Vascular Dysfunction in Glycogen Storage Disease Type I

作者: Angelina V. Bernier , Catherine E. Correia , Michael J. Haller , Douglas W. Theriaque , Jonathan J. Shuster

DOI: 10.1016/J.JPEDS.2008.10.048

关键词:

摘要: Objective To determine cardiovascular disease risk in a larger cohort of patients with glycogen storage (GSD) I through the use noninvasive measures arterial function and anatomy. Study design Carotid intima media thickness (IMT), radial artery tonometry, brachial reactivity were performed 28 GSD (13F/15M, mean age 23 years) control subjects (19F/4M, years). Results The primary outcome measure, left distal IMT was greater (0.500 ± 0.055 mm) than group (0.457 0.039 ( P = .002, adjusted for age, sex, body mass index). Mean augmentation index measured by tonometry higher (16.4% 14.0%) (2.4% 8.7%) .46). Conclusions is associated dysfunction evident increased index. Patients may be at disease.

参考文章(21)
Guilia Russo, Jane A Leopold, Joseph Loscalzo, Vasoactive substances: Nitric oxide and endothelial dysfunction in atherosclerosis☆ Vascular Pharmacology. ,vol. 38, pp. 259- 269 ,(2002) , 10.1016/S1537-1891(02)00250-1
O.N. Elpeleg, The Molecular Background of Glycogen Metabolism Disorders Journal of Pediatric Endocrinology and Metabolism. ,vol. 12, pp. 363- 380 ,(1999) , 10.1515/JPEM.1999.12.3.363
C. MÜHlhausen, R. Schneppenheim, U. Budde, M. Merkel, N. Muschol, K. Ullrich, R. Santer, Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia. Journal of Inherited Metabolic Disease. ,vol. 28, pp. 945- 950 ,(2005) , 10.1007/S10545-005-0184-9
Giuseppe Lippi, Martina Montagnana, Massimo Franchini, Emmanuel J. Favaloro, Giovanni Targher, The paradoxical relationship between serum uric acid and cardiovascular disease Clinica Chimica Acta. ,vol. 392, pp. 1- 7 ,(2008) , 10.1016/J.CCA.2008.02.024
Emile Levy, Jacques Letarte, Guy Lepage, Louise Thibault, Claude C. Roy, Plasma and lipoprotein fatty acid composition in glycogen storage disease type I. Lipids. ,vol. 22, pp. 381- 385 ,(1987) , 10.1007/BF02537265
Birgit Wittenstein, Marcus Klein, Barbara Finckh, Kurt Ullrich, Alfried Kohlschütter, Plasma antioxidants in pediatric patients with glycogen storage disease, diabetes mellitus, and hypercholesterolemia Free Radical Biology and Medicine. ,vol. 33, pp. 103- 110 ,(2002) , 10.1016/S0891-5849(02)00863-8
Catherine Boyer-Neumann, Dominique Meyer, Michel Odievre, Philippe Labrune, Anne Marfaing-Koka, Martine Wolf, Increased levels of hemostatic proteins are independent of inflammation in glycogen storage disease type Ia. Journal of Pediatric Gastroenterology and Nutrition. ,vol. 37, pp. 566- 570 ,(2003) , 10.1097/00005176-200311000-00011
Andrew D. Nguyen, Chi-Jiunn Pan, David A. Weinstein, Janice Y. Chou, Increased scavenger receptor class B type I-mediated cellular cholesterol efflux and antioxidant capacity in the sera of glycogen storage disease type Ia patients. Molecular Genetics and Metabolism. ,vol. 89, pp. 233- 238 ,(2006) , 10.1016/J.YMGME.2006.05.002
Lawson Mcdonald, Marion Edgill, CHANGES IN COAGULABILITY OF THE BLOOD DURING VARIOUS PHASES OF ISCHÆMIC HEART-DISEASE The Lancet. ,vol. 273, pp. 1115- 1118 ,(1959) , 10.1016/S0140-6736(59)90706-8