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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">16969</article-id><article-id pub-id-type="doi">10.36691/RJA16969</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Original studies</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Оригинальные исследования</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">Subcutaneous immunoglobulin 16.5 % given by the rapid push method in patients with inborn errors of immunity: a prospective multicenter study of quality of life</article-title><trans-title-group xml:lang="ru"><trans-title>Оценка качества жизни на фоне заместительной терапии иммуноглобулином для подкожного введения 16,5 % у пациентов с врожденным дефектом иммунной системы: результаты мультицентрового проспективного исследования</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9399-6534</contrib-id><name-alternatives><name xml:lang="en"><surname>Avedova</surname><given-names>Asmik Ya.</given-names></name><name xml:lang="ru"><surname>Аведова</surname><given-names>Асмик Яковлевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>asya.avedova95@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9083-4783</contrib-id><name-alternatives><name xml:lang="en"><surname>Deripapa</surname><given-names>Elena V.</given-names></name><name xml:lang="ru"><surname>Дерипапа</surname><given-names>Елена Васильевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Cand. 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xml:lang="en"><surname>Mashkovskaya</surname><given-names>Dina V.</given-names></name><name xml:lang="ru"><surname>Машковская</surname><given-names>Дина Валерьевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>mashkovskaya@child-hosp.ru</email><xref ref-type="aff" rid="aff5"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0007-8368-5250</contrib-id><name-alternatives><name xml:lang="en"><surname>Isakova</surname><given-names>Svetlana N.</given-names></name><name xml:lang="ru"><surname>Исакова</surname><given-names>Светлана Николаевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>hell_paradise@mail.ru</email><xref ref-type="aff" rid="aff16"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-9975-3632</contrib-id><contrib-id 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contrib-id-type="orcid">https://orcid.org/0000-0001-9826-9654</contrib-id><contrib-id contrib-id-type="spin">2028-9392</contrib-id><name-alternatives><name xml:lang="en"><surname>Shilova</surname><given-names>Tatiana V.</given-names></name><name xml:lang="ru"><surname>Шилова</surname><given-names>Татьяна Васильевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Cand. Sci. (Medicine)</p></bio><bio xml:lang="ru"><p>канд. мед. наук</p></bio><email>tanya920477@mail.ru</email><xref ref-type="aff" rid="aff18"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9755-8929</contrib-id><contrib-id contrib-id-type="spin">4140-9615</contrib-id><name-alternatives><name xml:lang="en"><surname>Zubova</surname><given-names>Elena S.</given-names></name><name xml:lang="ru"><surname>Зубова</surname><given-names>Елена Сергеевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>zubovaes@mail.ru</email><xref ref-type="aff" rid="aff19"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0008-7161-4540</contrib-id><name-alternatives><name xml:lang="en"><surname>Khabaeva</surname><given-names>Imani U.</given-names></name><name xml:lang="ru"><surname>Хабаева</surname><given-names>Имани Умаровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>el25kv@mail.ru</email><xref ref-type="aff" rid="aff20"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0001-6004-2558</contrib-id><name-alternatives><name xml:lang="en"><surname>Kitova</surname><given-names>Ekaterina V.</given-names></name><name xml:lang="ru"><surname>Китова</surname><given-names>Екатерина Владимировна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>Ek.Burova@yundex.ru</email><xref ref-type="aff" rid="aff21"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-7068-2601</contrib-id><name-alternatives><name xml:lang="en"><surname>Mandzhieva</surname><given-names>Anastasia G.</given-names></name><name xml:lang="ru"><surname>Манджиева</surname><given-names>Анастасия Геннадьевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>mandzhieva81@bk.ru</email><xref ref-type="aff" rid="aff22"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4321-7301</contrib-id><name-alternatives><name xml:lang="en"><surname>Starikova</surname><given-names>Svetlana Yu.</given-names></name><name xml:lang="ru"><surname>Старикова</surname><given-names>Светлана Юрьевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>Starikowa17@mail.ru</email><xref ref-type="aff" rid="aff23"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3403-4447</contrib-id><contrib-id contrib-id-type="spin">4893-7250</contrib-id><name-alternatives><name xml:lang="en"><surname>Pavlova</surname><given-names>Tatiana B.</given-names></name><name xml:lang="ru"><surname>Павлова</surname><given-names>Татьяна Борисовна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Cand. Sci. (Medicine)</p></bio><bio xml:lang="ru"><p>канд. мед. наук</p></bio><email>tabopav@rambler.ru</email><xref ref-type="aff" rid="aff24"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0005-1642-4398</contrib-id><name-alternatives><name xml:lang="en"><surname>Tyulyakova</surname><given-names>Elvira N.</given-names></name><name xml:lang="ru"><surname>Тюлякова</surname><given-names>Эльвира Николаевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>tabopav@rambler.ru</email><xref ref-type="aff" rid="aff25"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4451-3233</contrib-id><contrib-id contrib-id-type="spin">2836-2349</contrib-id><name-alternatives><name xml:lang="en"><surname>Grachev</surname><given-names>Nikolai S.