Congenital hepatic fibrosis in a child with autosomal dominant polycystic kidney disease.
The association of congenital hepatic fibrosis (CHF) with autosomal recessive polycystic kidney disease (ARPKD) is well known and occurs in approximately 50% of cases. However the association of CHF with autosomal dominant polycystic kidney disease (ADPKD) is less well known and less well documented. We report a child with neonatal onset of hypertension due to ADPKD who later develops portal hypertension due to CHF in childhood. A review of this rare association follows.
Kanaheswari
Y
Y
Department of Paediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia, Jalan Yaacob Latiff, Bandar Tun Razak, Cheras, 56000 Kuala Lumpur, Malaysia. kanahes@mail.hukm.ukm.my
Hamzaini
A H
AH
Wong
S W
SW
D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000150:complications* / Q000151:congenital* / Q000175:diagnosis; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis / Q000628:therapy