| Clinical presentation and echocardiographic findings of Thai patients with Marfan syndrome. | |
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MedLine Citation:
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PMID: 20364556 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: Marfan syndrome is an inherited disorder with systemic connective tissue involvement. The condition is clinically diverse and extremely pleiotropic. Though several articles had been published, no data from Thai individuals has ever been reported. Here we presented clinical manifestations and echocardiographic findings of Thai patients with Marfan syndrome. MATERIAL AND METHOD: Clinical and echocardiographic data were collected from series of Thai index cases with diagnosis of Marfan syndrome at Siriraj Hospital between 1995 and 2009. Demographic data, clinical presentations, and echocardiographic data were reviewed and analyzed. RESULTS: Fifty individuals and thirty echocardiograms were identified. Thirty eight individuals (76%) fulfilled clinical criteria for definite Marfan syndrome and twelve individuals (24%) were incomplete Marfan syndrome. The majority of those individuals had significant organ involvement including dilatation of ascending aorta (78%), ectopia lentis (54%), and skeletal involvement (96%). CONCLUSIONS: In summary, this is the first report of clinical manifestation and echocardiographic findings of Thai patients with Marfan syndrome. |
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Authors:
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Manop Pithukpakorn; Nithima Chaowalit |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of the Medical Association of Thailand = Chotmaihet thangphaet Volume: 93 Suppl 1 ISSN: 0125-2208 ISO Abbreviation: J Med Assoc Thai Publication Date: 2010 Jan |
Date Detail:
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Created Date: 2010-04-06 Completed Date: 2010-05-04 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7507216 Medline TA: J Med Assoc Thai Country: Thailand |
Other Details:
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Languages: eng Pagination: S43-7 Citation Subset: IM |
Affiliation:
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Division of Medical Genetics, Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aortic Aneurysm / diagnosis, genetics* Asian Continental Ancestry Group / genetics Echocardiography Ectopia Lentis / diagnosis, genetics* Female Humans Male Marfan Syndrome / diagnosis, genetics* Middle Aged Pedigree Thailand Young Adult |
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