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Some aspects of pathophysiology of pulmonary arterial hypertension and its epidemiology in the Czech Republic
Jansa, Pavel ; Aschermann, Michael (advisor) ; Widimský, Jiří (referee) ; Čerbák, Roman (referee)
1 Univerzita Karlova v Praze 1. lékařská fakulta Některé aspekty patofyziologie plicní arteriální hypertenze a její výskyt v České republice Some aspects of pathophysiology of pulmonary arterial hypertension and its epidemiology in the Czech Republic MUDr. Pavel Jansa Praha 2011 2 Abstract Pulmonary arterial hypertension (PAH) is a group of diseases characterized by a progressive increase of resistance and pressure in pulmonary vascular bed. In all types of PAH the same four pathological processes are reported: vasoconstriction, inflammation, thrombosis and remodelling. The genetic background is essential for the development of PAH. We aimed to investigate the role of polymorphisms of endothelial nitric oxide synthase (eNOS) genes in PAH. We studied 142 PAH patients and 189 healthy subjects. We examined 3 polymorphisms of the eNOS gene, including the Glu298Asp polymorphism, 27-base pair (bp) variable numbers of tandem repeats (VNTR) and -786 T/C promoter gene polymorphism. Prevalence of 27-bp VNTR allele A was higher in patients with PAH compared with healthy controls. Patients with PAH associated with connective tissue diseases had higher prevalence of AA genotype compared with other PAH subgroups. The Glu298Asp polymorphism and -786 T/C polymorphism are not associated with PAH. Thrombotic arteriopathy is...
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The role of metabolism and immune system in pulmonary hypertension
Křivonosková, Monika ; Plecitá, Lydie (advisor) ; Vašek, Daniel (referee)
Pulmonary arterial hypertension is a low-incidence, fatal, disease that affects the pulmonary vessels, which remodel, thicken and gradually reduce lumen. During this signaling molecules are excreted from endothelial cells, which act vasoconstrictively and proliferatively on smooth muscle cells. There is also a reduction in the production of vaodilators, which again has a vasoconstrictive effect. Accumulation of immune cells in adventitia promotes smooth muscle cell proliferation, migration and differentiation of fibroblasts into myofibroblasts, which causes changes in the composition of the extracellular matrix. Changes in metabolism within all artery layers related to the transition to glycolysis and increased glucose uptake promote cell proliferation and modulate signaling leading to infiltration and activation of immune cells resulting in artery remodeling.
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Some aspects of pathophysiology of pulmonary arterial hypertension and its epidemiology in the Czech Republic
Jansa, Pavel ; Aschermann, Michael (advisor) ; Widimský, Jiří (referee) ; Čerbák, Roman (referee)
1 Univerzita Karlova v Praze 1. lékařská fakulta Některé aspekty patofyziologie plicní arteriální hypertenze a její výskyt v České republice Some aspects of pathophysiology of pulmonary arterial hypertension and its epidemiology in the Czech Republic MUDr. Pavel Jansa Praha 2011 2 Abstract Pulmonary arterial hypertension (PAH) is a group of diseases characterized by a progressive increase of resistance and pressure in pulmonary vascular bed. In all types of PAH the same four pathological processes are reported: vasoconstriction, inflammation, thrombosis and remodelling. The genetic background is essential for the development of PAH. We aimed to investigate the role of polymorphisms of endothelial nitric oxide synthase (eNOS) genes in PAH. We studied 142 PAH patients and 189 healthy subjects. We examined 3 polymorphisms of the eNOS gene, including the Glu298Asp polymorphism, 27-base pair (bp) variable numbers of tandem repeats (VNTR) and -786 T/C promoter gene polymorphism. Prevalence of 27-bp VNTR allele A was higher in patients with PAH compared with healthy controls. Patients with PAH associated with connective tissue diseases had higher prevalence of AA genotype compared with other PAH subgroups. The Glu298Asp polymorphism and -786 T/C polymorphism are not associated with PAH. Thrombotic arteriopathy is...
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