Hepatitis autouinmune en niños. Presentación inicial como falla hepática fulminante / Autoimmune hepatitis in children. Initial presentation of fulminant hepatic failure
Acta gastroenterol. latinoam
;
30(4): 245-7, 2000. ilus, tab
Artículo
en Español
| LILACS
| ID: lil-272968
RESUMO
There are few cases reported of autoinmune hepatitis (AIH) tipe 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 per cent 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). EVOLUTION 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 per cent of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosupreessive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative treatment for this severe disease.
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Índice:
LILACS (Américas)
Asunto principal:
Fallo Hepático
/
Hepatitis Autoinmune
Tipo de estudio:
Estudio diagnóstico
/
Estudio observacional
/
Estudio pronóstico
/
Factores de riesgo
Límite:
Adolescente
/
Niño
/
Child, preschool
/
Femenino
/
Humanos
Idioma:
Español
Revista:
Acta gastroenterol. latinoam
Asunto de la revista:
Gastroenterologia
Año:
2000
Tipo del documento:
Artículo
País de afiliación:
Argentina
Institución/País de afiliación:
Hospital de Pediatría Juan P. Garrahan/AR
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