The effect of age and genotype on lung function in children with cystic fibrosis

2021 ◽  
Vol 31 (2) ◽  
pp. 159-166
Author(s):  
V. V. Shadrina ◽  
A. Yu. Voronkova ◽  
M. A. Starinova ◽  
O. I. Simonova ◽  
D. F. Sergienko ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Russian Federation ◽  
Age Groups ◽  
Forced Expiratory Volume ◽  
Functional Disorders ◽  
Significant Difference ◽  
The Russian Federation ◽  
Effect Of Age ◽  
Adolescent Period

Identifying the initial manifestations of pulmonary functional disorders associated with cystic fibrosis (CF) is essential for timely correction of the therapy and improvement of the prognosis.The aim of the study was to identify the age periods with the highest risk of a decrease in lung function in children with different CF genotypes.Methods. Lung function (LF) was analyzed using spirometry results in CF patients aged 6 to 18 years from the Registry of patients with cystic fibrosis in the Russian Federation 2018.Results. The forced expiratory volume in one second (FEV1) was statistically significantly lower in children aged 6 – 10 years with “severe” genotypes than in the children with “mild” genotypes. The lung function was reduced at 11 – 14 years of age in both “severe» and “mild” genotypes without any statistically significant difference. In general, the trend of a decrease in lung function in “severe” genotypes persists in the adolescent period from 15 to 18 years. The lung function is better in adolescents aged 15 – 18 with the “mild” variants of genotypes compared to the patients aged 11 – 14 years.Conclusion. The “severe” variants of CFTR negatively affect LF of CF patients in all age groups. The LF values in patients with “mild” genotypes at the age of 15 – 18 years were higher than in children aged 11 – 14 years with the same genotypes. There is a need for improved programs of medical care for adolescents with CF.

scholarly journals Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient

2017 ◽  
Vol 50 (5) ◽  
pp. 1700326 ◽  
Author(s):  
Gwyneth Davies ◽  
Janet Stocks ◽  
Lena P. Thia ◽  
Ah-Fong Hoo ◽  
Andrew Bush ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Early Life ◽  
Treatment Protocol ◽  
Forced Expiratory Volume ◽  
Z Score ◽  
Average Group ◽  
Significant Difference ◽  
Residual Capacity ◽  
High Risk Infants

With the advent of novel designer molecules for cystic fibrosis (CF) treatment, there is huge need for early-life clinical trial outcomes, such as infant lung function (ILF). We investigated the degree and tracking of ILF abnormality during the first 2 years of life in CF newborn screened infants.Forced expiratory volume in 0.5 s (FEV0.5), lung clearance index (LCI) and plethysmographic functional residual capacity were measured at ∼3 months, 1 year and 2 years in 62 infants with CF and 34 controls.By 2 years there was no significant difference in FEV0.5 z-score between CF and controls, whereas mean LCI z-score was 0.81 (95% CI 0.45–1.17) higher in CF. However, there was no significant association between LCI z-score at 2 years with either 3-month or 1-year results. Despite minimal average group changes in any ILF outcome during the second year of life, marked within-subject changes occurred. No child had abnormal LCI or FEV0.5 on all test occasions, precluding the ability to identify “high-risk” infants in early life.In conclusion, changes in lung function are mild and transient during the first 2 years of life in newborn screened infants with CF when managed according to a standardised UK treatment protocol. Their potential role in tracking disease to later childhood will be ascertained by ongoing follow-up.

scholarly journals Russian Experience of Transport Extracorporeal Membrane Oxygenation

2021 ◽  
Vol 9 (4) ◽  
pp. 521-528
Author(s):  
D. A. Shelukhin ◽  
A. V. Karpov ◽  
M. V. Ketskalo ◽  
K. K. Gubarev
Keyword(s):  
Extracorporeal Membrane Oxygenation ◽  
Clinical Experience ◽  
Russian Federation ◽  
Age Groups ◽  
Clinical Manifestations ◽  
Practical Experience ◽  
Hospital Survival ◽  
Membrane Oxygenation ◽  
Significant Difference ◽  
The Russian Federation

