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Research on behavioural phenotypes: velocardiofacial syndrome (deletion 22q11.2)

Published online by Cambridge University Press:  01 June 2000

Paul P Wang
Affiliation:
Children's Seashore House, Philadelphia, PA, USA
Michael F Woodin
Affiliation:
Children's Seashore House, Philadelphia, PA, USA
Rachel Kreps-Falk
Affiliation:
Children's Seashore House, Philadelphia, PA, USA
Edward M Moss
Affiliation:
Children's Seashore House, Philadelphia, PA, USA
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Abstract

Recent molecular genetics research has established that most cases of velocardiofacial syndrome (VCF) and of DiGeorge syndrome result from a submicroscopic deletion at chromosome 22q11.2. The medical features of this disorder include hypocalcemia, immunodeficiency, cleft palate, subtle facial dysmorphism, ‘conotruncal’ cardiac malformations (e.g. truncus arteriosus, tetralogy of Fallot, interrupted aortic arch, and certain types of ventricular septal defects), and other congenital malformations. While none of these complications is found in all patients with the 22q11.2 microdeletion, each is sufficiently common to be considered part of the disorder's medical phenotype.

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Copyright
2000 Mac Keith Press

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