Hydroxyurea treatment and neurocognitive functioning in sickle cell disease from school age to young adulthood.
Hydroxyurea treatment and neurocognitive functioning in sickle cell disease from school age to young adulthood.
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羟基脲治疗和神经认知功能在镰状细胞疾病中从学龄到成年。
DOI：
10.1111/bjh.17687
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发表时间：
2021-10
影响因子：
6.5
通讯作者：
Hankins JS
中科院分区：
文献类型：
--
作者：
Heitzer AM;Longoria J;Okhomina V;Wang WC;Raches D;Potter B;Jacola LM;Porter J;Schreiber JE;King AA;Kang G;Hankins JS
神经认知障碍在镰状细胞病（SCD）中很常见，并与显著的功能限制相关。在横断面分析中，我们研究了羟基脲（HU）治疗与SCD患者学龄至青年期神经认知功能之间的关系。共有215名HbSS/HbSβ0-地中海贫血患者（71% HU治疗）和149名HbSC/HbSβ+-地中海贫血患者（20% HU治疗）在学龄（8-9岁）、青春期早期（12-13岁）、青春期晚期（16-17岁）和成年早期（19-24岁）四个发育阶段之一完成了神经认知测量。对于多次评估的参与者，只包括最近的评估。在调整了社会脆弱性、HU治疗和性别的多变量分析中，HbSS/ HbSS β0-地中海贫血患者年龄越大，总体智商（IQ）每年降低0.55分[标准误差(SE) = 0.18，错误发现率调整P值(PFDR) = 0.01]。在调整了社会脆弱性、性别和治疗持续时间后，HbSS/ hbs - β0-地中海贫血患者较早开始HU （n = 152）与大多数领域的神经认知测试得分较高相关，包括智商[估计（SE） 0.77(0.25)/年，PFDR = 0.01]。这些结果支持早期使用HU来限制SCD的有害神经认知影响，同时强调需要采取其他措施来进一步减轻神经认知恶化。
Neurocognitive impairment is common in sickle cell disease (SCD) and is associated with significant functional limitations. In a cross-sectional analysis, we examined the association between hydroxyurea (HU) treatment and neurocognitive functioning from school-age to young adulthood in individuals with SCD. A total of 215 patients with HbSS/HbSβ0-thalassaemia (71% HU treated) and 149 patients with HbSC/HbSβ+-thalassaemia (20% HU treated) completed neurocognitive measures at one of four developmental stages: school-age (age 8–9 years), early adolescence (age 12–13 years), late adolescence (age 16–17 years) and young adulthood (ages 19–24 years). For participants with multiple assessments, only the most recent evaluation was included. In multivariable analysis adjusted for social vulnerability, HU treatment and sex, older age was associated with a reduction in overall intelligence quotient (IQ) of 0.55 points per year of life [standard error (SE) = 0·18, false discovery rate adjusted P value (PFDR) = 0.01] for patients with HbSS/HbSβ0-thalassaemia. Earlier initiation of HU (n = 152) in HbSS/HbSβ0-thalassaemia was associated with higher scores on neurocognitive measures across most domains, including IQ [estimate (SE) 0·77 (0·25)/year, PFDR = 0·01], after adjusting for social vulnerability, sex and treatment duration. These results support the early use of HU to limit the detrimental neurocognitive effects of SCD, while highlighting the need for additional measures to further mitigate neurocognitive deterioration.
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影响因子：
8
作者：
Armstrong, F. Daniel;Elkin, T. David;Wang, Winfred C.
通讯作者：
Wang, Winfred C.
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