Department of Psychiatry, Drexel University College of Medicine, Philadelphia, PA.
Division of Hematology, St. Christopher's Hospital for Children, Philadelphia, PA.
J Pediatr Psychol. 2021 Jul 20;46(6):710-718. doi: 10.1093/jpepsy/jsab015.
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.
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).
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.
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.
羟基脲 (HU) 用于镰状细胞病 (SCD) 儿童，以增加胎儿血红蛋白 (HF)，有助于减轻身体症状，并可能预防大脑微血管病。HU 对该人群认知的影响很少被研究。本研究探讨了 HU 使用状况与 SCD 儿童认知之间的关系。
37 名年龄在 4 岁零 11 个月至 11 岁之间的无明显中风或慢性输血史的 HbSS 或 HbS/β0 地贫（镰状细胞贫血；SCA）儿童 SCD 完成了神经心理学测试组合。还获得了其他医学、实验室和人口统计学数据。HU 组（n=9）和未服用 HU 组（n=28）的儿童在言语、非言语和注意力/执行功能 3 个领域的神经心理学功能进行了比较。
HU 组儿童在注意力/执行功能和非言语技能的标准化测量中表现明显优于未服用 HU 组儿童。两组的言语测量表现相似。
这些结果表明，HU 治疗不仅可以减轻身体症状，而且可能对 SCA 儿童的认知产生潜在益处，特别是在注意力/执行功能和非言语技能方面。需要更大样本和纵向研究进行复制。
