TY - JOUR
T1 - Repetitive and Self-injurious Behaviors in Children with Cornelia de Lange Syndrome
AU - Srivastava, Siddharth
AU - Clark, Bennett
AU - Landy-Schmitt, Colleen
AU - Offermann, Elizabeth A.
AU - Kline, Antonie D.
AU - Wilkinson, Samuel T.
AU - Grados, Marco A.
N1 - Funding Information:
Dr. Wilkinson has received contract funding administered through Yale University from Janssen and Sage Therapeutics for the conduct of clinical trials; he anticipates receiving contract funding from Oui Therapeutics and LivaNova. He has received consulting fees from Janssen, Oui Therapeutics, and Biohaven. Dr. Srivastava has received consulting fees from Guidepoint. The other authors have no other conflict of interest to disclose.
Funding Information:
Dr. Wilkinson gratefully acknowledges support from the Agency for Healthcare Research and Quality (5K12HS023000).
Publisher Copyright:
© 2020, Springer Science+Business Media, LLC, part of Springer Nature.
PY - 2021/5
Y1 - 2021/5
N2 - 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.
AB - 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.
KW - Cornelia de Lange syndrome
KW - Repetitive behaviors
KW - Self-injurious behaviors
KW - Stereotypies
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U2 - 10.1007/s10803-020-04617-x
DO - 10.1007/s10803-020-04617-x
M3 - Article
C2 - 32809170
AN - SCOPUS:85089477192
SN - 0162-3257
VL - 51
SP - 1748
EP - 1758
JO - Journal of Autism and Developmental Disorders
JF - Journal of Autism and Developmental Disorders
IS - 5
ER -