Document Detail


Fragile X syndrome is less common than previously estimated.
MedLine Citation:
PMID:  9032640     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In 1986, a population study of school children in the city of Coventry gave an overall prevalence in males and females for fragile X syndrome of 1/952. The 29 children diagnosed as having fragile X syndrome in this study have been re-evaluated with molecular diagnostic techniques. Eighteen of the original 29 children have been found not to have the expansion of the FMR1 gene associated with fragile X syndrome. Revised prevalence figures have been calculated giving rise to an overall prevalence figure of 1/2720 (range 1/2198-1/3089). If the four children lost to follow up are also assumed not to have the fragile X syndrome, the revised prevalence figure was 1/5714 (range 1/4762-1/6349). Clinical review of boys with severe mental retardation from this and a subsidiary study show that the clinical features of head circumference greater than the 50th centile, testicular volume greater than the 50th centile, and IQ between 35 and 70 remain helpful in distinguishing boys with fragile X syndrome from those who have non-specific mental retardation.
Authors:
J E Morton; S Bundey; T P Webb; F MacDonald; P M Rindl; S Bullock
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of medical genetics     Volume:  34     ISSN:  0022-2593     ISO Abbreviation:  J. Med. Genet.     Publication Date:  1997 Jan 
Date Detail:
Created Date:  1997-04-10     Completed Date:  1997-04-10     Revised Date:  2010-09-13    
Medline Journal Info:
Nlm Unique ID:  2985087R     Medline TA:  J Med Genet     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  1-5     Citation Subset:  IM    
Affiliation:
Clinical Genetics Unit, Birmingham Women's Hospital, Edgbaston, UK.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Blotting, Southern
Child
Cytogenetics
Diagnosis, Differential
Female
Fragile X Syndrome / diagnosis,  epidemiology*
Genetic Testing*
Great Britain / epidemiology
Humans
In Situ Hybridization
Intelligence Tests
Male
Mental Retardation / diagnosis,  etiology
Prevalence
Sex Chromosome Aberrations
X Chromosome*
Comments/Corrections

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