Document Detail


Preconceptional and prenatal screening for fragile X syndrome: experience with 40,000 tests.
MedLine Citation:
PMID:  17705235     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: To determine the carrier frequency of fragile X syndrome, and the rate of expansion from premutation (PM) carrier to full mutation (FM) fetus. METHODS: Results were analyzed on women with no family history of fragile X syndrome, or who were PM/FM carriers, who were tested between January 1994 and June 2004. PM was defined 55-199 repeats, FM above 200. RESULTS: Out of 40 079 women screened, 5 FM and 255 PM carriers were detected. There was no significant difference in carrier frequency between those with versus those without family history of mental retardation or developmental abnormalities: 1 in 128 (28/3596) versus 1 in 157 (232/36 483). However, the median of repeats differed significantly: 58 and 66 repeats, respectively, (P < 0.0001). Invasive prenatal diagnosis was carried out in 370 pregnancies (7 FM and 363 PM). Thirty FM fetuses were detected. There was a lower expansion rate in cases without a family history: 10% (17/169 PMs) compared to 50% (11/22 PMs) in those with a history, but this could be accounted for by the difference in allele size. CONCLUSION: There is now sufficient information on screening parameters and prenatal diagnosis of fragile X syndrome to offer testing to women of reproductive age.
Authors:
Michal Berkenstadt; Liat Ries-Levavi; Howard Cuckle; Leah Peleg; Gad Barkai
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Publication Detail:
Type:  Evaluation Studies; Journal Article    
Journal Detail:
Title:  Prenatal diagnosis     Volume:  27     ISSN:  0197-3851     ISO Abbreviation:  Prenat. Diagn.     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2007-10-29     Completed Date:  2008-02-11     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8106540     Medline TA:  Prenat Diagn     Country:  England    
Other Details:
Languages:  eng     Pagination:  991-4     Citation Subset:  IM    
Affiliation:
Danek-Gertner Institute of Human Genetics, Sheba Medical Center, Tel-Hashomer, Israel. mberken@sheba.health.gov.il
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MeSH Terms
Descriptor/Qualifier:
Autistic Disorder / diagnosis,  genetics
Female
Fetal Diseases / diagnosis,  genetics
Fragile X Syndrome / diagnosis*,  genetics
Gene Frequency
Genetic Testing*
Heterozygote Detection
Humans
Male
Mental Retardation / diagnosis,  genetics
Pregnancy
Preimplantation Diagnosis / methods*
Prenatal Diagnosis / methods*
Retrospective Studies

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


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