reticulin fibrosis, rebound thrombocytopenia and cataracts. This is an important new

  10. [Multi-facetted clinical presentation of thrombotic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Niemann, C.U.; Jurlander, J.; Daugaard, G.;

    2009-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathic disease. TTP is due to reduced activity of the von Willebrand factor which cleaves ADAMTS13. The disease is characterized by thrombocytopenia (<20 billion/l) intravascular Coombs-negative haemolysis and schistocytes in blood...... smears. Determination of the ADAMTS13-activity is now becoming available as a routine analysis. We present two cases that illustrate the multi-facetted clinical presentation under which TTP occurs. The importance of access to ADAMTS13 measurements is stressed Udgivelsesdato: 2009/1/26...

  11. [An unusual coincidence of thrombotic thrombocytopenic purpura and pernicious anemia].

    Science.gov (United States)

    Zamir, D; Polychuck, I; Reitblat, T; Leibovitz, I; Lugassy, G

    2002-08-01

    A 52 year old man was admitted for hospitalization due to dizziness and weakness that appeared in the previous 2 weeks. Anemia and thrombocytopenia, as well as elevated levels of lactic dehydrogenase, reticulocytosis and schistocytes on blood smear, all suggested thrombotic thrombocytopenic purpura. However, B12 deficiency was also diagnosed. The diagnosis of pernicious anemia was reassured by both fundic biopsy and the existence of antiparietal cells antibodies and anti-intrinsic cells antibodies. A few courses of plasmapheresis along with parenteral B12 stabilized his physical condition and he was released with no need for further treatment, and only required ambulatory follow-up. PMID:12222131

  12. A Case of Immune Thrombocytopenic Purpura After Rabies Vaccination.

    Science.gov (United States)

    Fulbright, Joy M; Williams, Sarah E; Pahud, Barbara A

    2015-10-01

    We describe a case of immune thrombocytopenic purpura (ITP) occurring 15 days after the first dose of a 4-dose rabies vaccination series. ITP is thought to be an immune-mediated process triggered by an infection or toxin. There is little evidence in the literature beyond case reports of an association of ITP with vaccines other than with the measles, mumps, and rubella vaccine. This is the third reported case of ITP associated with rabies vaccination. Because of the rare occurrence of this adverse event relative to the severity of rabies infection, the benefits of rabies vaccination, when indicated, outweigh the low and possible risk of ITP. PMID:26165405

  13. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo; Rocca, Mara A; Miller, Andrew David; Schmierer, Klaus; Frederiksen, Jette; Gass, Achim; Gama, Hugo; Tilbery, Charles P; Rocha, Antonio J; Flores, José; Barkhof, Frederik; Seewann, Alexandra; Palace, Jacqueline; Yousry, Tarek; Montalban, Xavier; Enzinger, Christian; Fazekas, Franz

    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...

  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. Effects of the polysaccharide nucleic acid fraction of bacillus Calmette-Guérin on the production of interleukin-2 and interleukin-10 in the peripheral blood lymphocytes of patients with chronic idiopathic urticaria.

    Science.gov (United States)

    Li, Na; Cao, Na; Niu, Yan-Dong; Bai, Xiu-Hui; Lu, Jie; Sun, Yu; Yu, Min; Sun, Li-Xin; Duan, Xin-Suo

    2013-09-01

    Urticaria is one of the most frequent dermatoses and its prevalence in the general population is estimated to be ~20%, whereas a substantial percentage of the cases may be classified as chronic idiopathic urticaria (CIU). The inflammatory response presenting with spontaneous wheals exhibits pro-inflammatory characteristics, involving a prominent role for lymphocytes with a mixed Th1/Th2 response in which interleukin (IL)-2 and IL-10 are prominently secreted by Th1 and Th2 cells, respectively. In CIU patients, it was demonstrated that IL-10 production was elevated and IL-2 reduced compared to controls. Therefore, inhibition of IL-10 and promotion of IL-2 production by the lymphocytes, indicating Th2 inhibition and Th1 promotion, may facilitate the treatment of CIU. Whether the polysaccharide nucleic acid fraction of bacillus Calmette-Guérin (BCG-PSN), which possesses multiple immunomodulatory properties, has that potential, remains to be elucidated. In this study, BCG-PSN was used on lymphocytes isolated from CIU patients, with healthy donors used as controls. Immunocytochemistry and ELISA were used to detect IL-2 and IL-10 production. It was demonstrated that the IL-2 production by the lymphocytes in the CIU group was significantly lower compared to that in the healthy control group and it increased sequentially with the increase of the concentration of BCG-PSN used. By contrast, the IL-10 production by the lymphocytes in the CIU group was significantly higher compared to that in the healthy control group and decreased sequentially with the increase of the concentration of BCG-PSN used. Thus, it may be concluded that the BCG-PSN has the potential to promote IL-2 and inhibit IL-10 production in the lymphocytes of CIU patients, facilitating the treatment of CIU. PMID:24649015

  18. Comparison between Sensitivity of Autologous Skin Serum Test and Autologous Plasma Skin Test in Patients with Chronic Idiopathic Urticaria for Detection of Antibody against IgE or IgE Receptor (FcεRIα

    Directory of Open Access Journals (Sweden)

    Vahid Sajedi

    2011-06-01

    Full Text Available Intradermal injection of autologous serum and plasma elicit a cutaneous reactivity in almost 45-60% of patients with Chronic Idiopathic Urticaria (CIU.   This reactivity is associated with the presence of auto antibodies against IgE or IgE receptors. This study was carried out to compare the cutaneous reactivity of autologous serum and plasma skin tests in a series of patients with CIU for diagnosis of auto ntibodies against IgE or IgE receptor.Fifty eight patients with CIU were injected intradermally with autologous serum and plasma (anticoagulated by  citrate.  Histamine  was  used  as  positive  control  and  normal  saline  as negative control. The study group was checked by routine laboratory tests (CBC, U/A etc, allergens with skin prick tests, and serum IgE level, and auto antibodies against thyroid as well. Duration of urticaria was another factor which was assessed.There was no significant difference between positive ASST and positive APST patients for the above mentioned tests. 77.6% of the patients were Positive for APST and 65.5% were ASST positive. Duration of urticaria was longer in patients with positive ASST and APST than ASST and APST negative patients, although the difference was not statistically significant.Autologous serum skin test (ASST and autologous plasma skin test (APST could be used for estimation of duration and severity of urticaria and planning for the treatment.

  19. 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

  20. 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

  1. Immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia and mumps.

    Science.gov (United States)

    Kurekci, A Emin; Atay, A Avni; Demirkaya, Erkan; Sarici, S Umit; Ozcan, Okan

    2006-03-01

    Immune thrombocytopenic purpura in childhood is characterized by a typical history of acute development of purpura and bruising in an otherwise healthy child. In children it usually follows a viral infection (eg, mumps, rubella) or immunization. We report for the first time a child with acute lymphoblastic leukemia who developed immune thrombocytopenic purpura due to mumps during the maintenance phase of acute lymphoblastic leukemia treatment. PMID:16679943

  2. Pharmacotherapy in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Gaurav Acharya

    2014-10-01

    Full Text Available Idiopathic pulmonary fibrosis or cryptogenic fibrosing alveolitis is a form of chronic, progressive interstitial lung disease causing scarring of lung tissue and usually affect adults. Treatment is usually aimed at controlling inflammation and thus slowing the process of fibrosis. With only few patients responding to treatment and the disease being ultimately fatal with poor progression, the underlying lesion was considered to be fibrotic rather than inflammatory. Fibrotic foci, deposition of collagen, and lack of inflammatory cells are a predominant finding. Pirfenidone and N-acetyl cysteine are the only effective pharmacotherapy available till date. Interim results of PANTHER Trial clearly indicate more risk with triple therapy. However, in Indian patients, trial of steroid therapy may be tried when there is doubt of chronic hypersensitivity pneumonitis. BIBF 1120 has also shown positive results in Phase II clinical trial and shows a positive response in deteriorating lung function. Supplemental oxygen, education of patient, pulmonary rehabilitation, and Streptococcus pneumoniae and influenza vaccine are the most important supportive care. Pulmonary rehabilitation should be used as a treatment in the majority of patients. [Int J Basic Clin Pharmacol 2014; 3(5.000: 761-763

  3. 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

  4. 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.

  5. Dengue Virus Infection Triggering Thrombotic Thrombocytopenic Purpura in Pregnancy.

    Science.gov (United States)

    Deepanjali, Surendran; Naik, Raghuramulu R; Mailankody, Sharada; Kalaimani, Sivamani; Kadhiravan, Tamilarasu

    2015-11-01

    We report a case of thrombotic thrombocytopenic purpura (TTP) that immediately followed symptomatic dengue virus infection in a pregnant lady. The patient developed dengue fever at 16 weeks of gestation, resulting in spontaneous abortion. Subsequently, fever reappeared with persistent thrombocytopenia and jaundice. Investigations revealed microangiopathic hemolysis; there was no evidence of disseminated intravascular coagulation. The TTP episode resolved after six cycles of therapeutic plasma exchange with fresh-frozen plasma. An ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif 13 repeats) activity assay, done during convalescence, showed normal activity. The patient had an uneventful second pregnancy and has remained free of TTP recurrence for more than 2 years now. We review the pathophysiological basis of TTP in dengue infection, and suggest that jaundice with disproportionate elevation of serum aspartate aminotransferase level in a patient with dengue should arouse the suspicion of TTP. PMID:26283741

  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. 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 ...

  8. Optimal management of idiopathic scoliosis in adolescence.

    Science.gov (United States)

    Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

    2013-01-01

    Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%-3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid 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

  9. Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management.

    Science.gov (United States)

    Kumar, Sathish

    2016-04-01

    Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. In sJIA, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of sJIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. Recent data suggests that early cytokine blockage might abrogate chronic, destructive, therapy resistant arthritis phase, reflecting a potential "window of opportunity" in the care of children with sJIA. PMID:26916892

  10. 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.

  11. Idiopathic Pulmonary Haemosiderosis

    OpenAIRE

    Gaurav Vishal; Ekta Dalal; Neelam Raval

    2013-01-01

    We report a case of idiopathic pulmonary hemosiderosis (IPH) in a 8year old female who presented with anaemia, breathlessness, puffiness of face and grade 4 clubbing. She had been investigated a number of times at multiple hos-pitals and had received multiple blood transfusions considering iron deficiency anaemia. Diagnosis of IPH was sus-pected on basis of clinical history of persistent respiratory distress, hypoxemia, repeated anaemia episodes and diffuse bilateral infiltrates on chest X ra...

  12. 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...

