The dynamics of some health indicators in adult patients with cystic fibrosis, observed in the research institute of pulmonology in 2003-2018

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Abstract

Background: Evaluation of the main clinical and functional indicators and their relationship with the treatment and diagnostic care at different time periods can contribute to the development of further effective medical strategies and globally lead to improved care for cystic fibrosis patients.

Aim: An assessment of the dynamics of some clinical and functional indicators in adult CF patients, who have been observed ed Research Institute of Pulmonology during the last 15 years(2003–2018).

Methods: The comparative analysis was performed on the data of adult patients with cystic fibrosis, observed at 31.12.2003 and at the end of October of 2018. The group of patients in 2003 consists of 80 and in 2018 — of 667 patients. The comparative analysis between the groups was carried out according to the following indicators: age, survival, lung function, nutritional status, proportion of infection with pathogenic microflora, identification of mutations in the cystic fibrosis gene and diagnosis in adult patients.

Results: An increase in patient’s age, survival rate, the proportion of identified mutations in cystic fibrosis gene and infection of Burkholderia cepacia complex in the group of patients in 2018 was revealed. There were no differences in the state of lung function, nutritional status, the ratio of the living and dead patients and proportion of patients diagnosed in adulthood between groups.

Conclusion: The progress in the treatment through the introduction of modern methods and therapeutic programs leads to improved survival and an increase in the number of adult patients with cystic fibrosis.

About the authors

S. A. Krasovskiy

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia; D.D. Pletnev City Clinical Hospital, Moscow Healthcare Department; Research Centre of Medical Genetics

Author for correspondence.
Email: sa_krasovsky@mail.ru
ORCID iD: 0000-0001-9642-0947
SPIN-code: 3385-6489

канд. мед. наук, старший научный сотрудник лаборатории муковисцидоза; врач-пульмонолог 2-го пульмонологического отделения; старший научный сотрудник научно-клинического отдела муковисцидоза

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682; Moscow

E. L. Amelina

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia

Email: eamelina@mail.ru
ORCID iD: 0000-0002-5356-9415
SPIN-code: 6341-4662

канд. мед. наук, заведующая лабораторией муковисцидоза

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682

U. V. Gorinova

National Medical Research Center for Children’s Health

Email: ygorinova@yandex.ru
ORCID iD: 0000-0002-3881-3483

канд. мед. наук, врач-педиатр отделения муковисцидоза

Russian Federation, 2, b.1, Lomonosovsky prospekt, Moscow, 119991

A. V. Chernyak

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia

Email: achi2000@mail.ru
SPIN-code: 9328-6440

канд. мед. наук, заведующий лабораторией функциональных и ультразвуковых методов исследований

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682

M. V. Afanasjeva

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia

Email: usa-mariya@yandex.ru
SPIN-code: 7271-7408

научный сотрудник лаборатории муковисцидоза

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682

N. A. Krilova

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia

Email: nataliya_2007_78@mail.ru
ORCID iD: 0000-0002-3788-0887

научный сотрудник лаборатории муковисцидоза

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682

G. L. Shumkova

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia

Email: galina2212@yandex.ru
ORCID iD: 0000-0001-9076-2348
SPIN-code: 5053-3013

научный сотрудник лаборатории муковисцидоза

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682

O. G. Zonenko

Russian National Research Medical University named after N.I. Pirogov

Email: o.zonenko@mail.ru
ORCID iD: 0000-0002-9142-2318
SPIN-code: 7726-6224

ассистент кафедры госпитальной педиатрии имени академика В.А. Таболина 

Russian Federation, 1, Ostrovityanova street, Moscow, 117997

A. R. Tatarskiy

Research Institute of Pulmonology of the Federal Medical-Biological Agency of Russia

Email: alexey.tatarsky@gmail.com
SPIN-code: 9734-2366

докт. мед. наук, профессор, руководитель образовательного центра

Russian Federation, 28, Orekchovy Boulevard, Moscow, 115682 

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Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. Dynamics of the number of adult patients with cystic fibrosis at the Research Institute of Pulmonology for the period 1993-2018.

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3. Fig. 2. Age structure of patients in 2003

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4. Fig. 3. The age structure of patients in 2018

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5. Fig. 4. Survival curves for groups of 2018 (green curve) and 2013 (blue curve)

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6. Fig. 5. The structure of the distribution of patients with various indicators of FEV1 in 2003

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7. Fig. 6. The distribution structure of patients with various indicators of FEV1 in 2018

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Copyright (c) 2018 Krasovskiy S.A., Amelina E.L., Gorinova U.V., Chernyak A.V., Afanasjeva M.V., Krilova N.A., Shumkova G.L., Zonenko O.G., Tatarskiy A.R.

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