Research paperHyperlipidaemia does not impair vascular endothelial function in glycogen storage disease type 1a
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Glycogen storage disease type I patients with hyperlipidemia have no signs of early vascular dysfunction and premature atherosclerosis
2021, Nutrition, Metabolism and Cardiovascular DiseasesCitation Excerpt :Similar observations have been published in 3 previous studies and are summarized in Table 1. Lee et al. found no evidence of atherosclerosis in 6 adult GSD Ia patients using a non-invasive ultrasonographic method to assess endothelial function in the brachial arteries [12]. Ubels et al. studied 9 adolescent GSD Ia patients at a mean age of 22.7 ± 3.4 years and found comparable IMT segments in both groups with even thinner segments in the patient group [6].
Ischemic stroke in an adult with glycogen storage disease type i
2010, Journal of Clinical NeuroscienceCitation Excerpt :Marked hypertriglyceridemia and hypercholesterolemia from infancy are common features of GSD-I. Despite the high-risk lipid profile, GSD-I has not been shown definitively to cause premature atherosclerosis. Small studies found young adults with GSD-I to be no different from controls when evaluated for endothelium-dependent and independent arterial responses, blood pressure, ankle-brachial index, or intima-media thickness (IMT) of carotid and femoral arteries.3,4 Recently, the largest study to date assessed the development of atherosclerosis in GSD-I patients, with an average age of 23 years.5
An association among iron, copper, zinc, and selenium, and antioxidative status in dyslipidemic pediatric patients with glycogen storage disease types IA and III
2010, Journal of Trace Elements in Medicine and BiologyA monocentric pilot study of an antioxidative defense and hsCRP in pediatric patients with glycogen storage disease type IA and III
2009, Nutrition, Metabolism and Cardiovascular DiseasesCitation Excerpt :Predominantly, pediatric patients with hepatic glycogen storage disease (GSD) have a raised lipid profile expressed as elevated triglyceride concentrations or mixed hyperlipidemia. In contrast to other diseases associated with exposure to cardiovascular risk factors at an early age, such as familial hypercholesterolemia,2 it has been shown that glycogen storage disease type Ia (GSD Ia) patients do not show any signs of endothelial dysfunction or premature atherosclerosis.3,4 Glycogen storage disease type Ia (von Gierke disease; OMIM + 232,200) is caused by a deficiency in glucose-6-phosphatase, which catalyzes the terminal step in both glycogenolysis and gluconeogenesis in the liver, kidney, and small intestine.5
Vascular Dysfunction in Glycogen Storage Disease Type I
2009, Journal of Pediatrics