[Results of surgical treatment of generalized emphysema of the lungs].

An analysis of examination and treatment results was made in 123 patients with generalized emphysema of the lungs and respiratory failure of II-III degree. The patients were divided into two groups according to the age: younger than 40 years old (group A - 9 patients),40 years old and older (group B - 114). A surgical reduction of lung volume was performed to correct the respiratory failure in 69 patients. The rate of postoperative complications consisted of 14.7% in group A and it was 42.2% in group B.

[Surgical treatment of respiratory failure in young patients with diffuse lung emphysema].

An analysis of results of examination and treatment of 8 young patients (from 25 to 17 years of age) with generalized emphysema of the lungs, pulmonary failure of the II and III degrees was made. The functional examination of the patients before operation has revealed changed respiration by obstructive type. Symptoms of dysplasia of the connective tissue were found in 4 patients. Surgical reduction of the lung volume was performed in 5 patients for correction of respiratory failure. There were neither serious complications nor lethality. Dyspnea by MMRC scale decreased in all the patients at minimum by 1 point during the first months after operation. Three years later 4 out of the operated patients (80%) had retained positive effect of operation. In patients who had no operative treatment the frequency of infectious complications, level of dyspnea during three years of follow-up remained at the same level, while functional indices continued worsening. The surgical method of treatment of severe respiratory failure allowed tolerance of physical exercise to be increased and quality of life of patients with diffuse lung emphysema to be improved.

[Use of inhaled tobramycin in patients with cystic fibrosis].

The results of a few studies evaluating the efficacy and safety of nebulized tobramycin solution used in patients with cystic fibrosis have been published to date. Pulmonary deposition of inhaled tobramycin and its lung produced concentrations are rather high (10-25 times greater than the minimum suppressing concentration) and systemic absorption is extremely low. The treatment makes it possible to improve external respiratory function, to lower the colonization of the bronchi with Pseudomonas aeruginosa, and, in many cases, to achieve its eradication. Inhaled tobramycin reduces the sputum level of inflammatory cytokines in patients with cystic fibrosis, which slows down irreversible lung changes. Inhaled antibacterial therapy results in a reduction in the number of exacerbations, the frequency of admissions, and the need for systemic antibiotics.

[Outcomes of the long-term treatment with a novel mucolytic drug pulmozin in mucoviscidosis patients]. / Opyt dlitel'nogo primeneniia novogo mukoliticheskogo preparata "pul'mozim" u bol'nykh mukovistsidozom.

AIM: To study clinical effectiveness and safety of a new mucolytic drug pulmozim (Switzerland) adjuvant to basic therapy in mucoviscidosis patients of different age in Russia. MATERIAL AND METHODS: 15 patients with mucoviscidosis aged 5-36 years, functional lung capacity and FEV-1 at least 40% received pulmozim for 30 days. The drug was given in a single daily dose 2.5 mg in inhalations. The study included three stages: initial 14 days--discontinuation of all mucolytic drugs, 30-day course of pulmozim, 14-day follow-up without pulmozim and other mucolytics. RESULTS: Pulmozim in the above regimen was effective in patients with mixed and moderate mucoviscidosis. The drug facilitates respiration, improves sputum rheology, normalizes general condition of the patient, is simple for use, has good organoleptic properties. CONCLUSION: Pulmozim is an effective mucolytic drug in combined treatment of mucoviscidosis.

[Genetic factors in predisposition to bronchial asthma]. / Geneticheskie faktory predraspolozhennosti k bronkhial'noi astme.

The ratio between the normal (+) and null (0) alleles of the genes encoding glutatione S-transferases M1 (GSTM1) and T1 (GSTT1) were studied in normal individuals from northwestern Russia (control group) and in patients with bronchial asthma (BA). The frequency of the GSTM1 0/0 genotype in the population sample was statistically significantly lower (37.8%) than in the BA patients (82.1%; chi 2 = 16.8; P < 0.001; w chi 2 = 15.7; alpha = 0.01). For the GSTT1 gene, similar data were obtained. The frequency of the GSTT1 0/0 genotype in healthy donors was statistically significantly higher (16.3%) than in the BA patients (73.7%; chi 2 = 28.5; P < 0.001; w chi 2 = 23.22; alpha = 0.01). A significant preponderance of the compound homozygotes for the GSTM1 and GSTT1 null alleles among the BA patients was observed. The frequency of the GSTM1 0/0, GSTT1 0/0 individuals among the patients was 57.9%, while it was only 4.7% among the controls (chi 2 = 27.4; P < 0.001).

