Department of Pediatrics, University of Colorado School of Medicine, Aurora, Colorado, USA.
eXtraordinarY Kids Program, Children's Hospital Colorado, Aurora, Colorado, USA.
Am J Med Genet C Semin Med Genet. 2020 Jun;184(2):414-427. doi: 10.1002/ajmg.c.31785. Epub 2020 May 25.
Sex chromosome aneuploidies (SCAs) are the most commonly occurring aneuploidies in children with a collective prevalence rate of 1 in 500 live births. Prior research has documented SCAs are associated with an increased risk for early expressive language and gross motor delays, learning disorders, ADHD, autism spectrum disorder, anxiety, and executive function problems. Although SCAs have been historically underdiagnosed in young children, recent advances in noninvasive prenatal testing have resulted in an increasing nationwide cohort of infants with confirmed diagnoses. Consequently, early childhood support systems must prepare for an influx of children with known risks for associated developmental delays and potential school problems. This national survey aimed to update our understanding of current early childhood intervention services for young children with SCA in the United States and to describe parent perspectives and priorities. Descriptive statistics, chi-square tests, and logistic regression models controlling for parent education revealed a majority of respondents reported receiving public early childhood intervention services with speech therapy as the most common service. There were significant differences in early childhood intervention services by timing of diagnosis (prenatal vs. postnatal), number of sex chromosomes (trisomy vs. tetra/pentasomy), and geographic location. Parents described interventions as desirable and effective yet also difficult to obtain due to issues with the SCA phenotype, lack of provider knowledge, and challenges navigating the intervention systems. Results support the need for enhanced provider training in SCAs, policy change for early childhood intervention qualification criteria for SCA conditions, and collaboration between medical and early childhood settings.
性染色体非整倍体 (SCAs) 是儿童中最常见的非整倍体,其总患病率为每 500 例活产儿中有 1 例。先前的研究表明,SCAs 与早期表达性语言和粗大运动发育迟缓、学习障碍、注意力缺陷多动障碍、自闭症谱系障碍、焦虑和执行功能问题的风险增加有关。尽管 SCAs 在幼儿中以前被低估,但非侵入性产前检测的最新进展导致全国范围内确诊患有该病的婴儿数量不断增加。因此,幼儿支持系统必须为已知存在相关发育迟缓风险和潜在学校问题的大量儿童做好准备。本全国性调查旨在更新我们对美国 SCA 幼儿当前幼儿期干预服务的理解,并描述家长的观点和优先事项。描述性统计、卡方检验和控制家长教育的逻辑回归模型显示,大多数受访者报告接受了公共幼儿期干预服务,其中言语治疗是最常见的服务。早期幼儿干预服务在诊断时间(产前与产后)、性染色体数量(三体与四体/五体)和地理位置方面存在显著差异。家长描述了干预措施是可取且有效的,但由于 SCA 表型问题、提供者知识缺乏以及干预系统难以导航等问题,干预措施也难以获得。研究结果支持需要加强提供者在 SCAs 方面的培训,为 SCA 条件的幼儿期干预资格标准制定政策改变,并在医疗和幼儿期环境之间开展合作。