</given-names></name><name xml:lang="ru"><surname>Грачев</surname><given-names>Николай Сергеевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Medicine), Professor</p></bio><bio xml:lang="ru"><p>д-р мед. наук, профессор</p></bio><email>nick-grachev@yandex.ru</email><xref ref-type="aff" rid="aff1"/></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>MD, Dr. Sci. (Medicine), Professor</p></bio><bio xml:lang="ru"><p>д-р мед. наук, профессор</p></bio><email>shcher26@hotmail.com</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Dmitry Rogachev National Medical 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><aff-alternatives id="aff2"><aff><institution xml:lang="en">National Research Center Institute of Immunology</institution></aff><aff><institution xml:lang="ru">Государственный научный центр «Институт иммунологии»</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">Clinical City Hospital No. 52</institution></aff><aff><institution xml:lang="ru">Городская клиническая больница № 52</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">Children’s City Clinical Hospital No. 9 named after G.N. Speransky</institution></aff><aff><institution xml:lang="ru">Детская городская клиническая больница № 9 имени Г.Н. Сперанского</institution></aff></aff-alternatives><aff-alternatives id="aff5"><aff><institution xml:lang="en">Republican Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Республиканская детская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff6"><aff><institution xml:lang="en">Stavropol State Medical University</institution></aff><aff><institution xml:lang="ru">Ставропольский государственный медицинский университет</institution></aff></aff-alternatives><aff-alternatives id="aff7"><aff><institution xml:lang="en">Regional Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Областная детская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff8"><aff><institution xml:lang="en">Altai State Medical University</institution></aff><aff><institution xml:lang="ru">Алтайский государственный медицинский университет</institution></aff></aff-alternatives><aff-alternatives id="aff9"><aff><institution xml:lang="en">Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhletsova</institution></aff><aff><institution xml:lang="ru">Архангельская областная детская клиническая больница имени П.Г. Выжлецова</institution></aff></aff-alternatives><aff-alternatives id="aff10"><aff><institution xml:lang="en">Nizhny Novgorod Regional Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Нижегородская детская областная клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff11"><aff><institution xml:lang="en">First Saint Petersburg State Medical University named after I.P. Pavlov</institution></aff><aff><institution xml:lang="ru">Первый Санкт-Петербургский государственный медицинский университет имени И.П. Павлова</institution></aff></aff-alternatives><aff-alternatives id="aff12"><aff><institution xml:lang="en">First Saint Petersburg State Medical University named after I.P. Pavlov, Saint Petersburg</institution></aff><aff><institution xml:lang="ru">Первый Санкт-Петербургский государственный медицинский университет имени И.П. Павлова</institution></aff></aff-alternatives><aff-alternatives id="aff13"><aff><institution xml:lang="en">Voronezh Regional Children’s Clinical Hospital No. 1</institution></aff><aff><institution xml:lang="ru">Воронежская областная детская клиническая больница № 1</institution></aff></aff-alternatives><aff-alternatives id="aff14"><aff><institution xml:lang="en">Republican Children’s Clinical Hospital named after E.P. Glinka</institution></aff><aff><institution xml:lang="ru">Республиканская детская клиническая больница имени Е.П. Глинки</institution></aff></aff-alternatives><aff-alternatives id="aff15"><aff><institution xml:lang="en">Regional Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Детская республиканская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff16"><aff><institution xml:lang="en">Research Institute of Fundamental and Clinical Immunology</institution></aff><aff><institution xml:lang="ru">Научно-исследовательский институт фундаментальной и клинической иммунологии</institution></aff></aff-alternatives><aff-alternatives id="aff17"><aff><institution xml:lang="en">Children’s Republican Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Детская республиканская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff18"><aff><institution xml:lang="en">Chelyabinsk Regional Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Челябинская областная детская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff19"><aff><institution xml:lang="en">Children’s Regional Hospital</institution></aff><aff><institution xml:lang="ru">Краевая детская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff20"><aff><institution xml:lang="en">Regional Children’s Clinical Hospital named after N.N. Silishcheva</institution></aff><aff><institution xml:lang="ru">Областная детская клиническая больница имени Н.Н. Силищевой</institution></aff></aff-alternatives><aff-alternatives id="aff21"><aff><institution xml:lang="en">Republican Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Республиканская клиническая инфекционная больница</institution></aff></aff-alternatives><aff-alternatives id="aff22"><aff><institution xml:lang="en">Regional Clinical Hospital No. 1</institution></aff><aff><institution xml:lang="ru">Областная клиническая больница № 1</institution></aff></aff-alternatives><aff-alternatives id="aff23"><aff><institution xml:lang="en">City Children’s Clinical Hospital No. 2 named after V.P. Bisyarina</institution></aff><aff><institution xml:lang="ru">Городская детская клиническая больница № 2 имени В.