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.
- 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®
- 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
- 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
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,
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