title: Long-Term Fish Oil Lipid Emulsion Use in Children With Intestinal Failure-Associated Liver Disease [Formula: see text].
abstract: Fish oil lipid emulsion (FOLE) and multidisciplinary care for infants with intestinal failure (IF) have been associated with reduced morbidity and mortality due to IF-associated liver disease (IFALD). With increased survival, a greater proportion of infants with IF are now able to remain on parenteral nutrition (PN) in the long term. The purpose of this study was to examine outcomes in children with IFALD who have required long-term PN and FOLE therapy due to chronic IF.
  
  
  
  A review of prospectively collected data was performed for children with IFALD who required at least 3 years of PN and FOLE therapy due to chronic IF. Outcomes examined include the incidence of death, transplantation, and essential fatty acid deficiency (EFAD), as well as growth parameters and the biochemical markers of liver disease.
  
  
  
  Of 215 patients with IFALD treated from 2004-2015, 30 required PN and FOLE therapy for at least 3 years (median, 4.6 years). To date, no patients have died, required transplantation, or developed EFAD. Biochemical markers of liver disease normalized within the first year of therapy with no recurrent elevations in the long term. Weight-for age and length-for-age z scores improved and PN dependence decreased in the first year of therapy, with a stable rate of growth in the long term.
  
  
  
  Children with IFALD who required long-term PN and FOLE for chronic IF had no mortality, need for transplantation, EFAD, or recurrence of liver disease in the long term, allowing for continued intestinal rehabilitation.
authors.affiliation: 1 Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Prathima
authors.initials: P
authors.lastname: Nandivada
authors.affiliation: 1 Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Gillian L
authors.initials: GL
authors.lastname: Fell
authors.affiliation: 2 Clinical Research Center, Boston Children's Hospital, Boston, Massachusetts, USA.
authors.forename: Paul D
authors.initials: PD
authors.lastname: Mitchell
authors.affiliation: 1 Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Alexis K
authors.initials: AK
authors.lastname: Potemkin
authors.affiliation: 1 Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Alison A
authors.initials: AA
authors.lastname: O'Loughlin
authors.affiliation: 3 Department of Pharmacy, Boston Children's Hospital, Boston, Massachusetts, USA.
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: Gura
authors.affiliation: 1 Vascular Biology Program and Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Mark
authors.initials: M
authors.lastname: Puder
mesh_terms: D015415:Biomarkers / Q000097:blood; D002657:Child Development / Q000187:drug effects; D002908:Chronic Disease; D023381:Endpoint Determination; D005217:Fat Emulsions, Intravenous / Q000008:administration & dosage*; D005228:Fatty Acids, Essential / Q000008:administration & dosage / Q000097:blood / Q000172:deficiency; D005260:Female; D005395:Fish Oils / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007410:Intestinal Diseases / Q000150:complications / Q000628:therapy*; D017093:Liver Failure / Q000150:complications / Q000628:therapy*; D008297:Male; D010288:Parenteral Nutrition; D012189:Retrospective Studies; D013024:Soybean Oil / Q000008:administration & dosage
chemical_list: D015415:Biomarkers; D005217:Fat Emulsions, Intravenous; D005228:Fatty Acids, Essential; D005395:Fish Oils; D013024:Soybean Oil
keywords: IFALD; PNALD; fish oil lipid emulsion; intestinal failure-associated liver disease; long term; parenteral nutrition; parenteral nutrition-associated liver disease