Diagnostic criteria for acute liver failure due to Wilson disease

查看全文 作　　者：Christoph [1]Eisenbach;Olivia [1]Sieg;Wolfgang [1]Stremmel;Jens [1]Encke;Uta [1]Merle

机构地区：[1]University Hospital of Heidelberg, Department of Gastroenterology and Hepatology, Im Neuenheimer Feld 410, Heidelberg D-69120, Germany

出　　处：《World Journal of Gastroenterology》索引2007年第13卷第11期,共4页

摘　　要：AIM: To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF). METHODS: We compared findings of patients presenting with ALF due to WD to those with ALF of other etiologies. RESULTS: Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 ± 43 vs 1982 ± 938, P < 0.0001 and 87 ± 44 vs 2756 ± 2941, P = 0.037, respectively), low choline esterase activity (1.79 ± 1.2 vs 4.30 ± 1.2, P = 0.009), high urine copper concentrations (93.4 ± 144.0 vs 3.5 ± 1.8, P = 0.001) and low hemoglobin (7.0 ± 2.2 vs 12.6 ± 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation. CONCLUSION: In ALF, these criteria can help establish a diagnosis of WD. Where applicable, slit- lamp examination for presence of Kayser-Fleischer rings and liver biopsy for determination of hepatic copper concentration still remain important for the diagnosis of ALF due to WD. The need for liver transplantation should be evaluated carefully as the prognosis is not necessarily fatal. 关键词：肝豆状核变性急性肝衰竭诊断标准铜代谢

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Diagnostic criteria for acute liver failure due to Wilson disease

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