  13. Clinical Features and Treatment Outcomes of Primary Immune Thrombocytopenic Purpura in Hospitalized Children Under 2-Years Old

    Science.gov (United States)

    Farhangi, H; Ghasemi, A; Banihashem, A; Badiei, Z; Jarahi, L; Eslami, G; Langaee, T

    2016-01-01

    Background Immune thrombocytopenic purpura (ITP) is the most prevalent cause of thrombocytopenia in children. Despite the importance of ITP in children under 2-years old, only a few publications are available in the literature.ITP usually presents itself as isolated thrombocytopenia and mucocutaneous bleeding. Materials and Methods This study was conducted on 187 under 2-year-old children diagnosed with ITP and treated at Dr. Sheikh Hospital from 2004 to 2011.In this retrospective study, clinical symptoms, laboratory findings, history of viral infections, vaccination history, and treatment efficacy in children under 2-years old with ITP were investigated.Patients were followed for one year after being discharged from the hospital. Results The risk of the disease developing into chronic form was higher in older children (0.001). ITP in children under 3-months old was significantly associated with vaccination (p=0.007). There was no significant differences between male and female patients in regards to newly diagnosed ITP, persistent, and chronic disease status (p = 0.21). No significant difference in bleeding symptoms was observed between patients under 3-months old and 3 to 24-months old (p=0.18). Conclusion Infantile ITP respond favorably to treatment. The risk of the disease developing into chronic form is higher in 3-to-24-month-old children compared to under-three-month olds.

  14. 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)

  15. Idiopathic incus necrosis: Analysis of 4 cases.

    Science.gov (United States)

    Kansu, Leyla; Yilmaz, Ismail; Akdogan, Volkan; Avci, Suat; Ozluoglu, Levent

    2013-02-01

    We evaluated ossicular chain reconstruction in patients with idiopathic incus necrosis who have conductive hearing loss and an intact ear drum. The study included four patients (3 women and 1 man; the ages of the patients were 22, 31, 35, and 56 years, respectively) with unilateral conductive hearing loss, no history of chronic serous otitis media, an intact ear drum, normal middle ear mucosa, and necrosis of the long processes of the incus. On preoperative pure tone audiometry, air-bone gaps were 24, 25, 38, and 33 dB. Bilateral tympanometry and temporal bone computed tomography results were normal. All 4 patients underwent an exploratory tympanotomy. During the operation, the mucosa of the middle ear was normal, with a mobile stapes foot plate and malleus. No evidence of any granulation tissue was found; however, necrosis of the incus long processes was seen. For ossicular reconstruction, we used tragal cartilage between the incus and the stapes in 1 patient; in the other 3 patients, glass ionomer bone cement was used (an interposition cartilage graft also was used in the patients who received the glass ionomer bone cement). In all patients, air-bone gaps under 20 dB were established in the first year after surgery. In the ossicular disorders within the middle ear, the incus is the most commonly affected ossicle. While, the most common cause of these disorders is chronic otitis media, it may be idiopathic rarely. Several ossicular reconstruction techniques have been used to repair incudostapedial discontinuity. PMID:23460219

  16. 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

    haematology analysers and commonly used methods for testing platelet function. The test principles, advantages and disadvantages of each test are described. We also evaluate the current literature regarding the clinical utility of the test for prediction of bleeding in thrombocytopenia in haematological and...... 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. To...... fully uncover the clinical utility of platelet-related tests, information about the prevalence of platelet function defects in thrombocytopenic conditions is required. Finally, knowledge of the performance in thrombocytopenic samples from patients is essential....

  17. 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...... 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....

  18. Idiopathic Pulmonary Haemosiderosis

    Directory of Open Access Journals (Sweden)

    Gaurav Vishal

    2013-06-01

    Full Text Available We report a case of idiopathic pulmonary hemosiderosis (IPH in a 8year old female who presented with anaemia, breathlessness, puffiness of face and grade 4 clubbing. She had been investigated a number of times at multiple hos-pitals and had received multiple blood transfusions considering iron deficiency anaemia. Diagnosis of IPH was sus-pected on basis of clinical history of persistent respiratory distress, hypoxemia, repeated anaemia episodes and diffuse bilateral infiltrates on chest X ray. [Natl J Med Res 2013; 3(3.000: 292-293

  19. Successful management of thrombotic thrombocytopenic purpura associated with pregnancy.

    Science.gov (United States)

    Uğur Bilgin, Aynur; Karaselek, Mehmet Ali; Camlı, Kazım

    2014-06-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, severe, potentially life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, altered mental status, fever, and renal abnormalities. It can be seen at any age or sex but affects women of childbearing age more commonly. Pregnancy is known as one of the most common precipitating events for the onset of TTP and occurs mostly in the late third trimester or during the puerperium. Because of relatively low prevalence of pregnancy-related TTP, here we report the clinical characteristics and successful outcomes of 7 women with pregnancy-related TTP. Median age of patients was 25 (19-32). While 4 out of 7 women were primiparous, others were multiparous. Total plasma exchange (TPE) procedure was started within 24h after admission to our hospital. All patients got into complete remission without any maternal mortality. Fetal mortality was found to be 28%. Pregnancy-related TTP is still associated with high maternal and fetal mortality rates. However, the prognosis of TTP has improved dramatically with early diagnosis and plasma- based therapies. PMID:24667160

  20. 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

  1. 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.

  2. 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

  3. 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...

  4. 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....... As in normal bone marrow, argyrophilic fibres and type III collagen displayed a close co-distribution, which was also demonstrated for type IV collagen and laminin. While normal bone marrow sinusoids had discontinuous basement membranes, fibrosing bone marrow was characterized by endothelial cell...

  5. 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.

  6. 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

  7. 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.

  8. 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.

  9. 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.

  10. Medical image of teh week: idiopathic pulmonary hemosiderosis

    OpenAIRE

    Reyes N; Snyder L; Arteaga V

    2014-01-01

    No abstract available. Article truncated at 150 words. The patient is a 40 year-old man who was diagnosed with Idiopathic Pulmonary Hemosiderosis (IPH) at the age of three. He has recurrent episodes of hemoptysis several times a year that are controlled with increased doses of prednisone. He is chronically on 10 mg daily which usually control his symptoms. A HRCT scan of the chest shows predominantly upper lung cystic changes both subpleural and clustered with a honeycomb appearance superimpo...

  11. 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...

  12. 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...

  13. 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

  14. Low serum levels of interleukin-6 in children with post-infective acute thrombocytopenic purpura.

    Science.gov (United States)

    Gangarossa, S; Romano, V; Munda, S E; Sciotto, A; Schilirò, G

    1995-08-01

    Interleukin-6 plays an important role in host defense mechanisms and it appears to be a major mediator of the acute-phase response. IL-6 is also an important thrombocytopoietic factor. High serum levels of IL-6 are present in reactive thrombocytosis. The number and function of circulating platelets are the major factors that affect megakaryocytopoiesis by thrombopoietin. High levels of thrombopoietin have been observed in patients with thrombocytopenic purpura. To evaluate a possible thrombopoietin-like function of IL-6, we measured IL-6 levels in the serum of patients affected by post-infective acute thrombocytopenic purpura using a sensitive ELISSA assay. As controls, we studied normal subjects and patients with reactive thrombocytosis. No significant difference was observed between thrombocytopenic patients and normal controls. High IL-6 levels were present in patients with reactive thrombocytosis. In conclusion, we had not observed high levels of IL-6 in acute thrombocytopenic purpura and, very probably, IL-6 is not involved in the regulation of platelet mass for the hemostatic function. The thrombocytopoietic activity of IL-6 is another acute-phase response and it is consistent with the other functions of this cytokine. This suggests an active participation of platelets in host defense mechanisms. PMID:7628586

  15. 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.

  16. New Advances in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Jing-Long Huang

    2012-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA comprises a group ofheterogeneous disorders of chronic arthritis in childhood withno apparent etiology. Juvenile idiopathic arthritis is the mostcommon pediatric rheumatic disease and is associated withsignificant long-term morbidity and mortality. There have beenmajor advances in recent years in our understanding of thepathogenesis of JIA, the definition of disease control, and biological treatments for JIA. Multiple environmental and geneticfactors have been linked with the onset and / or the exacerbation of JIA, including perinatal factors, viral and bacterialinfections, epigenetic factors, and malnutrition. However, nosingle causative factor has been identified to date. As ourunderstanding of the complex network of immune cells andinflammatory cytokines has improved, biologics have beendeveloped to modulate the inflammatory processes. Indeed, anumber of such biologics have been demonstrated effective for the treatment of JIA.Although biologic agents may alleviate the inflammation associated with JIA and preventdisability caused by joint destruction, continued and comprehensive observation is requiredto determine the long-term outcomes associated with such treatment

  17. 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

  18. 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

  19. 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

  20. 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

  1. Role of PET in Diagnosis of Idiopathic Retroperitoneal Fibrosis - Case Study

    International Nuclear Information System (INIS)

    Idiopathic retroperitoneal fibrosis, together with inflammatory aneurysm of the abdominal aorta and perianeurysmal retroperitoneal fibrosis, belongs to a group of diseases called chronic periaortitis. Chronic periaortitis is a rare entity characterized by the development of an inflammatory fibrotic process surrounding large vessels and retroperitoneal organs. Imaging modalities such as ultrasonography, CT and MRI are used and usually a needle biopsy is needed to confirm the diagnosis. The inflammatory process can be depicted also by positron emission tomography (PET) and there is also the possibility of the fusion of the CT and PET images. A case of a 60-years old man with idiopathic retroperitoneal fibrosis is presented.

  2. 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 ...

  3. Idiopathic Thoracic Epidural Lipomatosis with Chest Pain

    OpenAIRE

    Lee, Sang-Beom; Park, Hyung-Ki; Chang, Jae-Chil; Jin, So-Young

    2011-01-01

    Spinal epidural lipomatosis (SEL) is an overgrowth of the normally encapsulated adipose tissue in the epidural space around the spinal cord in the thoracic and lumbar spine causing compression of the neural components. Idiopathic SEL in non-obese patients is exceptional. Idiopathic SEL can result in thoracic myelopathy and lumbar radiculopathy. A thoracic radiculopathy due to idiopathic SEL has not been reported yet. We report a case of idiopathic SEL with intractable chest pain and paresthes...

  4. 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.

  5. 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...

  6. 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...

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

    Science.gov (United States)

    Flanagan, Frances; Glackin, Louisa; Slattery, Dubhfeasa M

    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. PMID:22573417

  8. 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.

