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Sample records for chronic idiopathic thrombocytopenic

  1. Changes in splenic microcirculatory pathways in chronic idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Schmidt, E E; MacDonald, I C; Groom, A C

    1991-09-15

    The spleen plays a central role in the pathogenesis of chronic idiopathic thrombocytopenic purpura (ITP); it produces massive quantities of antiplatelet antibodies, leading to accelerated phagocytosis of platelets. Lymphoid hyperplasia typically occurs in the spleen, characterized by large numbers of lymphatic nodules with active germinal centers. Whether changes in splenic microcirculatory pathways also occur is not known. We have studied this question by scanning electron microscopy of corrosion casts, comparing spleens removed from patients with ITP with normal spleens obtained from organ transplant donors. The casts demonstrate two major changes in microcirculatory pathways in ITP. Firstly, a striking proliferation of arterioles and capillaries is found in the white pulp and marginal zone (MZ), seen as extensive vascularization in 92.3% of lymphatic nodules (n = 191) versus 0.6% (n = 224) in normal spleens. Secondly, the marginal sinus, a series of flattened, anastomosing vascular spaces between the white pulp and MZ, is absent in 89.4% of lymphatic nodules versus 4.9% in normal spleens. The cause of these microcirculatory changes, which may not be exclusive to ITP, is presently unknown. Absence of the marginal sinus may affect distribution of blood flow through the MZ such that platelets spend increased amounts of time in the proximity of macrophages. In the presence of antiplatelet antibodies found in ITP spleens, this delayed transit would lead to greatly increased platelet destruction.

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

    Institute of Scientific and Technical Information of China (English)

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

    2007-01-01

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

  3. Multiple cycles of recombinant human thrombopoietin therapy in a patient with chronic refractory idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Hua, Baolai; Zou, Nong; Wang, Shujie; Zhu, Tienan; Zhao, Yongqiang

    2005-06-01

    We describe a 41-year-old woman with chronic idiopathic thrombocytopenic purpura who received recombinant human thrombopoietin (rhTPO) therapy. rhTPO was administrated subcutaneously at a dosage of 1.0 mug/kg daily for a maximum of 14 days until the platelet count was more than 50 x 10/l. The patient received three cycles (six, 13, and eight doses each) of rhTPO, each initiated when the platelet counts was less than 10 x 10/l. The platelet count increased to above 50 x 10/l on days 5, 11 and 8, and peaked at 456 x 10/l, 130 x 10/l and 82 x 10/l on days 9, 15 and 13 in the three respective cycles, each followed by a gradual decline. The durations of platelet counts at more than 50 x 10/l in the three cycles were 13, 7 and 10 days, respectively. rhTPO was well tolerated with no adverse event observed. Antibodies to rhTPO by enzyme-linked immunosorbent assay were not detected. Our observations suggested that rhTPO could transiently increase the peripheral platelet count in patients with chronic refractory idiopathic thrombocytopenic purpura. The reasons why the peak platelet counts decreased and the duration of response shortened after successive cycles of treatment were unclear.

  4. Idiopathic thrombocytopenic purpura

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    L Kayal

    2014-01-01

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

  5. Dentoalveolar trauma in a patient with chronic idiopathic thrombocytopenic purpura: a case report.

    Science.gov (United States)

    Finucane, David; Fleming, Padraig; Smith, Owen

    2004-01-01

    A case is presented of a 13-year-old boy with chronic idiopathic thrombocytopenic purpura (ITP) who sustained traumatic labial luxation of both lower central incisors, with partial alveolar fracture resulting in displacement of the labial alveolar plate. Intravenous immunoglobulin (Fleibogamma, 1 g/kg body weight x 2 days) was administered, resulting in the patient's platelet count rising from 15,000/mm3 to 70,000/mm3. Under general anesthesia, the displaced lower labial alveolus and luxated teeth were repositioned and splinted 2 days following trauma. Healing was uneventful. Subsequently, both lower central incisors became nonvital and were endodontically treated. The dental treatment of this patient with ITP is discussed in terms of emergency management, and subsequent care.

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

    Science.gov (United States)

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

    2009-06-08

    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.

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

    Directory of Open Access Journals (Sweden)

    Maryam Maghbool

    2009-07-01

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

  8. Rapidly calcified all of multiple intracranial hemorrhages occurred in a patient with Chronic idiopathic thrombocytopenic purpura

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    Seong Rok Han, M.D., Ph.D.

    2016-03-01

    Full Text Available We report a 38-year-old female patient with chronic idiopathic thrombocytopenic purpura (ITP who occurred in rapidly calcified all of multiple intracranial hemorrhage (ICH lesions. The patient was admitted with poor oral intake and confused mentality. Neurologic examination revealed drowsy consciousness but no motor weakness. She had been diagnosed ITP 5 years ago, however, she was not taking any medication at the time of presentation. Brain CT demonstrated that multiple ICHs, which were located in left frontal lobe, left temporal lobe and right cerebellar hemisphere. Platelet count was 10,000 cells/mm3. The patient was treated with conservative management, which included corticosteroids and platelet transfusion. Follow-up CT performed 12 days after the admission revealed that multiple ICHs were grossly resolving state. Interestingly, high attenuated lesions were seen all of multiple ICH sites, which were considered calcification. After 30 months after hemorrhage, follow-up brain CT showed prominent calcification of all of previous multiple ICH lesions. The patient was well- being state.

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

    Institute of Scientific and Technical Information of China (English)

    卢燕

    2001-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2005-01-01

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

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

    Science.gov (United States)

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

    2012-06-01

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

  12. A Remarkable Improvement of Patients with Idiopathic Thrombocytopenic Purpura after appendectomy including Carcinoid tumor

    OpenAIRE

    Hamidreza Alizadeh-Otaghvar; Saeedeh Firoozbakht; Sahar Montazeri; Samaneh Khazraie; Marjan Bani Ahmad; Maryam Hajiloo

    2011-01-01

    The assistance of chronic idiopathic thrombocytopenic purpura and appendiceal carcinoid tumor is so rare and has not been reported before .we present here in a 11 year old boy that is the known case of chronic idiopathic thrombocytopenic purpura (with the platelet count of 20000 when admitted). He reffered due to anorexia, pain and tenderness of right lower quadrant of abdomen and vomiting. He also had leukocytosis and abdominal free fluid in sonographic report. These findings suggested the d...

  13. Idiopathic thrombocytopenic purpura: laboratory diagnosis and management

    Directory of Open Access Journals (Sweden)

    Alvina Alvina

    2016-02-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP or immune thrombocytopenic purpura is a disease characterized by low platelet count (<150,000/ìL caused by autoantibody-mediated platelet destruction and the absence of other causes of thrombocytopenia. Acute primary ITP is more common in children 2-6 years of age, with similar incidence between males and females, while the chronic form is usually encountered in adults with median age of 40-45 years. The clinical signs of ITP are purpura, ecchymosis, petechiae and gastrointestinal tract bleeding, gingival bleeding, epistaxis, and urinary tract bleeding. Spontaneous mucosal, intracranial, and gastrointestinal hemorrhage may occur at platelet counts of <10000/ìL. To date, the diagnosis of ITP is still arrived at by exclusion, i.e. by elimination of other causes of thrombocytopenia. The diagnosis of ITP also requires a medical history (anamnesis, physical examination, platelet count, and examination of a peripheral blood smear. The latter examination in ITP shows low numbers of normal-sized platelets, occasionally also giant platelets, while erythrocytes and leukocytes have a normal morphology. The bone marrow is usually normal or shows increased megakaryocytes. Assessment of antithrombocyte antibody may assist in establishing the diagnosis of ITP. Management of ITP is based on platelet count and severity of bleeding. Treatment is aimed at interfering with antibodies that damage the platelets, by inhibiting the functions of macrophage Fcã receptors and decreasing the production of antiplatelet antibodies. Thrombopoietin (TPO receptor agonists including eltrombopag and romiplostim have offered an important new option in treating ITP.

  14. 儿童特发性血小板减少性紫癜急性转慢性危险因素分析%Risk factors of acute idiopathic thrombocytopenic purpura developing into chronic idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王缉干; 罗建明

    2012-01-01

    目的 探讨影响小儿急性特发性血小板减少性紫癜(AITP)发展成慢性特发性血小板减少性紫癜(CITP)的危险因素.方法 选择2006年5月至2010年4月于广西医科大学一附院诊断AITP住院患儿138例,对患儿临床表现、实验室检查、治疗方案等16个相关因素分别进行单因素成组对照研究.对有意义的单因素,再运用非条件Logistic多因素回归模型分析,以期找到有意义的因素.结果 病程(患儿起病至治疗时间)、ORh(D)+型血、用含有大剂量丙种球蛋白的治疗方案、血小板上升正常时间都是影响AITP患儿转为慢性的主要因素,而与发病年龄、治疗初用血小板、血小板开始回升时间、性别、有无前驱显性感染、治疗前血小板数、血小板平均体积、血小板平均分布系数、骨髓巨核细胞数、幼稚巨核细胞数、颗粒型巨核细胞数、有无幼稚淋巴细胞数无关.结论 AITP患儿早期治疗时用有大剂量丙种球蛋白的治疗方案是改善预后的关键,治疗时血小板回升正常时间晚、ORh(D)+血型(相对于A和B血型)是转为慢性的高危因素.%Objectives To investigate the risk factors for children with acute idiopathic thrombocytopenic purpura (AITP) developing into chronic idiopathic thrombocytopenic purpura (CITP). Methods A total of 138 patients with AITP from May 2006 to April 2010 in the First Affiliated Hospital of Guangxi Medical University were Selected and telephoned for retrospective analysis. The 16 related factors, including clinical presentation, laboratory tests and treatment programs, were performed single case-control study. For those significant single factors, multi-factor non-conditional Logistic regression analysis was used to find meaningful factors. Results The main influencing factors were the onset-to-treatment time,0 Rh (D) + type blood, treatment with gamma globulin and platelets increasing to normal time. The age of onset, early treatment

  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. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

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    Michelle Fog Andersen

    2015-01-01

    Full Text Available 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.

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

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    Sherif Ali Eltawansy

    2014-01-01

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

  18. A Remarkable Improvement of Patients with Idiopathic Thrombocytopenic Purpura after appendectomy including Carcinoid tumor

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    Hamidreza Alizadeh-Otaghvar

    2011-09-01

    Full Text Available The assistance of chronic idiopathic thrombocytopenic purpura and appendiceal carcinoid tumor is so rare and has not been reported before .we present here in a 11 year old boy that is the known case of chronic idiopathic thrombocytopenic purpura (with the platelet count of 20000 when admitted. He reffered due to anorexia, pain and tenderness of right lower quadrant of abdomen and vomiting. He also had leukocytosis and abdominal free fluid in sonographic report. These findings suggested the diagnosis of acute appendicitis and the patient underwent appendectomy, after the operation, the platelet count became normal. The pathologic report of the specimen of the appendix was carcinoid tumor. Now the question is whether the appendiceal carcinoid tumor can be the reason of symptoms of chronic idiopathic.

  19. The potential role of thrombopoietin in idiopathic thrombocytopenic purpura

    NARCIS (Netherlands)

    von dem Borne, A; Folman, C; van den Oudenrijn, S; de Jong, S; de Haas, M

    2002-01-01

    Thrombopoietin (TPO) plays a central role in the pathogenesis of idiopathic thrombocytopenic purpura (ITP), as it does in other immune-mediated thrombocytopenias. Because TPO is bound and internalized by platelets, it is destroyed together with platelets at an accelerated rate in the macrophage syst

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

    Science.gov (United States)

    2016-01-01

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

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

    NARCIS (Netherlands)

    Houwerzijl, Ewout Johan

    2008-01-01

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

  2. The analysis of factors of acute Idiopathic thrombocytopenic purpura conversing to chronic Idiopathic thrombocytopenic purpura in children%儿童特发性血小板减少性紫癜急性转慢性危险因素分析

    Institute of Scientific and Technical Information of China (English)

    周丹; 杨萍; 田立新; 冯玉兰; 宋丽琼

    2013-01-01

    Objective:To analyze the factors of cute Idiopathic thrombocytopenic purpura (AITP) conversing to chronic Idiopathic thrombocytopenic purpura (CITP). Methods:Choosed 156 children of AITP to analyze by lab, diagnosis and treatment, effect and prognosis. Methods:There were 12 index having difference, among these 2 index were continuous variable (weight, peak platelet). Implement multi-factor analysis of these 12 factors, and screened medical history, age, low count of WBC, weight,high count of marrow megakaryocyte and peak of platelet. Conclusion:Dignosising AITP patients with count of marrow megakaryocyte, peak of platelet to determine prognosis is effective.%目的:分析致使 AITP(即为:儿童特发性急性的血小板减少性紫癜)转变为 CITP(即为:儿童特发性慢性的血小板减少性紫癜)的相关因素。方法:随机对156例患儿分组,行调查分析、实验室分析、诊治方法分析、诊治效果分析、机体预后情况分析。结果:在所有因素指标之中,12项指标存在显著差异,P<0.05,其中,有2项是连续型变量,包括体重(生病之时)、血小板峰值(诊治以后),对存在显著意义且经单因素分析的12项因素实施多因素分析(Logistic非条件回归模型),各危险因素分别为诊治前较长的病史、年龄大(发病之时)、白细胞计数在病初阶段降低或者正常、体重于发病时较大、骨髓巨核细胞于病初阶段数目高、血小板峰值于诊治后降低。结论:在初诊AITP病儿之时,检查骨髓巨核细胞,并于诊治中对血小板最大值予以观察,对判断预后很有价值,对AITP预后的改善有利的因素即为早确诊、早诊治。

  3. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

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    Joji Nagasaki

    2016-01-01

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

  4. Association of primary biliary cirrhosis with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    Nobuyuld Toshikuni; Ryumei Yamato; Haruhiko Kobashi; Ken Nishino; Nobu Inada; Ritsuko Sakanoue; Mitsuhiko Suehiro; Yoshinori Fujimura; Gotaro Yamada

    2008-01-01

    Although both primary biliary cirrhosis (PBC) and idiopathic thrombocytopenic purpura (ITP) are autoimmune diseases, the association of the 2 diseases is rare. Here, we report a case of ITP that developed during the follow-up of PBC in a 74-yearold man. The patient had been diagnosed with PBC 12 years previously, and had received treatment with ursodeoxycholic acid. The platelet count decreased from approximately 60 × 109/L to 8 × 109/L, and the association of decompensated liver cirrhosis (PBC) with ITP was diagnosed. Steroid and immune gamma globulin therapy were successful in increasing the platelet count.Interestingly, human leukocyte antigen genotyping detected the alleles DQBI*0601 and DRBI*0803, which are related to both PBC and ITP in Japanese patients.This case suggests common immunogenetic factors might be involved in the development of PBC and ITP.

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

    Science.gov (United States)

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

    2003-10-01

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

  6. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

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    Christopher Coleman

    2014-01-01

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

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

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    Hanjun Kim

    2014-06-01

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

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

    NARCIS (Netherlands)

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

    2007-01-01

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

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

    NARCIS (Netherlands)

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

    2004-01-01

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

  10. Mycophenolate mofetil (MMF) for the treatment of steroid-resistant idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Hou, Ming; Peng, Jun; Shi, Yan; Zhang, Chunqing; Qin, Ping; Zhao, Chuanli; Ji, Xuebin; Wang, Xueyong; Zhang, Maohong

    2003-06-01

    The treatment of chronic idiopathic thrombocytopenic purpura (ITP) is difficult in those unresponsive to corticosteroids and/or splenectomy. We attempted to induce durable response in 21 patients with refractory ITP by applying mycophenolate mofetil (MMF) (1.5-2.0 g/d), a novel immunosuppressive agent. Overall response rate was 62% (13 of 21), including 24% (five of 21) in complete response (CR), 29% (six of 21) in partial response (PR), and 10% (two of 21) in minor response (MR). The response rates for non-splenectomized and splenectomized ITP patients were 64% (nine of 14) and 57% (four of seven), respectively (P > 0.05). 39% (five of 13) responders relapsed as a result of dose reduction or withdraw of MMF, and 61% (eight of 13) responders maintained their effectiveness for a median of 24 wk. Sustained response was observed in three patients in whom MMF was withdrawn. MMF was well tolerated with only slight nausea and diarrhea recorded in 3 of 21 cases. No premature withdrawal was found in this study. CD3+ peripheral blood mononuclear cells (PBMC) and CD19+ PBMC were significantly reduced 12 wk after MMF administration in the responders. Platelet-associated antibodies against glycoproteins GPIIb/IIIa were detected in 13 of 21 (62%) patients before MMF treatment, and antibody levels were significantly decreased in responders 12 wk after MMF administration. This suggested that MMF might correct the immunologic abnormalities underlying the destruction of circulating platelets in ITP. We conclude that MMF could be used as a second-line agent for the treatment of steroid-resistant ITP before or after splenectomy and thereby is worth of further evaluation in randomized studies.

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

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    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.

  12. Squamous cell carcinoma larynx presenting as idiopathic thrombocytopenic purpura

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    Bekur R

    2015-01-01

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

  13. Chronic immune thrombocytopenic purpura. New agents.

    Science.gov (United States)

    Rodeghiero, F; Ruggeri, M

    2009-01-01

    First generation thrombopoietic growth factors (rhTPO and PEG-rHuMGDF), investigated in the early 2000s, proved effective in increasing platelet count in normal volunteers, in thrombocytopenia due to chemotherapy and also in a few cases of immune thrombocytopenic purpura (ITP). These agents did not complete their clinical development since one of them induced antibodies in the recipients that cross reacted with endogenous thrombopoietin (TPO), thus causing thrombocytopenia. This promoted the ingenious design of a new generation of thrombopoietic growth factors having no sequence homology with natural TPO. The two main agents are romiplostim, a peptibody already approved for clinical use in USA and eltrombopag, a non-peptide, orally active small molecule. In open label and placebo-controlled trials both agents proved to predictably increase platelet count in normal volunteers and in patients with ITP. With appropriate dosages (1-10 microg/kg weekly sub cutaneously for romiplostim; 50-75 mg/die per os for eltrombopag ) a platelet increase becomes significant after 7-10 days and peaks between 10-14 days. By discontinuing treatment, platelet count returns to baseline level in 10-15 days. The response rate with both agents is above 70-80%, also in patients that had undergone several lines of treatment, or that have failed splenectomy. The response is maintained during the treatment, but is almost invariably lost even after several months of successful administration. Due to the lack of a curative potential and to the incomplete knowledge of long-term side effects, the place of these new drugs in the management of ITP is still unsettled and their use is best restricted to refractory patients or in preparation of splenectomy. It seems however that a new paradigm in the treatment of ITP has been established where the focus is not on reducing platelet consumption but on increasing platelet production.

  14. Effects of prednisone and splenectomy in patients with idiopathic thrombocytopenic purpura : only splenectomy induces a complete remission

    NARCIS (Netherlands)

    Louwes, H; Vellenga, E; Houwerzijl, EJ; de Wolf, JTM

    2001-01-01

    Idiopathic thrombocytopenic purpura (ITP) is a heterogeneous disease, whereby it is unclear if and in which way prednisone and splenectomy affect the platelet kinetics leading to a complete remission. To determine the effects of prednisone and splenectomy on the mean platelet life (MPL) and platelet

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

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    Enver Ay

    2012-03-01

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

  16. Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.

    Science.gov (United States)

    Caramazza, Domenica; Quintini, Gerlando; Abbene, Ignazio; Malato, Alessandra; Saccullo, Giorgia; Lo Coco, Lucio; Di Trapani, Rosa; Palazzolo, Roberto; Barone, Rita; Mazzola, Giuseppina; Rizzo, Sergio; Ragonese, Paolo; Aridon, Paolo; Abbadessa, Vincenzo; Siragusa, Sergio

    2010-12-01

    Idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare disease responsive to treatment with plasma exchange (PE) but with a high percentage of relapse or refractory patients. A severe deficiency of ADAMTS-13 (<5% of normal activity), congenital or caused by an autoantibody, may be specific for TTP and it has been proposed that severe ADAMTS-13 deficiency now defines TTP. B cells play a key role in both the development and the perpetuation of autoimmunity, suggesting that B-cell depletion could be a valuable treatment approach for patients with idiopathic TTP-HUS. This review of the literature focuses on the role of rituximab, a chimeric monoclonal antibody directed against CD20 antigen expressed by B lymphocytes, in patients with relapsing or refractory TTP-HUS with or without ADAMTS-13 deficiency, suggesting that rituximab may produce clinical remission in a significant proportion of patients. Rituximab therapy reduces plasma requirement and avoids complications related to salvage-immunosuppressive therapy. In conclusion, rituximab provides an effective, well-tolerated, and safe treatment option for patients with idiopathic TTP-HUS, thus giving an alternative approach to the current treatment based on PE.

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

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    Jorge García-Méndez

    2016-01-01

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

  18. Role of Helicobacter pylori Eradication Therapy on Platelet Recovery in Chronic Immune Thrombocytopenic Purpura

    Science.gov (United States)

    Sheema, Khan; Arshi, Naz; Farah, Naz; Imran, Sheikh

    2017-01-01

    Background. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. Objective. To assess the effect of Helicobacter pylori eradication therapy (HPET) on platelet count in Helicobacter pylori associated chronic immune thrombocytopenic purpura (chronic ITP) in adult. Materials and Methods. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015. A set of 85 patients diagnosed with chronic ITP were included in the study via convenient sampling. Patients with platelets count 3 months were selected. They were posed to first-line investigations which comprised complete blood count (CBC) and peripheral blood smear examination followed by second-line tests including bone marrow examination and Helicobacter pylori stool specific antigen (HpSA-EIA). Standard H. pylori eradication therapy was offered and the patients were assessed at regular intervals for 6 months. Results. Of the 85 study patients, 32 (37.6%) were male and 53 (62.3%) were female. Mean ages of H. pylori positive and negative subjects were 43.89 ± 7.06 and 44.75 ± 7.91 years, respectively. Bone marrow examination confirmed the diagnosis and excluded other related BM disorders. H. pylori stool antigen (HpSA) was detected in 34 (40%) patients and hence regarded as H. pylori positive; the rest were negative. Treatment with eradication therapy significantly improved the mean platelet counts from 48.56 ± 21.7 × 109/l to 94.2 ± 26.8 × 109/l. Conclusion. We concluded that the anti-H. pylori eradication therapy improves blood platelet counts in chronic immune thrombocytopenia.

  19. Role of Helicobacter pylori Eradication Therapy on Platelet Recovery in Chronic Immune Thrombocytopenic Purpura

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    Khan Sheema

    2017-01-01

    Full Text Available Background. Idiopathic thrombocytopenic purpura (ITP is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. Objective. To assess the effect of Helicobacter pylori eradication therapy (HPET on platelet count in Helicobacter pylori associated chronic immune thrombocytopenic purpura (chronic ITP in adult. Materials and Methods. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015. A set of 85 patients diagnosed with chronic ITP were included in the study via convenient sampling. Patients with platelets count 3 months were selected. They were posed to first-line investigations which comprised complete blood count (CBC and peripheral blood smear examination followed by second-line tests including bone marrow examination and Helicobacter pylori stool specific antigen (HpSA-EIA. Standard H. pylori eradication therapy was offered and the patients were assessed at regular intervals for 6 months. Results. Of the 85 study patients, 32 (37.6% were male and 53 (62.3% were female. Mean ages of H. pylori positive and negative subjects were 43.89 ± 7.06 and 44.75 ± 7.91 years, respectively. Bone marrow examination confirmed the diagnosis and excluded other related BM disorders. H. pylori stool antigen (HpSA was detected in 34 (40% patients and hence regarded as H. pylori positive; the rest were negative. Treatment with eradication therapy significantly improved the mean platelet counts from 48.56±21.7 × 109/l to 94.2±26.8 × 109/l. Conclusion. We concluded that the anti-H. pylori eradication therapy improves blood platelet counts in chronic immune thrombocytopenia.

  20. DNMT3B 579G>T promoter polymorphism and the risk for idiopathic thrombocytopenic purpura in a Chinese population.

    Science.gov (United States)

    Zhao, Haifeng; Du, Weiting; Gu, Dongsheng; Wang, Donghai; Xue, Feng; Ge, Jing; Sui, Tao; Yang, Renchi

    2009-01-01

    Epigenetics may influence the expression of numerous genes, which might contribute to autoimmune diseases. DNA methylation is mediated by DNA methyltransferases, especially DNA methyltransferase 3B (DNMT3B). Polymorphisms of the DNMT3B gene may influence DNMT3B activity on DNA methylation and increase the susceptibility to several diseases. The current study investigated the association between DNMT3B 579G>T and the risk for idiopathic thrombocytopenic purpura (ITP). The DNMT3B 579G>T polymorphisms were analyzed by PCR-RFLP. There was no significant difference in genotype and allele distribution between the ITP patient and the controls (p = 0.722 and 0.667, respectively). Similar results were observed between the 2 groups when stratified by age and disease course, including acute in childhood, chronic in childhood, acute in adult and chronic in adult. Importantly, this study showed a statistical difference in the distribution of SNP of DNMT3B between Chinese and Koreans or Americans. It is shown that the SNP of DNMT3B 579G>T may not be used on its own as a marker to predict the susceptibility to ITP in a Chinese population and that DNMT3B 579G>T promoter SNP varies from one ethnic population to another.

  1. Case of twin pregnancy complicated by idiopathic thrombocytopenic purpura treated with intravenous immunoglobulin: Review of the literature.

    Science.gov (United States)

    Zhao, W X; Yang, X F; Lin, J H

    2017-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an acquired thrombocytopenia without other clear cause of thrombocytopenia. It is not common in a singleton pregnancy and less common in twin pregnancy. We report a 33-year-old ITP pluripara whose first pregnancy was uneventful. She carried twin pregnancy, complicated by recurrent very low platelets, and gave birth to preterm twins. This patient received multiple courses of intravenous immunoglobulin (IVIG) and showed a significant platelet count improvement with IVIG therapy.

  2. 白介素-11联合激素治疗慢性特发性血小板减少性紫癜的起效时间及血小板变化情况研究%STUDY OF THE ONSET TIME AND THE CHANGE IN TREATMENT FOR CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA WITH INTERLEUKIN -11 AND HORMONE

    Institute of Scientific and Technical Information of China (English)

    郑冬丽

    2011-01-01

    [Objective]To investigate the onset time and platelet changes in the treatment of chronic idiopathic thrombo-cytopenic purpura with IL -11 combined with dexamethasone.[Methods]90 chronic idiopathic thrombocytopenic puipura patients as research subjects in our hospital from January 2006-January 2010 were randomly divided into three groups, of which A group carried out IL -11 treatment, B group treated with dexamethasone, C group were combined hormone Interleukin -11 treated, observed and compared the onset time and platelet changes in three groups.[Results]After treatment, the total effective rate in C group was significantly higher than that in group A, B, and the time that C group reached the normal level of platelets was significantly shorter with the A group, B group (P all < 0.05).After beingtreated, platelets were significantly higher in three groups than before treatment, C group with more obvious increase, showed statistically significant differences between groups (P all < 0.05).The side effects were insomnia, hypertension, hyperglycemia, secondary infection, moon face, central obesity, and gastrointestinal bleeding.The incidence of side effects in C group was significantly lower than A, B groups, it showed statistically significant difference (P both <0.05), but between A, B groups, it showed no significance (P > 0.05).[Conclusion]Interleukin -11 combined hormone treatment for chronic idiopathic thrombocytopenic purpura is with faster onset time and less side reactions, significantly improves platelet function, which is worthy of clinical application.%[目的]探讨白介素-11联合地塞米松治疗慢性特发性血小板减少性紫癜的起效时间及血小板变化情况.[方法]选取某院2006年1月~2010年1月住院治疗的90例慢性特发性血小板减少性紫癜患者作为研究对象,随机分为3组各30例,其中A组采用白介素-11进行治疗,B组采用地塞米松进行治疗,C组采用白介素-11联合

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

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    matteo molica

    2016-04-01

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

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

    Science.gov (United States)

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

    2013-04-01

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

  5. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura : a double-blind randomised controlled trial

    NARCIS (Netherlands)

    Kuter, David J.; Bussel, James B.; Lyons, Roger M.; Pullarkat, Vinod; Gernsheimer, Terry B.; Senecal, Francis M.; Aledort, Louis M.; George, James N.; Kessler, Craig M.; Sanz, Miguel A.; Liebman, Howard A.; Slovick, Frank T.; de Wolf, J. Th M.; Bourgeois, Emmanuelle; Guthrie, Troy H.; Newland, Adrian; Wasser, Jeffrey S.; Hamburg, Solomon I.; Grande, Carlos; Lefrere, Francois; Lichtin, Alan Eli; Tarantino, Michael D.; Terebelo, Howard R.; Viallard, Jean-Francois; Cuevas, Francis J.; Go, Ronald S.; Henry, David H.; Redner, Robert L.; Rice, Lawrence; Schipperus, Martin R.; Guo, D. Matthew; Nichol, Janet L.

    2008-01-01

    Background Chronic immune thrombocytopenic purpura (ITP) is characterised by accelerated platelet destruction and decreased platelet production. Short-term administration of the thrombopoiesis-stimulating protein, romiplostim, has been shown to increase platelet counts in most patients with chronic

  6. Vitamin D deficiency in chronic idiopathic urticaria.

    Science.gov (United States)

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

    2015-04-01

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

  7. Treatment of 37 Patients with Refractory Idiopathic Thrombocytopenic Purpura by Shengxueling(升血灵)

    Institute of Scientific and Technical Information of China (English)

    SHAO Ke-ding; ZHOU Yu-hong; SHEN Yi-ping; YE Bao-dong; GAO Rui-lan; ZHANG Yu

    2007-01-01

    Objective:To explore the clinical effect and possible mechanism of Shengxueling(升血灵,SXL),a Chinese medical preparation mainly consisting of ginseng saponins,in treating refractory idiopathic thrombocytopenic purpura(ITP).Methods:The selected 69 patients with ITP were randomly assigned to two groups,the 37 patients in the treated group were treated orally by SXL with the dose for adult as 60 mg twice a day for two weeks.Then when no marked rise of platelet count after that,the dose would be doubled and administered for another two weeks.Then the dose could be gradually reduced to the initiative level in patients who responded to the treatment,and if they did not,the treatment was regarded as ineffective and be terminated.The 32 patients in the control group were treated with ampeptide elemente instead of SXL,0.4 g each time three times a day in the first two weeks,and,if that was ineffective,0.2 g would be added each time and 1.8 g would be administered a day for two more weeks.Four weeks' treatment was regarded as one therapeutic course for both groups and the observation lasted for two successive courses in patients showing positive response.Results:In the 37 patients in the treated group,markedly effective was obtained in 7(19.0%),favorably effective in 15(40.5%),improved in 5(13.5%) and ineffective in 10(27.0%),the total effective rate being 59.5%.The corresponding number in the 32 patients in the control group was 4(12.5%),6(18.8%),3(9.4%),19(59.4%)and 31.3% respectively.Comparison showed the difference in therapeutic efficacy between the two groups was significant(P<0.05).Conclusion:SXL is a safe and effective preparation for treatment of ITP,showing an immediate effect which is obviously superior to that of ampeptide elemente with less adverse effect.

  8. Multidrug resistance-1 in T lymphocytes and natural killer cells of adults with idiopathic thrombocytopenic purpura: effect of prednisone treatment.

    Science.gov (United States)

    López-Karpovitch, Xavier; Graue, Gerardo; Crespo-Solís, Erick; Piedras, Josefa

    2008-07-01

    High P-glycoprotein-mediated multidrug resistance-1 (P-gp/MDR1) activity in lymphocytes from idiopathic thrombocytopenic purpura (ITP) patients may affect disease outcome. ITP treatment includes glucocorticoids that are substrates of P-gp; hence, P-gp functional activity and antigenic expression were assessed by flow cytometry in T and natural killer (NK) cells from ITP patients before and after prednisone therapy. Herein, patients' T and NK cells did not show increased MDR1 functional activity, whereas P-gp antigenic expression was significantly enhanced in both therapy-free and prednisone-treated patients. Prednisone treatment did not significantly modify the function and expression of MDR1 in T and NK cells of ITP patients.

  9. 儿童慢性特发性血小板减少性紫癜与幽门螺杆菌感染的临床研究%CLINICAL STUDY ON CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA AND HELICOBACTOR PYLORI IN CHILDREN

    Institute of Scientific and Technical Information of China (English)

    武国霞; 彭建霞; 张建菊; 武建辉; 任蓉

    2012-01-01

    [Objective] To investigate the relationship of chronic idiopathic thrombocytopenic purpura (cITP) and heli-cobactor pylori (HP) in children, and observe the clinical effect on HP eradiating therapy in children with cITP. [Methods] Children with cITP were delected by 13C urea breath teat. 14 children, who had positive results, were randomly divided into study group (n = 9) and control group (n = 5). The children were treated with immunotherapy and HP eradiating therapy in study group, the children were only treated with immunotherapy in control group. Clinical effects and the platelets before treatment and in the 7th day, 1st month, 2nd month and 3rd month after treatment were compared between groups. [Results] 14 of 66 children with cITP were positive for HP infection (21.2%). The clearances of HP were achieved in 7 out of 9 children with FTP in study group (77.8%). The analysis showed the platelets in the study group were higher than in the control group (P < 0.01) in the 7ui day, 1st month, 2nd month, 3rd month after treatment. The analysis of clinical effects of children showed the study group were better than the control group (P < 0.05). [Conclusion] The study had shown the prevalencde of HP infection in cITP children was 21.2%, the bacterium eradication were obtained in 7 of 9 HP positive children (77.8%) in study group. It is helpful to increase the platelets count and get clinical effects in cITP children combined with HP eradication treatment%[目的]探讨儿童慢性特发性血小板减少性紫癜(cITP)与幽门螺杆菌(HP)的关系,观察根除HP对儿童cITP的疗效.[方法]对cITP患儿进行13℃尿素呼吸试验检测,将确诊HP感染的14例患儿随机分成研究组(9例)和对照组(5例).研究组采用免疫治疗加根除HP治疗,而对照组只采用免疫治疗,比较两组治疗前以及治疗后d7、第1,2,3月血小板的数目和临床效果.[结果]66例cITP患儿中有HP感染14例(21.2%),研究组的9例中,其中7

  10. Púrpura trombocitopênica imunológica como manifestação inicial de lúpus eritematoso sistêmico juvenil Idiopathic thrombocytopenic purpura as initial manifestation of juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Josefina Aparecida Pellegrini Braga

    2003-12-01

    salientar a importância da determinação de auto-anticorpos para lúpus eritematoso sistêmico nas crianças com a forma crônica desta patologia.Patients with Idiopathic Thrombocytopenic Purpura (ITP present a high trend to develop Systemic Lupus Erythematosus (SLE, especially those with chronic presentation. Some authors observed that female gender, older patients and familial history of autoimmune disease in patients with Idiopathic Thrombocytopenic Purpura are factors that lead to increased susceptibility for the development of Systemic Lupus Erythematosus. Based on these facts, we decided to study 5 children with chronic Idiopathic Thrombocytopenic Purpura and late Systemic Lupus Erythematosus. In this paper, we describe the clinical and laboratorial features of 5 female children with Idiopathic Thrombocytopenic Purpura that later developed Systemic Lupus Erythematosus. All patients were girls, 3 Caucasian, with Idiopathic Thrombocytopenic Purpura onset age ranged between 6 years and 3 months and 12 years and 1 month (mean - 9 years and 2 months. The age at Systemic Lupus Erythematosus diagnosis ranged between 8 years and 13 years and 8 months (mean - 10 years and 10 months. Though, the gap between Idiopathic Thrombocytopenic Purpura and Systemic Lupus Erythematosus diagnosis ranged between 11 months and 2 years and 9 months (mean - 1 year and 10 months. All patients presented chronic Idiopathic Thrombocytopenic Purpura (thrombocytopenia lasts longer than 6 months. The Systemic Lupus Erythematosus classification criteria were (in decreasing frequency: malar erythema and positivity of ANA in 5 patients; arthritis, hematological (thrombocytopenia and immunological alterations (positivity of anti-DNA in 4 patients; photosensitivity and positivity of anti-cardiolipin in 3 patients. Other manifestations included oral ulcers, renal involvement, leukopenia and auto-immune hemolytic anemia, serositis (pericarditis, neurological involvement and positivity of anti-Sm antibody

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

    Energy Technology Data Exchange (ETDEWEB)

    Savolainen, S.

    1992-01-01

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

  12. Effect of Protein Kinase C on Proliferation and Apoptosis of T Lymphocytes in Idiopathic Thrombocytopenic Purpura Children

    Institute of Scientific and Technical Information of China (English)

    Changlin Wu; Fang Liu; Xuemin Zhou; Zhengwei Cheng; Xiaomeng Yang; Hong Xiao; Qun Chen; Kangrong Cai

    2005-01-01

    It is well-documented that T lymphocyte proliferation and apoptosis are abnormal in idiopathic thrombocytopenic purpura (ITP) children. However, the underlying regulation mechanisms especially in terms of signal transduction remain unknown. In this paper, we reported the changes of protein kinase C (PKC) activity in peripheral blood T lymphocytes and the effect of PKC on T lymphocyte proliferation and apoptosis. We demonstrated that in ITP children, the activator (PMA) and inhibitor (H-7) of PKC affected on T lymphocyte proliferation and apoptosis dramatically, but they altered little in healthy children. PKC activity was significantly enhanced in ITP children together with an increased expression of FasL on CD3+ T, CD4+ T and CD8+T cells, resulting in a positive correlation between PKC activity and the expression of FasL on T cells. While the PKC activity and the platelet count were negatively correlated. Taken together, our findings suggest that the PKC activation may enhance T lymphocytes activity, suppress T cell apoptosis and be involve in thrombocytes damage as a mechanism related to immune pathogenesis of ITP.

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

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  14. 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)

  15. Kinetics and sites of destruction of /sup 111/In-oxine-labeled platelets in idiopathic thrombocytopenic purpura: a quantitative study

    Energy Technology Data Exchange (ETDEWEB)

    Heyns, A.D.; Loetter, M.G.; Badenhorst, P.N.; de Kock, F.; Pieters, H.; Herbst, C.; van Reenen, O.R.; Kotze, H.; Minnaar, P.C.

