Fibrosis hepática congénita: un espectro clínico variable. Casos clínicos / Congenital hepatic fibrosis: report of five cases
Rev. méd. Chile
;
132(6): 733-741, jun. 2004. ilus
Artículo
en Español
| LILACS
| ID: lil-384222
ABSTRACT
Background: Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease. Aim: To describe the medical management of 5 children with CHF and to evaluate the presence and extension of the associated renal disease. Patients and methods: Retrospective review of the medical charts of 5 children with CHF, aged 2 to 14 years. Results: Three children presented autosomic recessive polycystic kidney disease, which was diagnosed before the appearance of liver disease manifestations. They presented a more severe liver damage, with a more aggressive clinical course requiring use of transjugular intrahepatic porto-systemic shunts (TIPS) or surgical porto-systemic shunts to control portal hypertension. The other two children, in whom the diagnosed was based on asymptomatic hepatomegaly, had normal renal function and structure with a more benign clinical course. Conclusions: The diagnosis of CHF should be suspected not only in children with polycystic kidney disease but in those children with persistent, hard consistency, left lobe predominance hepatomegaly (Rev Méd Chile 2004; 132: 733-41).
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Hígado
/
Cirrosis Hepática
Tipo de estudio:
Estudio de etiología
Límite:
Adolescente
/
Niño
/
Child, preschool
/
Femenino
/
Humanos
/
Masculino
Idioma:
Español
Revista:
Rev. méd. Chile
Asunto de la revista:
Medicina
Año:
2004
Tipo del documento:
Artículo
País de afiliación:
Chile
Institución/País de afiliación:
Pontificia Universidad Católica de Chile/CL
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