The purpose of this study is to determine if a fat emulsion comprised of omega-3 fatty acids, Omegaven, would be beneficial in the management of steatotic liver injury in parenteral nutrition (PN) by its inhibition of de novo lipogenesis, the reduction of arachidonic acid-derived inflammatory mediators, prevention of essential fatty acid deficiency through the presence of small amounts of arachidonic acid, and improved clearance of lipids from the serum.

Inclusion Criteria:

• Greater than 14 days old.
• Have PN-associated cholestasis defined as at least 2 consecutive direct bilirubin >2 mg/dL with anatomical or functional short gut (OR >4 mg/dL if intact intestine) obtained at least 1 week apart with a ratio of direct: total bilirubin > 0.4.
• Patients will be PN dependent (unable to meet nutritional needs solely by enteral nutrition) and are expected to require PN for at least 4 more weeks.
• Patients must have failed standard therapies for Parenteral Nutrition Associated Liver Disease (PNALD) including: cycling of PN, avoiding overfeeding, reduction/removal of copper and manganese from PN, advancement of enteral feeding, and use of ursodiol (i.e. Actigall).
• Signed patient informed consent.
• The patient is expected to have a reasonable possibility of survival.
• No other known etiology of cholestasis other than PNALD at time of Omegaven® initiation.

Exclusion Criteria:

• Causes of cholestasis other than PNALD including but not limited to Hepatitis C, Cystic fibrosis, biliary atresia, and alpha 1 anti-trypsin deficiency, prior to Omegaven® initiation.

• Known fish or egg allergy

• Any of the contraindications to use of Omegaven®:

  1. Active new infection at the time of initiation of Omegaven®
  2. Hemodynamic instability
  3. Use of medications with associated risk of bleeding, including nonsteroidal antiinflammatory drugs (NSAIDs)
  4. Active coagulopathy or bleeding
  5. Thrombocytopenia
  6. Unstable hyperglycemia
  7. Impaired lipid metabolism (triglycerides >1000 mg/dL) while on 1 g/kg/day or less of Intralipid
  8. History of severe hemorrhagic disorders (ie. hemophilia, Von Willebrand disease, etc.)
  9. Unstable diabetes mellitus
  10. Collapse and shock
  11. Stroke/ Embolism
  12. Cardiac infarction within the last 3 months
  13. Undefined coma status

• Enrollment in a clinical trial involving an investigational agent (unless approved by the designated physicians on the multidisciplinary team)

• The parent or guardian of child is unwilling to provide consent or assent