Duke University School of Nursing, Durham, NC.
Washington University School of Medicine, St. Louis, MO.
J Pediatr Hematol Oncol. 2021 Apr 1;43(3):104-111. doi: 10.1097/MPH.0000000000002091.
Sickle cell disease (SCD) is the most common abnormal genetic blood disease that affects ∼100,000 Americans. Approximately 20% to 37% of children with sickle cell anemia have silent cerebral infarcts by the age of 14 years old. Neurocognitive deficits are identified in infants and preschool children with SCD. The purpose of this systematic literature review is to provide a comprehensive understanding of the prevalence, severity, and the associated risk factors for neurodevelopmental delays (NDDs) in children with SCD 5 years of age and younger.
Systematic search of 6 databases identified 2467 potentially relevant publications and 8 were identified through a manual search. Only 24 articles met the inclusion criteria.
We identified an increased prevalence of NDDs (cognitive, motor, or both). Children experienced deficits with language, attention and behavior, executive functioning, school readiness and/or academic performance, and motor skills (fine and gross motor functioning). Risk factors include silent cerebral infarcts and strokes, SCD genotype (HbSS>HbSC), other biologic, and social factors.
NDDs are common in children ages 0 to 5 years old with SCD. There is an opportunity to improve adherence to national guideline recommendations and early detection practices by pediatricians, hematologists, and other health care providers.
镰状细胞病（SCD）是最常见的异常遗传性血液疾病，影响约 10 万名美国人。大约 20%至 37%的镰状细胞性贫血儿童在 14 岁时就有无症状性脑梗死。镰状细胞病婴儿和学龄前儿童存在神经认知缺陷。本系统文献综述的目的是全面了解 5 岁及以下 SCD 儿童神经发育迟缓（NDD）的患病率、严重程度和相关危险因素。
对 6 个数据库进行系统搜索，共确定了 2467 篇潜在相关文献，并通过手动搜索确定了 8 篇文献。只有 24 篇文章符合纳入标准。
我们发现 NDD（认知、运动或两者兼有）的患病率增加。儿童在语言、注意力和行为、执行功能、入学准备和/或学业成绩以及运动技能（精细和粗大运动功能）方面存在缺陷。危险因素包括无症状性脑梗死和中风、SCD 基因型（HbSS>HbSC）、其他生物学和社会因素。
NDD 在 0 至 5 岁 SCD 儿童中很常见。儿科医生、血液科医生和其他医疗保健提供者有机会改善对国家指南建议和早期检测实践的依从性。
