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<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Russian Journal of Allergy</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Allergy</journal-title><trans-title-group xml:lang="ru"><trans-title>Российский Аллергологический Журнал</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1810-8830</issn><issn publication-format="electronic">2686-682X</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">1447</article-id><article-id pub-id-type="doi">10.36691/RJA1447</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Help to the practitioner</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>В помощь практикующему врачу</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Hereditary angioedema. Clinical guidelines. (D84.1)</article-title><trans-title-group xml:lang="ru"><trans-title>Наследственный ангиоотек. Клинические рекомендации (D84.1)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5339-5566</contrib-id><contrib-id contrib-id-type="spin">8451-3075</contrib-id><name-alternatives><name xml:lang="en"><surname>Bliznetz</surname><given-names>Elena A.</given-names></name><name xml:lang="ru"><surname>Близнец</surname><given-names>Елена Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Cand. Sci. (Med.), Senior Research Associate</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук, старший научный сотрудник</p></bio><email>bliznetzelena@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2427-1417</contrib-id><name-alternatives><name xml:lang="en"><surname>Viktorova</surname><given-names>Ekaterina A.</given-names></name><name xml:lang="ru"><surname>Викторова</surname><given-names>Екатерина Андреевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Cand. Sci.(Med.)</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук</p></bio><email>vikat.10@mail.ru</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7398-0562</contrib-id><contrib-id contrib-id-type="spin">1109-2810</contrib-id><name-alternatives><name xml:lang="en"><surname>Vishneva</surname><given-names>Elena A.</given-names></name><name xml:lang="ru"><surname>Вишнева</surname><given-names>Елена Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Med.)</p></bio><bio xml:lang="ru"><p>доктор медицинских наук</p></bio><email>vishneva.e@yandex.ru</email><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3841-4762</contrib-id><contrib-id contrib-id-type="spin">8866-4534</contrib-id><name-alternatives><name xml:lang="en"><surname>Dzhobava</surname><given-names>Eliso M.</given-names></name><name xml:lang="ru"><surname>Джобава</surname><given-names>Элисо Мурмановна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, профессор</p></bio><email>super.lis9@yandex.ru</email><xref ref-type="aff" rid="aff5"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3556-969X</contrib-id><contrib-id contrib-id-type="spin">6715-5650</contrib-id><name-alternatives><name xml:lang="en"><surname>Il'ina</surname><given-names>Natalya I.</given-names></name><name xml:lang="ru"><surname>Ильина</surname><given-names>Наталья Ивановна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Professor</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, профессор</p></bio><email>instimmun@yandex.ru</email><xref ref-type="aff" rid="aff6"/><xref ref-type="aff" rid="aff7"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1669-8621</contrib-id><name-alternatives><name xml:lang="en"><surname>Kuzmenko</surname><given-names>Natalia B.</given-names></name><name xml:lang="ru"><surname>Кузьменко</surname><given-names>Наталья Борисовна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Cand. Sci.(Med.)</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук</p></bio><email>plunge@list.ru</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1606-205X</contrib-id><contrib-id contrib-id-type="spin">2063-7973</contrib-id><name-alternatives><name xml:lang="en"><surname>Latysheva</surname><given-names>Elena A.</given-names></name><name xml:lang="ru"><surname>Латышева</surname><given-names>Елена Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Senior Research Associate</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, ведущий научный сотрудник</p></bio><email>ealat@mail.ru</email><xref ref-type="aff" rid="aff8"/><xref ref-type="aff" rid="aff9"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1508-0640</contrib-id><contrib-id contrib-id-type="spin">8929-7644</contrib-id><name-alternatives><name xml:lang="en"><surname>Latysheva</surname><given-names>Tatiana V.</given-names></name><name xml:lang="ru"><surname>Латышева</surname><given-names>Татьяна Васильевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Professor</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, профессор</p></bio><email>tvlat@mail.ru</email><xref ref-type="aff" rid="aff9"/><xref ref-type="aff" rid="aff10"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6432-394X</contrib-id><contrib-id contrib-id-type="spin">7944-5159</contrib-id><name-alternatives><name xml:lang="en"><surname>Manto</surname><given-names>Irina A.