Relevance. The present time can be called a period of accumulation of experience of national health systems in different countries of the world in the application of transport extracorporeal membrane oxygenation (ECMO) technology at the pre- and inter-hospital stages of evacuation of patients to specialized ECMO-therapy centers. The role of such centers is to provide timely advice and, if necessary, perform inter-hospital evacuation.Material and methods. The study summarized and analyzed with the help of the national register “RosECMO” the own experience of 13 hospitals in the Russian Federation, who performed 68 inter-hospital evacuations under ECMO conditions by different modes of transport in patients of different age groups with symptoms of circulatory and respiratory failure. The following parameters were evaluated: characteristics of transport ECMO, clinical manifestations of potentially negative effects of transport, hospital survival, as well as the effect of experience (less and more than 10 cases of transport ECMO) of the presented clinics on the difference in the results obtained.Results. Connecting patients to the ECMO device reduces the likelihood of death on the SOFA and APACHE IV scales by 1.2 times (p <0.0001) and 1.4 times (p<0.0001), respectively. Despite the absence of deaths during inter-hospital transportation of patients under ECMO conditions, 14.93% of patients died within 3 days from the moment of their execution, without a significant difference in clinics with different practical experience. The overall hospital survival rate of ECMO transport scenarios in all 13 clinics of the Russian Federation was comparable to the data of the international register 48.52% versus 48.81%, at the same time it was significantly lower (1.3 times) in the group of clinics with less clinical experience 40% versus 52.08% (p<0.0001).Conclusion. The results of the first stage of the study we obtained indicate the prospects of using the method of extracorporeal membrane oxygenation at the stage of inter-hospital evacuation, due to the effective stabilization of the patient’s condition and a significant reduction in the risks of the likelihood of death. Clinics with less clinical experience showed significantly worse results of hospital survival of patients who underwent inter-hospital transportation under conditions of extracorporeal membrane oxygenation compared to clinics with more clinical experience, which can be a significant argument in adopting a model for the development of specialized regional centers for extracorporeal membrane oxygenation. The experience accumulated over the past six years and the analysis of new data from the register of transport cases of extracorporeal membrane oxygenation of the national healthcare system will make it possible to formulate the correct trajectory for the development of the method of extracorporeal membrane oxygenation and its application, including at the stage of pre- and inter-hospital evacuations of patients.

Chronic polypous rhinosinusitis and lung function in the profile of multi-organ pathology in children with cystic fibrosis in the Russian Federation

2021 ◽  
Vol 31 (2) ◽  
pp. 207-215
Author(s):  
D. P. Polyakov ◽  
N. A. Daykhes ◽  
A. S. Yunusov ◽  
O. V. Karneeva ◽  
A. S. Petrov ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Respiratory Tract ◽  
Chronic Rhinosinusitis ◽  
Russian Federation ◽  
Paranasal Sinuses ◽  
Respiratory Function ◽  
Organ Damage ◽  
Nonparametric Tests ◽  
The Russian Federation

Chronic rhinosinusitis (CRS), including chronic rhinosinusitis with nasal polyps (CRSwNP), is one of the manifestations of cystic fibrosis (CF). The paranasal sinuses (PNS) are considered as a reservoir of pathogenic microflora and a source of descending infection of the entire respiratory tract. Nevertheless, paranasal sinuses pathology is usually assessed in isolation from both the lower respiratory tract and the entire spectrum of multiple organ damage in CF.Aim. To determine the prevalence of CRSwNP and its correlation with other characteristics of CF in children in Russia.Methods. The study included data of 2216 children from 0 to 18 years old from 81 regions of the Russian Federation from the Registry of patients with cystic fibrosis in the Russian Federation for 2018. Children with an established diagnosis of CRSwNP (n = 599) were compared to those without this condition (n = 1 617). The prevalence was assessed in the subgroups by age, sex, respiratory function, nutritional status, microbiological status, complications, the need for various therapies, and the average duration of inpatient treatment during the year. The statistical analysis included parametric and nonparametric tests.Results. According to the Registry, the average prevalence of CRSwNP among children with CF was 27.03% and tended to increase with age. Children with CRSwNP had statistically significant higher incidence of homozygous F508del mutation, more common intermittent and chronic infection with P. aeruginosa, S. aureus (MSSA and MRSA), cystic fibrosis-associated diabetes mellitus with daily use of insulin, liver damage (as with or without cirrhosis), osteoporosis, a more frequent need for systemic antibacterial and inhalation (except for bronchodilators) therapy, as well as a relatively high need for hospital stay. Unexpectedly, the respiratory function indicators (FEV1, %, FVC, %) and body mass index turned out to be higher in the group of patients with nasal polyposis. A likely explanation is that the study included only children, and the lung function was not analyzed in the children and adolescents.Conclusion. The results suggest that CRSwNP is a predictor of adverse course of CF and requires attention from CF specialists and otorhinolaryngologists, as well as interdisciplinary collaborations.

