Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos

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dc.catalogadorpau
dc.contributor.authorHarris, Paul R.
dc.contributor.authorFodor, D.
dc.contributor.authorCavagnaro, F.
dc.contributor.authorDi Egidio, M.
dc.contributor.authorFava, M.
dc.date.accessioned2024-01-19T19:22:44Z
dc.date.available2024-01-19T19:22:44Z
dc.date.issued2004
dc.description.abstractBackground: Congenital hepalic fibrosis (CHF) is an autosomic dominant disease that has been associated will polycistic kidney disease. Aim. To describe The medical managment 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 polycistic 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 CHT should be suspected not only in children with polycystic kidney disease but in those children with persistent, hard consistently, left lobe predominance hepatomegaly.
dc.fechaingreso.objetodigital2024-04-10
dc.fuente.origenORCID-ene24
dc.identifier.doi10.4067/s0034-98872004000600010
dc.identifier.issn0717-6163
dc.identifier.urihttps://doi.org/10.4067/s0034-98872004000600010
dc.identifier.urihttps://publons.com/wos-op/publon/12716502/
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/80842
dc.information.autorucEscuela de Medicina; Harris Diez, Paul Richard; 0000-0001-6226-0957; 80706
dc.issue.numero6
dc.language.isoes
dc.nota.accesoContenido completo
dc.revistaRevista Médica de Chile
dc.rightsacceso abierto
dc.subjectHypertensiones_ES
dc.subjectPortalliver cirrhosispolycystic kidneyes_ES
dc.subjectAntosomal dominantportasystemic shuntes_ES
dc.subjectSurgicalportasystemic shuntes_ES
dc.subjectTransjugular intrahepatices_ES
dc.titleFibrosis Hepática congénita: un espectro clínico variable: Casos clínicoses_ES
dc.titleFibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
dc.title.alternativeCongenital hepatic fibrosis. Report of five cases
dc.typeartículo
dc.volumen132
sipa.codpersvinculados80706
sipa.trazabilidadORCID;2024-01-08
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