ssmuБюллетень сибирской медициныBulletin of Siberian Medicine1682-03631819-3684Siberian State Medical University, the Ministry of Healthcare of the Russian Federation10.20538/1682-0363-2019-3-144-154ssmu-2412Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEW AND LECTURESСтоматологические проявления первичных иммунодефицитовOral manifestations of primary immunodeficiencieshttps://orcid.org/0000-0002-1355-3048ДолгихМ. А.DolgikhM. A.

аспирант, 

620049, г. Екатеринбург, ул. Первомайская, 106

Postgraduate Student,

106, Pervomayskaya Str., Ekaterinburg, 620049

https://orcid.org/0000-0003-2763-9907БолковМ. А.BolkovM. A.

канд. мед. наук, ст. науч. сотрудник, 620049, г. Екатеринбург, ул. Первомайская, 106;

620002, г. Екатеринбург, ул. Мира, 19

PhD, Senior Researcher, 106, Pervomayskaya Str., Ekaterinburg, 620049;

19, Mira Str., Ekaterinburg, 620002

https://orcid.org/0000-0001-7496-0950ТузанкинаИ. А.TuzankinaI. A.

д-р мед. наук, заслуженный деятель науки Российской Федерации, профессор, гл. науч. сотрудник, 620049, г. Екатеринбург, ул. Первомайская, 106;

620002, г. Екатеринбург, ул. Мира, 19

DM, Honored Worker of Science of the Russian Federation, Professor, Сhief Researcher, 106, Pervomayskaya Str., Ekaterinburg, 620049;

19, Mira Str., Ekaterinburg, 620002

ituzan@yandex.ruhttps://orcid.org/0000-0003-3939-2956СаркисянН. Г.SarkisyanN. G.

канд. мед. наук., науч. сотрудник, 620049, г. Екатеринбург, ул. Первомайская, 106;

620002, г. Екатеринбург, ул. Мира, 19

PhD., Researcher, 106, Pervomayskaya Str., Ekaterinburg, 620049;

19, Mira Str., Ekaterinburg, 620002

https://orcid.org/0000-0001-9767-1932ОвсепянН. А.HovsepyanN. A.

аспирант,

620049, г. Екатеринбург, ул. Первомайская, 106

Postgraduate student, 

106, Pervomayskaya Str., Ekaterinburg, 620049

Институт иммунологии и физиологии Уральского отделения РАН (ИИФ УрО РАН)РоссияInstitute of Immunology and Physiology of the Ural Branch of the Russian Academy of Sciences (IIP UB RAS)Russian FederationИнститут иммунологии и физиологии Уральского отделения РАН (ИИФ УрО РАН); Уральский федеральный университет имени первого Президента России Б.Н. ЕльцинаРоссияInstitute of Immunology and Physiology of the Ural Branch of the Russian Academy of Sciences (IIP UB RAS); Federal State Autonomous Educational Institution of Higher Education “Ural Federal University named after the first President of Russia B.N. Yeltsin”Russian Federation201927102019183144154Copyright © Долгих М.А., Болков М.А., Тузанкина И.А., Саркисян Н.Г., Овсепян Н.А., 20192019Долгих М.А., Болков М.А., Тузанкина И.А., Саркисян Н.Г., Овсепян Н.А.Dolgikh M.A., Bolkov M.A., Tuzankina I.A., Sarkisyan N.G., Hovsepyan N.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://bulletin.ssmu.ru/jour/article/view/2412

Для понимания современного состояния проблемы стоматологических проявлений первичных иммунодефицитов проанализирована соответствующая зарубежная литература. В статье описаны стоматологические проявления многих первичных иммунодефицитов, которые могут быть как вторичным, так и основным симптомом. Представлены синдром тяжелого комбинированного иммунодефицита, гипер-IgE синдром, синдромы Вискотта – Олдрича, Ди Джорджи, дефицит STIM1 и дефицит ORAI1, дефицит NEMO и дефицит IκBα, общая вариабельная иммунная недостаточность, X-сцепленная агаммаглобулинемия, гипер-IgM синдром, селективный дефицит IgA, аутоиммунный лимфопролиферативный синдром, аутоиммунный полиýндокринный синдром 1-го типа, синдром Чедиака – Хигаши, дефицит CD70, синдромы тяжелой врожденной нейтропении, дефициты адгезии лейкоцитов, локализованный агрессивный пародонтит, синдром Папийона – Лефевра, хронический кожно-слизистый кандидоз, синдром Маршалла, гипер-IgD синдром, синдром Айкарди – Гутьереса 7-го типа, синдром херувизма, синдром CANDLE (хронический атипичный нейтрофильный дерматит с липодистрофией), PAPA (пиогенный артрит, гангренозная пиодермия и акне), хронический рецидивирующий мультифокальном остеомиелит, периодонтальный синдром Элерса – Данло, дефицит С1-ингибитора. Приведены данные о роли секреторных иммуноглобулинов, определяемых в слюнной жидкости.

To understand the current state of the issue of dental manifestations of primary immunodeficiencies, foreign literature on the problem has been analyzed. The article describes the dental manifestations of many primary immunodeficiencies, which can be both secondary and major symptoms. The article presents the data on the following syndromes: severe combined immunodeficiency, hyper IgE, Wiskott – Aldrich, DiGeorge, deficiency of STIM1 and ORAI1, NEMO deficiency and IκBα deficiency, common variable immunodeficiency, X-linked agammaglobulinemia, hyper IgM, selective IgA deficiency, autoimmune lymphoproliferative syndrome, autoimmune polyendocrine syndrome type 1, Chediak – Higashi syndrome, CD70 deficiency, severe congenital neutropenia syndromes, leukocyte adhesion deficiency, localized aggressive periodontitis, Papillon – Lefevre syndrome, chronic mucocutaneous candidiasis, Marshall syndrome, hyper IgD syndrome, Aicardi – Goutières syndrome type 7, cherubism syndrome, CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature), PAPA (pyogenic arthritis, pyoderma gangrenosum and acne), chronic recurrent multifocal osteomyelitis, periodontal Ehlers – Danlos syndrome, and C1 inhibitor deficiency. The role of secretory immunoglobulins of salivary fluid is described.

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The authors declare that there are no conflicts of interest present.