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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-3603</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>Муковисцидоз у взрослых: этиология, патогенез, перспективы лечения</article-title><trans-title-group xml:lang="en"><trans-title>Cystic fibrosis in adults: ethyology, pathogenesis, perspectives of treatment</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>Чучалин</surname><given-names>А. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Chuchalin</surname><given-names>A. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Воронина</surname><given-names>Л. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Voronina</surname><given-names>L. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кронина</surname><given-names>Л. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kronina</surname><given-names>L. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Самсонова</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Samsonova</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff xml:lang="ru" id="aff-1"><institution>НИИ пульмонологии МЗ РФ</institution><country>Russian Federation</country></aff><pub-date pub-type="collection"><year>1994</year></pub-date><pub-date pub-type="epub"><day>30</day><month>09</month><year>1994</year></pub-date><volume>0</volume><issue>3</issue><fpage>17</fpage><lpage>23</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Чучалин А.Г., Воронина Л.М., Кронина Л.А., Самсонова М.В., 1994</copyright-statement><copyright-year>1994</copyright-year><copyright-holder xml:lang="ru">Чучалин А.Г., Воронина Л.М., Кронина Л.А., Самсонова М.В.</copyright-holder><copyright-holder xml:lang="en">Chuchalin A.G., Voronina L.M., Kronina L.A., Samsonova M.V.</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/3603">https://journal.pulmonology.ru/pulm/article/view/3603</self-uri><abstract><p>.</p></abstract><trans-abstract xml:lang="en"><p>.</p></trans-abstract></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Блинова Т.В. Взаимосвязь мутации дельта Е 508 с особенностями иммунореактивности у больных муковисцидозом / / Пульмонология.— 1993.— No 2.— С.56—61.</mixed-citation><mixed-citation xml:lang="en">Блинова Т.В. Взаимосвязь мутации дельта Е 508 с особенностями иммунореактивности у больных муковисцидозом / / Пульмонология.— 1993.— No 2.— С.56—61.</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Болезни органов дыхания / Под ред. Н.Р.Палеева.— М.: Медицина, 1990.— С.201—210.</mixed-citation><mixed-citation xml:lang="en">Болезни органов дыхания / Под ред. Н.Р.Палеева.— М.: Медицина, 1990.— С.201—210.</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Воронина Л.М., Черняев А Л . и др. Цитологическая и цитобак-териоскопическая характеристика бронхиальных смывов при спелеотерапии / / Пульмонология.— 1994.— Приложение.— С. 166.</mixed-citation><mixed-citation xml:lang="en">Воронина Л.М., Черняев А Л . и др. Цитологическая и цитобак-териоскопическая характеристика бронхиальных смывов при спелеотерапии / / Пульмонология.— 1994.— Приложение.— С. 166.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">Гембицкая Т.Е. Особенности муковисцидоза у взрослых / / Сов. мед.— 1984.— No 4— С. 107—110.</mixed-citation><mixed-citation xml:lang="en">Гембицкая Т.Е. Особенности муковисцидоза у взрослых / / Сов. мед.— 1984.— No 4— С. 107—110.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Гембицкая Т.Е., Баранов B.C. Информация о работе IV североамериканской конференции по муковисцидозу / / Пульмонология.— 1993.— No 2.— С.52—55.</mixed-citation><mixed-citation xml:lang="en">Гембицкая Т.Е., Баранов B.C. Информация о работе IV североамериканской конференции по муковисцидозу / / Пульмонология.— 1993.— No 2.— С.52—55.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Гембицкая Т.В. и др. Организация помощи больным муковисцидозом и результаты длительного диспансерного наблюдения / / Там. же.— С.56— 61.</mixed-citation><mixed-citation xml:lang="en">Гембицкая Т.В. и др. Организация помощи больным муковисцидозом и результаты длительного диспансерного наблюдения / / Там. же.— С.56— 61.</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Горбенко П.П., Дубинская А.В., Осинин Ф.Г., Страшнова О.В. Галотерапия в профилактике и. лечении заболеваний органов дыхания: Метод, рекомендации.