Prolonged use of parenteral nutrition can lead to parenteral nutrition associated liver disease (PNALD). The purpose of this study is to determine the effect of treatment with a smaller amount lipid minimization) of our standard soybean oil based intravenous lipid emulsion (Intralipid) versus a fish-oil based lipid emulsion (Omegaven) in infants with severe cholestasis.

Infants meeting eligibility will be randomized to receive either 1g/kg/day of Intralipid® 20% or 1g/kg/day Omegaven® 10%. Infants randomized to Intralipid® whose conjugated bilirubin level rises >100umol/L will be crossed over to receive 1g/kg/day Omegaven®. Monitoring includes liver function tests (AST, ALT,ALP, GGT, Conjugated Bilirubin), Fatty Acid Profile (RBC and serum fatty acids; triene/tetraene ratio), INR (coagulation profile) and cytokine measure (inflammatory markers).

Inclusion Criteria:

• infants admitted to neonatal intensive care unit
• severe cholestasis, defined as conjugated bilirubin greater than 35
• receiving at least 60% calories by IV infusion and expected to require intravenous nutrition for at least an additional 28 days
• signed consent

Exclusion Criteria:

• hepatitis (TORCH or other viral infection)
• primary liver disease as etiology of cholestasis
• clinically severe bleeding not able to be managed with routine measures
• lethal congenital abnormalities
• congenital heart disease associated with right heart dysfunction