Gender
All
Age Group
1 Day to 17 Years
Accepting Healthy Volunteers
No
Inclusion Criteria:
1. Patient's parent(s) or legal guardian(s) has provided a signed and dated Informed
Consent Form (ICF).
2. Pediatric patient (<18 years) has been diagnosed with PNAC, defined as direct or
conjugated bilirubin (DBil) ≥ 2.0 mg/dL with no other known cause of liver
dysfunction at the time of enrollment and is expected to require Omegaven treatment
for at least eight weeks.
3. Patient has oral or enteral feeding intolerance or at least one gastrointestinal
disorder requiring PN.
Exclusion Criteria:
1. Patient has received Omegaven within four weeks before inclusion in the study
2. Patient has any other known cause of chronic liver disease such as hepatitis C,
cystic fibrosis, biliary atresia, alpha-1-antitrypsin deficiency, passive hepatic
congestion due to heart failure, etc.
3. Patient has known cirrhosis (liver biopsy is not required under this protocol).
4. Patient has been previously diagnosed with, or has prior evidence of, portal vein
thrombosis.
5. Patient has previously received a liver-only or liver-inclusive transplant.
6. Patient has hemodynamic instability due to any major cardiac anomaly.
7. Patient has a major life-threatening disease (e.g., sepsis requiring high-dose
vasopressors, acute respiratory distress syndrome, veno-occlusive disease, cancer).
8. Patient has multi-organ failure, septic shock, hypotension requiring pressor
therapy, persistent pulmonary hypertension requiring inhaled nitric oxides, or
requires extracorporeal membrane oxygenation (ECMO) or similar intervention.
9. Patient has renal failure and requires renal replacement therapy.
10. Patient has a severe hemorrhagic disorder.
11. Patient has severe hyperlipidemia or a severe disorder of lipid metabolism
characterized by hypertriglyceridemia (i.e., serum triglyceride level > 1,000
mg/dL).
12. Patient has a record of EFAD before inclusion in the study
13. Patient has been diagnosed with or is suspected to have an inborn error of
metabolism.
14. Patient has a known hypersensitivity to fish or egg protein or to any of the active
ingredients or excipients of Omegaven.
15. Patient is subject to treatment limitation.
16. Patient is enrolled in any other study with an investigational medicinal product
during the course of the current study.