title: Childhood autoimmune liver disease: indications and outcome of liver transplantation.
abstract: BACKGROUND
  Graft rejection and disease recurrence are well-recognized complications of liver transplantation (LT) for autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC). We describe indications and outcome of LT for childhood AIH and AISC.
  
  
  METHODS
  Twenty-year retrospective review of a cohort of children (n = 101) with AIH, AISC, or AIH/sclerosing cholangitis overlap syndrome from a single center.
  
  
  RESULTS
  AIH type 1 (AIH1, n = 67) was more common than AIH type 2 (AIH2, n = 18), AISC (n = 8), or overlap syndrome (n = 8). Overall, 18 patients (18%) required LT, the indications being failure of medical therapy (n = 16) and fulminant liver failure (n = 2). Patients with AIH who required LT had a more prolonged prothrombin time at presentation compared with those who did not undergo transplantation (P = 0.01). Patients with AIH1 who received LT had a lower aspartate transaminase (P = 0.009) and alanine transaminase (P = 0.02) levels at initial diagnosis compared with those with AIH1 who did not undergo transplantation. Post-LT, 11 patients (61%) had 18 episodes of rejection, most were steroid sensitive. Disease recurrence was observed in 7 patients (39%, median duration post-LT 33 months), more common in AIH2 (80% recurrence rate), and those taking cyclosporine (71%, 5/7 patients) compared with those taking tacrolimus (18%, 2/11 patients; P < 0.05) and in 3 of 3 children who did not have maintenance steroids post-LT. The overall 5- and 7-year post-LT survival rate was 94% and 88%, respectively.
  
  
  CONCLUSIONS
  LT is a good therapeutic option for progressive AIH and AISC, although recurrence of the primary autoimmune process limits the outcome.
authors.affiliation: Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.
authors.forename: Pei Fan
authors.initials: PF
authors.lastname: Chai
authors.forename: Way Seah
authors.initials: WS
authors.lastname: Lee
authors.forename: Rachel M
authors.initials: RM
authors.lastname: Brown
authors.forename: Jo L
authors.initials: JL
authors.lastname: McPartland
authors.forename: Katharine
authors.initials: K
authors.lastname: Foster
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: McKiernan
authors.forename: Deirdre A
authors.initials: DA
authors.lastname: Kelly
mesh_terms: D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D002648:Child; D015209:Cholangitis, Sclerosing / Q000276:immunology / Q000601:surgery*; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006084:Graft Rejection / Q000453:epidemiology; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000201:enzymology; D017114:Liver Failure, Acute / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000276:immunology / Q000401:mortality; D008297:Male; D011517:Prothrombin Time; D012008:Recurrence; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D016019:Survival Analysis; D015996:Survival Rate; D013577:Syndrome; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D016559:Tacrolimus