Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos

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: Ret...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Harris D,Paul, Fodor O,Daniel, Cavagnaro SM,Felipe, Egidio S,Marcia Di, Duarte G De C,Ignacio, Fava P,Mario
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2004
Materias:	Hypertension, portal Liver cirrhosis Polycystic kidney, antosomal dominant Portasystemic shunt, surgical Portasystemic shunt, transjugular intrahepatic
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872004000600010
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:scielo:S0034-98872004000600010
record_format	dspace
spelling	oai:scielo:S0034-988720040006000102014-08-14Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicosHarris D,PaulFodor O,DanielCavagnaro SM,FelipeEgidio S,Marcia DiDuarte G De C,IgnacioFava P,Mario Hypertension, portal Liver cirrhosis Polycystic kidney, antosomal dominant Portasystemic shunt, surgical Portasystemic shunt, transjugular intrahepatic 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)info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.132 n.6 20042004-06-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872004000600010es10.4067/S0034-98872004000600010
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Hypertension, portal Liver cirrhosis Polycystic kidney, antosomal dominant Portasystemic shunt, surgical Portasystemic shunt, transjugular intrahepatic
spellingShingle	Hypertension, portal Liver cirrhosis Polycystic kidney, antosomal dominant Portasystemic shunt, surgical Portasystemic shunt, transjugular intrahepatic Harris D,Paul Fodor O,Daniel Cavagnaro SM,Felipe Egidio S,Marcia Di Duarte G De C,Ignacio Fava P,Mario Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
description	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)
author	Harris D,Paul Fodor O,Daniel Cavagnaro SM,Felipe Egidio S,Marcia Di Duarte G De C,Ignacio Fava P,Mario
author_facet	Harris D,Paul Fodor O,Daniel Cavagnaro SM,Felipe Egidio S,Marcia Di Duarte G De C,Ignacio Fava P,Mario
author_sort	Harris D,Paul
title	Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
title_short	Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
title_full	Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
title_fullStr	Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
title_full_unstemmed	Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
title_sort	fibrosis hepática congénita: un espectro clínico variable: casos clínicos
publisher	Sociedad Médica de Santiago
publishDate	2004
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872004000600010
work_keys_str_mv	AT harrisdpaul fibrosishepaticacongenitaunespectroclinicovariablecasosclinicos AT fodorodaniel fibrosishepaticacongenitaunespectroclinicovariablecasosclinicos AT cavagnarosmfelipe fibrosishepaticacongenitaunespectroclinicovariablecasosclinicos AT egidiosmarciadi fibrosishepaticacongenitaunespectroclinicovariablecasosclinicos AT duartegdecignacio fibrosishepaticacongenitaunespectroclinicovariablecasosclinicos AT favapmario fibrosishepaticacongenitaunespectroclinicovariablecasosclinicos
_version_	1718436154611597312

Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos

Ejemplares similares