population genetics of hereditary hemochromatosis in saguenay lacsaintjean quebec canada hereditary hemochromatosis hh is an autosomal recessive disorder that has a high prevalence in caucasian populations based on hla typing in 18 families the gene frequency was estimated 012 the homozygote frequency was 0014 and the heterozygote frequency was 021 in saguenay lacsaintjean slsj a geographically isolated region of northeastern quebec the genealogical reconstruction showed that 15 of the 57 obligate carriers of the hh gene could be traced back to a unique ancestor in the 18th century the mean coefficients of inbreeding and kinship were 17 and 15 times respectively higher in the hh group than in three control groups the values of both coefficients were much higher than those found in other hh populations and in most of the other recessive disorders prevalent in slsj