The paper presents algorithms for adult outpatient care of coronavirus disease 2019 (COVID-19) and its assumption.

The paper presents algorithms for adult outpatient care of coronavirus disease 2019 (COVID-19) and its assumption.

An analysis of medical documents and morphological examination of resected lung samples was done in 25 children had been operated for repeated pneumonias. All the children (100 %) were diagnosed undifferentiated connective tissue dysplasia (CTD) with marked clinical features. Morphological substrate of the repeated pneumonias was various defects of the lung growth. The results showed that the CTD as a genetic systemic pathology provided repeated pneumonias in structural abnormalities of the lungs. This fact should be taken into account in the diagnostic work-up.

In order to find out the doctors knowledge about effective methods of smoking cessation, a survey of 86 internists and general practitioners, as well as 14 residents was conducted. Results of the survey confirm the need for systematic training of doctors on effective smoking cessation.

Background. The relevance of work is warranted by the necessity of a personalized approach to the rehabilitation of bronchopulmonary syndrome patients with connective tissue dysplasia (CTD). Aim. To assess the effectiveness of medical rehabilitation in patients with different clinical phenotypes of bronchopulmonary syndrome and CTD on the basis of the analysis of quality of life indicators.Materials and methods. 174 patients aged 18–40 years were divided into subgroups depending on the predominant clinical respiratory option syndrome. The results of a survey of 75 patients, matched by sex and age, with no signs of CTD were used as controls. Rehabilitation activities were carried out under outpatient conditions three times a week for 12 weeks, 2 times a year. The effectiveness of individual rehabilitation programs for each patient was evaluated after two courses of medical rehabilitation. The SF-36 questionnaire was used to assess the quality of life.Results. Before the start of rehabilitation programs integral indicators in persons with CTD were significantly lower than in persons without CTD. After carrying out of rehabilitation programs, the most significant increase of mean values was observed for the parameters of the psychological health component: mental health (+40 points), vitality (+23 points), role of emotional functioning (+25 points) and social functioning (+21 points). The parameters of the physical health component increased to a lesser extent, their gains ranged from +7 points for the parameters of the overall perception of health to +19 points for the intensity of pain parameters. The effect on the physical health components was limited by the variety of irreversible structural changes of various organs and systems, suggesting a longer monitoring period. However, during rehabilitation significant positive effects were reported for physical health as well. Different parameters of values of quality of life were identified in patients with different clinical variants of bronchopulmonary syndrome.Conclusion. A personalized approach that takes into account the clinical variant of the syndrome can significantly increase the reserve for improving the functional capacity and also for psychological and social adaptation of the patient.

The objective of this study was to define pathologic mechanisms of primary spontaneous pneumothorax in patients with connective tissue dysplasia (CTD). Methods. We followed-up 42 young patients with CTD and bullous emphysema in the lung CT without history of primary spontaneous pneumothorax. Results of clinical, functional, radiological and molecular testing were compared with morphological examination of resected lung tissue specimens from 165 patients underwent lung surgery for primary spontaneous pneumothorax. Results. Two mechanisms underlying emphysema in CTD patients were defined. Conclusion: The results supported a hypothesis of causative relationship between congenital CTD and bullous emphysema in young patients.

To study the respiratory symptomatology in patients with connective tissue dysplasia (CTD) were surveyed 305 young people. Clinical and functional components of bronchopulmonary syndrome in CTD were identified through a comprehensive examination, including the use of standardized questionnaires and scales, and functional methods.

Summary. Four clinical phenotypes of respiratory syndrome have been identified in patients with connective tissue dysplasia using the mathematical modeling method. All phenotypes were characterized by different clinical manifestations and functional disorders and had different independent predictors. The most common chronic bronchitis phenotype was related to tobacco smoking and included early obstructive disorders. Bullous phenotype also predominantly occurred in smokers and comprised apical subpleural bullae or blebs. A probable genetic basis of the bullous phenotype is mutations in alleles of the matrix metalloproteinases MMP1 and MMP9. Thoracodiaphragmatic phenotype was typical for patients with the chest and the spine deformities and was characterized by restrictive disorders. Hyperventilation phenotype was closely related to autonomic dysfunction and increased anxiety, predominantly in females.

Summary. Four clinical phenotypes of respiratory syndrome have been identified in patients with connective tissue dysplasia using the mathematical modeling method. All phenotypes were characterized by different clinical manifestations and functional disorders and had different independent predictors. The most common chronic bronchitis phenotype was related to tobacco smoking and included early obstructive disorders. Bullous phenotype also predominantly occurred in smokers and comprised apical subpleural bullae or blebs. A probable genetic basis of the bullous phenotype is mutations in alleles of the matrix metalloproteinases MMP1 and MMP9. Thoracodiaphragmatic phenotype was typical for patients with the chest and the spine deformities and was characterized by restrictive disorders. Hyperventilation phenotype was closely related to autonomic dysfunction and increased anxiety, predominantly in females.

Summary. This article presents results of examination of 50 young patients with connective tissue dysplasia not having any acute or chronic respiratory disease. All patients underwent inspiratory and expiratory helical CT with density gradient determination in different parts of the lungs. Typical CT findings were subpleural apical bullae and bleby (16 %), peribronchial fibrosis (40 %), local fibrosis (18 %), plevroapikal spikes (62 %), emphysema (4 %), air traps (18 %). The density gradient between exhalation and inhalation was reduced in the upper and lower lobes of the lungs.

Summary. To evaluate a role of polymorphic variants of alpha1-antitrypsin (AAT) genes and matrix metalloproteinase (MMP) genes for heritable susceptibility to bullous emphysema and primary spontaneous pneumothorax (PSP) in patients with connective tissue dysplasia (CTD), we analyzed polymorphic loci of PIZ (Glu342Lys), PIS (Glu264Val), MMP1 (1607insG), MMP9 (C-1562T), MMP12 (A-82G), and TIMP1 (С536Т) genes and studied serum AAT concentration. We did not find any significant difference between prevalence of the Z- and S-mutations of PI gene and serum AAT concentration between groups. MMP1 homozygous GG/GG genotype was associated with risk of PSP development (odds ratio (OR), 2.23; 95 % confidence interval (СI): 1.34–3.73), MMP1 GG allele (OR, 2.27; 95 % CI: 1.61–3.20), MMP9 heterozigous С/Т genotype (OR, 2.43; 95%CI: 1.37–4.31), MMP9 homozygous Т/Т genotype (OR, 4.38; 95 % CI: 1.12–20.00), MMP9 T allele (OR, 2.78; 95 % CI: 1.74–4.46). All alleles and genotypes were found significantly more often in patients with signs of CTD. No statistically significant difference for any polymorphic locus of the studied genes was seen between prevalence of allele variants in patients with PSP but not having CTD and in population sample.