Refractory cholestasis presenting as cholangiolitis in an Rh (E)-incompatible neonate.

Cholestasis in neonates is infrequently associated with Rh isoimmunization, and usually resolves within a month. The suggested pathophysiology is inspissated bile and hepatocellular damage. We report a rare case of refractory cholestasis presenting with cholangiolitis in a newborn with anti-E isoimmunisation. The cholangiolitis was disclosed by immunohistochemical investigation of conjugated hyperbilirubinaemia and by liver biopsy, which showed a number of CD8(+) lymphocytes within the portal tract damaging the interlobular bile duct. Bilirubin levels dramatically decreased after 14-day corticosteroid therapy (prednisolone, 2 mg/kg/day) implying that the cause of cholestasis could be immune-mediated cholangiolitis.