The aim of this study was to evaluate safety and efficacy of salbutamol, fenoterol and ipratropium bromide in patients with bronchial asthma (BA) associated with ischaemic heart disease (IHD). One hundred and twenty five patients with exacerbation of moderate to severe BA entered the study. Sixty four of them were diagnosed associated IHD (angina pectoris of II to III functional class and postinfarct cardiosclerosis). All the patients were divided into 4 group, receiving fenoterol, salbutamol, ipratropium bromide and Berodual correspondingly as via dosing aerosol and nebulizer. The bronchodilating effect of the drugs was assessed using peakflowmeter. Moreover, oxygen saturation, arterial blood pressure were measured and 24-hour ECG Holter monitoring was performed. As a result, nebulized therapy with short-acting β 2 -agonists gave a significant bronchodilating effect and did not cause considerable hemodynamic disorders and myocardial ischaemia. When using high doses of these drugs in patients with BA and associated IHD the efficacy/safety ratio decreased due to possible hemodynamic disturbances, hypoxemia and myocardial ischaemia induction. The alternative bronchodilator to control the bronchoobstructive syndrome in such patients is to be ipratropium bromide.

The study was aimed to Investigate severe asthma therapy in Russia. It involved 515 patients from 7 Russian cities according to the criteria of severe asthma. Individual registration cards were filled in considering dem ographic data, principal clinical features, drug therapy. It has been found that both inpatient and outpatient therapy of severe asthm a in Russia does not correspond to the standards recommended. The results confirmed high efficiency of inhaled corticosteroids when combined with long-acting β2-agonists. This therapy was found to be more effective then other regarding to symptoms, lung function param eters, rate of admission to a hospital and duration of hospital staying. Patients treated with this drug combination were satisfied by the treatment more often.

The study was designed to evaluate influence of 6-month therapy with salmeterol on clinical and functional parameters and quality of life in 20 patients with moderate to severe COPD. The disease symptoms were analyzed before and in 3 and 6 months of the treatment using the Piggario scale, the lung function parameters (FEV 1 , FVC, TLC, RV, Raw) were measures with spirography and body plethysmography, physical tolerance was assessed in 6-minute walking test, quality of life was evaluated with the Saint George's Respiratory Questionnaire. Results showed that Salmeterol reduced dyspnoea, cough, sputum production and improved tolerance to the cold air in the COPD patients. The 6-minute walking distance and FEV 1 significantly increased to the end of the study period. The quality of life improved greatly in all scales of the questionnaire. We concluded that salmeterol was reliably effective in COPD patients living at the strong continental climate of the Eastern Siberia.

The article describes a clinical case of a 14-year old patient with RDH5 mutations (OMIM *601617) in patient with fundus albipunctatus (OMIM #136880) and characteristic biomarkers of this disease with previously described pathogenic variant of nucleotic sequence in exon 3 of the RDH5 gene (NM_002905.3:c.500G>A), causing a missense change (p.Arg167His) in heterozygous state and previously not described pathogenic variant of nucleotic sequence in exon 5 of the RDH5 gene (NM_002905.3:c.838C>T), leading to a missense change (p.Arg280Cys) in heterozygous state with characteristic biomarkers of the disease. Best-corrected visual acuity (BCVA) was 20/20. Nyctalopia was accompanied by reduced b-wave of scotopic (dark-adapted 0.01) ERG and decreased amplitude of a- and b-waves of maximum (dark-adapted 3) ERG. Decreased amplitude of the a- and b-waves of photopic (light-adapted 3) ERG and the amplitude of high-frequency (light-adapted 30 Hz) Flicker ERG shows the involvement of retinal cone system in the process. Fundus autofluorescence imaging of both eyes produced fuzzy and grainy images with slight hyperfluorescence of retinal flecks. Optical coherence tomography showed focal thickening centered in the photoreceptor outer segment corresponding to the multiple discrete albipunctate dots.

