Acute cholestatic jaundice in children with sickle cell disease: hepatic crises or hepatitis?

Differentiating acute cholestatic jaundice resulting from hepatic vasoocclusive crises and hepatitis in children with sickle cell disease can be difficult. Both conditions result in hyperbilirubinemia, mainly of the conjugated type, and in elevation of serum transaminases. Five children with sickle cell disease, acute severe cholestatic jaundice and negative serology for hepatitis A and B presented in good general condition, with modest elevation of serum transaminases, and had an early uneventful recovery. Five children with sickle cell disease and serologically proved hepatitis A infection were sicker, exhibited a similar elevation of bilirubin concentration with marked elevation of the serum transaminases and recovered more slowly. The clinical course and outcome of hepatitis A in children with sickle cell disease was similar to that of hepatitis A in normal children. Unlike early reports acute cholestatic jaundice in our patients with sickle cell disease, whether caused by hepatitis or by hepatic vasoocclusive crises, was found to be benign with an uneventful recovery.