caa a22 4500 445883294 CHVBK 20180317145551.0 cr unu---uuuuu 170323e20110801xx s 000 0 eng 10.1007/s10157-011-0429-x doi (NATIONALLICENCE)springer-10.1007/s10157-011-0429-x Morphological and functional analyses of two infants with obstructive renal dysplasia [Elektronische Daten] [Kenichiro Miura, Takashi Sekine, Riki Nishimura, Yutaka Kanamori, Atsuhiro Yanagisawa, Kiyohide Sakai, Michio Nagata, Takashi Igarashi] Renal dysplasia associated with urinary tract obstruction comprises two distinct phenotypes, i.e., multicystic dysplastic kidney (MCDK) and obstructive renal dysplasia (ORD). MCDK is a common manifestation in infants with renal dysplasia, which is characterized by multiloculated thin-walled cysts with no functional parenchyma and an atretic ureter owing to pyelocalyceal occlusion early in fetal life. In contrast, ORD is an extremely rare condition which is caused by severe obstruction of the distal ureter or urethra. Here, we report two infants with ORD. Both patients manifested unilateral kidney enlargement with multiple cortical cysts, mild hydronephrosis, and marked dilatation of the ipsilateral ureter. Contralateral kidneys and urinary tracts revealed no apparent radiological abnormalities. Serial ultrasonographic studies of fetal and neonatal kidneys in both cases revealed that ureteral dilatation was evident at gestational week 16 and 27, respectively, and most of the cortical cysts disappeared within 1-3months after birth. The functions of the affected kidneys were severely impaired but evident at the time of birth. These manifestations were consistent with a diagnosis of ORD, and were distinct from the features of MCDK. Our observation of fetal and infantile kidneys in these two cases provides us with a better understanding of the pathogenesis of ORD. Japanese Society of Nephrology, 2011 Urinary tract obstruction nationallicence Multicystic dysplastic kidney nationallicence Cortical cyst nationallicence Glomerular cyst nationallicence Megaureter nationallicence Miura Kenichiro Department of Pediatrics, University of Tokyo, Tokyo, Japan aut Sekine Takashi Department of Pediatrics, University of Tokyo, Tokyo, Japan aut Nishimura Riki Department of Pediatrics, University of Tokyo, Tokyo, Japan aut Kanamori Yutaka Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan aut Yanagisawa Atsuhiro Department of Pediatrics, Ohta Nishinouchi Hospital, Kohriyama, Japan aut Sakai Kiyohide Department of Urology, Miyagi Children's Hospital, Sendai, Japan aut Nagata Michio Department of Molecular Pathology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan aut Igarashi Takashi Department of Pediatrics, University of Tokyo, Tokyo, Japan aut Clinical and Experimental Nephrology Springer Japan 15/4(2011-08-01), 602-606 1342-1751 15:4<602 2011 15 10157 https://doi.org/10.1007/s10157-011-0429-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10157-011-0429-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Miura Kenichiro Department of Pediatrics, University of Tokyo, Tokyo, Japan aut NATIONALLICENCE 700 1- Sekine Takashi Department of Pediatrics, University of Tokyo, Tokyo, Japan aut NATIONALLICENCE 700 1- Nishimura Riki Department of Pediatrics, University of Tokyo, Tokyo, Japan aut NATIONALLICENCE 700 1- Kanamori Yutaka Department of Pediatric Surgery, University of Tokyo, Tokyo, Japan aut NATIONALLICENCE 700 1- Yanagisawa Atsuhiro Department of Pediatrics, Ohta Nishinouchi Hospital, Kohriyama, Japan aut NATIONALLICENCE 700 1- Sakai Kiyohide Department of Urology, Miyagi Children's Hospital, Sendai, Japan aut NATIONALLICENCE 700 1- Nagata Michio Department of Molecular Pathology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan aut NATIONALLICENCE 700 1- Igarashi Takashi Department of Pediatrics, University of Tokyo, Tokyo, Japan aut NATIONALLICENCE 773 0- Clinical and Experimental Nephrology Springer Japan 15/4(2011-08-01), 602-606 1342-1751 15:4<602 2011 15 10157 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer