Document Detail


Carotid intima-media thickness is increased in patients with mucopolysaccharidoses.
MedLine Citation:
PMID:  21963080     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: The feasibility of carotid artery intima-media thickness (C-IMT), an established cardiovascular disease marker, as a cardiac risk marker in mucopolysaccharidosis (MPS) patients was explored.
OBJECTIVES: To determine if C-IMT is abnormal in MPS versus unaffected controls, and if C-IMT correlates with coronary artery diameter in MPS.
MATERIAL AND METHODS: Measurements of C-IMT via neck ultrasound and echocardiographic parameters, including coronary artery diameters, were obtained from MPS and control patients, and compared.
RESULTS: Sixteen MPS subjects (6 MPS I, 6 MPS II, 2 MPS III, 1 MPS VI, 1 MPS VII) and sixteen age, ethnicity, and gender-matched controls were enrolled. Median MPS and control subject ages were 8.3±4.5 and 8.6±4.3 years, respectively (p=0.73). Mean MPS and control C-IMTs were 0.54±0.070 and 0.48±0.034 mm (p=0.0029). No differences in left main, left anterior descending, or right coronary artery diameters were seen between MPS and controls. A significant proportion of MPS subjects had mitral insufficiency (14/16; p=0.0002), aortic insufficiency (10/16; p=0.0021), and left ventricular dilatation (7/16, p=0.037) versus controls. C-IMT did not correlate significantly with age, height, weight, coronary measurements, or duration of treatment.
CONCLUSION: C-IMT in MPS patients is increased compared to matched controls, likely reflective of arterial intima-medial glycosaminoglycan accumulation. MPS subjects demonstrated a high percentage of left-sided valvular insufficiency and ventricular dilatation. Additional studies should be performed in MPS patients to determine if C-IMT correlates with arterial elasticity, biomarkers of vascular dysfunction, and higher risk of cardiovascular events.
Authors:
Raymond Y Wang; Kelly K Covault; Eileen M Halcrow; Audrey J Gardner; Xiaoling Cao; Robert L Newcomb; Richard D Dauben; Anthony C Chang
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2011-09-10
Journal Detail:
Title:  Molecular genetics and metabolism     Volume:  104     ISSN:  1096-7206     ISO Abbreviation:  Mol. Genet. Metab.     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-11-25     Completed Date:  2012-03-15     Revised Date:  2014-02-21    
Medline Journal Info:
Nlm Unique ID:  9805456     Medline TA:  Mol Genet Metab     Country:  United States    
Other Details:
Languages:  eng     Pagination:  592-6     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Inc. All rights reserved.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Biological Markers
Carotid Intima-Media Thickness*
Case-Control Studies
Child
Child, Preschool
Coronary Vessels / pathology,  ultrasonography
Female
Heart Valve Diseases / etiology,  ultrasonography
Humans
Male
Mucopolysaccharidoses / complications,  ultrasonography*
Young Adult
Grant Support
ID/Acronym/Agency:
UL1 RR031985/RR/NCRR NIH HHS; UL1 RR031985/RR/NCRR NIH HHS; UL1 RR031985-01/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Biological Markers
Comments/Corrections

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