title: [The reproduction of families affected by Steinert's dystrophy in Saguenay (Quebec), 1885-1971:  demographic parameters].
abstract: "In the light of the literature in the field, one would expect that the myotonic dystrophy (or Steinert) gene carries a severe selective disadvantage. Indeed, this phenotype is commonly said to entail...sterility, infant mortality, and a lower nuptiality.  In the worst cases, it is not unlikely that the gene could eliminate itself through non-reproduction.  [A model is developed and] tested in the Saguenay (Quebec) population, known for its exceptionally high prevelance (1/475) of the disease.  Authors have been able to use a computerized demographic and genealogical data base in order to study several demographic variables (fertility, nuptiality, mortality, etc)....Case-control analyses produced very [few] differences between affected and control families, suggesting no significant selective disadvantage associated with the gene." (SUMMARY IN ENG AND ITA)
authors.forename: R
authors.initials: R
authors.lastname: Roy
authors.forename: M
authors.initials: M
authors.lastname: Declos
authors.forename: G
authors.initials: G
authors.lastname: Bouchard
authors.forename: J
authors.initials: J
authors.lastname: Mathieu
mesh_terms: D000569:Americas; D001695:Biology; D002170:Canada; D003710:Demography; D019049:Developed Countries; D004194:Disease; D005298:Fertility; D030342:Genetic Diseases, Inborn; D005823:Genetics; D005828:Genetics, Population; D008393:Marriage; D009026:Mortality; D009656:North America; D011153:Population; D011157:Population Dynamics; D011336:Probability; D012106:Research; D013223:Statistics as Topic
keywords: Americas; Biology; Canada; Demographic Factors; Developed Countries; Diseases; Fertility; Genetics; Genetics, Population; Hereditary Diseases; Mortality; North America; Northern America; Nuptiality; Population; Population Dynamics; Probability; Research Methodology; Statistical Studies; Studies