Intellectual abilities in a large sample of children with Velo–Cardio–Facial Syndrome: an update

Journal of Intellectual Disability Research - Tập 51 Số 9 - Trang 666-670 - 2007
Bert De Smedt1, Koenraad Devriendt, J. P. Fryns, Annick Vogels, Marc Gewillig, Ann Swillen
1Centre for Disability, Special Needs Education and Child Care, and Centre for Human Genetics, University of Leuven, Belgium. [email protected]

Tóm tắt

AbstractBackground  Learning disabilities are one of the most consistently reported features in Velo–Cardio–Facial Syndrome (VCFS). Earlier reports on IQ in children with VCFS were, however, limited by small sample sizes and ascertainment biases. The aim of the present study was therefore to replicate these earlier findings and to investigate intellectual abilities in a large sample of children with VCFS. In addition, we aimed to identify factors that may contribute to within‐syndrome variability in cognitive performance, such as the mode of inheritance of the deletion, sex, the presence of a heart defect and psychiatric morbidity.Method  IQ data of 103 children with VCFS (56 males, 47 females) were collected. Psychiatric diagnosis was additionally recorded.Results  Children with VCFS had a mean full‐scale IQ (FSIQ) of 73.48 (range: 50–109). There were no effects of sex, presence of a heart defect and psychiatric condition on intellectual profile. Inheritance of the deletion affected cognitive performance in VCFS, with children with familial deletions having significant lower FSIQ than children with a de novo deletion.Conclusions  Learning disabilities are very common in children with VCFS, although marked within syndrome variability is noted. One factor contributing to this variability seems to be the mode of inheritance of the deletion.

Từ khóa


Tài liệu tham khảo

American Psychiatric Association, 1994, Diagnostic and Statistical Manual of Mental Disorders

10.1207/s15326942dn2803_6

10.1542/peds.112.1.101

10.1007/s10803-005-5036-9

10.1002/(SICI)1096-8628(19990716)85:2<127::AID-AJMG6>3.0.CO;2-F

10.1002/ajmg.b.20144

10.1038/nn1572

10.1097/00008480-200110000-00016

10.1016/S0022-3476(99)70415-4

10.1111/j.1365-2788.2004.00620.x

10.1097/00125817-200101000-00017

OECD, 1999, Classifying educational programmes: Manual for ISCED‐97 implementation in OECD countries

10.1136/jmg.2004.030619

10.1136/jmg.34.10.798

10.1002/1098-2779(2000)6:2<142::AID-MRDD9>3.0.CO;2-H

10.1136/jmg.34.6.453

10.1002/(SICI)1096-8628(19981228)80:5<531::AID-AJMG20>3.0.CO;2-L

10.1097/00125817-200101000-00008