title: Liver Transplantation for Children With Primary Sclerosing Cholangitis and Autoimmune Hepatitis: UNOS Database Analysis.
abstract: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the pediatric end-stage liver disease era. We aimed to characterize the outcome of LT in children with AIH and PSC.
  
  
  
  Children 18 years or younger with PSC or AIH who had a first, isolated LT from 2002 to 2012 were identified from the United Network for Organ Sharing database. Graft and patient outcomes were studied.
  
  
  
  A total of 174 children with AIH and 113 with PSC were transplanted in the study period. One-year patient survival was 95.4% for AIH and 97.3% for PSC. Five-year patient survival was 91.4% for AIH and 92.9% for PSC. Patient survival was not significantly different between the 2 groups. Forty-four (25.2%) children with AIH were listed as status 1 for transplant (fulminant hepatic failure at presentation or acute-on-chronic disease). Patients transplanted as status 1 had significantly lower patient survival compared with patients transplanted with AIH and end-stage liver disease. The one- and five-year graft survival rates were not significantly different between patients with AIH and PSC.
  
  
  
  Children with AIH transplanted as status 1 had significantly lower patient survival rates but similar graft survival rates to children with chronic AIH. Children transplanted for AIH versus PSC showed no significant differences in patient or graft survival at both 1 and 5 years.
authors.affiliation: *Department of Pediatrics and RMTI, Mount Sinai Medical Center, New York †Fordham University, Bronx, NY.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Jossen
authors.forename: Rachel
authors.initials: R
authors.lastname: Annunziato
authors.forename: Hee-Sung
authors.initials: HS
authors.lastname: Kim
authors.forename: Jaime
authors.initials: J
authors.lastname: Chu
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000150:complications*; D016208:Databases, Factual; D058625:End Stage Liver Disease / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D019693:Hepatitis, Autoimmune / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017114:Liver Failure, Acute / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D015996:Survival Rate