Document Detail

High prevalence of vertebral artery tortuosity of Loeys-Dietz syndrome in comparison with Marfan syndrome.
MedLine Citation:
PMID:  20512544     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Loeys-Dietz syndrome (LDS) is a connective tissue disease caused by mutations in the genes encoding the transforming growth factor-beta receptor (TGFBR). LDS is associated with aneurysms or dissections of the aorta similar to Marfan syndrome (MFS) as well as arterial tortuosity and aneurysms in the peripheral arteries. The purpose of this study was to evaluate the arterial diseases of LDS to differentiate it from MFS. MATERIALS AND METHODS: A total of 10 LDS patients with an identified mutation in TGFBR (6 male, 4 female; mean age 36.3 years) and 20 MFS patients with an identified mutation in fibrilin-1 who were age- and sex-matched to the LDS subjects (12 male, 8 female; mean age 37.1 years) were reviewed. The prevalence of vertebral arterial tortuosity (VAT) and peripheral aneurysm (PAN) was studied using computed tomography angiography. RESULTS: In all, 9 of the 10 LDS patients had VAT, and five PANs were observed in 3 patients. In contrast, 8 (40%) of the MFS patients had VAT, and 1 patient had a PAN. LDS had a higher prevalence of VAT (P = 0.017) by Fisher's exact test. CONCLUSION: The VAT was highly prevalent among LDS patients. Thus, the presence of VAT has the potential to differentiate LDS from MFS.
Atsushi K Kono; Masahiro Higashi; Hiroko Morisaki; Takayuki Morisaki; Yoshiaki Tsutsumi; Koichi Akutsu; Hiroaki Naito; Kazuro Sugimura
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Publication Detail:
Type:  Comparative Study; Journal Article     Date:  2010-05-29
Journal Detail:
Title:  Japanese journal of radiology     Volume:  28     ISSN:  1867-108X     ISO Abbreviation:  Jpn J Radiol     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-05-31     Completed Date:  2010-09-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101490689     Medline TA:  Jpn J Radiol     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  273-7     Citation Subset:  IM    
Department of Radiology and Nuclear Medicine, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, 565-8565, Japan.
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MeSH Terms
Aneurysm / pathology,  radiography*
Chi-Square Distribution
Fibrin / genetics
Loeys-Dietz Syndrome / genetics,  pathology,  radiography*
Marfan Syndrome / genetics,  pathology,  radiography*
Middle Aged
Radiographic Image Interpretation, Computer-Assisted
Receptors, Transforming Growth Factor beta / genetics
Tomography, X-Ray Computed*
Vertebral Artery / pathology,  radiography*
Reg. No./Substance:
0/Receptors, Transforming Growth Factor beta; 9001-31-4/Fibrin

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