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Sample records for antiphospholipid antibody syndrome

  1. Antiphospholipid Antibody and Antiphospholipid Syndrome

    Institute of Scientific and Technical Information of China (English)

    吴竞生

    2008-01-01

    @@ Antiphospholipid antibodies (APA) APA is a big category for all kinds of negative charge phospholipid or lecithin - a protein complex autoantibodies or the same antibody, through its recognition of antigen (target protein) different, and phospholipids or lecithin - protein complex combination of various rely on the interference Phospholipid clotting and anti-coagulation factor, and promote endothelial cells, platelets, complement activation and play a role. APA including lupus anticoagulant(LA) and anticardiolipin antibody (ACA), In addition, there are anti-β2 glycoprotein-I (β2-GPI) antibody, anti-prothrombin (a- PT) antibody, anti-lysophosphatidic acid antibody and anti-phosphatidylserine antibody, and so on. APA as the main target of phospholipid-binding protein, including β2-GPI, prothrombin, annexin, protein C (PC) and protein S (PS), plasminogen, and so on.

  2. Antiphospholipid Antibodies and Antiphospholipid Syndrome during Pregnancy: Diagnostic Concepts

    OpenAIRE

    Roger A. Levy; dos Santos, Flavia Cunha; Guilherme R de Jesús; Nilson R. de Jesús

    2015-01-01

    Antiphospholipid syndrome (APS) comprises of a wide spectrum of clinical and obstetric manifestations linked to the presence of antiphospholipid antibodies (aPL). APS was described in the context of lupus, and later as an isolated syndrome or primary APS. The presence of aPL, especially the lupus anticoagulant test, is associated with adverse pregnancy outcomes, such as fetal death, recurrent early miscarriages, pre-eclampsia, and placental insufficiency, but does not seem to influence infert...

  3. Imaging spectrum of primary antiphospholipid antibody syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Kwon Ha; Won, Jong Jin [Wonkwang University Hospital, Iksan (Korea, Republic of); Ha, Hyun Kwon; Kim, Jung Hoon; Kim, Jeong Gon; Ki, Won Woo; Kim, Pyo Nyun; Lee, Moon Gyu; Auh, Yong Ho [Asan Medical Center, Seoul (Korea, Republic of)

    1998-04-01

    Antiphospholipid antibody syndrome is recognized as one of the most important causes of hypercoagulability. It can be clinically diagnosed if patients have experienced unexplained recurrent venous or arterial thrombosis, recurrent fetal loss, or thrombocytopenia in the presence of circulating autoantibodies to phospholipids, such as anticardiolipin antibody or lupus anticoagulant. Approximately half of all patients with this syndrome do not have associated systemic disease, and their condition is described as primary antiphospholipid antibody syndrome (PAPS). In the remainder, the syndrome is accompanied by systemic lupus erythematosus or other connective tissue diseases, and is known as secondary antiphospholipid syndrome (1). The purpose of this paper is to illustrate the systemic manifestation of PAPS, focusing on the radiological findings of CT, MR and angiography in clinically proven patients. (author). 8 refs., 10 figs.

  4. Antiphospholipid antibody syndrome presenting as transverse myelitis

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    Javvid M Dandroo

    2015-01-01

    Full Text Available The antiphospholipid syndrome (APS is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features, and a variety of other nonthrombotic neurological syndromes. Although the mechanism of neurological involvement in patients with APS is thought to be thrombotic in origin and endothelial dysfunction associated with antiphospholipid antibodies. APS presenting as acute transverse myelitis is very rarely seen with a prevalence rate of 1%. We are describing a foreigner female presenting as acute transverse myelitis which on evaluation proved to be APS induced. So far, very few cases have been reported in literature with APS as etiology.

  5. The antiphospholipid antibody syndrome: a case report

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    Luma HN

    2012-10-01

    Full Text Available Henry Namme Luma,1,2 Marie-Solange Doualla,1,2 Elvis Temfack,1 Servais Albert Fiacre Eloumou Bagnaka,1 Emmanuella Wankie Mankaa,3 Dobgima Fofung41Department of Internal Medicine, Douala General Hospital, Douala, Cameroon; 2Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon; 3Department of Radiology, Douala General Hospital Douala, Cameroon; 4Department of Abdominal Surgery, Daniel Muna Memorial Clinic, Douala, CameroonAbstract: Antiphospholipid antibody syndrome is defined by the presence of thromboembolic complications and/or pregnancy morbidity in the presence of persistently increased titers of antiphospholipid antibodies. Its clinical presentation can be diverse and any organ can be involved, with a current impact in most surgical and medical specialties. The authors present the case of a 43-year-old man who, over a 13-year period of follow-up, presented with thrombosis of the mesenteric vein, inferior vena cava, and axillary and subclavian veins in a setting where diagnostic and therapeutic options are limited and costly. Through this case report, the authors aim to describe the evolution of this complex pathology, which to date has not been described in the authors' milieu – probably because of its challenging diagnosis and the limited treatment options available. The authors conclude that clinicians need to have a high index of suspicion of APS in patients who present with a thrombotic episode – clinicians should investigate for the presence of antiphospholipid antibodies, as early diagnosis may influence the course of the disease. Furthermore, resources for the detection of antiphospholipid antibodies should be made readily available in resource-limited settings. Finally, patient education on the importance of drug compliance, periodic monitoring, and prevention of thrombosis is indispensable, especially as mortality could be associated with the effects of vascular thrombosis and/or the effects

  6. Antiphospholipid Antibodies and Antiphospholipid Syndrome During Pregnancy: Diagnostic Concepts

    Directory of Open Access Journals (Sweden)

    Roger A Levy

    2015-05-01

    Full Text Available Antiphospholipid syndrome comprises of a wide spectrum of clinical and obstetric manifestations linked to the presence of antiphospholipid antibodies. APS was described in the context of lupus and later as an isolated syndrome or primary APS. The Classification Criteria was designed for the definition of APS in epidemiologic and clinical studies is generally misused for clinical diagnostic decisions on an individual basis. The presence of aPL, especially the lupus anticoagulant test, is known to be a recurrence predictor for arterial events and fetal death. When the serologic assays are used it is important that the validated tests for anticardiolipin and anti-beta 2 glycoprotein I and cut-off are employed. Pathogenic mechanisms are various, beyond the inhibition of anticoagulant action of beta 2 glycoprotein I and other natural anticoagulants, including cell-mediated events involving endothelial and dendritic cells, monocytes and platelets. The mechanisms are not self-exclusive and may in fact be related, offering different targets for potential future therapies. During pregnancy a high-risk care setting, following a protocol of tests and prediction of events, is imperative. The relationship of aPL and infertility is not clear, w

  7. Antiphospholipid Antibody Syndrome Presenting with Hemichorea

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    Yezenash Ayalew

    2012-01-01

    Full Text Available A 25-year-old Bangladeshi lady presented to neurology with a three-month history of involuntary movements of her right arm, associated with loss of power. There was progression to the right leg, and she subsequently developed episodes of slurred speech and blurred vision. At the time of presentation, she was 12 weeks pregnant and the symptoms were reported to have started at conception. Past medical history was unremarkable apart from one first trimester miscarriage and there was no significant family history suggestive of a hereditary neurological condition. MRI of the head revealed no abnormalities but serology showed positive antinuclear antibodies (ANAs at a titre of 1/400. Further investigations revealed strongly positive anticardiolipin antibodies (>120 and positive lupus anticoagulant antibodies. The patient had a second miscarriage at 19 weeks gestation strengthening the possibility that the chorea was related to antiphospholipid antibody syndrome and she was started on a reducing dose of Prednisolone 40 mg daily and aspirin 300 mg daily. Six months later, she had complete resolution of neurological symptoms. There are several reports of chorea as a feature of antiphospholipid syndrome, but no clear consensus on underlying pathophysiology.

  8. The investigation of relationship between preeclampsia and antiphospholipid antibody syndrome

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    Mehmet Tayyar

    2013-03-01

    Full Text Available Aim. The aim of this study was evaluate the relationship between preeclampsia and antiphospholipid antibodies. Methods. A total of 116 pregnant women between 20th and 40th weeks of gestation admitted to our department were investigated. 63 of them were allocated our preeclampsia group and 53 of them were allocated our control group. Lupus anticoagulant, anti-cardiolipin antibodies (IG G ve M and antiphosphatidylserine antibodies (IG G ve M were measured. Results. There was no statistical significance between preeclampsia and control group for antiphospholipid antibodies but these were two times higher in preeclamptic group compared to control group. (22.2% in preeclampsia, 11.3% in control group p=0.193. Conclusions. In an unselected population we were not able to demonstrate an association between preeclampsia and antiphospholipid antibody syndrome but antiphospholipid antibody ratio elevated in women with preeclampsia. These findings show that, there is a need for large scale studies.

  9. Antiphospholipid syndrome.

    Science.gov (United States)

    Ruiz-Irastorza, Guillermo; Crowther, Mark; Branch, Ware; Khamashta, Munther A

    2010-10-30

    The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs. PMID:20822807

  10. The obstetric antiphospholipid syndrome

    NARCIS (Netherlands)

    Derksen, R. H. W. M.; de Grootb, Ph. G.

    2008-01-01

    The association of persistent presence of circulating antiphospholipid antibodies and thromboembolic events, (recurrent) pregnancy loss or both is termed antiphospholipid syndrome. Pregnancies in women with the syndrome should be regarded as at high-risk for complications. Optimal management consist

  11. Antiphospholipid syndrome.

    Science.gov (United States)

    George, Diane; Erkan, Doruk

    2009-01-01

    The antiphospholipid syndrome (APS) is an autoimmune systemic disease that is diagnosed when there is vascular thrombosis and/or pregnancy morbidity occurring with persistently positive antiphospholipid antibodies (aPL) (lupus anticoagulant test, anticardiolipin antibodies, and/or anti-beta(2)-glycoprotein I antibodies). Although International APS Classification Criteria have been formulated to provide a uniform approach to APS research, aPL may cause a spectrum of clinical manifestations, some of which are not included in these criteria. The main aPL-related cardiac manifestations include valve abnormalities (vegetations and/or thickening), myocardial infarction (MI), intracardiac thrombi, and myocardial microthrombosis. In this article, we will review the definition, etiopathogenesis, clinical manifestations, diagnosis, and treatment of aPL-related clinical events with emphasis on cardiac manifestations. PMID:19732604

  12. Primary antiphospholipid antibody syndrome with adrenal hemorrhage in a child : a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Lee, Soo Hyun; Kim, Hyun Joo; Yoo, Han Wook; Yoon, Chong Hyun [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-11-01

    Primary antiphospholipid antibody syndrome is a disease that is clinically diagnosed if a patient suffers recurrent thromboses, stroke, recurrent fetal loss, livedo reticularis, and thrombocytopenia, without evidence of systemic lupus erythematosus or other connective diseases. Adrenal hemorrhage in a patient with primary antiphospholipid antibody syndrome is a rarely recognized, but potentially catastrophic disorder. We recently encountered bilateral adrenal hemorrhaging in a child with antiphospholipid antibody syndrome and casem as well as reviewing the literature.

  13. Bilateral adrenal hemorrhage and primary antiphospholipid antibody syndrome

    International Nuclear Information System (INIS)

    Bilateral adrenal hemorrhage is an uncommon entity that is difficult to diagnose given the ambiquity of the clinical signs. Computerized tomography plays a major role in the diagnosis, disclosing enlarged adrenal glands presenting the hyperdense aspect that characterizes this disorders. We present a case of bilateral adrenal hemoorrhage in a patient diagnosed as having primary antiphospholipid antibody syndrome, which is a less common cause of adrenal hemorrhage than those classically reported, such as anticoagulant therapy, sepsis, shock and abdominal injury. (Author) 8 refs

  14. Antiphospholipid antibodies syndrome in ′Stroke in young′.

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    Mehndiratta M

    1999-04-01

    Full Text Available Antiphospholipid antibodies syndrome has emerged as an important entity responsible for stroke in young. Seven cases of young stroke (< 40 years of age with mean age of 30.1 years (age range 25-39 years, 2 males and 5 females, who tested positive for antiphospholipid antibodies are being reported. All subjects had completed strokes. Six had arterial ischaemic and one patient had venous stroke. One patient suffered from four episodes, three ischaemic and one intracerebral haemorrhage. Two patients suffered from foetal loss. Generalised tonic clonic seizures occurred in three patients. Deep vein thrombosis was observed in one case. Thrombocytopenia was not observed in any case. All the patients had elevated anticardiolipin antibodies (aCL IgM or IgG, while Lupus anticoagulant (LA was elevated in 4 cases. Six cases belonged to primary antiphospholipid antibodies syndrome and one to lupus like illness. Oral anticoagulants were administered to maintain a high intensity international normalized ratio (INR. No recurrences were observed during a follow up period of 6-18 months.

  15. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep;

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  16. Antiphospholipid syndrome

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    Pavlović Dragan M.

    2010-01-01

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

  17. Primary antiphospholipid antibody syndrome: neuroradiologic findings in 11 patients

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    Kim, Jung Hoon; Choi, Choong Gon; Choi, Soo Jung; Lee, Ho Kyu; Suh, Dae Chul [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2000-03-01

    To describe the neuroradiologic findings of primary antiphospholipid antibody syndrome (PAPS). During a recent two-year period, abnormally elevated antiphospholipid antibodies were detected in a total of 751 patients. In any cases in which risk factors for stroke were detected - hypertension, diabetes mellitus, hyperlipidemia, smoking, and the presence of SLE or other connective tissue diseases - PAPS was not diagnosed. Neuroradiologic studies were performed in 11 of 32 patients with PAPS. We retrospectively reviewed brain CT (n = 7), MR (n = 8), and cerebral angiography (n = 8) in 11 patients with special attention to the presence of brain parenchymal lesions and cerebral arterial or venous abnormalities. CT or MR findings of PAPS included nonspecific multiple hyper-intensity foci in deep white matter on T2-weighted images (5/11), a large infarct in the territory of the middle cerebral artery (4/11), diffuse cortical atrophy (2/11), focal hemorrhage (2/11), and dural sinus thrombosis (1/11). Angiographic findings were normal (5/8) or reflected either occlusion of a large cerebral artery (2/8) or dural sinus thrombosis (1/8). Neuroradiologic findings of PAPS are nonspecific but in young or middle- aged adults who show the above mentioned CT or MR findings, and in whom risk factors for stroke are not present, the condition should be suspected.

  18. Genetics Home Reference: antiphospholipid syndrome

    Science.gov (United States)

    ... Levin N, Andrade CA, Andreoli L, Chighizola CB, Porter TF, Salmon J, Silver RM, Tincani A, Branch DW. 14th International Congress on Antiphospholipid Antibodies Task Force report on obstetric antiphospholipid syndrome. Autoimmun Rev. 2014 ...

  19. Cutaneous Vasculitis in a Patient with Antiphospholipid Antibody Syndrome.

    Science.gov (United States)

    Sheth, Khushboo; Parke, Ann

    2016-02-01

    Antiphospholipid antibody syndrome (APS) is an acquired thrombophilia, caused by autoantibodies to anticardiolipin (aCL), or antibeta 2 glycoprotein I, or the presence of lupus anticoagulant (LA) in plasma. It is characterized by recurrent venous and/or arterial thrombi and/or pregnancy related morbidities. We present the case of a 52-year-old female with long-standing APS, who developed cutaneous vasculitis following a common cold. Most of the cutaneous manifestations of APS have been found to be thrombotic on histopathology without evidence of perivascular inflammation. Vasculitis is usually seen in APS patients with coexistent Systemic Lupus Erythematosus (SLE). However, our patient had evidence of vasculitis on skin biopsy and did not have SLE. Though rare, this is a disease process which must be considered in patients with primary APS which must be closely monitored for other vasculitic complications of APS, particularly diffuse alveolar hemorrhage. PMID:27024977

  20. An Approach to Differential Diagnosis of Antiphospholipid Antibody Syndrome and Related Conditions

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    Giacomo Emmi

    2014-01-01

    Full Text Available The antiphospholipid antibody syndrome is a systemic, acquired, immune-mediated disorder characterized by episodes of venous, arterial, or microcirculation thrombosis and/or pregnancy abnormalities, associated with the persistent presence of autoantibodies, confirmed at least in two occasions 12 weeks apart, directed to molecular complexes consisting of phospholipids and proteins. Antiphospholipid antibody syndrome should always be considered as a potential diagnosis especially for young patients presenting with a history of thrombotic events, in particular when they occur without any obvious external trigger or any inherited thrombophilic mutation (even if 2006 criteria do not exclude antiphospholipid antibody syndrome in patients with other inherited or acquired prothrombotic conditions, or for women with recurrent pregnancy losses or later fetal deaths. Many other disorders are able to mimic antiphospholipid antibody syndrome, so a broad range of alternative diagnoses should be investigated and ruled out during clinical workup.

  1. Pathogenic role of antiphospholipid antibodies

    NARCIS (Netherlands)

    Salmon, J. E.; de Groot, P. G.

    2008-01-01

    The antiphospholipid antibody syndrome (APS) is characterized by recurrent arterial and venous thrombosis and/or pregnancy in association with antiphospholipid (aPL) antibodies. The pathogenic mechanisms in APS that lead to in vivo injury are incompletely understood. Recent evidence suggests that AP

  2. [Antiphospholipid antibody syndrome in pediatric neurosurgery: a hemostasis problem].

    Science.gov (United States)

    Bocquet, R; Blanot, S; Dautzenberg, M D; Pierre-Kahn, A; Carli, P

    1999-11-01

    The case of a 11-year-old boy under anticoagulant therapy for a familial antiphospholipid antibody syndrome (SAAPF), who underwent surgery for a cerebrovascular malformation responsible for an intracerebral haematoma, is reported. Antivitamins K (AVK) were changed for unfractioned heparin (HNF), three days before. Heparin was discontinued two hours prior to surgery to obtain a normal peroperative coagulation. A vascular dural fistula was removed without any haemostatic problem. The neurological status rapidly returned to normal and tomodensitometry at day 1 showed a normal intracranial status. Heparin was readministered at h 16. Thrombocytopenia occurred at day 4 of heparin treatment. The change for a low weight molecular heparinoid, danaparoid (Orgaran), normalized the platelet count. The platelets aggregation tests were negative during thrombopenia. However, the test for antibodies against the PF4-heparin complex with the Elisa technique, was in favour of a heparin induced thrombocytopenia (TIH). In spite of its anecdotic occurrence due to cumulative thrombotic risks from the association of immunologic disorders (TIH and SAAPF), this case report underlines the value but also the risks of anticoagulant therapy in neurosurgery, when patients are at high risk for thrombosis.

  3. Bilateral Internal Carotid Artery Occlusion Associated with the Antiphospholipid Antibody Syndrome

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    Pria Anand

    2014-03-01

    Full Text Available A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies.

  4. Platelets and the antiphospholipid syndrome

    NARCIS (Netherlands)

    Urbanus, R. T.; Derksen, R. H. W. M.; de Groot, P. G.

    2008-01-01

    The antiphospholipid syndrome is a non-inflammatory autoimmune disease characterised by the presence of antiphospholipid antibodies in the plasma of patients with venous or arterial thrombosis or recurrent complications of pregnancy. The strong relation between the presence of antibodies against ani

  5. The antiphospholipid syndrome: still an enigma

    OpenAIRE

    Chaturvedi, Shruti; McCrae, Keith R

    2015-01-01

    Antiphospholipid syndrome (APS) is defined by clinical manifestations that include thrombosis and/or fetal loss or pregnancy morbidity in patients with antiphospholipid antibodies (aPL). Antiphospholipid antibodies are among the most common causes of acquired thrombophilia, but unlike most of the genetic thrombophilias are associated with both venous and arterial thrombosis. Despite an abundance of clinical and basic research on aPL, a unified mechanism that explains their prothrombotic activ...

  6. Systemic Lupus Erythematosus and Antiphospholipid Syndrome

    OpenAIRE

    Aleksandra Plavsic; Rada Miskovic; Sanvila Raskovic; Mirjana Bogic; Branka Bonaci Nikolic

    2014-01-01

    Antiphospholipid syndrome is an autoimmune disorder defined as association of vascular thrombosis and/or pregnancy complications with presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin and anti-β2 glycoprotein I). It is the most common cause of acquired thrombophilia, and can occur as an independent entity or in relation with other diseases, especially systemic lupus erythematosus. Presence of antiphospholipid syndrome in systemic lupus erythematosus is additional v...

  7. Systemic Lupus Erythematosus and Antiphospholipid Syndrome

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    Aleksandra Plavsic

    2014-09-01

    Full Text Available Antiphospholipid syndrome is an autoimmune disorder defined as association of vascular thrombosis and/or pregnancy complications with presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin and anti-β2 glycoprotein I. It is the most common cause of acquired thrombophilia, and can occur as an independent entity or in relation with other diseases, especially systemic lupus erythematosus. Presence of antiphospholipid syndrome in systemic lupus erythematosus is additional vaso occlusive factor in already present inflammation, bringing further risk for thrombotic events. Clinical and serological manifestations of antiphospholipid syndrome and systemic lupus erythematosus are very similar, so possible connection for these two autoimmune disorders is assumed.

  8. Antibodies to Phosphatidylserine/Prothrombin Complex in Antiphospholipid Syndrome: Analytical and Clinical Perspectives.

    Science.gov (United States)

    Peterson, Lisa K; Willis, Rohan; Harris, E Nigel; Branch, Ware D; Tebo, Anne E

    2016-01-01

    Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by thrombosis and/or pregnancy-related morbidity accompanied by persistently positive antiphospholipid antibodies (aPL). Current laboratory criteria for APS classification recommend testing for lupus anticoagulant as well as IgG and IgM anticardiolipin, and beta-2 glycoprotein I (anti-β2GPI) antibodies. However, there appears to be a subset of patients with classical APS manifestations who test negative for the recommended criteria aPL tests. While acknowledging that such patients may have clinical features that are not of an autoimmune etiology, experts also speculate that these "seronegative" patients may test negative for relevant autoantibodies as a result of a lack of harmonization and/or standardization. Alternatively, they may have aPL that target other antigens involved in the pathogenesis of APS. In the latter, autoantibodies that recognize a phosphatidylserine/prothrombin (PS/PT) complex have been reported to be associated with APS and may have diagnostic relevance. This review highlights analytical and clinical attributes associated with PS/PT antibodies, taking into consideration the performance characteristics of criteria aPL tests in APS with specific recommendations for harmonization and standardization efforts. PMID:26975968

  9. Thrombotic risk assessment in antiphospholipid syndrome: the role of new antibody specificities and thrombin generation assay.

    Science.gov (United States)

    Sciascia, Savino; Baldovino, Simone; Schreiber, Karen; Solfietti, Laura; Radin, Massimo; Cuadrado, Maria J; Menegatti, Elisa; Erkan, Doruk; Roccatello, Dario

    2016-01-01

    Antiphospholipid syndrome (APS) is an autoimmune condition characterized by the presence of antiphospholipid antibodies (aPL) in subjects presenting with thrombosis and/or pregnancy loss. The currently used classification criteria were updated in the international consensus held in Sidney in 2005. Vascular events seem to result of local procoagulative alterations upon triggers influence (the so called "second-hit theory"), while placental thrombosis and complement activation seem to lead to pregnancy morbidity. The laboratory tests suggested by the current classification criteria include lupus anticoagulant, a functional coagulation assay, and anticardiolipin and anti-β2-glycoprotein-I antibodies, generally detected by solid phase enzyme-linked immunosorbent assay. The real challenge for treating physicians is understanding what is the actual weight of aPL in provoking clinical manifestations in each case. As thrombosis has a multi-factorial cause, each patient needs a risk-stratified approach. In this review we discuss the role of thrombotic risk assessment in primary and secondary prevention of venous and arterial thromboembolic disease in patients with APS, focusing on new antibody specificities, available risk scoring models and new coagulation assays. PMID:27429595

  10. Catastrophic Antiphospholipid Syndrome.

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    El-Shereef, Rawhya R; El-Abedin, Zein; Abdel Aziz, Rashad; Talat, Ibrahim; Saleh, Mohammed; Abdel-Samia, Hanna; Sameh, Amro; Sharha, Mahmoud

    2016-01-01

    This paper reports one case of successfully treated patients suffering from a rare entity, the catastrophic antiphospholipid syndrome (CAPS). Management of this patient is discussed in detail. PMID:27375916

  11. Catastrophic Antiphospholipid Syndrome

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    Rawhya R. El-Shereef

    2016-01-01

    Full Text Available This paper reports one case of successfully treated patients suffering from a rare entity, the catastrophic antiphospholipid syndrome (CAPS. Management of this patient is discussed in detail.

  12. Catastrophic primary antiphospholipid syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Byun, Joo Nam [Chosun University Hospital, Gwangju (Korea, Republic of); Ryu, Sang Wan [Miraero21 Medical Center, Gwangju (Korea, Republic of)

    2006-09-15

    Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distension rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction.

  13. CEREBRAL SINUS THROMBOSIS IN A CASE OF ANTI-PHOSPHOLIPID ANTIBODY SYNDROME WITHOUT ASSOCIATED CONNECTIVE TISSUE INVOLVEMENT

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    Biswarup

    2014-12-01

    Full Text Available : The association of antiphospholipid antibodies with vascular thrombotic episodes is well established. In absence of other connective tissue disease such an association is very rare & known as the primary antiphospholipid antibody syndrome. Cerebral venous sinus thrombosis is associated with hypercoaguable states and a number of immune-mediated conditions. However the report of cerebral venous sinus thrombosis with antiphospholipid antibodies alone is limited. Here a case presenting with painful bilateral ophthalmoplegia with bilateral optic disc edema (due to raised intra cranial tension showing positive lupus anticoagulant in serum and right central venous sinus (transverse and sigmoid thrombosis on MRI and MR venogram is reported which showed clinical improvement with anticoagulant therapy

  14. Antiphospholipid Antibodies and Systemic Scleroderma

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    Awa Oumar Touré

    2013-03-01

    Full Text Available Objective: Antiphospholipid antibodies (APLs could be associated with an increased risk of vascular pathologies in systemic scleroderma. The aim of our study was to search for APLs in patients affected by systemic scleroderma and to evaluate their involvement in the clinical manifestations of this disease. Materials and Methods: We conducted a cross-sectional descriptive study, from January 2009 until August 2010, with patients received at the Department of Dermatology (Dakar, Senegal. Blood samples were taken at the hematology laboratory and were analyzed for the presence of APLs. Results: Forty patients were recruited. Various types of either isolated or associated APLs were found in 23 patients, i.e. 57.5% of the study population. The most frequently encountered antibody was IgG anti-β2 GPI (37.5% of the patients, followed by anticardiolipins (17.5% and lupus anticoagulants (5%. No statistically significant association of positive antiphospholipid-related tests to any of the scleroderma complications could be demonstrated. Conclusion: A high proportion of patients showing association of systemic scleroderma and APLs suggests the presence of a morbid correlation between these 2 pathologies. It would be useful to follow a cohort of patients affected by systemic scleroderma in order to monitor vascular complications following confirmation of the presence of antiphospholipid syndrome.

  15. Platelt receptors involved in the antiphospholipid syndrome

    NARCIS (Netherlands)

    Pennings, M.T.T.

    2007-01-01

    The antiphospholipid syndrome (APS) is a non-inflammatory autoimmune disease clinically characterized by the occurrence of either venous or arterial thrombosis or the presence of specific pregnancy complications. Serological criteria are the persistent presence of antibodies directed against cardiol

  16. Lupus anticoagulants and antiphospholipid antibodies

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000547.htm Lupus anticoagulants and antiphospholipid antibodies To use the sharing features on this page, please enable JavaScript. Lupus anticoagulants are antibodies against substances in the lining ...

  17. Systemic lupus erythematosis with antiphospholipid antibody syndrome: A mimic of Buerger′s disease

    Directory of Open Access Journals (Sweden)

    Vasugi Zoya

    2006-01-01

    Full Text Available This case report is about a past smoker who presented with history of recurrent ulcers and digital gangrene with claudication pain of the left foot for the past fifteen years. Clinical examination and angiogram showed disease involving the peripheral vessels of lowervlimb. This patient had been labeled as Buerger′s disease 15 years ago based on clinical and demographic profile of the illness. We felt that the progression of the disease despite the patient having stopped smoking 15 years ago along with the presence of elevated inflammatory markers in the blood with proteinuria was not in keeping with the nature of the disease. Furthur evaluation revealed that the patient had systemic lupus erythematosus with antiphospholipid antibody syndrome. This case highlights the need for a careful search for diseases, which can mimic Buerger′s disease in young smokers who present with peripheral vascular disease and who have an atypical clinical presentation or progression.

  18. Anticardiolipine antibodies in skin and muscle eluates of patients with primary and secondary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Z S Alekberova

    2004-01-01

    Full Text Available Objective. To detect anticardiolipin antibodies (АСА, anti-p2-GPl antibodies, C3 and C4 complement components in immune complexes including those containing АСА in skin and muscle eluates of pts with systemic lupus erythematosus (SLE and antiphospholipid syndrome (APS. Material and methods . In 7 pts (6 female and I male, 2 with primary APS, 3 with SLE+APS and 2 with SLE skin and muscle biopsies were taken. 6 from 7 pts had thrombotic complications. Eluates were obtained from frozen skin and skeletal muscle biopsies (size was 1,5x0,5 and 0,5x0,5 respectively. Because of small size of biopsies it was not possible to use traditional methods of tissue pounding such as sharp homogenization of tissues in homogenizers with pulverizing and subsequent process of freezing-unfreezing which lead to large protein loss and make impossible serological tissue analysis. Application of acid eluates method by T.E.W. Feltkamp and J,H. Boode of own modification allowed to minimize tissue protein loss and perform serological tissue analysis. Results. Serum of all 7 pts contained antiphospholipid antibodies - IgG-ACA in 3, combination of IgG- und IgM-ACA in 5. In 5 from 7 eluates lgG АСА exceeded 0,109 OO units were revealed. They contained СЗ, C4 and different protein products mostly immunoglobulines. Anti-(I2GP1 antiboddie;. were absent. Conclusion. For the first time presence of АСА in tissues of APS pts was showed which may be of particular interest in studying morphogenesis of local tissue disturbances with participation of immune complexes containing АСА.

  19. Origin and pathogenesis of antiphospholipid antibodies

    Directory of Open Access Journals (Sweden)

    C.M. Celli

    1998-06-01

    Full Text Available Antiphospholipid antibodies (aPL are a heterogeneous group of antibodies that are detected in the serum of patients with a variety of conditions, including autoimmune (systemic lupus erythematosus, infectious (syphilis, AIDS and lymphoproliferative disorders (paraproteinemia, myeloma, lymphocytic leukemias. Thrombosis, thrombocytopenia, recurrent fetal loss and other clinical complications are currently associated with a subgroup of aPL designating the antiphospholipid syndrome. In contrast, aPL from patients with infectious disorders are not associated with any clinical manifestation. These findings led to increased interest in the origin and pathogenesis of aPL. Here we present the clinical features of the antiphospholipid syndrome and review the origin of aPL, the characteristics of experimentally induced aPL and their historical background. Within this context, we discuss the most probable pathogenic mechanisms induced by these antibodies.

  20. Catastrophic antiphospholipid syndrome presenting as fever of unknown origin

    Directory of Open Access Journals (Sweden)

    Fatma I Al-Beladi

    2012-01-01

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

  1. Antiphospholipid Syndrome and Kidney Involvement: New Insights

    Directory of Open Access Journals (Sweden)

    José A. Martínez-Flores

    2016-07-01

    Full Text Available Antiphospholipid syndrome is an autoimmune disorder characterized by vascular thromboses and pregnancy morbidity associated with antiphospholipid antibodies: lupus anticoagulant, IgG or IgM anticardiolipin or anti-beta 2-glycoprotein I. The kidney is one of the major target organs in antiphospholipid syndrome (APS. However, beyond the known involvement of the kidney in primary and associated APS, we may be observing a new form of APS within the context of renal failure. This review describes the classical kidney manifestations of APS and provides new considerations to be taken into account.

  2. The effects of lupus and antiphospholipid antibody syndrome on foetal outcomes.

    Science.gov (United States)

    Nalli, C; Iodice, A; Andreoli, L; Lojacono, A; Motta, M; Fazzi, E; Tincani, A

    2014-05-01

    Systemic lupus erythematosus (SLE) is a multi-organ autoimmune disease that primarily affects women of childbearing-age. Antiphospholipid syndrome (APS) is a systemic autoimmune disorder defined by the occurrence of venous and arterial thrombosis, often multiple, and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). Recently, the long-term outcome of children born to patients with lupus and APS has become a major topic of interest both to patients and physicians. One of the major problems related to maternal disease is preterm delivery with all the consequences that this condition may bring. Prematurity may also be due to the presence of aPL; however, aPL do not generally display any thrombotic potential on neonates. Another complication may be neonatal lupus (NL), mediated by the presence of maternal antibodies (anti-Ro/SSA and anti-La/SSB). In addition, behaviour and neuropsychological outcomes have also been a matter of interest, but there are currently few data available. Beyond the biological influence of both maternal disease and autoimmune background, it is important to focus on the possible influence of maternal chronic illness on the neuropsychological development of her children. Whether aPL exposure could have a direct effect on brain development is still being debated. In children of mothers with APS, language delays have been noted and learning disabilities were described with a higher rate than the general age-school population. Several studies were performed on children born to lupus mothers: even if maternal lupus does not seem to impair intelligence levels, it may increase the occurrence of learning disabilities and particularly dyslexia in male children. To the best of our knowledge, no studies are available on the long-term outcome of children born to mothers with lupus or APS and particularly regarding the development of autoimmune diseases. Nevertheless, common experience of experts in the field is that these children do

  3. Antiphospholipid syndrome: A case study

    Energy Technology Data Exchange (ETDEWEB)

    Davies, T. [Royal Adelaide Hospital, Adelaide, SA (Australia). Department of Nuclear Medicine

    1998-03-01

    Full text: A forty-two-year-old male presented to the Royal Adelaide Hospital with symptoms of increasing shortness of breath, swelling in both ankles, petechial rash and blood in his sputum. Initial investigations showed cardiomegaly, right ventricular hypertrophy, patchy lung infiltrates, a platelet count of 1500 and a clotting time of 60 seconds. A V/Q scan indicated a high probability of pulmonary embolism. Further investigations showed that the patient was positive for lupus anticoagulant and cardiolipin antibodies. A diagnosis of primary antiphospholipid syndrome was made. The patient``s high risk of strokes and hemorrhaging prompted investigation by a {sup 99}mTc-HMPAO brain scan. Further V/Q scans were performed to follow up the initial finding of multiple pulmonary embolism and a R-L shunt study was performed to investigate a left subclavian murmur. The patient was admitted for four weeks and began treatment which included cyclaphosphamide, corticosteroids and plasmaphoresis and was discharged when stable. Over the next six months he was re admitted three times for relapse of antiphospholipid syndrome. On his fourth admission he collapsed and died five hours after admission. Cause of death was due to cardiac arrhythmia secondary to severe right ventricular hypertrophy and dilation. The effects of antiphospholipid syndrome was believed to be responsible for this outcome.

  4. Antiphospholipid antibody syndrome: the flow cytometric annexin A5 competition assay as a diagnostic tool.

    Science.gov (United States)

    Tomer, A; Bar-Lev, S; Fleisher, S; Shenkman, B; Friger, M; Abu-Shakra, M

    2007-10-01

    The mechanism underlying hypercoagulability in antiphospholipid antibody syndrome (APS) is uncertain. Here, we present a flow-cytometric assay (FCA) based on the hypothesis that anti-platelet-anionic-phospholipid autoantibodies (aPL) interfere with the activity of the natural anticoagulant protein annexin A5, thereby accelerating platelet procoagulant activity. This study assessed the clinical utility of the feasible FCA, which demonstrates the competition of the patient's aPL with the binding of annexin A5 to the platelet-anionic-phospholipids, in the diagnosis of APS. Sixty-two (94%) of 66 APS patients, 20 (51%) of 39 patients with systemic lupus erythematosus and two (4%) of 49 healthy individuals were positive by FCA. Compared with the anticardiolipin (aCL) assay, the relative sensitivity was 82% and the specificity 73.3%. However, 19 (25%) aCL-negative patients were positive by FCA; 12 were positive for lupus-anticoagulant (LA). Compared with LA assay, the relative sensitivity was 85% and the specificity 72.2%. However, 21 (26%) LA-negative patients were FCA-positive, 12 were positive for aCL. The FCA was particularly sensitive for APS patients with arterial (97.0%) and gestational vascular complications (100%) with overall sensitivity of 95% and specificity of 97%. Our findings suggest that the FCA is practical, sensitive and specific for the detection of clinically relevant aPL in the diagnosis of APS.

  5. Pulmonary hypertension in antiphospholipid syndrome.

    Science.gov (United States)

    Zuily, Stéphane; Wahl, Denis

    2015-01-01

    Pulmonary hypertension (PH) is a rare but life-threatening condition in antiphospholipid syndrome (APS) patients with or without systemic lupus erythematosus (SLE). The definition of PH is based on hemodynamic parameters estimated by transthoracic echocardiography (TTE) and confirmed by right heart catheterization (RHC). New evidence suggests that antiphospholipid antibodies (aPL) in SLE patients increase the risk of PH; however, studies yield conflicting results. Hypotheses regarding the impact of aPL on PH include large vessel and microvascular thrombosis, and endothelial remodeling. Natural history of PH is progressive worsening mainly due to recurrent pulmonary embolism. The management in APS patients includes anticoagulation; patients undergoing pulmonary endarterectomy need to be closely monitored because of an increased risk of thrombotic complications. PMID:25604574

  6. Impaired Fibrinolysis in the Antiphospholipid Syndrome

    OpenAIRE

    Krone, Katie A.; Allen, Kristi L.; McCrae, Keith R

    2010-01-01

    The antiphospholipid syndrome (APS) is characterized by venous and/or arterial thrombosis, or recurrent fetal loss, in the presence of antiphospholipid antibodies (APL). The pathogenesis of APS is multifaceted and involves numerous mechanisms including activation of endothelial cells, monocytes, and/or platelets; inhibition of natural anticoagulant pathways such as protein C, tissue factor inhibitor, and annexin A5; activation of the complement system; and impairment of the fibrinolytic syste...

  7. Post-surgical hemorrhagic infarction of the adrenal gland as the first clinical manifestation of antiphospholipid syndrome after 43 years of antibody-positivity.

    Science.gov (United States)

    Haselboeck, Johanna; Ringl, Helmut; Mueller, Catharina; Pabinger, Ingrid; Winkler, Stefan

    2013-11-01

    We report on a male patient who tested positive for antiphospholipid antibodies for 43 years without thromboembolic manifestation of antiphospholipid syndrome (APS). He has been followed up in a prospective cohort study since 2001. Following his second hip replacement surgery, the patient developed acute adrenal failure due to bilateral hemorrhagic infarction. Prophylactic anticoagulation, surgery, or an immunological reaction to blood transfusion may have triggered this late and unusually located primary manifestation of APS in our patient.

  8. β2GP1, Anti-β2GP1 Antibodies and Platelets: Key Players in the Antiphospholipid Syndrome

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    Yik C. Ho

    2016-05-01

    Full Text Available Anti-beta 2 glycoprotein 1 (anti-β2GP1 antibodies are commonly found in patients with autoimmune diseases such as the antiphospholipid syndrome (APS and systemic lupus erythematosus (SLE. Their presence is highly associated with increased risk of vascular thrombosis and/or recurrent pregnancy-related complications. Although they are a subtype of anti-phospholipid (APL antibody, anti-β2GP1 antibodies form complexes with β2GP1 before binding to different receptors associated with anionic phospholipids on structures such as platelets and endothelial cells. β2GP1 consists of five short consensus repeat termed “sushi” domains. It has three interchangeable conformations with a cryptic epitope at domain 1 within the molecule. Anti-β2GP1 antibodies against this cryptic epitope are referred to as ‘type A’ antibodies, and have been suggested to be more strongly associated with both vascular and obstetric complications. In contrast, ‘type B’ antibodies, directed against other domains of β2GP1, are more likely to be benign antibodies found in asymptomatic patients and healthy individuals. Although the interactions between anti-β2GP1 antibodies, β2GP1, and platelets have been investigated, the actual targeted metabolic pathway(s and/or receptor(s involved remain to be clearly elucidated. This review will discuss the current understanding of the interaction between anti-β2GP1 antibodies and β2GP1, with platelet receptors and associated signalling pathways.

  9. A Novel Dimeric Inhibitor Targeting Beta2GPI in Beta2GPI/Antibody Complexes Implicated in Antiphospholipid Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    A Kolyada; C Lee; A De Biasio; N Beglova

    2011-12-31

    {beta}2GPI is a major antigen for autoantibodies associated with antiphospholipid syndrome (APS), an autoimmune disease characterized by thrombosis and recurrent pregnancy loss. Only the dimeric form of {beta}2GPI generated by anti-{beta}2GPI antibodies is pathologically important, in contrast to monomeric {beta}2GPI which is abundant in plasma. We created a dimeric inhibitor, A1-A1, to selectively target {beta}2GPI in {beta}2GPI/antibody complexes. To make this inhibitor, we isolated the first ligand-binding module from ApoER2 (A1) and connected two A1 modules with a flexible linker. A1-A1 interferes with two pathologically important interactions in APS, the binding of {beta}2GPI/antibody complexes with anionic phospholipids and ApoER2. We compared the efficiency of A1-A1 to monomeric A1 for inhibition of the binding of {beta}2GPI/antibody complexes to anionic phospholipids. We tested the inhibition of {beta}2GPI present in human serum, {beta}2GPI purified from human plasma and the individual domain V of {beta}2GPI. We demonstrated that when {beta}2GPI/antibody complexes are formed, A1-A1 is much more effective than A1 in inhibition of the binding of {beta}2GPI to cardiolipin, regardless of the source of {beta}2GPI. Similarly, A1-A1 strongly inhibits the binding of dimerized domain V of {beta}2GPI to cardiolipin compared to the monomeric A1 inhibitor. In the absence of anti-{beta}2GPI antibodies, both A1-A1 and A1 only weakly inhibit the binding of pathologically inactive monomeric {beta}2GPI to cardiolipin. Our results suggest that the approach of using a dimeric inhibitor to block {beta}2GPI in the pathological multivalent {beta}2GPI/antibody complexes holds significant promise. The novel inhibitor A1-A1 may be a starting point in the development of an effective therapeutic for antiphospholipid syndrome.

  10. A novel dimeric inhibitor targeting Beta2GPI in Beta2GPI/antibody complexes implicated in antiphospholipid syndrome.

    Directory of Open Access Journals (Sweden)

    Alexey Kolyada

    Full Text Available BACKGROUND: β2GPI is a major antigen for autoantibodies associated with antiphospholipid syndrome (APS, an autoimmune disease characterized by thrombosis and recurrent pregnancy loss. Only the dimeric form of β2GPI generated by anti-β2GPI antibodies is pathologically important, in contrast to monomeric β2GPI which is abundant in plasma. PRINCIPAL FINDINGS: We created a dimeric inhibitor, A1-A1, to selectively target β2GPI in β2GPI/antibody complexes. To make this inhibitor, we isolated the first ligand-binding module from ApoER2 (A1 and connected two A1 modules with a flexible linker. A1-A1 interferes with two pathologically important interactions in APS, the binding of β2GPI/antibody complexes with anionic phospholipids and ApoER2. We compared the efficiency of A1-A1 to monomeric A1 for inhibition of the binding of β2GPI/antibody complexes to anionic phospholipids. We tested the inhibition of β2GPI present in human serum, β2GPI purified from human plasma and the individual domain V of β2GPI. We demonstrated that when β2GPI/antibody complexes are formed, A1-A1 is much more effective than A1 in inhibition of the binding of β2GPI to cardiolipin, regardless of the source of β2GPI. Similarly, A1-A1 strongly inhibits the binding of dimerized domain V of β2GPI to cardiolipin compared to the monomeric A1 inhibitor. In the absence of anti-β2GPI antibodies, both A1-A1 and A1 only weakly inhibit the binding of pathologically inactive monomeric β2GPI to cardiolipin. CONCLUSIONS: Our results suggest that the approach of using a dimeric inhibitor to block β2GPI in the pathological multivalent β2GPI/antibody complexes holds significant promise. The novel inhibitor A1-A1 may be a starting point in the development of an effective therapeutic for antiphospholipid syndrome.

  11. Valvular heart disease in antiphospholipid syndrome.

    Science.gov (United States)

    Zuily, Stéphane; Huttin, Olivier; Mohamed, Shirine; Marie, Pierre-Yves; Selton-Suty, Christine; Wahl, Denis

    2013-04-01

    Heart valve disease (HVD) is the most frequent cardiac manifestation in patients with antiphospholipid syndrome (APS), with prevalence of 30 %. The definition is based on the presence of thickening or vegetation of the valves (mainly mitral and aortic) as described by Libman and Sacks for patients with systemic lupus erythematosus (SLE). Transthoracic and/or transoesophageal echocardiography (TTE and TEE, respectively) enable early and accurate diagnosis and help avoid misdiagnosis as rheumatic valve disease. The presence of antiphospholipid antibodies (aPL) in SLE patients is associated with a threefold greater risk of HVD, confirming the crucial importance of these antibodies in the pathogenic process, leading to thrombotic manifestations on valves because of hypercoagulability. Natural history is characterized by worsening of HVD over time with an increased risk for stroke. APS patients undergoing valve-replacement surgery are at high risk of thrombotic and bleeding complications. Thus aPL-associated HVD has affects clinical management of APS patients. PMID:23456852

  12. The antiphospholipid syndrome: still an enigma.

    Science.gov (United States)

    Chaturvedi, Shruti; McCrae, Keith R

    2015-01-01

    Antiphospholipid syndrome (APS) is defined by clinical manifestations that include thrombosis and/or fetal loss or pregnancy morbidity in patients with antiphospholipid antibodies (aPL). Antiphospholipid antibodies are among the most common causes of acquired thrombophilia, but unlike most of the genetic thrombophilias are associated with both venous and arterial thrombosis. Despite an abundance of clinical and basic research on aPL, a unified mechanism that explains their prothrombotic activity has not been defined; this may reflect the heterogeneity of aPL and/or the fact that they may influence multiple pro- and/or antithrombotic pathways. Antiphospholipid antibodies are directed primarily toward phospholipid binding proteins rather than phospholipid per se, with the most common antigenic target being β2-glycoprotein 1 (β2GPI) although antibodies against other targets such as prothrombin are well described. Laboratory diagnosis of aPL depends upon the detection of a lupus anticoagulant (LA), which prolongs phospholipid-dependent anticoagulation tests, and/or anticardiolipin and anti-β2-glycoprotein 1 antibodies. Indefinite anticoagulation remains the mainstay of therapy for thrombotic APS, although new strategies that may improve outcomes are emerging. Preliminary reports suggest caution in the use of direct oral anticoagulants in patients with APS-associated thrombosis. Based on somewhat limited evidence, aspirin and low molecular weight heparin are recommended for obstetrical APS. There remains a pressing need for better understanding of the pathogenesis of APS in humans, for identification of clinical and laboratory parameters that define patients at greatest risk for APS-related events, and for targeted treatment of this common yet enigmatic disorder. PMID:26637701

  13. Antibody profile of pregnant women with antiphospholipid syndrome and pregnancy outcome after treatment with low dose aspirin and low-weight-molecular heparin.

    Science.gov (United States)

    Glasnović, Marija; Bosnjak, Ivica; Vcev, Aleksandar; Soldo, Ivan; Kosuta, Maja; Lenz, Bahrija; Glasnović-Horvatić, Elizabeta; Soldo-Butković, Silva; Mićunović, Nikola

    2007-03-01

    The aim of the research was to show our diagnostic and therapeutic experience with antiphospholipid syndrome (APS) in pregnant women. 36 pregnant women suspect on APS were included in the study: 32 with primary antiphospholipd syndrome (PAPS) and 4 with secondary antiphospholipid syndrome (SAPS). All pregnant women received low-molecular-weight-heparin (LMWH) and low dose aspirin (LDA) therapy. Control group represented 26 women with SAPS and previous bad reproductive anamnesis. Average pregnancy lasted 37.06 +/- 0.707 weeks. LMWH and LDA therapy was successful in 97.22%. Lupus anticoagulant (LA) was found to be more frequent in PAPS group (71.87%). Anticardiolipin antibodies (aCL) were found to be more frequent in SAPS (26.66%). For three patients (3.37%), PAPS was diagnosed due to a fact that they had positive antibeta2-glycoproteinl (antibeta-GP1). To make APS diagnosis, it is of great importance to search for all antiphospholipid antibodies. LMWH and low dose of acetylsalicylic acid should be the first choice therapy.

  14. Antiphospholipid antibodies in Brazilian hepatitis C virus carriers

    Directory of Open Access Journals (Sweden)

    A.M. Atta

    2008-06-01

    Full Text Available Hepatitis C, a worldwide viral infection, is an important health problem in Brazil. The virus causes chronic infection, provoking B lymphocyte dysfunction, as represented by cryoglobulinemia, non-organ-specific autoantibody production, and non-Hodgkin's lymphoma. The aim of this research was to screen for the presence of antiphospholipid autoantibodies in 109 Brazilian hepatitis C virus carriers without clinical history of antiphospholipid syndrome. Forty healthy individuals were used as the control group. IgA, IgG, and IgM antibodies against cardiolipin and β2-glycoprotein I were measured with an enzyme-linked immunosorbent assay, using a cut-off point of either 20 UPL or 20 SBU. While 24 (22.0% hepatitis C carriers had moderate titers of IgM anticardiolipin antibodies (median, 22.5 MPL; 95%CI: 21.5-25.4 MPL, only three carriers (<3% had IgG anticardiolipin antibodies (median, 23 GPL; 95%CI: 20.5-25.5 GPL. Furthermore, IgA anticardiolipin antibodies were not detected in these individuals. Male gender and IgM anticardiolipin seropositivity were associated in the hepatitis C group (P = 0.0004. IgA anti-β2-glycoprotein-I antibodies were detected in 29 of 109 (27.0% hepatitis C carriers (median, 41 SAU; 95%CI: 52.7-103.9 SAU. Twenty patients (18.0% had IgM anti-β2-glycoprotein I antibodies (median, 27.6 SMU; 95%CI: 23.3-70.3 SMU, while two patients had IgG antibodies against this protein (titers, 33 and 78 SGU. Antiphospholipid antibodies were detected in only one healthy individual, who was seropositive for IgM anticardiolipin. We concluded that Brazilian individuals chronically infected with hepatitis C virus present a significant production of antiphospholipid antibodies, mainly IgA anti-β2-glycoprotein I antibodies, which are not associated with clinical manifestations of antiphospholipid syndrome.

  15. Postpartum spontaneous colonic perforation due to antiphospholipid syndrome

    OpenAIRE

    Ahmed, Kamran; Darakhshan, Amir; Au, Eleanor; Khamashta, Munther A; Katsoulis, Iraklis E

    2009-01-01

    The antiphospholipid syndrome (APS) is a multi-systemic disease being characterized by the presence of antiphospholipid antibodies that involves both arterial and venous systems resulting in arterial or venous thrombosis, fetal loss, thrombocytopenia, leg ulcers, livedo reticularis, chorea, and migraine. We document a previously unreported case of a 37-year-old female in whom APS was first manifested by infarction and cecal perforation following cesarean section. At laparotomy the underlying ...

  16. Complement and thrombosis in the antiphospholipid syndrome.

    Science.gov (United States)

    Oku, Kenji; Nakamura, Hiroyuki; Kono, Michihiro; Ohmura, Kazumasa; Kato, Masaru; Bohgaki, Toshiyuki; Horita, Tetsuya; Yasuda, Shinsuke; Amengual, Olga; Atsumi, Tatsuya

    2016-10-01

    The involvement of complement activation in the pathophysiology of antiphospholipid syndrome (APS) was first reported in murine models of antiphospholipid antibody (aPL)-related pregnancy morbidities. We previously reported that complement activation is prevalent and may function as a source of procoagulant cell activation in the sera of APS patients. Recently, autoantibodies against C1q, a component of complement 1, were reported to be correlated with complement activation in systemic lupus erythematosus. These antibodies target neoepitopes of deformed C1q bound to various molecules (i.e., anionic phospholipids) and induce accelerated complement activation. We found that anti-C1q antibodies are more frequently detected in primary APS patients than in control patients and in refractory APS patients with repeated thrombotic events. The titer of anti-C1q antibodies was significantly higher in refractory APS patients than in APS patients without flare. The binding of C1q to anionic phospholipids may be associated with the surge in complement activation in patients with anti-C1q antibodies when triggered by 'second-hit' biological stressors such as infection. Such stressors will induce overexpression of anionic phospholipids, with subsequent increases in deformed C1q that is targeted by anti-C1q antibodies.

  17. Antibodies to endothelial cells and to beta 2-glycoprotein I in the antiphospholipid syndrome: prevalence and isotype distribution.

    Science.gov (United States)

    Navarro, M; Cervera, R; Teixidó, M; Reverter, J C; Font, J; López-Soto, A; Monteagudo, J; Escolar, G; Ingelmo, M

    1996-06-01

    The aim of this study was to analyse the prevalence and isotype distribution of antibodies to endothelial cells (aEC) and to beta 2-glycoprotein I (a beta 2GPI) in the antiphospholipid syndrome (APS). Fifteen patients with an APS [nine associated with systemic lupus erythematosus (SLE) and six "primary'] and 15 with SLE without an APS were prospectively studied. The aEC were determined by an enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein and the a beta 2GPI by ELISA using highly purified beta 2GPI. A positive titre of aEC was detected in 20 out of 30 patients (67%), but in none of the control group. Ten patients had both IgG and IgM isotypes, five had IgG only and five had only IgM. Thirteen patients with the APS (87%) were found to have a positive titre of aEC, while only seven with SLE but without a history of APS (47%) had aEC (P < 0.05). Nine patients with the APS (60%) had a positive titre of a beta 2GPI (four had both IgG and IgM isotypes, one had IgG only and four had only IgM), while none of the patients without an APS (0%) had these antibodies (P < 0.001). A significant association was also found between the presence of aPL and aEC (P < 0.05), as well as between aPL and a beta 2GPI (P < 0.001). Both aEC and a beta 2GPI can be found in the APS. This reinforces the theory that APS represents a complex autoimmune disorder in which several autoantibodies co-exist with aPL.

  18. The IgM isotype of anti-annexin A5 antibodies and multiple positivity of conventional antiphospholipid antibodies: increasing the number of clinical manifestations of primary antiphospholipid syndrome.

    Science.gov (United States)

    Bećarević, Mirjana; Stojanović, Ljudmila; Ignjatović, Svetlana; Dopsaj, Violeta

    2016-05-01

    We evaluated the importance of anti-annexin A5 antibodies (aanxA5 Abs) for clinical (thrombosis and/or recurrent pregnancy loss) and serologic (presence of antiphospholipid antibodies: lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2 glycoprotein I (aβ2GPI) antibodies) features of patients with primary antiphospholipid syndrome (PAPS). Our study included 70 patients with PAPS according to the international consensus criteria for APS. The mean age of the analyzed patients was 45.97 ± 12.72. The disease duration above 5 years was present in 31/70 of patients. Concentrations of analyzed antibodies were measured by ELISA. Cutoff values were set in accordance to the manufacturers' recommendations. History of recurrent pregnancy loss was associated with double positivity for aanxA5 IgM and LA (χ (2) = 4.000, P = 0.046) and triple positivity for aanxA5 IgM + LA + aβ2GPI IgM (χ (2) = 4.168, P = 0.041). Venous thromboses were associated with triple positivity for aanxA5 IgM + aCLIgG + aβ2GPI IgM (χ (2) = 3.965, P = 0.046). The IgG isotype of aanxA5 Abs was in positive correlation with aCL Abs of the IgG (r = 0.310, P = 0.009) and IgM (r = 0.254, P = 0.034) isotype. The presence of the clinical manifestations of PAPS is increasing with the number of positive conventional aPL and the IgM aanxA5 Abs tests. This new combination of Abs is beneficial even when the number of patients with positivity for aanxA5 Abs is low. This is important in further detection of patients prone to recurrence of thrombotic episodes. PMID:26971791

  19. Non-traumatic carotid dissection and stroke associated with anti-phospholipid antibody syndrome: Report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    Kluger Benzi

    2008-01-01

    Full Text Available Young adults with stroke frequently do not have any of the traditional risk factors associated with stroke, prompting a search for other mechanical and hypercoagulable causes. The authors report a young man presenting with stroke and subsequently diagnosed with a carotid dissection. Recurrent strokes while on heparin prompted a search for a second etiology and the patient was found to have antiphospholipid antibody syndrome. Although these conditions may be coincidental, we propose that their interaction was significant in this patient′s presentation. Other reports of this association will also be reviewed.

  20. Impaired Fibrinolysis in the Antiphospholipid Syndrome

    Science.gov (United States)

    Krone, Katie A.; Allen, Kristi L.; McCrae, Keith R.

    2010-01-01

    The antiphospholipid syndrome (APS) is characterized by venous and/or arterial thrombosis, or recurrent fetal loss, in the presence of antiphospholipid antibodies (APL). The pathogenesis of APS is multifaceted and involves numerous mechanisms including activation of endothelial cells, monocytes, and/or platelets; inhibition of natural anticoagulant pathways such as protein C, tissue factor inhibitor, and annexin A5; activation of the complement system; and impairment of the fibrinolytic system. Fibrinolysis—the process by which fibrin thrombi are remodeled and degraded—involves the conversion of plasminogen to plasmin by tissue plasminogen activator (tPA) or urokinase-type plasminogen activator, and is tightly regulated. Although the role of altered fibrinolysis in patients with APS is relatively understudied, several reports suggest that deficient fibrinolytic activity may contribute to the pathogenesis of disease in these patients. This article discusses the function of the fibrinolytic system and reviews studies that have reported alterations in fibrinolytic pathways that may contribute to thrombosis in patients with APL. Some of these mechanisms include elevations in plasminogen activator inhibitor-1 levels, inhibitory antibodies against tPA or other components of the fibrinolytic system, antibodies against annexin A2, and finally, antibodies to β2-glycoprotein-I (β2GPI) that block the ability of β2GPI to stimulate tPA-mediated plasminogen activation. PMID:20425534

  1. Discrimination of a lupus anticoagulant caused by antiphospholipid antibodies or rivaroxaban using taipan venom time

    NARCIS (Netherlands)

    Van Os, G.M.; De Laat, B.; Kamphuisen, P.W.; Meijers, J.C.; de Groot, P.G.

    2011-01-01

    The antiphospholipid syndrome (APS) is an autoimmune disease associated with the presence of antiphospholipid antibodies (APL) and the occurrence of thrombosis and pregnancy complications. One of the assays to detect APL is based on the prolongation of phospho-lipid dependent coagulation assays caus

  2. Mechanisms of Disease : antiphospholipid antibodies - from clinical association to pathologic mechanism

    NARCIS (Netherlands)

    de Laat, Bas; Mertens, Koen; de Groot, Philip G.

    2008-01-01

    The discovery that antiphospholipid antibodies recognize plasma proteins that bind to phospholipids rather than recognizing phospholipids themselves has been a major advance in research into antiphospholipid syndrome (APS). It is now established that beta(2)-glycoprotein I (beta(2)GPI) is the most i

  3. Antiphospholipid Antibodies in Lupus Nephritis.

    Science.gov (United States)

    Parodis, Ioannis; Arnaud, Laurent; Gerhardsson, Jakob; Zickert, Agneta; Sundelin, Birgitta; Malmström, Vivianne; Svenungsson, Elisabet; Gunnarsson, Iva

    2016-01-01

    Lupus nephritis (LN) is a major manifestation of systemic lupus erythematosus (SLE). It remains unclear whether antiphospholipid antibodies (aPL) alter the course of LN. We thus investigated the impact of aPL on short-term and long-term renal outcomes in patients with LN. We assessed levels of aPL cross-sectionally in SLE patients diagnosed with (n = 204) or without (n = 294) LN, and prospectively in 64 patients with active biopsy-proven LN (52 proliferative, 12 membranous), before and after induction treatment (short-term outcomes). Long-term renal outcome in the prospective LN cohort was determined by the estimated glomerular filtration rate (eGFR) and the Chronic Kidney Disease (CKD) stage, after a median follow-up of 11.3 years (range: 3.3-18.8). Cross-sectional analysis revealed no association between LN and IgG/IgM anticardiolipin or anti-β2-glycoprotein I antibodies, or lupus anticoagulant. Both aPL positivity and levels were similar in patients with active LN and non-renal SLE. Following induction treatment for LN, serum IgG/IgM aPL levels decreased in responders (p<0.005 for all), but not in non-responders. Both at active LN and post-treatment, patients with IgG, but not IgM, aPL had higher creatinine levels compared with patients without IgG aPL. Neither aPL positivity nor levels were associated with changes in eGFR from either baseline or post-treatment through long-term follow-up. Moreover, aPL positivity and levels both at baseline and post-treatment were similar in patients with a CKD stage ≥3 versus 1-2 at the last follow-up. In conclusion, neither aPL positivity nor levels were found to be associated with the occurrence of LN in SLE patients. However, IgG aPL positivity in LN patients was associated with a short-term impairment of the renal function while no effect on long-term renal outcome was observed. Furthermore, IgG and IgM aPL levels decreased following induction treatment only in responders, indicating that aPL levels are affected by

  4. Antiphospholipid Antibodies in Lupus Nephritis.

    Directory of Open Access Journals (Sweden)

    Ioannis Parodis

    Full Text Available Lupus nephritis (LN is a major manifestation of systemic lupus erythematosus (SLE. It remains unclear whether antiphospholipid antibodies (aPL alter the course of LN. We thus investigated the impact of aPL on short-term and long-term renal outcomes in patients with LN. We assessed levels of aPL cross-sectionally in SLE patients diagnosed with (n = 204 or without (n = 294 LN, and prospectively in 64 patients with active biopsy-proven LN (52 proliferative, 12 membranous, before and after induction treatment (short-term outcomes. Long-term renal outcome in the prospective LN cohort was determined by the estimated glomerular filtration rate (eGFR and the Chronic Kidney Disease (CKD stage, after a median follow-up of 11.3 years (range: 3.3-18.8. Cross-sectional analysis revealed no association between LN and IgG/IgM anticardiolipin or anti-β2-glycoprotein I antibodies, or lupus anticoagulant. Both aPL positivity and levels were similar in patients with active LN and non-renal SLE. Following induction treatment for LN, serum IgG/IgM aPL levels decreased in responders (p<0.005 for all, but not in non-responders. Both at active LN and post-treatment, patients with IgG, but not IgM, aPL had higher creatinine levels compared with patients without IgG aPL. Neither aPL positivity nor levels were associated with changes in eGFR from either baseline or post-treatment through long-term follow-up. Moreover, aPL positivity and levels both at baseline and post-treatment were similar in patients with a CKD stage ≥3 versus 1-2 at the last follow-up. In conclusion, neither aPL positivity nor levels were found to be associated with the occurrence of LN in SLE patients. However, IgG aPL positivity in LN patients was associated with a short-term impairment of the renal function while no effect on long-term renal outcome was observed. Furthermore, IgG and IgM aPL levels decreased following induction treatment only in responders, indicating that aPL levels are

  5. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients : Report of a Task Force at the 13th International Congress on Antiphospholipid Antibodies

    NARCIS (Netherlands)

    Ruiz-Irastorza, G.; Cuadrado, M. J.; Ruiz-Arruza, I.; Brey, R.; Crowther, M.; Derksen, R.; Erkan, D.; Krilis, S.; Machin, S.; Pengo, V.; Pierangeli, S.; Tektonidou, M.; Khamashta, M.

    2011-01-01

    The antiphospholipid syndrome (APS) is defined by the presence of thrombosis and/or pregnancy morbidity in combination with the persistent presence of circulating antiphospholipid antibodies: lupus anticoagulant, anticardiolipin antibodies and/or anti-beta 2-glycoprotein I antibodies in medium to hi

  6. Development of cerebral venous sinus thrombosis in an aplastic anemia patient with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    CHEN Jian-hua; YOU Xin; QIAN Min

    2010-01-01

    @@ Aplastic anemia (AA) is an acquired disorder in which bone marrow fails to produce or release sufficient amounts of blood cell. Antiphospholipid syndrome (APS)is an autoimmune disease characterised by recurrent arterial or venous thrombosis, pregnancy morbidity and the persistence of positive antiphospholipid antibodies (aPL), including anticardiolipin antibody (ACL) and lupus anticoagulant (LA).

  7. Evaluation of the diagnostic potential of antibodies to beta2-glycoprotein 1 domain 1 in Chinese patients with antiphospholipid syndrome.

    Science.gov (United States)

    Zhang, Shulan; Wu, Ziyan; Chen, Si; Li, Jing; Wen, Xiaoting; Li, Liubing; Zhang, Wen; Zhao, Jiuliang; Zhang, Fengchun; Li, Yongzhe

    2016-01-01

    In this study, we evaluated the clinical performance of anti-β2-glycoprotein 1 domain 1 antibodies (aβ2GP1-D1) in the diagnosis of antiphospholipid syndrome (APS). Sera from 229 subjects were tested, including 35 patients with primary APS, 51 patients with APS associated to other diseases, 30 patients with non-APS thrombosis, 32 patients with non-APS pregnancy-related morbidity, 42 patients with systemic lupus erythematosus, and 39 healthy controls (HC). Serum IgG aβ2GP1-D1, IgG/IgM anti-cardiolipin (aCL) and IgG/IgM aβ2GP1 were measured by a chemiluminescence assay. The levels of IgG aβ2GP1-D1 were significantly increased in patients with APS, compared with disease controls and HCs (p < 0.001). Significant correlation was identified between IgG aβ2GP1-D1 and IgG aβ2GP1 (p < 0.0001), indicating IgG aβ2GP1-D1 were the predominant domain-specific antibodies in IgG aβ2GP1 family. Importantly, aβ2GP1-D1, but not aβ2GP1 non-D1, was significantly correlated with thrombotic events. Interestingly, no significant correlation between IgG aβ2GP1-D1 and obstetric complications was observed. Additionally, significantly higher levels of IgG aβ2GP1-D1 were found in patients with triple aPL positivity, compared with patients with double and single aPL positivity. Our findings suggest a potential role of IgG aβ2GP1-D1 in identifying APS patients with high risk of thrombosis, shedding insight on the introduction of IgG aβ2GP1-D1 in China. PMID:27053361

  8. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy.

    Science.gov (United States)

    Chaturvedi, Swasti; Brandao, Leonardo; Geary, Denis; Licht, Christoph

    2011-06-01

    Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.

  9. CARDIAC TRANSPLANTATION IN YOUNG PATIENT WITH DILATED CARDIOMYOPATHY AND SECONDARY ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    E. V. Shlyakhto

    2013-01-01

    Full Text Available Patients with congestive heart failure have an increased incidence of thromboembolic events. The choice of me- dical management in patients with antiphospholipid antibodies and generalized thrombosis due to hypercoagula- bility is complex issue. We report heart transplant outcome in 15 years old patient with dilated cardiomyopathy and secondary anti-phospholipid syndrome

  10. Apheresis and intravenous immunoglobulins used in addition to conventional therapy to treat high-risk pregnant antiphospholipid antibody syndrome patients. A prospective study.

    Science.gov (United States)

    Ruffatti, Amelia; Favaro, Maria; Hoxha, Ariela; Zambon, Alessandra; Marson, Piero; Del Ross, Teresa; Calligaro, Antonia; Tonello, Marta; Nardelli, Giovanni B

    2016-06-01

    Pregnant women with triple antibody positive antiphospholipid syndrome (APS) who have had thrombosis or a history of early, severe pregnancy complications are generally considered at high risk of pregnancy loss. The objectives of this study were to investigate the efficacy and safety of a relatively new treatment protocol used in addition to conventional therapy in high-risk pregnant patients affected with primary APS. The study's two inclusion criteria were: (1) the presence of triple antiphospholipid positivity, (2) previous thrombosis and/or a history of one or more early, severe pregnancy complications. Eighteen pregnancies occurring between 2002 and 2015 in 14 APS patients, (mean age 34.8±3.6 SD) were monitored. All 14 (100%) patients had triple antiphospholipid positivity. In addition, six of them (42.8%) had a history of thrombosis, four (28.6%) had one or more previous early and severe pregnancy complications, and four (30.8%) met both clinical study criteria. The study protocol included weekly plasmapheresis or immunoadsorption and fortnightly 1g/kg intravenous immunoglobulins. Seventeen of the pregnancies (94.4%) produced live neonates, all born between the 26th and 37th weeks of gestation (mean 33.1±3.5 SD). One female (5.5%), born prematurely at 24 weeks, died of sepsis a week after birth. There were two cases (11.1%) of severe pregnancy complications. No treatment side effects were registered. Given the high live birth rate and the safety associated to it, the study protocol described here could be taken into consideration by medical teams treating high-risk APS pregnant patients. PMID:27088752

  11. Clinical Application of Revised Laboratory Classification Criteria for Antiphospholipid Antibody Syndrome: Is the Follow-Up Interval of 12 Weeks Instead of 6 Weeks Significantly Useful?

    Directory of Open Access Journals (Sweden)

    Sang Hyuk Park

    2016-01-01

    Full Text Available Background. According to revised classification criteria of true antiphospholipid antibody syndrome, at least one of three antiphospholipid antibodies should be present on two or more occasions at least 12 weeks apart. However, it can be inconvenient to perform follow-up tests with interval of 12 weeks. We investigated clinical application of follow-up tests with interval of 12 weeks. Method. Totals of 67, 199, and 332 patients tested positive initially for the lupus anticoagulants confirm, the anti-β2 glycoprotein-I antibody, and the anti-cardiolipin antibody test, respectively, from Jan 2007 to Jul 2009. We investigated clinical symptoms of patients, follow-up interval, and results of each test. Results. Among patients with initial test positive, 1.5%–8.5% were subjected to follow-up tests at interval of more than 12 weeks. Among 25 patients with negative conversion in tests, patients with interval of more than 12 weeks showed clinical symptom positivity of 33.3%, which was higher than that of 12.5% with 6–12 weeks. Among 34 patients with persistent test positive, clinical symptoms positivity trended to be more evident in patients at interval of 6–12 weeks (47.4% versus 26.7%, P=0.191 than more than 12 weeks. Conclusion. Less than 10% of patients with initial test positive had follow-up tests at interval of more than 12 weeks and the patients with persistent test positive at interval of more than 12 weeks showed trends toward having lower clinical symptoms than 6–12 weeks. More research is needed focused on the evidence that follow-up test at interval of more than 12 weeks should be performed instead of 6 weeks.

  12. Prevention of fetal loss in experimental antiphospholipid syndrome by in vivo administration of recombinant interleukin-3.

    OpenAIRE

    Fishman, P; Falach-Vaknine, E; Zigelman, R; Bakimer, R; Sredni, B.; Djaldetti, M; Shoenfeld, Y

    1993-01-01

    Antiphospholipid antibodies are strongly associated with arterial and venous thrombosis and with fetal loss. Recently an experimental model for antiphospholipid syndrome (APLS) was established in our laboratory. In this model, mice are immunized passively or actively with anticardiolipin antibodies and acquire the syndrome, which is characterized by prolonged activated partial thromboplastin time (APTT), thrombocytopenia, low fecundity rate, and fetal loss. In a normal process of pregnancy, l...