— Л., 1990.— С.1—13.</mixed-citation><mixed-citation xml:lang="en">Горбенко П.П., Дубинская А.В., Осинин Ф.Г., Страшнова О.В. Галотерапия в профилактике и. лечении заболеваний органов дыхания: Метод, рекомендации.— Л., 1990.— С.1—13.</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Капранов Н.Н. и др. Современные; достижения и актуальные вопросы в проблеме муковисцидоза / / Вести. Рос.’АМН.— 1992.— No 4— С.34—39.</mixed-citation><mixed-citation xml:lang="en">Капранов Н.Н. и др. Современные; достижения и актуальные вопросы в проблеме муковисцидоза / / Вести. Рос.’АМН.— 1992.— No 4— С.34—39.</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Рачинский С.В., Капранов Н.И. Муковисцидоз у детей / клиника, диагностика, лечение, вопросы медико-социальной адаптации/ — М., 1985.— С.1—24.</mixed-citation><mixed-citation xml:lang="en">Рачинский С.В., Капранов Н.И. Муковисцидоз у детей / клиника, диагностика, лечение, вопросы медико-социальной адаптации/ — М., 1985.— С.1—24.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">Рачинский С.В., Капранов Н.И., Торчинский М.10. Новое в изучении муковисцидоза / / Вопр. охр. мат.— 1989.— No 11 .— С.З—9.</mixed-citation><mixed-citation xml:lang="en">Рачинский С.В., Капранов Н.И., Торчинский М.10. Новое в изучении муковисцидоза / / Вопр. охр. мат.— 1989.— No 11 .— С.З—9.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Самильчук Е.И. Генная терапил наследственны*.заболеваний — реальность и перспективы / / Пульмонология.— 1993..— No 1.— С.6—9.</mixed-citation><mixed-citation xml:lang="en">Самильчук Е.И. Генная терапил наследственны*.заболеваний — реальность и перспективы / / Пульмонология.— 1993..— No 1.— С.6—9.</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Хафизова З.А. Клинические особенности муковисцидоза в зависимости от. генотипа: Дис. ... канд.мед.наук.— М . 1992.— С.1—154.</mixed-citation><mixed-citation xml:lang="en">Хафизова З.А. Клинические особенности муковисцидоза в зависимости от. генотипа: Дис. ... канд.мед.наук.— М . 1992.— С.1—154.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Чучалин А.Г., Самильчук Е.И. Муковисцидоз — состояние проблемы / / Тер. арх.— 1993.— No 3.— С.З—9.</mixed-citation><mixed-citation xml:lang="en">Чучалин А.Г., Самильчук Е.И. Муковисцидоз — состояние проблемы / / Тер. арх.— 1993.— No 3.— С.З—9.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Aitken M J. The role of mycobacterial infections in cystic fibrosis pulmonary disease / / Pediatr. Pulmonol.— 1991.— No 6.— P .160—162.</mixed-citation><mixed-citation xml:lang="en">Aitken M J. The role of mycobacterial infections in cystic fibrosis pulmonary disease / / Pediatr. Pulmonol.— 1991.— No 6.— P .160—162.</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Allan G.L., Phelan P.O. .Cystic Fibrosis: Survival to Adult Life. Ability to live with disability / / Med. J. A ust.— 1980.— Vol.l.— P .600—602.</mixed-citation><mixed-citation xml:lang="en">Allan G.L., Phelan P.O. .Cystic Fibrosis: Survival to Adult Life. Ability to live with disability / / Med. J. A ust.— 1980.— Vol.l.— P .600—602.</mixed-citation></citation-alternatives></ref><ref id="cit16"><label>16</label><citation-alternatives><mixed-citation xml:lang="ru">Altman R.P., Kulczycki L.L,, Randolph J.G.,.Clenathan J.E. Bronchoscopy Bronchial Lavage (BBL) in Children with Cystic Fibrosis / / J.-Pediatr. Surg.- 1973.— Vol.8, No 5.— P.809—81-4.</mixed-citation><mixed-citation xml:lang="en">Altman R.P., Kulczycki L.L,, Randolph J.G.,.Clenathan J.E. Bronchoscopy Bronchial Lavage (BBL) in Children with Cystic Fibrosis / / J.-Pediatr. Surg.- 1973.— Vol.8, No 5.— P.809—81-4.</mixed-citation></citation-alternatives></ref><ref id="cit17"><label>17</label><citation-alternatives><mixed-citation xml:lang="ru">Andersen D.H. Cystic Fibrosis of the Pancreas and its'Relation to Celiac Disease / / Am. J. Dis. Child.— 1938.— Vol.56.— P .344—399.</mixed-citation><mixed-citation xml:lang="en">Andersen D.H. Cystic Fibrosis of the Pancreas and its'Relation to Celiac Disease / / Am. J. Dis. Child.— 1938.— Vol.56.— P .344—399.</mixed-citation></citation-alternatives></ref><ref id="cit18"><label>18</label><citation-alternatives><mixed-citation xml:lang="ru">Andersen H.A., Fontana R.S. Transbronchioscopic Lung Biopsy for Diffuse Pulmonary Diseases: Technique and Results in 450 Cases / / Chest.