title: Carotid intima-media thickness is increased in patients with mucopolysaccharidoses.
abstract: 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.
  
  
  OBJECTIVE
  To determine if C-IMT is abnormal in MPS versus unaffected controls, and if C-IMT correlates with coronary artery diameter in MPS.
  
  
  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.
  
  
  CONCLUSIONS
  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.affiliation: Division of Metabolic Disorders, Pediatric Subspecialty Faculty, CHOC Children's, Orange, CA, USA. rawang@choc.org
authors.forename: Raymond Y
authors.initials: RY
authors.lastname: Wang
authors.forename: Kelly K
authors.initials: KK
authors.lastname: Covault
authors.forename: Eileen M
authors.initials: EM
authors.lastname: Halcrow
authors.forename: Audrey J
authors.initials: AJ
authors.lastname: Gardner
authors.forename: Xiaoling
authors.initials: X
authors.lastname: Cao
authors.forename: Robert L
authors.initials: RL
authors.lastname: Newcomb
authors.forename: Richard D
authors.initials: RD
authors.lastname: Dauben
authors.forename: Anthony C
authors.initials: AC
authors.lastname: Chang
mesh_terms: D000293:Adolescent; D015415:Biomarkers; D059168:Carotid Intima-Media Thickness; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003331:Coronary Vessels / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006349:Heart Valve Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications / Q000000981:diagnostic imaging*; D055815:Young Adult
chemical_list: D015415:Biomarkers