Document Detail


Klinefelter syndrome and associated fragile-X syndrome.
MedLine Citation:
PMID:  3585988     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
During screening of male individuals for Fragile-X syndrome in a residential facility for persons with mental retardation, the authors found a 21-year-old profoundly retarded man who displayed facial features and behaviour suggestive of Fragile-X syndrome. The chromosome analysis revealed 47,fra(X)(q27)fra(X)(q27)Y. His physically and intellectually normal sister had 14% of X chromosomes with a fragile site. Her two sons, who were subsequently examined, were found to have Fragile-X syndrome. Thus, the identification of Fragile-X syndrome in the proband during the screening process of a large institution led to the investigation of the proband's family and the subsequent diagnosis of Fragile-X syndrome in the proband's two nephews. The ascertainment of the two affected boys permitted prompt introduction of early intervention and special education services. Genetic counselling of other at-risk family members was carried out.
Authors:
S M Pueschel; M M O'Brien; T Padre-Mendoza
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of mental deficiency research     Volume:  31 ( Pt 1)     ISSN:  0022-264X     ISO Abbreviation:  J Ment Defic Res     Publication Date:  1987 Mar 
Date Detail:
Created Date:  1987-07-14     Completed Date:  1987-07-14     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0375401     Medline TA:  J Ment Defic Res     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  73-9     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Child, Preschool
Fragile X Syndrome / complications,  genetics*
Humans
Infant
Klinefelter Syndrome / complications*
Male
Pedigree
Sex Chromosome Aberrations / genetics*

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


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