西双版纳傣、布朗、基诺族7岁以下儿童地中海贫血与G6PD缺乏症的流行病学调查

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目的:了解云南省西双版纳州傣族、基诺族、布朗族的7岁以下儿童地中海贫血(地贫)和G6PD缺乏症的流行病学现状。方法:对云南省西双版纳州景洪、勐海、勐腊县的傣族、基诺族、布朗族的7岁以下儿童共2 781例,进行血液分析、红细胞脆性试验、Hb电泳、G6PD荧光斑点试验,并进行统计学分析。结果:所调查的地区地贫发生率33.7%,其中β-地贫发生率17.3%,α-地贫发生率16.4%。β-地贫发生率基诺族居首(28.9%),各民族之间差异有统计学意义;α-地贫以傣族居首(22.0%),布朗族和基诺族之间差异无统计学意义,其余各民族之间差异有统计学意义。α-地贫、β-地贫各地差异均有统计学意义。不同地区傣族的β-地贫差异均有统计学意义;α-地贫勐海与勐腊差异有统计学意义。同一地区不同民族α-地贫、β-地贫差异均有统计学意义。多因素分析表明筛查地贫有价值的指标为:MCV、RDW、靶形细胞、脆性试验。G6PD缺乏症发生率2.2%,男童大于女童。结论:地贫和G6PD缺乏症在云南省西双版纳州傣族、基诺族、布朗族的7岁以下儿童中属高发,其发生率在所调查的不同民族及地区有统计学差异。该调查为云南省西双版纳州少数民族进行遗传咨询及进行地贫和G6PD缺乏症的预防提供了有价值的基础资料。 OBJECTIVE: To understand the epidemiological status of thalassemia (Thalassemia) and G6PD deficiency in children under 7 years of age in Dai, Jino and Brang ethnic groups in Xishuangbanna of Yunnan Province. Methods: A total of 2 781 children under 7 years old from Jinghai, Menghai and Mengla counties in Yunnan Province were enrolled. Blood samples, erythrocyte fragility test, Hb electrophoresis and G6PD fluorescent speckle test were performed. Statistical analysis. Results: The incidence of thalassemia in the surveyed area was 33.7%, of which the incidence of β-thalassemia was 17.3% and the incidence of α-thalassemia was 16.4%. The incidence of β-thalassemia was the highest (28.9%), with significant differences among all ethnic groups. The α-thalassemia was predominantly Dai (22.0%), and there was no significant difference between Brown and Jino , The rest of the ethnic differences were statistically significant. α-thalassemia, β-thalassemia differences were statistically significant. There were significant differences in β-thalassemia among Dai people in different areas; the difference between α-thalassemia and Mengla was statistically significant. There were significant differences in α-thalassemia and β-thalassemia among different ethnic groups in the same area. Multivariate analysis showed that the valuable indicators for screening thalassemia were MCV, RDW, target cell, and brittleness test. The prevalence of G6PD deficiency was 2.2%, with boys outnumbering girls. CONCLUSIONS: Thalassemia and G6PD deficiency are high prevalence among children under 7 years of age in Dai, Jinuo and Blang ethnic groups in Xishuangbanna Prefecture of Yunnan Province. The incidence of these diseases is statistically different among different ethnic groups and regions under investigation. The survey provided valuable basic information for genetic counseling among ethnic minorities in Xishuangbanna Prefecture of Yunnan Province and prevention of thalassemia and G6PD deficiency.
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