Kaur M, Das G P, Verma I C
Department of Paediatrics, WHO Collaborating Centre in Genetics All India Institute of Medical Sciences, New Delhi.
Indian J Med Res. 1997 Mar;105:111-6.
A study of 2570 tribals comprising 973 from Kerala, 696 from Madhya Pradesh and 901 from Orissa revealed the frequency of sickle cell gene to vary from 0.05 to 0.31 among different communities. High frequency of the gene (0.145 or more) was observed among Chettys, Kurmars and Kondhs, who also had a substantial number of homozygous sicklers. None of those with sickle cell disease (HbSS) were more than 39 yr in age as compared with 9.9-35.3 per cent among heterozygotes (HbAS). Mean foetal haemoglobin in those with sickle cell disease varied between 9.7 to 13.5 per cent, although ti showed a slight positive correlation with total haemoglobin only among the Kondhs. Painful crises were universally observed among all tribals with sickle cell disease, with jaundice being present in 57.5 per cent of cases. Some carriers of sickle cell gene also complained of painful crises. A health plan for identifying homozygotes in infancy with appropriate medical management is highly desirable.
一项对2570名部落居民的研究，其中包括来自喀拉拉邦的973人、中央邦的696人和奥里萨邦的901人，结果显示不同社区中镰状细胞基因的频率在0.05至0.31之间变化。在切蒂人、库尔马人以及孔德人中观察到该基因的高频率（0.145或更高），这些人群中也有相当数量的纯合镰状细胞贫血患者。与杂合子（HbAS）中9.9% - 35.3%的比例相比，镰状细胞病（HbSS）患者中年龄最大的不超过39岁。镰状细胞病患者的平均胎儿血红蛋白在9.7%至13.5%之间变化，尽管仅在孔德人群体中胎儿血红蛋白与总血红蛋白呈轻微正相关。所有患有镰状细胞病的部落居民普遍出现疼痛性危象，57.5%的病例伴有黄疸。一些镰状细胞基因携带者也抱怨有疼痛性危象。制定一项在婴儿期识别纯合子并给予适当医疗管理的健康计划非常必要。