  9. Thrombotic thrombocytopenic purpura: MRI demonstration of persistent small cerebral infarcts after clinical recovery

    International Nuclear Information System (INIS)

    Abnormalities in the brain of patients with thrombotic thrombocytopenic purpura (TTP) are infrequent on MRI, often reversible and mainly limited to symptomatic stages of the disease. We report a case in which high-resolution MRI demonstrated multiple persistent small cortical infarcts after clinical remission. High-resolution MRI investigations may detect clinically latent but permanent brain damage, and complement clinical judgement in guiding therapeutic decisions. (orig.)

  10. 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

  11. 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...

  12. 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...

  13. 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

  14. 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.

  15. Radiological case: idiopathic pulmonary hemosiderosis

    OpenAIRE

    Nascimento , J.; Morais, L; Macedo, F.

    2012-01-01

    ABSTRACT We present a case of a four year-old child with a second episode of iron deficiency anaemia. Following fever, cough and hemoptysis a chest X-ray and chest CT were performed. Bilateral and patchy areas of ground glass and consolidation were noted in the setting of intrapulmonary hemorrhage. The final diagnosis was idiopathic pulmonary hemosiderosis.

  16. 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

  17. 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

  18. 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…

  19. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Westergaard, Jytte; Stoltze, Kaj;

    2006-01-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile...... idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases....

  20. 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.

  1. Púrpura trombocitopénico inmune asociado a infección por virus hepatitis C en paciente hemofílico: Caso clínico Immune thrombocytopenic purpura associated to hepatitis C virus infection: Report of one case

    Directory of Open Access Journals (Sweden)

    LILIAN PILLEUX C

    2010-09-01

    Full Text Available Hepatitis C Virus (HCV is a cause of secondary chronic immune thrombocytopenic purpura (ITP. We report a 43 year old man with mild Hemophilia A, who received blood transfusions during childhood, that consulted for epistaxis and ecchymoses. The laboratory showed a platelet count of 23.000/mm³, positive HCV serology and elevated transaminases. Steroids administered in pulses followed by oral doses resulted in a partial response requiring a second pulse and association of azathioprine. A steroidal diabetes appeared. Given his refractoriness, splenectomy and liver biopsy were performed. He continued on azathioprine maintaining a platelet count near 50.000/mm³ but continued with bleeding episodes. Liver biopsy showed a chronic active hepatitis.

  2. 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...

  3. 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

  4. The changing treatment landscape in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    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.

  5. A new direction in the pathogenesis of idiopathic pulmonary fibrosis?

    Directory of Open Access Journals (Sweden)

    Kolb Martin

    2002-01-01

    Full Text Available Abstract A recent review article suggested that idiopathic pulmonary fibrosis (IPF is a disease that is associated more with abnormal wound healing than with inflammation. Data derived from transgenic and gene transfer rodent models suggest that lung inflammation may be a necessary but insufficient component of IPF, and that at some point in the natural history of the disease IPF becomes no longer dependent on the inflammatory response for propagation. Altered microenvironment and involvement of epithelial cell/mesenchymal cell interaction are the most likely contributors to the pathogenesis of this chronic progressive disorder.

  6. Developments in the management of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    2015-07-01

    Idiopathic pulmonary fibrosis (IPF), a chronic progressive fibrotic interstitial lung disease (ILD) of unknown origin,(1) has seen significant change in its management over the last 15 years.(2) In addition, two antifibrotic drugs that have 'orphan designation' from the European Medicines Agency, ▼pirfenidone and ▼nintedanib, have received marketing authorisation approval for use in the UK.(3-5) Here we provide an overview of IPF and the importance of supportive and symptomatic care, and review the evidence for antifibrotic therapy. PMID:26159700

  7. Genetics and Idiopathic Interstitial Pneumonias.

    Science.gov (United States)

    Chu, Sarah G; El-Chemaly, Souheil; Rosas, Ivan O

    2016-06-01

    Significant progress has been made in elucidating the genetics of parenchymal lung diseases, particularly idiopathic interstitial pneumonias (IIPs). IIPs are a heterogeneous group of diffuse interstitial lung diseases of uncertain etiology, diagnosed only after known causes of interstitial lung disease have been excluded. Idiopathic pulmonary fibrosis is the most common IIP. Through candidate gene approaches and genome wide association studies, much light has been shed on the genetic origins of IIPs, enhancing our understanding of risk factors and pathogenesis. However, significant work remains to be accomplished in identifying novel genetic variants and characterizing the function of validated candidate genes in lung pathobiology, their interplay with environmental factors, and ultimately translating these discoveries to patient care. PMID:27231858

  8. 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...

  9. 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....

  10. 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...

  11. 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)

  12. 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.

  13. 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....

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. 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 ...

  17. 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...

  18. 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.

  19. 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

  20. A report of three cases with thrombotic thrombocytopenic purpura (TTP) secondary to an occult gastric adenocarcinoma

    International Nuclear Information System (INIS)

    Thrombotic thrombocytopenic purpura (TTP) is a disseminated form of thrombotic microangiopathy with clinical findings consisting of fever, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fluctuating neurologic impairment and renal dysfunction. However, Microangiopathic hemolytic anemia has been described in association with disseminated malignancies, most commonly adenocarcinoma of the breast or stomach. We present three patients with microangiopathic anemia in whom metastatic cancer was finally diagnosed; however, they died of refractory hemolytic anemia in the end. The occurrence of microangiopathic hemolytic anemia and thrombocytopenia in patients with disseminated malignant in gastric adenocarsinoma is well documented. Therefore, the diagnosis of tumor-associated TTP should be considered in unresponsive TTP patient treated with plasmapheresis. (author)

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

    Directory of Open Access Journals (Sweden)

    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.

  2. Idiopathic inflammatory myopathies and the lung

    Directory of Open Access Journals (Sweden)

    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.

  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. PMID:26053291

  4. 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

  5. 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

  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. 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

  9. 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...

  10. 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

  11. Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis.

    Science.gov (United States)

    Siu, K K; Li, Rever; Lam, S Y

    2015-04-01

    This report demonstrates pulmonary haemorrhage as a differential cause of anaemia. Idiopathic pulmonary hemosiderosis is a rare disease in children; it is classically described as a triad of haemoptysis, pulmonary infiltrates on chest radiograph, and iron-deficiency anaemia. However, anaemia may be the only presenting feature of idiopathic pulmonary hemosiderosis in children due to occult pulmonary haemorrhage. In addition, the serum ferritin is falsely high in idiopathic pulmonary hemosiderosis which increases the diagnostic difficulty. We recommend that pulmonary haemorrhage be suspected in any child presenting with iron-deficiency anaemia and persistent bilateral pulmonary infiltrates. PMID:25904566

  12. Abdominal Massage for the Treatment of Idiopathic Constipation in Children with Profound Learning Disabilities: A Single Case Study Design

    Science.gov (United States)

    Moss, Lucy; Smith, Melanie; Wharton, Sarah; Hames, Annette

    2008-01-01

    Chronic constipation is a common problem in people with learning disabilities. Treatment often involves dietary changes or long-term laxative use. The participants were five children with profound learning disabilities and additional physical difficulties. Their long-standing idiopathic constipation was managed by laxatives. Intervention lasted up…

  13. 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.

  14. 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

  15. Optimal management of idiopathic macular holes.

    Science.gov (United States)

    Madi, Haifa A; Masri, Ibrahim; Steel, David H

    2016-01-01

    This review evaluates the current surgical options for the management of idiopathic macular holes (IMHs), including vitrectomy, ocriplasmin (OCP), and expansile gas use, and discusses key background information to inform the choice of treatment. An evidence-based approach to selecting the best treatment option for the individual patient based on IMH characteristics and patient-specific factors is suggested. For holes without vitreomacular attachment (VMA), vitrectomy is the only option with three key surgical variables: whether to peel the inner limiting membrane (ILM), the type of tamponade agent to be used, and the requirement for postoperative face-down posturing. There is a general consensus that ILM peeling improves primary anatomical hole closure rate; however, in small holes (400 µm) and chronic holes (>1-year history) are usually treated with long-acting gas and posturing. Several studies on posturing and gas choice were carried out in combination with ILM peeling, which may also influence the gas and posturing requirement. Combined phacovitrectomy appears to offer more rapid visual recovery without affecting the long-term outcomes of vitrectomy for IMH. OCP is licensed for use in patients with small- or medium-sized holes and VMA. A greater success rate in using OCP has been reported in smaller holes, but further predictive factors for its success are needed to refine its use. It is important to counsel patients realistically regarding the rates of success with intravitreal OCP and its potential complications. Expansile gas can be considered as a further option in small holes with VMA; however, larger studies are required to provide guidance on its use. PMID:26834454

  16. Idiopathic interstitial pneumonias: Classification revision

    Directory of Open Access Journals (Sweden)

    Demosthenes Bouros MD, PhD, FCCP

    2010-01-01

    Full Text Available The American Thoracic Society (ATS, the European Respiratory Society (ERS and the Japan Respiratory Society (JRS are planning a revision of the 2002 ATS/ERS International Multidisciplinary Classification of Idiopathic Interstitial Pneumonias (IIPs1. In two years’ time it will be 10 years since its publication and with a view to publishing the revision after 10 years (i.e., in 2012, a steering committee has been established, which met in New Orleans during ATS congress in May 2010 and more recently in Barcelona during the ERS congress (Photo. The committee will meet again during the ATS and the ERS congresses that will be held in the next two years, with an additional meeting in Modena, Italy, in Αpril 2011.

  17. 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

  18. 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 ...

  19. 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...

  20. Developmental dyscalculia in children and adolescents with idiopathic epilepsies in a Brazilian sample

    OpenAIRE

    Ursula Thomé; Sandra Regina da Paixão Alves; Sabrina Mendonça Guerreiro; Célia Regina Carvalho Machado da Costa; Fernanda de Souza Moreira; Andrea Bandeira Lima; Maria Rita Ferreira Tavares; Heber de Souza Maia Filho

    2014-01-01

    Epilepsy is one of the most prevalent chronic disorders of childhood which can threaten child development and mental health. Among cognitive disorders, dyscalculia is one of the most important. In this study, 39 children and adolescents with idiopathic epilepsy underwent clinical and neuropsychological assessment to determine the intellectual level, math skills, reading and writing performance and neuropsychological profile. It was observed that the mathematical ability was below schooling ex...

  1. Medical and Surgical Treatment of Idiopathic Granulomatous Lobular Mastitis: A Benign Inflammatory Disease Mimicking Invasive Carcinoma

    OpenAIRE

    Gurleyik, Gunay; Aktekin, Ali; Aker, Fugen; Karagulle, Hikmet; Saglamc, Abdullah

    2012-01-01

    Purpose Idiopathic granulomatous lobular mastitis (IGLM) is a rare chronic inflammatory disease of the breast with obscure etiology that mimics invasive carcinoma both clinically and radiologically. The treatment of IGLM remains controversial. The aim of proper management is to use a combination of medical and surgical treatment of this benign condition to achieve a good cosmetic result and low recurrence rate. Methods A retrospective analysis of 19 patients with IGLM is performed based on th...

