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Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). 2009
Baris Turkbey, and
Iclal Ocak, and
Kailash Daryanani, and
Esperanza Font-Montgomery, and
Linda Lukose, and
Joy Bryant, and
Maya Tuchman, and
Parvathi Mohan, and
Theo Heller, and
William A Gahl, and
Peter L Choyke, and
Meral Gunay-Aygun
Molecular Imaging Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
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.
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