Document Detail


The fragile X in Sicily: an epidemiological survey.
MedLine Citation:
PMID:  3177477     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We have studied a group of 349 institutionalized propositi with mental retardation, and found 12 fra(X)-positive cases among 155 males (7.7%) and 8 fra(X)-positive cases among 194 females (4.1%). The males had characteristic manifestations of the Martin-Bell syndrome. Another 7 males, who were initially considered "borderline", having expression of fra(X) less than 4% and a non-characteristic phenotype, were eventually considered negative. Among 5,624 patients (2,764 males and 2,860 females) that were admitted to the Pediatric Department of the University of Catania during the period July 1986 - June 1987, 210 (120 males and 90 females) had mental retardation. Of these, 75 were analyzed for the presence of fra(X) (q27.3); 5 males (0.18% of all males) and 2 females (0.07% of all females) were fra(X)-positive. The males had the Martin Bell syndrome phenotype. The presence of fra(X) (q27) was confirmed in another 4 male propositi that were referred to our outpatient services with a clinical diagnosis of Martin-Bell syndrome.
Authors:
G Neri; S Sanfilippo; L Pavone; F Mollica; I Barberi; L Giuffrè; C Romano; T Mattina; M Cammarata; M G Ragusa
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  30     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:    1988 May-Jun
Date Detail:
Created Date:  1988-11-08     Completed Date:  1988-11-08     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7708900     Medline TA:  Am J Med Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  665-72     Citation Subset:  IM    
Affiliation:
Istituto di Genetica Umana, UCSC, Roma, Italy.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Child
Child, Preschool
Epidemiologic Methods
Female
Fragile X Syndrome / epidemiology*,  genetics
Humans
Italy
Male
Phenotype
Sex Chromosome Aberrations / epidemiology*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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