Changing the paradigm: omegaven for the treatment of liver failure in pediatric short bowel syndrome.
BACKGROUND
Parenteral omega-3 fatty acids, such as Omegaven, may benefit patients with pediatric short bowel syndrome (SBS) who develop parenteral nutrition-associated liver disease (PNALD).


METHODS
Retrospective cohort describing the outcome of all 12 children with SBS and advanced PNALD who were treated with Omegaven (target omega-6 to omega-3 fatty acid ratio = 1:1 to 2:1).


RESULTS
The median age was 7.5 (range 3.6-46) months, and median parenteral nutrition duration before starting Omegaven was 28.4 (range 15.3-55.3) weeks. Median initial serum conjugated bilirubin was 137 (range 54-203) micromol/L (8.06 [3.18-11.94] mg/dL). Of the 12 patients, 9 had complete and sustained resolution of hyperbilirubinemia within a median of 24 (range 7-37) weeks, and all are no longer being considered for liver transplantation. Improvements in markers of hepatic inflammation as well as nutritional status also were noted in these patients. Three patients received a liver-intestine transplant while taking Omegaven. There were no complications attributable to Omegaven.


CONCLUSIONS
Omegaven is associated with restoration of liver function in patients with SBS and advanced liver disease. Parenteral omega-3 fatty acids, such as Omegaven, have the potential to fundamentally alter the paradigm of neonatal SBS from one of early death or transplantation from liver failure to a more chronic disease. More children with SBS should achieve full enteral tolerance and those who do not have the capacity for intestinal adaptation should be able to survive and receive an intestinal graft when older.
Diamond
Ivan R
IR
The Hospital for Sick Children, Toronto, Canada.
Sterescu
Anca
A
Pencharz
Paul B
PB
Kim
Jae H
JH
Wales
Paul W
PW
D002675:Child, Preschool; D015331:Cohort Studies; D005217:Fat Emulsions, Intravenous; D015525:Fatty Acids, Omega-3 / Q000008:administration & dosage* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D007421:Intestine, Small / Q000637:transplantation; D008099:Liver / Q000187:drug effects / Q000502:physiology*; D017093:Liver Failure / Q000139:chemically induced / Q000188:drug therapy*; D008297:Male; D010289:Parenteral Nutrition, Total / Q000009:adverse effects*; D020127:Recovery of Function; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000628:therapy*
D005217:Fat Emulsions, Intravenous; D015525:Fatty Acids, Omega-3