[Sarcoidosis with Cardiac Involvement and Monoclonal Gammopathy].

The article presented a clinical case of 8-year follow-up of a patient with a relatively beneficial course of pulmonary sarcoidosis and intrathoracic lymph nodes and with gradually progressing myocardial injury. A specific feature of the case was that the patient had monoclonal gammopathy, which is extremely rarely reported. Morphological signs of paraproteinemic hemoblastosis and amyloidosis were not observed. The article considered a whole complex of studies, which is required for diagnosing cardiac sarcoidosis in a specific patient and addressed the issue of monoclonal gammopathy origination.

[Comorbidity of Chronic Obstructive Pulmonary Disease and Cardiovascular Diseases: Place of Therapy with Modern ß-Adrenoblockers].

Chronic obstructive pulmonary disease (COPD) is the fourth largest cause of worldwide mortality.  Presence of comorbidities is registered in 96% of COPD patients. The most important of these are cardiovascular diseases (coronary artery disease, arterial hypertension, chronic heart failure), which contribute to COPD patients' mortality in every third case. COPD and cardiovascular diseases have common risk factors and pathogenesis mechanisms. Cardioselective beta-blockers reduce morbidity risk and frequency of COPD exacerbation, are effective and safe in treatment of COPD patients.

Drug-Associated Acute Lung Injury During Amiodarqne Uptake.

In this article we present a clinical case of a quick recovery and restoration of pulmonary function after withdrawal of amiodarone in a patient with drug-associated acute lung injury induced by intake of amiodarone for two months.

[MODERN APPROACH TO THERAPY OF PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS].

A case of the treatment of a patients with idiopathic pulmonary fibrosis with pirfenidone and nintedanib is reported with reference to the role of these medications in therapy of his condition in the context of modern theory of its pathogenesis.

[A CASE OF FAMILIAL PULMONARY AND HEPATIC ECHINOCOCCOSIS)].

Echinococcus granulosus still remains a widespread disease. Moreover, there is a large number of patients in whom it is diagnosed rather late because its clinical presentation can be unspecific and symptoms vary. We report a familial case of the disease. Its specific feature was the onset with the bilateral pulmonary process and late involvement of the liver. This excluded other pulmonary diseases, such as pneumonia, tuberculosis or cancer.

[Exacerbation of chronic obstructive pulmonary disease: the role of the molecular genetic method for the identification of pathogenic agents].

The study dealt with etiological diagnostics of bacterial exacerbations of chronic obstructive pulmonary disease (COPD). Samples of bronchial secretion were obtained from 73 patients hospitalized in connection with COPD exacerbations and investigated by the culture method and PSR. The former technique revealed bacterial pathogens in 64 (82%) patients. Gram-positive bacteria (Streptococcus pneumoniae) predominated in patients with stage I and II COPD, Gram-negative (Pseudomonas aeruginosa) and mixt infection in those with stage III and IV COPD. CPR proved more sensitive for isolation of DNA of Gram-negative pathogens (Haemophilus influenzae and Enterobacteriaceae).

[EFFICIENT TREATMENT OF CHRONIC RESPIRATORY INSUFFICIENCY IN PATIENTS WITH KYPHOSCOLIOSIS].

We report efficient treatment of chronic respiratory insufficiency in patients with congenital kyphoscoliosis by non-invasive auxiliary ventilation and low-flow oxygen therapy. It proved possible to effectively control severe chronic respiratory insufficiency under conditions of a pulmonological ward without application of means and measures of intensive therapy.

[A case of lymphogranulomatosis in a patient with type 1 neurofibromatosis].

Neurofibromatosis is a common monogenic disease in man; its incidence is at least 1:3000-1:4000 population. It is inherited in the autosomal dominant mode with high penetrance and variable expression. A patient presenting with type 1 neurofibromatosis since the age of 3 mo who developed lymphogranulomatosis is reported. The patient 1 life prognosis is presently determined by the competing pathology. It is emphasized that neurofibromatosis is associated with different forms of lung lesions and creates a risk of neoplastic (including malignant) growth.

[Polymorphic variants of a surfactant protein C gene in patients with chronic obstructive pulmonary disease].

