<unk>  affects males and females in equal numbers. Its prevalence has been estimated to be 1 in 100,000 to 120,000 births worldwide. HT1 is especially prevalent in the Saguenay-Lac Saint-Jean region of Quebec is one in 1,850 births. The elevated frequency of this disorder within individuals of French-Canadian ancestry in Quebec is believed to be due to reduced genetic heterogeneity within the original founder population for the Saguenay-Lac Saint-Jean region. The initial settlement of Saguenay Lac-Saint-Jean (SLSJ) occurred between 1838 and 1911. From a total of 28,656 settlers, 75 percent originated from the neighboring Charlevoix region. The settling of the Charlevoix region itself started in 1675 when 599 founders of mostly French descent moved to this region from the Quebec City area. Worldwide,<unk>  I<unk>  affects about 1 person in 100,000. This<unk>  of<unk>  is much more common in Quebec, Canada. The overall incidence in Quebec is about 1 in 16,000 individuals. In the Saguenay-Lac-Saint-Jean region of Quebec,<unk>  1<unk>  affects 1 person in 1,846. The carrier rate has been estimated to be between 1 in 20 and 1 in 31.
Tyrosinemia type I
Aspartylglucosaminuria
Hawkinsinuria
Aldolase A deficiency
Essential fructosuria
Transaldolase deficiency
Phenylketonuria
Cystathioninuria