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成人斯蒂尔病并发暴发性肝衰竭

Fulminant hepatic failure in adult onset Still's disease.

作者信息

Dino O, Provenzano G, Giannuoli G, Sciarrino E, Pouyet M, Pagliaro L

机构信息

Clinica Medica R, University of Palermo, Italy.

出版信息

J Rheumatol. 1996 Apr;23(4):784-5.

PMID:8730149
Abstract

Adult onset Still's disease (AOSD) is a well characterized rheumatoid disorder with frequent liver involvement. This is usually asymptomatic but severe hepatic failure has occasionally been reported. We describe a 44-year-old woman who developed acute liver failure 2 months after presenting symptoms of AOSD. Full etiologic evaluation was done and all other causes of liver failure were excluded. She underwent emergency orthotopic liver transplantation but developed disseminated intravascular coagulation with acute renal failure, seizures, and coma, and died after 48 hours.

摘要

成人斯蒂尔病(AOSD)是一种特征明确的类风湿性疾病,常累及肝脏。这通常无症状,但偶尔有严重肝衰竭的报道。我们描述了一名44岁女性,在出现AOSD症状2个月后发生急性肝衰竭。进行了全面的病因评估,排除了所有其他肝衰竭原因。她接受了紧急原位肝移植,但出现了弥散性血管内凝血,并伴有急性肾衰竭、癫痫发作和昏迷,48小时后死亡。

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