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PMID: 8230158 Published · ppublish English Case Reports Journal Article

Velocardiofacial syndrome in a mother and daughter: variability of the clinical phenotype.

Journal of medical genetics ·Vol. 30 ·No. 10 ·1993-10-00 ·Pages 825-7

Holder SE, Winter RM, Kamath S, Scambler PJ

Abstract

We report a mother and daughter with features of the velocardiofacial (VCF) syndrome and monosomy for 22q11 identified using molecular techniques. The mother had surgery as a child for a cleft palate and a congenital heart defect, and her facial features were consistent with the diagnosis. The daughter had developmental delay, absent speech, scoliosis, and similar facial features, but no cleft palate or congenital heart defect. These cases illustrate the considerable intrafamilial variability of the phenotype of VCF syndrome. The clinical and molecular diagnosis of this syndrome is discussed. The phenotypic variability of the VCF syndrome means that many cases may be undiagnosed.

MeSH Terms
Abnormalities, Multiple/genetics,pathology Child Chromosome Deletion Cleft Palate/genetics Face/abnormalities Female Gene Deletion Genetic Variation Heart Defects, Congenital/genetics,pathology Humans Monosomy Neurocognitive Disorders/genetics Phenotype Syndrome Velopharyngeal Insufficiency/genetics,pathology
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Holder S E
Mothercare Unit of Clinical Genetics and Fetal Medicine, Institute of Child Health, London, UK.
Winter R M
Kamath S
Scambler P J
References (10)
10 references, click to expand
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Article Info
Journal
Journal of medical genetics
Abbr.
J Med Genet
ISSN
0022-2593
Published
1993-10-00
Pages
825-7
Language
English
Region
England
NLM ID
2985087R
PMCID
PMC1016563
Subset
IM
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