Department of Human Genetics, KU Leuven, Leuven, Belgium.
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Am J Med Genet A. 2018 Oct;176(10):2160-2166. doi: 10.1002/ajmg.a.38709. Epub 2018 Apr 25.
The 22q11.2 deletion syndrome (22q11.2 DS) places affected individuals at an increased risk for neurodevelopmental/cognitive, behavioral and social-emotional difficulties. Poor cognitive functioning and intellectual disabilities, attention and executive functioning deficits, learning disorders, emotional dysregulation and impairments in social processing are common among individuals with 22q11.2 DS. Identifying risk and protective/resilience factors that can be detected in early life and can predict neurodevelopmental outcomes for people with 22q11.2 DS is of significant clinical relevance and might allow for early detection and intervention. Given the focus of this review, we will discuss the possible contributing factors that influence the neurodevelopmental outcome in 22q1.2 DS, the cognitive phenotype in 22q11.2 DS, the different developmental trajectories across life span, and the implications for clinical practice and management.
22q11.2 缺失综合征(22q11.2DS)使受影响的个体面临神经发育/认知、行为和社会情感障碍的风险增加。认知功能差和智力残疾、注意力和执行功能缺陷、学习障碍、情绪失调以及社交处理受损在 22q11.2DS 患者中很常见。确定可在早期检测到的风险和保护/恢复力因素,并可预测 22q11.2DS 患者的神经发育结果具有重要的临床意义,可能有助于早期发现和干预。鉴于本综述的重点,我们将讨论可能影响 22q11.2DS 神经发育结果的相关因素、22q11.2DS 的认知表型、整个生命周期的不同发展轨迹,以及对临床实践和管理的影响。