Fibrosis Hepática congénita: un espectro clínico variable: Casos clínicos
| Autor: | Mario Fava P, Ignacio Duarte G. de C., Daniel Fodor O, Paul Harris D, Felipe Cavagnaro Sm, Marcia Di Egidio S |
|---|---|
| Jazyk: | Spanish; Castilian |
| Rok vydání: | 2004 |
| Předmět: |
Pathology
medicine.medical_specialty medicine.diagnostic_test business.industry Retrospective cohort study General Medicine medicine.disease Asymptomatic Polycystic kidney antosomal dominant Normal renal function Liver disease Portasystemic shunt transjugular intrahepatic El Niño Biopsy Liver cirrhosis Portasystemic shunt surgical Medicine Portal hypertension medicine.symptom Hypertension portal business Kidney disease |
| Zdroj: | Revista médica de Chile v.132 n.6 2004 SciELO Chile CONICYT Chile instacron:CONICYT |
| Popis: | 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) |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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