title: Reversal of Intestinal Failure-Associated Liver Disease by Switching From a Combination Lipid Emulsion Containing Fish Oil to Fish Oil Monotherapy.
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.
authors.affiliation: 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.
authors.forename: Sanghoon
authors.initials: S
authors.lastname: Lee
authors.affiliation: 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.
authors.forename: Hyo Jung
authors.initials: HJ
authors.lastname: Park
authors.affiliation: 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.
authors.forename: Jihye
authors.initials: J
authors.lastname: Yoon
authors.affiliation: 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.
authors.forename: Seul Hee
authors.initials: SH
authors.lastname: Hong
authors.affiliation: 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.
authors.forename: Chae-Youn
authors.initials: CY
authors.lastname: Oh
authors.affiliation: Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Suk-Koo
authors.initials: SK
authors.lastname: Lee
authors.affiliation: 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.
authors.forename: Jeong-Meen
authors.initials: JM
authors.lastname: Seo
mesh_terms: D000015:Abnormalities, Multiple / Q000628:therapy; D001663:Bilirubin / Q000378:metabolism; D002779:Cholestasis / Q000150:complications / Q000628:therapy; D003106:Colon / Q000002:abnormalities; D005217:Fat Emulsions, Intravenous / Q000494:pharmacology*; D005260:Female; D005395:Fish Oils / Q000494:pharmacology*; D020139:Gastroschisis / Q000150:complications / Q000628:therapy*; D006627:Hirschsprung Disease / Q000150:complications / Q000628:therapy*; D006801:Humans; D006932:Hyperbilirubinemia / Q000150:complications / Q000628:therapy; D007223:Infant; D007418:Intestinal Pseudo-Obstruction / Q000150:complications / Q000628:therapy; D008107:Liver Diseases / Q000150:complications / Q000628:therapy*; D008297:Male; D010288:Parenteral Nutrition / Q000379:methods; D014280:Triglycerides; D001743:Urinary Bladder / Q000002:abnormalities
chemical_list: D005217:Fat Emulsions, Intravenous; D005395:Fish Oils; D014280:Triglycerides; C568345:fish oil triglycerides; D001663:Bilirubin
keywords: fish oil; intestinal failure; intestinal failure–associated liver disease; intravenous lipid emulsion; parenteral nutrition