Document Detail


Vascular dysfunction in glycogen storage disease type I.
MedLine Citation:
PMID:  19101686     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Angelina V Bernier; Catherine E Correia; Michael J Haller; Douglas W Theriaque; Jonathan J Shuster; David A Weinstein
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2008-12-21
Journal Detail:
Title:  The Journal of pediatrics     Volume:  154     ISSN:  1097-6833     ISO Abbreviation:  J. Pediatr.     Publication Date:  2009 Apr 
Date Detail:
Created Date:  2009-03-27     Completed Date:  2009-04-14     Revised Date:  2014-05-09    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  588-91     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
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:
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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