Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
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Date
2004
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Abstract
Background: 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.
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Hypertension, Portalliver cirrhosispolycystic kidney, Antosomal dominantportasystemic shunt, Surgicalportasystemic shunt, Transjugular intrahepatic