</given-names></name><name xml:lang="ru"><surname>Манто</surname><given-names>Ирина Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Research Associate</p></bio><bio xml:lang="ru"><p>Доктор медицины, научный сотрудник, научный сотрудник</p></bio><email>irina.manto@yandex.ru</email><xref ref-type="aff" rid="aff8"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2209-7531</contrib-id><contrib-id contrib-id-type="spin">1312-2147</contrib-id><name-alternatives><name xml:lang="en"><surname>Namazova-Baranova</surname><given-names>Leyla S.</given-names></name><name xml:lang="ru"><surname>Намазова-Баранова</surname><given-names>Лейла Сеймуровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, профессор</p></bio><email>leyla.s.namazova@gmail.com</email><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3162-2510</contrib-id><contrib-id contrib-id-type="spin">3363-6170</contrib-id><name-alternatives><name xml:lang="en"><surname>Nenasheva</surname><given-names>Natalia M.</given-names></name><name xml:lang="ru"><surname>Ненашева</surname><given-names>Наталья Михайловна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p> Professor, Doctor of Medical Sciences</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, профессор</p></bio><email>1444031@gmail.com</email><xref ref-type="aff" rid="aff11"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5039-8473</contrib-id><contrib-id contrib-id-type="spin">9722-7961</contrib-id><name-alternatives><name xml:lang="en"><surname>Pampura</surname><given-names>Alexander N.</given-names></name><name xml:lang="ru"><surname>Пампура</surname><given-names>Александр Николаевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Professor. Veltischev Research and Clinical Institute for Pediatrics</p></bio><bio xml:lang="ru"><p>доктор медицинских наук, профессор.</p> <p>Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева</p></bio><email>apampura1@mail.ru</email><xref ref-type="aff" rid="aff12"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-0105-1833</contrib-id><contrib-id contrib-id-type="spin">6453-3097</contrib-id><name-alternatives><name xml:lang="en"><surname>Polyakov</surname><given-names>Aleksander V.</given-names></name><name xml:lang="ru"><surname>Поляков</surname><given-names>Александр Владимирович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Dr. Sci. (Bio), Professor</p></bio><bio xml:lang="ru"><p>доктор биологических наук, профессор</p></bio><email>apol@dnalab.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3678-7939</contrib-id><contrib-id contrib-id-type="spin">5508-1689</contrib-id><name-alternatives><name xml:lang="en"><surname>Selimzianova</surname><given-names>Lilia R.</given-names></name><name xml:lang="ru"><surname>Селимзянова</surname><given-names>Лилия Робертовна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Cand. Sci.(Med.), Senior Research Associate</p></bio><bio xml:lang="ru"><p>кандидат медицинских наук, в.н.с. </p></bio><email>lilysir@mail.ru</email><xref ref-type="aff" rid="aff13"/><xref ref-type="aff" rid="aff14"/><xref ref-type="aff" rid="aff15"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3113-4939</contrib-id><contrib-id contrib-id-type="spin">6759-0031</contrib-id><name-alternatives><name xml:lang="en"><surname>Shcherbina</surname><given-names>Anna Yu.</given-names></name><name xml:lang="ru"><surname>Щербина</surname><given-names>Анна Юрьевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>professor, head of Immunology Department Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), PhD</p></bio><bio xml:lang="ru"><p>профессор, зав. отделением иммунологии НМИЦ ДГОИ, исполнительный директор Национального общества экспертов в области первичных иммунодефицитов, член Национального общества детских гематологов и онкологов, член Европейского общества иммунодефицитов, доктор медицинских наук</p></bio><email>shcher26@hotmail.com</email><xref ref-type="aff" rid="aff16"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Federal State Budgetary Institution Research Centre for Medical Genetics</institution></aff><aff><institution xml:lang="ru">Федеральное государственное бюджетное научное учреждение "Медико-генетический научный центр имени академика Н.П. Бочкова"</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology</institution></aff><aff><institution xml:lang="ru">Федеральное государственное бюджетное учреждение "Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева" Министерства здравоохранения Российской Федерации</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences»</institution></aff><aff><institution xml:lang="ru">Федеральное государственное бюджетное учреждение здравоохранения Центральная клиническая больница Российской академии наук</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">Pirogov Russian National Research Medical University</institution></aff><aff><institution xml:lang="ru">Федеральное государственное автономное образовательное учреждение высшего образования "Российский национальный исследовательский медицинский университет имени Н.