— .1972.— Vol.62,— P.125—128.</mixed-citation><mixed-citation xml:lang="en">Andersen H.A., Fontana R.S. Transbronchioscopic Lung Biopsy for Diffuse Pulmonary Diseases: Technique and Results in 450 Cases / / Chest.— .1972.— Vol.62,— P.125—128.</mixed-citation></citation-alternatives></ref><ref id="cit19"><label>19</label><citation-alternatives><mixed-citation xml:lang="ru">Baltimore R.S., Christie C.D., Smith G.J Inimimohistopathologie localization of Pseudomonas aeruginasa in lung from.patients with cystic fibrosis. Implications for the pathogenesis of progressive lung deteioration / / Am. Rev. Respir.'Dis.— 1989.— Vol.140, No 6.— P.1650—1661.</mixed-citation><mixed-citation xml:lang="en">Baltimore R.S., Christie C.D., Smith G.J Inimimohistopathologie localization of Pseudomonas aeruginasa in lung from.patients with cystic fibrosis. Implications for the pathogenesis of progressive lung deteioration / / Am. Rev. Respir.'Dis.— 1989.— Vol.140, No 6.— P.1650—1661.</mixed-citation></citation-alternatives></ref><ref id="cit20"><label>20</label><citation-alternatives><mixed-citation xml:lang="ru">Bedard M. et al. Release of IL-8, IL-6 and colonystimulating factors by upper aisway epithelial cells: implication for cystic fibrosis / / Am. J. Respir Cells Microbiol.— 1993.— Vol.9, No 4.— P .455—462.</mixed-citation><mixed-citation xml:lang="en">Bedard M. et al. Release of IL-8, IL-6 and colonystimulating factors by upper aisway epithelial cells: implication for cystic fibrosis / / Am. J. Respir Cells Microbiol.— 1993.— Vol.9, No 4.— P .455—462.</mixed-citation></citation-alternatives></ref><ref id="cit21"><label>21</label><citation-alternatives><mixed-citation xml:lang="ru">Berger M. Infection, Immunity and Inflammation in the lung in cystic fibrosis / / Pediatr. Pulmonol.— 1988;— Suppl.2.—P . 71— 73.</mixed-citation><mixed-citation xml:lang="en">Berger M. Infection, Immunity and Inflammation in the lung in cystic fibrosis / / Pediatr. Pulmonol.— 1988;— Suppl.2.—P . 71— 73.</mixed-citation></citation-alternatives></ref><ref id="cit22"><label>22</label><citation-alternatives><mixed-citation xml:lang="ru">Berman G.M., Colman B.H. Nasal aspects of cystic fibrosis in children / / J. Laryng. Otol.— 1977.— Vol.91, No 2.— P. 133—139.</mixed-citation><mixed-citation xml:lang="en">Berman G.M., Colman B.H. Nasal aspects of cystic fibrosis in children / / J. Laryng. Otol.— 1977.— Vol.91, No 2.— P. 133—139.</mixed-citation></citation-alternatives></ref><ref id="cit23"><label>23</label><citation-alternatives><mixed-citation xml:lang="ru">Blancharsh C. Dental aspects of patients with cystic fibrosis: a preliminary clinical study / / J. Am. Dent. Ass.— 1977.— Vol.95, No 1.— P.106—110.</mixed-citation><mixed-citation xml:lang="en">Blancharsh C. Dental aspects of patients with cystic fibrosis: a preliminary clinical study / / J. Am. Dent. Ass.— 1977.— Vol.95, No 1.— P.106—110.</mixed-citation></citation-alternatives></ref><ref id="cit24"><label>24</label><citation-alternatives><mixed-citation xml:lang="ru">Boat F.T., Welsh J.M., Beaudet L.A. Cystic Fibrosis / / The Metabolic Basis of Inherited Disease. 6-th Ed.— New York, 1989,— P .2649—2679.</mixed-citation><mixed-citation xml:lang="en">Boat F.T., Welsh J.M., Beaudet L.A. Cystic Fibrosis / / The Metabolic Basis of Inherited Disease. 6-th Ed.— New York, 1989,— P .2649—2679.</mixed-citation></citation-alternatives></ref><ref id="cit25"><label>25</label><citation-alternatives><mixed-citation xml:lang="ru">Boucher R.C. Pathogenesis of Cystic Fibrosis in NaCl and V olume (Water) Absorbing Epithelia / / Pediatr. Pulmonol.— 1991.— No 6.— P .125.</mixed-citation><mixed-citation xml:lang="en">Boucher R.C. Pathogenesis of Cystic Fibrosis in NaCl and V olume (Water) Absorbing Epithelia / / Pediatr. Pulmonol.— 1991.— No 6.— P .125.</mixed-citation></citation-alternatives></ref><ref id="cit26"><label>26</label><citation-alternatives><mixed-citation xml:lang="ru">Brown J. Cystic Fibrosis / / Med. J. Aust.— 1992.— Vol.l, No 2.— P .67—70.</mixed-citation><mixed-citation xml:lang="en">Brown J. Cystic Fibrosis / / Med. J. Aust.— 1992.— Vol.l, No 2.— P .67—70.</mixed-citation></citation-alternatives></ref><ref id="cit27"><label>27</label><citation-alternatives><mixed-citation xml:lang="ru">Bruce M.C., Roncz L. et al. Biochemical and Pathologic Evidence for Proteolytic Destruction of Lung Connective Tissue in Cystic Fibrosis // Am. Rev. Respir. Dis.