Compassionate Use of Omegaven to Reverse Parenteral Nutrition Induced Cholestasis

Enrollment of subjects into this study will occur for up to 4 years. Subjects will receive
Omegaven at a dose of up to 1 g/kg body weight/day until they no longer require total
parenteral nutrition or until their conjugated/direct bilirubin has normalized and their
enteral lipid intake is sufficient to discontinue intravenous lipids.

Inclusion Criteria:

- Males and females ages one month of age to 18 years of age

- Patients with intestinal failure on TPN

- Patients who have a conjugated/direct bilirubin of ≥3 mg/dl for more than weeks and in
whom other causes of cholestasis have been excluded with reasonable certainty
utilizing biochemical, serologic, microbiologic, and radiographic techniques. Liver
biopsy is not required to rule out other disorders, but may be utilized at the
clinician's discretion

- Patients in whom reduction of IV soy-based lipid to an average <1.2g/kg body
weight/day has failed to reduce the conjugated/direct bilirubin within ≥ 30 days of
implementation

- Willing to use birth control during study participation for females of child- bearing
potential, as determined by investigator.

- Signed informed consent for use of Omegaven® obtained

Exclusion Criteria:

- Any of the contraindications to use of Omegaven®

- Impaired lipid metabolism (triglycerides >1000 mg/dL) while on

1g/kg/day or less of Intralipid

- History of severe hemorrhagic disorders (ie. hemophilia, Von Willebrand disease,
etc.)

- Unstable diabetes mellitus

- Collapse and shock

- Stroke/ Embolism

- Cardiac infarction within the last 3 months

- Undefined coma status

- Pregnancy (positive pregnancy test) prior to enrollment in the study for females
of child-bearing potential

- Females of child-bearing potential who are unwilling to use birth control during
study participation

- Parental decision to forego the use of Omegaven®

- Known fish or egg allergy

- Pregnancy

- Causes of liver disease other than PNAC