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
Artigo
em Espanhol
| 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 andmethods:
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:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Fígado
/
Cirrose Hepática
Tipo de estudo:
Estudo de etiologia
Limite:
Adolescente
/
Criança
/
Criança, pré-escolar
/
Feminino
/
Humanos
/
Masculino
Idioma:
Espanhol
Revista:
Rev. méd. Chile
Assunto da revista:
Medicina
Ano de publicação:
2004
Tipo de documento:
Artigo
País de afiliação:
Chile
Instituição/País de afiliação:
Pontificia Universidad Católica de Chile/CL
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