Hepatobiliary dysfunction in infants and children associated with long-term total parenteral nutrition. A clinico-pathologic study.

Hepatobiliary dysfunction is a well recognized complication in infants and children on long-term total parenteral nutrition. This clinical-pathological study of fifteen patients with this syndrome suggests that cholestasis is the primary pathogenetic mechanism. The cause of the cholestasis is not well understood, but does not appear to be primarily related to the type of intravenous fluids or the occurrence of sepsis. It is suggested that the prolonged fasting results in disruption of the normal gastro-intestinal mechanisms responsible for bile production and flow. This is supported by the pathological findings, the fact that hepatobiliary dysfunction develops late (usually around 2-3 months), the observation that elevated bile acids and direct hyperbilirubinemia occurs prior to any evidence of hepatocellular necrosis and the occurrence of cholelithiasis in some patients.