Abstract
We describe a 10 year old patient admitted to the Children's Hospital of Buffalo with hypocomplementemia associated with steroid responsive minimal change nephrotic syndrome. The sibling also had a low serum C3 concentration and all family members studied hadC 3 slow phenotypes. Factor I levels were at the lower limit of normal in the patient and his brother. Functional assays for CH50, total hemolyticC 3 and serum concentration of C2, C4−C9 and factors B and H were all within normal limits. This case confirms that a depressed serum complement level can occur in minimal change nephrotic syndrome and indicates that this depression could represent a preexisting inherited rather than an acquired deficiency. The findings are consistent with the pressence of a null or hypomorphicC 3 slow allele in hypocomplementemic family members. Additional studies are needed to resolve the association between the inherited partialC 3 deficiency and minimal change nephrotic syndrome.
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Springate, J.E., McLean, R.H., Winkelstein, J.A. et al. Hereditary partial deficiency of the third component of complement associated with minimal change nephrotic syndrome. Pediatr Nephrol 1, 608–610 (1987). https://doi.org/10.1007/BF00853597
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DOI: https://doi.org/10.1007/BF00853597