    1982-04-01

    Kinetics and quantification of the sites of destruction of /sup 111/In-oxine-labeled autologous platelets were investigated in eight patients with idiopathic thrombocytopenic purpura. The mean platelet count was 17 +/- 9 X 10(9)/liter; platelets were separated by differential centrifugation and labeled with 5.6 +/- 2.5 MBq /sup 111/In. Whole body and organ /sup 111/In-platelet distribution was quantitated with a scintillation camera and a computer-assisted imaging system acquisition matrix. Areas of interest were selected with the computer and organ /sup 111/In-radioactivity expressed as a percentage of whole body activity. Mean platelet survival was 49.5 +/- 29.6 hr and the survival curves were exponential. Equilibrium percentage organ /sup 111/In-radioactivity was (normal values in parentheses): spleen 33.7 +/- 8.8 (31.1 +/- 10.2); liver 16.1 +/- 9.5 (13.1 +/- 1.3); thorax 22.8 +/- 3.7 (28.2 +/- 5.6). Percentage organ /sup 111/In-activity at the time when labeled platelets had disappeared from the circulation was: spleen 44.5 +/- 16.4 (40 +/- 16); liver 16.0 +/- 11.5 (32.4 +/- 7.2); thorax 19.7 +/- 6.0 (17.7 +/- 10.3). Thorax activity corresponds to bone marrow radioactivity. Three patterns of platelet sequestration were evident. Three patients had mainly splenic sequestration, two mainly hepatic sequestration, and three diffuse reticuloendothelial system sequestration with a major component of platelets destroyed in the bone marrow. Splenectomy was performed in two patients. The pattern of /sup 111/In-platelet sequestration was not predictive of response of glucocorticoid therapy or indicative of the necessity for splenectomy. Quantitative /sup 111/In-labeled autologous platelet kinetic studies provide a new tool for the investigation of platelet disorders.U

  16. Chronic Idiopathic Intestinal Pseudo-obstruction.

    Science.gov (United States)

    Malagelada

    2000-08-01

    The definition of chronic idiopathic intestinal pseudo-obstruction (CIIP) is somewhat vague because it was based on clinical observations that preceded modern advances in the measurement of gut motility and neuromuscular integrity. Appropriate management of patients with CIIP requires an initial consideration, supported by pertinent diagnostic tests, of the tissue affected (muscle, nerves, both), extent and magnitude of gut propulsive failure, and extraintestinal disease.

  17. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

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

    NARCIS (Netherlands)

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

    2009-01-01

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

  19. AUTOLOGOUS SERUM SKIN TESTING (ASST) IN CHRONIC IDIOPATHIC URTICARIA

    OpenAIRE

    Arun; Suresh; Raghavendra; Vijay; Ramesh,; Asha; Manali; Jitendra

    2014-01-01

    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 A...

  20. Nursing experience of idiopathic thrombocytopenic purpura%特发性血小板减少性紫癜的护理体会

    Institute of Scientific and Technical Information of China (English)

    袁玖莲

    2015-01-01

    目的:本文通过回顾性分析56例特发性血小板减少性紫癜患者的临床资料,阐述了本病的心理护理、病情观察、出血的护理、用药护理、饮食护理、健康教育等护理措施。对特发性血小板减少性紫癜患者采取综合性的护理干预措施,可以有效地提高临床治疗效果,降低并发症的发生率,降低死亡率。%In this paper,through retrospectively analyzing the clinical datas of 56 patients with idiopathic thrombocytopenic purpura,the author expounded the mental nursing,observation,bleeding nursing,drug nursing,diet nursing and health education. Comprehensive nursing intervention measures for patients with idiopathic thrombocytopenic purpura can effectively improve the clinical treatment effect,reduce the incidence of complications and mortality.

  1. [Splenectomy in chronic idiopathic thrombopenic purpura in adults. Apropos of 49 cases].

    Science.gov (United States)

    Melki, J; Dauce, J P; Kunlin, A; Tilly, H; Julien, J P; Monconduit, M; Piguet, H

    1989-01-01

    The authors reviewed the case files of 49 adult patients undergoing splenectomy for chronic idiopathic thrombocytopenic purpura at the Centre Henri Becquerel between 1970 and 1987. Although the postoperative course was straightforward in 83.7% of cases, one reoperation for subphrenic abscess was necessary and there was one postoperative death. Remission from thrombocytopenia was obtained in 87.5% of the patients, but only transiently in 8.5% of them. No preoperative predictive factors could be demonstrated. An early postoperative rise in the platelet count to more than 500 G/litre appears to ensure a good subsequent result. Secondary infectious complications are not exceptional and can be fatal (one death in our series); they require prophylaxis by anti-pneumococcal vaccination. The place of prophylactic antibiotic therapy has yet to be defined.

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

    Science.gov (United States)

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

    2011-12-01

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

  3. Idiopathic thrombocytopenic purpura (ITP)

    Science.gov (United States)

    Blood tests will be done to check your platelet count . A bone marrow aspiration or biopsy may also be done. ... the disease usually goes away without treatment. Some children ... the platelet count in about half of people. However, other drug ...

  4. 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.

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

    Directory of Open Access Journals (Sweden)

    Nayereh Alizadeh

    2014-04-01

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

  6. 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.

  7. Megakaryocytic dysfunction in myelodysplastic syndromes and idiopathic thrombocytopenic purpura is in part due to different forms of cell death

    NARCIS (Netherlands)

    Houwerzijl, E. J.; Blom, N. R.; van der Want, J. J. L.; Vellenga, E.; de Wolf, J. T. M.

    2006-01-01

    Platelet production requires compartmentalized caspase activation within megakaryocytes. This eventually results in platelet release in conjunction with apoptosis of the remaining megakaryocyte. Recent studies have indicated that in low-risk myelodysplastic syndromes (MDS) and idiopathic thrombocyto

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

    Institute of Scientific and Technical Information of China (English)

    王晓格; 胡姬婷

    2014-01-01

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

  9. [Treatment of chronic refractory idiopathic thrombocytopenia purpura. 10 years experience at the Salvador Zubiran National Institute of Nutrition].

    Science.gov (United States)

    Pita-Ramírez, L; Hurtado-Monroy, R; Labardini-Méndez, J

    1992-01-01

    A total of 126 patients with chronic idiopathic thrombocytopenic purpura were diagnosed from January 1980 to January 1990 in our institute. In this group of patients, 21 were refractory to prednisone therapy, splenectomy or both, or had had a relapse after a good response with these treatments. They were given other therapies. There was enough information for evaluation in 16 of the 21 patients. The treatment responses were classified according to the post-therapy platelet counts: complete response (CR) = > 150 x 10(9)/L for more than three months; partial response (PR) = 50-150 x 10(9)/L for more than three months; any response (AR) = CR + PR; no response (NR) = < 50 x 10(9)/L. There were 15 women and one male. The median age was 41 years (range 11 to 65). 6-mercaptopurine was given in all patients with CR = 31.2%, PR = 18.8%, AR = 50% and NR = 50%. Seven patients received cyclophosphamide with CR = 28.6%, PR = 14.3%, AR = 42.9% and NR = 57%. Vincristine was given in four patients with only one PR. Interferon alpha 2B was given in four patients with two transitory PR. One patient received colchicine and vitamin C without response. It is concluded that 6-mercaptopurine and cyclophosphamide are useful drugs in refractory thrombocytopenic purpura.

  10. Analysis of clinical feature of infantile idiopathic thrombocytopenic purpura%婴儿特发性血小板减少性紫癜临床特点分析

    Institute of Scientific and Technical Information of China (English)

    刘小丹; 李学荣; 孙立荣

    2011-01-01

    目的 分析婴儿特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的特点.方法 从性别、临床表现、实验室检查、治疗效果等方面对77例ITP患儿进行观察和分析评价.结果婴儿组男性占75%,年长儿组男性占60%; 诱因为疫苗接种:婴儿组占28%,年长儿组占4%;诱因为感染:婴儿组为38%,年长儿组为58%;临床表现:婴儿组多见皮肤出血,出血程度较轻,年长儿组多见黏膜出血,或皮肤和黏膜混合出血,出血程度较重;治疗效果:婴儿组治愈+显效者30/32例(94%),年长儿组36/45例(84%),婴儿组无一例发展为慢性型,年长儿组3例发展为慢性型.结论 与年长儿组比较,婴儿组ITP患儿常见于男性,发病诱因主要为感染和免疫接种,出血程度较轻,疗效反应好,起效快,不易发展为慢性.%Objective To analyze the characteristics of infantile idiopathic thrombocytopenic purpura (ITP).Methods Clinical data of 77 ITP inpatients ( including 32 infants and 45 children older than 1 year of age)were observed.Clinical features, manifestations, laboratory findings and the treatment effect were analyzed and evaluated.Results Male accounted for 75% and 60% in infants and older children respectively; 28% of infants vs.4% of older children were induced by immunizations; 38% of infants vs.58% of older children were induced by infections; skin bleeding or mild hemorrhage were most commonly found in infants, while mucosal bleeding, mixed bleeding of skin and mucous membrane and severe hemorrhage were most commonly found in older children; the cure rate of infants was 30/32 (94%) compared with 36/45 (86%) in older children.None of infants vs.3 of older children developed into chronic ITP.Conclusion Infantile ITP preferentially affects boys.It is mainly induced by infection and immunization.It has mild hemorrhage, favorable therapeutic effect and lower susceptibility to chronic ITP.

  11. Unravelling the incidence and etiology of chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Visser, N.A.

    2016-01-01

    Chronic idiopathic axonal polyneuropathy (CIAP) is a sensory or sensorimotor polyneuropathy that has a slowly progressive course without severe disability. CIAP is diagnosed in a significant proportion of patients with polyneuropathy, but precise figures on the incidence of polyneuropathy and CIAP w

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

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    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.

  13. Chronic idiopathic intestinal pseudo-obstruction in an English bulldog.

    Science.gov (United States)

    Dvir, E; Leisewitz, A L; Van der Lugt, J J

    2001-05-01

    A case of chronic idiopathic intestinal pseudo-obstruction in an English bulldog is described. The dog was presented with chronic weight loss and vomiting. An intestinal obstruction was suspected based on clinical and radiological findings. A diagnosis of chronic idiopathic intestinal pseudo-obstruction was made on the basis of full thickness intestinal biopsies. The dog was refractory to any antiemetic therapy. Necropsy revealed marked atrophy and fibrosis of the tunica muscularis, together with a mononuclear cell infiltrate extending from the duodenum to the colon. This case was presented with clinical findings consistent with visceral myopathy in humans--namely, atony and dilatation of the whole gut--but the histological findings resembled sclerosis limited to the gastrointestinal tract.

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

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    V.I. Petrov

    2010-06-01

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

  15. Evaluation of H2 receptor antagonists in chronic idiopathic urticaria

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    Minocha Y

    1995-01-01

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

  16. Comparison Of Levocetirizine And Cetirizine In Chronic Idiopathic Urticaria

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    Rathi Sanjay K

    2004-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2008-01-01

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

  18. Idiopathic chronic hypertrophic craniocervical pachymeningitis: case report.

    Science.gov (United States)

    Botella, C; Orozco, M; Navarro, J; Riesgo, P

    1994-12-01

    A 55-year-old woman with a unique form of chronic hypertrophic pachymeningitis involving the posterior fossa and upper cervical spine is reported. Unlike other cases previously described, the clinical picture was dominated by signs of increased intracranial pressure, lower cranial nerve disorders, and a progressive cervical radiculomyelopathy. The diagnosis was made by means of a contrast-enhanced magnetic resonance imaging scan and confirmed by histological examination of the excised dura. Surgical treatment with removal of the hypertrophic dura provided temporary relief, although the natural history of the disease was not modified. Exhaustive bacteriological and histopathological studies failed to identify a specific cause for this diffuse hypertrophy of the cranial and cervical dura. The literature is reviewed, and other histologically documented cases are discussed.

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

    Science.gov (United States)

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

    2014-01-01

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

  20. A perspective for pediatric surgeons: chronic idiopathic intestinal pseudoobstruction.

    Science.gov (United States)

    Shaw, A; Shaffer, H; Teja, K; Kelly, T; Grogan, E; Bruni, C

    1979-12-01

    Chronic idiopathic intestinal pseudoobstruction (CIIP) is a disorder of alimentary tract motility with onset of symptoms in adolescence or young adulthood, characterized by recurring attacks of abdominal pain, distention, vomiting, and constipation often leading to malnutrition and death. Starting with an adolescent patient, treated for a succession of life-threatening attacks of pseudoobstruction, the authors have identified CIIP in four generations of a Virginia family. Studies show characteristic dilation and diminished motility of the esophagus and intestine. Surgery's role in CIIP is palliative, and operation should only be offered when medical measures have failed to control symptoms.

  1. 重组人血小板生成素在难治性血小板减少性紫癜中的应用%Application of recombinant human thrombopoietin on refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    曹慧琴; 任羽; 苏保雄; 郝彩霞; 刘卉; 刘宇宏

    2012-01-01

    Objective To evaluate the efficacy and safety of human recombinant thrombopoietin ( rhTPO ) on refractory idiopathic thrombocytopenic purpura ( ITP ). Methods 35 patients with ITP were divided into three groups: test group 1 ( treated with rhTPO combined danazol,n = 12 ),test group 2( only use rhTPO,n = 8 ),control group ( only use danazol,n = 15 ). Results The median counts of platelet in test group 1 was 6( 1. 0 ~ 15. 0 ) ×109/L before medi- cation;the maximum value increased to 97( 43 ~ 136 )× 109/L after 14 d of treatment(P 0.05).结论 rhTPO治疗难治性ITP安全、有效,rhTPO联合达那唑比单用达那唑起效快,能更好地控制出血症状.

  2. Púrpura trombocitopênica idiopática na infância: estudo de base populacional no Catar Idiopathic thrombocytopenic purpura in childhood: a population-based study in Qatar

    Directory of Open Access Journals (Sweden)

    Naima Al-Mulla

    2009-06-01

    Full Text Available OBJETIVO: Definir o padrão da púrpura trombocitopênica idiopática (PTI (aguda/crônica, e descrever seus sintomas e características clínicas em crianças com menos de 14 anos de idade em uma sociedade árabe recentemente desenvolvida. MÉTODOS: Este estudo descritivo retrospectivo foi realizado no Departamento de Pediatria do Hospital Geral de Hamad, Hamad Medical Corporation, Catar. Foram incluídas neste estudo 50 crianças com idade inferior a 14 anos e diagnóstico de PTI durante o período de 2000 a 2005. RESULTADOS: Das crianças estudadas (50, 62% foram diagnosticadas com PTI aguda e 38% com PTI crônica. A PTI aguda foi mais prevalente em meninos (64,5% em comparação com meninas (35,5%, enquanto que a PTI crônica apresentou uma distribuição quase igual em meninos (57,9% e meninas (42,1%. História de infecção viral foi comum em casos de PTI tanto aguda (71% quanto crônica (63,2%; 68% das crianças com PTI apresentaram contagem de plaquetas abaixo de 20x10(9/L ao diagnóstico. A maioria das crianças estudadas (74% foi tratada com imunoglobulina intravenosa. CONCLUSÕES: O estudo revelou uma alta incidência de PTI entre as crianças no Catar. As descobertas do estudo são semelhantes às de outros relatos internacionais.OBJECTIVE: To find the pattern of idiopathic thrombocytopenic purpura (ITP (acute/chronic and to describe presenting features and clinical characteristics of the disease in children below 14 years of age in a newly developed Arabian society. METHODS: This retrospective, descriptive study was carried out at the Pediatric Department of the Hamad General Hospital, Hamad Medical Corporation, Qatar. A total of 50 children below 14 years of age who were diagnosed with ITP during the period 2000-2005 were included. RESULTS: Among the studied children (50, 62% were diagnosed with acute ITP and 38% with chronic ITP. Acute ITP was more prevalent in boys (64.5% when compared with girls (35.5%, whereas for chronic ITP

  3. Rituximab treatment for symptomatic chronic ITP

    NARCIS (Netherlands)

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

    2006-01-01

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

  4. Inhibition of ileal bile acid transporter: An emerging therapeutic strategy for chronic idiopathic constipation.

    Science.gov (United States)

    Mosińska, Paula; Fichna, Jakub; Storr, Martin

    2015-06-28

    Chronic idiopathic constipation is a common disorder of the gastrointestinal tract that encompasses a wide profile of symptoms. Current treatment options for chronic idiopathic constipation are of limited value; therefore, a novel strategy is necessary with an increased effectiveness and safety. Recently, the inhibition of the ileal bile acid transporter has become a promising target for constipation-associated diseases. Enhanced delivery of bile acids into the colon achieves an accelerated colonic transit, increased stool frequency, and relief of constipation-related symptoms. This article provides insight into the mechanism of action of ileal bile acid transporter inhibitors and discusses their potential clinical use for pharmacotherapy of constipation in chronic idiopathic constipation.

  5. 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.

  6. 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

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

    Institute of Scientific and Technical Information of China (English)

    刘巧

    2010-01-01

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

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

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    Filiz Canpolat

    2009-03-01

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

  9. Clinical Characteristics of Idiopathic Thrombocytopenic Purpura in 401 Infants%婴幼儿特发性血小板减少性紫癜401例临床特点

    Institute of Scientific and Technical Information of China (English)

    潘艳莎; 贾苍松; 陈婷婷

    2011-01-01

    Objective To analyze clinical characteristics of infants with idiopathic thrombocytopenic purpura(ITP) from 1 month to 3 years old, and compare the therapeutic effects. Methods The data of 401 ITP children were collected. All of the patients were given hormone shock treatment and intravenous immunoglobulin. According to the extent of platelet count and bleeding improvement, the outcome included:complete remission, effective, invalid. The patients were divided into younger group( ≤1 year old) and older group( > 1 -3 years old)based on age, acute group( ≤6 months) and chronic group( >6 months)based on the course of disease, their clinical data were retrospectively analyzed by SPSS 12.0 software. Results 1. Although both the younger group and the older group had higher male/female ratio, there was no significant difference of the percentage of the male between younger group and older group(x2 =0. 682 ,P >0. 05 ). 2. The median of platelet count at admission in the younger group were statistically lower than those in the older group ( Z = 2. 668, P < 0. 05 ). 3. Both the median of megakrocytes( which produce platelets) and the increase ratio of megakrocytes in the younger group were statistically lower than that in the o1der group( Z = 2. 065 ,P < 0.05 ;x2 = 16. 322, P < 0. 001 ). 4. Under the same treatment, the time needed for the platelets to become normal ( ≥ 100 × 109 L -1 ) in the younger group were statistically lower than that in the older group( Z = 3. 542,P < 0. 001 ). 5. The median of platelet count at admission in the acute group were statistically lower than that in the chronic group ( Z = 2. 100,P < 0.05 ). 6. There was no significant difference in terms of hospital stays between the transfused patients and untransfused patients( Z = 1. 385, P > 0. 05 ). Conclusions The infants with ITP have higher male/female ratio, most possess unobvious incentive and mucocutaneous hemorrhage. The younger patients ( ≤ 1 year old) with lower

  10. [The idiopathic hypereosinophilic syndrome and chronic eosinophilic leukemia].

    Science.gov (United States)

    Chrobák, L; Voglová, J

    2005-12-01

    Idiopathic hypereosinophilic syndrome is a heterogenous group of hematological disorders characterized by eosinophilia (> 1.5 x 10(9)/l) persistent for more than 6 months, exclusion of reactive eosinophilia from other causes, such as parasitic infections or allergy, and evidence of end-organ damage. According to World Health Organization the exclusion includes all neoplastic disorders in which eosinophils are part of the neoplastic clone. Excluded should be also T cell population with aberant phenotype and abnormal cytokine production, recently considert also as "lymphocytic" variants of the HES [42]. HES has to be reclassified as chronic eosinophilic leukemia (CEL) when there is evidence for clonality based on the presence of chromosomal abnormalities or inactivation of X-chromosome in female patients. The successful empiric treatment of patients with tyrosine kinase inhibitor imatinib (Glivec) suggested the presence of an imatinib-sensitive tyrosine kinase inhibitor. The identification of a specific intersticial chromosome deletion del(4)(q12;q12) creating the FIP1L1-PDGFRA fusion gene confirmed this hypothesis. Patients carrying this gene should be reclassified as CEL and detection of this gene is a positive predictor for response to imatinib therapy. Effective doses of imatinib are 100 mg/day. The side effects are minimal. The only exception is an acute left ventricular dysfunction which has been reported in three patients within the first week of treatment with imatinib. Imatinib has been successfully used also in some patients with the constitutively activated thyrosine kinase ETV6-PDGFRbeta [1] and in systemic mast cell disease associated with eosinophilia. Other therapeutical options for HES/CEL have been mentioned. The resistence to imatinib and the possibilities how to overcome it are discussed.

  11. 婴儿期特发性血小板减少性紫癜的临床特点分析%Clinical Analysis of Infantile Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    王功僚; 邓仕华; 李慧娴; 黄积德

    2013-01-01

    Objective To analysis clinical characteristics of infantile idiopathic thrombocytopenic purpura (ITP).Methods From Jaunary 2006 to December 2011,a total of 130 ITP patients (including 67infants and 63 children) were enrolled in this study.They were divided into two groups according to their age,infant group (n=67) and young children group (n=63).The study protocol was approved by the Ethical Review Board of Investigation in Human Being of Guangxi Baise Maternal and Child Health-Care Hospital.Informed consent was obtained from all participates' parents.Clinical features of gender distribution,predisposing factors,clinical manifestations,blood test on admission,treatment effect and outcome were retrospectively reviewed.Results No significant difference of gender distribution in two groups were found (U=0.6499,P> 0.05).Prodromal infections within 4 weeks before the onset were found in 40.29% (27/67) of infant group and 60.32% (38/63) of young children group (U=2.2827,P<0.05).The history of vaccination within 4 weeks were found in 23.88% (16/67) of infant group,and zero (0.00) of young children group (U=4.2909,P<0.01).The median of platelet counts on admission in infant group (8 × 109/L) were significant lower than that in young children group (13× 109/L) (t =2.864,P<0.01).Clinical presentation of infant group were skin bleeding or mile hemorrhage,young children group showed mucosal bleeding or mixed bleeding of skin and mucous membrane and severe hemorrhage.Bleeding in other sites was observed predominantly in infant group compared with that in young children group (U=2.3238,P<0.05).The rates of therapeutic effects (complete remission + valid) in infant group was 100.00% with no chronic cases,and 92.06% (58/63) in young children group,11 cases (17.46%) were developed into chronic ITP in young children group.Conclusions The history of vaccination was the crux of infantile ITP.Infantile ITP has good efficacy,rapid response,lower susceptibility

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

    OpenAIRE

    2016-01-01

    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. Me...

  13. 儿童特发性血小板减少性紫癜脾切除术的围手术期处理%The perioperative management of splenectomy for Idiopathic thrombocytopenic purpura in children

    Institute of Scientific and Technical Information of China (English)

    金晟; 吴晔明

    2015-01-01

    Objetive To explore the perioperative methods of idiopathic thrombocytopenic purpura (ITP)in children. Methods Clinical data of 12 children suffering from ITP,who underwent splenectomy, their perioperative treatments and their effects were retrospectively analyzed. Results Our group included 12 children with the mean age of 7.1 years old (range 3y to 12y).The mean day of their illness was 3 years (range 1 .5y to 7y).Preoperatively,the platelet counts of 10 children were lower than normal count,with the mean count of 29.4 ×109 /L.Glucocorticoids combined with platelet transfusion were employed for 7 children whose platelet counts were lower than 30 ×109 /L.A second platelet transfusion combined with a large dose of glucocorticoids and immunoglobulin injections were done for 4 children whose platelet counts were not improved after the first transfusion.When their platelet counts were improved to >60 ×109 /L,they were treated by e-mergency splenectomy.All patients were successfully treated.The platelet counts of 2 patients,who were posi-tive in PAIg,were dropped to below normal count.During the follow-up of 10 months to 8 year,8 children were curing,while 2 patients were with durable remission. Conclusions Splenectomy is a safe and effective method for the children who suffer from chronic ITP and ITP which is ineffective for drug therapy.In addition, PAIg should be considered as a routine examination in children with ITP.%目的:探讨儿童特发性血小板减少性紫癜(ITP)脾切除的围手术期处理方法。方法回顾性分析2006年至2014年作者收治的12例 ITP 行脾切除术的患儿临床资料,探讨其围手术期的处理及治疗效果。结果本组12例平均年龄7.1(3~12)岁,病程1.5~7年,平均3年。10例术前血小板低于正常值,平均为29.4×109/L,最低5×109/L。术前对7例血小板<30×109/L 的患儿采用激素联合输血小板治疗,其中4例输注后血小板无法提升,

  14. Omalizumab for the Treatment of Chronic Idiopathic Urticaria: Systematic Review of the Literature.

    Science.gov (United States)

    Tonacci, Alessandro; Billeci, Lucia; Pioggia, Giovanni; Navarra, Michele; Gangemi, Sebastiano

    2017-02-22

    Omalizumab is recombinant humanized monoclonal antibody to immunoglobulin E. Guidelines for the treatment of chronic idiopathic urticaria (also known as chronic spontaneous urticaria) recommend the use of omalizumab as third-line therapy in addition to high doses of histamine receptor type 1 (H1 ) antihistamines when they are unsuccessful as first- and second-line therapy. We performed a systematic review of the literature to identify studies that evaluated the efficacy of omalizumab for the treatment of chronic idiopathic urticaria, in both controlled and real-world settings, to assess its potential role as a preferred therapy. The PubMed, ScienceDirect, LILACS (Latin American and Caribbean Health Sciences Literature), and Google Scholar databases were searched between January 1, 2000, and November 21, 2016. The search was limited to articles published in peer-reviewed journals in the English language, and 29 studies were included in this review. Omalizumab 300 mg administered every 4 weeks appears to be the most effective and safe dosage, with a rapid response time, for the treatment of chronic idiopathic urticaria, with few minor adverse effects, and appears to be safe in the offspring of pregnant patients who received the drug. However, as published studies of omalizumab are sparse, future studies are warranted. When findings are confirmed in larger studies, due to its efficacy, safety, and increased benefit/cost ratio, omalizumab could become the preferred method of treatment for chronic idiopathic urticaria in patients unresponsive to H1 antihistamines.

  15. Clinical study on recombinant human thrombopoietin in the treatment of refractory idiopathic thrombocytopenic purpura%重组人血小板生成素治疗难治性特发性血小板减少性紫癜临床观察

    Institute of Scientific and Technical Information of China (English)

    张晓丽; 刘延方; 孙慧; 谢新生; 姜中兴; 孙玲; 万鼎铭; 刘林湘

    2010-01-01

    Objective To observe the efficacy and toxicity of recombinant human thrombopoietin (rhTPO)in the treatment of chronic refractory idiopathic thrombocytopenic purpura(ITP).Methods Twenty- four patients with chronic ITP were administered with rhTPO subcutaneously at 300 IU/(kg · d)for 14 days or until the platelet count > 100 × 109/L.Results Out of 24 patients with ITP, 12 cases showed significant response and 5 cases showed good response to the treatment with rhTPO.The peak platelet count after treatment was(129 ± 97)× 109/L, significantly higher than the mean platelet count [(10 ±5)× 109/L]before treatment(t = 5.822, P <0.01).The agent was well tolerated in most patients.Conclusions rhTPO exerts satisfactory efficacy in the treatment of chronic ITP with mild side effects.%目的 观察重组人血小板生成素(rhTPO)治疗慢性难治性特发性血小板减少性紫癜(ITP)的临床疗效及不良反应.方法 24例慢性难治性ITP患者皮下注射rhTPO 300 IU/(kg·d),疗程14 d或血小板>100×109/L后停药.结果 24例患者rhTPO治疗后,显效12例,有效5例,用药前血小板平均值为(10±5)×109/L,治疗后血小板计数的升高的最高值为(129±97)×109/L,与用药前比较差异有统计学意义(t=5.822,P<0.01).多数患者对药物耐受良好.结论 rhTPO治疗慢性难治性ITP具有良好的疗效,不良反应轻微.

  16. Familial progressive neuronal disease and chronic idiopathic intestinal pseudo-obstruction.

    Science.gov (United States)

    Steiner, I; Steinberg, A; Argov, Z; Faber, J; Fich, A; Gilai, A

    1987-06-01

    Chronic idiopathic intestinal pseudo-obstruction (CIIP) is characterized by recurrent episodes of bowel obstruction without mechanical cause. In five members of two Jewish-Iranian families, CIIP was associated with progressive neuronal disease, starting before age 30, with ophthalmoplegia, sensorimotor peripheral neuropathy, and hearing loss. There was no evidence of CNS involvement. The pattern suggested autosomal recessive inheritance.

  17. Coexistence of chronic renal failure, hashimoto thyroiditis and idiopathic hypoparathyroidism: a rare case report.

    Science.gov (United States)

    Yildiz, Saliha; Soyoral, Yasemin; Demirkiran, Davut; Ozturk, Mustafa

    2014-04-01

    Hypoparathyroidism is an uncommon disease and its coexistence with chronic renal failure is quite rare. Hypocalcemia and hyperphosphatemia are seen in both diseases. Diagnosis of hypoparathyroidism may be overlooked when parathormone response is not evaluated in patients with chronic renal failure. A 19-year-old female patient who had been receiving hemodialysis for 3 years because of chronic renal failure was diagnosed as idiopathic hypoparathyroidism and hashimoto thyroiditis. When her medical records on the first admission and medical history were evaluated, hypoparathyroidism and hashimoto thyroiditis were seen to be present also when she was started hemodialysis. Idiopathic hypoparathyroidism should be suspected in case as absence of parathormone response to hypocalcemia in patients with chronic renal failure. It should be taken into consideration that hashimoto thyroiditis may accompany and required analysis should be done.

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

    Institute of Scientific and Technical Information of China (English)

    白松婷; 赵晓明; 盛光耀; 方营旗

    2011-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    龙远雄; 马利; 何丽云; 闫世艳; 王彬; 王双连

    2015-01-01

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

  20. 重组人血小板生成素治疗难治性特发性血小板减少性紫癜的循证评价%Evidence-based Evaluation of Recombinant Human Thromobopoietin for Refractory Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    黄琳; 任晓蕾; 刘一; 李玉珍

    2011-01-01

    目的:评价重组人血小板生成素(rhTPO)治疗成人慢性难治性特发性血小板减少性紫癜(ITP)的有效性与安全性.方法:采用循证医学的文献分析评价方法,计算机检索CBM、CNKI、MEDLINE、EMbase、Cochrane图书馆临床对照试验资料库等数据库,收集rhTPO治疗成人慢性难治性ITP的临床研究证据.结果:检索到rhTPO治疗成人慢性难治性ITP的文献有:自身前后对照试验5篇,随机对照试验2篇.按Jadad量表评分:1篇随机对照研究质量较高,为4分,其余文献均小于3分.5项自身对照研究结果基本一致,认为rhTPO可升高成人难治性ITP患者的血小板计数,且不良反应轻微.2项随机对照试验认为rhTPO联合达那唑治疗难治性ITP的效果明显优于单用达那唑,且对于单用达那唑治疗无效者,接受rhTPO治疗后血小板计数亦明显升高.结论:现有研究显示rhTPO在治疗成人慢性难治性ITP方面疗效确切、安全性好,但试验设计存在不足,尚需进行设计良好的大样本研究.%OBJECTIVE: To evaluate the efficacy and safety of recombinant human thromobopoietin (rhTPO) for adult chronic refractory idiopathic thrombocytopenic purpura (ITP).METHODS: By literature analysis based on evidence-based medicine, clinical trials about rhTPO in the treatment of adult chronic refractory ITP were retrieved from CMB, CNKI, MEDLINE, EMbase and Cochrane library.RESULTS: Of collected pertinent literature about rhTPO in the treatment of adult chronic refractory ITP, five were self-control studies and two were randomized controlled trials (RCTs).The quality of seven studies was evaluated according to Jadad scale, of which one scored four points, and the others below three points.The five self-control studies all supported that rhTPO could effectively elevate the platelet counts and was safe for patients with adult chronic refractory ITP.Two RCTs indicated that rhTPO combined with danazol was more effective than danazol for

  1. Lubiprostone: a new drug for the treatment of chronic idiopathic constipation.

    Science.gov (United States)

    Baker, Danial E

    2007-01-01

    Lubiprostone offers an additional alternative for patients with chronic idiopathic constipation. Lubiprostone is more efficacious than placebo in the treatment of chronic idiopathic constipation. In placebo-controlled clinical trials, lubiprostone therapy was generally well tolerated and was not associated with severe adverse effects; however, the high incidence of nausea may be problematic for some patients. The nausea may be alleviated or minimized by administering the dose with food, and some patients may require a dosage reduction to 24 mug once daily. The key limitations of the placebo-controlled clinical trials include the absence of information regarding the duration of the constipation and previous types of therapies that had been used to treat the constipation and the absence of an active control group. Comparative studies with other therapies (eg, saline laxatives, polyethylene glycol) used for constipation are necessary to determine the clinical and economic value of this agent relative to other forms of therapy.

  2. [Chronic cough and worsening dyspnea: a case of idiopathic tracheal stenosis].

    Science.gov (United States)

    Conti, Valentina; Calia, Nunzio; Pasquini, Claudio; Zardi, Silvia; Finetti, Cinzia; Stomeo, Francesco; Ravenna, Franco

    2013-04-01

    We report a case of idiopathic tracheal stenosis in a 75-year-old woman, who presented to our observation with a diagnosis of asthmatic bronchitis characterized by cough and exertional dyspnea, later complicated by the appearance of tirage. Biopsy of the lesion showed focal squamous metaplasia of the epithelium lining, multiple sclerosis and chronic inflammatory infiltration of the corium. The patient was treated with endoscopic destruction via rigid bronchoscopy, through the combined action of YAG laser and mechanical debulking.

  3. Small bowel volvulus in a patient with chronic idiopathic intestinal pseudo-obstruction

    OpenAIRE

    Youssef, Haney; Rashid, Sidi H; Cellador, Enrique Collantes; Baragwanath, Phil

    2009-01-01

    Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare syndrome of ineffectual gut motility associated with clinical, endoscopic and radiological exclusion of mechanical causes, as well as evidence of air–fluid levels in distended bowel loops. A case of small bowel volvulus in a patient with an established diagnosis of CIIP is presented. The case is illustrated by images of operative findings and computed tomography scan reconstruction, showing the classical appearances of small bo...

  4. Idiopathic erythrocytosis in a patient on chronic hemodialysis

    Directory of Open Access Journals (Sweden)

    Dong Hyun Lee

    2015-03-01

    Full Text Available A 78-year-old man on hemodialysis presented to our hospital with erythrocytosis. He had started hemodialysis 4 years previously, with a hemoglobin level of 9.8 g/dL, and was administered erythropoiesis stimulating agents and ferrous sulfate. Two years previously, his hemoglobin level increased to 14.5 g/dL and the treatment for anemia was discontinued. He continued hemodialysis thrice weekly; however, the hemoglobin level had increased to 17.0 g/dL at the time of presenting to our hospital. His serum erythropoietin level was 31.4 mIU/mL (range, 3.7–31.5 mIU/mL, carboxyhemoglobin level was 0.6% (range, 0–1.5%, and oxygen saturation in ambient air was 95.4%. The JAK2 V617F mutation was not observed and other bone marrow abnormalities were not identified. The patient was diagnosed with bladder cancer and a transurethral resection was performed. Eight months after the treatment of bladder cancer, his hemoglobin level was 15.1 g/dL, and he was diagnosed with idiopathic erythrocytosis.

  5. Clinical observation for cytomegalovirus infection of infant idiopathic thrombocytopenic purpura%婴儿特发性血小板减少性紫癜巨细胞病毒感染临床观察

    Institute of Scientific and Technical Information of China (English)

    王欣; 乔丽津

    2011-01-01

    Objective To investigate the effect of cytomegalovirus (CMV) infection on the patho-genesis of infant idiopathic thrombocytopenic purpura (FTP). Methods IgM/DNA of serum CMV were detected in 178 cases of infants ITP in our hospital from June of 2004 to December of 2009. According to the results of positive or negative, they were divided into observation group and control group. Their ages of onset, course of disease, inducing factors, bleeding degrees, lab examinations and outcome were analyzed retrospectively. Results The median ages of observation group (81 cases) and control group (97 cases) were three months and six months respectively; The prodromal infection rate for both groups were 37% and 42% respectively and there was no statistical difference (P >0. 05). The prodromal infection was mainly upper respiratory tract infection; 72% and 75% patients in two groups had vaccination history and there was no statistical difference ( P > 0.05); The degrees of bleeding in two groups had no statistical difference (P >0.05). The platelet counts of over half patients were less than 10 ×109/L when admitted into hospital. The mean values were 12.28 × 109/L and 11.67 × 109/L respectively and showed no statistical difference ( P > 0.05); The bone marrow smears of two groups appeared normal or increased number of megakaryocyte combined with maturation arrest and thrombocyte dysplasia; 86% (31/36) and 81% (26/32) patients in two groups had abnormal immunoglobulin and there was no statistical difference (P > 0.05). After treatment of prednisone and/or venous gamma globulin, the platelet counts of 75 cases (93% ) and 93 cases (96% ) in two groups turned to normal and there was no statistical difference ( P > 0. 05 ). Conclusions CMV infection is very common in small children and may be one of inducing factors for thrombocytopenic purpura. Clinical manifestation of ITP patients has no significant difference whether they have CMY infection or not. Infant ITP with or without

  6. Prolonged idiopathic gastric dilatation following revascularization for chronic mesenteric ischemia.

    Science.gov (United States)

    Gauci, Julia L; Stoven, Samantha; Szarka, Lawrence; Papadakis, Konstantinos A

    2014-01-01

    A 71-year-old female presented with nausea, emesis, early satiety, and abdominal distension following revascularization for chronic mesenteric ischemia. Computed tomography angiogram showed gastric dilatation. Esophagogastroduodenoscopy, small bowel follow through, and paraneoplastic panel were negative. Gastric emptying was delayed. Despite conservative management, she required a percutaneous endoscopic jejunostomy. The development of a prolonged gastroparetic state has not been previously described.

  7. 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

  8. Clinical Significance of Helicobacter Pylori in Children with Idiopathic Thrombocytopenic Purpura%幽门螺杆菌在儿童特发性血小板减少性紫癜中的临床意义

    Institute of Scientific and Technical Information of China (English)

    唐瑛; 王书春; 王鲁娟; 刘永; 王海英; 王占聚

    2013-01-01

    This study was aimed to investigate the clinic significance of helicobacter pylori (HP) in children with idiopathic thrombocytopenic purpura( ITP). The infection of HP in 92 ITP children was determined by 13C-Urea Breath Test, the same test was also performed on 66 healthy children. The 68 children infected with HP were randomly divided into 2 groups: single drug group treated only with corticosteroid and; combined drug group treated with corticosteroid and anti-helicobacter pylori treatment. The results showed that 68 patients infected with HP were found in 92 ITP children(74. 7% ), 26 patients infected with HP were observed in 66 healthy children (39.4% ), which was lower than that in ITP children (74.7% , P<0.05). After anti-helicobacterpylori therapy, the total effective rate and cure rate of ITP patients increased respectively from 73.5% to 94.1 % , and the total recurrence rate( 17.0% ) was much lower than single drug group( 47. 1% , P < 0. 05). Otherwise, after therapy the platelet count in both two groups increased continuously, and at the same time point, the platelet count in snti-helicobacterium pylori group was higher than that in the single drug group(P < 0. 05). It is concluded that the ITP children have a higher infection rate of HP, which may be involved in the pathogenesis of ITP. Anti-helicobacterium pylori therapy would help to improve the therapeutic efficacy and reduce the recurrence of ITP children.%本研究旨在探讨幽门螺杆菌(helicobacter pylori,HP)在儿童特发性血小板减少性紫癜(ITP)中的临床意义.用13C尿素呼气试验检测92例ITP儿童患者的感染情况,并检测66名健康儿童作为对照组.将ITP患儿中68例阳性者随机分为2组:复合用药治疗组34例,应用糖皮质激素+抗菌治疗;单药治疗组34例,用糖皮质激素治疗.结果表明:ITP患儿HP的感染率为74.7%,明显高于对照组(39.4%)(P<0.05).ITP患儿经治疗后,单药治疗组的总有效率(73.5

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

    Science.gov (United States)

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

    2006-01-01

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

  10. A rare combination of thrombotic thrombocytopenic purpura and antiphospholipid syndrome.

    Science.gov (United States)

    Viner, Maya; Murakhovskaya, Irina

    2016-11-24

    Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system. In the literature, there have only been nine cases of thrombotic thrombocytopenic purpura associated with primary antiphospholipid syndrome. Seven of the nine cases suffered from one or multiple strokes, a common feature in antiphospholipid syndrome, but an uncommon finding in thrombotic thrombocytopenic purpura. We introduce the possibility of an association between thrombotic thrombocytopenic purpura and the presence of antiphospholipid antibodies. Systematic testing of ADAMTS13 activity and anti-ADAMTS13 antibodies in patients who present with neurological symptoms and thrombocytopenia, in the presence of antiphospholipid antibodies, may help with the diagnosis of the rare thrombotic thrombocytopenic purpura-antiphospholipid syndrome combination.