  13. Antiphospholipid antibody: laboratory, pathogenesis and clinical manifestations

    Directory of Open Access Journals (Sweden)

    T. Ziglioli

    2011-06-01

    Full Text Available Antiphospholipid antibodies (aPL represent a heterogeneous group of antibodies that recognize various antigenic targets including beta2 glycoprotein I (β2GPI, prothrombin (PT, activated protein C, tissue plasminogen activator, plasmin and annexin A2. The most commonly used tests to detect aPL are: lupus anticoagulant (LAC, a functional coagulation assay, anticardiolipin antibody (aCL and anti-β2GPI antibody (anti-β2GPI, which are enzyme-linked immunoassay (ELISA. Clinically aPL are associated with thrombosis and/or with pregnancy morbidity. Apparently aPL alone are unable to induce thrombotic manifestations, but they increase the risk of vascular events that can occur in the presence of another thrombophilic condition; on the other hand obstetrical manifestations were shown to be associated not only to thrombosis but mainly to a direct antibody effect on the trophoblast.

  14. Successful treatment of post-exertion acute myocardial infarction by primary angioplasty and stenting in a patient with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Musuraca, Gerardo; Imperadore, Ferdinando; Terraneo, Clotilde; De Girolamo, Piergiuseppe; Cemin, Claudio; Bonmassari, Roberto; Vergara, Giuseppe

    2004-01-01

    Antiphospholipid syndrome is a disorder characterized by arterial and venous thromboses, thrombocytopaenia and stroke. Acute myocardial infarction is rarely associated with this syndrome. The treatment of these patients is a clinical challenge. This report is about a patient with antiphospholipid syndrome presenting with an acute myocardial infarction after an exercise test. The infarct-related coronary artery was successfully revascularized by primary angioplasty and stenting without any major bleeding complications. We think that the physical exertion could have favoured acute coronary thrombosis in this particular setting.

  15. [Hepatic hemorrhagic infarction in eclampsia and HELLP Syndrome associated with the antiphospholipid syndrome].

    Science.gov (United States)

    Enriquez, R; Gutierrez, A; Sirvent, A E; Saez, J; Palacios, F; Cabezuelo, J B

    1999-01-01

    A 33 year-old woman developed eclampsia with HELLP syndrome. Laboratory results revealed lupus anticoagulant and anticardiolipin antibodies. Imaging tests showed liver and spleen infarctions. The patients was given enoxaparin and supportive care and there was a good evolution. We discuss some aspects about liver infarction and its association with toxemia of pregnancy and the antiphospholipid syndrome.

  16. Anticorpos antinucleossomo e síndrome antifosfolipídica: estudo observacional Antinucleosome antibodies and primary antiphospholipid syndrome: an observational study

    Directory of Open Access Journals (Sweden)

    Alexandre Wagner Silva de Souza

    2012-06-01

    Full Text Available OBJETIVO: Avaliar a associação entre a presença de anticorpos antinucleossomo (anti-NCS e a síndrome antifosfolipídica primária (SAFP e o posterior desenvolvimento de lúpus eritematoso sistêmico (LES. MATERIAIS E MÉTODOS: Trinta e seis mulheres com o diagnóstico de SAFP foram avaliadas prospectivamente para manifestações de doenças reumáticas autoimunes e para a presença de anticorpos antifosfolípides, anticorpos antinucleares e anti-NCS/cromatina. RESULTADOS: Após um período médio de seguimento de 45,7 meses, anticorpos anti-NCS/cromatina foram detectados em apenas uma paciente (2,8%, que desenvolveu manifestações de LES tais como poliartrite, linfopenia, neurite óptica, lesões compatíveis com esclerose múltipla em substância branca cerebral e perfil de autoanticorpos altamente sugestivo de LES. CONCLUSÃO: A frequência de anticorpos anti-NCS/cromatina é baixa em pacientes com SAFP, e sua presença pode associar-se ao desenvolvimento de manifestações de LES.OBJECTIVE: To study the association of anti-nucleosome (anti-NCS antibodies in primary antiphospholipid syndrome (APS and the development of systemic lupus erythematosus (SLE during follow-up. MATERIALS AND METHODS: Thirty-six women with primary APS were evaluated prospectively for clinical features of systemic autoimmune diseases and for the presence of antiphospholipid antibodies, antinuclear antibodies and anti-NCS/chromatin antibodies. RESULTS: After a mean follow-up period of 45.7 months, anti-NCS/chromatin antibodies were detected in only one patient (2.8%, who developed features of SLE including polyarthritis, lymphopenia, optic neuritis, multiple sclerosis-like lesions, and an autoantibody profile suggestive of SLE. CONCLUSION: The frequency of anti-NCS/chromatin antibodies in primary APS patients is very low, and they may be associated with the development of SLE manifestations.

  17. Twenty-two years of failure to set up undisputed assays to detect patients with the antiphospholipid syndrome

    NARCIS (Netherlands)

    de Groot, Philip G.; Derksen, Ronald H. W. M.; de Laat, Bas

    2008-01-01

    The antiphospholipid syndrome is defined by the persistent presence of antiphospholipid antibodies in plasma of patients with a history of thrombosis and/or pregnancy morbidity. From the definition in 1985 onwards, confusion has arisen concerning who has the syndrome and who has not. Although the cl

  18. Genome-Wide Association Study of Antiphospholipid Antibodies

    Directory of Open Access Journals (Sweden)

    M. Ilyas Kamboh

    2013-01-01

    Full Text Available Background. The persistent presence of antiphospholipid antibodies (APA may lead to the development of primary or secondary antiphospholipid syndrome. Although the genetic basis of APA has been suggested, the identity of the underlying genes is largely unknown. In this study, we have performed a genome-wide association study (GWAS in an effort to identify susceptibility loci/genes for three main APA: anticardiolipin antibodies (ACL, lupus anticoagulant (LAC, and anti-β2 glycoprotein I antibodies (anti-β2GPI. Methods. DNA samples were genotyped using the Affymetrix 6.0 array containing 906,600 single-nucleotide polymorphisms (SNPs. Association of SNPs with the antibody status (positive/negative was tested using logistic regression under the additive model. Results. We have identified a number of suggestive novel loci with P

  19. Hemolysis, hemorrhage, headache, and hidden abortion: imaging findings in antiphospholipid syndrome

    International Nuclear Information System (INIS)

    Antiphospholipid antibodies are associated with arterial and venous thromboses, recurrent pregnancy loss, and organ infarction. Any vascular region can be affected. We present a 20-year-old woman suffering from secondary antiphospholipid syndrome with a unique combination of multifocal venous thromboses, pulmonary embolism, spontaneous abortion, and splenic infarction. Diversity of clinical symptoms and diagnostic imaging modalities are discussed with emphasis on cross-sectional imaging. The syndrome should be suspected in patients with thromboses and organ infarctions of otherwise undetermined etiology. (orig.)

  20. Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

    Science.gov (United States)

    Yelnik, Cécile M; Kozora, Elizabeth; Appenzeller, Simone

    2016-02-01

    Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases. PMID:26923254

  1. Anti-beta2 glycoprotein 1 and the anti-phospholipid syndrome.

    LENUS (Irish Health Repository)

    Keane, Pearse A

    2012-02-03

    PURPOSE: To describe a patient who presented with bilateral retinal vascular occlusion and the use of anti-beta2 glycoprotein 1 (GPI) antibody testing in the diagnosis of antiphospholipid syndrome. DESIGN: Observational case report. METHODS: Hematological investigations were performed on a 49-year-old man who presented with rapid onset of bilateral severe central retinal vein occlusion. RESULTS: Lupus anticoagulant and anticardiolipin antibody testing was negative. Markedly raised titers of anti-beta2 GPI antibodies were detected on two separate occasions. CONCLUSIONS: The raised titers of anti-beta2 GPI antibodies were considered to strongly suggest an underlying diagnosis of the antiphospholipid syndrome.

  2. Proliferative glomerulonephritis and primary antiphospholipid syndrome

    International Nuclear Information System (INIS)

    Little is known regarding the association of primary antiphospholipid syndrome (APLS) and proliferative glomerulonephiritis (GN). We describe a biopsy-documented case with primary APLS and proliferative (GN) with no evidence of thrombotic microangiopathy (TMA), and in the absence of other manifestations of systematic lupus erythematosus (SLE). She presented initially with left popliteal deep venous thrombosis and nephrotic syndrome. Her first pregnancy at the age of 26 years resulted in the intra-uterine fetal death at term. Two subsequent pregnancies ended up with miscarriages at 3 and 4 months of gestation. Urinalysis revealed glomerular red blood cells of 1.0000.000/ml and granular cast; proteinuria of 13.4grams/24 hours, which was non-selective; hemoglobin 12 gm/dl, normal white blood cell and platelets; serum albumin 2.6gm/dl; anti-nuclear antibody (ANA) and anti DNA were negative and complement levels normal. Lupus anticoagulant was positive leading to a diagnosis of primary APLS. The biopsy findings were consistent with membranoproliferative GN. She continued to have steroid-resistant proteinuria, but stable renal function after a 12-year follow up period. She had 2 pregnancies during this period and was delivered at term using caesarian section. She received heparin during the pregnancies. Later she developed hypertension easily controlled by atenolol. This case provides evidence that primary APLS can be associated with proliferative GN due to immune deposits and not only TMA as previously reported, and in the complete absence of SLE. Performing more renal biopsies in this group of patients may disclose a greater prevalence of proleferative GN and may help in devising a rationale for treatment. (author)

  3. Endothelial cell activation by antiphospholipid antibodies is modulated by Krüppel-like transcription factors

    OpenAIRE

    Allen, Kristi L.; Hamik, Anne; Jain, Mukesh K.; McCrae, Keith R

    2011-01-01

    Antiphospholipid syndrome is characterized by thrombosis and/or recurrent pregnancy loss in the presence of antiphospholipid antibodies (APLAs). The majority of APLAs are directed against phospholipid-binding proteins, particularly β2-glycoprotein I (β2GPI). Anti-β2GPI antibodies activate endothelial cells in a β2GPI-dependent manner through a pathway that involves NF-κB. Krüppel-like factors (KLFs) play a critical role in regulating the endothelial response to inflammatory stimuli. We hypoth...

  4. 'Criteria' aPL tests : Report of a Task Force and preconference workshop at the 13th International Congress on Antiphospholipid Antibodies, Galveston, Texas, April 2010

    NARCIS (Netherlands)

    Pierangeli, S. S.; de Groot, P. G.; Dlott, J.; Favaloro, E.; Harris, E. N.; Lakos, G.; Ortel, T.; Meroni, P. L.; Otomo, K.; Pengo, V.; Tincani, A.; Wong, R.; Roubey, R.

    2011-01-01

    Current classification criteria for definite antiphospholipid syndrome (APS) mandate the use of one or more of three positive 'standardized' laboratory assays to detect antiphospholipid antibodies (aPL) (viz: anticardiolipin [aCL] IgG and IgM; anti-beta(2)glycoprotein I [anti-beta(2)GPI] antibodies

  5. Avaliação da pesquisa de anticorpos antifosfolipídios para o diagnóstico da síndrome antifosfolípide Evaluation of antiphospholipid antibodies testing for the diagnosis of antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Paula Gonçalves Perches

    2009-06-01

    June 2006. RESULTS: 106 participants (mean age of 42.2 ± 14.1 years at inclusion and female gender in 84% of patients were included in the study. The prevalence of thrombosis was 17.9% (19/106 patients and pregnancy morbidity was 12.3% (13/106 patients. The antiphospholipid syndrome (APS was confirmed in 23.6% (25/106 patients, and it was primary in 68% (17/25 patients and secondary in 32% (8/25 patients. The ACL antibodies were found in 97.1% (103/106 and LAC in 11.4% (5/44 of the serum samples tested. IgM, IgG and IgA ACL isotypes were respectively found in 100%, 23.3% and in 4.9% of these ACL positive sera. For APS diagnosis the sensitivity of IgM ACL was 92% and its specificity was 1.2%, while IgG ACL had a sensitivity of 40% and specificity of 82.5%. The absence of IgG ACL had a high negative predictive value for APS diagnosis (81.4%.The analysis of the Receiver Operating Characteristic (ROC curve showed larger area under the curve for ACL IgG and LAC. CONCLUSION: In a random sample of individuals with positive antiphospholipid antibodies, IgG ACL and LAC showed a larger specificity for APS diagnosis which had been characterized by a higher prevalence of thrombosis.

  6. New developments in lupus-associated antiphospholipid syndrome

    NARCIS (Netherlands)

    Lockshin, M. D.; Derksen, R. H. W. M.

    2008-01-01

    Systemic lupus erythematosus is the disease in which the antiphospholipid syndrome was first described more than 20 years ago and which is the most frequent underlying disorder in secondary antiphospholipid syndrome. With respect to pathogenic concepts and treatment, the subjects of this review, no

  7. Basilar artery thrombosis in the setting of antiphospholipid syndrome.

    Science.gov (United States)

    Saad, Amin F; Nickell, Larry T; Heithaus, R Evans; Shamim, Sadat A; Opatowsky, Michael J; Layton, Kennith F

    2014-07-01

    Antiphospholipid syndrome is an autoimmune disorder characterized by arterial or venous thrombosis, recurrent first-trimester pregnancy loss, and multiple additional clinical manifestations. We describe a man with severe atherosclerotic basilar artery stenosis and superimposed in situ thrombus who was found to have antiphospholipid syndrome.

  8. Basilar artery thrombosis in the setting of antiphospholipid syndrome

    OpenAIRE

    Saad, Amin F.; Nickell, Larry T.; Heithaus, R. Evans; Shamim, Sadat A.; Opatowsky, Michael J.; Layton, Kennith F.

    2014-01-01

    Antiphospholipid syndrome is an autoimmune disorder characterized by arterial or venous thrombosis, recurrent first-trimester pregnancy loss, and multiple additional clinical manifestations. We describe a man with severe atherosclerotic basilar artery stenosis and superimposed in situ thrombus who was found to have antiphospholipid syndrome.

  9. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies.

    Science.gov (United States)

    Schreiber, K

    2016-04-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from the PROMISSE cohort will hopefully identify serological biomarkers, possibly genes, and in addition, give valuable information about underlying disease mechanisms. PMID:26811370

  10. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies.

    Science.gov (United States)

    Schreiber, K

    2016-04-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from the PROMISSE cohort will hopefully identify serological biomarkers, possibly genes, and in addition, give valuable information about underlying disease mechanisms.

  11. Dyspnoea in-patient with antiphospholipid syndrome; case Presentation and literature revision

    International Nuclear Information System (INIS)

    The antiphospholipid syndrome (AFS) is characterized by vascular thrombosis and/or pregnancy morbidity associated to presence of antiphospholipid antibodies (anticardiolipin and/or lupus anticoagulant), This syndrome may occur alone primary -PAFS-) or in association with systemic lupus erythematosus (secondary -SAFS, Both primary and secondary AFS can be responsible for systemic manifestations other than vascular, among them pulmonary, Here is presented a patient with dyspnoea due to pulmonary hypertension (PHT) and interstitial pulmonary disease (IPD), in whom a diagnostic of SAFS was done, The pulmonary manifestations of the AFS are revised including pulmonary thromboembolism, PHT, IPD, pulmonary hemorrhage, and adult respiratory syndrome with multisystemic failure

  12. Cerebrospinal fluid and serum antiphospholipid antibodies in multiple sclerosis, Guillain-Barré syndrome and systemic lupus arythematosus

    Directory of Open Access Journals (Sweden)

    Paulo E. Marchiorji

    1990-12-01

    Full Text Available Immuneglobulins isotypes (IgG and IgM for myelin basic protein (MBP, cerebrosides (CER, gangliosides (GANG and cardiolipin (CARD were detected in the cerebrospinal fluid (CSF from 33 patients with multiple sclerosis (MS, 18 with Guillain-Barré syndrome (GBS and 30 with systemic lupus erythematosus (SLE. In MS patients occurred positive and significant levels of IgG-MBP in 51,5% (p<0.05 and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels foi IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p<0.05, 53% for IgM-MBP. 3&.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long -as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p<0.05 Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD. only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphosphohoid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.

  13. Genetic aspects of the antiphospholipid syndrome: An update.

    Science.gov (United States)

    Sebastiani, Gian Domenico; Iuliano, Annamaria; Cantarini, Luca; Galeazzi, Mauro

    2016-05-01

    Studies on the immunogenetic predisposition to antiphospholipid syndrome (APS) and on other non-genetic and epigenetic factors are summarised and discussed. Family studies suggest a genetic predisposition to APS. It appears that this genetic predisposition is in part accounted for by the HLA system, the most consistent associations being those with DR4 and DRw53. Furthermore, it appears that lupus anticoagulant (LA) and anticardiolipin (aCL) antibodies are both associated with the same HLA antigens. Population studies suggest that HLA genes have a role in conferring susceptibility to develop primary APS, with some differences in different ethnic groups. Other genes, outside the MHC, give their contribution to the development of this autoimmune syndrome, such as IRF5, STAT4 and those related to inherited thrombophilia--factor V Leiden and G20210A prothrombin polymorphisms. Finally, post-transcriptional modifications of anti-beta2GPI antibodies could be implicated too.

  14. Antiphospholipid syndrome; its implication in cardiovascular diseases: a review

    Directory of Open Access Journals (Sweden)

    Goudevenos John

    2010-11-01

    Full Text Available Abstract Antiphospholipid syndrome (APLS is a rare syndrome mainly characterized by several hyper-coagulable complications and therefore, implicated in the operated cardiac surgery patient. APLS comprises clinical features such as arterial or venous thromboses, valve disease, coronary artery disease, intracardiac thrombus formation, pulmonary hypertension and dilated cardiomyopathy. The most commonly affected valve is the mitral, followed by the aortic and tricuspid valve. For APLS diagnosis essential is the detection of so-called antiphospholipid antibodies (aPL as anticardiolipin antibodies (aCL or lupus anticoagulant (LA. Minor alterations in the anticoagulation, infection, and surgical stress may trigger widespread thrombosis. The incidence of thrombosis is highest during the following perioperative periods: preoperatively during the withdrawal of warfarin, postoperatively during the period of hypercoagulability despite warfarin or heparin therapy, or postoperatively before adequate anticoagulation achievement. Cardiac valvular pathology includes irregular thickening of the valve leaflets due to deposition of immune complexes that may lead to vegetations and valve dysfunction; a significant risk factor for stroke. Patients with APLS are at increased risk for thrombosis and adequate anticoagulation is of vital importance during cardiopulmonary bypass (CPB. A successful outcome requires multidisciplinary management in order to prevent thrombotic or bleeding complications and to manage perioperative anticoagulation. More work and reporting on anticoagulation management and adjuvant therapy in patients with APLS during extracorporeal circulation are necessary.

  15. Antiphospholipid Syndrome during pregnancy: the state of the art.

    Science.gov (United States)

    Di Prima, Fosca A F; Valenti, Oriana; Hyseni, Entela; Giorgio, Elsa; Faraci, Marianna; Renda, Eliana; De Domenico, Roberta; Monte, Santo

    2011-04-01

    Obstetric complications are the hallmark of antiphospholipid syndrome. Recurrent miscarriage, early delivery, oligohydramnios, prematurity, intrauterine growth restriction, fetal distress, fetal or neonatal thrombosis, pre-eclampsia/eclampsia, HELLP syndrome, arterial or venous thrombosis and placental insufficiency are the most severe APS-related complication for pregnant women. Antiphospholipid antibodies promote activation of endothelial cells, monocytes and platelets, causing an overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. These factors, associated with the typical changes in the hemostatic system during normal pregnancy, result in a hypercoagulable state. This is responsible of thrombosis that is presumed to provoke many of the pregnancy complications associated with APS. Obstetric care is based on combined medical-obstetric high-risk management and treatment with the association between aspirin and heparin. This review aims to deter- mine the current state of the art of APS by investigating the knowledge achievements of recent years, to provide the most appropriate diagnostic and therapeutic management for pregnant women suffering from this syndrome. PMID:22439075

  16. Recurrent miscarriage and antiphospholipid antibodies: prognosis of subsequent pregnancy

    NARCIS (Netherlands)

    D.M. Cohn; M. Goddijn; S. Middeldorp; J.C. Korevaar; F. Dawood; R.G. Farquharson

    2010-01-01

    Summay Background: Although women with antiphospholipid antibodies (APLAs) are at increased risk of recurrent miscarriage, the outcome of a subsequent pregnancy is not clearly elucidated. Objectives: To assess the pregnancy outcome of a subsequent pregnancy in women with APLAs and compare this outco

  17. Recurrent miscarriage and antiphospholipid antibodies: prognosis of subsequent pregnancy.

    NARCIS (Netherlands)

    Cohn, D.M.; Goddijn, M.; Middeldorp, S.; Korevaar, J.C.; Dawood, F.; Farquharson, R.G.

    2010-01-01

    Background: Although women with antiphospholipid antibodies (APLAs) are at increased risk of recurrent miscarriage, the outcome of a subsequent pregnancy is not clearly elucidated. Objectives: To assess the pregnancy outcome of a subsequent pregnancy in women with APLAs and compare this outcome with

  18. PREVENTION OF THROMBOSES IN ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    Lyubov Valeryevna Kondratyeva

    2009-01-01

    Patients with antiphospholipid (aPL antibodies and venous thromboses need long-term moderate-intensity warfarin therapy. Patients with ischemic strokes without other indications for the use of anticoagulants may be given either warfarin or ASA. In the latter case, there is no need for laboratory control or an individual dose adjustment. The primary prevention of thromboses in the presence of aPL is also performed with ASA. When pregnancy occurs, women with obstetric manifestations of APS may be given small-dose ASA in combination with heparins. To reduce the risk of hemorrhages, warfarin dosage adjustment is initiated with the minimum doses (<5 mg/day. Novel ASA formulations, such as ASA with the unabsorbed antacid magnesium hydroxide, have been developed to prevent gastrointestinal tract complications.

  19. Prevalence of antiphospholipid antibodies in patients with fetal loss.

    OpenAIRE

    Out, H.J.; Bruinse, H.W.; Christiaens, G. C.; Vliet, M. van; Meilof, J.F.; De Groot, P G; Smeenk, R. J.; Derksen, R H

    1991-01-01

    The prevalence of antiphospholipid and antinuclear antibodies in 102 patients with at least three unexplained miscarriages before a gestational age of 12 weeks, or at least one intrauterine fetal death after 12 weeks, was investigated and compared with the prevalence in 102 normal pregnant controls. Six patients had a history of thrombosis and six had 'lupus-like' disease. Twenty one patients had anticardiolipin antibodies compared with 10 controls. Serum samples of nine patients and one cont...

  20. Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

    Institute of Scientific and Technical Information of China (English)

    Tatiana; M; Reshetnyak; Natalia; V; Seredavkina; Maria; A; Satybaldyeva; Evgeniy; L; Nasonov; Vasiliy; I; Reshetnyak

    2015-01-01

    The antiphospholipid syndrome(APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of BuddChiari syndrome(BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.

  1. Patient with antiphospholipid syndrome accompanied by pre-eclampsia who developed hellp syndrome and eclampsia after abortion

    Institute of Scientific and Technical Information of China (English)

    WANG Yong-qing; NIU Ji-hong; WANG Jia-lue; YE Rong-hua; ZHAO Yang-yu

    2012-01-01

    Antiphospholipid syndrome(APS)refers to a group of clinical symptoms and signs caused by antiphospholipid antibody(aPLA).We reported a rare case of poor outcome of a pregnant woman with APS.The pregnant woman had APS,hemolytic anemia,elevated liver function and low platelet count(HELLP)syndrome,and eclampsia and had a poor outcome from a second pregnancy.She was treated with antispasmodics,sedatives,and anti-hypertensive agents,along with anticoagulant therapy and infusion of immunoglobulin.APS during pregnancy often makes pregnancy even more complex and risky.Obstetricians should carry out anticoagulation treatment throughout the perinatal period.

  2. Annexin A2: biology and relevance to the antiphospholipid syndrome

    OpenAIRE

    Cockrell, E; Espinola, RG; McCrae, KR

    2008-01-01

    Antiphospholipid antibodies (aPL), the majority of which are directed against β2-glycoprotein I (β2GPI), are associated with an increased incidence of venous and arterial thrombosis. The pathogenesis of antiphospholipid/anti-β2GPI-associated thrombosis has not been defined, and is likely multifactorial. However, accumulating evidence suggests an important role for endothelial cell activation with the acquisition of a procoagulant phenotype by the activated endothelial cell. Previous work demo...

  3. Postpartum spontaneous colonic perforation due to antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    Kamran Ahmed; Amir Darakhshan; Eleanor Au; Munther A Khamashta; Iraklis E Katsoulis

    2009-01-01

    The antiphospholipid syndrome (APS) is a multi-systemic disease being characterized by the presence of antiphospholipid antibodies that involves both arterial and venous systems resulting in arterial or venous thrombosis, fetal loss, thrombocytopenia, leg ulcers, livedo reticularis, chorea,and migraine. We document a previously unreported case of a 37-year-old female in whom APS was first manifested by infarction and cecal perforation following cesarean section. At laparotomy the underlying cause of colonic perforation was not clear and after resection of the affected bowel an ileo-colostomy was performed. The diagnosis of APS was established during post-operative hospital stay and the patient was commenced on warfarin.Eventually, she made a full recovery and had her stoma reversed after 4 mo. Pregnancy poses an increased risk of complications in women with APS and requires a more aggressive approach to the obstetric care. This should include full anticoagulation in the puerperium and frequent doppler ultrasound monitoring of uterine and umbilical arteries to detect complications such as preeclampsia and placental insufficiency.

  4. Cutaneous necrosis associated with the antiphospholipid syndrome and mycosis fungoides.

    Science.gov (United States)

    Hill, V A; Whittaker, S J; Hunt, B J; Liddell, K; Spittle, M F; Smith, N P

    1994-01-01

    The development of extensive cutaneous necrosis in a patient with tumour-stage mycosis fungoides is described. Skin biopsies showed a lymphomatous infiltrate, and thrombosis of dermal blood vessels. Investigation revealed the presence of anticardiolipin antibodies, a lupus anticoagulant, and low free protein S, which contributed to a prothrombotic state. Antiphospholipid antibodies have been detected in non-Hodgkin's lymphoma, but clinical manifestations are uncommon. Such autoantibodies may be produced by neoplastic lymphoid cells. The frequency with which antiphospholipid antibodies occur in mycosis fungoides is currently unknown.

  5. Association of anti-phospholipid antibodies with connective tissue diseases

    OpenAIRE

    Reena Rai; Swetha, T.

    2015-01-01

    Background: The antiphospholipid antibodies (APLA) are directed against phospholipids and their binding proteins and are frequently found in association with connective tissue disorders. Systemic lupus erythematoses (SLE) with APLA may cause a diagnostic dilemma as there are several manifestations like haemolytic anemia, thrombocytopenia, neurologic manifestations, leg ulcerations, serositis proteinuria which overlap in both these conditions. We conducted a study to find out the association o...

  6. Recurrent miscarriage and antiphospholipid antibodies: prognosis of subsequent pregnancy.

    OpenAIRE

    Cohn, D.M.; Goddijn, M.; Middeldorp, S.; Korevaar, J.C.; Dawood, F.; Farquharson, R G

    2010-01-01

    Background: Although women with antiphospholipid antibodies (APLAs) are at increased risk of recurrent miscarriage, the outcome of a subsequent pregnancy is not clearly elucidated. Objectives: To assess the pregnancy outcome of a subsequent pregnancy in women with APLAs and compare this outcome with women with unexplained recurrent miscarriage. Methods: We performed a cohort study among all women who attended the Miscarriage Clinic at Liverpool Women’s Hospital between 1987 and 2006 after bei...

  7. Update on anti-phospholipid antibodies in SLE: the Hopkins' Lupus Cohort.

    Science.gov (United States)

    Petri, M

    2010-04-01

    Anti-phospholipid antibodies are common in patients in the Hopkins' Lupus Cohort: 47% have anti-cardiolipin, 32.5% anti-beta(2)-glycoprotein I and 26% lupus anticoagulant (by dRVVT confirmatory testing). Systemic lupus erythematosus patients with the lupus anticoagulant at baseline have a 50% chance of a deep venous thrombosis/pulmonary embolus in the next 20 years. Anti-phospholipid antibodies differ in their association with thrombosis: the lupus anticoagulant is most strongly associated with arterial and venous thrombosis and is the only anti-phospholipid antibody associated with myocardial infarction. Anti-phospholipid antibodies are not associated with atherosclerosis.

  8. Association of anti-phospholipid antibodies with connective tissue diseases

    Directory of Open Access Journals (Sweden)

    Reena Rai

    2015-01-01

    Full Text Available Background: The antiphospholipid antibodies (APLA are directed against phospholipids and their binding proteins and are frequently found in association with connective tissue disorders. Systemic lupus erythematoses (SLE with APLA may cause a diagnostic dilemma as there are several manifestations like haemolytic anemia, thrombocytopenia, neurologic manifestations, leg ulcerations, serositis proteinuria which overlap in both these conditions. We conducted a study to find out the association of antiphospholipid antibodies with connective tissue diseases and compared the clinical and laboratory parameters between antiphoshpolipid antibody positive and antiphoshpolipid antibody negative group. Materials and Methods: This study was carried out in 102 patients diagnosed with connective tissue diseases. APLA testing was done at baseline and for those positive, the test was repeated after 12 weeks. Results: 14.7 % of patients with connective tissue diseases tissue had positive antiphoshpolipid antibodies. Positive antiphoshpolipid antibody was detected in 73.3% of patients with SLE group, 13.3% of patients with mixed connective tissue disease (MCTD and 13.3% of patients with systemic sclerosis. APLA positivity was seen in SLE patients with leg ulcers (87.2%, neurologic manifestation (72.7%, hemolytic anemia (62.3%, thrombocytopenia (72.7%, serositis (27.8% and proteinuria(19.6%. Conclusions: Antiphoshpolipid antibodies should be tested in all patients with connective tissue disease.

  9. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus.

    Science.gov (United States)

    Tincani, A; Rebaioli, C B; Taglietti, M; Shoenfeld, Y

    2006-10-01

    Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. All the anatomical heart structures can be affected, and multiple pathogenic mechanisms have been reported. Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman-Sacks verrucous endocarditis, myocarditis and pericarditis. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis.

  10. Confirmation of antiphospholipid antibody positivity: a year’s results in a cohort of 113 patients

    Directory of Open Access Journals (Sweden)

    A. Ruffatti

    2011-06-01

    Full Text Available Objective: To evaluate the confirmation rate of antiphospholipid antibodies (aPL, to analyze their behaviour at confirmation time, and to study the clinical value of their confirmation. Methods: Blood samples from 380 subjects, enrolled in this study from June 1, 2007 to May 31, 2008, were tested for anti-cardiolipin (aCL and anti-beta2glycoprotein (aβ2GPI antibodies using an ELISA method and for Lupus anticoagulant (LA using a series of clotting tests. The samples of the 113 subjects resulting positive at the first testing time were assayed again to confirm antiphospholipid positivity. Results: aPL positivity was confirmed in 67 out of the 113 subjects (59.3%. Medium-high antibody levels of all, except IgM aCL, aPL/ELISA had a significantly higher confirmation rate with respect to that in subjects with low levels. The confirmation rate in the category I antibody patients (multiple positivity was higher than that in the category II antibody subjects (single positivity. LA positivity was confirmed only when it was associated to other aPL. The cut-off of 40 GPL produced a confirmation rate equal to that resulting from a 99th percentile cut-off. Confirmation of aPL positivity made it possible for us to confirm the diagnosis of antiphospholipid syndrome (APS in 8 out of the 113 subjects originally resulting positive (7,1%. APS clinical features were vascular thrombosis in 4 of these and pregnancy morbidity in the other 4. Conclusions: Our data emphasize aPL positivity confirmation selectivity, and medium-high antibody levels and category I antibodies (multiple positivity had the best confirmation rates.

  11. Overlapping humoral autoimmunity links rheumatic fever and the antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Blank, M; Krause, I; Magrini, L;

    2006-01-01

    Rheumatic fever (RF) and the antiphospholipid syndrome (APS) are autoimmune diseases that share similar cardiac and neurological pathologies. We assessed the presence of shared epitopes between M protein, N-acetyl-beta-D-glucosamine (GlcNAc) and beta2 glycoprotein-I (beta2GPI), the pathogenic mol...

  12. Renal artery thrombosis and hypertension in a 13 year old girl with antiphospholipid syndrome.

    Science.gov (United States)

    Ostuni, P A; Lazzarin, P; Pengo, V; Ruffatti, A; Schiavon, F; Gambari, P

    1990-01-01

    The case of a 13 year old girl with renal artery thrombosis and hypertension is described. A cerebrovascular accident and a probable occlusion of the superior mesenteric artery also occurred. Very high levels of 'lupus anticoagulant', anticardiolipin antibodies as well as false positive Venereal Disease Research Laboratory tests were repeatedly shown. Moreover, the patient fulfilled at least four classification criteria for systemic lupus erythematosus, but only a slight positivity for antinucleolar antibodies was present. The striking relation between antiphospholipid antibody levels and clinical events and the treatment of this complex syndrome are discussed. Images PMID:2108619

  13. [Diagnostic and therapeutic approach to pregnant women suspect on antiphospholipid syndrome].

    Science.gov (United States)

    Glasnović, Marija; Bosnjak, Ivica; Vcev, Aleksandar; Kosuta, Maja; Lenz, Bahrija; Glasnović-Horvatić, Elizabeta

    2008-01-01

    Antiphospholipid syndrome includes the presence of antiphospholipid antibodies, vascular thrombosis and reproductive function disturbances. The aim was to show our diagnostic and therapeutic experiences. 62 women were included in study, 32 with primary antiphospholipd syndrome (PAPS), and 30 with secondary antiphospholipid syndrome (SAPS). 36 were pregnant and studied prospectively throughout pregnancy and six weeks after the delivery. Lupus-anticoagulant (LA) was positive in 23 patients with PAPS (71.9%), and in 10 patients with SAPS (33.3%). In SAPS group anticardiolipin antibodies (aCL) was positive in 8 patients (26.6%) compared to PAPS group with 3 aCL positive patients (9.4%). Antibeta2glycoprotein1 (antibeta2GP1) was positive in 3 patients with PAPS. Complications in previous pregnancies were in 25 cases (69.4%) spontaneous abortion, in 7 cases (19.4%) preeclampsia with intrauterine growth restriction (IUGR) in 3 patients. In 4 cases the complication was fetal death in utero. Average pregnancy lasted 37.06+/-0.707 weeks. Therapy with low dose aspirin and low-molecular-weight heparin was successful in 97.2%.

  14. Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    B. Routy

    2013-01-01

    Full Text Available Catastrophic antiphospholipid syndrome (CAPS is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported.

  15. Antiphospholipid Syndrome: Expanding the Spectrum of Autoimmune Thrombosis

    OpenAIRE

    Gómez Puerta, José Alfredo

    2007-01-01

    The antiphospholipid syndrome (APS) is an acquired prothrombotic syndrome characterized by venous or arterial thromboses and pregnancy morbidity. It can present as primary APS without any discernable underlying disease, or in association with systemic autoimmune disease [usually systemic lupus erythematosus (SLE)], infections (mainly chronic viral infections) and malignant process, among others. It may also occur rapidly over days or weeks, when it is known as "catastrophic" APS (CAPS).The fi...

  16. Elevated antiphospholipid antibody titers and adverse pregnancy outcomes: analysis of a population-based hospital dataset

    Directory of Open Access Journals (Sweden)

    Nuwayhid Bahij S

    2009-03-01

    Full Text Available Abstract Background The primary objective of this study was to determine if elevated antiphospholipid antibody titers were correlated with the presence of preeclampsia/eclampsia, systemic lupus erythematosus (SLE, placental insufficiency, and a prolonged length of stay (PLOS, in women who delivered throughout Florida, USA. Methods Cross-sectional analyses were conducted using a statewide hospital database. Prevalence odds ratios (OR were calculated to quantify the association between elevated antiphospholipid antibody titers and four outcomes in 141,286 women who delivered in Florida in 2001. The possibility that the relationship between elevated antiphospholipid antibody titers and the outcomes of preeclampsia/eclampsia, placental insufficiency, and PLOS, may have been modified by the presence of SLE was evaluated in a multiple logistic regression model by creating a composite interaction term. Results Women with elevated antiphospholipid antibody titers (n = 88 were older, more likely to be of white race and not on Medicaid than women who did not have elevated antiphospholipid antibody titers. Women who had elevated antiphospholipid antibody titers had an increased adjusted odds ratio for preeclampsia and eclampsia, (OR = 2.93 p = 0.0015, SLE (OR = 61.24 p Conclusion This exploratory epidemiologic investigation found moderate to very strong associations between elevated antiphospholipid antibody titers and four important outcomes in a large sample of women.

  17. Antiphospholipid antibodies predict progression of abdominal aortic aneurysms.

    Directory of Open Access Journals (Sweden)

    Christina Duftner

    Full Text Available Antiphospholipid antibodies (aPLs frequently occur in autoimmune and cardiovascular diseases and correlate with a worse clinical outcome. In the present study, we evaluated the association between antiphospholipid antibodies (aPLs, markers of inflammation, disease progression and the presence of an intra-aneurysmal thrombus in abdominal aortic aneurysm (AAA patients. APLs ELISAs were performed in frozen serum samples of 96 consecutive AAA patients and 48 healthy controls yielding positive test results in 13 patients (13.5% and 3 controls (6.3%; n.s.. Nine of the 13 aPL-positive AAA patients underwent a second antibody testing >12 weeks apart revealing a positive result in 6 cases. APL-positive patients had increased levels of inflammatory markers compared to aPL-negative patients. Disease progression was defined as an increase of the AAA diameter >0.5 cm/year measured by sonography. Follow-up was performed in 69 patients identifying 41 (59.4% patients with progressive disease. Performing multipredictor logistic regression analysis adjusting for classical AAA risk factors as confounders, the presence of aPLs at baseline revealed an odds ratio of 9.4 (95% CI 1.0-86.8, p = 0.049 to predict AAA progression. Fifty-five patients underwent a computed tomography in addition to ultrasound assessment indicating intra-aneurysmal thrombus formation in 82.3%. Median thrombus volume was 46.7 cm3 (1.9-377.5. AAA diameter correlated with the size of the intra-aneurysmal thrombus (corrcoeff = 0.721, p<0.001, however neither the presence nor the size of the intra-aneurysmal thrombus were related to the presence of aPLs. In conclusion, the presence of aPLs is associated with elevated levels of inflammatory markers and is an independent predictor of progressive disease in AAA patients.

  18. 36-Year-Old Female with Catastrophic Antiphospholipid Syndrome Treated with Eculizumab: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Marianna Strakhan

    2014-01-01

    Full Text Available Catastrophic antiphospholipid syndrome (CAPS is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL. CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor.

  19. Annexin A2: biology and relevance to the antiphospholipid syndrome

    Science.gov (United States)

    Cockrell, E; Espinola, RG; McCrae, KR

    2012-01-01

    Antiphospholipid antibodies (aPL), the majority of which are directed against β2-glycoprotein I (β2GPI), are associated with an increased incidence of venous and arterial thrombosis. The pathogenesis of antiphospholipid/anti-β2GPI-associated thrombosis has not been defined, and is likely multifactorial. However, accumulating evidence suggests an important role for endothelial cell activation with the acquisition of a procoagulant phenotype by the activated endothelial cell. Previous work demonstrated that endothelial activation by antiphospholipid/anti-β2GPI antibodies is β2GPI-dependent. We extended these observations by defining annexin A2 as an endothelial β2GPI binding site. We also observed that annexin A2 plays a critical role in endothelial cell activation induced by anti-β2GPI antibodies, and others have described direct endothelial activation by anti-annexin A2 antibodies in patients with aPL. Similar findings have been reported using human monocytes, which also express annexin A2. Because annexin A2 is not a transmembrane protein, how binding of β2GPI/anti-β2GPI antibodies, or anti-annexin A2 antibodies, to endothelial annexin A2 causes cellular activation is unknown. Recent studies, however, suggest an important role for the Toll-like receptor family, particularly TLR4. In this article, we review the role of these interactions in the activation of endothelial cells by aPL. The influence of these antibodies on the ability of annexin A2 to enhance t-PA-mediated plasminogen activation is also discussed. PMID:18827060

  20. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

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    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  1. The significance of antiphospholipid antibodies in pregnant women with chronic hypertension

    NARCIS (Netherlands)

    Zeeman, GG; Alexander, JM; McIntire, DD; Leveno, KJ

    2004-01-01

    The objective of this study was to perform antiphospholipid antibody screening in women with chronic hypertension to assess whether the presence of such antibodies is associated with adverse pregnancy outcome. Serum for anticardiolipin antibodies and lupus anticoagulant was obtained in pregnant wome

  2. The coexistence of antiphospholipid syndrome and systemic lupus erythematosus in Colombians.

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    Juan-Sebastian Franco

    Full Text Available OBJECTIVES: To examine the prevalence and associated factors related to the coexistence of antiphospholipid syndrome (APS and systemic lupus erythematosus (SLE in a cohort of Colombian patients with SLE, and to discuss the coexistence of APS with other autoimmune diseases (ADs. METHOD: A total of 376 patients with SLE were assessed for the presence of the following: 1 confirmed APS; 2 positivity for antiphospholipid (aPL antibodies without a prior thromboembolic nor obstetric event; and 3 SLE patients without APS nor positivity for aPL antibodies. Comparisons between groups 1 and 3 were evaluated by bivariate and multivariate analysis. RESULTS: Although the prevalence of aPL antibodies was 54%, APS was present in just 9.3% of SLE patients. In our series, besides cardiovascular disease (AOR 3.38, 95% CI 1.11-10.96, p = 0.035, pulmonary involvement (AOR 5.06, 95% CI 1.56-16.74, p = 0.007 and positivity for rheumatoid factor (AOR 4.68, 95%IC 1.63-14.98, p = 0.006 were factors significantly associated with APS-SLE. APS also may coexist with rheumatoid arthritis, Sjögren's syndrome, autoimmune thyroid diseases, systemic sclerosis, systemic vasculitis, dermatopolymyositis, primary biliary cirrhosis and autoimmune hepatitis. CONCLUSIONS: APS is a systemic AD that may coexist with other ADs, the most common being SLE. Awareness of this polyautoimmunity should be addressed promptly to establish strategies for controlling modifiable risk factors in those patients.

  3. Trombose da artéria renal e síndrome do anticorpo antifosfolípide: um relato de caso Renal arterial thrombosis and the antiphospholipid antibody syndrome: a case report

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    Célia S. Macedo

    2001-12-01

    a history of abdominal pain, pallor, lethargy, and anuria for 36 hours. On physical examination, the patient showed malnutrition, high blood pressure, moderate edema, and hypochondrial pain. Laboratory findings included: urea=112mg/dl, serum creatinine= 4.5 mg/dl, blood pH= 7.47, blood bicarbonate= 12.8 mmol/L, K=7.2 mEq/L. Peritoneal dialysis was started and maintained for 11 days. After 7 weeks, the patient still needed anti-hypertensive drugs and the renal function was still abnormal. Renal biopsy was performed and revealed renal infarction. The result of Doppler ultrasonography revealed absent renal blood flow on the right side. Renal arteriography showed total occlusion of the right renal artery. Results for collagen diseases were negative. A right nephrectomy was performed and the blood pressure was controlled. The child was hospitalized again at 5 years and 8 months old with episodes of absence seizures and abdominal and precordial pain. Anticardiolipin antibody test was positive. The child is now 7 years old, asymptomatic, with negative anticardiolipin antibody, and has been under regular follow-up. COMMENTS: children with arterial thrombosis should be investigated for a possible association with the antiphospholipid antibody syndrome even in the absence of collagen disease.

  4. Acute myocardial infarction in young adults with Antiphospholipid syndrome: report of two cases and literature review

    OpenAIRE

    Leila Abid; Faten Frikha; Zouhir Bahloul; Samir Kammoun

    2011-01-01

    Abstract Acute myocardial infarction (AMI) is rarely associated with antiphospholipid syndrome. The treatment of these patients is a clinical challenge. We report the observations of 2 young adults (1 woman and 1 man), admitted in our acute care unit for acute myocardial infarction (AMI). A coagulopathy work-up concludes the existence of antiphospholipid syndrome (APS) in the 2 cases. APS syndrome was considered primary in 2 cases. All patients presented an intense inflammatory syndrome (high...

  5. The autoimmune tautology with a focus on antiphospholipid syndrome.

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    Franco, J-S; Anaya, J-M

    2014-10-01

    Autoimmune diseases (ADs) are often diagnosed according to classification criteria; however, they share similar subphenotypes including signs and symptoms, non-specific autoantibodies and other immune changes, which are prone to taxonomic problems. Polyautoimmunity is defined as the presence of more than one AD in a single patient. The close relationship between antiphospholipid syndrome (APS) and systemic lupus erythematosus has been studied throughout the years. However, APS may coexist with several other ADs confirming polyautoimmunity in this systemic disease. Herein, we summarized the common characteristics shared between APS and others ADs in light of the autoimmune tautology (that is, common mechanisms of autoimmune diseases).

  6. Cardiac manifestations in antiphospholipid syndrome - a brief review of the literature

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    Đoković Aleksandra

    2015-01-01

    Full Text Available Antiphospholipid syndrome (APS or Hughes syndrome represents a systemic autoimmune disorder characterized by arterial and/or venous thrombosis, multiple and recurrent fetal losses, accompanied by persistently elevated levels of antiphospholipid antibodies (aPL. This syndrome is considered primary if unassociated with any other connective tissue disease, or secondary if it appears in association with other autoimmune disorders, mainly systemic lupus erythematosus. Cardiac manifestations in APS are integral part of the syndrome. aPL are involved in the pathogenesis of pseudoinfective endocarditis (Libman Sacks and other valvular manifestations presented as their thickening and dysfunction. Intracardiac thrombi and myxomas, pulmonary hypertension and left ventricular dysfunction are also distinguishing features of APS. On the other hand, accelerated atherosclerosis, proven in APS and also aPL mediated, is accountable for the development of coronary and peripheral artery disease. This leads to higher cardiovascular mortality rate in the population of patients with low incidence of the traditional atherosclerosis risk factors. Furthermore, recent studies implied that presence of certain aPL could be a risk factor for a specific cardiac manifestation. Bearing all this in mind, early diagnosis of cardiac manifestations, control and abolition of traditional risk factors, as well as close cardiac follow-up of APS patients, are crucial in reducing their cardiovascular mortality.

  7. Human Monoclonal Antiphospholipid Antibodies Disrupt the Annexin A5 Anticoagulant Crystal Shield on Phospholipid Bilayers

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    Rand, Jacob H.; Wu, Xiao-Xuan; Quinn, Anthony S.; Chen, Pojen P.; McCrae, Keith R.; Bovill, Edwin G.; Taatjes, Douglas J.

    2003-01-01

    The antiphospholipid (aPL) syndrome is an autoimmune condition that is marked by recurrent pregnancy losses and/or systemic vascular thrombosis in patients who have antibodies against phospholipid/co-factor complexes. The mechanism(s) for pregnancy losses and thrombosis in this condition is (are) not known. Annexin A5 is a potent anticoagulantprotein, expressed by placental trophoblasts and endothelial cells, that crystallizes over anionic phospholipids, shielding them from availability for coagulation reactions. We previously presented data supporting the hypothesis that aPL antibody-mediated disruption of the anticoagulant annexin A5 shield could be a thrombogenic mechanism in the aPL syndrome. However, this has remained a subject of controversy. We therefore used atomic force microscopy, a method previously used to study the crystallization of annexin A5, to image the effects of monoclonal human aPL antibodies on the crystal structure of the protein over phospholipid bilayers. In the presence of the aPL monoclonal antibodies (mAbs) and β2-GPI, the major aPL co-factor, structures presumed to be aPL mAb-antigen complexes were associated with varying degrees of disruption to the annexin A5 crystallization pattern over the bilayer. In addition, measurements of prothrombinase activity on the phospholipid bilayers showed that the aPL mAbs reduced the anti-coagulant effect of annexin A5 and promoted thrombin generation. These data provide morphological evidence that support the hypothesis that aPL antibodies can disrupt annexin A5 binding to phospholipid membranes and permit increased generation of thrombin. The aPL antibody-mediated disruption of the annexin A5 anticoagulant shield may be an important prothrombotic mechanism in the aPL syndrome. PMID:12937161

  8. Type III mixed cryoglobulinemia and antiphospholipid syndrome in a patient with partial DiGeorge syndrome.

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    Chang, Alice D; Tachdjian, Raffi; Gallagher, Kerry; McCurdy, Deborah K; Lassman, Charles; Stiehm, E Richard; Yadin, Ora

    2006-01-01

    We studied a 14 year-old boy with partial DiGeorge syndrome (DGS), status post complete repair of Tetralogy of Fallot, who developed antiphospholipid syndrome (APS) and type III mixed cryoglobulinemia. He presented with recurrent fever and dyspnea upon exertion secondary to right pulmonary embolus on chest computed tomography (CT). Coagulation studies revealed homozygous methylene tetrahydrofolate reductase 677TT mutations, elevated cardiolipin IgM antibodies, and elevated beta(2)-glycoprotein I IgM antibodies. Infectious work-up revealed only positive anti-streptolysin O (ASO) and anti-DNAse B titers. Autoimmune studies showed strongly positive anti-platelet IgM, elevated rheumatoid factor (RF), and positive cryocrit. Renal biopsy for evaluation of proteinuria and hematuria showed diffuse proliferative glomerulonephritis (DPGN) with membranoproliferative features consistent with cryoglobulinemia. Immunofixation showed polyclonal bands. Our patient was treated successfully with antibiotics, prednisone, and mycophenolate mofetil (MMF). This is the first report of a patient with partial DGS presenting with APS and type III mixed cryoglobulinemia possibly due to Streptococcal infection.

  9. Type III Mixed Cryoglobulinemia and Antiphospholipid Syndrome in a Patient With Partial DiGeorge Syndrome

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    Alice D. Chang

    2006-01-01

    Full Text Available We studied a 14 year-old boy with partial DiGeorge syndrome (DGS, status post complete repair of Tetralogy of Fallot, who developed antiphospholipid syndrome (APS and type III mixed cryoglobulinemia. He presented with recurrent fever and dyspnea upon exertion secondary to right pulmonary embolus on chest computed tomography (CT. Coagulation studies revealed homozygous methylene tetrahydrofolate reductase 677TT mutations, elevated cardiolipin IgM antibodies, and elevated β2-glycoprotein I IgM antibodies. Infectious work-up revealed only positive anti-streptolysin O (ASO and anti-DNAse B titers. Autoimmune studies showed strongly positive anti-platelet IgM, elevated rheumatoid factor (RF, and positive cryocrit. Renal biopsy for evaluation of proteinuria and hematuria showed diffuse proliferative glomerulonephritis (DPGN with membranoproliferative features consistent with cryoglobulinemia. Immunofixation showed polyclonal bands. Our patient was treated successfully with antibiotics, prednisone, and mycophenolate mofetil (MMF. This is the first report of a patient with partial DGS presenting with APS and type III mixed cryoglobulinemia possibly due to Streptococcal infection.

  10. The role of anti-annexin A2 antibodies in antiphospholipid syndrome%抗膜联蛋白A2抗体在抗磷脂综合征中的作用

    Institute of Scientific and Technical Information of China (English)

    敖文; 郑辉; 陈晓微; 申艳; 杨程德

    2009-01-01

    Objective This study has explored the role of antibody against annexin A2 in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Methods Using purified recombinant annexin A2, IgG anti-annexin A2 antibody was measured by ELISA in 101 APS patients, 41 SLE patients with thrombosis, 124 SLE patients without thrombosis and 120 healthy controls. Results The positive rate of IgG anti-annexin A2 antibody in APS patients and SLE patients with thrombosis was 21.8%, 26.8%, respectively, they were all significantly higher than in SLE patients without thrombosis (6.5%). IgG anti-annexin A2 antibody was associated with thrombosis and/or pregnancy morbidity (P<0.01). Conclusion Anti-annexin A2 antibody is associated with thrombosis and/or pregnancy mnrbidity. It suggests that anti-annexin A2 antibody may be helpful in identifying in some potential AIRS.%目的 研究抗膜联蛋白A2抗体在抗磷脂综合征(APS)、系统性红斑狼疮(SLE)的血栓/病态妊娠中的可能作用.方法 先用分子克隆方法表达纯化出重组膜联蛋白A2,然后以重组膜联蛋白A2为抗原,采用酶联免疫吸附试验(ELISA)法分别检测了,101例APS患者,41例SLE合并血栓患者,124例无血栓的SLE患者及120名健康人的血清中IgG型抗膜联蛋白A2抗体水平.结果 APS组和SLE合并血栓组的IgC型抗膜联蛋白A2抗体阳性率分别为21.8%,26.8%,均品著高于单纯SLE组(6.5%)(P值均<0.0.).IgG型抗膜联蛋白A2抗体与血栓/病态妊娠有关联(P<0.01).IgG型抗膜联蛋白A2抗体对血栓/病态妊娠诊断的敏感性、特异性、预测值分别为0.232、0.935、0.805.结论 IgG型抗膜联蛋白A2抗体与APS和SLE患者的血栓/病态妊娠表现相关,将有助于一些潜在的APS患者的诊断.

  11. The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome

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    Doruk Erkan

    2016-03-01

    Full Text Available Antiphospholipid syndrome (APS is characterized by thrombosis (arterial, venous, small vessel and/or pregnancy morbidity occurring in patients with persistently positive antiphospholipid antibodies (aPL. Catastrophic APS is the most severe form of the disease, characterized by multiple organ thromboses occurring in a short period and commonly associated with thrombotic microangiopathy (TMA. Similar to patients with complement regulatory gene mutations developing TMA, increased complement activation on endothelial cells plays a role in hypercoagulability in aPL-positive patients. In mouse models of APS, activation of the complement is required and interaction of complement (C 5a with its receptor C5aR leads to aPL-induced inflammation, placental insufficiency, and thrombosis. Anti-C5 antibody and C5aR antagonist peptides prevent aPL-mediated pregnancy loss and thrombosis in these experimental models. Clinical studies of anti-C5 monoclonal antibody in aPL-positive patients are limited to a small number of case reports. Ongoing and future clinical studies of complement inhibitors will help determine the role of complement inhibition in the management of aPL-positive patients.

  12. Antiphospholipid Syndrome and Libman-Sacks Endocarditis in a Bioprosthetic Mitral Valve.

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    Sladek, Eric H; Accola, Kevin D

    2016-02-01

    This report describes one the first cases of antiphospholipid syndrome and Libman-Sacks endocarditis in a bioprosthetic valve. A redo mitral valve replacement was carried out owing to early deterioration of the prior valve. Initially it was considered secondary to rheumatic heart disease; however, pathology analysis and autoimmune workup revealed antiphospholipid syndrome with Libman-Sacks endocarditis. We believe certain populations with mitral valve stenosis may have an underlying antiphospholipid syndrome. As a result, there needs to be a lower threshold for identifying this disease.

  13. Determinación de anticuerpos anti-β2glicoproteína I en pacientes con síndrome antifosfolípido Anti- β2 glycoprotein antibodies in patients with antiphospholipid syndrome

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    Oscar Uribe Uribe

    2004-09-01

    and with the clinical manifestations of the Antiphospholipid Syndrome (APS. In this study 80 women with APS (35 from the Rheumatology Service and 45 with a history of recurrent spontaneous abortion, RSA were included, as well as 5 women with rheumatic diseases but no APS, 27 RSA-women without APS and 20 healthy women in their reproductive age. The presence of IgG and IgM anticardiolipin antibodies (aCL, anti- β2GPI antibodies by ELISA method and lupus anticoagulant by the test of activated partial thromboplastin time was investigated. Additionally the clinical manifestations associated to APS were registered. In the group of women with APS, 25.7% (9/35 of those with rheumatic diseases and 4.4% /2/45 of the ones with RSA were positive for anti- β2GPI while none of the women without APS or the controls had such positive reaction. There was a significant association at titers of 3+ (highly positive between the presence of anti- β2GPI antibodies and IgG and IgM aCL in contrast to anti- β2GPI-negative individu als. The positivity of lupus anticoagulant also correlated with the presence of anti- β2GPI antibodies. There was no significant correlation between any specific clinical manifestation and the presence of anti- β2GPI antibodies. In conclusion, the determination of anti- β2GPI antibodies was highly specific in patients with APS but did not associate with any clinical manifestation of the syndrome.

  14. NEW ORAL ANTICOAGULANTS IN THE THERAPY OF ANTIPHOSPHOLIPID SYNDROME

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    M. A. Satybaldyeva

    2016-01-01

    Full Text Available The vitamin K antagonist warfarin is an essential medicine from a group of anticoagulants, which is used to treat antiphospholipid syndrome (APS. However, it has a number of disadvantages especially in patients who need longterm and frequently lifetime prevention of thromboses. New oral anticoagulants, such as dabigatran etexilate (Pradaxa®, rivaroxaban (Xarelto®, apixaban (Eliquis and others, have been recently synthesized. Unlike warfarin, they are administered at fixed doses, require neither routine monitoring nor diet, and interact with drugs only in small amounts. The new oral anticoagulants have been approved for certain indications, but the data of performed trials are inapplicable to patients with APS. These medicines are expected to improve quality of life in patients with this condition. 

  15. Antibodies Against Annexin V and Prothrombin, Their Correlation with Other Anti-phospholipid Antibodies in Recurrent Pregnancy Loss

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    Objective To study the findings of serum antibodies against annexin V, prothrombin,ph-inositol, ph-acid, ph-ethanolamine, ph-serine, ph-glycerol, cardiolipin, and beta2-glycoprotein I and analyze the trophoblast annexin V receptorsMethods Sera from 156 patients aged 26-41 years with recurrent pregnancy loss (3-7 times) were investigated. Eighty-four fertile healthy women aged 24-38 years were included in a control group. ELISA methods were used for detecting a panel of sera anti-phospholipid antibodies. Immunolocalization of annexin Vreceptors in 143trophoblast specimens of 156patients was investigated by the immunofluorescence technique using Annexin V-FITC, Apoptosis and Annexin V-CY3 commercial kits.Results Positivity for anti-phospholipid antibodies mainly against ph-serine, phethanolamine, and ph-inositol was found together in 80. 8% (126 out of 156 patients),anti-prothrombin antibodies in 12% (18), and anti-annexin Vantibodies in 13. 5%(21) women. No significant levels of anti-phospholipid antibodies were found in 6controls. Placenta immunohistopathology also exhibited some changes manifested by the presence of apoptotic and necrotic cells in trophoblast, and very few microthrombotization in some intervillous spaces.Conclusion Our detailed study demonstrated the prevalence of majority of antiphospholipid antibodies as a high risk factor for repeated reproductive failure. Very low microthrombosis in placentas could be explained by the changes of haemocoagulation properties out of uterus.

  16. Acute adrenal failure as the presenting feature of primary antiphospholipid syndrome in a child

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    Improda Nicola

    2012-09-01

    Full Text Available Abstract Introduction Antiphospholipid syndrome (APS is characterized by recurrent arterial and venous thrombosis and detection of antiphospholipid antibodies (aPLs. This syndrome may be associated with connective tissue disorders, or with malignancies, but it may also appear in isolated form (primary APS. We report on a pediatric patient presenting with acute adrenal failure as the first manifestation of primary APS. Case report A previously healthy 11-year-old boy developed fever, abdominal pain, and vomiting. An abdominal computed tomography scan showed nodular lesions in the adrenal glands. He was referred to our Department and a diagnosis of APS and acute adrenal failure was considered, based on positive aPLs (IgG and IgM, elevated ACTH levels and low cortisol levels. Other features were anemia, thrombocytopenia, elevated inflammatory parameters, hypergammaglobulinemia, prolonged partial thromboplastin time, positive antinuclear, anticardiolipin, anti-platelet antibodies, with negative double-stranded DNA antibodies. Lupus anticoagulant and Coomb’s tests were positive. MRI revealed a bilateral adrenal hemorrhage. A treatment with intravenous metylprednisolone, followed by oral prednisone and anticoagulant, was started, resulting in a progressive improvement. After 2 months he also showed hyponatremia and elevated renine levels, indicating a mineralcocorticoid deficiency, requiring fludrocortisones therapy. Conclusion The development of acute adrenal failure from bilateral adrenal haemorrhage in the context of APS is a rare but life-threatening event that should be promptly recognized and treated. Moreover, this case emphasizes the importance of the assessment of aPLs in patients with acute adrenal failure in the context of an autoreaction.

  17. Catastrophic Antiphospholipid Syndrome Presenting as Ischemic Pancreatitis in Systemic Lupus Erythematosus

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    Ajit Vyas

    2009-09-01

    Full Text Available Context Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week. Case report We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery. Conclusion Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients’ survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.

  18. A Case of Microangiopathic Antiphospholipid-Associated Syndromes during Pregnancy: Review of the Literature

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    Nobuhiro Suzumori

    2012-01-01

    Full Text Available Microangiopathic antiphospholipid-associated syndromes (MAPSs are reported as encompassing several conditions mainly affecting the microvasculature of selected organs: the liver in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet; kidney, brain, and skin in TTP (thrombotic thrombocytopenic purpura. It is predominant in patients with catastrophic antiphospholipid syndrome (APS. A recent report suggests that APS is not only a thrombotic disease but also associated with microangiopathic features, and it can explain the greater prevalence of HELLP syndrome in these patients. We here report a case of MAPS during pregnancy associated with systemic lupus erythematosus (SLE in early second trimester.

  19. Enfermedad celiaca asociada a síndrome antifosfolípido Celiac disease associated with antiphospholipid syndrome

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    O. Jorge

    2008-02-01

    Full Text Available Introducción: la enfermedad celiaca puede asociarse a patologías de etiología inmunológica. Presentamos su asociación con síndrome antifosfolípido. Caso 1: mujer, 26 años, diagnosticada de enfermedad celiaca. Seis meses después queda embarazada, presentando muerte fetal. Al año siguiente nuevo embarazo. Anticuerpos anticardiolipina IgG: 20 GPL U/ml (valor normal Introduction: celiac disease may be associated with pathologies of immune etiology. We present its association with antiphospholipid syndrome. Case 1: a 26-year-old female was diagnosed with celiac disease. Six months later she became pregnant, and experienced fetal death. The following year she became pregnant again. IgG anticardiolipin antibodies: 20 GPL U/ml (normal value < 11, and IgM anticardiolipin antibodies: 9 MPL U/ml (n. v. < 10. Hematological tests were otherwise uneventful. Medicated with acetylsalicylic acid she had a normal pregnancy. Case 2: a 48-year-old female diagnosed with celiac disease presented with thrombosis in her left lower limb and renal infarction. Hematological tests showed no prothrombotic alterations (antiphospholipid antibodies were not measured. A year and a half later she had thrombosis in a finger of her hand. IgG anticardiolipin antibodies: 10 GPL (n. v. < 13, and IgM anticardiolipin antibodies: 35 MPL (n. v. < 12. Case 3: a 38-year-old female was diagnosed with celiac disease. Some time later she experienced two spontaneous abortions and a transient ischemic cerebral attack. Nowadays, she is in her sixth month of pregnancy. IgM anticardiolipin antibodies: 75 MPL/ml (n. v. up to 20, and IgG anticardiolipin antibodies within normal values. Hematological tests revealed no other prothrombotic alterations. Discussion: antiphospholipid syndrome is characterized by arterial and venous thrombosis, and spontaneous fetal death. Its association with celiac disease has been described in few cases. Celiac disease is associated with spontaneous fetal

  20. Heart Failure with Multi-organ Thrombosis: A Case of Antiphospholipid Syndrome Co-existing with Cardiomyopathy

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    Xueqi Li and Shipeng Wei

    2012-09-01

    Full Text Available Antiphospholipid Syndrome (APS is an autoimmune disease featured by venous or arterial thrombosis, fetal losses and thrombocytopenia in the presence of antiphospholipid antibodies. Here we reported one case of antiphospholipid syndrome together with dilated cardiomyopathy. A 46-year-old female patient complaining short of breath was found enlargement of atrial and ventricular compartments. The ecletrocardiogram and blood test revealed anteroseptal myocardial infarction, while no pulmonary thrombosis was present and therefore diagnosis of dilated cardiomyopathy was made. There were also thrombi formed in the cardial chambers and deep venous. During hospitalization, there was an onset of ischemic brain stroke and head MRI showed newly developed small infarctions. An elevation of anticardiolipin immunoglobulin A (ACAIGA was detected from the blood sample. The patient was discharged after being treated with anticoagulant, corticosteroid and other medicines for improving heart function. In our case, APS is the basic cause leading to multi-organ thrombosis and heart failure is mainly due to dilated cardiomyopathy, thus independent of APS. So this is the first time that cardiomyopathy co-existing with APS was reported.

  1. Detection of multiple annexin autoantibodies in a patient with recurrent miscarriages, fulminant stroke and seronegative antiphospholipid syndrome.

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    Scholz, Philipp; Auler, Markus; Brachvogel, Bent; Benzing, Thomas; Mallman, Peter; Streichert, Thomas; Klatt, Andreas R

    2016-01-01

    Anti-phospholipid syndrome (APS) is one of the main causes for recurrent miscarriages. The diagnosis of APS is based on the occurrence of clinical symptoms such as thrombotic events or obstetric complications as well as the detection of antiphospholipid antibodies directed against β2-glycoprotein I and cardiolipin, or a positive lupus anticoagulant assay. However, there is a subpopulation of patients with clinical symptoms of APS, but the lack of serological markers (seronegative APS). In addition, a large proportion of patients with unexplained recurrent miscarriages exist. These cases may be attributed, at least in part, to a seronegative APS.
The presence of autoantibodies against annexins is potentially associated with APS. Here we used immunoassays and immunoblots to detect autoantibodies directed against annexin A1-5, and A8, respectively, in a patient with a seronegative APS and a history of six recurrent pregnancy losses and fulminant stroke. We found strong IgM isotype antibody reactivity directed against annexin A2 and annexin A8, and moderate to weak IgM isotype antibody reactivity directed against annexin A1, A3, and A5. Further studies will evaluate the diagnostic value of IgM isotype antibodies against annexin A1-A5, and A8 for seronegative APS and recurrent miscarriages. PMID:27346975

  2. Antiphospholipid antibodies and pregnancy outcomes in women heterozygous for Factor V Leiden

    Science.gov (United States)

    Manuck, Tracy; Branch, D. Ware; Lai, Yinglei; Sibai, Baha; Spong, Catherine Y.; Wendel, George; Wenstrom, Katharine; Samuels, Philip; Caritis, Steve N.; Sorokin, Yoram; Miodovnik, Menachem; O’Sullivan, Mary J.; Conway, Deborah; Wapner, Ronald J.

    2010-01-01

    Antiphospholipid antibodies are associated with a spectrum of pregnancy complications, including preeclampsia and small for gestational age (SGA) fetuses. We sought to assess anticardiolipin and anti-β2-glycoprotein I (anti-β2-GPI) IgG and IgM antibody prevalence and the relationship of these antibodies to pregnancy complications in women with the Factor V Leiden (FVL) mutation. The study comprised a secondary analysis of a multicenter, prospective observational study of FVL prevalence among 5,188 asymptomatic pregnant women. A subset of 362 women (117 FVL heterozygotes, 245 matched controls) had serum collected at the time of the original study and underwent serum analysis for anticardiolipin and anti-β2-GPI IgG and IgM as a part of this analysis. The primary outcome was preeclampsia and/or SGA (<10%). The overall prevalence of anticardiolipin and anti-β2-GPI IgG and IgM antibodies was low and did not vary with FVL status. Forty-seven women (13.0%) developed preeclampsia and/or SGA. There were no differences in primary outcome rates between women with and without aPL antibodies, regardless of FVL mutation status. Among FVL carriers, the presence of antiphospholipid antibodies does not appear to contribute to adverse pregnancy outcome. PMID:20439118

  3. Varfarin in the complex treatment of antiphospholipid syndrome: preliminary results

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    T M Reshetnyak

    2003-01-01

    Full Text Available Objective. To assess efficacy and tolerance of varfarin in prophylaxis and therapy of thrombotic complications in patients with antiphospholipid syndrome (APS. Methods. 20 pts with APS (5 male and 15 female received varfarin during a year. 8 of them had primary APS (PAPS and 12 -systemic lupus erythematosus with APS (SLE+APS. 2 other pts (I with SLE+APS and I with PAPS received varfarin during the last 4 years. Nobody from 9 pts with PAPS received corticosteroids (CS. In SLE+APS pts CS dose varied from 4 to 20 mg/day and was not increased during follow up. During the study prothrombine time (PT was examined with thromboplastin ( manufactured by Renam having international sensitivity index 1,2 and international normalization relation (INR. Depending on treatment scheme APS pts were divided into 3 groups. Group 1 included 8 pts with INR<2,0, Group 2-7 with INR >3,0, group 3 - 7 pts with INR<2,0 receiving as additional treatment thrombo ASS 100 mg/day and vasonit from 600 to 1200 mg/day. Results. Two pts with INR = 1,8 had thrombosis recurrence (due to leg thrombophlebitis. There were no recurrences in other groups. 2 from 22 pts had "large" bleedings. "Small" bleedings episodes were noted in 7 from 22 pts. Largely that were subcutaneous bleedings (in 4 pts no more than 5 cm of size. Two pts receiving varfarin with INR 1,8 and 2,4 had renal colic. Conclusion. Our preliminary results prove the necessity of inclusion of varfarin in the treatment of pts with APS and thrombosis but intensive anticoagulant effect is not always desired.

