title: The natural history of cirrhosis from parenteral nutrition-associated liver disease after resolution of cholestasis with parenteral fish oil therapy.
abstract: OBJECTIVE
  To determine the natural history of cirrhosis from parenteral nutrition-associated liver disease (PNALD) after resolution of cholestasis with fish oil (FO) therapy.
  
  
  BACKGROUND
  Historically, cirrhosis from PNALD resulted in end-stage liver disease, often requiring transplantation for survival. With FO therapy, most children now experience resolution of cholestasis and rarely progress to end-stage liver disease. However, outcomes for cirrhosis after resolution of cholestasis are unknown and patients continue to be considered for liver/multivisceral transplantation.
  
  
  METHODS
  Prospectively collected data were reviewed for children with cirrhosis because of PNALD who had resolution of cholestasis after treatment with FO from 2004 to 2012. Outcomes evaluated included need for liver/multivisceral transplantation, mortality, and the clinical progression of liver disease.
  
  
  RESULTS
  Fifty-one patients with cirrhosis from PNALD were identified, with 76% demonstrating resolution of cholestasis after FO therapy. The mean direct bilirubin decreased from 6.4 ± 4 mg/dL to 0.2 ± 0.1 mg/dL (P < 0.001) 12 months after resolution of cholestasis, with a mean time to resolution of 74 days. None of the patients required transplantation or died from end-stage liver disease. Pediatric End-Stage Liver Disease scores decreased from 16 ± 4.6 to -1.2 ± 4.6, 12 months after resolution of cholestasis (P < 0.001). In children who remained PN-dependent, the Pediatric End-Stage Liver Disease score remained normal throughout the follow-up period.
  
  
  CONCLUSIONS
  Cirrhosis from PNALD may be stable rather than progressive once cholestasis resolves with FO therapy. Furthermore, these patients may not require transplantation and show no clinical evidence of liver disease progression, even when persistently PN-dependent.
authors.affiliation: *Department of Surgery and Vascular Biology Program †The Clinical Research Program, Biostatistics Core ‡Department of Pharmacy, Boston Children's Hospital, Harvard Medical School, Boston, MA.
authors.forename: Prathima
authors.initials: P
authors.lastname: Nandivada
authors.forename: Melissa I
authors.initials: MI
authors.lastname: Chang
authors.forename: Alexis K
authors.initials: AK
authors.lastname: Potemkin
authors.forename: Sarah J
authors.initials: SJ
authors.lastname: Carlson
authors.forename: Eileen
authors.initials: E
authors.lastname: Cowan
authors.forename: Alison A
authors.initials: AA
authors.lastname: Oʼloughlin
authors.forename: Paul D
authors.initials: PD
authors.lastname: Mitchell
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: Gura
authors.forename: Mark
authors.initials: M
authors.lastname: Puder
mesh_terms: D000886:Anthropometry; D015415:Biomarkers / Q000097:blood; D002779:Cholestasis / Q000188:drug therapy* / Q000209:etiology; D018450:Disease Progression; D005260:Female; D005395:Fish Oils / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000401:mortality / Q000601:surgery; D008103:Liver Cirrhosis / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D010288:Parenteral Nutrition / Q000009:adverse effects*; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D005395:Fish Oils