Gamberini M R, Canella R, Lucci M, Vullo C, Barrai I
Pediatric Division, USL 31, University of Ferrara, Italy.
Am J Med Genet. 1991 Jan;38(1):103-6. doi: 10.1002/ajmg.1320380123.
The reproductive behavior in 1984 of families segregating for Cooley anemia in Ferrara was compared with that of a control group of families, matched for some biological variables which affect fertility. At the resolution power of the sample, it was found that there is no significant difference in these variables due to segregation for Cooley anemia, and it appears that there is no longer significant reproductive compensation in thalassemic couples, although a tendency to compensate does still exist. The increased life span of children affected by Cooley anemia, due to improvements in treatment in the past decade, is probably the main reason why the compensatory reproductive behaviour of the past has almost disappeared.
对费拉拉地区患有库利贫血的家系在1984年的生殖行为,与一组在影响生育力的一些生物学变量上相匹配的对照家系进行了比较。在该样本的分辨能力下,发现由于库利贫血的遗传分离,这些变量没有显著差异,并且似乎在地中海贫血夫妇中不再存在显著的生殖补偿现象,尽管补偿的趋势仍然存在。由于过去十年治疗方法的改进,受库利贫血影响的儿童寿命延长,这可能是过去的补偿性生殖行为几乎消失的主要原因。