Hereditary nephritis (Alport's syndrome)-1983

In the 81 years that have passed since the first description of the pedigree ultimately augmented by Alport, and in the 56 years since the latter description, insight into the pathogenesis of lethal familial hematuria has been limited. Work during the past decade has focused on the antigenic and chemical composition of the glomerular basement membrane. It is ever more likely that lethal familial hematuria of Alport type is a genetically heterogeneous phenotype.