科妮莉亚·德朗热综合征患儿的重复及自伤行为
Repetitive and Self-injurious Behaviors in Children with Cornelia de Lange Syndrome.
作者信息
Srivastava Siddharth, Clark Bennett, Landy-Schmitt Colleen, Offermann Elizabeth A, Kline Antonie D, Wilkinson Samuel T, Grados Marco A
机构信息
Department of Neurology, Boston Children's Hospital, Boston, MA, USA.
Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
出版信息
J Autism Dev Disord. 2021 May;51(5):1748-1758. doi: 10.1007/s10803-020-04617-x.
Cornelia de Lange syndrome (CdLS) is associated with repetitive and self-injurious behaviors (RBs, SIB). Evaluating children with CdLS, this study: (1) characterizes the spectrum of RBs; (2) characterizes the impact and severity of RBs including SIB; (3) describes how age and adaptive functioning relate to RBs including SIB. Fifty children (5-17 years) with CdLS were assessed with Children's Yale-Brown Obsessive Compulsive Scale Modified for PDD; Aberrant Behavior Checklist (ABC); Vineland Adaptive Behaviors Scales (VABS). All children had ≥ 1 type of RB; 44% had some form of SIB. 64% spent > 1 h/day displaying RBs. Lower VABS adaptive functioning was associated with higher stereotypy and SIB scores (ABC). In children with CdLS, RBs including SIB are common, impactful, and associated with lower adaptive functioning.
科妮莉亚·德朗格综合征（CdLS）与重复和自伤行为（RB、SIB）有关。在对患有CdLS的儿童进行评估时，本研究：（1）描述RB的范围；（2）描述RB包括SIB的影响和严重程度；（3）描述年龄和适应性功能如何与包括SIB在内的RB相关。对50名年龄在5至17岁的患有CdLS的儿童使用针对广泛性发育障碍修改的儿童耶鲁-布朗强迫量表、异常行为检查表（ABC）、文兰适应性行为量表（VABS）进行评估。所有儿童都有≥1种RB类型；44%有某种形式的SIB。64%的儿童每天花费超过1小时表现出RB。较低的VABS适应性功能与较高的刻板行为和SIB分数（ABC）相关。在患有CdLS的儿童中，包括SIB在内的RB很常见、有影响，并且与较低的适应性功能相关。
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