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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 pub-id-type="doi">10.18093/0869-0189-2011-0-1-111-114</article-id><article-id custom-type="elpub" pub-id-type="custom">pulmo-259</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>ЗАМЕТКИ ИЗ ПРАКТИКИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PRACTICAL NOTES</subject></subj-group></article-categories><title-group><article-title>СЛУЧАЙ МНОЖЕСТВЕННЫХ ГЕНЕТИЧЕСКИХ НАРУШЕНИЙ В РАЗЛИЧНЫХ СИСТЕМАХ ЗАЩИТЫ ЛЕГКИХ</article-title><trans-title-group xml:lang="en"><trans-title>A CASE OF MULTIPLE GENETIC LESIONS IN DIFFERENT SYSTEMS OF THE LUNG DEFENSE</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>Дидковский</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Didkovsky</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д. м. н., проф., заслуженный врач России, зав. лабораторией клинической иммунологии </p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: 8-499-782-33-39.</p><p> </p></bio><bio xml:lang="en"><p>д. м. н., проф., заслуженный врач России, зав. лабораторией клинической иммунологии</p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: 8-499-782-33-39.</p></bio><email xlink:type="simple">didkovski@rambler.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Жарова</surname><given-names>М. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Zharova</surname><given-names>M. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к. б. н., науч. сотрудник лаборатории клинической иммунологии </p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: (499) 782-33-25.</p></bio><bio xml:lang="en"><p>к. б. н., науч. сотрудник лаборатории клинической иммунологии</p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: (499) 782-33-25.</p></bio><email xlink:type="simple">marusia.v@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Каблашова</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kablashova</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>ст. ординатор отделения иммунотерапии и детоксикации, врач высшей категории </p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: (499) 782-33-25.</p></bio><bio xml:lang="en"><p>ст. ординатор отделения иммунотерапии и детоксикации, врач высшей категории</p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: (499) 782-33-25.</p></bio><email xlink:type="simple">vladkom@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Малашенкова</surname><given-names>И. К.</given-names></name><name name-style="western" xml:lang="en"><surname>Malashenkova</surname><given-names>I. K.</given-names></name></name-alternatives><bio xml:lang="ru"><p>ст. науч. сотрудник лаборатории клинической иммунологии </p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: (499) 782-33-25.</p></bio><bio xml:lang="en"><p>ст. науч. сотрудник лаборатории клинической иммунологии</p><p>119992, Москва, ул. Малая Пироговская, 1а.</p><p>Тел.: (499) 782-33-25.</p></bio><email xlink:type="simple">didkovski@rambler.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГУ "НИИ физико6химической медицины" ФМБА России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>ФГУ "НИИ физико6химической медицины" ФМБА России</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2011</year></pub-date><pub-date pub-type="epub"><day>28</day><month>02</month><year>2011</year></pub-date><volume>0</volume><issue>1</issue><fpage>111</fpage><lpage>114</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Дидковский Н.А., Жарова М.А., Каблашова Н.А., Малашенкова И.К., 2011</copyright-statement><copyright-year>2011</copyright-year><copyright-holder xml:lang="ru">Дидковский Н.А., Жарова М.А., Каблашова Н.А., Малашенкова И.К.</copyright-holder><copyright-holder xml:lang="en">Didkovsky N.A., Zharova M.S., Kablashova N.A., Malashenkova I.K.</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/259">https://journal.pulmonology.ru/pulm/article/view/259</self-uri><abstract><p>Случай множественных генетических нарушений в различных системах защиты легких</p></abstract><trans-abstract xml:lang="en"><p>A case of multiple genetic lesions in different systems of the lung defense</p></trans-abstract><kwd-group xml:lang="ru"><kwd>буллезная болезнь легких</kwd><kwd>генетические дефекты</kwd><kwd>металлопротеиназы</kwd><kwd>белки сурфактанта</kwd><kwd>эпоксидгидролаза</kwd></kwd-group><kwd-group xml:lang="en"><kwd>bulleous lung disease</kwd><kwd>genetic lesions</kwd><kwd>metalloproteinases</kwd><kwd>surfactant proteins</kwd><kwd>epoxide hydrolase</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Sandford A.J., Joos L., Pare P.D. 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