Cystic fibrosis (CF) is an autosomal recessive disorder with a prevalence at 
birth estimated at 1/2000-1/2500 livebirths in Caucasian populations. Some 127 
CF individuals are known in Saguenay-Lac-St-Jean (SLSJ), a geographically 
isolated region of Quebec. The prevalence at birth was estimated at 1/902 live 
borns, and the carrier rate was estimated at 1/15 inhabitants in the SLSJ 
region. The mean inbreeding coefficient was only slightly elevated in the CF 
group compared with three control groups, and was due to remote consanguinity. 
The mean kinship coefficient was 2.4 times higher in the CF group than in the 
control groups. In SLSJ region, the places of origin of the CF individuals and 
their parents did not show a clustered nonuniform distribution. Endogamy was not 
higher in the CF group than in control groups.