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

    Directory of Open Access Journals (Sweden)

    Brian E Lacy

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

  12. B淋巴细胞激活因子表达水平在儿童特发性血小板减少性紫癜诊断及预后判断中的意义%Value of B-cell activating factor expression in the diagnosis and prognosis of children with idiopathic thrombocyto-penic purpura

    Institute of Scientific and Technical Information of China (English)

    仲玉强; 王清

    2014-01-01

    Objective To study the value of B-cell activating factor (BAFF) expression levels in the diagnosis and prog-nosis of children with idiopathic thrombocytopenic purpura. Methods Children with idiopathic thrombocytopenic purpura (ITP) admitted to our hospital from September 2011 to September 2013 were enrolled in observation group, and healthy check-up chil-dren during the same period were enrolled as controls. The serum levels of B-cell activating factor, platelet antibodies (PAIgG, PAIgM, PAIgA) and platelet count were detected, and curative effect in children with ITP was observed. Results The BAFF, PAIgG, PAIgM and PAIgA levels were higher and platelet count were less in both acute and chronic ITP children than those in normal controls. The difference was signiifcant (P<0.05). The results of linear regression analysis indicated that BAFF levels were positively correlated with PAIgG, PAIgM and PAIgA levels (β=1.87~2.25, P<0.05), and were negatively correlated with platelet count (β=-2.42, P<0.05). Meanwhile, BAFF levels were negatively correlated with curative effect in ITP children (β=-1.88, P<0.05). When the value of BAFF was 1.35, the sensitivity for predicting effective treatment was 0.84 and the speciifcity was 0.75. The area under the ROC curve was 0.85 (95%CI:0.73~0.98, P=0.000). Conclusions The serum BAFF expression was increased and closely related to the level of antiplatelet antibody, platelet count and treatment effect in ITP children, and therefore, was a good indicator for the diagnosis and prognosis of ITP.%目的:研究B淋巴细胞激活因子(BAFF)表达水平在儿童特发性血小板减少性紫癜(ITP)诊断及预后中的应用价值。方法将2011年9月—2013年9月期间收治的初诊ITP患儿纳入研究组,同期健康体检儿童纳入对照组,检测两组对象血清中BAFF、抗血小板抗体PAIgG、PAIgM、PAIgA水平及血小板计数,观察治疗效果。结果急性型和慢性型ITP患儿外周血BAFF、PAIgG

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

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    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.

  14. Chronic idiopathic intestinal pseudo-obstruction treated with jejunostomy: case report and literature review

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    Carlos Renato dos Reis Lemos

    Full Text Available CONTEXT: Chronic idiopathic intestinal pseudo-obstruction is a very rare condition. CASE REPORT: This study describes a male patient who had presented obstructive symptoms for 24 years. He had been treated clinically and had undergone two previous operations in different services, with no clinical improvement or correct diagnosis. He was diagnosed with intestinal obstruction without mechanical factors in our service and underwent jejunostomy, which had a significant decompressive effect. The patient was able to gain weight and presented improvements in laboratory tests. Jejunostomy is a relatively simple surgical procedure that is considered palliative but, in this case, it was resolutive.

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

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    Joern K Pomper

    2013-11-01

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

  16. Transanal Irrigation for Refractory Chronic Idiopathic Constipation: Patients Perceive a Safe and Effective Therapy

    Science.gov (United States)

    Minty, Ian; Bain, Iain M.; Cundall, Jeremy; Yiannakou, Yan

    2017-01-01

    Background. Transanal irrigation (TAI) can successfully treat neurogenic bowel dysfunction (NBD), but patient perception of its use in chronic idiopathic constipation (CIC) is unknown. Objective. To evaluate patient perceptions of the efficacy and safety of TAI for CIC and whether there are predictive factors of perceived treatment response. Methods. Prospective data collection of baseline physiology and symptom severity; retrospective evaluation of efficacy and safety perceptions using a snapshot survey. All patients fulfilling the Rome III criteria for functional constipation with chronic idiopathic aetiology were included. The main outcome measure was the duration of patients' usage of TAI. Results. 102 patients reported 21,476 irrigations over 119 patient years, with a mean duration of therapy use of 60.5 weeks [SD 73.2 : SE 7.3]. Overall symptom improvement included general well-being (65%), rectal clearance (63%), bloating (49%), abdominal pain (48%), and bowel frequency (42%). 68 patients (67%) were “moderately better” or “very much better” on a satisfaction question. Reported complications were minor. No correlation was demonstrated between duration of therapy use and baseline measures. Conclusion. A significant proportion of CIC sufferers use TAI as a long-term or bridging therapy and perceive it as safe. This therapy demands a prospective investigation of efficacy and safety. PMID:28115930

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

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    Ekrem Akdeniz

    2016-01-01

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

  18. 不同剂量丙种球蛋白联合地塞米松治疗小儿特发性血小板减少性紫癜的临床疗效分析%Effect observation of different doses Dexamethasone combined with gamma globulin in the treatment of idiopathic thrombocytopenic purpura with children

    Institute of Scientific and Technical Information of China (English)

    覃黄正

    2014-01-01

    Objective To explore the clinical effect of different dose Dexamethasone combined with gamma globulin in the treatment of idiopathic thrombocytopenic purpura with children. Methods 120 patients with Idiopathic thrombocytopenic purpura in our hosipta were randomly divided intocontrol group, low-dose group and high dose group, each group had 40 cases. All patients were received treatment using dexamethasone injection, children with high-dose group were given Globulin 400mg/(kg·d), 1 times a day, once every 5d;children withlow-dose group were given Globulin 200mg/(kg·d), 1 times a day, once every 5d;the clinical efficacy, platelets rise time, platelet recovery time, time to stop bleeding of three group were observation and compared. Results The clinical efficacy of high-dose group, the rise time of platelets and platelet recovery time, time to stop the bleeding was compared between low-dose group and high dose group, the difference was not statistically significant (P > 0.05), compared with control group, the differences were statistically significant (P < 0.05). Conclusion Gamma globulin and dexamethasone can significantly improve pediatric idiopathic thrombocytopenic purpura clinical symptoms, the rapid increase platelet count, and small doses of gamma globulinhad similartreatment effect with high dose, it is worthy of wide application in clinical.%目的:探讨不同剂量丙种球蛋白联合地塞米松治疗小儿重症特发性血小板减少性紫癜的疗效观察。方法将120例小儿特发性血小板减少性紫癜患儿随机分为对照组,小剂量组和大剂量组,每组各40例,所有患者均接受采用地塞米松注射液治疗,大剂量组患儿给予丙种球蛋白400mg/(kg·d),1次/d,连用5d;小剂量组患儿给予丙种球蛋白200mg/(kg·d),1次/d,连用5d,观察比较三组临床疗效,血小板上升时间,血小板恢复正常时间,出血停止时间。结果小剂量组与大剂量组临床疗

  19. An effect analysis of different doses of gamma globulin combined with prednisone in the treatment of idiopathic thrombocytopenic purpura%不同剂量的丙种球蛋白联合强的松治疗特发性血小板减少性紫癜的效果分析

    Institute of Scientific and Technical Information of China (English)

    潘凤娟; 钟翠鸣; 钟华

    2015-01-01

    Objective To compare the efficacy of low dose and high dose gamma globulin combined with prednisone in the treatment of idiopathic thrombocytopenic purpura for obtaining a better cost - effect ratio treatment scheme. Methods Between March 2010 and May 2014 a total of 64 pediatric patients with idio-pathic thrombocytopenic purpura cared at Pediatrics Department of our hospital were enrolled in this study,a hidden random number table method was used to allocate the patients to two groups.Thirty- two cases in group A were given 400 mg/(kg · d)gamma globulin for 5 days,after that they received oral prednisone. Thirty-two cases in group B were given 200 mg/(kg·d)gamma globulin for 5 days,after that they received oral prednisone,after drug administration,platelet count was measured,and the platelet elevating time,mean hemostasis time,time to platelet peak and the platelet value were all recorded every day.The efficacy rate was compared between the two groups. Results After treatment,comparison of mean platelet elvating time,av-erage hemostasis time,mean platelet peak value between the two groups of pediatric children showed no signif-icant differences (P > 0.05 ).Group A patients had shorter average peak time than group B,group paired comparison yielded statistical difference (P 0.05 ). Conclusion Low dose gamma globulin combined with prednisone in treatment of idiopathic thrombocytopenic purpura can obtain high potency ratio,can quickly increase platelet and control of hemorrhage.%目的:比较大剂量和小剂量丙种球蛋白联合强的松治疗特发性血小板减少性紫癜(ITP)的效果,寻求效价比高的治疗方案。方法选择我院儿科2010年3月~2014年5月住院的64例特发性血小板减少性紫癜患儿,采用隐匿数字随机法分为两组,A 组32例患儿给予400 mg/(kg·d)丙种球蛋白治疗5 d 后口服强的松,B 组32例患儿给予200 mg/(kg·d)丙种球蛋白治疗5 d 后口服强的松,用药后每天检测血小板计数,

  20. Spirulina did not ameliorate idiopathic chronic fatigue in four N-of-1 randomized controlled trials.

    Science.gov (United States)

    Baicus, Cristian; Baicus, Anda

    2007-06-01

    Idiopathic chronic fatigue is an exclusion diagnosis established when no chronic disease is found. Spirulina platensis is an alga with a rich content of proteins, vitamins, minerals and amino acids and is considered as a bioactive additive with multiple effects, among them being effects against fatigue. However, despite the worldwide utilization of Spirulina, there are only a few quality studies with it and none concerning fatigue. The N-of-1 randomized trials are made on one patient, and by this kind of study the efficacy of a treatment on that particular patient can be assessed. A series of four N-of-1 double-blind, randomized trials were performed on four physicians who complained of chronic fatigue. Each patient was his own control and received three pairs of treatments comprising 4 weeks of spirulina and 4 weeks of placebo. Spirulina platensis was administered in a dose of 3 g/day. For each pair, the order of treatments was randomized. Outcome measures were severity of fatigue measured on a 10-point scale. The scores of fatigue were not significantly different between spirulina and placebo. Spirulina administered in a dose of 3 g/day did not ameliorate fatigue more than the placebo in any of the four subjects, and possibly it has no effect on chronic fatigue.

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

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    Fengyi Shen

    2014-01-01

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

  2. Acute immune thrombocytopenic purpura triggered by insect bite

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    Namdev R

    2009-03-01

    Full Text Available Idiopathic Thrombocytopenic Purpura (ITP is a blood disorder characterized by an abnormal decrease in the number of platelets in the blood. Acute thrombocytopenic purpura is most commonly seen in young children, with a sudden onset, following a viral illness, vaccination or an insect bite. The report is a rare case of ITP, which was triggered by a honey-bee bite and caused continuous intermittent bleeding from the gingival sulcular region of the maxillary left permanent first molar, and ecchymotic spots on the forehead, scalp, and lower limbs. A complete hemogram revealed severe thrombocytopenia with platelet count as low as 15,000/mm3. The patient was immediately hospitalized and administered platelet replacement and medication. With this report, the authors, as dentists, emphasize the significance of recognition, early diagnosis, and referral of such patients with bleeding disorders to specialized centers, for prompt treatment.

  3. Small bowel volvulus in a patient with chronic idiopathic intestinal pseudo-obstruction.

    Science.gov (United States)

    Youssef, Haney; Rashid, Sidi H; Cellador, Enrique Collantes; Baragwanath, Phil

    2009-01-01

    Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare syndrome of ineffectual gut motility associated with clinical, endoscopic and radiological exclusion of mechanical causes, as well as evidence of air-fluid levels in distended bowel loops. A case of small bowel volvulus in a patient with an established diagnosis of CIIP is presented. The case is illustrated by images of operative findings and computed tomography scan reconstruction, showing the classical appearances of small bowel volvulus. The patient recovered well after surgery and is maintained on parenteral nutrition. CIIP is a heterogeneous disorder in which the primary aims of management are nutrition, pain control and the avoidance of unnecessary repeated laparotomies. However, even in the presence of an established diagnosis of CIIP, surgeons should be vigilant to the possibility that an operable mechanical obstruction may still occur.

  4. 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.

  5. [Chronic idiopathic intestinal pseudo-obstruction: visceral myopathy. Report of 4 cases].

    Science.gov (United States)

    de Pini, A F; de Dávila, M T; Marín, A; Guastavino, E; Ruiz, J A; De Rosa, S

    1993-01-01

    Chronic intestinal pseudo-obstruction is the term applied to a heterogeneous group of functional motility disorders sharing a common clinical expression: signs and symptoms of bowel obstruction in absence of mechanical occlusion. It is caused by ineffective intestinal propulsion. The chronic form of intestinal pseudo-obstruction may be primary or secondary. Primary pseudo-obstruction or chronic idiopathic pseudo-obstruction (CIIP) defines a group of propulsive disorders having no recognized underlying diseases. This study presents four female patients, aged between 4 months to 7 years, and makes a review of the literature. The symptoms, very similar in three of them, were bilious vomiting, abdominal distention and constipation, alternating with diarrhea and malnutrition. The fourth patient, different from the others in the age of onset and evolution, only had severe constipation and abdominal bloating. The diagnostic was made by full thickness biopsies during laparotomy, getting specimens by mapping, at different heights of intestine and stomach. Samples were studied by optic and electronic microscopy and visceral myopathies were found. None of them had urinary disorders. Medical treatment consisted of total parental nutrition and/or enteral nutrition. Cisapride was not effective in the two patients who received it.

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

    Science.gov (United States)

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

    2016-01-01

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

  7. Abdominal Pain: A Comparison between Neurogenic Bowel Dysfunction and Chronic Idiopathic Constipation

    Science.gov (United States)

    Finnerup, Nanna Brix; Christensen, Peter

    2013-01-01

    Introduction. Most spinal-cord-injured patients have constipation. One-third develop chronic abdominal pain 10 years or more after injury. Nevertheless, very little is known about the nature of abdominal pain after spinal cord injury (SCI). It may be neuropathic or caused by constipation. Aim. To compare characteristics of abdominal pain in SCI with able-bodied with chronic idiopathic constipation (CIC). Subjects and Methods. 21 SCI and 15 CIC patients were referred for treatment of bowel symptoms. Constipation-related symptoms were assessed with the Cleveland Constipation Scoring System and the International Spinal Cord Injury Basic Bowel Function Data Set. Characteristics of abdominal pain were described using the Brief Danish Pain Questionnaire. Total gastrointestinal transit times (GITT) were measured by radiopaque markers. Results. Seventeen (81%) SCI and 14 (93%) CIC patients reported abdominal pain or discomfort within the last month (P = 0.38). Pain was considered more intense by CIC than by SCI patients (P GITT. Conclusion. Most characteristics of abdominal pain among SCI patients resemble those of CIC. This indicates that constipation is a major cause of pain after SCI. PMID:24159329

  8. Abdominal Pain: A Comparison between Neurogenic Bowel Dysfunction and Chronic Idiopathic Constipation

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    Pia Møller Faaborg

    2013-01-01

    Full Text Available Introduction. Most spinal-cord-injured patients have constipation. One-third develop chronic abdominal pain 10 years or more after injury. Nevertheless, very little is known about the nature of abdominal pain after spinal cord injury (SCI. It may be neuropathic or caused by constipation. Aim. To compare characteristics of abdominal pain in SCI with able-bodied with chronic idiopathic constipation (CIC. Subjects and Methods. 21 SCI and 15 CIC patients were referred for treatment of bowel symptoms. Constipation-related symptoms were assessed with the Cleveland Constipation Scoring System and the International Spinal Cord Injury Basic Bowel Function Data Set. Characteristics of abdominal pain were described using the Brief Danish Pain Questionnaire. Total gastrointestinal transit times (GITT were measured by radiopaque markers. Results. Seventeen (81% SCI and 14 (93% CIC patients reported abdominal pain or discomfort within the last month (. Pain was considered more intense by CIC than by SCI patients (. Only minor differences were found in patient’s qualitative description of abdominal pain or in the location of pain. In neither SCI nor CIC was pain associated with GITT. Conclusion. Most characteristics of abdominal pain among SCI patients resemble those of CIC. This indicates that constipation is a major cause of pain after SCI.

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

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    Sibel Arınç

    2016-08-01

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

  10. Neurogenic chronic idiopathic intestinal pseudo-obstruction, patent ductus arteriosus, and thrombocytopenia segregating as an X linked recessive disorder.

    Science.gov (United States)

    FitzPatrick, D R; Strain, L; Thomas, A E; Barr, D G; Todd, A; Smith, N M; Scobie, W G

    1997-08-01

    We present a family with three affected males in two generations with congenital neurogenic chronic idiopathic intestinal pseudo-obstruction (CIIP), patent ductus arteriosus, and large platelet thrombocytopenia apparently segregating as an X linked recessive disorder. The pattern of segregation of DNA markers within the family is consistent with linkage to the previously described neurogenic CIIP (CIIPX) locus at Xq28. This combination may represent a new contiguous gene disorder and appears to have a good prognosis with supportive therapy.

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

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    George James N

    2008-02-01

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

  12. 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

  13. Metabolic Syndrome, Neurotoxic 1-Deoxysphingolipids and Nervous Tissue Inflammation in Chronic Idiopathic Axonal Polyneuropathy (CIAP)

    Science.gov (United States)

    Hube, Larissa; Dohrn, Maike F.; Karsai, Gergely; Hirshman, Sarah; Van Damme, Philip; Schulz, Jörg B.; Weis, Joachim; Hornemann, Thorsten; Claeys, Kristl G.

    2017-01-01

    Aim Chronic idiopathic axonal polyneuropathy (CIAP) is a slowly progressive, predominantly sensory, axonal polyneuropathy, with no aetiology being identified despite extensive investigations. We studied the potential role of the metabolic syndrome, neurotoxic 1-deoxysphingolipids (1-deoxySLs), microangiopathy and inflammation in sural nerve biopsies. Methods We included 30 CIAP-patients, 28 with diabetic distal symmetrical polyneuropathy (DSPN) and 31 healthy controls. We assessed standardised scales, tested for the metabolic syndrome, measured 1-deoxySLs in plasma, performed electroneurography and studied 17 sural nerve biopsies (10 CIAP; 7 DSPN). Results One third of the CIAP-patients had a metabolic syndrome, significantly less frequent than DSPN-patients (89%). Although the metabolic syndrome was not significantly more prevalent in CIAP compared to healthy controls, hypercholesterolemia did occur significantly more frequent. 1-deoxySLs were significantly and equally elevated in both patient groups compared to healthy controls. Mean basal lamina thickness of small endoneurial vessels and the number of CD68- or CD8-positive cells in biopsies of CIAP- and DSPN-patients did not differ significantly. However, the number of leucocyte-common-antigen positive cells was significantly increased in CIAP. Conclusions A non-significant trend towards a higher occurrence of the metabolic syndrome in CIAP-patients compared to healthy controls was found. 1-deoxySLs were significantly increased in plasma of CIAP-patients. Microangiopathy and an inflammatory component were present in CIAP-biopsies. PMID:28114358

  14. Lubiprostone for chronic idiopathic constipation and irritable bowel syndrome with constipation.

    Science.gov (United States)

    Saad, Richard; Chey, William D

    2008-08-01

    Lubiprostone, a locally acting highly selective type-2 chloride channel activator, has been US FDA approved since January 2006 for the treatment of adults with chronic idiopathic constipation and FDA approved since April 2008 for the treatment of woman aged 18 years or older suffering from irritable bowel syndrome (IBS) with constipation. Through activation of the type-2 chloride channels located on the luminal side of intestinal epithelial cells, it promotes fluid secretion, increasing the liquid content of stool and accelerating small bowel as well as colonic transit. Lubiprostone has demonstrated efficacy with respect to increasing weekly spontaneous bowel movements and improving stool consistency, straining and constipation severity, both in short- and long-term studies. It has also demonstrated efficacy in the treatment of IBS with constipation, with beneficial effects on global symptoms, abdominal pain, constipation-related symptoms and overall quality of life. There is no evidence of a rebound in constipation or IBS symptoms following cessation of lubiprostone. In general, lubiprostone is well tolerated, with the most common side effects including nausea, headache and diarrhea.

  15. 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.

  16. Chronic idiopathic intestinal pseudo-obstruction: the need for a multidisciplinary approach to management.

    Science.gov (United States)

    Silk, D B A

    2004-08-01

    At the outset of the research programme into irritable bowel syndrome (IBS) it was perceived that there was a need to develop a symptom-based classification for the patients. Four groups of patients were identified, those with spastic colon syndrome, diarrhoea-predominant spastic colon syndrome, functional diarrhoea and midgut dysmotility. While working with outpatients with IBS it was noted how some of them had suffered symptoms for many years; specifically, a group of patients satisfying the criteria for midgut dysmotility had also suffered from particularly severe and intractable intestinal symptoms. These patients underwent 24 h ambulatory studies of small intestinal motility and the majority were found to have manometric features of chronic idiopathic intestinal pseudo-obstruction (CIIP). To characterise the cause, laparoscopic full-thickness small intestine and colonic biopsies have been obtained in forty-five of the latter group of patients. Of these patients 58% have been found to have complete or partial deficiency of alpha-actin epitope staining in the inner circular layer of small intestinal smooth muscle. This deficiency is believed to represent an important biomarker rather than the cause of CIIP, since alpha-actin epitope deficiency has been observed in association with enteric neuropathy and myopathies. In relation to the management of CIIP patients, a multidisciplinary model is proposed incorporating management of co-morbid psychological and psychiatric pathology, abdominal and musculoskeletal pain, fatigue, urological symptoms and nutrition. A six-stage nutritional management plan for these patients is presented.

  17. Isolated idiopathic chronic pancreatitis associated with a compound heterozygosity for two mutations of the CFTR gene.

    Science.gov (United States)

    Reboul, Marie-Pierre; Laharie, David; Amouretti, Michel; Lacombe, Didier; Iron, Albert

    2003-01-01

    We report the case of a patient suffering from idiopathic chronic pancreatitis (ICP) and compound heterozygous for mutations G542X and S1235R of the cystic fibrosis transmembrane regulator (CFTR) gene. The patient had normal sweat test and no other clinical sign usually linked with a typical or moderate pathology (bronchiectasis, nasal polyposis, congenital absence of the vas deferens) of the CFTR gene. G542X is a severe mutation, which is usually found in classical cystic fibrosis when associated with other severe mutations. S1235R is a quite rare abnormality recently reported as being potentially pathogenic when combined in trans with a second CF mutation. Our case is quite similar to the only other six patients in the literature in whom only the pancreas is affected and who bear a rare mutation with moderate effect. The history and the clinical features of our patient indicate an unambiguous isolated ICP in which the presence of the S1235R mutation--in trans with regard to G542X--is likely responsible for the ICP phenotype. This case could throw light on some of the as yet poorly known abnormalities of the CFTR gene in the ICP phenotype.

  18. Role of cytotoxin associated protein A in the pathogenesis of idiopathic thrombocytopenic purpura%幽门螺杆菌细胞毒素相关蛋白A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)

    雷瑚仪; 赵谢兰

    2012-01-01

    Objective Helicobacter Pylori (Hp) infection is now considered one of the causes of idiopathic thrombocytope-nic purpura (ITP) . This study aimed to explore the mechanism of Hp infection causing ITP by investigating the relation of cytotoxin associated protein A of Hp with the PA-IgG level and PLT count in ITP patients. Methods We detected Hp using the rapid urease test (RUT) and 14C urea breath test (14C UBT). We divided 60 patients with ITP into three groups, Hp- , Hp+ cagA- , and Hp+ cagA+ , determined the PLT counts and levels of cagA and PA-IgG before and after anti-Hp therapy, and compared them among the three groups. Results The PA-IgG level was significantly higher ( P 0. 05 ) . Compared with the baseline, the PA-IgG level was significantly decreased and PLT count remarkably increased in the Hp + cagA + group ( P 0. 05). Conclusion Helicobacter pylori increases the PA-IgG level and reduces the PLT count in ITP patients, which may be associated with cagA and involved the pathogenesis of ITP.%目的 目前,认为幽门螺杆菌( helicobacter pylori,Hp)感染是引起特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的病因之一.研究幽门螺杆菌细胞毒素相关基因蛋白A(cytotoxin-associated gene protein,cagA)与特发性血小板减少性紫癜的血小板相关IgG抗体(platelet associated IgG,PA-IgG)及血小板数量变化的关系有助于探索Hp感染引起ITP的机制.文中通过检测ITP患者血清中cagA、PA-IgG和PLT数量,分析Hp感染与ITP发生的关系. 方法 快速尿素酶试验(rapid urease test,RUT)及14C尿素呼气试验(UBT)检测Hp.60例ITP患者分为Hp-、Hp+ cagA -及Hp+ cagA+3组,酶联免疫法测定外周血cagA、PA-IgG,用血细胞仪测定血小板计数,并比较抗Hp治疗前后的变化. 结果 Hp+ cagA+组的PA-IgG水平与Hp-和Hp+ cagA -组比较明显升高(P<0.05),血小板计数与Hp-和Hp+ cagA -组比较明显降低(P<0.05).Hp+ cagA -组的PA-IgG水平及血小板计数

  19. 升血小板胶囊联合激素治疗特发性血小板减少性紫癜疗效观察%Clinical effect of platelet increasing capsules combined with prednisone on treatment of idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    梁利杰; 游晓英

    2011-01-01

    目的 观察升血小板胶囊联合激素治疗特发性血小板减少性紫癜(ITP)的临床疗效.方法 选择临床及实验室确诊的ITP患者,将之分为3组:单用泼尼松组、单用升血小板胶囊组、泼尼松联用升血小板胶囊组.结果 泼尼松组有效率为88.23%,升血小板胶囊组有效率为80.00%,泼尼松和升血小板胶囊联用组有效率为94.44%.结论 治疗特发性血小板减少性紫癜,升血小板胶囊配合激素更能提高疗效,减少不良反应.%Objective The purpose of this study is to observe the clinical effect of platelet Increasing capsules (Traditional Chinese Medicine) combined with prednisone on the treatment of idiopathic thrombocytopenic purpura(ITP).Methods Patients were diagnosed by ITP based on their clinical and laboratory examinations.The patients with ITP were then divided into three groups: prednisone group, platelet increasing capsule group and platelet increasing capsule combined with prednisone group.Results The efficiency rate is 88.23% in patients treated with prednisone, 80.00% in patients treated with platelet increasing capsules, and 94.44% in patients treated with both.Conclusions The platelet increasing capsules combined with prednisone greatly increased plateletan improv clinical symptoms, and reduce side effect and toxicity of prednisoe, provide a better strategy for treating ITP.

  20. 小剂量利妥昔单抗治疗难治性血小板减少性紫癜序贯维持的临床研究%Clinical research of low-dose rituximab therapy and sequential maintenance for patients with refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王吉刚; 周凡; 刘彦琴; 刘景华; 白颖; 徐宪平; 董援利; 孟广晗; 佟丹江

    2016-01-01

    Objective To investigate the efficacy and safety of low-dose rituximab therapy and sequential maintenance for patients with refractory idiopathic thrombocytopenic purpura. Methods Thirty-three patients with refractory idiopathic thrombocytopenic purpura received intravenous rituximab at the dose of 100 mg once a week for 4 consecutive weeks. Complete blood cell count and serum concentrations of immunoglobulin (IgG,IgM and IgA) were monitored regularly. The numbers of CD3+ and CD19+ CD20+ lymphocyte cells were assayed by flow cytometry before and after therapy. Twenty-five patients with responses(complete response and response) were divided into maintained group (12 patients) and control group (13 patients) by random digits table method. The patients in maintained group were treated with rituximab 100 mg every 6 months. The efficacy of maintenance therapy was evaluated through long-term follow-up. Results The complete response(CR) rate, response (R) rate and no response(NR) rate were 48.48%(16/33), 27.27%(9/33) and 24.24% (8/33), respectively. As a result, total effective rate was 75.76% (25/33). There were no significant changes of peripheral blood white blood cell count,hemoglobin,serum immunoglobulin and CD3+lymphocyte counts before and after treatment (P>0.05). However, CD19+ CD20+ cells were almost depleted in patients treated with rituximab: (3.71±2.64)×106/L vs. (279.33±92.78)×106/L, P<0.01. Five patients suffered from allergic response, and 1 patient developed pneumonia and respiratory failure. The relapse rates of maintained group and control group were 1/12 and 4/13, respectively. Conclusions Treatment with low-dose rituximab may be an effective and safe approach in patients with idiopathic thrombocytopenic purpura. Relapse rates can be decreased through maintenance therapy with refractory low-dose rituximab. However, the optimal therapeutic schedule need further investigation.%目的:探讨小剂量利妥昔单抗治疗难治性特发性血小板

  1. The clinical significance of immune-related marker detection in idiopathic thrombocytopenic purpura%特发性血小板减少性紫癜患者免疫相关指标的检测及其临床意义

    Institute of Scientific and Technical Information of China (English)

    陈剑芳; 杨林花; 冯建军; 常丽贤; 刘秀娥; 鹿育晋

    2010-01-01

    Objective To assess the clinical significance of detecting the immune markers in idiopathic thrombocytopenic purpura (ITP). Methods The frequencies of circulating B cells secreting platelet-specific antibody, platelet-specific antibody, the percentage of T lymphocyte subsets, the percentage of reticulated platelet and the level of thrombopoietin in 64 ITP patients and 31 healthy controls were measured with enzyme-linked immunospot assay (ELISPOT),modified monoclonal antibody immunobilization of platelet antigens assay (MAIPA), flow cytometry and sandwich enzyme-linked innnunosorbent assay respectively. Results Compared with the controls[1.3 ± 0. 5/105 peripheral blood mononuclear cell (PBMC), (0.33±0.06,0.41±0.03), (22.08±4.54)% and (8.19±2.46)%], the frequencies of circulating B cells secreting platelet-specific antibody (7.6±4.6/105 PBMC in acute ITP group, 5.3±3.0/105 PBMC in chronic ITP group), platelet-specific antibody (including the anti-GP Ⅱ b/Ⅲa antibody, anti-GP Ⅰ b/X antibody) (0.51 ±0.11, 0.48±0.06 in acute ITP group; 0.49±0.10,0.46±0.09 in chronic ITP group), the percentage of CD8+ T Lymphocyte (27.09±9.86 ) %, the percentage of reticulated platelet in ITP patients[the megakaryocyte cytosis group (24. 85 ± 19. 18)%, the normal megakaryocyte group (23.89±18.90)%]were significantly increased ( all P<0.05).The frequencies of circulating B cells secreting platelet-specific antibody in acute ITP patients were notably increased (P<0.05) compared to the chronic ITP patients. In T lymphocyte subsets, the percentage of CD3+T lymphocyte and CD4+ T lymphocyte and the ratio of CD4+/CD8+ in the patients with ITP[(60.88±14.59)%, (28.41±10.55)%, 1.18±0.59]were notably decreased than those in the healthy controls [(69.89±6.43)%, (35.38±5.05) %, 1.64±0.29, P<0.05]. There was no apparent difference of the level of thrombopoietin between ITP patients with megakaryocyte cytosis (72. 09 ± 41.64 ) and health controls (75.37± 26. 32, P > 0

  2. A controlled investigation of the cause of chronic idiopathic axonal polyneuropathy.

    Science.gov (United States)

    Hughes, R A C; Umapathi, T; Gray, I A; Gregson, N A; Noori, M; Pannala, A S; Proteggente, A; Swan, A V

    2004-08-01

    To investigate the aetiology of chronic idiopathic axonal polyneuropathy (CIAP), 50 consecutive patients were compared with 50 control subjects from the same region. There were 22 patients with painful neuropathy and 28 without pain, 26 with sensory neuropathy and 24 with sensory and motor neuropathy. The typical picture was a gradually progressive sensory or sensory and motor neuropathy. It caused mild or sometimes moderate disability, and reduced the quality of life. There was no evidence that alcohol, venous insufficiency, arterial disease or antibodies to peripheral nerve antigens played a significant part. There was a possible history of peripheral neuropathy in the first or second-degree relatives of six patients and no controls (P = 0.01), and claw toes were present in 12 patients and four controls (P = 0.03). Thirty-two per cent of the patients and 14% of the controls had impaired glucose tolerance or fasting hyperglycaemia but, after adjusting for age and sex, the difference was not significant (P = 0.45), even in the painful neuropathy subgroup. The mean (SD) fasting insulin concentrations were significantly (P = 0.01) higher in the patients [75.9 (44.4) mmol/l] than the controls [47.3 (37.9) mmol/l], and the mean was higher still in the painful neuropathy subgroup [92.2 (37.1) mmol/l] (P environmental toxin exposure and hypertriglyceridaemia, but not glucose intolerance or alcohol overuse as significant risk factors that deserve further investigation as possible causes of CIAP.

  3. [An autopsy case of atypical Friedreich's ataxia with chronic idiopathic intestinal pseudo-obstruction].

    Science.gov (United States)

    Nagata, T; Aoki, M; Hasegawa, T; Shiga, Y; Hayashi, T; Higuchi, J; Abe, K; Tanno, T; Konno, H; Itoyama, Y

    2001-07-01

    We report a 58-year-old man with slowly progressive muscle atrophy and weakness in the four extremities, accompanying cerebellar ataxia and sensory impairment of all modalities. He was a product of consanguineous marriage. His neurological manifestations began in childhood. He was admitted to our hospital because of marked abdominal distension and pretibial edema with hypoalbuminemia and hyperlipidemia. Neuroimaging studies showed marked atrophy of the cerebellum and spinal cord. Nerve conduction studies presented with slowing and sural nerve biopsy revealed demyelination with onion-bulbs. Abdominal distension was interpreted to be caused by chronic idiopathic intestinal pseudo-obstruction (CIIP), leading to protein-losing gastroenteropathy and hypalbuminemia caused by the CIIP. He died of DIC by myelodysplasic syndrome and DIC, two years later. Postmortem study demonstrated with severe loss of anterior horn cells and gliosis in the spinal cord. The Clarke's column was also affected. There was symmetrical degeneration in the dorsal column and corticospinal tracts. The cerebellum showed atrophy of molecular layer, prominent loss of Purkinje's cells and sparse granular cell layer, but no obvious change in the dentate nucleus. Neuronal loss in the dorsal root ganglia was remarkable. There were no alternations in the cerebral cortex, striatum, thalamus, subthalamic nucleus, and pontine nucleus, except for mild changes in substantia nigra and inferior olivary nucleus. This case was clinically suspected either of variant of Friedreich's ataxia or an early onset ataxia associated with hypoalbuminemia (EOAHA), although marked autonomic dysfunction was atypical. But the postmortem study, demonstrated with marked neuronal loss in anterior horn cells and cerebellan cortex and rather suggested an independent category of this case.

  4. 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 = ANA(-) 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 ANA(-) 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.

  5. 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.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  7. Fatal hepatic failure associated with graft rejection following reduced-intensity stem-cell transplantation for chronic idiopathic myelofibrosis (CIMF).

    Science.gov (United States)

    Miyakoshi, Shigesaburo; Kami, Masahiro; Kishi, Yukiko; Murashige, Naoko; Yuji, Koichiro; Kusumi, Eiji; Matsumura, Tomoko; Onishi, Yasushi; Kobayashi, Kazuhiko; Kim, Sung-Won; Hamaki, Tamae; Takaue, Yoichi; Taniguchi, Shuichi

    2004-12-01

    A 54-year-old man with chronic idiopathic myelofibrosis (CIMF) underwent RIST. His clinical course had been uneventful until day 60, when splenomegaly reappeared. Hepatic dysfunction developed on day 75. Recipient-type hematopoiesis increased to 51% on day 90. After rapid tapering of cyclosporin, serum levels of AST and ALP normalized in parallel with recovery of complete chimerism on day 134. Yet, jaundice progressed. He died of liver failure on day 176. Postmortem examination revealed neither GVHD nor VOD. Graft rejection following RIST for CIMF may lead to fatal hepatic damage through extramedullary hematopoiesis in the liver or cytokine-mediated immune dysregulations.

  8. NFATc3 and VIP in Idiopathic Pulmonary Fibrosis and Chronic Obstructive Pulmonary Disease

    Science.gov (United States)

    Szema, Anthony M.; Forsyth, Edward; Ying, Benjamin; Hamidi, Sayyed A.; Chen, John J.; Hwang, Sonya; Li, Jonathan C.; Sabatini Dwyer, Debra; Ramiro-Diaz, Juan M.; Giermakowska, Wieslawa; Gonzalez Bosc, Laura V.