П. Бисяриной</institution></aff></aff-alternatives><aff-alternatives id="aff24"><aff><institution xml:lang="en">Irkutsk State Regional Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Иркутская государственная областная детская клиническая больница</institution></aff></aff-alternatives><aff-alternatives id="aff25"><aff><institution xml:lang="en">Kaluga Regional Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Калужская областная детская клиническая больница</institution></aff></aff-alternatives><pub-date date-type="preprint" iso-8601-date="2024-12-20" publication-format="electronic"><day>20</day><month>12</month><year>2024</year></pub-date><pub-date date-type="pub" iso-8601-date="2024-12-28" publication-format="electronic"><day>28</day><month>12</month><year>2024</year></pub-date><volume>21</volume><issue>4</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>451</fpage><lpage>468</lpage><history><date date-type="received" iso-8601-date="2024-09-11"><day>11</day><month>09</month><year>2024</year></date><date date-type="accepted" iso-8601-date="2024-11-08"><day>08</day><month>11</month><year>2024</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2024, Pharmarus Print Media</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2024, Фармарус Принт Медиа</copyright-statement><copyright-year>2024</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="2027-01-28"/></permissions><self-uri xlink:href="https://rusalljournal.ru/raj/article/view/16969">https://rusalljournal.ru/raj/article/view/16969</self-uri><abstract xml:lang="en"><p><bold>BACKGROUND:</bold> Replacement therapy with immunoglobulin preparations is the basic therapy for patients with impaired antibody formation in the context of inborn errors of immunity. The introduction of replacement therapy with subcutaneous immunoglobulin (SCIG) into clinical immunological practice has allowed patients with inborn errors of immunity, regardless of age, to carry out therapy independently at home. This circumstance has played a leading role in improving the quality of life of patients.</p> <p><bold>AIM:</bold> To assess the quality of life of patients and adherence to therapy during the transition from intravenous immunoglobulin replacement therapy to SCIG therapy.</p> <p><bold>MATERIALS AND METHODS:</bold> A total of 233 patients of different ages with inborn errors of immunity were enrolled in the study. All patients were switched from intravenous immunoglobulin to SCIG 16.5 % therapy. SCIG was administered every 7–10 days for at least 12 months via the rapid push method. Assessment of quality of life and adherence to SCIG therapy was carried out 6 and 12 months after the start of therapy using a questionnaire.</p> <p><bold>RESULTS:</bold> Based on the results of the assessment of the quality of life of patients using a questionnaire, patients of all age groups under 18 years and their parents demonstrated improvement in the social sphere, academic performance at school, and physical activity against the background of switching to SCIG. According to the survey, 84 % of patients did not experience technical difficulties and 83 % wanted to continue the SCIG infusion.</p> <p><bold>CONCLUSION</bold><italic>:</italic> Performing manual infusion of SCIG at home has a positive effect on the quality of life of patients and helps improve adherence to therapy.</p></abstract><trans-abstract xml:lang="ru"><p><bold>Обоснование.</bold> Заместительная терапия препаратами иммуноглобулинов является базовой терапией пациентов с нарушением антителообразования в рамках врожденного дефекта иммунной системы. Внедрение в клиническую иммунологическую практику заместительной терапии препаратами иммуноглобулинов для подкожного введения (ПКИГ) позволило пациентам с врожденным дефектом иммунной системы независимо от возраста получать терапию самостоятельно в домашних условиях. Это сыграло ведущую роль в улучшении качества жизни пациентов.</p> <p><bold>Цель исследования</bold> – оценка качества жизни пациентов и приверженности к терапии на фоне перехода с заместительной терапии внутривенным иммуноглобулином на ПКИГ.</p> <p><bold>Материалы и методы.</bold> В исследование первоначально включены 233 пациента с врожденным дефектом иммунной системы разных возрастных групп. Все пациенты рекрутированы с заместительной терапии внутривенным иммуноглобулином на терапию ПКИГ 16,5 % (Cutaquig). Терапия ПКИГ проводилась пациентами и/или их родителями методом rapid push (быстрые толчки) с интервалом 1 раз в 7–10 дней на протяжении 12 мес. Оценка качества жизни и приверженности к терапии ПКИГ проводилась через 6 и 12 мес от начала лечения методом анкетирования.</p> <p><bold>Результаты.</bold> Результаты оценки качества жизни методом анкетирования пациентов всех возрастных групп младше 18 лет и их родителей продемонстрировали улучшение в социальной сфере, успеваемости в школе и физической активности на фоне переключения на ПКИГ. Данные опроса пациентов и/или их родителей показали, что 84 % пациентов не имели технических сложностей при самостоятельном введении препарата ручным методом и 83 % предпочли продолжить терапию ПКИГ.</p> <p><bold>Заключение.</bold> Выполнение инфузии ПКИГ ручным методом в домашних условиях положительно сказывается на качестве жизни пациентов и способствует улучшению приверженности к терапии.</p></trans-abstract><kwd-group xml:lang="en"><kwd>inborn error of immunity</kwd><kwd>subcutaneous immunoglobulin</kwd><kwd>intravenous immunoglobulin</kwd><kwd>quality of life</kwd><kwd>rapid push</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>врожденный дефект иммунитета</kwd><kwd>иммуноглобулин для подкожного введения</kwd><kwd>иммуноглобулин для внутривенного введения</kwd><kwd>качество жизни</kwd><kwd>ручной метод</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">Cunningham-Rundles C. 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