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灾难性抗磷脂综合征——一例罕见病例报告

Catastrophic Antiphospholid Syndrome - An Unusual Case Report.

作者信息

Madkaiker Sneha

机构信息

Critical Care Medicine, Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India.

出版信息

Indian J Crit Care Med. 2019 Jun;23(6):276-280. doi: 10.5005/jp-journals-10071-23180.

Abstract

Antiphospholipid syndrome (APLS) is characterised by venous or arterial thrombosis and/or adverse pregnancy outcome in the presence of persistent laboratory evidence of antiphospholipid antibodies. Catastrophic Antiphospholipid Syndrome (CAPS) is a severe and rare form of antiphospholipid syndrome characterised by multiple site thrombosis involving small, medium and large blood vessels occurring over a short period of time (usually 1 week) causing multiorgan failure. We present an unusual case of left upper limb acute arterial thrombosis with purpura fulminans like skin lesions precipitated by swine flu (H1N1) infection with adult respiratory distress syndrome subsequently developing acute renal failure, retinal infarcts, multiple acute cerebral infarcts, cardiac valvular vegetations and hemolytic anemia with recurrent bleeding episodes. A positive lupus anticoagulant confirmed the diagnosis of CAPS. In spite of early initiation of triple therapy (anticoagulation, high dose steroids, plasmapheresis) our patient did not survive. This rare case of probable CAPS is presented with an aim to study the clinical manifestations, laboratory findings, efficacy of therapy and prognosis in the medical ICU. Madkaiker S. Catastrophic Antiphospholid Syndrome - An Unusual Case Report. Indian J Crit Care Med 2019;23(6):276-280.

摘要

抗磷脂综合征(APLS)的特征是在存在抗磷脂抗体的持续实验室证据的情况下出现静脉或动脉血栓形成和/或不良妊娠结局。灾难性抗磷脂综合征(CAPS)是抗磷脂综合征的一种严重且罕见的形式,其特征是在短时间内(通常为1周)发生涉及小、中、大血管的多部位血栓形成,导致多器官功能衰竭。我们报告了一例不寻常的病例,一名患者因感染甲型H1N1流感,出现成人呼吸窘迫综合征,随后发展为急性肾衰竭、视网膜梗死、多发性急性脑梗死、心脏瓣膜赘生物和溶血性贫血伴反复出血发作,进而引发左上肢急性动脉血栓形成并伴有暴发性紫癜样皮肤病变。狼疮抗凝物阳性确诊为CAPS。尽管早期开始了三联治疗(抗凝、高剂量类固醇、血浆置换),我们的患者仍未存活。本文报告这例可能的CAPS罕见病例,旨在研究重症医学科中该疾病的临床表现、实验室检查结果、治疗效果及预后。 Madkaiker S. 灾难性抗磷脂综合征——一例不寻常病例报告。《印度危重症医学杂志》2019年;23(6):276 - 280。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f9c7/6698347/6ffd05ab2e90/ijccm-23-276-g001.jpg

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