α-thalassemia in Bantu population from Congo-Brazzaville:: Its interaction with sickle cell anemia
α-thalassemia in Bantu population from Congo-Brazzaville:: Its interaction with sickle cell anemia
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DOI：
10.1159/000022899
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发表时间：
2000-03-01
期刊：
影响因子：
1.8
通讯作者：
Galactéros, F
中科院分区：
文献类型：
--
作者：
Mouélé, R;Pambou, O;Galactéros, F
缺失型α（+）地中海贫血在来自班图人的四组无关个体中调查了（-α（3.7））（新生儿、正常成人、镰状细胞性状携带者、镰状细胞性贫血患者），新生儿（f = 0.40）与成人（f = 0.36）、镰状细胞与非镰状细胞间（-α（3.7））染色体核型相似。镰状细胞性贫血患者（SS患者）的（-α（3.7））染色体频率在年龄分层时没有变化。患有（-α/α α，-α/-α）和不患有（α α/α α）α（+）地中海贫血的SS患者的血液学特征与牙买加和美国镰状细胞贫血患者中报道的相似，α（+）地中海贫血对镰状细胞性状携带者中血红蛋白S的百分比有影响。因此，刚果人群中α（+）-地中海贫血的高频率可能是由于这种疾病具有有利于生存的选择性优势。然而，α（+）-地中海贫血的频率不受年龄的影响。尽管在这种选择性热带环境中，α（+）-地中海贫血与其他地方一样显著影响镰状细胞贫血患者的血液学特征，然而，我们的数据没有提供α（+）-地中海贫血增加SS患者存活率的证据，Copyright（C）1999 S. Karger AG，巴塞尔。
Deletional alpha(+)-thalassemia (-alpha(3.7)) was investigated in four groups of unrelated individuals from the Bantu population (newborns, normal adults, sickle cells trait carriers, sickle cell anemia patients) of Brazzaville, Congo, The frequency of the (-alpha(3.7)) chromosome was similar between newborns (f = 0.40) and adult subjects (f = 0.36), and between sicklers and nonsickler subjects. The frequency of the (-alpha(3.7)) chromosome in sickle cell anemia patients (SS patients) did not change when age was stratified. The hematological characteristics of SS patients with (-alpha/alpha alpha, -alpha/-alpha) and without (alpha alpha/alpha alpha) alpha(+)-thalassemia were similar to those reported in Jamaican and US sickle cell anemia patients, alpha(+)-Thalassemia had an effect on the percentage of hemoglobin S in sickle cell trait carriers. Thus, the high frequency of alpha(+)-thalassemia in the Congolese population presumably results from this disorder having a selective advantage favoring survival, However, the frequency of alpha(+)-thalassemia was not affected by age, Although in this selective tropical environment, alpha(+)-thalassemia as elsewhere markedly affects the hematological characteristics of sickle cell anemia patients, however our data provide no evidence that alpha(+)-thalassemia increases survival of SS patients, Copyright (C) 1999 S. Karger AG, Basel.