Russian registry of patients with cystic fibrosis: lessons and perspectives

Q4 Medicine

Pulmonologiya Pub Date : 2023-04-12 DOI:10.18093/0869-0189-2023-33-2-171-181

E. Kondratyeva, A. Voronkova, N. Kashirskaya, S. Krasovsky, M. Starinova, E. Amelina, S. Avdeev, S. Kutsev

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引用次数: 4

Abstract

A registry of patients with cystic fibrosis (CF) of the Russian Federation has been compiled annually since 2011. Analysis of the national registry with large amounts of clinical and laboratory data helps understand changes in demographic indicators, plan measures to improve the quality of medical care and evaluate their effectiveness.Aim. To analyze health status of patients with cystic fibrosis in the Russian Federation and the dynamics of key clinical and laboratory parameters from 2011 to 2021. Methods. The health status of CF patients was assessed using the registry data from 2011 to 2021.Results. The analysis revealed an increase in the total number of patients from 1,026% in 2011 to 3,969 in 2021, in the number of patients identified by neonatal screening from 28.8% to 53.5%, and in coverage by genetic testing from 91.8 to 93.6%. At the same time, the number of mutations detected dropped from 80 to 90.5% and the number of patients with unidentified mutations decreased from 9.5 to 3.2%. The mean age at diagnosis of cystic fibrosis did not change (3.3 ± 5.5 in 2011 and 3.1 ± 6.2 in 2021) despite an increase in the number of patients diagnosed through neonatal screening. There was a difference in M ± SD age from 2011 to 2021 (11.5 ± 8.9 in 2011 and 14 ± 9.8 in 2021). The proportion of adult patients was 24.95% in 2011 and 27.4% in 2021. The therapy changed over 11 years - the number of courses of intravenous therapy decreased from 70.9 to 36.4%, the number of patients using inhaled antipseudomonal therapy expanded to 45%, the number of patients using hypertonic sodium chloride solution expanded from 8.7 to 70.7%, the use of glucocorticoids decreased. The targeted therapy was introduced in 2018, and the number of patients receiving pathogenetic drugs is growing.Conclusion. The observed changes are indicative of the health status of Russian patients with cystic fibrosis. Analysis of registries helps improve the organization of medical care, predict and implement sanitary and epidemic measures, plan therapy, and assist the regions in organizing outpatient monitoring and microbiological control. The registry is analyzed to organize health care for adult patients.

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俄罗斯囊性纤维化患者登记:经验教训和观点

自2011年以来，每年编制俄罗斯联邦囊性纤维化(CF)患者登记册。对国家登记的大量临床和实验室数据进行分析，有助于了解人口指标的变化，计划改善医疗质量的措施，并评估其有效性。目的:分析2011 - 2021年俄罗斯联邦囊性纤维化患者的健康状况及关键临床和实验室参数的动态。方法。使用2011 - 2021年的登记数据评估CF患者的健康状况。分析显示，患者总数从2011年的1026%增加到2021年的3969人，新生儿筛查确定的患者数量从28.8%增加到53.5%，基因检测的覆盖率从91.8%增加到93.6%。与此同时，检测到的突变数量从80%下降到90.5%，未识别突变的患者数量从9.5%下降到3.2%。尽管通过新生儿筛查诊断的患者数量增加，但囊性纤维化诊断的平均年龄没有变化(2011年为3.3±5.5岁，2021年为3.1±6.2岁)。2011年至2021年M±SD年龄差异(2011年为11.5±8.9,2021年为14±9.8)。2011年成人患者比例为24.95%，2021年为27.4%。治疗方法在11年中发生了变化——静脉治疗的疗程数从70.9%下降到36.4%，使用吸入抗假单胞菌治疗的患者数量增加到45%，使用高渗氯化钠溶液的患者数量从8.7增加到70.7%，糖皮质激素的使用减少。2018年引入靶向治疗，接受致病性药物治疗的患者数量不断增加。观察到的变化表明了俄罗斯囊性纤维化患者的健康状况。登记分析有助于改善医疗保健组织，预测和实施卫生防疫措施，计划治疗，并协助地区组织门诊监测和微生物控制。对注册表进行分析，以便为成年患者组织保健服务。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pulmonologiya Medicine-Pulmonary and Respiratory Medicine

CiteScore

1.40

自引率

0.00%

发文量

期刊介绍： The aim of this journal is to state a scientific position of the Russian Respiratory Society (RRS) on diagnosis and treatment of respiratory diseases based on recent evidence-based clinical trial publications and international consensuses. The most important tasks of the journal are: -improvement proficiency qualifications of respiratory specialists; -education in pulmonology; -prompt publication of original studies on diagnosis and treatment of respiratory diseases; -sharing clinical experience and information about pulmonology service organization in different regions of Russia; -information on current protocols, standards and recommendations of international respiratory societies; -discussion and consequent publication Russian consensus documents and announcement of RRS activities; -publication and comments of regulatory documents of Russian Ministry of Health; -historical review of Russian pulmonology development. The scientific concept of the journal includes publication of current evidence-based studies on respiratory medicine and their discussion with the participation of Russian and foreign experts and development of national consensus documents on respiratory medicine. Russian and foreign respiratory specialists including pneumologists, TB specialists, thoracic surgeons, allergists, clinical immunologists, pediatricians, oncologists, physiologists, and therapeutists are invited to publish article in the journal.