Safety Study to Evaluate the Occurrence of EFAD in Pediatric Patients With PNAC Who Require More Than Eight Weeks of Omegaven Treatment

Prior treatment

• Patient has received Omegaven within four weeks before inclusion in the study
• Patient has been previously diagnosed with, or has prior evidence of, portal vein thrombosis.
• Patient has previously received a liver-only or liver-inclusive transplant.

Other illness

• 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.
• Patient has hemodynamic instability due to any major cardiac anomaly.
• Patient has a major life-threatening disease (e.g., sepsis requiring high-dose vasopressors, acute respiratory distress syndrome, veno-occlusive disease, cancer).
• 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.
• Patient has renal failure and requires renal replacement therapy.
• Patient has a severe hemorrhagic disorder.
• Patient has a known hypersensitivity to fish or egg protein or to any of the active ingredients or excipients of Omegaven.

Lab values

• Patient has severe hyperlipidemia or a severe disorder of lipid metabolism characterized by hypertriglyceridemia (i.e., serum triglyceride level \> 1,000 mg/dL).

Other

• Patient has known cirrhosis (liver biopsy is not required under this protocol).
• Patient has a record of EFAD before inclusion in the study
• Patient has been diagnosed with or is suspected to have an inborn error of metabolism.
• Patient is subject to treatment limitation.
• Patient is enrolled in any other study with an investigational medicinal product during the course of the current study.

• Patient's parent(s) or legal guardian(s) has provided a signed and dated Informed Consent Form (ICF).
• 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.
• Patient has oral or enteral feeding intolerance or at least one gastrointestinal disorder requiring PN.