Balgir R S, Murmu B, Dash B P
Division of Human Genetics, Regional Medical Research Centre (ICMR), Chandrasekharpur, Orissa, India.
J Assoc Physicians India. 1999 Oct;47(10):987-90.
Hemoglobinopathy and allied hemolytic disorders are important genetic and public health problems in Orissa. These cause high degree of hemolytic anemia, morbidity and mortality in the vulnerable populations. A total of 465 Ashram School children aged 6-15 years belonging to Bathudi, Bhumiz, Kolha and Santal tribes in six localities of Mayurbhanj district of Orissa were screened for hemoglobinopathy, glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, ABO and Rhesus blood groups serology and any other hereditary condition. The sickle cell trait (Hb AS) was detected in Santal (1.0%), Bathudi (1.0%) and Bhumiz (0.9%) tribals. No case of homozygous sickle cell disease was detected among the tribes of Mayurbhanj district. The beta-thalassemia trait was detected in Santal (8.0%), Kolha (2.0%), Bhumiz (1.7%) and other tribal (3.8%) students. Sickle cell hemoglobinopathy and beta-thalassemia are prevalent in this district among the tribes, but the frequency is very low. The prevalence of G-6-PD deficiency is considerably high (7.7-9.8%) among the tribes of Mayurbhanj district in Orissa. Out of total 43 G-6-PD deficient subjects, there were 32 males, 9 heterozygote females and 2 homozygous females. This shows that the antimalarial drugs should be administered with caution as these cause hemolytic anemia, sometimes fatal also. The distribution of ABO and Rhesus blood groups shows the preponderance of B blood group (33.8%) over O (29.6%) and 2.1% cases of Rhesus negativity were detected among the Bathudi tribe. This pattern is consistent with the characteristic features of tribal populations in India.
血红蛋白病及相关溶血性疾病是奥里萨邦重要的遗传和公共卫生问题。这些疾病在易感人群中导致高度的溶血性贫血、发病率和死亡率。对奥里萨邦梅奥尔布汉杰区六个地区的465名年龄在6至15岁、属于巴图迪、布米兹、科尔哈和桑塔尔部落的阿什拉姆学校儿童进行了血红蛋白病、葡萄糖-6-磷酸脱氢酶（G-6-PD）缺乏症、ABO和恒河猴血型血清学以及任何其他遗传性疾病的筛查。在桑塔尔（1.0%）、巴图迪（1.0%）和布米兹（0.9%）部落中检测到镰状细胞性状（Hb AS）。在梅奥尔布汉杰区的部落中未检测到纯合子镰状细胞病病例。在桑塔尔（8.0%）、科尔哈（2.0%）、布米兹（1.7%）和其他部落（3.8%）的学生中检测到β地中海贫血性状。镰状细胞血红蛋白病和β地中海贫血在该地区的部落中普遍存在，但频率很低。奥里萨邦梅奥尔布汉杰区的部落中G-6-PD缺乏症的患病率相当高（7.7 - 9.8%）。在总共43名G-6-PD缺乏症患者中，有32名男性、9名杂合子女性和2名纯合子女性。这表明抗疟药物应谨慎使用，因为这些药物会导致溶血性贫血，有时甚至是致命的。ABO和恒河猴血型的分布显示B血型（33.8%）比O血型（29.6%）占优势，并且在巴图迪部落中检测到2.1%的恒河猴阴性病例。这种模式与印度部落人口的特征一致。
