caa a22 4500 475755421 CHVBK 20180406123536.0 cr unu---uuuuu 170329e20000201xx s 000 0 eng 10.1007/s002470050035 doi (NATIONALLICENCE)springer-10.1007/s002470050035 Appearance of autosomal recessive polycystic kidney disease in magnetic resonance imaging and RARE-MR-urography [Elektronische Daten] [S. Kern, L.-B. Zimmerhackl, F. Hildebrandt, B. Ermisch-Omran, M. Uhl] Purpose. To describe the appearance of autosomal recessive polycystic kidney disease (ARPKD) on MRI and RARE-MR urography. Materials and 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. Springer-Verlag Berlin Heidelberg, 2000 Kern S. Division of Paediatric Radiology, Department of Diagnostic Radiology, University Hospital Freiburg, Hugstetterstr. 55, D-79 106 Freiburg, Germany, DE aut Zimmerhackl L.-B Division of Paediatric Nephrology, Department of Paediatrics, University Hospital Freiburg, Freiburg, Germany, DE aut Hildebrandt F. Division of Paediatric Nephrology, Department of Paediatrics, University Hospital Freiburg, Freiburg, Germany, DE aut Ermisch-Omran B. Division of Paediatric Nephrology, Department of Paediatrics, University Hospital Freiburg, Freiburg, Germany, DE aut Uhl M. Division of Paediatric Radiology, Department of Diagnostic Radiology, University Hospital Freiburg, Hugstetterstr. 55, D-79 106 Freiburg, Germany, DE aut https://doi.org/10.1007/s002470050035 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002470050035 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kern S. Division of Paediatric Radiology, Department of Diagnostic Radiology, University Hospital Freiburg, Hugstetterstr. 55, D-79 106 Freiburg, Germany, DE aut NATIONALLICENCE 700 1- Zimmerhackl L.-B Division of Paediatric Nephrology, Department of Paediatrics, University Hospital Freiburg, Freiburg, Germany, DE aut NATIONALLICENCE 700 1- Hildebrandt F. Division of Paediatric Nephrology, Department of Paediatrics, University Hospital Freiburg, Freiburg, Germany, DE aut NATIONALLICENCE 700 1- Ermisch-Omran B. Division of Paediatric Nephrology, Department of Paediatrics, University Hospital Freiburg, Freiburg, Germany, DE aut NATIONALLICENCE 700 1- Uhl M. Division of Paediatric Radiology, Department of Diagnostic Radiology, University Hospital Freiburg, Hugstetterstr. 55, D-79 106 Freiburg, Germany, DE aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer