Abstract
32 children with FN were studied, after exclusion of finnish type. They belonged to 17 families totalizing 39 affected subjects. When compared to sporadic nephrosis (N) FN showed a higher incidence of infantile onset (11/32 vs 9/249: p < 0.001) of males affected (81 vs 71%), of steroid resistance (16/32 vs 65/249 p < 0.01) and poor outcome, but appeared as a heterogeneous entity. Two groups were separated according to steroid (S) response: 16 children from 9 families were S-responders, 16 from 8 families were S-resistant. No discordance was found in any of the families. S-responsive FN showed the same age and sex distribution as sporadic N and similar good prognosis. It affected both sexes in 5 families, two generations in 3 and 2 siblings in 6 families. S-resistant FN affected exclusively siblings (3 in 3 families, 2 in the others), boys except for 2 sisters, and children under 3 (11/16 under 1 year). Two pairs of siblings had microcephaly and dysmorphy. Three died from complications, 6 deteriorated to renal failure after 1 to 14 years and 7 had normal renal function after 1 to 15 years. Renal biopsy showed in S-responders 6 minimal changes (MC) and one focal lesion (FL), and in S-resistant 8 MC, 1 FL and 4 mesangial sclerosis (including 3 siblings). In conclusion: S-sensitive FN do not differ from sporadic N. The facilitating role of environmental or genetic factors is not prominent but cannot be excluded. S-resistance FN appears as (a) different entities(y) and is consistent with a heriditary disease suggesting a recessive transmission.
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Kleinknecht, C., Gonzalez, G., Gubler, M. et al. FAMILIAL NEPHROSIS (FN). Pediatr Res 14, 1003 (1980). https://doi.org/10.1203/00006450-198008000-00180
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DOI: https://doi.org/10.1203/00006450-198008000-00180