The structure of consumption of tobacco and nicotine-containing products among the population of the Russian Federation

2020 ◽  
Vol 30 (4) ◽  
pp. 453-462
Author(s):  
O. O. Salagay ◽  
G. M. Sakharova ◽  
N. S. Antonov
Keyword(s):  
Russian Federation ◽  
Electronic Cigarettes ◽  
Age Groups ◽  
Opinion Poll ◽  
Age Group ◽  
Tobacco Products ◽  
Men And Women ◽  
Significant Difference ◽  
The Russian Federation ◽  
Structure Of Consumption

In the Russian Federation, after the adoption of Federal Law “On the Protection of Citizens' Health from the Effects of Tobacco Smoke and the Consequences of Tobacco Use”, the prevalence of smoking tobacco has significantly decreased. Reducing the consumption of smoking tobacco products led to the emergence of a new aggressive policy of tobacco companies, which resulted in the emergence of new “innovative” products and the concepts of “less harmful tobacco product” or “less harmful nicotine-containing product”. The undoubtedly aggressive advertising campaigns of tobacco companies and manufacturers of electronic cigarettes have affected the smoking behavior of tobacco consumers, which has begun to change in recent years.The purpose of this study was to study new trends in the structure of consumption of tobacco and nicotine-containing products in various age groups of the Russian Federation, as well as among men and women.Methods. Analysis of the structure of consumption of tobacco and nicotine-containing products was carried out as part of an online public opinion poll to study the level of awareness of measures to reduce the prevalence of tobacco products in the Russian Federation, initiated in 2019 by the Ministry of Health of the Russian Federation. A total of 1 282 respondents from 73 regions of the Russian Federation took part in the survey. Among the respondents there were 447 (34.9%) men and 835 women (65.1%) aged 18 years and younger to 70 years. To conduct a comparative analysis of the structure of use of tobacco and nicotine-containing products, the frequencies of their use in different age groups, among men and women, were calculated. A statistically significant difference between the groups was confirmed by analyzing the dependence in the contingency table using the χ2 criterion.Results. A total of 432 respondents reported the use of tobacco and nicotine-containing products, which amounted to 33.7% of all respondents. The most used products were smoking tobacco (57.64%), electronic cigarettes (34.03%) and waterpipe (33.80%). However, in different age groups, the structure of their use was significantly different (p < 0.05). The highest level of electronic cigarette use, including vapors containing nicotine and electronic nicotine delivery product, were found in the age group of 18 years and younger (58.82%). In the older age groups, a significant decrease in the level of consumption was observed. Chewing tobacco and snuff were consumed by consumers significantly less than other tobacco products. Smokeless tobacco was mainly consumed by consumers aged 18 years and younger, its level was 20.58%. The highest level of waterpipe consumption was found among consumers in the age group of 19 – 29 years (53.85%), it was slightly less in the group of 18 years and younger (44.11%). The higher level of waterpipe consumption was detected (40.35%) among women than it was among men (26.47%). Many consumers indicated that they consumed several types of tobacco and nicotine-containing products. The most popular combination of products was found among consumers under the age of 30, and 20.59% of consumers aged 18 and younger consumed at the same time almost all the types of tobacco and nicotine-containing products that were presented on the market.Conclusion. The study revealed new trends in preferences for consumption of tobacco and nicotine-containing products. The new trends have significantly changed the structure of the consumption of these products among the population of Russian Federation.

Epidemic process of COVID-19 in the Russian Federation: interim results. 1th report

2020 ◽  
Vol 18 (3) ◽  
pp. 7-14
Author(s):  
N.Yu. Pshenichnaya ◽  
◽  
I.A. Lizinfeld ◽  
G.Yu. Zhuravlev ◽  
А.A. Ploskireva ◽  
...  
Keyword(s):  
Russian Federation ◽  
Age Groups ◽  
Effective Control ◽  
Labor Migrants ◽  
Epidemic Process ◽  
Mild Disease ◽  
Significant Difference ◽  
The Russian Federation ◽  
Restrictive Measures ◽  
Peak Value