One of the most important purposes of treatment of asthma is achievement and maintenance of control of the disease. The present study investigated relationship between a level of asthma control and bronchial hyperreactivity (BHR). This study involved 145 outpatients aged 18 to 34 yrs. The control of asthma was evaluated using the Asthma Control Test (ACT). The ACT score of 25 indicated the full control of asthma, the scores between 20 and 24 indicated the good control and the scores < 20 suggested the poor control. BHR was assessed in the methacholine challenge test; PD20  was the dose of methacholine causing 20 % fall in FEV 1 . PD 20 ≤ 0.046 mg suggested high BHR, PD 20 ranged 0.047 mg to 0.228 mg indicated moderate BHR, and PD 20 of 0.229–0.471 mg corresponded to low BHR. Asthma was fully controlled in 17 mild asthma patients (22 %) and was poorly controlled in 13 of them (17 %). No-one of moderate and severe asthma patients had the full control of the disease. Good control of moderate and severe asthma was found in 22 (50 %) and 2 (8 %) respectively, poor control was estimated in 22 (50 %) and 23 (92 %) patients respectively. The mean methacholine concentrations in mild, moderate and severe asthma groups were 0.304 ± 0.011 mg, 0.211 ± 0.019 mg and 0.101 ± 0.015 mg respectively (p = 0.03). BHR was low in all patients with fully controlled asthma with no regards to its severity. The patients with well controlled asthma had predominantly low and moderate BHR, and 9 % of them had high BHR. BHR was also high in 57 % of the patients with poorly controlled asthma. There was a close correlation between the ACT score and PD20 of methacholine (r = 0.78; р < 0.001). In conclusion, the results revealed insufficient quality of outpatient treatment of asthma, particularly of moderate and severe asthma. ACT is reasonable to be used in wide clinical practice, especially in primary care settings.

Biological therapy of bronchial asthma (BA) is a modern method of treating severe forms of the disease, that are uncontrolled by traditional pharmacotherapeutic approaches. Currently, 5 monoclonal antibody (AT) preparations are registered in the world for the treatment of severe bronchial asthma (SBA) of the T2 endotype (T2-SBA) – antibodies, binding to immunoglobulin (Ig) E (anti-IgE – omalizumab), interleukin antagonists (IL)-5 (anti-IL-5 – mepolizumab, resizumab) and its receptor (anti-IL-5Rα – benralizumab), as well as antibodies, that selectively bind to the IL-4 and -13 receptor (anti-IL-4 /13Rα – dupilumab). The article presents data on the effectiveness of these drugs in relation to the key characteristics of SBA, formulates clinical and laboratory criteria, the study of which in real practice can potentially predict the likelihood of a clinical response to a particular type of biological therapy. An algorithm is proposed for choosing a targeted therapy strategy for patients with SBA, clinically associated with allergies, for patients with severe non-allergic eosinophilic BA and for patients with eosinophilic BA of a combined phenotype.

Frequency and clinical manifestations of respiratory disease in systemic connective tissue diseases (CTD) differ significantly including clinical course and outcomes. Pulmonary  abnormalities  are seen in 20% to 95% of patients with CTD ranging from subtle to life-threatening disease and could be fatal. Commonly, CTD-related interstitial lung disease (ILD)  is crucial for prognosis. Interstitial  pneumonias  are the most frequent variant of respiratory disease in patients with CTD.  CTD-related ILDs comprise 15% to 25% of all ILDs. Usually, respiratory disease develops in symptomatic CTD,  but in some cases, ILD can be the first appearance  of CTD which precedes systemic symptoms that could significantly impede early diagnosis. Therefore,  diagnostic workup in ILD should consider extrapulmonary  manifestations  of CTD.  Moreover,  an intrinsic part of diagnostic algorithm should be specific laboratory assessment including measurement  of autoantibodies.  Combination of specific antibodies and clinical features provides higher probability of CTD diagnosis.

Frequency and clinical manifestations of respiratory disease in systemic connective tissue diseases (CTD) differ significantly including clinical course and outcomes. Pulmonary  abnormalities  are seen in 20% to 95% of patients with CTD ranging from subtle to life-threatening disease and could be fatal. Commonly, CTD-related interstitial lung disease (ILD)  is crucial for prognosis. Interstitial  pneumonias  are the most frequent variant of respiratory disease in patients with CTD.  CTD-related ILDs comprise 15% to 25% of all ILDs. Usually, respiratory disease develops in symptomatic CTD,  but in some cases, ILD can be the first appearance  of CTD which precedes systemic symptoms that could significantly impede early diagnosis. Therefore,  diagnostic workup in ILD should consider extrapulmonary  manifestations  of CTD.  Moreover,  an intrinsic part of diagnostic algorithm should be specific laboratory assessment including measurement  of autoantibodies.  Combination of specific antibodies and clinical features provides higher probability of CTD diagnosis.

