Congenital hepatic fibrosis. Report of five cases.

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dc.article.numberG0715
dc.catalogadorpau
dc.contributor.authorHarris, Paul R.
dc.contributor.authorFodor, D.
dc.contributor.authorCavagnaro, F.
dc.contributor.authorDi Egidio, M.
dc.contributor.authorDuarte, I.
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 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.
dc.fuente.origenORCID-ene24
dc.identifier.issn0016-5085
dc.identifier.urihttp://www.scopus.com/inward/record.url?eid=2-s2.0-12444299955&partnerID=MN8TOARS
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/80840
dc.information.autorucEscuela de Medicina; Harris Diez, Paul Richard; 0000-0001-6226-0957; 80706
dc.issue.numero6
dc.language.isoen
dc.nota.accesoContenido completo
dc.revistaRevista Médica de Chile
dc.rightsacceso abierto
dc.subjectHypertensiones_ES
dc.subjectPortales_ES
dc.subjectLiver cirrhosises_ES
dc.subjectPolycystic kidneyes_ES
dc.subjectAntosomal dominantes_ES
dc.subjectPortasystemic shuntes_ES
dc.subjectSurgicales_ES
dc.subjectPortasystemic shuntes_ES
dc.subjectTransjugular intrahepatices_ES
dc.titleCongenital hepatic fibrosis. Report of five cases.es_ES
dc.title.alternativeFibrosis hepática congénita: Un espectro clínico variable. Casos clínicos
dc.typeartículo
dc.volumen132
sipa.codpersvinculados80706
sipa.trazabilidadORCID;2024-01-08
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