Two cases of congenital nephrotic syndrome.
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.
Iitaka
Kikuo
K
Department of Pediatrics, Yamato City Hospital, 8-3-6 Fukaminishi, Yamato, Kanagawa 242-8602, Japan. kikuo.iitaka@gov.city.yamato.kanagawa.jp
Motoyama
Osamu
O
Hojo
Midori
M
Iwanami
Naomi
N
Koshino
Hiroe
H
Nakamura
Shinya
S
Moriya
Shunsuke
S
D001706:Biopsy; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007678:Kidney Glomerulus / Q000473:pathology* / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D009392:Nephrectomy; D009402:Nephrosis, Lipoid / Q000151:congenital / Q000473:pathology / Q000601:surgery; D009404:Nephrotic Syndrome / Q000151:congenital / Q000473:pathology* / Q000601:surgery; D011507:Proteinuria / Q000151:congenital / Q000473:pathology* / Q000601:surgery; D012075:Remission, Spontaneous