Objective Hydroxyurea (HU) is used in children with sickle cell disease (SCD) to increase fetal hemoglobin (HF), contributing to a decrease in physical symptoms and potential protection against cerebral microvasculopathy. There has been minimal investigation into the association between HU use and cognition in this population. This study examined the relationship between HU status and cognition in children with SCD. Methods Thirty-seven children with SCD HbSS or HbS/β0 thalassaemia (sickle cell anemia; SCA) ages 4:0–11 years with no history of overt stroke or chronic transfusion completed a neuropsychological test battery. Other medical, laboratory, and demographic data were obtained. Neuropsychological function across 3 domains (verbal, nonverbal, and attention/executive) was compared for children on HU (n = 9) to those not taking HU (n = 28). Results Children on HU performed significantly better than children not taking HU on standardized measures of attention/executive functioning and nonverbal skills. Performance on verbal measures was similar between groups. Conclusions These results suggest that treatment with HU may not only reduce physical symptoms, but may also provide potential benefit to cognition in children with SCA, particularly in regard to attention/executive functioning and nonverbal skills. Replication with larger samples and longitudinal studies are warranted.

中文翻译：

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羟基脲的使用与镰状细胞性贫血的非语言和执行技能相关

目的羟基脲 (HU) 用于患有镰状细胞病 (SCD) 的儿童，以增加胎儿血红蛋白 (HF)，有助于减少身体症状和潜在的脑微血管病保护。在该人群中，对 HU 使用与认知之间的关联的调查很少。本研究探讨了 SCD 儿童的 HU 状态与认知之间的关系。方法 37 名年龄在 4:0-11 岁且没有明显中风或慢性输血史的 SCD HbSS 或 HbS/β0 地中海贫血（镰状细胞性贫血；SCA）儿童完成了一项神经心理学测试。获得了其他医学、实验室和人口统计数据。将 HU (n = 9) 与未服用 HU (n = 28) 的儿童的 3 个领域（语言、非语言和注意力/执行力）的神经心理功能进行了比较。结果 使用 HU 的儿童在注意力/执行功能和非语言技能的标准化测量上明显优于未使用 HU 的儿童。各组之间口头测量的表现相似。结论 这些结果表明，使用 HU 治疗不仅可以减轻身体症状，还可以为 SCA 儿童的认知提供潜在的益处，特别是在注意力/执行功能和非语言技能方面。有必要进行更大样本的复制和纵向研究。但也可能对 SCA 儿童的认知产生潜在益处，特别是在注意力/执行功能和非语言技能方面。有必要进行更大样本的复制和纵向研究。但也可能对 SCA 儿童的认知产生潜在益处，特别是在注意力/执行功能和非语言技能方面。有必要进行更大样本的复制和纵向研究。