  2. 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...

  3. A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis

    OpenAIRE

    Chin, Chana I.C.; Kohn, Shirleen Loloyan; Keens, Thomas G.; Margetis, Monique F.; Kato, Roberta M.

    2015-01-01

    Background Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology characterized by chronic pulmonary hemorrhage and presents with a triad of anemia, hemoptysis and pulmonary infiltrates. IPH is a diagnosis of exclusion with a variable and disparate clinical course. Despite existing therapies, few children achieve full remission while others have recurrent hemorrhage, progressive lung damage, and premature death. Methods We surveyed physicians who care for patients wit...

  4. Psychological Profile in Children and Adolescents with Severe Course Juvenile Idiopathic Arthritis

    OpenAIRE

    Emanuela Russo; E. Trevisi; Zulian, F; M. A. Battaglia; Viel, D.; Facchin, D.; Chiusso, A.; Martinuzzi, A

    2012-01-01

    Objective. Juvenile Idiopathic Arthritis (JIA) is the most common chronic pediatric rheumatic disease. It is recognized that only reliance on clinical signs of disease outcome is inadequate for understanding the impact of illness and its treatment on child's life and functioning. There is a need for a multidisciplinary and holistic approach to children with arthritis which considers both physical and emotional functioning. This study investigated the psychosocial functioning of children and a...

  5. 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...

  6. 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...

  7. 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...

  8. 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 ...

  9. 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. ...

  10. 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.

  11. [Chronic cough: common problem, discontended patients].

    Science.gov (United States)

    Koskela, Heikki; Purokivi, Minna

    2014-01-01

    The prevalence of chronic cough is 10 to 15%. It has a strong negative impact on the patients' quality of life and it often causes depression. Many patients find medications unhelpful. Successful management of chronic cough requires the identification of the underlying condition like chronic rhinosinusitis, asthma, and asthma-like syndrome, and esophageal reflux disease. If the underlying condition cannot be identified or if the drug trials fail to help, the patient probably suffers from idiopathic chronic cough. A new paradigm has been introduced in which chronic cough is regarded as a primary condition. PMID:25558624

  12. 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....

  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. Adolescent-onset idiopathic hemifacial spasm

    OpenAIRE

    Jianfeng Liang; Zhuangli Guo; Li Zhang; Yanbing Yu

    2014-01-01

    Aims: To study the clinical manifestations, illness-related factors and microsurgical management of adolescent-onset idiopathic hemifacial spasm. Materials and Methods: Of the 1221 microvascular decompression procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3%) were in adolescent age (≤18). Results: Clinical manifestations in the adolescent-onset were typical, but milder when compared with late-onset. Gender, thickening and adhesion of the arachnoid...

  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. 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

  17. Controversies in the diagnosis and management of childhood acute immune thrombocytopenic purpura.

    Science.gov (United States)

    Segel, George B; Feig, Stephen A

    2009-09-01

    Acute immune thrombocytopenic purpura (ITP) occurs most commonly in young children who present with severe isolated thrombocytopenia and purpura. A marrow examination is not required unless glucocorticoids are used, lest treatment mask incipient acute lymphoblastic leukemia, but controversy exists here. The recommendations for evaluation and management remain controversial, since prospective controlled trials have not been done. There is some consensus based on experience and empiric data. Almost all children with acute ITP will recover completely without therapy. Although the various treatments may increase the platelet count, they do not influence the outcome of the illness, may increase cost, and cause significant side effects. Therefore, careful observation may be the best management option for the patient with ITP, in the absence of severe bleeding. The data available relevant to these issues are discussed. PMID:19165890

  18. 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

  19. Immune thrombocytopenic purpura induced by intestinal tuberculosis in a liver transplant recipient.

    Science.gov (United States)

    Lugao, Renata dos Santos; Motta, Marina Pamponet; de Azevedo, Matheus Freitas Cardoso; de Lima, Roque Gabriel Rezende; Abrantes, Flávia de Azevedo; Abdala, Edson; Carrilho, Flair José; Mazo, Daniel Ferraz de Campos

    2014-07-01

    A variety of clinical manifestations are associated directly or indirectly with tuberculosis. Among them, haematological abnormalities can be found in both the pulmonary and extrapulmonary forms of the disease. We report a case of immune thrombocytopenic purpura (ITP) associated with intestinal tuberculosis in a liver transplant recipient. The initial management of thrombocytopenia, with steroids and intravenous immunoglobulin, was not successful, and the lack of tuberculosis symptoms hampered a proper diagnostic evaluation. After the diagnosis of intestinal tuberculosis and the initiation of specific treatment, a progressive increase in the platelet count was observed. The mechanism of ITP associated with tuberculosis has not yet been well elucidated, but this condition should be considered in cases of ITP that are unresponsive to steroids and intravenous immunoglobulin, especially in immunocompromised patients and those from endemic areas. PMID:25009408

  20. 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.

  1. Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

    Science.gov (United States)

    Portillo, Karina; Guasch Arriaga, Ignasi; Ruiz-Manzano, Juan

    2015-10-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management. PMID:26099364

  2. Elevated common acute lymphoblastic leukemia antigen expression in pediatric immune thrombocytopenic purpura.

    Science.gov (United States)

    Cornelius, A S; Campbell, D; Schwartz, E; Poncz, M

    1991-01-01

    Bone marrow examination is often performed in thrombocytopenic children to distinguish immune thrombocytopenic purpura (ITP) from acute leukemia. We describe a patient with thrombocytopenia and 50% common acute lymphoblastic leukemia antigen (CALLA) positivity in his marrow who was subsequently shown to have ITP. CALLA (CD10) is a surface antigen found in early B-lymphocytes and is elevated in most cases of childhood acute lymphoblastic leukemia (ALL). This case prompted us to prospectively study the frequency of immature lymphocyte populations in children with ITP. Fourteen patients with acute ITP and five with other conditions were studied. The two groups were comparable with respect to age: ITP mean, 4.3 (range 0.3-15.5) years; control mean, 5.8 (0.6-13.8) years. The ITP group had a significantly higher percentage of CD10 positive bone marrow lymphocytes (p = 0.007). Five of the 10 patients younger than 4 years of age in the ITP group had CD10 levels of greater than 30%, which is in the leukemic range, whereas none of the control patients had a CD10 levels of greater than 17% (p = 0.003). There was good correlation between CD10 positivity and B4 positivity indicating that both of these markers arise from the same population of immature B-lymphocytes. None of the ITP patients who were older than 4 years had a CD10 level of greater than 30%. We conclude that it is common to have an increase in the proportion of immature lymphocytes in the marrow of young children with ITP. The cause of this increase in CD10 positive cells is unknown.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1827572

  3. [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

  4. Activation of Oncogenic Pathways in Idiopathic Pulmonary Fibrosis

    Directory of Open Access Journals (Sweden)

    Giulia M. Stella

    2014-10-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. The most recent hypotheses on IPF pathogenesis suggest a central role of epithelial cell damage, followed by a dysregulated molecular cross talk between epithelial cells and fibroblasts. Thus, IPF progression has often been assimilated to that of cancer, and several signaling patterns appear to be disrupted in both diseases. Here, we analyze the expression in an IPF series of a panel of molecules, which are known to play a role in tumorigenic process. Our findings, although preliminary, reveal that IPF landscape is enriched in neoplastic potential expressed in a context of complex genomic polyclonality and cellular heterogeneity. These results provide a rationale for further investigations aimed to exploit—in a similar fashion to cancer—targeted therapies for a “precision medicine” approach to IPF.

  5. 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

  6. Medical image of teh week: idiopathic pulmonary hemosiderosis

    Directory of Open Access Journals (Sweden)

    Reyes N

    2014-07-01

    Full Text Available No abstract available. Article truncated at 150 words. The patient is a 40 year-old man who was diagnosed with Idiopathic Pulmonary Hemosiderosis (IPH at the age of three. He has recurrent episodes of hemoptysis several times a year that are controlled with increased doses of prednisone. He is chronically on 10 mg daily which usually control his symptoms. A HRCT scan of the chest shows predominantly upper lung cystic changes both subpleural and clustered with a honeycomb appearance superimposed on a background of diffuse ground glass opacification. Typical HRCT findings include patchy scattered areas of ground glass opacity and consolidation that usually involve the perihilar and lower aspects of the lungs. However, case reports of rare findings of multiple honeycomb cystic changes have been reported that are thought to be a result of progressive fibrotic changes from hemosiderin deposition in the interstitium (1. These honeycomb cysts may represent sites of more severe and recurrent alveolar hemorrhage in adults ...

  7. 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

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

    Science.gov (United States)

    Chogtu, Bharti; Malik, Daliparty Vasudev

    2016-01-01

    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

  9. 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.

  10. 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.

  11. 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

  12. Chronic Cutaneous Hyalophomycosis by Paecilomyces

    OpenAIRE

    BOUFFLETTE, Nicolas; Arrese Estrada, Jorge; Leonard, Philippe; Nikkels, Arjen

    2014-01-01

    Paecilomyces lilacinus is a ubiquitous saprophytic fungus that rarely causes infections in humans, frequently affecting the eyes and the skin. Cutaneous and subcutaneous infections mainly occur in immunocompromised hosts but have occasionally been reported in immunocompetent patients. The clinical spectrum is highly heterogeneous and diagnosis is often delayed. A 60-year-old woman with idiopathic chronic necrotizing vasculitis treated since 10 years with a series of immunosuppressive thera...

  13. 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)

  14. 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)

  15. 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)

  16. 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.

  17. 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 ...

  18. 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...

  19. 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

  20. 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.

  1. 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...

  2. 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

  3. 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

  4. 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...