    2017-01-01

    Idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD) are both debilitating lung diseases which can lead to hypoxemia and pulmonary hypertension (PH). Nuclear Factor of Activated T-cells (NFAT) is a transcription factor implicated in the etiology of vascular remodeling in hypoxic PH. We have previously shown that mice lacking the ability to generate Vasoactive Intestinal Peptide (VIP) develop spontaneous PH, pulmonary arterial remodeling and lung inflammation. Inhibition of NFAT attenuated PH in these mice suggesting a connection between NFAT and VIP. To test the hypotheses that: 1) VIP inhibits NFAT isoform c3 (NFATc3) activity in pulmonary vascular smooth muscle cells; 2) lung NFATc3 activation is associated with disease severity in IPF and COPD patients, and 3) VIP and NFATc3 expression correlate in lung tissue from IPF and COPD patients. NFAT activity was determined in isolated pulmonary arteries from NFAT-luciferase reporter mice. The % of nuclei with NFAT nuclear accumulation was determined in primary human pulmonary artery smooth muscle cell (PASMC) cultures; in lung airway epithelia and smooth muscle and pulmonary endothelia and smooth muscle from IPF and COPD patients; and in PASMC from mouse lung sections by fluorescence microscopy. Both NFAT and VIP mRNA levels were measured in lungs from IPF and COPD patients. Empirical strategies applied to test hypotheses regarding VIP, NFATc3 expression and activity, and disease type and severity. This study shows a significant negative correlation between NFAT isoform c3 protein expression levels in PASMC, activity of NFATc3 in pulmonary endothelial cells, expression and activity of NFATc3 in bronchial epithelial cells and lung function in IPF patients, supporting the concept that NFATc3 is activated in the early stages of IPF. We further show that there is a significant positive correlation between NFATc3 mRNA expression and VIP RNA expression only in lungs from IPF patients. In

  9. 个体化剂量丙种球蛋白联合地塞米松治疗儿童重症特发性血小板减少性紫癜78例疗效观察%Therapeutic effects of individual-dose intravenous immunoglobulin combined with dexamethasone on 78 children with severe idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    李建厂; 贾秀红; 唐慎华; 朱淑霞; 杨华琴

    2011-01-01

    目的 观察个体化剂量静脉注射用丙种球蛋白(IVIG)联合地塞米松治疗儿童特发性血小板减少性紫癜(ITP)的疗效.方法 重症ITP患儿入院后均给地塞米松及IVIG[400 mg/(kg·d)],3 d后测外周血血小板计数,≥100×109/L停用IVIG,继用地塞米松;若<100×109/L,再继续用原剂量IVIG 2 d.结果 治疗3 d后,78例患儿中,37例外周血血小板计数升至100×109/L以上,余41例继续治疗后,31例升至100×109/L以上.结论 个体化剂量IVIG联合地塞米松是治疗儿童重症ITP的有效办法,可节省一定医疗费用.%Objective To evaluate the effect of individual-dose immunoglobulin combined with dexamethasone on 78 children with severe idiopathic thrombocytopenic purpura (ITP). Methods All the children with ITP were treated with dexamethasone and immunoglobulin (400 mg/kg · d). The platelet counts were detected after 3 day ' s treatment. The patients with platelet ≥ 100 × 109/L continued the treatment of dexamethasone. The others with platelet < 100 × 109/L added the treatment of immunoglobulin (400 mg/kg · d) for 2 days. Results The platelet counts of 37 children were elevated more than 100 ×109/L, and those of 41 children were still lower than 100 × 109/L after 3 day' s treatment. After additional treatment of dexamethasone and immunoglobulin for 2 days, 31 cases got a platelet increase of more than 100 × 109/L. Conclusions Treatment of individual-dose immunoglobulin combined with dexarnethasone is an effective way to treat children with severe ITP. It reduced a lot of medical cost for patients and their family.

  10. Effect of hemodiafiltration with endogenous reinfusion on overt idiopathic chronic inflammation in maintenance hemodialysis patients: a multicenter longitudinal study.

    Science.gov (United States)

    Borrelli, Silvio; Minutolo, Roberto; De Nicola, Luca; De Simone, Emanuele; De Simone, Walter; Zito, Bruno; Guastaferro, Pasquale; Nigro, Filippo; Iulianiello, Giuseppe; Credendino, Olga; Bassi, Antonio; Leone, Luigi; Capuano, Maria; Auricchio, Maria Rita; Conte, Giuseppe

    2014-10-01

    Chronic inflammation is widely diffuse in maintenance hemodialysis (MHD) patients and is associated with poor survival. Hemodiafiltration with endogenous reinfusion (HFR) is a dialysis technique, highly biocompatible, able to adsorb proinflammatory cytokines and to decrease amino acids and antioxidants loss. These features could be helpful in MHD patients affected by idiopathic chronic inflammation, but this issue remains to be elucidated. We performed a multicenter longitudinal study to assess the effect of the switching from bicarbonate HD to HFR in patients with serum C-reactive Protein (CRP) > 5 mg/L coupled with albumin inflammation. At baseline, serum levels of CRP (18.7[7.0-39.4] mg/L) and albumin (3.5[3.3-3.7] g/dL) were significantly correlated (r = -0.49; P = 0.028). The effect on CRP and albumin was almost evident in the first 4 months and remained stable until to eighth month. A strict correlation (R = -0.49; 0.040) between percentage change of CRP (-35%) and albumin (+14%) after 8 months of HFR. These effects were associated with the reduction of IL-6, IL-1β, and TNF-α and the increment of pre-albumin and leptin, whereas the serum levels of Branched Chain Amino Acid (BCAA) remained unchanged. In MHD patients affected by idiopathic chronic inflammation the switching from BHD to HFR is associated with improvement of inflammation. Whether these favorable effects may modify the outcomes of these high-risk patients needs to be confirmed by studies ad hoc.

  11. 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....

  12. Tuberculosis presenting as immune thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Bahadir-Erdogan Beril

    2004-09-01

    Full Text Available Abstract Background Although various hematologic abnormalities are seen in tuberculosis, immune thrombocytopenic purpura is a rare event. Case Presentation We report a case of a 29 year-old male who was presented with immune thrombocytopenia-induced hemoptysis, macroscopic hematuria and generalized petechiae. The patient was found to have clinical, microbiological and radiological evidence of active pulmonary tuberculosis. The immune thrombocytopenic purpura was successfully treated with anti-tuberculous drugs combined with corticosteroids and high dose immune globulin therapy. Conclusion Immune thrombocytopenic purpura can be one of the hematological manifestations of tuberculosis which has a global prevalence with increasing incidence secondary to HIV infection.

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

    Science.gov (United States)

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

    2016-01-01

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

  14. Predict Value of Serum B-Cell Activating Factor on Treatment Effectiveness of Children with Idiopathic Thrombocytopenic Purpura in Diagnosis%血清B淋巴细胞激活因子水平对初诊特发性血小板减少性紫癜患儿疗效的预测价值

    Institute of Scientific and Technical Information of China (English)

    葛小丽; 韩志君; 过毅; 陈洪敏

    2012-01-01

    目的 评价血小板减少性紫癜(ITP)患儿初诊时血清B淋巴细胞激活因子(BAFF)对其疗效的预测价值.方法 采用ELISA法检测初诊37例ITP患儿与37例隐睾及腹股沟疝患儿(对照组)血清BAFF水平,分析ITP患儿血清BAFF水平与其血小板计数、治疗效果的关系.采用受试者工作曲线法(ROC)分析血清BAFF水平对治疗效果的预测价值.结果 ITP患儿血清BAFF水平较对照组增高(P=0.02),且与血小板计数呈负相关(R2=0.39,P<0.01).初诊时血清BAFF水平较低的患儿,其治疗效果相对较好(P<0.01).采用初诊血清BAFF水平预测患儿治疗效果,其曲线下面积(AUC)为0.85(95% CI0.72 ~0.97,P<0.01),当血清BAFF值为850 ng·L-1时,预测敏感性为0.71(95% CI 0.48 ~0.89),特异性为0.88(95% CI 0.62 ~0.98).结论 初诊血清BAFF水平有助于ITP患儿最终治疗效果的预测.%Objective To evaluate the predictive efficiency of serum B - cell activating factor (BAFF) on the treatment effectiveness in children with idiopathic thrombocytopenic purpura (ITP) in diagnosis. Methods Serum BAFF level of 37 children with ITP and controls ( consisted of cryptorchidism and inguinal hernia) were detected by enzyme - linked immunosorbent assay (ELISA). The correlations between serum BAFF and platelet count and treatment effectiveness were analyzed. The predictive efficiency of BAFF on ITP patients' treatment effectiveness was evaluated by receiver operating characteristics (ROC) curve analysis. Results Increased serum BAFF, which negatively correlated with platelet count, was observed in children with ITP. Higher serum BAFF at diagnosis was associated with worse treatment effectiveness, and vice versa. For treatment effectiveness predicting, the area under curve (AUC) for serum BAFF level was 0. 85 (95%CI0.72 -0.97, P<0.01). At the cut-off value of 850 ng·L-1 , the sensitivity and specificity for serum BAFF level were 0.71 (95%CI 0.48 -0. 88) and 0. 88 (95% CI0.62 - 0

  15. Clinical Analysis and Clinical Economic Evaluation of Two Treatments for Children with Idiopathic Thrombocytopenic Purpura%特发性血小板减少性紫癜2种治疗方案比较及经济学评价

    Institute of Scientific and Technical Information of China (English)

    韩金芬; 何志旭; 张昌华

    2012-01-01

    Objective To investigate the clinical efficacy and economic evaluation of 2 treatments for 70 children with idiopathic throm-bocytopenic purpura(ITP). Methods The glucocorticoid (GC) alone treatment and GC combined with intravenous immunoglobulin(IVIG) treatment were retrospectively analyzed, and the economic evaluation was made by cost - effectiveness analysis methods. The data were analyzed by SPSS 14.0 software. Results There was no statistical difference in the side effects and follow - up visits between 2 groups. The cases with platelet counts of (10 -25) x 109 L ' or less than 10 X 109 L"1 and severe bleeds in IVIG plus GC treatment group were more than that in GC treatment group. The days which the platelets reached the normal level from the 1" day to the 7' day, the effective rate and significant effective rate in IVIG plus GC treatment group were higher or better than that in GC treatment group. The result of economic evaluation in GC treatment group was better than that in IVIG plus GC treatment group. Conclusions The cases with platelet counts of (10 -25) x 10' L1 or less than 10 x 109 L1, severe bleeds should be treated with IVIG plus GC treatment, while the others with GC treatment. Many factors should be considered to choose the ways of clinical treatment, including the clinical curative effect and economics.%目的 探讨70例特发性血小板减少性紫癜(ITP)患儿中2种治疗方案的临床疗效及经济学评价.方法 对静脉用丙种球蛋白(IVIG)与地塞米松(GC)静脉联合治疗组及单用GC治疗组进行回顾性分析,并应用成本-效果分析法进行临床经济学评价.采用SPSS 14.0软件进行统计学分析.结果 2组ITP患儿治疗后不良反应、回访情况比较差异均无统计学意义.IVIG+ GC组PLT下降极重度[ (10 ~25) ×109 L-1]、重度(<10×109L-1)、严重出血例数均高于GC组.2组治疗第1-7天PLT上升至正常天数比较,有效率、显效率比较,IVIG+ GC组均优于GC组.应用

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

    Institute of Scientific and Technical Information of China (English)

    路桂云

    2013-01-01

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

  17. [Clinical analysis of 200 cases of idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    García-Stivalet, Lilia Adela; Muñoz-Flores, Aarón; Montiel-Jarquín, Alvaro José; Barragán-Hervella, Rodolfo Gregorio; Bejarano-Huertas, Ruth; García-Carrasco, Mario; López-Colombo, Aurelio

    2014-01-01

    INTRODUCCIÓN: la púrpura trombocitopénica idiopática se caracteriza por la extravasación de sangre en el tejido subcutáneo, membranas, mucosas o piel, que puede generar manifestaciones clínicas de sangrado como lesiones equimóticas, petequias de aparición brusca, epistaxis, gingivorragia y complicaciones graves como hemorragia intracraneal, debido a destrucción plaquetaria mediada por anticuerpos dirigidos contra la superficie de las plaquetas. El objetivo de este informe es presentar las características clínicas de los pacientes con púrpura trombocitopénica idiopática en un hospital de tercer nivel de atención, con la finalidad de tener estadísticas para estudios analíticos posteriores. MÉTODOS: se realizó un estudio descriptivo de 200 pacientes atendidos en el servicio de hematología con diagnóstico de púrpura trombocitopénica idiopática. Se describen sus manifestaciones clínicas, el diagnóstico y el tratamiento médico y quirúrgico empleados.

  18. Autoimmune thyroid disease as a risk factor for angioedema in patients with chronic idiopathic urticaria: a case-control study

    Directory of Open Access Journals (Sweden)

    Ruy Felippe Brito Gonçalves Missaka

    Full Text Available CONTEXT AND OBJECTIVE: An association between chronic idiopathic urticaria (CIU and autoimmune thyroid disease (ATD has been reported. However, there have not been any reports on whether ATD raises the risk of angioedema, which is a more severe clinical presentation of CIU. Thus, the aim of the present study was to evaluate whether the risk of angioedema is increased in patients with CIU and ATD. DESIGN AND SETTING: Case-control study including 115 patients with CIU at a tertiary public institution. METHODS: The patients were evaluated with regard to occurrence of angioedema and presence of ATD, hypothyroidism or hyperthyroidism. RESULTS: Angioedema was detected in 70 patients (60.9%. There were 22 cases (19.1% of ATD, 19 (16.5% of hypothyroidism and nine (7.8% of hyperthyroidism. The risk among patients with ATD was 16.2 times greater than among those without this thyroid abnormality (confidence interval, CI = 2.07-126.86. The odds ratio for hypothyroidism was 4.6 (CI = 1.00-21.54 and, for hyperthyroidism, 3.3 (CI = 0.38-28.36. CONCLUSIONS: Patients with CIU and ATD presented greater risk of angioedema, which reinforces the idea that a relationship exists between this allergic condition and thyroid autoimmunity. This finding could imply that such patients require specifically directed therapy.

  19. 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.

  20. Chronic idiopathic intestinal pseudo-obstruction caused by acquired visceral neuropathy localised in the left colon: report of two cases.

    Science.gov (United States)

    Suzuki, H; Amano, S; Matsumoto, K; Kitagawa, T; Masuda, T

    1987-07-01

    Two cases of chronic idiopathic intestinal pseudo-obstruction (CIIP) are reported. One is a 51-year-old man, and the other is a 47-year-old woman. Both patients presented with severe constipation and barium enema showed a marked dilation of the right colon, and a narrowing in the left colon. Studies done on the motility of the colon and anorectum revealed normal resting pressure profiles of the anorectom, a normal recto-anal reflex, and a normal resting tone of the collapsed colon. Administration of methacholine chloride, however, provoked large, non-propulsive movements in the collapsed colon, which were inhibited by the administration of atropin sulfate. Histologic examination disclosed a marked decrease in neurons and an increase of Schwann cells in the myenteric plexus of the collapsed colon. CIIP due to acquired visceral neuropathy localised in the left colon, was diagnosed as a result of manometric and histologic findings. One case was cured surgically, by a left hemi-colectomy, and the other was cured medically using choline antagonists and laxatives.

  1. A novel locus for syndromic chronic idiopathic intestinal pseudo-obstruction maps to chromosome 8q23-q24.

    Science.gov (United States)

    Deglincerti, Alessia; De Giorgio, Roberto; Cefle, Kivanc; Devoto, Marcella; Pippucci, Tommaso; Castegnaro, Giovanni; Panza, Emanuele; Barbara, Giovanni; Cogliandro, Rosanna F; Mungan, Zeynel; Palanduz, Sukru; Corinaldesi, Roberto; Romeo, Giovanni; Seri, Marco; Stanghellini, Vincenzo

    2007-08-01

    Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare and severe clinical syndrome characterized by symptoms and signs of intestinal occlusion, in the absence of any mechanical obstruction of the gut lumen. In the attempt to identify the genetic basis of CIIP, we analyzed a Turkish pedigree with a high degree of consanguinity in which three siblings presented with a syndromic form of CIIP. All affected family members were characterized by recurrent, self-limiting subocclusive episodes, long-segment Barrett esophagus, and a variety of minor cardiac valve or septal defects. In some patients full-thickness intestinal biopsy samples were obtained and tissues were processed for immunohistochemistry using antibodies to different markers of the intestinal neuromuscular tract. Full-thickness biopsies of the gut wall showed abnormalities of both the neural and muscular components suggesting an underlying intestinal neuro-myopathy. Blood samples were collected for DNA extraction from each available family member and DNAs were genotyped using 382 microsatellites spanning the entire genome with the aim to take advantage of the homozygosity mapping approach. Linkage analysis identified a new syndromic locus on chromosome 8q23-q24 (multipoint LOD score=5.01). Our data strongly support the presence of a new genetic locus associated with CIIP, long-segment Barrett esophagus, and cardiac involvement on chromosome 8.

  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 < 0.05). Linear trends were noted for reductions in mean number of wheals and interference with daily activities and sleep with rupatadine 10 and 20 mg over the 4-week treatment period. The two most frequently reported AEs were somnolence (2.90% for placebo, 4.29% for 5 mg-, 5.41% for 10 mg- and 21.43% for 20 mg-rupatadine-treated group) and headache (4.35% for placebo, 2.86% for 5 mg-, 4.05% for 10 mg- and 4.29% for 20 mg-rupatadine-treated group). These findings suggest that rupatadine 10 and 20 mg is a fast-acting, efficacious and safe treatment for the management of patients with moderate-to-severe CIU. Rupatadine decreased pruritus severity, in a dose- and time-dependent manner.

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

    Science.gov (United States)

    Khorshied, Mervat Mamdooh; El-Ghamrawy, Mona Kamal

    2012-12-10

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Edwin; Owen, Richard [University of Alberta, Department of Radiology and Diagnostic Imaging, Edmonton (Canada); Bruce, Garth [University of Saskatchewan, Department of Pediatrics, Royal University Hospital, Saskatoon (Canada); Wiebe, Sheldon [University of Saskatchewan, Department of Medical Imaging, Royal University Hospital, Saskatoon (Canada)

    2011-11-15

    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.)

  5. 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...

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

    Institute of Scientific and Technical Information of China (English)

    叶华觅; 郝建萍; 赵芳; 王欢; 郭新红

    2013-01-01

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

  7. 利妥昔单抗治疗糖皮质激素无效的特发性血小板减少性紫癜疗效观察%Rituximab treatment for adults with steroid-resistant idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王文; 俞庆宏; 张海燕; 初晓霞; 陈峰; 张春青; 周郁鸿; 侯明

    2008-01-01

    Objective To investigate the efficacy and safety as well as the effects of rituximab on B-lymphocytes and anti-platelet glycoprotein-specific antibodies,in patients with steroid-resistant idiopathic thrombocytopenic purpura(ITP).Methods Twelve steroid-resistant ITP patients,16 to 54 years old,received intravenous rituximab at the dose of 375 mg/m2 once-weekly for 4 weeks.Lab studies included CBC,serum concentrations of IgG,IgM and IgA.CD+3,CD+4,CD+8,CD+19,CD+20 cell numbers were assayed by flow cytometry and anti-platelet glycoprotein-specific antibodies(GP Ⅱ b/Ⅲ a,GP Ⅰ b/Ⅸ)were assayed by monoclonal antibody-specific immobilisation of platelet antigens prior to and following rituximab therapy.Results A complete response(platelet counts ≥100×109/L)was observed in 4 cases,a partial response (platelet counts between 50 and 100×109/L)in 3 cases,a minor response(platelet counts between 30 and 50×109/L)in 2 cases,and non response(platelet counts<30×109/L)in 3 cases.Responses were sustained 0.5 to 12 months(median 5 months).After 4 weeks of rituximab therapy,anti-platelet glycoprotein-specific antibodies(GP Ⅱ b/Ⅲ a,GP Ⅰ b/Ⅸ)disappeared except one NR patient and CD+19/CD+20 cells were almost depleted in all patients(295.0±86.4)×106/L vs(4.1±2.2)×106/L(P<0.01).As expected,the T cell counts,and the serum concentrations of IgG,IgM and IgA were not changed after therapy.No severe side effects were observed.Conclusion Rituximab may be an effective and safe treatment for adults with steroid-resistant ITP.%目的 探讨利妥昔单抗治疗特发性血小板减少性紫癜(ITP)的疗效、安全性及治疗前后B细胞、血小板膜糖蛋白(GP)特异性自身抗体的变化.方法 利妥昔单抗(375 mg/m2,每周1次,连用4周)治疗12例糖皮质激素治疗无效的ITP患者,监测治疗前后的血常规、血清免疫球蛋白定量(IgG、IgM、IgA)、血小板GPⅡb/Ⅲa和(或)GP Ⅰ b/Ⅸ特异性自身抗体、CD+3、CD+4、CD+8、CD+19

  8. 特发性血小板减少性紫癜患者血小板相关抗体的表达及意义%The Expression and Significance of Platelet-associated Antibodies in Patients with Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    李杪

    2014-01-01

    目的:探讨特发性血小板减少性紫癜(ITP)患者患病时PAIg的表达评价其在ITP中的临床意义。方法选取40例ITP患儿作为研究对象,同期门诊体检正常儿童20例为对照组,应用酶联免疫吸附法(ELISA法)测定血小板相关抗体(PAIgA、PAIgG)、采取单克隆抗体特异性俘获血小板抗原(MAIPA法)测定血小板特异性抗体抗血小板膜糖蛋白抗体(GPIIb/IIIa、GPIb/IX),全自动血液分析仪测定血小板计数。结果 PAIgA、PAIgG、GPIIb/IIIa、GPIb/IX、血小板计数对照组分别为(4.22±0.78)ng/107PA、(23.17±3.46)ng/107PA、(0.33±0.01)A值、(0.31±0.01)A值、(137.82±42.37)×109/L,ITP组分别为(45.58±5.32) ng/107PA、(243.34±12.21)ng/107PA、(0.42±0.06)A值、(0.41±0.05)A值、(35.50±21.35)×109/L,两组以上各项指标之间比较均差异有统计学意义(P<0.05);ITP组血小板计数与特异性抗体GPIIb/IIIa、GPIb/IX之间具有负相关,相关系数分别为-0.24(P<0.05)、-0.31(P<0.05)。结论在ITP患儿中血小板相关抗体及特异性抗体均有明显升高,呈现阳性表达,ELISA 法、MAIPA法联合检测有助于鉴别免疫性与非免疫性血小板减少型紫癜,能提高早期诊断率,并且有助于指导临床治疗。%Objective To understand the expression of PAIg in patients with idiopathic thrombocytopenic purpura (ITP) and evalu-ate its clinical significance in ITP. Methods 40 children with ITP were selected as the subjects, and 20 normal children underwent outpatient physical examination during the same period were selected as the control group. Enzyme linked immunosorbent assay (ELISA) was used to detect platelet-associated antibodies (PAIgA, PAIgG), monoclonal antibody immobilization of platelet antigen assay (MAIPA) was adopted to determine platelet specific antibodies against platelet membrane glycoprotein antibody (GPIIb/IIIa, GPIb/IX), and full

  9. 重组人血小板生成素治疗特发性血小板减少性紫癜的临床疗效%Clinical efficacy and safety of rhTPO in the treatment of idiopathic thrombocytopenic purpura: a randomized controlled trial

    Institute of Scientific and Technical Information of China (English)

    张春青; 王琳; 秦平; 陈峰; 陈春燕; 侯明

    2011-01-01

    目的 评价重组人血小板生成素(rhTPO)对糖皮质激素治疗无效的成人特发性血小板减少性紫癜(ITP)的有效性与安全性.方法 选择对糖皮质激素治疗无效的ITP患者74例,分为rhTPO+达那唑组(观察组)37例,达那唑组(对照组)37例.观察组皮下注射rhTPO 1.0μg/kg(300U/kg),1次/d,共14 d,停用rhTPO后再观察14 d.对照组口服达那唑14 d后,如血小板≤20×109/L可皮下注射rhTPO(1.0μg/kg),1次/d,14 d,停用rhTPO后观察14 d.两组在整个试验阶段口服达那唑200mg/次,3次/d.比较两组前14 d血小板计数的最高值和不同时间点血小板计数值的曲线下面积及有效率,比较对照组应用rhTPO前后的血小板计数及其差值.结果 观察组前14 d血小板计数和升高值均显著高于对照组(P<0.01).14 d内血小板计数值的曲线下面积中位数明显高于对照组(P<0.001);观察组rhTPO治疗14d内显效者(血小板计数升高至≥100×109/L)(16例、43.24%)明显高于对照组(3例、8.10%)(P<0.01).观察组良效者(血小板计数升高≥30×109/L)(9例、24.32%)与对照组(10例、27.03%)差异无统计学意义(P>0.01).观察组近期有效observation率(显效+良效)明显高于对照组(P<0.05).对照组14 d血小板升高未达有效的患者给予rhTPO治疗后血小板计数与使用rhTPO前相比明显升高,有效率达到62.50%.两组均无严重不良事件发生.结论 rhTPO治疗糖皮质激素治疗无效的ITP患者安全、有效.%Objective To investigate the efficacy and safety of recombinant human thrombopoietin (rhTPO) in the treatment of adult steroid-resistant idiopathic thrombocytopenic purpura (ITP). Methods 74 patients with steroid-resistant ITP were randomly divided into the observation group and the control group, 37 in each group. Patients in the observation group were treated with 1.0 μg/kg · d of rhTPO, ih(300 U/kg · d)and danazol, 600 mg/d, three times a day, for 14 d. Patients in the control group were

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

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    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.

  11. Once daily levocetirizine for the treatment of allergic rhinitis and chronic idiopathic urticaria

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    E Nettis

    2008-12-01

    Full Text Available E Nettis1, G F Calogiuri2, E Di Leo1, F Cardinale3, L Macchia1, A Ferrannini1, A Vacca1,41Section of Allergology and Clinical Immunology, Department of Internal Medicine and Infectious Diseases; 24th Pneumology Department, Pneumologic Hospital A Galateo, San Cesario di Lecce, Italy; 3Department of Biomedicina dell’Età Evolutiva, Pediatrics Unit “S Maggiore”; 4Department of Internal Medicine and Clinical Oncology, University of Bari Medical School, Bari, ItalyAbstract: Levocetirizine is the pharmacologically active enantiomer of cetirizine. It is a potent histamine H-1 receptor antagonist with anti-inflammatory and antiallergic properties. The review analyses the levocetirizine’s properties in terms of safety and efficacy both in allergic rhinitis and urticarioid syndromes.Keywords: allergic rhinitis, chronic idiopatic urticaria, levocetirizine

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

    Institute of Scientific and Technical Information of China (English)

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

    2009-01-01

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

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

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    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.

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

    Institute of Scientific and Technical Information of China (English)

    王兴兰; 杨恩品

    2012-01-01

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

  15. Moxifloxacin (Avelox Induced Thrombotic Thrombocytopenic Purpura

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    Sikander P. Surana

    2012-01-01

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

  16. Thrombocytopenic purpura: Importance of early diagnosis

    OpenAIRE

    Linklater, David M.; Voth, Arnold

    1996-01-01

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

  17. Thrombotic Thrombocytopenic Purpura Associated with Pneumococcal Sepsis

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    Jeffrey R Schriber

    1993-01-01

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

  18. Thrombopoietin mimetic agents in the management of immune thrombocytopenic purpura.

    Science.gov (United States)

    Newland, Adrian

    2007-10-01

    Thrombopoietin (TPO) is a potent endogenous cytokine and the principal regulator of platelet production. Advances in the understanding of the structure of TPO enabled development of the first generation of thrombopoietic growth factors, recombinant human thrombopoietin (rhTPO) and pegylated human recombinant megakaryocyte growth and development factor (PEG-rHuMGDF). Clinical results showed that these agents were effective in promoting increases in platelet counts in a variety of thrombocytopenic disorders. However, clinical development was halted when studies demonstrated risk for autoantibody formation with cross-reactivity to endogenous TPO. A second generation of thrombopoietic growth factors, including TPO peptide and nonpeptide mimetics and TPO agonist antibodies, utilizing different mechanisms from recombinant growth factors to promote platelet production, are currently in development. The TPO peptide mimetic AMG 531 and the nonpeptide mimetic eltrombopag are in advanced clinical trials and have both resulted in dose-dependent increases in platelets in healthy subjects and in significant increases in platelets in patients with chronic immune thrombocytopenic purpura (ITP). Clinical trials are also being conducted to examine the efficacy and safety of eltrombopag to treat thrombocytopenia in hepatitis C virus (HCV)-infected individuals. These agents appear to be well tolerated and the formation of autoantibodies appears to be limited to first-generation growth factors. Increases in marrow reticulin have been demonstrated with some growth factors, but this appears to be a reversible phenomenon and is not associated with formation of collagen fibrosis. There appears to be no increased incidence of thrombotic events in patients who achieve high platelet counts with growth factor treatments, and although occasional thrombotic events have been reported, their association to the treatment is uncertain. While there is evidence that activation of signaling pathways

  19. Treatment of 36 cases of thrombotic thrombocytopenic purpura

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    Suvajdžić-Vuković Nada D.

    2004-01-01

    Full Text Available Thirty-six patients (pts. with thrombotic thrombocytopenic purpura (TTP were treated between May 1990 and May 2003. There were 31 women and 5 men; the average age was 37 years. Twenty-five cases were idiopathic and 11 secondary (3 infection - related, 5 occurred during pregnancy and 3 were drug - associated. The mean lag period between the first symptoms and the diagnosis was 8.5 days (in 14 pts. £ 5; in 22 > 5. On diagnosis neurological symptoms were present in 31, bleeding in 33, fever in 21 and renal impairment in 27 patients. The mean hemoglobin was 67.5 g/L, the mean platelet count was 10´109/L, and the mean reticulocytosis was 17%. The mean serum LDH was 1457 IU. Treatment included plasma exchange (PE in 24 pts. and only plasma infusions in 12 pts. There were 24 complete responders (20 on PE and 12 deaths (4 on PE; PE significantly improved survival (p<0.01. There were 5 treatment-related complications due to the infection and bleeding, 17 exacerbations and 4 relapses. The mean time delay before the onset of symptoms and the treatment initiation lasted for 9 days suggesting the poor disease recognition; the mean time delay from diagnosis to PE institution was 6 days, indicating postponed PE. The mean treatment duration in all patients was 18 days; the mean number of PE cycles needed for the platelet count stabilization was 9. Good prognostic indicators of survival were: the longer prodromal period (>5 days, the secondary form of TTP and the absence of coma at presentation. The use of PE significantly improved survival. TTP is a severe disorder requiring early recognition and diagnosis in general medical care facilities, which should lead to the timely treatment with PE.

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

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    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.

  1. Cough in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    M.J.G. Van Manen (Mirjam J.G.); S.S. Birring (Surinder S.); C. Vancheri (Carlo); V. Cottin (Vincent); Renzoni, E.A. (Elisabetta A.); Russell, A.-M. (Anne-Marie); M.S. Wijsenbeek (Marlies)

    2016-01-01

    textabstractMany 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,

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

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    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.

  3. Thrombotic thrombocytopenic purpura concomitant with autoimmune thyroiditis

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    Ali Bay

    2011-12-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is characterized by disseminated thrombotic occlusions located in the microcirculation, microangiopathic hemolytic anemia, thrombocytopenia, fever, and renal and neurologic abnormalities. A 14 year old girl admitted to our hospital complaining bruising on her body and prolonged menstrual bleeding. On her physical examination there were very common bruising on four extremities. On the laboratory studies, Hemoglobin was 9 g/dL; Hematocrit, 24%; white blood count 11600/mm3 and thrombocyte count, 9.000/mm3. According to these findings our first diagnose was idiopatic thrombocytopenic purpura so intravenous immunoglobulin was given to patient for two days. Bone marrow aspiration was performed because of persisting thrombocytopenia despite two days IVIG therapy. Increased number of megakaryocytes was seen in bone marrow. Some accompanying symptoms like headache, numbness in per oral region and extremities, difficulty in speaking, and fluctuation in consciousness for short time occurred. The patient was reevaluated; because thrombocytopenia persisted and some neurological symptoms was observed. Due to these findings we thought that TTP was the diagnosed and plasma exchange was started. Increase was seen in platelet count in the second days of treatment. TTP should be considered in children presenting with atypical thrombocytopenia.

  4. Idiopathic scoliosis.

    Science.gov (United States)

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

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

  5. Evidence-based Treatment for a Newly Diagnosed Acute Idiopathic Thrombocytopenic Purpura in Children%一例初诊儿童急性特发性血小板减少性紫癜患者的循证治疗

    Institute of Scientific and Technical Information of China (English)

    吴星; 徐之良

    2010-01-01

    目的 结合1例初诊儿童急性特发性血小板减少性紫癜患者的循证治疗经过,总结评价治疗儿童急性特发性血小板减少性紫癜的最佳证据,为临床实践提供参考.方法 充分评估患者情况后,提出临床问题,从cochrane图书馆(2009年第4期)Medline(PbuMed网站1993年1月至2009年10月)中国知网知识总库上进行检索.检索检索主题词为:ildren acute immune thrombocytopenic purpura, platelet count, intracranial hemorrhage, Corticosteroids ,intravenous immuneglobulin,mega-dose methylprednisolon refractory itp, Rituximaba, splenectomy, human, children 0~8 years, systematic review, meta-analysis, randomized controlled trials.结果 检索出与不同问题相关的随机对照实验30篇,系统评价或Meta分析6篇,通过对检索结果进行分析,为患者制定了合理的治疗方案.经1年随访证实,该方案适合患者.结论 采用循证治疗的方法,为初治的儿童特发性血小板减少性紫癜患者确定合理的治疗目标和治疗方案,可有效提高治疗效果.

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

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    Roussos Charis

    2010-05-01

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

  7. Thrombocytopenic purpura as initial presentation of acute hepatitis A.

    Science.gov (United States)

    Scott, Julius Xavier; Gnananayagam, Ebor Jacob; Gupta, Sanjay; Simon, Anna; Mukhopadhya, Ashish

    2003-01-01

    Extrahepatic immune manifestations are rare in hepatitis A virus infection. We report a 4 1/2-year-old girl who presented with thrombocytopenic purpura as initial manifestation of hepatitis A virus infection. She responded to steroid therapy.

  8. 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

  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.

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

    Science.gov (United States)

    Chamanara, Elham; Raeeskarami, Seyed-Reza

    2016-01-01

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

  11. Treatment Algorithm for Chronic Idiopathic Constipation and Constipation-Predominant Irritable Bowel Syndrome Derived from a Canadian National Survey and Needs Assessment on Choices of Therapeutic Agents

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    Yvonne Tse

    2017-01-01

    Full Text Available Background. Chronic idiopathic constipation (CIC and constipation-predominant irritable bowel syndrome (IBS-C are common functional lower gastrointestinal disorders that impair patients’ quality of life. In a national survey, we aimed to evaluate (1 Canadian physician practice patterns in the utilization of therapeutic agents listed in the new ACG and AGA guidelines; (2 physicians satisfaction with these agents for their CIC and IBS-C patients; and (3 the usefulness of these new guidelines in their clinical practice. Methods. A 9-item questionnaire was sent to 350 Canadian specialists to evaluate their clinical practice for the management of CIC and IBS-C. Results. The response rate to the survey was 16% (n=55. Almost all (96% respondents followed a standard, stepwise approach for management while they believed that only 24% of referring physicians followed the same approach. Respondents found guanylyl cyclase C (GCC agonist most satisfying when treating their patients. Among the 69% of respondents who were aware of published guidelines, only 50% found them helpful in prioritizing treatment choices and 69% of respondents indicated that a treatment algorithm, applicable to Canadian practice, would be valuable. Conclusion. Based on this needs assessment, a treatment algorithm was developed to provide clinical guidance in the management of IBS-C and CIC in Canada.

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

    Science.gov (United States)

    Bech, Per; Lunde, Marianne; Møller, Stine Bjerrum

    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 the anxiety and depression subscales within the neuroticism scale and the extraversion and introversion subscales within the extraversion scale. When measuring states of depression and anxiety, the Melancholia Scale and the Hamilton Anxiety Scale were used. Results. We identified acceptable subscales of anxiety and depression in the Eysenck factor of neuroticism and extraversion versus introversion subscales within the Eysenck factor of extraversion. Focusing on the item of "Does your mood often go up and down?" we showed a statistically significant association with melancholia and anxiety for patients with a positive score on this item. Conclusion. Within the Eysenck factor of neuroticism it is important to differentiate between the anxiety and depression subscales. The clinimetric analysis of the Eysenck factor of extraversion identified valid subscales.

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

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    S. Mešanović

    2014-06-01

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

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

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    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.

  15. Mastitis crónica granulomatosa idiopática: reporte de un caso Idiopathic chronic granulomatous mastitis: a case report

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    Juan Takano

    2010-12-01

    Full Text Available La mastitis cónica granulomatosa idiopática es una enfermedad rara que se presenta en mujeres, preferentemente en edad reproductiva, con características clínicas, mamográficas, ecográficas y citológicas sugestivas de cáncer mamario. Se informa un caso de patología mamaria en paciente mujer de 35 años de edad, portadora de tumoración eritematosa, sensible, localizada en mama izquierda. La lesión fue tratada como "mastitis" con antibióticos y corticoides durante dos semanas. La persistencia de tumoración y sensibilidad, así como los controles mamográficos y ecográficos sugestivos de neoplasia, decidieron la extirpación quirúrgica. El diagnóstico histopatológico fue de mastitis crónica granulomatosa idiopática.Idiopathic chronic granulomatous mastitis is a rare disease in women at the reproductive life with clinic, mammographic, echographic and cytological characteristics suggestive of breast cancer. We report a case of breast pathology in a 35 years old woman who had a sensitiveness and eritematous tumor localized in the left breast. With the diagnosis of "mastitis", she was treated with antibiotics and corticoids for two weeks. Because the lesion and the sensitiveness persist and the mammographic and ecographic controls were suggestive of malignancy, the tumor was surgically removing. The histopathological diagnosis was idiopatic chronic granulomatous mastitis.

  16. Acquired platelet function defect

    Science.gov (United States)

    Acquired qualitative platelet disorders; Acquired disorders of platelet function ... blood clotting. Disorders that can cause problems in platelet function include: Idiopathic thrombocytopenic purpura Chronic myelogenous leukemia Multiple ...

  17. Idiopathic Scoliosis

    OpenAIRE

    2014-01-01

    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...

  18. [Thrombotic thrombocytopenic purpura disclosing cancer: apropos of 2 cases].

    Science.gov (United States)

    Girard, P; Tardy, B; Page, Y; Mosnier, J F; Tardy-Poncet, B; Bertrand, J C

    1995-01-21

    Thrombotic thrombocytopenic purpura (TTP) causes severe haemolytic anaemia, thrombopenia, fever and neurological and renal involvement. Currently five large aetiologic groups have been identified: viral or bacterial infection, drugs, conjunctive tissue diseases, pregnancy and solid tumours. We observed two cases resulting from an adenocarcinoma. In the first case, a 71-year-old man with chronic silicosis, the presenting signs were asthenia, fever, epistaxis with diffus purpura and spontaneous haematomas of the lower limbs. Diagnosis of TTP was based on routine laboratory tests and the patient responded well to fresh frozen plasma. On the 5th day of treatment, haemoglobin level dropped sharply and melana occurred. Upper digestive tract endoscopy revealed a tumoural formation of the antrum-fundic junction and histology examination of the biopsy confirmed the diagnosis of adenocarcinoma. Ten months after gastrectomy the patient was in excellent health with no relapse of the TTP. In the second case, the presenting signs included spontaneous haematomas, rectorrhagia and low grade fever. Microscopic haematuria and renal failure were observed in addition to the biological syndrome of TTP. The patient responded poorly to fresh frozen plasma and packed cell transfusions. Plasma exchange was equally unsuccessful. The disease continued a fulminant course and the diagnosis of adenocarcinoma located in a pulmonary lymph nodes was made at autopsy. These rare cases of TTP caused by cancer emphasize the importance of a thorough aetiological research. Plasma exchange has been shown to be effective but mortality at 1 year approximately 85% in cancer related cases. Early diagnosis and specific anti-cancer therapy might improve prognosis. We report our personal experience with 16 other similar cases.

  19. Juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Krupa H Bhatt

    2014-01-01

    Full Text Available Juvenile Idiopathic Arthritis (JIA is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  20. Refractory thrombotic thrombocytopenic purpura following acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Ebisa Bekele; Bethel Shiferaw; Alexandra Sokolova; Arpan Shah; Phillip Saunders; Alida Podrumar; Javed Iqbal

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13) activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6%after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  1. Idiopathic Scoliosis

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    Jens Ivar Brox

    2014-07-01

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

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

    NARCIS (Netherlands)

    Groot, E.

