Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

Pediatr Radiol. 2009 Feb;39(2):100-11. doi: 10.1007/s00247-008-1064-x. Epub 2008 Dec 17.


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.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cholangiopancreatography, Magnetic Resonance / methods*
  • Diagnosis, Differential
  • Female
  • Fibrosis / congenital
  • Fibrosis / diagnosis
  • Humans
  • Infant, Newborn
  • Liver / diagnostic imaging*
  • Liver / pathology*
  • Liver Cirrhosis / congenital*
  • Liver Cirrhosis / diagnosis*
  • Male
  • Middle Aged
  • Polycystic Kidney, Autosomal Recessive / diagnosis*
  • Radiography
  • Ultrasonography / methods*
  • Young Adult