Dupuytren's disease recurs more frequently than generally assumed. 24 typical family trees derived from the literature are analyzed here.
The main conclusions that can be derived are the following:
1) Monogenic heredity is confirmed.
2) The sex ratio in the affected families is 61,2 m: 38,8 f.
3) The sex ratio in unaffected families is 38 m: 43 f.
4) The expressivity of the disease is variable. Penetrance, which is incomplete, varies according to the sex. In our selected material, the penetrance rate is 93,6% in the males and 42,9% in the females.
5) Males transmit the gene in 68% of the cases; females in 32%.
6) Incomplete sex linkage can be excluded.
7) A comparison is made between the really observed values and the theoretical rates for normal and affected men and women for the chance of an auto-somatic dominance. These observations make possible the conclusion that the number of affected females is too low as compared with unaffected females and affected males.
8) A new theory is suggested concerning the mechanism of heredity based on the different statistical values. The responsible factor would be deadly for 60% of the XX affected zygotes. But 60% of the affected X gametes produced by the mother, being identified by an essential incompatibility for the unaffected X spermatozoid, would be electively fecundated by a Y gamate. This machanism would explain the excess of affected males, the small proportion of affected females and the sex-ratio of 61,2: 38,8.
The concordance between the observed values and the theoretically expected values of such a kind seem to convalidate our new theory.