目前在镰状细胞病治疗中使用的抗镰状细胞药物的不同治疗干预措施和作用机制

N. Imaga, O. Taiwo
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引用次数: 2

摘要

镰状细胞病是一种由镰状血红蛋白引起的遗传性疾病。在许多形式的疾病中,由于镰状血红蛋白异常聚合,红细胞可在脱氧时改变形状。血红蛋白相互粘在一起,使细胞具有坚硬的表面和镰刀状,并在此过程中破坏红细胞膜,导致细胞卡在血管中。这会剥夺下游组织的氧气,导致缺血和梗塞(可能导致器官损伤),如中风。该病最常见于非洲裔人群,地中海裔、拉丁裔、东印度裔和阿拉伯裔人群发病率较低(按此顺序)。在尼日利亚、加蓬、加纳和刚果共和国等非洲国家,镰状细胞特征的患病率在20%至30%之间,该疾病影响约2-3%的人口。从植物中提取的草药制剂被称为“植物药”,即使不能根除错误的S基因,也能有效地使患者摆脱危机状态,并使他们在社会中过上稳定的生活。这篇综述强调了镰状细胞病管理中使用的一些治疗选择,以期启发该主题的未来研究。
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Different Therapeutic Interventions and Mechanisms of Action of Antisickling Agents Currently in Use in Sickle Cell Disease Management
Sickle cell disease is a genetic disorder caused by sickle haemoglobin. In many forms of the disease, the red blood cells can change shape upon deoxygenation due to abnormal sickle haemoglobin polymerisation. The haemoglobin proteins stick to each other, causing the cell to have a rigid surface and sickle shape and in the process damaging the red blood cell membrane, causing the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischaemia and infarction (which may cause organ damage), such as stroke. Incidences of the disease are found most commonly in people of African descent and less commonly in people of Mediterranean, Latino, East Indian, and Arab descent (in that order). In African countries such as Nigeria, Gabon, Ghana, and the Republic of Congo, the prevalence of the sickle cell trait is between 20% and 30%, with the disease affecting ˜2–3% of the population. Herbal formulations prepared from plants are known as phytomedicines and are effective in keeping the patient out of a crisis state and enabling them to live stable lives in society, even though the faulty S gene is not eradicated but instead managed. This review highlights some of the therapeutic options in use in the management of sickle cell disease with a view to inspiring future research on this subject.
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