Omegaven Protocol:Intermediate Size Patient Population
Parenteral Nutrition Associated Liver Disease
About this trial
This is an expanded access trial for Parenteral Nutrition Associated Liver Disease
Eligibility Criteria
Inclusion Criteria:
- 1. Inborn and transferred newborns born between the gestational age of 22-40 weeks who develop chronic liver failure, secondary to parenteral nutrition.
2. Patients will be PN dependent (unable to meet nutritional needs soley by enteral nutrition) and are expected to require PN for at least another 30 days 3. Patients must have diagnosis of parenteral nutrition associated liver disease (PNALD) as defined by serum direct bilirubin greater than 2mg/dL on 2 consecutive occasions 4. have had PNALD for at least four weeks prior to planned Omegaven initiation 5. Direct bilirubin > 2.0 mg/dl 6. Signed patient informed consent. 7. 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:
- 2. Other causes of chronic liver disease (Hepatitis C, Cystic fibrosis, biliary atresia, and alpha 1 anti-trypsin deficiency,) 3. severe and/or unstable concomitant systemic disease such as complex congenital cardiac disease, renal failure, autoimmune disease, sepsis, inborn error of metabolism, genetic liver disease, 4. bleeding disorder; 5. biochemical disturbance with potential of worsening with proposed treatment, e.g. persistent hyperglycemia, hypertriglyceridemia, hypercalcemia.
6. 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.