Abstract
Twelve infants with idiopathic C.N.S. starting before 5 month of age were studied. All died. Autopsy was performed in 10, and renal biopsy only in 2. According to their glomerular changes they were divided in 4 groups: I) Minimal change: 4, no one developed renal failure(RF); tubular microcysts were present in 2. Of 2 sibs, both with autopsy, only one had “microcystic kidney”. II) Fetal glomerular sclerosis: 2, both with tubular microcysts and RF. III) Proliferative glomerulonephritis: 2, both died of infection without RF; autopsy was not performed. IV) Diffuse complex glomerulopathy: 4, all of them with RF, mesangial proliferation and basement thickening, one had Wilms' tumor and another masculine pseudohermaphroditism. Diffuse mesangial sclerosis (Habib) was not found in any of the 12 infants.
Systematization of C.N.S. is still difficult. Non specifficity of tubular microcysts, and the importance of differentiating between fetal glomerular sclerosis and focal sclerosing glomerular sclerosis and focal sclerosing glomerulopathy are stressed.
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Gallo, G. 54 CONGENITAL HEPHROTIC SYNDROME (C.N.S.). Pediatr Res 15, 192 (1981). https://doi.org/10.1203/00006450-198102000-00111
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DOI: https://doi.org/10.1203/00006450-198102000-00111
