Reference Center for Sickle Cell Disease, Hôpital Necker-Enfants malades, AP-HP, Université Paris Descartes, Labex GR-Ex, Paris, France; and.
Centre Hospitalier Mère-Enfant, CEFA/Monkole, Kinshasa, Democratic Republic of the Congo.
Hematology Am Soc Hematol Educ Program. 2019 Dec 6;2019(1):490-495. doi: 10.1182/hematology.2019000053.
As more children are appropriately being diagnosed, the burden of sickle cell disease is increasing greatly in Africa and in high-resource countries such as the United States and Europe. Early management is mandatory, but newborn screening is not implemented everywhere. Point-of-care testing devices are increasingly being used in low-resource countries, showing good sensitivity and specificity. Because the diagnosis is often traumatic for the families, the announcement should be made by an experienced person. The development of care networks is urgently required to facilitate daily life by defining the respective functions of nearby and highly specialized health care professionals, who should work in close collaboration. Comprehensive programs targeting the prevention of pneumococcal infections, malaria in infested zones, and stroke may substantially improve patient care. Hydroxyurea is increasingly being used, but whether it should be systematically prescribed in all children is debated, and its access is still limited in many African countries. Yearly checkups should be organized early in life in order to screen and then treat any organ impairment. Enhancing parents' and patients' knowledge and skills is mandatory.
随着越来越多的儿童得到适当诊断,镰状细胞病在非洲以及美国和欧洲等资源丰富的国家的负担大大增加。早期管理是强制性的,但并非所有地方都实施新生儿筛查。即时检测设备在资源匮乏的国家越来越多地被使用,显示出良好的敏感性和特异性。由于诊断通常对家庭来说是创伤性的,因此应由有经验的人宣布。迫切需要发展护理网络,通过定义附近和高度专业化的医疗保健专业人员的各自职能来便利日常生活,他们应该密切合作。针对预防肺炎球菌感染、疫区疟疾和中风的综合计划可能会大大改善患者的护理。羟基脲的使用越来越多,但是否应在所有儿童中系统使用仍存在争议,在许多非洲国家,其获取仍然有限。应在生命早期定期进行体检,以筛查和治疗任何器官损伤。必须提高家长和患者的知识和技能。