Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)
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ARPKD/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.
KeywordsAutosomal recessive polycystic kidney disease Congenital hepatic fibrosis US MR cholangiography Children
We thank the ARPKD/CHF Alliance and all patients and their families who generously participated in this investigation. Supported by the intramural research programs of the National Human Genome Research Institute, National Cancer Institute, National Institute of Diabetes and Digestive and Kidney Diseases, and the National Institutes of Health Clinical Center.