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

Research output: Contribution to journalReview article

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.

Original languageEnglish (US)
Pages (from-to)100-111
Number of pages12
JournalPediatric radiology
Volume39
Issue number2
DOIs
StatePublished - Feb 1 2009
Externally publishedYes

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Radiology Nuclear Medicine and imaging

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  • Cite this

    Turkbey, B., Ocak, I., Daryanani, K., Font-Montgomery, E., Lukose, L., Bryant, J., Tuchman, M., Mohan, P., Heller, T., Gahl, W. A., Choyke, P. L., & Gunay-Aygun, M. (2009). Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatric radiology, 39(2), 100-111. https://doi.org/10.1007/s00247-008-1064-x