  4. Investigation of potential correlations between the antiphospholipid syndrome and some parasitary and bacterial infections in Romanian patients

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    Marian Ghervan

    2013-12-01

    Full Text Available The association of the antiphospholipid syndrome (APS with some parasitary and bacterial infections was investigated on a statistically significant group of 6,657 patients with various vasculary disorders (ocular and neurological, from district clinics of Bucharest and throughout the country. Patients were investigated in the ambulatory service of the Clinic of Neurosurgery of Saint Pantelimon Emergency Hospital, Bucharest, over a period of six years (2004-2009. Most of them (96.4 % were diagnosed with antiphospholipid syndrome, in the Clinic of Hematology, Fundeni Clinical Institute, Bucharest, using specific blood tests. The patients diagnised with antiphospholipid syndrome have been tested for nine visceral parasitary diseases and bacterial sepsis by three ways: i serological investigations (ELISA IgM for Toxoplasma gondii, Larva migrans visceralis, Cysticercus sp., Trichinella sp., Giardia intestinalis, Chlamydia trachomatis, Chlamydia pneumoniae, Mycoplasma pneumoniae, Borrelia burgdorferi sensu lato, and IgG for Toxoplasma gondii ii statistical analyses (Bravais-Pearson correlation coefficient R2 and iii evaluation of the effect of the anti-parasitary and antimicrobial treatments upon the symptoms of the antiphospholipid syndrome. A statistically significant positive linear correlation was established between the antiphospholipid syndrome and eight of the nine etiological agents, i.e. (Giardia intestinalis, Borrelia burgdorferi, Chlamydia trachomatis, Chlamydia pneumoniae, Larva migrans visceralis, Mycoplasma pneumoniae, Toxoplasma gondii, Cysticercus sp.. The etiologic treatments for parasitoses and bacterial sepsis have proved to eliminate the respective parasitic and bacterial agents, and also to improve the health status and to turn specific antiphospholipid syndrome tests into negative. Coexistence of the antiphospholipid syndrome with some parasitic and bacterial infection in 96.6 % of patients, the statistically positive

  5. Measuring IgA Anti-β2-Glycoprotein I and IgG/IgA Anti-Domain I Antibodies Adds Value to Current Serological Assays for the Antiphospholipid Syndrome

    Science.gov (United States)

    Pericleous, Charis; Ferreira, Isabel; Borghi, Orietta; Pregnolato, Francesca; McDonnell, Thomas; Garza-Garcia, Acely; Driscoll, Paul; Pierangeli, Silvia; Isenberg, David; Ioannou, Yiannis; Giles, Ian; Meroni, Pier Luigi; Rahman, Anisur

    2016-01-01

    Introduction Currently available clinical assays to detect antiphospholipid antibodies (aPL) test for IgG and IgM antibodies to cardiolipin (aCL) and β2-glycoprotein I (aβ2GPI). It has been suggested that testing for IgA aPL and for antibodies to Domain I (DI), which carries the key antigenic epitopes of β2GPI, could add value to these current tests. We performed an observational, multicenter cohort study to evaluate the utility of IgG, IgM and IgA assays to each of CL, β2GPI and DI in APS. Methods Serum from 230 patients with APS (n = 111), SLE but not APS (n = 119), and 200 healthy controls were tested for IgG, IgM and IgA aCL, aβ2GPI and aDI activity. Patients with APS were further classified into thrombotic or obstetric APS. Logistic regression and receiver operator characteristic analyses were employed to compare results from the nine different assays. Results All assays displayed good specificity for APS; IgG aCL and IgG aβ2GPI assays however, had the highest sensitivity. Testing positive for IgA aβ2GPI resulted in a higher hazard ratio for APS compared to IgM aβ2GPI. Positive IgG, IgM or IgA aDI were all associated with APS, and in subjects positive for aCL and/or aβ2GPI, the presence of aDI raised the hazard ratio for APS by 3–5 fold. IgG aCL, aβ2GPI, aDI and IgA aDI were associated with thrombotic but not obstetric complications in patients with APS. Conclusion Measuring IgG aDI and IgA aβ2GPI and aDI may be useful in the management of patients with APS, particularly thrombotic APS. PMID:27253369

  6. Diffusion tensor imaging in patients with obstetric antiphospholipid syndrome without neuropsychiatric symptoms

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, Fabricio R. [University Hospital Center of Nimes and Research Team EA 2415, Department of Radiology (France); Macri, Francesco; Beregi, Jean-Paul [University Hospital Center of Nimes and Research Team EA 2415, Department of Radiology (France); Montpellier University, Faculty of Medicine, Montpellier (France); Jackowski, Marcel P. [University of Sao Paulo, Department of Computer Science, Institute of Mathematics and Statistics, Sao Paulo (Brazil); Kostis, William J. [Harvard Medical School, Massachusetts General Hospital, Boston, MA (United States); Athinoula A. Martinos Center for Biomedical Imaging, Charlestown, MA (United States); Gris, Jean-Christophe [Montpellier University, Faculty of Medicine, Montpellier (France); University Hospital Center of Nimes, Department and Laboratory of Hematology (France); Mekkaoui, Choukri [University Hospital Center of Nimes and Research Team EA 2415, Department of Radiology (France); Montpellier University, Faculty of Medicine, Montpellier (France); Harvard Medical School, Massachusetts General Hospital, Boston, MA (United States); Athinoula A. Martinos Center for Biomedical Imaging, Charlestown, MA (United States)

    2016-04-15

    To evaluate white matter (WM) integrity in neurologically asymptomatic antiphospholipid syndrome (APS) using diffusion tensor imaging (DTI) in women with no thrombotic history but with pregnancy loss. Imaging was performed with a 3 T scanner using structural MRI (T1-weighted, fluid attenuation inversion recovery [FLAIR]) and DTI sequences in 66 women with APS and a control group of 17 women. Women with APS were further categorized as positive for lupus anticoagulant (LA) and/or aβ2GPI-G antibodies (LA/aβ2GPI-G-positive, N = 29) or negative (LA/aβ2GPI-G-negative, N = 37) for both. Tract-based spatial statistics of standard DTI-based indices were compared among groups. Women with APS had significantly lower fractional anisotropy (p < 0.05) associated with higher mean diffusivity and radial diffusivity compared to the control group. There was a stronger association of abnormal DTI features among women positive for LA and/or aβ2GPI-IgG antibodies than those who were negative. DTI appears sensitive to subtle WM changes in women with APS with no thrombotic history but with pregnancy loss, compatible with alterations in axonal structure and in the myelin sheath. The preferential association of abnormal DTI features with the two most pathogenic aPLAbs reinforces the pathophysiological relevance of our findings. (orig.)

  7. Adalimumab-associated antiphospholipid syndrome: a case report and review of the literature.

    Science.gov (United States)

    Hemmati, Iman; Kur, Jason

    2013-07-01

    This study aims for the presentation of the first reported case of adalimumab-associated antiphospholipid syndrome (APS) and review of the literature on adalimumab-induced vasculitis and APS. A case of APS associated with adalimumab use in a 67-year-old woman is reported. The English medical literature was reviewed for antitumor necrosis factor (TNF) agents and their association with APS and vasculitis. Adalimumab is a fully humanized monoclonal antibody targeted against TNF alpha that is widely used in the treatment of rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, psoriatic arthritis, psoriasis, and Crohn's disease. Literature review reveals several cases of anti-TNF-induced vasculitis including cases associated with adalimumab. We report the first case of adalimumab-induced APS in a 67-year-old woman who developed APS and vasculitis associated with de novo positive anti-cardiolipin (aCL) antibody following the third dose of adalimumab therapy for the treatment of spondyloarthropathy. This is the first case demonstrating that a short course of adalimumab therapy may induce immunoglobulin M aCL autoantibodies leading to APS. With the growing use of anti-TNF medications in immune-mediated and inflammatory diseases, adalimumab and other anti-TNF medications should be considered as a possible explanation for APS.

  8. Diffusion tensor imaging in patients with obstetric antiphospholipid syndrome without neuropsychiatric symptoms

    International Nuclear Information System (INIS)

    To evaluate white matter (WM) integrity in neurologically asymptomatic antiphospholipid syndrome (APS) using diffusion tensor imaging (DTI) in women with no thrombotic history but with pregnancy loss. Imaging was performed with a 3 T scanner using structural MRI (T1-weighted, fluid attenuation inversion recovery [FLAIR]) and DTI sequences in 66 women with APS and a control group of 17 women. Women with APS were further categorized as positive for lupus anticoagulant (LA) and/or aβ2GPI-G antibodies (LA/aβ2GPI-G-positive, N = 29) or negative (LA/aβ2GPI-G-negative, N = 37) for both. Tract-based spatial statistics of standard DTI-based indices were compared among groups. Women with APS had significantly lower fractional anisotropy (p < 0.05) associated with higher mean diffusivity and radial diffusivity compared to the control group. There was a stronger association of abnormal DTI features among women positive for LA and/or aβ2GPI-IgG antibodies than those who were negative. DTI appears sensitive to subtle WM changes in women with APS with no thrombotic history but with pregnancy loss, compatible with alterations in axonal structure and in the myelin sheath. The preferential association of abnormal DTI features with the two most pathogenic aPLAbs reinforces the pathophysiological relevance of our findings. (orig.)

  9. Antiphospholipid Antibodies in Women Undergoing In Vitro Fertilization Treatment: Clinical Value of IgA Anti-β2glycoprotein I Antibodies Determination

    Directory of Open Access Journals (Sweden)

    Odile Paulmyer-Lacroix

    2014-01-01

    Full Text Available Implantation failure could be related to antiphospholipid antibodies (aPL. We retrospectively analyzed the usefulness of aPL determination in women undergoing IVF. Conventional aPL of the antiphospholipid syndrome, lupus anticoagulant (LA, anticardiolipin antibodies (aCL, anti-β2glycoprotein I (aβ2GPI antibodies, and IgG and IgM isotypes as well as IgA isotype were analyzed in women presenting with at least two implantation failures after in vitro fertilization (IVF. In a population of 40 IVF patients, a total prevalence of 20% (8/40 of aPL was found, significantly different from that of the control population (100 healthy blood donors, P<0.0005. Among the panels of aPL tested, aβ2GPI IgA antibodies were the most prevalent (62.5% 5/8, significantly higher in IVF patients (12.5%, 5/40 than in controls (1%, 1/100 (P=0.01. No difference according to the numbers of IVF attempts and success of embryo implantation was found between aPL positive and negative IVF patients. In contrast, no accomplished pregnancy with full-term live birth was observed in aPL positive IVF patients. Altogether our data led us to propose aPL assessment, in particular aβ2GPI IgA antibodies, in support of IVF treated women. In a perspective way, an early aPL detection could be the basis for defining novel therapeutic strategy.

  10. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

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    Venkat Sainaresh Vellanki

    2013-01-01

    Full Text Available In the presence of systemic lupus erythematosus or related autoimmune disorders, antiphospholipid syndrome (APS is termed secondary APS. Pregnancy-related renal failure due to SAPS is rarely reported in the literature. We present the case of a young primgravida woman with bilateral renal cortical necrosis due to secondary APS in late pregnancy.

  11. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period

    DEFF Research Database (Denmark)

    Cervera, R; Serrano, R; Pons-Estel, G J;

    2015-01-01

    OBJECTIVES: To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later. METHODS: In 1999, we started an observational study of 1000 APS ...

  12. GINGIVAL HYPERPLASIA AND THE ANTIPHOSPHOLIPID SYNDROME WITH AUTOIMMUNE THROMBOCYTOPENIA – A CASE STUDY

    OpenAIRE

    M.C. Giurgiu; H.T. DUMITRIU

    2012-01-01

    Introduction. The periodontal disease is a microbial inflammatory pathology, to which local and/systemic favourizing factors are associated, leading finally to teeth losses. Scope. To establish an antibiotic treatment against a particular type of gingival hyperplasia, favourized by an antiphospholipid syndrome with thrombocytopenia. Materials and method. Following the realization of the antibiogramme, a systemic antibiotic treatment was given to a patient with gingi...

  13. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

    OpenAIRE

    Venkat Sainaresh Vellanki; Sriramnaveen Parvathina; Sivaramakrishna Gondi; Manjusha Yadla; Krishna Kishore Chenu; Sivakumar Vishnubhotla

    2013-01-01

    In the presence of systemic lupus erythematosus or related autoimmune disorders, antiphospholipid syndrome (APS) is termed secondary APS. Pregnancy-related renal failure due to SAPS is rarely reported in the literature. We present the case of a young primgravida woman with bilateral renal cortical necrosis due to secondary APS in late pregnancy.

  14. Evaluation of the hypolipidemic activity of 6,7-dimethoxycoumarin on placental tissue factor mRNA expression in experimental anti-phospholipid syndrome

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    Annamalai Amarnath

    2013-01-01

    Full Text Available Background: Anti-phospholipid syndrome is a thrombogenic and systemic autoimmune disorder that influences fetal life throughout gestation period. Over expression of tissue factor on the surface of monocyte(s is reported to be a major causative agent in inducing anti-phospholipid antibody-mediated placental thrombosis and fetal loss in pregnant women. The over expression of tissue factor is proposed to be due to high levels of blood cholesterol and oxidized lipoproteins. Objective: In this study, we report the lipid-lowering property and anti-tissue factor activity of one of the naturally occurring coumarin derivates 6,7-dimethoxycoumarin, found aplenty in Chinese medicinal plant Artemisia scoparia, and its effect on tissue factor mRNA expression in experimental anti-phospholipid syndrome. Materials and Methods: Adult female mice were immunized with cardiolipin and beta-2-glycoprotein-1 to induce experimental anti-phospholipid syndrome. Female mice with high titer of aCL were allowed to mate with male, and the female mice were treated with 6,7-dimethoxycoumarin on a daily dose of 5 mg/kg body weight from day 3 to day 15 of gestation. On day 18 of pregnancy, all the animals were dissected to measure biochemical parameters in blood, and TF mRNA expression levels were measured in placenta. Results: Treatment with 6,7-dimethoxycoumarin significantly reduced the levels of cholesterol and plasma lipids by its potent hypolipidemic property, which eventually reduced the over-expression tissue factor at mRNA levels in placenta. We believe that further studies in animal model would reveal the potential therapeutic properties of 6,7-dimethoxycoumarin against anti-phospholipid syndrome. Conclusion: The 6,7-dimethoxycoumarin is capable to reduce the expression of TF in placenta at the mRNA level and thrombus generation indirectly by its potent anti-TF and anti-oxidant activities.

  15. Antiphospholipid Syndrome - A Case Report of Pulmonary Thromboembolism, Followed with Acute Myocardial Infarction in Patient with Systemic Sclerosis

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    Marija Vavlukis

    2015-11-01

    CONCLUSION: The acquired antiphospholipid syndrome is common condition in patients with systemic autoimmune diseases, but relatively rare in patients with systemic sclerosis. Never the less, we have to be aware of it when treating the patients with systemic sclerosis.

  16. Vaccinations and secondary immune thrombocytopenia with antiphospholipid antibodies by human papillomavirus vaccine.

    Science.gov (United States)

    Bizjak, Mojca; Bruck, Or; Kanduc, Darja; Praprotnik, Sonja; Shoenfeld, Yehuda

    2016-04-01

    A 13-year-old girl developed immune thrombocytopenic purpura (ITP) and concomitant positive antiphospholipid antibodies (aPL) following vaccination with a quadrivalent human papillomavirus (HPV) vaccine. During the course of a disease, she developed clinical manifestation with bleeding and she was treated with intravenous immunoglobulins. Consequently, the number of her platelets remained critically low and she was put on corticosteroids and rituximab. Since then, her platelet count remain within the normal range, but her aPL are still present. PMID:27312165

  17. C-reactive protein in antiphospholipid syndrome: relationship with cardiovascular pathology

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    N V Seredavkina

    2009-01-01

    Full Text Available Objective. To assess relationship of high sensitivity C reactive protein (hsCRP level in pts with antiphospholipid syndrome (APS with clinico-laboratory features and cardiovascular pathology. Material and methods. 206 pts were included. 58 from them had primary APS (PAPS, 72 –systemic lupus erythematosus (SLE with APS and 76 – SLE. 29 from 76 pts of the latter group were positive on anticardiolipin antibodies (ACA – SLE with antiphospholipid antibodies (APhL and 47 – low positive or negative on ACA – SLE without APhL. 72 persons without autoimmune diseases were included into control group. CRP (with high sensitivity immuno-nephelometric assay, APhL (with solid phase immuno-enzyme assay, plasma lipids were evaluated, sonography with measurement of intima-media complex (IMC thickness of common carotid arteries, carotid artery bulbs and internal carotid arteries, electrocardiography (ECG, echocardiography (EchoCG, Holter ECG monitoring were performed. Results. HsCRP serum level in pts was significantly higher than in control: 2,55 [0,71; 7,04] mg/l (varied from 0,15 to 39,85 vs 0,68 [0,26; 1,97] mg/l (varied from 0,1 to 9,61, p<0,001. Most high hsCRP concentration was found in SLE with APS (p=0,02. HsCRP level in pts with PAPS with history of combined or isolated arterial thrombosis was significantly higher than in pts with SLE and APS having the same localization of thrombosis. HsCRP concentration less than 3 mg/l correlated with duration of postthrombotic period in pts with PAPS. HsCRP level also correlated with triglyceride concentration, body mass index, summated coronary risk and magistral arteries IMC thickness. Conclusion. HsCRP elevation in pts with APS was associated with development of combined and arterial thrombosis as well as with traditional risk factors of atherosclerosis.

  18. [Antiphospholipid syndrome with autoimmune hemolytic anemia which mimics thrombotic thrombocytopenic purpura].

    Science.gov (United States)

    Karasawa, Naoki; Taniguchi, Yasuhiro; Hidaka, Tomonori; Katayose, Keiko; Kameda, Takuro; Side, Kotaro; Shimoda, Haruko; Nagata, Kenji; Kubuki, Yoko; Matsunaga, Takuya; Shimoda, Kazuya

    2010-04-01

    A 67-year-old woman was admitted to the hospital for lethargy, fever, hemolytic anemia, thrombocytopenia, and consciousness disturbance. Direct Coombs test was positive, and anti-cardiolipin beta2-glycoprotein I antibody was detected. She was diagnosed with antiphospholipid syndrome complicated with autoimmune hemolytic anemia (AIHA). She demonstrated variable consciousness disturbance, inability to distinguish right from left, dysgraphia and dyscalculia. Multiple cerebral infarctions, especially dominant cerebral hemisphere infarctions, were observed on magnetic resonance imaging. A ventilation-perfusion scan demonstrated the presence of a ventilation-perfusion mismatch in both lung fields, and multiple veinous embolisms in the right femoral, bilateral the great saphenous and popliteal veins. Therefore, pulmonary embolism and thrombophlebitis were diagnosed. Based on these findings, it was necessary to distinguish this diagnosis from thrombotic thrombocytopenic purpura (TTP). As ADAMTS-13 activity was within the normal range, TTP was denied. Thereafter, the patient was treated with 1 mg/kg of prednisolone for AIHA, 3 mg of warfarin, and 3500 units of low-molecular-weight heparin for thrombosis, and her condition improved. PMID:20467225

  19. Antiphospholipid Syndrome: primary or secondary to Systemic Lupus Erythematosus? Description of a clinical case of avitaminosis D in premenopausal woman with pseudo-Cushing syndrome

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    Mauro Turrin

    2014-06-01

    Full Text Available Low vitamin D levels have been described in obese individuals and in some autoimmune diseases, such as Systemic Lupus Erythematosus (SLE and primary antiphospholipid syndrome (pAPS. In particular, more than 50% of premenopausal women with pAPS have hypovitaminosis D. In this issue we report a case of an obese, premenopausal, and hypertensive woman with pseudo-Cushing syndrome, affected by deep venous thrombosis associated with pulmonary embolism after rib fracture who presented hypovitaminosis D. 7 years before, diagnosis of pAPS had been made after the detection of thrombocytopenia (present at a young age and arterial ischemia of a lower limb. For seven years she was treated with acetylsalicylic acid without complications. We found positive anti-dsDNA antibodies, a triple antiphospholipid antibodies (aPL positivity and levels of vitamin D < 4 µg/l. The case report arises some questions: is vitamin D deficiency due to obesity or APS? Is the positivity of anti-dsDNA indicative of progression to SLE? Is preventive therapy with hydroxychloroquine indicated? Does the high-risk aPL profile justify a high-intensity and life-long anticoagulation regimen?http://dx.doi.org/10.7175/cmi.v8i2.912

  20. Transient Antiphospholipid Syndrome Associated with Primary Cytomegalovirus Infection: A Case Report and Literature Review

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    Tsuyoshi Nakayama

    2014-01-01

    Full Text Available Viral infection is known to induce transient autoimmunity in humans. Acute cytomegalovirus (CMV infection is implicated in occasional thrombosis formation. We here, for the first time, report a 19-year-old female who had an acute CMV infection, leading to a deep venous thrombosis and a pulmonary embolism along with transient appearance of lupus anticoagulant. The pathological role of antiphospholipid antibodies in CMV-mediated thrombosis is discussed.

  1. [Maternal adrenal necrosis in the third trimester of pregnancy: a rare complication of antiphospholipid syndrome].

    Science.gov (United States)

    Legendre, G; Vauthier-Brouzes, D; Cornet, A; Al Hawari, M; Renard-Penna, R; Piette, J-C; Dommergues, M

    2008-04-01

    Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy.

  2. AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION): 5-Year Update.

    Science.gov (United States)

    Barbhaiya, Medha; Andrade, Danieli; Erkan, Doruk

    2016-10-01

    Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) is the first-ever international network created to design and conduct large-scale, multicenter clinical trials and research in persistently antiphospholipid antibody (aPL)-positive patients. Since its inception in 2010, the APS ACTION has made important strides toward our goal of international research collaboration and data sharing. Through the dedication and hard work of 50 APS ACTION members, collaborative international projects are currently underway including a multicenter web-based registry and repository of aPL-positive patients, a randomized controlled clinical trial assessing the efficacy of hydroxychloroquine for primary thrombosis prevention in persistently aPL-positive but thrombosis-free patients, standardization of aPL testing through the use of core laboratories worldwide, identification of the limitations in the existing aPL/APS literature, and conducting observational research studies to further our understanding of the disease. Thus far, APS ACTION has held annual workshops and summits with the aim of facilitating international collaboration and developing initiatives to recruit young scholars to APS research. This paper describes updates related to the organization's structure, ongoing research efforts, and recent accomplishments and discusses future directions. PMID:27646150

  3. A role for Toll-like receptor mediated signals in neutrophils in the pathogenesis of the anti-phospholipid syndrome.

    Directory of Open Access Journals (Sweden)

    Gerd Gladigau

    Full Text Available The anti-phospholipid syndrome (APS is characterized by recurrent thrombosis and occurrence of anti-phospholipid antibodies (aPL. aPL are necessary, but not sufficient for the clinical manifestations of APS. Growing evidence suggests a role of innate immune cells, in particular polymorphonuclear neutrophils (PMN and Toll-like receptors (TLR to be additionally involved. aPL activate endothelial cells and monocytes through a TLR4-dependent signalling pathway. Whether this is also relevant for PMN in a similar way is currently not known. To address this issue, we used purified PMN from healthy donors and stimulated them in the presence or absence of human monoclonal aPL and the TLR4 agonist LPS monitoring neutrophil effector functions, namely the oxidative burst, phagocytosis, L-Selectin shedding and IL-8 production. aPL alone were only able to induce minor activation of PMN effector functions at high concentrations. However, in the additional presence of LPS the activation threshold was markedly lower indicating a synergistic activation pathway of aPL and TLR in PMN. In summary, our results indicate that PMN effector functions are directly activated by aPL and boosted by the additional presence of microbial products. This highlights a role for PMN as important innate immune effector cells that contribute to the pathophysiology of APS.

  4. Pre-conception preparation at the antiphospholipid syndrome as way to improve reproductive health

    OpenAIRE

    Gulyash Tanysheva; Saule Kabylova; Sholpan Kinayatova; Aizat Zhumazhanova

    2014-01-01

    Introduction: Reproductive health is characterized by the condition of the woman in association with the course of pregnancy and childbirth. In this case, the absence of disease plays a fundamental role. Unfortunately, conditions that can negatively impact reproductive health and cause deterioration of pregnancy and delivery outcomes are frequent in women of reproductive age. Antiphospholipid syndrome (APS) is one of the leading conditions that can negatively affect reproductive health and le...

  5. Pulmonary manifestations of systemic lupus erythematosus patients with and without antiphospholipid syndrome

    OpenAIRE

    Hamdani, Muhammad Afzal; Saud Al-Arfaj, Abdul Rahman; Parvez, Khalid; Naseeb, Faisal; Ibrahim, Abdalla El Fateh; Cal, Joseph Hope

    2015-01-01

    Objective: To uncover the pulmonary manifestations of Systemic Lupus Erythematosus (SLE) patients alone and to compare findings with antiphospholipid syndrome (APS) associated with SLE. Methods: This cross sectional comparative study was carried out at King Khalid University Hospital (KKUH)/King Saud University (KSU), a tertiary care hospital, Riyadh, Kingdom of Saudi Arabia. From June 2012 to March 2014, 96 diagnosed SLE patients with respiratory symptoms were included in the study and divid...

  6. Transient Pulmonary Edema Following Adrenal Infarction in a Patient with Primary Anti-Phospholipid Syndrome

    OpenAIRE

    Ozawa, K; Tazawa, K.; D. Kishida; Fukushima, K.; Matsuda, M.; Ikeda, S

    2012-01-01

    We report a patient with primary anti-phospholipid syndrome (APS) who developed pulmonary edema following sudden-onset pain in the left, lower back of the chest. Radiological examinations demonstrated fresh infarction of the left adrenal gland but no obvious thrombi in pulmonary arteries. The patient quickly recovered from pulmonary edema with anti-coagulation therapy alone. Primary APS may have caused adrenal infarction in the patient, leading to transient pulmonary edema via microthrombosis...

  7. 抗磷脂综合征与妊娠丢失的研究进展%Research progress in relationship of antiphospholipid syndrome and pregnancy loss

    Institute of Scientific and Technical Information of China (English)

    王艳梅; 凌国灿

    2012-01-01

    自身免疫性疾病或免疫功能异常影响妊娠,在自身免疫疾病中,抗磷脂综合征(APS)占了一定比例.本文就抗磷脂抗体(APA)的形成及APA导致妊娠丢失的作用机制进行阐述,旨为今后APS的临床治疗带来更多的启示.%Autoimmue disease or abnormal immunologic function can affect pregnancy. In autoimmune diseases, the antiphospholipid syndrome (APS) has accounted for a certain proportion. This paper illustrated the formation of antiphospholipid antibody (APA) and the mechanism of pregnancy loss caused by APA. It brings some enlightenment for the future APS treatment.

  8. Frequency of antiphospholipid antibodies in patients with infectious diseases using three different ELISA methods Freqüência de anticorpos antifosfolípides em pacientes com doenças infecciosas usando três diferentes testes de ELISA

    OpenAIRE

    Mittermayer Barreto Santiago; Reinaldo Martinelli; Reis, Mitermayer G.; Eliana Almeida Reis; Albert Ko; Roberto Dias Fontes; Moacir Paranhos Silva; Eliane Goes Nascimento; Ricardo Espinola; Nigel Harris; Azzudin Gharavi; Silvia Pierangeli

    2006-01-01

    OBJECTIVE: The standard enzyme-linked immunosorbent assay (ELISA) for anticardiolipin (aCL) antibodies is the most important test for the diagnosis of antiphospholipid syndrome (APS). However, the test is also positive in some infectious diseases and other non-related syndromes. It has been suggested that the detection of antibodies to a mixture of phospholipids or to beta2-glycoprotein I (beta2-GP I) has higher specificity for APS than the standard aCL ELISA. The aim of the present work is t...

  9. Avaliação clínico-laboratorial de pacientes com síndrome antifosfolípide primária segundo a frequência de anticorpos antinucleares (FAN Hep-2 Clinical and laboratory evaluation of patients with primary antiphospholipid syndrome according to the frequency of antinuclear antibodies (ANA Hep-2

    Directory of Open Access Journals (Sweden)

    Jozélio Freire de Carvalho

    2010-06-01

    Full Text Available OBJETIVO: Avaliar a frequência de manifestações clínicas e laboratoriais em pacientes com síndrome antifosfolípide primária (SAFP com anticorpos antinucleares positivos (FAN Hep-2+, comparados àqueles com esses anticorpos negativos (FAN Hep-2 -. PACIENTES E MÉTODOS: Estudo transversal em 58 pacientes (82,8% mulheres com SAFP. Foram avaliados os dados demográficos, clínicos, comorbidades, medicações e anticorpos antifosfolípides. RESULTADOS: Dos 58 pacientes incluídos no estudo, vinte (34,5% apresentaram presença de FAN Hep-2. Comparando-se o grupo de pacientes FAN Hep-2+ com aqueles FAN Hep-2 -, verificou-se que ambos os grupos de pacientes com SAFP não diferiram estatisticamente em relação aos dados demográficos, bem como em relação ao tempo de doença. Em relação às manifestações clínicas e laboratoriais, o grupo com FAN Hep-2 + apresentou maior frequência de trombose venosa profunda (85 versus 52,6%, P = 0,04, uma frequência estatística e significativamente maior de anticardiolipina IgG (85 versus 52,6%, P = 0,02 e uma tendência para anticardiolipina IgM (80% versus 52,6%, P = 0,05, bem como maiores medianas desses anticorpos [33 (0-128 versus 20 (0-120 GPL, P = 0,008] e [33 (0-120 versus 18,5 (0-120 MPL, P = 0,009]. Tal diferença não foi observada no que se refere a outras manifestações da SAF, presença de comorbidades, estilo de vida e uso de medicações. CONCLUSÃO: Pacientes com SAFP que apresentam FAN Hep-2+ têm maior frequência de trombose venosa profunda e anticardiolipinas IgG e IgM.OBJECTIVE: To evaluate the frequency of clinical and laboratory manifestations in patients with primary antiphospholipid syndrome (PAPS with positive antinuclear antibodies (ANA Hep-2+ compared to those in whom this antibody is negative (ANA Hep-2-. PATIENTS AND METHODS: This is a transversal study with 58 patients (82.8% females with PAPS. Demographic and clinical data, comorbidities, medications, and

  10. Anti-Phospholipid Antibodies in Patients Undergoing Total Joint Replacement Surgery

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    Melissa Simpson

    2012-01-01

    Full Text Available Background. Patients undergoing joint replacement remain at increased risk for venous thromboembolism (VTE compared to other types of surgery, regardless of thromboprophylactic regimen. The pathophysiologic processes rendering this group of patients at risk for VTE are multifactorial. Procedure-specific and patient-specific exposures play a role in the postoperative development of VTE, including the development of anti-phospholipid antibodies (aPL. Methods. We measured three aPL (anti-cardiolipin, anti-β2 glycoprotein, and lupus anticoagulant in 123 subjects undergoing total knee or hip arthroplasty to describe the presence of these antibodies preoperatively and to describe the rate of postoperative seroconversion among those people who were negative preoperatively. Postoperative antibodies were measured at day 7, 14, and 21. Results. The prevalence of aPL antibodies in the preoperative period was 44%, positive subjects were more likely to be smokers (P=0.05 and were less likely to have undergone a previous arthroplasty procedure (P=0.002. Subjects seroconverted in a 21 day postoperative period at a rate of 79%. Conclusions. These pilot data suggest that the prevalence of aPL in this population both preoperatively and postoperatively is higher than previously expected. Further studies are needed to describe aPL in a larger population and to establish their clinical significance in populations undergoing joint replacement surgeries.

  11. Clinical and neuroimaging correlates of antiphospholipid antibodies in multiple sclerosis: a preliminary study

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    Gonzalez-Toledo Eduardo

    2007-10-01

    Full Text Available Abstract Background The presence of antiphospholipid antibodies (APLA in multiple sclerosis (MS patients has been reported frequently but no clear relationship between APLA and the clinical and neuroimaging features of MS have heretofore been shown. We assessed the clinical and neuroimaging features of MS patients with plasma APLA. Methods A consecutive cohort of 24 subjects with relapsing-remitting (RR MS were studied of whom 7 were in remission (Rem and 17 in exacerbation (Exc. All subjects were examined and underwent MRI of brain. Patients' plasma was tested by standard ELISA for the presence of both IgM and IgG antibodies using a panel of 6 targets: cardiolipin (CL, β2 glycoprotein I (β2GPI, Factor VII/VIIa (FVIIa, phosphatidylcholine (PC, phosphatidylserine (PS and phosphatidylethanolamine (PE. Results In exacerbation up to 80% of MS subjects had elevated titers of IgM antibodies directed against the above antigens. However, in remission, less than half of MS patients had elevated titers of IgM antibodies against one or more of the above antigens. This difference was significant, p Conclusion The findings of this preliminary study show that increased APLA IgM is associated with exacerbations of MS. Currently, the significance of this association in pathogenesis of MS remains unknown. However, systematic longitudinal studies to measure APLA in larger cohorts of patients with relapsing-remitting MS, particularly before and after treatment with immunomodulatory agents, are needed to confirm these preliminary findings.

  12. Immunization with anticardiolipin cofactor (beta-2-glycoprotein I) induces experimental antiphospholipid syndrome in naive mice.

    Science.gov (United States)

    Blank, M; Faden, D; Tincani, A; Kopolovic, J; Goldberg, I; Gilburd, B; Allegri, F; Balestrieri, G; Valesini, G; Shoenfeld, Y

    1994-08-01

    Beta-2-GPI is a 50 kDa glycoprotein which is known to be a serum co-factor, with a role in determining the binding of pathogenic anticardiolipin antibodies to phospholipids. Immunization of naive mice with beta-2-GPI resulted in elevated levels of antibodies directed against negatively charged phospholipids (cardiolipin, phosphotidylserine, phosphatidylinositol). The presence of increased titres of antiphospholipid antibodies in the sera of the mice was later followed by prolonged activated partial thromboplastin time (APTT), thrombocytopenia, and when the mice were mated, by a high percentage of fetal resorptions in the uterus. These data point to the ability of beta-2-GPI to induce pathogenic anti-cardiolipin antibodies following active immunization. PMID:7980847

  13. The role of anti-phospholipid antibodies in autoimmune reproductive failure.

    Science.gov (United States)

    Pantham, Priyadarshini; Abrahams, Vikki M; Chamley, Lawrence W

    2016-05-01

    Anti-phospholipid antibodies (aPL) are autoantibodies that are associated with thrombosis and a range of pregnancy complications including recurrent pregnancy loss and pre-eclampsia. The three clinically relevant, well-characterized aPL are anti-cardiolipin antibodies, lupus anticoagulant and anti-beta-2-glycoprotein I (β2GPI) antibodies. aPL do not bind directly to phospholipids but instead bind to a plasma-binding 'cofactor'. The most extensively studied cofactor is β2GPI, whose role in pregnancy is not fully elucidated. Although the pathogenicity of aPL in recurrent pregnancy loss is well established in humans and animal models, the association of aPL with infertility does not appear to be causative. aPL may exert their detrimental effects during pregnancy by directly binding trophoblast cells of the placenta, altering trophoblast signalling, proliferation, invasion and secretion of hormones and cytokines, and by increasing apoptosis. Heparin is commonly used to treat pregnant women with aPL; however, as thrombotic events do not occur in the placentae of all women with aPL, it may exert a protective effect by preventing the binding of aPL to β2GPI or by acting through non-thrombotic pathways. The aim of this review is to present evidence summarizing the current understanding of this field. PMID:26884418

  14. Immunoadsorbent plasmapheresis for a patient with antiphospholipid syndrome during pregnancy.

    OpenAIRE

    Kobayashi, S; Tamura, N.; Tsuda, H.; Mokuno, C; Hashimoto, H.; Hirose, S

    1992-01-01

    The case of a 34 year old woman with systemic lupus erythematosus with a history of three previous recurrent abortions and lupus anticoagulant and anticardiolipin antibodies is reported. Immunoadsorbent plasmapheresis with a dextran sulphate column was used to remove lupus anticoagulant, anticardiolipin antibodies, and antibodies to DNA during her fourth pregnancy in combination with low doses of aspirin and prednisolone. Although during the course of treatment prednisolone was transiently in...

  15. USE OF ENZYMES IN COMPLEX TREATMENT OF ANTIPHOSPHOLIPID SYNDROME IN WOMEN WITH REPRODUCTIVE LOSSES OF ANDIJAN STATE

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    Gulnoza Maniyozova

    2014-11-01

    Full Text Available Obstetric complications developing at antiphospholipid syndrome are serious medical-social problem. Seeking of the treatment methods sets conditions for urgency of the article reviewed. Enzyme preparations are widely applied for treatment of women with fecundity disorder and hemorheology changes. Research objective was in clinico-laboratorial evaluation of Serrata drug impact in antiphospholipid syndrome in women with reproductive losses of Andijan state. Methods: Data of 43 women in the non-pregnancy state with reproductive losses (RL in past history with revealed APS had been studied by us. Results: Thus, conducted therapy with Serrata was conductive to haemostasis. Conducted researches confirm the safety of application.

  16. Prevalence and isotype distribution of antiphospholipid antibodies in unselected Chilean patients with venous and arterial thrombosis.

    Science.gov (United States)

    Palomo, Iván; Pereira, Jaime; Alarcón, Marcelo; Vásquez, Marcela; Pinochet, Carmen; Vélez, María T; Sandoval, Jorge; Icaza, Gloria; Pierangeli, Silvia

    2004-04-01

    Antiphospholipid antibodies (aPL) are a heterogeneous family of antibodies associated with thrombotic events and other complications. The objective of this study was to investigate the prevalence of aPL in a group of Chilean patients with thrombosis. Two hundred and twenty-six patients with venous and arterial thrombosis and 95 healthy controls were studied. Anticardiolipin (aCL), anti-beta(2 )glycoprotein I (anti-beta(2)GPI), and antiprothrombin (aPT) antibodies were determined. Eighty-eight out of 226 (38.9%) patients with thrombosis had some type of aPL. Fifty-seven patients (25.2%) were positive for aCL, 31 (13.7%) for aPT, and 14 (6.2%) for anti-beta(2)GPI antibodies. Twelve patients (5.3%) were positive for more than one aPL. IgG, IgM and IgA isotypes were observed in aCL, anti-beta(2)GPI, and aPT antibodies. Twenty-six out of 92 (28.3%) patients with venous thrombosis and 31/134 (23.1%) patients with arterial thrombosis were positive for aCL antibodies. With regard to the control group (4/95=4.2%), the odd ratios (OR) were 5.2 (1.3-19.8; p0.01) and 5.7 (1.6-22.3; p0.01), respectively. Additionally, we observed statistically significant OR with aPT and anti-beta(2)GPI antibodies; in the first, with venous and arterial thrombosis, and in the second, only with arterial thrombosis. Our results show a significant prevalence of aPL, predominantly aCL and aPT antibodies, in patients with thrombosis. Additionally, aCL and aPT antibodies appear to be a risk factor for venous and arterial thrombosis, and anti-beta(2)GPI antibodies appear to be a risk factor for arterial thrombosis. PMID:15045627

  17. Coagulopathy triggered autoimmunity: experimental antiphospholipid syndrome in factor V Leiden mice

    Science.gov (United States)

    2013-01-01

    Background We investigated interactions between genetically and autoimmune-mediated coagulopathies by inducing experimental antiphospholipid syndrome (eAPS) in mice carrying the factor V Leiden (FVL) mutation. Methods eAPS was induced in heterozygous and homozygous FVL transgenic mice (C57BL/6 background) by immunization with β2-glycoprotein I (β2-GPI). Autoantibody levels were measured at 1 and 5 months post-immunization. Mice were tested at 4 months post-immunization for behavior and cognitive function in the staircase, elevated plus-maze, and swim T-maze tests. Brains were removed and analyzed by immunohistochemistry for inflammatory markers and neurodegenerative processes. Results A single immunization with β2-GPI induced significantly higher and longer-lasting immune responses, and this was dependent on the number of FVL alleles. At 1 and 5 months post-immunization, levels of antibodies rose from 1.17 ± 0.07 to 1.62 ± 0.17 (optical density units; ODU) in homozygous FVL mice, compared with stable levels of 0.59 ± 0.17 and 0.48 ± 0.16 ODU in heterozygous FVL mice and a drop from 1.62 ± 0.21 to 0.61 ± 0.13 ODU in wild-type mice. Behavioral and cognitive clinical features of eAPS were also correlated with FVL allele load, as assessed by the elevated plus-maze (altered anxiety), staircase (hyperactivity and higher exploration), and swim T-maze (impaired learning) tests. Histological studies identified significant neurodegenerative changes in both grey and white matter in the eAPS-FVL brains. In spite of the potential interaction of two prothrombotic disease states, there were no ischemic lesions seen in this group. Conclusions The results indicate that genetically mediated coagulopathies increase the risk of developing coagulation-targeted autoimmune responses, and suggest the importance of antibody-mediated neurodegenerative processes in the brain in APS. PMID:23566870

  18. Myocardial infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    ZHANG Bo; JIANG Da-ming; ZHOU Xu-chen; QI Guo-xian

    2011-01-01

    This case report we presented aims to report a-31-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) who developed myocardial infarction (Ml) and also aims to discuss the possible mechanisms. The results showed that traditional risk factors alone do not cause coronary heart disease with SLE, and SLE-related factors influence the atherogenic process. We found that although SLE patients with acute Ml benefit from percutaneous coronary intervention (PCI) therapy, it is very important to choose the reasonable antithrombotic strategies in patients with SLE and APS undergoing PCI who require oral anticoagulant therapy.

  19. Imaging findings in the rare catastrophic variant of the primary antiphospholipid syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Thuerl, Christina; Altehoefer, Carsten; Laubenberger, Joerg [Freiburg Univ. (Germany). Abt. Radiologie; Spyridonidis, Alexandros [Freiburg Univ. (DE). Abt. Innere Medizin 1 (Haematologie und Onkologie)

    2002-03-01

    We report imaging findings in a case of the rare catastrophic variant of antiphospholipid syndrome (CAPS) characterized by widespread microvascular occlusions, which may lead to multiple organ failure. We present a case of a 66-year-old woman with bone marrow necrosis, acute acalculous cholecystitis (AAC), focal liver necrosis, subtle patchy splenic infarctions, and bilateral adrenal infarction. The demonstration of multiple microvascular organ involvement (three or more) is crucial for the diagnosis of the catastrophic variant of APS. This can be performed radiologically intra-vitam. Imaging can even reveal subclinical microinfarctions, which are often only diagnosed at autopsy. (orig.)

  20. Effect of Low Molecular Weight Heparins (LMWHs on antiphospholipid Antibodies (aPL-mediated inhibition of endometrial angiogenesis.

    Directory of Open Access Journals (Sweden)

    Silvia D'Ippolito

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity in the presence of circulating antiphospholipid antibodies (aPL. Different pathogenic mechanisms for aPL-mediated pregnancy failure have been proposed. In particular a direct effect of aPL on both maternal and fetal side of the placental tissue has been reported, since their reactivity with β2-glycoprotein I (β2GPI makes them adhere to trophoblast and human endometrial endothelial cell (HEEC membranes. β2GPI can be recognized by aPL that, once bound, interfere with both trophoblast functions and with the HEEC differentiation.APS patients can be successfully treated with Low Molecular Weight Heparin (LMWH. Recent reports suggest that LMWH acts through mechanisms alternative to its well known anticoagulant effect, because of its ability to bind β2GPI. In our previous studies, we showed that LMWH is able to reduce the aPL binding to trophoblasts and restore cell invasiveness and differentiation. So far, however, no study has described its effects on endometrial angiogenesis.The aim of our research was to evaluate whether two LMWHs, tinzaparin and enoxaparin, have an effect on the aPL-inhibited endometrial angiogenesis. This prompted us to investigate: (i in vitro HEEC angiogenesis through a Matrigel assay; (ii VEGF secretion by ELISA; (iii matrix metalloproteinase-2 (MMP-2 activity by gelatin zymography; (iv Nuclear Factor-κB (NF-κB DNA binding activity by colorimetric assay; (v STAT-3 activation by a sandwich-ELISA kit. Furthermore, using an in vivo murine model we investigated the LMWHs effects on angiogenesis.We demonstrated that the addition of LMWHs prevents aPL-inhibited HEEC angiogenesis, both in vitro and in vivo, and is able to restore the aPL inhibited NF-κB and/or STAT-3 activity, the VEGF secretion and the MMPs activity.The demonstration of a beneficial role for LMWHs on the aPL-inhibited HEEC angiogenesis

  1. THE STUDY OF PRODUCTION AND MECHANISM OF ANTIPHOSPHOLIPID ANTIBODIES IN PATIENTS WITH CORONARY HEART DISEASE

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To assess whether there was strong association between antiphospholipid antibodies(APA) and coronary heart disease(CHD), to study the environmental factors of APA production and APA pathogenic mechanism in patients with CHD.Methods Blood samples from 76 patients with CHD and 30 controls were tested for anticardiolipin antibodies IgG(ACA-IgG),human cytomegalovirus IgG,IgM(HCMV-IgG,IgM) by enzyme-link immunosorbant assay(ELISA) and 6-keto-PGF1a,endothelin(ET) by radioimmunoassay(RIA).Results A total of 27 patients(35.53%) were ACA positive in 76, as compared to 2 of 30(6.67%) healthy individuals, P<0.05. There was no difference in ACA among acute myocardial infarction(AMI,39.13%), old myocardial infarction(OMI,26.53%), unstable angina pectoris(UA,38.40%), P>0.05. The number of ACA positive subjects was higher in HCMV infection patients with CHD than no HCMV infectious patients with CHD. There was no PGI2 and ET level difference between ACA-IgG positive and negative CHD.Conclusion There are strong association between APA and CHD. The HCMV infection may be an environmental factor of APA production in CHD patients with raised ACA. The alteration of PGI2 and ET are not the pathogenic mechanism of ACA in patients with CHD.

  2. The hidden world of anti-phospholipid antibodies and female infertility: A literature appraisal.

    Science.gov (United States)

    Chighizola, Cecilia B; de Jesus, Guilherme R; Branch, D Ware

    2016-06-01

    Even though the association of anti-phospholipid antibodies (aPL) with infertility is debated, infertile women are commonly screened for aPL. To review evidence, a systematic PubMed search was conducted to retrieve papers addressing (i) the association between aPL and infertility, (ii) the positivity rate of criteria and non-criteria aPL in women with infertility, (iii) the association between aPL and assisted reproduction technologies (ART) outcome, (iv) the efficacy of medical treatments on ART outcome, and (v) the effects of ART on thrombotic risk. A total of 46 papers were considered; several limitations emerged: (i) wide heterogeneity in study populations, (ii) non-prospective design in 90% of studies, and (iii) aPL cutoffs not conforming to international guidelines in more than 75% of studies; aPL positivity not confirmed in 89% of studies. Most studies evinced an association between infertility and anti-β2GPI antibodies and almost all non-criteria aPL. The association rate with infertility was below 50% for lupus anti-coagulant, anti-cardiolipin antibodies (aCL), and anti-phosphatidic acid antibodies. According to our estimates, overall positivity rates of criteria and non-criteria aPL tests are 6% and 3% among infertile women, 1% and 2% among controls, respectively. A significant difference in the positivity rate of patients versus controls emerged for aCL only. Five of 18 studies reported a detrimental effect of aPL on ART outcome. Only one of the six studies assessing the effects of treatment on ART outcome among aPL-positive infertile women reported a benefit. All relevant studies reported no increase in the rate of thrombosis among aPL-positive women undergoing ART. PMID:26827907

  3. GINGIVAL HYPERPLASIA AND THE ANTIPHOSPHOLIPID SYNDROME WITH AUTOIMMUNE THROMBOCYTOPENIA – A CASE STUDY

    Directory of Open Access Journals (Sweden)

    M.C. Giurgiu

    2012-12-01

    Full Text Available Introduction. The periodontal disease is a microbial inflammatory pathology, to which local and/systemic favourizing factors are associated, leading finally to teeth losses. Scope. To establish an antibiotic treatment against a particular type of gingival hyperplasia, favourized by an antiphospholipid syndrome with thrombocytopenia. Materials and method. Following the realization of the antibiogramme, a systemic antibiotic treatment was given to a patient with gingival hyperplasia and antiphospholipid syndrome with autoimmune thrombocytopenia. Periodontal evolution was followed by monitorization of the periodontal indices: plaque, calculus, bleeding and periodontal pockets indices. Results. Thrombocytopenia and the cortisone treatment required a complex antibiotic treatment, followed by gingival debridement, after normalization of thrombocytes, by periodontal surgical therapy. 4 weeks after the end of the periodontal treatment, no periodontal pockets were present, any more. Conclusions. The general condition of the patient plays an important role in the evolution and treatment of the periodontal diseases. Treatment of gingival hyperplasia requires antibiotherapy and, in some cases, even administration of antibiotics

  4. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period : A multicentre prospective study of 1000 patients

    NARCIS (Netherlands)

    Cervera, R.; Serrano, R.; Pons-Estel, G. J.; Ceberio-Hualde, L.; Shoenfeld, Y.; De Ramón, E.; Buonaiuto, V.; Jacobsen, S.; Zeher, M. M.; Tarr, T.; Tincani, A.; Taglietti, M.; Theodossiades, G.; Nomikou, E.; Galeazzi, M.; Bellisai, F.; Meroni, P. L.; Derksen, R. H W M; De Groot, P. G D; Baleva, M.; Mosca, S.; Bombardieri, M.; Houssiau, F.; Gris, J. C.; Quéré, I.; Hachulla, E.; Vasconcelos, C.; Fernández-Nebro, A.; Haro, M.; Amoura, Z.; Miyara, M.; Tektonidou, M.; Espinosa, G.; Bertolaccini, M. L.; Khamashta, M. A.

    2015-01-01

    Objectives: To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later. Methods: In 1999, we started an observational study of 1000 APS pat

  5. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period : a multicentre prospective study of 1000 patients

    NARCIS (Netherlands)

    Cervera, R.; Khamashta, M. A.; Shoenfeld, Y.; Camps, M. T.; Jacobsen, S.; Kiss, E.; Zeher, M. M.; Tincani, A.; Kontopoulou-Griva, I.; Galeazzi, M.; Bellisai, F.; Meroni, P. L.; Derksen, R. H. W. M.; de Groot, P. G.; Gromnica-Ihle, E.; Baleva, M.; Mosca, M.; Bombardieri, S.; Houssiau, F.; Gris, J-C; Quere, I.; Hachulla, E.; Vasconcelos, C.; Roch, B.; Fernandez-Nebro, A.; Piette, J-C; Espinosa, G.; Bucciarelli, S.; Pisoni, C. N.; Bertolaccini, M. L.; Boffa, M-C; Hughes, G. R. V.

    2009-01-01

    Objectives: To identify the main causes of morbidity and mortality in patients with antiphospholipid syndrome (APS) during a 5-year period and to determine clinical and immunological parameters with prognostic significance. Methods: The clinical and immunological features of a cohort of 1000 patient

  6. Human monoclonal antiphospholipid antibodies disrupt the annexin A5 anticoagulant crystal shield on phospholipid bilayers: evidence from atomic force microscopy and functional assay.

    Science.gov (United States)

    Rand, Jacob H; Wu, Xiao-Xuan; Quinn, Anthony S; Chen, Pojen P; McCrae, Keith R; Bovill, Edwin G; Taatjes, Douglas J

    2003-09-01

    The antiphospholipid (aPL) syndrome is an autoimmune condition that is marked by recurrent pregnancy losses and/or systemic vascular thrombosis in patients who have antibodies against phospholipid/co-factor complexes. The mechanism(s) for pregnancy losses and thrombosis in this condition is (are) not known. Annexin A5 is a potent anticoagulant protein, expressed by placental trophoblasts and endothelial cells, that crystallizes over anionic phospholipids, shielding them from availability for coagulation reactions. We previously presented data supporting the hypothesis that aPL antibody-mediated disruption of the anticoagulant annexin A5 shield could be a thrombogenic mechanism in the aPL syndrome. However, this has remained a subject of controversy. We therefore used atomic force microscopy, a method previously used to study the crystallization of annexin A5, to image the effects of monoclonal human aPL antibodies on the crystal structure of the protein over phospholipid bilayers. In the presence of the aPL monoclonal antibodies (mAbs) and beta(2)-GPI, the major aPL co-factor, structures presumed to be aPL mAb-antigen complexes were associated with varying degrees of disruption to the annexin A5 crystallization pattern over the bilayer. In addition, measurements of prothrombinase activity on the phospholipid bilayers showed that the aPL mAbs reduced the anti-coagulant effect of annexin A5 and promoted thrombin generation. These data provide morphological evidence that support the hypothesis that aPL antibodies can disrupt annexin A5 binding to phospholipid membranes and permit increased generation of thrombin. The aPL antibody-mediated disruption of the annexin A5 anticoagulant shield may be an important prothrombotic mechanism in the aPL syndrome. PMID:12937161

  7. Thin-section chest CT findings in systemic lupus erythematosus with antiphospholipid syndrome: A comparison with systemic lupus erythematosus without antiphospholipid syndrome

    International Nuclear Information System (INIS)

    Purpose: To assess thin-section chest CT findings in systemic lupus erythematosus (SLE) with antiphospholipid syndrome (APS), in comparison with SLE without APS. Materials and methods: We retrospectively reviewed the medical records and thin-section CT findings of 17 consecutive patients with an established diagnosis of SLE with APS, comparing with 37 consecutive SLE patients without APS, between 2004 and 2008, and patients who had other autoimmune disease, such as Sjögren syndrome, were excluded. No significant differences were seen between the two groups in age, gender, smoking habits, or history of steroid pulse and biological therapy. CT images of 2 mm thickness obtained with a 16- or 64-detector row CT were retrospectively evaluated by two radiologists in consensus on ultra high-resolution gray-scale monitors. Results: The frequency of thin-section CT abnormalities was higher in SLE with APS group (82%) than in SLE without APS group (43%). Ground-glass opacity (59%), architectural distortion (47%), reticulation (41%), enlarged peripheral pulmonary artery (29%), and mosaic attenuation (29%) were significantly more common in the SLE with APS group than in the SLE without APS group (Fisher's exact test, p < 0.01). Conclusion: SLE patients with APS have increased prevalence of thin-section chest CT abnormalities than those without APS.

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

    Institute of Scientific and Technical Information of China (English)

    陈李华; 姜玲玲; 厉有名; 彭清璧

    2003-01-01

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

  9. Prevalence of deep venous thrombosis in the lower limbs and the pelvis and pulmonary embolism in patients with positive antiphospholipid antibodies

    Energy Technology Data Exchange (ETDEWEB)

    Kinuya, Keiko; Kakuda, Kiyoshi; Matano, Sadaya; Sato, Shigehiko; Sugimoto, Tatsuho [Tonami General Hospital, Toyama (Japan); Asakura, Hidesaku; Kinuya, Seigo; Michigishi, Takatoshi; Tonami, Norihisa

    2001-12-01

    Antiphospholipid antibodies (AA) are immunoglobulins that cross-react with phospholipid on cell membrane, and are therefore associated with a hypercoagulable state manifested by arterial/venous thromboses. We aimed to determine the prevalence of deep venous thrombosis in the lower limbs and the pelvic region (DVT) and pulmonary embolism (PE) in patients with positive AA. Sixty-six patients (48 female, 18 male) with positive lupus anticoagulant (LA) and/or positive anticardiolipin antibody (aCL) underwent radionuclide (RN) venography with 370 MBq of {sup 99m}Tc-MAA. Pulmonary perfusion scintigraphy was performed in 58 patients. Fifteen patients had positive LA and positive aCL (LA+/aCL+), 33 patients had positive LA only (LA+/aCL-) and 18 patients had positive aCL only (LA-/aCL+). Forty-three patients were diagnosed with primary antiphospholipid syndrome (APS) and 19 were diagnosed with APS associated with SLE. DVT was detected in 21 of 66 patients (32%). Patients with LA+/aCL+ showed higher prevalence of DVT (53%) as compared to LA+/aCL- (27%) and LA-/aCL+ (22%). PE was found in 13 of 58 patients (22%). The prevalence of PE was higher in patients with positive aCL (33% in LA+/aCL+; 36% in LA-/aCL+) than in patients with negative aCL (10%). Because of the high prevalence of DVT and PE in patients with AA, RN scintigraphy must be recommended in screening for these clinical troubles. These results indicate that the prevalence of DVT and PE may vary in subgroups of AA. (author)

  10. Dilazep and dipyridamole inhibit tissue factor expression on monocytes induced by IgG from patients with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    Hong ZHON

    2004-01-01

    AIM: To investigate whether antiplatelet agents, dilazep and dipyridamole, inhibit tissue factor (TF) expression on monocytes induced by IgG from patients with antiphospholipid syndrome (APS). METHODS: Freshly isolated peripheral blood monocytes were allowed to adhere on plastic and then cultured in media containing patient or control antibodies and/or other agonists with or without dilazep or dipyridamole. The TF activity on monocytes was investigated by measuring factor VIIa-dependent generation of factor Xa, using a chromogenic substrate and the TF mRNA expression was examined by real-time PCR (TaqMan PCR). RESULTS: The TF activity on monocytes induced by APS IgG (250 mg/L) was inhibited by dilazep (0.15-150 μmol/L) and dipyridamole (0.2-200 μrmol/L) in a dose-dependent fashion. But, the TF mRNA expression induced by APS IgG was not inhibited. Theophylline (500 μmol/L), an adenosine receptor antagonist, could counteract the inhibitory effect of dilazep and dipyridamole on TF activity. CONCLUSION: Antiplatelet agents, dilazep and dipyridamole, block APS IgG-induced monocytes TF expression at a post-transcriptional level, partly by adenosine receptor pathway. Pharmacological agents that block monocytes TF activity, such as dilazep and dipyridamole, are a novel therapeutic approach in APS.

  11. Cardiac thrombus with risk of fulminant pulmonary embolism in paediatric antiphospholipid syndrome.

    Science.gov (United States)

    Albinski, M; Hufnagel, M; Schelling, J; Fleck, T; Siepe, M; Zieger, B; Stiller, B

    2016-07-01

    Antiphospholipid Syndrome (APS) describes a systemic disease caused by autoantibodies to membrane components. Involving coagulation pathways, complement factors and immune cells, it results in thrombosis in any blood vessel. Its clinical presentation varies considerably depending upon the organ affected. Paediatric data on APS remain sparse. Most case reports focus on catastrophic APS with multiple small-vessel occlusions and a life-threatening course. Here, we report on a 15-year-old patient with deep vein thrombosis and a right ventricular tumour posing the risk of a fulminant pulmonary embolism. The tumour was surgically removed. Histology revealed it to be a thrombus. The patient fully recovered and is currently treated with long term anticoagulation. PMID:27138626

  12. Rivaroxaban - a safe therapeutic option in patients with antiphospholipid syndrome? Our experience in 23 cases.

    Science.gov (United States)

    Haładyj, Ewa; Olesińska, Marzena

    2016-01-01

    In the therapeutic approach to patients with antiphospholipid syndrome (APS) with thrombotic manifestations, oral vitamin K antagonists (VKA) remain the standard of care. However, the use of VKA is very often associated with inability to achieve a therapeutic dose even in patients maintaining nutritional and therapeutic restrictions. The non-vitamin-K oral anticoagulants (NOAC) have a lot of advantages, but their efficacy and safety in APS have not been proven. We present 23 patients with APS treated with rivaroxaban in our department. Recurrence of thrombosis was observed only in 1 patient. No major or minor bleeding occurred. It proves the efficacy of treatment with rivaroxaban, but our observations require further prospective, randomized studies.

  13. Rivaroxaban – a safe therapeutic option in patients with antiphospholipid syndrome? Our experience in 23 cases

    Science.gov (United States)

    Olesińska, Marzena

    2016-01-01

    In the therapeutic approach to patients with antiphospholipid syndrome (APS) with thrombotic manifestations, oral vitamin K antagonists (VKA) remain the standard of care. However, the use of VKA is very often associated with inability to achieve a therapeutic dose even in patients maintaining nutritional and therapeutic restrictions. The non-vitamin-K oral anticoagulants (NOAC) have a lot of advantages, but their efficacy and safety in APS have not been proven. We present 23 patients with APS treated with rivaroxaban in our department. Recurrence of thrombosis was observed only in 1 patient. No major or minor bleeding occurred. It proves the efficacy of treatment with rivaroxaban, but our observations require further prospective, randomized studies. PMID:27504026

  14. The Flare Up of Catastrophic Antiphospholipid Syndrome: a Report of an Immunosuppressive Withdrawal-Induced Case

    Directory of Open Access Journals (Sweden)

    Sayyed Gholamreza Mortazavimoghaddam

    2011-09-01

    Full Text Available Antiphospholipid syndrome (APS is a systemic disease that causes venous and arterial thrombosis in virtually any organ. Sometimes it is complicated into pulmonary infarction and cavitation, pulmonary hypertension, and catastrophic course with high morbidity and mortality. The present case is a 35-year-old woman with one episode of postpartum deep veins thrombosis (DVT 12 years earlier and the second one after the second labor two years later. In spite of usual therapy for each episode of DVT, the condition had progressed into severe pulmonary hypertension. The diagnosis of primary APL syndrome was confirmed four years ago. She had been on warfarin, low dose of steroid, and azathioprine since the diagnosis of APL syndrome. After one year treatment with steroid and azathiprine the patient showed progressive well being; however, because of hyperglycemia the steroid tapered and discontinued. She had several attacks of paroxismal atrial tachycardia in the last year. On the last time, she presented with severe dyspnea, hemoptesis, and lower limbs edema. Chest radiography and Lung CT scan demonsterated the presence of lung cavitations. Because of high suspicious for fungal pulmonary infection, azathioprine was also discontinued. However, constellation renal failure, hemodynamic instability, and confusion caused the patient to succumb to death. The definite diagnosis of lung cavitations was not obtained

  15. The presentation and evaluation of a case of systemic Lupus erythematosus and anthiphospholipid antibody syndrome with primary clinical manifestation of chorea

    Directory of Open Access Journals (Sweden)

    Asgary S

    1998-06-01

    Full Text Available Manifestation of chorea in patients with systemic lupus erythematosus (SLE and antiphospholipid antibody syndrome (APA synd. is not common. Moreover, primary presentation of the disease with chorea is rare and only few such cases are reported in literature in recent years. We report here the case of a 28 year old woman who was first seen at the age of 10 with clinical manifestations of chorea. Later she developed deep vein thrombosis, thrombocytpenia, stroke, cardiac valve involvement and recurrent abortions. Laboratory investigations confirmed the diagnosis of SLE and the presence of antiphospholipid antibodies. We present this patient as a case of SLE and antiphospholipid antibody syndrome with chorea being her primary clinical presentation

  16. Are the current attempts at standardization of antiphospholipid antibodies still useful? Emerging technologies signal a shift in direction.

    Science.gov (United States)

    Andreoli, Laura; Rizzini, Silvia; Allegri, Flavio; Meroni, Pierluigi; Tincani, Angela

    2008-06-01

    The pathogenic role of antiphospholipid antibodies (aPL) has been widely established over past years in several experimental models and clinical studies. Accordingly, the detection of aPL by immunoassays (anticardiolipin antibodies; anti-beta2 glycoprotein I antibodies) has become a routine practice in the clinical workup of patients with systemic autoimmune diseases. aPL are mostly assayed using commercial ELISA kits, whose performance has not been found to be sufficiently concordant among the different manufacturers. In the past years, collaborative groups have spent considerable effort to reach some form of standardization but this process is still ongoing. Such lack of standardization has recently become even more crucial, as manufacturers have had to face an increasing demand for fully automated tests for aPL, like those test systems that have been developed for other autoantibodies (e.g., antinuclear antibodies, anti-ENA antibodies). We therefore report our recent experience with two newly developed automated methods for anticardiolipin antibodies testing. In particular, we discuss the results obtained using routine samples, as we believe that these better reflect the "real-life" situation in which those automated methods will operate. We also mention other emerging technologies in the field of aPL detection.

  17. A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy

    Science.gov (United States)

    Koyama, Yasushi; Suzuki, Yasuo

    2016-01-01

    A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy. PMID:27672472

  18. Apheresis in high risk antiphospholipid syndrome pregnancy and autoimmune congenital heart block.

    Science.gov (United States)

    Ruffatti, Amelia; Favaro, Maria; Brucato, Antonio; Ramoni, Veronique; Facchinetti, Myriam; Tonello, Marta; Del Ross, Teresa; Calligaro, Antonia; Hoxha, Ariela; Grava, Chiara; De Silvestro, Giustina

    2015-12-01

    In the first part a prospective cohort study was reported to evaluate the efficacy and safety of a treatment protocol including plasma exchange (PE) or PE plus intravenous immunoglobulins (IVIG) or immunoadsorption (IA) plus IVIG administered in addition to conventional therapy to 22 pregnant women with high-risk APS. The results indicate that PE or IA treatments administered along with IVIG and conventional antithrombotic therapy could be a valuable and safe therapeutic option in pregnant APS women with triple antiphospholipid antibody positivity along with a history of thrombosis and/or one or more severe pregnancy complications. In the second part the efficacy and safety of PE combined with IVIG and steroids were evaluated for the treatment of 10 patients with autoimmune congenital heart block (CHB) by comparing maternal features, pregnancy outcome and side effects with those of 24 CHB patients treated with steroids only. The patients treated with the combined therapy showed a statistically significant regression of 2nd degree blocks, an increase in heart rate at birth and a significantly lower prevalence of pacing in the first year of life. Moreover, no side effects were observed except for a few steroid-related events. If these results are confirmed by large-scale studies, the apheretic procedures could lead to improved outcomes in the treatment of these devastating diseases. PMID:26626966

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

    Directory of Open Access Journals (Sweden)

    Karoline Mayer-Pickel

    2015-01-01

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

  20. Efficacy of plasma exchange and immunoadsorption in systemic lupus erythematosus and antiphospholipid syndrome: A systematic review.

    Science.gov (United States)

    Kronbichler, Andreas; Brezina, Biljana; Quintana, Luis F; Jayne, David R W

    2016-01-01

    Extracorporeal treatments have been used since the 1970s in the management of systemic lupus erythematosus (SLE). A randomised controlled trial comparing the efficacy of standard of care (SOC) combined with plasma exchange against SOC alone in patients with lupus nephritis revealed no difference in terms of renal outcome. Subsequently, initial expectations have been dampened and further experience with plasma exchange is mainly limited to observational studies and single case reports. Beneficial effects have been reported in patients with refractory disease course or in pregnancy with prior complications due to SLE and antiphospholipid syndrome. A more specific form of extracorporeal treatment, immunoadsorption (IAS), has emerged as a valuable option in the treatment of SLE. In line with the plasma exchange experience, IAS seems to have beneficial effects in patients with refractory disease, contraindications to standard immunosuppression or during pregnancy. The mechanism IAS relates to autoantibody removal but for plasma exchange removal of activated complement components, coagulation factors, cytokines and microparticles may also be relevant. Both treatment forms have good safety profiles although reactions to blood product replacement in plasma exchange and procedure related complications such as bleeding or catheter-related infections have occurred. There is a need to more clearly define the clinical utility of plasma exchange and IAS in refractory lupus and APS subgroups. PMID:26318215

  1. ASSOCIATION OF ANTI-PHOSPHATIDYLCHOLINE ANTIBODIES WITH ACUTE MYOCARDIAL INFARCTION: A COMPARATIVE STUDY

    OpenAIRE

    Abdolreza Sotoodeh Jahromi; Mohammad Shojaei; Mohammad Reza Farjam; Abdolhossien Madani

    2013-01-01

    Many factors play a role in Acute Myocardial Infarction (AMI). One those anti-Phospholipid (aPL) antibodies, that may act in the induction of immunological response leading to the development of AMI. Anti-Phosphatidylcholines (PC) antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of anti-PC antibody in AMI might shed light on etiologic mechanisms in the pathogenesis of acute coronary syndromes. T...

  2. Anti-Cardiolipin Antibody in Acute Myocardial Infarction

    OpenAIRE

    Abdolreza S. Jahromi; Mohammad Shojaie; Samira Dana; Abdoulhossain Madani

    2010-01-01

    Problem statement: Myocardial infarction is the combined result of environmental and personal factors. Data concerning the relation between anti-Phospholipid (aPL) antibodies and myocardial infarction in subjects without evidence of overt autoimmune disease are conflicting. Anticardiolipin antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of Anticardiolipin antibody in Acute Myocardial Infarction...

  3. ANTI-PHOSPHATIDYLSERINE ANTIBODIES IN ACUTE MYOCARDIAL INFARCTION

    OpenAIRE

    Abdolreza Sotoodeh Jahromi; Mohammad Shojaei; Mohammad Reza Farjam; Abdolhossien Madani

    2013-01-01

    Acute Myocardial Infarction (AMI) is the combined result of environmental factors and personal predispositions. Many factors play a role in AMI including anti-Phospholipid (aPL) antibodies, that may act in the induction of immunological response leading to the development of AMI. Anti-Phosphatidylserine (PS) antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of anti-PS antibody in AMI might shed l...

  4. Risk of thrombosis in patients with primary immune thrombocytopenia and antiphospholipid antibodies: A systematic review and meta-analysis.

    Science.gov (United States)

    Moulis, Guillaume; Audemard-Verger, Alexandra; Arnaud, Laurent; Luxembourger, Cécile; Montastruc, François; Gaman, Amelia Maria; Svenungsson, Elisabet; Ruggeri, Marco; Mahévas, Matthieu; Gerfaud-Valentin, Mathieu; Brainsky, Andres; Michel, Marc; Godeau, Bertrand; Lapeyre-Mestre, Maryse; Sailler, Laurent

    2016-03-01

    Antiphospholipid antibodies (aPL) are common in ITP, but their role for the occurrence of ITP-related thrombosis is controversial. We performed a systematic review and a meta-analysis to investigate the risk of thrombosis associated with lupus anticoagulant (LA), anticardiolipin (aCL) and anti-β2GP-I antibodies in primary ITP. The literature search was run on Medline, Cochrane and ISI Web of Science from January 1st 1980 to December 31st 2014. Unpublished studies were searched in meeting abstracts. The main analysis assessed the risk of all thromboses (arterial or venous) associated with the presence of LA, aCL or anti-β2GP-I antibodies. Random-effect models were used to calculate odds ratios (OR) and their 95% confidence intervals (CI). Searches in electronic databases retrieved 776 citations. Twelve additional studies from unpublished literature were added. Eventually, 10 cohort studies totalizing 1574 patients were included in the analysis. The pooled OR for the risk of all thromboses associated with LA was 6.11, 95% CI [3.40-10.99]; it was 2.14, 95% CI [1.11-4.12] with aCL. The ORs were similar when stratifying on the type of thrombosis (arterial vs. venous). Only two studies assessed the risk of thrombosis associated with anti-β2GP-I antibody positivity; consequently, no pooled OR was computed for these antibodies. This meta-analysis highly suggests that LA positivity, and to a less extent aCL antibodies, are associated with an enhanced risk of thrombosis in primary ITP patients. Further prospective studies are needed to identify the factors associated with the risk of thrombosis among LA patients before assessing prevention strategies.

  5. Thromboprophylaxis in carriers of antiphospholipid antibodies (APL) without previous thrombosis: "Pros" and "Cons".

    Science.gov (United States)

    Ceccarelli, Fulvia; Chighizola, Cecilia; Finazzi, Guido; Meroni, Pier Luigi; Valesini, Guido

    2012-06-01

    The presence of anti-phospholipid (aPL) is necessary but not sufficient to induce a thrombotic event. The "second hit" hypothesis suggested that an additional trigger may be needed to develop a vascular event in aPL carriers. In this article, pro and con of primary thromboprophylaxis in aPL carriers is deeply discussed, concluding that univocal data are not available, due to conflicting results of available clinical trials. However, in clinical practice the primary thromboprophylaxis is not indicated in all unselected asymptomatic aPL carriers, and the best strategy begin with the assessment of the peculiar risk profile of the subject. Thus, it is mandatory to eliminate modifiable prothrombotic risk factors (i.e. smoking, oral contraceptive), to treat the irreversible risk factors (i.e. hypertension, diabetes) and to introduce an aggressive prophylaxis with subcutaneous LMWH in high-risk situations (i.e. surgical procedures with prolonged immobilization). A different evaluation should be addressed to aPL carriers with a concomitant autoimmune disease that are considered as an additional pro-thrombotic risk factor. Similarly, concomitant positivity for more than one anti-phospholipid test confer a stronger risk of developing the thrombotic manifestations. Specific trials with larger cohorts of patients are needed to better clarify this issue.

  6. Hyperhomocysteinemia - an additional risk factor of thrombosis in systemic lupus erythematosus and antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    I. E. Shirokova

    2003-01-01

    Full Text Available Objective. To assess homocystein (HC level in systemic lupus erythematosus (SLE with antiphospholipid syndrome (APS and its relation to thrombosis development and blood lipide spectrum disturbances. Material and methods. 32 pts (12 male and 20 female with mean age 36 12 years and mean disease duration 13 11 years were included. 8 pts had SLE without APS, 13 - SLE with APS and 11 - primary APS (PAPS. All pts were divided into 2 groups depending on blood HC level. 26 pts with HC level more than 12 mcg/d! were included In group 1 and 6 pts with HC level less than 12 mcg/dl - in group 2. HC level was measured with high efficacious liquid chromatography (HELC. Lipid-protein blood spectrum was assessed in all pts. Results. Elevated HC level was revealed in 26 from 32 pts: in 16 with SLE (including 12 pts with APS and in 10 with PAPS. HC concentration did not depend on APS presence, but frequence of hyperhomocysteinemia (HHC significantly associated with APS and thrombotic complications. 20 from 26 (76,9% pts with HHC had thrombosis history. Only I from 6 (16,7% pts with normal HC level had thrombosis history (exact Fisher test p=0,02. HC level did not depend on age and sex. Changes of blood lipid-protein indices were revealed in most pts. Lipid spectrum disturbances were confined largely to cholesterol elevation due to increase of atherogenic lipoproteins cholesterol. Only 22% of pts showed decrease of antiatherogenic lipoproteins concentration. Bblood lipid-protein spectrum indices did not depend on HC level. Conclusion. HHC is present in 84,6% of pts with APS (primary and secondary. In pts with APS HHC is more frequent than in pts without APS. HHC is associated with thrombotic complications. HHC and lipid-protein spectrum disturbances are independent risk factors of thrombotic complications in pts with SLE and APS.

