title: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF).
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.
authors.affiliation: Molecular Imaging Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Baris
authors.initials: B
authors.lastname: Turkbey
authors.forename: Iclal
authors.initials: I
authors.lastname: Ocak
authors.forename: Kailash
authors.initials: K
authors.lastname: Daryanani
authors.forename: Esperanza
authors.initials: E
authors.lastname: Font-Montgomery
authors.forename: Linda
authors.initials: L
authors.lastname: Lukose
authors.forename: Joy
authors.initials: J
authors.lastname: Bryant
authors.forename: Maya
authors.initials: M
authors.lastname: Tuchman
authors.forename: Parvathi
authors.initials: P
authors.lastname: Mohan
authors.forename: Theo
authors.initials: T
authors.lastname: Heller
authors.forename: William A
authors.initials: WA
authors.lastname: Gahl
authors.forename: Peter L
authors.initials: PL
authors.lastname: Choyke
authors.forename: Meral
authors.initials: M
authors.lastname: Gunay-Aygun
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D049448:Cholangiopancreatography, Magnetic Resonance / Q000379:methods*; D003937:Diagnosis, Differential; D005260:Female; D005355:Fibrosis / Q000151:congenital / Q000175:diagnosis; D006801:Humans; D007231:Infant, Newborn; D008099:Liver / Q000000981:diagnostic imaging* / Q000473:pathology*; D008103:Liver Cirrhosis / Q000151:congenital* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D017044:Polycystic Kidney, Autosomal Recessive / Q000175:diagnosis*; D011859:Radiography; D014463:Ultrasonography / Q000379:methods*; D055815:Young Adult