| Vascular dysfunction in glycogen storage disease type I. | |
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MedLine Citation:
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PMID: 19101686 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To determine cardiovascular disease risk in a larger cohort of patients with glycogen storage disease (GSD) I through the use of noninvasive measures of arterial function and anatomy. STUDY DESIGN: Carotid intima media thickness (IMT), radial artery tonometry, and brachial artery reactivity were performed in 28 patients with GSD I (13F/15M, mean age 23 years) and 23 control subjects (19F/4M, mean age 23 years). RESULTS: The primary outcome measure, mean left distal IMT was greater in the GSD cohort (0.500+/-0.055 mm) than in the control group (0.457+/-0.039 mm) (P= .002, adjusted for age, sex, and body mass index). Mean augmentation index measured by radial artery tonometry was higher in the GSD cohort (16.4%+/-14.0%) than in the control group (2.4%+/-8.7%) (P< .001). No significant difference was observed between mean brachial artery reactivity in the GSD cohort (6.3%+/-4.9% change) versus control subjects (6.6%+/-5.1% change) (P= .46). CONCLUSIONS: GSD I is associated with arterial dysfunction evident by increased IMT and augmentation index. Patients with GSD I may be at increased risk for cardiovascular disease. |
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Authors:
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Angelina V Bernier; Catherine E Correia; Michael J Haller; Douglas W Theriaque; Jonathan J Shuster; David A Weinstein |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2008-12-21 |
Journal Detail:
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Title: The Journal of pediatrics Volume: 154 ISSN: 1097-6833 ISO Abbreviation: J. Pediatr. Publication Date: 2009 Apr |
Date Detail:
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Created Date: 2009-03-27 Completed Date: 2009-04-14 Revised Date: 2013-04-18 |
Medline Journal Info:
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Nlm Unique ID: 0375410 Medline TA: J Pediatr Country: United States |
Other Details:
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Languages: eng Pagination: 588-91 Citation Subset: AIM; IM |
Affiliation:
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Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL 32610-0296, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Atherosclerosis / epidemiology, pathology* Brachial Artery / pathology Carotid Artery, Common / pathology Case-Control Studies Cohort Studies Endothelium, Vascular / pathology* Female Florida / epidemiology Glycogen Storage Disease Type I / epidemiology, pathology* Humans Male Manometry Radial Artery / pathology Risk Tunica Intima / pathology |
| Grant Support | |
ID/Acronym/Agency:
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K23 RR 017560/RR/NCRR NIH HHS; M01 RR 00082/RR/NCRR NIH HHS; M01 RR000082/RR/NCRR NIH HHS |
| Comments/Corrections | |
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