title: Appearance of autosomal recessive polycystic kidney disease in magnetic resonance imaging and RARE-MR-urography.
abstract: OBJECTIVE
  To describe the appearance of autosomal recessive polycystic kidney disease (ARPKD) on MRI and RARE-MR urography.
  
  
  METHODS
  Seven boys and one girl (aged 3 months to 14 years, median 2.5 years) were evaluated. Images were obtained with 0.23-T and 1.5-T MR systems using T1-weighted (T1-W) spin-echo, T2-weighted (T2-W) turbo-spin-echo and RARE-MR-urography sequences. Signal intensities, morphological appearance of the affected kidneys and, specifically, the picture of the urinary tract on RARE-MR-urography were evaluated.
  
  
  RESULTS
  All children showed kidney enlargement, reniform but humpy kidney shape, homogeneously grainy renal parenchyma, normal renal pelvis and normal calyces. Signal intensity was hyperintense in T2-W images in all cases. In six cases (n = 7), T1-W images were hypointense. On RARE-MR urography a hyperintense, linear radial pattern was seen in the cortex and medulla which represents the characteristic microcystic dilatation of collecting ducts in ARPKD. Three boys and the girl presented with a few circumscribed small subcapsular cysts.
  
  
  CONCLUSIONS
  In order to confirm the diagnosis of ARPKD, RARE-MR urography seems to be a non-invasive imaging tool that shows directly the microcystic dilated water-filled collecting ducts.
authors.affiliation: Department of Diagnostic Radiology, University Hospital Freiburg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Kern
authors.forename: L B
authors.initials: LB
authors.lastname: Zimmerhackl
authors.forename: F
authors.initials: F
authors.lastname: Hildebrandt
authors.forename: B
authors.initials: B
authors.lastname: Ermisch-Omran
authors.forename: M
authors.initials: M
authors.lastname: Uhl
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007089:Image Enhancement; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications / Q000175:diagnosis*; D012189:Retrospective Studies; D014567:Urography / Q000379:methods*