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Updated: Aug 16, 2026
Assessing Signaling Properties of Ectodermal Epithelia During Craniofacial Development
Published on: March 24, 2011
Growth characteristics of children with ectodermal dysplasia syndromes
Kathleen J Motil1, Timothy J Fete, J Kennard Fraley
1Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA. kmotil@bcm.tmc.edu
Insights
Children with ectodermal dysplasia (ED) syndromes experience significant weight deficits from infancy through adolescence. Height deficits are primarily observed in ED types other than hypohidrotic ectodermal dysplasia (HED).
Area of Science:
- Pediatric Endocrinology
- Genetics
- Growth and Development
Background:
- Ectodermal dysplasia (ED) syndromes are a group of genetic disorders characterized by abnormalities in ectodermal structures.
- Clinical observations suggest potential growth abnormalities in affected children, necessitating further investigation.
Purpose of the Study:
- To characterize the longitudinal growth patterns in a cohort of children diagnosed with ED syndromes.
- To investigate whether linear and ponderal growth abnormalities differ among the clinical variants of ED.
Main Methods:
- A cohort of 138 children with ED was studied, with 74% having hypohidrotic ED (HED).
- Standardized height and weight measurements were collected and converted to z scores for comparison with a reference population.
- Statistical analyses including linear regression and t tests were employed to identify growth abnormalities.
Main Results:
- Children with ED syndromes exhibited significantly lower mean weight-for-age, weight-for-height, and BMI-for-age z scores compared to the reference population.
- Weight deficits persisted from infancy through adolescence, with significant increases in weight-for-age and weight-for-height z scores over time.
- Height deficits were specifically noted in children with ED syndromes other than HED.
Conclusions:
- Weight deficits are a common and persistent feature of ED syndromes from early childhood into adolescence.
- Height deficits are primarily associated with non-HED forms of ectodermal dysplasia.
- Clinical vigilance for growth abnormalities is crucial in managing children with ED syndromes.
Objective:
Clinical observations suggested that growth abnormalities may be present in children with ectodermal dysplasia (ED) syndromes. This study characterizes the longitudinal pattern of growth in a cohort of children with the ED syndromes. We hypothesized that (1) linear and ponderal growth abnormalities are present in children with ED from infancy through adolescence, and (2) linear and ponderal growth abnormalities differ among the clinical variants of these disorders.
Methods:
We studied 138 children who had ED and were registered with the National Foundation for Ectodermal Dysplasias, 74% of whom had clinical features consistent with the hypohidrotic EDs (HEDs). Height (or length) and weight measurements were obtained by standardized techniques and from review of available medical records. We converted these measurements to weight-for-height (children younger than 5 years and <103 cm in length) or BMI (children > or =2 years old). Height, weight, weight-for-height, and BMI were converted to age- and gender-specific z scores. We applied linear regression, 1-sample t tests, and analysis of variance to detect linear and ponderal growth abnormalities in children with ED compared with a reference population.
Results:
Mean weight-for-age, weight-for-height, and BMI-for-age z scores but not height-for-age z score, were significantly lower in children with the ED syndromes than in the reference population. Mean weight-for-age and weight-for-height z scores but not BMI-for-age or height-for-age z scores increased significantly with increasing age. The mean height-for-age z score of children with the ED syndromes other than the HEDs was significantly lower than that of children with the HEDs.
Conclusions:
Growth abnormalities, measured as weight deficits, were present at an early age in children with the ED syndromes and persisted through adolescence. Height deficits were seen only in children with ED syndromes other than HEDs. Clinicians should evaluate carefully children with ED syndromes for growth abnormalities.
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