  5. Optimal management of idiopathic macular holes

    Directory of Open Access Journals (Sweden)

    Madi HA

    2016-01-01

    Full Text Available Haifa A Madi,1,* Ibrahim Masri,1,* David H Steel1,2 1Sunderland Eye Infirmary, Sunderland, 2Institute of Genetic Medicine, Newcastle University, International Centre for Life, Newcastle, UK *These authors contributed equally to this work Abstract: This review evaluates the current surgical options for the management of idiopathic macular holes (IMHs, including vitrectomy, ocriplasmin (OCP, and expansile gas use, and discusses key background information to inform the choice of treatment. An evidence-based approach to selecting the best treatment option for the individual patient based on IMH characteristics and patient-specific factors is suggested. For holes without vitreomacular attachment (VMA, vitrectomy is the only option with three key surgical variables: whether to peel the inner limiting membrane (ILM, the type of tamponade agent to be used, and the requirement for postoperative face-down posturing. There is a general consensus that ILM peeling improves primary anatomical hole closure rate; however, in small holes (<250 µm, it is uncertain whether peeling is always required. It has been increasingly recognized that long-acting gas and face-down positioning are not always necessary in patients with small- and medium-sized holes, but large (>400 µm and chronic holes (>1-year history are usually treated with long-acting gas and posturing. Several studies on posturing and gas choice were carried out in combination with ILM peeling, which may also influence the gas and posturing requirement. Combined phacovitrectomy appears to offer more rapid visual recovery without affecting the long-term outcomes of vitrectomy for IMH. OCP is licensed for use in patients with small- or medium-sized holes and VMA. A greater success rate in using OCP has been reported in smaller holes, but further predictive factors for its success are needed to refine its use. It is important to counsel patients realistically regarding the rates of success with

  6. 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.

  7. 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

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

    Science.gov (United States)

    Wannhoff, Andreas; Müller, Oliver J; Friedrich, Kilian; Rupp, Christian; Klöters-Plachky, Petra; Leopold, Yvonne; Brune, Maik; Senner, Mirja; Weiss, Karl-Heinz; Stremmel, Wolfgang; Schemmer, Peter; Katus, Hugo A; Gotthardt, Daniel N

    2014-01-01

    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 future

  9. Idiopathic pulmonary fibrosis: an Australian perspective.

    Science.gov (United States)

    Prasad, J; Holland, A E; Glaspole, I; Westall, G

    2016-06-01

    Idiopathic pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a dismal median survival of 3 years. Patients typically develop progressive dyspnoea and increasing exercise limitation. With a rising incidence and prevalence, an unpredictable disease course and limited treatment options, it is rapidly becoming an important public health concern. To date, lung transplantation has been the sole viable hope for treatment for those who qualify. However, the landscape of idiopathic pulmonary fibrosis management is changing, with the recent emergence of novel pharmacotherapy shown to have a favourable influence on the natural history of this disease. PMID:27257148

  10. Management of severe and rigid idiopathic scoliosis.

    Science.gov (United States)

    Teixeira da Silva, Luis Eduardo Carelli; de Barros, Alderico Girão Campos; de Azevedo, Gustavo Borges Laurindo

    2015-07-01

    Frequently, severe idiopathic scoliosis patients are first seen in a spine centre after years of deformity evolution, presenting with large curves, severe rib hump, shoulder and trunk imbalance and cardiorespiratory complications related to neglected scoliosis. Severe rigid idiopathic scoliosis has traction, internal temporary distraction, releases, osteotomies and apical vertebral resection are often used in combination to achieve optimal results. Indications must be tailored by surgeons considering resources, deformity characteristics and patient's profile. Vertebral resection procedures may have potential neurological and clinical risks and should be one of the last treatment options performed by experienced surgical team. Neuromonitoring is essential during these procedures. PMID:26033753

  11. Atypical presentation of acute idiopathic megacolon in a 14-year-old patient

    Directory of Open Access Journals (Sweden)

    B. Barakat

    2012-12-01

    Full Text Available In clinical practice the term “megacolon” is used to indicate a marked dilatation of the cecum and the sigmoid colon (>12 and 6.5 cm, respectively (1. From a clinical standpoint, a megacolon can be classified as chronic or acute depending on its clinical presentation. Chronic megacolon typically refers to a congenital disorder in which the enteric nervous system (ENS supplying the colon does not develop properly, thereby leaving the distal segments of the viscus without myenteric and submucosal ganglia (i.e. Hirschsprung’s disease (2. Other cases of non-aganglionic chronic megacolon can be secondary to variety of conditions such as Chagas’ disease and neurodegenerative diseases (e.g. Parkinson’s and Alzheimer’s diseases, leading to or associated with ENS abnormalities (3. The acute form of megacolon, also referred to as Ogilvie’s syndrome, is characterized by a predominant involvement of the cecum and right colon usually affecting elderly patients undergoing surgery (e.g. orthopedic procedures or taking medications altering gut motility (e.g. opioids or antidepressants (4. Some forms of acute megacolon, however, can be idiopathic in origin since no underlying etiology can be identified. Patients with acute idiopathic megacolon usually have a longstanding history of constipation, often accompanied by laxative abuse, and their clinical presentation is characterized by abdominal distension and severe pain with radiological evidence of stool impacted in the colon and rectum (1, 4. The case herein reported represents an unusual form of acute idiopathic megacolon characterized by massive descending and sigmoid colon distension complicated with a volvulus in a 14-year-old boy with no Hirschsprung’s disease. In addition, just to increase the peculiarity of this case report, the patient had an unremarkable clinical record, and never suffered from chronic constipation in the past.

  12. Acquired thrombotic thrombocytopenic purpura after vascular prosthesis implantation for impending rupture of an abdominal aortic aneurysm.

    Science.gov (United States)

    Naito, Chiaki; Ogawa, Yoshiyuki; Yanagisawa, Kunio; Ishizaki, Takuma; Mihara, Masahiro; Handa, Hiroshi; Isonishi, Ayami; Hayakawa, Masaki; Matsumoto, Masanori; Nojima, Yoshihisa

    2016-01-01

    Acquired thrombotic thrombocytopenic purpura (TTP) is caused by autoantibodies against ADAMTS13. TTP patients run a rapidly fatal course unless immediate plasma exchange (PEX) is initiated upon diagnosis. Herein, we report a 72-year-old man with TTP, which developed after he underwent artificial blood vessel replacement surgery for an abdominal aneurysm with impending rupture. In the perioperative period, the patient received several platelet transfusions for severe thrombocytopenia (minimum platelet count: 0.6×10(4)/μl). Thereafter, he was admitted to our department for rapidly progressing coma with multiple cerebral infarctions, and was transferred to the ICU. Based on the tentative diagnosis of TTP, we immediately began PEX and steroid pulse therapy. The diagnosis was confirmed thereafter by markedly reduced ADAMTS13 activity (<0.5%) and his being positive for the ADAMTS13 inhibitor. We performed PEX for five consecutive days and administered high-dose prednisolone (PSL). On the second hospital day (HD), his platelet count rose along with improvement of his consciousness level. The ADAMTS13 inhibitor was not detected on the 10th HD. TTP did not relapse and his general condition improved despite tapering of PSL. In this case, by closely monitoring ADAMTS13-related parameters and minimizing the number of plasma exchanges, the patient was able to achieve a remission without the use of boosting inhibitors. PMID:27076251

  13. [Sudden death associated with myocardial damage caused by microthrombi in a patient with thrombotic thrombocytopenic purpura].

    Science.gov (United States)

    Yamamoto, Kiyoko; Hattori, Yukinori; Shimada, Koki; Araki, Yoko; Adachi, Tatsuya; Tsushita, Keitaro

    2015-11-01

    We describe a 35-year-old woman with Down's syndrome who was admitted to a clinic with anorexia and vomiting. Since laboratory findings showed anemia (Hb 7.4 g/dl) and thrombocytopenia (0.5 × 10⁴/μl), she was transferred to our hospital for treatment. Further laboratory examinations revealed schistocytes, LDH elevation, and a negative Coombs' test. Thrombotic thrombocytopenic purpura (TTP) was suspected. Plasma exchange (PEX) and prednisolone administration were thus immediately initiated. Prior to these treatments, ADAMTS13 activity was less than 5% and inhibitors were detected at a level of 0.8 Bethesda U/ml. Although her platelet count had risen to 13.0 × 10⁴/μl by day 6 (post 4 sessions of PEX), it had decreased to 1.8 × 10⁴/μl on day 7. Despite ongoing PEX, thrombocytopenia persisted. On day 21, she suddenly died. Autopsy findings revealed no evidence of myocardial necrosis or coronary artery thrombosis. Extensive microthrombi were, however, detected in precapillary arterioles, capillaries, and post-capillary venules of the heart. Therefore, this patient's sudden death was clinically suspected to have been caused by cardiomyopathy, which had produced cardiogenic shock. PMID:26666721

  14. Genetic variations in complement factors in patients with congenital thrombotic thrombocytopenic purpura with renal insufficiency.

    Science.gov (United States)

    Fan, Xinping; Kremer Hovinga, Johanna A; Shirotani-Ikejima, Hiroko; Eura, Yuka; Hirai, Hidenori; Honda, Shigenori; Kokame, Koichi; Taleghani, Magnus Mansouri; von Krogh, Anne-Sophie; Yoshida, Yoko; Fujimura, Yoshihiro; Lämmle, Bernhard; Miyata, Toshiyuki

    2016-03-01

    The congenital form of thrombotic thrombocytopenic purpura (TTP) is caused by genetic mutations in ADAMTS13. Some, but not all, congenital TTP patients manifest renal insufficiency in addition to microangiopathic hemolysis and thrombocytopenia. We included 32 congenital TTP patients in the present study, which was designed to assess whether congenital TTP patients with renal insufficiency have predisposing mutations in complement regulatory genes, as found in many patients with atypical hemolytic uremic syndrome (aHUS). In 13 patients with severe renal insufficiency, six candidate complement or complement regulatory genes were sequenced and 11 missense mutations were identified. One of these missense mutations, C3:p.K155Q mutation, is a rare mutation located in the macroglobulin-like 2 domain of C3, where other mutations predisposing for aHUS cluster. Several of the common missense mutations identified in our study have been reported to increase disease-risk for aHUS, but were not more common in patients with as compared to those without renal insufficiency. Taken together, our results show that the majority of the congenital TTP patients with renal insufficiency studied do not carry rare genetic mutations in complement or complement regulatory genes. PMID:26830967

  15. 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

  16. [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

  17. The role of N-acetylcysteine in the treatment of thrombotic thrombocytopenic purpura.

    Science.gov (United States)

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

    2016-05-01

    Thrombotic thrombocytopenic purpura (TTP) is an acute, thrombotic microangiopathy with a high mortality rate if left untreated. Plasma exchange (PEX) is the current standard of care. However, a significant number of patients are refractory to this treatment. N-acetylcysteine (NAC) was recently suggested as a potential therapeutic adjunct for patients with TTP. This study reports a series of three patients with TTP successfully treated with NAC in addition to standard therapy. Detailed chart reviews on these patients were conducted. We discuss clinical features, laboratory findings and management of three patients who presented with microangiopathic hemolytic anemia and thrombocytopenia. Anti-ADAMTS13 antibodies and low levels of ADAMTS13 were detected and confirmed the diagnosis of acquired TTP. Based upon their severe presentation or lack of response to initial treatment with PEX, corticosteroids and other immunosuppressive agents, NAC was added. Under this combined treatment, all three patients hada significant clinical improvement of symptoms with concurrent normalization of platelet count and ADAMTS13 activity level. This report highlights the potential therapeutic utility of NAC in the treatment of TTP. Randomized controlled studies will be required to better characterize the risk-to-benefit ratio of NAC in the treatment of TTP. PMID:26245827

  18. 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...

