Homozygous appearance of distal myopathy

L Welander - Acta Genetica et Statistica Medica, 1957 - JSTOR
L Welander
Acta Genetica et Statistica Medica, 1957JSTOR
In the present family (p. 35) both parents (aged 82 and 81) had distal myopathy, of 27 and
19 years' duration respectively. The parents were unrelated, and the same disease was
known in the ancestry of both. They had 16 living children, aged 35 to 59; seven had distal
myopathy, and nine were so far normal. If the disease was inherited as a dominant trait, the
expectancy of affected children in a family of 16 would be 12, according to the expanded
binomial formula. Considering the age distribution (35 to 59 years), and the age at onset …
In the present family (p. 35) both parents (aged 82 and 81) had distal myopathy, of 27 and 19 years' duration respectively. The parents were unrelated, and the same disease was known in the ancestry of both. They had 16 living children, aged 35 to 59; seven had distal myopathy, and nine were so far normal.
If the disease was inherited as a dominant trait, the expectancy of affected children in a family of 16 would be 12, according to the expanded binomial formula. Considering the age distribution (35 to 59 years), and the age at onset observed (range 34 to 82 years, mean 50 years), as a matter of fact this number could not be expected. Which mode of calculation will then give the most faithful expectation? A simple calculation was performed with consideration only to the age
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