Register of Patients as an Effective Method of Diagnosing Primary Immunodeficiencies

Сизякина, Л П; Andreeva, I I

doi:10.15690/pf.v10i5.831

Cited by 3 publications

(2 citation statements)

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“…Отсутствие точного диагноза у таких больных озна-чает безвозвратную потерю времени для назначения адекватной патогенетически обоснованной терапии, раз-витие необратимых повреждений внутренних органов и, как следствие, невозможность вернуть человека на иной, качественно новый уровень жизни [5,11].…”

Section: актуальностьunclassified

Neonatal Screening for Severe Combined Immune Deficiency in Russia: Glorious Future or Tomorrow’s Reality?

Дерябина¹,

Tuzankina²,

Власова³

et al. 2017

Vopr. sovr. pediatr.

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Section: актуальностьunclassified

Neonatal Screening for Severe Combined Immune Deficiency in Russia: Glorious Future or Tomorrow’s Reality?

Дерябина¹,

Tuzankina²,

Власова³

et al. 2017

Vopr. sovr. pediatr.

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“…Многие авторы отмечают важность ведения регистров для оценки эффективности оказания медицинской помощи, учета пациентов, страдающих редкими (орфанными) заболеваниями, которые требуют дорогостоящих методов лечения, таких как высокотехнологическая медицинская помощь или дорогостоящие препараты [9,10].…”

Section: введение / Introductionunclassified

Patient registers and account registries of compulsory medical insurance: integration and replaceability

2019

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Калькулятор прямых медицинских расходов, связанных с применением энзалутамида и абиратерона у больных метастатическим кастрационно-резистентным раком предстательной железы, ранее не получавших химиотерапию Моделирование влияния ферментозаместительной терапии на развитие жизнеугрожающих исходов у пациентов с болезнью Фабри Том 11 2018 FARMAKOEKONOMIKA Modern Pharmacoeconomic and Pharmacoepidemiology 2018 Vol. 11 No4 www.pharmacoeconomics.ru ISSN 2070-4909 (print) ISSN 2070-4933 (online) Данная интернет-версия статьи была скачана с сайта http://www.pharmacoeconomics.ru. Не предназначено для использования в коммерческих целях. Информацию о репринтах можно получить в редакции. Тел.: +7 (495) 649-54-95;эл. почта: info@irbis-1.ru.

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Republican registry of primary immune deficiencies in the chuvash republic and description of postvaccinal immunity disorders in a pregnant patient with common variable immune deficiency

et al. 2021

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In recent years, primary immunodeficiencies have turned from the class of rare diseases to the category of more common disorders which may be encountered by doctors of any clinical discipline. The first case of primary immunodeficiency disorder (PID) in Chuvashia was detected in 1993. Since that time, the Department of Internal Diseases with the Course of Clinical Immunology at the I. Ulyanov Chuvash State University registered all the cases of PID diagnosed in the region, introducing them into the Republican Registry of PID. The study was aimed for searching epidemiological indexes, clinical and laboratory manifestations of PID in Chuvash region. The study was based on the patient data obtained by retrospective analysis of 85 case histories of PID patients, treated at different departments of the Republican Clinical Hospital, and the City Chuvash Pediatric Clinical Hospital of Public Health Ministry in 2000-2019, as well as on 49 outpatient records of the patients included into the Regional PID Registry. Various forms of PIDs were diagnosed according to the criteria developed by the European Society for Immunodeficiency and the Pan-American Group on Immunodeficiency (1999). The results of this study showed that the incidence of PID in the Chuivash Region is 3.4:100,000. The incidence of common variable immune deficiency (CVID), the most common form of PID in the region, was 1.58 per 100,000 population. The average age at the time of CVID diagnosis in Chuvash patients was 30.4±16.1 years, and the age of CVID debut was 11.3±15.0 years. The delay in proper diagnosis from the moment of clinical manifestation of CVID was, on average, 17.9 years in the region. At the time of CVID diagnosis, the patients showed marked decrease in the levels of 3 or 2 immunoglobulin classes (IgG and IgA), and T-helper cell contents (CD3+CD4+) in peripheral blood. Prevalence of selective IgA deficiency with сlinical symptoms was 0.83 per 100,000 population of the region, and the incidence of the asymptomatic form of this PID was 1 : 167. In patients with selective IgA deficiency, there were also disorders in the T cell system manifesting as decreased relative number of cytotoxic T-cells as well as elevated IgG and IgM levels. The age of diagnosis of X-linked agammaglobulinemia in the region was 3.5±3.0 years. In addition to disturbances of humoral adaptive immunity in children with this disease, a decrease in absolute T cell numbers was detected. In conclusion, the article describes disturbances of postvaccinal immunity in a pregnant patient with CVID, with asymptomatic clinical course, thus leading to false interpretation of the serological markers of TORCH infections and wrong strategy of pregnancy management.

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Register of Patients as an Effective Method of Diagnosing Primary Immunodeficiencies

Cited by 3 publications

References 0 publications

Neonatal Screening for Severe Combined Immune Deficiency in Russia: Glorious Future or Tomorrow’s Reality?

Neonatal Screening for Severe Combined Immune Deficiency in Russia: Glorious Future or Tomorrow’s Reality?

Patient registers and account registries of compulsory medical insurance: integration and replaceability

Republican registry of primary immune deficiencies in the chuvash republic and description of postvaccinal immunity disorders in a pregnant patient with common variable immune deficiency

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