Safety Study of SMOFlipid to Evaluate the Risk of Developing EFAD and/or PNAC in Pediatric and Adult Patients

Age Limits: and Adults

Inclusion Criteria:

1. Male or female patients, at least 1 month of age.

2. Patients who require PN for at least 5 days/week.

3. Patients who receive 80% or more of their total energy requirements as PN atenrollment and who are expected to receive 80% or more of their total energyrequirements as PN for at least 56 days.

4. Written informed consent. In case of pediatric patients, informed consent must beobtained from parent(s) or legal representatives. If possible, the assent of thepediatric patient must also be obtained (according to local law).

Exclusion Criteria:

1. Use of any other lipid injectable emulsion than SMOFlipid within 6 months prior tostudy participation

2. Known hypersensitivity to fish, egg, soybean, or peanut proteins, or to any of theactive ingredients or excipients of SMOFlipid.

3. Hyperlipidemia or disorders of lipid metabolism characterized byhypertriglyceridemia (serum triglyceride concentration >250 mg/dL in infants or >400mg/dL in older pediatric and adult patients).

4. Inborn errors of amino acid metabolism.

5. Cardiopulmonary instability (including pulmonary edema, cardiac insufficiency,myocardial infarction, acidosis and hemodynamic instability requiring significantvasopressor support).

6. Hemophagocytic syndrome.

7. Liver enzymes (either AST, or ALT, or GGT) exceeding 2 x upper limit of normal range

8. Direct bilirubin exceeding 2 x upper limit of normal range

9. INR exceeding 2 x upper limit of normal range and patient not receiving oralanticoagulants.

10. Any known hepatic condition outside of IFALD that will increase direct bilirubin ≥2.0 mg/dL.

11. Clinically significant abnormal levels of any serum electrolyte (sodium, potassium,magnesium, calcium, chloride, phosphate).

12. Active bloodstream infection demonstrated by positive blood culture at screening.

13. Severe renal failure (eGFR <15 ml/min per 1.73 m2) including patients on renalreplacement therapy.

14. Abnormal blood pH, oxygen saturation, or carbon dioxide.

15. Pregnancy or lactation.

16. Participation in another interventional clinical study.

17. Unlikely to survive longer than 56 days.