rospedРоссийский педиатрический журнал имени М.Я. СтуденикинаM.Ya. Studenikin Russian Pediatric JournalФГАУ «НМИЦ здоровья детей» Минздрава России10.46563/1560-9561-2023-26-6-455-458oqipgtrosped-387Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCLINICAL CASEПервичное иммунодефицитное состояние у ребёнка 9 летPrimary immunodeficiency in the 9-year childhttps://orcid.org/0009-0005-3836-0428АлискандиеваЗулейха АлаудиновнаAliskandievaZuleykha A.

Канд. мед. наук, доцент каф. факультетской и госпитальной педиатрии ФГБОУ ВО «Дагестанский государственный медицинский университет

e-mail: aliskandieva@mail.ru

MD, PhD, Associate Prof. of the Department of Faculty and Hospital Pediatrics, Pediatrics of the FPC and Teaching Staff of the Dagestan State Medical University, Dagestan State Medical University, Makhachkala, 367000, Russian Federation

e-mail: aliskandieva@mail.ru

aliskandieva@mail.ruhttps://orcid.org/0000-0002-6023-7726АлискандиевАлаудин МагомедовичAliskandievAlaudin M.

Доктор мед. наук, проф., зав. каф. факультетской и госпитальной педиатрии ФГОУ ВО ДГМУ Минздрава России

e-mail: alaudin@rambler.ru

alaudin@rambler.ruhttps://orcid.org/0009-0009-8495-1017ИдрисоваРахмат МагомедовнаIdrisovaRakhmat M.

Канд. мед. наук, врач высшей категории, ассистент каф. педиатрии ФГБОУ ВО ДГМУ Минздрава России, зав. отделением МСКТ МРТ ДРКБ

e-mail: rayka-9999@mail.ru

rayka-9999@mail.ruhttps://orcid.org/0009-0007-8820-3839ИзраиловМагомед ИсрапиловичIzrailovMagomed I.

Канд. мед. наук, доцент, зав. каф. педиатрии ФПК и ППС ФГБОУ ВО ДГМУ Минздрава России

e-mail: izrailov74@mail.ru

izrailov74@mail.ruhttps://orcid.org/0009-0009-3927-271XАмироваКистаман МурадовнаAmirovaKistaman М.

Клинический ординатор, ФГБОУ ВО ДГМУ Минздрава России

e-mail: kistamanamirova@mail.ru

kistamanamirova@mail.ruФГБОУ ВО «Дагестанский государственный медицинский университет»Dagestan State Medical University202306012024266455458Copyright © Алискандиева З.А., Алискандиев А.М., Идрисова Р.М., Израилов М.И., Амирова К.М., 20242024Алискандиева З.А., Алискандиев А.М., Идрисова Р.М., Израилов М.И., Амирова К.М.Aliskandieva Z.A., Aliskandiev A.M., Idrisova R.M., Izrailov M.I., Amirova K.М.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.rosped.ru/jour/article/view/387

Первичные иммунодефицитные состояния представляют собой гетерогенную группу нарушений, вызванных дефектами различных звеньев иммунной системы, которые клинически проявляются в виде рецидивирующих инфекционных и аутоиммунных заболеваний разной степени тяжести. Описано собственное клиническое наблюдение для определения принципов диагностики и лечения первичных иммунодефицитных состояний у детей в современных условиях. Установлено, что средний возраст постановки диагноза иммунодефицитного состояния составляет в лучшем случае 30 дней, но часто это время увеличивается до нескольких лет. Представлены также данные регистра первичных иммунодефицитных состояний Республики Дагестан по диагностике и постоянной заместительной терапии иммунодефицитных состояний.

Участие авторов

Участие авторов:Алискандиева З.А. — концепция и дизайн исследования, статистическая обработка материала, написание текста;Алискандиева З.А., Идрисова Р.М., Амирова К.М. — сбор и обработка материала;Алискандиев А.М., Израилов М.И. — редактирование.Все соавторы — утверждение окончательного варианта статьи, ответственность за целостность всех частей статьи.

Финансирование

Финансирование. Исследование не имело финансовой поддержки.

Конфликт интересов

Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов.

Поступила 16

Поступила 16.10.2023Принята к печати 28.11.2023Опубликована 27.12.2023

 

 

Primary immunodeficiency conditions are a heterogeneous group of disorders caused by defects in various parts of the immune system, manifested in the form of recurrent infectious and autoimmune diseases of varying severity. The authors describe own clinical observation to determine the principles of diagnosis and treatment of primary immunodeficiency in children in modern conditions. The average age of diagnosis of an immunodeficiency condition has been established to be best over 30 days, but often this time increases to several years. The data of the enrolment of primary immunodeficiency states of the Republic of Dagestan on the diagnosis and permanent replacement therapy of immunodeficiency states is also presented.

Acknowledgment

Acknowledgment. The study had no sponsorship.

Conflict of interest

Conflict of interest. The authors declare no conflict of interest.

Contribution

Contribution:Aliskandieva Z.A. — concept and design of the study, statistical processing of the material;Idrisova R.M., Amirova K.M. — collection and processing of material;Aliskandieva Z.A. — writing the text;Aliskandiev A.M., Israilov M.I. — editing the text.All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Received

Received: October 16, 2023Accepted: November 28, 2023Published: December 27, 2023

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