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肝硬化难治性出血的一种不寻常原因。

An Unusual Cause of Refractory Bleeding in Cirrhosis.

作者信息

Alvarado Meagan, Li Darrick K

机构信息

Department of Medicine, Section of Digestive Diseases, Yale School of Medicine, New Haven, CT.

出版信息

ACG Case Rep J. 2024 Sep 12;11(9):e01498. doi: 10.14309/crj.0000000000001498. eCollection 2024 Sep.

Abstract

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the development of antibodies against factor VIII. AHA has previously been reported in association with malignancy and autoimmune disorders, but rarely with liver disease. A prolonged activated partial thromboplastin time is the initial laboratory manifestation of this condition but may be challenging to interpret in the setting of abnormal markers of coagulation typically seen in cirrhosis. We present a case of AHA in a patient with decompensated cirrhosis resulting in refractory bleeding and highlight the complexities of interpreting abnormal coagulation factors in patients with cirrhosis.

摘要

获得性血友病A(AHA)是一种罕见的出血性疾病,由针对凝血因子VIII的抗体产生所致。此前已有AHA与恶性肿瘤和自身免疫性疾病相关的报道,但与肝脏疾病相关的报道较少。活化部分凝血活酶时间延长是该病的初始实验室表现,但在肝硬化患者常见的凝血异常标志物背景下可能难以解读。我们报告一例失代偿期肝硬化患者发生AHA导致难治性出血的病例,并强调解读肝硬化患者异常凝血因子的复杂性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3226/11392490/9d4761f719e9/ac9-11-e01498-g001.jpg

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