— 1985.— Vol.132, No 3.— P .529—535.</mixed-citation><mixed-citation xml:lang="en">Bruce M.C., Roncz L. et al. Biochemical and Pathologic Evidence for Proteolytic Destruction of Lung Connective Tissue in Cystic Fibrosis // Am. Rev. Respir. Dis.— 1985.— Vol.132, No 3.— P .529—535.</mixed-citation></citation-alternatives></ref><ref id="cit28"><label>28</label><citation-alternatives><mixed-citation xml:lang="ru">Carnoy C., Ramphal R. et al. Altered carbohydrate composition of salivary mucins from patients with cystic fibrosis and the adgesion of Pseudomonas aeruginosa / / Am. J. Respir. Cell Molec Biol.— 1993.— Vol.9, No 3.— P.323—335.</mixed-citation><mixed-citation xml:lang="en">Carnoy C., Ramphal R. et al. Altered carbohydrate composition of salivary mucins from patients with cystic fibrosis and the adgesion of Pseudomonas aeruginosa / / Am. J. Respir. Cell Molec Biol.— 1993.— Vol.9, No 3.— P.323—335.</mixed-citation></citation-alternatives></ref><ref id="cit29"><label>29</label><citation-alternatives><mixed-citation xml:lang="ru">Church J., Keens T. et al. Normal neuthrophill and monocyte chemotaxis in patients with cystic fibrosis / / J. Pediatr.— 1979.— Vol.95.— P .272—274.</mixed-citation><mixed-citation xml:lang="en">Church J., Keens T. et al. Normal neuthrophill and monocyte chemotaxis in patients with cystic fibrosis / / J. Pediatr.— 1979.— Vol.95.— P .272—274.</mixed-citation></citation-alternatives></ref><ref id="cit30"><label>30</label><citation-alternatives><mixed-citation xml:lang="ru">Ciofu C., Popescu-Miclosanu S. et al. Clinical and histopathologic polymorphism in cystic fibrosis / / Rev. Pediatr. Obstet. Ginecol. Pediatr.— 1989.— Vol.38, No 1,— P.61—74.</mixed-citation><mixed-citation xml:lang="en">Ciofu C., Popescu-Miclosanu S. et al. Clinical and histopathologic polymorphism in cystic fibrosis / / Rev. Pediatr. Obstet. Ginecol. Pediatr.— 1989.— Vol.38, No 1,— P.61—74.</mixed-citation></citation-alternatives></ref><ref id="cit31"><label>31</label><citation-alternatives><mixed-citation xml:lang="ru">Cohn J.A. et al. Protein Phosphorilation Responses in Normal and Cystic Fibrosis Epithelial Cell lines / / Am. J. Respir. Cell Molec. .Biol.— 1993.— V ol.9,No 4 .— P.401—404.</mixed-citation><mixed-citation xml:lang="en">Cohn J.A. et al. Protein Phosphorilation Responses in Normal and Cystic Fibrosis Epithelial Cell lines / / Am. J. Respir. Cell Molec. .Biol.— 1993.— V ol.9,No 4 .— P.401—404.</mixed-citation></citation-alternatives></ref><ref id="cit32"><label>32</label><citation-alternatives><mixed-citation xml:lang="ru">Collins F .S. Cystic Fibrosis: M olecular Biology and Therapeutic Implications / / Science.— 1992.— V ol.256.— P .774—779.</mixed-citation><mixed-citation xml:lang="en">Collins F .S. Cystic Fibrosis: M olecular Biology and Therapeutic Implications / / Science.— 1992.— V ol.256.— P .774—779.</mixed-citation></citation-alternatives></ref><ref id="cit33"><label>33</label><citation-alternatives><mixed-citation xml:lang="ru">Cowen L., Mok J. et al. Psychologic Adjusment of the family with a member who has Cystic Fibrosis / / Pediatrics.— 1986.— Vol.74, No-5.— P.745—753.</mixed-citation><mixed-citation xml:lang="en">Cowen L., Mok J. et al. Psychologic Adjusment of the family with a member who has Cystic Fibrosis / / Pediatrics.— 1986.— Vol.74, No-5.— P.745—753.</mixed-citation></citation-alternatives></ref><ref id="cit34"><label>34</label><citation-alternatives><mixed-citation xml:lang="ru">Crystal R:G. New Frontiers in Therapy / / Pediatr. Pulmonol.— 1991.— N. 6 — P 64—65.</mixed-citation><mixed-citation xml:lang="en">Crystal R:G. New Frontiers in Therapy / / Pediatr. Pulmonol.— 1991.— N. 6 — P 64—65.</mixed-citation></citation-alternatives></ref><ref id="cit35"><label>35</label><citation-alternatives><mixed-citation xml:lang="ru">Cystic Fibrosis: Manual of Diagnosis and Management / Ed. M.C. Goodchild, J.A. Dodge. 2-nd Ed.— London, 1992.— P .27—46.</mixed-citation><mixed-citation xml:lang="en">Cystic Fibrosis: Manual of Diagnosis and Management / Ed. M.C. Goodchild, J.A. Dodge. 2-nd Ed.— London, 1992.— P .27—46.</mixed-citation></citation-alternatives></ref><ref id="cit36"><label>36</label><citation-alternatives><mixed-citation xml:lang="ru">Davis P.B. Cystic Fibrosis in Adults. / / Cystic Fibrosis / Ed. L.M.Taussing.