Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)
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- Turkbey, B., Ocak, I., Daryanani, K. et al. Pediatr Radiol (2009) 39: 100. doi:10.1007/s00247-008-1064-x
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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.