Lower respiratory infection in patients with cystic fibrosis in Russian Federation according to the National Register, 2014

Красовский, С. А.; Амелина, Е. Л.; Kondratyeva, E.I.; Шагинян, И А; Чернуха, М Ю; Черняк, А. В.; Каширская, Н. Ю.; Аветисян, Л Р; Поликарпова, С. В.; Пивкина, Н. В.; Воронкова, А. Ю.; Usacheva, M.; Калинина, Ю. Э.; Kanukova, N.; Kozyreva, L.; Кондакова, Ю. А.; Konovalova, L.; Корнеева, Т. Ю.; Горинова, Ю. В.; Lavrova, A.E.; Ledeneva, Larisa; Ledneva, V.; Лягуша, Д. Э.; Golubtsova, O.; Мерзлова, Н. Б.; Mukhacheva, V.; Nikonova, V.; Неретина, А Ф; Новикова, О. Б.; Осипова, Елена Владимировна; Usacheva, O.V.; Pavlov, Pavlin; Пинегина, Ю. С.; Петрова, А И; Полякова, Ю. В.; Ибрагимова, Д. Ф.; Ponomareva, Tatiana A.; Protasova, T.; Пятеркина, О. Г.; Sergienko, D.F. Sergienko; Kapranov, N.

doi:10.18093/0869-0189-2016-26-4-421-435

Pulʹmonologiâ (Mosk.)

2016

DOI: 10.18093/0869-0189-2016-26-4-421-435

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Lower respiratory infection in patients with cystic fibrosis in Russian Federation according to the National Register, 2014

С. А. Красовский¹,

Е. Л. Амелина²,

E.I. Kondratyeva³

et al.

Abstract: РезюмеВажнейшим прогностическим фактором при муковисцидозе (МВ) является характер респираторной инфекции. В России разнообра зию микробного пейзажа нижних дыхательных путей у больных МВ посвящены единичные работы на ограниченных выборках боль ных. Общий анализ на большой группе пациентов в рамках единого регистра ранее не проводился. Цель. Выявление особенностей хро нической инфекции респираторного тракта у больных МВ в России по данным национального Регистра. Материалы и методы. Сведе ния о пациентах (n = 2 1… Show more

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Cited by 2 publications

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Early eradication of Pseudomonas aeruginosa infection in cystic fibrosis

Каширская¹,

Амелина²,

Красовский³

2017

Pulʹmonologiâ (Mosk.)

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Информация об авторах Каширская Наталия Юрьевна-д. м. н., профессор, главный научный сотрудник лаборатории генетической эпидемиологии Федерального государственного бюджетного научного учреждения «Медико генетический научный центр»; тел.:

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Early eradication of Pseudomonas aeruginosa infection in cystic fibrosis

Каширская¹,

Амелина²,

Красовский³

2017

Pulʹmonologiâ (Mosk.)

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Characteristics of CF patients over 18 years of age in the Republic of Tatarstan

Shakirova,

Gizatullina

2023

Medicinskij sovet

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Introduction. Cystic fibrosis is a hereditary disease characterized by the defeat of all exocrine glands, vital organs and systems. Currently, the life expectancy of patients with cystic fibrosis is increasing due to the development of new therapies.Goal. Analysis of the results of a comprehensive examination of patients with cystic fibrosis under the supervision of a pulmonologist of Republican Clinical Hospital (RCH) of the Ministry of Health of the Republic of Tatarstan.Materials and methods. The analysis of the database of patients with cystic fibrosis, older than 18 years, registered in the Regional Center of cystic fibrosis of the Republic of Tatarstan (RT).Results. As of April 2023, 47 patients over the age of 18 with a diagnosis of cystic fibrosis were registered in the RCH of the Ministry of Health of the Republic of Tatarstan. The diagnosis was made on the basis of a positive sweat sample and/or a genetic study. The age of patients is from 18 to 39 years (26.1 ± 1.1 years). Women – 49%, men – 51%. Median body weight – 51.5 kg, height – 165 cm, BMI – 18.05 kg/m2. 25 patients (53.2%) had a BMI of less than 18.5 kg/m2. Among patients in RT, the most common mutation is F508del. When assessing the microbiological profile of the respiratory tract, chronic infection was detected in 37 patients (78.7%). Ps. aeruginosa (66.0%) and S.aureus (35.6%) were the most frequently sown. The average FVC indicators were 72.6 ± 4.3% predicted values, the average FEV1 indicators were 61.6 ± 28.1% predicted values.Conclusion. On the territory of the Republic of Tatarstan in patients with cystic fibrosis, the F508del mutation was detected in 30 (66.7%) adult patients, 9 of whom receive targeted therapy elecsacaftor / tezacaftor / ivacaftor+ ivacaftor with a pronounced positive effect. The high incidence of P. aeruginosa infection (66.0%) and a decrease in respiratory function among adult patients indicate a more severe course of cystic fibrosis.

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Lower respiratory infection in patients with cystic fibrosis in Russian Federation according to the National Register, 2014

Cited by 2 publications

References 10 publications

Early eradication of Pseudomonas aeruginosa infection in cystic fibrosis

Early eradication of Pseudomonas aeruginosa infection in cystic fibrosis

Characteristics of CF patients over 18 years of age in the Republic of Tatarstan

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