title: Psychoeducational characteristics of children with hypohidrotic ectodermal dysplasia.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Developmental Pediatrics, Department of Pediatrics, Saint Louis University, St. Louis, MO 63014, USA.
authors.forename: Rolanda A
authors.initials: RA
authors.lastname: Maxim
authors.forename: Samuel H
authors.initials: SH
authors.lastname: Zinner
authors.forename: Hisako
authors.initials: H
authors.lastname: Matsuo
authors.forename: Theresa M
authors.initials: TM
authors.lastname: Prosser
authors.forename: Mary
authors.initials: M
authors.lastname: Fete
authors.forename: Terry L
authors.initials: TL
authors.lastname: Leet
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Fete
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004476:Ectodermal Dysplasia / Q000150:complications / Q000523:psychology*; D005260:Female; D006801:Humans; D007007:Hypohidrosis / Q000150:complications / Q000523:psychology*; D008297:Male; D010353:Patient Education as Topic / Q000379:methods*