Document Detail


The fragile X syndrome: history, diagnosis, and treatment.
MedLine Citation:
PMID:  6348096     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The fragile X (marker X) syndrome is a relatively common form of X-linked mental retardation. The karyotypic hallmark of the syndrome consists of a pronounced constriction near the terminus of the long arm of the X chromosome (fragile site), expressed in vitro only under conditions where thymidylate production is blocked (reduced folate levels and/or addition of methotrexate or 5-fluorodeoxyuridine). Clinical features associated with the syndrome include macroorchidism, large or prominent ears, and significant emotional dysfunction. In the present review, historical, diagnostic, biochemical, and clinical aspects of this syndrome are presented. Recent anecdotal reports of clinical improvement following high dose folic acid treatment will be discussed.
Authors:
R J Hagerman; P McBogg; P J Hagerman
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Publication Detail:
Type:  Historical Article; Journal Article; Review    
Journal Detail:
Title:  Journal of developmental and behavioral pediatrics : JDBP     Volume:  4     ISSN:  0196-206X     ISO Abbreviation:  J Dev Behav Pediatr     Publication Date:  1983 Jun 
Date Detail:
Created Date:  1983-09-23     Completed Date:  1983-09-23     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8006933     Medline TA:  J Dev Behav Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  122-30     Citation Subset:  IM; Q    
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MeSH Terms
Descriptor/Qualifier:
Australia
Canada
Chromosome Fragile Sites
Chromosome Fragility
Folic Acid / therapeutic use
Fragile X Syndrome / diagnosis*,  drug therapy,  history
Genetic Markers
Heterozygote
History, 19th Century
History, 20th Century
Humans
Prenatal Diagnosis
Sex Chromosome Aberrations / diagnosis*
United States
Chemical
Reg. No./Substance:
0/Genetic Markers; 59-30-3/Folic Acid

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


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