Francis R B, Kasper C K
JAMA. 1983 Dec 16;250(23):3192-5.
Reproduction rates in 294 men with severe hemophilia, 327 of their mothers, and 215 of their sisters were compared with Vital Statistics data for the general US population matched for exact age, calendar year between 1940 and 1977, and, for women, parity. Before 1950, reproduction rates in hemophiliacs and in mothers did not differ from those of the general population, but thereafter the rates fell notably. Births to sisters who were para 0 were lower than expected beginning in 1965, but births to sisters who had had one or more nonhemophiliac children were similar to those expected of the general population. The decline in fecundity may be caused by greater availability of genetic counseling and birth control measures and by social changes. Many prospective parents would welcome the opportunity to have unafflicted children but refuse selective abortion. Preconception gender selection would be appropriate for this sex-linked disorder.
对294名重度血友病男性患者、他们的327位母亲以及215位姐妹的生育率,与美国普通人群按确切年龄、1940年至1977年历年以及(针对女性)产次匹配的生命统计数据进行了比较。1950年之前,血友病患者及其母亲的生育率与普通人群无异,但此后这些比率显著下降。从1965年开始,未生育过的姐妹的生育率低于预期,但生育过一个或多个非血友病患儿的姐妹的生育率与普通人群预期的生育率相似。生育力下降可能是由于遗传咨询和节育措施的可及性提高以及社会变革所致。许多准父母会欢迎有机会生育无病的孩子,但拒绝选择性堕胎。对于这种伴性遗传疾病,孕前性别选择是合适的。