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在整个非洲大陆实现羟基脲的有效性:多中心 REACH 研究在撒哈拉以南非洲的入组和基线特征。

Realizing effectiveness across continents with hydroxyurea: Enrollment and baseline characteristics of the multicenter REACH study in Sub-Saharan Africa.

机构信息

Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

KEMRI/Wellcome Trust Research Programme, Kilifi, Kenya.

出版信息

Am J Hematol. 2018 Aug;93(4):537-545. doi: 10.1002/ajh.25034. Epub 2018 Jan 27.

Abstract

Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries. Following comprehensive training of local research teams, REACH was approved by local Ethics Committees and achieved full enrollment ahead of projections with 635 participants enrolled over a 30-month period, despite half of families living >12 km from their clinical site. At enrollment, study participants (age 5.4 ± 2.4 years) had substantial morbidity, including a history of vaso-occlusive pain (98%), transfusion (68%), malaria (85%), and stroke (6%). Significant differences in laboratory characteristics were noted across sites, with lower hemoglobin concentrations (P < .01) in Angola (7.2 ± 1.0 g/dL) and the DRC (7.0 ± 0.9 g/dL) compared to Kenya (7.4 ± 1.1 g/dL) and Uganda (7.5 ± 1.1 g/dL). Analysis of known genetic modifiers of SCA demonstrated a high frequency of α-thalassemia (58.4% with at least a single α-globin gene deletion) and G6PD deficiency (19.7% of males and 2.4% of females) across sites. The CAR β-globin haplotype was present in 99% of participants. The full enrollment to REACH confirms the feasibility of conducting high-quality SCA research in Africa; this study will provide vital information to guide safe and effective dosing of hydroxyurea for children with SCA living in Africa.

摘要

尽管羟基脲在高收入国家治疗镰状细胞贫血(SCA)方面的安全性和疗效已得到充分描述,但在撒哈拉以南非洲地区,这种药物仍然难以获得,因为该地区每年有超过 75%的 SCA 患儿出生,并且存在许多合并症。跨越大陆实现羟基脲疗效(REACH,ClinicalTrials.gov NCT01966731)是一项前瞻性、I/II 期开放标签羟基脲试验,旨在评估羟基脲治疗撒哈拉以南非洲四个国家 SCA 儿童的可行性、安全性和益处。在对当地研究团队进行全面培训后,REACH 获得了当地伦理委员会的批准,并在预期之前实现了全额入组,在 30 个月的时间内招募了 635 名参与者,尽管一半的家庭距离其临床地点超过 12 公里。在入组时,研究参与者(年龄 5.4 ± 2.4 岁)有严重的发病情况,包括血管阻塞性疼痛(98%)、输血(68%)、疟疾(85%)和中风(6%)病史。各研究点之间的实验室特征存在显著差异,安哥拉(7.2 ± 1.0 g/dL)和刚果民主共和国(7.0 ± 0.9 g/dL)的血红蛋白浓度较低(P <.01),而肯尼亚(7.4 ± 1.1 g/dL)和乌干达(7.5 ± 1.1 g/dL)。对 SCA 已知遗传修饰因子的分析表明,各研究点的α-地中海贫血发生率较高(58.4%至少有一个α-珠蛋白基因缺失)和 G6PD 缺乏症(19.7%的男性和 2.4%的女性)。CAR β-珠蛋白单倍型存在于 99%的参与者中。REACH 的全面入组证实了在非洲开展高质量 SCA 研究的可行性;这项研究将为指导在非洲生活的 SCA 儿童安全有效使用羟基脲提供重要信息。

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