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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Terapevticheskii arkhiv</journal-id><journal-title-group><journal-title xml:lang="en">Terapevticheskii arkhiv</journal-title><trans-title-group xml:lang="ru"><trans-title>Терапевтический архив</trans-title></trans-title-group></journal-title-group><issn publication-format="print">0040-3660</issn><issn publication-format="electronic">2309-5342</issn><publisher><publisher-name xml:lang="en">LLC Obyedinennaya Redaktsiya</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">31818</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Editorial article</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Передовая статья</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Approaches to therapy for pulmonary hypertension: Role of the endothelin receptor antagonist bosentan</article-title><trans-title-group xml:lang="ru"><trans-title>Подходы к терапии легочной артериальной гипертензии: роль антагониста рецепторов эндотелина бозентана</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Avdeev</surname><given-names>S N</given-names></name><name xml:lang="ru"><surname>Авдеев</surname><given-names>С Н</given-names></name></name-alternatives><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en"></institution></aff><aff><institution xml:lang="ru">«НИИ пульмонологии» ФМБА России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2015-09-15" publication-format="electronic"><day>15</day><month>09</month><year>2015</year></pub-date><volume>87</volume><issue>9</issue><issue-title xml:lang="en">VOL 87, NO9 ()</issue-title><issue-title xml:lang="ru">ТОМ 87, №9 (2015)</issue-title><fpage>64</fpage><lpage>71</lpage><history><date date-type="received" iso-8601-date="2020-04-10"><day>10</day><month>04</month><year>2020</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2015, Consilium Medicum</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2015, ООО "Консилиум Медикум"</copyright-statement><copyright-year>2015</copyright-year><copyright-holder xml:lang="en">Consilium Medicum</copyright-holder><copyright-holder xml:lang="ru">ООО "Консилиум Медикум"</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by-nc-sa/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://ter-arkhiv.ru/0040-3660/article/view/31818">https://ter-arkhiv.ru/0040-3660/article/view/31818</self-uri><abstract xml:lang="en"><p>Pulmonary hypertension (PH) is a specific clinical group of severe and rare diseases with similar morphological, hemodynamic, and therapeutic characteristics. Despite the fact that there have been international conciliative documents and advances in drug therapy for PH, the long-term prognosis of the disease in these patients remains rather poor. Clinical trials have demonstrated that bosentan therapy in patients with PH improves pulmonary hemodynamics and exercise endurance and delays the development of the disease. According to the data of long-term studies, as compared to the historical control, bosentan used as a first-line drug can improve survival in PH patients.</p></abstract><trans-abstract xml:lang="ru"><p>Легочная артериальная гипертензия (ЛАГ) — специфическая клиническая группа тяжелых и редких заболеваний со сходными морфологическими, гемодинамическими и терапевтическими характеристиками. Несмотря на появление международных согласительных документов и успехи в медикаментозной терапии ЛАГ, долгосрочный прогноз исхода заболевания у таких пациентов остается достаточно плохим. Клинические исследования продемонстрировали, что терапия бозентаном пациентов с ЛАГ приводит к улучшению легочной гемодинамики, переносимости физических нагрузок и замедлению развития заболевания. Согласно данным длительных исследований, бозентан, назначенный как препарат терапии первой линии, позволяет улучшить выживаемость пациентов ЛАГ, по сравнению с историческим контролем.</p></trans-abstract><kwd-group xml:lang="en"><kwd>pulmonary hypertension</kwd><kwd>endothelin receptor antagonists</kwd><kwd>bosentan</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>легочная артериальная гипертензия</kwd><kwd>антагонисты рецепторов эндотелина</kwd><kwd>бозентан</kwd></kwd-group></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB. Definitions and diagnosis of pulmonary hypertension. 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