| Popis: |
A kindred of fifty-five members has been described of whom twenty-eight, extending through four generations, have renal disease. In addition, four members transmitted the disorder but were apparently normal. The renal disease appears to be more severe in males, eight having died in uremia. The clinical course is compatible with glomerulonephritis but autopsy findings show elements of glomerulonephritis, pyelonephritis, interstitial fibrosis and vascular disease. Deafness and a history of allergy have been noted in several members. The genetic analysis is consistent with autosomal dominant inheritance with nonrandom segregation and preferential association of the autosome bearing the gene with the X chromosome during meiosis. The possible pathogenic role of streptococcal infections is discussed. |
