Expanded Access of Omegaven IV Fat Emulsion to Infants and Children With PNALD

The etiology of parenteral nutrition associated liver disease (PNALD) is currently considered
multifactorial. Available treatment options for this disease process are limited and have
achieved moderate success at best. Unlike conventional intravenous fat emulsions, Omegaven™
is comprised solely of fish oils containing primarily omega-3 fatty acids. The investigators
hypothesize that administering Omegaven™ to patients with PNALD in place of conventional fat
emulsion may reverse cholestasis allowing patients to be maintained on adequate PN until they
are able to ingest adequate nutrition enterally.

Inclusion Criteria:

1. Pediatric patients aged 0 - 18 yrs

2. Patients will be PN dependent (unable to meet nutritional needs solely by enteral
nutrition). It will be assumed that patients will require parenteral nutrition for at
least 30 days from the inclusion into the study.

3. Patients must have parenteral nutrition associated liver disease (PNALD) as defined as
a direct bilirubin of 2mg/dl or more. Other causes of liver disease should be
excluded. A liver biopsy is not necessary for treatment.

4. 2 consecutive direct bilirubins > 2.0 mg/dl. one week apart.

5. Signed patient informed consent.

6. The patient must have utilized standard therapies as clinically appropriate to prevent
the progression of his/her liver disease. Such therapies could include 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. Documented cause of chronic liver disease other than parenteral nutrition associated
liver disease.

2. An allergy to any seafood product, egg protein, and/or previous allergy to Omegaven.

3. Active coagulopathy characterized by ongoing bleeding or by a requirement for clotting
factor replacement (e.g. fresh frozen plasma or cryoprecipitate) to maintain
homeostasis.

4. Impaired lipid metabolism or severe hyperlipidemia with or without pancreatitis.(e.g.
(triglycerides > 1000mg/dl while receiving intralipid 1g/kg/day or less)

5. Unstable diabetes mellitus or hyperglycemia (Blood glucose >200 mg/dL) at the time of
initiation of Omegaven

6. Currently being treated for stroke, embolism, collapse and shock, myocardial
infarction.

7. Cholestasis due to any reason other than parenteral associated liver disease

8. Active new infection at time of initiation of Omegaven

9. Hemodynamic instability (e.g. receiving vasopressors) at time of initiation of
Omegaven.

10. The patient may not be enrolled in any other clinical trial involving an
investigational agent (unless approved by the designated physicians on the
multidisciplinary team).

11. Known pregnancy.