Cystic fibrosis: a HLA associated hereditary disease?

Twelve homozygote patients and thirty-two heterozygote gene carriers from families with cystic fibrosis were HLA-typed. Diagnostic criteria were sweat electrolyte concentration, pancreatic enzyme levels from duodenal juice and stool, Szczepanski's bromide test in the group of homozygotes, and the latter only in the cases of heterozygotes. In comparison with 130 healthy blood donors typed for 29 HLA antigens. B18 proved to be more frequent in the group of patients and gene carriers, with 50 and 31%, respectively, and 14% in the normal population. The association seems to be stronger in the homozygotes than in the heterozygotes (p less than 0.02, respectively).