Abstract
C4 and factor B typing were performed in 37 pediatric patients with primary IgA nephropathy. Null alleles for C4B occurred with a frequency of 26% in patients, as compared to 15% in healthy controls (NS). The phenotype of C4B deficiency (homozygous C4B null), however, was found in 16% of patients and 4% of controls (P<0.05). Comparison of observed C4B phenotypes with those predicted from the Hardy-Weinberg equilibrium also confirmed an excess of C4B deficiency (P<0.0005). In contrast, there was no evidence of distortion in the frequencies of the C4A null allele or phenotype, or of the factor B alleles. The data suggest that C4B deficiency may be one of multiple interacting factors contributing to the development of this glomerulopathy.
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Supported in part by grant no. RR00123 from the General Clinical Research Centers Branch National Institutes of Health. Presented in part at the 1986 meeting of the American Pediatric Society and the Society for Pediatric Research
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Welch, T.R., Berry, A. & Beischel, L.S. C4 isotype deficiency in IgA nephropathy. Pediatr Nephrol 1, 136–139 (1987). https://doi.org/10.1007/BF00849283
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DOI: https://doi.org/10.1007/BF00849283