Persistence of elevated aminotransferases in Wilson's despite adeguate therapy.

Wilson's disease (WD) is characterized by a dramatic build-up of intracellular hepatic copper with subsequent hepatic and neurological abnormalities.1, 2 Prompt recognition of WD is crucial, because if not treated, the disease is fatal.2, 3 Most childhood cases of WD present with liver disease that mimics various liver disorders, while the typical neurological signs are usually absent. Hypertransaminasemia may be the only altered laboratory sign in early WD. Histological evidence of severe liver disease may occur in children with isolated hypertransaminasemia.4, 5 The response of serum transaminase levels to penicillamine and zinc treatment in WD is poorly understood, because the children with WD in earlier studies had a relatively advanced disease, and treatment efficacy was evaluated from parameters (i.e., jaundice, ascites, and neurological symptoms) other than serum transaminases.6–9