D-青霉胺在以急性肝衰竭伴溶血为表现的威尔逊病中的应用
D-penicillamine in Wilson's disease presenting as acute liver failure with hemolysis.
作者信息
Vielhauer W, Eckardt V, Holtermüller K H, Lüth J B, Schulte B, Prellwitz W, Sonntag W
出版信息
Dig Dis Sci. 1982 Dec;27(12):1126-9. doi: 10.1007/BF01391452.
PMID:7172963
Abstract
Wilson's disease in a young woman presenting with an acute course is described. The clinical manifestations were fulminant hepatic failure associated with marked intravascular hemolysis. Immediate D-penicillamine and high-dose steroid therapy did not influence the course of the disease. Necropsy revealed an increased hepatic copper content and cirrhosis with extensive necrosis of the liver.
摘要
本文描述了一名年轻女性患威尔逊氏病且病程呈急性的情况。临床表现为暴发性肝衰竭并伴有明显的血管内溶血。立即给予青霉胺和大剂量类固醇治疗并未影响疾病进程。尸检显示肝脏铜含量增加以及伴有广泛肝坏死的肝硬化。
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