Characteristics of lipid metabolism and the cardiovascular system in glycogenosis types I and III

• Main Authors: N V Polenova, T V Strokova, A V Starodubova
• Format: Article
• Language: Russian
• Published: "Consilium Medicum" Publishing house 2017-08-01
• Series: Терапевтический архив
• Subjects: glycogen storage disease; dyslipidemia; subclinical atherosclerosis; rare (orphan) diseases; lipid paradox
• Online Access: https://ter-arkhiv.ru/0040-3660/article/viewFile/32324/pdf

Glycogen storage disease (GSD) is an inherited metabolic disorder characterized by early childhood lipid metabolic disturbances with potentially proatherogenic effects. The review outlines the characteristics of impaired lipid composition and other changes in the cardiovascular system in GSD types I and III. It analyzes the factors enabling and inhibiting the development of atherosclerosis in patients with GSD. The review describes the paradox of vascular resistance to the development of early atherosclerosis despite the proatherogenic composition of lipids in the patients of this group.