title: Persistence of hepatic fibrosis in pediatric intestinal failure patients treated with intravenous fish oil lipid emulsion.
abstract: BACKGROUND
  Pediatric intestinal failure (PIF) is a life-altering chronic condition with significant morbidity and mortality. Omegaven® therapy has been used to treat children with advanced intestinal failure associated liver disease. Our objective was to determine the evolution of hepatic fibrosis in PIF patients who received Omegaven® and describe their clinical outcome.
  
  
  METHODS
  A retrospective review in PIF patients who received Omegaven® was performed. Patients were included if they had liver biopsies completed before Omegaven® therapy and after resolution of hyperbilirubinemia. Biopsy results were evaluated to determine the degree of fibrosis, inflammation, and cholestasis. Clinical and biochemical data was collected.
  
  
  RESULTS
  Six patients were identified. Assessment of fibrosis at last follow-up demonstrated improvement in 2 patients and progression or stable fibrosis in 4/6. All patients demonstrated reduction in cholestasis and inflammation. One patient received a liver/intestine transplant and a second is listed, both of them with progressive fibrosis. One patient achieved full enteral nutrition, while the rest remain partially parenteral nutrition dependent.
  
  
  CONCLUSIONS
  Use of Omegaven® is associated with reduced cholestasis and inflammation, but with persistence or worsening of fibrosis in some patients. A subset of patients with progressive fibrosis may develop portal hypertension and progressive liver disease.
authors.affiliation: Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Christina
authors.initials: C
authors.lastname: Belza
authors.affiliation: Division of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Rory
authors.initials: R
authors.lastname: Thompson
authors.affiliation: Division of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Gino R
authors.initials: GR
authors.lastname: Somers
authors.affiliation: Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Nicole
authors.initials: N
authors.lastname: de Silva
authors.affiliation: The Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Kevin
authors.initials: K
authors.lastname: Fitzgerald
authors.affiliation: Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Karen
authors.initials: K
authors.lastname: Steinberg
authors.affiliation: Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Glenda
authors.initials: G
authors.lastname: Courtney-Martin
authors.affiliation: Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada; The Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Paul W
authors.initials: PW
authors.lastname: Wales
authors.affiliation: Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada; Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Canada. Electronic address: yaron.avitzur@sickkids.ca.
authors.forename: Yaron
authors.initials: Y
authors.lastname: Avitzur
mesh_terms: D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004750:Enteral Nutrition; D005217:Fat Emulsions, Intravenous / Q000627:therapeutic use*; D005260:Female; D005395:Fish Oils / Q000627:therapeutic use*; D005500:Follow-Up Studies; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D007410:Intestinal Diseases / Q000150:complications* / Q000628:therapy; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D016031:Liver Transplantation; D008297:Male; D010289:Parenteral Nutrition, Total; D012189:Retrospective Studies; D016037:Single-Blind Method; D016896:Treatment Outcome; D014280:Triglycerides
chemical_list: D005217:Fat Emulsions, Intravenous; D005395:Fish Oils; D014280:Triglycerides; C568345:fish oil triglycerides
keywords: Cholestasis; Fish oil lipid emulsion; Intestinal failure associated liver disease; Liver–intestine transplantation; Parenteral nutrition; Pediatrics