Sickle cell disease can be life-threatening or chronically debilitating for both children and adults. Worldwide, more than 300 000 children are born with sickle cell disease every year, over 75% of whom in sub-Saharan Africa. Increased awareness and early interventions, such as neonate screening and comprehensive care, have led to considerable reductions in mortality in children younger than 5 years in high-income countries. However, sickle cell disease prevention and care have largely been neglected in Africa. Without intervention, 50-90% of affected children in many sub-Saharan African countries die before their fifth birthday. Fortunately, increasing initiatives in sub-Saharan Africa are piloting interventions such as neonate screening and comprehensive care, and as mortality declines, quality of life and increased life expectancy become major targets for interventions. Hydroxyurea (hydroxycarbamide) and haematopoietic stem-cell transplantation have already been shown to be effective therapies in high-income countries, but are either not widely accessible or too expensive for most African populations. These challenges are being alleviated by numerous networks evolving through international collaborations that are positively changing the outlook of sickle cell disease management in sub-Saharan Africa. In this Series paper, we describe the epidemiology, pathophysiology, clinicobiological profile, and psychosocial effects of sickle cell disease in sub-Saharan Africa. We highlight transferable strategies already used for the successful management of the condition and key strategies and recommendations for affordable and comprehensive care on the continent. TRANSLATION: For the French translation of the abstract see Supplementary Materials section.

中文翻译：

---

撒哈拉以南非洲的镰状细胞病：预防和护理的可转移策略。


对于儿童和成人来说，镰状细胞病可能会危及生命或造成慢性衰弱。全球每年有超过 300 000 名儿童出生时患有镰状细胞病，其中 75% 以上生活在撒哈拉以南非洲地区。提高认识和早期干预措施（例如新生儿筛查和综合护理）已导致高收入国家 5 岁以下儿童的死亡率大幅下降。然而，镰状细胞病的预防和护理在非洲很大程度上被忽视。如果不进行干预，许多撒哈拉以南非洲国家中 50-90% 的受影响儿童会在五岁生日前死亡。幸运的是，撒哈拉以南非洲地区越来越多的举措正在试点新生儿筛查和综合护理等干预措施，随着死亡率下降，生活质量和延长预期寿命成为干预措施的主要目标。羟基脲（羟基脲）和造血干细胞移植已被证明是高收入国家的有效疗法，但对于大多数非洲人群来说要么无法广泛使用，要么过于昂贵。通过国际合作不断发展的众多网络正在缓解这些挑战，这些网络正在积极改变撒哈拉以南非洲镰状细胞病管理的前景。在本系列论文中，我们描述了撒哈拉以南非洲地区镰状细胞病的流行病学、病理生理学、临床生物学概况和心理社会影响。我们重点介绍已经用于成功管理病情的可转移策略，以及非洲大陆负担得起的综合护理的关键策略和建议。翻译：有关摘要的法文翻译，请参阅补充材料部分。