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JPEN J Parenter Enteral Nutr. 2016 Mar;40(3):437-40. doi: 10.1177/0148607114567200. Epub 2015 Jan 5.

Reversal of Intestinal Failure-Associated Liver Disease by Switching From a Combination Lipid Emulsion Containing Fish Oil to Fish Oil Monotherapy.

Author information

1
Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Intestinal Rehabilitation Team, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
2
Intestinal Rehabilitation Team, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Department of Pharmaceutical Services, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
3
Intestinal Rehabilitation Team, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Department of Clinical Nutrition, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
4
Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
5
Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Intestinal Rehabilitation Team, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea jm0815.seo@samsung.com.

Abstract

Intestinal failure-associated liver disease (IFALD) is a serious complication of parenteral nutrition (PN). Studies have shown that the amount and content of intravenous lipid emulsions (LEs) used is closely related to the development of IFALD. We report 2 cases of IFALD reversed by switching from a combination lipid emulsion containing fish oil to fish oil monotherapy (Omegaven; Fresenius Kabi Austria Gmbh, Graz, Austria). Patients initially received PN containing SMOFlipid 20% (SMOF; Fresenius Kabi Austria Gmbh, Graz, Austria), 2.0-3.0 g/kg/d, over 24 hours. When IFALD developed, LE was switched from SMOF to Omegaven starting at 1.0 g/kg/d over 12 hours. Case 1 was an 11-month-old girl with a diagnosis of extensive Hirschsprung disease up to the proximal jejunum. She developed direct bilirubinemia at 3 months, and the patient's LE was switched to Omegaven. A decrease in direct bilirubin was observed after 60 days on Omegaven, and IFALD was completely resolved after 90 days. Case 2 was a 1-month-old boy with a history of gastroschisis diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome. He could not tolerate any oral feeds and was kept on full PN. He had elevated direct bilirubin and developed IFALD since 5 weeks. Omegaven treatment was initiated at 5 months. Direct bilirubin rose to 8 mg/dL during the first month on Omegaven. Then a gradual decrease in direct bilirubin was observed, and after 5 months on Omegaven, IFALD was completely resolved. In conclusion, 2 infants with advanced IFALD showed reversal of cholestasis by switching from SMOF to Omegaven monotherapy.

KEYWORDS:

fish oil; intestinal failure; intestinal failure‚Äďassociated liver disease; intravenous lipid emulsion; parenteral nutrition

PMID:
25560679
DOI:
10.1177/0148607114567200
[Indexed for MEDLINE]

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