Phytosterolemia in children with parenteral nutrition—Associated cholestatic liver disease

Background: Lipid emulsions used for parenterai nutrition (PN) contain phytosterols. Our hypothesis was that these phytosterols can accumulate and contribute to cholestatic liver disease and other complications of PN, e.g., thrombocytopenia (which occurs in hereditary phytosterolemia). Methods: Using gas chromatography-mass spectrometry, plasma concentrations of sterols were measured in 29 children aged 2 months to 9 years receiving PN and in 29 age-matched controls. The children receiving PN fell into two subgroups: 5 with severe PN-associated cholestatic liver disease (bilirubin level, >100 μmol/L; aspartate aminotransferase [AST]level, >200 U/L) and 24 with a bilirubin level of