    2009-01-01

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

  3. Prediction of melatonin efficacy by pretreatment dim light melatonin onset in children with idiopathic chronic sleep onset insomnia.

    Science.gov (United States)

    van der Heijden, Kristiaan B; Smits, Marcel G; van Someren, Eus J W; Boudewijn Gunning, W

    2005-06-01

    Research has shown efficacy of melatonin treatment to advance sleep-wake rhythms in insomnia. In healthy adults, direction and magnitude of the phase shift depends on the timing of administration relative to the phase position of the circadian system. Therefore, in the present study we investigated whether in children with chronic sleep onset insomnia (SOI) efficacy of melatonin treatment in the early evening could be predicted from dim light melatonin onset (DLMO), a phase marker of the circadian system. We combined data of two previously published double blind, randomized, placebo-controlled trials in 110 participants, aged 6-12 years. Sleep was actigraphically estimated, and saliva collected, at baseline and in the third week of a 4-week treatment period with 5 mg melatonin or placebo at 18:00 or 19:00 hours. Primary outcome measures were pre- to post-treatment changes in dim light melatonin onset (DeltaDLMO), sleep onset (DeltaSO), sleep latency (DeltaSL), and total sleep duration (DeltaTSD). Melatonin advanced DLMO with +1:12 h (P melatonin-treated group, but not in the placebo-treated group, pretreatment DLMO was significantly related to DeltaDLMO [F(1, 29) = 7.28, P = 0.012] and DeltaSO [F(1, 25) = 7.72, P = 0.010]. The time interval between treatment administration and pretreatment DLMO (INT) was only significantly related to DeltaSO [F(1,26) = 5.40, P = 0.028]. The results suggest that in children with SOI, the efficacy of early evening melatonin to advance sleep onset and endogenous melatonin onset increases the later the pretreatment DLMO is.

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

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    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.

  5. 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

  6. DNMT3B promoter polymorphism and risk of immune thrombocytopenic purpura in pediatric Egyptians.

    Science.gov (United States)

    Shaheen, Iman A; Abukhalil, Reham E; Ali, Dina K; Afifi, Rasha A

    2012-10-01

    Idiopathic (immune) thrombocytopenic purpura (ITP) is a heterogeneous clinical disorder characterized by immune-mediated platelet destruction. Epigenetic changes in gene expression, including DNA methylation and histone modifications, might contribute to autoimmunity. Polymorphisms of the DNA methyltransferase 3B (DNMT3B) gene may influence DNMT3B activity on DNA methylation and increase the susceptibility to several diseases. The current study investigated the association between a single nucleotide polymorphism (SNP) in the promoter of DNMT3B gene and the risk for ITP in pediatric Egyptians. DNMT3B SNP was genotyped by PCR-restriction fragment length polymorphism in 71 pediatric ITP patients and 82 healthy controls matched for age and sex. The C/C wild genotype was not detected in ITP patients or in the controls. The frequencies of the T/T and C/T genotypes were 93.9 and 6.1% in the controls and 91.5 and 6.1% in ITP patients, respectively. There was no significant difference in either genotypes or allelic distribution between ITP patients and the controls. In conclusion, this polymorphism was almost equally distributed between ITP patients and the controls. These results demonstrated that this SNP may not be used as a stratification marker to predict the susceptibility to childhood ITP in Egypt.

  7. 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.

  8. [Idiopathic interstitial pneumonias in 2016].

    Science.gov (United States)

    Debray, M-P; Borie, R; Danel, C; Khalil, A; Majlath, M; Crestani, B

    2017-02-01

    Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.

  9. 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.

  10. Extrahepatic manifestations of chronic viral hepatitis.

    Science.gov (United States)

    Pyrsopoulos, N T; Reddy, K R

    2001-02-01

    Hepatitis B (HBV) and C (HCV) viruses are well-recognized causes for chronic hepatitis, cirrhosis, and even for hepatocellular carcinoma. Apart from liver disease, these viral infections are known to be associated with a spectrum of extrahepatic manifestations. The prevalence of clinically significant extrahepatic manifestations is relatively low, but it can be associated with significant morbidity and even mortality. An awareness and recognition of these manifestations is of paramount importance in facilitating early diagnosis and in offering treatment. However, treatments are not necessarily effective, and patients may continue with disabling extrahepatic manifestations. Hepatitis B virus has been well recognized as causing a variety of manifestations that include skin rash, arthritis, arthralgia, glomerulonephritis, polyarteritis nodosa, and papular acrodermatitis. More recently, infection with hepatitis C virus has elicited considerable interest for its role in a spectrum of extrahepatic manifestations. Among the best-reported are cryoglobulinemia, glomerulonephritis, high titer of autoantibodies, idiopathic thrombocytopenic purpura, lichen planus, Mooren's corneal ulcer, Sjögren's syndrome, porphyria cutanea tarda, and necrotizing cutaneous vasculitis. The precise pathogenesis of these extrahepatic complications has not been determined, although the majority represent the clinical expression of autoimmune phenomena.

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

    NARCIS (Netherlands)

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

    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

  12. Clinical efficacy of lower dose rituximab for chronic refractory immune thrombocytopenic purpura%小剂量利妥昔单抗治疗复发难治性原发免疫性血小板减少症的临床观察

    Institute of Scientific and Technical Information of China (English)

    李燕; 王晓敏; 毛敏; 张晓燕; 富玲; 艾合买江; 张莲兴

    2012-01-01

    目的 探讨小剂量利妥昔单抗治疗复发难治性原发免疫性血小板减少症(ITP)的疗效及安全性.方法 研究纳入20例复发难治性ITP患者,给予利妥昔单抗100 mg静脉滴注,每周1次,连用4周,动态观察血常规、肝肾功能及凝血功能.采用流式细胞术检测治疗前后CD3+、CD4+、CD8+、CD19+淋巴细胞数.免疫比浊法定量检测治疗前后血清免疫球蛋白(IgG、lgM、IgA)水平.用ELISA方法检测血小板膜糖蛋白抗体.治疗前后各项检测指标比较采用配对t检验.结果 治疗后中位起效时间为18d,PLT达峰值时间为(24±7)d.治疗后PLT[(124±106)×109/L]显著高于治疗前[(13±5)×109/L](P<0.01).11例(55%)患者达完全反应(CR),4例(20%)有效(R),5例(25%)无效(NR).中位疗效持续时间为8(5~23)个月.治疗前后外周血WBC、HGB、血清免疫球蛋白以及CD3+、CD4+、CD8+淋巴细胞数无明显变化,CD19+淋巴细胞数治疗后[(50.53±29.11)×106/L]较治疗前[(125.65±14.12)×106/L]明显下降(P<0.01).3例患者治疗前血小板自身抗体检测阳性,治疗后均为阴性.1例患者在首次输注利妥昔单抗后发生轻微不良反应.结论 小剂量利妥昔单抗是一种治疗复发难治性ITP安全有效的药物,但其最佳用药方案、长期疗效以及不良反应有待临床进一步观察验证.%Objective To investigate the efficacy and safety as well as the effects of lower dose of rituximab on B-lymphocytes,serum immunoglobulin,and platelet glycoprotein-specific antibodies in patients with chronic refractory immune thrombocytopenic purpura (ITP).Methods Twenty chronic refractory ITP patients,median age 47(20 to 60) years old,received intravenous rituximab at the dose of 100mg once weekly for 4 consecutive weeks.Laboratory studies included complete blood cell count,regular monitoring of liver and kidney functions,blood coagulation and serum concentrations of IgG,IgM and IgA. CD3 +,CD4 +,CD8 +,CD19 +,CD20 + cell

  13. Splenectomy during pregnancy: treatment of refractory immune thrombocytopenic purpura

    OpenAIRE

    Mahey, Reeta; Kaur, Simran Deep; Chumber, Sunil; Kriplani, Alka; Bhatla, Neerja

    2013-01-01

    Immune thrombocytopenic purpura (ITP) complicates 1–2/10 000 pregnancies and accounts for 5% of cases of pregnancy-associated thrombocytopenia. Corticosteroids and intravenous immunoglobulin remain the first-line therapy in pregnancy, and a majority of pregnant women respond to this conventional therapy. Other cytotoxic and immunosuppressive agents used for treatment in non-pregnant patients, for example, danazol, cyclophosphamide, vinca alkaloids and azathioprine, are potential teratogens an...

  14. Thrombotic thrombocytopenic purpura in the first trimester of pregnancy

    Directory of Open Access Journals (Sweden)

    Pooja Sikka

    2013-01-01

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

  15. 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

  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. 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

    with a dose regimen of 375 mg/m2 i.v. approximately once weekly for 4 consecutive weeks. Six patients received a fixed dose of 500 mg disregarding their weight supplemented by 100 mg of methylprednisone i.v. or 50-100 mg of Pred given as premedication together with an antihistamine just before infusion...

  18. [Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification].

    Science.gov (United States)

    Barrera, Ana Madeleine; Vargas, Leslie

    2016-12-01

    Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged.

  19. 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.

  20. Cough in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Mirjam J.G. van Manen

    2016-09-01

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

  1. 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

  2. Glucocorticoids in juvenile idiopathic arthritis.

    Science.gov (United States)

    Malattia, Clara; Martini, Alberto

    2014-05-01

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

  3. Idiopathic chondrolysis - diagnostic difficulties

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Scougall, J.

    1984-07-01

    Four cases of idiopathic chondrolysis of the hip in three white girls and one Maori girl are reported. The authors stress the causes why a disease with characteristic clinical and radiographic appearances and normal biochemical findings presents diagnostic difficulties. It is suspected that idiopathic chondrolysis is a metabolic disorder of chondrocytes, triggered by environment circumstances in susceptible individuals. Idiopathic chondrolysis is probably one of the most common causes of coxarthrosis in women.

  4. AMEGAKARYOCYTIC THROMBOCYTOPENIC PURPURA‎: A FIFTEEN YEAR EXPERIENCE

    Directory of Open Access Journals (Sweden)

    M. Bakhshi

    2006-07-01

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

  5. 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

  6. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Meltzer, Eric B; Noble, Paul W

    2008-01-01

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

  7. 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

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

    Directory of Open Access Journals (Sweden)

    Işın Yaprak

    2010-09-01

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

  9. Bleeding tendency and platelet function during treatment with romiplostim in children with severe immune thrombocytopenic purpura.

    Science.gov (United States)

    Suntsova, Elena V; Demina, Irina M; Ignatova, Anastasia A; Ershov, Nikolay M; Trubina, Natalia M; Dobrynina, Juliya; Serkova, Irina V; Supik, Zhanna S; Orekhova, Ekaterina V; Hachatryan, Lili A; Kotskaya, Natalia N; Pshonkin, Aleksey V; Maschan, Aleksey A; Novichkova, Galina A; Panteleev, Mikhail A

    2017-03-07

    It has been suggested that platelet function in chronic immune thrombocytopenic purpura (ITP) may be abnormal. Thrombopoietin mimetics used for treatment can affect it, but the data remain limited. We investigated platelet function of 20 children diagnosed with severe ITP (aged 1-16 years, 12 females and eight males). Platelet functional activity in whole blood was characterized by flow cytometry before and after stimulation with SFLLRN plus collagen-related peptide. Levels of CD42b, PAC1, and CD62P, but not CD61 or annexin V, were significantly increased (P < 0.05) in resting platelets of patients before treatment compared with healthy donors. On average, PAC1 and CD62P in patients after activation were also significantly elevated, although some patients failed to activate integrins. Romiplostim (1-15 μg/kg/week s.c.) was prescribed to seven patients, with clinical improvement in six. Interestingly, one patient had clinical improvement without platelet count increase. Eltrombopag (25-75 mg/day p.o.) was given to four patients, with positive response in one. Others switched to romiplostim, with one stable positive response, one unstable positive response, and one non-responding. Platelet quality improved with romiplostim treatment, and their parameters approached the normal values. Our results suggest that platelets in children with severe ITP are pre-activated and abnormal, but improve with treatment.

  10. Immune thrombocytopenic purpura might be an early hematologic manifestation of undiagnosed human immunodeficiency virus infection.

    Science.gov (United States)

    Lai, Shih-Wei; Lin, Hsien-Feng; Lin, Cheng-Li; Liao, Kuan-Fu

    2017-03-01

    Little research focuses on the association between immune thrombocytopenic purpura and human immunodeficiency virus infection in Taiwan. This study investigated whether immune thrombocytopenic purpura might be an early hematologic manifestation of undiagnosed human immunodeficiency virus infection in Taiwan. We conducted a retrospective population-based cohort study using data of individuals enrolled in Taiwan National Health Insurance Program. There were 5472 subjects aged 1-84 years with a new diagnosis of immune thrombocytopenic purpura as the purpura group since 1998-2010 and 21,887 sex-matched and age-matched, randomly selected subjects without immune thrombocytopenic purpura as the non-purpura group. The incidence of human immunodeficiency virus infection at the end of 2011 was measured in both groups. We used the multivariable Cox proportional hazards regression model to measure the hazard ratio and 95 % confidence interval (CI) for the association between immune thrombocytopenic purpura and human immunodeficiency virus infection. The overall incidence of human immunodeficiency virus infection was 6.47-fold higher in the purpura group than that in the non-purpura group (3.78 vs. 0.58 per 10,000 person-years, 95 % CI 5.83-7.18). After controlling for potential confounding factors, the adjusted HR of human immunodeficiency virus infection was 6.3 (95 % CI 2.58-15.4) for the purpura group, as compared with the non-purpura group. We conclude that individuals with immune thrombocytopenic purpura are 6.47-fold more likely to have human immunodeficiency virus infection than those without immune thrombocytopenic purpura. We suggest not all patients, but only those who have risk factors for human immunodeficiency virus infection should receive testing for undiagnosed human immunodeficiency virus infection when they develop immune thrombocytopenic purpura.

  11. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

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

  12. 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.

  13. The genetic epidemiology of idiopathic scoliosis

    OpenAIRE

    Gorman, Kristen Fay; Julien, Cédric; Moreau, Alain

    2012-01-01

    Purpose Idiopathic scoliosis is a complex developmental syndrome defined by an abnormal structural curvature of the spine. High treatment costs, chronic pain/discomfort, and the need for monitoring at-risk individuals contribute to the global healthcare burden of this musculoskeletal disease. Although many studies have endeavored to identify underlying genes, little progress has been made in understanding the etiopathogenesis. The objective of this comprehensive review was to summarize geneti...

  14. Atherosclerosis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2012-01-01

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

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

    DEFF Research Database (Denmark)

    Lisse, I; Hasselbalch, H; Junker, P

    1991-01-01

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

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

    DEFF Research Database (Denmark)

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

    2009-01-01

    only the negatively phrased items was used to measure the pure anxiolytic effect. The analgesic effect was measured by the Visual Analogue Scale (VAS) for bodily pain. The General Health Questionnaire (GHQ-12) was used to identify minor affective states. RESULTS: In total 171 patients with chronic non......BACKGROUND: We have re-analysed our previous double-blind, placebo-controlled clomipramine study, changing the focus from depression to anxiety both in the response analysis and in the classification of minor affective states. METHODS: The Spielberger State Anxiety Symptom Scale (SSASS) including......, the effect size was below 0.40. LIMITATIONS: No attempt has been made to measure the degree of pure neuropathic pain in the patients. CONCLUSIONS: In patients with chronic non-malignant pain, clomipramine is superior to placebo as regards anxiolytic effect measured by Spielberger State Anxiety Symptom Scale...

  17. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  18. 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.

  19. Idiopathic atrophie blanche.

    Science.gov (United States)

    Amato, Lauretta; Chiarini, Caterina; Berti, Samantha; Massi, Daniela; Fabbri, Paolo

    2006-01-01

    A 41-year-old woman presented with a 3-year history of purpuric lesions followed by superficial, painful ulcers and development of lesions on the lower legs and on the dorsa of the feet, particularly in the summer. The patient was asymptomatic during the winter months. On physical examination she had irregular, scleroatrophic, white-ivory, coalescent lesions on a livedoid basis, with purpuric and, in some lesions, pigmented borders with numerous telangiectatic capillaries. These lesions were localized on the medial sides of the lower legs and on the dorsa of the feet (Figure 1). Laboratory investigations were normal or negative, including complete blood cell count, platelets, coagulation indexes, erythrocyte sedimentation rate, serum immunoglobulins, antinuclear antibodies, anti-double-stranded DNA, anticardiolipin, antiphospholipids, antineutrophilic cytoplasmic antibodies, circulating immunocomplexes, complement fractions (C3, C4), cryoglobulins, rheumatoid factor, and Rose-Waaler reaction. The only laboratory abnormality was an elevated fibrinogen level (472 mg/dL). Doppler velocimetry excluded a chronic venous insufficiency. Thoracic x-ray and abdominal ultrasound were normal. A digital photoplethysmograph revealed functional Raynaud's phenomenon. A biopsy specimen taken from a purpuric lesion showed an atrophic epidermis with parakeratosis and focal spongiosis. An increased number of small-sized vessels were observed within a sclerotic dermis. Most of the vessels in the upper dermis were dilated and showed endothelial swelling; some were occluded due to amorphous hyaline microthrombi (Figure 2). There were fibrinoid deposits around the vessels with thickening of the vessel walls. Extravasated erythrocytes were found throughout the upper and mid-dermis. There was a sparse perivascular lymphocytic infiltrate but no vasculitis. Direct immunofluorescence showed a perivascular microgranular deposit of IgM (+), C3 (++), and fibrinogen/fibrin (+++). On the basis of

  20. Idiopathic pulmonary artery aneurysm.

    Science.gov (United States)

    Kotwica, Tomasz; Szumarska, Joanna; Staniszewska-Marszalek, Edyta; Mazurek, Walentyna; Kosmala, Wojciech

    2009-05-01

    Pulmonary artery aneurysm (PAA) is an uncommon lesion, which may be associated with different etiologies including congenital cardiovascular diseases, systemic vasculitis, connective tissue diseases, infections, and trauma. Idiopathic PAA is sporadically diagnosed by exclusion of concomitant major pathology. We report a case of a 56-year-old female with an idiopathic pulmonary artery dilatation identified fortuitously by echocardiography and confirmed by contrast-enhanced computed tomography. Neither significant pulmonary valve dysfunction nor pulmonary hypertension and other cardiac abnormalities which might contribute to the PAA development were found. Here, we describe echocardiographic and computed tomography findings and review the literature on PAA management.

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

    Science.gov (United States)

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

    2016-10-01

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

  2. Immune thrombocytopenic purpura as sole manifestation in a case of acute hepatitis A.

    Science.gov (United States)

    Tanir, Gönül; Aydemir, Cumhur; Tuygun, Nilden; Kaya, Ozge; Yarali, Neşe

    2005-12-01

    Acute hepatitis due to hepatitis A virus is usually a benign selflimiting disease during childhood. Although many viral infections such as hepatitis B virus, Parvovirus, and Epstein-Barr virus are associated with extrahepatic autoimmune phenomena, such manifestations are rare in patients with acute hepatitis A infection. Immune thrombocytopenia is a benign, self-limiting disease in children, responding well to treatment and generally associated with viral infections. Immune thrombocytopenic purpura is rarely reported as a manifestation of acute hepatitis A. We report a five-year-old boy with immune thrombocytopenic purpura as the sole manifestation of anicteric acute hepatitis A infection. Acute hepatitis A should be included in the differential diagnosis of immune thrombocytopenic purpura.

  3. 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

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

    Directory of Open Access Journals (Sweden)

    Frederico Figueiredo Amâncio

    2014-06-01

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

  5. Takotsubo cardiomyopathy and thrombotic thrombocytopenic purpura preceding a lupus diagnosis: a case report.

    Science.gov (United States)

    Georgiades, F; Demosthenous, S; Braimi, M; Tsitskari, T; Psarelis, S

    2015-11-01

    Takotsubo cardiomyopathy, a rare stress-related cardiomyopathy, has been observed in a few cases secondary to systemic lupus erythematosus (SLE). Herein, we report an unusual case where a postmenopausal woman presented initially with Takotsubo syndrome, later developed thrombotic thrombocytopenic purpura and cerebrovascular events, initially without clinical or laboratory features of SLE. During the course of her illness, she was found to satisfy four of the Systemic Lupus International Collaborating Clinics classification criteria for a SLE diagnosis. This unique presentation of our patient, initially with Takotsubo cardiomyopathy, the development of thrombotic thrombocytopenic purpura and cerebrovascular events preceding the diagnosis of SLE illustrates the importance of clinical observation and follow-up.

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

    Institute of Scientific and Technical Information of China (English)

    李学燕; 于海凤

    2014-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1997-04-14

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

  8. 重组人瘦素对慢性特发性血小板减少性紫癜患者骨髓巨核细胞的影响%Effect of recombinant human leptin on megakaryocyte in patients with chronic idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王晓燕; 蔡艳霞; 田文洪; 赵友恒; 杨仁池

    2014-01-01

    目的:探讨瘦素对 CITP 骨髓巨核细胞的影响。方法以急性巨核细胞白血病细胞株 M07e 和 CITP 骨髓 CD34+细胞经诱导分化的巨核细胞为研究对象。运用 RT-PCR 检测 M07e 细胞瘦素受体 mRNA 表达,运用 Brdu-ELISA 法检测瘦素对 M07e 细胞的增殖作用,运用 Annexin V/ PI 双标流式检测瘦素对 M07e 细胞的凋亡作用。经TPO、SCF 诱导磁珠分选后获得的 CITP 骨髓 CD34+细胞分化为巨核细胞,运用流式测定瘦素对其分化过程中CD41a +、CD61+的表达以及 CD41a +细胞的凋亡是否有影响。结果瘦素受体长型 Ob-RL 和短型 Ob-RS 在 M07e细胞均有 mRNA 表达。瘦素对 M07e 细胞的增殖有促进作用,并呈现一定的剂量依赖性,浓度为5 ng/ ml 即显示出促增殖效应(P =0.037),但浓度在5、10、25 ng/ ml 之间无明显统计学差异,浓度为100 ng/ ml 时其增殖效应最大。在时间依赖效应上,瘦素作用48 h,其细胞增殖明显高于24 h,而作用72 h 其细胞增殖介于两者之间(P =0.000)。瘦素对 M07e 细胞具有抑制凋亡作用(P =0.001),而且在作用72小时后凋亡率最高。瘦素对骨髓 CD34+细胞分化为巨核细胞过程中 CD41a +、CD61+表达的影响,与不加瘦素组相比无明显统计学差异(P >0.05)。对 CD41a +细胞凋亡的影响与不加瘦素组相比亦无明显统计学差异(P >0.05)。结论瘦素通过促进 M07e 细胞的增殖,抑制其凋亡来参与巨核细胞白血病的发生、发展。瘦素在 TPO + SCF 诱导 CITP 骨髓巨核细胞分化中无明显协同作用,对分化产生 CD41a +巨核细胞的凋亡亦无明显作用。%Objective:To explore whether leptin plays a role in megakaryocyte of CITP. Methods:Megakaryocytic leu-kemia cell line M07e,megakaryocyte differentiated from bone marrow CD34 + cells of CITP were used in this study. The mR-NA expression of leptin receptor(ob-R)in M07e was detected by RT-PCR. The proliferative effect of leptin on M07e was investigated with Brdu-ELISA assay and the apoptosis of cells was measured with flow cytometry after Annexin V labeling and PI staining . Bone marrow CD34 + cells were selected by magnetic cell sorting(MACS),and TPO,SCF were used in the expansion system of megakaryocyte progenitor. The influences of leptin on the expression of megakaryocytic specific mono-clonal antibodies CD41a ,CD61 and the apoptosis of megakaryocyte were measured with flow cytometry. Results:There was mRNA expression of ob-RL and ob-RS in M07e. Leptin promoted the proliferation of M07e in a dose and time dependent manner. The effect of proliferation was the most significant at the concentration of 100ng/ ml and after cultured for 48h. Lep-tin inhibited the apoptosis of M07e. Compared with the control group,leptin had no effect on the expression of CD41a , CD61 and the apoptosis of megakaryocyte during the period of megakaryocyte differentiation from bone marrow CD34 + cells of CITP. Conclusion:Leptin may play an important role in human megakaryocytic leukemia by promoting the proliferation and inhibiting the apoptosis of M07e. Leptin had no synergistic effect on megakaryocyte differentiation of ITP induced by TPO and SCF. There was also no significant influence on the apoptosis of CD41a + cells.

  9. The Effects of Thrombopoietin and Interleukin-11 on Bone Marrow Megakaryocytic Progenitors in Patients with Chronic Idiopathic Thrombocytopenic Purpura in Vitro%血小板生成素和白细胞介素-11对慢性特发性血小板减少性紫癜巨核细胞的影响

    Institute of Scientific and Technical Information of China (English)

    廖小梅; 唐雪梅; 邓承祺; 牛挺; 孟文彤

    2001-01-01

    目的探讨重组人血小板生成素(rhTPO)及联用重组人白细胞介素-11(rhIL-11)对慢性特发性血小板减少性紫瘢(CITP)患者骨髓巨核祖细胞增殖和成熟的影响.方法采用血浆凝块法对21例CITP患者骨髓巨核祖细胞进行体外培养,培养体系中加入不同浓度的rhTPO,或同时加rhIL-11两者联用.培养14天后经SZ-21(GPⅢa)单抗和ABC试剂盒染色观察集落生长数,用MCDS-2010型超清晰度骨髓细胞分析系统进行巨核细胞的面积和直径测定.结果CITP组骨髓巨核细胞面积及直径明显低于对照组(P<0.05).rhTPO对CITP患者的巨核细胞集落形成单位(CFU-MK)总集落数、巨核细胞直径与面积均有促进作用,但无浓度依赖性.体外最适浓度为10ng/ml;rhTPO与rhIL-11联用组较单用组的CFU-MK、总集落数及面积、直径均显著增加.结论CITP患者骨髓巨核细胞存在成熟障碍,体外rhTPO单用及与rhIL-11联用均可促进CITP患者巨核祖细胞的增殖与成熟,其联用较单用促进作用更强.

  10. 紫茜胶囊对慢性原发性血小板减少性紫癜患者IL-2、sIL-2R的影响%The Effect on IL - 2 and sIL - 2R of chronic Idiopathic Thrombocytopenic Purpura treated by Zi Qian capsule

    Institute of Scientific and Technical Information of China (English)

    孙凤; 朱云丽

    2007-01-01

    目的:探讨紫茜胶囊对慢性原发性血小板减少性紫癜(CIIp)患者血清IL-2及sIL-2R的影响及作用机理.方法:将64例CITP患者分为紫茜胶囊治疗组和升血小板胶囊对照组各32例,用ELISA方法检测CITp患者治疗前后血清IL-2、sIL-2R的含量变化.结果:CITP患者的IL-2水平明显低于正常对照组,sIL-2R则高于正常对照组,二者经治疗后均有明显改善,但治疗组优于对照组.结论:血清IL-2及sIL-2R水平变化与CITP的发病有关,紫茜胶囊可通过降低IL-2、升高sIL-2R改善CITP患者的细胞免疫功能.

  11. Therapeutic effect of cyclosp-ionrin A on chronic idiopathic thrombocytopenic purpura with diabetes mellitus%环胞素A治疗慢性原发性血小板减少性紫癜合并糖尿病的疗效观察

    Institute of Scientific and Technical Information of China (English)

    耿惠

    2001-01-01

    目的探讨环胞素A(CsA)治疗合并糖尿病(DM)的慢性原发性血小板减少性紫癜(CIIP)的疗效.方法对19例合并DM的CITP给予CsA治疗,用长春新碱(VCR)静脉滴注22例作为对照治疗,比较两组治疗总有效率及T细胞亚群各指标.结果两组治疗总有效率差异有显著性,治疗组优于对照组(P<0.05).CsA组治疗前后T细胞亚群中CD8+及CD4+/CD8+变化显著(分别为P<0.05,P<0.01).而对照组T细胞亚群各指标变化差异无显著性.结论CsA治疗合并DM的CITP较传统治疗有疗效高等特点,可作为合并较严重DM的CTTP治疗有效手段之一.

  12. Juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Prakken, Berent; Albani, Salvatore; Martini, Alberto

    2011-01-01

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

  13. High-dose acetylcysteine in idiopathic pulmonary fibrosis.

    NARCIS (Netherlands)

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

    2005-01-01

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

  14. 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.

  15. 双侧丘脑底核慢性电刺激治疗原发性帕金森病%Treatment of idiopathic Parkinson's disease with chronic electronic deep brain stimulation of bilateral subthalamic nucleus

    Institute of Scientific and Technical Information of China (English)

    骆承章; 曹胜武; 赵春生

    2013-01-01

    Objective To evaluate the surgical approaches and clinical efficacy of electronic deep brain stimulation(DBS) in bilateral subthalamic nucleus (STN) for the treatment of Parkinson's disease.Methods Fourteen patients with idiopathic Parkinson's disease were treated with chronic electronic bilateral subthalamic nucleus deep brain stimulation(STN-DBS).The target was established by cranial magnetic resonance(MRI) and intraoperative microelectrode recording(MER).The patients were followed up for 6 months to 8 years.The outcomes of motor symptoms and medication dose in the 6th month after surgery were analyzed.Results The Unified Parkinson's Disease Rating Scale (UPDRS) motor score was significantly improved in the 6th month after surgery (P<0.05).The UPDRS motor score was improved on an average by 41.8% under on-status and 51.6% under offstatus.Patients were able to gain a better life quality by a long-term stimulation parameters and medication dose adjustment.The medication dose was reduced averagely by 55.6% in the 6th month after surgery.Conclusion The bilateral subthalamic nucleus stimulation is an effective method for the treatment of idiopathic Parkinson's disease.%目的 探讨双侧丘脑底核-脑深部电刺激(STN-DBS)治疗原发性帕金森病(PD)的手术方法及临床疗效.方法 采用头颅核磁共振及术中微电极记录电生理技术明确靶点定位,对14例原发性PD患者行STN-DBS手术.随访6个月-8年,评估手术对患者运动症状的改善及术后6个月患者服药剂量减少情况.结果 术后6个月患者统一用帕金森评分量表(UPDRS),运动检查评分有明显改善(P<0.05):“开”状态下平均改善率为41.8%,“关”状态下平均改善率为51.6%.患者术后通过调整刺激参数及药物剂量,均能获得满意的生活质量.于术后6个月左右评估,患者服用抗帕金森药物剂量平均减少55.6% (P<0.05).结论 STN-DBS术治疗原发性PD疗效确切.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-09-01

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

  17. Trulance Approved for Chronic Constipation

    Science.gov (United States)

    ... news/fullstory_163171.html Trulance Approved for Chronic Constipation Drug designed to stimulate upper gastrointestinal tract To ... U.S. Food and Drug Administration to treat persistent constipation of unknown (idiopathic) cause in adults. Some 42 ...

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

    DEFF Research Database (Denmark)

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

    2014-01-01

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

  19. Refractory thrombotic thrombocytopenic purpura in a 16-year-old girl: successful treatment with bortezomib

    NARCIS (Netherlands)

    Balen, T. van; Schreuder, M.F.; Jong, H. de; Kar, N.C.A.J. van de

    2014-01-01

    We present a case of a 16-year-old girl with autoimmune thrombotic thrombocytopenic purpura (TTP), refractory to plasma exchange and high-dose prednisone. Despite the additional treatment with rituximab, she developed renal and neurological complications with ongoing hemolysis and thrombocytopenia.

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

    NARCIS (Netherlands)

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

    2006-01-01

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

  1. 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. ... PDF Open All Close All Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  2. Idiopathic noncirrhotic portal hypertension: current perspectives

    Directory of Open Access Journals (Sweden)

    Riggio O

    2016-07-01

    Full Text Available Oliviero Riggio,1 Stefania Gioia,1 Ilaria Pentassuglio,1 Valeria Nicoletti,1 Michele Valente,2 Giulia d’Amati2 1Department of Clinical Medicine, Center for the Diagnosis and Treatment of Portal Hypertension, 2Department of Radiological, Oncological, and Pathological Sciences, Sapienza University of Rome, Rome, Italy Abstract: The term idiopathic noncirrhotic portal hypertension (INCPH has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals, splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. Keywords: idiopathic portal hypertension, obliterative portal venopathy, esophageal varices, splenomegaly

  3. 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

  4. Juvenile idiopathic arthritis: a clinical overview

    Energy Technology Data Exchange (ETDEWEB)

    Davidson, J

    2000-02-01

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

  5. 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.

  6. Paraplegia Caused by Diffuse Idiopathic Skeletal Hyperostosis: A Case Report

    Directory of Open Access Journals (Sweden)

    Sevgi Ižkbali Afsar

    2014-02-01

    Full Text Available Diffuse idiopathic skeletal hyperosteosis (DISH, also known as Forestier disease, is an idiopathic chronic disorder characterized by the calcification of the anterior longitudinal ligament of the vertebral column and various extraspinal ligaments. It is usually asymptomatic but the most common symptoms are spinal pain, limited range of spinal motion and dysphagia. Spinal canal stenosis and myelopathia may rarely be seen. We present with the relevant literature a 64-year-old male who developed paraplegia due to thoracal spinal stenosis on a background of DISH.

  7. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    Science.gov (United States)

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

    2015-01-01

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

  8. Understanding the relationship between pain and emotion in idiopathic headaches.

    Science.gov (United States)

    Bussone, G; Grazzi, L

    2013-05-01

    The aim of this paper is to review hypotheses regarding pain mechanisms in headache and relationships between headache pain and the brain's emotional network. There is evidence that chronic pain in idiopathic headaches is, in part, an emotional response induced by alterations in the homeostasis of the interoceptive system--a system that integrates nociceptive information with the emotional network (mediating emotional awareness). These findings suggest that idiopathic headaches are probably due to both an altered pain matrix on the one hand, and an altered affective-cognitive state on the other.

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

    Science.gov (United States)

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

    1998-09-01

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

  10. Idiopathic Normal Pressure Hydrocephalus

    OpenAIRE

    2016-01-01

    Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” ...

  11. Acute Idiopathic Scrotal Edema

    Directory of Open Access Journals (Sweden)

    Micheál Breen

    2013-01-01

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

  12. 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.

  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. Level of IL-16 and Reticulated Platelets Percentage during the Clinical Course of Immune Thrombocytopenic Purpura in Children.

    Science.gov (United States)

    Abd El-Glil, Reem R; Assar, Effat H

    2015-01-01

    Immune thrombocytopenic purpura (ITP) is an immune-mediated acquired disease with transient or persistent decrease of thrombocytes number in the blood. Cytokines play important roles in the immune regulation and are known to be deregulated in autoimmune diseases. This study aimed to investigate serum IL-16 levels in relation to reticulated platelets in children with ITP and platelet count. Twenty six children with ITP (11 with newly diagnosed ITP, 9 with persistent ITP and 6 with chronic ITP) and 12 age-matched healthy children controls were studied. Serum level of IL-16 and reticulated platelets count were assessed by Enzyme Linked Immunosorbent Assay (ELISA) and flow cytometry respectively. Serum IL-16 levels were significantly higher in patients as compared to controls (P platelets were also elevated in patients compared to controls and the increase was significant in newly diagnosed group (P platelets and platelets counts (r = -0.284, P = 0.028, r = 0.274 P = 0.25) respectively. It is concluded that IL-16 may be valuable in predicting the clinical course of pediatrics ITP. Measurement of reticulated platelets may provide significant information about thrombopoietic activity during the clinical course of ITP in children.

  15. Idiopathic fistula-in-ano

    Institute of Scientific and Technical Information of China (English)

    Sherief Shawki; Steven D Wexner

    2011-01-01

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

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

    Science.gov (United States)

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

    2014-06-01

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

  17. 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.

  18. Hypercalcemia in idiopathic myelofibrosis

    DEFF Research Database (Denmark)

    Voss, A; Schmidt, K; Hasselbalch, H;

    1992-01-01

    A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low level...... of circulating type I procollagen propeptide (PICP) and lack of progression of the bone marrow fibrosis during almost 6 months of follow-up point to an in vivo inhibition of type I collagen synthesis by 1,25-dihydroxyvitamin D3....

  19. [Idiopathic progressive subglottic stenosis].

    Science.gov (United States)

    Sittel, C

    2014-07-01

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

  20. Idiopathic granulomatous orchitis.

    Science.gov (United States)

    Roy, Somak; Hooda, Shveta; Parwani, Anil V

    2011-05-15

    Idiopathic granulomatous orchitis is a rare inflammatory process of the testis of unknown etiology. It is characterized by presence of non-specific granulomatous inflammation and admixed multinucleated giant cells. It usually presents as a testicular mass which is highly suspicious of malignancy. Histologically, there is extensive destruction of seminiferous tubules with tubular or interstitial pattern of granulomatous inflammation and prominent collagen fibrosis. Trauma and possible auto-antibodies against sperms have been postulated to be the underlying mechanism. Differential diagnoses include intratubular germ-cell neoplasia, malignant lymphomas, and malakoplakia. Orchiectomy is currently the most appropriate therapy for this condition.

  1. Idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Nitin Hemchandra Dani

    2015-01-01

    Full Text Available Idiopathic gingival fibromatosis (IGF is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth.

  2. Idiopathic gingival fibromatosis

    Science.gov (United States)

    Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep

    2015-01-01

    Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525

  3. Idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne M; Jensen, Rigmor H

    2015-01-01

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

  4. 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

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

    Institute of Scientific and Technical Information of China (English)

    谭李萍

    2015-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Gruber, O.; Cramon, D.Y. von [Day-Care Clinic for Cognitive Neurology, University of Leipzig (Germany); Max Planck Institute of Cognitive Neuroscience, Leipzig (Germany); Wittig, I. [Day-Care Clinic for Cognitive Neurology, University of Leipzig (Germany); Wiggins, C.J. [Max Planck Institute of Cognitive Neuroscience, Leipzig (Germany)

    2000-08-01

    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.)

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

    Directory of Open Access Journals (Sweden)

    Vivek Chowdhry

    2013-01-01

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

  8. Rotavirus-associated immune thrombocytopenic purpura in children: A retrospective study

    OpenAIRE

    Ai, Qi; Yin, Jing; Chen, Sen; Qiao, Lijin; Luo, Na

    2016-01-01

    Certain studies have previously indicated that an association may exist between rotavirus infection and primary immune thrombocytopenic purpura (ITP). The present retrospective study aimed to investigate whether rotavirus may cause ITP in children. Firstly, the incidence of ITP in children with or without rotavirus diarrhea was compared. A 14.58% incident rate was observed in children with rotavirus diarrhea compared with a 7.22% incident rate in children without rotavirus diarrhea. Subsequen...

  9. Juvenile idiopathic arthritis

    Science.gov (United States)

    ... arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... wrists, or knees. It also affects the eyes. Spondyloarthritis of children resembles the disorder in adults and ...