  19. Skin prick test in patients with chronic allergic skin disorders

    OpenAIRE

    Pooja Bains; Alka Dogra

    2015-01-01

    Background: Chronic allergic skin disorders are the inflammatory and proliferative conditions in which both genetic and environmental factors play important roles. Chronic idiopathic urticaria (CIU) and atopic dermatitis (AD) are among the most common chronic allergic skin disorders. These can be provoked by various food and aeroallergens. Skin prick tests (SPTs) represent the cheapest and most effective method to diagnose type I hypersensitivity. Positive skin tests with a history suggestive...

  20. 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.

  1. 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.

  2. 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 ...

  3. Idiopathic cervical osteomyelitis presenting as dysphagia

    OpenAIRE

    Ghaly GA; Espeso A; Fish B

    2010-01-01

    We discuss a case of idiopathic cervical epidural abscess, complicated by osteomyelitis, presenting with dysphagia as the main complaint. No predisposing factors were identified and blood cultures were negative. Case was treated conservatively by long course of IV antibiotics. We present a review of presentation of spinal epidural abscesses and indications for surgical intervention.

  4. 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.

  5. 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

  6. 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.

  7. Idiopathic Aortic Root to Right Atrial Fistula.

    Science.gov (United States)

    Campisi, Salvatore; Cluzel, Armand; Vola, Marco; Fuzellier, Jean Francois

    2016-06-01

    An aorta to right atrium fistula is rare. We report a case of idiopathic aortic root to right atrial fistula with right heart failure and review the literature. doi: 10.1111/jocs.12751 (J Card Surg 2016;31:373-375). PMID:27109166

  8. 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.

  9. Adolescent-onset idiopathic hemifacial spasm

    Directory of Open Access Journals (Sweden)

    Jianfeng Liang

    2014-01-01

    Full Text Available Aims: To study the clinical manifestations, illness-related factors and microsurgical management of adolescent-onset idiopathic hemifacial spasm. Materials and Methods: Of the 1221 microvascular decompression procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3% were in adolescent age (≤18. Results: Clinical manifestations in the adolescent-onset were typical, but milder when compared with late-onset. Gender, thickening and adhesion of the arachnoid membrane at the cerebellopontine angle and a small volume of posterior cranial fossa might be important illness-related factors for adolescent-onset idiopathic hemifacial spasm. Arterial compression was the main cause. Typical compression of offending arteries was observed in 14 patients. Compression of petrous bone crest was found in one patient. Immediate effective rate was 100%, and curative rate was 75%. The curative rate, effective rate and delayed resolution rate during the follow-up period (mean: 22.9 months were 87.5%, 100% and 12.5%, respectively. There was no recurrence. The postoperative complications (2; 12.5% included: One patient had transient moderate facial palsy and decreased hearing and one patient had transient decreased hearing and tinnitus. Conclusions: This suggests that microvascular decompression is effective in adolescent-onset idiopathic hemifacial spasm.

  10. 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 ...

  11. 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

  12. Safety and feasibility of a home-based six week resistance training program in juvenile idiopathic arthritis

    OpenAIRE

    Van Oort, Cameron; Tupper, Susan M.; Rosenberg, Alan M; Farthing, Jonathan P.; Baxter-Jones, Adam D

    2013-01-01

    Background Juvenile idiopathic arthritis (JIA), among the most common chronic diseases of childhood, can be associated with attenuated physical activity levels, reduced fitness, decreased functionality and pain. This pilot study aimed to determine the safety, feasibility and effect of a six week resistance training program in children with JIA. Methods Youth (8-18 years) with JIA participated in a home-based resistance training program. Participants reported pain on an electronic diary once a...

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

    Directory of Open Access Journals (Sweden)

    Hikmet Tekin Nacaroglu

    2015-10-01

    Full Text Available 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. Case Presentation: Although there were no growth and developmental delay and gastrointestinal symptoms like chronic diarrhea, chronic constipation, vomiting, abdominal bloating and pain in the two patients with IPH, they were diagnosed with Lane Hamilton Syndrome. After initiation of gluten-free diet, their IPH symptoms disappeared and hemoglobin levels were observed to return to normal. Conclusions: Even if there were no gastrointestinal symptoms in a patient with IPH, celiac disease should be investigated. These patients may benefit from gluten free diet and IPH symptoms may disappear.

  14. 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

  15. Phenotypic analysis of bone marrow lymphocytes from children with acute thrombocytopenic purpura.

    Science.gov (United States)

    Guiziry, Dalai E L; El, Gendy Wessam; Farahat, Nahla; Hassab, Hoda

    2005-01-01

    Hematogones are benign immature B cells that commonly populate the bone marrow of children. Their presence has been noted to interfere with the flow-cytometric analysis of acute lymphoblastic leukemia (ALL), because their immunophenotype is similar to B-precursor cell lymphoblasts. Immune-mediated thrombocytopenia is a clinical condition characterized by increased platelet destruction due to sensitization of platelets by autoantibodies. The aim of this study was to determine the incidence and clinical impact of bone marrow hematogones in cases of acute immune thrombocytopenic purpura (ITP) among children. This was done by immunophenotyping of bone marrow lymphocytes of ITP cases and controls and follow up of cases. This study was done on 25 cases of ITP, 12 females and 13 males, their age ranged from 2 to 13 years. A control group was included in the study, 15 cases of apparently healthy children with matching age and sex taken from among bone marrow donors. Cases and controls were subjected to bone marrow lymphocyte immunophenotyping with flow-cytometry to verify the presence of hematogones. A statistically significant increase in the percentage of hematogones was demonstrated in their bone marrows. An increased percentage of CD10+ lymphocytes was demonstrated; with a mean of 18+/-15.2%, CD19+ with a mean of 27+/-16.3% and CD34+ with a mean of 3.7+/-3.2%. No correlation was found between the percentage of hematogones and peripheral platelet count or bone marrow lymphocytic count. In conclusion, there is an increase in the bone marrow hematogones in ITP cases in comparison to normal controls. This could be the sequence of an immunological response to the cause which determined the disease, or the regeneration of the stem cell compartment following transient damage. PMID:16734134

  16. 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

  17. Immune thrombocytopenic purpura (ITP) associated with vaccinations: a review of reported cases.

    Science.gov (United States)

    Perricone, Carlo; Ceccarelli, Fulvia; Nesher, Gideon; Borella, Elisabetta; Odeh, Qasim; Conti, Fabrizio; Shoenfeld, Yehuda; Valesini, Guido

    2014-12-01

    Immune thrombocytopenic purpura (ITP) is an autoimmune condition characterized by low platelet count with mucocutaneous and other bleedings. Clinical manifestations may range from spontaneous formation of purpura and petechiae, especially on the extremities, to epistaxis, bleeding at the gums or menorrhagia, any of which occur usually if the platelet count is below 20,000 per μl. A very low count may result in the spontaneous formation of hematomas in the mouth or on other mucous membranes. Fatal complications, including subarachnoid or intracerebral, lower gastrointestinal or other internal bleeding can arise due to an extremely low count. Vaccines may induce ITP by several mechanisms. Vaccine-associated autoimmunity may stem not only from the antigen-mediated responses but also from other constituents of the vaccine, such as yeast proteins, adjuvants, and preservatives diluents. The most likely is through virally induced molecular mimicry. The binding of pathogenic autoantibodies to platelet and megakaryocytes may cause thrombocytopenia by different mechanisms, such as opsonization, direct activation of complement, or apoptotic pathways. The autoantibodies hypothesis is not sufficient to explain all ITP cases: In the anti-platelet antibody-negative cases, a complementary mechanism based on T cell immune-mediated mechanism has been suggested. In particular, T cell subsets seem dysregulated with an increased production of pro-inflammatory cytokines, as IFN-γ and TNF, and chemokines, as CXCL10. Vaccines are one of the most striking discoveries in human history that changed dramatically life expectancy. Nonetheless, the occurrence of adverse events and autoimmune phenomena has been described following vaccination, and ITP may represent one of this. PMID:25427992

  18. 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.

  19. A systematic approach for the diagnosis and treatment of idiopathic peptic ulcers.

    Science.gov (United States)

    Chung, Chen-Shuan; Chiang, Tsung-Hsien; Lee, Yi-Chia

    2015-09-01

    An idiopathic peptic ulcer is defined as an ulcer with unknown cause or an ulcer that appears to arise spontaneously. The first step in treatment is to exclude common possible causes, including Helicobacter pylori infection, infection with other pathogens, ulcerogenic drugs, and uncommon diseases with upper gastrointestinal manifestations. When all known causes are excluded, a diagnosis of idiopathic peptic ulcer can be made. A patient whose peptic ulcer is idiopathic may have a higher risk for complicated ulcer disease, a poorer response to gastric acid suppressants, and a higher recurrence rate after treatment. Risk factors associated with this disease may include genetic predisposition, older age, chronic mesenteric ischemia, smoking, concomitant diseases, a higher American Society of Anesthesiologists score, and higher stress. Therefore, the diagnosis and management of emerging disease should systematically explore all known causes and treat underlying disease, while including regular endoscopic surveillance to confirm ulcer healing and the use of proton-pump inhibitors on a case-by-case basis. PMID:26354049

  20. Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient

    Directory of Open Access Journals (Sweden)

    J.J. Egan

    2011-06-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF is a chronic, progressive, fatal form of diffuse interstitial lung disease. Management of IPF requires an orderly approach, with regular evaluations and implementation of both pharmacological and nonpharmacological treatments. Pulmonary rehabilitation can relieve patients from the distressing symptoms of IPF and improve quality of life. Oxygen therapy is central to treatment of all patients. Lung transplantation enhances survival in selected patients. Mechanical ventilation may be used in patients with acute exacerbations, but the prognosis is poor in these cases. Palliative care focuses on symptom management, advance directives and end-of-life planning. Patient support groups may also play an important role.

