Diagnostic criteria for acute liver failure

Aims: Wilson disease is an uncommon cause of acute liver failure. Early identification is crucial because of the high mortality without liver transplantation. Valid diagnostic criteria for acute liver failure due to Wilson disease have not been established.

Methods: We compared findings of patients presenting with acute liver failure due to Wilson disease to those with acute liver failure of other etiologies.

Results: Previously described criteria such as low alkaline phosphatase activity, low alkaline phosphatase to total bilirubin ratio or high AST to ALT ratio failed to identify patients with acute liver failure due to Wilson disease. Significantly different were low ALT and AST activities (P <0.0001 and P=0.037, respectively), low choline esterase activity (P=0.009), high urine copper concentrations (P=0.001) and low hemoglobin (P <0.0001) in patients with acute liver failure caused by Wilson disease compared to other etiologies. Interestingly, 4 out of 7 patients with acute liver failure due to Wilson disease survived without liver transplantation.

Conclusions: In acute liver failure, these criteria can help establishing a diagnosis of Wilson disease. 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 acute liver failure due to Wilson disease. The need for liver transplantation should be evaluated carefully as the prognosis is apparently not necessarily fatal.