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The Scientific World Journal
Volume 2012, Article ID 532371, 8 pages
Research Article

Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia

1Division of Developmental Pediatrics, Department of Pediatrics, Saint Louis University, St. Louis, MO 63014, USA
2Division of Developmental Medicine, Department of Pediatrics, University of Washington, Seattle, WA 98195-5852, USA
3Department of Sociology and Criminal Justice, Saint Louis University, St. Louis, MO 63108, USA
4School of Education, Texas Christian University, Forth Worth, TX 76129-0002, USA
5National Foundation for Ectodermal Dysplasias, Mascoutah, IL 62258, USA
6Department of Pediatrics, Saint Louis University, St. Louis, MO 63108, USA
7Department of Community Health, Saint Louis University, St. Louis, MO 63108, USA
8Department of Child Health, University of Missouri, Columbia, MO 65212, USA

Received 24 October 2011; Accepted 17 November 2011

Academic Editors: B. M. Piraccini and M. Priolo

Copyright © 2012 Rolanda A. Maxim et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Objective. Hypohidrotic ectodermal dysplasia (HED) is an X-linked hereditary disorder characterized by hypohidrosis, hypotrichosis, and anomalous dentition. Estimates of up to 50% of affected children having intellectual disability are controversial. Method. In a cross-sectional study, 45 youth with HED (77% males, mean age 9.75 years) and 59 matched unaffected controls (70% males, mean age 9.79 years) were administered the Kaufman Brief Intelligence Test and the Kaufman Test of Educational Achievement, and their parents completed standardized neurodevelopmental and behavioral measures, educational, and health-related information regarding their child, as well as standardized and nonstandardized data regarding socioeconomic information for their family. Results. There were no statistically significant differences between the two groups in intelligence quotient composite and educational achievement scores, suggesting absence of learning disability in either group. No gender differences within or between groups were found on any performance measures. Among affected youth, parental education level correlated positively with (1) cognitive vocabulary scores and cognitive composite scores; (2) educational achievement for mathematics, reading, and composite scores. Conclusion. Youth affected with HED and unaffected matched peers have similar profiles on standardized measures of cognition, educational achievement, and adaptive functioning although children with HED may be at increased risk for ADHD.