  10. Idiopathic intracranial hypertension in a child with Duchenne muscular dystrophy.

    Science.gov (United States)

    Weig, Spencer G; Zinn, Matthias M; Howard, James F

    2011-12-01

    Duchenne muscular dystrophy is an X-linked, recessively inherited disorder characterized by progressive weakness attributable to the absence of dystrophin expression in muscle. In multiple studies, the chronic administration of corticosteroids slowed the loss of ambulation that develops in mid to late childhood. Corticosteroids, however, frequently produce unacceptable side effects, including Cushingoid appearance and weight gain. Deflazacort, an oxazoline analogue of prednisolone, produces equivalent benefits on muscle with fewer reported Cushingoid side effects. We present a 9-year-old boy with Duchenne muscular dystrophy who developed morbid obesity and subsequent idiopathic intracranial hypertension after 2 years of receiving deflazacort. Although deflazacort is typically thought to produce less obesity than prednisone, severe Cushingoid side effects may occur in some individuals. To our knowledge, this description is the first of idiopathic intracranial hypertension complicating chronic corticosteroid treatment of Duchenne muscular dystrophy.

  11. 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.

  12. Idiopathic Normal Pressure Hydrocephalus

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    Basant R. Nassar BS

    2016-04-01

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

  13. Idiopathic short stature.

    Science.gov (United States)

    Pasquino, A M; Albanese, A; Bozzola, M; Butler, G E; Buzi, F; Cherubini, V; Chiarelli, F; Cavallo, L; Drop, S L; Stanhope, R; Kelnar, C J

    2001-07-01

    Idiopathic short stature (ISS) is a term used to describe the status of children with short stature that cannot be attributed to a specific cause. Many children diagnosed as having ISS have partial GH insensitivity, which can result from disturbances at various points of the GH-IGF-I axis. Several clinical studies on spontaneous growth in ISS showed that adult height was almost in the range of target height. GH treatment led to adult height not significantly higher than the pretreatment predicted adult height in most reports. No metabolic side effects have been observed, even when the dose was higher than in GH deficiency. Manipulation of puberty with gonadotrophin releasing hormone analogues reported by a few authors in a small number of children has shown conflicting results. Long-term psychological benefits of GH therapy for short normal children have not been demonstrated to date.

  14. Idiopathic Bilateral Bloody Tearing

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    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.

  15. [Juvenile idiopathic arthritis].

    Science.gov (United States)

    Herlin, Troels

    2002-08-19

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis. In addition to the clinical characteristics, genetic and biochemical differences suggest that JIA could be regarded as a general term covering various diseases. Complications described are uveitis, temporomandibular joint affection and growth disturbances. The therapeutic strategy should be planned individually according to age, subtype and disease activity and carried out as teamwork with several specialties. Drugs showing significant effectiveness in controlled studies are primarily methotrexate and sulphasalazine. An immunomodulating agent, etanercept, a soluble TNF alpha-receptor fusion protein, has shown a promising effect in severe polyarticular JIA refractory to methotrexate treatment.

  16. 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.

  17. Managing juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

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

    2016-01-01

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

  18. Systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Cimaz, Rolando

    2016-09-01

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

  19. 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.

  20. Idiopathic Retroperitoneal Hematoma

    Directory of Open Access Journals (Sweden)

    Tomoyuki Abe

    2010-09-01

    Full Text Available A 34-year-old female presented with sudden onset of severe abdominal pain in a flank distribution. A large mass was palpable in the right upper quadrant on physical examination. Abdominal contrast-enhanced computed tomography showed a well-defined, right-sided, retroperitoneal cystic lesion located between the abdominal aorta and the inferior vena cava (IVC. The tumor size was 55 × 58 mm, and it compressed the gallbladder and the duodenum. Upper gastrointestinal radiography revealed a stricture of the second portion of the duodenum by the tumor. T2-weighted magnetic resonance imaging showed that the whole part was hyperintense with hypointense rims, but the inner was partially hypointense. Based on the radiological findings, the preoperative differential diagnosis included retroperitoneal teratoma, Schwannoma, abscess, and primary retroperitoneal tumor. On laparotomy, the tumor was located in the right retroperitoneal cavity. Kocher maneuver and medial visceral rotation, which consists of medial reflection of the upper part of right colon and duodenum by incising their lateral peritoneal attachments, were performed. Although a slight adhesion to the IVC was detected, the tumor was removed safely. Thin-section histopathology examination detected neither tumor tissues nor any tissues such as adrenal gland, ovarian tissue, or endometrial implants. The final pathological diagnosis was idiopathic retroperitoneal hematoma; the origin of the bleeding was unclear. The patient was discharged without any complication 5 days after the operation.

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

    Directory of Open Access Journals (Sweden)

    Oliveira Raimundo Antônio Gomes

    2003-01-01

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

  2. 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.

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

    Science.gov (United States)

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

    2016-02-01

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

  4. Immune Thrombocytopenic Purpura and Gastritis by H. pylori Associated With Type 1 Diabetes Mellitus

    Science.gov (United States)

    Correa, Ricardo; Flores-Guevara, Igor; Espinoza Morales, Frank; Mejia, Christian R

    2016-01-01

    We present the 15th case reported worldwide and 3rd case reported in Latin America of immune thrombocytopenic purpura associated with Type 1 diabetes mellitus in Scopus, MEDLINE, and SciELO. An 11-year-old male patient of mixed ethnicity with immune thrombocytopenic purpura, Type 1 diabetes mellitus, and gastritis due to H. pylori presented to the emergency room with petechiae, ecchymosis, and gingival and conjunctival bleeding that had been worsening for the past three months. The patient had a body mass index of 18.85 kg/m2 (P75). A biochemical analysis showed 1×109 platelets/L, increased prothrombin time, increased partial thromboplastin time, and an HbA1C of 7.84% on admission. He was prescribed a single dose of intravenous methylprednisolone 750 mg in 100 mL of NaCl and daily oral 50 mg prednisolone, with intravenous 250 mg tranexamic acid every eight hours. The patient’s glycemic control was continued with the administration of insulin glargine (30 units every 24 hours) and prandial insulin glulisine (five to eight units per meal). Before admission, the patient was on a prescribed treatment of sitagliptin 50 mg and metformin 850 mg, but this was suspended in the emergency room. For the eradication of H. pylori he was prescribed amoxicillin 500 mg every eight hours, oral clarithromycin 335 mg every 12 hours, and IV omeprazole 40 mg. After 15 days, he showed disease resolution and he was discharged to his home with orders to follow-up with pediatrics, hematology, and endocrinology services. The first-line treatment for immune thrombocytopenic purpura patients with active bleeding and a platelet count < 30,000 platelets/μl is the administration of corticosteroids and inmunoglobulin. PMID:27026836

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

    Directory of Open Access Journals (Sweden)

    Ashvin K Tadakamalla

    2011-01-01

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

  6. 601 Autoimmune Thrombocytopenic Purpura Associated with Common Variable Immunodeficiency

    Science.gov (United States)

    Mendieta, Elizabeth; Del Rivero, Leonel Gerardo

    2012-01-01

    Background Common variable immunodeficiency (CVID) is a condition characterized by antibody deficiency, and therefore susceptible to recurrent pyogenic infections, cancer and autoimmune diseases. It is a heterogeneous syndrome in primary immunodeficiencies and clinically the most important is often diagnosed in adulthood. Autoimmunity occurs in 5% of the general population, in patients with CVID the percentage increased to 20 to 48%, cytopenias being the most common cause of autoimmunity in these patients. Autoimmune thrombocytopenic purpura and autoimmune hemolytic anemia are the most common autoimmune consequences, occurring in 5% to 8% of all patients with CVID. Some patients develop these disorders before the diagnosis of CVID. Methods We present the case of a woman of 45 year old, with a history of lower respiratory tract and urinary tract infections in recurrent Pulmonary Tuberculosis. Enter the program short-course treatment strictly supervised for pulmonary tuberculosis with appropriate response. Autoinmune thrombocytopenic purpura refractory to steroids (WWTP) for performing splenectomy. Results Anti DNA antibodies, anti nuclear, anti-protease, C. ANCA/PR3 antimieloperoxidasa, serology for hepatitis B, C, HIV negative. Serum immunoglobulins were as follow: IgG, 158 mg/dL (normal 700 to 1600), IgM, 55 mg/dL (normal 40–230), IgA, 36 mg/dL (normal 70–400), and, IgE, 38.7 IU/mL (normal 0–100) in more than 2 occasions with values below 2 standard deviations. CD4 T lymphocytes (19%) CD4/CD8 ratio (0.54). Conclusions Meets diagnostic criteria for Common Variable Immunodeficiency (CVID) and starting treatment with intravenous immunoglobulin at a dose of 400 mg/kg (every 21 days) with significant clinical improvement and has even managed to integrate into your daily activities. Today, he continues with danazol for WWTP. Therefore, CVID is necessary to consider in the differential diagnosis of autoimmune thrombocytopenic purpura and autoimmune hemolytic anemia

  7. Severe Thrombocytopenic Purpura in a Child with Brucellosis: Case Presentation and Review of the Literature

    Science.gov (United States)

    Perogiannaki, Aikaterini; Chaliasos, Nikolaos

    2017-01-01

    Brucellosis is still endemic and a significant public health problem in many Mediterranean countries, including Greece. It is a multisystemic disease with a broad spectrum of clinical manifestations including hematological disorders, such as anemia, pancytopenia, leucopenia, and thrombocytopenia. Thrombocytopenia is usually moderate and attributed to bone marrow suppression or hypersplenism. Rarely, autoimmune stimulation can cause severe thrombocytopenia with clinically significant hemorrhagic manifestations. We present the case of a girl with severe thrombocytopenic purpura as one of the presenting symptoms of Brucella melitensis infection. Treatment with intravenous immunoglobulin and the appropriate antimicrobial agents promptly resolved the thrombocyte counts. A review of similar published cases is also presented. PMID:28127481

  8. 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.

  9. 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.

  10. Male idiopathic oligoasthenoteratozoospermia.

    Science.gov (United States)

    Cavallini, Giorgio

    2006-03-01

    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.

  11. 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.

  12. Adolescent idiopathic scoliosis: current perspectives

    Directory of Open Access Journals (Sweden)

    Miyanji F

    2014-01-01

    Full Text Available Firoz MiyanjiBritish Columbia Children's Hospital, Vancouver, BC, CanadaAbstract: Adolescent idiopathic scoliosis (AIS remains a common and potentially severe musculoskeletal disorder. Although its etiology is largely unknown, ongoing research endeavors continue to improve our understanding of its potential origin and its natural history. Advances in understanding its true three-dimensional nature have sought to improve classification and management strategies. This review will attempt to highlight and give an overview of the current concepts in adolescent idiopathic scoliosis.Keywords: AIS, current, natural history, management strategies

  13. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or 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...

  14. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    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......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...

  15. 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.

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

    Institute of Scientific and Technical Information of China (English)

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

    2014-01-01

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

  17. Immune Thrombocytopenic Purpura Associated with Hepatitis A Infection in a Five-year Old Boy: A Case Report.

    Science.gov (United States)

    Urganci, N; Kilicaslan, O; Kalyoncu, D; Yilmaz, S

    2014-09-01

    Acute hepatitis A infection is usually a self-limiting disease and mostly asymptomatic in children younger than six years old. Extrahepatic autoimmune manifestations such as immune thrombocytopenic purpura (ITP) have been reported rarely in children with acute hepatitis A infection. We report herein a paediatric case with ITP which is due to hepatitis A virus infection.

  18. 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.

  19. Genetics in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Albers, Heleen Marion

    2015-01-01

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

  20. Diagnosis and classification of juvenile idiopathic arthritis.

    Science.gov (United States)

    Eisenstein, Eli M; Berkun, Yackov

    2014-01-01

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

  1. Australian Paediatric Rheumatology Group standards of care for the management of juvenile idiopathic arthritis.

    Science.gov (United States)

    Munro, Jane; Murray, Kevin; Boros, Christina; Chaitow, Jeffrey; Allen, Roger C; Akikusa, Jonathan; Adib, Navid; Piper, Susan E; Singh-Grewal, Davinder

    2014-09-01

    This standards document outlines accepted standards of management for children, adolescents and young adults with juvenile idiopathic arthritis (JIA) in Australia. This document acknowledges that the chronic inflammatory arthritis conditions (JIA) in childhood are different diseases from inflammatory arthritis in adults and that specific expertise is required in the care of children with arthritis.

  2. 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…

  3. Chronic hypersensitivity pneumonitis

    Science.gov (United States)

    Pereira, Carlos AC; Gimenez, Andréa; Kuranishi, Lilian; Storrer, Karin

    2016-01-01

    Hypersensitivity pneumonitis (HSP) is a common interstitial lung disease resulting from inhalation of a large variety of antigens by susceptible individuals. The disease is best classified as acute and chronic. Chronic HSP can be fibrosing or not. Fibrotic HSP has a large differential diagnosis and has a worse prognosis. The most common etiologies for HSP are reviewed. Diagnostic criteria are proposed for both chronic forms based on exposure, lung auscultation, lung function tests, HRCT findings, bronchoalveolar lavage, and biopsies. Treatment options are limited, but lung transplantation results in greater survival in comparison to idiopathic pulmonary fibrosis. Randomized trials with new antifibrotic agents are necessary. PMID:27703382

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

    Science.gov (United States)

    Espada, Graciela

    2009-10-01

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

  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. Physiotherapy in pauciarticular juvenile idiopathic arthritis. Case study.

    Science.gov (United States)

    Zuk, Beata; Kaczor, Zofia; Zuk-Drążyk, Berenika; Księżopolska-Orłowska, Krystyna

    2014-01-01

    Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood and adolescence. This term encompasses a group of chronic systemic inflammatory diseases of the connective tissue which cause arthritis in patients under 16 years of age lasting at least 6 weeks. The authors presented the characteristic features of physiotherapy based on functional examination results on the basis of two cases of girls with pauciarticular JIA treated according to an established pharmacological regimen. Physiotherapy should be introduced at an early stage of the disease. Kinesiotherapy preceded by history-taking and a functional examination of the patient, has to focus on both primary and secondary joint lesions.

  7. Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis

    Science.gov (United States)

    Park, Tae Joon; Seo, Won Deok; Kim, Sang Young; Cho, Jae Hoon; Kim, Dae Hyun

    2016-01-01

    Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic progressive and diffuse inflammatory fibrosis of the spinal dura mater. Though treatment of IHSP is surgical decompression with steroid therapy, treatment for recurrent IHSP is controversial. Our patient was diagnosed with IHSP based on magnetic resonance imaging (MRI) and underwent laminectomy for decompression following steroid pulse therapy. Despite maintenance of steroid therapy, the patient experienced 3 recurrences. As an alternative immunosuppressant medication, methotrexate was introduced with low-dose steroid. Fortunately, the symptom was resolved, and a decrease of dura thickening was revealed on MRI. We present the case and suggest that methotrexate might be an effective treatment modality for recurrent IHSP.

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

    Science.gov (United States)

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

    2007-08-01

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

  9. Inheritance and genetics in idiopathic scoliosis

    OpenAIRE

    Grauers, Anna

    2015-01-01

    Idiopathic scoliosis is the most common spine deformity, affecting approximately 3% of children and adolescents. Its aetiology is still unknown. However, relatives of individuals with idiopathic scoliosis have a higher risk of developing scoliosis compared to the general population. The aim of this thesis was to improve our understanding of the hereditary and genetic background of idiopathic scoliosis. Self - reported data on scoliosis in twins (n=64,578) i...

  10. Asperger syndrome associated with idiopathic infantile nystagmus--a report of 2 cases.

    Science.gov (United States)

    Kumar, Anil; Sarvananthan, Nagini; Proudlock, Frank; Thomas, Mervyn; Roberts, Eryl; Gottlob, Irene

    2009-01-01

    Asperger syndrome is a severe and chronic developmental disorder. It is closely associated with autism and is grouped under autism spectrum disorder (ASD). Various eye movement abnormalities in AS have been reported in literature such as increased errors and latencies on the antisaccadic task implicating dysfunction of the prefrontal cortex, impairment of the pursuit especially for targets presented in the right visual hemisphere, suggesting disturbance in the left extrastraite cortex. There are no reports in the literature of association between idiopathic infantile nystagmus (IIN) and AS. We report 2 cases of Asperger syndrome associated with idiopathic infantile nystagmus.

  11. Idiopathic myelofibrosis with generalized periostitis in a 4-year-old girl.

    Science.gov (United States)

    Walia, Mandeep; Mehta, Rajesh; Paul, Premila; Saluja, Sumita; Kapoor, Sujala; Sharma, Monika

    2005-05-01

    Idiopathic myelofibrosis, a chronic myeloproliferative disorder of unknown origin, is characterized by splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, teardrop erythrocytes, and myelofibrosis. It is a rare disorder in childhood. The authors describe a 4-year-old girl with features consistent with idiopathic myelofibrosis, who also had generalized solid laminated periosteal reaction involving all long bones. The presence of thrombocytopenia at the onset and lack of leukocytosis were in contrast to the reported features seen in children. Recent case reports describe a relatively indolent course in children. Spontaneous remissions have also been described in pediatric cases. The fulminant course of this patient without any features of malignant transformation was noteworthy in this regard.

  12. Rotavirus-associated immune thrombocytopenic purpura in children: A retrospective study.

    Science.gov (United States)

    Ai, Qi; Yin, Jing; Chen, Sen; Qiao, Lijin; Luo, Na

    2016-10-01

    Certain studies have previously indicated that an association may exist between rotavirus infection and primary immune thrombocytopenic purpura (ITP). The present retrospective study aimed to investigate whether rotavirus may cause ITP in children. Firstly, the incidence of ITP in children with or without rotavirus diarrhea was compared. A 14.58% incident rate was observed in children with rotavirus diarrhea compared with a 7.22% incident rate in children without rotavirus diarrhea. Subsequently, the clinical features of ITP children with or without rotavirus infection were compared. The results indicated that ITP children with rotavirus infection were significantly younger, showed significantly decreased mean platelet volume (MPV) levels and presented a significantly higher frequency of bleeding score of 3 against ITP children without rotavirus infection. In conclusion, these findings suggest that rotavirus serves a causative role in ITP.

  13. Adolescent idiopathic scoliosis: current perspectives

    OpenAIRE

    2014-01-01

    Firoz MiyanjiBritish Columbia Children's Hospital, Vancouver, BC, CanadaAbstract: Adolescent idiopathic scoliosis (AIS) remains a common and potentially severe musculoskeletal disorder. Although its etiology is largely unknown, ongoing research endeavors continue to improve our understanding of its potential origin and its natural history. Advances in understanding its true three-dimensional nature have sought to improve classification and management strategies. This review will attem...

  14. Platelet destruction in autoimmune thrombocytopenic purpura: kinetics and clearance of indium-111-labeled autologous platelets

    Energy Technology Data Exchange (ETDEWEB)

    Stratton, J.R.; Ballem, P.J.; Gernsheimer, T.; Cerqueira, M.; Slichter, S.J.

    1989-05-01

    Using autologous /sup 111/In-labeled platelets, platelet kinetics and the sites of platelet destruction were assessed in 16 normal subjects (13 with and three without spleens), in 17 studies of patients with primary autoimmune thrombocytopenic purpura (AITP), in six studies of patients with secondary AITP, in ten studies of patients with AITP following splenectomy, and in five thrombocytopenic patients with myelodysplastic syndromes. In normal subjects, the spleen accounted for 24 +/- 4% of platelet destruction and the liver for 15 +/- 2%. Untreated patients with primary AITP had increased splenic destruction (40 +/- 14%, p less than 0.001) but not hepatic destruction (13 +/- 5%). Compared with untreated patients, prednisone treated patients did not have significantly different spleen and liver platelet sequestration. Patients with secondary AITP had similar platelet counts, platelet survivals, and increases in splenic destruction of platelets as did patients with primary AITP. In contrast, patients with myelodysplastic syndromes had a normal pattern of platelet destruction. In AITP patients following splenectomy, the five nonresponders all had a marked increase (greater than 45%) in liver destruction compared to five responders (all less than 40%). Among all patients with primary or secondary AITP, there was an inverse relationship between the percent of platelets destroyed in the liver plus spleen and both the platelet count (r = 0.75, p less than 0.001) and the platelet survival (r = 0.86, p less than 0.001). In a stepwise multiple linear regression analysis, total liver plus spleen platelet destruction, the platelet survival and the platelet turnover were all significant independent predictors of the platelet count. Thus platelet destruction is shifted to the spleen in primary and secondary AITP. Failure of splenectomy is associated with a marked elevation in liver destruction.

  15. 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 (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.

  16. Gastaut type idiopathic occipital epilepsy

    Directory of Open Access Journals (Sweden)

    I. V. Volkov

    2015-01-01

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

  17. [Chronic inflammatory demyelinating polyradiculoneuropathy].

    Science.gov (United States)

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

    2010-06-01

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

  18. 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.

  19. 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.

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

    Science.gov (United States)

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

    2014-01-01

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

  1. Etiology and Pathogenesis of Idiopathic Achalasia.

    Science.gov (United States)

    Pressman, Amanda; Behar, Jose

    2017-03-01

    This review examines the etiology and pathogenesis of idiopathic achalasia. This disease is clinically characterized by dysphagia of solids and liquids due to the presence of simultaneous or absent esophageal contractions and impaired or absent relaxation of the lower esophageal sphincter. It includes a review of (a) etiology and pathogenesis of this inflammatory process that damage the ganglion cells of the Auerbach plexus that is limited to the esophagus; (b) genetic abnormalities and polymorphisms associated with this disease that may help explain its heterogeneity expressed by the different motility abnormalities of its phenotypes as well as differences in its clinical progression. These different genetic abnormalities may be responsible for the slow progression of types I or II phenotypes; (c) indirect evidence of viruses present in these patients that may initiate its development; (d) the abnormalities of the muscle layer that may be responsible for the dilation of the body of the esophagus that ultimately causes the sigmoid-like esophagus in the very last phase of this disease. This progression to the end-stage phase tends to occur in about 5% of patients. And, (e) the chronic inflammatory abnormalities in the squamous mucosa that may be the cause of the dysplastic and neoplastic changes that may lead to squamous cell carcinoma whose incidence in this disease is increased. These mucosal abnormalities are usually present in patients with markedly dilated body of the esophagus and severe food stasis.

  2. Clinical outcome measures in juvenile idiopathic arthritis.

    Science.gov (United States)

    Consolaro, Alessandro; Giancane, Gabriella; Schiappapietra, Benedetta; Davì, Sergio; Calandra, Serena; Lanni, Stefano; Ravelli, Angelo

    2016-04-18

    Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality of life. In order to optimize JIA management, the paediatric rheumatologist has begun to regularly use measurements of disease activity developed, validated and endorsed by international paediatric rheumatology professional societies in an effort to monitor the disease course over time and assess the efficacy of therapeutic interventions in JIA patients.A literature review was performed to describe the main outcome measures currently used in JIA patients to determine disease activity status.The Juvenile Disease Activity Score (JADAS), in its different versions (classic JADAS, JADAS-CRP and cJADAS) and the validated definitions of disease activity and response to treatment represent an important tool for the assessment of clinically relevant changes in disease activity, leading more and more to a treat-to-target strategy, based on a tight and thorough control of the patient condition. Moreover, in recent years, increasing attention on the incorporation of patient-reported or parent-reported outcomes (PRCOs), when measuring the health state of patients with paediatric rheumatic diseases has emerged.We think that the care of JIA patients cannot be possible without taking into account clinical outcome measures and, in this regard, further work is required.

  3. Juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Clarke, Sarah L N; Sen, Ethan S; Ramanan, Athimalaipet V

    2016-04-27

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.

  4. Ultrastructural differences between diabetic and idiopathic gastroparesis

    NARCIS (Netherlands)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2016-05-01

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

  6. Analysis of scapular muscle EMG activity in patients with idiopathic neck pain: a systematic review.

    Science.gov (United States)

    Castelein, Birgit; Cools, Ann; Bostyn, Emma; Delemarre, Jolien; Lemahieu, Trees; Cagnie, Barbara

    2015-04-01

    It is proposed that altered scapular muscle function can contribute to abnormal loading of the cervical spine. However, it is not clear if patients with idiopathic neck pain show altered activity of the scapular muscles. The aim of this paper was to systematically review the literature regarding the differences or similarities in scapular muscle activity, measured by electromyography ( = EMG), between patients with chronic idiopathic neck pain compared to pain-free controls. Case-control (neck pain/healthy) studies investigating scapular muscle EMG activity (amplitude, timing and fatigue parameters) were searched in Pubmed and Web of Science. 25 articles were included in the systematic review. During rest and activities below shoulder height, no clear differences in mean Upper Trapezius ( = UT) EMG activity exist between patients with idiopathic neck pain and a healthy control group. During overhead activities, no conclusion for scapular EMG amplitude can be drawn as a large variation of results were reported. Adaptation strategies during overhead tasks are not the same between studies. Only one study investigated timing of the scapular muscles and found a delayed onset and shorter duration of the SA during elevation in patients with idiopathic neck pain. For scapular muscle fatigue, no definite conclusions can be made as a wide variation and conflicting results are reported. Further high quality EMG research on scapular muscles (broader than the UT) is necessary to understand/draw conclusions on how scapular muscles react in the presence of idiopathic neck pain.

  7. Renal Involvement in Idiopathic Inflammatory Myopathies.

    Science.gov (United States)

    Cucchiari, David; Angelini, Claudio

    2017-02-01

    Renal involvement in idiopathic inflammatory myopathies is not as uncommon as was previously thought, as it develops in about one fifth of patients. Clinical presentation includes either acute kidney injury or chronic glomerulonephritis. The former usually develops abruptly during acute phases of rhabdomyolysis: in this case, kidney injury is caused by the toxic effects that myoglobinuria has on the kidney tubules, including cast formation and iron-induced oxidative stress and the development of a third space into the injured muscles. The latter instead has an autoimmune nature, a pleomorphic histological picture, and a more indolent course, with the exception of crescentic glomerulonephritis. Accurate diagnosis and management is crucial for these patients, as timely evaluation and treatment can prevent most of the complications. In the setting of rhabdomyolysis-induced acute kidney injury, the necessity of dialysis can be avoided through aggressive hydration and alkalinization, in order to force diuresis and avoid acidosis and hyperkalemia. In immune-mediated glomerulonephritis, renal biopsy is of undoubtedly value in the diagnostic process and can add prognostic and therapeutic information. In these forms, the development of chronic kidney disease can be prevented or at least delayed by the institution or modification of immunosuppressive treatment. Moreover, the use of drugs that inhibit the renin-angiotensin-aldosterone system and some lifestyle modifications, such as smoking cessation, weight loss, and salt restriction have also value in reducing proteinuria and the progression of kidney damage. In this review, we have summarized the currently available evidence and the different case series in an attempt to provide the readers with the most complete and practical notions that are needed to handle these delicate patients.

  8. Practical management of Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Kishaba, Tomoo

    2015-07-22

    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.

  9. 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.

  10. Effect of the herbal medicine dai-kenchu-to on gastrointestinal motility in patients with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) and chronic idiopathic intestinal pseudo-obstruction (CIIP): report of two cases.

    Science.gov (United States)

    Hirakawa, Hitoshi; Ueno, Shigeru; Matuda, Hiromitu; Hinoki, Tomoya; Kato, Yuko

    2009-04-20

    Dai-kenchu-to (DKT), a traditional Japanese herbal medicine (Kampo medicine), composed of zanthoxylum fruit, ginseng root, dried ginger rhizome and malt sugar, is clinically effective for postoperative ileus and chronic constipation. MMIHS and CIIP are severe motility disorder associated with high morbidity. The aim of this study was to evaluate the effect of DKT on functional intestinal obstruction. DKT was clinically effective for gastrointestinal motility in a case with MMIHS, but not effective in one with CIIP. MMIHS and CIIP are speculated to have different pathogenesis regarding gastrointestinal pseudo-obstruction based upon the effect of this drug.

  11. "Immune Complexes in Juvenile Idiopathic Arthritis"

    Directory of Open Access Journals (Sweden)

    Terry Lynn Moore

    2016-05-01

    Full Text Available Abstract for invited review in Molecular Mechanisms of Immune Complex Pathophysiology thematic issue to be published in Frontiers in Immunology. Immune Complexes(ICin Juvenile Idiopathic Arthritis (JIA Terry L. Moore, MD, FAAP, FACR, MACR Professor of Internal Medicine,Pediatrics, and Molecular Biology and Immunology Director of Adult and Pediatric Rheumatology Saint Louis University School of Medicine Saint Louis, Missouri 631`04,USA Juvenile idiopathic arthritis (JIA reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs and associated complement activation by-products in their sera. ICs have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column,C1q solid phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these IC s have shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF, isotypes of anti-cyclic citrullinated (CCP peptide antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We

  12. Imaging of juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

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

    2006-08-15

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

  13. Epidemiology of adolescent idiopathic scoliosis

    OpenAIRE

    Konieczny, Markus Rafael; Senyurt, Hüsseyin; Krauspe, Rüdiger

    2012-01-01

    Adolescent idiopathic scoliosis is a common disease with an overall prevalence of 0.47–5.2 % in the current literature. The female to male ratio ranges from 1.5:1 to 3:1 and increases substantially with increasing age. In particular, the prevalence of curves with higher Cobb angles is substantially higher in girls than in boys: The female to male ratio rises from 1.4:1 in curves from 10° to 20° up to 7.2:1 in curves >40°. Curve pattern and prevalence of scoliosis is not only influenced by gen...

  14. Qualitative Dermatoglyphics In Idiopathic Epilepsy

    Directory of Open Access Journals (Sweden)

    Ranganath Priya

    2004-01-01

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

  15. 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...

  16. [Chronic inflammatory bowel diseases in cats].

    Science.gov (United States)

    Ghermai, A K

    1989-01-01

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

  17. Idiopathic subglottic stenosis: a familial predisposition.

    Science.gov (United States)

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

    2013-03-01

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

  18. 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…

  19. [The non-specific environmental syndromes MCS (Multiple Chemical Sensitivity), IEI (Idiopathic Environmental Intolerance) and SBS (Sick Building Syndrome)].

    Science.gov (United States)

    Csef, H

    1998-11-30

    This review starts with a clinical description of the most common unspecific environmental diseases, such as Multiple Chemical Sensitivities (MCS), Idiopathic Environmental Intolerances (IEI) and Sick Building Syndrome (SBS). These syndromes are very controversial discussed between scientific medicine and "clinical ecology". In addition, they have fundamental similarities to Chronic Fatigue Syndrome (CFS) and Fibromyalgia. Finally the spectrum of therapeutic approaches is discussed.

  20. Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome.

    Science.gov (United States)

    Watanabe, Ryu; Shirai, Tsuyoshi; Tajima, Yumi; Ohguchi, Hiroto; Onishi, Yasushi; Fujii, Hiroshi; Takasawa, Naruhiko; Ishii, Tomonori; Harigae, Hideo

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice.

  1. Acute pancreatitis-induced thrombotic thrombocytopenic purpura with recurrent acute pancreatitis.

    Science.gov (United States)

    Fujino, Yasuhisa; Inoue, Yoshihiro; Onodera, Makoto; Kikuchi, Satoshi; Sato, Masayuki; Kojika, Masahiro; Sato, Hisaho; Suzuki, Keijiro; Matsumoto, Masanori

    2016-04-01

    Recent successive reports on acute pancreatitis-induced thrombotic thrombocytopenic purpura (TTP) have revealed that TTP-related microvascular damage is an aggravating factor of acute pancreatitis. Here, we report the case of a 26-year-old man diagnosed with acute pancreatitis due to high alcohol consumption. The patient was unconscious as he had taken an overdose of medication, and presented with fever and renal failure due to acute pancreatitis on admission. Although the pancreatitis subsequently improved, the symptoms were still observed; on the next day, he exhibited hemoglobinuria, anemia, and thrombocytopenia. Moreover, general blood examinations indicated the presence of schistocytes and reduced activity of ADAMTS13 (a disintegrin-like metalloproteinase with thrombospondin type 1 motif 13) to 47 %. Thus, the patient was diagnosed with TTP, and plasma exchange was performed. After the development of TTP, the acute pancreatitis recurred, but a severe pathogenesis was prevented by plasma exchange. Thus, ADAMTS13 activity may be useful for predicting a severe pathogenesis of acute pancreatitis. In ADAMTS13-deficient cases, plasma exchange may be an effective technique for preventing aggravation of acute pancreatitis.

  2. 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

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

    Directory of Open Access Journals (Sweden)

    Alex Shimanovsky

    2016-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP is characterized by a decreased platelet count caused by excess destruction of platelets and inadequate platelet production. In many cases the etiology is not known, but viral illness is thought to play a role in the development of some cases of ITP. The current (2011 American Society of Hematology ITP guidelines recommend initial diagnostic studies to include testing for HIV and Hepatitis C. The guidelines suggest that initial treatment consist of observation, therapy with corticosteroids, IVIG or anti D. While most cases respond to the standard therapy such that the steroids may be tapered and the platelet counts remain at a hemostatically safe level. Some patients with ITP are dependent on long term steroid maintenance and the thrombocytopenia persists with the tapering of the steroids. Recent case reports demonstrate that ITP related to cytomegalovirus (CMV can persist in spite of standard therapy and that antiviral therapy maybe indicated. Herein we report a case of a 26-year-old female with persistent ITP that resolved after the delivery of a CMV infected infant and placenta. Furthermore we review the current literature on CMV-associated ITP and propose that the current ITP guidelines be amended to include assessment for CMV as part of the work-up for severe and refractory ITP prior to splenectomy.

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

    Directory of Open Access Journals (Sweden)

    Mariaserena Pioli Di Marco

    2013-12-01

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

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

    Science.gov (United States)

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

    2016-01-01

    Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all). Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03). Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

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

    Directory of Open Access Journals (Sweden)

    Suella Martino

    Full Text Available Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP. Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04. Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all. Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03. Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

  7. Common variable immunodeficiency unmasked by treatment of immune thrombocytopenic purpura with Rituximab

    DEFF Research Database (Denmark)

    Mogensen, Trine H; Jensen, Jens Magnus Bernth; Petersen, Charlotte C;

    2013-01-01

    BACKGROUND: Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs of immunod......BACKGROUND: Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs...... of immunodeficiency, this is not always recognized. Despite novel insight into the molecular immunology of common variable immunodeficiency, several areas of uncertainty remain. In addition, the full spectrum of immunological effects of the B cell depleting anti-CD20 antibody Rituximab has not been fully explored....... To our knowledge this is the first report of development of CVID in a patient with normal immunoglobulin prior to Rituximab treatment. CASE PRESENTATION: Here we describe the highly unusual clinical presentation of a 34-year old Caucasian male with treatment refractory immune thrombocytopenic purpura...

  8. 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.

  9. Idiopathic arterial calcification in childhood

    Energy Technology Data Exchange (ETDEWEB)

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

    2004-08-01

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

  10. Idiopathic Renal Infarction Mimicking Appendicitis

    Science.gov (United States)

    Lisanti, Francesco; Scarano, Enrico

    2017-01-01

    Renal infarction is a rare cause of referral to the emergency department, with very low estimated incidence (0.004%–0.007%). Usually, it manifests in patients aged 60–70 with risk factors for thromboembolism, mostly related to heart disease, atrial fibrillation in particular. We report a case of idiopathic segmental renal infarction in a 38-year-old patient, presenting with acute abdominal pain with no previous known history or risk factors for thromboembolic diseases. Because of its aspecific clinical presentation, this condition can mimic more frequent pathologies including pyelonephritis, nephrolithiasis, or as in our case appendicitis. Here we highlight the extremely ambiguous presentation of renal infarct and the importance for clinicians to be aware of this condition, particularly in patients without clear risk factors, as it usually has a good prognosis after appropriate anticoagulant therapy. PMID:28203466

  11. 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.

  12. Idiopathic Inflammatory Myopathies: An update

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    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

  13. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

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

    2012-07-01

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

  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. Idiopathic inflammatory myopathies: diagnosis, treatment and outcome

    NARCIS (Netherlands)

    van de Vlekkert, J.

    2015-01-01

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

  16. Nonchylous idiopathic pleural effusion in the newborn

    Directory of Open Access Journals (Sweden)

    Geeta Gathwala

    2011-01-01

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

  17. Prevalence and Management of Back Pain in Adolescent Idiopathic Scoliosis Patients: A Retrospective Study

    Directory of Open Access Journals (Sweden)

    Jean Théroux

    2015-01-01

    Full Text Available BACKGROUND: Back pain (BP has often been associated with adolescent idiopathic scoliosis (AIS, which is a three-dimensional deviation of the vertebral column. In adolescents, chronic pain appears to be a predictor of health care utilization and has a negative impact on physical, psychological and family well-being. In this population, BP tends to be persistent and may be a predictor of BP in adulthood.

  18. Idiopathic scoliosis: etiological concepts and hypotheses

    OpenAIRE

    2013-01-01

    Scoliosis is diagnosed as idiopathic in 70 % of structural deformities affecting the spine in children and adolescents, probably reflecting our current misunderstanding of this disease. By definition, a structural scoliosis should be the result of some primary disorder. The goal of this article is to give a comprehensive overview of the currently proposed etiological concepts in idiopathic scoliosis regarding genetics, molecular biology, biomechanics, and neurology, with particular emphasis o...

  19. Unilateral Idiopathic Calcinosis Cutis: A Case Report

    Science.gov (United States)

    Alsaif, Fahad; Abduljabbar, Amr M.

    2017-01-01

    Calcinosis cutis is a rare disorder characterized by the deposition of calcium in the skin and subcutaneous tissue. Unilateral idiopathic calcinosis cutis has only rarely been reported in the literature. Here, we report the case of a 7-year-old healthy girl who presented with multiple asymptomatic hard nodules on the right side of her body. Histopathological, radiological, and extensive blood investigations confirmed the diagnosis of unilateral idiopathic calcinosis cutis. PMID:28203159

  20. Cognitive function in idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne Maria; Fagerlund, Birgitte; Forchhammer, Hysse Birgitte;

    2014-01-01

    OBJECTIVE: To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. DESIGN: Prospective case-control study. SETTING: Neurological department, ophthalmological department and a terti......OBJECTIVE: To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. DESIGN: Prospective case-control study. SETTING: Neurological department, ophthalmological department...

  1. 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....

  2. 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.

  3. Classification of idiopathic interstitial pneumonias using anti–myxovirus resistance-protein 1 autoantibody

    Science.gov (United States)

    Hamano, Yoshimasa; Kida, Hiroshi; Ihara, Shoichi; Murakami, Akihiro; Yanagawa, Masahiro; Ueda, Ken; Honda, Osamu; Tripathi, Lokesh P.; Arai, Toru; Hirose, Masaki; Hamasaki, Toshimitsu; Yano, Yukihiro; Kimura, Tetsuya; Kato, Yasuhiro; Takamatsu, Hyota; Otsuka, Tomoyuki; Minami, Toshiyuki; Hirata, Haruhiko; Inoue, Koji; Nagatomo, Izumi; Takeda, Yoshito; Mori, Masahide; Nishikawa, Hiroyoshi; Mizuguchi, Kenji; Kijima, Takashi; Kitaichi, Masanori; Tomiyama, Noriyuki; Inoue, Yoshikazu; Kumanogoh, Atsushi

    2017-01-01

    Chronic fibrosing idiopathic interstitial pneumonia (IIP) can be divided into two main types: idiopathic pulmonary fibrosis (IPF), a steroid-resistant and progressive disease with a median survival of 2–3 years, and idiopathic non-specific interstitial pneumonia (INSIP), a steroid-sensitive and non-progressive autoimmune disease. Although the clinical courses of these two diseases differ, they may be difficult to distinguish at diagnosis. We performed a comprehensive analysis of serum autoantibodies from patients definitively diagnosed with IPF, INSIP, autoimmune pulmonary alveolar proteinosis, and sarcoidosis. We identified disease-specific autoantibodies and enriched KEGG pathways unique to each disease, and demonstrated that IPF and INSIP are serologically distinct. Furthermore, we discovered a new INSIP-specific autoantibody, anti–myxovirus resistance-1 (MX1) autoantibody. Patients positive for anti-MX1 autoantibody constituted 17.5% of all cases of chronic fibrosing IIPs. Notably, patients rarely simultaneously carried the anti-MX1 autoantibody and the anti–aminoacyl-transfer RNA synthetase autoantibody, which is common in chronic fibrosing IIPs. Because MX1 is one of the most important interferon-inducible anti-viral genes, we have not only identified a new diagnostic autoantibody of INSIP but also obtained new insight into the pathology of INSIP, which may be associated with viral infection and autoimmunity. PMID:28230086

  4. Chronic autoimmune urticaria : Where we stand ?

    Directory of Open Access Journals (Sweden)

    Goh C

    2009-01-01

    Full Text Available It is well-recognized that 30-40% of chronic idiopathic urticaria is autoimmune in nature. Chronic autoimmune urticaria is caused by anti-FcåRI and less frequently, by anti-IgE autoantibodies that lead to mast cell and basophil activation, thereby giving rise to the release of histamine and other proinflammatory mediators. Activation of the classical complement pathway and formation of C5a are important in dermal mast cell activation. C5a is also a neutrophil and eosinophil chemoattractant. Chronic autoimmune urticaria has been found to be associated with autoimmune thyroid disease. The autologous serum skin test is used as a screening test for chronic autoimmune urticaria and has a sensitivity and specificity of about 70 and 80%, respectively. The current gold standard diagnostic test is the basophil histamine release assay. The treatment of chronic autoimmune urticaria, as in chronic idiopathic urticaria, is with H1 antihistamines. Oral corticosteroids may be used during acute flares. Refractory cases have been shown to respond to cyclosporine and other immunomodulators. The prevalence of chronic autoimmune urticaria in Singapore is similar to that reported in Western countries at about 42%. The presence of thyroid autoimmunity appears to be higher than reported, with 22.5% of patients with chronic idiopathic urticaria here, exhibiting presence of thyroid autoantibodies.

  5. Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    高维强; 苏健; 白霞; 王兆钺; 阮长耿

    2004-01-01

    Background Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. In this study we investigated the von Willebrand factor-cleaving protease (vWF-cp) activity deficiency in patients with TTP.Methods The plasma or serum vWF-cp activity was measured using a sensitive enzyme-linked immunosorbent assay (ELISA) by detecting the residual collagen binding activity (R-CBA) of von Willebrand factor (vWF) before and after digestion by vWF-cp. Multimers of vWF in plasma of patients with TTP were also analyzed by SDS-agarose electrophoresis. Moreover, the serum vWF-cp activities were compared between the patients with TTP and those with tumors.Results The coefficient of variation for intra-batch and inter-batch of the assay were 3.60% and 8.35%. The plasma and serum vWF-cp activity in healthy individuals were (78.79±9.17)% (n=30) and (79.47±10.78)% (n=53), respectively, while the plasma vWF-cp activity in 5 patients with TTP was markedly decreased [(21.83±19.98)%, P<0.001]. The unusually large vWF multimers were observed in two plasma samples of the patients with TTP. Although the vWF-cp activities in patients with benign and malignant tumors were also decreased (P<0.03 and P<0.001, respectively), they were relatively high in comparison with that of TTP patients (P<0.001).Conclusion Measurement of the vWF-cp activity using R-CBA is a simple and rapid method for diagnosing TTP. The vWF-cp activity in patients with TTP was markedly lower than those of patients with tumors.

  6. Pituitary Adenoma and Hyperprolactinemia Accompanied by Idiopathic Granulomatous Mastitis

    Directory of Open Access Journals (Sweden)

    Sebahattin Destek

    2017-01-01

    Full Text Available Idiopathic granulomatous mastitis (IGM is a rare chronic inflammatory disease of the breast, and its etiology remains not fully elucidated. IGM is observed more often in patients with autoimmune disease. Hyperprolactinemia is observed during pregnancy, lactation, and a history of oral contraceptive use. A 39-year-old patient with no history of oral contraceptive use presented with complaints such as redness, pain, and swelling in her left breast. Ultrasound and magnetic resonance imaging (MRI revealed a suspicious inflamed mass lesion. Core biopsy was performed to exclude breast cancer and to further diagnose. The breast abscess was drained and steroids were given for treatment. In order to monitor any progression during the three months of treatment, hormone levels were routinely examined. Prolactin level was above the reference range, and pituitary MRI revealed a pituitary prolactinoma. After treatment with prolactin inhibitors, IGM also improved with hyperprolactinemia. This report emphasizes attention to hyperprolactinemia in cases of IGM diagnosis and treatment.

  7. Pituitary Adenoma and Hyperprolactinemia Accompanied by Idiopathic Granulomatous Mastitis

    Science.gov (United States)

    Destek, Sebahattin; Ahioglu, Serkan; Serin, Kursat Rahmi

    2017-01-01

    Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory disease of the breast, and its etiology remains not fully elucidated. IGM is observed more often in patients with autoimmune disease. Hyperprolactinemia is observed during pregnancy, lactation, and a history of oral contraceptive use. A 39-year-old patient with no history of oral contraceptive use presented with complaints such as redness, pain, and swelling in her left breast. Ultrasound and magnetic resonance imaging (MRI) revealed a suspicious inflamed mass lesion. Core biopsy was performed to exclude breast cancer and to further diagnose. The breast abscess was drained and steroids were given for treatment. In order to monitor any progression during the three months of treatment, hormone levels were routinely examined. Prolactin level was above the reference range, and pituitary MRI revealed a pituitary prolactinoma. After treatment with prolactin inhibitors, IGM also improved with hyperprolactinemia. This report emphasizes attention to hyperprolactinemia in cases of IGM diagnosis and treatment. PMID:28321344

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

    Science.gov (United States)

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

    2016-01-01

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

  9. Assessment and Management of Pain in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Jennifer N Stinson

    2012-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is a common chronic childhood illness. Pain is the most common and distressing symptom of JIA. Pain has been found to negatively impact all aspects of functioning, including physical, social, emotional and role functions. Children with arthritis continue to experience clinically significant pain despite adequate doses of disease-modifying antirheumatic drugs and anti-inflammatory agents. The present article reviews the prevalence and nature of pain in JIA, the biopsychosocial factors that contribute to the pain experience, current approaches to assessing pain in this population, and ways of managing both acute and persistent pain using pharmacological, physical and psychological therapies. Finally, new approaches to delivering disease self-management treatment for youth with JIA using the Internet will be outlined.

  10. 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.

  11. Diagnosis and treatment of adolescent idiopathic scoliosis.

    Science.gov (United States)

    Burton, Monique S

    2013-11-01

    Scoliosis is defined as a lateral curvature of the spine greater than 10 degrees on radiography that is typically associated with trunk rotation. The three major types of scoliosis are congenital, idiopathic, and neuromuscular. Idiopathic scoliosis is divided into three subcategories based on the age of onset. Infantile idiopathic scoliosis affects patients younger than 3 years, juvenile idiopathic scoliosis appears in children between 3 and 10 years, and adolescent idiopathic scoliosis (AIS) occurs in skeletally immature patients older than 10 years. AIS is the most common form of idiopathic scoliosis. Approximately 2% to 4% of children aged 10 to 16 years have some degree of spinal curvature. Although some researchers view routine screening for AIS as controversial, well-child examinations and sports physicals are an optimal time to evaluate for AIS in the clinical setting. In 2008, the American Academy of Orthopaedic Surgeons, the Scoliosis Research Society, the Pediatric Orthopaedic Society of North America, and the American Academy of Pediatrics convened a task force to review the issues related to scoliosis screening and issued an information statement concluding that although screening has limitations, the potential benefits that patients with idiopathic scoliosis receive from early treatment can be substantial. Recommendations are now that females are screened twice, at age 10 and 12 years, and males once at age 13 or 14 years. Screening during routine well-child examinations and/or school-based evaluations will help identify patients who need ongoing monitoring. The evaluation of curvatures in conjunction with the level of skeletal maturity will help to guide the management of the curvature.

  12. Genetics Home Reference: familial idiopathic basal ganglia calcification

    Science.gov (United States)

    ... idiopathic basal ganglia calcification ( FIBGC , formerly known as Fahr disease) is a condition characterized by abnormal deposits of ... on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). Am J Hum Genet. 1999 Sep;65(3): ...

  13. 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.

  14. 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)

  15. Lubiprostone for chronic constipation in adults.

    Science.gov (United States)

    2014-04-01

    Lubiprostone (Amitiza-Sucampo Pharma Europe), a chloride-channel activator is licensed for the treatment of chronic idiopathic constipation in adults. It received a marketing authorisation in the UK in September 2012. In this article, we consider the evidence for lubiprostone in the management of constipation and how the treatment fits with current management strategies for constipation.

  16. Chronic hypersensitivity pneumonitis

    Directory of Open Access Journals (Sweden)

    Pereira CA

    2016-09-01

    Full Text Available Carlos AC Pereira,1 Andréa Gimenez,2 Lilian Kuranishi,2 Karin Storrer 2 1Interstitial Lung Diseases Program, 2Pulmonology Postgraduate, Federal University of São Paulo, São Paulo, Brazil Abstract: Hypersensitivity pneumonitis (HSP is a common interstitial lung disease resulting from inhalation of a large variety of antigens by susceptible individuals. The disease is best classified as acute and chronic. Chronic HSP can be fibrosing or not. Fibrotic HSP has a large differential diagnosis and has a worse prognosis. The most common etiologies for HSP are reviewed. Diagnostic criteria are proposed for both chronic forms based on exposure, lung auscultation, lung function tests, HRCT findings, bronchoalveolar lavage, and biopsies. Treatment options are limited, but lung transplantation results in greater survival in comparison to idiopathic pulmonary fibrosis. Randomized trials with new antifibrotic agents are necessary. Keywords: interstitial lung diseases, extrinsic allergic alveolitis, diffuse lung disease, lung immune response, HRCT, farmers lung

  17. Idiopathic Circumscripta Calcinosis Cutis of the Fingers

    Directory of Open Access Journals (Sweden)

    Ömer Çalka

    2013-05-01

    Full Text Available Calcinosis cutis is an uncommon disorder characterized by deposition of insoluble calcium salts in the skin. Based on the etiology of the deposition calcinosis cutis may be devided into four major groups namely metastatic, dystrophic, idiopathic, and iatrogenic. The pathophysiology of calcinosis cutis remains unclear. The dystrophic form is the most common whereas the idiopathic one is the rarest. Idiopathic calcinosis cutis occurs in the absence of any identifiable causes and is not associated with drug use. Idiopathic calcinosis cutis has two major types called localized (circumscript and generalized (universalis. Localized type generally involves the vulva, scrotum, penis and breast. A definitive diagnosis requires the histologic demonstration of the accumulation of calcium in the skin and exclusion of other clinic types.There is not an effective treatment for the disease. A 47-year-old woman referred to our outpatient clinic for painless, yellowish-white nodules on her hand enduring for 30 years. A diagnosis of idiopathic calcinosis cutis was made by clinical and histological findings. We present this case because localization of lesions in our case was previously unreported in the literature to our knowledge.

  18. 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

  19. Idiopathic non-specific interstitial pneumonia.

    Science.gov (United States)

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP.

  20. Chronic pancreatitis: controversies in etiology, diagnosis and treatment Pancreatitis crónica: controversias respecto a la etiología, el diagnóstico y el tratamiento

    OpenAIRE

    Draganov, P.; 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...

  1. Osteoarthritis, osteoarthrosis, and idiopathic condylar resorption.

    Science.gov (United States)

    Mercuri, Louis G

    2008-05-01

    The term "osteoarthritis" has classically been defined as a low-inflammatory arthritic condition. The term "osteoarthrosis," a synonym for osteoarthritis in the medical orthopedic literature, has recently come to be identified in the dental/temporomandibular joint (TMJ) disorders literature with any noninflammatory arthritic condition that results in similar degenerative changes as in osteoarthritis. The term "idiopathic condylar resorption," also known as "progressive condylar resorption," is described as a dysfunctional remodeling of the TMJ manifested by morphologic change, decreased ramal height, progressive mandibular retrusion in the adult, or decreased mandibular growth in the juvenile. This article discusses the diagnosis and management of osteoarthritic TMJ disorders and idiopathic condylar resorption.

  2. Clinical variants of idiopathic torsion dystonia.

    Science.gov (United States)

    Fahn, S

    1989-06-01

    Some patients with dystonic movements and postures not known to be caused by environmental or degenerative disorders can be segregated from classical-appearing idiopathic torsion dystonia on the basis of distinctive clinical and pharmacologic features. Many of them should be considered within the family of dystonia, as clinical variants of idiopathic torsion dystonia, while others are better classified as being part of other families of dyskinesias. In the former group are paradoxical dystonia, myoclonic dystonia, diurnal dystonia, and dopa-responsive dystonia. The latter group consists of dystonic tics and the various entities comprising paroxysmal dystonia, namely kinesigenic, nonkinesigenic and hypnogenic dystonia.

  3. An unusual case of idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Vikender S Yadav

    2013-01-01

    Full Text Available Idiopathic gingival fibromatosisis, a condition of undetermined cause can develop as an isolated disorder, but mostly it is associated with some syndrome. It usually begins at the time of eruption of permanent teeth but can develop with the eruption of deciduous dentition and rarely present at birth. This case report describes an unusual case of non-syndromic generalized idiopathic gingival fibromatosis in a 15-year-old male present since birth. Surgical treatment in the form of ledge and wedge procedure with internal bevel gingivectomy was performed. No recurrence of enlargement was seen after 2 years of follow-up.

  4. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    Science.gov (United States)

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

  5. 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.

  6. B淋巴细胞刺激因子对慢性特发性荨麻疹患者产生抗FcεRI抗体和抗IgE抗体的影响%The Affect of the Serum B Lymphocytes Stimulating Factor(BlyS)on the Anti-IgE Antibody and Anti-FcεRI Antibody of the Patients with Chronic Idiopathic Urticaria

    Institute of Scientific and Technical Information of China (English)

    李正学

    2015-01-01

    目的:探讨慢性特发性荨麻疹患者血清B淋巴细胞刺激因子(BlyS)对抗IgE抗体和抗FcεRI抗体的影响。方法:选取慢性特发性荨麻疹患者100例作为疾病组,选取同期体检健康者100例作为健康组。检测两组受试者血清BlyS、抗IgE抗体及抗FcεRI抗体水平;分离培养疾病组患者外周血中的B淋巴细胞,分为两份保存,在其中一份培养液中加入有效水平的BlyS,检测抗IgE抗体及抗FcεRI抗体水平,作为试验组,将另一份作为空白对照组。分析血清BlyS与抗IgE抗体及抗FcεRI抗体之间的关系。结果:疾病组血清BlyS、抗IgE抗体及抗FcεRI抗体水平明显高于健康组,差异具有统计学意义(P<0.05)。试验组培养液中抗IgE抗体及抗FcεRI抗体水平明显高于对照组,差异具有统计学意义(P<0.05);试验组中抗IgE抗体及抗FcεRI抗体水平与BlyS呈正相关(r=0.765、0.722,P<0.05)。结论:慢性特发性荨麻疹患者血清BlyS可刺激B淋巴细胞产生抗IgE抗体和抗FcεRI抗体,这可能与慢性特发性荨麻疹的发病机制有关。%Objective:To investigate the affect of the serum B lymphocytes stimulating factor(BlyS)on the anti-IgE antibody and anti-FcεRI antibody of the patients with chronic idiopathic urticaria. Method:100 cases of patients with chronic idiopathic urticaria were selected as disease group,100 cases of healthy people in the same period were selected as the healthy group. The serum level of the BlyS,anti-IgE antibody and anti-FcεRI antibodies were detected;the B lymphocytes from the peripheral blood of the disease group patients were isolated and cultured,and divided into two shares,add BlyS in one share as the experimental group,another share as blank control group,the level of the anti-IgE antibody and anti-FcεRI antibodies were detected. The relationship among the BlyS,anti-IgE antibody and anti-FcεRI antibodies were analyzed. Result

  7. Meandering main pancreatic duct as a relevant factor to the onset of idiopathic recurrent acute pancreatitis.

    Directory of Open Access Journals (Sweden)

    Wataru Gonoi

    Full Text Available BACKGROUND: Meandering main pancreatic duct (MMPD, which comprises loop type and reverse-Z type main pancreatic duct (MPD, has long been discussed its relation to pancreatitis. However, no previous study has investigated its clinical significance. We aimed to determine the non-biased prevalence and the effect of MMPD on idiopathic pancreatitis using non-invasive magnetic resonance (MR technique. METHODS AND FINDINGS: A cross-sectional study performed in a tertiary referral center. The study enrolled 504 subjects from the community and 30 patients with idiopathic pancreatitis (7 acute, 13 chronic, and 10 recurrent acute. All subjects underwent MR scanning and medical examination. MMPD was diagnosed when the MPD in the head of pancreas formed two or more extrema in the horizontal direction on coronal images of MR cholangiopancreatography, making a loop or a reverse-Z shaped hairpin curves and not accompanied by other pancreatic ductal anomaly. Statistical comparison was made among groups on the rate of MMPD including loop and reverse-Z subtypes, MR findings, and clinical features. The rate of MMPD was significantly higher for all idiopathic pancreatitis/idiopathic recurrent acute pancreatitis (RAP (20%/40%; P<0.001/0.0001; odds ratio (OR, 11.1/29.0 than in the community (2.2% but was not higher for acute/chronic pancreatitis (14%/8%; P = 0.154/0.266. Multiple logistic regression analysis revealed MMPD to be a significant factor that induces pancreatitis/RAP (P<0.0001/0.0001; OR, 4.01/26.2. Loop/reverse-Z subtypes were found more frequently in idiopathic RAP subgroup (20%/20%; P = 0.009/0.007; OR, 20.2/24.2 than in the community (1.2%/1.0%. The other clinical and radiographic features were shown not associated with the onset of pancreatitis. CONCLUSIONS: MMPD is a common anatomical variant and might be a relevant factor to the onset of idiopathic RAP.

  8. Chronic Idiopathic Cough and Chronic Cough in Idiopathic Pulmonary Fibrosis | EU Clinical Trials Register [EU Clinical Trials Register

    Lifescience Database Archive (English)

    Full Text Available nhaled corticosteroids, benzonatate, dextromethorphan, carbetapentane, H1 antihistamines, leukotriene modifi...eeks of the Screening Visit: Prednisone, narcotic antitussives, baclofen, gabapentin, inhaled corticosteroids, benzonatate, dextromet...horphan, carbetapentane, H1 antihistamines, leukotriene

  9. 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.

  10. Idiopathic Spinal Epidural Abscess: A Case Report

    Directory of Open Access Journals (Sweden)

    Chaitali Biswas

    2011-11-01

    Full Text Available Epidural abscess is a potentially life-threatening disease which can lead to medical-surgical emergency. Idiopathic spinal epidural abscess (SEA with atypical manifestations is extremely rare. We describe such a case which led to severe neurological compromise and was not associated with any known risk factors.

  11. Idiopathic Spinal Epidural Abscess: A Case Report

    OpenAIRE

    Chaitali Biswas; Anirban Pal; Saswata Bharati; Nitesh Sinha

    2011-01-01

    Epidural abscess is a potentially life-threatening disease which can lead to medical-surgical emergency. Idiopathic spinal epidural abscess (SEA) with atypical manifestations is extremely rare. We describe such a case which led to severe neurological compromise and was not associated with any known risk factors.

  12. Vitamin A and Idiopathic Intracranial Hypertension

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-11-01

    Full Text Available Vitamin A levels were measured in the cerebrospinal fluid of a total of 78 patients having idiopathic intracranial hypertension (IIH;n=20, elevated pressure of other causes (E-ICP;n=19, and normal pressure (N-ICP;n=39, in a study at the University of Utah, Salt Lake City, UT.

  13. Sialosis, Gout Induced or Idiopathic? Case Report.

    Science.gov (United States)

    Naik, Keyur; Mandel, Louis

    2017-02-01

    Sialosis is observed in relation to diabetes, alcoholism, and malnutrition. An assumed relation between gout and sialosis is probably based on confusion that originated from the therapeutic use of phenylbutazone for gout and the sialadenitis that the medication caused. This report describes a case of sialosis in a patient with a longstanding history of gout that was idiopathic in origin.

  14. GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

    Directory of Open Access Journals (Sweden)

    A. A. Chernova

    2012-01-01

    Full Text Available Published data demonstrating genetic determination of sick sinus syndrome is presented. The definition of this pathology is presented; the main symptoms are described, as well as genes that influence the development of idiopathic sick sinus syndrome, their polymorphisms and role in disorders of the cardiovascular system.

  15. The human microbiome and juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Verwoerd, Anouk; ter Haar, Nienke M.; de Roock, Sytze; Vastert, Sebastiaan J.; Bogaert, Debby

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The

  16. Pirfenidone treatment in idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Salih, Goran Nadir; Shaker, Saher Burhan; Madsen, Helle Dall;

    2016-01-01

    BACKGROUND: Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect...

  17. 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.

  18. Neocortical gamma oscillations in idiopathic generalized epilepsy

    DEFF Research Database (Denmark)

    Benedek, Krisztina; Berenyi, Antal; Gombkoto, Peter

    2016-01-01

    Objective: Absence seizures in patients with idiopathic generalized epilepsy (IGE) may in part be explained by a decrease in phasic GABAA (type-A c-aminobutyric acid) receptor function, but the mechanisms are only partly understood. Here we studied the relation between ictal and interictal spike-...

  19. Idiopathic erythrocytosis in IgA nephropathy

    Science.gov (United States)

    Mahesh, E.; Madhyastha, P. R.; Kalashetty, M.; Gurudev, K. C.; Bande, S.; John, M. M.

    2017-01-01

    We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.

  20. 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.

  1. Etiology and pathogenesis of adolescent idiopathic scoliosis

    NARCIS (Netherlands)

    Schlösser, Tom P C; Colo, Dino; Castelein, RM

    2015-01-01

    Despite many years of dedicated research into the etio-pathogenesis, not one single cause for adolescent idiopathic scoliosis has been identified. The purpose of this review is to give a comprehensive overview of the current evidence and main etiological theories. Intrinsic causal mechanisms are fou

  2. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

    BACKGROUND AND OBJECTIVE: Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currentl...

  3. Selective fusion in adolescent idiopathic scoliosis

    Institute of Scientific and Technical Information of China (English)

    WANG Ting; XU Jian-guang; ZENG Bing-fang

    2008-01-01

    @@ Despite the continual evolution in the surgical treatment of adolescent idiopathic scoliosis (AIS), the goals of surgery remain to correct and stabilize the deformity in three dimensions, to maintain equilibrium of the shoulders and trunk, and to leave as many mobile spinal segments as possible.

  4. 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.

  5. Capsaicin in Idiopathic Rhinitis A Hot Topic

    NARCIS (Netherlands)

    J.B. van Rijswijk

    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

  6. The lived experience with idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Kaldan, Gudrun; Marsaa, Kristoffer

    2016-01-01

    The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family...

  7. 胸腺五肽联合地氯雷他定治疗慢性特发性荨麻疹的临床观察%Clinical Observation of Thymopentin-5 Combined with Desloratadine in Treatment of Chronic Idiopathic Urticaria

    Institute of Scientific and Technical Information of China (English)

    晏勇; 余霞萍; 陈希林; 刘长山; 林应花

    2013-01-01

    目的:探讨胸腺五肽联合地氯雷他定治疗慢性特发性荨麻疹的临床疗效。方法:治疗慢性特发性荨麻疹患者180例,按照随机数字表法将其分为治疗组和对照组各90例。治疗组:口服地氯雷他定分散片5 mg,1次/d,同时胸腺五肽针1 mg加入0.9%氯化钠注射液250 mL,静脉滴注,1次/d;对照组:口服地氯雷他定片5 mg,1次/d。两组均连续用药4周后,随访观察12周,评定疗效并记录皮损变化和不良反应。结果:治疗组痊愈率为95.6%,总有效率为97.8%,对照组痊愈率为90%,总有效率为95.6%。两组短期症状控制效果都非常有效,差异无统计学意义(P>0.05)。两组痊愈患者复发情况观察结果显示,治疗组停药超过12周未复发(优等)病例52例(60.5%),对照组11例(13.6%);治疗组停药后4周以内复发(差等)病例9例(10.4%),对照组38例(46.9%),两组比较差异均有统计学意义(P0.05). The two group of patients relapse were observed. The results showed,the treatment group was more than 12 weeks in withdrawal recurrence(top)of 52 cases(60.5%),11 cases in the control group(13.6%),the treatment group 4 weeks after withdrawal recurrence within 9(low)cases(10.4%),38 cases in the control group(46.9%),and the difference of two indicates above between the two groups were no statistically significant(P<0.01).Conclusion:Thymopentin-5 combined with desloratadine in treatment of chronic idiopathic urticaria obvious curative effect,can effectively control the nettle rash and itching,little side effect,low recurrence rate,long-term effect is obviously higher than that of single desloratadine therapy.

  8. Opana ER abuse and thrombotic thrombocytopenic purpura (TTP)-like illness: a rising risk factor in illicit drug users.

    Science.gov (United States)

    Kapila, Aaysha; Chhabra, Lovely; Chaubey, Vinod K; Summers, Jeffery

    2014-03-03

    We report the case of a 22 year-old-woman who presented with upper extremity cellulitis secondary to an infiltration of illicit intravenous drug use. She confessed to the intravenous use of Opana ER (an extended release oral formulation of oxymorphone) which is an opioid drug approved only for oral use. She was found to have clinical evidence of profound thrombotic microangiopathy which resulted due to the intravenous use of Opana ER. She showed full clinical improvement after withholding drug and supportive clinical care. Recent report of Opana ER intravenous abuse was published from Tennessee county and has now been increasingly recognised as one of the causes of thrombocytopenia which mimicks clinically as thrombotic thrombocytopenic purpura. Physicians should be aware of this association as the lack of familiarity to this can pose serious management dilemmas for our patients (especially the polysubstance abusers).

  9. Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome).

    Science.gov (United States)

    Korkmaz, Huseyin; Bugdaci, Mehmet Sait; Temel, Tuncer; Dagli, Mehmet; Karabagli, Pinar

    2013-04-01

    Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) associated with Evans syndrome; combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) has rarely been reported. We report the case of a 53-year-old patient who presented with weakness, myalgia, arthralgia, shortness of breath and purpura. Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. Diagnose of PBC-AIH overlap was made by clinical, serological and histological investigations. AIHA and ITP was identified with clinical-laboratory findings and bone marrow puncture. She was treated with IVIG followed by prednisolone and ursodeoxycholic acid. Hemoglobin-thrombocytes increased rapidly and transaminases improved at day 8. We have reported the first case in the literature with AIH-PBC overlap syndrome concurrent by ITP and AIHA which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including AIH, PBC, ITP and AIHA.

  10. Acute clinical onset chronic inflammatory demyelinating polyneuropathy in a dog.

    Science.gov (United States)

    Molín, Jéssica; Márquez, Mercedes; Raurell, Xavier; Matiasek, Kaspar; Ferrer, Isidre; Pumarola, Martí

    2011-09-01

    We report a case of acute-onset ambulatory paraparesis with electrophysiological abnormalities compatible with axonal and demyelinating lesions in a Rottweiler dog. Although the clinical findings were compatible with acute canine idiopathic polyneuropathy, postmortem investigations revealed a chronic demyelinating polyneuropathy affecting the nerve roots. Due to the combination of acute clinical presentation and chronic pathologic features, this case is consistent with the acute-onset form of chronic inflammatory demyelinating polyneuropathy (A-CIDP).

  11. Novel self-epitopes derived from aggrecan, fibrillin, and matrix metalloproteinase-3 drive distinct autoreactive T-cell responses in juvenile idiopathic arthritis and in health

    NARCIS (Netherlands)

    S.S.M. Kamphuis (Sylvia); K. Hrafnkelsdóttir (Kolbrún); M. Klein (Mark); W. de Jager (Wilco); M.H. Haverkamp (Margje); J.H.M. van Bilsen (Jolanda); S. Albani (Salvatore); W. Kuis (Wietse); M.H.M. Wauben (Marca); B.J. Prakken (Berent)

    2006-01-01

    textabstractJuvenile idiopathic arthritis (JIA) is a heterogeneous autoimmune disease characterized by chronic joint inflammation. Knowing which antigens drive the autoreactive T-cell response in JIA is crucial for the understanding of disease pathogenesis and additionally may provide targets for an

  12. Comparison of platelet counts by sysmex XE 2100 and LH-750 with the international flow reference method in thrombocytopenic patients

    Directory of Open Access Journals (Sweden)

    Tina Dadu

    2013-01-01

    Full Text Available Background: There are several methods for counting platelets, of which the international flow reference method (IRM is considered to be the gold standard. We compared the platelet count given by this method to the count given by automated analyzers using other methods, such as optical fluorescence and impedance. Aims: The aim of this study is to compare the platelet counts obtained by Sysmex XE 2100 by Impedance (Sysmex-I, optical florescence (Sysmex-O and reported (Sysmex-R based on the switching algorithm and LH-750 by Impedance (LH-750 with the IRM in thrombocytopenic blood samples. To calculate the sensitivity, specificity, positive predictive value (PPV and negative predictive value (NPV of various technologies at the clinically relevant transfusion thresholds of 10 × 10 9 /l and 20 × 10 9 /l. Materials and Methods: A total of 118 blood samples with platelet count of <50 × 10 9 /l were selected for the study. Platelet counts of all samples were analyzed by all methods using the Sysmex analyzer, LH-750 and IRM in parallel within 6 h of collection. Statistical Analysis Used: Pearson correlation, bland Altman analysis, sensitivity and specificity, PPV and NPV. Results and Conclusions: Sysmex-R had the least Bias and 95% limits of agreement (95%LA range and thus correlated best with IRM values. LH-750 had a higher Bias compared to Sysmex-O and Sysmex-R, but a strikingly similar 95% LA ensures similar results in all three methods. In fact, in the oncology subset, it had the narrowest 95% LA, which made it the best performer in this subgroup. Of the three Sysmex results, Sysmex-I had the highest bias, widest 95% LA and highest potential risk of over transfusion. Hence, Sysmex-R and LH-750 were found to be reliable tools for estimation of platelet count in thrombocytopenic patients.

  13. Chronic pancreatitis

    Science.gov (United States)

    Chronic pancreatitis - chronic; Pancreatitis - chronic - discharge; Pancreatic insufficiency - chronic; Acute pancreatitis - chronic ... alcohol abuse over many years. Repeated episodes of acute ... chronic pancreatitis. Genetics may be a factor in some cases. ...

  14. Different microcirculatory and interstitial matrix patterns in idiopathic dilated cardiomyopathy and Chagas' disease: a three dimensional confocal microscopy study

    OpenAIRE

    Higuchi, M.; Fukasawa, S; De Brito, T.; Parzianello, L; G. Bellotti; Ramires, J

    1999-01-01

    OBJECTIVE—To analyse the morphological aspects of the extracellular matrix and microcirculation to clarify whether chronic Chagas' cardiopathy (CCC) is an accurate model to study the pathogenesis of idiopathic dilated cardiomyopathy (IDCM).
DESIGN—Thick histological myocardial sections were prepared to analyse collagen, and microcirculation was examined during confocal laser and light microscopy.
SETTING—The specimens were prepared at the pathology service of the Heart Institute of São Paulo,...

  15. Staphylococcus aureus septicemia presenting as disseminated intravascular coagulation - thrombotic thrombocytopenic purpura overlap and thrombus in inferior vena cava, right atrium and right ventricle: a case report

    Directory of Open Access Journals (Sweden)

    Khwaja Saifullah Zafar

    2015-01-01

    Full Text Available Staphylococcal sepsis following furunculosis and complicated by suspected deep vein thrombosis and septic inferior vena caval, right atrium, right ventricle emboli accompanied by disseminated intravascular coagulation (DIC - thrombotic thrombocytopenic overlap in a 65 years old lady is presented. She was managed successfully with antibiotics and anticoagulation. The case is reported for its rarity and brings to light the vivid manifestations of septicemia specially staphylococcal. [Int J Res Med Sci 2015; 3(1.000: 368-372

  16. Anatomical Consideration in Catheter Ablation of Idiopathic Ventricular Arrhythmias.

    Science.gov (United States)

    Yamada, Takumi; Kay, G Neal

    2016-01-01

    Idiopathic ventricular arrhythmias (VAs) are ventricular tachycardias (VTs) or premature ventricular contractions (PVCs) with a mechanism that is not related to myocardial scar. The sites of successful catheter ablation of idiopathic VA origins have been progressively elucidated and include both the endocardium and, less commonly, the epicardium. Idiopathic VAs usually originate from specific anatomical structures such as the ventricular outflow tracts, aortic root, atrioventricular (AV) annuli, papillary muscles, Purkinje network and so on, and exhibit characteristic electrocardiograms based on their anatomical background. Catheter ablation of idiopathic VAs is usually safe and highly successful, but can sometimes be challenging because of the anatomical obstacles such as the coronary arteries, epicardial fat pads, intramural and epicardial origins, AV conduction system and so on. Therefore, understanding the relevant anatomy is important to achieve a safe and successful catheter ablation of idiopathic VAs. This review describes the anatomical consideration in the catheter ablation of idiopathic VAs.

  17. Dysregulated innate immune function in the aetiopathogenesis of idiopathic inflammatory myopathies.

    Science.gov (United States)

    Day, Jessica; Otto, Sophia; Proudman, Susanna; Hayball, John D; Limaye, Vidya

    2017-01-01

    The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of systemic muscle conditions that are believed to be autoimmune in nature. They have distinct pathological features, but the aetiopathogenesis of each subtype remains largely unknown. Recently, there has been increased interest in the complex role the innate immune system plays in initiating and perpetuating these conditions, and how this may differ between subtypes. This article summarises the traditional paradigms of IIM pathogenesis and reviews the accumulating evidence for disturbances in innate immune processes in these rare, but debilitating chronic conditions.

  18. Surgical treatment for primary lung cancer combined with idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Watanabe, Atsushi; Miyajima, Masayoshi; Mishina, Taijiro; Nakazawa, Junji; Harada, Ryo; Kawaharada, Nobuyoshi; Higami, Tetsuya

    2013-05-01

    Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. IPF is associated with an increased risk of lung cancer, and lung cancer patients with IPF undergoing pulmonary resection for non-small cell lung cancer have increased postoperative morbidity and mortality. Especially, postoperative acute exacerbation of IPF (AEIPF) causes fatal status and long-term outcomes are worse than for patients without IPF, although certain subgroups have a good long-term outcome. A comprehensive review of the current literature pertaining to AEIPF and the late phase outcome after the context of a surgical intervention was performed.

  19. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy.

    Science.gov (United States)

    Carr, Laurie L; Kern, Jeffrey A; Deutsch, Gail H

    2016-09-01

    Although incidental reactive pulmonary neuroendocrine cell hyperplasia (PNECH) is seen on biopsy specimens in adults with chronic lung disease, disorders characterized by marked PNECH are rare. Primary hyperplasia of neuroendocrine cells in the lung and obstructive lung disease related to remodeling or physiologic constriction of small airways define diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) in the adult and neuroendocrine cell hyperplasia of infancy (NEHI) in children. DIPENCH and NEHI share a similar physiology, typical imaging appearance, and increased neuroendocrine cells on biopsy. However, there are important differences related to the underlying disease mechanisms leading to disparate outcomes.

  20. Idiopathic intracranial hypertension and transverse sinus stenoses

    DEFF Research Database (Denmark)

    Skyrman, Simon; Fytagoridis, Anders; Andresen, Morten

    2013-01-01

    An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed to be asso......An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed...... to be associated with the IIH. Initial treatment consisted of symptom relief by a temporary lumbar drain for cerebrospinal fluid (CSF) diversion, while the pros and cons of a more permanent solution by insertion of a ventriculoperitoneal shunt (VPS) or bilateral transverse sinus stent was discussed. A VPS...

  1. Clinical characteristics of idiopathic portal hypertension

    Institute of Scientific and Technical Information of China (English)

    Ozgur Harmanci; Yusuf Bayraktar

    2007-01-01

    Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

  2. Idiopathic gingival enlargement and its management

    Directory of Open Access Journals (Sweden)

    Shetty Arvind

    2010-01-01

    Full Text Available Idiopathic gingival enlargement is a proliferative fibrous lesion of the gingival tissue that causes esthetic and functional problems. Both genetically and pharmacologically induced forms of gingival enlargement exist. This case report addresses the diagnosis and treatment of a case of idiopathic gingival enlargement in a 13-year-old female. The patient presented with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal / occlusal third of the tooth resulting in difficulty in speech and mastication since last three years. Patient also gave a history of surgical treatment being carried out four years back in upper anterior region suggesting of recurrence. Biopsy report confirmed the diagnosis of gingival hyperplasia. Gingivectomy was carried out in all four quadrants by using four different methods.

  3. Idiopathic gingival enlargement and its management

    Directory of Open Access Journals (Sweden)

    Pawan Kumar

    2015-01-01

    Full Text Available Increase in the size of gingiva referred to as "gingival enlargement," usually overfills the interproximal spaces, ballooning out over the teeth and sometimes even protruding into the oral cavity. It may occur as localized enlargement in relation to a single tooth, a group of teeth, or may be generalized involving the entire dentition. It is multifactorial in origin having the influence of bacterial plaque, systemic factors or conditions, genetic predisposition (hereditary or familial, and various medications. Sometimes, it may appear as diffuse enlargement of gingiva without any specific etiologic factor. It is then considered as idiopathic enlargement. In this case report, an idiopathic gingival enlargement was successfully treated using gingivectomy by ledge and wedge procedure. The surgical technique performed yielded functionally as well as esthetically satisfying results. Gingivectomy provides a viable option to manage diffuse enlargement cases. This procedure provides the functional as well as esthetic demands and at the same time, it maintains the integrity of periodontium.

  4. [Pathogenesis of the idiopathic inflammatory myopathies].

    Science.gov (United States)

    Riebeling-Navarro, Carlos; Nava, Arnulfo

    2009-11-01

    The inflammatory myopathies, commonly described as idiopathic, are a group of acquired diseases characterized by an inflammatory infiltrate of the skeletal muscle. On the basis of clinical and immuno-pathological features, three major diseases can be identified: dermatomiositis (DM), polymyositis (PM) and inclusion body myositis (IBM). Immunopathogenesis mechanisms are crucial for discriminating between the three different subsets of inflammatory myopathies. DM is a complement-mediated microangiopathy affecting skin and muscle. PM and IBM are T cell-mediated disorders, where CD8-positive cytotoxic T cells invade muscle fibres expressing MHC class I antigens. This article summarizes the main immunopathological markers. The impact of this new knowledge must be defined in relation to potential therapeutic targets for idiopathic inflammatory myopathies.

  5. [Biologics for treatment of juvenile idiopathic arthritis. Consensus statement of the 7th Wörlitzer Expertengespräche 2004 for the German Arbeitsgemeinschaft Kinder- und Jugendrheumatologie].

    Science.gov (United States)

    Horneff, G

    2006-03-01

    The group of biologics for the treatment of rheumatic diseases is continuously growing. They have become an important option not only for treatment of so far untreatable chronic inflammatory or rheumatic disease, but also for juvenile idiopathic arthritis. In addition, the velocity and the degree of improvement is better than with to conventional therapies. Furthermore, toxicity and risks seem to be lower with higher safety and compatibility. Although the data are scarce, they are widely used. Therefore, the German Arbeitsgemeinschaft Kinder- und Jugendrheumatologie is updating the current recommendation for the treatment of juvenile idiopathic arthritis using biologics.

  6. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summariz...

  7. Idiopathic intracranial hypertension in female homozygous twins.

    OpenAIRE

    Fujiwara, S; Sawamura, Y; Kato, T.; Abe, H.; Katusima, H

    1997-01-01

    The authors report on female homozygous twins with idiopathic intracranial hypertension. At the age of 12 years, both twins simultaneously developed visual disturbances with photophobia. At the age of 19 years, an ophthalmological examination disclosed papilloedema in both their eyes. At the age of 22 years, a lumbar puncture showed raised CSF pressure over (200 mm H2O) in both twins. Their neurological and radiological examinations were extremely similar; both of them had severely impaired v...

  8. Optimal management of idiopathic scoliosis in adolescence

    OpenAIRE

    Kotwicki T; Chowanska J; Kinel E; Czaprowski D; Tomaszewski M; Janusz P

    2013-01-01

    Tomasz Kotwicki,1 Joanna Chowanska,1,2 Edyta Kinel,3 Dariusz Czaprowski,4,5 Marek Tomaszewski,1 Piotr Janusz1 1Department of Pediatric Orthopedics, University of Medical Sciences, Poznan, Poland; 2National Scoliosis Foundation, Stoughton, MA, USA; 3Department of Rehabilitation, University of Medical Sciences, Poznan Poland; 4Department of Physiotherapy, Józef Rusiecki University College, Olsztyn, 5Rehasport Clinic, Poznan, Poland Abstract: Idiopathic scoliosis is a three-dimension...

  9. 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

  10. JUVENILE IDIOPATHIC ARTHRITIS – A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Paresh H

    2012-11-01

    Full Text Available ABSTRACT: The prevalence of Juvenile idiopathic arthritis (JIA is 0.86 per 1000 children. Subcutaneous nodules have been reported in 5% to 10% of children with JIA. Approximately 90% of patients with RA and subc utaneous nodules test positive for rheumatoid factor (RF, and approximately 40% o f all RF-seropositive patients with RA have subcutaneous nodules, whereas only 6% in volvement is seen in seronegative cases. We hereby report a case of atypical Juvenile idiopathic arthritis (JIA in a 6 year old, female child with joint pain & myalgia along with subcutaneous nodules over the dorsum of feet, hands and elbows. Joint pain initial ly involving the left ankle, slowly progressed to involve the knee, shoulder, wrist, metacar pophalangeal and interphalangeal joints over a period of one year. Joint involvement was not symmetric. RF was Negative. Fundoscopy examination was normal. Histopathological examinat ion revealed a central zone of Fibrinoid necrosis surrounded by epithelioid h istiocytes and occasional lymphocytes. Differential diagnosis of Rheumatoid Nodule (R N or Subcutaneous Granuloma Annulare (SGA or Necrobiosis Lipoidica Diabeticorum was made. In light of clinicopathological findings, both SGA and NLD were ruled out a nd the diagnosis of Juvenile idiopathic arthritis presenting as RF-negative polyarthritis was made.

  11. Brace Treatment in Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Ali Sehirlioglu

    2012-01-01

    Full Text Available Adolescent idiopathic scoliosis (AIS, the most common pediatric spine problem, is a structural lateral and rotatory curvature of the spine arising in otherwise normal children during puberty. It occurs before skeletal maturity. Although there is still no cause for AIS, the natural history of AIS has been established in the literature very well. The aim of nonoperative treatment is mainly an attempt to prevent progression of the curve. Bracing and surgery have been used for large and or progressive curves currently. Many conservative treatments are available for adolescents with idiopathic scoliosis (AIS. Although there are numerous studies in literature that have tried to summarize the results of treatment, the evidence for their accepted use is still unclear. Many clinicians skeptical about the efficacy of conservative treatments. Because there is no consistency of both the inclusion criteria and the definitions of brace effectiveness. The definition of success or who should be included in the analysis have never been universally agreed upon. The Scoliosis Research Society established parameters for all future AIS bracing studies to be able to make comparison among studies more valid and reliable. These guidelines may standardize orthotic studies by recommending inclusion and assessment criteria and allow the promotion of the effectiveness of different braces and decrease the suspicion about their usefulness. Orthotic treatment in adolescent idiopathic scoliosis is used to control spinal curvatures while waiting for skeletal maturation.

  12. Pulmonary function in children with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Tsiligiannis Theofanis

    2012-03-01

    Full Text Available Abstract Idiopathic scoliosis, a common disorder of lateral displacement and rotation of vertebral bodies during periods of rapid somatic growth, has many effects on respiratory function. Scoliosis results in a restrictive lung disease with a multifactorial decrease in lung volumes, displaces the intrathoracic organs, impedes on the movement of ribs and affects the mechanics of the respiratory muscles. Scoliosis decreases the chest wall as well as the lung compliance and results in increased work of breathing at rest, during exercise and sleep. Pulmonary hypertension and respiratory failure may develop in severe disease. In this review the epidemiological and anatomical aspects of idiopathic scoliosis are noted, the pathophysiology and effects of idiopathic scoliosis on respiratory function are described, the pulmonary function testing including lung volumes, respiratory flow rates and airway resistance, chest wall movements, regional ventilation and perfusion, blood gases, response to exercise and sleep studies are presented. Preoperative pulmonary function testing required, as well as the effects of various surgical approaches on respiratory function are also discussed.

  13. 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.

  14. B淋巴细胞刺激因子对慢性特发性荨麻疹患者产生抗FcεRI抗体和抗IgE抗体的影响%Influence of B lymphocyte stimulator on the production of anti-FcεRI and anti-IgE antibodies by B lymphocytes from patients with chronic idiopathic urticaria

    Institute of Scientific and Technical Information of China (English)

    康尔恂; 李杰; 孙丽伟; 韩春玉; 闫丽萍; 杨建

    2013-01-01

    目的 探讨B淋巴细胞刺激因子(BlyS)能否刺激慢性特发性荨麻疹(CIU)患者B淋巴细胞产生抗FcεRI抗体或抗IgE抗体.方法 设CIU患者组和健康对照组.ELISA法测定血清中BlyS、抗FcεRI抗体和抗IgE抗体水平,分离培养受试者外周血B淋巴细胞,在培养液中加入BlyS,检测培养液中抗FcεRI抗体和抗IgE抗体水平,分析BlyS与抗FcεRI抗体和抗IgE抗体产生的相关性.结果 CIU患者血清BlyS水平显著高于健康对照组(t=3.04,P< 0.01),抗FcεRI抗体和抗IgE抗体水平均显著高于健康对照组(t=3.51,P<0.01;t=3.29,P< 0.01).CIU患者血清中抗FcεRI抗体和抗IgE抗体水平与BlyS水平呈正相关(r=0.93,P<0.01;r=0.91,P< 0.01);CIU患者外周血B淋巴细胞培养液中加入有效浓度BlyS后,B淋巴细胞培养液中抗FcεRI抗体和抗IgE抗体水平均显著高于不加BlyS的空白对照(t=3.67,P< 0.01;t=3.56,P< 0.01),2种抗体在培养液中的水平与BlyS浓度呈正相关(r=0.96,P< 0.01;r=0.91,P< 0.01);抗FcεRI抗体和抗IgE抗体在CIU患者血清与培养液中的检出符合率分别为94.76%和87.84%.结论 CIU患者血液中BlyS水平增高可刺激B淋巴细胞产生抗FcεRI抗体或抗IgE抗体,可能与CIU发病有关.%Objective To explore if B lymphocyte stimulator (BlyS) stimulates B lymphocytes from patients with chronic idiopathic urticaria (CIU) to produce anti-high affinity IgE receptor (FcεRI) or anti-IgE antibodies.Methods Totally,300 CIU patients and 300 health controls were enrolled in this study.Blood samples were obtained from these subjects.Peripheral blood B lymphocytes were isolated and cultured in vitro for 72 hours.Then,BlyS of various concentrations (2,4,8,16 ng/ml) was added to the culture medium of B lymphocytes followed by another 72-hour culture.Enzyme-linked immunosorbent assay was performed to determine the serum levels of BlyS,anti-FcεRI and anti-IgE antibodies,as well as the supernatant levels of anti

  15. Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss.

    Science.gov (United States)

    Lok, Julie Y C; Yip, Nelson K F; Chong, Kelvin K L; Li, C L; Young, Alvin L

    2015-08-01

    Idiopathic hypertrophic pachymeningitis is a rare inflammatory condition with diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. We report on a 28-year-old Chinese woman with a history of granulomatous mastitis 7 years previously and oligomenorrhoea, headache, blurred vision, and raised prolactin level 2 years previously, that was diagnosed as prolactinoma and treated conservatively with bromocriptine. However, she had recurrent bilateral vision loss when the bromocriptine was stopped. Her symptoms were resolved by high-dose steroid injection but remained steroid-dependent. Serial magnetic resonance imaging scan showed progressive diffuse thickening of the pachymeningitis with disappearance of pituitary apoplexy. Lumbar puncture showed lymphocytosis with no organisms. Open biopsy of the meninges was performed and histology showed features of inflammatory infiltrates and vasculitis. This is an unusual presentation of a rare condition in this age-group, with co-existing granulomatous mastitis and chronic otitis media, and is a diagnostic challenge mimicking pituitary macroadenoma and meningioma in initial magnetic resonance imaging scans.

  16. 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.

  17. Treatment with gabapentin of 11 dogs with refractory idiopathic epilepsy.

    Science.gov (United States)

    Platt, S R; Adams, V; Garosi, L S; Abramson, C J; Penderis, J; De Stefani, A; Matiasek, L

    Eleven dogs diagnosed with refractory idiopathic epilepsy were treated orally with gabapentin for a minimum of three months at an initial dose of 10 mg/kg every eight hours. They were all experiencing episodes of generalised tonic-clonic seizures and had been treated chronically with a combination of phenobarbital and potassium bromide at doses sufficient to reach acceptable therapeutic serum levels without causing significant side effects. In each dog, the number of seizures per week, the average duration of the seizures and the number of days on which seizures occurred were compared for the three months before and after they were treated with gabapentin. A minimum 50 per cent reduction in the number of seizures per week was interpreted as a positive response to gabapentin, and six of the dogs showed a positive response. After the addition of gabapentin, both the number of seizures per week (P= 0.005) and the number of days with any seizures in a one-week period (P=0.03) were significantly reduced. Mild side effects of ataxia and sedation were observed in five of the dogs, but they were not severe enough to warrant the treatment being discontinued during the trial.

  18. 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.

  19. Idiopathic scoliosis; a biomechanical and functional anatomical study.

    NARCIS (Netherlands)

    Veldhuizen, Albert Gerrit

    1985-01-01

    Although many types of causes of scoliosis are known, the idiopathic variety comprises the largest group and as its name indicates, its a etiology is unknown. Idiopathics coliosis develops in a previously normal vertebral column and has been recognized as an entity for more than a century.

  20. Abnormal glomerular basement membrane in idiopathic multicentric osteolysis

    NARCIS (Netherlands)

    Bakker, SJL; Vos, GD; Verschure, PDMM; Mulder, AH; Tiebosch, TMG

    1996-01-01

    The primary cause of nephropathy in idiopathic multicentric osteolysis is as yet unknown. We report a young girl with idiopathic multicentric osteolysis and nephropathy. An abnormal glomerular basement membrane was the only abnormality found in a renal biopsy taken 2 years before the development of

  1. Perceived health status in self-reported adolescent idiopathic scoliosis

    DEFF Research Database (Denmark)

    Andersen, Mikkel Ø; Thomsen, Karsten; Kyvik, Kirsten O

    2010-01-01

    A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort.......A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort....

  2. 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.

  3. Study of idiopathic inflammatory myopathies with special reference to borderland between idiopathic inflammatory myopathies and muscular dystrophies

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    Khadilkar Satish

    2008-01-01

    Full Text Available Background: Idiopathic inflammatory myopathies (IIMs form important treatable myopathies, hence it is important to recognize and categorize them. In some cases, the differential diagnosis between IIM and muscular dystrophies can be difficult. Aim: To study the clinical and laboratory features of patients with IIMs and compare and contrast this group with limb girdle muscular dystrophies (LGMDs. Setting and Design: A prospective study for the period of five years [1999-2004] was undertaken at a tertiary neuromuscular center. Materials and Methods: Bohan and Peter criteria were used for the diagnosis of IIM and Bushby criteria were used for the diagnosis of LGMD. Patients underwent history, clinical examination, hematological tests, electrophysiological studies and muscle biopsy. The biopsies were studied for histology and immunocytochemistry. A clinical scoring system was evolved to differentiate IIM from LGMD and was validated in a blinded manner. Receiver operator curves were used as the statistical method to analyze the sensitivity and specificity. Results and Conclusions: In the IIM group, dermatomyositis was most common, followed by polymyositis, occurring in young females. Overlap group was less common. In patients with polymyositis, onset in upper girdle was associated with adverse outcome. The scoring system helped to differentiate IIM from LGMD, mainly using clinical pointers. This was particularly valuable in chronic cases.

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

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    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

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

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    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 .

  6. Immunogenetics of juvenile idiopathic arthritis: A comprehensive review.

    Science.gov (United States)

    Hersh, Aimee O; Prahalad, Sampath

    2015-11-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthropathy of childhood. Juvenile idiopathic arthritis is believed to be a complex genetic trait influenced by both genetic and environmental factors. Twin and family studies suggest a substantial role for genetic factors in the predisposition to JIA. Describing the genetics is complicated by the heterogeneity of JIA; the International League of Associations for Rheumatology (ILAR) has defined seven categories of JIA based on distinct clinical and laboratory features. Utilizing a variety of techniques including candidate gene studies, the use of genotyping arrays such as Immunochip, and genome wide association studies (GWAS), both human leukocyte antigen (HLA) and non-HLA susceptibility loci associated with JIA have been described. Several of these polymorphisms (e.g. HLA class II, PTPN22, STAT4) are shared with other common autoimmune conditions; other novel polymorphisms that have been identified may be unique to JIA. Associations with oligoarticular and RF-negative polyarticular JIA are the best characterized. A strong association between HLA DRB1:11:03/04 and DRB1:08:01, and a protective effect of DRB1:15:01 have been described. HLA DPB1:02:01 has also been associated with oligoarticular and RF-negative polyarticular JIA. Besides PTPN22, STAT4 and PTPN2 variants, IL2, IL2RA, IL2RB, as well as IL6 and IL6R loci also harbor variants associated with oligoarticular and RF-negative polyarticular JIA. RF-positive polyarticular JIA is associated with many of the shared epitope encoding HLA DRB1 alleles, as well as PTPN22, STAT4 and TNFAIP3 variants. ERA is associated with HLA B27. Most other associations between JIA categories and HLA or non-HLA variants need confirmation. The formation of International Consortia to ascertain and analyze large cohorts of JIA categories, validation of reported findings in independent cohorts, and functional studies will enhance our understanding of the genetic

  7. The Role of Gender in Juvenile Idiopathic Arthritis-Associated Uveitis

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    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.

  8. Idiopathic thoracic aortic aneurysm at pediatric age.

    Science.gov (United States)

    Marín-Manzano, E; González-de-Olano, D; Haurie-Girelli, J; Herráiz-Sarachaga, J I; Bermúdez-Cañete, R; Tamariz-Martel, A; Cuesta-Gimeno, C; Pérez-de-León, J

    2009-03-01

    A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

  9. The Electrophysiology in Idiopathic Senile Macular Hole

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    Of 12 patients with idiopathic senile full- thickness macular hole, 3 had bilateral involvement, 9 had monocular macular hole. Flash ERG and pattern VEP were performed in the bilateral eyes of all patients. The abnormal rate of the pattern VEP was 93.3% when we used 15' checkboard stimulus, the changes of the VEP appeared as delayed latencies, reduced amplitudes or malformation of P100. The abnormal rate of the flash ERG was 53.3%, showing primary characteristics of reduced amplitudes of cone response b...

  10. "Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V

    2013-10-15

    The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children.

  11. Genetics of idiopathic generalized epilepsy: An overview

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    D. K. V. Prasad

    2013-01-01

    Full Text Available Idiopathic generalized epilepsy (IGE is a common type of epilepsy. Strong support for a genetic role in IGE comes from twin and family studies. Several subtypes of IGE have been reported but families often have members affected with different subtypes. Major advances have been made in the understanding of genetic basis of monogenic inherited epilepsies. However, most IGEs are complex genetic diseases and some susceptible IGE genes are shared across subtypes that determine subtypes in specific combinations. The high throughput technologies like deoxyribonucleic acid microarrays and sequencing technologies have the potential to identify causative genes or loci in non-familial cases.

  12. Idiopathic calcinosis cutis of nasal dorsum

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    Shrinath D.P Kamath

    2012-01-01

    Full Text Available A 23-year-old lady presented with a slowly progressing firm mass on the nasal dorsum since 8 months. Her biochemical, haematological and collagen vascular disease screening tests were normal. Radiographs of the nasal bones showed a subcutaneous calcifying lesion with no evidence of nasal bone erosion. A diagnosis of idiopathic calcinosis cutis (ICC was made. The mass was excised and soft tissue defect was augmented with silicone prosthesis. The histopathology with the haematoxylin and eosin staining and von Kossa stain confirmed the diagnosis of calcinosis cutis. This is an unusual presentation of ICC involving the nasal dorsum requiring surgery and nasal dorsal augmentation with silicone prosthesis.

  13. Juvenile idiopathic arthritis: the paediatric perspective

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    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.)

  14. Update on idiopathic hirsutism: diagnosis and treatment.

    Science.gov (United States)

    Erem, C

    2013-01-01

    Idiopathic hirsutism (IH) is defined as hirsutism in conjunction with normal ovulatory function and normal serum androgen levels. The pathogenesis of IH is still not clear. Increased peripheral 5alpha-reductase enzyme activity and abnormalities of androgen receptor gene polymorphisms have been postulated to explain the pathogenesis of this disorder. It is diagnosed in women who have hirsutism, normal ovulatory function, and normal levels of serum total or free testosterone. Combination treatment of IH, including androgen suppression, peripheral androgen blockade and cosmetic methods is most effective.

  15. Idiopathic gingival enlargement: A case report

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    Rajesh Shah

    2015-09-01

    Full Text Available Idiopathic gingival fibromatosis is a relatively rare condition characterized by the proliferation of the gingival tissues resulting in masticatory, esthetics, phonetics and psychological disturbances. We present a case with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal/occlusal third of the tooth in the left maxillary region. Gingivectomy was carried out in all four quadrants. Periodic recalls showed maintenance of good oral hygiene and one year follow-up revealed no recurrence.JCMS Nepal. 2015;11(1: 26-28

  16. Idiopathic esophagopleural fistula in the newborn

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    Iannaccone, G.; Cozzi, F.; Roggini, M.; Capocaccia, P.

    1982-07-01

    Idiopathic rupture of the esophagus in the neonate is a rare event, probably related to the same mechanism of ischemic necrosis responsible for other 'spontaneous' g.i. tract perforations in the newborn. The laceration is usually located on the right aspect of the distal esophagus and is complicated by esophagopleural fistula and hydropneumothorax. Plain chest film and esophagography are diagnostic. The condition is an emergency one and usually carries a bad prognosis without prompt surgical repair. A typical case is reported in a baby who survived without early surgery; a residual tiny blind pouch and a small hiatal hernia required surgery at 1 year of age.

  17. Behavioral conditioning and idiopathic environmental intolerance.

    Science.gov (United States)

    Giardino, N D; Lehrer, P M

    2000-01-01

    Idiopathic environmental intolerance (IEI) is a poorly understood condition that may involve disturbances in immunologic, neurologic, endocrine, behavioral, emotional, and cognitive processes. This chapter reviews theories and evidence that behavioral conditioning processes, including pharmacologic sensitization, conditioned immunomodulation, and conditioned odor and taste aversions, may play a role in the development and maintenance of IEI. It also reviews the psychophysiologic concepts of individual response specificity and situational response stereotypy as potential explanations for the individual differences observed in specific responses to environmental stimuli in patients with IEI. Finally, the treatment implications of a conditioning account of IEI are discussed as part of a more comprehensive treatment approach that incorporates other behavioral and nonbehavioral strategies.

  18. Idiopathic environmental intolerance: case definition issues.

    Science.gov (United States)

    Kreutzer, R

    2000-01-01

    Case definitions of the same phenomenon may be different for different purposes. Case definitions usually become more specific over time as more information about the condition becomes available. Idiopathic environmental intolerance is one of many labels for a heterogeneous group of conditions in which subjects describe multiple symptoms that are attributed to exposure to extremely low doses of common chemicals. Dr. Kreutzer presents issues in case definition for clinical purposes and for population-based studies, and makes recommendations for the clinician and for the public health investigator.

  19. Autonomic symptoms in idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Ferini-Strambi, Luigi; Oertel, Wolfgang; Dauvilliers, Yves

    2014-01-01

    , and sexual dysfunction. Our results show that compared to control subjects with a similar overall age and sex distribution, patients with iRBD experience significantly more problems with gastrointestinal, urinary, and cardiovascular functioning. The most prominent differences in severity of autonomic......Patients with idiopathic REM sleep behavior disorder (iRBD) are at very high risk of developing neurodegenerative synucleinopathies, which are disorders with prominent autonomic dysfunction. Several studies have documented autonomic dysfunction in iRBD, but large-scale assessment of autonomic...

  20. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?

    Science.gov (United States)

    Prendiville, Julie S; Tucker, Lori B; Cabral, David A; Crawford, Richard I

    2004-01-01

    The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic

  1. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon

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    Legakis Nikolaos

    2006-02-01

    Full Text Available Abstract Background Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. Case presentation A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Conclusion Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  2. Idiopathic juxtafoveolar retinal telangiectasis: A current review

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    Nowilaty Sawsan

    2010-01-01

    Full Text Available Idiopathic juxtafoveolar retinal telangiectasis (IJFT, also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities.

  3. Idiopathic Adrenal Hematoma Masquerading as Neoplasm

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    Kazuki Sasaki

    2012-04-01

    Full Text Available We report herein a case of idiopathic adrenal hematoma. A 59-year-old Japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity. The tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland. His chief complaint was weight loss of 8 kg over the previous 6 months. He had no past medical history and took no medications, including no anticoagulants. Laboratory data were almost normal except for a slight elevation of PIVKA-II. The origin of the tumor was found to be the adrenal gland, as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries. A fine needle biopsy of the lesion was unable to confirm the diagnosis. Open right adrenalectomy was performed. The histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue. In the absence of any obvious etiology, the diagnosis was idiopathic adrenal hematoma.

  4. Idiopathic pulmonary fibrosis: current and future directions.

    Science.gov (United States)

    Soo, E; Adamali, H; Edey, A J

    2017-01-21

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and is increasingly recognised. Prior to the advent of effective therapies, achieving an early diagnosis was arguably of little prognostic consequence given IPF was considered an untreatable and uniformly fatal disease. The advent of new drug treatments has given hope for the future and raised the profile of IPF. International management guidelines highlight the critical role of radiology as part of an interstitial lung disease multidisciplinary team approach in reaching an accurate and early diagnosis of IPF. The diagnostic criteria and levels of diagnostic confidence for the radio-pathological pattern associated with the clinical syndrome of IPF, usual interstitial pneumonia (UIP), appear seemingly straightforward; however, with increasing research and recognition of radiopathological interobserver variability, limitations of this classification model are becoming increasingly apparent. This review describes ancillary radiological features, comorbidities, and emerging new entities that potentially co-exist with IPF. Beyond diagnosis radiology is developing as a key prognostic tool to inform longitudinal patient evaluation. These diagnostic and prognostic clinical challenges and the future role of radiology in IPF are discussed.

  5. RADIOFREQUENCY ABLATION OF IDIOPATHIC RIGHT VENTRICULAR TACHYCARDIA

    Institute of Scientific and Technical Information of China (English)

    华伟; JituVohra

    1998-01-01

    This paper presents our experieaee with radioreqencey ablation (RFA) for idiopathic ventricular tschycardia (VT) arising from right ventricle in 12 patients(pts). The age range d patients was 21~50, with a mean of 38. 5 years. Ten out of 12 were females, 1 patient had eandia failure due to almost incessant VT while the rest had normal left ventricular function.Twelve pts had VT arising from the fight ventricle; of those, 9 were from the outflow truct, 2 from the RV apex, and l from the RV inflow. In all tats the diagnostic study and therapeutic RFA were combined in a single procedure, pacemapping and local aetlvition time were used to guide the site of RFA in Ors with VT arising froth the tight ventricle.RFA was successful in 11 of the 12 pts ( 91%). Ntmaher of RF applications were 1~27, mean 9. 6; fluoroscopy time were 4~75, mean 26. 9 minutes. RFA for idiopathic RV has a high success rate. This mode of treament should be considered as a nonphartaaeologieal curative treatment for symptomatic pts.

  6. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    Science.gov (United States)

    Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

    2016-01-01

    Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

  7. Idiopathic membranous nephropathy: an autoimmune disease.

    Science.gov (United States)

    Makker, Sudesh P; Tramontano, Alfonso

    2011-07-01

    For more than 50 years researchers have debated the evidence for an autoimmune basis of human idiopathic membranous nephritis (MN). Work published in the past 2 years has substantially strengthened the belief that MN is indeed an autoimmune disease of the kidney. Autoantibodies of the IgG4 subclass to at least three podocyte membrane proteins including phospholipase A(2)-receptor, aldose reductase, and manganese superoxide dismutase have been detected by immunoblotting in sera as well as in acid eluates prepared from renal biopsy tissue of patients with this disease, using either whole tissue or microdissected glomeruli from frozen sections. In each case the podocyte antigen has been shown to co-localize with the subepithelial glomerular immune deposits in renal tissue of the same patients. It is not certain if any of these podocyte proteins is an inciting/primary autoantigen or whether they are secondary antigens recruited by intermolecular epitope-spreading, initiating from a yet-to-be-discovered autoantigen. Although it is clear that autoantibodies to podocyte membrane proteins are elicited in idiopathic MN and contribute to the formation of the subepithelial deposits, many questions remain concerning the triggers for their development and their contribution toward proteinuria and progression of the disease.

  8. Idiopathic spontaneous pneumomediastinum: radiologic and clinical features

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Young; Kim, Su Young; Kim, Yong Hoon; Hwang, Yoon Joon; Seo, Jung Wook; Han, Yoon Hee; Cha, Soon Joo; Hur, Gham [College of Medicine, Inje Univ., Goyang (Korea, Republic of)

    2004-07-01

    To evaluate the clinical presentations, radiological characteristics, and natural history of healthy adolescents presenting with idiopathic spontaneous pneumomediastinum. We retrospectively reviewed the simple radiographs of 14 consecutive patients (11 males) with spontaneous pneumomediastinum, who were examined over a period of 8 years, and analyzed their clinical history, radiographic findings including distribution, combined subcutaneous emphysema, mediastinal widening, pneumothorax, pleural effusion, and resolving period on follow up chest radiographs. We also obtained CT images of 7 patients for the assessment of additional information. The most common complaint at the time of presentation was chest pain and chest discomfort (8/14), followed by neck discomfort (6/14). The chest radiograph was of diagnostic value in alI cases. The main distribution of the pneumomediastinum was cervical (14/14), upper lung (13/14) and lower lung (6/14). Combined subcutaneous emphysema was observed in 6 patients. However, there were no cases of mediastinal widening, pneumothorax or pleural effusion. Complete resolution of the pneumomediastinum on the radiograph was observed after 10 days (mean 5.6), following purely conservative treatment. There was no additional information on the CT images, as compared with that on the radiographs. Idiopathic spontaneous pneumomediastinum is a benign entity that usually goes undiagnosed, but which responds very weIl to conservative treatment.

  9. Pediatric Idiopathic Intracranial Hypertension: A Case Report

    Directory of Open Access Journals (Sweden)

    Divya Nandwani, OD

    2015-08-01

    Full Text Available Background: Idiopathic intracranial hypertension (IIH presents differently in prepubertal children as compared to postpubertal children and adults. In adults, IIH is most common in obese women of child-bearing age. However, when occurring in prepubertal children, IIH has been shown to have no predilection for gender and does not correlate with obesity. This case report exemplifies the rare occurrence of IIH in a pediatric patient. Case Report: A five-year-old Hispanic female patient was seen at the University Eye Center for a longstanding hypotropia secondary to a likely congenital fourth nerve palsy with an exotropia component. After a strabismus surgery and a series of follow-up visits, bilateral swollen optic nerves were observed, and a prompt referral to the emergency room was made. Consequently, she was diagnosed with IIH. A treatment regimen of acetazolamide was initiated with subsequent improvement of the clinical presentation of increased intracranial pressure, confirmed by the reduction of swelling of her optic nerves. Conclusions: Bilateral swelling of the optic nerves can be an emergency, especially in children. It is important urgently to rule out causes for increased intracranial pressure. If treatment is significantly delayed, or if no treatment is given to a patient with this condition, loss of visual function may occur. In addition to reporting a rare circumstance of pediatric idiopathic intracranial hypertension, this case report serves to remind eye care practitioners of the importance of monitoring the ocular health of patients closely, even in young, healthy children.

  10. Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

    Directory of Open Access Journals (Sweden)

    Mercier Pierre

    2009-08-01

    Full Text Available Abstract Background Adolescent idiopathic scoliosis is characterized by a three-dimensional deviation of the vertebral column and its etiopathogenesis is unknown. Various factors cause idiopathic scoliosis, and among these a prominent role has been attributed to the vestibular system. While the deficits in sensorimotor transformations have been documented in idiopathic scoliosis patients, little attention has been devoted to their capacity to integrate vestibular information for cognitive processing for space perception. Seated idiopathic scoliosis patients and control subjects experienced rotations of different directions and amplitudes in the dark and produced saccades that would reproduce their perceived spatial characteristics of the rotations (vestibular condition. We also controlled for possible alteration of the oculomotor and vestibular systems by measuring the subject's accuracy in producing saccades towards memorized peripheral targets in absence of body rotation and the gain of their vestibulo-ocular reflex. Results Compared to healthy controls, the idiopathic scoliosis patients underestimated the amplitude of their rotations. Moreover, the results revealed that idiopathic scoliosis patients produced accurate saccades to memorized peripheral targets in absence of body rotation and that their vestibulo-ocular reflex gain did not differ from that of control participants. Conclusion Overall, results of the present study demonstrate that idiopathic scoliosis patients have an alteration in cognitive integration of vestibular signals. It is possible that severe spine deformity developed partly due to impaired vestibular information travelling from the cerebellum to the vestibular cortical network or alteration in the cortical mechanisms processing the vestibular signals.

  11. Life-threatening postpartum hemolysis, elevated liver functions tests, low platelets syndrome versus thrombocytopenic purpura - Therapeutic plasma exchange is the answer

    Directory of Open Access Journals (Sweden)

    Prashant Nasa

    2011-01-01

    Full Text Available The differential diagnosis of life-threatening microangiopathic disorders in a postpartum female includes severe preeclampsia-eclampsia, hemolysis, elevated liver functions tests, low platelets syndrome and thrombotic thrombocytopenic purpura. There is considerable overlapping in the clinical and laboratory findings between these conditions, and hence an exact diagnosis may not be always possible. However, there is considerable maternal mortality and morbidity associated with these disorders. This case underlines the complexity of pregnancy-related microangiopathies regarding their differential diagnosis, multiple organ dysfunction and role of therapeutic plasma exchange in their management.

  12. Idiopathic Calcium Nephrolithiasis And Hypercalciuria: The Role Of Genes

    Science.gov (United States)

    Gambaro, Giovanni; Abaterusso, Cataldo

    2007-04-01

    Idiopathic calcium nephrolithiasis and hypercalciuria are multifactorial disease conditions, the pathogenesis of which involves the interaction of environmental and individual factors. Data support a strong role of genes in the pathogenesis of these two conditions. Findings obtained in monogenic disorders characterized by renal calcium stones, and/or hypercalciuria, and/or nephrocalcinosis have proposed a number of genes as candidate genes in the pathogenesis of the common idiopathic calcium nephrolithiasis and hypercalciuria. The physiological role of these genes, and findings in monogenic disorders and idiopathic, multifactorial disorders will be presented.

  13. PReS-FINAL-2148: Rheumates@work a cognitive behavioural internet based intervention promoting physical activity in children with juvenile idiopathic arthritis: Preliminary results of a randomized clinical trail

    NARCIS (Netherlands)

    Bos, J; Armbrust, W.; Geertzen, J.; Sauer, P.; Dijkstra, P.; Van Brussel, M.; Cappon, J.; Lelieveld, O.

    2013-01-01

    Introduction: Juvenile Idiopathic Arthritis (JIA) is a chronic disease in which periods of active inflammation alternate with periods of inactive disease in an unpredictable way. Although impairments are most pronounced in children with disease activity, deficits like fatigue, decreased physical act

  14. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    Background: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term card

  15. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    BACKGROUND: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term card

  16. A murine model for human immune thrombocytopenic purpura and comparative analysis of multiple gene expression in bone marrow and spleen

    Institute of Scientific and Technical Information of China (English)

    Hong Wei; Xinchun Ding; Jiangong Ren; Ka Liu; Pingping Tan; Daquan Li; Runlin Z.Ma

    2008-01-01

    Homeostasis of platelet number in human and other mammals is well maintained for prevention of minor bleeding and for other im-munological functions, but the exact molecular mechanism responsible for immune thrombocytopenic purpura (ITP) has not been fullyunderstood. In an effort to identify genetic factors involved in initiation of platelet production in response to bleeding injury or plateletdestruction, we have successfully generated an animal model of human ITP via intraperitoneal injection of anti-platelet antibody into theBalb/c mouse. Platelet counts were dropped dramatically in animals that received antibody injection within 4 h, maintained at the mini-mum level for a period of 44 h, started to rebound after 48 h, and reached to the maximum at 144 h (6 days). Final homeostasis reached atapproximately 408 h (17 days), following a minor cycle of platelet number fluctuation. Using semi-quantitative RT-PCR, we assessed andcompared mRNA level of CD41, c-myb, c-mpl, caspase-3, caspase-9, GATA-1, and Bcl-xl in bone marrow and spleen. Alteration ofmRNA expression was correlated with the change of platelet level, and an inverse relationship was found for expression of the genes be-tween bone marrow and spleen. No transcription was detectable for any of the seven genes in bone marrow at the time when plateletnumber reached the maximum (144 h). In contrast, mRNA transcripts of the seven genes were found to be at the highest level in spleentissue. This is the first study of simultaneous detection of multiple platelet related genes in a highly reproducible ITP animal model. Ourresults provided the supportive evidence that expression of the above seven genes are more related to negative regulation of plateletnumber in spleen tissue, at least in the model animals.

  17. The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura

    KAUST Repository

    Lancellotti, S.

    2015-08-13

    Congenital thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy, inherited with autosomal recessive mode as a dysfunction or severe deficiency of ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin 1 repeats Nr. 13), caused by mutations in the ADAMTS-13 gene. About 100 mutations of the ADAMTS-13 gene were identified so far, although only a few characterised by in vitro expression studies. A new Asp to Gly homozygous mutation at position 173 of ADAMTS-13 sequence was identified in a family of Romanian origin, with some members affected by clinical signs of TTP. In two male sons, this mutation caused a severe (< 3 %) deficiency of ADAMTS-13 activity and antigen level, associated with periodic thrombocytopenia, haemolytic anaemia and mild mental confusion. Both parents, who are cousins, showed the same mutation in heterozygous form. Expression studies of the mutant ADAMTS-13, performed in HEK293 cells, showed a severe decrease of the enzyme’s activity and secretion, although the protease was detected inside the cells. Molecular dynamics found that in the D173G mutant the interface area between the metalloprotease domain and the disintegrin-like domain significantly decreases during the simulations, while the proline-rich 20 residues linker region (LR, 285–304) between them undergoes extensive conformational changes. Inter-domain contacts are also significantly less conserved in the mutant compared to the wild-type. Both a decrease of the inter-domain contacts along with a substantial conformational rearrangement of LR interfere with the proper maturation and folding of the mutant ADAMTS-13, thus impairing its secretion.

  18. Experience of buffy coat pooling of platelets as a supportive care in thrombocytopenic dengue patients: A prospective study

    Directory of Open Access Journals (Sweden)

    Kabita Chatterjee

    2014-01-01

    Full Text Available Random donor platelet (RDP is not sufficient to improve the platelet count in most thrombocytopenic patients. Single donor platelet (SDP or buffy coat pooled platelet (BCPP are the two choices to provide a full therapeutic dose of platelets. However, there are constraints in the preparation of SDP due to stringent donor selection procedure, time required for procedure, and need of special expensive equipments and kits. BCPP is widely practiced, especially in the European countries, since 1995. In India, we decided to adopt the procedure of buffy coat pooling of platelets, especially for economically backward patients and for emergencies. This study was prospectively conducted from September 2009 to September 2010. A total of 129 units of BCPP [tested prior for viral markers by enzyme-linked immunosorbent assay (ELISA and individual donor nucleic acid amplification test (ID-NAT] were issued to 129 patients suffering from dengue and were included in this study. For comparison between efficacy of SDP and BCCP, patients were divided into two groups of 50 each. The post-transfusion platelet counts of the patients were noted after 2 hours of transfusion for each type of component. The platelet yield varied from 2.5 to 4.4 Χ 10ΉΉ in BCPP samples. The samples analyzed were sterile without any contamination. The different biochemical parameters were analyzed in detail. The observed post-transfusion platelet recovery and corrected count increment (CCI at 1 hour and 24 hours after BCPP transfusion were similar to that after SDP transfusion. Hence, we concluded that BCPP can be a low cost alternative to SDP in the times of emergencies like dengue and non-affordability by the patient for SDP.

  19. Report of a family with idiopathic knuckle pads and review of idiopathic and disease-associated knuckle pads.

    Science.gov (United States)

    Hyman, Charles H; Cohen, Philip R

    2013-05-15

    Knuckle pads are a rare, frequently overlooked, thickening of the skin usually overlying the extensor surface of the proximal interphalangeal joints. They are well- circumscribed, benign lesions that generally do not require treatment. Idiopathic knuckle pads must be differentiated from similar appearing lesions or trauma-induced pseudo-knuckle pads. Knuckle pads have been observed in association with autosomal dominant conditions such as Bart-Pumphrey syndrome, Dupuytren's contracture, Ledderhose disease, and Peyronie's disease. To the best of our knowledge, idiopathic familial knuckle pads have not previously been described in the English language literature. We describe a sister and brother with familial idiopathic knuckle pads with no associated conditions.

  20. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.