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Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF) Turkbey B; Ocak I; Daryanani K; Font-Montgomery E; Lukose L; Bryant J; Tuchman M; Mohan P; Heller T; Gahl WA; Choyke PL; Gunay-Aygun MPediatr Radiol 2009[Feb]; 39 (2): 100-11ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.|Adolescent[MESH]|Adult[MESH]|Child[MESH]|Child, Preschool[MESH]|Cholangiopancreatography, Magnetic Resonance/*methods[MESH]|Diagnosis, Differential[MESH]|Female[MESH]|Fibrosis/congenital/diagnosis[MESH]|Humans[MESH]|Infant, Newborn[MESH]|Liver Cirrhosis/*congenital/*diagnosis[MESH]|Liver/*diagnostic imaging/*pathology[MESH]|Male[MESH]|Middle Aged[MESH]|Polycystic Kidney, Autosomal Recessive/*diagnosis[MESH]|Radiography[MESH]|Ultrasonography/*methods[MESH]|Young Adult[MESH] |
