ArticleJournal of inherited metabolic disease2013
Endothelial function in children and adolescents with mucopolysaccharidosis.
Article in Journal of inherited metabolic disease, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 19 citations in OpenAlex.
- Arsenic exposure from drinking water and endothelial dysfunction in Bangladeshi adolescents.Environmental research · 2022Article
- Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders.Molecular genetics and metabolism reports · 2018Article
- Vascular function in asthmatic children and adolescents.Respiratory research · 2017Article
- Immune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type I.PloS one · 2016Article
- Human pulmonary artery endothelial cells in the model of mucopolysaccharidosis VI present a prohypertensive phenotype.Molecular genetics and metabolism reports · 2015Article
- Younger age is associated with lower reactive hyperemic index but not lower flow-mediated dilation among children and adolescents.Atherosclerosis · 2014Article
- Glycosaminoglycan metabolism defects and atherosclerosis: frequent association of endothelial dysfunction in patients with Mucopolysaccharidosis.Journal of inherited metabolic disease · 2014Article
- Carotid intima-media thickness is increased in patients with treated mucopolysaccharidosis types I and II, and correlates with arterial stiffness.Molecular genetics and metabolism · 2014Article
- Perioperative medullary complications in spinal and extra-spinal surgery in mucopolysaccharidosis: a case series of three patients.JIMD reports · 2014Article
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundAlthough coronary artery pathology is a prominent feature of mucopolysaccharidosis (MPS), it may be underestimated by coronary angiography because of its diffuse nature. It is also generally assumed that cardiovascular risk is increased in MPS and reduced following hematopoietic stem cell transplantation (HSCT) or enzyme replacement therapy (ERT), but this has never been formally evaluated. Non-invasive methods of assessing vascular endothelial function may provide a measure of cardiovascular risk in MPS. We evaluated endothelial function, using digital reactive hyperemia, in youth with MPS and in healthy controls.
methodsDigital reactive hyperemic index (RHI) was measured in 12 children and adolescents (age 10.3 ± 3.9 years old; 11 boys) with treated MPS and nine age- and gender-matched (11.4 ± 4.0; 8 boys) healthy controls. An independent t-test was used to compare RHI between individuals with MPS and controls.
resultsChildren and adolescents with MPS (MPS type II: N = 5; type I: N = 4; type VI: N = 3) whether treated by HSCT (N = 4) or ERT (N = 8) had significantly lower RHI compared to controls (MPS 1.22 ± 0.19 vs. controls 1.46 ± 0.32, p < 0.05).
conclusionThese preliminary findings suggest that children and adolescents with treated MPS have significantly poorer endothelial function when compared to healthy controls. Further investigation into the utility of endothelial function for risk stratification and the long term implications of reduced endothelial function in MPS is warranted.
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