title: Characteristics and outcome of autoimmune liver disease in Asian children.
abstract: BACKGROUND
  Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population.
  
  
  METHODS
  Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia.
  
  
  RESULTS
  At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [survival with native liver vs. LT or death (duration between onset of disease and treatment; median ± standard error) = 2.5 ± 2.9 months vs. 24.0 ± 13.3 months; p = 0.012].
  
  
  CONCLUSIONS
  Although remission was achieved in the majority of patients with prednisolone and/or azathioprine therapy, delay in seeking diagnosis and treatment adversely affects the outcome of childhood AILD in Malaysia.
authors: Department of Paediatrics, University Malaya Medical Center, 59100, Kuala Lumpur, Malaysia, leews@um.edu.my.
authors: Way S
authors: WS
authors: Lee
authors: Su H
authors: SH
authors: Lum
authors: Chooi B
authors: CB
authors: Lim
authors: Sze Y
authors: SY
authors: Chong
authors: Kim M
authors: KM
authors: Khoh
authors: Ruey T
authors: RT
authors: Ng
authors: Kai M
authors: KM
authors: Teo
authors: Christopher C M
authors: CC
authors: Boey
authors: Jayalakshmi
authors: J
authors: Pailoor
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D044466:Asian People; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D016572:Cyclosporine / Q000627:therapeutic use; D057210:Delayed Diagnosis; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D015212:Inflammatory Bowel Diseases / Q000150:complications; D008103:Liver Cirrhosis / Q000209:etiology; D016031:Liver Transplantation; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008296:Malaysia; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D015996:Survival Rate; D061665:Time-to-Treatment; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone; D001379:Azathioprine