title: [Autoimmune hepatitis in children. Initial presentation as fulminant hepatic failure].
abstract: There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF.
  
  
  METHODS
  Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7%) were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day).
  
  
  RESULTS
  Patients 1 and 3 died while waiting for liver transplant (LT) at 72 and 48 hours after initiating medical treatment. Patient 2 underwent LT3 days after starting treatment, with excellent evolution at 3 years and 7 months of follow up.
  
  
  CONCLUSIONS
  1--AIH type 2 was very infrequent in our group. 2--33% of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosuppressive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative treatment for this severe disease.
authors.affiliation: Servicios de Gastroenterología, Hospital de Pediatría Juan P. Garrahan. Bs. As. Argentina. lcuarterol@intramed.Net.ar
authors.forename: M
authors.initials: M
authors.lastname: Cuarterolo
authors.forename: S
authors.initials: S
authors.lastname: López
authors.forename: M
authors.initials: M
authors.lastname: G de Dávila
authors.forename: C
authors.initials: C
authors.lastname: Mondiglio
authors.forename: A
authors.initials: A
authors.lastname: Roy
authors.forename: M
authors.initials: M
authors.lastname: Zelazko
authors.forename: M
authors.initials: M
authors.lastname: Centeno
authors.forename: D
authors.initials: D
authors.lastname: Bes
authors.forename: O
authors.initials: O
authors.lastname: Imventarza
authors.forename: M
authors.initials: M
authors.lastname: Ciocca
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications / Q000473:pathology; D017093:Liver Failure / Q000150:complications*; D016031:Liver Transplantation; D012737:Sex Factors