部落镰状细胞贫血患者的胎儿血红蛋白升高:不幸中的万幸?

D. Trivedi, Priyali Purandare
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摘要

镰状细胞性贫血是一种分子疾病。世卫组织认为这是一个全球性的公共卫生问题。在印度,这种疾病在部落社区很常见。HbS 增高是罪魁祸首。治疗研究的重点是维持高水平的 HbF 和减少 2、3、BPG,以针对疾病。评估自然代偿血红蛋白变体在古吉拉特邦东北部镰状细胞贫血患者中的作用。对随机挑选的 50 名患病 5 年以上的镰状细胞性贫血患者进行前瞻性病例对照分析研究。50 名年龄和性别匹配的健康对照对象。研究对象包括 50 名镰状细胞性贫血患者和健康对照组。通过碱性血红蛋白电泳分析总血红蛋白水平、NESTROFT 法进行的西克林测试和血红蛋白变异。通过口头询问记录了临床危机的频率。结果使用 SPSS 20 版进行分析。采用学生非配对 t 检验来评估差异的显著性。P 值小于 0.05 视为具有统计学意义。我们观察到总血红蛋白水平下降,HbF 和 HbA2 水平升高,HbA0 水平下降。部落 SCA 患者 HbF 的补偿性升高表明临床危机发生的频率较低。镰状细胞性贫血患者的 HbA0 降低可通过部落患者 HbF 和 HbA2 的自然升高得到补偿,这对他们的总体健康产生了有益的影响。
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Elevated foetal haemoglobin in tribal sickle cell anaemic patients: Blessing in disgust?
Sickle cell anaemia is a molecular disease. WHO recognises it as a global public health problem. In India, it is common among tribal communities. Increased HbS is a culprit. Therapeutic research is focused on maintaining high levels of HbF and decreasing 2,3, BPG to target disease. To assess the role of naturally compensated haemoglobin variants in tribal Sickle cell anaemic patients of North East Gujarat. Prospective, analytical, case control study conducted on randomly selected fifty tribal Sickle cell anaemic patients having disease for more than 5 years. Fifty age and sex matched, healthy control subjects. Each fifty Tribal sickle cell anaemic patients and healthy control were included in the study. Total Haemoglobin level, Sickling test by NESTROFT method and Haemoglobin variants were analysed by alkaline haemoglobin electrophoresis. Frequencies of clinical crises were recorded by oral questioning. The results were analysed using SPSS version 20. Student unpaired t- test was employed to assess the significance of the differences. P-values < 0.05 considered statistically significant. We observed decreased levels of total haemoglobin, high levels of HbF and HbA2 along with reduced HbA0. Compensatory increase of HbF in tribal SCA patients have shown lower frequency of clinical crises. Reduced HbA0 in sickle cell anaemia is compensated by naturally elevated HbF and HbA2 in tribal patients which has a beneficial influence on their general health.
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