ACUTE LIVER-FAILURE IN WILSONS-DISEASE

Background: Establishing an early diagnosis is crucial to successfully treat mostly young patients with sudden onset acute hepatic failure as the initial symptom of Wilson's disease. Recognition of the entity of Wilsonian fulminant hepatitis is important, because liver transplantation improves survival if performed in a timely fashion. Methods: Retrospective case analysis regarding characteristic profile of standard labaratory parameters and clinical course of acute Wilsonian hepatic failure. Results: In two female patients (age 17 and 27 years) with non-autoimmune hermolysis serum AST and ALT levels were only moderately elevated with a conspicuously diminished ALT activity and relatively low serum alkaline phosphatase (AP) levels. Despite immediate application of D-Penicillamin one patient died from complications of multiorgan failure. Conclusion: In fulminant Wilsonian hepatic failure the AP/bilirubin ratio is usually below 2. Additionally calculation of free copper concentration in serum and renal excretion of ionic copper in combination with non-autoimmune hemolysis provide clues to establish an early diagnosis of Wilsonian hepatic failure.