  7. Survival in primary antiphospholipid syndrome. A single-centre cohort study.

    Science.gov (United States)

    Ames, Paul R J; Merashli, Mira; Chis Ster, Irina; D'Andrea, Giovanna; Iannaccone, Luigi; Marottoli, Vincenzo; Margaglione, Maurizio; Brancaccio, Vincenzo

    2016-06-01

    The vascular mortality of antiphospholipid syndrome (APS) ranges from 1.4 % to 5.5 %, but its predictors are poorly known. It was the study objective to evaluate the impact of baseline lupus anticoagulant assays, IgG anticardiolipin (aCL), plasma fibrinogen (FNG) and von Willebrand factor (VWF), platelets (PLT) and of genetic polymorphisms of methylenetetrahydrofolate reductase C677T, of prothrombin G20210A and of paraoxonase-1 Q192R on survival in primary APS (PAPS). Cohort study on 77 thrombotic PAPS and 33 asymptomatic carriers of aPL (PCaPL) seen from 1989 to 2015 and persistently positive for aPL as per annual review. At baseline all participants were tested twice for the ratios of kaolin clotting time (KCTr), activated partial thromboplastin time (aPTTr), dilute Russell viper venom time (DRVVTr), IgG aCL, FNG, VWF and once for PLT. All thrombotic PAPS were on warfarin with regular INR monitoring. During follow-up 11 PAPS deceased (D-PAPS) of recurrent thrombosis mostly arterial, despite adequate anticoagulation yielding an overall vascular mortality of 10 %. D-PAPS had the strongest baseline aPTTr and DRVVTr and the highest mean baseline IgG aCL, FNG, VWF and PLT. Cox proportional hazards model identified baseline DRVVTr and FNG as main predictors of mortality with adjusted hazard ratios of 5.75 (95 % confidence interval [CI]: 1.5, 22.4) and of 1.03 (95 %CI: 1.01, 1.04), respectively. In conclusion, plasma DRVVTr and FNG are strongly associated with the risk of vascular death in PAPS; while FNG lowering agents exist further research should be directed at therapeutic strategies able to dampen aPL production. PMID:26864875

  8. Anti-β2 Glycoprotein-I Antibody in Acute Myocardial Infarction

    OpenAIRE

    Mohammad Shojaei; Abdolreza S. Jahromi; Hamideh Ebadat; Seyed H. Moosavy; Bita Seddigh; Abdolhossien Madani

    2011-01-01

    Problem statement: Ischemic cardiac manifestations have been reported in a various percentage of patients with anti-phospholipid antibodies. Data concerning the relation between anti- Phospholipid (aPL) antibodies and myocardial infarction in subjects without evidence of overt autoimmune disease are conflicting. Anti-beta2 glycoprotein-I (anti-beta2-GPI) antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. Th...

  9. THE PRESENCE OF ANTI-PHOSPHATIDYLETHANOLAMINE ANTIBODIES IN ACUTE MYOCARDIAL INFARCTION

    OpenAIRE

    Abdolreza Sotoodeh Jahromi; Mohammad Shojaei; Mohammad Reza Farjam; Abdolhossien Madani

    2013-01-01

    Acute Myocardial Infarction (AMI) is a clinical manifestation of coronary atherothrombosis and is the important causes of death. Many factors play a role in AMI. Anti-Phospholipid (aPL) antibodies may act in the induction of immunological response leading to the development of AMI. Anti-Phosphatidylethanolamine (aPEA) antibody has been detected in various autoimmune diseases and anti-phospholipid antibody syndrome. The study of aPEA antibody in AMI might shed light on etiologic mechanisms in ...

  10. Antiphospholipid Antibodies are Associated with Low Levels of Complement C3 and C4 in Patients with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Garabet, L; Gilboe, I-M; Mowinckel, M-C; Jacobsen, A F; Mollnes, T E; Sandset, P M; Jacobsen, E-M

    2016-08-01

    Complement activation and low complement levels are common in systemic lupus erythematosus (SLE). Antiphospholipid antibodies (aPL) are found in about 30-40% of patients with SLE. This study aimed to investigate the association between aPL and complement levels in patients with SLE. Serum samples were collected from 269 patients with SLE enrolled in the Norwegian Systemic Connective Tissue and Vasculitis Registry (NOSVAR) during 2003-2009, and from 353 controls. All samples were analysed for anti-β2 glycoprotein 1 (anti-β2GP1) and anticardiolipin antibodies (aCL), C-reactive protein (CRP) and complement components C3 and C4. Median CRP level was significantly higher in cases than in controls (2.06 versus 0.90 mg/l; P C3 levels were similar in cases (1.03 g/l) and controls (1.00 g/l), whereas median C4 levels were 0.16 g/l in cases versus. 0.19 in controls (P C3 levels (0.92 versus. 1.07 g/l; P = 0.001) and C4 levels (0.11 versus 0.16 g/l; P C3 and C4 values in aPL-positive SLE patients may reflect a higher consumption of C3 and C4 due to more pronounced complement activation in aPL-positive SLE patients compared to SLE patients without aPL. PMID:27135178

  11. Anti-phospholipid antibodies in patients with Plasmodium falciparum malaria

    DEFF Research Database (Denmark)

    Jakobsen, P H; Morris-Jones, S D; Hviid, L;

    1993-01-01

    of phospholipids (PL) during an acute Plasmodium falciparum infection. The anti-PL antibody titre returned to preinfection levels in most of the donors 30 days after the disease episode. IgG titres against PI, PC and CL were low. In Gambian children with malaria, IgM antibody titres against PI and PC...

  12. Thrombolytic Therapy for Cerebral Vein Thrombosis in Antiphospholipid Syndrome Secondary to Systemic Lupus Erythematosus

    OpenAIRE

    Mehrzad Hajialilo; Hamid Noshad; Reza Mohammadian; Ali Reza Khabbazi; Mohammad Hossein Daghighi; Parviz Saleh

    2012-01-01

    A 20-year-old woman was admitted to a Gynecology Hospital in her 6th month of pregnancy for high blood pressure and tonic-clonic seizure. Primary diagnosis was eclampsia, and for that reason she underwent cesarean section. She also had headache on frontal and parietal areas without nausea or vomiting. There was not a focal neurological sign. Rheumatology consultation was requested. Sys-temic lupus erythematosus and secondary antiphospholipid (APS) was confirmed. The patient had headache that ...

  13. Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Bures Vladimir

    2005-04-01

    Full Text Available Abstract We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon. Conclusion Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.

  14. Apresentação pediátrica da síndrome antifosfolípide Paediatric antiphospholipid syndrome presentation

    Directory of Open Access Journals (Sweden)

    Juliana de Oliveira Sato

    2008-12-01

    Full Text Available OBJETIVO: Descrever as características clínicas, laboratoriais e de desfecho de uma série de casos com diagnóstico definido de síndrome antifosfolípide (SAF pediátrica. MÉTODOS: Estudo observacional-retrospectivo de referência pediátrica terciária, que identificou os casos por meio de evento vascular, trombose venosa ou oclusão arterial, determinação de anticorpos anticardiolipina (IgG e IgM e teste do anticoagulante lúpico. RESULTADOS: Foram identificados cinco casos atendidos nos últimos cinco anos, sendo dois meninos e três meninas. A trombose venosa ocorreu em seios venosos cerebrais (2, fibular (2, poplítea (1, femoral (1, intestinal (1, renal (1, acompanhados por oclusão arterial intestinal (1, de artéria renal (1 e artéria digital (1, esta resultando gangrena periférica como evento recorrente durante anticoagulação com warfarina. Um abortamento espontâneo ocorreu em uma adolescente em vigência de púrpura trombocitopênica, evoluindo com anemia hemolítica (síndrome de Evans e desfecho fatal por hemorragia. A investigação laboratorial em todos os casos resultou, pelo menos, uma determinação positiva de anticardiolipina IgG e/ou IgM, sendo considerados como SAF primária. Três dos casos estão em seguimento com anticoagulação oral. CONLUSÃO: A trombose venosa cerebral e de extremidades foram os eventos mais freqüentes. A presente série alerta para a investigação e o diagnóstico precoces, com abordagem multidisciplinar para o tratamento.OBJECTIVE: To describe clinical and laboratorial features as well as outcome in a paediatric series with defined diagnosis of antiphospholipid syndrome. METHODS: A descriptive-retrospective report from a pediatric tertiary referral, with case ascertainment by vascular events identification, either venous thrombosis or arterial occlusion, anti-cardiolipin antibodies (IgG and IgM titres and lupus anticoagulant tests. RESULTS: Five cases, being two boys and three girls

  15. Anti-phospholipid antibodies restore mesenteric ischemia/reperfusion-induced injury in complement receptor 2/complement receptor 1-deficient mice.

    Science.gov (United States)

    Fleming, Sherry D; Egan, Ryan P; Chai, Chunyan; Girardi, Guillermina; Holers, V Michael; Salmon, Jane; Monestier, Marc; Tsokos, George C

    2004-12-01

    Complement receptor 2-deficient (Cr2(-/-)) mice are resistant to mesenteric ischemia/reperfusion (I/R) injury because they lack a component of the natural Ab repertoire. Neither the nature of the Abs that are involved in I/R injury nor the composition of the target Ag, to which recognition is lacking in Cr2(-/-) mice, is known. Because anti-phospholipid Abs have been shown to mediate fetal growth retardation and loss when injected into pregnant mice, we performed experiments to determine whether anti-phospholipid Abs can also reconstitute I/R injury and, therefore, represent members of the injury-inducing repertoire that is missing in Cr2(-/-) mice. We demonstrate that both murine and human monoclonal and polyclonal Abs against negatively charged phospholipids can reconstitute mesenteric I/R-induced intestinal and lung tissue damage in Cr2(-/-) mice. In addition, Abs against beta2 glycoprotein I restore local and remote tissue damage in the Cr2(-/-) mice. Unlike Cr2(-/-) mice, reconstitution of I/R tissue damage in the injury-resistant Rag-1(-/-) mouse required the infusion of both anti-beta2-glycoprotein I and anti-phospholipid Ab. We conclude that anti-phospholipid Abs can bind to tissues subjected to I/R insult and mediate tissue damage. PMID:15557203

  16. Anticorpos antifosfolípides em mulheres com antecedentes de perdas gestacionais: estudo caso-controle Antiphospholipid antibodies in women with recurrent pregnancy loss: a case-control study

    Directory of Open Access Journals (Sweden)

    Olívia Lúcia Nunes Costa

    2005-06-01

    250 pregnant and non-pregnant women, seen at a public maternity between March of 2003 and June of 2004. The women were divided in two groups according to their obstetric past: 100 women with previous pregnancy loss, defined as the obstetric criteria of the antiphospholipid syndrome (cases and 150 healthy women with record of two or more successful pregnancies (controls. Lupus anticoagulant was determined by aTTP and dRVVT tests. IgG and IgM anticardiolipin antibodies (aCL were determined by a specific immunoassay (ELISA and results were expressed as GPL or MPL units. RESULTS: Lupus anticoagulant was positive in 5% of the cases and 2% of the controls (p = 0.27. The IgG aCL antibodies were present in 18% of the cases and 8,7% of the controls (p = 0.028 ;OR = 2.3; IC 95% = 1-53 and IgM aCL in 5% of the cases and 1% of the controls (p = 0,21. CONCLUSIONS: Antiphospholipid antibodies were more prevalent in women with pregnancy loss (28% than in general obstetric population (17%. Women with IgG aCL antibodies have higher chances of pregnancy loss.

  17. Increased Performances of the Biological Diagnosis of the Antiphospholipid Syndrome by the Use of a Multiplex Assay

    Directory of Open Access Journals (Sweden)

    M. Sénant

    2015-01-01

    Full Text Available Antiphospholipid syndrome (APS is characterized by development of venous and/or arterial thrombosis and pregnancy morbidity. Biological criteria are the persistent presence of lupus anticoagulant (LA and/or anti-cardiolipin (aCL and/or anti-B2GP1 autoantibodies’ positivity. The assays’ performances are of crucial importance. We evaluated a multiplex assay allowing simultaneous detection of IgG anti-cardiolipin, anti-B2GP1, and anti-factor II. 300 samples were tested. Patients were categorized according to clinical scores of APS from 0 to 3 based on presence or not of arterial or venous thrombosis, fetal loss, and autoimmunity. We used a multiplex assay for APS for simultaneous detection of aCL, anti-B2GP1, and factor II and compared its performances to ELISA assays. Presence of LA was also assessed. We performed a correlation study of the tested assays and compared their clinical efficacy by ROC curve analysis. We obtained significantly higher performances with the multiplex assay than ELISA with higher area under the curve (AUC. The disease rate increased with the number of positive markers from 9% for 1 marker to 100% for 4 markers positive for patients with high risk scores. The multiplex APS assay exhibited higher performances particularly in case of primary APS and is useful for rapid diagnosis of APS.

  18. Thrombolytic Therapy for Cerebral Vein Thrombosis in Antiphospholipid Syndrome Secondary to Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Mehrzad Hajialilo

    2012-03-01

    Full Text Available A 20-year-old woman was admitted to a Gynecology Hospital in her 6th month of pregnancy for high blood pressure and tonic-clonic seizure. Primary diagnosis was eclampsia, and for that reason she underwent cesarean section. She also had headache on frontal and parietal areas without nausea or vomiting. There was not a focal neurological sign. Rheumatology consultation was requested. Sys-temic lupus erythematosus and secondary antiphospholipid (APS was confirmed. The patient had headache that continued several days after cesarean section, therefore, brain magnetic resonance im-aging (MRI and magnetic resonance venography (MRV were performed, and cerebral vein thrombosis was documented. Distal segment of right lateral sinus and sigmoid sinus were not ap-peared in brain MRV. Abnormal hypersignal intensity of right lateral sinus/coronal T2 was detected. Thrombolytic therapy with 20 mg tissue plasminogen activator on right sigmoid and transverse sinus was performed by an interventional neurologist. After this procedure, the patient's headache healed and she was discharged in a good condition.

  19. Mal de Hansen, anticorpos antifosfolípides e obstrução das artérias fibulares Leprosy, antiphospholipid antibodies and bilateral fibular arteries obstruction

    Directory of Open Access Journals (Sweden)

    Liz Wallin

    2009-04-01

    Full Text Available Pacientes com Mal de Hansen (MH podem se apresentar com quadro clínico e laboratorial sugestivo de doenças reumáticas, o que exige um exercício cuidadoso de diagnósticos diferenciais. Descreve-se aqui o caso de uma jovem com MH que se apresentou com lesões cutâneas sugestivas de vasculite, obstrução de vasos fibulares, FAN e anticorpos antifosfolípides positivos sem muitos estigmas da doença cutânea, ilustrando essa dificuldade.Patients with Hansen's disease (HD may present themselves with clinical and laboratorial features that resemble rheumatic disorders. This requires a careful exercise of differential diagnosis. We describe here a case of a young woman with HD that presented with vasculitic like lesions, fibular vessels obstruction, ANA and antiphospholipid antibodies without typical signs of cutaneous disease, illustrating this clinical difficulty.

  20. Role of anti-thrombotic therapy for recurrent pregnancy loss due to anti-phospholipid syndrome

    International Nuclear Information System (INIS)

    Background: Recurrent pregnancy loss is a major health problem effecting 1 to 2% of women of reproductive age. Its causes range from chromosomal abnormalities to endocrinological factors and thrombophilia related factors. Treating thrombophilia s especially anti phospholipid syndrome with low dose aspirin and low molecular weight heparin improves foetal outcome. This study will add local data to already existing knowledge. Method: Sixty selected patients from gynaecology OPD of Aero Hospital with clinical and/or serological findings of anti phospholipid syndrome from February 2009 to January 2011 were given aspirin 75 mg once daily and enoxaparine 40 mg subcutaneously once daily from 6 - 8 weeks to 35 and 37 weeks respectively. Results : Ninety-three percent of patients achieved live birth. Out of these 75% patients delivered at term and 18% had preterm delivered. Four (7%) had early pregnancy loss and only one had early neonatal death due to extreme prematurity. None of patients experienced any major hemorrhagic complications . Conclusion: Use of low dose aspirin and low molecular weight heparin is safe in pregnancy and improve foetal outcome in patients with recurrent pregnancy loss due to anti phospholipids syndrome. (author)

  1. Statement of Retraction: "Mohamad Goldust, Mahnaz Talebi, Jafar Majidi, Mohammad Amin Rezazadeh Saatlou, and Elham Rezaee. Evaluation of antiphospholipid antibodies in youths suffering from cerebral ischemia.".

    Science.gov (United States)

    Lyons, Kelly; Pahwa, Rajesh

    2013-08-01

    The Editors and Publisher would like to inform the readers the following article has been retracted from publication in the International Journal of Neuroscience: Mohamad Goldust, Mahnaz Talebi, Jafar Majidi, Mohammad Amin Rezazadeh Saatlou, Elham Rezaee. Evaluation of antiphospholipid antibodies in youths suffering from cerebral ischemia. Int J Neurosci. 2013 Mar;123(3):1247-57. Dr. Mahnaz Talebi contacted the Editors of the International Journal of Neuroscience to inform them that this article was a graduation thesis for his student Dr. Mohamadali Arami at Tabriz University of Medical Sciences, Tabriz, Iran, and previously published in print and in Persian by the Iranian Journal of Neurology: Mahnaz Talebi, Jafar Majidi, Mohamadali Arami, Seyed Ali Saderddini. Evaluation of antiphospholipid antibodies in youths suffering from cerebral ischemia. Iran J Neuro. 2005 Spring;15(3):26-34. Moreover, Dr. Talebi said he was listed as an author of the article published in the International Journal of Neuroscience without his knowledge or consent. When queried, Dr. Mohamad Goldust, the corresponding author of the article published in the International Journal of Neuroscience, admitted that he listed Dr. Talebi as a coauthor improperly and asked for the manuscript to be retracted; he did not respond to our questions regarding whether this manuscript was previously published in the Iranian Journal of Neurology or whether this manuscript was the original work of the authors listed in the published the International Journal of Neuroscience article. The coauthors listed on the article published in the International Journal of Neuroscience were contacted several times but did not respond to our queries. Since the article in the Iranian Journal of Neurology was published in Persian, we contacted Dr. Shahriar Nafissi, Editor in Chief of the Iranian Journal of Neurology, who confirmed that the two articles in question were the same. Our policy in this respect is clear: the

  2. IgA Antibodies in Rett Syndrome

    Science.gov (United States)

    Reichelt, K. L.; Skjeldal, O.

    2006-01-01

    The level of IgA antibodies to gluten and gliadin proteins found in grains and to casein found in milk, as well as the level of IgG to gluten and gliadin, have been examined in 23 girls with Rett syndrome and 53 controls. Highly statistically significant increases were found for the Rett population compared to the controls. The reason for this…

  3. Characterization of beta2-glycoprotein I-dependent and -independent "antiphospholipid" antibodies from lupus-prone NZW/BXSB F1 hybrid male mice.

    Science.gov (United States)

    Thiagarajan, P; Le, A; Shapiro, S S

    1997-10-01

    Male (NZW x BXSB)F1 (W/BF1) mice develop a systemic lupus-like syndrome characterized by thrombocytopenia, coronary vascular disease, nephritis, and anticardiolipin antibodies. Three stable hybridoma cell lines secreting monoclonal anticardiolipin antibodies were developed from these mice by fusing their splenic lymphocytes with nonsecreting myeloma cell line, NS-1. Monoclonal antibody A1.17 reacted with cardiolipin in a beta2-Glycoprotein I-dependent manner. The epitope for this antibody consisted of beta2-glycoprotein I bound to cardiolipin or immobilized on plastic plates. Other anionic phospholipid-binding proteins, such as prothrombin or annexin V, had no significant effect in the reactivity of these antibodies. The specificity is similar to the autoimmune anticardiolipin antibodies described in patients with systemic lupus erythematosus and other infectious diseases. In contrast, monoclonal antibodies A1.72 and A1.84 reacted with cardiolipin in the absence of beta2-glycoprotein I. Beta2-glycoprotein I, either in the fluid phase or bound to cardiolipin, inhibited the binding of these antibodies. The specificity of the latter two antibodies was similar to that described in patients with syphilis and allied disorders. Both types of antibodies had lupus anticoagulant properties. Thus lupus-prone male (NZW x BXSB)F1 (W/BF1) mice develop both beta2-glycoprotein I-dependent and beta2-glycoprotein I-independent anticardiolipin antibodies.

  4. 抗磷脂综合征165例临床特征与分型%Classification and clinical analysis of 165 patients with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    徐娜; 张尧; 张文; 赵岩; 曾小峰

    2009-01-01

    Objective Based on 2006 revised classification criteria of definite antiphospholipid syndrome(APS)and definition of new APS subsets,we investigated clinical features of APS patients,correlation between thrombotic events and related antibodies Was assessed as well.Methods 165 patients with APS were enrolled and analysed retrospectively.Results 124 female and 41 male patients were included.116 cases(70.3%),34 cases(20.6%),10 cases(6.1%),and 5 cases(3.0%)were classified as definite APS,probable APS,seronegative APS,and microangiopathic APS(MAPS) respectively.Among 124 patients accompanied with other diseases,113(91.1%)were autoimmune diseases,mostly systemic lupus erythematosus(79.6%).121(73.3%)patients had episodes of thrombosis,with majority of deep vein thrombosis.The patients with positive test of lupus anticoagulant (LA)only were more likely to have episodes of thrombosis than with positive anticardiolipid antibody(ACL) only(86.0%versus 55.7%;P<0.05).Among 61 patients with prolonged APTT,the presence of LA was detected in 50(82.0%)patients,and ACL in 34(55.7%)patients.Conclusion APS patients could be classified into several subgroups according to their clinical features.Venous thromboses are more common than arterial thromboses,and positive of LA correlates thrombosis more than ACL%目的 分析抗磷脂综合征(APS)的临床特征与分型.方法 根据2006年更新的APS分类诊断标准及新的临床亚型的定义,回顾性分析北京协和医院住院的165例APS患者的临床分型,总结患者的临床表现及治疗,并分析抗磷脂抗体与血栓的相关性.结果 165例患者中,男:女为1:3.不同临床亚型的分类包括确诊APS 116例(70.3%),可能APS 34例(20.6%),血清阴性APS 10例(6.1%),微血管性APS 5例(3.0%).124例(75.2%)合并其他疾病,其中113例(91.1%)合并自身免疫病,以系统性红斑狼疮常见(79.6%).合并血栓者121例(73.3%),其中静脉血栓68例(56.2%),以下肢深静脉血栓最常见.仅有狼

  5. [Ruptured aneurysm at the anterior wall of the internal carotid artery in a patient with systemic lupus erythematosus and secondary antiphospholipid syndrome].

    Science.gov (United States)

    Chonan, Masashi; Fujimura, Miki; Inoue, Takashi; Tominaga, Teiji

    2011-07-01

    A 60 year-old woman, who had a 45-year history of systemic lupus erythematosus (SLE) and secondary antiphospholipid syndrome, presented with subarachnoid hemorrhage due to a ruptured aneurysm at the anterior wall of the non-branching site of the right internal carotid artery. She underwent radical surgery on the day of onset. In light of the possibility of arterial dissection, we performed extracranial-intracranial bypass prior to careful exploration of the aneurysm. Based on the finding of saccular aneurysm, she ultimately underwent neck clipping of the aneurysm without complication. Postoperative course was uneventful, and she did not suffer from cerebral vasospasm. We recommend early surgical intervention in patients with aneurysmal SAH associated with SLE, while intrinsic pathologies of SLE such as fragile vascular structure and the risk for ischemic complication should be considered.

  6. Hiper-homocisteinemia e síndrome antifosfolípide primária Hyperhomocysteinemia and primary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Jozélio Freire de Carvalho

    2009-08-01

    associations in patients with primary antiphospholipid syndrome (PAPS. PATIENTS AND METHODS: This is a transversal study with 27 patients (88% women with PAPS (Sapporo criteria. Demographic and clinical data, as well as comorbidities, medications, antiphospholipid antibodies, and blood concentrations of homocysteine, measured by high resolution liquid chromatography, were evaluated. RESULTS: Six (22% out of 27 patients had high levels of homocysteine (98.7 ± 8.9 versus 8.0 ± 2.9 ¼M, P = 0.0008. Comparison between the group of patients with hyperhomocysteinemia and the group of patients with normal serum homocysteine levels did not show differences in the demographic data (age, white, weight, height, and body mass index or in the duration of the disease (64 ± 39.6 versus 77.9 ± 61.3 months, P = 0.32. Differences in disease manifestations (arterial, venous, and obstetric events, deep venous thrombosis, pulmonary thromboembolism, thrombocytopenia, acute myocardial infarction, angina, and stroke, comorbidities (hypertension and hyperlipidemia, life style (physical activity, past and present smoking, as well as the use of medications (past and present use of corticosteroids, statins, chloroquine, and acetylsalicylic acid, were not observed between both groups. The prevalence and titers of anticardiolipin antibodies were similar in both groups of patients. CONCLUSION: Hyperhomocysteinemia can be detected in approximately one fourth of the PAPS patients, and it is not associated with distinct clinical and laboratorial characteristics of this disorder.

  7. Frequency of antiphospholipid antibodies in patients with infectious diseases using three different ELISA methods Freqüência de anticorpos antifosfolípides em pacientes com doenças infecciosas usando três diferentes testes de ELISA

    Directory of Open Access Journals (Sweden)

    Mittermayer Barreto Santiago

    2006-02-01

    Full Text Available OBJECTIVE: The standard enzyme-linked immunosorbent assay (ELISA for anticardiolipin (aCL antibodies is the most important test for the diagnosis of antiphospholipid syndrome (APS. However, the test is also positive in some infectious diseases and other non-related syndromes. It has been suggested that the detection of antibodies to a mixture of phospholipids or to beta2-glycoprotein I (beta2-GP I has higher specificity for APS than the standard aCL ELISA. The aim of the present work is to compare the diagnostic specificity of three different antiphospholipid (aPL assays in patients with infectious diseases. METHODS: Antiphospholipid antibodies were searched by three ELISA techniques, namely standard aCL, APhL® ELISA kit and anti-beta2-GP I, in sera of patients with infectious diseases, including syphilis (69, leptospirosis (33 and visceral leishmaniasis (30. RESULTS: The frequency of positivity of IgG aPL in patients with syphilis, leptospirosis and Kala-azar was 13/69 (19%, 9/33 (27% and 2/30 (6%, respectively, using standard ELISA, versus only 1/69 (1.4%, 0/33 (0% and 0/30 (0% positivity by the APhL® ELISA kit. The positivity of the isotype IgM aPL was 10/69 (14%, 4/33 (12% and 1/30 (3%, respectively, by the standard ELISA, and 1/69 (1.4%, 0/33 (0% and 0/30 (0% by the APhL® ELISA kit. The presence of significant levels of IgG anti-beta2GPI was observed in 14/69 cases of syphilis (20%, 6/33 cases of leptospirosis (18% and 16/30 cases of Kala-azar (53%. The APhL® ELISA kit had superior performance showing the highest specificity: 97% (95% CI: 92%-99% for IgG compared to 81% (95% CI: 74%-87% for standard ELISA and 72% (95% CI: 64%-79% for anti-beta2 GPI assay. CONCLUSIONS: The APhL® ELISA kit proved to be significantly more specific than the aCL standard ELISA and the anti-beta2GPI ELISA, and it should be used to help in the diagnosis and confirmation of APS.OBJETIVO: O ensaio de enzyme-linked immunosorbent assay (ELISA para a pesquisa de

  8. Patient-level analysis of five international cohorts further confirms the efficacy of aspirin for the primary prevention of thrombosis in patients with antiphospholipid antibodies.

    Science.gov (United States)

    Arnaud, Laurent; Mathian, Alexis; Devilliers, Hervé; Ruffatti, Amelia; Tektonidou, Maria; Forastiero, Ricardo; Pengo, Vittorio; Lambert, Marc; Lefevre, Guillaume; Martinez-Zamora, Maria Angeles; Balasch, Juan; Wahl, Denis; Amoura, Zahir

    2015-03-01

    We performed an individual patient meta-analysis to determine whether aspirin has a significant protective effect on the risk of first thrombosis among patients with antiphospholipid antibodies (aPL). Five international cohort studies with available individual patient-level data, reporting on primary prophylaxis with continuous treatment with low-dose aspirin in patients with aPL were included. The main outcome was the occurrence of a first thrombotic in patients with aPL treated with low-dose aspirin compared to those not treated with low-dose aspirin. Pooled Hazard Ratios (HRs) and 95%CIs were calculated using frailty models. We pooled data from 497 subjects and 79 first thrombotic events (3469 patient-years of follow-up). After adjustment on cardiovascular risk factors, aPL profiles, and treatment with hydroxychloroquine, the HR for the risk of a first thrombosis of any type in aPL carriers treated with low-dose aspirin versus those not treated with aspirin was 0.43 (95%CI 0.25-0.75). Subgroup analysis showed a protective effect of aspirin against arterial (HR: 0.43 [95%CI: 0.20-0.93]) but not venous (HR: 0.49 [95%CI: 0.22-1.11]) thrombosis. Subgroup analysis according to underlying disease revealed a protective effect of aspirin against arterial thrombosis for systemic lupus erythematosus (SLE) (HR: 0.43 [95%CI: 0.20-0.94]) and asymptomatic aPL carriers (HR: 0.43 [95%CI 0.20-0.93]). We found no independent protective effect of hydroxychloroquine. This individual patient data meta-analysis shows that the risk of first thrombotic event as well of first arterial thrombotic event is significantly decreased among SLE patients and asymptomatic aPL individuals treated by low-dose aspirin.

  9. Successful pregnancy after rituximab in a women with recurrent in vitro fertilisation failures and anti-phospholipid antibody positive.

    LENUS (Irish Health Repository)

    Ng, C T

    2012-02-01

    We report a case of successful pregnancy after rituximab in a patient with a history of in vitro fertilisation (IVF) failures and positive anti-cardiolipin antibody (ACA). Following a course of rituximab, her ACA became negative and she successfully conceived with IVF treatment. This is the first case in literature describing the use of rituximab therapy in this clinical scenario.

  10. Síndrome antifosfolípide e trombocitopenia na infância Síndrome antifosfolípido y trombocitopenia en la infancia: relato de caso Antiphospholipid syndrome and thrombocytopenia in childhood

    Directory of Open Access Journals (Sweden)

    Roberta Bittencourt F. Turini

    2012-09-01

    eight months old with severe thrombocytopenia associated with a positive anticardiolipin antibody. Data were collected by clinical history, physical examination, and laboratorial exams. Diagnosis was confirmed according to criteria established for the antiophospholipid syndrome, associated with the presence of the most common manifestations of the syndrome in children: livedo reticularis and thrombocytopenia. COMMENTS: The antiphospholipid syndrome is an uncommon pediatric disease, and clinical manifestations such as decreased platelet number should be considered.

  11. Anti-aquaporin-4 antibody-positive dorsal midbrain syndrome.

    Science.gov (United States)

    Lee, Juyoun; Jeong, Seong-Hae; Park, Sang Min; Sohn, Eun Hee; Lee, Ae Young; Kim, Jae-Moon; Jo, Hyun-Jin; Lee, Yeon-Hee; Kim, Ji-Soo

    2015-04-01

    Neuromyelitis optica spectrum disorders (NMOSD) can cause various ocular motor disorders in addition to optic neuritis. Ocular motor findings associated with NMOSD include spontaneous vertical and gaze-evoked nystagmus, wall-eyed bilateral internuclear ophthalmoplegia, and trochlear nerve palsy. The association between dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity has not been reported. Here, we report a patient displaying typical dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity.

  12. The catastrophic antiphospholipid syndrome/Asherson's syndrome:an overview and update%恶性抗磷脂综合征的临床研究新进展

    Institute of Scientific and Technical Information of China (English)

    沈思钰(综述); 朱培元; 傅晓东(审校)

    2015-01-01

    Catastrophic antiphospholipid syndrome (CAPS), i.e.Asherson's syndrome, is characterized by diffuse small vessel thrombosis and acute multiple organ involvement which resulting in multiorgan failure over a short period of time. Although less than 1% of patients with the antiphospholipid syndrome ( APS ) develop this complication, unfortunately, the mortality is extreme high.The purpose of this paper is to summarize characteristics of CAPS which including clinical manifestation, pathogenesis, classification criteria, therapy strategy and prognosis.In parallel with CAPS registry, CAPS task force should pay more attention in advanced treatment recommendations revised which maintaining clinical long-term remission and increasing survival rate in the future.%恶性抗磷脂综合征( CAPS)是一种短期内进行性的广泛血栓形成导致多脏器功能衰竭直至死亡的自身免疫病。本文主要根据临床注册研究,探讨其临床表现、发病机制、诊断标准、治疗推荐及预后等。本病发病率低但死亡率极高,未来研究应在CAPS注册的基础上,更关注于治疗方案的不断改良及如何维持长期临床缓解、降低死亡率的研究。

  13. Anti-glutamic acid decarboxylase antibody positive neurological syndromes.

    Science.gov (United States)

    Tohid, Hassaan

    2016-07-01

    A rare kind of antibody, known as anti-glutamic acid decarboxylase (GAD) autoantibody, is found in some patients. The antibody works against the GAD enzyme, which is essential in the formation of gamma aminobutyric acid (GABA), an inhibitory neurotransmitter found in the brain. Patients found with this antibody present with motor and cognitive problems due to low levels or lack of GABA, because in the absence or low levels of GABA patients exhibit motor and cognitive symptoms. The anti-GAD antibody is found in some neurological syndromes, including stiff-person syndrome, paraneoplastic stiff-person syndrome, Miller Fisher syndrome (MFS), limbic encephalopathy, cerebellar ataxia, eye movement disorders, and epilepsy. Previously, excluding MFS, these conditions were calledhyperexcitability disorders. However, collectively, these syndromes should be known as "anti-GAD positive neurological syndromes." An important limitation of this study is that the literature is lacking on the subject, and why patients with the above mentioned neurological problems present with different symptoms has not been studied in detail. Therefore, it is recommended that more research is conducted on this subject to obtain a better and deeper understanding of these anti-GAD antibody induced neurological syndromes. PMID:27356651

  14. A Study of Anti Beta-2 Glycoprotein I and Anti-Prothrombin Antibodies in Patients with Unexplained Recurrent Pregnancy Losses.

    Science.gov (United States)

    Singh, Angad; Nangia, Anita; Sharma, Sunita; Puri, Manju

    2016-06-01

    To compare the levels of IgG and IgM anti beta-2 glycoprotein I antibodies and IgG and IgM anti prothrombin antibodies among women with unexplained recurrent pregnancy losses and women with at least 2 live issues. To compare the prevalence of newer anti beta-2 glycoprotein I & anti prothrombin antibodies with conventional Lupus anticoagulant & anticardiolipin antibodies. 50 women with recurrent pregnancy losses & 50 matched controls were evaluated for the presence of: Lupus anticoagulant-screened by LA sensitive aPTT& DRVV and confirmatory Staclot Assay. ELISA kits were used for detecting IgG & IgM anticardiolipin, anti beta-2 glycoprotein I & anti prothrombin antibodies. 11/50 (22 %) women in study group and none in control group had circulating antiphospholipid antibodies. 2 cases (4 %) had lupus anticoagulant. 1 case (2 %) had anticardiolipin antibody & 6 cases (12 %) were positive for anti beta-2 Glycoprotein I antibody (p value = 0.027). 3 cases (6 %) had anti prothrombin antibody. All were mutually exclusive except for one. Women with recurrent pregnancy losses should be tested for anti beta-2 Glycoprotein I antibodies & anti prothrombin antibodies in addition to conventional lupus anticoagulant and anticardiolipin antibodies. This approach can decrease the incidence of SNAP (seronegative antiphospholipid syndrome) cases while establishing the true prevalence of antiphospholipid syndrome. PMID:27065583

  15. Clinical characteristics of 174 Chinese patients with antiphospholipid syndrome and comparison with European patients%抗磷脂综合征174例临床特点及与欧洲数据的比较分析

    Institute of Scientific and Technical Information of China (English)

    陈晓微; 申艳; 孙传银; 杨程德

    2010-01-01

    Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) in a cohort of Chinese patients. Methods From January 1996 to October 2009, 174 patients with defined APS were retrospectively studied,χ2 test was selected. Results The cohort consisted of 151(86.8%) female and 23 (13.2%) male. Mean age at study was (42±13) years (range: 14-75 years). Thirty-one (17.8%) patients had primary APS, 143 (82.2% ) patients had APS associated with other diseases, including 138 cases of systemic lupus erythematosus (SLE). One hundred and forty-one (81.0%) had thrombosis episodes, among which the most common presenting manifestations were stroke (27.6%), deep venous thrombosis (27.6%) and pulmonary embolism (6.3% ). Stroke was more prevalent in Chinese than European patients. Forty-five patients (31.9%) had thrombotic recurrences and 62 patients (44.0%) had more than two sites of thrombosis. Patients with primary APS had higher rates of rethrombosis than those with secondary APS (P<0.05). Fetal morbidity rate of 126 married women in our study was 50.0%. Seven of APS patients with APS nephropathy were characterized by thrombotic microangiopathy. The presence of anticardiolipin antibodies was detected in 112 patients (64.4% ), anti-beta-2 glycoprotein Ⅰ antibodies in 103 patients (59.1%) and lupus anticoagulant in 50 patients (28.7%). Conclusion APS is characterized by recurrent thrombosis, pregnancy morbidity and positive antiphospholipid antibodies tests. Stroke, deep venous thrombosis and pulmonary embolism are the most common manifestations both in Chinese and European patients. Rethrombosis is more prominent in primary APS. Thrombotic microangiopathy is one of the most common histological changes of APS nephropathy. Multiple tests for aPL are proposed.%目的 分析中国人群抗磷脂综合征(AIDS)的临床特点,以提高对这一疾病的认识.方法 回顾性分析1996-2009年在上海仁济医院诊治的APS患者的临

  16. 抗磷脂综合征发病机制及诊治进展%Pathogenesis and progress of anti phospholipids syndrome

    Institute of Scientific and Technical Information of China (English)

    张晶; 周彬

    2011-01-01

    抗磷脂综合征(antiphospholipid syndrome,APS)主要表现为血栓形成或病态妊娠,实验室检测抗磷脂抗体(antiphospholipid antibody,APL)阳性,如抗心磷脂抗体(anticardiolipin antibody,ACA)、抗β2-糖蛋白1(β2-glycoprotein l,β2-GP1)抗体及狼疮抗凝物(lupus anticoagulant,LAC).β2-GP1及其抗体在APS发病机理中的作用日益受到人们的重视.APS累及全身多个系统,治疗上除肝素、华法林和阿司匹林外,还可试用免疫抑制剂、大剂量丙种球蛋白等,其他与发病机制相关的免疫治疗仍在进一步研究中.%The major features of antiphospholipid syndrome(APS) are thrombosis and morbidity pregnancy with antiphospholipid antibodies positived, which includes anticardiolipin antibody, antiβ2-glycoprotein 1 antibody and lupus anticoagulant.β2-glycoprotein 1 and the antibodies plays an increasingly important role in pathogenesis of APS.Except heparin, warfarin and aspirin, we can choose immunosuppressive agents or high dose immunoglobulin G to treat the APS.Other immunotheropies related with the pathogenesis are still in study.

  17. Síndrome antifosfolípide e gestação: tratamento com heparina e aspirina em doses baixas Antiphospholipid syndrome and pregnancy: treatment with heparin and low-dose aspirin

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    Marcelo de Amorim Aquino

    1999-05-01

    Full Text Available Objetivos: determinar a eficácia e a segurança do tratamento de gestantes com síndrome antifosfolípide com a heparina associada à aspirina em baixas doses, e determinar possíveis fatores agravantes da síndrome. Métodos: 17 pacientes portadoras da síndrome antifosfolípide foram submetidas a rigoroso acompanhamento pré-natal. A heparina foi utilizada na dose de 10.000 UI/dia e a aspirina na dose de 100 mg/dia. Foram analisados resultados perinatais e maternos, mediante a utilização do teste do chi² e do teste exato de Fischer. Resultados: o índice de recém-nascidos vivos foi de 88,2% nas gestações tratadas dessas pacientes contra 13,3% nas gestações prévias não-tratadas dessas mesmas pacientes. Foi alta a incidência de complicações gestacionais: oligoidrâmnio (40%, sofrimento fetal anteparto (33,3%, crescimento intra-uterino retardado (33,3%, diabetes mellitus gestacional (29,4%, pré-eclâmpsia (23,5% e prematuridade (60%. A presença do lúpus eritematoso sistêmico foi indicação de mau prognóstico. Nenhum efeito colateral significativo foi observado durante o tratamento. Conclusões: o tratamento adotado se mostrou efetivo na obtenção de maior índice de recém-nascidos vivos, seguro, mas incapaz de impedir a alta incidência de complicações maternas e perinatais associadas à síndrome. O lúpus eritematoso sistêmico se mostrou um fator agravante da síndrome antifosfolípide.Purpose: to determine the effectiveness and the safety of treatment with heparin and low-dose aspirin in pregnant women with antiphospholipid syndrome, and to determine possible deteriorating factors for this syndrome. Methods: 17 patients with antiphospholipid syndrome were submitted to a rigorous antenatal care. Patients were treated with a fixed dose of heparin (10,000 IU/day associated with low-dose aspirin (100 mg/day. We analyzed perinatal and maternal results, using chi² test and Fischer's exact test. Results: the overall live

  18. Sleep disturbances in voltage-gated potassium channel antibody syndrome.

    Science.gov (United States)

    Barone, Daniel A; Krieger, Ana C

    2016-05-01

    Voltage-gated potassium channels (VGKCs) are a family of membrane proteins responsible for controlling cell membrane potential. The presence of antibodies (Ab) against neuronal VGKC complexes aids in the diagnosis of idiopathic and paraneoplastic autoimmune neurologic disorders. The diagnosis of VGKC Ab-associated encephalopathy (VCKC Ab syndrome) should be suspected in patients with subacute onset of disorientation, confusion, and memory loss in the presence of seizures or a movement disorder. VGKC Ab syndrome may present with sleep-related symptoms, and the purpose of this communication is to alert sleep and neurology clinicians of this still-under-recognized condition. In this case, we are presenting the VGKC Ab syndrome which improved after treatment with solumedrol. The prompt recognition and treatment of this condition may prevent the morbidity associated with cerebral atrophy and the mortality associated with intractable seizures and electrolyte disturbances.

  19. 抗磷脂抗体对妊娠丢失筛查的临床价值%Clinical value of antiphospholipid antibody in the screening for pregnancy loss

    Institute of Scientific and Technical Information of China (English)

    张浩如

    2013-01-01

    Objective To investigate the relationship between history of pregnancy loss and antiphospholipid antibodies (APA),including anticardiolipin (ACA) and lupus anti-coagulant antibodies (LA).Methods One hundred and fifty patients with history of unexplained pregnancy loss as study group and 120 normal nonpregnant women as control group.The study group was further divided into three subgroups:embryo growth arrest (n =36),stillbirth (n =44),and recurrent abortion (n =70)levels of serum APA(including ACA and LA) were determined repectively and analyzed.Results The positive rates of APA,ACA,LA in the study group were significantly higher than those in control group (P < 0.05).The above significance was true in both stillbirth and recurrent abortion groups but not in embryo growth arrest group.Conclusions Levels of serum APA are associated with pregnancy loss,especially about recurrent abortion and stillbirth.We suggest routine screening of serum APA should be performed in patients with history of fetal wastage for the sake of early treatment.%目的 探讨妊娠丢失与抗磷脂抗体(APA)[包括抗心磷脂抗体(ACA)和狼疮抗凝抗体(LA)]的关系.方法 观察组为150例有妊娠丢失史的患者,其中分为胚胎停育组(36例)、死胎组(44例)和复发性流产组(70例).对照组为同期120例正常孕妇,分别测定观察组和对照组静脉血清APA水平,并进行对比分析.结果 整个观察组APA、ACA、LA的阳性率均高于对照组,差异有统计学意义(P<0.05).胚胎停育组与对照组比较,差异无统计学意义(P>0.05).死胎组和复发性流产组的APA、ACA、LA阳性率分别与对照组比较,差异均有统计学意义(P<0.05).结论 APA与妊娠丢失有关,尤其对于复发性流产和死胎者.因此,对有不良孕产史的患者常规筛查APA,有利于尽早对因治疗.

  20. Anti-Cardiolipin Antibody in Acute Myocardial Infarction

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    Abdolreza S. Jahromi

    2010-01-01

    Full Text Available Problem statement: Myocardial infarction is the combined result of environmental and personal factors. Data concerning the relation between anti-Phospholipid (aPL antibodies and myocardial infarction in subjects without evidence of overt autoimmune disease are conflicting. Anticardiolipin antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of Anticardiolipin antibody in Acute Myocardial Infarction (AMI might shed light on etiologic mechanisms in the pathogenesis of acute coronary syndromes. The purpose of the present study was to determine association of plasma aPL antibodies, namely, anti-Cardiolipin (aCL antibodies, with AMI. Approach: This study recruited 45 patients with the diagnosis of AMI according to WHO criteria in their first 24 h of admission. Thirty six matched individuals were studied as the control group with normal coronary artery angiography. Samples were tested for IgG-class antibodies to cardiolipin by an ELISA and the results were compared. Results: There were not significant differences between plasma level of aCLAs IgG in the patients with AMI on admission ant the control group. Also aCLAs IgG was not correlated with hypertension, diabetes mellitus, hyperlipidemia, sex, age and smoking. Conclusion: Our findings suggest that aCLAs IgG are not indicative of hypercoagulable state in patients with AMI.

  1. Estudo comparativo entre a síndrome antifosfolípide primária e a secundária: características clínico-laboratoriais em 149 pacientes Comparative study between primary and secondary antiphospholipid syndrome: clinic and laboratorial characteristics of 149 patients

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    Caio Robledo D'Angioli Costa Quaio

    2008-12-01

    .017 were more prevalent in the first group. Except cataract that was more frequently observed in secondary APS group (10% vs. 0, p = 0.017, no other significant difference was found regarding comorbidities. In relation to autoantibodies, the secondary APS patients had a more significant prevalence of ANA (99% vs. 60%, p < 0.0001, anti-ENA (45.9% vs. 22%, p = 0.007 e anti-Ro/SS-A (43% vs. 8%, p < 0.0001. Antiphospholipid antibodies (IgM anticardiolipin and lupus anticoagulant did not differ between the groups, except IgG anticardiolipin that was more found in secondary APS group (84.7% vs. 60%, p = 0.0018, CONCLUSIONS: the present study showed that primary APS had more arterial thrombotic events, more specifically Sneddon's syndrome and limbs ischemia, than secondary APS. It was also reinforced the role of autoantibodies to identify patients with APS associated to SLE.

  2. [IgG antibodies against toxic shock syndrome toxin 1 in human immunoglobulins].

    Science.gov (United States)

    Dickgiesser, N; Kustermann, B

    1986-07-15

    IgG antibodies against toxic shock syndrome toxin-1 in human immunoglobulins were determined using the ELISA technique. Of the drugs for intramuscular application, hemogamma and beriglobin contained the highest amount of antibodies. The highest concentration of antibodies in drugs for intravenous application was found in Pseudomonas polyglobin and in Venimmun. PMID:3762013

  3. Anti-β2GPI antibodies stimulate endothelial cell microparticle release via a nonmuscle myosin II motor protein-dependent pathway.

    Science.gov (United States)

    Betapudi, Venkaiah; Lominadze, George; Hsi, Linda; Willard, Belinda; Wu, Meifang; McCrae, Keith R

    2013-11-28

    The antiphospholipid syndrome is characterized by thrombosis and recurrent fetal loss in patients with antiphospholipid antibodies (APLAs). Most pathogenic APLAs are directed against β2-glycoprotein I (β2GPI), a plasma phospholipid binding protein. One mechanism by which circulating antiphospholipid/anti-β2GPI antibodies may promote thrombosis is by inducing the release of procoagulant microparticles from endothelial cells. However, there is no information available concerning the mechanisms by which anti-β2GPI antibodies induce microparticle release. In seeking to identify proteins phosphorylated during anti-β2GPI antibody-induced endothelial activation, we observed phosphorylation of nonmuscle myosin II regulatory light chain (RLC), which regulates cytoskeletal assembly. In parallel, we observed a dramatic increase in the formation of filamentous actin, a two- to fivefold increase in the release of endothelial cell microparticles, and a 10- to 15-fold increase in the expression of E-selectin, intercellular adhesion molecule 1, vascular cell adhesion molecule 1, and tissue factor messenger RNA. Microparticle release, but not endothelial cell surface E-selectin expression, was blocked by inhibiting RLC phosphorylation or nonmuscle myosin II motor activity. These results suggest that distinct pathways, some of which mediate cytoskeletal assembly, regulate the endothelial cell response to anti-β2GPI antibodies. Inhibition of nonmuscle myosin II activation may provide a novel approach for inhibiting microparticle release by endothelial cells in response to anti-β2GPI antibodies. PMID:23954892

  4. Miller-Fisher Syndrome: Are Anti-GAD Antibodies Implicated in Its Pathophysiology?

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    Ioannis E. Dagklis

    2016-01-01

    Full Text Available Miller-Fisher syndrome (MFS is considered as a variant of the Guillain-Barre syndrome (GBS and its characteristic clinical features are ophthalmoplegia, ataxia, and areflexia. Typically, it is associated with anti-GQ1b antibodies; however, a significant percentage (>10% of these patients are seronegative. Here, we report a 67-year-old female patient who presented with the typical clinical features of MFS. Workup revealed antibodies against glutamic acid decarboxylase (GAD in relatively high titers while GQ1b antibodies were negative. Neurological improvement was observed after intravenous gamma globulin and follow-up examinations showed a continuous clinical amelioration with simultaneous decline of anti-GAD levels which finally returned to normal values. This case indicates that anti-GAD antibodies may be associated with a broader clinical spectrum and future studies in GQ1b-seronegative patients could determine ultimately their clinical and pathogenetic significance in this syndrome.

  5. Guillain-Barré syndrome- and Miller Fisher syndrome-associated Campylobacter jejuni lipopolysaccharides induce anti-GM1 and anti-GQ1b Antibodies in rabbits.

    NARCIS (Netherlands)

    M.A. de Klerk; H.P. Endtz (Hubert); B.C. Jacobs (Bart); J.D. Laman (Jon); F.G.A. van der Meché; P.A. van Doorn (Pieter); C.W. Ang (Wim)

    2001-01-01

    textabstractCampylobacter jejuni infections are thought to induce antiganglioside antibodies in patients with Guillain-Barre syndrome (GBS) and Miller Fisher syndrome (MFS) by molecular mimicry between C. jejuni lipopolysaccharides (LPS) and gangliosides. We used purifi

  6. Production and Characterization of Monoclonal Antibodies of Shrimp White Spot Syndrome Virus Envelope Protein VP28

    Institute of Scientific and Technical Information of China (English)

    Wan-gang GU; Jun-fa YUAN; Ge-lin XU; Li-juan LI; Ni LIU; Cong ZHANG; Jian-hong ZHANG; Zheng-li SHI

    2007-01-01

    BALB/c mice were immunized with purified White spot syndrome virus (WSSV).Six monoclonal antibody cell lines were selected by ELISA with VP28 protein expressed in E.coll in vitro neutralization experiments showed that 4 of them could inhibit the virus infection in crayfish.Westernblot suggested that all these monoclonal antibodies were against the conformational structure of VP28.The monoclonal antibody 7B4 was labeled with colloidal gold particles and used to locate the VP28 on virus envelope by immunogold labeling.These monoclonal antibodies could be used to develop immunological diagnosis methods for WSSV infection.

  7. Evaluation of salivary gland protein 1 antibodies in patients with primary and secondary Sjogren's syndrome.

    Science.gov (United States)

    Shen, Long; Kapsogeorgou, Efstathia K; Yu, Meixing; Suresh, Lakshmanan; Malyavantham, Kishore; Tzioufas, Anthanasios G; Ambrus, Julian L

    2014-11-01

    Sjogren's syndrome (SS) has been associated with the expression of anti-Ro and anti-La antibodies. Anti-salivary gland protein 1 (SP1) antibodies have recently been identified in patients with SS. The current work involved a cross sectional study to determine whether anti-SP1 antibodies were identified in particular subgroups of patients with SS. The results of this study revealed that anti-SP1 antibodies were present in the sera of 52% of SS patients while anti-Ro/anti-La was present in 63% of patients. 19% of patients had anti-SP1 without anti-Ro/anti-La. Patients with SS and lymphoma expressed anti-Ro, anti-La and anti-SP1 together. In SS associated with RA, 50% had antibodies anti-SP1 while 40% had anti-Ro/anti-La. In conclusion, anti-SP1 antibodies are commonly seen in both primary and secondary SS and rarely in normal controls. Future studies are needed to determine the roles and timing of expression of anti-SP1 antibodies in Sjogren's syndrome.

  8. Hipertensión pulmonar tromboembólica crónica con y sin síndrome antifosfolípido Chronic thromboembolic pulmonary hypertension with and without antiphospholipid syndrome

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    Cecilia Colorio

    2007-06-01

    Full Text Available La hipertensión pulmonar tromboembólica crónica (HPTC está dada por la obstrucción de arterias pulmonares y sus ramas por trombos organizados. El objetivo fue evaluar las características y evolución de la HPTC en pacientes con y sin síndrome antifosfolípido (SAF. Se analizaron retrospectivamente todos los pacientes con HPTC en nuestra institución entre junio de 1993 y junio del 2005. De los 38 pacientes evaluados, 16 tenían SAF (grupo A y 22 pacientes (grupo B presentaban estudio de trombofilia normal (n = 10 u otras anormalidades trombofílicas (n = 12. Ambos grupos fueron similares en cuanto a la media de edad (41 vs. 50 años, la clase funcional que presentaban al diagnóstico (81 vs. 100% en clase III-IV, y la presencia de trombosis en otros territorios (31 vs. 27%. El 62.5% de pacientes del grupo A y el 54.5% del grupo B fueron sometidos a tromboendarterectomía pulmonar. La media de seguimiento fue de 45 meses (rango de 0.5 a 144. Al final del mismo todos los pacientes con SAF y todos los pacientes operados estaban en clase funcional I-II. La mediana de sobrevida desde el momento del diagnóstico alcanzó los 59 meses para el grupo A vs. 27 meses en el grupo B (p = 0.199. La media de sobrevida del total de los pacientes operados fue de 56 meses para el grupo A vs. 33 meses para el grupo B (p = 0.08. En conclusión: Los pacientes con HPTC y SAF tendieron a lograr una mejor sobrevida que los pacientes con HPTC sin SAF. Aquellos con HPTC y SAF que fueron operados lograron mejoría de su clase funcional y tendieron a una mayor sobrevida respecto de los no operados.Chronic thromboembolic pulmonary hypertension (CTE-PH is defined as the chronic obstruction by organized thrombi in pulmonary artery and their branches causing pulmonary hypertension. The objective is to evaluate features and outcome of CTE-PH in patients with and without coexisting antiphospholipid syndrome (APS. All patients studied at our Institution with CTE-PH between

  9. Anti-natrium/iodide symporter antibodies and other anti-thyroid antibodies in children with Turner's syndrome.

    Science.gov (United States)

    Kucharska, Anna M; Czarnocka, Barbara; Demkow, Urszula

    2013-01-01

    Antibodies against the Na/I symporter (anti-NIS ab) have been found in adult patients with autoimmune thyroid diseases. As easily available for the immune system, NIS can play a role in the initial stage of autoimmune thyroid diseases. Children with Turner's syndrome (TS) being at high risk of autoimmune thyroid disease development seem a valuable group for the investigation of the early autoimmune process. The aim of the study was to investigate the presence of anti-NIS ab and its potential clinical significance in TS children. Fifty four girls with TS were examined (age 11.9 ± 2.46 years), and 23 healthy girls with normal thyroid function, free of autoimmune diseases. Anti-NIS antibodies were measured by the in-house ELISA method and the Western blotting. Sera considered positive for anti-NIS ab were used for the iodide uptake bioassay using COS7 cells stably transfected with hNIS. In all patients the thyroid function, antithyroid antibodies presence and thyroid ultrasonography were evaluated. In 20% of the patients a subclinical hypothyroidism was diagnosed and 70.4% had antithyroid antibodies (anti-TPO - 64.8% and Anti-Tg - 24%). Anti-NISab were present in 14.8% girls with TS and in none of the control group. Their presence was unrelated to other antithyroid antibodies titre or patients' age. A positive correlation between the anti-NIS ab presence and the hypothyroidism was found (p < 0.04). Anti-NIS ab-positive sera did not suppress iodine uptake. In conclusion, anti-NIS antibodies were present in 14.8% of children with TS and they were related to the presence of hypothyroidism. PMID:22836628

  10. Inhibition of middle east respiratory syndrome coronavirus infection by anti-CD26 monoclonal antibody

    NARCIS (Netherlands)

    K. Ohnuma (Kei); B.L. Haagmans (Bart); R. Hatano (Ryo); V.S. Raj (Stalin); H. Mou (Huihui); S. Iwata (Satoshi); R.L. Dang (Rong); B.J. Bosch (Berend Jan); C. Morimoto (Chikao)

    2013-01-01

    textabstractWe identified the domains of CD26 involved in the binding of Middle East respiratory syndrome coronavirus (MERS-CoV) using distinct clones of anti-CD26 monoclonal antibodies (MAbs). One clone, named 2F9, almost completely inhibited viral entry. The humanized anti-CD26 MAb YS110 also sign

  11. Rare Association of Anti-Hu Antibody Positive Paraneoplastic Neurological Syndrome and Transitional Cell Bladder Carcinoma

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    S. Lukacs

    2012-01-01

    Full Text Available Introduction. Paraneoplastic encephalomyelitis (PEM and subacute sensory neuronopathy (SSN are remote effects of cancer, usually associated with small-cell lung carcinoma and positive anti-Hu antibody. We describe the rare association of bladder transitional cell carcinoma (TCC with anti-Hu antibody positivity resulting in this paraneoplastic neurological syndrome. Patient. A 76-year-old female presented with bilateral muscle weakness and paraesthesia of the upper and lower limbs in a length-dependent “glove and stocking” distribution. Central nervous system symptoms included cognitive problems, personality change, and truncal ataxia. Case notes and the literature were reviewed. Result. Autoantibody screening was positive for anti-Hu antibody (recently renamed antineuronal nuclear antibody 1, ANNA-1. The diagnosis of PEM and SSN was supported by MRI and lumbar puncture results. A superficial bladder TCC was demonstrated on CT and subsequently confirmed on histology. No other primary neoplasm was found on full-body imaging. The neurological symptoms were considered to be an antibody-mediated paraneoplastic neurological syndrome and improved after resection of the tumour. Discussion. The association of anti-Hu positive paraneoplastic neurological syndrome and TCC has not been described in the literature previously. We emphasize the need for detailed clinical examination and the importance of a multidisciplinary thought process and encourage further awareness of this rare association.

  12. Anti-β2 Glycoprotein-I Antibody in Acute Myocardial Infarction

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    Mohammad Shojaei

    2011-01-01

    Full Text Available Problem statement: Ischemic cardiac manifestations have been reported in a various percentage of patients with anti-phospholipid antibodies. Data concerning the relation between anti- Phospholipid (aPL antibodies and myocardial infarction in subjects without evidence of overt autoimmune disease are conflicting. Anti-beta2 glycoprotein-I (anti-beta2-GPI antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of anti-beta2-GPI antibody in Acute Myocardial Infarction (AMI might shed light on etiologic mechanisms in the pathogenesis of acute coronary syndromes. The purpose of the present study was to determine association of plasma aPL antibodies, namely, antibeta2- GPI antibodies, with AMI. This study was designed to investigate whether prevalence of antibeta2- GPI antibodies, in patients who had acute myocardial infarction and to analyze their relationship with traditional cardiovascular risk factors. Approach: We investigated the prevalence of anti-beta2- GPI IgG in a well characterized group of patients with AMI as a case group. Sera from 74 patients with AMI and from 76 healthy subjects, matched for age and sex as a control group. Using ELISA to evaluate the presence of IgG isotype of anti-beta2-GPI autoantibodies in their sera. Results: The prevalence of anti-beta2-GPI IgG in the control group (10.50% resulted significantly lower than in patients with AMI (37.80% (pConclusion: Our findings suggest that anti-beta2-GPI IgG antibodies seemed to behave as independent risk factors for myocardial infarction, which may represent a link between autoimmunity and atherosclerosis in patients with acute myocardial infarction. Further studies with bigger patients are needed to explore association of anti-β2-GPI IgG with STEMI and NSTEMI.

  13. [Psychosis as debut of antiphospholipid syndrome].

    Science.gov (United States)

    del Rio-Casanova, Lucía; Paz-Silva, Eduardo; Requena-Caballero, Ignacio; Diaz-Llenderrozas, Francisco; Gomez-Trigo Baldominos, Jesús; de la Cruz-Davila, Alberto; Paramo-Fernandez, Mario

    2014-06-01

    Introduccion. El sindrome antifosfolipido (SAF) es un trastorno autoinmune que determina un estado de hipercoagulabilidad caracterizado por eventos tromboticos, abortos de repeticion y la presencia de anticuerpos antifosfolipido. Puede presentarse de manera aislada (SAF primario) o asociado a lupus eritematoso sistemico u otras enfermedades autoinmunes (SAF secundario). Las manifestaciones neuropsiquiatricas del SAF incluyen jaqueca, epilepsia, corea, demencia y psicosis. Se realiza una revision bibliografica de la sintomatologia neuropsiquiatrica en general y psicotica en particular de los pacientes afectos de SAF. Caso clinico. Mujer de 23 años afecta de un SAF primario que comenzo con manifestaciones clinicas unicamente neuropsiquiatricas consistentes en sintomatologia psicotica (dos episodios de clinica delirante) y movimientos anormales coreiformes y hemibalisticos que inicialmente se interpretaron como clinica conversiva. Conclusiones. Se discute la patogenesis de la sintomatologia motora y psicotica. La etiologia de los sintomas neuropsiquiatricos todavia no se conoce con claridad, pero la trombosis de pequeños vasos en el cerebro podria explicar parte de los sintomas. Asimismo, se revisa el papel que la medicacion antipsicotica y antitrombotica tiene para estos pacientes. En la actualidad, nuestra paciente se mantiene asintomatica sin necesidad de terapia antipsicotica y recibe tratamiento unicamente con un agente antiagregante y otro antipaludico.

  14. Anti-Ganglioside antibodies in Guillain-Barre Syndrome : Do They indicate Prognosis?

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    Menon Ashok

    2003-01-01

    Full Text Available This study aimed to detect anti-ganglioside antibodies in the sera of patients with Guillain-Barre syndrome and correlate their presence with clinical features, electrophysiological studies and outcome. Twenty patients with GBS were evaluated clinically and electrophysiologically. Serological assays for antibodies against GM1, GD1a and GD1b gangliosides were carried out by ELISA, Twelve patients tested positive; two had antibodies against all three gangliosides, one against both GM1 and GD1a, one against GM1, GD1a or GD1b alone were seen in two, five and one patient respectively. No significant correlation was noted between the presence or type of antibody with clinical features, electrophysiological findings and outcome.

  15. Morvan's syndrome with anti contactin associated protein like 2 – voltage gated potassium channel antibody presenting with syndrome of inappropriate antidiuretic hormone secretion

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    Anjani Kumar Sharma

    2016-01-01

    Full Text Available Morvan's syndrome is a rare autoimmune disorder characterized by triad of peripheral nerve hyperexcitability, autonomic dysfunction, and central nervous system symptoms. Antibodies against contactin-associated protein-like 2 (CASPR2, a subtype of voltage-gated potassium channel (VGKC complex, are found in a significant proportion of patients with Morvan's syndrome and are thought to play a key role in peripheral as well as central clinical manifestations. We report a patient of Morvan's syndrome with positive CASPR2–anti-VGKC antibody having syndrome of inappropriate antidiuretic hormone as a cause of persistent hyponatremia.

  16. Morvan's syndrome with anti contactin associated protein like 2 – voltage gated potassium channel antibody presenting with syndrome of inappropriate antidiuretic hormone secretion

    Science.gov (United States)

    Sharma, Anjani Kumar; Kaur, Manminder; Paul, Madhuparna

    2016-01-01

    Morvan's syndrome is a rare autoimmune disorder characterized by triad of peripheral nerve hyperexcitability, autonomic dysfunction, and central nervous system symptoms. Antibodies against contactin-associated protein-like 2 (CASPR2), a subtype of voltage-gated potassium channel (VGKC) complex, are found in a significant proportion of patients with Morvan's syndrome and are thought to play a key role in peripheral as well as central clinical manifestations. We report a patient of Morvan's syndrome with positive CASPR2–anti-VGKC antibody having syndrome of inappropriate antidiuretic hormone as a cause of persistent hyponatremia.

  17. Morvan's syndrome with anti contactin associated protein like 2 – voltage gated potassium channel antibody presenting with syndrome of inappropriate antidiuretic hormone secretion

    Science.gov (United States)

    Sharma, Anjani Kumar; Kaur, Manminder; Paul, Madhuparna

    2016-01-01

    Morvan's syndrome is a rare autoimmune disorder characterized by triad of peripheral nerve hyperexcitability, autonomic dysfunction, and central nervous system symptoms. Antibodies against contactin-associated protein-like 2 (CASPR2), a subtype of voltage-gated potassium channel (VGKC) complex, are found in a significant proportion of patients with Morvan's syndrome and are thought to play a key role in peripheral as well as central clinical manifestations. We report a patient of Morvan's syndrome with positive CASPR2–anti-VGKC antibody having syndrome of inappropriate antidiuretic hormone as a cause of persistent hyponatremia. PMID:27695240

  18. Anticorpos antinucleossomo e síndrome antifosfolipídica: estudo observacional Antinucleosome antibodies and primary antiphospholipid syndrome: an observational study

    OpenAIRE

    Alexandre Wagner Silva de Souza; Silene Peres Keusseyan; Neusa Pereira da Silva; Emilia Inoue Sato; Luis Eduardo Coelho Andrade

    2012-01-01

    OBJETIVO: Avaliar a associação entre a presença de anticorpos antinucleossomo (anti-NCS) e a síndrome antifosfolipídica primária (SAFP) e o posterior desenvolvimento de lúpus eritematoso sistêmico (LES). MATERIAIS E MÉTODOS: Trinta e seis mulheres com o diagnóstico de SAFP foram avaliadas prospectivamente para manifestações de doenças reumáticas autoimunes e para a presença de anticorpos antifosfolípides, anticorpos antinucleares e anti-NCS/cromatina. RESULTADOS: Após um período médio de segu...

  19. Glutamic acid decarboxylase antibody-positive paraneoplastic stiff limb syndrome associated with carcinoma of the breast

    Directory of Open Access Journals (Sweden)

    Agarwal Pankaj

    2010-01-01

    Full Text Available Stiff limb syndrome (SLS is a rare "focal" variant of stiff person syndrome which presents with rigidity and painful spasms of a distal limb, and abnormal fixed foot or hand postures. Anti-glutamic acid decarboxylase antibodies (GAD-Ab are variably present in most cases. Most reported cases of SLS are unassociated with cancer. We describe a patient with SLS as a paraneoplastic manifestation of breast carcinoma, in whom GAD-Ab was present. The patient responded very well to oral diazepam, baclofen and steroids.This is the third reported case of SLS as a paraneoplastic accompaniment to cancer.

  20. 抗磷脂抗体及凝血因子Ⅻ缺乏与视网膜静脉阻塞的相关研究%The correlation of antiphospholipid antibody and factor Ⅻ deficiency in patients with retinal vein occlusion

    Institute of Scientific and Technical Information of China (English)

    孙红晶; 李毓敏

    2009-01-01

    目的 观察抗磷脂抗体(APA)及凝血因子Ⅻ(F Ⅻ)缺乏在视网膜静脉阻塞(RVO)发病中的作用.方法 对RVO患者33例(33眼)及正常对照组30例(30眼),采用ELISA法检测抗心磷脂抗体(ACA)(IgG、IgM、IgA);APTT法检测LA;一期法测定FⅫ活性.采用Fisher检验进行统计学分析.结果 RVO组APAs总阳性率24.24%(8/33)与对照组6.67%(2/30)比较差异无统计学意义(P=0.085).RVO组ACA阳性率18.18%(6/33)与对照组(0/30)比较差异有统计学意义(P=0.025).≤50岁组及>50岁组RVO患者LA总阳性率与同年龄对照组相比差异均无统计学意义(P=0.160,P=0.206).RVO组FⅫ缺乏率42.42%(14/33)与对照组13.33%(4/30)比较差异有统计学意义(P=0.013).≤50岁组及>50岁组RVO患者FⅫ缺乏与同年龄对照组比较差异无统计学意义(P=0.206,P=0.052).结论 研究表明ACA阳性及FⅫ缺乏引起的凝血障碍与RVO发病相关.%Objective Antiphospholipid antibody and factor Ⅻ deficiency are among the coagulation disorders that have been implicated in many thrombembolic events. The aim of this study was to investigate the prevalence of antiphospholipid antibodies and factor Ⅻ deficiency in patients with retinal vein occlusion (RVO). Methods The investigation was a case control study. The periphery blood was collected from a cohort of 33 consecutive patients with RVO and 30 age- and gender-matched normal subjects. Anticardiolipin antibody (ACA) was detected by ELISA as binding index (BI) =A value/standard A value. The lupus anticoagulant antibody was examined by APTT test and the activity of factor Ⅻ was detected. This study was approved by The Human Research Ethics Committee of this hospital, and written informed consent was obtained from all the subjects before initiation of any study protocol. Results The total positive rate of APA in RVO group was 24. 24% (8/33), showing a insignificant difference in comparison with control group (6. 67%, 2/30) (P = 0. 085). The positive rate of

  1. A importância da intervenção nutricional na redução do peso corpóreo em pacientes com síndrome do anticorpo antifosfolípide The importance of nutritional intervention in the reduction of body weight in patients with the antiphospholipid antibody

    Directory of Open Access Journals (Sweden)

    Karin Klack

    2008-06-01

    Full Text Available OBJETIVO: Avaliar a eficácia da intervenção nutricional na redução do excesso de peso (EP, em pacientes com síndrome do anticorpo antifosfolípide (SAF. MÉTODO: Incluídos 40 pacientes, acima de 18 anos, com diagnóstico de SAF primária ou secundária, acompanhados no Serviço de Reumatologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP e recrutados no período de outubro de 2005 a fevereiro de 2006. Foram coletados dados demográficos e realizados a revisão de prontuários, a mensuração de peso e da altura, o cálculo do índice de massa corpórea (IMC atual e a adequação da dieta pelo IMC ideal. Foi realizada avaliação na primeira consulta e após intervalo mínimo de dois meses. RESULTADOS: A média de idade dos pacientes foi de 41 anos, sendo 93% de mulheres. Os pacientes com SAF primária perfaziam 25%, com média de dois anos da doença. A trombose venosa ocorreu em 63%, arterial em 48% e manifestações obstétricas em 27%. Na primeira consulta, 68% apresentavam EP, 27% eram eutróficos e 5% estavam com baixo peso (BP. Após três meses de intervenção, os eutróficos mantiveram o peso e os de BP tornaram-se eutróficos, segundo o IMC. Interessantemente, entre os pacientes com EP (n = 27, 82% emagreceram, 14% engordaram e 4% se mantiveram. Especificamente, 11 pacientes apresentaram 1% a 3% de perda ponderal de peso, oito perderam de 4% a 7%, dois reduziram 8% a 9% e um reduziu 13,6% com o acompanhamento nutricional. CONCLUSÃO: Foi demonstrado no presente estudo que a intervenção nutricional conseguiu atingir metas para redução de peso, possibilitando diminuição no risco trombótico num curto período, sendo, portanto, uma modalidade terapêutica inicial e de eleição para corrigir o EP em pacientes com SAF.OBJECTIVE: To evaluate the efficacy of the nutricional intervention in the loss of the weight excess (WE, in patients with the antiphospholipid antibody syndrome (APS

  2. Anti-phospholipid Sindrome. A case report Síndrome antifosfolípido. Presentación de caso

    OpenAIRE

    Grace A. de Lara; Eduardo B. Hernández Ortiz; José M. Carles Zerquera

    2007-01-01

    A case of a young female patient suffering from an anti-phospholipid syndrome whose evolution was accompanied by repeated episodes of jaundice and, who finally deceased due to a cerebrovascular ischemia is presented. Although the appearance of this syndrome is not frequent, it is very important to know its particularities as well as to take into account its possible presence in young patients with past history of abortions and hypercoagulabilty episodes.

