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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">mimmun</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинская иммунология</journal-title><trans-title-group xml:lang="en"><trans-title>Medical Immunology (Russia)</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1563-0625</issn><issn pub-type="epub">2313-741X</issn><publisher><publisher-name>SPb RAACI</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15789/1563-0625-RRO-2148</article-id><article-id custom-type="elpub" pub-id-type="custom">mimmun-2148</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>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL ARTICLES</subject></subj-group></article-categories><title-group><article-title>Республиканский регистр первичных иммунодефицитов в чувашской республике и описание нарушения поствакцинального иммунитета у беременной пациентки с общей вариабельной иммунной недостаточностью</article-title><trans-title-group xml:lang="en"><trans-title>Republican registry of primary immune deficiencies in the chuvash republic and description of postvaccinal immunity disorders in a pregnant patient with common variable immune deficiency</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5899-6352</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Карзакова</surname><given-names>Л. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Karzakova</surname><given-names>L. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, заведующий кафедрой внутренних болезней с курсом клинической иммунологии;</p><p>428015, Чувашская Республика, г. Чебоксары, Московский пр., 15</p></bio><bio xml:lang="en"><p>PhD, MD (Medicine), Professor, Head, Department of Internal Diseases with a Course of Clinical Immunology, </p><p>428015, Chuvash Republic, Cheboksary, Moskovsky ave., 15</p></bio><email xlink:type="simple">luizak58@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2883-6307</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мучукова</surname><given-names>О. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Muchukova</surname><given-names>O. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., ассистент кафедры внутренних болезней с курсом клинической иммунологии;врач аллерголог-иммунолог поликлиники,</p><p>г. Чебоксары, Чувашская Республика</p></bio><bio xml:lang="en"><p>PhD (Medicine), Assistant Professor, Department of Internal Diseases with a Course of Clinical Immunology;</p><p>Clinical Allergologist/Immunologist, Ambulance Department,</p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">olgahealth@yandex.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2368-5084</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Луткова</surname><given-names>Т. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Lutkova</surname><given-names>T. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., доцент кафедры внутренних болезней с курсом клинической иммунологии,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>PhD (Medicine), Associate Professor, Department of Internal Diseases with a Course of Clinical Immunology,</p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">lts21@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2277-9425</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кудряшов</surname><given-names>С. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Kudryashov</surname><given-names>S. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., доцент кафедры внутренних болезней с курсом клинической иммунологии,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>PhD (Medicine), Associate Professor, Department of Internal Diseases with a Course of Clinical Immunology, </p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">medicpro21@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6470-7724</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Журавлева</surname><given-names>Н. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zhuravleva</surname><given-names>N. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., доцент кафедры внутренних болезней с курсом клинической иммунологии,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>PhD (Medicine), Associate Professor, Department of Internal Diseases with a Course of Clinical Immunology,</p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">zhuravlevanv@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1808-6845</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ухтерова</surname><given-names>Н. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Ukhterova</surname><given-names>N. D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., доцент кафедры внутренних болезней с курсом клинической иммунологии,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>PhD (Medicine), Associate Professor, Department of Internal Diseases with a Course of Clinical Immunology,</p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">55dd@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6962-4266</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гаврилова</surname><given-names>Э. