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Two cases of congenital nephrotic syndrome
Authors:Kikuo?Iitaka  author-information"  >  author-information__contact u-icon-before"  >  mailto:kikuo.iitaka@gov.city.yamato.kanagawa.jp"   title="  kikuo.iitaka@gov.city.yamato.kanagawa.jp"   itemprop="  email"   data-track="  click"   data-track-action="  Email author"   data-track-label="  "  >Email author,Osamu?Motoyama,Midori?Hojo,Naomi?Iwanami,Hiroe?Koshino,Shinya?Nakamura,Shunsuke?Moriya
Affiliation:(1) Department of Pediatrics, Yamato City Hospital, 8-3-6 Fukaminishi, Yamato, Kanagawa, 242-8602, Japan;(2) Department of Pediatrics, Toho University Hospital, Tokyo, Japan;(3) Department of Pediatrics, Kitasato University Hospital, Kanagawa, Japan
Abstract:The clinical course and renal histological changes in two patients with congenital nephrotic syndrome are presented. Both patients developed edema and proteinuria at 2 days of age. Steroids and immunosuppressive drugs were not used in either patient. One patient showed gradual disappearance of nephrotic syndrome and proteinuria during the 16-month follow-up. In the other patient, the nephrotic state persisted and nephrotic complications necessitated bilateral nephrectomy when she was 12 months old. Renal biopsy showed minimal change lesions in one patient, while the nephrectomized kidneys showed mild mesangial proliferation and matrix increase in the glomeruli. No tubular dilatation or microcysts were observed in either patient.
Keywords:Congenital nephrotic syndrome  Finnish type  Minimal change lesion  Arterial thrombosis  Nephrec-tomy  Spontaneous remission
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