  3. Serum antibodies against frameshift peptides in microsatellite unstable colorectal cancer patients with Lynch syndrome.

    Science.gov (United States)

    Reuschenbach, Miriam; Kloor, Matthias; Morak, Monika; Wentzensen, Nicolas; Germann, Anja; Garbe, Yvette; Tariverdian, Mirjam; Findeisen, Peter; Neumaier, Michael; Holinski-Feder, Elke; von Knebel Doeberitz, Magnus

    2010-06-01

    High level microsatellite instability (MSI-H) occurs in about 15% of colorectal cancer (CRCs), either as sporadic cancers or in the context of hereditary non-polyposis cancer or Lynch syndrome. In MSI-H CRC, mismatch repair deficiency leads to insertion/deletion mutations at coding microsatellites and thus to the translation of frameshift peptides (FSPs). FSPs are potent inductors of T cell responses in vitro and in vivo. The present study aims at the identification of FSP-specific humoral immune responses in MSI-H CRC and Lynch syndrome. Sera from patients with history of MSI-H CRC (n = 69), healthy Lynch syndrome mutation carriers (n = 31) and healthy controls (n = 52) were analyzed for antibodies against FSPs using peptide ELISA. Reactivities were measured against FSPs derived from genes frequently mutated in MSI-H CRCs, AIM2, TGFBR2, CASP5, TAF1B, ZNF294, and MARCKS. Antibody reactivity against FSPs was significantly higher in MSI-H CRC patients than in healthy controls (P = 0.036, Mann-Whitney) and highest in patients with shortest interval between tumor resection and serum sampling. Humoral immune responses in patients were most frequently directed against FSPs derived from mutated TAF1B (11.6%, 8/69) and TGFBR2 (10.1%, 7/69). Low level FSP-specific antibodies were also detected in healthy mutation carriers. Our results show that antibody responses against FSPs are detectable in MSI-H CRC patients and healthy Lynch syndrome mutation carriers. Based on the high number of defined FSP antigens, measuring FSP-specific humoral immune responses is a highly promising tool for future diagnostic application in MSI-H cancer patients.

  4. Synthetic LNA/DNA nano-scaffolds for highly efficient diagnostics of nucleic acids and autoimmune antibodies

    DEFF Research Database (Denmark)

    Astakhova, Irina Kira

    2014-01-01

    Herein novel fluorescent oligonucleotides for homogeneous (all-in-solution) detection of nucleic acids and autoimmune antibodies (autoantibodies) are described. The probes are prepared by highly efficient copper-catalyzed click chemistry between novel alkyne-modified locked nucleic acid (LNA...... of the monoclonal human autoantibody is achieved. It makes the novel "clickable" LNA/DNA complexes a very promising tool in molecular diagnostics of both nucleic acids and autoantibodies against DNA. The latter are produced under several autoimmune conditions including antiphospholipide syndrome and systemic lupus...

  5. Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Wisnieski, J J; Naff, G B

    1989-09-01

    Urticaria, angioedema, and arthritis are cardinal features of hypocomplementemic urticarial vasculitis syndrome (HUVS). Considered to be an immune complex-mediated disorder, HUVS has been differentiated from systemic lupus erythematosus (SLE), based on its clinical manifestations and the C1q precipitin (C1q-p) reaction, which is manifested as gel precipitation of C1q by a small percentage of HUVS IgG molecules. This phenomenon has been attributed to an Fc region abnormality, and the responsible IgG molecules are said to possess C1q-p activity. We purified IgG from 4 HUVS patients and confirmed that HUVS IgG contains C1q binding activity. F(ab')2 fragments from these patients also bound to C1q, as measured by 2 different C1q binding methods at physiologic ionic strength; HUVS IgG Fc fragments did not bind to C1q. Preincubation of HUVS F(ab')2 fragments with antibody to human F(ab')2 prevented subsequent binding to C1q. We conclude that IgG antibodies to C1q are present in HUVS serum, and it is likely that these antibodies are C1q-p. Because the clinical manifestations of HUVS and the presence of anti-C1q antibodies have been described in patients with SLE, our findings support the concept that HUVS is an autoimmune syndrome related to SLE. PMID:2528353

  6. Bickerstaff’s brainstem encephalitis, Miller Fisher syndrome and Guillain-Barré syndrome overlap in an asthma patient with negative anti-ganglioside antibodies

    OpenAIRE

    Han Chongyu; Wang Yuan; Jia Jianping; Ji Xunming; Fredrickson Vance; Ding Yuchuan; Sun Wei; Xu Jia; Sun Yong-Xin

    2012-01-01

    Abstract Background Bickerstaff’s brainstem encephalitis (BBE), together with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) were considered to form a continuous clinical spectrum. An anti-GQ1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders; however, other studies have found a positive anti-GM1 instead of anti-GQ1b antibody. Case presentation Here we report a 20-year-old male patient with overlapping BBE, MFS and GBS. The pat...

  7. Manifestações neuropsiquiátricas em crianças e adolescentes com lúpus eritematoso sistêmico juvenil: associação com anticorpos antifosfolípide? Neuropsychiatric manifestations of children and adolescents with juvenile systemic lupus erythematosus: is there an association with antiphospholipid antibodies?

    Directory of Open Access Journals (Sweden)

    Cássia Maria Passarelli Lupoli Barbosa

    2006-10-01

    Full Text Available OBJETIVO: estudar a freqüência de anticorpos antifosfolípide (aFL em pacientes com lúpus eritematoso sistêmico juvenil (LESJ e sua possível associação com manifestações neuropsiquiátricas. MÉTODOS: análise retrospectiva de prontuários de 64 pacientes com LESJ, de acordo com os critérios do American College of Rheumatology (ACR, acompanhados por um período mínimo de seis meses. Foram consideradas manifestações neuropsiquiátricas: cefaléia, convulsão, acidente vascular cerebral (AVC, coréia, neuropatia medular e periférica, além de alterações do comportamento, com ou sem psicose. Duas dosagens de anticorpos anticardiolipina foram realizadas com intervalo de dois meses e foram considerados positivos os títulos de IgG maiores que 20 e de IgM maiores que 12. O anticoagulante lúpico foi dosado em 32 pacientes. A análise estatística foi realizada através do teste de Fisher com nível de significância OBJECTIVE: to study the frequency of antiphospholipid antibodies (aPL in patients with juvenile systemic lupus erythematosus (JSLE and the possible association to neuropsychiatric manifestations. METHODS: retrospective analysis of charts of 64 JSLE patients according to the American College of Rheumatology (ACR classification criteria, followed for at least six months. The neuropsychiatric manifestations were defined by the presence of: headache, seizure, cerebrovascular accident (CVA, chorea, medular or peripheral neuropathy and behavior disturbances with psichosis or not. The aPL were tested in two occasions with an interval of two months. Values greater than 20 for IgG or 12 for IgM were considered as positive. The lupus anticoagulant was tested in 32 patients. The statistical analysis was performed using the Fisher’s exact test with a significance level of 0,05. RESULTS: 38 (59.4% out of 64 JSLE patients had neuropsychiatric manifestations. APL antibodies were presented in 29 patients (45.3%. We did not observe a

  8. Correlation between hsCRP and Anti-beta2GPI Antibody in Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Meiriza Djohari

    2011-08-01

    Full Text Available BACKGROUND: Several researches reported that inflammatory and immunological mechanism such as autoantibody to β2-glycoprotein I (anti β2GPI appear as related factors in initiation and progress of atherosclerosis lesion in patient with autoimmune disease. Antibody to β2GPI titers are correlated with atherosclerosis and in vitro studies showed that they enhance oxidized low density lipoprotein (ox-LDL uptake by macrophages. Immunization with auto-antigen β2GPI elicits an immune response to influence lesion progression that mostly happens in autoimmune subjects. The metabolic syndrome (MetS is combination of several metabolic disorders such as obesity, dyslipidemia, Diabetes Mellitus (DM and conditions due to inflammation and stress oxidative. The Correlation between inflammatory markers such as High sensitivity C-Reactive Protein (hsCRP and anti-β2GPI antibody in MetS needs to be further investigated. METHODS: This was an observational study with cross sectional design on subject with MetS as determined by the International Diabetes Federation (IDF 2005’s criteria. RESULTS: There was a positive and significant correlation between hsCRP and anti-β2GPI antibody in MetS group (r=0.406; p≤0.05 as compared to non-MetS group. We found that there was elevated level of anti-β2GPI antibody in hsCRP of 3-10 mg/L. CONCLUSIONS: Anti-β2GPI antibody may be elevated in subjects with MetS who have low grade of inflammation as shown by hsCRP. KEYWORDS: metabolic syndrome, inflammation, autoantigen, atherosclerosis, obesity.

  9. Lepromatous leprosy patients produce antibodies that recognise non-bilayer lipid arrangements containing mycolic acids

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    Isabel Baeza

    2012-12-01

    Full Text Available Non-bilayer phospholipid arrangements are three-dimensional structures that form when anionic phospholipids with an intermediate structure of the tubular hexagonal phase II are present in a bilayer of lipids. Antibodies that recognise these arrangements have been described in patients with antiphospholipid syndrome and/or systemic lupus erythematosus and in those with preeclampsia; these antibodies have also been documented in an experimental murine model of lupus, in which they are associated with immunopathology. Here, we demonstrate the presence of antibodies against non-bilayer phospholipid arrangements containing mycolic acids in the sera of lepromatous leprosy (LL patients, but not those of healthy volunteers. The presence of antibodies that recognise these non-bilayer lipid arrangements may contribute to the hypergammaglobulinaemia observed in LL patients. We also found IgM and IgG anti-cardiolipin antibodies in 77% of the patients. This positive correlation between the anti-mycolic-non-bilayer arrangements and anti-cardiolipin antibodies suggests that both types of antibodies are produced by a common mechanism, as was demonstrated in the experimental murine model of lupus, in which there was a correlation between the anti-non-bilayer phospholipid arrangements and anti-cardiolipin antibodies. Antibodies to non-bilayer lipid arrangements may represent a previously unrecognised pathogenic mechanism in LL and the detection of these antibodies may be a tool for the early diagnosis of LL patients.

  10. Bickerstaff’s brainstem encephalitis, Miller Fisher syndrome and Guillain-Barré syndrome overlap in an asthma patient with negative anti-ganglioside antibodies

    Directory of Open Access Journals (Sweden)

    Han Chongyu

    2012-06-01

    Full Text Available Abstract Background Bickerstaff’s brainstem encephalitis (BBE, together with Miller Fisher syndrome (MFS and Guillain-Barré syndrome (GBS were considered to form a continuous clinical spectrum. An anti-GQ1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders; however, other studies have found a positive anti-GM1 instead of anti-GQ1b antibody. Case presentation Here we report a 20-year-old male patient with overlapping BBE, MFS and GBS. The patient had a positive family history of bronchial asthma and had suffered from the condition for over 15 years. He developed BBE symptoms nine days after an asthma exacerbation. During the course of illness, he had significantly elevated IgE levels in both serum and cerebrospinal fluid. Serologic analysis of antibodies against ganglioside complexes (anti-GDIa, anti-GDIb, anti-GM1, anti-GM2, anti-GM3, anti-GQIb and anti-GTIb antibodies showed negative results. Conclusions Since asthma has recently been related to autoimmune disease, our case supports an autoimmune mechanism underlying the clinical spectrum composed of BBE, MFS and GBS. However, contrary to a proposed anti-GQ1b antibody syndrome, we would suggest that pathogenesis of this clinical spectrum is not limited to anti-ganglioside antibodies.

  11. Sildenafil no tratamento da hipertensão pulmonar associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio Sildenafil for treatment of pulmonary hypertension in association with systemic lupus erythematosus and anti-phospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Eduardo José do Rosário e Souza

    2003-10-01

    Full Text Available Hipertensão pulmonar grave é uma doença debilitante, com expectativa de vida reduzida, que acomete adultos jovens. Complicações pleuropulmonares no lúpus eritematoso sistêmico ocorrem em 50% a 70% dos pacientes. A hipertensão pulmonar grave no lúpus eritematoso sistêmico é rara e tem prognóstico reservado. Descreve-se, pela primeira vez, um paciente com hipertensão pulmonar grave associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio secundário que apresentou boa resposta ao uso do sildenafil oral, após falha do tratamento convencional com corticosteróides, ciclofosfamida, warfarin e diltiazem.Severe pulmonary hypertension is a debilitating disease with short life expectancy that often affects young people. Pleuropulmonary complications of systemic lupus erythematosus occur in 50-70% of patients. Severe symptomatic pulmonary hypertension in systemic lupus erythematosus is rare and carries a bad prognosis because a fatal outcome can occur within months. The authors describe, for the first time, a patient with systemic lupus erythematosus with severe pulmonary hypertension and secondary antiphospholipid syndrome who responded favorably to oral sildenafil, after unsuccessful use of prednisone, intravenous cyclophosphamide, warfarin and diltiazem.

  12. 系统性红斑狼疮患者抗磷脂抗体与视网膜血管病关系的研究%Relationship between Retinal Vasculopathy and Antiphospholipid Antibodies in Patients with Systemic Lupus Erythematosus

    Institute of Scientific and Technical Information of China (English)

    刘岩; 熊毅彤; 翁欢; 徐康; 李秋华

    2003-01-01

    目的:研究系统性红斑狼疮(systemic lupus erythematosus,SLE)抗磷脂抗体等血清免疫学标志物与患者视网膜血管病的关系.方法:从2002年6月至2003年1月,对164例SLE患者作最佳矫正视力检查、眼压测量、裂隙灯检查和眼底检查,血清免疫学检查指标包括抗心磷脂抗体(anticardiolipin antibody, ACL)、狼疮抗凝物(lupus anticoagulant,LA)、抗核抗体(antinuclear antibody,ANA)和抗ds-DNA抗体等.结果:164例SLE患者中26例(16%)检查到有视网膜血管病,其中19例(73%)检测到抗磷脂抗体(17例ACL和2例LA).结论:SLE患者易发生视网膜血管病变,抗磷脂抗体的存在可能与其有较高的相关性.

  13. Cronkhite-Canada syndrome showing elevated levels of antinuclear and anticentromere antibody.

    Science.gov (United States)

    Ota, Seisuke; Kasahara, Akinori; Tada, Shoko; Tanaka, Takehiro; Umena, Sachio; Fukatsu, Haruka; Noguchi, Toshio; Matsumura, Tadashi

    2015-02-01

    A 56-year-old female initially visited an otorhinolaryngologist because of an impaired sense of taste in September, 2010 and was referred to our facility in October, 2010. She was diagnosed with Basedow's disease for which she underwent subtotal thyroidectomy in 1984 and arthritis involving multiple joints, primarily affecting her hands. In addition, the anticentromere antibody (ACA) level was markedly high. On physical examination, alopecia as well as hyperpigmentation of the dorsum of the hands and back was observed. Dystrophic changes of the fingernails and a bilateral thumb abduction deformity were observed. Antinuclear antibodies were elevated. Gastrointestinal endoscopy and colonoscopy revealed the mucosa carpeted with strawberry-like polypoid lesions. Histopathological examination of the biopsied specimen of the stomach revealed a corkscrew-like appearance. Thus, the patient was diagnosed with Cronkhite-Canada syndrome (CCS). She admitted to our hospital in November, 2010. Oral prednisolone was administered with success. In July, 2012, her antimitochondrial M2 antibody level was elevated. To the best of our knowledge, the present case is the first patient with CCS, a history of Basedow's disease, and elevated levels of ACA and antimitochondrial M2 antibody. We consider the present case suggests CCS could be caused by immunological abnormality. PMID:25518819

  14. Dye Labelled Monoclonal Antibody Assay for Detection of Toxic Shock Syndrome Toxin -1 from Staphylococcus Aureus

    Directory of Open Access Journals (Sweden)

    V Javid Khojasteh

    2011-12-01

    Full Text Available Objective: The aim of study was to develop a rapid assay, dye labelled monoclonal antibody assay (DLMAA, using non-radioactive organic synthetic dyes for identification of Toxic Shock Syndrome Toxin-1 (TSST-1 producing strains of Staphylococcus aureus.Materials and Methods: The assay protocol required only two simple steps; addition of TSST-1 antigen to a nitrocellulose membrane and then adding a colloidal dye labelled antibody (D/A suspension detection reagent.Results: The sensitivity and specificity of the assay was determined relative to positive and negative strains compared to an ELISA assay. Overall 100% agreement was found between both assays. The sensitivity for detection of TSST-1 was 30 ng.Conclusion: The DLMAA did not require handling and disposal of radioactive materials. It is a rapid qualitative technique for detection of TSST-1 toxin at room temperature within a short time.

  15. The Prevalence of Anticardiolipin Antibody in Patients with Systemic Lupus Erythematosus and Its Association with Clinical Manifestations

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    Zahra Basiri

    2013-01-01

    Full Text Available The central immunological disturbance in systemic lupus erythematosus (SLE is autoantibody production. Some of these antibodies affecting components of the cell nucleus are the major characteristics of SLE. The present study was aimed to assess importance of anticardiolipin (ACL antibody and its association with clinical state in SLE patients. A cross sectional study was performed on 100 patients with SLE referred to rheumatology outpatient clinic in Ekbatan hospital in Hamadan (Iran between 2007 and 2008. Serum samples were extracted and screened for IgG and IgM using an ACL enzyme-linked immunosorbent assay. Up to 36% of patients were positive for ACL antibody that was more frequent in women than men (39.8% versus 8.3%. No association was revealed between ACL antibody and age. Clinical manifestations of antiphospholipid antibody syndrome were observed in 23.0% of patients that was more prevalent in ACL positive group compared with ACL negative group (41.7% versus 125%. The prevalence of other manifestations including pregnancy-related disorders (recurrent abortion, central nervous system defects, and deep vein thrombosis was 33.3%, 25.0%, and 30.6% in ACL positive group and was 9.4%, 7.8%, and 7.8% in ACL negative group that all were more frequent in the former group. The prevalence of thrombocytopenia was also higher in ACL positive group than another group (22.2% versus 15.6%. Among ACL positive patients with clinical manifestations of antiphospholipid antibody syndrome, 86.6% had medium to high titer of ACL. Our study emphasized value of (ACL antibody to assess clinical status in SLE patients

  16. Middle East Respiratory Syndrome Coronavirus Antibody Reactors Among Camels in Dubai, United Arab Emirates, in 2005

    OpenAIRE

    Alexandersen, S; Kobinger, G P; Soule, G; Wernery, U

    2014-01-01

    We tested, using a low starting dilution, sequential serum samples from dromedary camels, sheep and horses collected in Dubai from February/April to October of 2005 and from dromedary camels for export/import testing between Canada and USA in 2000–2001. Using a standard Middle East respiratory syndrome coronavirus (MERS-CoV) neutralization test, serial sera from three sheep and three horses were all negative while sera from 9 of 11 dromedary camels from Dubai were positive for antibodies supp...

  17. THE PRESENCE OF ANTI-PHOSPHATIDYLETHANOLAMINE ANTIBODIES IN ACUTE MYOCARDIAL INFARCTION

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    Abdolreza Sotoodeh Jahromi

    2013-01-01

    Full Text Available Acute Myocardial Infarction (AMI is a clinical manifestation of coronary atherothrombosis and is the important causes of death. Many factors play a role in AMI. Anti-Phospholipid (aPL antibodies may act in the induction of immunological response leading to the development of AMI. Anti-Phosphatidylethanolamine (aPEA antibody has been detected in various autoimmune diseases and anti-phospholipid antibody syndrome. The study of aPEA antibody in AMI might shed light on etiologic mechanisms in the pathogenesis of coronary atherothrombosis and AMI. This study was aimed to evaluate whether prevalence of aPEA antibodies, in patients with AMI and to analyze their relationship with traditional cardiovascular risk factors. The prevalence of aPEA IgG and IgM in a well characterized group of patients with AMI as a case group and in age and sex matched healthy subjects as a control group. Sera from two groups were tested to evaluate the presence of aPEA IgG and IgM isotypes by ELISA method. The frequencies of positive test for aPEA IgG were 12.22 and 2.22% among patients and controls respectively with significant difference (p = 0.007. The aPEA IgM frequencies were 3.33 and 0.00% in patients and the controls, with significant difference (p = 0.005. According to the results of this study, aPEA antibodies have a role in AMI, independent risk factors for AMI, which may represent a link between autoimmunity and coronary atherothrombosis. Further studies with larger sample size of patients and healthy people are needed to explore the role of aPEA antibodies in coronary atherothrombosis.

  18. Kinetics of Severe Acute Respiratory Syndrome (SARS) Coronavirus-Specific Antibodies in 271 Laboratory-Confirmed Cases of SARS

    OpenAIRE

    He, Zhongping; Dong, Qingming; Zhuang, Hui; Song, Shujing; Peng, Guoai; Luo, Guangxiang; Dwyer, Dominic E.

    2004-01-01

    The sensitivities and specificities of an immunofluorescence assay and an enzyme immunoassay for detection of antibodies specific for severe acute respiratory syndrome coronavirus (SARS-CoV) were compared for 148 laboratory-confirmed SARS cases. The appearance and persistence of SARS-CoV-specific antibodies were assessed, with immunoglobulin G detected in 59% of samples collected within 14 days and persisting for 60 to 95 days after the onset of illness.

  19. [Anti-VGKC antibody-associated limbic encephalitis/Morvan syndrome].

    Science.gov (United States)

    Misawa, Tamako; Mizusawa, Hidehiro

    2010-04-01

    Anti-voltage-gated potassium channel antibodies (anti-VGKC-Ab) cause hyperexcitability of the peripheral nerve and central nervous system. Peripheral nerve hyperexcitability is the chief manifestation of Issacs syndrome and cramp-fasciculation syndrome. Morvan syndrome is characterized by neuromyotonia with autonomic and CNS involvement. Manifestations involving the CNS without peripheral involvement are characteristic of limbic encephalitis and epilepsy. The clinical features of anti-VGKC-Ab-associated limbic encephalitis are subacute onset of episodic memory impairment, disorientation and agitation. Hyponatremia is also noted in most patients. Cortico-steroid therapy, plasma exchange and intravenous immunoglobulin are effective in treating to not only the clinical symptoms but also hyponatremia. Unlike other anti-VGKC-Ab-associated neurological disorders, paraneoplastic cases are rare. Thus, anti-VGKC-Ab-associated limbic encephalopathy is considered to be an autoimmune, non-paraneoplastic, potentially treatable encephalitis. Morvan syndrome is characterized by widespread neurological symptoms involving the peripheral nervous system (neuromyotonia), autonomic system (hyperhidrosis, severe constipation, urinary incontinence, and cardiac arrhythmia) and the CNS (severe insomnia, hallucinations, impairment of short-term memory and epilepsy). Many patients have an underlying tumor, for example thymoma, lung cancer, testicular cancer and lymphoma; this indicates the paraneoplastic nature of the disease. Needle electro-myography reveals myokimic discharge. In nerve conduction study, stimulus-induced repetitive descharges are frequently demonstrated in involved muscles. Plasma exchange is an effective treatment approach, and tumor resection also improves symptoms. Both VGKC-Ab-associated limbic encephalitis and Morvan syndrome can be successfully treated. Therefore, when these diseases are suspected, it's important to measure the anti-VGKC-Ab level.

  20. 偏头痛相关性脑卒中发病中抗磷脂抗体的作用%Relationship between antiphospholipid antibodies and migraine related cerebral infarction

    Institute of Scientific and Technical Information of China (English)

    刘昌勤; 解翠红; 孙圣刚

    2004-01-01

    目的:研究和探讨偏头痛与缺血性卒中的相关性以及抗磷脂抗体在偏头痛相关性卒中发病中的作用. 方法:运用问卷调查方式统计普通人群及脑梗死患者偏头痛的患病率,将脑梗死患者按有否偏头痛病史分为两组,用 ELISA方法分别测定其血清抗心磷脂抗体( anticardiolipin antibodies , ACA)水平. 结果:①脑梗死患者偏头痛的患病率与普通人群偏头痛的患病率分别为 20.0 %和 6.0%,二者之间差异有显著性意义(χ2=13.2671,P< 0.01).②脑梗死伴有偏头痛史者和无偏头痛史者,其 ACA阳性率分别为 41.7 %和 19.5 %,二者差异具有显著性意义(χ 2=5.0133,P< 0.05). 结论:偏头痛病史与脑梗死具有明显相关性.抗磷脂抗体可能参与偏头痛所相关的脑梗死的发病机制.%AIM:To study the relationship between migraine and cerebral infarction, and also to explore whether anticardiolipin antibodies(ACA) play a role in the mechanisms of migraine-related cerebral infarction. METHODS:Patients with cerebral infarction and control subjects received a questionnaire that is mainly relative to migraine history.Besides,the patients with cerebral infarction also received sera IgG-ACA detection of enzyme-linked immunoadsordent assay(ELISA). RESULTS:Twenty percent patients had a history of migraine,while only 6.0% of the control subjects had the incidence of migraine.They had statistically significant difference(χ 2=13.2671,P< 0.01) .The patients with cerebral infarction were divided into two groups:patients with migraine history and patients without migraine history.The positive rates of ACA in the stroke patients with migraine history and that of those without migraine history were 41.67% and 19.5% respectively. They also had statistically significant difference(χ 2=5.0133,P< 0.05) . CONCLUSION:The study suggested that migraine history was relative to cerebral infarction and ACA might play an important role in the mechanisms of migraine

  1. Increased expression of Matrix Metalloproteinase 9 in liver from NZB/W F1 mice received antibody against human parvovirus B19 VP1 unique region protein

    Directory of Open Access Journals (Sweden)

    Hsu Gwo-Jong

    2009-01-01

    Full Text Available Abstract Background Human parvovirus B19 infection has been postulated to the anti-phospholipid syndrome (APS in autoimmunity. However, the influence of anti-B19-VP1u antibody in autoimmune diseases is still obscure. Methods To elucidate the effect of anti-B19-VP1u antibodies in systemic lupus erythematosus (SLE, passive transfer of rabbit anti-B19-VP1u IgG was injected intravenously into NZB/W F1 mice. Results Significant reduction of platelet count and prolonged thrombocytopenia time were detected in anti-B19-VP1u IgG group as compared to other groups, whereas significant increases of anti-B19-VP1u, anti-phospholipid (APhL, and anti-double strand DNA (dsDNA antibody binding activity were detected in anti-B19-VP1u group. Additionally, significant increases of matrix metalloproteinase-9 (MMP9 activity and protein expression were detected in B19-VP1u IgG group. Notably, phosphatidylinositol 3-phosphate kinase (PI3K and phosphorylated extracellular signal-regulated kinase (ERK proteins were involved in the induction of MMP9. Conclusion These experimental results firstly demonstrated the aggravated effects of anti-B19-VP1u antibody in disease activity of SLE.

  2. Evaluation of anticardiolipin antibodies in tobacco users and non-tobacco users with severe chronic periodontal disease

    Science.gov (United States)

    Yadalam, Pradeep K.; Rajapandian, K.; Ravishankar, P. L.; Vartharajan, Kalaivani; Subramaniam, Srinath; Dinakar, Mithra

    2016-01-01

    Aims: Many studies have proven that b2-glycoprotein-I-dependent anticardiolipin is elevated in periodontal diseases. Systemic lupus erythematosus and antiphospholipid syndrome, which are usually associated with high antiphospholipid antibodies, are more prone to adverse pregnancy outcomes and cardiovascular sequelae. Therefore, the aim of the present study is to evaluate IgG, IgM anticardiolipin antibodies in tobacco users and non-tobacco users with severe chronic periodontal disease. Materials and Methods: Based on the Armitage classification, 2000, 40 severe periodontitis (group D) (mean clinical attachment loss greater than 2.5 mm) male patients were selected for the study with the age range of 35–65 years and good general health from the Department of periodontics, SRM Kattankulathur Dental College, Chennai. They were classified as smokers (20 subjects) and non-smokers (20 subjects). Blood samples were collected and IgG, IgM antibodies were semi-quantitatively analyzed by enzyme-linked immunosorbent assay. The data thus collected were statistically analyzed by independent student's t-test. Results: Results showed that smokers with severe periodontitis exhibited marked increase in anticardiolipin IgG, IgM compared to non-smokers. They showed a positive correlation and statistical significance (P periodontitis, which indicates that these patients are more prone to coronary heart disease. PMID:27382544

  3. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

    Science.gov (United States)

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome. The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%). In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  4. Clinical, electrophysiological subtypes and antiganglioside antibodies in childhood Guillain-Barré syndrome

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    Meena A Kannan

    2011-01-01

    Full Text Available Background: Guillain-Barré syndrome (GBS has been the most common cause of flaccid paralysis in children after the decline in the incidence of poliomyelitis. There are not any published data from the Indian subcontinent documenting electrophysiological patterns and antiganglioside antibodies in pediatric GBS. Materials and Methods: The study population included children with GBS referred for electrodiagnostic evaluation and also children with GBS admitted to our institute between August 2006 and July 2007. Nerve conduction studies were done to determine GBS subtypes and serum antiganglioside antibodies were measured using enzyme-linked immunosorbent assay (ELISA. Clinical and electrophysiological features were correlated with antiganglioside antibody results. Results: Of the 43 (male to female ratio = 2.1:1 children studied, 97.6% had motor weakness, 76.7% had cranial nerve palsies, 13.9% had autonomic disturbances and respiratory paralysis was found in 9.3% children. Antecedent illness was recorded in 69.8% children. The GBS subtype distribution was as follows: acute inflammatory demyelinating polyradiculoneuropathy (AIDP in 21 (48.8%, acute motor axonal neuropathy (AMAN in 19 (44.2%, and 3 (6.9% children were unclassified. The severity of illness was similar in both AMAN and AIDP subtypes and the recovery in both the subtypes was complete without any significant difference in the duration of recovery. Preceding diarrheal illness was more common in AMAN subtype as compared to AIDP subtype (57.9% vs. 4.7%, P = 0.007. Sensory symptoms were more common in AIDP subtype than in AMAN subtype (66.6% vs. 21%, P = 0.03}. The commonest ganglioside antibody was IgM GM2. Anti GM3 antibodies were exclusively seen in children with AMAN and IgG GD1b was significantly associated with (36.7 vs. 4%; P = 0.007 AMAN subtype. IgG GT1b was identified in 50% of patients with AIDP as compared to 22.7% in patients with AMAN. Conclusion: In this study, AMAN subtype

  5. Onconeuronal and antineuronal antibodies in patients with neoplastic and non-neoplastic pulmonary pathologies and suspected for paraneoplastic neurological syndrome

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    Michalak S

    2009-12-01

    Full Text Available Abstract Objective Onconeuronal antibodies are important diagnostic tool in patients with suspicion of paraneoplastic neurological syndromes (PNS. However, their role in PNS pathophysiology and specificity for particular neurological manifestation remains unclear. The aim of this study was to evaluate onconeuronal and antineuronal antibodies in patients with pulmonary pathologies and suspected for PNS. Materials and methods Twenty one patients with pulmonary pathologies were selected from the database of 525 consecutive patients with suspicion of PNS. Patients' sera were screened for the presence of onconeuronal and antineuronal antibodies by means of indirect immunofluorescence; the presence was confirmed by Western blotting. Clinical data were obtained from medical records, hospital data base, and questionnaire-based direct telephone contact with patients. Results Among 21 patients, aged 54 ± 11, with pulmonary pathologies, the most frequent neurological manifestations were neuropathies. Typical PNS included paraneoplastic cerebellar degeneration (PCD and limbic encephalitis (LE. We found cases with multiple onconeuronal antibodies (anti-Ri and anti-Yo and coexisting PNS (PCD/LE. Well-defined onconeuronal antibodies were identified in 23.8% of patients. Among antineuronal antibodies, the most frequent were anti-MAG (23.8%. ROC curves analysis revealed high sensitivity of onconeuronal and antineuronal antibodies for typical PNS and lower for pulmonary malignancies. Conclusions Tests for antibodies are highly sensitive for the diagnosis of typical paraneoplastic neurological syndromes. Anti-myelin and anti-MAG antibodies are associated with non-neoplastic pulmonary diseases. Patients with well-defined onconeuronal antibodies require careful screening and follow-up, because the PNS diagnosis indicates a high probability of an underlying malignancy.

  6. [Anticardiolipin antibodies in patients with systemic lupus erythematosus].

    Science.gov (United States)

    Petrović, R; Petrović, M; Novicić-Sasić, D; Damjanov, N

    1994-01-01

    The aim of the study was to determine the prevalence and to evaluate clinical significance of anticardiolipin antibodies in cohort of 60 patients with systemic lupus erythematosus. The measurement of autoantibodies was carried out by standardized ELISA method using MELISA anticardiolipin IgG and IgM kits (Walker Diagnostics, Cambridgeshire, UK) A positive result indicated a value in GPL or MPL U/ml more than 3 SD above the mean value obtained with control sera of 48 healthy pearsons. IgG isotype alone, and both isotupe of anticardiolipin antibodies were found in 30 percent, in 6,7 percent and in 11,7 percent of patients, respectively. High or medium levels of IgG anticardiolipin antibodies were found in all 6 patients with actual venous or arterial thrombosis, but in only 3 out of 10 patients with history of thromboembolic features. All 6 patients with actual thrombocytopenia and 3 female with recent spontaneus abortion also had elevated levels of the same isotype. Total anticardiolipin antibodies (IgG and IgM) were significantly associated with recent or history of thrombocytopenia. In conclusion, we emphasize the association of IgG anticardiolipin antibodies with recent events of antiphospholipid syndrome in patients with systemic lupus erythematosus. PMID:18173204

  7. Anti-neutrophil cytoplasmic antibody-associated Pauci-immune crescentic glomerulonephritis complicating Sjögren's syndrome.

    Science.gov (United States)

    Wang, Wei-Jei; Wu, Hau-Shin; Chu, Tzong-Shinn

    2011-07-01

    Sjögren's syndrome is a chronic autoimmune disease, characterized by specific autoimmune antibodies anti-Ro and anti-La, and it can involve multiple organs, such as the kidneys, lungs, muscles, and nervous system. The most common renal complication of Sjögren's syndrome is tubulointerstitial nephritis, and glomerulonephritis is relatively uncommon. We report the case of an 86-year-old man presenting with recurrent fever, poor appetite, decreased salivary secretion, and body weight loss. Laboratory investigation revealed that serum creatinine was 4.2 mg/dL, proteinuria was 3+, and there was microscopic hematuria. Positive perinuclear anti-neutrophil cytoplasmic antibody, anti-Ro, and anti-La antibodies were detected. Renal biopsy showed crescentic glomerulonephritis with scanty immune complex deposition. The patient was diagnosed with primary Sjögren's syndrome complicated with rapidly progressive glomerulonephritis with positive anti-neutrophil cytoplasmic antibody. Unlike the patients of other case reports, our patient's renal function did not recover after immunosuppressant treatment, and he finally received long-term hemodialysis. Pauci-immune glomerulonephritis is a rare renal complication of Sjögren's syndrome, and progress to renal failure in such patients is possible.

  8. Indirect ELISA with Recombinant GP5 for Detecting Antibodies to Porcine Reproductive and Respiratory Syndrome Virus

    Institute of Scientific and Technical Information of China (English)

    Yan Chen; Hong Tian; Jian-Hui He; Jin-Yin Wu; You-jun Shang; Xiang-tao Liu

    2011-01-01

    Porcine reproductive and respiratory syndrome is caused by the PRRS virus(PRRSV), which has six structural proteins(GP2, GP3, GP4, GP5, M and N). GP5 and N protein are important targets for serological detection by enzyme-linked immunosorbent assay(ELISA)and other methods. Toward this goal, we developed an indirect ELISA with recombinant GP5 antigens and this method was validated by comparison to the LSI PRRSV-Ab ELISA kit. The results indicated that the optimal concentration of coated recombinant antigen was 0.2 μg/well for a serum dilution of 1:40. The rate of agreement with the LSI PRRSV-Ab kit was 88.7%(266/300). These results support the potential use of recombinant GP5 as an antigen for indirect ELISA to detect PRRSV antibodies in pigs.

  9. Anti-ganglioside antibodies in patients with Guillain Barré syndrome and other neurological disorders

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    C Vaishnavi

    2013-01-01

    Full Text Available A study was performed on 59 Guillain-Barré syndrome (GBS cases, 58 neurological controls (NC and 60 non-neurological controls (NNC to investigate the association of anti-ganglioside antibodies in GBS and other neurological disorders. Campylobacter jejuni was isolated from 5.7% of GBS patients. Anti-ganglioside immunoglobulin G was present in 82% and immunoglobulin M in 46% in acute inflammatory demyelinating polyneuropathy patients, 70% and 44% respectively in acute motor axonal neuropathy subgroup and 38% each in acute motor sensory axonal neuropathy subgroup. Though high intensity of anti-gangliosides was present in the GBS patients, the NC patients also had adequate anti-gangliosides compared with the NNC group.

  10. Thrombomodulatory Effect of Anti-B2-Glycoprotein I Antibodies on Crystalline Annexin A5 on Phospholipid Bilayers, as Observed by Atomic Force Microscopy

    Science.gov (United States)

    Irman, Špela; Škarabot, Miha; Muševič, Igor; Rozman, Blaž; Božič, Borut

    2011-01-01

    Antibodies against β2-glycoprotein I are a subset of very heterogeneous family of antiphospholipid antibodies. It is well recognised that anti-β2-glycoprotein I antibodies are the main pathogenic players in the autoimmune disease known as antiphospholipid syndrome. Many mechanisms have been proposed through which these autoantibodies could cause microplacental, arterial or venous thrombosis. One of the suggested mechanisms is an antiphospholipid antibody-mediated disruption of annexin A5 protective crystalline shield on negatively charged phospholipid membranes. In current report the study of β2-glycoprotein I, anti-β2-glycoprotein I antibodies and annexin A5 interactions was performed on in vitro model of planar solid-supported phospholipid bilayers and visualized by atomic force microscopy. Planar phospholipid bilayers comprised 30 % L-α-phosphatidylserine and 70 % L-α-phosphatidylcholine. For the study of interactions 10 mg/l annexin A5, 0.15 g/l β2-glycoprotein I, 10 g/l of IgG fraction from healthy blood donor, 10 g/l of IgG fraction from a patient with anti-β2-glycoprotein I antibodies and 0.4 g/l of isolated IgG anti-β2-glycoprotein I antibodies from the same patients in Hepes buffered saline with 1.5 mM Ca2+ were used. We confirmed the clustering of β2-glycoprotein I on planar phospholipid bilayers. We also found that in the presence of annexin A5, β2-glycoprotein I does not bind to planar phospholipid bilayers. However, when adding the anti-β2-glycoprotein I antibodies, the growth of β2-glycoprotein I-anti-β2-glycoprotein I antibodies complexes in the presence of incompletely crystallized annexin A5 on planar phospholipid bilayers was observed. Results confirm the possible thrombomodulatory activity of anti-β2-glycoprotein antibodies through their effect on crystalline annexin A5. In addition, the hypothesis that the presence of possibly pathologic antigen-antibody pair itself is not sufficient to start the pathological process is confirmed

  11. Characterization of polyclonal antibodies against nonstructural protein 9 from the porcine reproductive and respiratory syndrome virus

    Directory of Open Access Journals (Sweden)

    Mengmeng ZHAO,Juanjuan QIAN,Jiexiong XIE,Tiantian CUI,Songling FENG,Guoqiang WANG,Ruining WANG,Guihong ZHANG

    2016-06-01

    Full Text Available Porcine reproductive and respiratory syndrome (PRRS is considered to be one of the most important infectious diseases impacting the swine industry and is characterized by reproductive failure in late term gestation in sows and respiratory disease in pigs of all ages. The nonstructural protein 9 gene, Nsp9, encoding the RNA-dependent RNA polymerase, is generally regarded as fairly conserved when compared to other viral proteins. Antibodies against Nsp9 will be of great importance for the diagnosis and treatment of the causal agent, PRRS virus. A study was undertaken to generate polyclonal antibodies against the immunodominant Nsp9. For this purpose, the Nsp9 was expressed in Escherichia coli and subsequently used as an antigen to immunize New Zealand rabbits. Antiserum was identified via an indirect ELISA, and then verified based on the ability to react with both naturally and artificially expressed Nsp9. Results of virus neutralization test showed that this antiserum could not neutralize the PRRSV. Nevertheless, this antiserum as a diagnostic core reagent should prove invaluable for further investigations into the mechanism of PRRS pathogenesis.

  12. Middle East respiratory syndrome coronavirus antibody reactors among camels in Dubai, United Arab Emirates, in 2005.

    Science.gov (United States)

    Alexandersen, S; Kobinger, G P; Soule, G; Wernery, U

    2014-04-01

    We tested, using a low starting dilution, sequential serum samples from dromedary camels, sheep and horses collected in Dubai from February/April to October of 2005 and from dromedary camels for export/import testing between Canada and USA in 2000-2001. Using a standard Middle East respiratory syndrome coronavirus (MERS-CoV) neutralization test, serial sera from three sheep and three horses were all negative while sera from 9 of 11 dromedary camels from Dubai were positive for antibodies supported by similar results in a MERS-CoV recombinant partial spike protein antibody ELISA. The two negative Dubai camels were both dromedary calves and remained negative over the 5 months studied. The six dromedary samples from USA and Canada were negative in both tests. These results support the recent findings that infection with MERS-CoV or a closely related virus is not a new occurrence in camels in the Middle East. Therefore, interactions of MERS-CoV at the human-animal interface may have been ongoing for several, perhaps many, years and by inference, a widespread pandemic may be less likely unless significant evolution of the virus allow accelerated infection and spread potential in the human population.

  13. Sneddon's syndrome: it is all in the ectoderm.

    Science.gov (United States)

    Rutter-Locher, Zoe; Chen, Zhongbo; Flores, Lorena; Basu, Tanya; Creamer, Daniel; Weeks, Robert; Arya, Roopen; Nashef, Lina

    2016-08-01

    A 51-year-old man gave a 2-year history of worsening mobility, cognitive decline and headaches. He had a history of thromboembolic stroke, recurrent transient ischaemic attacks and a spontaneous intraventricular haemorrhage. On examination, he had livedo reticularis and perniosis and a systolic murmur. Catheter cerebral angiography showed peripheral small-vessel and medium-vessel vasculopathy resulting in pruning of the distal cortical vessels and tortuous irregular distal collaterals. Skin biopsy showed subtle vasculopathy with ectasia of capillaries and postcapillary venules but no frank vasculitis or arterial thrombosis. Repeated serum antiphospholipid antibody titres were negative. The clinical features, skin biopsy and angiogram findings strongly supported a diagnosis of Sneddon's syndrome. Clinicians should consider Sneddon's syndrome in patients with livedo reticularis and stroke. There are treatment dilemmas in this situation when ischaemic and haemorrhagic cerebral events coexist. PMID:26968196

  14. The meaning of anti-Ro and anti-La antibodies in primary Sjögren's syndrome.

    Science.gov (United States)

    Hernández-Molina, Gabriela; Leal-Alegre, Gustavo; Michel-Peregrina, Martha

    2011-01-01

    Anti-SSA/Ro and anti-La/SSB are the hallmark antibodies in primary Sjögren's syndrome (pSS), being present in 60-70%. These antibodies have been associated with an earlier disease onset, glandular dysfunction and extraglandular manifestations as well as with other B cells activation markers. In addition an immunogenetic background is important for the autoantibody formation, having a stronger association with HLA-DR2 and HLA-DR3. Anti-Ro/SSA and anti-La/SSB antibodies are useful in the diagnosis of pSS and help to identify more "active" patients, however their association with response to treatment is unclear. Herein we review the evidence regarding the association of these antibodies with HLA background, demographic, clinical, glandular dysfunction, other serologic features and response to treatment in patients with pSS.

  15. Chorea associated with anti-phospholipid antibodies: case report.

    Science.gov (United States)

    Demonty, J; Gonce, M; Ribai, P; Verellen-Dumoulin, C; Hustinx, R

    2010-01-01

    A seventeen year-old boy developed left sided chorea in a few days, subsequently involving the four limbs. Although he presented a marfanoid phenotype, genetic analysis of the Fibrillin 1 was normal. The genes for familial chorea and Huntington's disease were also negative. Biological tests showed normal serum homocystein, but revealed very high levels of anti-beta2-GP1 IgG, anticardiolipin and lupus anticoagulant, which remained at similar values for a period of over three months. Electroencephalogram and cerebral magnetic resonance imaging (MRI) showed no abnormalities. Brain PET-scan disclosed bilateral striatal hypermetabolism. The patient was treated with methylprednisolone and low dose of acetylsalicylic acid. He improved markedly after six weeks of treatment, and choreic movements disappeared completely after two months. A control PET-scan performed at this time showed reversion of striated hypermetabolism to a normal pattern. The pathogenic aspects of this relatively rare case of chorea are discussed. PMID:21128564

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

    Institute of Scientific and Technical Information of China (English)

    陈李华; 姜玲玲; 厉有名; 彭清璧

    2003-01-01

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

  17. Comparison of a commercial ELISA and an immunoperoxidase monolayer assay to detect antibodies directed against porcine respiratory and reproductive syndrome virus

    NARCIS (Netherlands)

    Nodelijk, G.; Wensvoort, G.; Kroese, B.; Leengoed, van L.A.M.G.; Colijn, E.; Verheijden, J.H.M.

    1996-01-01

    A commercially available enzyme-linked immunosorbent assay (ELISA) for the detection of antibodies against porcine respiratory and reproductive syndrome virus (PRRSV) was compared to an immunoperoxidase monolayer assay (IPMA). Serum samples used were collected from pigs experimentally infected with

  18. Clinical relevance of the determination of anti-GQ1b antibodies in Miller Fisher and Guillain-Barré syndromes.

    Science.gov (United States)

    Goffette, S; Jeanjean, A; Pierret, F; Peeters, A; Sindic, C J

    1998-12-01

    Anti-GQ1b antibodies were assayed by an enzyme-linked immunosorbent assay in sera from patients with non-neurological disorders (N = 20), and with various neurological disorders (N = 59), including nine cases of Miller Fisher syndrome, 16 cases of Guillain-Barré syndrome and one case of acute post-infectious ophthalmoparesis. Such antibodies were found in most cases (8 out of 9) of Miller Fisher syndrome, and at very high titres, in one case of Guillain-Barré syndrome characterised by an initial ophthalmoparesis, and in the case of isolated post-infectious ophthalmoparesis. The latter was characterised by a long-lasting occurrence of these antibodies. Anti-GQ1b antibodies are specific for an immune-mediated neuropathy of the cranial, especially oculomotor, nerves.

  19. Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

    OpenAIRE

    Konstantinov, Konstantin N.; Harris, Alexis A.; Barry, Marc; Murata, Glen H; Tzamaloukas, Antonios H.

    2013-01-01

    A woman diagnosed with mixed connective tissue disease (MCTD) developed an anti-myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA) and nephrotic syndrome with normal serum creatinine. Percutaneous kidney biopsy showed pauci-immune glomerulonephritis with superimposed immune complex deposition. After treatment with cyclophophamide and prednisone, proteinuria decreased progressively to a level of 0.4 g/g creatinine, ANCA became undetectable, while serum creatinine remained normal ...

  20. Application of GP5 protein to develop monoclonal antibody against porcine reproductive and respiratory syndrome virus.

    Science.gov (United States)

    Tian, Hong; Cheng, Yan; Wu, Jin-yang; He, Jian-hui; Shang, You-jun; Liu, Xiang-tao

    2011-08-01

    In this study, a panel of monoclonal antibodies (mAbs) against Porcine reproductive and respiratory syndrome virus(PRRSV), named as 8C9 and4B4, were produced by fusing SP2/0 myeloma cells and spleen cells of BALB/c mice immunized with the PRRSV (TCID(50)=5.5), screened by the indirect ELISA and subjected to several limiting dilutions. mAbs were then identified by biological characterization. Among the two fusion cell strains, 8C9 belonged to the IgG1 subclass and 4B4 belonged to the IgG2a subclass. The titers in cell culture supernatant and abdomen liquor reached to 1:10(4)and 1:10(5), respectively. The specificity test indicated that the two cells had specific reactions for the PRRSV and GP5 protein respectively, and no reaction with Classical swine fever virus (CSFV) or Swine vesicular disease virus (SVDV). The molecular weights of the heavy chain and light chain were about 45.0 kDa and 25.0 kDa, respectively. In neutralization activity tests, the results showed that the prepared mAb 4B4 can protect 50% of cells with no CPE in dilution up to 1:512, but mAB 8C9 has no neutralization activities to PRRSV. PMID:21847758

  1. Application of GP5 Protein to Develop Monoclonal Antibody against Porcine Reproductive and Respiratory Syndrome Virus

    Institute of Scientific and Technical Information of China (English)

    Hong Tian; Yan Cheng; Jin-yang Wu; Jian-hui He; You-jun Shang; Xiang-tao Liu

    2011-01-01

    In this study,a panel of monoclonal antibodies (mAbs) against Porcine reproductive and respiratory syndrome virus(PRRSV),named as 8C9 and4B4,were produced by fusing SP2/0 myeloma cells and spleen cells of BALB/c mice immunized with the PRRSV (TCID50=5.5),screened by the indirect ELISA and subjected to several limiting dilutions.mAbs were then identified by biological characterization.Among the two fusion cell strains,8C9 belonged to the IgG1 subclass and 4B4 belonged to the IgG2a subclass.The titers in cell culture supernatant and abdomen liquor reached to 1:104and 1:105,respectively.The specificity test indicated that the two cells had specific reactions for the PRRSV and GP5 protein respectively,and no reaction with Classical swine fever virus (CSFV) or Swine vesicular disease virus (SVDV).The molecular weights of the heavy chain and light chain were about 45.0 kDa and 25.0 kDa,respectively.In neutralization activity tests,the results showed that the prepared mAb 4B4 can protect 50% of cells with no CPE in dilution up to 1:512,but mAB 8C9 has no neutralization activities to PRRSV.

  2. Histological Features of Antiphospholipid Nephropathy in Patients with Systemic Lupus Erythematosus

    International Nuclear Information System (INIS)

    Objective:To determine the histological features of renal biopsies of Systemic Lupus Erythematosus (SLE) patients with and without antiphospholipid antibodies in Saudi population. Study Design: Cross-sectional, comparative study. Place and Duration of Study: King Khalid University Hospital, Riyadh, Saudi Arabia, from January to December 2013. Methodology: Consecutive SLE patients admitted to King Khalid University Hospital, Riyadh for renal biopsy for evaluation of proteinuria or deterioration of renal function were recruited. SLE patients with renal involvement were divided in two groups. Group one included patients with positive APS antibodies and group two included patients with negative APS antibodies. The histological features of renal biopsies of the two patients groups were compared. Data was analyzed using simple statistical analysis. Results: The mean age of APS antibodies-positive patients was 30.37 ± 10.714 years while mean age of APS negative patients was 33.62 ± 11.717 years (p=0.224). Twenty five (83.33 percentage) patients were females and 5 (16.67 percentage) patients were males in APS positive patients while 42 (89.36 percentage) were females and 5 (10.63 percentage) were males in group two. Acute lesions like thrombotic microangiopathy were in 2 (6.7 percentage) of APS positive patients while chronic lesions like focal cortical atrophy was found in 6 (20 percentage) and fibrous intimal hyperplasia was found in 9 (30 percentage). Other significant histological findings in APS antibodies positive group were glomerular basement membrane wrinkling in 12 (40 percentage), glomerular double wall contour in 17 (56.7 percentage), fibrous adhesions in 11 (36.7 percentage) patients with APS antibodies. Conclusion:Systemic Lupus Erythematosus (SLE) patients with positive APS antibodies has specific histological findings suggesting an important role of APS antibodies in the pathogenesis of APS nephropathy. (author)

  3. 联合检测血清β-HCG、雌二醇、孕酮及抗心磷脂抗体预测先兆流产妊娠结局%Study on combined detection of serum β-HCG, estradiol, progesterone and antiphospholipid antibodies in predicting the outcome of threatened abortion in early pregnancy

    Institute of Scientific and Technical Information of China (English)

    夏书琴; 马宏生; 王维琴; 贾海琴; 刘举珍

    2015-01-01

    Objective To explore the clinical value of combined detection of serum beta human chorionic gonadotropin (β-hCG),estradiol (E2),progesterone (P) and antiphospholipid antibodies (ACA) in predicting the outcomes of early threatened abortion.Methods 80 pregnant women were chosen from six to eight weeks singleton pregnancies,50 cases of threatened abortion and 30 cases of normal pregnancy.The serum levels of β-HCG,estradiol,progesterone and ACA-IgG,ACA-IgM and ACA-IgA were detected.The threatened abortion patients were given intramuscular progesterone 20 mg/day/time for 1 week,then,the pregnancy outcomes were analyzed and the serum levels of β-HCG,estradiol and progesterone were explored.Results The serum β-HCG,estradiol and progesterone of the threatened abortion group [(5 140.77 ± 2 365.47) IU/L,(680.28 ± 306.06) pg/mL,(15.59 ± 3.65) pg/L]were lower than those of the normal pregnant women [(14 520.4 ± 11 016.24) IU/L,(1 142.81 ± 670.57) pg/mL,(27.6 ± 4.78) pg/L] (t =3.224,P =0.003 ; t =2.43,P =0.022 ; t =7.72,P =0.001).According to pregnancy outcome,patients with threatened miscarriages were divided into the two groups,including 45 patients with continuing pregnancies and 5 patients with inevitable miscarriages.The serum levels of β-HCG,estradiol and progesterone in ongoing pregnancies group [(10 829.42 ± 6 926.28) IU/L,(832.9 ± 397.91) pg/mL,(25.62 ± 4.51) pg/L] were higher than those in inevitable miscarriages [(6 310.14 ± 2 593.19) IU/L,(487.14 ± 275.47) pg/mL,(13.6 ±4.84) pg/L] (P < 0.05) ;The serum levels of ACA-IgG,ACA-IgM and ACA-IgA in 80 cases tested,only 2 cases who appeared in the threatened abortion group were detected with the positive of serum ACA-IgG,accounted for 4% (2/50) (x2 =68.05,P =0.000),occurred spontaneous abortion in the end.Conclusion Combined detection of serum ACA,β-HCG,estradiol and progesterone may be helpful for prognosis of threatened abortion.However,the clinical predictive value of ACA needs further study in the

  4. In vivo neutralization assays by monoclonal antibodies against white spot syndrome virus in crayfish (Cambarus proclarkii)

    Institute of Scientific and Technical Information of China (English)

    WANG Yinan; ZHAN Wenbin; XING Jing; JIANG Yousheng

    2008-01-01

    The neutralizing activities of eight monoclonal antibodies (MAbs) against white spot syndrome virus (WSSV) (2D2,2B2,1D2,1DS,1C2,4A1,6A4 and 6B4) were analyzed by in vivo experiments.Gills from WSSV-infected shrimp were homogenized and ten-fold serially diluted by PBS,and then incubated with MAbs (hybridoma culture supernatant),respectively.The mixture of WSSV and MAbs were injected into crayfish (Procambarus clarkii).Mter challenge,the death rates of crayfish were counted to determine the neutralizing activities of MAbs.At the same time,the mixture of myeloma culture supernatant and WSSV or PBS was served as positive or negative control,respectively.The results showed that,at each virus dilution,the mean time to death of the crayfish injected with MAb-treated virus was significantly longer than that in the positive control,though they all showed 100% mortality within 25 d,and meanwhile,few crayfish died in the negative control.Among the eight MAbs,2D2,2B2,1D2 and 1DS,especially the former two,delayed the mortality significantly,and 1C2,4Al and 6A4 delayed the mortality as well but not so efficiently,while MAb 6B4 was efficient only when the virus concentration increased.The results indicated that the anti-WSSV MAbs can neutralize WSSV in different virus dilutions.

  5. Atomic force microscopy-based antibody recognition imaging of proteins in the pathological deposits in Pseudoexfoliation Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Creasey, Rhiannon [School of Chemical and Physical Sciences, Flinders University of SA, GPO Box 2100, Adelaide, SA 5001 (Australia); Sharma, Shiwani [School of Medicine, Ophthalmology, Flinders University of SA, GPO Box 2100, Adelaide, SA 5001 (Australia); Gibson, Christopher T. [School of Chemical and Physical Sciences, Flinders University of SA, GPO Box 2100, Adelaide, SA 5001 (Australia); Craig, Jamie E. [School of Medicine, Ophthalmology, Flinders University of SA, GPO Box 2100, Adelaide, SA 5001 (Australia); Ebner, Andreas [Institute for Biophysics, Johannes Kepler Universitaet Linz, Altenbergerstr. 69, A-4040 Linz (Austria); Becker, Thomas [Nanochemistry Research Institute, Curtin University, GPO Box U1987, Perth, 6845 WA (Australia); Hinterdorfer, Peter [Institute for Biophysics, Johannes Kepler Universitaet Linz, Altenbergerstr. 69, A-4040 Linz (Austria); Voelcker, Nicolas H., E-mail: nico.voelcker@flinders.edu.au [School of Chemical and Physical Sciences, Flinders University of SA, GPO Box 2100, Adelaide, SA 5001 (Australia)

    2011-07-15

    The phenomenon of protein aggregation is of considerable interest to various disciplines, including the field of medicine. A range of disease pathologies are associated with this phenomenon. One of the ocular diseases hallmarked by protein aggregation is the Pseudoexfoliation (PEX) Syndrome. This condition is characterized by the deposition of insoluble proteinaceous material on the anterior human lens capsule. Genomic and proteomic analyses have revealed an association of specific genetic markers and various proteins, respectively, with PEX syndrome. However, the ultrastructure of the protein aggregates is poorly characterized. This study seeks to build capacity to determine the molecular nature of PEX aggregates on human lens capsules in their native state by AFM-based antibody recognition imaging. Lysyl oxidase-Like 1 (LOXL1), a protein identified as a component of PEX aggregates, is detected by an antibody-modified AFM probe. Topographical AFM images and antibody recognition images are obtained using three AFM-based techniques: TREC, phase and force-volume imaging. LOXL1 is found to be present on the lens capsule surface, and is localized around fibrous protein aggregates. Our evaluation shows that TREC imaging is best suited for human tissue imaging and holds significant potential for imaging of human disease tissues in their native state. -- Highlights: {yields} Atomic force microscopy techniques were applied to diseased human tissues. {yields} LOXL1 protein was detected on the small fibers of Pseudoexfoliation deposits. {yields} PicoTREC was the optimum technique for investigating protein aggregates.

  6. Monoclonal antibody panels for acute leukemia and myelodysplastic syndrome diagnosis. Results of a co-operative quality control group.

    Science.gov (United States)

    Basso, G; Bernasconi, P; Chianese, R; Crovetti, G; Garbaccio, G; Iavarone, A; Pautasso, M; Santagostino, A; Stacchini, A

    2001-01-01

    The need for standardization criteria and result reproducibility in immunophenotyping hematological diseases has increased along with their clinical importance. Our group "Policentric Study Group on Immunological Markers", is composed of 40 laboratories. Its aim, over recent years, has been to find a standardized way of immunophenotypic analysis applicable to various hematological diseases. The objective of this study is to contribute to the debate concerning standardization of monoclonal antibody panels and immunophenotypic analysis procedures in acute leukemia (AL) and myelodysplastic syndrome (MDS), with the following targets: to improve interlaboratory reproducibility of the immunophenotyping data, and interpretative results; to study, with improved feasibility, correlation between immunophenotype and clinical or biological findings on a large number of AL and MDS cases; to verify the utility of the proposed monoclonal antibody panels for proper AL and MDS classification, and to detect minimal residual disease. In the field of AL and MDS our experience is based on about 1800 and 700 cases respectively analyzed over the last five years. Starting from these experiences and data of the literature we have elaborated the proposed panels of monoclonal antibodies and the methods of analysis. We have suggested a standardized immunophenotypic approach to study AL and MDS. In particular our work has focused on the gating strategy. This aims at drawing a gate of analysis having high purity and recovery, and on the choice of monoclonal antibody combinations for multiparametric analysis, particularly the normal antigen expression on each step of lineage differentiation or their clinically relevant aberrant expressions. A standardized criteria has become a necessary starting point in any kind of analytical process. In the field of acute leukemias and myelodysplastic syndromes the work of this polycentric group has focused on the pre-analytical and analytical steps to be

  7. No evidence for XMRV nucleic acids, infectious virus or anti-XMRV antibodies in Canadian patients with chronic fatigue syndrome.

    Directory of Open Access Journals (Sweden)

    Imke Steffen

    Full Text Available The gammaretroviruses xenotropic murine leukemia virus (MLV-related virus (XMRV and MLV have been reported to be more prevalent in plasma and peripheral blood mononuclear cells of chronic fatigue syndrome (CFS patients than in healthy controls. Here, we report the complex analysis of whole blood and plasma samples from 58 CFS patients and 57 controls from Canada for the presence of XMRV/MLV nucleic acids, infectious virus, and XMRV/MLV-specific antibodies. Multiple techniques were employed, including nested and qRT-PCR, cell culture, and immunoblotting. We found no evidence of XMRV or MLV in humans and conclude that CFS is not associated with these gammaretroviruses.

  8. Interferon β-1b-neutralizing antibodies 5 years after clinically isolated syndrome

    DEFF Research Database (Denmark)

    Hartung, H-P; Freedman, M S; Polman, C H;

    2011-01-01

    To determine the frequency and consequences of neutralizing antibodies (NAbs) in patients with a first event suggestive of multiple sclerosis (MS) treated with interferon β-1b (IFNβ-1b).......To determine the frequency and consequences of neutralizing antibodies (NAbs) in patients with a first event suggestive of multiple sclerosis (MS) treated with interferon β-1b (IFNβ-1b)....

  9. Effect of oral administration of recombinant human β2 glycoprotein 1 on Treg/Th17 ratio of mice with experimental anti-phospholipid syndrome%口服rhβ2GP1对EAPS小鼠Treg/Th17比率的影响

    Institute of Scientific and Technical Information of China (English)

    徐立; 马静茹; 刘磊; 戴鹤玲; 付嘉; 方艳秋; 谭岩

    2015-01-01

    目的:通过口服低剂量抗原(rhβ2GP1)干预EAPS疾病进展过程中小鼠外周血CD4+ CD25+ Treg和Th17数量和功能变化,探讨EAPS口服耐受机制.方法:ELISA法检测小鼠外周血浆中IL-17、IL-2、IL-6、TGF-β水平变化;计算小鼠流产率(%)、活化部分凝血活酶时间(APTT)和血小板计数(PLT)判断APS病理改变;流式细胞术检测小鼠PBMC中CD4+ CD25+ Treg及Th17细胞变化.结果:与模型组相比,耐受组小鼠IL-17、IL-2、IL-6水平明显降低,APTT缩短,TGF-β、PLT升高,流产率降低,均差异显著(P<0.05);耐受组小鼠PBMC中CD4+ CD25+ Treg和Th17细胞百分率及两者的比率与模型组相比差异显著(P<0.05),而与对照组则无差异(P>0.05).结论:CD4+ CD25+ Treg/Th17在EAPS口服耐受的诱导中发挥重要作用.%Objective:To explore the oral tolerance mechanism of experimental anti-phospholipid syndrome (EAPS) by intervening numerical and functional changes of CD4 + CD25 + regulatory T (Treg) and Th17 in peripheral blood of mice in the course of EAPS progression with oral administration of low-dose recombinant human β2 glycoprotein 1 (rhβ2 GP1).Methods:ELISA was used to detect the changes of interleukin-17 (IL-17),interleukin-2 (IL-2),interleukin-6 (IL-6),and transforming growth factor-β (TGF-β) levels in peripheral plasma of mice; the abortion rate,activated partial thromboplastin time (APTT),and platelet count (PLT) were calculated ; pathological changes of APS were judged ; flow cytometry was applied to detect the changes of CD4 + CD25 + Treg and Th17-cells in peripheral blood mononuclear cells.Results:Compared with model group,the levels of IL-17,IL-2,and IL-6 in tolerance group decreased significantly (P < 0.05),APTT shortened significantly (P < 0.05),TGF-β level and PLT increased significantly (P < 0.05),abortion rate decreased significantly (P < 0.05) ; there were statistically significant differences in the percentages of CD4 + CD25 + Treg and Thl7 cells and

  10. Anticardiolipin antibodies in pathogenesis of infertility

    Directory of Open Access Journals (Sweden)

    Lončar Dragan

    2010-01-01

    Full Text Available Background/Aim. Antiphospholipid syndrome (APS is an autoimmune disorder clinically characterized by arterial or venous thrombosis and/or specific obstetric complications and presence of antiphospholipid antibodies (aPL in the serum. It occurs in 0.3% of pregnant women, while 1% of them have two spontaneous abortions. The aim of this study was to analyze the frequency of biphospholipid antibodies in pregnant women with recurrent spontaneous abortions. Methods. We analyzed 60 pregnant women who had two or more recurrent miscarriages. The control group included 60 healthy pregnant women. We analyzed titres of anticardiolipin (aCL IgG and/or IgM with high titres (> 20 U/mL, lupus anticoagulant (LAC antibodies and anti-beta-2 glycoprotein (b2-GP1 IgG as well as parameters of coagulation status of pregnant women. Results. Analyzing Spearman's rank correlation coefficient in a group of affected patients, we noticed a slightly positive correlation of lupus anticoagulants (LAC with aCL antibodies of both classes, while the correlation with b2GP1 IgG was negative. Both classes of aCL antibodies and antib2GP1 IgG were in a discrete positive correlation with the given variables. In the control group, there was a lack of consistency in correlation of the study variables with LAC-aCl IgG, compared to the affected patients, and there was a standard negative coefficient of correlation with anti-b2GP1 IgG. The correlation ratio of anti-b2GP1 IgG was negative for all studied test parameters. Analysis of hemostatic parameters showed a statistically significant difference in the concentration of fibrinogen (p < 0.01 and thrombocyte count (p < 0.05 between the study and the control group of pregnant women. Lower mean values of fibrinogen (2.90 ± 0.45 g/L and lower thrombocyte count [(179.20 ± 6.00 × 109] were found in the study group of pregnant women with secondary infertility compared to the mean values of fibrinogen (3.60 ± 0.55 g/L and thrombocyte count

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

    Directory of Open Access Journals (Sweden)

    Kistler Thomas

    2011-06-01

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

  12. Dissecting the Role of Anti-ganglioside Antibodies in Guillain-Barré Syndrome: an Animal Model Approach.

    Science.gov (United States)

    Asthana, Pallavi; Vong, Joaquim Si Long; Kumar, Gajendra; Chang, Raymond Chuen-Chung; Zhang, Gang; Sheikh, Kazim A; Ma, Chi Him Eddie

    2016-09-01

    Guillain-Barré syndrome (GBS) is an autoimmune polyneuropathy disease affecting the peripheral nervous system (PNS). Most of the GBS patients experienced neurological symptoms such as paresthesia, weakness, pain, and areflexia. There are also combinations of non-neurological symptoms which include upper respiratory tract infection and diarrhea. One of the major causes of GBS is due largely to the autoantibodies against gangliosides located on the peripheral nerves. Gangliosides are sialic acid-bearing glycosphingolipids consisting of a ceramide lipid anchor with one or more sialic acids attached to a neutral sugar backbone. Molecular mimicry between the outer components of oligosaccharide of gangliosides on nerve membrane and lipo-oligosaccharide of microbes is thought to trigger the autoimmunity. Intra-peritoneal implantation of monoclonal ganglioside antibodies secreting hybridoma into animals induced peripheral neuropathy. Recent studies demonstrated that injection of synthesized anti-ganglioside antibodies raised by hybridoma cells into mice initiates immune response against peripheral nerves, and eventually failure in peripheral nerve regeneration. Accumulating evidences indicate that the conjugation of anti-ganglioside monoclonal antibodies to activating FcγRIII present on the circulating macrophages inhibits axonal regeneration. The activation of RhoA signaling pathways is also involved in neurite outgrowth inhibition. However, the link between these two molecular events remains unresolved and requires further investigation. Development of anti-ganglioside antagonists can serve as targeted therapy for the treatment of GBS and will open a new approach of drug development with maximum efficacy and specificity. PMID:26374552

  13. Human Antibodies Against Middle East Respiratory Syndrome Coronavirus | NCI Technology Transfer Center | TTC

    Science.gov (United States)

    The National Cancer Institute is seeking statements of capability or interest from parties interested in collaborative research to co-develop antibody-based therapeutic against MERS-CoV, including animal studies, cGMP manufacturing, and clinical trials.

  14. Stroke and the antiphospholipid syndrome : consensus meeting Taormina 2002

    NARCIS (Netherlands)

    Brey, RL; Chapman, J; Levine, [No Value; Ruiz-Irastorza, G; Derksen, RHWM; Khamashta, M; Shoenfeld, Y

    2003-01-01

    Ischaemic stroke is the only neurological manifestation accepted as a clinical diagnostic criterion for the antiphospholipidsyndrome (APS). This association is reasonably well established in patients first diagnosed with APS but is less clear in randomly selected stroke patients who test positive on

  15. A new trick for an ancient drug: quinine dissociates antiphospholipid immune complexes.

    Science.gov (United States)

    Bezati, E; Wu, X-X; Quinn, A S; Taatjes, D J; Rand, J H

    2015-01-01

    Quinine, a quinoline derivative, is an ancient antipyretic drug with antimalarial properties that has been phased out by more effective synthetic candidates. In previous studies we discovered that hydroxychloroquine (HCQ), a synthetic antimalarial with structural similarities to quinine, reduced the binding of antiphospholipid (aPL) immune complexes to phospholipid bilayers. We performed ellipsometry and atomic force microscopy (AFM) studies to measure the effect of quinine on dissociation of anti-β2-glycoprotein I (anti-β2GPI) immune complexes. We found that quinine desorbed pre-formed β2GPI-aPL immunoglobulin (Ig)G complexes from phospholipid bilayers at significantly lower molar concentrations than HCQ. Quinine also inhibited the formation of immune complexes with a higher efficacy than HCQ at equivalent drug concentrations of 0.2 mg/ml (0.192 ± 0.025 µg/cm(2) for quinine vs. 0.352 ± 0.014 µg/cm(2) for HCQ, p quinine disintegrated immune complexes bound to planar phospholipid layers. The desorptive and inhibitory effects of the old drug, quinine, toward β2GPI-aPL IgG complexes and β2GPI were significantly more pronounced compared to the synthetic antimalarial, HCQ. The results suggest that the quinoline core of the molecule is a critical domain for this activity and that side chains may further modulate this effect. The results also indicate that there may yet be room for considering new activities of very old drugs in devising clinical trials on potential non-anticoagulant treatments for antiphospholipid syndrome (APS).

