Department of Pediatric Psychology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
Res Dev Disabil. 2013 Sep;34(9):2937-45. doi: 10.1016/j.ridd.2013.06.001. Epub 2013 Jun 29.
Patients with 22q11DS are at risk of behavioral problems and cognitive impairment. Recent studies suggest a possible intellectual decline in 22q11DS children. To date it is unknown if cognitive development is related to the behavioral problems in 22q11DS. We studied 53 children with 22q11DS who underwent cognitive and behavioral assessments at 9.5 years (T1) and 15.3 years (T2). In about one third, IQ data obtained at 7.5 years (T0) were also available. Results showed that internalizing behaviors intensified while externalizing behaviors decreased. Simultaneously, in about a third a significant decline in IQ was found, which, surprisingly, was unrelated to the behavioral changes. It can be concluded that children with 22q11DS follow a unique developmental trajectory. Cognitive deterioration is severe in some but does not appear to predict behavioral problems in early adolescence.
22q11DS 患者存在行为问题和认知障碍风险。最近的研究表明，22q11DS 儿童可能存在智力下降。目前尚不清楚认知发育是否与 22q11DS 的行为问题有关。我们研究了 53 名 22q11DS 患儿，他们在 9.5 岁（T1）和 15.3 岁（T2）时接受了认知和行为评估。约三分之一的患儿还在 7.5 岁（T0）时获得了智商数据。结果显示，内化行为加剧，外化行为减少。同时，约三分之一的患儿的智商显著下降，但令人惊讶的是，智商下降与行为变化无关。可以得出结论，22q11DS 患儿具有独特的发育轨迹。在一些患儿中，认知恶化严重，但似乎不会预测青春期早期的行为问题。