— New York, 1984.— P.408—433.</mixed-citation><mixed-citation xml:lang="en">Davis P.B. Cystic Fibrosis in Adults. / / Cystic Fibrosis / Ed. L.M.Taussing.— New York, 1984.— P.408—433.</mixed-citation></citation-alternatives></ref><ref id="cit37"><label>37</label><citation-alternatives><mixed-citation xml:lang="ru">Davis P.B., di-Sant Agnese P.A. Diagnosis and Treatment of Cystic Fibrosis. An Update / / Chest.— 1984.— Vol.85, No 6.— P .802—809.</mixed-citation><mixed-citation xml:lang="en">Davis P.B., di-Sant Agnese P.A. Diagnosis and Treatment of Cystic Fibrosis. An Update / / Chest.— 1984.— Vol.85, No 6.— P .802—809.</mixed-citation></citation-alternatives></ref><ref id="cit38"><label>38</label><citation-alternatives><mixed-citation xml:lang="ru">Dovey M., Wisseman C.L. et al. Ultrastructural morphology of the iung in cystic fibrosis / / J. Submicrosc. Cytol. Pathol.— 1989,— Vol.21, No 3.— P.521—534.</mixed-citation><mixed-citation xml:lang="en">Dovey M., Wisseman C.L. et al. Ultrastructural morphology of the iung in cystic fibrosis / / J. Submicrosc. Cytol. Pathol.— 1989,— Vol.21, No 3.— P.521—534.</mixed-citation></citation-alternatives></ref><ref id="cit39"><label>39</label><citation-alternatives><mixed-citation xml:lang="ru">Efthimion J., Hodson M. et a'L Importance of Viruses and Legionella pneumonia in respiratory exacerbations of young adults with cystic fibrosis / / Thorax.— 1984.— Vol.39, No 2.— P.150—154.</mixed-citation><mixed-citation xml:lang="en">Efthimion J., Hodson M. et a'L Importance of Viruses and Legionella pneumonia in respiratory exacerbations of young adults with cystic fibrosis / / Thorax.— 1984.— Vol.39, No 2.— P.150—154.</mixed-citation></citation-alternatives></ref><ref id="cit40"><label>40</label><citation-alternatives><mixed-citation xml:lang="ru">Fick R.B., Stillwell P.C. Controversies in the mamagement of pulmonary disease due to cystic fibrosis / / Chest.— 1989.— Vol.95, No .6,— P. 1319—1327.</mixed-citation><mixed-citation xml:lang="en">Fick R.B., Stillwell P.C. Controversies in the mamagement of pulmonary disease due to cystic fibrosis / / Chest.— 1989.— Vol.95, No .6,— P. 1319—1327.</mixed-citation></citation-alternatives></ref><ref id="cit41"><label>41</label><citation-alternatives><mixed-citation xml:lang="ru">Hendersson W.R., Chi E.Y.Degranulation of cystic fibrosis nasal polyp mast cells / / J. Pathol.- 1992.— V ol.166, No 4.— P/395—404.</mixed-citation><mixed-citation xml:lang="en">Hendersson W.R., Chi E.Y.Degranulation of cystic fibrosis nasal polyp mast cells / / J. Pathol.- 1992.— V ol.166, No 4.— P/395—404.</mixed-citation></citation-alternatives></ref><ref id="cit42"><label>42</label><citation-alternatives><mixed-citation xml:lang="ru">Hodson M.E. Bronchiectasis and Cystic Fibrosis / / Br. Med. J.— 1978.— Vol.l.— P.971—973.</mixed-citation><mixed-citation xml:lang="en">Hodson M.E. Bronchiectasis and Cystic Fibrosis / / Br. Med. J.— 1978.— Vol.l.— P.971—973.</mixed-citation></citation-alternatives></ref><ref id="cit43"><label>43</label><citation-alternatives><mixed-citation xml:lang="ru">Hodson M.E. Lung-transplantation for cystic fibrosis / / Cystic Fibrosis, Basic and Clinical R esearch / Ed. N. Noily, S.S. Pedersen.— Amsterdam, 1992.— P. 185—192.</mixed-citation><mixed-citation xml:lang="en">Hodson M.E. Lung-transplantation for cystic fibrosis / / Cystic Fibrosis, Basic and Clinical R esearch / Ed. N. Noily, S.S. Pedersen.— Amsterdam, 1992.— P. 185—192.</mixed-citation></citation-alternatives></ref><ref id="cit44"><label>44</label><citation-alternatives><mixed-citation xml:lang="ru">Hodson M.E. Cystic Fibrosis / / Postgrad. Med..-J.— 1984.— Vol.60.— P .225—233.</mixed-citation><mixed-citation xml:lang="en">Hodson M.E. Cystic Fibrosis / / Postgrad. Med..-J.— 1984.— Vol.60.— P .225—233.</mixed-citation></citation-alternatives></ref><ref id="cit45"><label>45</label><citation-alternatives><mixed-citation xml:lang="ru">Hodson M.E., Madden B.P. et al. Non invasive mechanical ventilation for cystic- fibrosis patients —- a potential bridge to transplantation / / .Eui. Respir. J.— 1991.— Vol.4.— P.524—527.</mixed-citation><mixed-citation xml:lang="en">Hodson M.E., Madden B.P. et al. Non invasive mechanical ventilation for cystic- fibrosis patients —- a potential bridge to transplantation / / .Eui. Respir. J.