Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Department of Pediatrics, Division of Pediatric Hematology, Oncology, and Bone Marrow Transplantation, Cohen Children's Medical Center of New York, New Hyde Park, New York.
Pediatr Blood Cancer. 2020 May;67(5):e28196. doi: 10.1002/pbc.28196. Epub 2020 Feb 21.
Children with sickle cell disease (SCD) are at risk for neurocognitive deficits, which can lead to effects on academic performance and later job attainment. However, screening in children at high risk for poor academic performance (PAP) in a clinic setting has been limited. The goal was to identify young children with SCD at high risk for PAP via administration of a standardized screening tool at the clinic visit.
Parents of 20 patients were asked to complete the Behavior Assessment System for Children, 3rd edition (BASC-3) Parent Rating Scale. Children ages six to nine years and all SCD genotypes were included. Those patients who scored at least 1 standard deviation below the mean were considered high risk. Statistics was used to associate demographic, academic, and laboratory data with risk status (RS).
Four of 20 patients (20%) were found to be at risk by the BASC-3. A significant association was found between those with a history of PAP and RS (P = 0.001). A trend toward association was found between baseline hemoglobin, reticulocyte count, and RS. Children not at risk had a higher hemoglobin level and lower reticulocyte count (P = 0.37 and P = 0.20, respectively). Those on hydroxyurea were significantly less likely to score as at risk (P = 0.014), whereas those with siblings may be at greater risk (P = 0.037).
CONCLUSION(S): A parent-directed screening tool may identify children with SCD in need of additional school support. Further prospective studies are necessary to understand correlations found between hemoglobin, reticulocyte count, and hydroxyurea treatment and risk for PAP.
镰状细胞病（SCD）患儿存在神经认知缺陷风险，这可能导致学习成绩下降，并影响日后就业。然而，在诊所环境中对学习成绩不佳（PAP）高风险的儿童进行筛查受到限制。本研究旨在通过在就诊时使用标准化筛查工具来识别 SCD 患儿中存在 PAP 高风险的儿童。
要求 20 名患者的家长填写儿童行为评估系统第 3 版（BASC-3）家长评定量表。纳入年龄为 6 至 9 岁且具有所有 SCD 基因型的儿童。评分低于均值 1 个标准差的患儿被认为存在高风险。采用统计学方法将人口统计学、学业和实验室数据与风险状况（RS）相关联。
通过 BASC-3，20 名患者中有 4 名（20%）被确定为高风险。具有 PAP 病史与 RS 之间存在显著关联（P=0.001）。基线血红蛋白和网织红细胞计数与 RS 之间存在关联趋势。无风险患儿的血红蛋白水平较高，网织红细胞计数较低（P=0.37 和 P=0.20）。未服用羟基脲的患儿评分明显较低（P=0.014），而有兄弟姐妹的患儿可能面临更高的风险（P=0.037）。
家长指导的筛查工具可能会识别出需要额外学校支持的 SCD 患儿。需要进一步的前瞻性研究来了解血红蛋白、网织红细胞计数和羟基脲治疗与 PAP 风险之间的相关性。