AIM: To investigate effects of polymorphic variants of surfactant protein C gene (SFTPC) on the course of the disease in patients with chronic obstructive pulmonary disease (COPD). MATERIAL AND METHODS: Sixty COPD patients were examined with COPD of stage 0-IV. The following parameters were studied: smoking index (SI), bronchial patency, lung pathology by the findings of high resolution computed tomography, polymorphisms of SFTPC gene by the data of polymerase chain reaction (PCR) followed by mass-spectrometric detection. A trend was found to correlation between a polymorphic variant of A138C and COPD severity (p = 0.056). A CC genotype occurred more frequently in patients with COPD stages III and IV. A significant correlation exists between a polymorphic variant of A186G and the disease course severity (p = 0.23). Genotypes AA or AG are more typical for patients with COPD stage 0, I and II; GG genotype--stage III and IV. Genotype CC SFPTC A138C and genotype GG SFTPC A186G are significantly associated with pulmonary emphysema (p = 0.004 and p = 0.01, respectively). CONCLUSION: Polymorphic variants of SFTPC affect COPD course.

[Efficacy of a cardioselective beta1-adrenoblocker bisoprolol in patients with chronic obstructive pulmonary disease in combination with ischemic heart disease].

AIM: To study effects of a selective beta1-adrenoblocker (B1AB) bisoprolol fumarate (conkor, Nikomed, Germany) on severity of pulmonary hypertension (PH), bronchial obstruction and bioelectric activity of the myocardium in patients with chronic obstructive pulmonary disease (COPD) associated with ischemic heart disease (IHD). MATERIAL AND METHODS: Effects of an 8-week course of bisoprolol therapy on pulmonary hypertension, its efficacy and safety were studied in 30 IHD patients with COPD of stage III-IV on broncholytic therapy. The comparison group consisted of 45 IHD patients with COPD untreated with basic bisoprolol therapy. The following parameters were assessed: chest x-ray data, mean pulmonary artery pressure (MPAP) by echocardiography findings, frequency of anginal attacks by ECG monitoring, bronchial permeability by investigation of external respiration function, partial pressure and carbonic gas in blood, oxygen blood saturation (pO2, pCO2 and SaO2). RESULTS: Bisoprolol has decreased MPAP from 23.8 +/- 0.8 mmHg to 21.9 +/- 1.0 mmHg, by 8%, p < 0.05; frequency of anginal attacks, heart rate from 82.2 +/- 1.4 to 73.2 +/- 1.5 b/min; number of episodes of ST segment depression from 2.35 +/- 0.43 to 0.95 +/- 0.22, p < 0.01; total duration of ST segment depression from 10.1 +/- 2.54 to 2.89 +/- 0.76 min, p < 0.01; number of supraventricular and ventricular extrasystoles for 24 hours from 194.5 +/- 74.4 to 96.2 +/- 27.4 and from 239.1 +/- 124.9 to 111.3 +/- 44.1, respectively. Parameters of a 6 min walk test improved from 326.7 to 442 m, p < 0.01. Bisoprolol had no negative effect on bronchial obstruction. CONCLUSION: Bisoprolol is well tolerated, effective and safe in COPD patients with IHD.

[Types of structural-functional disorders in severe chronic obstructive pulmonary disease]. / Tipy strukturno-funktsional'nykh narushenii pri tiazhelom techenii khronicheskikh obstruktivnykh zabolevanii legkikh.

To differentiate approaches to assessment of treatment and prognosis specificities in a severe course of chronic obstructive pulmonary diseases (COPD), a study was made of 30 patients with long-term COPD. The patients were divided into groups by the severity of respiratory insufficiency which was investigated by saturation of 0(2) and gas composition of arterialized capillary blood, flow-volume curve and bodyplethysmography, ECG, echo-CG. High resolution computed tomography of the lungs was used to evaluate diffuse changes of pulmonary tissue and its morphological rearrangement. Basing on the rhoentgenomorphological picture, most typical variants of pulmonary tissue changes were identified. Comparison with the clinical and laboratory-instrumental data allowed to characterize four structural-functional types which occur in severe COPD. Etiological and pathogenetic features of the above types (the role of smoking, in particular) and their contribution to development of pulmonary hypertension, treatment and prognosis are discussed.

[Bronchiolar-alveolar cancer in idiopathic fibrosing alveolitis]. / Bronkhiolo-al'veoliarnyi rak na fone idiopaticheskogo fibroziruiushchego al'veolita.

An observation of bronchiolo-alveolar carcinoma that developed in the presence of the idiopathic fibrosing alveolitis (IFA) in a woman of 57, is described. The duration of IFA was 12 years. Diffuse pneumosclerosis with the development of the so-called "honey-comb" lungs was observed. Numerous confluent foci of the bronchiolo-alveolar carcinoma in both the lungs are found in the presence of alterations typical for IFA (diffuse sclerosis of alveolar septa, microcystosis). The cause of death was progressing respiratory deficiency.