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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">actabiomedica</journal-id><journal-title-group><journal-title xml:lang="ru">Acta Biomedica Scientifica</journal-title><trans-title-group xml:lang="en"><trans-title>Acta Biomedica Scientifica</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2541-9420</issn><issn pub-type="epub">2587-9596</issn><publisher><publisher-name>Scientific Centre for Family Health and Human Reproduction Problems</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.12737/21492</article-id><article-id custom-type="elpub" pub-id-type="custom">actabiomedica-159</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>СЛУЧАЙ ИЗ ПРАКТИКИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CASE REPORTS</subject></subj-group></article-categories><title-group><article-title>Первичная цилиарная дискинезия. Клиническое наблюдение</article-title><trans-title-group xml:lang="en"><trans-title>Primary ciliary dyskinesia. Clinical observation</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>Павлова</surname><given-names>Т. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Pavlova</surname><given-names>T. B.</given-names></name></name-alternatives><email xlink:type="simple">tabopav@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>Шинкарёва</surname><given-names>В. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Shinkaryova</surname><given-names>V. M.</given-names></name></name-alternatives><email xlink:type="simple">vm_shinkareva@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ГБОУ ДПО «Иркутская государственная медицинская академия последипломного образования» Минздрава России; ГБУЗ «Иркутская государственная областная детская клиническая больница»</institution></aff><aff xml:lang="en"><institution>Irkutsk State Medical Academy of Continuing Education; Irkutsk State Regional Children’s Clinical Hospital</institution></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ГБУЗ «Иркутская государственная областная детская клиническая больница»</institution></aff><aff xml:lang="en"><institution>Irkutsk State Regional Children’s Clinical Hospital</institution></aff></aff-alternatives><pub-date pub-type="collection"><year>2016</year></pub-date><pub-date pub-type="epub"><day>28</day><month>02</month><year>2016</year></pub-date><volume>1</volume><issue>1</issue><fpage>75</fpage><lpage>77</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Павлова Т.Б., Шинкарёва В.М., 2016</copyright-statement><copyright-year>2016</copyright-year><copyright-holder xml:lang="ru">Павлова Т.Б., Шинкарёва В.М.</copyright-holder><copyright-holder xml:lang="en">Pavlova T.B., Shinkaryova V.M.</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://www.actabiomedica.ru/jour/article/view/159">https://www.actabiomedica.ru/jour/article/view/159</self-uri><abstract><p>В статье приведено собственное наблюдение первичной цилиарной дискинезии, иллюстрирующее трудности диагностики этого заболевания. Сложность данного клинического случая определялась отсутствием инверсии внутренних органов у пациента с ранними симптомами хронического бронхита и хронического синусита. Первичная цилиарная дискинезия относится к орфанным заболеваниям, поэтому осведомлённость врачей первичного звена в отношении этой патологии низкая, что способствует высокой инвалидизации пациентов.</p></abstract><trans-abstract xml:lang="en"><p>We give an example of our own clinical observation of primary ciliary dyskinesia showing difficulties of this disease diagnosis. The complexity of this clinical picture is in the absence of situs viscerum inversus of the patient with early symptoms of chronic bronchitis and chronic sinusitis. From birth patient was noticed to have wheezing during acute respiratory viral infection (4 episodes), first year -bilateral pneumonia, and later - 3-4 pneumonias per year. Gastroesophageal reflux disease was diagnosed on third year of life, surgical treatment was used. Year 4 - according to computerized tomography data middle lobe syndrome was detected. We performed middle lobectomy. Then we revealed morphologically-cylindrical bronchiectasis. Severe bronchial asthma and allergic rhinitis were diagnosed in 4,5 years. Despite the fact that baseline inhalation and antimicrobial therapy was done, general condition of the patient did not get better. On year 9 we did bronchoscopy with brush biopsy of bronchial mucosa. The result is sharp decrease of ciliary function. All in all, even though the patient had classical symptoms of primary ciliary dyskinesia and first symptoms showed during the first year of life, right diagnose was stated only when the patient turned nine. Primary ciliary dyskinesia is classified as rare (orphan) diseases, that is why awareness of primary care physicians against this pathology is low, resulting in high disability of patients.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>первичная цилиарная дискинезия</kwd><kwd>синдром Картагенера</kwd><kwd>клиническое наблюдение</kwd></kwd-group><kwd-group xml:lang="en"><kwd>primary ciliary dyskinesia</kwd><kwd>Kartagener syndrome</kwd><kwd>clinical observation</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">Аллергология и иммунология: национальное руководство / Под ред. Р.М. Хаитова, Н.И. Ильиной. -М.: ГЭОТАР-Медиа, 2009. - 656 с</mixed-citation><mixed-citation xml:lang="en">Аллергология и иммунология: национальное руководство / Под ред. Р.М. Хаитова, Н.И. 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