— 1991.— Vol.4.— P.524—527.</mixed-citation></citation-alternatives></ref><ref id="cit46"><label>46</label><citation-alternatives><mixed-citation xml:lang="ru">Hodson-M.E., Penketh A.R.L., Batten J.C. Aerosol Carbennicillin and Gentamicim treatment of Pseudomonas infection in patients with cystic fibrosis / Lancet.— 1981.— Vol.2, No 8253.— P .1 137—1139.</mixed-citation><mixed-citation xml:lang="en">Hodson-M.E., Penketh A.R.L., Batten J.C. Aerosol Carbennicillin and Gentamicim treatment of Pseudomonas infection in patients with cystic fibrosis / Lancet.— 1981.— Vol.2, No 8253.— P .1 137—1139.</mixed-citation></citation-alternatives></ref><ref id="cit47"><label>47</label><citation-alternatives><mixed-citation xml:lang="ru">Hodson M.E., Roberts C.M. et al. Oral ciprofloxacin compared with conventional intravenous treatment for Pseudomonas aeruginosa infection in adults with Cystic Fibrosis / / Lancet.— 1987.— Vol.1, No 8526,— P;235—237.</mixed-citation><mixed-citation xml:lang="en">Hodson M.E., Roberts C.M. et al. Oral ciprofloxacin compared with conventional intravenous treatment for Pseudomonas aeruginosa infection in adults with Cystic Fibrosis / / Lancet.— 1987.— Vol.1, No 8526,— P;235—237.</mixed-citation></citation-alternatives></ref><ref id="cit48"><label>48</label><citation-alternatives><mixed-citation xml:lang="ru">Howie A.D., Sack B.H.R. Cystic Fibrosis in adolescents and young adults / / Scot- Med. J:— 1979.— Vol.24, No 3.— P. 193—198.</mixed-citation><mixed-citation xml:lang="en">Howie A.D., Sack B.H.R. Cystic Fibrosis in adolescents and young adults / / Scot- Med. J:— 1979.— Vol.24, No 3.— P. 193—198.</mixed-citation></citation-alternatives></ref><ref id="cit49"><label>49</label><citation-alternatives><mixed-citation xml:lang="ru">Hunt B., Geddes D. Newly diagnosted cystic fibrosis in middle and later life / / Thorax.— 1985.— V ol.40, No 1.— P .23—26.</mixed-citation><mixed-citation xml:lang="en">Hunt B., Geddes D. Newly diagnosted cystic fibrosis in middle and later life / / Thorax.— 1985.— V ol.40, No 1.— P .23—26.</mixed-citation></citation-alternatives></ref><ref id="cit50"><label>50</label><citation-alternatives><mixed-citation xml:lang="ru">Khaghani A., Madden B. et al. Heart-Lung Transplantation for Cystic Fibrosis / / Pediatr. Pulmonol.— 1991.— No 6.— P. 128—129.</mixed-citation><mixed-citation xml:lang="en">Khaghani A., Madden B. et al. Heart-Lung Transplantation for Cystic Fibrosis / / Pediatr. Pulmonol.— 1991.— No 6.— P. 128—129.</mixed-citation></citation-alternatives></ref><ref id="cit51"><label>51</label><citation-alternatives><mixed-citation xml:lang="ru">Knowles M.R., Stutts- M.J. et al. Abnormal Ion Permetation Through Cystic Fibrosis Respiratory Epithelium / / Science.— 1983.— Vol.221, No 9,— P .1067—1070.</mixed-citation><mixed-citation xml:lang="en">Knowles M.R., Stutts- M.J. et al. Abnormal Ion Permetation Through Cystic Fibrosis Respiratory Epithelium / / Science.— 1983.— Vol.221, No 9,— P .1067—1070.</mixed-citation></citation-alternatives></ref><ref id="cit52"><label>52</label><citation-alternatives><mixed-citation xml:lang="ru">Lethem M.I., Dowell M.L. et al. Nucleotide regulation of goblet cells in human airway epithelial explants: normal exocytosis in cystic fibrosis / / Am. J. Respir. Cell Microbiol.— 1993.— Vol.9, No 3.— P .315—322.</mixed-citation><mixed-citation xml:lang="en">Lethem M.I., Dowell M.L. et al. Nucleotide regulation of goblet cells in human airway epithelial explants: normal exocytosis in cystic fibrosis / / Am. J. Respir. Cell Microbiol.— 1993.— Vol.9, No 3.— P .315—322.</mixed-citation></citation-alternatives></ref><ref id="cit53"><label>53</label><citation-alternatives><mixed-citation xml:lang="ru">Meyer K.C., Zimmerman J. Neuthrophil mediators, Pseudomonas and pulmonary disfunction in cystic fibrosis // J. Lab. Clin. M ed.— 1993.— Vol.121, N. 5.— P .654—661.</mixed-citation><mixed-citation xml:lang="en">Meyer K.C., Zimmerman J. Neuthrophil mediators, Pseudomonas and pulmonary disfunction in cystic fibrosis // J. Lab. Clin. M ed.— 1993.— Vol.121, N. 5.— P .654—661.</mixed-citation></citation-alternatives></ref><ref id="cit54"><label>54</label><citation-alternatives><mixed-citation xml:lang="ru">Millis R.M., Young R.C., Kulczycki L.L. Validation ofTherapeutic Bronchoscopic Bronchial Washing in Cystic Fibrosis / / Chest.