Cholestasis secondary to panhypopituitarism in an infant.

Cholestasis occurring in infancy should be evaluated completely to exclude hepatic as well as endocrine or metabolic causes. A rapid diagnosis should be made to ensure that hepatic and neurologic complications are prevented. We describe a rare case of infant cholestasis due to panhypopituitarism resulting in hypoglycemia and liver biopsy findings consistent with bile duct paucity. Existing bile ducts were noted to be small, and electron microscopy demonstrated diminutive and atrophied biliary cells with a diminished bile duct lumen size. Hypoglycemia and cholestasis resolved with treatment of the underlying panhypopituitarism. Panhypopituitarism should be considered in any infant who presents with cholestasis, hypoglycemia, and other manifestations of pituitary malfunction. Growth hormone deficiency may affect bile duct formation as demonstrated in this patient.