Labie D, Richin C, Pagnier J, Gentilini M, Nagel R L
Hum Genet. 1984;65(3):300-2. doi: 10.1007/BF00286522.
We have studied the incidence of hemoglobinopathies in 1059 individuals in Upper Volta. We have found that this population has a high frequency of HbS and HbC and alpha-thalassemia. The gene frequency of HbS was high (0.1 for the beta s gene) in the arid Sahel portion of Upper Volta accompanied by a lower frequency for HbC (0.05 for the beta c gene). The reverse was true in the humid Savanna region of this country (0.03 for the beta s gene and 0.14 for the beta c gene). There was no age dependency of the HbS gene frequency, but alpha-thalassemia, detectable in HbS heterozygotes, showed a statistically significant decrease with age. No homozygote for HbS was detected after the age of 1 year, and SC and CC genotypes were found at a lower incidence than expected. The environmental and medical conditions in Upper Volta preclude the survival of SS individuals and decrease the survival of SC and CC genotypes.
我们对布基纳法索1059名个体的血红蛋白病发病率进行了研究。我们发现，这一人群中HbS、HbC和α地中海贫血的频率较高。在布基纳法索干旱的萨赫勒地区，HbS的基因频率较高（βs基因频率为0.1），而HbC的频率较低（βc基因频率为0.05）。在该国湿润的热带稀树草原地区情况则相反（βs基因频率为0.03，βc基因频率为0.14）。HbS基因频率不存在年龄依赖性，但在HbS杂合子中可检测到的α地中海贫血随年龄增长呈统计学显著下降。1岁以后未检测到HbS纯合子，且SC和CC基因型的发生率低于预期。布基纳法索的环境和医疗条件使SS个体无法存活，并降低了SC和CC基因型的存活率。