  16. A COMPARATIVE STUDY OF ANTI-PHOSPHATIDYL INOSITOLE ANTIBODIES IN PATIENTS WITH MYOCARDIAL INFARCTION AND HEALTHY SUBJECTS

    Directory of Open Access Journals (Sweden)

    Abdolreza Sotoodeh Jahromi

    2014-01-01

    Full Text Available Immune system and inflamation is widely known to play a key role in the development and progression of cardiovascular diseases. Anti-Phospholipid (aPL antibodies may act in the induction of immunological response leading to the development of Acute Myocardial Infarction (AMI. Anti-Phosphatidyl Inositole (PI Antibody (Ab has been seen in various diseases including rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. Although there are a few studies on the association of some autoantibodies with AMI, more epidemiological data are required to confirm their significance as independent risk factors in cardiovascular diseases. Moreover, the data on the relationship of autoantibodies with traditional risk factors of AMI is rare. The study of anti-PI Ab in AMI may lead to understand of etiology of ischemic heart disease. This study was conducted to determine whether prevalence of anti-PI Abs, in patients who had AMI and to analyze their association with traditional cardiovascular risk factors. The prevalence of anti-PI IgG and IgM in a well characterized group of patients with AMI as a case group and in age and sex matched healthy subjects as control group. Sera from the case and the control groups were tested to evaluate the presence of IgG and IgM isotypes to anti-PI by ELISA method. The prevalence of anti-PI IgG and also IgM in the case group resulted significantly higher than in the control group with AMI (p<0.005. The findings of this study suggest that anti-PI Abs seemed to have a role in AMI independent risk factors for AMI and may represent a link between autoimmunity and atherosclerosis in patients with AMI. Comprehensive studies are recommended to explore the exact role of anti-PI Abs in AMI.

  17. Spontaneous coronary artery dissection in the context of positive anticardiolipin antibodies and clinically undiagnosed systemic lupus erythematosus.

    Science.gov (United States)

    Nisar, M K; Mya, T

    2011-11-01

    Spontaneous coronary artery dissection (SCAD) is an extremely uncommon condition that can lead to fatal acute myocardial infarction. There have been very few case reports of SCAD in patients with systemic lupus erythematosus (SLE) and even fewer in association with antiphospholipid antibodies - mainly postpartum. This is the first reported case of SCAD in a patient who was confirmed to have SLE and tested positive for anticardiolipin antibody and lupus anticoagulant. This case demonstrates the importance of carefully considering the differential diagnoses of SCAD at presentation. It also highlights the need for further research to explore the link between SLE, antiphospholipid antibodies and SCAD.

  18. The Hemagglutinin Stem-Binding Monoclonal Antibody VIS410 Controls Influenza Virus-Induced Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Baranovich, Tatiana; Jones, Jeremy C; Russier, Marion; Vogel, Peter; Szretter, Kristy J; Sloan, Susan E; Seiler, Patrick; Trevejo, Jose M; Webby, Richard J; Govorkova, Elena A

    2016-04-01

    Most cases of severe influenza are associated with pulmonary complications, such as acute respiratory distress syndrome (ARDS), and no antiviral drugs of proven value for treating such complications are currently available. The use of monoclonal antibodies targeting the stem of the influenza virus surface hemagglutinin (HA) is a rapidly developing strategy for the control of viruses of multiple HA subtypes. However, the mechanisms of action of these antibodies are not fully understood, and their ability to mitigate severe complications of influenza has been poorly studied. We evaluated the effect of treatment with VIS410, a human monoclonal antibody targeting the HA stem region, on the development of ARDS in BALB/c mice after infection with influenza A(H7N9) viruses. Prophylactic administration of VIS410 resulted in the complete protection of mice against lethal A(H7N9) virus challenge. A single therapeutic dose of VIS410 given 24 h after virus inoculation resulted in dose-dependent protection of up to 100% of mice inoculated with neuraminidase inhibitor-susceptible or -resistant A(H7N9) viruses. Compared to the outcomes in mock-treated controls, a single administration of VIS410 improved viral clearance from the lungs, reduced virus spread in lungs in a dose-dependent manner, resulting in a lower lung injury score, reduced the extent of the alteration in lung vascular permeability and protein accumulation in bronchoalveolar lavage fluid, and improved lung physiologic function. Thus, antibodies targeting the HA stem can reduce the severity of ARDS and show promise as agents for controlling pulmonary complications in influenza. PMID:26787699

  19. Gangrena de pavilhão auricular como primeira manifestação de síndrome do anticorpo antifosfolípide Gangrene of the auricle as the first sign of antiphospholipid antibody syndrome

    OpenAIRE

    Erika Bettini de Sá; Adson da Silva Passos; Mariana Cecconi; Maria Lourdes Peris Barbo; José Eduardo Martinez; Gilberto Santos Novaes

    2011-01-01

    A síndrome do anticorpo antifosfolípide (SAF), mais comum em mulheres, manifesta-se clinicamente como trombose e/ou abortamentos de repetição. Anemia hemolítica autoimune e manifestações neurológicas, cardíacas e cutâneas são comuns. Relata-se o caso de um paciente do gênero masculino cuja manifestação inicial da doença foi gangrena em pavilhão auricular, e o diagnóstico de SAF se deu por meio de biópsia de pele do membro inferior, que mostrava vasculopatia trombótica, sem evidência de vascul...

  20. Incidence of Ascaris suum-specific antibodies in Austrian patients with suspected larva migrans visceralis (VLM) syndrome.

    Science.gov (United States)

    Schneider, Renate; Auer, Herbert

    2016-03-01

    The pig roundworm, Ascaris suum, is commonly found in domestic pigs all over the world. The transmission to humans takes place by ingestion of infective A. suum eggs present in soil because pig manure is widely used as fertilizer. The possible role of A. suum in the human visceral larva migrans (VLM) syndrome has been discussed controversially during past decades, even though various case reports, particularly from Japan document pulmonal, hepatic and even cerebral symptoms caused by migrating A. suum larvae after ingestion of infected row meat (liver) or contaminated vegetables. We examined 4481 sera by A. suum immunoblot (As-IB) and 5301 sera by Toxocara-ELISA from patients with symptoms associated with the VLM syndrome during three consecutive years (2012-2014). The incidence of A. suum-specific antibodies was 13.2 %, the incidence of T. canis specific antibodies 12.9 % and from a part of the As-IB positive sera (n = 417) additional Toxocara serology was performed to demonstrate the specificity of our tests. Only 56 out of the 417 (13.4 %) sera showed antibodies to both helminth species demonstrating that double infections exist. Interestingly the age distribution of the patients showed that 2.8 % of the Ascaris-positive patients were younger than 21 years, while in the Toxocara-positive group 13.4 % were <21 years. These results are in accordance with a Dutch study suspecting different ways of transmission as cause for this interesting age distribution. Due to the fact that large amounts of untreated pig manure are used as fertilizer and that the expulsion of adult A. suum worms causing intestinal ascariosis is extremely rare in Central European countries, the zoonotic potential of A. suum is considerably underestimated. We suggest that the performance of reliable immunoserological tests, in all industrialized countries where pigs are raised and their manure is used as fertilizer, could help to assess the actual potential of A. suum as causative agent of

  1. Middle East respiratory syndrome coronavirus (MERS-CoV) RNA and neutralising antibodies in milk collected according to local customs from dromedary camels, Qatar, April 2014

    NARCIS (Netherlands)

    Reusken, C B; Farag, E A; Jonges, M; Godeke, G J; El-Sayed, A M; Pas, S D; Raj, V S; Mohran, K A; Moussa, H A; Ghobashy, H; Alhajri, F; Ibrahim, A K; Bosch, B J; Pasha, S K; Al-Romaihi, H E; Al-Thani, M; Al-Marri, S A; AlHajri, M M; Haagmans, B L; Koopmans, M P

    2014-01-01

    Antibodies to Middle East respiratory syndrome coronavirus (MERS-CoV) were detected in serum and milk collected according to local customs from 33 camels in Qatar, April 2014. At one location, evidence for active virus shedding in nasal secretions and/or faeces was observed for 7/12 camels; viral RN

  2. Gemcitabine-induced hemolytic uremic syndrome mimicking scleroderma renal crisis presenting with Raynaud's phenomenon, positive antinuclear antibodies and hypertensive emergency.

    Science.gov (United States)

    Yamada, Yuichiro; Suzuki, Keisuke; Nobata, Hironobu; Kawai, Hirohisa; Wakamatsu, Ryo; Miura, Naoto; Banno, Shogo; Imai, Hirokazu

    2014-01-01

    A 58-year-old woman who received gemcitabine for advanced gallbladder cancer developed an impaired renal function, thrombocytopenia, Raynaud's phenomenon, digital ischemic changes, a high antinuclear antibody titer and hypertensive emergency that mimicked a scleroderma renal crisis. A kidney biopsy specimen demonstrated onion-skin lesions in the arterioles and small arteries along with ischemic changes in the glomeruli, compatible with a diagnosis of hypertensive emergency (malignant hypertension). The intravenous administration of a calcium channel blocker, the oral administration of an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker and the transfusion of fresh frozen plasma were effective for treating the thrombocytopenia and progressive kidney dysfunction. Gemcitabine induces hemolytic uremic syndrome with accelerated hypertension and Raynaud's phenomenon, mimicking scleroderma renal crisis.

  3. Novel Camelid Antibody Fragments Targeting Recombinant Nucleoprotein of Araucaria hantavirus: A Prototype for an Early Diagnosis of Hantavirus Pulmonary Syndrome

    Science.gov (United States)

    Pereira, Soraya S.; Moreira-Dill, Leandro S.; Morais, Michelle S. S.; Prado, Nidiane D. R.; Barros, Marcos L.; Koishi, Andrea C.; Mazarrotto, Giovanny A. C. A.; Gonçalves, Giselle M.; Zuliani, Juliana P.; Calderon, Leonardo A.; Soares, Andreimar M.; Pereira da Silva, Luiz H.; Duarte dos Santos, Claudia N.; Fernandes, Carla F. C.; Stabeli, Rodrigo G.

    2014-01-01

    In addition to conventional antibodies, camelids produce immunoglobulins G composed exclusively of heavy chains in which the antigen binding site is formed only by single domains called VHH. Their particular characteristics make VHHs interesting tools for drug-delivery, passive immunotherapy and high-throughput diagnosis. Hantaviruses are rodent-borne viruses of the Bunyaviridae family. Two clinical forms of the infection are known. Hemorrhagic Fever with Renal Syndrome (HFRS) is present in the Old World, while Hantavirus Pulmonary Syndrome (HPS) is found on the American continent. There is no specific treatment for HPS and its diagnosis is carried out by molecular or serological techniques, using mainly monoclonal antibodies or hantavirus nucleoprotein (N) to detect IgM and IgG in patient serum. This study proposes the use of camelid VHHs to develop alternative methods for diagnosing and confirming HPS. Phage display technology was employed to obtain VHHs. After immunizing one Lama glama against the recombinant N protein (prNΔ85) of a Brazilian hantavirus strain, VHH regions were isolated to construct an immune library. VHHs were displayed fused to the M13KO7 phage coat protein III and the selection steps were performed on immobilized prNΔ85. After selection, eighty clones recognized specifically the N protein. These were sequenced, grouped based mainly on the CDRs, and five clones were analyzed by western blot (WB), surface plasmon resonance (SPR) device, and ELISA. Besides the ability to recognize prNΔ85 by WB, all selected clones showed affinity constants in the nanomolar range. Additionaly, the clone KC329705 is able to detect prNΔ85 in solution, as well as the native viral antigen. Findings support the hypothesis that selected VHHs could be a powerful tool in the development of rapid and accurate HPS diagnostic assays, which are essential to provide supportive care to patients and reduce the high mortality rate associated with hantavirus infections. PMID

  4. Novel camelid antibody fragments targeting recombinant nucleoprotein of Araucaria hantavirus: a prototype for an early diagnosis of Hantavirus Pulmonary Syndrome.

    Directory of Open Access Journals (Sweden)

    Soraya S Pereira

    Full Text Available In addition to conventional antibodies, camelids produce immunoglobulins G composed exclusively of heavy chains in which the antigen binding site is formed only by single domains called VHH. Their particular characteristics make VHHs interesting tools for drug-delivery, passive immunotherapy and high-throughput diagnosis. Hantaviruses are rodent-borne viruses of the Bunyaviridae family. Two clinical forms of the infection are known. Hemorrhagic Fever with Renal Syndrome (HFRS is present in the Old World, while Hantavirus Pulmonary Syndrome (HPS is found on the American continent. There is no specific treatment for HPS and its diagnosis is carried out by molecular or serological techniques, using mainly monoclonal antibodies or hantavirus nucleoprotein (N to detect IgM and IgG in patient serum. This study proposes the use of camelid VHHs to develop alternative methods for diagnosing and confirming HPS. Phage display technology was employed to obtain VHHs. After immunizing one Lama glama against the recombinant N protein (prNΔ₈₅ of a Brazilian hantavirus strain, VHH regions were isolated to construct an immune library. VHHs were displayed fused to the M13KO7 phage coat protein III and the selection steps were performed on immobilized prNΔ₈₅. After selection, eighty clones recognized specifically the N protein. These were sequenced, grouped based mainly on the CDRs, and five clones were analyzed by western blot (WB, surface plasmon resonance (SPR device, and ELISA. Besides the ability to recognize prNΔ85 by WB, all selected clones showed affinity constants in the nanomolar range. Additionaly, the clone KC329705 is able to detect prNΔ₈₅ in solution, as well as the native viral antigen. Findings support the hypothesis that selected VHHs could be a powerful tool in the development of rapid and accurate HPS diagnostic assays, which are essential to provide supportive care to patients and reduce the high mortality rate associated with

  5. Meta-analysis of anti-muscarinic receptor type 3 antibodies for the diagnosis of Sjogren syndrome.

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    Chuiwen Deng

    Full Text Available To conduct a meta-analysis to evaluate the diagnostic value of anti-muscarinic receptor type 3 (M3R antibodies in Sjögren syndrome (SS.Two databases, PUBMED and the Cochrane Library, were systematically searched. Approximately 2,000 participants from several studies were included in this research. STATA 11.2 software and Meta-DiSc 1.4 was used to conduct the meta-analysis.Eleven studies were included in the meta-analysis. The pooled DOR was 13.00 (95% CI, 6.00-26.00. The sensitivity was 0.43 (95% CI, 0.28-0.58 and the specificity was 0.95 (95%CI, 0.91-0.97. The LR+ and LR- were 7.90 (95% CI, 4.70-13.40, 0.61 (95% CI, 0.46-0.79, respectively. The AUC was 0.89 (95% CI, 0.86-0.92.The anti-M3R antibody had high specificity but relatively low sensitivity for the diagnosis of SS.

  6. Paraneoplastic Neurological Syndromes associated with anti-Hu antibodies: are T lymphocytes involved or not?

    NARCIS (Netherlands)

    M.T. de Graaf (Marieke)

    2011-01-01

    textabstractIn paraneoplastic neurological syndromes (PNS), ectopic expression of onconeural antigens by the tumor triggers an immune response that not only reacts with the tumor but also with the same antigens expressed in the nervous system. This immune response inhibits tumor growth. However, the

  7. Anti-Lipid IgG Antibodies Are Produced via Germinal Centers in a Murine Model Resembling Human Lupus

    Science.gov (United States)

    Wong-Baeza, Carlos; Reséndiz-Mora, Albany; Donis-Maturano, Luis; Wong-Baeza, Isabel; Zárate-Neira, Luz; Yam-Puc, Juan Carlos; Calderón-Amador, Juana; Medina, Yolanda; Wong, Carlos; Baeza, Isabel; Flores-Romo, Leopoldo

    2016-01-01

    Anti-lipid IgG antibodies are produced in some mycobacterial infections and in certain autoimmune diseases [such as anti-phospholipid syndrome, systemic lupus erythematosus (SLE)]. However, few studies have addressed the B cell responses underlying the production of these immunoglobulins. Anti-lipid IgG antibodies are consistently found in a murine model resembling human lupus induced by chlorpromazine-stabilized non-bilayer phospholipid arrangements (NPA). NPA are transitory lipid associations found in the membranes of most cells; when NPA are stabilized they can become immunogenic and induce specific IgG antibodies, which appear to be involved in the development of the mouse model of lupus. Of note, anti-NPA antibodies are also detected in patients with SLE and leprosy. We used this model of lupus to investigate in vivo the cellular mechanisms that lead to the production of anti-lipid, class-switched IgG antibodies. In this murine lupus model, we found plasma cells (Gr1−, CD19−, CD138+) producing NPA-specific IgGs in the draining lymph nodes, the spleen, and the bone marrow. We also found a significant number of germinal center B cells (IgD−, CD19+, PNA+) specific for NPA in the draining lymph nodes and the spleen, and we identified in situ the presence of NPA in these germinal centers. By contrast, very few NPA-specific, extrafollicular reaction B cells (B220+, Blimp1+) were found. Moreover, when assessing the anti-NPA IgG antibodies produced during the experimental protocol, we found that the affinity of these antibodies progressively increased over time. Altogether, our data indicate that, in this murine model resembling human lupus, B cells produce anti-NPA IgG antibodies mainly via germinal centers. PMID:27746783

  8. A Case of Marine-Lenhart Syndrome with a Negative TSH Receptor Antibody Titer Successfully Treated with a Fixed, Low Dose of I131

    Directory of Open Access Journals (Sweden)

    Masahiro Takei

    2014-01-01

    Full Text Available We herein describe a case of Marine-Lenhart syndrome with a negative TSH receptor antibody titer. A 75-year-old female presented to our hospital with malaise, palpitations, and mild fine tremors. She did not have any signs suggestive of Graves’ ophthalmopathy, including conjunctival injection, periorbital edema, or proptosis. Her laboratory data were negative for thyroid autoantibodies, including anti-thyroid peroxidase antibodies, anti-thyroglobulin antibodies, and anti-TSH receptor antibodies (TRAb. Ultrasonography of the thyroid gland revealed a tumor in the right lobe. The remaining thyroid gland had an inhomogeneous and rough texture with a high color Doppler flow. I123 scintigraphy disclosed a hot nodule in the right thyroid gland corresponding to the tumor detected on ultrasonography, suggesting Plummer disease. Furthermore, there was an increased uptake of radionuclide in the rest of the thyroid gland, despite the suppressed level of TSH and negative titer of TRAb, suggesting underlying Graves’ disease. The present findings suggested a diagnosis of Marine-Lenhart syndrome with a negative TRAb titer. Treatment with 10 mCi of radioiodine was highly effective in treating hyperthyroidism in this case. A negative TSH receptor antibody titer does not necessarily rule out the existence of Graves’ disease in patients with Plummer disease.

  9. Clinical Associations of Biallelic and Monoallelic TNFRSF13B Variants in Italian Primary Antibody Deficiency Syndromes.

    Science.gov (United States)

    Pulvirenti, Federica; Zuntini, Roberta; Milito, Cinzia; Specchia, Fernando; Spadaro, Giuseppe; Danieli, Maria Giovanna; Pession, Andrea; Quinti, Isabella; Ferrari, Simona

    2016-01-01

    We assessed the prevalence of TNFRSF13B mutations and the clinical correlates in an Italian cohort of 189 CVID, 67 IgAD patients, and 330 healthy controls to substantiate the role of TACI genetic testing in diagnostic workup. We found that 11% of CVID and 13% of IgAD carried at least one mutated TNFRSF13B allele. Seven per cent of CVID had monoallelic-mutations and 4% had biallelic-mutations. The frequency of C104R monoallelic-mutations was not higher than that found in healthy controls. Biallelic-mutations were exclusively found in CVID. CVID patients carrying monoallelic-mutations had an increased prevalence of lymphadenopathy, granulomata, and autoimmune cytopenias. CVID carrying biallelic-mutations had a low prevalence of autoimmunity in comparison with TACI wild-type CVID. Moreover, biallelic-mutated CVID had higher frequency of switched memory B-cells and higher IgM and IgA antibodies to polysaccharide antigens than TACI wild-type and monoallelic-mutated CVID. TACI-mutated IgAD patients had only monoallelic-mutations and did not display clinical difference from IgAD wild-type patients. In conclusion, TNFRSF13B genetic screening of antibody deficiencies may allow the identification of mutational patterns. However, as with counseling for risk assessment, geneticists should be aware that the interpretation of genetic testing for TACI mutations is difficult and the potential impact on clinical management is still limited. PMID:27123465

  10. Clinical Associations of Biallelic and Monoallelic TNFRSF13B Variants in Italian Primary Antibody Deficiency Syndromes

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    Federica Pulvirenti

    2016-01-01

    Full Text Available We assessed the prevalence of TNFRSF13B mutations and the clinical correlates in an Italian cohort of 189 CVID, 67 IgAD patients, and 330 healthy controls to substantiate the role of TACI genetic testing in diagnostic workup. We found that 11% of CVID and 13% of IgAD carried at least one mutated TNFRSF13B allele. Seven per cent of CVID had monoallelic-mutations and 4% had biallelic-mutations. The frequency of C104R monoallelic-mutations was not higher than that found in healthy controls. Biallelic-mutations were exclusively found in CVID. CVID patients carrying monoallelic-mutations had an increased prevalence of lymphadenopathy, granulomata, and autoimmune cytopenias. CVID carrying biallelic-mutations had a low prevalence of autoimmunity in comparison with TACI wild-type CVID. Moreover, biallelic-mutated CVID had higher frequency of switched memory B-cells and higher IgM and IgA antibodies to polysaccharide antigens than TACI wild-type and monoallelic-mutated CVID. TACI-mutated IgAD patients had only monoallelic-mutations and did not display clinical difference from IgAD wild-type patients. In conclusion, TNFRSF13B genetic screening of antibody deficiencies may allow the identification of mutational patterns. However, as with counseling for risk assessment, geneticists should be aware that the interpretation of genetic testing for TACI mutations is difficult and the potential impact on clinical management is still limited.

  11. Anti-centromere antibody-seropositive Sjögren's syndrome differs from conventional subgroup in clinical and pathological study

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    Ida Hiroaki

    2010-07-01

    Full Text Available Abstract Background To clarify the clinicopathological characteristics of primary Sjögren's syndrome (pSS with anti-centromere antibody (ACA. Methods Characteristics of 14 patients of pSS with ACA were evaluated. All patients were anti-SS-A/Ro and SS-B/La antibodies negative (ACA+ group without sclerodactyly. The prevalence of Raynaud's phenomenon (RP, titer of IgG and focus score (FS in the minor salivary glands (MSGs were determined. Quantification analysis of Azan Mallory staining was performed to detect collagenous fiber. Forty eight patients in whom ACA was absent were chosen as the conventional (ACA- pSS group. Results Prevalence of ACA+ SS patients was 14 out of 129 (10.85% pSS patients. RP was observed in 61.5% of the patients with ACA. The level of IgG in the ACA+ group was significantly lower than that of the ACA- group (p = 0.018. Statistical difference was also found in the FS of MSGs from the ACA+ group (1.4 ± 1.0 as compared with the ACA- group (2.3 ± 1.6 (p = 0.035. In contrast, the amount of fibrous tissue was much higher in the ACA+ group (65052.2 ± 14520.6 μm2 versus 26251.3 ± 14249.8 μm2 (p = 1.3 × 10-12. Conclusions Low cellular infiltration but with an increase in fibrous tissues may explain the clinical feature of a high prevalence of RP and normal IgG concentration in ACA+ pSS.

  12. Mild leptospirosis with three-year persistence of IgG- and IgM-antibodies, initially manifesting as carpal tunnel syndrome.

    Science.gov (United States)

    Finsterer, Josef; Stöllberger, Claudia; Sehnal, Ernst; Stanek, Gerold

    2005-08-01

    Long-term persistence of IgG- and IgM-antibodies against leptospira after mild leptospirosis has not been reported. In a 45-year-old female pet-shop worker with carpal tunnel syndrome, accompanied by arthralgias, coughing, repeatedly elevated temperature, followed by easy fatigability, personality change, memory and speech disturbance, blurred vision, myalgia and swollen lymph nodes, leptospirosis was diagnosed, based upon history, clinical findings, and serological investigations. After the described symptoms had disappeared following doxycyclin for 2 weeks, IgG- and IgM-antibodies against leptospira remained positive during the next three years. This case illustrates that leptospirosis may start as carpal tunnel syndrome and that the severity of leptospirosis does not seem to be related to the intensity of the humoral immune response against the causative agent. PMID:16038755

  13. DNA vaccine-generated duck polyclonal antibodies as a postexposure prophylactic to prevent hantavirus pulmonary syndrome (HPS.

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    Rebecca Brocato

    Full Text Available Andes virus (ANDV is the predominant cause of hantavirus pulmonary syndrome (HPS in South America and the only hantavirus known to be transmitted person-to-person. There are no vaccines, prophylactics, or therapeutics to prevent or treat this highly pathogenic disease (case-fatality 35-40%. Infection of Syrian hamsters with ANDV results in a disease that closely mimics human HPS in incubation time, symptoms of respiratory distress, and disease pathology. Here, we evaluated the feasibility of two postexposure prophylaxis strategies in the ANDV/hamster lethal disease model. First, we evaluated a natural product, human polyclonal antibody, obtained as fresh frozen plasma (FFP from a HPS survivor. Second, we used DNA vaccine technology to manufacture a polyclonal immunoglobulin-based product that could be purified from the eggs of vaccinated ducks (Anas platyrhynchos. The natural "despeciation" of the duck IgY (i.e., Fc removed results in an immunoglobulin predicted to be minimally reactogenic in humans. Administration of ≥ 5,000 neutralizing antibody units (NAU/kg of FFP-protected hamsters from lethal disease when given up to 8 days after intranasal ANDV challenge. IgY/IgYΔFc antibodies purified from the eggs of DNA-vaccinated ducks effectively neutralized ANDV in vitro as measured by plaque reduction neutralization tests (PRNT. Administration of 12,000 NAU/kg of duck egg-derived IgY/IgYΔFc protected hamsters when administered up to 8 days after intranasal challenge and 5 days after intramuscular challenge. These experiments demonstrate that convalescent FFP shows promise as a postexposure HPS prophylactic. Moreover, these data demonstrate the feasibility of using DNA vaccine technology coupled with the duck/egg system to manufacture a product that could supplement or replace FFP. The DNA vaccine-duck/egg system can be scaled as needed and obviates the necessity of using limited blood products obtained from a small number of HPS survivors. This

  14. Development and application of a blocking enzyme-linked immunosorbent assay (ELISA) to differentiate antibodies against live and inactivated porcine reproductive and respiratory syndrome virus.

    Science.gov (United States)

    Cong, Yanlong; Huang, Zhiqiang; Sun, Yixue; Ran, Wei; Zhu, Lisai; Yang, Guilian; Ding, Xuemei; Yang, Zhanqing; Huang, Xiao; Wang, Chunfeng; Ding, Zhuang

    2013-09-01

    The aim of this study was to establish a method that could differentiate antibodies against live and inactivated vaccines of porcine reproductive and respiratory syndrome virus (PRRSV). A blocking ELISA (b-ELISA) was established using the PRRSV non-structural protein, Nsp9, as the antigen and a monoclonal antibody, 2D6, against the Nsp9 protein as the capture antibody. The test was validated by using 415 clinical sera in the b-ELISA compared to a commercial kit based on the indirect ELISA using the nucleocapsid (N) protein as antigen. Significant differences were observed for the data obtained by the two detection methods. This may be due to the commercial kit detecting antibodies elicited by live and inactivated virus, whereas the b-ELISA only detects antibodies produced by any active viral replication, such as natural infection or live vaccination. Therefore, the b-ELISA in this study is able to distinguish between antibodies against live and inactivated viruses in pigs.

  15. The Goodpasture-like syndrome in mice induced by intravenous injections of anti-type IV collagen and anti-laminin antibody.

    OpenAIRE

    Yaar, M; Foidart, J. M.; Brown, K S; Rennard, S. I.; Martin, G R; Liotta, L.

    1982-01-01

    Laminin and Type IV collagen are both components of basement membrane. Antibodies to these two proteins, when injected into mice, were found to accumulate in all basement membranes examined, but at highest levels in kidney, liver, and spleen. An acute respiratory distress syndrome was noted shortly after injection. A transient segmental proliferative glomerulonephritis was observed both in the heterologous (early) and autologous (late) phase. The glomerular basement membrane of mice injected ...

  16. Characterization and Diagnostic Use of a Monoclonal Antibody for VP28 Envelope Protein of White Spot Syndrome Virus

    Institute of Scientific and Technical Information of China (English)

    Chong-lin Hou; Yu Cao; Rong-hui Xie; Yi-zhen Wang; Hua-hua Du

    2011-01-01

    The gene encoding the VP28 envelope protein of White spot syndrome virus (WSSV)was cloned into expression vector pET-30a and transformed into the Escherichia coli strain BL21.After induction,the recombinant VP28 (rVP28) protein was purified and then used to immunize Balb/c mice for monoclonal antibody (MAb) production.It was observed by immuno-electron microscopy the MAbs specific to rVP28 could recognize native VP28 target epitopes of WSSV and dot-blot analysis was used to detect natural WSSV infection.Competitive PCR showed that the viral level was approximately 104 copies/mg tissue in the dilution of gill homogenate of WSSV-infected crayfish at the detection limit of dot-blot assay.Our results suggest that dot-blot analysis with anti-rVP28 MAb could rapidly and sensitively detect WSSV at the early stages of WSSV infection.

  17. α-Synuclein and anti-α-synuclein antibodies in Parkinson's disease, atypical Parkinson syndromes, REM sleep behavior disorder, and healthy controls.

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    Lynnae M Smith

    Full Text Available α-synuclein is thought to play a key role in Parkinson's disease (PD because it is the major protein in Lewy bodies, and because its gene mutations, duplication, and triplication are associated with early-onset PD. There are conflicting reports as to whether serum and plasma concentrations of α-synuclein and anti-α-synuclein antibodies differ between PD and control subjects. The objectives of this study were to compare the levels of α-synuclein and its antibodies between individuals with typical PD (n=14, atypical Parkinson syndromes (n=11, idiopathic rapid eye movement sleep behavior disorder (n=10, and healthy controls (n=9, to assess the strength of association between these serum proteins, and to determine group sizes needed for a high probability (80% power of detecting statistical significance for 25% or 50% differences between typical PD and control subjects for these measurements. Analysis of log-transformed data found no statistically significant differences between groups for either α-synuclein or its antibodies. The concentrations of these proteins were weakly correlated (Spearman rho=0.16. In subjects with typical PD and atypical Parkinson syndromes, anti-α-synuclein antibody levels above 1.5 µg/ml were detected only in subjects with no more than four years of clinical disease. Power analysis indicated that 236 and 73 samples per group would be required for an 80% probability that 25% and 50% differences, respectively, in mean α-synuclein levels between typical PD and control subjects would be statistically significant; for anti-α-synuclein antibodies, 283 and 87 samples per group would be required. Our findings are consistent with those previous studies which suggested that serum concentrations of α-synuclein and its antibodies are not significantly altered in PD.

  18. Evidence that pentoxifylline reduces anti-CD3 monoclonal antibody-induced cytokine release syndrome.

    Science.gov (United States)

    Alegre, M L; Gastaldello, K; Abramowicz, D; Kinnaert, P; Vereerstraeten, P; De Pauw, L; Vandenabeele, P; Moser, M; Leo, O; Goldman, M

    1991-10-01

    Pretreatment with pentoxifylline (PTX), a methylxanthine known for its beneficial effects on tissue lesions induced by the injection of endotoxin or recombinant cytokines, was shown to decrease the systemic release of tumor necrosis factor and interleukin 2 occurring after the administration of the anti-CD3 monoclonal antibody 145-2C11 in mice. In parallel, PTX attenuated the hypothermia and the rise in blood urea nitrogen observed in this model. The protective effect of PTX on the toxicity of 145-2C11 was confirmed by the reduction of the mortality among D-galactosamine-sensitized animals. The mitigation by PTX of the release of cytokines did not affect the immunosuppression entailed by 145-2C11 as assessed by the unmodified cytotoxic T lymphocytes (CTL) unresponsiveness against alloantigens measured 48 hr after the injection of the mAb. In vitro experiments on human peripheral blood leukocytes indicated that PTX alone or in synergy with methylprednisolone (m-PDS) also inhibited the release of TNF and IL-2 induced by OKT3. Finally, in a preliminary pilot trial conducted in kidney transplant recipients, we observed that pretreatment with PTX (20 mg/kg i.v.) in addition to m-PDS (2 g i.v.) reduced by half the amount of TNF released in the blood stream after the first injection of OKT3, while no further reduction of the low levels of IL-2 was found. PMID:1833865

  19. Avascular osteonecrosis in patients with SLE: relation to corticosteroid therapy and anticardiolipin antibodies.

    Science.gov (United States)

    Migliaresi, S; Picillo, U; Ambrosone, L; Di Palma, G; Mallozzi, M; Tesone, E R; Tirri, G

    1994-02-01

    Sixty-nine unselected SLE patients were studied to evaluate the prevalence of avascular osteonecrosis (AVN) and its relationship with steroid therapy and with anticardiolipin antibodies (aCL). All the patients were under treatment with corticosteroids. AVN occurred in seven occurred in seven of the 69 patients (10.14%) and was not related to corticosteroid intake. Seventeen of the 69 patients were also treated with methylprednisolone pulse therapy (MPPT) and cumulated the highest corticosteroid doses but none of them suffered from AVN. Excluding the 17 MPPT-treated SLE patients, corticosteroid intake was significantly higher in the AVN-SLE patients. Abnormal IgG and/or IgM aCL serum levels were found in two of the seven AVN-SLE patients and in 24 of the 62 non-AVN SLE, without a statistically significant difference. None of the seven AVN-SLE patients showed features of antiphospholipid syndrome. We conclude that in SLE patients a continuous high-dose steroid treatment may be considered a risk factor for AVN. On the contrary, MPPT regimen may reduce this risk. Anticardiolipin antibodies might represent an added factor which could play a role in some patients but not in all. PMID:8025584

  20. [VGKC-complex antibodies].

    Science.gov (United States)

    Watanabe, Osamu

    2013-04-01

    Various antibodies are associated with voltage-gated potassium channels (VGKCs). Representative antibodies to VGKCs were first identified by radioimmunoassays using radioisotope-labeled alpha-dendrotoxin-VGKCs solubilized from rabbit brain. These antibodies were detected only in a proportion of patients with acquired neuromyotonia (Isaacs' syndrome). VGKC antibodies were also detected in patients with Morvan's syndrome and in those with a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC" antibodies are mainly directed toward associated proteins (for example LGI-1 and CASPR-2) that complex with the VGKCs themselves. The "VGKC" antibodies are now commonly known as VGKC-complex antibodies. In general, LGI-1 antibodies are most commonly detected in patients with limbic encephalitis with syndrome of inappropriate secretion of antidiuretic hormone. CASPR-2 antibodies are present in the majority of patients with Morvan's syndrome. These patients develop combinations of CNS symptoms, autonomic dysfunction, and peripheral nerve hyperexcitability. Furthermore, VGKC-complex antibodies are tightly associated with chronic idiopathic pain. Hyperexcitability of nociceptive pathways has also been implicated. These antibodies may be detected in sera of some patients with neurodegenerative diseases (for example, amyotrophic lateral sclerosis and Creutzfeldt-Jakob disease).

  1. An antibody to the GM1/GalNAc-GD1a complex correlates with development of pure motor Guillain-Barré syndrome with reversible conduction failure.

    Science.gov (United States)

    Ogawa, Go; Kaida, Ken-ichi; Kuwahara, Motoi; Kimura, Fumihiko; Kamakura, Keiko; Kusunoki, Susumu

    2013-01-15

    Antibodies to a ganglioside complex consisting of GM1 and GalNAc-GD1a (GM1/GalNAc-GD1a) are found in sera from patients with Guillain-Barré syndrome (GBS). To elucidate the clinical significance of anti-GM1/GalNAc-GD1a antibodies in GBS, clinical features of 58 GBS patients with IgG anti-GM1/GalNAc-GD1a antibodies confirmed by enzyme-linked immunosorbent assay and thin layer chromatography immunostaining were analyzed. Compared to GBS patients without anti-GM1/GalNAc-GD1a antibodies, anti-GM1/GalNAc-GD1a-positive patients more frequently had a preceding respiratory infection (n=38, 66%, pGalNAc-GD1a-positive patients for whom electrophysiological data were available, 14 had conduction blocks (CBs) at intermediate segments of motor nerves, which were not followed by evident remyelination. Eight of 10 bedridden cases were able to walk independently within one month after the nadir. These results show that the presence of anti-GM1/GalNAc-GD1a antibodies correlated with pure motor GBS characterized by antecedent respiratory infection, fewer cranial nerve deficits, and CBs at intermediate sites of motor nerves. The CB may be generated through alteration of the regulatory function of sodium channels in the nodal axolemma.

  2. Comparative Study of ELISA and IIFT in Detecting Chlamydia Pneumoniae Specific IgG Antibodies in Patients of Acute Coronary Syndrome (ACS

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    Bhuva S P, Bhuva P J, Javdekar T B, Jain M R, Mulla S A

    2012-01-01

    Full Text Available Background: Chlamydia pnaumoniae is reported to be associated with coronary heart disease (CHD. Efficacy of available serologic tests for detecting C.pneumoniae antibodies has been debated. The aim of present study was to compare Enzyme Linked Immunosorbant Assay (ELISA and Indirect Immunofluorescent Test (IIFT for detecting specific antichlamydial antibodies in patients of Acute Coronary Syndrome (ACS. Materials and Methods: Serum samples from 100 patients of ACS and 90 healthy controls were tested for the presence of Chlamydial IgG antibodies using ELISA and IIFT. To assess agreement between ELISA and IIFT, we used ‘nominal scale variables”. Agreement analysis was done using sensitivity, specificity, positive and negative predictive values (PPV and NPV and diagnostic accuracy of the test. Results: The ELISA and IIFT detected C.pneumoniae IgG antibodies in 66% and 48% respectively in patients of ACS, and 29% and 20% respectively of healthy controls. In patients of ACS, sensitivity and specificity of ELISA as compared to IIFT were 70.8% and 38.4% respectively. The PPV of ELISA for C.pneumoniae was 51.5% and NPV was 58.8%. The diagnostic accuracy of ELISA for C.pneumoniae was 54.6%. The two tests correlated in 54% of samples with a moderate agreement of =0.51. Conclusions: The results of present study indicate that ELISA test was inferior to IIFT in detecting C.pneumoniae antibodies in patients of ACS.

  3. Annexin V and anti-Annexin V antibodies: two interesting aspects in acute myocardial infarction

    Directory of Open Access Journals (Sweden)

    Kholoosi Ensieh

    2009-07-01

    Full Text Available Abstract Background Myocardial infarction is the combined result of environmental factors and personal predispositions. Prothrombotic factors might play an important role in this phenomenon. Annexin V (ANV is a calcium-dependent glycoprotein widely present in various tissues exerting a potent anticoagulant effect in vitro by reducing plaque adhesion and aggregation. Anti-annexin V antibodies (aANVAs are detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of ANV in Acute Myocardial Infarction (AMI might shed light on hypercoagulability mechanisms in the pathogenesis of acute coronary syndromes. This study was conducted to investigate the association of plasma ANV, aANVAs and anti-cardiolipin antibodies (aCLAs with AMI. Methods This study recruited 45 patients with the diagnosis of AMI according to WHO criteria in their first 24 hours of admission. 36 matched individuals were studied as the control group with normal coronary artery angiography. Plasma levels of ANV, aANVAs and aCLAs were determined by enzyme-linked immunosorbent assay and the results were compared. Results Plasma ANV levels in the patients with AMI on admission were significantly lower than those in the control group (p = 0.002. Positive test for aANVAs were found to be present in a significant number of our patients (p = 0.004. The studied groups were similar in their rate of patients with positive aCLAs tests. ANV, aANVAs and aCLAs were not correlated with hypertension, diabetes mellitus, hyperlipidemia, sex, age and smoking. Conclusion Our findings suggest that low plasma ANV levels along with positive aANVAs tests in patients with AMI are indicative of hypercoagulable state that is not related to the traditional cardiovascular risk factors.

  4. An ORF2 protein-based ELISA for porcine circovirus type 2 antibodies in post-weaning multisystemic wasting syndrome.

    Science.gov (United States)

    Blanchard, Ph; Mahé, D; Cariolet, R; Truong, C; Le Dimna, M; Arnauld, C; Rose, N; Eveno, E; Albina, E; Madec, F; Jestin, A

    2003-07-17

    Porcine circovirus type 2 (PCV2) plays a crucial role in the pathogenesis of post-weaning multisystemic wasting syndrome (PMWS) in swine. As PCV2 displays significant homology with PCV1 (a non-pathogenic virus) at the nucleotide and amino-acid level, a discriminative antigen is needed for specific serological diagnosis. The ORF2-encoded capsid protein from PCV2 was used to develop an indirect enzyme-linked immunosorbent assay (ELISA). GST-fused capsid protein from PCV2 and GST alone (both expressed in recombinant baculovirus-infected cells) were used as antigens for serodiagnosis. The specificity of the ELISA for detection of PCV2 antibodies was demonstrated in sera from pigs experimentally infected with PCV1, PCV2 and other swine viruses. The semi-quantitative nature of the test was evaluated versus an immunoperoxidase monolayer assay (IPMA). The ELISA was performed on 322 sera from pigs in eight Brittany herds and compared with IPMA. The sensitivity (98.2%) and specificity (94.5%) of this test were considered suitable for individual serological detection. High PCV2 seroprevalence was found in sows and pigs at the end of the growth phase (18-19 weeks) in all eight herds. The seroprevalence in piglets (11-17 weeks) was statistically correlated with clinical symptoms of PMWS (93% in affected versus 54%, in non-affected farms). A cohort study performed in PMWS-free farms showed that 57% of piglets exhibited active seroconversion after 13 weeks, indicating that PCV2 infection occurred earlier in PMWS-affected piglets. PMID:12814886

  5. Younger patients with primary Sjögren's syndrome are more likely to have salivary IgG anti-muscarinic acetylcholine receptor type 3 antibodies.

    Science.gov (United States)

    Jayakanthan, K; Ramya, J; Mandal, Santosh Kumar; Sandhya, P; Gowri, M; Danda, Debashish

    2016-03-01

    Acetylcholine type 3 receptor (M3R) is recognized as an autoantigen in primary Sjögren's syndrome (pSS). Assay of anti-M3R antibody levels in serum is fraught with low sensitivity for diagnosis of pSS. Salivary assay is more likely to improve the diagnostic accuracy. Patients with pSS classified either by the American European Consensus Group (AECG) or American college of Rheumatology (ACR) criteria, attending rheumatology clinic between October 2014 and July 2015 were included. Hospital staff and lupus patients constituted healthy and disease controls, respectively. Evaluation of pSS included clinical evaluation, laboratory tests, ESSDAI and ESSPRI scoring. Unstimulated saliva was collected by the spitting method. Salivary IgG antibody against M3R (anti-M3R) was quantified by indirect ELISA. In this study, 43 patients with pSS, 34 with lupus and 42 healthy controls were recruited. The frequency of anti-M3R antibody levels was 55.81, 17.64 and 7 % for pSS, lupus and healthy controls, respectively. Area under the Receiver Operator Characteristic was 0.7791 (95 % CI,, 0.67-0.87). Sensitivity and specificity of the assay for diagnosis of pSS were 44.19 and 88.16 %, respectively. Salivary anti-M3R IgG antibody positivity was associated with lower age, shorter disease duration and higher globulin levels in our cohort. Salivary anti-M3R IgG antibody assay has high specificity in pSS; younger patients and those with hyperglobulinemia more frequently tested positive for this antibody. PMID:26809799

  6. Genetic Syndromes, Maternal Diseases and Antenatal Factors Associated with Autism Spectrum Disorders (ASD)

    Science.gov (United States)

    Ornoy, Asher; Weinstein- Fudim, Liza; Ergaz, Zivanit

    2016-01-01

    Autism spectrum disorder (ASD) affecting about 1% of all children is associated, in addition to complex genetic factors, with a variety of prenatal, perinatal, and postnatal etiologies. In addition, ASD is often an important clinical presentation of some well-known genetic syndromes in human. We discuss these syndromes as well as the role of the more important prenatal factors affecting the fetus throughout pregnancy which may also be associated with ASD. Among the genetic disorders we find Fragile X, Rett syndrome, tuberous sclerosis, Timothy syndrome, Phelan–McDermid syndrome, Hamartoma tumor syndrome, Prader-Willi and Angelman syndromes, and a few others. Among the maternal diseases in pregnancy associated with ASD are diabetes mellitus (PGDM and/or GDM), some maternal autoimmune diseases like antiphospholipid syndrome (APLS) with anti-β2GP1 IgG antibodies and thyroid disease with anti-thyroid peroxidase (TPO) antibodies, preeclampsia and some other autoimmune diseases with IgG antibodies that might affect fetal brain development. Other related factors are maternal infections (rubella and CMV with fetal brain injuries, and possibly Influenza with fever), prolonged fever and maternal inflammation, especially with changes in a variety of inflammatory cytokines and antibodies that cross the placenta and affect the fetal brain. Among the drugs are valproic acid, thalidomide, misoprostol, and possibly SSRIs. β2-adrenergic receptor agonists and paracetamol have also lately been associated with increased rate of ASD but the data is too preliminary and inconclusive. Associations were also described with ethanol, cocaine, and possibly heavy metals, heavy smoking, and folic acid deficiency. Recent studies show that heavy exposure to pesticides and air pollution, especially particulate matter < 2.5 and 10 μm in diameter (PM2.5 and PM10) during pregnancy is also associated with ASD. Finally, we have to remember that many of the associations mentioned in this review are

  7. Genetic Syndromes, Maternal Diseases and Antenatal Factors Associated with Autism Spectrum Disorders (ASD)

    Science.gov (United States)

    Ornoy, Asher; Weinstein- Fudim, Liza; Ergaz, Zivanit

    2016-01-01

    Autism spectrum disorder (ASD) affecting about 1% of all children is associated, in addition to complex genetic factors, with a variety of prenatal, perinatal, and postnatal etiologies. In addition, ASD is often an important clinical presentation of some well-known genetic syndromes in human. We discuss these syndromes as well as the role of the more important prenatal factors affecting the fetus throughout pregnancy which may also be associated with ASD. Among the genetic disorders we find Fragile X, Rett syndrome, tuberous sclerosis, Timothy syndrome, Phelan–McDermid syndrome, Hamartoma tumor syndrome, Prader-Willi and Angelman syndromes, and a few others. Among the maternal diseases in pregnancy associated with ASD are diabetes mellitus (PGDM and/or GDM), some maternal autoimmune diseases like antiphospholipid syndrome (APLS) with anti-β2GP1 IgG antibodies and thyroid disease with anti-thyroid peroxidase (TPO) antibodies, preeclampsia and some other autoimmune diseases with IgG antibodies that might affect fetal brain development. Other related factors are maternal infections (rubella and CMV with fetal brain injuries, and possibly Influenza with fever), prolonged fever and maternal inflammation, especially with changes in a variety of inflammatory cytokines and antibodies that cross the placenta and affect the fetal brain. Among the drugs are valproic acid, thalidomide, misoprostol, and possibly SSRIs. β2-adrenergic receptor agonists and paracetamol have also lately been associated with increased rate of ASD but the data is too preliminary and inconclusive. Associations were also described with ethanol, cocaine, and possibly heavy metals, heavy smoking, and folic acid deficiency. Recent studies show that heavy exposure to pesticides and air pollution, especially particulate matter < 2.5 and 10 μm in diameter (PM2.5 and PM10) during pregnancy is also associated with ASD. Finally, we have to remember that many of the associations mentioned in this review are

  8. Genetic Syndromes, Maternal Diseases and Antenatal Factors Associated with Autism Spectrum Disorders (ASD).

    Science.gov (United States)

    Ornoy, Asher; Weinstein-Fudim, Liza; Ergaz, Zivanit

    2016-01-01

    Autism spectrum disorder (ASD) affecting about 1% of all children is associated, in addition to complex genetic factors, with a variety of prenatal, perinatal, and postnatal etiologies. In addition, ASD is often an important clinical presentation of some well-known genetic syndromes in human. We discuss these syndromes as well as the role of the more important prenatal factors affecting the fetus throughout pregnancy which may also be associated with ASD. Among the genetic disorders we find Fragile X, Rett syndrome, tuberous sclerosis, Timothy syndrome, Phelan-McDermid syndrome, Hamartoma tumor syndrome, Prader-Willi and Angelman syndromes, and a few others. Among the maternal diseases in pregnancy associated with ASD are diabetes mellitus (PGDM and/or GDM), some maternal autoimmune diseases like antiphospholipid syndrome (APLS) with anti-β2GP1 IgG antibodies and thyroid disease with anti-thyroid peroxidase (TPO) antibodies, preeclampsia and some other autoimmune diseases with IgG antibodies that might affect fetal brain development. Other related factors are maternal infections (rubella and CMV with fetal brain injuries, and possibly Influenza with fever), prolonged fever and maternal inflammation, especially with changes in a variety of inflammatory cytokines and antibodies that cross the placenta and affect the fetal brain. Among the drugs are valproic acid, thalidomide, misoprostol, and possibly SSRIs. β2-adrenergic receptor agonists and paracetamol have also lately been associated with increased rate of ASD but the data is too preliminary and inconclusive. Associations were also described with ethanol, cocaine, and possibly heavy metals, heavy smoking, and folic acid deficiency. Recent studies show that heavy exposure to pesticides and air pollution, especially particulate matter PM2.5 and PM10) during pregnancy is also associated with ASD. Finally, we have to remember that many of the associations mentioned in this review are only partially proven, and not

  9. Genetic Syndromes, Maternal Diseases and Antenatal Factors Associated with Autism Spectrum Disorders (ASD).

    Science.gov (United States)

    Ornoy, Asher; Weinstein-Fudim, Liza; Ergaz, Zivanit

    2016-01-01

    Autism spectrum disorder (ASD) affecting about 1% of all children is associated, in addition to complex genetic factors, with a variety of prenatal, perinatal, and postnatal etiologies. In addition, ASD is often an important clinical presentation of some well-known genetic syndromes in human. We discuss these syndromes as well as the role of the more important prenatal factors affecting the fetus throughout pregnancy which may also be associated with ASD. Among the genetic disorders we find Fragile X, Rett syndrome, tuberous sclerosis, Timothy syndrome, Phelan-McDermid syndrome, Hamartoma tumor syndrome, Prader-Willi and Angelman syndromes, and a few others. Among the maternal diseases in pregnancy associated with ASD are diabetes mellitus (PGDM and/or GDM), some maternal autoimmune diseases like antiphospholipid syndrome (APLS) with anti-β2GP1 IgG antibodies and thyroid disease with anti-thyroid peroxidase (TPO) antibodies, preeclampsia and some other autoimmune diseases with IgG antibodies that might affect fetal brain development. Other related factors are maternal infections (rubella and CMV with fetal brain injuries, and possibly Influenza with fever), prolonged fever and maternal inflammation, especially with changes in a variety of inflammatory cytokines and antibodies that cross the placenta and affect the fetal brain. Among the drugs are valproic acid, thalidomide, misoprostol, and possibly SSRIs. β2-adrenergic receptor agonists and paracetamol have also lately been associated with increased rate of ASD but the data is too preliminary and inconclusive. Associations were also described with ethanol, cocaine, and possibly heavy metals, heavy smoking, and folic acid deficiency. Recent studies show that heavy exposure to pesticides and air pollution, especially particulate matter < 2.5 and 10 μm in diameter (PM2.5 and PM10) during pregnancy is also associated with ASD. Finally, we have to remember that many of the associations mentioned in this review are

  10. Imaging of bronchial carcinoid tumors associated to Cushing syndrome with 111In-Octreoscan scintigraphy and immunoscintigraphy with anti-chromogranin monoclonal antibodies. Report of two cases.

    Science.gov (United States)

    Carretta, A; Chiesa, G; Magnani, P; Songini, C; Melloni, G; Zannini, P; Grossi, A

    1997-04-01

    Bronchial carcinoid tumors are neuroendocrine neoplasms capable of expressing somatostatin receptors and of secreting neuromediators such as ACTH and chromogranins. Radiologic appearance is usually non-specific and has to be distinguished from benign pulmonary nodules and other malignant diseases. Standard radiological techniques have limited accuracy in the evaluation of such lesions. Radioisotopic imaging techniques may increase the specificity of diagnostic assessment. The role of immunoscintigraphy with anti-chromogranin A and B monoclonal antibodies (MoAbs) and of 111In-Octreoscan scintigraphy is evaluated in two cases of bronchial carcinoid tumors associated to Cushing syndrome. PMID:9201136

  11. The SSB-positive/SSA-negative antibody profile is not associated with key phenotypic features of Sjögren's syndrome

    DEFF Research Database (Denmark)

    Baer, Alan N; McAdams DeMarco, Mara; Shiboski, Stephen C;

    2015-01-01

    established SS, we compared anti-SSA/anti-SSB reactivity profiles against concurrent phenotypic features. We fitted logistic regression models to explore the association between anti-SSA/anti-SSB reactivity profile and each key SS phenotypic feature, controlling for potential confounders. RESULTS: Among 3297...... participants, 2061 (63%) had negative anti-SSA/anti-SSB, 1162 (35%) had anti-SSA with or without anti-SSB, and 74 (2%) anti-SSB alone. Key SS phenotypic features were more prevalent and had measures indicative of greater disease activity in those participants with anti-SSA, either alone or with anti-SSB, than......OBJECTIVE: To determine whether the Sjögren's syndrome B (SSB)-positive/Sjögren's syndrome A (SSA)-negative antibody profile is associated with key phenotypic features of SS. METHODS: Among registrants in the Sjögren's International Collaborative Clinical Alliance (SICCA) with possible or...

  12. Association of Anti-GT1a Antibodies with an Outbreak of Guillain-Barre Syndrome and Analysis of Ganglioside Mimicry in an Associated Campylobacter jejuni Strain.

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    Maojun Zhang

    Full Text Available An outbreak of Guillain-Barré syndrome (GBS, subsequent to Campylobacter jejuni enteritis, occurred in China in 2007. Serum anti-ganglioside antibodies were measured in GBS patients and controls. Genome sequencing was used to determine the phylogenetic relationship among three C. jejuni strains from a patient with GBS (ICDCCJ07001, a patient with gastroenteritis (ICDCCJ07002 and a healthy carrier (ICDCCJ07004, which were all associated with the outbreak. The ganglioside-like structures of the lipo-oligosaccharides of these strains were determined by mass spectrometry. Seventeen (53% of the GBS patients had anti-GT1a IgG antibodies. GT1a mimicry was found in the lipo-oligosaccharides of strain ICDCCJ07002 and ICDCCJ07004; but a combination of GM3/GD3 mimics was observed in ICDCCJ07001, although this patient had anti-GT1a IgG antibodies. A single-base deletion in a glycosyltransferase gene caused the absence of GT1a mimicry in ICDCCJ07001. The phylogenetic tree showed that ICDCCJ07002 and ICDCCJ07004 were genetically closer to each other than to ICDCCJ07001. C. jejuni, bearing a GT1a-like lipo-oligosaccharide, might have caused the GBS outbreak and the loss of GT1a mimicry may have helped ICDCCJ07001 to survive in the host.

  13. A novel cell-based assay for inhibitory anti-muscarinic type 3 receptor antibodies in primary Sjögren's syndrome.

    Science.gov (United States)

    Bastian, Isabell; Gordon, Tom P; Jackson, Michael W

    2015-12-01

    Inhibitory autoantibodies acting at the muscarinic acetylcholine receptor type 3 (M3R) are postulated to mediate autonomic dysfunction, including decreased salivary and lacrimal gland output and extra-glandular manifestations, in patients with primary Sjögren's syndrome. However, the contention that anti-M3R antibodies are pathogenic in patients remains untested, due to a lack of assays both sophisticated enough to detect inhibitory anti-M3R antibodies yet suitable for screening large patient cohorts. In the current study, we have established a cell-based bioassay of M3R activity, based on dual transfection of the M3R and a luciferase reporter gene. The bioassay is capable of capturing real-time agonist-mediated signalling of the M3R, which is inhibited specifically by patient IgG that have previously been demonstrated to have anti-M3R activity. The assay can be run in multi-well culture plates, and analysed using simple luminescence readers. As such, the new bioassay incorporating M3R-mediated luciferase transduction is the first assay adaptable to common diagnostic platforms that is capable of determining the presence in patient serum of functionally active anti-M3R autoantibodies. The new bioassay should prove useful for large cohort screening studies aiming to correlate the presence in patients of inhibitory anti-M3R antibodies with symptoms of both glandular and extra-glandular autonomic dysfunction. PMID:26584897

  14. 孕妇抗磷脂抗体、胰岛素样生长因子-I与胎儿生长受限的相关性研究%Correlation study of pregnant women's antiphospholipid antibody and insulin-like growth factor I and fetal growth restriction

    Institute of Scientific and Technical Information of China (English)

    班正贺; 黎丽嫦; 翁碧艳

    2014-01-01

    Objective To investigate the relationship between the pregnant women's expression levels of anti-cardiolipin antibody (ACA) and placental tissue insulin-like growth factor-1 (IGF-I)and their role in fetal growth restriction (FGR). Methods The enzyme-linked immunosorbent assay (ELISA) was used to detect the serum ACA-IgG and IgM of 63 pregnant women with normal full-term pregnancy (control group)and 63 patients with fetal growth restriction(study group);The immunohistochemical method and multimedia color pathological image analysis technique were used to measure the IGF-I expression level in placenta tissues. Results The positive rate of ACA in the study group was 19.0% and that in the control group was 3.2%,with significant difference between the two groups(P 0.05),but were significantly different in placenta weight and fetal body weight (P < 0.05). Conclusion Positive serum ACA or decreased IGF-I expression levels in placenta tissues of the pregnant women is one of the risk factors of FGR.Whether ACA can decrease the IGF-I expression level in placenta tissues needs further study.%目的:探讨胎儿生长受限(FGR)孕妇抗心磷脂抗体(ACA)与胎盘组织胰岛素样生长因子-I(IGF-I)表达水平间的关系及两者在FGR发生中的作用。方法采用酶联免疫吸附法(ELISA法)测定63例正常足月妊娠孕妇(对照组)及63例胎儿生长受限孕妇(研究组)血清中ACA-IgG、IgM;利用免疫组织化学方法及多媒体彩色病理图像分析技术测定胎盘组织IGF-I表达水平。结果研究组抗心磷脂抗体阳性率为19.0%,对照组为3.2%,两组比较,有显著性差异(P<0.05);研究组胎盘组织IGF-1表达水平明显低于对照组(P<0.05);ACA阳性组与ACA阴性组两组比较,胎盘组织IGF-I表达水平无显著差异(P>0.05),胎盘重量、胎儿体重有显著差异(P<0.05)。结论孕妇血清ACA阳性或胎盘组织IGF-I表达水平下降是导致FGR

  15. Screening for celiac disease in Down's syndrome patients revealed cases of subtotal villous atrophy without typical for celiac disease HLA-DQ and tissue transglutaminase antibodies

    Institute of Scientific and Technical Information of China (English)

    Oivi Uibo; Kaupo Teesalu; Kaja Metsküla; Tiia Reimand; Riste Saat; Tarvo Sillat; Koit Reimand; Tiina Talvik; Raivo Uibo

    2006-01-01

    AIM: To investigate the prevalence of celiac disease (CD) as well as CD marker antibodies and susceptibility HLA-DQ haplotypes in 134 karyotyped Down's syndrome (DS) patients.METHODS: Immunoglobulin A (IgA) and G (IgG)type anti-gliadin antibodies (AGA), IgA type anti-tissue transglutaminase (tTG) antibodies (anti-tTG) with antigen of guinea pig and human source were determined by enzyme-linked immunosorbent assay and endomysium antibodies (EMA) by indirect immunofluoresence test.HLA-DQA1*0501/DQB1*0201 (DQ2) was revealed by polymerase chain reaction. Celiac disease was diagnosed by revised ESPGHAN criteria.RESULTS: 41% of DS patients had AGA, 6.0% IgAanti-tTG with guinea pig antigen, and 3.0 % IgA EMA (all positive for anti-tTG with human tTG). Subtotal villous atrophy was found in 5 out of 9 DS patients who had agreed to small bowel biopsy. One of them had DQA1*0501/DQB1*0201 and anti-tTG and EMA i.e. typical for CD markers (this case also fulfilled the ESPGHAN diagnostic criteria), but other four lacked these markers. Three non-biopsied DS patients had also most probably CD because DQA1*0501/DQB1*0201 and IgA anti-tTG (EMA) were detected. Thus, the prevalence of CD among our DS patients population is 3.0 % (95 %of confidence interval [CI]: 0.1-5.9 %).CONCLUSION: We confirm the increased frequency of CD among DS patients. In addition, we have revealed a subgroup of patients with subtotal villous atrophy but without characteristic for CD immunological and genetic markers. Whether these cases represent CD (with atypical immunopathogenesis) or some other immune enteropathy, requires further investigations.

  16. [A case of anti-aquaporin 4 antibody-positive Sjögren syndrome associated with a relapsed myelitis in pregnancy].

    Science.gov (United States)

    Tsugawa, Jun; Tsuboi, Yoshio; Inoue, Hirosato; Baba, Yasuhiko; Yamada, Tatsuo

    2010-01-01

    It is known that pregnancy influences the relapsing rate of multiple sclerosis (MS); however, interaction between pregnancy and relapse of neuromyelitis optica (NMO), a distinct disease from MS, remains unclear. A 34-year-old woman who 1 year previously had clinical history of Sjögren syndrome complicated by myelitis with the presence of anti-AQP4 antibody in her serum, although there was no optic neuritis involvement, was neurologically normal at time of becoming pregnant. In the 22nd week of her pregnancy, however, she developed abdominal belt-shaped numbness and sensory impairment followed by weakness of bilateral lower limb leading to difficulty of her gait. MR imaging revealed hyperintense lesions within the spinal cord extending from C2 to T2 vertebral level with marked spinal cord swelling, indicating relapse of myelitis associated with anti-AQP4 antibody. She was treated with intravenous corticosteroid with marked benefits for her neurological status; she was able to walk without assistance after the treatment. However, in the 30th week she relapsed with myelitis at T2 to T9 vertebral level on MR imaging. Intravenous steroid administration again elicited improvement. She delivered a baby via Caesarean section at 34 weeks of pregnancy. After delivery, she started taking oral corticosteroid as preventive therapy for further relapse of myelitis; thus far she has had no relapse at 7 months of follow-up. There are few reports regarding the influence of pregnancy on anti-AQP4 antibody-positive myelitis. Although further investigation should be done to clarify the difference of immunological changes during pregnancy between NMO and conventional MS, our case together with previous reports indicate increased risk of relapse during pregnancy in NMO. It is necessary to remain vigilant against possible risk of relapse during pregnancy in patients with NMO and/or positive anti-AQP4 antibody. Intravenous steroid administration seems safe and effective against relapse of

  17. Evaluation by indirect immunofluorescent assay and enzyme linked immunosorbent assay of the dynamic changes of serum antibody responses against severe acute respiratory syndrome coronavirus

    Institute of Scientific and Technical Information of China (English)

    MO Hong-ying; XU Jun; REN Xiao-lan; ZENG Guang-qiao; TAN Ya-xia; CHEN Rong-chang; Moira Chan-Yeung; ZHONG Nan-shan

    2005-01-01

    Background Severe acute respiratory syndrome coronavirus (SARS-CoV) is a newly emerging virus that gives rise to SARS patients with high rates of infectivity and fatality. To study the humoral immune responses to SARS-CoV, the authors evaluated IgG and IgM specific antibodies in patients' sera.Methods Two methods, enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescent assay (IFA), were used to detect specific serum IgG and IgM against SARS-CoV in 98 SARS patients and 250 controls consisting of patients with pneumonia, health-care professionals and healthy subjects. The serum antibody profiles were investigated at different times over one and a half years in 18 of the SARS patients. Results The sensitivity and specificity of ELISA for detecting IgG against SARS-CoV were 100.0% and 97.2% and for IgM 89.8% and 97.6% respectively; the figures using IFA for IgG were 100.0% and 100.0% and for IgM 81.8% and 100.0% respectively. During the first seven days of the antibodies trace test, no IgG and IgM were detected, but on day 15, IgG response increased dramatically, reaching a peak on day 60, remaining high up to day 180 and decreasing gradually until day 540. On day 15, IgM was detected, rapidly reached a peak, then declined gradually until day 180 when IgM was undetectable. Conclusion The detection of antibodies against SARS virus is helpful in the clinical diagnosis of SARS.

  18. Functional effects of anticardiolipin antibodies.

    Science.gov (United States)

    Harris, E N; Pierangeli, S S

    1996-10-01

    The 'lupus anticoagulant' phenomenon is the best documented functional effect of antiphospholipid (aPL) antibodies, occurring either by inhibition of the prothrombinase and/or Factor X activation reactions. Understanding the mechanism by which aPL antibodies inhibit phospholipid dependent coagulation reactions may yield important clues about their 'thrombogenic effects' in vivo. We conducted a series of studies to determine the specificity, diversity, and mechanism by which aPL antibodies inhibit phospholipid dependent reactions. Results showed that purified immunoglobulins with lupus anticoagulant and anti-cardiolipin activities were absorbed by negatively charged phospholipids and both activities were recovered from the phospholipid-antibody precipitate. Purified aPL antibodies inhibited the prothrombinase reaction in a plasma free system in which beta 2-glycoprotein 1 (beta 2-GP1) was absent. Affinity purified aPL antibodies had 25-50 times the inhibitory activity of immunoglobulin preparations. The phospholipid binding proteins, beta 2-GPI and placental anticoagulant protein I (PAP I), independently inhibited the prothrombinase reaction, and when these proteins were combined with aPL, inhibition of the prothrombinase reaction was additive. Antibodies of syphilis had no inhibitory effect, partially accounted for by lack of specificity for phosphotidylserine (PS). Although aPL antibodies inhibited the protein C activation reaction, there was no correlation of these activities with inhibition of the prothrombinase reaction. Together, these results show that aPL exert their effects by interaction with negatively charged phospholipids, in particular phosphotidylserine, but lack of correlation between inhibition of the prothrombinase and protein C activation reactions, suggests that the nature of the coagulation protein is also important. PMID:8902763

  19. Cross-reacting antibacterial auto-antibodies are produced within coronary atherosclerotic plaques of acute coronary syndrome patients.

    Directory of Open Access Journals (Sweden)

    Filippo Canducci

    Full Text Available Coronary atherosclerosis, the main condition predisposing to acute myocardial infarction, has an inflammatory component caused by stimuli that are yet unknown. We molecularly investigated the nature of the immune response within human coronary lesion in four coronary plaques obtained by endoluminal atherectomy from four patients. We constructed phage-display libraries containing the IgG1/kappa antibody fragments produced by B-lymphocytes present in each plaque. By immunoaffinity, we selected from these libraries a monoclonal antibody, arbitrarily named Fab7816, able to react both with coronary and carotid atherosclerotic tissue samples. We also demonstrated by confocal microscopy that this monoclonal antibody recognized human transgelin type 1, a cytoskeleton protein involved in atherogenesis, and that it co-localized with fibrocyte-like cells transgelin+, CD68+, CD45+ in human sections of coronary and carotid plaques. In vitro fibrocytes obtained by differentiating CD14+ cells isolated from peripheral blood mononuclear cells also interacted with Fab7816, thus supporting the hypothesis of a specific recognition of fibrocytes into the atherosclerotic lesions. Interestingly, the same antibody, cross-reacted with the outer membrane proteins of Proteus mirabilis and Klebsiella pneumoniae (and possibly with homologous proteins of other enterobacteriaceae present in the microbiota. From all the other three libraries, we were able to clone, by immunoaffinity selection, human monoclonal antibodies cross-reacting with bacterial outer membrane proteins and with transgelin. These findings demonstrated that in human atherosclerotic plaques a local cross-reactive immune response takes place.

  20. Evaluation of a blocking ELISA for screening of antibodies against porcine reproductive and respiratory syndrome (PRRS) virus

    DEFF Research Database (Denmark)

    Sørensen, K.J.; Bøtner, Anette; Madsen, E.S.;

    1997-01-01

    A blocking Elisa was developed for the detection of antibodies against PRRS virus with a view to satisfying the need for examination of blood samples on a large scale. The test was evaluated in comparison with an indirect Elisa and the immunoperoxidase monolayer assay. The blocking Elisa...... was sensitive and specific. It had a higher capacity and was cheaper to perform than the immunoperoxidase monolayer assay and the indirect Elisa. It was comparable to the immunoperoxidase monolayer assay and better than the indirect Elisa in detecting antibodies formed early after infection, and it was superior...... to both the immunoperoxidase monolayer assay and the indirect Elisa in detecting antibodies at a late stage of infection....

  1. Altered Traffic of Cardiolipin during Apoptosis: Exposure on the Cell Surface as a Trigger for “Antiphospholipid Antibodies”

    Directory of Open Access Journals (Sweden)

    Valeria Manganelli

    2015-01-01

    Full Text Available Apoptosis has been reported to induce changes in the remodelling of membrane lipids; after death receptor engagement, specific changes of lipid composition occur not only at the plasma membrane, but also in intracellular membranes. This paper focuses on one important aspect of apoptotic changes in cellular lipids, namely, the redistribution of the mitochondria-specific phospholipid, cardiolipin (CL. CL predominantly resides in the inner mitochondrial membrane, even if the rapid remodelling of its acyl chains and the subsequent degradation occur in other membrane organelles. After death receptor stimulation, CL appears to concentrate into mitochondrial “raft-like” microdomains at contact sites between inner and outer mitochondrial membranes, leading to local oligomerization of proapoptotic proteins, including Bid. Clustering of Bid in CL-enriched contacts sites is interconnected with pathways of CL remodelling that intersect membrane traffic routes dependent upon actin. In addition, CL association with cytoskeleton protein vimentin was observed. Such novel association also indicated that CL molecules may be expressed at the cell surface following apoptotic stimuli. This observation adds a novel implication of biomedical relevance. The association of CL with vimentin at the cell surface may represent a “new” target antigen in the context of the apoptotic origin of anti-vimentin/CL autoantibodies in Antiphospholipid Syndrome.

  2. Monitoring porcine reproductive and respiratory syndrome virus infection status in swine herds based on analysis of antibodies in meat juice samples

    DEFF Research Database (Denmark)

    Mortensen, Sten; Strandbygaard, Bertel; Bøtner, Anette;

    2001-01-01

    An indirect ELISA test was developed as a novel tool aimed at monitoring the herd infection status of swine herds. Meat juice samples from pig carcasses were analysed for the presence of antibodies against porcine reproductive and respiratory syndrome virus (PRRSV). A study of samples from herds...... with known PRRS status was undertaken. The PRRS status of the herds was evaluated based on the analysis of blood samples by another serological test (blocking ELISA) capable of differentiating between infection with PRRSV of the American type and European type. The specificity of the indirect ELISA test...... and herd level criteria for assessing the PRRS status of herds by the new test were developed. Herds were classified as PRRS negative or PRRS seropositive based on 10 meat juice samples collected randomly at slaughter throughout a 3-month-period. Herd PRRS status classification by the indirect ELISA...

  3. Comparison of antibody assays for detection of autoantibodies to Ro 52, Ro 60 and La associated with primary Sjögren's syndrome.

