Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia

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...

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Main Authors: Rolanda A. Maxim, Samuel H. Zinner, Hisako Matsuo, Theresa M. Prosser, Mary Fete, Terry L. Leet, Timothy J. Fete
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:The Scientific World Journal
Online Access:http://dx.doi.org/10.1100/2012/532371
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author Rolanda A. Maxim
Samuel H. Zinner
Hisako Matsuo
Theresa M. Prosser
Mary Fete
Terry L. Leet
Timothy J. Fete
author_facet Rolanda A. Maxim
Samuel H. Zinner
Hisako Matsuo
Theresa M. Prosser
Mary Fete
Terry L. Leet
Timothy J. Fete
author_sort Rolanda A. Maxim
collection DOAJ
description 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.
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spelling doaj-art-99e8884417e5445ba748aa518315bac82025-02-03T07:23:32ZengWileyThe Scientific World Journal1537-744X2012-01-01201210.1100/2012/532371532371Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal DysplasiaRolanda A. Maxim0Samuel H. Zinner1Hisako Matsuo2Theresa M. Prosser3Mary Fete4Terry L. Leet5Timothy J. Fete6Division of Developmental Pediatrics, Department of Pediatrics, Saint Louis University, St. Louis, MO 63014, USADivision of Developmental Medicine, Department of Pediatrics, University of Washington, Seattle, WA 98195-5852, USADepartment of Sociology and Criminal Justice, Saint Louis University, St. Louis, MO 63108, USASchool of Education, Texas Christian University, Forth Worth, TX 76129-0002, USANational Foundation for Ectodermal Dysplasias, Mascoutah, IL 62258, USADepartment of Pediatrics, Saint Louis University, St. Louis, MO 63108, USADepartment of Child Health, University of Missouri, Columbia, MO 65212, USAObjective. 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.http://dx.doi.org/10.1100/2012/532371
spellingShingle Rolanda A. Maxim
Samuel H. Zinner
Hisako Matsuo
Theresa M. Prosser
Mary Fete
Terry L. Leet
Timothy J. Fete
Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia
The Scientific World Journal
title Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia
title_full Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia
title_fullStr Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia
title_full_unstemmed Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia
title_short Psychoeducational Characteristics of Children with Hypohidrotic Ectodermal Dysplasia
title_sort psychoeducational characteristics of children with hypohidrotic ectodermal dysplasia
url http://dx.doi.org/10.1100/2012/532371
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