REGIONAL REGISTER OF PRIMARY IMMUNODEFICIENCES OF THE REPUBLIC OF BASHKORTOSTAN

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Abstract

Primary immunodeficiency (PID) is a diverse group of genetically determined diseases, including more than 330 diseases. Early detection of PID and selection of adequate therapy aff ects the prognosis of the disease, improves the quality of life and life expectancy of patients significantly. PID register is the main tool for solving the tasks. Registration of patients with PID in the Republic of Bashkortostan (RB) is carried out on the basis of the Republican Children Clinical Hospital (RCCH) of Ufa and at March 2019, the number of registered patients increased to 223 people. Currently, 126 children are observed in the RCCH of Ufa, 43 patients have been transferred to the adult network. Distinctive features of the regional register of the RB are the presence of patients for whom the diagnosis of PID was set at autopsy and the absence of patients with Nij megen syndrome. The solution of current problems related to the diagnosis and treatment of PID is possible only with state support and teamwork of doctors of various specialties.

About the authors

R. M. Khairullina

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Author for correspondence.
Email: fake@neicon.ru

Prof., PhD, Head of the Immunology Laboratory with the Department of Clinical Immunology,

Ufa

Russian Federation

D. D. Prolygina

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Email: fake@neicon.ru

PhD, allergist-immunologist,

Ufa

Russian Federation

L. R. Kalmetyeva

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Email: fake@neicon.ru

PhD, allergist-immunologist, 

Ufa

Russian Federation

G. A. Davletbaeva

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Email: fake@neicon.ru

PhD, allergist-immunologist, 

Ufa

Russian Federation

I. A. Mirsayapova

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Email: fake@neicon.ru

PhD, allergist-immunologist,

Ufa

Russian Federation

G. U. Makarova

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Email: fake@neicon.ru

PhD, Head of the Allergology Department,

Ufa

Russian Federation

L. G. Ibragimova

Republican Children Clinical Hospital of the Ministry of Health of the Republic of Bashkortostan

Email: fake@neicon.ru

allergist-immunologist,

Ufa

Russian Federation

References

  1. Кузьменко Н. Б., Варламова Т. В., Мерсиянова И. В., Райкина Е. В., Бобрынина В. О., Щербина А. Ю. Молекулярно-генетическая диагностика первичных иммунодефицитных состояний. Вопросы гематологии / онкологии и иммунопатологии в педиатрии. 2016, 15 (1), 10–16.
  2. Щербина А. Ю., Пашанов Е. Д. Практическое руководство по детским болезням. Т. 8. Иммунология детского возраста. Медпрактика-М, М. 2006, 432 с.
  3. Сизякина Л. П., Андреева И. И. Создание регистра пациентов как эффективный инструмент диагностики первичных иммунодефицитов. Педиатрическая фармакология. 2013, 10 (5), 94–96.

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Copyright (c) 2019 Khairullina R.M., Prolygina D.D., Kalmetyeva L.R., Davletbaeva G.A., Mirsayapova I.A., Makarova G.U., Ibragimova L.G.

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This work is licensed under a Creative Commons Attribution 4.0 International License.
СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № 77 - 11525 от 04.01.2002.


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