— 1977.— Vol.71.— P .508—513.</mixed-citation><mixed-citation xml:lang="en">Millis R.M., Young R.C., Kulczycki L.L. Validation ofTherapeutic Bronchoscopic Bronchial Washing in Cystic Fibrosis / / Chest.— 1977.— Vol.71.— P .508—513.</mixed-citation></citation-alternatives></ref><ref id="cit55"><label>55</label><citation-alternatives><mixed-citation xml:lang="ru">Park R.W ., Grand R.J. Gasrointestinal manifestations of Cystic Fibrosis: A review / / Gastroenterology.— 1981.— Vol.81, No 6.— P .1143—1161.</mixed-citation><mixed-citation xml:lang="en">Park R.W ., Grand R.J. Gasrointestinal manifestations of Cystic Fibrosis: A review / / Gastroenterology.— 1981.— Vol.81, No 6.— P .1143—1161.</mixed-citation></citation-alternatives></ref><ref id="cit56"><label>56</label><citation-alternatives><mixed-citation xml:lang="ru">Pederson S. Model system sweat glands. Aspect of the calcium mediated biological response in cultered sweat duct glands / / Cystic Fibrosis, Basic and Clinical Research.— Amsterdam, 1992.— P .35—48.</mixed-citation><mixed-citation xml:lang="en">Pederson S. Model system sweat glands. Aspect of the calcium mediated biological response in cultered sweat duct glands / / Cystic Fibrosis, Basic and Clinical Research.— Amsterdam, 1992.— P .35—48.</mixed-citation></citation-alternatives></ref><ref id="cit57"><label>57</label><citation-alternatives><mixed-citation xml:lang="ru">Penketh A.R.L., Wise A. et al. Cystic Fibrosis in adolescents and adults / / Thorax.— 1987.— Vol.42.— P.526—532.</mixed-citation><mixed-citation xml:lang="en">Penketh A.R.L., Wise A. et al. Cystic Fibrosis in adolescents and adults / / Thorax.— 1987.— Vol.42.— P.526—532.</mixed-citation></citation-alternatives></ref><ref id="cit58"><label>58</label><citation-alternatives><mixed-citation xml:lang="ru">Pennington J., W olff S., Puziss M,. Summary oT a workshop on infections in patients with cystic fibrosis / / J. Infect. Dis.— 1979.— Vol. 140.— P.-252—256.</mixed-citation><mixed-citation xml:lang="en">Pennington J., W olff S., Puziss M,. Summary oT a workshop on infections in patients with cystic fibrosis / / J. Infect. Dis.— 1979.— Vol. 140.— P.-252—256.</mixed-citation></citation-alternatives></ref><ref id="cit59"><label>59</label><citation-alternatives><mixed-citation xml:lang="ru">Perobelli S. The. physiological situation of independently living cystic fibrosis adults / / Cystic Fibrosis, Basic and .Clinical Research.— Amsterdam, 1992.- P.526—532.</mixed-citation><mixed-citation xml:lang="en">Perobelli S. The. physiological situation of independently living cystic fibrosis adults / / Cystic Fibrosis, Basic and .Clinical Research.— Amsterdam, 1992.- P.526—532.</mixed-citation></citation-alternatives></ref><ref id="cit60"><label>60</label><citation-alternatives><mixed-citation xml:lang="ru">Plotkowsky M.C., Bajolet-Laudinat O'., Puchelle E. Cellular and molecular mechanisms of bacterial adhesion to respiratory mucosa / / Eur. Respir. J.— 1093.— Vol.6, No 6.— P.903—916.</mixed-citation><mixed-citation xml:lang="en">Plotkowsky M.C., Bajolet-Laudinat O'., Puchelle E. Cellular and molecular mechanisms of bacterial adhesion to respiratory mucosa / / Eur. Respir. J.— 1093.— Vol.6, No 6.— P.903—916.</mixed-citation></citation-alternatives></ref><ref id="cit61"><label>61</label><citation-alternatives><mixed-citation xml:lang="ru">Plotkowsky M.C., Chevillard M. et al. Epithelial respiratory cells from cystic fibrosis patients do not possess specific Pseudomonas aeruginosa adgesive properties / / J. Med. Microbiol.— 1992.— Vol.36, No 2,— P.104—111.</mixed-citation><mixed-citation xml:lang="en">Plotkowsky M.C., Chevillard M. et al. Epithelial respiratory cells from cystic fibrosis patients do not possess specific Pseudomonas aeruginosa adgesive properties / / J. Med. Microbiol.— 1992.— Vol.36, No 2,— P.104—111.</mixed-citation></citation-alternatives></ref><ref id="cit62"><label>62</label><citation-alternatives><mixed-citation xml:lang="ru">Rose J., Gamble J.G., Lewiston N. Back pain and spinal deformity in cystic fibrosis / / Pediatr. Pulmonol.— 1991.— No 6.— P .111—112.</mixed-citation><mixed-citation xml:lang="en">Rose J., Gamble J.G., Lewiston N. Back pain and spinal deformity in cystic fibrosis / / Pediatr. Pulmonol.