Objective. To perform interim analysis of epidemiological parameters of COVID-19 in the Russian Federation. Material and methods. The article is based on the analysis of individual monitoring data carried out by Federal Service for Surveillance on Consumer Rights Protection and Human Wellbeing for January–June 2020. Results. The epidemic process in Moscow and Moscow region (MR) differed from that in other regions. In Moscow and MR,daily increase of new coronavirus cases was characterized by high absolute numbers and varied between 5.0% and 29.2% in March–April 2020. The peak (7,446 new cases) was registered on May 6; after that, the number of new cases was rapidly declining and decreased by 84.9% from the peak value by June 30. This was ensured by strict restrictive measures and effective control in Moscow and MR. In other regions, daily increase of new cases varied between 2.9% and 52.6%. The peak incidence was registered on May 21 (6,021 new cases), whereas the plateau phase was observed between May 14 and June 17, 2020 (5,808–6,021 new cases daily). Only by June 28, the number of new cases decreased by 43.8% from the peak value. During this time, the number of new cases in Moscow and MR was already 3 times lower. This can be explained by less severe restrictions in other regions. Half of patients (50.1%) had mild disease. The majority of patients were aged 41–64 (44.4%) and 18–40 years (30.2%). In the age group ≥40 years, women prevailed; however, they usually had milder disease, which was observed in all age groups ≥18 years. Conclusion. The characteristics of the epidemic process in Russia were largely determined by the restrictive measures introduced in March 2020. These measures significantly reduced both the epidemic intensity of COVID-19 and the role of children of various age groups in it. The earlier start of active circulation of SARS-CoV-2 in Moscow and MR, as well as its subsequent spread to the regions by labor migrants who returned from the capital led to a significant difference in the dynamics of new cases at the decline of the epidemic. Key words: age groups, COVID-19, Russia, severity, epidemic process.

Analysis of vitamin D deficiency in patients of different age groups

2020 ◽  
pp. 64-70
Author(s):  
Timur Minasov ◽  
Radik Nurlygayanov ◽  
Angela Vershinina ◽  
Yulia Soboleva ◽  
Damir Akhmeldinov ◽  
...  
Keyword(s):  
Vitamin D ◽  
Blood Serum ◽  
Vitamin D Deficiency ◽  
Russian Federation ◽  
Age Groups ◽  
Upper Limit ◽  
The Russian Federation ◽  
Physiological Values

The results of a study of the concentration of vitamin D in blood serum of 3455 residents of Ufa are presented. Physiological values of 25(OH)D were observed in 34.9 % (274) of men and 31.7 % (847) of women, which is 32.4 % (1121) of all examined. Vitamin D deficiency was detected in 67.1 % of the examined. The level of 25(OH)D in the blood serum of residents is on average 75 nmol/L, which is the upper limit of vitamin D deficiency. Vitamin D deficiency is extremely common among residents of this region of the Russian Federation (67.1 %).

Does body position, age, and heart rate induce IOP’s changes?

2021 ◽  
pp. 112067212110233
Author(s):  
Marcelina Sobczak ◽  
Magdalena Asejczyk ◽  
Malwina Geniusz
Keyword(s):  
Heart Rate ◽  
Body Position ◽  
Age Groups ◽  
Cardiovascular Parameters ◽  
Entire Cohort ◽  
Significant Difference ◽  
Group A ◽  
The Difference ◽  
Group B ◽  
Effect Of Age

Objectives: The main goal of this research was to determine the differences between the values of intraocular pressure (IOP) in the supine and sitting positions, and to assess the effect of age and cardiovascular parameters. Methods: Seventy-two healthy adults were enrolled and classified into age groups: 20–30 years (group A), 31–40 years (group B), and 41–71 years (group C). Corneal biometry and cardiovascular parameters, such as heart rate (HR), were measured. IOP measurements were taken in the sitting position (IOPS) and in the supine position (IOPL) using the iCare® Pro tonometer. Results: A significant difference between the IOPS and IOPL in the entire cohort was found ( p < 0.001). Regarding the age subgroups, a significant difference ( p < 0.001) between the IOPS and IOPL was obtained in group A (2.6 ± 1.6 mmHg) and group C (1.5 ± 1.3 mmHg). There were no significant differences in the IOPS between groups. The highest IOP values were obtained for group A. The correlations between HR and IOPS are statistically significant for group A and group B, and for HR and IOPL-S for group B only. Multivariate analysis showed that HR has a significant influence on the difference in IOP in the two body positions. Conclusion: A statistically significant difference between the effect of age and the values of IOPS and IOPL was shown. Cardiovascular parameters showed some relevant statistical dependencies, but with a rather marginal significance in young people. The influence of body position for the measurement of IOP for healthy subjects does not seem to matter, despite the fact that there are some dependencies that are statistically significant.

