Cholestasis Reversal: Efficacy of IV Fish Oil

The purpose of this study is to determine whether the omega-3 fatty acid emulsion (Omegaven),
when used in lieu of the conventional soy-based fat emulsion (Intralipid), is effective in
the treatment of parenteral nutrition associated liver disease (PNALD).

Inclusion Criteria:

1. Patients will be PN dependent (unable to meet nutritional needs solely by enteral
nutrition) and are expected to require PN for at least another 30 days

2. Patients considered eligible for study participation must have parenteral nutrition
associated liver disease (PNALD) as defined as a direct bilirubin of > 2 mg/dl or
currently on Omegaven through another protocol. Other causes of liver disease should
be excluded. A liver biopsy is not necessary for treatment.

3. Direct bilirubin > 2.0 mg/dl or already on Omegaven through another protocol

4. Signed patient informed consent.

5. The patient must have utilized standard therapies to prevent the progression of
his/her liver disease including surgical treatment, cyclic PN, avoiding overfeeding,
reduction/removal of copper and manganese from PN, advancement of enteral feeding, and
the use of ursodiol (i..e., Actigall®).

Exclusion Criteria:

1. Pregnancy

2. Other causes of chronic liver disease (Hepatitis C, biliary atresia, and alpha 1
anti-trypsin deficiency).

3. Enrollment in any other clinical trial involving an investigational agent (unless
approved by the designated physicians on the multidisciplinary team)

4. The parent or guardian or child unwilling to provide consent or assent

In rare instances, patients diagnosed with PNALD may later be found to have liver disease
due to other causes in addition to the use of PN (i.e., inborn errors of metabolism, viral
infections ). Such causes may not be known at the time of enrollment and will not preclude
them from continuing in the study. For the sake of statistical analysis, however, these
patients will be excluded although all data will be collected and reviewed.