Cholestasis associated with long-term parenteral hyperalimentation.

Abstract

Three infants receiving long-term parenteral hyperalimentation developed obstructive jaundice characterized by parallel elevations of total and direct bilirubin with normal serum enzyme determinations. Two infants died from complications of their primary surgical lesion while the third survived with complete reversal of the hepatic involvement. Liver specimens in two patients revealed a unique form of hepatitis having parenchymal cholestasis and periportal infiltration with a marked eosinophilic myelopoiesis. The histologic findings are compatible with complications of a bile secretory defect. Deficiency of a specific amino acid, essential to the formation of normal bile salts, or long-standing intestinal obstruction, could predispose to abnormal or inadequately conjugated bile salts interfering with micelle formation and the normal flow of bile into the canaliculus.