    Science.gov (United States)

    Trier, Nicole Hartwig; Nielsen, Inger Ødum; Friis, Tina; Houen, Gunnar; Theander, Elke

    2016-06-01

    Anti-Ro(52/60) and anti-La constitute the hallmark autoantibodies in primary Sjögren's syndrome, being present in 40-70% of sera. Several anti-Ro/La assays exist, but antibody detection appears to be assay-specific, thus the aim of this study was to compare several anti-Ro/La assays. In total, 96 sera from individuals with primary Sjögren's syndrome and 114 healthy controls were tested for anti-Ro 52/60 and anti-La in 17 immunoassays. Especially the immunoassays used for detection of anti-Ro 52 differed in their sensitivity (48-79%), while only small differences in sensitivities were observed for the anti-Ro 60 (69-77%) anti-La (39-44%) assays. Concordances of 65%, 79% and 73% for the anti-Ro 52, anti-Ro 60 and anti-La assays were found, respectively. The majority of the assays yielded high specificities, primarily ranging from 97 to 100%, except from a single anti-Ro 60 assay, which yielded a specificity of 79%. Occasionally, reactivity levels were increased in a few assays, indicating that false-positive results can be obtained when applying assays of reduced specificity. In general, the commercial assays appeared to perform better than the in-house analyses. When correcting the in-house assays for background reactivity, sensitivities were reduced by approximately 7%, 17%, and 19% for anti-Ro 52, anti-Ro 60 and anti-La assays, respectively, illustrating the pitfalls when applying immunoassays for detection of autoantibodies, which in theory may apply to commercial assays as well. Finally, increased total sensitivities were obtained when combining assays. These studies contribute to clarify the clinical utility of immunoassays for detection of autoantibodies of Ro 52, Ro 60 and La and illustrate that the most efficient strategy to maximize antibody sensitivity is to combine several assays.

  4. Immune Dysfunction in Rett Syndrome Patients Revealed by High Levels of Serum Anti-N(Glc IgM Antibody Fraction

    Directory of Open Access Journals (Sweden)

    Anna Maria Papini

    2014-01-01

    Full Text Available Rett syndrome (RTT, a neurodevelopmental disorder affecting exclusively (99% female infants, is associated with loss-of-function mutations in the gene encoding methyl-CpG binding protein 2 (MECP2 and, more rarely, cyclin-dependent kinase-like 5 (CDKL5 and forkhead box protein G1 (FOXG1. In this study, we aimed to evaluate the function of the immune system by measuring serum immunoglobulins (IgG and IgM in RTT patients (n=53 and, by comparison, in age-matched children affected by non-RTT pervasive developmental disorders (non-RTT PDD (n=82 and healthy age-matched controls (n=29. To determine immunoglobulins we used both a conventional agglutination assay and a novel ELISA based on antibody recognition by a surrogate antigen probe, CSF114(Glc, a synthetic N-glucosylated peptide. Both assays provided evidence for an increase in IgM titer, but not in IgG, in RTT patients relative to both healthy controls and non-RTT PDD patients. The significant difference in IgM titers between RTT patients and healthy subjects in the CSF114(Glc assay (P=0.001 suggests that this procedure specifically detects a fraction of IgM antibodies likely to be relevant for the RTT disease. These findings offer a new insight into the mechanism underlying the Rett disease as they unveil the possible involvement of the immune system in this pathology.

  5. Cocaine-associated retiform purpura: a C5b-9-mediated microangiopathy syndrome associated with enhanced apoptosis and high levels of intercellular adhesion molecule-1 expression.

    Science.gov (United States)

    Magro, Cynthia M; Wang, Xuan

    2013-10-01

    Cocaine-associated retiform purpura is a recently described entity characterized by striking hemorrhagic necrosis involving areas of skin associated with administration of cocaine. Levamisole, an adulterant in cocaine, has been suggested as the main culprit pathogenetically. Four cases of cocaine-associated retiform purpura were encountered in the dermatopathology practice of C. M. Magro. The light microscopic findings were correlated with immunohistochemical and immunofluorescence studies. All 4 cases showed a very striking thrombotic diathesis associated with intravascular macrophage accumulation. Necrotizing vasculitis was noted in 1 case. Striking intercellular adhesion molecule-1 (ICAM-1)/CD54 expression in vessel wall along with endothelial expression of caspase 3 and extensive vascular C5b-9 deposition was observed in all biopsies examined. Cocaine-induced retiform purpura is a C5b-9-mediated microvascular injury associated with enhanced apoptosis and prominent vascular expression of ICAM-1, all of which have been shown in prior in vitro and in vivo murine models to be a direct effect of cocaine metabolic products. Antineutrophilic cytoplasmic antibody and antiphospholipid antibodies are likely the direct sequelae of the proapoptotic microenvironment. The inflammatory vasculitic lesion could reflect the downstream end point reflective of enhanced ICAM-1 expression and the development of antineutrophilic cytoplasmic antibody. Levamisole likely works synergistically with cocaine in the propagation of this syndromic complex. PMID:23392134

  6. Antibody response to Shiga toxins in Argentinean children with enteropathic hemolytic uremic syndrome at acute and long-term follow-up periods.

    Directory of Open Access Journals (Sweden)

    Romina J Fernández-Brando

    Full Text Available Shiga toxin (Stx-producing Escherichia coli (STEC infection is associated with a broad spectrum of clinical manifestations that include diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome (HUS. Systemic Stx toxemia is considered to be central to the genesis of HUS. Distinct methods have been used to evaluate anti-Stx response for immunodiagnostic or epidemiological analysis of HUS cases. The development of enzyme-linked immunosorbent assay (ELISA and western blot (WB assay to detect the presence of specific antibodies to Stx has introduced important advantages for serodiagnosis of HUS. However, application of these methods for seroepidemiological studies in Argentina has been limited. The aim of this work was to develop an ELISA to detect antibodies against the B subunit of Stx2, and a WB to evaluate antibodies against both subunits of Stx2 and Stx1, in order to analyze the pertinence and effectiveness of these techniques in the Argentinean population. We studied 72 normal healthy children (NHC and 105 HUS patients of the urban pediatric population from the surrounding area of Buenos Aires city. Using the WB method we detected 67% of plasma from NHC reactive for Stx2, but only 8% for Stx1. These results are in agreement with the broad circulation of Stx2-expressing STEC in Argentina and the endemic behavior of HUS in this country. Moreover, the simultaneous evaluation by the two methods allowed us to differentiate acute HUS patients from NHC with a great specificity and accuracy, in order to confirm the HUS etiology when pathogenic bacteria were not isolated from stools.

  7. Antibody response to Shiga toxins in Argentinean children with enteropathic hemolytic uremic syndrome at acute and long-term follow-up periods.

    Science.gov (United States)

    Fernández-Brando, Romina J; Bentancor, Leticia V; Mejías, María Pilar; Ramos, María Victoria; Exeni, Andrea; Exeni, Claudia; Laso, María del Carmen; Exeni, Ramón; Isturiz, Martín A; Palermo, Marina S

    2011-04-29

    Shiga toxin (Stx)-producing Escherichia coli (STEC) infection is associated with a broad spectrum of clinical manifestations that include diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome (HUS). Systemic Stx toxemia is considered to be central to the genesis of HUS. Distinct methods have been used to evaluate anti-Stx response for immunodiagnostic or epidemiological analysis of HUS cases. The development of enzyme-linked immunosorbent assay (ELISA) and western blot (WB) assay to detect the presence of specific antibodies to Stx has introduced important advantages for serodiagnosis of HUS. However, application of these methods for seroepidemiological studies in Argentina has been limited. The aim of this work was to develop an ELISA to detect antibodies against the B subunit of Stx2, and a WB to evaluate antibodies against both subunits of Stx2 and Stx1, in order to analyze the pertinence and effectiveness of these techniques in the Argentinean population. We studied 72 normal healthy children (NHC) and 105 HUS patients of the urban pediatric population from the surrounding area of Buenos Aires city. Using the WB method we detected 67% of plasma from NHC reactive for Stx2, but only 8% for Stx1. These results are in agreement with the broad circulation of Stx2-expressing STEC in Argentina and the endemic behavior of HUS in this country. Moreover, the simultaneous evaluation by the two methods allowed us to differentiate acute HUS patients from NHC with a great specificity and accuracy, in order to confirm the HUS etiology when pathogenic bacteria were not isolated from stools.

  8. Presence of Middle East respiratory syndrome coronavirus antibodies in Saudi Arabia : a nationwide, cross-sectional, serological study

    NARCIS (Netherlands)

    Müller, Marcel A; Meyer, Benjamin; Corman, Victor M; Al-Masri, Malak; Turkestani, Abdulhafeez; Ritz, Daniel; Sieberg, Andrea; Aldabbagh, Souhaib; Bosch, Berend-J; Lattwein, Erik; Alhakeem, Raafat F; Assiri, Abdullah M; Albarrak, Ali M; Al-Shangiti, Ali M; Al-Tawfiq, Jaffar A; Wikramaratna, Paul; Alrabeeah, Abdullah A; Drosten, Christian; Memish, Ziad A

    2015-01-01

    BACKGROUND: Scientific evidence suggests that dromedary camels are the intermediary host for the Middle East respiratory syndrome coronavirus (MERS-CoV). However, the actual number of infections in people who have had contact with camels is unknown and most index patients cannot recall any such cont

  9. Middle east respiratory syndrome coronavirus spike protein delivered by modified vaccinia virus ankara efficiently induces virus-neutralizing antibodies

    NARCIS (Netherlands)

    F. Song (Fei); R. Fux (Robert); L.B.V. Provacia (Lisette); A. Volz (Asisa); M. Eickmann; S. Becker (Stephan); A.D.M.E. Osterhaus (Albert); B.L. Haagmans (Bart); G. Suttera (Gerd)

    2013-01-01

    textabstractMiddle East respiratory syndrome coronavirus (MERS-CoV) has recently emerged as a causative agent of severe respiratory disease in humans. Here, we constructed recombinant modified vaccinia virus Ankara (MVA) expressing full-length MERS-CoV spike (S) protein (MVA-MERS-S). The genetic sta

  10. Anti-Aquaporin-4 Antibody-Positive Neuromyelitis Optica Presenting with Syndrome of Inappropriate Antidiuretic Hormone Secretion as an Initial Manifestation

    Directory of Open Access Journals (Sweden)

    H. Nakajima

    2011-10-01

    Full Text Available The distribution of neuromyelitis optica (NMO-characteristic brain lesions corresponds to sites of high aquaporin-4 (AQP4 expression, and the brainstem and hypothalamus lesions that express high levels of AQP4 protein are relatively characteristic of NMO. The syndrome of inappropriate antidiuretic hormone secretion (SIADH is one of the important causes of hyponatremia and results from an abnormal production or sustained secretion of antidiuretic hormone (ADH. SIADH has been associated with many clinical states or syndromes, and the hypothalamic-neurohypophyseal system regulates the feedback control system for ADH secretion. We report the case of a 63-year-old man with NMO, whose initial manifestation was hyponatremia caused by SIADH. Retrospective analysis revealed that the serum anti-AQP4 antibody was positive, and an MRI scan showed a unilateral lesion in the hypothalamus. SIADH recovered completely with regression of the hypothalamic lesion. As such, NMO should even be considered in patients who develop SIADH and have no optic nerve or spinal cord lesions but have MRI-documented hypothalamic lesions.

  11. Pathogenic effects of maternal antinuclear antibodies during pregnancy in women with lupus

    OpenAIRE

    Rafael Herrera-Esparza; Juan José Bollain-y-Goytia; Esperanza Avalos-Díaz

    2014-01-01

    Lupus is an autoimmune disease that primarily affects young women of childbearing age. Fertility rates in lupus patients depend on various factors, including disease activity, nephritis, and the presence of antiphospholipid antibodies; however, after lupus patients become pregnant, different factors may affect the course of pregnancy, such as the production of autoantibodies, pre-existing renal disease, and eclampsia, among others. The placenta is a temporary hemochorial organ that prevents i...

  12. Presence of Mycoplasma fermentans in the bloodstream of Mexican patients with rheumatoid arthritis and IgM and IgG antibodies against whole microorganism

    Directory of Open Access Journals (Sweden)

    Salinas Salvador

    2009-08-01

    Full Text Available Abstract Background Increasing evidence incriminates bacteria, especially Mycoplasma fermentans, as possible arthritogenic agents in humans. The purpose of this study was to investigate M. fermentans in the bloodstream of patients with rheumatoid arthritis. Methods Two hundred and nineteen blood samples from patients with rheumatoid arthritis, systemic lupus erythematosus, antiphospholipid syndrome, and healthy individuals were screened by bacterial culture and direct PCR in order to detect mycoplasmas; IgM and IgG against M. fermentans PG18 were also detected by ELISA and Immunoblotting assays in patients with rheumatoid arthritis and healthy individuals. Results Blood samples from patients with antiphospholipid syndrome and healthy individuals were negative for mycoplasma by culture or direct PCR. In blood samples from patients with systemic lupus erythematosus were detected by direct PCR M. fermentans in 2/50 (2%, M. hominis in 2/50 (2% and U. urealyticum in 1/50 (0.5%. In patients with RA M. fermentans was detected by culture in 13/87 blood samples and in 13/87 by direct PCR, however, there was only concordance between culture and direct PCR in six samples, so M. fermentans was detected in 20/87(23% of the blood samples from patients with RA by either culture or PCR. Antibody-specific ELISA assay to M. fermentans PG18 was done, IgM was detected in sera from 40/87 patients with RA and in sera of 7/67 control individuals, IgG was detected in sera from 48/87 RA patients and in sera from 7/67 healthy individuals. Antibody-specific immunoblotting to M. fermentans PG18 showed IgM in sera from 35/87 patients with RA and in sera from 4/67 healthy individuals, IgG was detected in sera from 34/87 patients and in sera from 5/67 healthy individuals. Conclusion Our findings show that only M. fermentans produce bacteremia in a high percentage of patients with RA. This finding is similar to those reported in the literature. IgM and IgG against M

  13. Diagnostic and prognostic significance of measuring antibodies to alpha-fodrin compared to anti-Ro-52, anti-Ro-60, and anti-La in primary Sjogren's syndrome

    DEFF Research Database (Denmark)

    Pelck, R.; Manthorpe, R.; Locht, Henning

    2008-01-01

    OBJECTIVE: To compare sensitivity and specificity of autoantibodies to alpha-fodrin with conventional anti-Ro and anti-La antibodies in patients with primary Sjogren's syndrome (pSS). Data on internal organ manifestations were correlated with presence of autoantibodies. METHODS: We collected...... not diagnostically superior to conventional anti-Ro/La testing. IgG anti-La had the highest specificity (97%). A highly significant association was found between presence of anti-La and internal organ manifestations (OR 6, 95% CI 2.99-12.03) or hematological abnormalities. The pattern of autoantibodies...... was relatively independent of disease duration, indicating that these antibodies appeared early in pSS, probably even years before the first symptoms were manifest. CONCLUSION: We could not confirm that antibodies to alpha-fodrin had higher specificity or sensitivity than anti-Ro/La. Anti-La antibodies were...

  14. Thyroid Antibodies

    Science.gov (United States)

    ... be limited. Home Visit Global Sites Search Help? Thyroid Antibodies Share this page: Was this page helpful? Also known as: Thyroid Autoantibodies; Antithyroid Antibodies; Antimicrosomal Antibody; Thyroid Microsomal Antibody; ...

  15. [Neuroimmunological diseases associated with VGKC complex antibodies].

    Science.gov (United States)

    Watanabe, Osamu

    2013-05-01

    Antibodies to voltage-gated potassium channels(VGKC) were first identified by radioimmunoassay of radioisotope labeled alpha-dendrotoxin-VGKCs solubilized from rabbit brain. These antibodies were found only in a proportion of patients with acquired neuromyotonia (Isaacs' syndrome). VGKC antibodies were also detected in Morvan's syndrome and in a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC" antibodies are mainly directed toward associated proteins(for example LGI-1, Caspr-2) that complex with the VGKCs themselves. The "VGKC" antibodies are now usually known as VGKC-complex antibodies. In general, LGI-1 antibodies are most common in limbic encephalitis with SIADH. Caspr-2 antibodies are present in the majority of patients with Morvan's syndrome. These patients develop combinations of CNS symptoms, autonomic dysfunction, and peripheral nerve hyperexcitability.

  16. No evidence of misdiagnosis in patients with multiple sclerosis and repeated positive anticardiolipin antibody testing based on magnetic resonance imaging and long term follow‐up

    Science.gov (United States)

    Liedorp, M; Sanchez, E; van Hoogstraten, I M W; von Blomberg, B M E; Barkhof, F; Polman, C H

    2007-01-01

    Objective To determine whether patients with definite multiple sclerosis (MS) and repeated positive anticardiolipin antibody (aCL Ab) testing fulfil the recently updated criteria for the antiphospholipid syndrome (APS). Also, to determine if these patients form a separate subgroup in terms of long term follow‐up and MRI characteristics. Design A blinded case control study comparing MRI patterns between aCL Ab positive and negative MS patients with a clinical follow‐up of 7 years. Participants 8 (5.6%; male:female ratio 2:6; 6 relapsing–remitting subtype, 1 primary progressive subtype and 1 neuromyelitis optica (NMO)) of 143 consecutive patients with definite MS or NMO (71% relapsing–remitting, 18% secondary progressive and 6% primary progressive disease course; 4% NMO) showed repeated positive aCL Ab testing. Setting Outpatient clinic of a tertiary MS centre in the Netherlands. Results All eight aCL Ab positive patients had levels below 40 MPL/GPL units, with the majority of intervals between tests of at least 12 weeks. After follow‐up, none of the patients fulfilled the criteria for APS. No specific MRI features were present compared with 24 matched aCL Ab negative patients. Conclusions No aCL Ab positive MS patient fulfilled the criteria for APS, arguing against a possible misdiagnosis or coexistence. PMID:17878195

  17. Seroprevalence of hepatitis A antibodies in a group of normal and Down Syndrome children in Porto Alegre, Southern Brazil

    Directory of Open Access Journals (Sweden)

    Ferreira Cristina Targa

    2002-01-01

    Full Text Available The high incidence of Hepatitis A and B in institutionalized patients with Down Syndrome (DS is not fully understood. Under poor hygienic conditions, immunological alterations might predispose individuals to these infections. Sixty three DS children between 1 and 12 years old living at home with their families were examined for anti-HAV and compared to age-matched controls (64 healthy children. This cross-sectional study was carried out from May, 1999, to April, 2000, at the Hospital de Clínicas of Porto Alegre, southern Brazil. Groups were compared in terms of age, sex, skin color, and family income (> R$ 500 and < R$ 500/ month by the chi-square test, with Yates' correction and for the prevalence of anti-HAV (Fisher's exact test. In the DS group (n=63, the mean age was 4.4 ± 3.3 years, 94% of the patients were white and 51% were female. Family income was <= R$ 500/month in 40 cases (63%. In the control group (n=64, the mean age was 4.8 ± 2.7 years, 81% of the patients were white and 56% were female. Family income was <= R$ 500 in 20 patients (31%. DS children's families had a significantly lower income (P<0.0005. In the DS group there were 6 positive (9.5% anti-HAV cases, and all came from low-income families (less than R$ 500/ month. In the control group, 3 cases (4.7% were positive for anti-HAV (two were from a low-income family and one was from a higher income family. These differences were not significant. Our data indicate that Hepatitis A is not a special risk for mentally retarded DS outpatients, even in a developing country like Brazil.

  18. An international multicentre-laboratory evaluation of a new assay to detect specifically lupus anticoagulants dependent on the presence of anti-beta2-glycoprotein autoantibodies

    NARCIS (Netherlands)

    de laat, B.; Derksen, R. H. W. M.; Reber, G.; Musial, J.; Swadzba, J.; Bozic, B.; Cucnik, S.; Regnault, V.; Forastiero, R.; Woodhams, B. J.; de Groot, Ph. G.

    2011-01-01

    Background: Antiphospholipid syndrome (APS) is diagnosed by the simultaneous presence of vascular thrombosis and/or pregnancy morbidity and detection of antiphospholipid antibodies in plasma. Objectives: We have shown that prolongation of clotting time by anti-beta2-glycoprotein I (beta2GPI) antibod

  19. Pregnancy in Systemic Lupus Erythematosus Patients with Nephritis

    OpenAIRE

    Panagiotis Pateinakis; Athina Pyrpasopoulou

    2014-01-01

    Pregnancy in patients with lupus nephritis is a challenging clinical situation. Although not absolutely contraindicated, it is associated with increased risk for foetal and maternal complications, including foetal loss, preterm delivery, intrauterine growth retardation, hypertension, pre-eclampsia, nephritis flare, and, rarely, maternal death. The complication rate is further increased in the presence of antiphospholipid antibodies or the antiphospholipid syndrome. Proliferative classes of ne...

  20. Glutamate receptor antibodies in neurological diseases: anti-AMPA-GluR3 antibodies, anti-NMDA-NR1 antibodies, anti-NMDA-NR2A/B antibodies, anti-mGluR1 antibodies or anti-mGluR5 antibodies are present in subpopulations of patients with either: epilepsy, encephalitis, cerebellar ataxia, systemic lupus erythematosus (SLE) and neuropsychiatric SLE, Sjogren's syndrome, schizophrenia, mania or stroke. These autoimmune anti-glutamate receptor antibodies can bind neurons in few brain regions, activate glutamate receptors, decrease glutamate receptor's expression, impair glutamate-induced signaling and function, activate blood brain barrier endothelial cells, kill neurons, damage the brain, induce behavioral/psychiatric/cognitive abnormalities and ataxia in animal models, and can be removed or silenced in some patients by immunotherapy.

    Science.gov (United States)

    Levite, Mia

    2014-08-01

    .g., chronic progressive limbic Encephalitis, Paraneoplastic Encephalitis or Herpes Simplex Virus Encephalitis), Schizophrenia, Mania, Stroke, or Sjorgen syndrome. In some patients, the anti-NMDA-NR2A/B antibodies are present in both the serum and the CSF. Some of the anti-NMDA-NR2A/B antibodies cross-react with dsDNA, while others do not. Some of the anti-NMDA-NR2A/B antibodies associate with neuropsychiatric/cognitive/behavior/mood impairments in SLE patients, while others do not. The anti-NMDA-NR2A/B antibodies can undoubtedly be very pathogenic, since they can kill neurons by activating NMDA receptors and inducing 'Excitotoxicity', damage the brain, cause dramatic decrease of membranal NMDA receptors expressed in hippocampal neurons, and also induce behavioral cognitive impairments in animal models. Yet, the concentration of the anti-NMDA-NR2A/B antibodies seems to determine if they have positive or negative effects on the activity of glutamate receptors and on the survival of neurons. Thus, at low concentration, the anti-NMDA-NR2A/B antibodies were found to be positive modulators of receptor function and increase the size of NMDA receptor-mediated excitatory postsynaptic potentials, whereas at high concentration they are pathogenic as they promote 'Excitotoxcity' through enhanced mitochondrial permeability transition. (4) Anti-mGluR1 antibodies were found thus far in very few patients with Paraneoplastic Cerebellar Ataxia, and in these patients they are produced intrathecally and therefore present in much higher levels in the CSF than in the serum. The anti-mGluR1 antibodies can be very pathogenic in the brain since they can reduce the basal neuronal activity, block the induction of long-term depression of Purkinje cells, and altogether cause cerebellar motor coordination deficits by a combination of rapid effects on both the acute and the plastic responses of Purkinje cells, and by chronic degenerative effects. Strikingly, within 30 min after injection of anti-mGluR1

  1. Glutamate receptor antibodies in neurological diseases: anti-AMPA-GluR3 antibodies, anti-NMDA-NR1 antibodies, anti-NMDA-NR2A/B antibodies, anti-mGluR1 antibodies or anti-mGluR5 antibodies are present in subpopulations of patients with either: epilepsy, encephalitis, cerebellar ataxia, systemic lupus erythematosus (SLE) and neuropsychiatric SLE, Sjogren's syndrome, schizophrenia, mania or stroke. These autoimmune anti-glutamate receptor antibodies can bind neurons in few brain regions, activate glutamate receptors, decrease glutamate receptor's expression, impair glutamate-induced signaling and function, activate blood brain barrier endothelial cells, kill neurons, damage the brain, induce behavioral/psychiatric/cognitive abnormalities and ataxia in animal models, and can be removed or silenced in some patients by immunotherapy.

    Science.gov (United States)

    Levite, Mia

    2014-08-01

    .g., chronic progressive limbic Encephalitis, Paraneoplastic Encephalitis or Herpes Simplex Virus Encephalitis), Schizophrenia, Mania, Stroke, or Sjorgen syndrome. In some patients, the anti-NMDA-NR2A/B antibodies are present in both the serum and the CSF. Some of the anti-NMDA-NR2A/B antibodies cross-react with dsDNA, while others do not. Some of the anti-NMDA-NR2A/B antibodies associate with neuropsychiatric/cognitive/behavior/mood impairments in SLE patients, while others do not. The anti-NMDA-NR2A/B antibodies can undoubtedly be very pathogenic, since they can kill neurons by activating NMDA receptors and inducing 'Excitotoxicity', damage the brain, cause dramatic decrease of membranal NMDA receptors expressed in hippocampal neurons, and also induce behavioral cognitive impairments in animal models. Yet, the concentration of the anti-NMDA-NR2A/B antibodies seems to determine if they have positive or negative effects on the activity of glutamate receptors and on the survival of neurons. Thus, at low concentration, the anti-NMDA-NR2A/B antibodies were found to be positive modulators of receptor function and increase the size of NMDA receptor-mediated excitatory postsynaptic potentials, whereas at high concentration they are pathogenic as they promote 'Excitotoxcity' through enhanced mitochondrial permeability transition. (4) Anti-mGluR1 antibodies were found thus far in very few patients with Paraneoplastic Cerebellar Ataxia, and in these patients they are produced intrathecally and therefore present in much higher levels in the CSF than in the serum. The anti-mGluR1 antibodies can be very pathogenic in the brain since they can reduce the basal neuronal activity, block the induction of long-term depression of Purkinje cells, and altogether cause cerebellar motor coordination deficits by a combination of rapid effects on both the acute and the plastic responses of Purkinje cells, and by chronic degenerative effects. Strikingly, within 30 min after injection of anti-mGluR1

  2. Haematology and neurology

    Science.gov (United States)

    Austin, Steven; Cohen, Hannah; Losseff, Nick

    2007-01-01

    This review aims to update the reader on advances in the understanding of haematological conditions that may arise in neurological practice. Thrombophilia, antiphospholipid antibody syndrome, thrombotic thrombocytopenic purpura, sickle cell and clonal disorders associated with neuropathy are discussed. PMID:17369588

  3. Blood Clotting and Pregnancy

    Medline Plus

    Full Text Available ... blood conditions. back to top Antiphospholipid Antibody Syndrome: A Patient's Journey back to top Where Can I ... and help move hematology forward. Learn more Find a Hematologist Search a database of practicing hematologists in ...

  4. Vaccination of Plasmid DNA Encoding Somatostatin Gene Fused with GP5 Gene of Porcine Reproductive and Respiratory Syndrome Virus Induces Anti-GP5 Antibodies and Promotes Growth Performance in Immunized Pigs

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Somatostatin (SS) is a hormone that inhibits the secretion of growth hormone. Immunization against SS can promote the growth of animals. This paper described the effects of DNA immunization on the growth and antibody response in mice and pigs immunized with a plasmid DNA encoding SS fused with GP5 of porcine reproductive and respiratory syndrome virus (PRRSV). A fragment of 180 bp encoding partial SS gene was amplified by PCR from the genomic DNA of peripheral blood mononuclear cells of pigs, and cloned as a fusion gene with PRRSV GP5 in plasmid pISGRTK3. Three times of immunization with the resulting plasmid pISG-SS/GP5 induced anti-GP5 antibodies in BALB/c mice and pigs, as demonstrated by GP5-specific ELISA and immunoblotting. Compared with pigs immunized with empty vector pISGRTK3, the growth performance of pigs immunized with pISG-SS/GP5 was increased by 11.1% on the 13th week after the last vaccination. The results indicated the plasmid DNA encoding SS and PRRSV GP5 fusion gene elicited anti-GP5 antibodies and improved the growth performance of immunized pigs.

  5. Plasma levels of plasminogen activator inhibitor type 1, factor VIII, prothrombin activation fragment 1+2, anticardiolipin, and antiprothrombin antibodies are risk factors for thrombosis in hemodialysis patients.

    Science.gov (United States)

    Molino, Daniela; De Santo, Natale G; Marotta, Rosa; Anastasio, Pietro; Mosavat, Mahrokh; De Lucia, Domenico

    2004-09-01

    Patients with end-stage renal disease are prone to hemorrhagic complications and simultaneously are at risk for a variety of thrombotic complications such as thrombosis of dialysis blood access, the subclavian vein, coronary arteries, cerebral vessel, and retinal veins, as well as priapism. The study was devised for the following purposes: (1) to identify the markers of thrombophilia in hemodialyzed patients, (2) to establish a role for antiphospholipid antibodies in thrombosis of the vascular access, (3) to characterize phospholipid antibodies in hemodialysis patients, and (4) to study the effects of dialysis on coagulation cascade. A group of 20 hemodialysis patients with no thrombotic complications (NTC) and 20 hemodialysis patients with thrombotic complications (TC) were studied along with 400 volunteer blood donors. Patients with systemic lupus erythematosus and those with nephrotic syndrome were excluded. All patients underwent a screening prothrombin time, activated partial thromboplastin time, fibrinogen (Fg), coagulation factors of the intrinsic and extrinsic pathways, antithrombin III (AT-III), protein C (PC), protein S (PS), resistance to activated protein C, prothrombin activation fragment 1+2 (F1+2), plasminogen, tissue type plasminogen activator (t-PA), plasminogen tissue activator inhibitor type-1 (PAI-1), anticardiolipin antibodies type M and G (ACA-IgM and ACA-IgG), lupus anticoagulant antibodies, and antiprothrombin antibodies type M and G (aPT-IgM and aPT-IgG). The study showed that PAI-1, F 1+2, factor VIII, ACA-IgM, and aPT-IgM levels were increased significantly over controls both in TC and NTC, however, they could distinguish patients with thrombotic complications from those without, being increased maximally in the former group. The novelty of the study is represented by the significant aPT increase that was observed in non-systemic lupus erythematosus hemodialysis patients, and particularly in those with thrombotic events. In addition

  6. Association of beta2-glycoprotein I IgG and IgM antibodies with thrombosis and thrombocytopenia

    DEFF Research Database (Denmark)

    Voss, Anne-Sofie Boertmann; Jacobsen, Søren; Heegaard, Niels Henrik Helweg

    2001-01-01

    Antiphospholipid antibodies (APA) have been known for decades. Their relation to clinical manifestations, primarily thromboses and thrombocytopenia, was recognised in the 1980s. In this clinical study two cohorts of patients, a population-based (84 patients with systemic lupus erythematosus (SLE......)) and a hospital-based (87 patients with SLE and 53 with other connective tissue diseases) were investigated for APA and associated clinical manifestations. Anticardiolipin antibodies (ACA) of IgG and IgM classes were found in 13 and 38% of the population-based patients and in 29 and 58% of the hospital......-based patients, respectively. The corresponding figures for antibodies against beta2-glycoprotein I (anti-beta2GPI) were 15 and 10% in the population-based patients and 14 and 8% in the hospital-based cohort. Anti-beta2GPI antibodies were always found in association with the corresponding immunoglobulin class...

  7. Beta2-glycoprotein I dependent anticardiolipin antibodies and lupus anticoagulant in patients with recurrent pregnancy loss.

    Directory of Open Access Journals (Sweden)

    Kumar K

    2002-01-01

    Full Text Available AIM: The present study was aimed to define the incidence of antiphospholipid antibodies of different types lupus anticoagulant (LAC, venereal disease research laboratory test (VDRL and Beta2-glycoprotein I dependent anticardiolipin antibodies Beta2 I aCL in our cohort of population experiencing recurrent pregnancy loss (RPL from Andhra Pradesh, South India. SETTING AND DESIGN: A referral case-control study at a tertiary centre over a period of 5 years. PARTICIPANTS: 150 couples experiencing 3 or more recurrent pregnancy losses with similar number of matched controls. MATERIAL AND METHODS: LAC activity was measured by the activated partial thromboplastin time (aPTT according to the method of Proctor and Rapaport with relevant modifications. VDRL analysis was performed by the kit method supplied by Ranbaxy Diagnostics Limited and Beta2 Glycoprotein I dependent anticardiolipin antibodies were estimated by ELISA kit (ORGen Tech, GmbH, Germany with human Beta2 Glycoprotein I as co-factor. STATISTICAL ANALYSIS: Statistical analysis was performed using Student′s t test. RESULTS: LAC activity was found positive in 11 women (10.28%. The mean +/- SE Beta2 I aCL concentration in the study group was 14.53 (micro/ml +/- 1.79 (range 0 to 90.4 micro/ml which was higher than the control group with a mean +/- SE of 7.26 (micro/ml +/- 0.40 (range 0 to 18 u/ml. The binding of the antibodies to the antigen was observed in 40.24% (n=33 of the cases compared to 6.09% (n=5 in controls. VDRL test was positive in 7(2.34% individuals (3 couples and 1 male partner and none among controls. CONCLUSIONS: The present study indicates the importance of antiphospholipid antibodies in women experiencing RPL and suggests the usefulness of screening for these antibodies as a mandatory routine for instituting efficient therapeutic regimens for a successful outcome of pregnancy.

  8. A Novel Anti-Histone H1 Monoclonal Antibody, SSV Monoclonal Antibody, Improves Lung Injury and Survival in a Mouse Model of Lipopolysaccharide-Induced Sepsis-Like Syndrome

    Directory of Open Access Journals (Sweden)

    Toru Kusano

    2015-01-01

    Full Text Available Background. Histones play important roles in both host defenses and inflammation related to microbial infection. A peptide mimotope (SSV was identified from a novel histone H1 monoclonal antibody (16G9 mAb that was shown to inhibit the mixed lymphocyte reaction. In the present study, an anti-SSV producing hybridoma was established. We investigated the effects of SSV mAb in a mouse acute inflammation model induced by intraperitoneal injection of lipopolysaccharide (LPS. Methods. SSV mAb was generated and characterized. Mice were treated with SSV mAb or a control IgG antibody prior to LPS injection. Evaluation of survival rate and lung tissue on histological score was performed. The levels of inflammatory cytokines and histones H1, H3, and H4 in plasma and lung tissue were measured by ELISA. Results. Competitive ELISA revealed that SSV mAb binds to histone H1. SSV mAb improved lung injury and prolonged the survival of LPS-injected mice. Increased levels of histones H1, H3, and H4 and inflammatory cytokines (TNF-α, IL-1β, and IL-6 in plasma and lung tissue after LPS injection were ameliorated by SSV mAb. Conclusion. SSV mAb is shown to have anti-inflammatory activity and organ-protective effects, highlighting the importance of controlling histone H1 as well as H3 and H4 levels during inflammation.

  9. Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies.

    Science.gov (United States)

    Corominas, Hèctor; Ortiz-Santamaría, Vera; Castellví, Iván; Moreno, Mireia; Morlà, Rosa; Clavaguera, Teresa; Erra, Alba; Martínez-Pardo, Silvia; Ordóñez, Sergi; Santo, Pilar; Reyner, Patricia; González, Maria José; Codina, Oriol; Gelman, Mario Saul; Juanola-Roura, Xavier; Olivé, Alex; Torrente-Segarra, Vicenç

    2016-03-01

    The aim of this study was to assess nailfold capillaroscopic (NC) findings in patients with primary Sjögren's syndrome (PSS) with and without Raynaud's phenomenon (RP) as well as in the presence of positive anti-SSA/Ro and anti-SSB/La antibodies. Videocapillaroscopy was performed in 150 patients with PSS. Data collected included demographics, presence of RP, PSS symptoms, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, and NC findings. RP was present in 32% of PSS, keratoconjunctivitis sicca in 91%, oral xerosis in 93%, and skin or genital xerosis in 53%. In patients with positive anti-SSA/Ro (75%) and positive anti-SSB/La (40%), NC showed normal findings in 53% of cases and non-specific in 36%. In patients with PSS, NC was normal in 51% of cases and non-specific in 34%. Scleroderma pattern was found in 14 patients. RP associated with PSS had non-specific capillaroscopy in 40% of cases (p = 0.1). Pericapillary haemorrhages (p = 0.06) and capillary thrombosis (p = 0.2) were not increased, but more dilated capillaries were detected in 48% of cases. Patients with positive anti-Ro and/or anti-La have not a distinct NC profile. Patients with RP associated with PSS had more dilated capillaries, but neither pericapillary haemorrhages nor capillary thrombosis was observed.

  10. Peripheral nerve P2 basic protein and the Guillain-Barre syndrome : In vitro demonstration of P2-specific antibody-secreting cells

    NARCIS (Netherlands)

    Luijten, J.A.F.M.; Jong, W.A.C. de; Demel, R.A.; Heijnen, C.J.; Ballieux, R.E.

    1984-01-01

    An immune response to the peripheral nerve basic protein P2 may be operative in the pathogenesis of the Guillain-Barré syndrome (GBS). A method is described for the purification of P2 of human origin. Purified P2 was used to investigate whether lymphocytes derived from peripheral blood of GBS patien

  11. Optimisation of sandwich ELISA based on monoclonal antibodies for the specific measurement of pregnancy-associated plasma protein (PAPP-A) in acute coronary syndrome

    DEFF Research Database (Denmark)

    Rossen, Marie; Iversen, Kasper; Teisner, Ane;

    2007-01-01

    OBJECTIVES: PAPP-A has become the principal biochemical serum marker in first trimester screening for Down syndrome, the original data being based on results of a radioimmunoassay (RIA). Recent observations using sandwich ELISA technology have proposed PAPP-A as a potential marker in patients wit...

  12. Basis for defective proliferation of peripheral blood T cells to anti-CD2 antibodies in primary Sjögren's syndrome.

    Science.gov (United States)

    Gerli, R; Bertotto, A; Agea, E; Lanfrancone, L; Cernetti, C; Spinozzi, F; Rambotti, P

    1990-12-01

    Anti-CD2-induced T cell proliferation was analyzed in the peripheral blood samples of 31 primary and 8 secondary untreated Sjögren's syndrome patients. Anti-CD2-stimulated PBMC proliferation was very low in about one-third of primary Sjögren's syndrome samples, despite the number of CD2+ cells being similar in primary and secondary Sjögren's syndrome and normal PBMC samples. The depressed response to anti-CD2 was mainly found in anti-Ro+/La+ patients. Experiments on purified T cells demonstrated that a defect at the T cell level was responsible for the anti-CD2 unresponsiveness. Cell proliferation failure was associated with poor IL-2 and IL-2 receptor mRNA expression and, consequently, IL-2 and IL-2 receptor synthesis. Since defective anti-CD2-induced mitogenesis could be reversed by phorbol myristate acetate, but not calcium ionophore A23187, it is probably correlated with impaired protein kinase C activation. Comparison of anti-CD2-triggered PBMC proliferation in treated and untreated patients and a long-term study of nine patients showed that the defect is a stable characteristic in primary Sjögren's syndrome patients, but that it can be reversed by pharmacological immunosuppression. PMID:1979333

  13. The diagnostic value of anti-SSA antibody in primary Sj(o)gren's syndrome%抗SSA抗体在原发性干燥综合征的诊断价值

    Institute of Scientific and Technical Information of China (English)

    李梦涛; 董怡; 赵岩; 郑文洁; 苏金梅; 吴庆军; 蒋颖; 徐东; 张文; 张奉春

    2010-01-01

    Objective According to international classification criteria (2002) on Sjogren' s syndrome, labial pathology was still considered as a major criterion for diagnosis. Standard labial biopsy was hard to be carried out in China. This study is to evaluate whether the invasive labial biopsy could be replaced by noninvasive detection of serum anti-SSA antibody. Methods 181 Chinese patients with the initial diagnosis of primary Sjogren's syndrome in Peking Union Medical College Hospital (PUMCH) were enrolled in Sjogren's International Collaborative Clinical Alliance (SICCA). All patients received standard labial biopsies (area of salivary gland tissues≥4 mm~2) and focal score (FS) of focal lymphatic sialadenitis were confirmed by pathologists from school of stomatology,University California of San Francisco (UCSF). Anti-SSA antibodies in sera of all patients were detected by double immunodiffusion (DID), Western blot in PUMCH and by enzyme-linked immunosorbent assay (EIJSA) in central laboratory of SICCA. The correlation between labial pathological findings and serum anti-SSA antibody was studied by X~2 test and the concordance was calculated by unweighted Kappa. Results(1)Bivariate analysis revealed strong associations of FS > 1 with the presence of anti-SSA antibody by DID (83.9% vs 42. 0%, P 1 was strongly associated with the presence of anti-SSA antibody by Western blot (83.0% vs 51.7%, P 1 was strongly associated with the presence of anti-SSA antibody by ELISA (81.5% vs 38.6%, P 1 finding is strongly associated with anti-SSA antibody. Positive results of anti-SSA antibodies by DID or ELISA may indicate FS > 1, thus labial biopsy could relatively be avoided, negative results may need further standard labial biopsy procedure to confirm the diagnosis of Sjogren's syndrome.%目的 探讨抗SSA抗体在原发性干燥综合征(ss)的诊断价值.方法 选北京协和医院风湿免疫科临床初诊为原发性SS的患者181例,所有病例均按照SS国际合作联

  14. [Isaacs's syndrome and associated diseases].

    Science.gov (United States)

    Watanabe, Osamu

    2013-01-01

    Isaacs' syndrome is an antibody-mediated potassium channel disorder. Clinical symptoms of Isaacs' syndrome are characterized by muscle cramp, slow relaxation following muscle contraction, and hyperhidrosis. Hyperexcitability of the peripheral nerve cause these symptoms, which are relieved by administration of Na channel blockers and immunotherapy.The target channel proteins are voltage-gated potassium channels (VGKCs). The suppression of voltage-gated outward K(+) current by antibodies induces hyperexcitability of the peripheral nerve. Electrophysiological findings show that antibodies may not directly block the kinetics of VGKCs, but may decrease channel density. From the electrophysiological, pharmacologic and immunologic view points, the site of origin of spontaneous discharges is located principally in the distal portion of the motor nerve."VGKC antibodies" are also detected in Morvan syndrome (severe insomnia with neuromyotonia and various autonomic disorders) and in a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC antibodies" are mainly directed toward associated proteins (for example LGI-1, CASPR-2) that complex with VGKCs themselves. The "VGKC antibodies" are now usually known as VGKC-complex antibodies. In general, LGI-1 antibodies are most common in limbic encephalitis with SIADH. CASPR-2 antibodies are present in the majority of patients with Morvan syndrome.

  15. [Isaacs's syndrome and associated diseases].

    Science.gov (United States)

    Watanabe, Osamu

    2013-01-01

    Isaacs' syndrome is an antibody-mediated potassium channel disorder. Clinical symptoms of Isaacs' syndrome are characterized by muscle cramp, slow relaxation following muscle contraction, and hyperhidrosis. Hyperexcitability of the peripheral nerve cause these symptoms, which are relieved by administration of Na channel blockers and immunotherapy.The target channel proteins are voltage-gated potassium channels (VGKCs). The suppression of voltage-gated outward K(+) current by antibodies induces hyperexcitability of the peripheral nerve. Electrophysiological findings show that antibodies may not directly block the kinetics of VGKCs, but may decrease channel density. From the electrophysiological, pharmacologic and immunologic view points, the site of origin of spontaneous discharges is located principally in the distal portion of the motor nerve."VGKC antibodies" are also detected in Morvan syndrome (severe insomnia with neuromyotonia and various autonomic disorders) and in a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC antibodies" are mainly directed toward associated proteins (for example LGI-1, CASPR-2) that complex with VGKCs themselves. The "VGKC antibodies" are now usually known as VGKC-complex antibodies. In general, LGI-1 antibodies are most common in limbic encephalitis with SIADH. CASPR-2 antibodies are present in the majority of patients with Morvan syndrome. PMID:24291881

  16. Myasthenic syndromes.

    Science.gov (United States)

    Farrugia, M E

    2011-03-01

    The neuromuscular junction is vulnerable to autoimmune attack both at the pre-synaptic nerve terminal and at the post-synaptic muscle membrane. Antibodies directed to the nicotinic acetylcholine receptor at the muscle surface are the cause of myasthenia gravis in the majority of cases. Myasthenia gravis is an acquired condition, characterised by weakness and fatigability of the skeletal muscles. The ocular muscles are commonly affected first, but the disease often generalises. Treatment includes symptom control and immunosuppression. The thymus gland plays an important role in the pathogenesis of myasthenia gravis and thymectomy is indicated in certain subgroups. Lambert-Eaton myasthenic syndrome is associated with antibodies directed to the voltage-gated calcium channel antibodies at the pre-synaptic nerve terminal. It is an acquired condition and, in some cases, may be paraneoplastic, often secondary to underlying small cell lung carcinoma. Clinical presentation is distinct from myasthenia gravis, with patients often first presenting with lower limb muscle fatigability and autonomic symptoms. Congenital myasthenic syndromes are inherited neuromuscular disorders due to mutations in proteins at the neuromuscular junction. Various phenotypes exist depending on the protein mutation. Treatment is directed towards symptom control and immunosuppression is not indicated. PMID:21365067

  17. A phase I study evaluating the pharmacokinetics, safety and tolerability of an antibody-based tissue factor antagonist in subjects with acute lung injury or acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Morris Peter E

    2012-02-01

    Full Text Available Abstract Background The tissue factor (TF-dependent extrinsic pathway has been suggested to be a central mechanism by which the coagulation cascade is locally activated in the lungs of patients with acute lung injury and acute respiratory distress syndrome (ALI/ARDS and thus represents an attractive target for therapeutic intervention. This study was designed to determine the pharmacokinetic and safety profiles of ALT-836, an anti-TF antibody, in patients with ALI/ARDS. Methods This was a prospective, randomized, placebo-controlled, dose-escalation Phase I clinical trial in adult patients who had suspected or proven infection, were receiving mechanical ventilation and had ALI/ARDS (PaO2/FiO2 ≤ 300 mm. Eighteen patients (6 per cohort were randomized in a 5:1 ratio to receive ALT-836 or placebo, and were treated within 48 hours after meeting screening criteria. Cohorts of patients were administered a single intravenously dose of 0.06, 0.08 or 0.1 mg/kg ALT-836 or placebo. Blood samples were taken for pharmacokinetic and immunogenicity measurements. Safety was assessed by adverse events, vital signs, ECGs, laboratory, coagulation and pulmonary function parameters. Results Pharmacokinetic analysis showed a dose dependent exposure to ALT-836 across the infusion range of 0.06 to 0.1 mg/kg. No anti-ALT-836 antibody response was observed in the study population during the trial. No major bleeding episodes were reported in the ALT-836 treated patients. The most frequent adverse events were anemia, observed in both placebo and ALT-836 treated patients, and ALT-836 dose dependent, self-resolved hematuria, which suggested 0.08 mg/kg as an acceptable dose level of ALT-836 in this patient population. Conclusions Overall, this study showed that ALT-836 could be safely administered to patients with sepsis-induced ALI/ARDS. Trial registration ClinicalTrials.gov: NCT01438853

  18. Metabolic syndrome and central retinal artery occlusion

    Directory of Open Access Journals (Sweden)

    Kosanović-Jaković Natalija

    2005-01-01

    Full Text Available Background. The accumulation of risk factors for central retinal artery occlusion can be seen in a single person and might be explained by the metabolic syndrome. Case report. We presented the case of a 52-year-old man with no light perception in his right eye. The visual loss was monocular and painless, fundoscopy showed central retinal artery occlusion and the laboratory investigation showed the raised erythrocyte sedimentation rate of 105 mm/h and the raised C-reactive protein of 22 mg/l. Specific laboratory investigations and fluorescein angiography excluded the presence of vasculitis, collagen vascular diseases, hypercoagulable state and antiphospholipid syndrome. Conclusion. The patient met all the five of the National Cholesterol Education Program (NCEP criteria for the metabolic syndrome: hypertension, abnormal lipid profile, abnormal glucose metabolism, obesity and hyperuricemia. Measurement of C-reactive protein is useful for the assessment of therapeutic systemic effect on any abnormality in the metabolic syndrome. Individual therapy for all risk factors in the metabolic syndrome is necessary to prevent complications such as cardiovascular, retinal vascular diseases and stroke.

  19. Anticuerpos anti 21 hidroxilasa séricos en pacientes con anticuerpos antifracción microsomal: Síndrome poliendocrino autoinmune Seric 21- hydroxilase antibodies in patients with anti-microsomal fraction antibodies: Autoimmune polyendocrine syndrome

    Directory of Open Access Journals (Sweden)

    Silvia Botta

    2007-04-01

    Full Text Available El síndrome poliendocrino autoinmune (SPA es la asociación de enfermedades endocrinas autoinmunes con otros desórdenes autoinmunes no endocrinos. Los tipos 1, 2 y 4 presentan adrenalitis autoinmune, esto indica la presencia de autoanticuerpos, y su marcador serológico específico es el anti 21 hidroxilasa (a21-OH. El SPA tipo 2 es la asociación de adrenalitis, enfermedad tiroidea y/o diabetes mellitus inducidas por autoanticuerpos. Como componentes menores, pueden estar asociados entre otros, vitiligo, alopecia y miastenia. Nuestros objetivos fueron: establecer la prevalencia de a21-OH séricos en pacientes con anticuerpos anti fracción microsomal (AFM positivos, enfermedad tiroidea autoinmune y/o afecciones endocrinas y no endocrinas autoinmunes; diagnosticar formas incompletas de SPA y estudiar individuos con probable riesgo de progresión a un SPA completo. Estudiamos 72 pacientes AFM positivos y 60 sujetos tomados como grupo control, AFM negativos. Hallamos a21-OH elevados en dos pacientes: A= 47 U/ml, hipotiroidismo autoinmune y miastenia; y B= 8.75 U/ml, hipotiroidismo autoinmune y vitiligo; ambos con ausencia de insuficiencia adrenal. La prevalencia de a21-OH encontrada fue del 2.8%. Las pacientes A y B corresponden a un SPA tipo 2 incompleto y latente en relación al componente adrenal. Considerando a los a21-OH marcadores de enfermedad autoinmune latente, el eventual riesgo de evolución hacia la afección clínica sugiere la necesidad de estrechos controles clínicos y bioquímicos periódicos.Autoimmune polyendocrine syndrome (APS is the association of autoimmune endocrine diseases, with other autoimmune nonendocrine disorders. APS types 1, 2 and 4 include autoimmune adrenalitis; this suggests the presence of autoantibodies. A specific serological marker for these is the anti 21- hydroxilase autoantibody (a21-OH. APS type 2 is the association of autoimmune adrenalitis, to autoimmune thyroid disease and/or diabetes mellitus, all

  20. Budd-Chiari Syndrome: Two Cases with Different Courses

    Directory of Open Access Journals (Sweden)

    Shinjiro Inomata

    2008-08-01

    Full Text Available We report two cases of Budd-Chiari syndrome. Case 1: A 57-year-old man presented with leg edema and esophageal varices. Cavography showed obstruction of the inferior vena cava with antiphospholipid syndrome. Further, the patient showed positive serology for hepatitis C virus and consumed large quantities of alcohol. Percutaneous transluminal angioplasty was performed on this patient and anticoagulants administered; leg edema and esophageal varices were ameliorated although liver biopsy showed cirrhosis without evident congestion. More than 9 months since the diagnosis, restenosis of the inferior vena cava has not occurred. Case 2: A 73-year-old woman presented abdominal pain but no edema or varices. Cavography showed membranous obstruction of the inferior vena cava which required no therapy. Manifestation of portal hypertension was not present and liver function was maintained although liver biopsy showed obvious congestion. These cases showed untypical features against histopathology, and careful observation will be required for emergence of hepatocellular carcinoma.

  1. [Hypercoagulable workup in ophthalmology. When and what].

    Science.gov (United States)

    Muñoz-Negrete, F J; Casas-Lleras, P; Pérez-López, M; Rebolleda, G

    2009-07-01

    Most ophthalmologic disorders secondary to hypercoagulabe state are due to the confluence of congenital and adquired factors. A systematic workup is mandatory. Most of congenital coagulation disorders cause venous trombosis and are inherited autosomal dominantly. In order of frequency these are factor V Leiden mutation (activated protein C resistance), G20210A mutation of the prothrombin gen and protein C, protein S, and antithrombin III deficiencies. Sickle cell anemia can determine arerial and venous thrombosis. In relation with arterial occlusion, the markers most frequently involved are homcysteine fasting levels and the markers of antiphospholipid antibody syndrome. Both of them can also determine venous thrombosis. Several acquired factors can lead to hypoercoagulable state, especially hyperhomocysteinemia, antiphospholipid antibody syndrome, hepatic disease, alcohol and tobacco intake, oral contraceptives, immobilization, surgeries and malignancies. In central venous occlusion is only necessary to rule out hyperhomocysteinemia and antiphospholipid antibody syndrome in young patients without known risk factors. In central artery occlusion, hypercoagulable workup is only recommended for patients less than 50 years-old with unknown emboli source. In this cases protein C, protein S, and antithrombin III deficiencies, homocystein, sickle cell disease and antiphospholipid antibody syndrome will ruled out. In non arteritic ischemic optic neuropathy hypercoagulable work up is not necessary. In amaurosis fugax without known emboli source, it is recommended to rule out etiologies of arterial occlusion, especially antithrombin III deficiencies, homocystein, sickle cell disease and antiphospholipid antibody syndrome. PMID:19658050

  2. Antineutrophil cytoplasmic antibodies

    Directory of Open Access Journals (Sweden)

    G.D. Sebastiani

    2011-06-01

    Full Text Available Antineutrophil cytoplasmic antibodies (ANCA are predominantly IgG autoantibodies directed against constituents of primary granules of neutrophils and monocytes’ lysosomes. Although several antigenic targets have been identified, those ANCA directed to proteinase 3 or myeloperoxidase are clinically relevant, whereas the importance of other ANCA remains unknown. Both are strongly associated with small vessel vasculitides, the ANCA-associated vasculitides, which include Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, and the localised forms of these diseases (eg, pauci-immune necrotising and crescentic glomerulonephritis. ANCA is a useful serological test to assist in diagnosis of small-vessel vasculitides. 85-95% of patients with Wegener’s granulomatosis, microscopic polyangiitis, and pauci-immune necrotising and crescentic glomerulonephritis have serum ANCA. ANCA directed to either proteinase 3 or myeloperoxidase are clinically relevant, yet the relevance of other ANCA remains unknown. Besides their diagnostic potential, ANCA might be valuable in disease monitoring. In addition, data seem to confirm the long-disputed pathogenic role of these antibodies. There is increasing evidence that myeloperoxidase- ANCA are directly involved in the pathogenesis of necrotizing vasculitis. This is less clear for proteinase 3-ANCA, markers for Wegener’s granulomatosis. With respect to proteinase 3-ANCA, complementary proteinase 3, a peptide translated from the antisense DNA strand of proteinase 3 and homologous to several microbial peptides, may be involved in induction of proteinase 3-antineutrophil cytoplasmic autoantibodies.

  3. Glycopeptide profiling of beta-2-glycoprotein I by mass spectrometry reveals attenuated sialylation in patients with antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Kondo, Akira; Miyamoto, Toshiaki; Yonekawa, Osamu;

    2009-01-01

    or exposing the Gly40-Arg43 motif towards the anti-beta2GPI autoantibody. To test this hypothesis we used mass spectrometry (MS) for comparative glycopeptide profiling of human beta2GPI obtained from blood serum from four healthy test subjects and six APS patients. It revealed significant differences...... structures, as compared to controls. These data indicate that some APS patients have beta2GPI molecules with a reduced number of negatively charged sialic acid units in the glycan structure at Asn-143. This alteration of the electrostatic properties of the glycan moiety may attenuate the intramolecular...

  4. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients

    DEFF Research Database (Denmark)

    Cervera, R; Khamashta, M A; Shoenfeld, Y;

    2008-01-01

    patients with APS from 13 European countries who had been followed up from 1999 to 2004 were analysed. RESULTS: 200 (20%) patients developed APS-related manifestations during the 5-year study period. Recurrent thrombotic events appeared in 166 (16.6%) patients and the most common were strokes (2...

  5. Annexin A5 anticoagulant activity in children with systemic lupus erythematosus and the association with antibodies to domain I of β2-glycoprotein I.

    Science.gov (United States)

    Wahezi, D M; Ilowite, N T; Wu, X X; Pelkmans, L; Laat, B; Schanberg, L E; Rand, J H

    2013-06-01

    Children with systemic lupus erythematosus (SLE) have a high prevalence of antiphospholipid (aPL) antibodies and are at increased risk for aPL-related thrombosis. We investigated the association between annexin A5 anticoagulant activity and antibodies to the domain I portion of β2-glycoprotein I (anti-DI antibodies), and propose a potential mechanism for the pathogenesis of aPL-related thrombosis. Using samples from 183 children with SLE collected during the Atherosclerosis Prevention in Pediatric Lupus Erythematosus (APPLE) trial, we examined resistance to the anticoagulant effects of annexin A5, using the annexin A5 resistance (A5R) assay, and evaluated for anti-DI IgG antibodies. Children with SLE had higher frequency of anti-D1 antibodies (p = 0.014) and significantly reduced A5R compared to pediatric controls: mean A5R = 172 ± 30% versus 242 ± 32% (p antibodies had significantly lower mean A5R levels compared to those with negative anti-DI antibodies: mean A5R = 155 ± 24% versus 177 ± 30% (p antibodies (p = 0.013) and lupus anticoagulant (LA) (p = 0.036) were both independently associated with reduced A5R. Children with SLE have significantly reduced annexin A5 anticoagulant activity that is associated with the presence of LA and anti-DI antibodies.

  6. Are Toll-Like Receptors and Decoy Receptors Involved in the Immunopathogenesis of Systemic Lupus Erythematosus and Lupus-Like Syndromes?

    Directory of Open Access Journals (Sweden)

    Giuliana Guggino

    2012-01-01

    Full Text Available In this paper we focus our attention on the role of two families of receptors, Toll-like receptors (TLR and decoy receptors (DcR involved in the generation of systemic lupus erythematosus (SLE and lupus-like syndromes in human and mouse models. To date, these molecules were described in several autoimmune disorders such as rheumatoid arthritis, antiphospholipids syndrome, bowel inflammation, and SLE. Here, we summarize the findings of recent investigations on TLR and DcR and their role in the immunopathogenesis of the SLE.

  7. Monoclonal antibodies

    International Nuclear Information System (INIS)

    Monoclonal antibodies (MAbs) are antibodies having single specificity for a given antigen site (epitope). The development of hybridoma technology and the relative ease by which MAbs can be prepared has revolutionized many aspects of serological applications in diagnosis and differentiation of disease producing agents. The property of monospecificity offers advantages in diagnostic applications over polyclonal sera in that tests can be defined exactly with regard to the antigen detected and the affinity of reaction between the given antigenic site and the monoclonal reagent. In addition, MAbs offer better possibilities for test standardization, because the same reagent can be used in different laboratories. Such an MAb can be supplied by a central laboratory or 'grown' from hybridoma cells, ensuring that the resultant product is identical from laboratory to laboratory and that the part of the test involving the MAb reaction is the same. The methodologies for inoculation regimes, mice, cloning methods, selection of fusion partners, etc., have been validated extensively in developed country laboratories. The decision to establish a MAb production facility must be examined on a strict cost-benefit basis, since it is still expensive to produce a product. There are many MAbs available that should be sought to allow exploitation in developing tests. If a production facility is envisaged, it should produce reagents for national needs, i.e. there should be a clear problem oriented approach whereby exact needs are defined. In the field of veterinary applications, MAbs are the central reagent in many immunoassays based on the enzyme linked immunosorbent assay (ELISA). The development of specific tests for diagnosing diseases is dominated by MAbs and has been fuelled by a strong research base, mainly in developed countries allied to developing countries through the study of related diseases. Thus, there are very many assays dependent on MAbs, some of which form the basis of

  8. Acute renal infarction associated with homozygous methylenetetrahydrofolate reductase mutation C677T and IgA beta-2-glycoprotein antibodies.

    Science.gov (United States)

    Vlachostergios, Panagiotis J; Dufresne, François

    2015-07-01

    Arterial thrombosis of the kidney(s) is a rare clinical entity usually presenting as a result of cardioembolic disease, though rare inherited hypercoagulable states have also been implicated. Within this context, both hyperhomocysteinemia triggered by a mutated methylenetetrahydrofolate reductase (MTHFR) gene product and the presence of antiphospholipid antibodies have been separately associated with arterial thrombotic events, including renal artery embolism. We present a case of combined homozygous MTHFR C677T mutation and IgA beta-2-glycoprotein antibody positivity resulting in acute renal infarction and previous silent myocardial infarction. An acute and otherwise unexplained thrombotic event of unusual location always warrants further investigation, which should include testing for hereditary thrombophilic disorders.

  9. Morvan Syndrome

    Science.gov (United States)

    Maskery, Mark; Chhetri, Suresh K.; Dayanandan, Rejith; Gall, Claire

    2016-01-01

    A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management. PMID:26740856

  10. Yttrium Y 90 Anti-CD45 Monoclonal Antibody BC8 Followed by Donor Stem Cell Transplant in Treating Patients With High-Risk Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, or Myelodysplastic Syndrome

    Science.gov (United States)

    2016-09-29

    Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Chronic Myelomonocytic Leukemia; Previously Treated Myelodysplastic Syndrome; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Secondary Acute Myeloid Leukemia

  11. 抗磷脂综合征

    Institute of Scientific and Technical Information of China (English)

    李萍

    2003-01-01

    @@ 抗磷脂综合征(antiphospholipid syndrome,APS)的主要临床表现为反复的血管性血栓形成、自发性流产和血小板减少,并伴有血清狼疮抗凝因子(lupus anticoagulant,LA)阳性、和(或)抗磷脂抗体(antiphospholipid antibody,APL)中度或高度阳性.

  12. Monoclonal antibodies.

    Science.gov (United States)

    2009-01-01

    The ability to produce and exploit monoclonal antibodies (mAbs) has revolutionized many areas of biological sciences. The unique property of an mAb is that it is a single species of immunoglobulin (IG) molecule. This means that the specificity of the interaction of the paratopes on the IG, with the epitopes on an antigenic target, is the same on every molecule. This property can be used to great benefit in immunoassays to provide tests of defined specificity and sensitivity, which improve the possibilities of standardization. The performance of assays can often be determined relating the actual weight of antibody (hence the number of molecules) to the activity. Often the production of an mAb against a specific epitope is the only way that biological entities can be differentiated. This chapter outlines the areas involving the development of assays based on mAbs. The problems involved address include the physical aspects of mAbs and how they may affect assay design and also the implications of results based on monospecific reagents. Often these are not fully understood, leading to assays that are less than satisfactory, which does not justify the relatively high cost of preparing and screening of mAbs. There are many textbooks and reviews dealing with the preparation of mAbs, the principles involved, and various purification and manipulative methods for the preparation of fragments and conjugation. There has been little general information attempting to summarize the best approaches to assay design using mAbs. Much time can be wasted through bad planning, and this is particularly relevant to mAbs. A proper understanding of some basic principles is essential. It is beyond the scope of this chapter to discuss all aspects, but major areas are highlighted. PMID:19219589

  13. Beals Syndrome

    Science.gov (United States)

    ... Boards & Staff Annual Report & Financials Contact Us Donate Marfan & Related Disorders What is Marfan Syndrome? What are ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of ...

  14. Pathogenic effects of maternal antinuclear antibodies during pregnancy in women with lupus

    Directory of Open Access Journals (Sweden)

    Rafael Herrera-Esparza

    2014-11-01

    Full Text Available Lupus is an autoimmune disease that primarily affects young women of childbearing age. Fertility rates in lupus patients depend on various factors, including disease activity, nephritis, and the presence of antiphospholipid antibodies; however, after lupus patients become pregnant, different factors may affect the course of pregnancy, such as the production of autoantibodies, pre-existing renal disease, and eclampsia, among others. The placenta is a temporary hemochorial organ that prevents immunological conflict due to exposure to alloantigens at the maternal-fetal interface; placental regulatory T cells play a major role in maternal-fetal tolerance. Typically, significant amounts of maternal IgG class antibodies cross the placenta and enter the fetal circulation. This transition depends on the distribution of Fc receptors along the syncytiotrophoblast. The production of antinuclear antibodies (ANA is a hallmark of lupus, and these autoantibodies can form immune complexes that are typically trapped in the placenta during gestation. However, the entry of ANA into the fetal circulation depends on the IgG-ANA concentration and the FcR placental density. Maternal antinuclear antibodies with anti-Ro or anti-La specificity might be pathogenic to the fetus if transfused by the placental pathway and could induce neonatal pathologies, such as neonatal lupus and congenital heart block. Here, we review the experimental and clinical data supporting a pathogenic role for maternal autoantibodies transmitted to the fetus

  15. [Antinuclear antibodies].

    Science.gov (United States)

    Cabiedes, Javier; Núñez-Álvarez, Carlos A

    2010-01-01

    Anti-nuclear antibodies (ANA) are immunoglobulin directed against autologous cell nuclear and cytoplasmic components. Besides the autoimmune ANA there are other ANA that can be detected in circulation, like natural and infectious ANA. Because of its high sensibility, detection of the ANA must be done by indirect immunofluorescence (IIF) as screening test and all of those positive samples are convenient to confirm its specificity by ELISA, western blot or other techniques. Positive ANA detected by IIF must be evaluated taking in to account the pattern and titer. The following recommended step is the specificity characterization (reactivity against extractable nuclear antigens [ENA], dsDNA, etc.) which is useful for the diagnosis and follow up of patients with autoimmune diseases, and by such reasoning, its detection must be performed in an orderly and reasonable way using guides or strategies focused to the good use and interpretation of the autoantibodies. The objective of this review is to present a compilation of the literature and our experience in the detection and study of the ANA.

  16. A study on the expression of anti-mitochondriai antibody in the brain of patients with MELAS syndrome%抗线粒体抗体在MELAS综合征脑活检组织的表达观察

    Institute of Scientific and Technical Information of China (English)

    戚晓昆; 姚生; 王海燕; 朴月善; 卢德宏; 袁云

    2009-01-01

    Objective To investigate the pathological changes and pathogenesis of the MELAS syndrome (mitochondrial encephalopathy lactic acidosis strok-like episodes ) by using the method of immunohistochemical staining in the brain biopsy specimens with anti-mitochondrial antibody (AMA).Methods We performed immunohistochemical staining in 3 confirmed MELAS patients' paraffin-imbed brain biopsy specimens.Results Small vessel proliferation and the uneven thickness of the wall were found in the 3 MELAS patients.A lot of brown deposits was shown in the wall of small vessels and also noted in neurons.Conclusions The main pathological change in the MELAS brain biopsy immunohistochemical staining with AMA was the small vessel proliferation,indicating that abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the lesion sites.This finding was consistent with the electron microscopic discovery and valuable for the diagnosis of MELAS.%目的 对线粒体脑肌病伴随乳酸血症和卒中样发作(MELAS综合征)患者脑活检以探讨其病理变化及发病机制.方法 收集海军总医院确诊的3例MELAS综合征患者脑活检组织石蜡标本,行AMA免疫组化染色.结果 3例MELAS综合征患者脑组织AMA免疫组化染色光镜观察发现病变脑组织及蛛网膜下腔小血管异常增生,血管肇薄厚不均,可见到大量浓染的棕褐色沉积物;神经元细胞中也可见到大量浓染的棕褐色沉积物.结论 MELAS综合征患者AMA免疫组化染色的主要病理特点是病变部位血管异常增生,提示病变部位血管平滑肌、内皮细胞及神经细胞内大量异常线粒体堆积,与电镜下发现大量异常线粒体堆积对线粒体脑肌病的诊断有同样价值.

  17. Antisynthetase Syndrome: A Rare Cause for ILD

    OpenAIRE

    Devi, HJ Gayathri; Pasha, Md Majeed; Padmaja, Mantha Sathya; Halappa, Sujith

    2016-01-01

    Anti-Synthetase Syndrome (ASS) is a rare autoimmune disorder characterized by Interstitial Lung Disease (ILD), inflammatory myositis, fever, Raynaud’s phenomenon, mechanic’s hand, and inflammatory polyarthritis in the setting of antibodies against amino acyl-transfer RNA synthetases, with anti-Jo-1 antibody being the most common. It can sometimes present as interstitial lung disease without any other expression of the syndrome. Clinical and radiological features can be similar to atypical pne...

  18. Syndrome in question: antisynthetase syndrome (anti-PL-7)*

    Science.gov (United States)

    Esposito, Ana Cláudia Cavalcante; Gige, Tatiana Cristina; Miot, Hélio Amante

    2016-01-01

    Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies.

  19. Auriculotemporal Syndrome (Frey Syndrome).

    Science.gov (United States)

    Motz, Kevin M; Kim, Young J

    2016-04-01

    Frey syndrome is a common sequela of parotidectomy, and although it is not frequently manifested clinically, it can cause significant morbidity for those affected. Frey syndrome results from synkinetic autonomic reinnervation by transected postganglionic parasympathetic nerve fiber within the parotid gland to the overlying sweat glands of the skin. Many surgical techniques have been proposed to prevent the development of Frey syndrome. For those who develop clinical symptoms of Frey syndrome, objective testing can be performed with a Minor starch-iodine test. Some of the current methods to prevent and treat symptomatic Frey syndrome are reviewed. PMID:26902982

  20. Inflammatory response and the endothelium.

    Science.gov (United States)

    Meroni, P L; Borghi, M O; Raschi, E; Ventura, D; Sarzi Puttini, P C; Atzeni, F; Lonati, L; Parati, G; Tincani, A; Mari, D; Tedesco, F

    2004-01-01

    Antiphospholipid-mediated endothelium perturbation plays a role in antiphospholipid syndrome (APS)-associated vasculopathy. Antiphospholipid antibodies activate endothelium both in vitro and in vivo experimental models by inducing a pro-inflammatory/-coagulant phenotype; the antibodies recognize beta2 glycoprotein I (beta2GPI) on human endothelial cells (EC) from different parts of the vasculature. In spite of such large in vitro evidence, few studies have addressed the issue whether or not a comparable endothelial perturbation might be detectable in vivo. We investigated several indirect ex vivo parameters of endothelial dysfunction: plasma levels of soluble adhesion molecules (sADM), soluble thrombomodulin (sTM), von Willebrand factor (vWF) and tissue plasminogen activator (t-PA) by solid-phase assays. The study included: patients with primary antiphospholipid syndrome (n=32), with the syndrome secondary to non-active systemic lupus erythematosus (SLE, n=10), six patients with persistent antiphospholipid positivity at medium/high titre without any clinical manifestation of the syndrome. Fifty-two age and sex matched healthy subjects have been enrolled as controls. In addition, circulating endothelial cells identified by flow cytometry and the brachial artery flow-mediated vasodilation (FMV) were evaluated in 26 patients (20 primary and 6 lupus syndromes) and 30 healthy controls. Plasma levels of soluble adhesion molecules did not differ from controls, while a significant increase in von Willebrand factor titres (P<0.05) was found. No significant difference was found regarding the number of circulating endothelial cells and flow-mediated vasodilation. As a whole, these findings do suggest that antiphospholipid antibodies per se are not able to support a full-blown endothelial perturbation in vivo. As shown in antiphospholipid syndrome experimental animal models, a two-hit hypothesis is suggested.

