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Damage of the Cardiovascular System in Lysosomal Storage Disease – Mucopolysaccharidosis

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source: Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego

year: 2021

authors: Pyvovar SM,Rudyk IS,Lozykc TV

summary/abstract:

The analysis of literature data reflecting the issues of the pathology of the cardiovascular system in mucopolysaccharidosis are presented. It was found out that heart and vessels damage is one of the cardinal signs of this pathology, often leading to death. Cardiac pathology is recorded in all types of mucopolysaccharidosis, but it is most significant for patients with three clinical variants of Hurler syndrome, Hunter, and Maroteaux-Lamy syndromes. Typical signs of damage to the cardiovascular system in mucopolysaccharidosis are thickening of the valves with the development of their dysfunction (while the severity of damage to the left-sided valves is more pronounced), myocardial hypertrophy, conduction disturbance, coronary artery disease, arterial hypertension. Many researchers emphasize the difficulties of clinical and functional examination of the cardiovascular system in patients with mucopolysaccharidosis, which is due to the presence of physical and intellectual limitations in patients, ands a gradual increase in symptoms. For the treatment of cardiovascular pathology at mucopolysaccharidosis, medical and surgical methods are used, including enzyme replacement therapy and stem cell transplantation.

organization: Government Institution "L.T. Malaya Therapy National Institute of the National Academy of Medical Sciences of Ukraine", Kharkiv, Ukraine.

DOI: 16;49(294):448-452

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