Cholestasis Reversal: Efficacy of IV Fish Oil

Study Overview
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Brief Summary
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The purpose of this study is to determine whether Omegaven is effective in the treatment of parenteral nutrition associated liver disease (PNALD).

Detailed Description
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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).

Official Title
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Cholestasis Reversal: Efficacy of IV Fish Oil

Conditions
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Parenteral Nutrition Associated Liver Disease, Short Bowel Syndrome, Gastrointestinal Disease

Intervention / Treatment
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* Drug: Omegaven®


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: 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 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. Direct bilirubin > 2.0 mg/dl or already on Omegaven through another protocol Signed patient informed consent. 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: Pregnancy Other causes of chronic liver disease (Hepatitis C, biliary atresia, and alpha 1 anti-trypsin deficiency). Enrollment in any other clinical trial involving an investigational agent (unless approved by the designated physicians on the multidisciplinary team) 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.

Ages Eligible for Study
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Maximum Age: 17 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Omegaven<br>1g/kg/day for duration of study participation for all participants | Drug: Omegaven®<br>* 10% Omegaven® 1g/kg/day, IV (in the vein) until the patient no longer requires parenteral nutrition or until participation in the study is terminated<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Efficacy of parenteral administration of fish oil derived fat emulsion (Omegaven®) to reverse established parenteral nutrition associated liver disease |  | 1 year | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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PNALD, Omegaven, Parenteral Nutrition Associated Liver Disease, Cholestasis, Short Bowel Syndrome