И. Пирогова" Министерства здравоохранения Российской Федерации</institution></aff></aff-alternatives><aff-alternatives id="aff5"><aff><institution xml:lang="en">LLC group of clinics Crede Expert</institution></aff><aff><institution xml:lang="ru">ООО группа клиник Кредэ Эксперто</institution></aff></aff-alternatives><aff-alternatives id="aff6"><aff><institution xml:lang="en">Russian Assotiation of Allergology and Clinical Immunology</institution></aff><aff><institution xml:lang="ru">Государственный научный центр «Институт иммунологии» Федерального медико-биологического агентства</institution></aff></aff-alternatives><aff-alternatives id="aff7"><aff><institution xml:lang="en">National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia</institution></aff><aff><institution xml:lang="ru">Российская ассоциация аллергологов и клинических иммунологов</institution></aff></aff-alternatives><aff-alternatives id="aff8"><aff><institution xml:lang="en">National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia</institution></aff><aff><institution xml:lang="ru">Государственный научный центр «Институт иммунологии» Федерального медико-биологического агентства</institution></aff></aff-alternatives><aff-alternatives id="aff9"><aff><institution xml:lang="en">National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia</institution></aff><aff><institution xml:lang="ru">Государственный научный центр Институт иммунологии Федерального медико-биологического агентства</institution></aff></aff-alternatives><aff-alternatives id="aff10"><aff><institution xml:lang="en">Moscow State University of Medicine and Dentistry named after A.I. Evdokimov</institution></aff><aff><institution xml:lang="ru">Московский государственный медико-стоматологический университет имени А.И. Евдокимова</institution></aff></aff-alternatives><aff-alternatives id="aff11"><aff><institution xml:lang="en">Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГБОУ ДПО Российская медицинская академия непрерывного профессионального образования Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff12"><aff><institution xml:lang="en">Pirogov Russian National Research Medical</institution></aff><aff><institution xml:lang="ru">ФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова»</institution></aff></aff-alternatives><aff-alternatives id="aff13"><aff><institution xml:lang="en">Central Clinical Hospital of the Russian Academy of Sciences</institution></aff><aff><institution xml:lang="ru">Центральная клиническая больница Российской академии наук</institution></aff></aff-alternatives><aff-alternatives id="aff14"><aff><institution xml:lang="en">Pirogov Russian National Research Medical University</institution></aff><aff><institution xml:lang="ru">Российский национальный исследовательский медицинский университет имени Н.И. Пирогова</institution></aff></aff-alternatives><aff-alternatives id="aff15"><aff><institution xml:lang="en">The First Sechenov Moscow State Medical University (Sechenov University)</institution></aff><aff><institution xml:lang="ru">Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)</institution></aff></aff-alternatives><aff-alternatives id="aff16"><aff><institution xml:lang="en">Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева</institution></aff></aff-alternatives><pub-date date-type="preprint" iso-8601-date="2021-05-25" publication-format="electronic"><day>25</day><month>05</month><year>2021</year></pub-date><pub-date date-type="pub" iso-8601-date="2021-06-26" publication-format="electronic"><day>26</day><month>06</month><year>2021</year></pub-date><volume>18</volume><issue>2</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>77</fpage><lpage>114</lpage><history><date date-type="received" iso-8601-date="2021-04-14"><day>14</day><month>04</month><year>2021</year></date><date date-type="accepted" iso-8601-date="2021-04-19"><day>19</day><month>04</month><year>2021</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2021, Pharmarus Print Media</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2021, Фармарус Принт Медиа</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="en">Pharmarus Print Media</copyright-holder><copyright-holder xml:lang="ru">Фармарус Принт Медиа</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/" start_date="2023-07-06"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by-nc-nd/4.0/</ali:license_ref></license></permissions><self-uri xlink:href="https://rusalljournal.ru/raj/article/view/1447">https://rusalljournal.ru/raj/article/view/1447</self-uri><abstract