Agarwal M B
Department of Haematology, Bombay Hospital Institute of Medical Sciences, Mumbai, India.
Indian J Pediatr. 2003 Aug;70(8):649-54. doi: 10.1007/BF02724255.
Sickle cell disease is numerically as common as thalassaemia. However, it affects relatively under privileged population i.e. tribal population belonging to economically poor class and having inadequate access to education and modern health facilities. A recent explosion acknowledged in understanding the pathogenesis of this disease has lead to newer dimensions in treatment. Some of these viz. prevention of overwhelming bacterial infection, present indications and controversies regarding blood transfusion, prevention of stroke, acute chest syndrome, hydroxyurea therapy--probably the best disease modifying agent at the moment, stem cell transplantation--a cure and certain promising experimental therapies including gene therapy have been discussed in this review.
镰状细胞病在数量上与地中海贫血一样常见。然而,它影响的是相对贫困的人群,即经济贫困阶层的部落人口,他们获得教育和现代医疗设施的机会不足。最近在理解这种疾病发病机制方面取得的重大进展为治疗带来了新的方向。本文综述了其中的一些内容,即预防严重细菌感染、输血的当前指征和争议、预防中风、急性胸综合征、羟基脲疗法(可能是目前最好的疾病修饰药物)、干细胞移植(一种治愈方法)以及某些有前景的实验性疗法,包括基因疗法。