Neurocognitive functioning in preschool children with sickle cell disease.
Neurocognitive functioning in preschool children with sickle cell disease.
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DOI：
10.1002/pbc.29531
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发表时间：
2022-03
影响因子：
3.2
通讯作者：
Hankins, Jane S.
中科院分区：
文献类型：
--
作者：
Heitzer, Andrew M.;Cohen, Diana L.;Okhomina, Victoria I.;Trpchevska, Ana;Potter, Brian;Longoria, Jennifer;Porter, Jerlym S.;Estepp, Jeremie H.;King, Allison;Henley, Misham;Kang, Guolian;Hankins, Jane S.
关键词：
患有镰状细胞病（SCD）的儿童经历神经发育迟缓;然而，对学龄前儿童的研究有限。本研究旨在探讨神经认知风险和保护因素与学龄前儿童SCD。62例年龄在3 ~ 6岁（平均=4.77岁）的SCD患者（60%HbSS/HbSβ0-地中海贫血; 40%HbSC/HbSβ+-地中海贫血）接受了神经心理学评估作为常规系统监测。未根据疾病严重程度、既往中枢神经系统检查结果或现有认知问题选择患者。34例患者（82% HbSS/HbSβ0-地中海贫血）在进行神经心理学评估时接受了羟基脲（HU）处方。平均而言，这些患者在2.15岁（标准差=1.45）时接受了HU处方。平均剂量为28.8 mg/kg/天。除基因型外，基于HU治疗状态的医学或人口统计学因素无组间差异。HbSS/HbSβ0-地中海贫血患者在智力、语言理解和入学准备方面的评分低于正常预期（错误发现率校正的p值[pFDR]<0.05）。年龄、镰状基因型和HU治疗暴露与测量的神经认知结果无关（pFDR>0.05）。在社区层面上，更大的社会脆弱性与智力功能、语言理解、视觉控制和入学准备以及父母报告的执行功能障碍的测量结果相关（pFDR<0.05）。更大的家庭社会经济地位与学业准备呈正相关。患有严重SCD（HbSS/HbSβ0-地中海贫血）的学龄前儿童在智力和学术准备方面的表现低于年龄预期。社会人口因素是比疾病严重程度或疾病改善治疗更强的神经认知表现驱动因素。需要针对家庭和更广泛的社区环境进行神经发育干预。
Children with sickle cell disease (SCD) experience neurodevelopmental delays; however, there is limited research with preschool age children. This study examined neurocognitive risk and protective factors in preschoolers with SCD. Sixty-two patients with SCD (60% HbSS/HbSβ0-thalassemia; 40% HbSC/HbSβ+-thalassemia) between the ages of 3 and 6 years (Mean=4.77 years) received a neuropsychological evaluation as routine systematic surveillance. Patients were not selected for disease severity, prior central nervous system findings, or existing cognitive concerns. Thirty-four patients (82% HbSS/HbSβ0-thalassemia) were prescribed hydroxyurea (HU) at the time of their neuropsychological evaluation. On average, these patients had been prescribed HU at 2.15 (Standard Deviation=1.45) years of age. The average dose was 28.8 mg/kg/day. Besides genotype, there were no group differences in medical or demographic factors based on HU treatment status. Patients with HbSS/HbSβ0-thalassemia scored below normative expectations on measures of intelligence, verbal comprehension, and school readiness (false discovery rate adjusted p-value [pFDR]<0.05). Age, sickle genotype, and HU treatment exposure were not associated with measured neurocognitive outcomes (pFDR>0.05). Greater social vulnerability at the community level was associated with poorer performance on measures of intellectual functioning, verbal comprehension, visuomotor control, and school readiness, as well as parent report of executive dysfunction (pFDR<0.05). Greater household socioeconomic status was positively associated with academic readiness. Preschoolers with severe SCD (HbSS/HbSβ0-thalassemia) perform below age expectations on measures of intelligence and academic readiness. Sociodemographic factors were stronger drivers of neurocognitive performance than disease severity or disease-modifying treatment. Neurodevelopmental interventions targeting the home and broader community environment are needed.
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作者：
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