Prevalence of β-Thalassemia Trait and Abnormal Hemoglobins in Sanliurfa Province in Southeast Turkey
Prevalence of β-Thalassemia Trait and Abnormal Hemoglobins in Sanliurfa Province in Southeast Turkey
复制标题
DOI：
10.3109/03630269.2014.978008
复制
发表时间：
2014-01-01
期刊：
影响因子：
1
通讯作者：
Vural, Mehmet
中科院分区：
文献类型：
--
作者：
Incebiyik, Adnan;Genc, Ahmet;Vural, Mehmet
地中海贫血是土耳其最常见的遗传性疾病之一，特别是在该国的地中海地区。本研究的目的是确定在土耳其东南部地区Sanliurfa省申请婚前筛查的夫妇中β -地中海贫血（β -thal）特征和异常血红蛋白（Hbs）的频率，该省首次报道了β - thalia和异常Hbs的发病率。在本研究中，为了检测β -thal特征和异常Hbs在土耳其Sanliurfa省的流行情况，共对37,962对申请婚前筛查的夫妇进行了分析。从2011年1月到2014年3月，分别用细胞计数器和高效液相色谱（HPLC）进行红细胞（RBC）计数和Hb分离。高Hb A(2) （bbb3.5 %）值的β -thal在三留尔法省的患病率为2.44% （n = 1853）。此外，Hb异常率为1.57% (1193/75,924),Hb S (HBB: C . 20t > A), Hb C （HBB: C . 19g > A）和Hb D- Punjab （HBB: C . 364g > C）分别为0.50,0.38和0.69。本研究首次确定了出生频率最高的三柳尔法省乙型肝炎和异常乙型肝炎的发病频率。我们报告说，与土耳其其他城市相比，β -thal特征的频率处于高风险水平。由于在三留尔法省β -thal的高风险，婚前筛查计划将在告知父母β -thal后代方面具有重要价值。
Thalassemia is one of the most common hereditary disorders in Turkey, especially in the Mediterranean region of the country. The purpose of this study was to determine the frequency of the beta-thalassemia (beta-thal) trait and abnormal hemoglobins (Hbs) in couples who applied for premarital screening in Sanliurfa Province, in the southeastern region of Turkey, a province with the first reported incidence of beta-thal and abnormal Hbs. In the present study, in order to detect the prevalence of the beta-thal trait and abnormal Hbs in Sanliurfa Province, Turkey, a total of 37,962 couples who applied for premarital screening were analyzed. From January 2011 through March 2014, red blood cell (RBC) counts and Hb fractionation were carried out by a cell counter and high performance liquid chromatography (HPLC), respectively. The prevalence of beta-thal with high Hb A(2) (>3.5%) values was found at rates of 2.44% (n = 1853) in Sanliurfa Province. Additionally, the abnormal Hb rate was 1.57% (1193/75,924), and Hb S (HBB: c.20T > A), Hb C (HBB: c.19G > A) and Hb D- Punjab (HBB: c.364G > C) were reported as 0.50, 0.38 and 0.69, respectively. This study is the first to establish the frequency of beta-thal and abnormal Hbs in Sanliurfa Province, which has the highest birth frequency. We report that the frequency of the beta-thal trait is at a high-risk level compared to other cities in Turkey. Due to the high risk of beta-thal in Sanliurfa Province, a premarital screening program would be of great value in informing parents about offspring with beta-thal.