尼日利亚的α地中海贫血：它与镰状细胞病的相互作用。

Alpha-thalassaemia in Nigeria: its interaction with sickle-cell disease.

作者信息

Falusi A G, Esan G J, Ayyub H, Higgs D R

出版信息

Eur J Haematol. 1987 Apr;38(4):370-5. doi: 10.1111/j.1600-0609.1987.tb00013.x.

DOI:10.1111/j.1600-0609.1987.tb00013.x

Abstract

We have determined the molecular basis and frequency of alpha-thalassaemia in Nigeria. The alpha-thalassaemia determinant in this population is caused by only one type of single alpha globin gene deletion (-alpha 3.7). Comparison of the haematological features of those patients who have sickle-cell disease with (-alpha/alpha alpha, -alpha/-alpha) or without (alpha alpha/alpha alpha) alpha-thalassaemia showed similar trends to those reported in Jamaican and U.S. patients with these interactions. However, in contrast to studies in some other African populations we have shown that the frequency of alpha-thalassaemia in Nigeria is the same (0.24) in patients with or without homozygous sickle-cell disease (AA, AS and AC genotypes).

摘要

我们已经确定了尼日利亚α地中海贫血的分子基础和发病率。该人群中的α地中海贫血决定因素仅由一种单一α珠蛋白基因缺失类型（-α3.7）引起。对患有镰状细胞病且伴有（-α/αα，-α/-α）或不伴有（αα/αα）α地中海贫血的患者的血液学特征进行比较，结果显示出与牙买加和美国有这些相互作用的患者所报道的趋势相似。然而，与其他一些非洲人群的研究不同，我们已经表明，在尼日利亚，患有或未患有纯合镰状细胞病（AA、AS和AC基因型）的患者中α地中海贫血的发病率相同（0.24）。