WS02.3 Liver cirrhosis and transplantation in cystic fibrosis in the Russian Federation

2021 ◽  
Vol 20 ◽  
pp. S4
Author(s):  
E. Zhekaite ◽  
E. Kondratyeva ◽  
A. Voronkova ◽  
V. Sherman ◽  
T. Dzhanbekov ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Cirrhosis ◽  
Russian Federation ◽  
The Russian Federation

MORBIDITY, MORTALITY AND RELIABILITY OF ACCOUNTING OF PATIENTS OF DIFFERENT AGE GROUPS WITH LIVER CANCER IN RUSSIA

2020 ◽  
pp. 240-245
Author(s):  
В. М. Мерабишвили ◽  
В. К. Гуркало ◽  
Э. Н. Мерабишвили
Keyword(s):  
Liver Cancer ◽  
Russian Federation ◽  
Malignant Tumors ◽  
Primary Liver Cancer ◽  
Age Groups ◽  
Federal District ◽  
Cancer Register ◽  
The North ◽  
The Russian Federation ◽  
North Western

Рак печени (РП) - тяжелейшая онкопатология. В России смертность превышает число первично-учтенных больных в среднем. Ежегодно в России регистрируют более 8 тыс. (8 810 - в 2018 г.) случаев рака печени и более 10 тыс. (10 018 - в 2018 г.) случаев смерти от этой причины. Использованы стандартные методы медицинской статистики, все зарегистрированные первичные случаи РП в России. Углубленная разработка материала проведена на основе базы данных Популяционного ракового регистра Северо-Западного Федерального округа РФ. Проанализированы данные всего комплекса доступной информации в целом по России. Установлен прирост заболеваемости РП населения России за 18 лет в «грубых» показателях и абсолютных числах на 20% и в стандартизованных - на 1,22%. У женщин отмечено снижение учтенных случаев РП на 4,55%. Смертность населения России в стандартизованных показателях снизилась за 18 лет практически на 3%. Превышение числа умерших над заболевшими от РП существенно возрастает в старших возрастных группах. Индекс достоверности учета (ИДУ) - это отношение абсолютных чисел или «грубых» показателей смертности населения к заболеваемости. Для всех злокачественных новообразований (ЗНО) величина в среднем по России составила 0,47, практически совпадающая со среднеевропейской. ИДУ у мужчин - 0,55, выше расчетных показателей для женщин - 0,40, что в первую очередь связано с более благоприятной структурой заболеваемости женщин ЗНО. Медиана выживаемости первичных больных РП составляет немногим более 3 мес. Пятилетняя выживаемость больных РП даже на ранних стадиях составляет у лиц моложе 60 лет с I стадией - 25,3%, старше 60 лет - только 17,2%, со II стадией - 17,9 и 11% соответственно. Liver cancer is a severe oncopathology. Mortality exceeds the number of primary-accounted patients in Russia on average. Every year in Russia more than eight thousand (8 810 in 2018) cases of liver cancer and more than 10 000 (10 018 in 2018) deaths from this cause are registered. Used Standard methods of medical statistics, all registered primary cases of liver cancer in Russia. In-depth development of the material was carried out on the basis of the database of the Population cancer register of the North-Western Federal district of the Russian Federation. The analysis of the data of the whole complex of available information in Russia as a whole is carried out. In-depth data development was carried out on the basis of the database of the Population cancer register of the North-Western Federal district of the Russian Federation. The increase in the incidence of liver cancer in Russia over 18 years in «rough» indicators and absolute numbers by 20 % and in standardized by 1,22 % was established. In women, there was a decrease in reported cases of liver cancer by 4,55 %. The mortality rate of the Russian population in standardized indicators has decreased by almost 3 % over 18 years. The excess of the number of deaths over those from liver cancer increases significantly in older age groups. The index of reliability of accounting is the ratio of absolute numbers or «rough» indicators of mortality to morbidity. For all malignant tumors, its value on average in Russia was 0,47, almost coinciding with the average European. The index of reliability of accounting for men is 0,55, higher than the calculated indicators for women - 0,40, which is primarily due to a more favorable structure of the incidence of malignant tumors in women. The median survival of primary liver cancer patients is a little more than three months. The fi ve-year survival rate of patients with liver cancer even in the early stages is in the first among persons younger than 60 years - 25,3 %, over 60 years - only 17,2 %, in the second, respectively, 17,9 and 11%.

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