— 1991.— No 6.— P .111—112.</mixed-citation></citation-alternatives></ref><ref id="cit63"><label>63</label><citation-alternatives><mixed-citation xml:lang="ru">Seddon D.J., Hodson M.E. Surgical management of pneumothorax in cystic fibrosis / / Thorax.— 1988.— Vol.43.— P .739—740.</mixed-citation><mixed-citation xml:lang="en">Seddon D.J., Hodson M.E. Surgical management of pneumothorax in cystic fibrosis / / Thorax.— 1988.— Vol.43.— P .739—740.</mixed-citation></citation-alternatives></ref><ref id="cit64"><label>64</label><citation-alternatives><mixed-citation xml:lang="ru">Stead R.J., Skypale G. et al. Enteric coated microspheres of pancreatin in the treatment of cystic fibrosis: comparison with a standard enteric coated preparations / / Ibid.— 1987.— Vol.42, No 7.— P.533—537.</mixed-citation><mixed-citation xml:lang="en">Stead R.J., Skypale G. et al. Enteric coated microspheres of pancreatin in the treatment of cystic fibrosis: comparison with a standard enteric coated preparations / / Ibid.— 1987.— Vol.42, No 7.— P.533—537.</mixed-citation></citation-alternatives></ref><ref id="cit65"><label>65</label><citation-alternatives><mixed-citation xml:lang="ru">Suter S. Lung inflammation and antiinflammatory treatment / / Cystic Fibrosis, Basic and Clinical Research.— Amsterdam, 1992.— P .177—184.</mixed-citation><mixed-citation xml:lang="en">Suter S. Lung inflammation and antiinflammatory treatment / / Cystic Fibrosis, Basic and Clinical Research.— Amsterdam, 1992.— P .177—184.</mixed-citation></citation-alternatives></ref><ref id="cit66"><label>66</label><citation-alternatives><mixed-citation xml:lang="ru">Taussing L.M., Landau L.G., Marcs M.G. Bronchial Lavage / / Cystic Fibrosis / Ed. L.M. Taussing.— New York, 1984.— P.149—150.</mixed-citation><mixed-citation xml:lang="en">Taussing L.M., Landau L.G., Marcs M.G. Bronchial Lavage / / Cystic Fibrosis / Ed. L.M. Taussing.— New York, 1984.— P.149—150.</mixed-citation></citation-alternatives></ref><ref id="cit67"><label>67</label><citation-alternatives><mixed-citation xml:lang="ru">Van-Haren E.H., Hoptman W.P. et al. Pancreatic morphology and function in adult patients with cystic fibrosis / / Scand. J. Gastroenterol.— 1992.— Vol.27, No 8.— P .695—698.</mixed-citation><mixed-citation xml:lang="en">Van-Haren E.H., Hoptman W.P. et al. Pancreatic morphology and function in adult patients with cystic fibrosis / / Scand. J. Gastroenterol.— 1992.— Vol.27, No 8.— P .695—698.</mixed-citation></citation-alternatives></ref><ref id="cit68"><label>68</label><citation-alternatives><mixed-citation xml:lang="ru">Welsh M.J. Search for basic defect / / Pediatr. Pulmonol.— 1988.— Suppl.2.— P .21—22.</mixed-citation><mixed-citation xml:lang="en">Welsh M.J. Search for basic defect / / Pediatr. Pulmonol.— 1988.— Suppl.2.— P .21—22.</mixed-citation></citation-alternatives></ref><ref id="cit69"><label>69</label><citation-alternatives><mixed-citation xml:lang="ru">Wewers M. Pathogenesis of emphysema. Assesment of basic science concepts through clinical investigation / / Chest.— 1989.— Vol.95, No P.190—195.</mixed-citation><mixed-citation xml:lang="en">Wewers M. Pathogenesis of emphysema. Assesment of basic science concepts through clinical investigation / / Chest.— 1989.— Vol.95, No P.190—195.</mixed-citation></citation-alternatives></ref><ref id="cit70"><label>70</label><citation-alternatives><mixed-citation xml:lang="ru">Willmott R.W . Allergic Bronchopulmonary Aspergillesis. Diagnosis and Treatment / / Pediatr. Pulmonol.— 1991.— No 6.— P.162—163.</mixed-citation><mixed-citation xml:lang="en">Willmott R.W . Allergic Bronchopulmonary Aspergillesis. Diagnosis and Treatment / / Pediatr. Pulmonol.— 1991.— No 6.— P.162—163.</mixed-citation></citation-alternatives></ref><ref id="cit71"><label>71</label><citation-alternatives><mixed-citation xml:lang="ru">Wood R.E. Factors Affecting Survival of Populations / / Cystic Fibrosis / Ed. L.M. Taussing.— New York, 1984.— P.438—445.</mixed-citation><mixed-citation xml:lang="en">Wood R.E. Factors Affecting Survival of Populations / / Cystic Fibrosis / Ed. L.M. Taussing.— New York, 1984.— P.438—445.</mixed-citation></citation-alternatives></ref></ref-list><fn-group><fn fn-type="conflict"><p>The authors declare that there are no conflicts of interest present.</p></fn></fn-group></back></article>