  1. Idiopathic pulmonary fibrosis: an epithelial/fibroblastic cross-talk disorder

    Directory of Open Access Journals (Sweden)

    Selman Moisés

    2001-10-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis is a chronic and usually progressive lung disorder of unknown etiology. A growing body of evidence suggests that, in contrast to other interstitial lung diseases, IPF is a distinct entity in which inflammation is a secondary and non-relevant pathogenic partner. Evidence includes the presence of similar mild/moderate inflammation either in early or late disease, and the lack of response to potent anti-inflammatory therapy. Additionally, it is clear from experimental models and some human diseases that it is possible to have fibrosis without inflammation. An evolving hypothesis proposes that IPF may result from epithelial micro-injuries and abnormal wound healing.

  2. Clinical Significance of Serum Autoantibodies in Idiopathic Interstitial Pneumonia

    OpenAIRE

    Kang, Bo Hyoung; Park, Jin Kyeong; Roh, Jae Hyung; Song, Jin Woo; Lee, Chang Keun; Kim, Miyoung; Jang, Se Jin; Colby, Thomas V.; Kim, Dong Soon

    2013-01-01

    Although autoantibodies are routinely screened in patients with idiopathic interstitial pneumonia, there are no reliable data on their clinical usefulness. The aim of this study was to investigate the prognostic value of autoantibodies for predicting the development of new connective tissue disease in these patients and also mortality. We conducted retrospective analysis of the baseline, and follow-up data for 688 patients with idiopathic interstitial pneumonia (526 with idiopathic pulmonary ...

  3. Dietary guidance of selenium supplementation for incipient idiopathic scoliotic patients

    Directory of Open Access Journals (Sweden)

    Jin-li GUO

    2013-11-01

    Full Text Available The study found that there was a correlation between trace element Se and idiopathic scoliosis, and selenium deficiency was probably one of the precipitating factor. According to Chinese dietary reference intakes and selenium content in different food, the plan for filling selenium is conducted, the dietary guidance of filing selenium for incipient idiopathic scoliotic patients is provided, and the disease development is observed so that the basis for etiology and prevention of idiopathic scoliosis can be provided.

  4. [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 6weeks duration in patients aged less than 16years 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

  5. 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...

  6. 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...

  7. 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). ...

  8. Idiopathic Hypertrophic Pyloric Stenosis in Identical Twins.

    OpenAIRE

    Ju-Bei Yen; Man-Shan Kong; Wan-Jao Wu; Chen-Sheng Huang; Kuei-Wen Chang

    2003-01-01

    Idiopathic hypertrophic pyloric stenosis (IHPS) was thought to be a congenital diseasetraditionally, even though several published reports assumed IHPS was an acquired disease.The pathogenesis and inheritance patterns of IHPS are not fully understood. Except for thefamilial recurrence of IHPS, concordance of IHPS in monozygotic or dizygotic twins wasalso noted, but occurrence in female twins is rare. From July 1992 through June 2000, 130patients were diagnosed with IHPS in our hospital includ...

  9. 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...

  10. A surgical challenge: Idiopathic scrotal elephantiasis

    OpenAIRE

    Brotherhood, Hilary Laurel; Metcalfe, Michael; Goldenberg, Larry; Pommerville, Peter; Bowman, Cameran; Naysmith, David

    2014-01-01

    Scrotal elephantiasis is a condition rarely encountered in developed nations. It is endemic in tropical regions due to the presence of filariasis (Wucheria bancrofti). We report 2 cases of idiopathic scrotal elephantiasis in Canadian citizens with no history of travel to endemic filariasis regions, malignancy, surgery or radiation. Both patients underwent complete excision of the involved tissue with reconstruction. We found that for advanced cases of scrotal lymphedema, surgery is currently ...

  11. A Case of Idiopathic Thyrotropin (TSH) Deficiency

    OpenAIRE

    Ichida, Tatsuya; Kajita, Yoshihiro

    1997-01-01

    The first case of idiopathic thyrotropin (TSH) deficiency in an old woman with thyroid functioning adenoma was reported. She got subtotal thyroidectomy before about four years of her admission to our hospital because of fatigability, puffy face and leg edema. At that time, she had low TSH and free T4 levels despite replacement therapy with desiccated thyroid. No response of only serum TSH after adminstration of combined stimulant containing TRH and repeated TRH suggested the failure of TSH se...

  12. 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...

  13. Vitamin D metabolites in idiopathic infantile hypercalcaemia.

    OpenAIRE

    Martin, N D; Snodgrass, G J; Cohen, R D; Porteous, C E; Coldwell, R D; Trafford, D J; Makin, H L

    1985-01-01

    Metabolites of vitamin D were measured in plasma from 83 patients with idiopathic infantile hypercalcaemia syndrome who were mentally handicapped but had normal calcium values at the time of the study. No significant difference was detected in the mean plasma concentrations of 25-hydroxyvitamin D2, 1,25-dihydroxyvitamin D, 24,25-dihydroxyvitamin D3, or 25,26-dihydroxyvitamin D3 between patients and age matched controls. The mean plasma concentration of 25-hydroxyvitamin D3 was significantly l...

  14. 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...

  15. 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 ...

  16. Idiopathic pulmonary hemosiderosis - a diagnostic challenge

    OpenAIRE

    Bakalli, Ilirjana; Kota, Luljeta; Sala, Durim; Celaj, Ermela; Kola, Elmira; Lluka, Robert; Sallabanda, Sashenka

    2014-01-01

    Idiopathic pulmonary hemosiderosis is a rare disorder that can occur at any age and is characterized by the triad of hemoptysis, iron deficiency anemia and diffuse pulmonary infiltrates. The clinical course is exceedingly variable especially in children and a substantial proportion of this age group is undiagnosed. It is probably due to the fact that iron deficiency anemia may be the first and the only manifestation of IPH, preceding other symptoms and signs by several months and IPH is not c...

  17. Two Sisters with Idiopathic Pulmonary Hemosiderosis

    OpenAIRE

    Mehmet Gencer; Erkan Ceylan; Muharrem Bitiren; Ahmet Koc

    2007-01-01

    Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage with unknown etiology. In the present report, the presentations of two sisters are described: one sister had IPH, eosinophilia and a high serum immunoglobulin E (IgE) level; and the other had IPH, pneumothorax, eosinophilia and a high serum IgE level. Both cases had quite unusual presentations. The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. He...

  18. Surgical treatment analysis of idiopathic esophageal achalasia

    OpenAIRE

    de Aquino, José Luis Braga; SAID, Marcelo Manzano; PEREIRA, Douglas Rizzanti; do AMARAL, Paula Casals; LIMA, Juliana Carolina Alves; LEANDRO-MERHI, Vânia Aparecida

    2015-01-01

    Background Idiopathic esophageal achalasia is an inflammatory disease of unknown origin, characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter in response to swallowing, with consequent dysphagia. Aim To demonstrate the results of surgical therapy in these patients, evaluating the occurred local and systemic complications. Methods Were studied retrospectively 32 patients, 22 of whom presented non-advanced stage of the disease (Stage I/II) and 10 wi...

  19. 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...

  20. 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...

  1. 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 ...

  2. Idiopathic pulmonary fibrosis: a historical review.

    OpenAIRE

    Homolka, J.

    1987-01-01

    Hamman and Rich are generally considered to have been the first to describe idiopathic pulmonary fibrosis (IPF) as a new clinical and pathological entity. However, several earlier reports in the German-language literature described autopsy findings consistent with IPF from a contemporary point of view. The author discusses these and later reports in a review of the history, diagnosis and treatment of the disease.

  3. 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 ...

  4. 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

  5. Invasive diagnostic techniques in idiopathic interstitial pneumonias.

    Science.gov (United States)

    Poletti, Venerino; Ravaglia, Claudia; Gurioli, Carlo; Piciucchi, Sara; Dubini, Alessandra; Cavazza, Alberto; Chilosi, Marco; Rossi, Andrea; Tomassetti, Sara

    2016-01-01

    Fibrosing interstitial lung diseases (f-ILDs) represent a heterogeneous group of disorders in which the aetiology may be identified or, not infrequently, remain unknown. Establishing a correct diagnosis of a distinct f-ILD requires a multidisciplinary approach, integrating clinical profile, physiological and laboratory data, radiological appearance and, when appropriate, histological findings. Surgical lung biopsy is still considered the most important diagnostic tool as it is able to provide lung samples large enough for identification of complex patterns such as usual interstitial pneumonitis (UIP) and nonspecific interstitial pneumonitis. However, this procedure is accompanied by significant morbidity and mortality. Bronchoalveolar lavage is still a popular diagnostic tool allowing identification of alternative diagnoses in patients with suspected idiopathic pulmonary fibrosis (IPF) when an increase in lymphocytes is detected. Conventional transbronchial lung biopsy has a very low sensitivity in detecting the UIP pattern and its role in this clinical-radiological context is marginal. The introduction of less invasive methods such as transbronchial cryobiopsy show great promise to clinical practice as they can be used to obtain samples large enough to morphologically support a diagnosis of IPF or other idiopathic interstitial pneumonias, along with fewer complications. Recent advances in the field suggest that less invasive methods of lung sampling, without significant side effects, in combination with other diagnostic methods could replace the need for surgical lung biopsy in the future. Indeed, these new multidisciplinary procedures may become the main diagnostic work-up method for patients with suspected idiopathic interstitial pneumonia. PMID:26682637

  6. 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

  7. 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.

  8. 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.

  9. 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 ...

  10. 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. PMID:26452110

  11. 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

  12. 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

  13. Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history.

    Science.gov (United States)

    Sgalla, Giacomo; Biffi, Alice; Richeldi, Luca

    2016-04-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing lung disorder of unknown aetiology whose diagnosis involves the careful exclusion of secondary causes for pulmonary fibrosis and the presence of a pattern of usual interstitial pneumonia (UIP) at either high-resolution computed tomography (HRCT) scan or surgical lung biopsy. Despite great efforts made in establishing precise, universally acknowledged diagnostic criteria for IPF, its ascertainment remains a challenge, especially in those individuals presenting with atypical HRCT patterns. With new drugs emerging, establishing a precise diagnosis is becoming a clinically relevant issue. Although regarded as a rare disease, IPF epidemiology is controversial due to studies relying on old data and adopting mixed, incomparable methodologies for cases definition. Overall, the prevalence and incidence appear to be increasing over the last decades, suggesting that in earlier studies they might have been underestimated because of diagnostic uncertainty. IPF is invariably progressive, although its clinical course might greatly vary on an individual basis, with episodes of severe acute respiratory deterioration (acute exacerbations) being unpredictable. A deeper understanding of the mechanisms responsible for an accelerated course of the disease and the identification of biomarkers of progression would lead to a better stratification of the disease, essential for delivering individualized therapeutic strategies. PMID:26595062

  14. 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.

  15. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review.

