Pediatric Radiology

, Volume 39, Issue 2, pp 100–111

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

Authors

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

DOI: 10.1007/s00247-008-1064-x

Cite this article as:
Turkbey, B., Ocak, I., Daryanani, K. et al. Pediatr Radiol (2009) 39: 100. doi:10.1007/s00247-008-1064-x

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 diseaseCongenital hepatic fibrosisUSMR cholangiographyChildren

Copyright information

© Springer-Verlag 2008