Carotid intima-media thickness is increased in patients with mucopolysaccharidoses |
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Authors: | Wang Raymond Y Covault Kelly K Halcrow Eileen M Gardner Audrey J Cao Xiaoling Newcomb Robert L Dauben Richard D Chang Anthony C |
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Affiliation: | a Division of Metabolic Disorders, Pediatric Subspecialty Faculty, CHOC Children's, Orange, CA, USAb Department of Pediatrics, University of California-Irvine School of Medicine, Irvine, CA, USAc Vascular Laboratory, Division of Cardiovascular Services, St Joseph's Hospital, Orange, CA, USAd Division of Cardiology, Pediatric Subspecialty Faculty, CHOC Children's, Orange, CA, USAe Institute for Clinical and Translational Science, University of California-Irvine, Irvine, CA, USAf Division of Neurology, St Joseph's Hospital, Orange, CA, USAg Heart Institute, CHOC Children's, Orange, CA, USA |
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Abstract: | BackgroundThe 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.ObjectivesTo determine if C-IMT is abnormal in MPS versus unaffected controls, and if C-IMT correlates with coronary artery diameter in MPS.Material and methodsMeasurements of C-IMT via neck ultrasound and echocardiographic parameters, including coronary artery diameters, were obtained from MPS and control patients, and compared.ResultsSixteen 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.ConclusionC-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. |
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Keywords: | C-IMT, carotid intima-media thickness MPS, mucopolysaccharidosis GAG, glycosaminoglycan ERT, enzyme replacement therapy HSCT, hematopoietic stem cell transplant CHOC, Children's Hospital of Orange County IVSd, end-diastolic intraventricular septal thickness LVPWd, end-diastolic left ventricular posterior wall thickness IDU, α-iduronidase IDS, iduronate sulfatase SGSH, N-sulfoglucosamine sulfhydrolase ARSB, arylsulfatase B GUSB, β-glucuronidase |
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