    Science.gov (United States)

    Raghu, Ganesh; Amatto, Valeria C; Behr, Jürgen; Stowasser, Susanne

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies published in English from January 1990 to January 2015 identified via MEDLINE and EMBASE were included; bibliographies of articles were also searched.Among the 126 studies included, prevalence of pulmonary hypertension (PH) was 3-86%, 6-91% for obstructive sleep apnoea, 3-48% for lung cancer and 6-67% for chronic obstructive pulmonary disease (COPD). Nonrespiratory comorbidities included ischaemic heart disease (IHD) (3-68%) and gastro-oesophageal reflux (GER) (0-94%). Mortality was highest among patients with IPF and lung cancer. Most studies assessed relatively small samples of patients with IPF.PH, COPD, lung cancer, GER and IHD are significant comorbidities; differences in IPF severity, case definitions and patient characteristics limited the comparability of findings. The identification and prompt treatment of comorbidities may have a clinically significant impact on overall outcome that is meaningful for patients with IPF. PMID:26424523

  16. 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

  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. Long term functional outcome of idiopathic inflammatory myopathies

    Directory of Open Access Journals (Sweden)

    G. Pasero

    2011-09-01

    Full Text Available The idiopathic inflammatory myopathies (IIM comprise a group of diseases characterized by chronic inflammation of the skeletal muscles. The definition of the long-term outcome of IIM, has been limited by the difficulty in objectively evaluating the rate of muscle function impairment. Aim of our study, was to define the long term outcome in a group of 37 IIM patients, followed at our centres between 1979 and 1999. A protocol, evaluating CK levels, muscle function, and disability in daily life activity was prospectively designed. The disease outcome was defined on the basis the patient’s functional evaluation. Disease activity was defined as the presence of an increase in serum CK levels associated with an increase of therapy. At the end of follow up, 27% of the patients had an active disease and 35% had a poor functional outcome. On the basis of our results we could distinguish three different outcomes of IIM: (i good functional outcome (65%; (ii poor functional outcome with inactive disease (13.5%; (iii poor functional outcome and active disease (21,5%. In conclusion, although IIM seem to have a good evolution in terms of disease activity, in about 46% of patients they are associated with an elevated incidence of functional impairement, probably attributable both to the disease’s damage and to the side effects of therapy.

  19. Idiopathic Hypertrophic Cranial Pachymeningitis Misdiagnosed as Acute Subtentorial Hematoma

    Science.gov (United States)

    Park, Ik-Seong; Kim, Hoon; Chung, Eun Yong

    2010-01-01

    A case of idiopathic hypertrophic cranial pachymeningitis (IHCP) misdiagnosed as an acute subdural hematoma is reported. A 37-year-old male patient presented with headache following head trauma 2 weeks earlier. Computerized tomography showed a diffuse high-density lesion along the left tentorium and falx cerebri. Initial chest X-rays revealed a small mass in the right upper lobe with right lower pleural thickening, which suggested lung cancer, such as an adenoma or mediastinal metastasis. During conservative treatment under the diagnosis of a subdural hematoma, left cranial nerve palsies were developed (3rd and 6th), followed by scleritis and uveitis involving both eyes. Magnetic resonance imaging (MRI) revealed an unusual tentorium-falx enhancement on gadolinium-enhanced T1-weighted images. Non-specific chronic inflammation of the pachymeninges was noticed on histopathologic examination following an open biopsy. Systemic steroid treatment was initiated, resulting in dramatic improvement of symptoms. A follow-up brain MRI showed total resolution of the lesion 2 months after steroid treatment. IHCP should be included in the differential diagnosis of subtentorial-enhancing lesions. PMID:20856672

  20. 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)

  1. 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...

  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. 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

  4. Idiopathic environmental intolerances (IEI): from molecular epidemiology to molecular medicine.

    Science.gov (United States)

    De Luca, C; Scordo, G; Cesareo, E; Raskovic, D; Genovesi, G; Korkina, L

    2010-07-01

    Inherited or acquired impairment of xenobiotics metabolism is a postulated mechanism underlying environment-associated pathologies such as multiple chemical sensitivity, fibromyalgia, chronic fatigue syndrome, dental amalgam disease, and others, also collectively named idiopathic environmental intolerances (IEI). In view of the poor current knowledge of their etiology and pathogenesis, and the absence of recognised genetic and metabolic markers of the diseases. They are often considered "medically unexplained syndromes",. These disabling conditions share the features of polysymptomatic multi-organ syndromes, considered by part of the medical community to be aberrant responses triggered by exposure to low-dose organic and inorganic chemicals and metals, in concentrations far below average reference levels admitted for environmental toxicants. A genetic predisposition to altered biotransformation of environmental chemicals, drugs, and metals, and of endogenous low-molecular weight metabolites, caused by polymorphisms of genes coding for xenobiotic metabolizing enzymes, their receptors and transcription factors appears to be involved in the susceptibility to these environment-associated pathologies, along with epigenetic factors. Free radical/antioxidant homeostasis may also be heavily implicated, indirectly by affecting the regulation of xenobiotic metabolizing enzymes, and directly by causing increased levels of oxidative products, implicated in the chronic damage of cells and tissues, which is in part correlated with clinical symptoms. More systematic studies of molecular epidemiology, toxico- and pharmaco-genomics, elucidating the mechanisms of regulation, expression, induction, and activity of antioxidant/detoxifying enzymes, and the possible role of inflammatory mediators, promise a better understanding of this pathologically increased sensitivity to low-level chemical stimuli, and a solid basis for effective individualized antioxidant- and/or chelator

  5. 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

  6. IDIOPATHIC PYODERMA GANGRENOSUM: REPORT IN A THREE YEAR OLD CHILD

    Directory of Open Access Journals (Sweden)

    Vidyadhar Kinhal

    2015-01-01

    Full Text Available Pyoderma gangrenosum is a rare, non - infectious and destructive skin disease. Its exact etiopathogenesis is not clearly understood and is believed to be due to immune dysfunction. Several theories have been postulated; however none are consistent in all patients. Pyoderma gangrenosum mostly affects adults and rarely in children. An underlying systemic diseas e is often seen in most of the cases. A three - year - old male child presented with a non - healing ulcer over left thigh. Clinical features, histopathology, other investigations and dramatic response to corticosteroids suggested a diagnosis of idiopathic pyo derma gangrenosum. Associated idiopathic thrombocytosis was also seen. Idiopathic pyoderma gangrenosum is very rare in children. Idiopathic pyoderma gangrenosum associated with idiopathic thrombocytosis and keloidal scarring has not been reported earlier

  7. Radiographic Evaluation of Idiopathic Osteosclerosis in Patients Referring to Mashhad Dental School from November 2002 to May 2004

    Directory of Open Access Journals (Sweden)

    M Eatemadi-Sadjadi

    2006-07-01

    Full Text Available Introduction: Idiopathic osteosclerosis is a common radiopaque lesion in the jaw bones, which is asymptomatic and usually detected accidentally in various radiographs. The purpose of this study was to determine the radiographic features of idiopathic osteosclerosis in the jawbones of patients referring to the maxillofacial radiology center of Mashhad Dental Faculty. Methods: In this descriptive study, panoramic radiographs of 300 patients ( 125 males, 175 females who had referred to the maxillofacial radiology department were evaluated for the presence of idiopathic osteosclerosis. The radiographic information included location, number, shape, size, relationship with teeth, pattern of density ( trabecular-cortical and demographic data ( age, sex and evidence of disease in the gastrointestinal tract and kidneys were also recorded . Then data was analyzed using chi-square and t-student test. Results : A total of 27 patients (9%, exhibited 40 foci of idiopathic osteosclerosis with different shapes and sizes (4 cm2 to 1/09 cm 2 , most of which had well defined borders (65% and trabecular density (62/5% . The most common region for these lesions was in the mandible, especially in the second premolar (47/5% and first molar (42/5% regions and the majority had no connection to the teeth (57/5%. The lesions were most prevalent in the second decade of life (29/6% followed by third and fourth decades. (25/9% Conclusion: The results of this study were the same as that of previous similar studies. In addition, chronic renal failure was seen in 1/3 of patients with idiopathic osteosclerosis .

  8. 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.

  9. 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

  10. 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

  11. Idiopathic Hypertrophic Pyloric Stenosis in Identical Twins.

    Directory of Open Access Journals (Sweden)

    Ju-Bei Yen

    2003-12-01

    Full Text Available Idiopathic hypertrophic pyloric stenosis (IHPS was thought to be a congenital diseasetraditionally, even though several published reports assumed IHPS was an acquired disease.The pathogenesis and inheritance patterns of IHPS are not fully understood. Except for thefamilial recurrence of IHPS, concordance of IHPS in monozygotic or dizygotic twins wasalso noted, but occurrence in female twins is rare. From July 1992 through June 2000, 130patients were diagnosed with IHPS in our hospital including one pair of female twins. Wepresent the finding in the twins and review the associated articles about the pathogenesis andinheritance patterns of IHPS.

  12. 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.

  13. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    Science.gov (United States)

    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. PMID:26508184

  14. 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

  15. 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.)

  16. 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

  17. Genetics of idiopathic generalized epilepsy: An overview

    Directory of Open Access Journals (Sweden)

    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.

  18. Idiopathic Horner's syndrome in Collie dogs

    International Nuclear Information System (INIS)

    Seven cases of idiopathic Horner's Syndrome in the Collie are described. Five males and two females presented with unilateral miosis, ptosis of the upper eyelid, enophthalmos and protrusion of the third eyelid. Thorough examination, pharmacological testing with phenylephrine, complete blood counts and radiography of the tympanic bullae and thorax were performed. The etiology was not identified in any of the cases. Clinical signs improved with pharmacologic testing within 20-40 min. In five dogs, total resolution of clinical signs was observed at 4 and 16 weeks after their initial appearance. Pharmacological testing suggested that the deficit could be at the preganglionic neuron

  19. 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

  20. Prenatal sonographic monitoring of idiopathic megacystis.

    Science.gov (United States)

    Cavoretto, Paolo; Lesma, Arianna; Spagnolo, Daniele; Valsecchi, Luca; Negretto, Alice; Candiani, Massimo

    2013-02-01

    This case report is of a female fetus diagnosed with severe idiopathic megacystis at 21 weeks of pregnancy. Sonographic monitoring demonstrated normal amniotic fluid volume and renal structures, absence of hydronephrosis, ureteral dilation, and associated abnormalities. Conservative management was chosen with postnatal confirmation of diagnosis. The neonate presented seizures, and subsequent magnetic resonance imaging demonstrated transverse sinus thrombosis with adjacent ischemic damage. At present, the infant is 2 years old, and she is thriving normally and maintains urinary continence with spontaneous voidings. PMID:21953421