Affiliation:
1. Division of Child and Adolescent Psychiatry University of Washington Seattle Washington USA
2. Seattle Children's Autism Center Seattle Washington USA
3. Seattle Children's Research Institute Seattle Children's Hospital Seattle Washington USA
4. Division of Endocrinology and Diabetes Seattle Children's Hospital, University of Washington Seattle Washington USA
Abstract
AbstractPrader–Willi syndrome (PWS) is a rare genetic disorder caused by the loss of paternal genes on chromosome 15. The Aberrant Behavior Checklist (ABC) is a standardized rating scale for assessing problematic behaviors in persons with developmental disabilities. Our study aims to describe ABC scores in youth with PWS and track their change over time. The analysis included 69 patients. Mean ABC scores were compared in four age groups (5–8, 9–12, 13–16, and 17–22 years). A statistically significant difference was found only in the Irritability subscale, with lower scores in the 5–8 age group compared to the 9–12 age group. For change over time, scores for Irritability, Lethargy, Stereotypic Behavior, Hyperactivity subscales, and Total score were likely to decrease after age 12. Irritability subscale scores of males were predicted to increase more than those of females between ages of 5 and 12 . The Lethargy score in the nondeletion group had a greater reduction than the deletion group in the 12–20 year range. This study highlights the need for systematic collection and characterization of behavioral data given the burden of maladaptive behaviors that often persist for a lifetime.
Reference34 articles.
1. The aberrant behavior checklist: A behavior rating scale for the assessment of treatment effects;Aman M. G.;American Journal of Mental Deficiency,1985
2. Psychometric characteristics of the aberrant behavior checklist;Aman M. G.;American Journal of Mental Deficiency,1985
3. Reliability of the aberrant behavior checklist and the effect of variations in instructions;Aman M. G.;American Journal of Mental Deficiency,1987
4. The Global Prader–Willi Syndrome Registry: Development, Launch, and Early Demographics
5. Fases nutricionales en Síndrome de Prader-Willi