36岁女性急性肾衰竭和血小板减少1例报告

M. Stoian
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摘要

背景:抗磷脂综合征(APS)是一种获得性、免疫介导的血栓性疾病,单独发生(原发性APS, PAPS)或与其他自身免疫性疾病相关,主要是系统性红斑狼疮(SLE)(继发性APS),其特征是静脉或动脉血栓形成复发和/或妊娠发病率与抗磷脂抗体(aPL)和/或狼疮抗凝剂(LA)相关。病例报告:一名36岁妇女因急性肾功能衰竭和血小板减少症入院。该妇女表现为急性疾病,其特征是呼吸道症状和发热的前驱症状,对抗生素治疗无反应,随后进行性累及多个器官。有酶和功能证据表明心肌坏死导致间歇性,严重的心力衰竭,以及需要透析的急性肾功能衰竭,实验室证据表明胰腺损伤,肺浸润伴呼吸衰竭,中枢神经系统受累,并伴有混淆。肾活检后发现肾周血肿和血小板减少。抗磷脂抗体综合征的实验室诊断标准是IgG抗心磷脂抗体升高、狼疮抗凝血试验阳性、IgM抗心磷脂抗体升高。结论:该患者具有抗磷脂抗体综合征,并发急性血栓性血管病变,引起心肌、胰腺、肾脏和肺部缺血性损伤。肾间质炎症比单纯缺血性损伤更严重,许多反应要么是药物过敏反应,要么是干性干燥综合征
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acute Renal Failure and Thrombocytopenia To a 36-Year -Old Woman: Case Report
Background: Antiphospholipid syndrome (APS) is an acquired, immune-mediated thrombophilia occurring alone (primary APS, PAPS) or in association with other autoimmune diseases, mainly systemic lupus erythematous (SLE), (secondary APS), characterized by recurrent venous or arterial thrombosis and /or pregnancy morbidity in association with antiphospholipid antibodies (aPL) and/or lupus anticoagulant (LA). Case report: A 36-year-old woman was admitted to the hospital because of acute renal failure and thrombocytopenia. This woman presented with an acute illness characterized by a prodrome of respiratory symptoms and fever that were unresponsive to antibiotic therapy, followed by the progressive involvement of multiple organs. There was enzymatic and functional evidence of myocardial necrosis leading to intermittent, severe heart failure, as well as acute renal failure requiring dialysis, laboratory evidence of pancreatic injury, pulmonary infiltrates with respiratory failure, and central nervous system involvement, with confusion. After a renal biopsy was detected a perinephric hematoma and thrombocytopenia. The laboratory criteria for the diagnosis of the antiphospholipid-antibody syndrome are an elevated value for IgG anticardiolipin antibody, a positive test for lupus anticoagulant, and an elevated value for IgM anticardiolipin antibody. Conclusions: This patient has the antiphospholipid antibody syndrome, with an acute thrombotic angiopathy that caused ischemic damage in the myocardium, pancreas, kidneys and lungs. The renal interstitial inflammation is more severe than that expected from the ischemic injury alone and many reflect either a hypersensitivity drug reaction or in view of the dry Sjogren’s syndrome
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