Rozšířený přístup k tukové emulzi Omegaven IV pro kojence a děti s PNALD

Inclusion Criteria:

1. Pediatric patients aged 0 - 18 yrs
2. Patients will be PN dependent (unable to meet nutritional needs solely by enteral nutrition). It will be assumed that patients will require parenteral nutrition for at least 30 days from the inclusion into the study.
3. Patients must have parenteral nutrition associated liver disease (PNALD) as defined as a direct bilirubin of 2mg/dl or more. Other causes of liver disease should be excluded. A liver biopsy is not necessary for treatment.
4. 2 consecutive direct bilirubins > 2.0 mg/dl. one week apart.
5. Signed patient informed consent.
6. The patient must have utilized standard therapies as clinically appropriate to prevent the progression of his/her liver disease. Such therapies could include 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:

1. Documented cause of chronic liver disease other than parenteral nutrition associated liver disease.
2. An allergy to any seafood product, egg protein, and/or previous allergy to Omegaven.
3. Active coagulopathy characterized by ongoing bleeding or by a requirement for clotting factor replacement (e.g. fresh frozen plasma or cryoprecipitate) to maintain homeostasis.
4. Impaired lipid metabolism or severe hyperlipidemia with or without pancreatitis.(e.g. (triglycerides > 1000mg/dl while receiving intralipid 1g/kg/day or less)
5. Unstable diabetes mellitus or hyperglycemia (Blood glucose >200 mg/dL) at the time of initiation of Omegaven
6. Currently being treated for stroke, embolism, collapse and shock, myocardial infarction.
7. Cholestasis due to any reason other than parenteral associated liver disease
8. Active new infection at time of initiation of Omegaven
9. Hemodynamic instability (e.g. receiving vasopressors) at time of initiation of Omegaven.
10. The patient may not be enrolled in any other clinical trial involving an investigational agent (unless approved by the designated physicians on the multidisciplinary team).
11. Known pregnancy.