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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
The mild form of mucopolysaccharidosis type I (Scheie syndrome) is associated with increased ascending aortic stiffness
Heart and Vessels, Volume 23, No. 2, Year 2008
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Description
Mucopolysaccharidosis type I (MPS IS) is a rare autosomal recessive disease caused by a deficiency of the lysosomal enzyme α-iduronidase, which is involved in the degradation of sulfated glycosaminoglycans (GAGs). The deficiency results in the intra-and pericellular accumulation of the GAGs heparan sulfate and dermatan sulfate. Eight adult patients with typical features of MPS IS aged 31.5 ± 6.8 years (five men) were included and compared to age-and gender-matched controls. With transthoracic echocardiography, cyclic ascending aortic diameter changes were measured and ascending aortic elastic properties were calculated to characterize aortic elasticity. In MPS IS patients, aortic stiffness index was significantly increased (23.1 ± 10.4 vs 3.9 ± 1.5, P < 0.001), while aortic distensibility was significantly decreased (1.6 ± 0.8 vs 1.6 ± 1.9 Ca2/dynes 10-6, P < 0.001) compared to age-and sex-matched controls. The results of the present study demonstrate that in addition to the known cardiac complications, MPS IS patients have an impairment of ascending aortic elasticity. Further follow-up studies are needed to examine arterial elasticity using other methods in this patient population, and to detect possible effects of enzyme replacement therapy. © Springer Japan 2008.
Authors & Co-Authors
Nemes, Attila
Netherlands, Rotterdam
Erasmus Mc
Hungary, Szeged
Szegedi Tudományegyetem Szte
Timmermans, R. G.M.
Netherlands, Rotterdam
Erasmus Mc
Wilson, John H.Paul
Netherlands, Rotterdam
Erasmus Mc
Soliman, O. I.I.
Netherlands, Rotterdam
Erasmus Mc
Egypt, Cairo
Al-azhar University
Krenning, Boudewijn J.
Netherlands, Rotterdam
Erasmus Mc
Ten Cate, Folkert J.
Netherlands, Rotterdam
Erasmus Mc
Geleijnse, Marcel L.
Netherlands, Rotterdam
Erasmus Mc
Statistics
Citations: 20
Authors: 7
Affiliations: 3
Identifiers
Doi:
10.1007/s00380-007-1013-x
ISSN:
09108327
Research Areas
Health System And Policy
Noncommunicable Diseases
Study Design
Cross Sectional Study
Cohort Study
Participants Gender
Male