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Gavrilova</surname><given-names>E. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., доцент кафедры внутренних болезней с курсом клинической иммунологии,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>PhD (Medicine), Associate Professor, Department of Internal Diseases with a Course of Clinical Immunology, </p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">ges2201@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3658-6579</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сидоров</surname><given-names>И. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Sidorov</surname><given-names>I. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., врач отделения оториноларингологии и челюстно-лицевой хирургии,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>PhD, MD (Medicine), Clinician, Department of Otorhinolaryngology and Maxillofacial Surgery, </p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">ivansv68@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7069-5029</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Одинцова</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Odintsova</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>студентка,</p><p>г. Чебоксары</p></bio><bio xml:lang="en"><p>Student, </p><p>Cheboksary, Chuvash Republic</p></bio><email xlink:type="simple">anastasia.od.99@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Чувашский государственный университет имени И.Н. Ульянова»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I. Ulyanov Chuvash State University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГБОУ ВО «Чувашский государственный университет имени И.Н. Ульянова»;&#13;
БУ «Республиканская клиническая больница» Министерства здравоохранения Чувашской Республики</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I. Ulyanov Chuvash State University;&#13;
Republican Clinical Hospital, Cheboksary</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>БУ «Республиканская детская клиническая больница» Министерства здравоохранения Чувашской Республики</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Republican Pediatric Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>24</day><month>04</month><year>2021</year></pub-date><volume>23</volume><issue>2</issue><fpage>353</fpage><lpage>368</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Карзакова Л.М., Мучукова О.М., Луткова Т.С., Кудряшов С.И., Журавлева Н.В., Ухтерова Н.Д., Гаврилова Э.С., Сидоров И.А., Одинцова А.В., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Карзакова Л.М., Мучукова О.М., Луткова Т.С., Кудряшов С.И., Журавлева Н.В., Ухтерова Н.Д., Гаврилова Э.С., Сидоров И.А., Одинцова А.В.</copyright-holder><copyright-holder xml:lang="en">Karzakova L.M., Muchukova O.M., Lutkova T.S., Kudryashov S.I., Zhuravleva N.V., Ukhterova N.D., Gavrilova E.S., Sidorov I.A., Odintsova A.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://www.mimmun.ru/mimmun/article/view/2148">https://www.mimmun.ru/mimmun/article/view/2148</self-uri><abstract><p>В последние годы первичные иммунодефициты из разряда редко встречающихся заболеваний переходят в число заболеваний, с которыми может встретиться врач любой специальности. Первый случай первичных иммунодефицитных заболеваний (ПИДЗ) был выявлен в Чувашии в 1993 г. С тех пор на кафедре внутренних болезней с курсом клинической иммунологии Чувашского государственного университета имени И.Н. Ульянова ведется регистрация всех диагностированных в регионе случаев ПИДЗ с внесением их в Республиканский регистр ПИДЗ. Целью настоящего исследования явилось изучение эпидемиологических показателей, клинико-лабораторных проявлений ПИДЗ на территории Чувашии. Материалом исследования служили данные о больных, полученные в ходе ретроспективного анализа 85 историй болезни больных ПИДЗ, находившихся на стационарном лечении в различных отделениях БУ «Республиканская клиническая больница» и БУ «Городская детская клиническая больница» Минздрава Чувашии в 2000-2019 гг., а также 49 амбулаторных карт пациентов, включенных в региональный регистр ПИДЗ. Диагностику различных форм ПИДЗ осуществляли в соответствии с критериями, разработанными Европейским Обществом по имму нодефицитам и Панамериканской группой по иммунодефицитам (1999). Результаты исследования показали, что частота распространенности ПИДЗ в регионе составляет 3,4:100 000. Заболеваемость наиболее распространенной формы ПИДЗ в регионе – общей вариабельной иммунной недостаточности (ОВИН) – составила 1,58 на 100 000 населения. Средний возраст на момент постановки диагноза ОВИН у больных Чувашии равнялся 30,4±16,1 годам, возраст дебюта ОВИН – 11,3±15,0 годам. Задержка постановки диагноза с момента клинической манифестации ОВИН составила в регионе в среднем 17,9 лет. На момент выставления диагноза ОВИН у больных обнаруживалось выраженное снижение уровней иммуноглобулинов 3-х или 2-х классов (IgG и IgА) и содержания в периферической крови Т-хелперных клеток (CD3+CD4+). Показатель распространенности