Лекарственно-индуцированные интерстициальные поражения легких (ЛИИПЛ) являются одной из наиболее частых форм лекарственных пневмопатий. ЛИИПЛ составляют около 3% в структуре всей интерстициальной патологии легких, и это требует такого же диагностического подхода, как и при интерстициальных заболеваниях легких другого генеза или идиопатических интерстициальных пневмониях. Лекарственные средства (ЛС) индуцируют различные варианты поражения паренхимы легких, нередко сочетая в себе несколько патогистологических паттернов. ЛИИПЛ чаще протекают изолированно, однако пневмопатии могут быть и частью системных патологических процессов, индуцированных ЛС. Характерных клинических, гистологических и радиологических особенностей для абсолютного большинства ЛИИПЛ не существует, что обусловливает гиподиагностику лекарственных пневмопатий. Основным диагностическим критерием ЛИИПЛ является хронологическая зависимость между приемом ЛС и возникновением клинических проявлений. Диагностические трудности нередко обусловлены поздней клинико-рентгенологической манифестацией (длительный период времени от начала приема ЛС или после его отмены) либо отсутствием улучшения состояния после прекращения приема потенциально «виновного» ЛС. Однако своевременная диагностика ЛИИПЛ представляется чрезвычайно важной, поскольку во многих случаях отмена препарата способствует купированию и разрешению патологического процесса.

Different strategies for disease control in real clinical practice are compared in terms of dynamics of functional parameters in patients with persistent bronchial asthma. This prospective multicentre surveillance study was carried out in 19 Russian clinics using the common protocol. The patients were divided in 3 groups in accordance with the changes of basal antiinflammatory therapy during the study period. Group A--stepwise increase in the extent of combined salmoterol/fluticason therapy, group B--long-term stable-dose salmoterol/fluticason therapy, group C--salmoterol/fluticason therapy with gradual decrease of the dose and/or transition to an alternative variant. Statistical analysis using Statistica 6.0 program included data from 543 patients. The results suggest that the two first modalities increased the level of control (ACT test) and improved characteristics of external respiration throughout the study period. Strategy 3 was associated with a decrease in the external respiration function and the level of control.

Целью исследования явилось изучение взаимосвязи клинико-функциональных нарушений, качества жизни (КЖ) и влияние на них терапии тиотропия бромидом (ТБ) у больных хронической обструктивной болезнью легких (ХОБЛ). У 138 пациентов с ХОБЛ различной степени тяжести проанализированы респираторные симптомы, параметры спиро и бодиплетизмографии, толерантность к физической нагрузке и КЖ. Результаты показали значительное влияние гиперинфляции легких на выраженность одышки, доминирующей в снижении КЖ и толерантности к физическим нагрузкам. Применение ТБ в течение 6 мес. при тяжелой ХОБЛ достоверно уменьшает выраженность одышки и гиперинфляции легких, повышая физическую толерантность и КЖ. Таким образом, уменьшение легочной гиперинфляции является наиболее важным фактором улучшения клиникофункционального статуса и КЖ у больных ХОБЛ.

The trial was aimed аt comparative evaluation of hyperinfl ation reversibility in chronic obstructive pulmonary disease (COPD) and asthma. Th e reversibility parameters of spirometry and bodyplethysmography in the bronchodilator test were analyzed in 35 outpatients (21 with COPD and 14 with asthma). Results revealed the post bronchodilator parameters of bodyplethysmography considerably diff er between COPD patients and asthma patients. In particular, the residual volume (RV) of COPD patients in majority of cases (95,2 %) is not normalized by bronchodilator inhalation and RV remains above 120 % from reference values, whereas RV reduces in asthma patients (92,9 %) and aft er bronchodilator RV is