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Int. J. Mol. Sci. 2017, 18(3), 637; doi:10.3390/ijms18030637

The Carotid Intima-Media Thickness and Arterial Stiffness of Pediatric Mucopolysaccharidosis Patients Are Increased Compared to Both Pediatric and Adult Controls

1
Division of Metabolic Disorders, CHOC Children’s Specialists, Orange, CA 92868, USA
2
Department of Pediatrics, University of California-Irvine School of Medicine, Orange, CA 92868, USA
3
Division of Biostatistics, University of Minnesota School of Public Health, Minneapolis, MN 55455, USA
4
School of Kinesiology, University of Minnesota, Minneapolis, MN 55455, USA
5
Department of Pediatrics, University of Minnesota Medical School, Minneapolis, MN 55455, USA
*
Author to whom correspondence should be addressed.
Academic Editor: Ritva Tikkanen
Received: 30 January 2017 / Revised: 25 February 2017 / Accepted: 8 March 2017 / Published: 15 March 2017
View Full-Text   |   Download PDF [837 KB, uploaded 15 March 2017]   |  

Abstract

Treatments for mucopolysaccharidoses (MPSs) have increased longevity, but cardiovascular disease causes mortality in a significant percentage of survivors. Markers must be developed to predict MPS cardiac risk and monitor efficacy of investigational therapies.MPS patients underwent carotid artery ultrasonography from which carotid intima-media thickness (cIMT) and three measures of arterial stiffness were calculated: carotid artery distensibility (cCSD), compliance (cCSC), and incremental elastic modulus (cIEM). MPS carotid measurements were compared to corresponding data from pediatric and adult healthy cohorts. 33 MPS patients (17 MPS I, 9 MPS II, 4 MPS IIIA, and 3 MPS VI; mean age 12.5 ± 4.7 years), 560 pediatric controls (age 13.1 ± 4.0 years), and 554 adult controls (age 39.2 ± 2.2 years) were studied. Age and sex-adjusted aggregate MPS cIMT (0.56 ± 0.05 mm) was significantly greater than both pediatric (+0.12 mm; 95% CI +0.10 to +0.14 mm) and adult (+0.10 mm; 95% CI +0.06 to +0.14 mm) control cohorts; similar findings were observed for all MPS subtypes. Mean MPS cIMT approximated the 80th percentile of the adult cohort cIMT. MPS patients also demonstrated significantly increased adjusted arterial stiffness measurements, evidenced by reduced cCSD, cCSC, and increased cIEM, compared to pediatric and adult control cohorts. Regardless of treatment, MPS patients demonstrate increased cIMT and arterial stiffness compared to healthy pediatric and adult controls. These data suggest that relatively young MPS patients demonstrate a “structural vascular age” of at least 40 years old. View Full-Text
Keywords: mucopolysaccharidosis; vascular; intima; media; thickness; stiffness; outcome; treatment; lysosomal; structure; function mucopolysaccharidosis; vascular; intima; media; thickness; stiffness; outcome; treatment; lysosomal; structure; function
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MDPI and ACS Style

Wang, R.Y.; Rudser, K.D.; Dengel, D.R.; Braunlin, E.A.; Steinberger, J.; Jacobs, D.R.; Sinaiko, A.R.; Kelly, A.S. The Carotid Intima-Media Thickness and Arterial Stiffness of Pediatric Mucopolysaccharidosis Patients Are Increased Compared to Both Pediatric and Adult Controls. Int. J. Mol. Sci. 2017, 18, 637.

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