Abstract
Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease. To describe the medical management of 5 children with CHF and to evaluate the presence and extension of the associated renal disease. Retrospective review of the medical charts of 5 children with CHF, aged 2 to 14 years. 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. 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.
Highlights
Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease
Las principales indicaciones en niños han sido atresia biliar, que de por sí son malos candidatos, debido a su edad de presentación, su rápida progresión a cirrosis y la alta frecuencia de alteraciones anatómicas en la vena portal en este grupo de pacientes, fibrosis hepática congénita (FHC), síndrome de BuddChiari, y fibrosis quística
El diagnóstico de la fibrosis hepática congénita (FHC) debe sospecharse, no sólo en niños portadores de ARPKD, sino también en niños que presentan una hepatomegalia persistente, dura y de predominio del lóbulo izquierdo, para iniciar el estudio y manejo adecuado
Summary
Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease. El objetivo de este trabajo es presentar una serie clínica con cinco niños que presentaron FHC, de los cuales dos no presentaron alteraciones ecográficas ni de función renal, poniendo de manifiesto la existencia de pacientes con las lesiones hepáticas características pero sin compromiso renal evidente.
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