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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">cfpd</journal-id><journal-title-group><journal-title xml:lang="ru">Бюллетень физиологии и патологии дыхания</journal-title><trans-title-group xml:lang="en"><trans-title>Bulletin Physiology and Pathology of Respiration</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1998-5029</issn><publisher><publisher-name>Дальневосточный научный центр физиологии и патологии дыхания</publisher-name></publisher></journal-meta><article-meta><article-id custom-type="elpub" pub-id-type="custom">cfpd-519</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>Статьи</subject></subj-group></article-categories><title-group><article-title>ПРОГРЕССИРУЮЩАЯ ЛЕГОЧНАЯ ДИСТРОФИЯ, КАК ОДИН ИЗ ВАРИАНТОВ БУЛЛЕЗНОЙ ЭМФИЗЕМЫ ЛЕГКИХ</article-title><trans-title-group xml:lang="en"><trans-title>PROGRESSIVE PULMONARY DYSTROPHY AS ONE OF THE VARIANTS OF BULLOUS LUNGS EMPHYSEMA</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>Леншин</surname><given-names>Анатолий Васильевич</given-names></name><name name-style="western" xml:lang="en"><surname>Lenshin</surname><given-names>Anatoliy V.</given-names></name></name-alternatives><email xlink:type="simple">lenshin42@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>Ильин</surname><given-names>Андрей Валерьевич</given-names></name><name name-style="western" xml:lang="en"><surname>Il'in</surname><given-names>Andrey V.</given-names></name></name-alternatives><email xlink:type="simple">alero82@yandex.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>Одиреев</surname><given-names>Андрей Николаевич</given-names></name><name name-style="western" xml:lang="en"><surname>Odireev</surname><given-names>Andrey N.</given-names></name></name-alternatives><email xlink:type="simple">bulleten.fpd@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>Шелудько</surname><given-names>Елизавета Григорьевна</given-names></name><name name-style="western" xml:lang="en"><surname>Shelud'ko</surname><given-names>Elizaveta G.</given-names></name></name-alternatives><email xlink:type="simple">dncfpd@ramn.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Дальневосточный научный центр физиологии и патологии дыхания Сибирского отделения РАМН</institution></aff><aff xml:lang="en"><institution>Far Eastern Scientific Center of Physiology and Pathology of Respiration SB RAMS</institution></aff></aff-alternatives><pub-date pub-type="collection"><year>2012</year></pub-date><pub-date pub-type="epub"><day>19</day><month>02</month><year>2020</year></pub-date><volume>0</volume><issue>45</issue><fpage>104</fpage><lpage>114</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Леншин А.В., Ильин А.В., Одиреев А.Н., Шелудько Е.Г., 2020</copyright-statement><copyright-year>2020</copyright-year><copyright-holder xml:lang="ru">Леншин А.В., Ильин А.В., Одиреев А.Н., Шелудько Е.Г.</copyright-holder><copyright-holder xml:lang="en">Lenshin A.V., Il'in A.V., Odireev A.N., Shelud'ko E.G.</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://cfpd.elpub.ru/jour/article/view/519">https://cfpd.elpub.ru/jour/article/view/519</self-uri><abstract><p>Прогрессирующая легочная дистрофия («исчезающее легкое») - редкое заболевание неизвестной этиологии. Оно характеризуется выраженными дистрофическими легочными изменениями, проявляющимися трансформацией нормальной легочной ткани в гигантские воздушные буллы, занимающие не менее 1/3 объема легких. Для этого заболевания характерно прогрессирующее течение, преимущественно односторонний характер поражения, несоответствие объема поражения легочной ткани и скудности клинических проявлений. Нами проанализирован собственный, на протяжении ряда лет, опыт диагностики и динамического наблюдения за 5 пациентами с прогрессирующей легочной дистрофией. Исход заболевания, если не применять хирургическую коррекцию (операцию редукции объема, трансплантацию легкого) в подавляющем большинстве случаев фатальный. В работе проиллюстрированы различные методы лучевой диагностики: начиная от широко распространенных рутинных и до современных высокотехнологичных. Представлены результаты эксклюзивной разработки - метод трехмерной волюметрии для количественной оценки вентиляционной функции легких при мультиспиральной компьютерной томографии с использованием инспираторно-экспираторного теста.</p></abstract><trans-abstract xml:lang="en"><p>Progressive pulmonary dystrophy («disappearing lung») is a rare disease of unknown etiology. It is characterized by intensive dystrophic pulmonary changes revealed through the transformation of normal lung tissue into huge air bullas taking 1/3 of lungs volume. This disease is characterized by progressive clinical course, predominantly one-sided character of affection, inconsistency between the scope of affection and bareness of clinical presentations. Personal experience of diagnostics and dynamic examination of 5 patients with progressive pulmonary dystrophy during several years was analyzed. The clinical outcome without surgical correction (the operation of the volume reduction, lung transplantation) is fatal in most cases. The work illustrates different methods of radiodiagnostics, ranging from widely-spread routine ones to modern high-technological ones. The results of exclusive development, namely the method of three dimensional volumetry for quantitative estimation of lungs ventilation function at multispiral computer-aided tomography with the use of inspiratory and expiratory test are presented.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>прогрессирующая легочная дистрофия</kwd><kwd>синдром исчезающего легкого</kwd><kwd>клиническая и лучевая диагностика</kwd><kwd>компьютерная томография</kwd><kwd>инспираторно-экспираторный тест</kwd><kwd>трехмерная волюметрия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>progressive pulmonary dystrophy</kwd><kwd>the syndrome of disappearing lung</kwd><kwd>clinical and radio diagnostics</kwd><kwd>computer-aided tomography</kwd><kwd>inspiratory and expiratory test</kwd><kwd>three dimensional volumetry</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">Вишневский А.А, Борисов В.В., Рустамов И.Р. 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