Cluster of cystic fibrosis cases in a limited area of Brittany (France)

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Abstract
Cystic fibrosis in the northern sector of the French department of Finistere is 1:1787 live births. Within this sector a concentration of the disease was found in a small area. The minimal frequency in this area, from 1946-1972, was calculated as 1:377 live births, the gene frequency being 0.0515. Genealogic analysis, going back to the beginning of the 18th century, showed a relationship between 8 of the 10 families to which the patients belonged. The origin of the deleterious genes may be explained by at least 5 primary ancestral couples living in the 18th century. Random drift is the most probable explanation for the concentration of cystic fibrosis in this region.

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