Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF).
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.
Turkbey
Baris
B
Molecular Imaging Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
Ocak
Iclal
I
Daryanani
Kailash
K
Font-Montgomery
Esperanza
E
Lukose
Linda
L
Bryant
Joy
J
Tuchman
Maya
M
Mohan
Parvathi
P
Heller
Theo
T
Gahl
William A
WA
Choyke
Peter L
PL
Gunay-Aygun
Meral
M
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