[Pulmonary arterial hypertension in patients with hereditary hemorrhagic telangiectasia (Osler-Rendu disease)].
Pulmonary arterial pressure was studied using a noninvasive method in 23 patients with hereditary hemorrhagic telangiectasia (HHT) without clinical and x-ray signs of arterial-venous shunting in the lungs. It was established that pulmonary arterial hypertension was typical of HHT patients. A significant decrease in the volume and frequency of hemorrhages in 12 of 17 HHT patients was combined with a statistically significant decrease in pulmonary arterial pressure as a result of HBO therapy. It was assumed that pulmonary microcirculatory disorders played an important role in HHT pathogenesis and were expressed in metabolic derangement of a number of biologically active substances in the lungs resulting in the development of telangiectasia.
D000328:Adult; D000368:Aged; D001165:Arteriovenous Malformations / Q000150:complications; D001794:Blood Pressure; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006931:Hyperbaric Oxygenation; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008168:Lung / Q000098:blood supply*; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities / Q000503:physiopathology*; D011667:Pulmonary Veins / Q000002:abnormalities; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy
Legochnaia arterial'naia gipertenziia u bol'nykh nasledstvennoĭ gemorragicheskoĭ teleangiektazieĭ (bolezn'iu Randiu-Oslera).