Growth characteristics of children with ectodermal dysplasia 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.
Motil
Kathleen J
KJ
Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA. kmotil@bcm.tmc.edu
Fete
Timothy J
TJ
Fraley
J Kennard
JK
Schultz
Rebecca J
RJ
Foy
Thomas M
TM
Ochs
Ulrike
U
Sybert
Virginia P
VP
D000293:Adolescent; D000328:Adult; D001827:Body Height; D015992:Body Mass Index; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D004476:Ectodermal Dysplasia / Q000150:complications*; D005260:Female; D006130:Growth Disorders / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D013577:Syndrome