[Congenital hepatic fibrosis. Report of five cases].
BACKGROUND
Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease.


OBJECTIVE
To describe the medical management of 5 children with CHF and to evaluate the presence and extension of the associated renal disease.


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.
Departamento de Pediatría, Secciones de Gastroenterología Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago. pharris@med.puc.cl
Paul
P
Harris
Daniel
D
Fodor
Felipe
F
Cavagnaro
Marcia
M
Di Egidio
Ignacio
I
Duarte
Mario
M
Fava
D000293:Adolescent; D044963:Biopsy, Fine-Needle; D002648:Child; D002675:Child, Preschool; D018618:Echocardiography, Doppler, Color; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000150:complications / Q000151:congenital* / Q000473:pathology*; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D012189:Retrospective Studies