[Autoimmune hepatitis in children. Initial presentation as fulminant hepatic failure].
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.
Cuarterolo
M
M
Servicios de Gastroenterología, Hospital de Pediatría Juan P. Garrahan. Bs. As. Argentina. lcuarterol@intramed.Net.ar
López
S
S
G de Dávila
M
M
Mondiglio
C
C
Roy
A
A
Zelazko
M
M
Centeno
M
M
Bes
D
D
Imventarza
O
O
Ciocca
M
M
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