Early motor development in young children with 22q.11 deletion syndrome and a conotruncal heart defect.
Velocardiofacial syndrome is identified by a submicroscopic deletion of chromosome 22q.11 (del22q.11). This study presents data on the early motor development and behaviour of 11 children (8 males, 3 females) with del22q.11 (mean age 41mo, SD 9.7mo) with a congenital heart defect. To control for the impact of the congenital heart defect, a control group of 19 children (15 males, 4 females; mean age 46mo, SD 9mo) with the same types of congenital heart defects but without del22q.11 was selected. Motor development in both groups was measured with the Peabody Developmental Motor Scales-2. Behaviour was assessed with the Child Behaviour Checklist. Children with del22q.11 scored significantly lower (p<0.05) on motor performance than the children of the control group. Most deficient motor skills were found for the subtests Locomotion and Stationary. On the behaviour questionnaire, a statistically significant (p<0.05) difference between the two groups was found only for the subscale Withdrawn. These data reveal a significant motor delay in many young children with del22q.11, which is not caused by the presence of a congenital heart defect or by behavioural features.
Swillen
Ann
A
Department of Human Genetics, University Hospital Gasthuisberg, University of Leuven, Herestraat 49, 3000 Leuven, Belgium. Ann.Swillen@uz.kuleuven.ac.be
Feys
Hilde
H
Adriaens
Tamara
T
Nelissen
Loes
L
Mertens
Luc
L
Gewillig
Marc
M
Devriendt
Koen
K
Fryns
Jean-Pierre
JP
D016022:Case-Control Studies; D002657:Child Development; D002675:Child, Preschool; D002872:Chromosome Deletion; D002892:Chromosomes, Human, Pair 22; D015897:Comorbidity; D004062:DiGeorge Syndrome / Q000150:complications* / Q000235:genetics; D005260:Female; D006330:Heart Defects, Congenital; D006801:Humans; D007223:Infant; D008297:Male; D019957:Motor Skills Disorders / Q000209:etiology*