xml:lang="en"><p>Hereditary angioedema is a rare, potentially life-threatening genetic disease that manifests with skin and mucous/submucosal swelling that occurs under the influence of bradykinin. The clinical guidelines for hereditary angioedema (code for the International Statistical Classification of Diseases and Related Health Problems D84.1) were developed in December 2020 by the Russian Association of Allergology and Clinical Immunology, Association of Medical Geneticists, National Association of Experts in the Field of Primary Immunodeficiencies, Union of Pediatricians of Russia. In December 2020, the project was approved at a meeting of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (December 10, 2020 No. 743/12).</p> <p>The clinical guidelines are devoted to optimizing the clinical care of patients with hereditary angioedema. The topic is relevant owing to the under-diagnosis of hereditary angioedema and insufficient awareness of doctors of various specialties about this problem, as well as the difficulties in managing this category of patients. The clinical guidelines contain information about the epidemiology, etiology, classification, pathogenesis, and common clinical manifestations of hereditary angioedema. Thus, the paper described actual diagnostic approaches and differential diagnosis of hereditary angioedema and established its diagnostic criteria. Medical care algorithms to patients with hereditary angioedema at the prehospital and hospital stages are also described. The principles of pharmacotherapy, including long-term prophylaxis, short-term prophylaxis, and acute therapy, for hereditary angioedema are also presented. Treatment regimens are stratified according to patients’ age and gender. Non-drug measures for the prevention hereditary angioedema attacks are considered. Sections were also added in the edition of the new clinical guidelines of hereditary angioedema. Clinical guidelines for hereditary angioedema are intended for practicing doctors of all specialties, students and teachers of medical universities, residents, graduate students of medical universities.</p></abstract><trans-abstract xml:lang="ru"><p>Наследственный ангиоотёк ― редкое, потенциально жизнеугрожающее генетически детерминированное заболевание, проявляющееся в виде отёков кожи и слизистых/подслизистых оболочек, возникающих под воздействием брадикинина. Российской ассоциацией аллергологов и клинических иммунологов, Ассоциацией медицинских генетиков, Национальной ассоциацией экспертов в области первичных иммунодефицитов, Союзом педиатров России были разработаны клинические рекомендации по наследственному ангиоотёку (код по Международной статистической классификации болезней и проблем, связанных со здоровьем, D84.1). В декабре 2020 года проект был утверждён на заседании научно-практического совета Министерства здравоохранения Российской Федерации (протокол от 10.12.2020 № 743/12).</p> <p>Представленные клинические рекомендации посвящены оптимизации помощи больным наследственным ангиоотёком. Актуальность темы обусловлена гиподиагностикой наследственного ангиоотёка и недостаточной осведомлённостью врачей различных специальностей о проблеме, а также многочисленными сложностями ведения данной категории больных.</p> <p>В клинических рекомендациях содержится информация об эпидемиологии, этиологии, классификации, патогенезе, характерных клинических проявлениях наследственного ангиоотёка. Подробно описаны современные подходы к диагностике и дифференциальной диагностике наследственного ангиоотёка, сформулированы критерии постановки диагноза. Изложен порядок оказания медицинской помощи пациентам с наследственным ангиоотёком на догоспитальном и госпитальном этапах. Отражены принципы фармакотерапии больных наследственным ангиоотёком, включающие долгосрочную профилактику, краткосрочную профилактику, купирование острых атак. Схемы лечения стратифицированы с учётом возраста и пола пациентов. Рассматриваются немедикаментозные мероприятия по профилактике обострений наследственного ангиоотёка.</p> <p>В новом издании клинических рекомендаций добавлены разделы с информацией для пациентов и паспорт больного наследственным ангиоотёком.</p> <p>Клинические рекомендации по наследственному ангиоотёку предназначены для практикующих врачей всех специальностей, студентов и преподавателей медицинских вузов, ординаторов, аспирантов медицинских вузов.</p></trans-abstract><kwd-group xml:lang="en"><kwd>angioedema</kwd><kwd>hereditary angioedema</kwd><kwd>C1-inhibitor</kwd><kwd>bradykinin</kwd><kwd>clinical guidelines</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>ангиоотёк</kwd><kwd>наследственный ангиоотёк</kwd><kwd>С1-ингибитор</kwd><kwd>брадикинин</kwd><kwd>клиничекие рекомендации</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">Maurer M. Magerl M, Ansotegui I, et al. 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