SummaryThis study was aimed at development of cystic fibrosis (CF) patient register for those living at Moscow and Moscow region. The CF patients living in Moscow region were found to have some clinical, genetic and bacteriological differences from CF patients living at the Western Europe. The median survival in 2003-2012 was 39.5 years. Aging of CF patients was associated to reduction in respiratory function, occurrence of more aggres sive respiratory pathogens, increase in respiratory and gastrointestinal complication rate. Key words: cystic fibrosis, register, age related features. РезюмеЦелью исследования явилось создание регистра больных муковисцидозом (МВ), проживающих в Москве и Московской области. Пока зано, что больные МВ московского региона имеют ряд клинико генетических и микробиологических особенностей по сравнению с пациентами ряда западных стран. Медиана выживаемости за период 2003-2012 гг. составила 39,5 лет. Продемонстрировано, что взрос ление больного МВ сопровождается снижением респираторной функции, сменой микрофлоры дыхательных путей на более агрессив ную, нарастанием частоты осложнений со стороны органов дыхания и пищеварения. Ключевые слова: муковисцидоз, регистр, возрастные особенности.

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Хроническая инфекция нижних дыхательных путей является ключевым признаком у больных муковисцидозом (МВ). Она является ведущим фактором, определяющим тяжесть клинического течения и прогноз заболевания. При изучении микрофлоры нижних дыхательных путей различ-ных возрастных групп детей, больных МВ, исследователями различных стран установлено, что основными возбудителями инфекции легких у больных МВ являются Р. aeruginosa, S. aureus и H. influenzae [1]. Показано, что в первые годы жизни у больных МВ доминирует золотистый стафилококк, а затем основным возбудителем становится синегнойная палочка [2, 3]. В последние годы было конста-С.В. ПОЛИКАРПОВА 1 , к.м.н., Е.И. КОНДРАТЬЕВА 2 , д.м.н., профессор, Л.А. ШАБАЛОВА 2 , к.м.н., Н.В. ПИВКИНА 1 , С.В. ЖИЛИНА 1 , А.Ю. ВОРОНКОВА 2 , В.Д. ШЕРМАН 2 , к.м.н., В.С. НИКОНОВА 2 , Н.И. КАПРАНОВ 2 , д.м.н., профессор, Н.Ю. КАШИРСКАЯ 2 , С.Ю. СЕМЫКИН 3 , Е.Л. АМЕЛИНА 4 , С.А. КРАСОВСКИЙ 4 1 Городская клиническая больница №15 им. О.М. Филатова Департамента здравоохранения г. Москвы 2 Медико-генетический научный центр, Москва

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Since the beginning of the COVID-19 epidemic, the European cystic fibrosis society (ECFS) has decided to launch a special ECFS-COVID-19 program to collect information on the of COVID-19 characteristics in the patients with cystic fibrosis (CF). The results of the program should help timely and efficiently provide the patients with CF with the necessary care. Initially, it was assumed that COVID-19 would be severe in CF patients. The aim. Тo assess the prevalence and characteristics of COVID-19 in patients with cystic fibrosis (CF) in the Russian Federation (RF). Methods. 6 cases (4 children and 2 adults) of COVID-19 in Russian CF patients were analyzed. Results. There are 405,843 infected with SARS-CoV-2 in Russia, the incidence of coronavirus infection in Russia was 1.4 cases per 1 thousand people. According to the Ministry of Health of the RF, as of December 2019, there were 3,931 patients with CF (2,823 children and 1,108 adults). The incidence of COVID-19 was 1.5 per 1000 patients with CF (1.4 : 1,000 for children and 1.8 : 1,000 for adults). The incidence was not higher than in the General population. The diagnosis of COVID-19 was confirmed in 4 boys and 2 women, 3 of the patients were infected with Pseudomonas aeruginosa and 2 – with Achromobacter spp. Mild disease was seen in 5 patients including all the children. Pneumonia was registered in 3 patients. One child with COVID-19 had abdominal syndrome. 2 patients – 1 adult and 1 child – needed in-patient care. Additional antibiotics were given to 4 patients, 2 of them received i/v antibiotics. One adult patient was on the lung transplantation waiting list. This woman had long-term oxygen therapy and BiPAP noninvasive respiratory support before the infection with SARS-CoV-2, FEV1 was 24 %pred. Conclusion. Despite the fact that patients with CF are at risk of severe COVID-19, to date, in the described cases, COVID-19 infection has not led to a significant deterioration of the symptoms of CF. Not a single fatal outcome in Russian patients with CF has been recorded.

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Aim: to assess clinical and genetic, microbiological and functional characteristics of patients with cystic fibrosis living in Moscow and Moscow region as of January 31, 2010. Patients and methods: 359 patients were examined. Mean age was 12,1 ± 9,6 years old (0,2-43,2), median age-11,0 (16,0) years. Adult rate (patients older than 18 years old) was 30,6%. Median age at the moment of diagnosis confirmation was 1,0 (5,0) years; 3,8% of all patients (12,4% of adults) were diagnosed in adulthood. Genetic screening for the purpose of cystic fibrosis mutations detection and microbiological assay of lower respiratory tract discharge were performed. Results: the prevalence of the most common mutation in the world-F508del-was 52,96%, a relatively high frequency of «mild» genotypes was observed among adult patients. Interestingly, a high percentage of Burkholderia cepacia complex (8,7%) in the structure of microbiological pattern of the respiratory tract was noted. A significant increase of pneumothorax, haemoptysis, hypoxemic respiratory failure and diabetes mellitus with fasting hyperglycemia frequency was established, while hepatocirrhosis with portal hypertension prevalence was comparable between adults and patients. Conclusions: patients with cystic fibrosis from Moscow and Moscow region have a number of clinical and genetic and microbiological special features in comparison to patients from other regions of Russian Federation and certain western countries.

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