Safer Parenteral Nutrition in Pediatric Short Bowel Syndrome to Decrease Liver Damage

Inclusion Criteria:

- The targeted population for enrollment is the cohort of patients with TPN-dependent
short bowel syndrome, defined as any pediatric patient who, following abdominal
surgery, has a residual small bowel length less than 25% of that predicted for
gestational age or requires postoperative TPN for more than 42 days because of
gastrointestinal intolerance and who has developed TPN-associated liver disease
sufficient to pose a significant risk for progression to liver failure based on the
following criteria:

- Total serum bilirubin concentration greater than 3 mg/dL after a total duration
of TPN greater than 2 months in the absence of a proven episode of bacteremia
within the preceding 3 weeks.

PLUS EITHER:

• Platelet count less than 200,000/μL after a total duration of TPN greater than 2 months
in the absence of a proven episode of bacteremia within the preceding 3 weeks.

OR

• Serum albumin concentration less than 3.2 mg/dL after a total duration of TPN greater
than 2 months in the absence of a proven episode of bacteremia within the preceding 3
weeks.

Patients with coagulopathy due to parenteral nutrition-associated liver disease (INR > 1.2)
will be potential candidates for enrollment, because patients with an elevated INR
exceeding 2 have demonstrated resolution of coagulopathy after treatment with Omegaven®.
Similarly, patients with hyperlipidemia will be potential candidates for enrollment.

Alternatively, patients currently receiving Omegaven that was initiated at another center
because of intestinal failure with liver disease that do not need to meet the lab criteria
listed above. The subject may continue Omegaven under this protocol at the discretion of
the Principle Investigator.

Exclusion Criteria:

Patients with a history of the following will be excluded from enrollment in this protocol:

- Allergy to fish or egg protein.

- Liver disease proven or suspected to be caused by a process other than TPN-dependent
short bowel syndrome, including but not limited to hepatitis C, hepatitis B, cystic
fibrosis, biliary atresia, Alagille syndrome, familial intrahepatic cholestasis, and
alpha-1-antitrypsin deficiency.

- Refusal of third party providers to reimburse hospital for the cost of Omegaven®.