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<article article-type="research-article" dtd-version="1.3" 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" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">pulmo</journal-id><journal-title-group><journal-title xml:lang="ru">Пульмонология</journal-title><trans-title-group xml:lang="en"><trans-title>PULMONOLOGIYA</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0869-0189</issn><issn pub-type="epub">2541-9617</issn><publisher><publisher-name>Scientific and Practical Journal “PULMONOLOGIYA” LLC</publisher-name></publisher></journal-meta><article-meta><article-id custom-type="elpub" pub-id-type="custom">pulmo-2713</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОБЗОРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW</subject></subj-group></article-categories><title-group><article-title>Молекулярные основы и клинические аспекты недостаточности альфа 1-антитрипсин</article-title><trans-title-group xml:lang="en"><trans-title>Molecular basis and clinical aspects of alfa 1 -antitripsin deficiency</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пузырев</surname><given-names>В. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Pusyrev</surname><given-names>V. P.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Савюк</surname><given-names>В. Я.</given-names></name><name name-style="western" xml:lang="en"><surname>Savyuk</surname><given-names>V. Ya.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff xml:lang="ru" id="aff-1"><institution>НИИ медицинской генетики ТНЦ СО РАМН</institution><country>Russian Federation</country></aff><pub-date pub-type="collection"><year>2003</year></pub-date><pub-date pub-type="epub"><day>08</day><month>09</month><year>2021</year></pub-date><volume>0</volume><issue>1</issue><fpage>105</fpage><lpage>115</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Пузырев В.П., Савюк В.Я., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Пузырев В.П., Савюк В.Я.</copyright-holder><copyright-holder xml:lang="en">Pusyrev V.P., Savyuk V.Y.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://journal.pulmonology.ru/pulm/article/view/2713">https://journal.pulmonology.ru/pulm/article/view/2713</self-uri><abstract><p>.</p></abstract></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Кравчук О.И.. 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