селективного дефицита IgA, проявляющегося клинически, составил 0,83 на 100 000 населения региона, частота встречаемости бессимптомной формы данного ПИДЗ – 1:167. У пациентов с селективным дефицитом IgА обнаруживались нарушения в Т-клеточной системе, проявляющиеся в уменьшении относительного числа цитотоксических Т-клеток. Уровни IgG и IgМ оказались повышены. Возраст постановки диагноза X-сцепленной агаммаглобулинемии в регионе составил 3,5±3,0 года. Помимо нарушения в гуморальном звене адаптивного иммунитета у детей с данным заболеванием выявлялось уменьшение абсолютного числа Т-лимфоцитов. В заключение приведено описание нарушения поствакцинального иммунитета у беременной пациентки с ОВИН, бессимптомное течение которой привело к ложной трактовке показателей серологических маркеров ТОРЧ-инфекций и ошибкам в тактике ведения беременности.</p></abstract><trans-abstract xml:lang="en"><p>In recent years, primary immunodeficiencies have turned from the class of rare diseases to the category of more common disorders which may be encountered by doctors of any clinical discipline. The first case of primary immunodeficiency disorder (PID) in Chuvashia was detected in 1993. Since that time, the Department of Internal Diseases with the Course of Clinical Immunology at the I. Ulyanov Chuvash State University registered all the cases of PID diagnosed in the region, introducing them into the Republican Registry of PID. The study was aimed for searching epidemiological indexes, clinical and laboratory manifestations of PID in Chuvash region. The study was based on the patient data obtained by retrospective analysis of 85 case histories of PID patients, treated at different departments of the Republican Clinical Hospital, and the City Chuvash Pediatric Clinical Hospital of Public Health Ministry in 2000-2019, as well as on 49 outpatient records of the patients included into the Regional PID Registry. Various forms of PIDs were diagnosed according to the criteria developed by the European Society for Immunodeficiency and the Pan-American Group on Immunodeficiency (1999). The results of this study showed that the incidence of PID in the Chuivash Region is 3.4:100,000. The incidence of common variable immune deficiency (CVID), the most common form of PID in the region, was 1.58 per 100,000 population. The average age at the time of CVID diagnosis in Chuvash patients was 30.4±16.1 years, and the age of CVID debut was 11.3±15.0 years. The delay in proper diagnosis from the moment of clinical manifestation of CVID was, on average, 17.9 years in the region. At the time of CVID diagnosis, the patients showed marked decrease in the levels of 3 or 2 immunoglobulin classes (IgG and IgA), and T-helper cell contents (CD3+CD4+) in peripheral blood. Prevalence of selective IgA deficiency with сlinical symptoms was 0.83 per 100,000 population of the region, and the incidence of the asymptomatic form of this PID was 1 : 167. In patients with selective IgA deficiency, there were also disorders in the T cell system manifesting as decreased relative number of cytotoxic T-cells as well as elevated IgG and IgM levels. The age of diagnosis of X-linked agammaglobulinemia in the region was 3.5±3.0 years. In addition to disturbances of humoral adaptive immunity in children with this disease, a decrease in absolute T cell numbers was detected. In conclusion, the article describes disturbances of postvaccinal immunity in a pregnant patient with CVID, with asymptomatic clinical course, thus leading to false interpretation of the serological markers of TORCH infections and wrong strategy of pregnancy management.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>регистр первичных иммунодефицитов</kwd><kwd>ОВИН</kwd><kwd>селективный дефицит IgA</kwd><kwd>х-сцепленная  агаммаглобулинемия</kwd><kwd>поствакцинальный иммунитет</kwd></kwd-group><kwd-group xml:lang="en"><kwd>register of primary immunodeficiency</kwd><kwd>CVID</kwd><kwd>selective IgA deficiency</kwd><kwd>X-linked agammaglobulinaemia</kwd><kwd>postvaccinal immunity</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Авторы выражают благодарность студентам медицинского факультета ФГБОУ ВО «Чувашский государственный университет имени И.Н. Ульянова»: Мельниковой Марии Алексеевне, Шошориной Марии Сергеевне и Егорову Льву Валерьевичу за внесение базы данных пациентов Чувашии с различными формами ПИДЗ в таблицу Excel для проведения статистической обработки.</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Карзакова Л.М., Ванькина Н.Н., Борисова Л.В., Мучукова О.М., Андреева И.И. Изучение иммунного статуса населения, проживающего в промышленно насыщенной зоне Чувашии. В кн.: Артемьева Е.Г., Батраков С.Н., ред. Иммунодефицитные состояния в клинике внутренних болезней. Чебоксары: Изд-во Чуваш. ун-та, 1999. С. 38-41.</mixed-citation><mixed-citation xml:lang="en">Karzakova L.M., Vankina N.N., Borisova L.V., Muchukova O.M., Andreeva I.I. Study of the immune status of the population living in the industrially saturated zone of Chuvashia. 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