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Diagnostic criteria for acute liver failure due to Wilson disease

机译:威尔逊病引起的急性肝衰竭的诊断标准

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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.
机译:目的:描述由于威尔逊病(WD)引起的急性肝衰竭的诊断标准,这是急性肝衰竭(ALF)的罕见原因。方法:我们比较了由WD引起的ALF患者和其他病因的ALF患者的发现。结果:先前描述的标准,例如低碱性磷酸酶活性,低碱性磷酸酶与总胆红素的比率或高天冬氨酸氨基转移酶(AST)与丙氨酸氨基转移酶(ALT)的比率,未能鉴定出因WD导致的ALF患者。低ALT和AST活性(分别为53±43 vs 1982±938,P <0.0001和87±44 vs 2756±2941,P = 0.037),胆碱酯酶活性低(1.79±1.2 vs 4.30±1.2)有显着差异,P = 0.009),WD引起的ALF患者尿铜浓度高(93.4±144.0 vs 3.5±1.8,P = 0.001)和低血红蛋白(7.0±2.2 vs 12.6±1.8,P <0.0001)病因。有趣的是,由于WD而导致的ALF的7例患者中有4例在没有肝移植的情况下得以存活。结论:在ALF中,这些标准可以帮助建立WD的诊断。在适用的情况下,裂隙灯检查是否存在Kayser-Fleischer环和肝活检以确定肝铜浓度对于诊断WD引起的ALF仍然很重要。肝移植的必要性应仔细评估,因为其预后不一定致命。

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