Immunoglobulin replacement therapy in patients with primary immunodeficiency diseases in Russia

Mukhinа, Anna; Abramova, Irina; Kuzmenko, N. B.; Родина, Ю. А.; Латышева, Т В; Latysheva, Elena A.; Костинова, А. М.; Ilina, Natalia; Катрышева, А. С.; Сударикова, Е. В.; Zimin, S.B.; Корсунский, А. А.; Barycheva, Lyudmila; Kubanova, Leila Timurovna; Кальметьева, Л. Р.; Пролыгина, Д. Д.; Давлетбаева, Г. А.; Шакирова, Л. Г.; Gracheva, Elena; Селезнева, О. С.; Кузьмичева, К. П.; Рычкова, О.А.; Demikhova, E.V.; Демихов, В. Г.; Фролова, И. О.; Timofeeva, Elena; Дурягина, С. Н.; Горенькова, А. В.; Власова, Е. В.; Laba, O.M.; Шахова, Н. В.; Кутлянцева, А. Ю.; Novichkova, Galina; Румянцев, А. Г.; Shcherbina, Anna

doi:10.24287/1726-1708-2020-19-4suppl-18-29

Voprosy gematologii/onkologii i immunopatologii v pediatrii

2021

DOI: 10.24287/1726-1708-2020-19-4suppl-18-29

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Immunoglobulin replacement therapy in patients with primary immunodeficiency diseases in Russia

Anna Mukhinа

Irina Abramova

N. B. Kuzmenko

et al.

Abstract: According to the data from Russian primary immunodeficiencies (PID) registry 71% of registered patients were treated with immunoglobulins (IG). Regular immunoglobulin substitutions were reported in 90% of patients with primary antibody deficiencies (PAD), 86% -with syndromic PID and 91% of patients with combined T and B cell defects. The study was supported by Academic

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Inborn errors of immunity: features of epidemiology and continuity of medical care in the Republic of Tatarstan

Skorokhodkina,

Luntsov,

Sibgatullina

et al. 2024

Russian Journal of Allergy

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Rationale: Inborn errors of the immune system represent the field of modern medicine, in particular clinical immunology, which demonstrates a high rate of accumulation of new data on this problem, which is realized in the current change in ideas about the prevalence of these diseases, revision of classification, and introduction of new treatment methods. Accordingly, many classical paradigms regarding primary immunodeficiencies (PID) have now been revised: taking into account more effective and early diagnosis, as well as modern treatment options, the prognosis of patient survival has largely changed in a positive direction, and the traditional idea of the predominance of patients with early PID childhood is no longer relevant. Objective: to assess the effectiveness and level of continuity of medical care in the Republic of Tatarstan based on an epidemiological analysis of a group of patients diagnosed with PID. Methods: analysis of the register of patients living in the Republic of Tatarstan with an established diagnosis of PID, observed from 1994 to 2023. A total of 240 patients were included in the study. Results: An epidemiological analysis of primary immunity errors in the Republic of Tatarstan (RT) revealed a prevalence of 6.0 per 100,000 population, with a high proportion of patients over 18 years of age (53%), with the largest group of patients having defects in antibody production (42 %). Conclusions: the increasing relevance of the problem of PID requires special attention to the organization of medical care for patients with PID, increasing continuity between specialized pediatric services and the adult network, as well as developing issues of interdisciplinary interaction

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Inborn errors of immunity: features of epidemiology and continuity of medical care in the Republic of Tatarstan

Skorokhodkina,

Luntsov,

Sibgatullina

et al. 2024

Russian Journal of Allergy

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show abstract

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Immunoglobulin replacement therapy in patients with primary immunodeficiency diseases in Russia

Cited by 1 publication

References 17 publications

Inborn errors of immunity: features of epidemiology and continuity of medical care in the Republic of Tatarstan

Inborn errors of immunity: features of epidemiology and continuity of medical care in the Republic of Tatarstan

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