Pediatric Radiology

, Volume 39, Issue 2, pp 100–111

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

  • Baris Turkbey
  • Iclal Ocak
  • Kailash Daryanani
  • Esperanza Font-Montgomery
  • Linda Lukose
  • Joy Bryant
  • Maya Tuchman
  • Parvathi Mohan
  • Theo Heller
  • William A. Gahl
  • Peter L. Choyke
  • Meral Gunay-Aygun
Review

Abstract

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.

Keywords

Autosomal recessive polycystic kidney disease Congenital hepatic fibrosis US MR cholangiography Children 

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Baris Turkbey
    • 1
  • Iclal Ocak
    • 1
    • 2
  • Kailash Daryanani
    • 3
  • Esperanza Font-Montgomery
    • 4
  • Linda Lukose
    • 4
  • Joy Bryant
    • 4
  • Maya Tuchman
    • 4
  • Parvathi Mohan
    • 5
  • Theo Heller
    • 6
  • William A. Gahl
    • 4
  • Peter L. Choyke
    • 1
  • Meral Gunay-Aygun
    • 4
    • 7
  1. 1.Molecular Imaging Program, National Cancer InstituteNational Institutes of HealthBethesdaUSA
  2. 2.Department of RadiologyUniversity of Pittsburgh Medical CenterPittsburghUSA
  3. 3.Clinical Center, Department of RadiologyNational Institutes of HealthBethesdaUSA
  4. 4.National Human Genome Research Institute, Medical Genetics BranchNational Institutes of HealthBethesdaUSA
  5. 5.Department of Pediatric GastroenterologyGeorge Washington UniversityWashingtonUSA
  6. 6.National Institute of Diabetes and Digestive and Kidney DiseasesNational Institutes of HealthBethesdaUSA
  7. 7.Intramural Program, Office of Rare Diseases, Office of the DirectorsNational Institutes of HealthBethesdaUSA

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