  1. Iodine I 131 Monoclonal Antibody BC8, Fludarabine Phosphate, Cyclophosphamide, Total-Body Irradiation and Donor Bone Marrow Transplant in Treating Patients With Advanced Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, or High-Risk Myelodysplastic Syndrome

    Science.gov (United States)

    2016-07-18

    Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Chronic Myelomonocytic Leukemia; Previously Treated Myelodysplastic Syndrome; Refractory Anemia With Excess Blasts; Refractory Anemia With Ring Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Refractory Cytopenia With Multilineage Dysplasia and Ring Sideroblasts

  2. Paraneoplastic syndromes and the kidney

    Directory of Open Access Journals (Sweden)

    de Oliveira Filgueira Pedro Henrique

    2010-01-01

    Full Text Available A paraneoplastic syndrome is defined as a group of symptoms that develop when substances released by some cancer cells disrupt the normal function of the surrounding cells and tissue. Paraneoplastic renal syndromes are diseases that indirectly compromise tubular and glomerular function by electrolyte imbalance, hormone-producing tumors or deposition of antigen-antibody complexes in the glomeruli. In order to describe the most common paraneoplastic syndromes, which may compromise the renal function, an extensive review was performed of papers, including case reports, guidelines, meta-analysis and other scientific publications. Renal function can be affected by many paraneoplactic syndromes: hypercalcemia in malignancies, syndrome of inappropriate sec-retion of antidiuretic hormone, tumor lysis syndrome, renin-producing tumors and paraneoplastic glomerulopathies. An early diagnosis and effective treatment might improve quality of life and alter prognosis of these patients.

  3. Measurement of Serum Anti-Myelin Antibody and Its Clinical Significance in Patients with Guillain-Barré Syndrome%格林-巴利综合征患者血清抗髓鞘脂抗体水平测定及临床意义

    Institute of Scientific and Technical Information of China (English)

    李玉芬; 崔黎黎; 戚其学

    2013-01-01

      目的探讨抗髓鞘脂抗体在格林-巴利综合征(Guillain-Barré syndrome,GBS)发病机制中的作用及其临床意义。方法用ELISA方法测定50例GBS患者和40名正常对照组血清中抗髓鞘脂抗体含量,并对其中28例患者进行动态观察。结果GBS患者血清中抗髓鞘脂抗体水平显著高于正常对照组(0.34±0.16 vs 0.22±0.08,P<0.01),GBS患者抗髓鞘脂抗体的阳性率为20%,动态观查发现GBS患者血清中抗髓鞘脂抗体水平的下降与临床症状的改善相一致。结论血清抗髓鞘脂抗体水平升高在GBS的发病机制中具有重要意义,其水平变化的观测对GBS的诊断、治疗及预防具有一定的临床意义。%Objective To investigate the roles of serum anti-myelin antibody in the pathogenesis of Guillain-Barré syndrome(GBS).Methods Serum anti-myelin antibody levels of 50 GBS patients and 40 normal controls were measured by ELISA,and dynamic changes of anti-myelin antibody levels in 28 GBS patients were also observed.Results Serum anti-myelin antibody levels in GBS patients were higher than those in normal controls(0.34±0.16 vs 0.22±0.08,P<0.01).Positive rate of serum anti-myelin antibody in GBS patients was 20%.Decline of serum anti-myelin levels was in parallel with symptom improvement in GBS patients.Conclusions Higher serum anti-myelin antibody level played an important role in the pathogenesis of GBS.Dynamic change of serum anti-myelin antibody level was important for the diagnosis,treatment and prevention of GBS.

  4. A cardiorenal-pulmonary-cutaneous-muscle syndrome

    OpenAIRE

    Schöpp, Sebastian; Elitok, Saban; Bieringer, Markus; Schneider, Wolfgang; Luft, Friedrich C.

    2013-01-01

    Anti-synthetase syndrome is a relatively recently described auto-immune disease characterized by auto-antibodies to enzymes that acetylate transfer RNA (tRNA). Interstitial pulmonary disease and inflammatory myopathy are regular findings. Our patient also exhibited a lupus-like glomerulonephritis. An important clue was the presence of ‘mechanics’ hands. Nephrologists need to be aware of this syndrome.

  5. Prevention of reovirus type 2-induced diabetes-like syndrome in DBA/1 suckling mice by treatment with antibodies against intercellular adhesion molecule-1 and lymphocyte function-associated antigen-1.

    OpenAIRE

    Hayashi, T.; Yamamoto, S.; Onodera, T.

    1995-01-01

    Reovirus type 2-induced diabetes-like syndrome in suckling mice is considered to be an animal model for human insulin-dependent diabetes mellitus. We have previously demonstrated that immunopathologic pancreatic islet cell damage might be relevant to reovirus type 2 infection. In this study the involvement of adhesion molecules in the development of reovirus type 2-induced diabetes-like syndrome was examined. In infected mice infiltration of mononuclear cells mixed with polymorphonuclear leuc...

  6. The detection of antibodies to ganglioside in patient with Guillain-Barré syndrome%吉兰-巴雷综合征患者血清和脑脊液抗神经节苷脂抗体检测的意义

    Institute of Scientific and Technical Information of China (English)

    杨奎; 李晓光; 陈军; 刘宁; 鲁雅琴; 陈琳

    2012-01-01

    目的 通过测定吉兰-巴雷综合征( Guillain-Barré Syndrome,GBS)患者血清和脑脊液中抗神经节苷脂GMI抗体水平,探讨其临床意义.方法 应用ELISA法检测44例GBS患者血清和脑脊液中抗GM1抗体水平.结果 (1) GBS组血清和脑脊液中抗GMI-IgG抗体水平与其他神经疾病(OND)组、正常对照(NC)组相比有显著性差异(P<0.05);血清中抗GMI -IgM抗体与NC组相比有显著性差异(P<0.05),但与OND组相比无显著性差异(P>0.05);脑脊液中抗GMI -IgM抗体与OND组、NC组相比有显著性差异(P<0.05).(2)轻重型GBS患者血清和脑脊液中抗GM1抗体的阳性率差异无显著性(P>0.05).(3)急性炎性脱髓鞘性多发性神经根神经病(AIDP)患者血清和脑脊液中抗GM1抗体阳性率与急性运动轴索型神经病(AMAN)及急性运动感觉轴索型神经病( AMSAN)中抗GMl抗体阳性率者相比无显著性差异(P>0.05).结论 抗GM1抗体可能在吉兰-巴雷综合征的发病中起重要作用,但抗体水平与疾病严重程度无关.%To study the clinical significance of antibody to GM1 in sera and CSF of patients with Guil-lain-Barre syndrome( GBS). Method The levels of antibody to GM1 in sera and CSF of 44 patients with GBS were detected by enzyme-linked immunosorbent assay(ELBA). Result Compared to normal control and patient with other neurological disease, the frequency of the antibody to GM1 in sera and CSF were much higher in GBS group( P 0.05). Conclusion The study demonstrates that the higher frequency of antibodies to GMl-IgG both in sera and CSF of patient with GBS indicate that antibody to GM1 might play an important role in pathogenesis of Guillain-Barr£ syndrome, while antibodies to GM1 were not associated with a worse prognosis.

  7. 77 FR 9678 - Prospective Grant of Exclusive License: The Development of Human Anti-CD22 Monoclonal Antibodies...

    Science.gov (United States)

    2012-02-17

    ... disease such as lupus and Sjogren's syndrome. The specific antibodies covered by this technology are... Human Anti-CD22 Monoclonal Antibodies for the Treatment of Human Cancers and Autoimmune Disease AGENCY... Antibodies Specific for CD22'' , PCT Application PCT/US2009/124109 entitled ``Human and Improved...

  8. Bi Syndrome (Arthralgia Syndrome)

    Institute of Scientific and Technical Information of China (English)

    ZHANG En-qin

    2010-01-01

    @@ The word 'Bi' (痹) in Chinese means an obstruction.Bi Syndrome refers the syndrome characterized by the obstruction of qi and blood in the meridians due to the invasion of external pathogenic wind, cold and dampness, manifested as soreness, pain, numbness,heavy sensation, swelling of joints and limbs, limitation of movements and so on.

  9. Monoclonal antibodies and cancer

    International Nuclear Information System (INIS)

    The usefulness of radiolabeled monoclonal antibodies for imaging and treatment of human (ovarian) cancer was investigated. A review of tumor imaging with monoclonal antibodies is presented. Special attention is given to factors that influence the localization of the antibodies in tumors, isotope choice and methods of radiolabeling of the monoclonal antibodies. Two monoclonal antibodies, OC125 and OV-TL3, with high specificity for human epithelial ovarian cancer are characterized. A simple radio-iodination technique was developed for clinical application of the monoclonal antibodies. The behavior of monoclonal antibodies in human tumor xenograft systems and in man are described. Imaging of tumors is complicated because of high background levels of radioactivity in other sites than the tumor, especially in the bloodpool. A technique was developed to improve imaging of human tumor xenographs in nude mice, using subtraction of a specific and a non-specific antibody, radiolabeled with 111In, 67Ga and 131I. To investigate the capability of the two monoclonal antibodies, to specifically localize in human ovarian carcinomas, distribution studies in mice bearing human ovarian carcinoma xenografts were performed. One of the antibodies, OC125, was used for distribution studies in ovarian cancer patients. OC125 was used because of availability and approval to use this antibody in patients. The same antibody was used to investigate the usefulness of radioimmunoimaging in ovarian cancer patients. The interaction of injected radiolabeled antibody OC125 with circulating antigen and an assay to measure the antibody response in ovarian cancer patients after injection of the antibody is described. 265 refs.; 30 figs.; 19 tabs

  10. 抗磷脂抗体(APA)对人和动物生殖的影响%The Effect of Antiphospholipid Antibodies (APA) on Reproduction of Human and Animals

    Institute of Scientific and Technical Information of China (English)

    赫晓燕; 范瑞文

    2003-01-01

    有关研究表明,动物许多自身免疫性疾病或免疫功能异常能够影响妊娠,与自身抗体--抗磷脂抗体( APA)有关.文章就抗磷脂抗体( APA)的种类、性质、作用机制、对人和动物生殖的影响进行了综述.

  11. Relationship between the pregnancy and the antipbospholipid antibody in systemic lupus erythematosus%SLE患者妊娠中抗磷脂抗体与妊娠的关系

    Institute of Scientific and Technical Information of China (English)

    章群

    2008-01-01

    系统性红斑狼疮(SLE)是累及全身多系统的自身免疫性疾病,好发于育龄期妇女.患者妊娠期间抗磷脂抗体滴度的变化是不良妊娠结局可能的危险因素.抗磷脂抗体与凝血失衡,缺陷胎盘的产生、补体激活以及相关蛋白间的交互作用等是导致母体和胎儿损伤的重要因素.%Systemic Lupus Erythrematosus(SLE)is a common autoimmune disease,affecting several organs.Women in childbearing years are frequently affected.Positive for antiphospholipid antibody is a probable risk factor to bad pregnancy outcome in pregnant woman with SLE.Antiphospholipid antibody,disequilibrium in blood clotting mechanism,placental defect and the interaction among them ale important factors for the damage to patients and fetuses.

  12. Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?

    OpenAIRE

    Hongliang Zhang; Chunkui Zhou; Limin Wu; Fengming Ni; Jie Zhu; Tao Jin

    2013-01-01

    Paraneoplastic neurological syndromes (PNSs) occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, especially small cell lung cancer (SCLC), lymphoma, and gynecological tumors. The finding of highly specific antibodies directed against onconeural antigens has revolutionized the diagnosis and promot...

  13. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, ...

  14. [Neurological syndromes, encephalitis].

    Science.gov (United States)

    Yamamoto, Tomotaka; Tsuji, Shoji

    2010-06-01

    The remote effects of malignant tumors in most cases of paraneoplastic neurological syndromes(PNS)are mediated by autoimmune processes against antigens shared by the tumor cells and the nervous tissue(onconeural antigens). Onconeural (or paraneoplastic)antibodies are broadly categorized into two groups according to the location of the corresponding onconeural antigens, inside or on the surface of neurons. Antibodies established as clinically relevant diagnostic markers for PNS are designated as well-characterized onconeural antibodies (or classical antibodies)that target intracellular antigens(Hu, Yo, Ri, CV2/CRMP5,Ma2, and amphiphysin). They also serve as useful markers in detecting primary tumors. Recent identification of new antibodies as markers of subtypes of limbic encephalitis has also expanded the concept of autoimmune limbic encephalitis. These autoantibodies are directed to neuronal cell-surface antigens including neurotransmitter receptors(NMDA, AMPA, and GABAB receptors)and ion channels(VGKC). They are less frequently associated with cancer, so that they cannot be used as specific markers for PNS. Autoimmune limbic encephalitis with anti-neuronal cell surface antobodies and paraneoplastic limbic encephalitis with classical antibodies overlap in some clinical features but are pathophysiologically distinct. Classical antibodies are not simple tumor markers. They seem to be closely related to the disease mechanisms because specific intrathecal synthesis has been shown in PNS patients. However, attempts to produce an animal model of PNS by passive transfer of these antibodies have been unsuccessful, and there is no direct evidence demonstrating the pathogenic role of classical antibodies. Instead, some circumstantial evidence, including pathological studies showing extensive infiltrates of T cells in the CNS of the patients, supports the hypothesis that cytotoxic-T cell mechanisms cause irreversible neuronal damage. On the other hand, humoral immune

  15. Factor V G1691A (Leiden is a major etiological factor in Egyptian Budd-Chiari syndrome patients

    Directory of Open Access Journals (Sweden)

    Tawhida Y. Abdel Ghaffar

    2011-12-01

    Full Text Available Objective: Budd-Chiari syndrome is a multifactorial disease in which several prothrombotic disorders may predispose patients to the development of thrombosis at this uncommon location (hepatic veins. The aim of this study was to determine the prevalence and characteristics of inherited thrombophilia in Egyptian Budd-Chiari syndrome patients.Materials and Methods: The study included 47 Budd-Chiari syndrome patients (20 children and 27 adults. Genotyping of Factor V G1691A (Leiden, prothrombin G20210A (PT, and methylenetetrahydrofolate reductase C677T were performed using real-time PCR and fluorescence melting curve detection analysis.Results: Factor V Leiden was observed in 29 patients (61.7%. It is the only factor that caused Budd-Chiari syndrome in 18 of the patients and in 5 of the patients with inferior vena cava involvement. Myeloproliferative disease was noted in 12 (25.5% patients, antiphospholipid syndrome in 5 (10.6%, and Behcet’s disease in 3 (6.4%. Interestingly, 3 of the children with Budd-Chiari syndrome had lipid storage disease.Conclusion: Factor V Leiden was a major etiological factor in Egyptian Budd-Chiari syndrome patients, which may have been related to the high frequency of this mutation in the study region. Factor V Leiden was also a strong thrombophilic factor and the leading cause of inferior vena cava thrombosis in these patients. Lipid storage disease should be included as a risk factor for Budd-Chiari syndrome.

  16. Iodine I 131 Monoclonal Antibody BC8, Fludarabine Phosphate, Total Body Irradiation, and Donor Stem Cell Transplant Followed by Cyclosporine and Mycophenolate Mofetil in Treating Patients With Advanced Acute Myeloid Leukemia or Myelodysplastic Syndrome

    Science.gov (United States)

    2015-11-16

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes

  17. Radiolabeled Monoclonal Antibody Therapy, Fludarabine Phosphate, and Low-Dose Total-Body Irradiation Followed by Donor Stem Cell Transplant and Immunosuppression Therapy in Treating Older Patients With Advanced Acute Myeloid Leukemia or High-Risk Myelodysplastic Syndromes

    Science.gov (United States)

    2015-11-16

    Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Secondary Myelodysplastic Syndromes; Untreated Adult Acute Myeloid Leukemia

  18. Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy

    OpenAIRE

    Ogata, Hidenori; Yamasaki, Ryo; Hiwatashi, Akio; Oka, Nobuyuki; Kawamura, Nobutoshi; Matsuse, Dai; Kuwahara, Motoi; Suzuki, Hidekazu; Kusunoki, Susumu; Fujimoto, Yuichi; Ikezoe, Koji; Kishida, Hitaru; Tanaka, Fumiaki; Matsushita, Takuya; Murai, Hiroyuki

    2015-01-01

    Objective To investigate anti-neurofascin 155 (NF155) antibody-positive chronic inflammatory demyelinating polyneuropathy (CIDP). Methods Sera from 50 consecutive CIDP patients diagnosed in our clinic, 32 patients with multiple sclerosis, 40 patients with other neuropathies including 26 with Guillain–Barré syndrome (GBS)/Fisher syndrome, and 30 healthy controls were measured for anti-NF antibodies by flow cytometry using HEK293 cell lines stably expressing human NF155 or NF186. Four additiona...

  19. [A spectrum of neurological diseases with anti-VGKC antibody].

    Science.gov (United States)

    Arimura, Kimiyoshi; Watanabe, Osamu; Nagado, Tatsui

    2007-11-01

    Anti-VGKC antibody causing peripheral nerve hyperexcitability is already an established clinical entity. Recently, many patients with non-herpetic limbic encephalitis (NHLE) with anti-VGKC antibody have been reported. The characteristic clinical features are low serum Na+ concentration and good response to immunotherapy. Anti-VGK antibody positive NHLE is relatively frequent among immune-mediated NHLE. It is important to know that this disease is responsive to immunotherapy. Furthermore, anti-VGKC antibody is also positive in some intractable epilepsies. These findings suggest that anti-VGKC is correlated with hyperexcitability in both the peripheral and central nervous system and that the spectrum of anti-VGKC antibody syndrome is now expanding.

  20. Sustained systemic delivery of monoclonal antibodies by genetically modified skin fibroblasts

    DEFF Research Database (Denmark)

    Noël, D; Pelegrin, M; Brockly, F;

    2000-01-01

    In vivo production and systemic delivery of therapeutic antibodies by engineered cells might advantageously replace injection of purified antibodies for treating a variety of life-threatening diseases, including cancer, acquired immunodeficiency syndrome, and autoimmune diseases. We report here...... that skin fibroblasts retrovirally transduced to express immunoglobulin genes can be used for sustained long-term systemic delivery of cloned antibodies in immunocompetent mice. Importantly, no anti- idiotypic response against the ectopically expressed model antibody used in this study was observed....... This supports the notion that skin fibroblasts can potentially be used in antibody-based gene/cell therapy protocols without inducing any adverse immune response in treated individuals....

  1. Heavy chain only antibodies

    DEFF Research Database (Denmark)

    Moghimi, Seyed Moein; Rahbarizadeh, Fatemeh; Ahmadvand, Davoud;

    2013-01-01

    Unlike conventional antibodies, heavy chain only antibodies derived from camel contain a single variable domain (VHH) and two constant domains (CH2 and CH3). Cloned and isolated VHHs possess unique properties that enable them to excel conventional therapeutic antibodies and their smaller antigen-...... for combating HER2+ breast cancer. © 2013 by Tabriz University of Medical Sciences.......Unlike conventional antibodies, heavy chain only antibodies derived from camel contain a single variable domain (VHH) and two constant domains (CH2 and CH3). Cloned and isolated VHHs possess unique properties that enable them to excel conventional therapeutic antibodies and their smaller antigen......-binding fragments in cancer targeting and therapy. VHHs express low immunogenicity, are highly robust and easy to manufacture and have the ability to recognize hidden or uncommon epitopes. We highlight the utility of VHH in design of new molecular, multifunctional particulate and immune cell-based systems...

  2. Engineering antibody therapeutics.

    Science.gov (United States)

    Chiu, Mark L; Gilliland, Gary L

    2016-06-01

    The successful introduction of antibody-based protein therapeutics into the arsenal of treatments for patients has within a few decades fostered intense innovation in the production and engineering of antibodies. Reviewed here are the methods currently used to produce antibodies along with how our knowledge of the structural and functional characterization of immunoglobulins has resulted in the engineering of antibodies to produce protein therapeutics with unique properties, both biological and biophysical, that are leading to novel therapeutic approaches. Antibody engineering includes the introduction of the antibody combining site (variable regions) into a host of architectures including bi and multi-specific formats that further impact the therapeutic properties leading to further advantages and successes in patient treatment. PMID:27525816

  3. Postural Orthostatic Tachycardia With Chronic Fatigue After HPV Vaccination as Part of the "Autoimmune/Auto-inflammatory Syndrome Induced by Adjuvants": Case Report and Literature Review.

    Science.gov (United States)

    Tomljenovic, Lucija; Colafrancesco, Serena; Perricone, Carlo; Shoenfeld, Yehuda

    2014-01-01

    We report the case of a 14-year-old girl who developed postural orthostatic tachycardia syndrome (POTS) with chronic fatigue 2 months following Gardasil vaccination. The patient suffered from persistent headaches, dizziness, recurrent syncope, poor motor coordination, weakness, fatigue, myalgias, numbness, tachycardia, dyspnea, visual disturbances, phonophobia, cognitive impairment, insomnia, gastrointestinal disturbances, and a weight loss of 20 pounds. The psychiatric evaluation ruled out the possibility that her symptoms were psychogenic or related to anxiety disorders. Furthermore, the patient tested positive for ANA (1:1280), lupus anticoagulant, and antiphospholipid. On clinical examination she presented livedo reticularis and was diagnosed with Raynaud's syndrome. This case fulfills the criteria for the autoimmune/auto-inflammatory syndrome induced by adjuvants (ASIA). Because human papillomavirus vaccination is universally recommended to teenagers and because POTS frequently results in long-term disabilities (as was the case in our patient), a thorough follow-up of patients who present with relevant complaints after vaccination is strongly recommended.

  4. Importance of anticomplement immunofluorescence antibody titration for diagnosing varicella-zoster virus infection in Bell's palsy.

    OpenAIRE

    Shigeta, S; Baba, M.; Ogata, M; Nozaki, H.; Okuaki, A; Nakamura, S.

    1986-01-01

    Anticomplement Immunofluorescence was used for antibody titration against varicella-zoster virus (VZV) in 43 patients with peripheral facial palsy. Nine of 31 patients (29%) with Bell's palsy and eight of 12 patients (75%) with Ramsey-Hunt syndrome had anticomplement immunofluorescence antibody titres of greater than or equal to 1/10. On the other hand, none of 14 patients with herpes simplex virus (HSV) infection and 51 healthy adults showed anticomplement immunofluorescence antibody titres ...

  5. RBC Antibody Screen

    Science.gov (United States)

    ... the baby is Rh-positive and the mother's antibody status is negative for anti-D, the mother is given additional RhIG. This test also may be used to help diagnose autoimmune-related hemolytic anemia ... when a person produces antibodies against his or her own RBC antigens. This ...

  6. Nephrotic syndrome

    Science.gov (United States)

    ... the following: Antinuclear antibody Cryoglobulins Complement levels Glucose tolerance test Hepatitis B and C antibodies HIV test ... PhD, and the A.D.A.M. Editorial team. Related MedlinePlus Health Topics Kidney Diseases Browse the ...

  7. Selection of Recombinant Human Antibodies.

    Science.gov (United States)

    Tomszak, Florian; Weber, Susanne; Zantow, Jonas; Schirrmann, Thomas; Hust, Michael; Frenzel, André

    2016-01-01

    Since the development of therapeutic antibodies the demand of recombinant human antibodies is steadily increasing. Traditionally, therapeutic antibodies were generated by immunization of rat or mice, the generation of hybridoma clones, cloning of the antibody genes and subsequent humanization and engineering of the lead candidates. In the last few years, techniques were developed that use transgenic animals with a human antibody gene repertoire. Here, modern recombinant DNA technologies can be combined with well established immunization and hybridoma technologies to generate already affinity maturated human antibodies. An alternative are in vitro technologies which enabled the generation of fully human antibodies from antibody gene libraries that even exceed the human antibody repertoire. Specific antibodies can be isolated from these libraries in a very short time and therefore reduce the development time of an antibody drug at a very early stage.In this review, we describe different technologies that are currently used for the in vitro and in vivo generation of human antibodies. PMID:27236551

  8. Anti-insulin antibody test

    Science.gov (United States)

    Insulin antibodies - serum; Insulin Ab test; Insulin resistance - insulin antibodies; Diabetes - insulin antibodies ... You appear to have an allergic response to insulin Insulin no longer seems to control your diabetes

  9. 组蛋白诱导抗核抗体产生及其对肾脏损害%Induction of antinuclear antibodies and systemic lupus erythematosus like syndrome by immunization with histone

    Institute of Scientific and Technical Information of China (English)

    马爱妞; 吴厚生

    2001-01-01

    目的 寻找系统性红斑狼疮中诱导抗核抗体(ANA)生成的真正的自身核成分免疫原。方法 从ConA活化的脾淋巴细胞中提取组蛋白免疫同系BALB/c小鼠,ELISA方法测定IgG类抗组蛋白、抗dsDNA抗体,免疫荧光法检测抗核抗体核型和免疫复合物在肾小球内的沉积,免疫印迹法测定抗可溶性核抗原抗体,考马斯亮蓝法检测尿蛋白含量,光镜下观察肾脏形态变化,电镜下观察肾小球电子致密物。结果 活性组蛋白诱导IgG类抗组蛋白、抗dsDNA等多种抗核抗体生成,且诱发同系小鼠产生SLE样肾脏病理变化。结论 活性组蛋白是诱发抗核抗体生成、造成肾损害的免疫原之一。%Objective To identify the immunogen capable of inducingproduction of antinuclear antibodies and triggering renal injury. Methods BALB/c mice were immunized with active histone purified from syngeneic spleen cells following Con A activation. The patterns of antinuclear antibodies and immune complexes in glomeruli were observed by immunofluorescence staining. The antibodies against determinants of extractable nuclear antigen (ENA) were demonstrated by immunoblot assay. The dense deposits in glomeruli and kidney injury were observed under electron microscopy and microscopy. Results Active histone induced the production of anti-histone, anti-dsDNA, anti-Ro/SS-A, anti-La/SS-B and anti-J0-1. The nuclear patterns of antinuclear antibodies included homogeneous, speckled, centromeric, peripheral, cytoplasmic, nucleolar patterns, etc. The mice immunized with active histone had the glomerulonephritis and the deposits of IgG immune complex in the glomeruli and in the matrix of the mesangial region and the protein urea. However, the mice immunized with resting histone, which was purified from the syngeneic spleen cells without Con A stimulation failed to do the same. Conclusion Active histone could be one of the immunogens driving the production of anti

  10. Effects of anti-Hu antibodies from patients with paraneoplastic neurological syndrome on expression of fetal rats cultured neurons N-cadherin%观察副肿瘤神经综合征患者抗Hu抗体对培养胎鼠神经细胞N-cadherin的表达及意义

    Institute of Scientific and Technical Information of China (English)

    丁欣; 赵雷; 姜志茹; 王汐; 于洁; 尹美珺

    2011-01-01

    Objective In this study, we examined the effects of the anti-Hu autoantibodies on primary-cultured neurons N-cadherin of fetal rats in patients with paraneoplastic neurological syndromes to explore the etiology of immunopathogenesis. Methods These effects of serum IgG fractions ( from the health persons with AB type and the anti-Hu antibody negative persons without neurological symptoms) containing the anti-Hu antibody on the cultured neurons on mouse-brain-derived neurons in a primary culture system, observing the transmutation of cell and finding the target antigens of N-cadherin were expressed by immunohistochemistry. Result The serum IgGs from patients with PNS and anti-Hu antibody showed that cells were integrated and connected with long and thick dendrites in IgG group. The target antigens were expressed of N-cadherin by immunohistochemistry. However, there was no direct proof of above transmutation of cells. No apoptotic neurons were found and no cell surface were stained by the cell adhesion factor of N-cadherin. Conclusion The serum IgG fractions contained the anti-Hu antibody on the cultured neurons, suggests that their effects are through the binding of the certain antibody to affect the normal function of neuronal cell. This finding indicates that effects are not through the active immunization with Hu antibody, but by some other factors contained in IgG fractions.%目的 观察副肿瘤神经综合征(PNS)患者自身抗体(抗Hu抗体)对胎鼠培养神经细胞的直接影响及N-cadherin的表达,探讨PNS免疫学的发病机制.方法 实验组抗Hu抗体阳性PNS患者血清IgG、对照组血清IgG添加到由胎儿鼠脑分离出的培养神经细胞,观察细胞的变化,通过免疫组化观察N-cadherin的表达.结果 实验组添加抗Hu抗体阳性IgG的培养神经细胞可见细胞互相融合,突起出现粗而长相互连接的形态变化,免疫组化发现大量N-cadherin的表达;而对照组未看到上述细胞形态的变化,

  11. Laboratory and Clinic of Anti-phosphatide Antobody Syndrome%抗磷脂抗体综合征的实验与临床

    Institute of Scientific and Technical Information of China (English)

    胡俊; 李新蕊

    2005-01-01

    抗磷脂抗体(antiphospholipid antibody,APA)的本质仍不清楚,但大多学者认为是一组酸性磷脂的异质性自身抗体的总称.它包括狼疮抗凝因子(lupus anticoagulant,LA)、抗心磷脂抗体(anticardiolipin antibodies,ACA)两大类,抗磷脂酸抗体、抗磷脂酰丝氨酸抗体、抗磷脂酰乙醇胺抗体、抗磷脂酰胆碱抗体、抗磷脂酰肌醇抗体等均可归于这两大类中.

  12. Anti-B cell antibody therapies for inflammatory rheumatic diseases

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Jayne, David R W

    2014-01-01

    Several monoclonal antibodies targeting B cells have been tested as therapeutics for inflammatory rheumatic diseases. We review important observations from randomized clinical trials regarding the efficacy and safety of anti-B cell antibody-based therapies for rheumatoid arthritis, systemic lupus...... erythematosus, antineutrophil cytoplasmic antibody-associated vasculitis, polymyositis/dermatomyositis, and primary Sjögren's syndrome. For some anti-B cell agents, clinical benefits have been convincingly demonstrated, while other B cell-targeted therapies failed to improve outcomes when added to standard...... and functions in rheumatic disorders. Future studies should also evaluate how to maintain disease control by means of conventional and/or biologic immunosuppressants after remission-induction with anti-B cell antibodies....

  13. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  14. Cushing's Syndrome

    Science.gov (United States)

    Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone ... cause your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

  15. Cushing's Syndrome

    Science.gov (United States)

    ... Cushing's syndrome, also called hypercortisolism , is a rare endocrine disorder caused by chronic exposure of the body's tissues ... removing the tumor while minimizing the chance of endocrine deficiency or long-term ... for Cushing's Syndrome Clinical Trials ...

  16. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of ...

  17. National Down Syndrome Society

    Science.gov (United States)

    ... info@ndss.org Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ... More » Follow us Down Syndrome What Is Down Syndrome? Down Syndrome Facts Myths & Truths Preferred Language Guide Q& ...

  18. Learning about Marfan Syndrome

    Science.gov (United States)

    ... genetic terms used on this page Learning About Marfan Syndrome What is Marfan syndrome? What are the ... Syndrome Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most ...

  19. HIV Antibody Test

    Science.gov (United States)

    ... be limited. Home Visit Global Sites Search Help? HIV Antibody and HIV Antigen (p24) Share this page: Was this page helpful? Also known as: HIV Screening Tests; AIDS Test; AIDS Screen; HIV Serology; ...

  20. Antinuclear antibody panel

    Science.gov (United States)

    ... blood may be due to: Chronic liver disease Collagen vascular disease Drug-induced lupus erythematosus Myositis (inflammatory muscle disease) ... Saunders; 2011:chap 51. Read More Antibody Arthritis Collagen vascular disease Drug-induced lupus erythematosus Liver disease Scleroderma Systemic ...

  1. Anti-cartilage antibody.

    Science.gov (United States)

    Greenbury, C L; Skingle, J

    1979-08-01

    Antibody to cartilage has been demonstrated by indirect immunofluorescence on rat trachea in the serum of about 3% of 1126 patients with rheumatoid arthritis. Titres ranged from 1:20 to 1:640. The antibody was not found in 284 patients with primary or secondary osteoarthritis or in 1825 blood donors, nor, with the exception of two weak reactors, in 1314 paraplegic patients. In most cases the antibody appears to be specific for native type II collagen. Using this as an antigen in a haemagglutination test 94% of anti-cartilage sera were positive, whereas among 100 rheumatoid control sera there were only three weak positives. More than 80% of patients with antibody had some erosion of articular cartilage, but there was no correlation with age, sex, duration of disease, nor any recognisable clinical event or change.

  2. Incidence of anticardiolipin antibodies and lupus anticoagulant factor among women experiencing unexplained recurrent abortion and intrauterine fetal death.

    Science.gov (United States)

    Al Jameil, Noura; Tyagi, Poonam; Al Shenefy, Amal

    2015-01-01

    The aim of this research study was to estimate anticardiolipin (IgG & IgM) antibodies (aCL) and lupus anticoagulant (LA) factor in patients of recurrent unexplained pregnancy loss and intrauterine fetal deaths (IUFD). 82 women were selected for this study by virtue of having more than two consecutive unexplained pregnancy losses in their first trimester and were referred by the department of Obstetrics and Gynecology, King Saud Medical City Hospital, Riyadh, KSA. All patients had gone through a standardized investigation sequence. Lupus anticoagulants and Anticardiolipin antibodies (IgM and IgG) were detected in the serum by the enzyme linked immunosorbent assay method. To check the significance of aCL and LA, two-tailed t-test was done. Non parametric data was calculated either by Chi-Square test or Fischer exact test when relevant. Total 82 females grouped as 52 cases of recurrent (≥2) mainly first and second trimester miscarriage and 30 cases of recurrent (≥2) late intrauterine fetal death. Lupus anticoagulants was observed in twenty one (21) cases (25.6%) while anticardiolipin antibodies IgM and or IgG positive cases were estimated in forty four (44) cases (53.65%). The prevalence of APS in both studied group was thirty five (35) cases (42.68%). Antiphospholipid antibodies are calculated as the most important reason for recurrent abortion. The patients with unexplained recurrent pregnancy loss must be advised to go for a screening test for all this aPL antibodies.

  3. Antibody tumor penetration

    Science.gov (United States)

    Thurber, Greg M.; Schmidt, Michael M.; Wittrup, K. Dane

    2009-01-01

    Antibodies have proven to be effective agents in cancer imaging and therapy. One of the major challenges still facing the field is the heterogeneous distribution of these agents in tumors when administered systemically. Large regions of untargeted cells can therefore escape therapy and potentially select for more resistant cells. We present here a summary of theoretical and experimental approaches to analyze and improve antibody penetration in tumor tissue. PMID:18541331

  4. Expression of Recombinant Antibodies

    OpenAIRE

    Frenzel, André; Hust, Michael; Schirrmann, Thomas

    2013-01-01

    Recombinant antibodies are highly specific detection probes in research, diagnostics, and have emerged over the last two decades as the fastest growing class of therapeutic proteins. Antibody generation has been dramatically accelerated by in vitro selection systems, particularly phage display. An increasing variety of recombinant production systems have been developed, ranging from Gram-negative and positive bacteria, yeasts and filamentous fungi, insect cell lines, mammalian cells to transg...

  5. Dumping Syndrome

    Science.gov (United States)

    ... Disease Organizations​​ (PDF, 341 KB)​​​​​ Alternate Language URL Dumping Syndrome Page Content On this page: What is ... Nutrition Points to Remember Clinical Trials What is dumping syndrome? Dumping syndrome occurs when food, especially sugar, ...

  6. Antibody informatics for drug discovery

    DEFF Research Database (Denmark)

    Shirai, Hiroki; Prades, Catherine; Vita, Randi;

    2014-01-01

    for antibody rational design using computational approaches to affinity and stability improvement, as well as ab-initio and homology-based antibody modeling; (ii) resources for antibody sequences, structures, and immune epitopes and open drug discovery resources for development of antibody drugs; and (iii...

  7. Neonatal lupus syndromes.

    Science.gov (United States)

    Buyon, J P; Rupel, A; Clancy, R M

    2004-01-01

    The neonatal lupus syndromes (NLS), while quite rare, carry significant mortality and morbidity in cases of cardiac manifestations. Although anti-SSA/Ro-SSB/La antibodies are detected in > 85% of mothers whose fetuses are identified with congenital heart block (CHB) in a structurally normal heart, when clinicians applied this testing to their pregnant patients, the risk for a woman with the candidate antibodies to have a child with CHB was at or below 1 in 50. While the precise pathogenic mechanism of antibody-mediated injury remains unknown, it is clear that the antibodies alone are insufficient to cause disease and fetal factors are likely contributory. In vivo and in vitro evidence supports a pathologic cascade involving apoptosis of cardiocytes, surface translocation of Ro and La antigens, binding of maternal autoantibodies, secretion of profibrosing factors (e.g., TGFbeta) from the scavenging macrophages and modulation of cardiac fibroblasts to a myofibroflast scarring phenotype. The spectrum of cardiac abnormalities continues to expand, with varying degrees of block identified in utero and reports of late onset cardiomyopathy (some of which display endocardial fibroelastosis). Moreover, there is now clear documentation that incomplete blocks (including those improving in utero with dexamethasone) can progress postnatally, despite the clearance of the maternal antibodies from the neonatal circulation. Better echocardiographic measurements which identify first degree block in utero may be the optimal means of approaching pregnant women at risk. Prophylactic therapies, including treatment with intravenous immunoglobulin, await larger trials. In order to achieve advances at both the bench and bedside, national research registries established in the US and Canada are critical.

  8. Engineering antibodies for cancer therapy.

    Science.gov (United States)

    Boder, Eric T; Jiang, Wei

    2011-01-01

    The advent of modern antibody engineering has led to numerous successes in the application of these proteins for cancer therapy in the 13 years since the first Food and Drug Administration approval, which has stimulated active interest in developing more and better drugs based on these molecules. A wide range of tools for discovering and engineering antibodies has been brought to bear on this challenge in the past two decades. Here, we summarize mechanisms of monoclonal antibody therapeutic activity, challenges to effective antibody-based treatment, existing technologies for antibody engineering, and current concepts for engineering new antibody formats and antibody alternatives as next generation biopharmaceuticals for cancer treatment.

  9. Pathogenic and Epiphenomenal Anti-DNA Antibodies in SLE

    Directory of Open Access Journals (Sweden)

    Mirjana Pavlovic

    2010-01-01

    Full Text Available The discoveries of natural and the development of manufactured highly efficient catalytic antibodies (abzymes opens the door to many practical applications. One of the most fascinating is the use of such antibodies in human therapy and prevention (vaccination, of cancer, AIDS, autoimmune diseases. A special entity of naturally occurring DNA hydrolytic anti-DNA antibodies is emerging within past decades linked to autoimmune and lymphoproliferative disorders, such as systemic lupus erythematosus (SLE, multiple sclerosis (MS, Sjogren Syndrome (SS, B - Chronic lymphocytic leucosis (B-CLL, and Multiple Myeloma (MM. The origin of the antibodies is unknown. The underlying mechanisms of these activities are suggested to be penetration into the living cells and translocation in the nucleus, with recognition of the specific binding sites at particular (ss or ds DNA. There are controversies in the literature whether hydrolysis is a sequence-specific event. The interplay between anti-DNA antibodies and DNA is not yet elucidated. This molecular “twist” also suggests that anti-DNA antibodies with DNA hydrolytic capacity could be the organism's immune response to a microbial attack, with microbial DNA, or specific genes within microbial DNA sequence, as a target for neutralization. The catalytic antibody-based approach can become a key tool in selective chemotherapeutic strategies.

  10. Seroprevalence of dengue virus antibodies in healthy Jamaicans.

    Science.gov (United States)

    Brown, Michelle G; Vickers, Ivan E; Salas, Rose Alba; Smikle, Monica F

    2009-01-01

    Dengue fever, a mosquito borne viral infection, is endemic to Jamaica. The seroprevalence of dengue IgG and IgM antibodies were determined in 277 healthy Jamaicans by enzyme linked immunosorbent assay (ELISA). The seroprevalence of dengue IgG antibodies was 100% (277/277) while dengue IgM antibodies were found in 3.6% (10/277). A statistically significant association was found between the presence of dengue IgM antibodies and gender (males 10/105, 9.5% vs females 0/172, 0.0%); chi(2) = 17.0, p=0.000.The high seroprevalence rate of dengue IgG antibodies and the presence of dengue IgM in the healthy population are in keeping with the endemicity of the virus in Jamaica. Therefore tests for dengue IgG antibodies are of limited usefulness in Jamaica and can be safely excluded from diagnostic testing as a cost saving measure. Serological diagnosis of current dengue infection should be centred around the dengue IgM tests although the limitations in the predictive values of such tests should also be considered. The results also suggest that the risk of emergence of the more severe forms of dengue, dengue haemorrhagic fever (DHF) and dengue shock syndrome (DSS) in the Jamaican population, due to the presence of enhancing antibodies, is high. PMID:19996526

  11. Refeeding syndrome.

    Science.gov (United States)

    Fernández López, M T; López Otero, M J; Alvarez Vázquez, P; Arias Delgado, J; Varela Correa, J J

    2009-01-01

    Refeeding syndrome is a complex syndrome that occurs as a result of reintroducing nutrition (oral, enteral or parenteral) to patients who are starved or malnourished. Patients can develop fluid-balance abnormalities, electrolyte disorders (hypophosphataemia, hypokalaemia and hypomagnesaemia), abnormal glucose metabolism and certain vitamin deficiencies. Refeeding syndrome encompasses abnormalities affecting multiple organ systems, including neurological, pulmonary, cardiac, neuromuscular and haematological functions. Pathogenic mechanisms involved in the refeeding syndrome and clinical manifestations have been reviewed. We provide suggestions for the prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk, reintroduce nutrition cautiously and correct electrolyte and vitamin deficiencies properly.

  12. Comparison of two ELISA kits for the detection of the IgG antibodies against hemorrhagic fever with renal syndrome%2种检测人肾综合征出血热IgG抗体ELISA试剂盒的比较

    Institute of Scientific and Technical Information of China (English)

    李卓; 曾汉玉; 马樱; 刘媛; 张戎; 刘美宁; 陈丽华

    2015-01-01

    Objective To investigate the performance differences in detecting hemorrhagic fever with renal syndrome (HFRS) IgG Antibodies between domestic and imported ELISA kits. Methods Domestic and imported ELISA kits were used to detect HFRS IgG antibodies of the serum samples from 50 HFRS vaccinators, and the consistency, sensitivity, precision and methodology of the two ELISA kits were analyzed. Results Precision test showed that both the domestic and the imported ELISA kits had good precision, with the batch coefficient of variation of less than 5%. The positive consistency rate of the two ELISA kits was 100%, the negative consis-tency rate was 20%, and the similarity rate was 60%. The sensitivity of the domestic ELISA kits was 5 times higher than that of the imported ELISA kits, and the difference was significant between the two ELISA kits (P<0.05). Conclusions The sensitivity of the do-mestic ELISA kits is higher than that of the imported ELISA kits, the specificity of the imported ELISA kits is higher than that of the domestic ELISA kits, and both the domestic ELISA kits and the imported ELISA kits have good precision for the detection of HFRS IgG antibodies. The selection and application of the domestic or the imported ELISA kits depend on specific requirements.%目的 探讨国产与进口人肾综合征出血热(hemorrhagic fever with renal syndrome, HFRS)IgG抗体ELISA检测试剂盒的性能差异. 方法 采用国产和进口ELISA试剂盒分别检测50例HFRS疫苗接种者血清样本中抗人HFRS IgG抗体含量,分析比较2种ELISA试剂盒的一致性、灵敏性、精密度以及方法学等的差异. 结果 2种ELISA试剂盒的精密度良好,批内变异系数均<5%;2种试剂阳性一致率为100%,阴性一致率为20%,相似率为60%;灵敏性检测结果显示,2种ELISA试剂盒的灵敏性差异较大(P<0.05),国产ELISA试剂盒的灵敏度约是进口ELISA试剂盒的5倍. 结论 国产人HFRS IgG抗体ELISA检测试剂盒灵敏性好,进

  13. 抗磷脂抗体相关性复发性流产的诊治

    Institute of Scientific and Technical Information of China (English)

    林其德; 苏小玲

    2013-01-01

    Numerous studies show that antiphospho-lipid antibodies can cause recurrent miscarriage, unexplained recurrent miscarriage associated with an-tiphospholipid antibodies are antiphospholipid antibody syndrome scope. The studies showed that path-ogenesis of antiphospholipid antibodies including pla-cental hypercoagulable state, thrombosis and direct damage of trophoblast cell,could lead to miscarriage. Treatment for antiphospholipid antibody-positive patients with recurrent miscarriage could be short course and individualized. The dose and duration of aspirin, adrenal corticosteroids and low molecular weight heparin therapy should be adjusted , according to antiphospholipid antibodies, platelet aggregation and the D-D dimer level in the peripheral blood of patients. However, the diagnosis and treatment has yet to be further explored.%大量研究表明抗磷脂抗体可导致复发性流产,原因不明复发性流产伴抗磷脂抗体阳性属于抗磷脂综合征范畴.发病机理研究表明抗磷脂抗体可以通过高凝状态、血栓形成和直接损伤滋养细胞导致流产.抗磷脂抗体阳性的复发性流产患者的治疗应该是短疗程、个体化.阿司匹林、肾上腺皮质激素和低分子肝素的剂量和疗程主要根据患者外周血抗磷脂抗体水平、血小板聚集情况、D-D聚体水平变化进行调整.但其诊治尚待进一步的探讨.

  14. Bilateral sudden hearing loss following habitual abortion: a case report and review of literature.

    Science.gov (United States)

    Yin, Tuanfang; Huang, Fengying; Ren, Jihao; Liu, Wei; Chen, Xing; Li, Lihua; Xie, Dinghua; Lu, Yongde

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) is usually unilateral and can be associated with tinnitus and vertigo. The most common causes of this disease are known to be the vascular and viral agents, but immune disorders are involved in the development of sudden deafness. The antiphospholipid syndrome (APS) is an acquired autoimmune system disorder, which is defined as the presence of antiphospholipid antibodies (APA) in the patient's blood, then cause venous and/or arterial thrombosis in various organs of the body, for example, thrombosis can occur in the placenta and/or the inner ear. As a result, it can cause abortion and/or sudden deafness. Bilateral SSNHL following habitual abortion is a rare clinical event. Here, we report a case of 32-year-old woman who presented with bilateral sudden hearing loss following recurrent pregnancy loss (RPL) as the first manifestation of primary antiphospholipid syndrome. Combine the literature, the diagnosis, clinical implication and treatment are discussed.

  15. Cerebellar Ataxia and Glutamic Acid Decarboxylase Antibodies

    Science.gov (United States)

    Ariño, Helena; Gresa-Arribas, Nuria; Blanco, Yolanda; Martínez-Hernández, Eugenia; Sabater, Lidia; Petit-Pedrol, Mar; Rouco, Idoia; Bataller, Luis; Dalmau, Josep O.; Saiz, Albert; Graus, Francesc

    2016-01-01

    IMPORTANCE Current clinical and immunologic knowledge on cerebellar ataxia (CA) with glutamic acid decarboxylase 65 antibodies (GAD65-Abs) is based on case reports and small series with short-term follow-up data. OBJECTIVE To report the symptoms, additional antibodies, prognostic factors, and long-term outcomes in a cohort of patients with CA and GAD65-Abs. DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study and laboratory investigations at a center for autoimmune neurologic disorders among 34 patients with CA and GAD65-Abs, including 25 with long-term follow-up data (median, 5.4 years; interquartile range, 3.1-10.3 years). MAIN OUTCOMES AND MEASURES Analysis of clinicoimmunologic features and predictors of response to immunotherapy. Immunochemistry on rat brain, cultured neurons, and human embryonic kidney cells expressing GAD65, GAD67, α1-subunit of the glycine receptor, and a repertoire of known cell surface autoantigens were used to identify additional antibodies. Twenty-eight patients with stiff person syndrome and GAD65-Abs served as controls. RESULTS The median age of patients was 58 years (range, 33-80 years); 28 of 34 patients (82%) were women. Nine patients (26%) reported episodes of brainstem and cerebellar dysfunction or persistent vertigo several months before developing CA. The clinical presentation was subacute during a period of weeks in 13 patients (38%). Nine patients (26%) had coexisting stiff person syndrome symptoms. Systemic organ-specific autoimmunities (type 1 diabetes mellitus and others) were present in 29 patients (85%). Twenty of 25 patients with long-term follow-up data received immunotherapy (intravenous immunoglobulin in 10 and corticosteroids and intravenous immunoglobulin or other immunosuppressors in 10), and 7 of them (35%) improved. Predictors of clinical response included subacute onset of CA (odds ratio [OR], 0.50; 95% CI, 0.25-0.99; P = .047) and prompt immunotherapy (OR, 0.98; 95% CI, 0.96-0.99; P = .01). Similar

  16. Coeliac disease in Williams syndrome

    Science.gov (United States)

    Giannotti, A.; Tiberio, G.; Castro, M.; Virgilii, F.; Colistro, F.; Ferretti, F.; Digilio, M. C.; Gambarara, M.; Dallapiccola, B.

    2001-01-01

    BACKGROUND—Coeliac disease (CD) has been reported in several patients affected by chromosomal disorders, including Down syndrome (DS) and Turner syndrome (TS). CD has also been found in sporadic Williams syndrome (WS) patients. In this study, CD was evaluated in a consecutive series of patients with WS, in order to estimate if the prevalence of CD in WS patients is higher than in the general population.
METHODS AND RESULTS—A consecutive series of 63 Italian patients with WS was studied by analysing the dosage of antigliadin antibodies (AGA) IgA and antiendomisium antibodies (AEA). In patients with positive AGA and AEA, small bowel biopsy was performed. The prevalence of CD in our WS population was compared with that estimated in a published series of 17 201 Italian students. Seven WS patients were found to be positive for AGA IgA and AEA. Six of them underwent small bowel biopsy, which invariably disclosed villous atrophy consistent with CD. The prevalence of CD in the present series of WS patients was 9.5% (6/63), compared to 0.54% (1/184) in the Italian students (p<0.001).
CONCLUSION—The present results suggest that the prevalence of CD in WS is higher than in the general population and is comparable to that reported in DS and TS. AGA and AEA screening is recommended in patients with WS.


Keywords: Williams syndrome; coeliac disease PMID:11694549

  17. Antibody affinity maturation

    DEFF Research Database (Denmark)

    Skjødt, Mette Louise

    linker for yeast surface display of scFv and scFab fragments, we compared a series of different Gly-Ser-based linkers in display and antigen binding proficiency. We show that these formats of the model antibody can accommodate linkers of different lengths and that introduction of alanine or glutamate......-2. Based on the presented data we suggest that affinity maturation of the model antibody proceeds through multiple incremental steps of subtle improvements. We moreover conclude that the best affinity improved candidates are likely to be obtained through optimization of both the antigen...... fragments by in vivo homologous recombination large combinatorial antibody libraries can easily be generated. We have optimized ordered assembly of three CDR fragments into a gapped vector and observed increased transformation efficiency in a yeast strain carrying a deletion of the SGS1 helicase...

  18. Paraneoplastic Syndromes of the Central Nervous System

    NARCIS (Netherlands)

    J.W.B. Moll (Wibe)

    1996-01-01

    textabstractIn recent years a continuous stream of new information on clinical, pathological and immunological aspects of paraneoplastic neurological syndromes has been published. In this survey, we will discuss current opinions on the value of anti-neuronal antibody detection for establishing a dia

  19. Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome: Case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Beatriz De-Leon-Bojorge; Samuel Zaltzman-Girsevich; Arturo Ortega-Salgado; Adelina Prieto-Patron; Ruth Córdoba-Córdoba; Marco Yamazaki-Nakashimada

    2006-01-01

    Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population. We describe a 17-year-old patient with SLE, who developed nephritis that was well controlled with medications. Initial treatment consisted of azathioprine, aspirin and prednisone with stable control of her symptoms. Two years later she developed a right quadrant abdominal pain,and an abdominal ultrasound revealed microlithiasic cholecystitis. Open cholecystectomy was performed and the histopathological findings revealed vasculitis with thrombotic microangiopathy in the gallbladder. This case presentation illustrates that calculous or acalculous cholecystitis should be considered as a manifestation of active SLE and APS.

  20. Hypereosinophilic syndromes

    Directory of Open Access Journals (Sweden)

    Goldman Michel

    2007-09-01

    Full Text Available Abstract Hypereosinophilic syndromes (HES constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia (> 1.5 × 109/L for more than six consecutive months associated with evidence of eosinophil-induced organ damage, where other causes of hypereosinophilia such as allergic, parasitic, and malignant disorders have been excluded. Prevalence is unknown. HES occur most frequently in young to middle-aged patients, but may concern any age group. Male predominance (4–9:1 ratio has been reported in historic series but this is likely to reflect the quasi-exclusive male distribution of a sporadic hematopoietic stem cell mutation found in a recently characterized disease variant. Target-organ damage mediated by eosinophils is highly variable among patients, with involvement of skin, heart, lungs, and central and peripheral nervous systems in more than 50% of cases. Other frequently observed complications include hepato- and/or splenomegaly, eosinophilic gastroenteritis, and coagulation disorders. Recent advances in underlying pathogenesis have established that hypereosinophilia may be due either to primitive involvement of myeloid cells, essentially due to occurrence of an interstitial chromosomal deletion on 4q12 leading to creation of the FIP1L1-PDGFRA fusion gene (F/P+ variant, or to increased interleukin (IL-5 production by a clonally expanded T cell population (lymphocytic variant, most frequently characterized by a CD3-CD4+ phenotype. Diagnosis of HES relies on observation of persistent and marked hypereosinophilia responsible for target-organ damage, and exclusion of underlying causes of hypereosinophilia, including allergic and parasitic disorders, solid and hematological malignancies, Churg-Strauss disease, and HTLV infection. Once these criteria are fulfilled, further testing for eventual pathogenic classification is warranted using appropriate cytogenetic and functional approaches. Therapeutic

  1. Antithyroglobulin Antibodies and Antimicrosomal Antibodies in Various Thyroid Diseases

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Gwon Jun; Hong, Key Sak; Choi, Kang Won; Lee, Kyu; Koh, Chang Soon; Lee, Mun Ho; Park, Sung Hoe; Chi, Je Geun; Lee, Sang Kook [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1979-03-15

    The authors investigated the incidence of antithyroglobulin antibodies and antibodies and antimicrosomal antibodies measured by tanned red cell hemagglutination method in subjects suffering from various thyroid disorders. 1) In 15 normal patients, neither suffering from any thyroid diseases nor from any other autoimmune disorders, the antithyroglobulin antibodies were all negative, but the antimicrosomal antibody was positive only in one patient (6.7%). 2) The antithyroglobulin antibodies were positive in 31.5% (34 patients) of 108 patients with various thyroid diseases, and the antimicrosomal antibodies were positive in 37.0% (40 patients). 3) of the 25 patients with Graves' diseases, 7 patients (28.0%) showed positive for the antithyroglobulin antibodies, and 9 (36.0%) for the antimicrosomal antibodies. There was no definite differences in clinical and thyroid functions between the groups with positive and negative results. 4) Both antibodies were positive in 16 (88.9%) and 17 (94.4%) patients respectively among 18 patients with Hashimoto's thyroiditis, all of them were diagnosed histologically. 5) Three out of 33 patients with thyroid adenoma showed positive antibodies, and 3 of 16 patients with thyroid carcinoma revealed positive antibodies. 6) TRCH antibodies demonstrated negative results in 2 patients with subacute thyroiditis, but positive in one patient with idiopathic primary myxedema. 7) The number of patients with high titers(>l:802) was 16 for antithyroglobulin antibody, and 62.5% (10 patients) of which was Hashimoto's thyroiditis. Thirteen (65.0) of 20 patients with high titers (>l:802) for antimicrosomal antibody was Hashimoto's thyroiditis. TRCH test is a simple, sensitive method, and has high reliability and reproducibility. The incidences and titers of antithyroglobulin antibody and antimicrosomal antibody are especially high in Hashimoto's thyroiditis.

  2. Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances

    International Nuclear Information System (INIS)

    A 28-year-old woman presented with generalised livedo reticularis, dementia, epilepsy, and pyramidal and extrapyramidal signs. Multiple focal infarcts were seen on MRI. Angiography demonstrated widespread cerebromeningeal angiomatosis with multiple small and medium size arterial occlusions. A lifelong personal and family history of mental handicap in the absence of anticardiolipin antibodies suggests Divry-Van Bogaert syndrome, not previously been reported in a female. Similarities to Sneddon's syndrome are discussed. (orig.)

  3. Churg-Strauss syndrome and hemorragic vasculitis

    Directory of Open Access Journals (Sweden)

    Rui Moreira Marques

    2011-12-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the lefthand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome.

  4. 基于上转化发光免疫层析技术建立发热伴血小板减少综合征病毒总抗体快速检测方法%Rapid detection of severe fever with thrombocytopenia syndrome virus (SFTSV)total antibodies by up-converting phosphor technology (UPT)-based lateral-flow assay

    Institute of Scientific and Technical Information of China (English)

    黄超; 张文帅; 张黎; 温恬; 史凤娟; 曾晓燕; 迟莹; 史智扬; 焦永军

    2015-01-01

    目的:基于上转化发光(UPT)免疫层析技术,建立发热伴血小板减少综合征病毒(SFTSV)总抗体的现场快速检测方法。方法将 SFTSV 重组 NP 蛋白与上转化发光颗粒(UCP)偶联,制备 UCP-NP 免疫层析试纸条,评价该试纸条检测SFTSV 总抗体的灵敏性、特异性和稳定性,并检测 SFTSV 血清254份,与酶联免疫法(ELISA)比较。结果该方法可在15 min内完成 SFTSV 总抗体检测,可检测1∶500稀释度的 SFTSV 阳性血清,与其他出血热病毒无交叉反应,加样14 d 内稳定性较高。UPT 免疫层析法与 ELISA 法检测临床血清样品一致性极高(Kappa=0.967),约登指数为0.973。结论建立了基于 UPT 免疫层析技术的 SFTSV 总抗体快速检测方法,该方法灵敏、特异,且操作简便、快速,结果稳定,适合在基层门诊和体检现场推广。%Objective To develop a method for rapid and on-site detection of severe fever with thrombocytopenia syndrome virus (SFTSV)total antibodies by up-converting phosphor technology (UPT)-based lateral-flow assay.Methods SFTSV re-combinant NP protein was conjugated to up-converting phosphor particles (UCP),UPT-NP analysis strips were assembled. The sensitivity,specificity and stability of UPT method to detect total antibodies were evaluated;254 clinical sera were tested by UPT method and ELISA to study the consistence of above 2 methods.Results UPT method can analyze total antibodies a-gainst SFTSV in 15 minutes,with the detection limit of 1∶500 dilution of original positive SFTSV sera samples;no cross reac-tion with other hemorrhagic fever virus were detected.UPT method exhibited high stability in 14 days upon sample loading. The results of UPT method were highly consistent with ELISA (Kappa=0.967),with Youden's index of 0.973.Conclusion Rapid detection of SFTSV total antibodies by UPT-based lateral-flow assay was developed successfully,which was sensitive , specific

  5. Waardenburg syndrome.

    OpenAIRE

    Read, A P; Newton, V E

    1997-01-01

    Auditory-pigmentary syndromes are caused by physical absence of melanocytes from the skin, hair, eyes, or the stria vascularis of the cochlea. Dominantly inherited examples with patchy depigmentation are usually labelled Waardenburg syndrome (WS). Type I WS, characterised by dystopia canthorum, is caused by loss of function mutations in the PAX3 gene. Type III WS (Klein-Waardenburg syndrome, with abnormalities of the arms) is an extreme presentation of type I; some but not all patients are ho...

  6. Metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Gogia Atul

    2006-02-01

    Full Text Available The Metabolic syndrome is a widely prevalent and multi-factorial disorder. The syndrome has been given several names, including- the metabolic syndrome, the insulin resistance syndrome, the plurimetabolic syndrome, and the deadly quartet. With the formulation of NCEP/ATP III guidelines, some uniformity and standardization has occurred in the definition of metabolic syndrome and has been very useful for epidemiological purposes. The mechanisms underlying the metabolic syndrome are not fully known; however resistance to insulin stimulated glucose uptake seems to modify biochemical responses in a way that predisposes to metabolic risk factors. The clinical relevance of the metabolic syndrome is related to its role in the development of cardiovascular disease. Management of the metabolic syndrome involves patient-education and intervention at various levels. Weight reduction is one of the main stays of treatment. In this article we comprehensively discuss this syndrome- the epidemiology, pathogenesis, clinical relevance and management. The need to do a comprehensive review of this particular syndrome has arisen in view of the ever increasing incidence of this entitiy. Soon, metabolic syndrome will overtake cigarette smoking as the number one risk factor for heart disease among the US population. Hardly any issue of any primary care medical journal can be opened without encountering an article on type 2 diabetes, dyslipidemia or hypertension. It is rare to see type 2 diabetes, dyslipidemia, obesity or hypertension in isolation. Insulin resistance and resulting hyperinsulinemia have been implicated in the development of glucose intolerance (and progression to type 2 diabetes, hypertriglyceridemia, hypertension, polycystic ovary yndrome, hypercoagulability and vascular inflammation, as well as the eventual development of atherosclerotic cardiovascular disease manifested as myocardial infarction, stroke and myriad end organ diseases. Conversely

  7. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    Dayane Cristine Issaho

    2015-04-01

    Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

  8. Sweet Syndrome

    OpenAIRE

    Kasapçopur, Özgür; Sever, Lale; Çalışkan, Salim; Kodakoğlu, Ramazan; Mat, Cem; Kaner, Gültekin; Arısoy, Nil

    1996-01-01

    Sweet syndrome is a vasculitis characterized with fever leucocytosis neutrophilia and dermal neutrophilic infiltration In children Sweet syndrome usually occurs with secondary to infection and in adults to malignancy We report a Sweet syndrome in a five years old girl with respiratory infections otitis dactylitis long lasting fever and cutaneous rash A neutrophilic dermal infiltration is noted in cutaneous biopsy These signs have disappeared with corticosteroid treatment In conclusion Sweet s...

  9. Brugada syndrome

    Directory of Open Access Journals (Sweden)

    Bockeria O.L.

    2015-03-01

    Full Text Available Brugada syndrome is characterized by sudden death associated with one of several ECG patterns including incomplete right bundle-branch block and ST-segment elevation in the anterior precordial leads. According to the ECG patterns there are three types of Brugada syndrome. Brugada syndrome is genetically determined and has an autosomal dominant pattern of transmission in about 50% of familial cases. Nowadays implantation of cardioverter-defibrillator is the only proven method of sudden cardiac death prevention.

  10. Marfan Syndrome (For Teens)

    Science.gov (United States)

    ... How Can I Help a Friend Who Cuts? Marfan Syndrome KidsHealth > For Teens > Marfan Syndrome Print A ... a genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, ...

  11. What Is Down Syndrome?

    Science.gov (United States)

    ... NDSS Home » Down Syndrome » What Is Down Syndrome? What Is Down Syndrome? In every cell in the ... chromosome 21 causes the characteristics of Down syndrome. What Causes Down Syndrome? Regardless of the type of ...

  12. 抗α-胞衬蛋白抗体对干燥综合征诊断价值的荟萃分析%Diagnostic value of anti-α-fodrin antibody testing for Sj(o)gren's syndrome: a meta-analysis

    Institute of Scientific and Technical Information of China (English)

    黄清水; 乐爱平; 罗忠勤; 李俊明; 王文强; 鞠北华; 万腊根

    2008-01-01

    Objective To evaluate the diagnostic accuracy of anti-α-fodrin antibody for Sjogren's syndrome (SS). Methods Qualified literatures on evaluation of anti-ot-fodrin antibody in diagnosis of SS in English and Chinese published between January 1997 and December 2007 were retrieved from the Cocharane Library, Medline, Embase, and China National Knowledge Infrastructure (CNKI) databases, etc. Two reviewers independently assessed the methodological quality of each study with the tool QUADAS ( quality assessment of diagnostic accuracy studies). Statistical analysis was performed by employing MATLAB, Review Manager 4.2 and Meta-Discl. 4. A reefs-analysis of the reported sensitivity and specificity of each study and Summary Receiver Operating Characteristic (SROC) curve was performed. Results Eighteen literatures were included at last. After testing the heterogeneity of the included articles, proper effect model was selected to calculate the pooled weighted sensitivity and specificity with 95% confidence interval: for anti-α-fodrin antibody IgG, the sensitivity was 0.40 [95% CI(0.37-0.43)] and the specificity was 0.82 [95% CI(0.79-0.84)], the area under the curve (AUC) of SROC was 0.8029 (SE=0.0580). Eight studies tested anti-α-fodrin antibody lgA, the pooled weighted sensitivity and specificity with 95% confidence interval were 0.34 [95% CI(0.30-0.38) ] and 0. 83 [95% CI(0.79-0.86) ] respectively, the AUC of SROC was 0.6374 (SE=0.1841), the synthesis data all showed heterogeneity. The subgroups were analyzed to identify the sources of heterogeneity according to age, race, assay method, agent source, diagnostic criteria, and country. There was homogenicity among the 4 studies from China, and the 6 studiesfrom Japan,the AUC of SROC were 0.7343 (SE=0.0448) and 0. 9273 (SE=0.0394), respectively. Conclusion Diagnostic criteria, agent source, assay method, age, race, and country are the important sources of heterogeneity. Anti-a-fedrin antibodies IgG and IgA have relatively

  13. Monoclonal antibodies in myeloma

    DEFF Research Database (Denmark)

    Sondergeld, P.; van de Donk, N. W. C. J.; Richardson, P. G.;

    2015-01-01

    The development of monoclonal antibodies (mAbs) for the treatment of disease goes back to the vision of Paul Ehrlich in the late 19th century; however, the first successful treatment with a mAb was not until 1982, in a lymphoma patient. In multiple myeloma, mAbs are a very recent and exciting add...

  14. Prediction of Antibody Epitopes

    DEFF Research Database (Denmark)

    Nielsen, Morten; Marcatili, Paolo

    2015-01-01

    self-proteins. Given the sequence or the structure of a protein of interest, several methods exploit such features to predict the residues that are more likely to be recognized by an immunoglobulin.Here, we present two methods (BepiPred and DiscoTope) to predict linear and discontinuous antibody...

  15. [Myasthenia gravis and myasthenic syndromes].

    Science.gov (United States)

    Marouf, W; Sieb, J P

    2009-08-01

    Neuromuscular transmission is compromised in a variety of disorders due to immunological, toxic or congenital mechanisms. Myasthenia gravis (MG) is the most frequent among these disorders. In about 15% of cases, MG is associated with a second autoimmune disorder mainly seen in rheumatologists. Some of the drugs used in rheumatology can exacerbate MG or even trigger immunologically the occurrence of MG. In most MG patients, antibodies to the acetylcholine receptor (AChR) are present, but around 10% have AChR antibodies that are only identified by novel methods, and up to 5% have muscle-specific kinase antibodies which define a different subgroup of myasthenia. Among those MG patients with anti-AChR antibodies, a number of clinical subtypes can be identified including early-onset MG (onset syndrome (LEMS). The abnormality in LEMS is a presynaptic failure to acetylcholine release caused by antibodies to voltage-gated calcium channels. More than half of LEMS patients have small-cell lung cancer. PMID:19609783

  16. Importance of anticomplement immunofluorescence antibody titration for diagnosing varicella-zoster virus infection in Bell's palsy.

    Science.gov (United States)

    Shigeta, S; Baba, M; Ogata, M; Nozaki, H; Okuaki, A; Nakamura, S

    1986-11-01

    Anticomplement Immunofluorescence was used for antibody titration against varicella-zoster virus (VZV) in 43 patients with peripheral facial palsy. Nine of 31 patients (29%) with Bell's palsy and eight of 12 patients (75%) with Ramsey-Hunt syndrome had anticomplement immunofluorescence antibody titres of greater than or equal to 1/10. On the other hand, none of 14 patients with herpes simplex virus (HSV) infection and 51 healthy adults showed anticomplement immunofluorescence antibody titres of greater than or equal to 1/10. The anticomplement immunofluorescence antibody titre in two patients with Ramsey-Hunt syndrome increased later and decreased sooner than the indirect immunofluorescence antibody titre, becoming undetectable at 66 and 104 days, respectively, after onset of the disease. There was no cross reaction between anti-VZV and anti-HSV antibodies in the patients who showed a positive antibody rise for VZV. As the acute stage of VZV infection is obscure in the patients with peripheral facial palsy without herpes the screening of anticomplement immunofluorescence antibody to VZV at titres greater than or equal to 1/10 may be useful for the diagnosis of VZV infection in patients with peripheral facial palsy.

  17. All that seems sepsis is not sepsis

    Directory of Open Access Journals (Sweden)

    Vivek S Guleria

    2013-01-01

    Full Text Available Catastrophic antiphospholipid antibody syndrome (CAPS resembles severe sepsis in its acute presentation, with features of systemic inflammatory response syndrome (SIRS leading to multiple organ dysfunction. Infections are the best known triggers of CAPS. This emphasizes the need for early diagnosis and aggressive treatment as the mortality is as high as 50%. We present a 42-year-old woman who developed SIRS postoperatively and was eventually diagnosed as CAPS.

  18. Recombinant antibodies and tumor targeting

    OpenAIRE

    Sheikholvaezin, Ali

    2006-01-01

    Different antibody derived constructs are rapidly advancing as putative tools for treatment of malignant diseases. Antibody engineering has added significant new technologies to modify size, affinities, solubility, stability and biodistribution properties for immunoconjugates. In the present thesis, the aim was to increase our knowledge on how new recombinant antibodies could be tailored to optimize localization to experimental tumors in mice. One hybridoma, producing the monoclonal antibody ...

  19. Activation of human complement by immunoglobulin G antigranulocyte antibody.

    Science.gov (United States)

    Rustagi, P K; Currie, M S; Logue, G L

    1982-01-01

    The ability of antigranulocyte antibody to fix the third component of complement (C3) to the granulocyte surface was investigated by an assay that quantitates the binding of monoclonal anti-C3 antibody to paraformaldehyde-fixed cells preincubated with Felty's syndrome serum in the presence of human complement. The sera from 7 of 13 patients with Felty's syndrome bound two to three times as much C3 to granulocytes as sera from patients with uncomplicated rheumatoid arthritis. The complement-activating ability of Felty's syndrome serum seemed to reside in the monomeric IgG-containing serum fraction. For those sera capable of activating complement, the amount of C3 fixed to granulocytes was proportional to the amount of granulocyte-binding IgG present in the serum. Thus, complement fixation appeared to be a consequence of the binding of antigranulocyte antibody to the cell surface. These studies suggest a role for complement-mediated injury in the pathophysiology of immune granulocytopenia, as has been demonstrated for immune hemolytic anemia and immune thrombocytopenia. PMID:7174786

  20. Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?

    Directory of Open Access Journals (Sweden)

    Hongliang Zhang

    2013-01-01

    Full Text Available Paraneoplastic neurological syndromes (PNSs occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, especially small cell lung cancer (SCLC, lymphoma, and gynecological tumors. The finding of highly specific antibodies directed against onconeural antigens has revolutionized the diagnosis and promoted the understanding of these syndromes and led to the current hypothesis of an autoimmune pathophysiology. Accumulating data strongly suggested direct pathogenicity of these antibodies. The field of PNS has expanded rapidly in the past few years with the discovery of limbic encephalitis associated with glutamic acid decarboxylase (GAD 65, the voltage (VGKC-gated potassium channel complex, the methyl (N-NMDA-D-aspartate, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA, and gamma aminobutyric acid (GABA (B receptors, and so forth. Despite this, the clinical spectrum of these diseases has not yet been fully investigated. The clinical importance of these conditions lies in their frequent response to immunotherapies and, less commonly, their association with distinctive tumors. This review provides an overview on the pathogenesis and diagnosis of PNS, with emphasis on the role of antibodies in limbic encephalitis.