[Correlation of results of immunocytochemical, electrophysiologic, and molecular studies in patients with cystic fibrosis]. / Korreliatsiia rezul'tatov immunotsitokhimicheskikh, élektrofiziologicheskikh i molekuliarnykh issledovanii u bol'nykh mukovistsidozom.

In 13 cystic fibrosis (CF) patients of 5 to 23 years of age with a known mutation spectrum of gene CFTR, sweat chloride values and nasal-potential differences (NPD) were measured and localization characteristics of the protein product of gene CFTR in the cells of nasal epithelium were studied. Sweat Chloride values were normal or boundary (24 to 62 mM/l) in six CF patients. In seven CF patients, these values were significantly above the estimates for the control group. On average, the NPD values were -44.7 +/- 2.2 mV (from -32.5 to -68.9 mV) and -17.2 +/- 1.8 mV (from -6.8 to -30.2 mV) in CF patients and the control group, respectively. Histochemical studies clearly revealed the localization of the CFTR protein on the apical membrane of the nasal epithelium. Depending on the type of mutation, the protein product of gene CFTR was either absent or regularly distributed in the cytoplasm in CF patients; it was not detected in the apical membrane. Thus, NPD measurements and the analysis of the localization of the protein product of gene CFTR in scrapes of nasal epithelium were shown to be additional, highly informative methods of CF diagnostics.

[Clinical significance of specific features of HLA antigen incidence in patients with mucoviscidosis and in their relatives]. / Klinicheskoe znachenie osobennostei raspredeleniia antigenov sistemy HLA u bol'nykh mukovistsidozom i ikh rodstvennikov.

The distribution of antigens in the HLA system was studied in 57 patients with mucoviscidosis and in their 43 relatives. The examinees were found to have increased incidence of the HLA antigens B40, CW2, CW3, CW4. The haplotype AIB40 was associated with the mutation carriage of the CF gene deletion F508 both in patients and their relations heterozygous for this mutation. The analysis of the level of HLA antigens in the tissue of CF patients may predict the development of particular clinical features of mucoviscidosis and the nature of the course of the disease.

[Outpatient treatment and follow-up of adolescents and adults with mucoviscidosis in a specialized center]. / Opyt dispansernogo nabliudeniia i ambulatornogo lecheniia podrostkov i vzroslykh mukovistziodozom v usloviiakh spetsializirovannogo tsentra.

Of 49 mucoviscidosis patients benefited more those who received adequate home care. These patients had only 1-2 episodes of pulmonary inflammation aggravation a year, remained socially active, avoided hospitalization. It is emphasized that such patients need continuous follow-up and check-ups.

[Etiology of the infectious process in pulmonary and mixed forms of mucoviscidosis in children]. / Etiologiia infektsionnogo protsessa pri legochnoi i smeshannoi formakh mukovistsidoza u detei.

The specific features of an infectious process were studied in 150 children treated for mucoviscidosis at the State Pulmonology Research Center, Ministry of Health and Medical Industry of the Russian Federation. Hemophilic bacilli, Staphylococcus aureus, and Pseudomonas aeruginosa were fond to play the leading role in the etiology of the infectious process in the bronchopulmonary system. Pneumococcal infection was first ascertained to be important in the course of the disease. Developmental stages of a pyoinfectious process from the onset of its contamination, acute infection to chronic one were followed up. The most severe, prognostically unfavourable course of the disease was demonstrated to be associated with Pseudomonas aeruginosa infection.

[Genetic aspects of chronic lung diseases (early diagnosis and prevention)]. / Geneticheskie aspekty khronicheskikh zabolevanii legkikh (ranniaia diagnostika i profilaktika).

According to the data of the Institute of Pulmonology of the USSR Ministry of Public Health about 20 per cent of the patients in the specialized pulmonological department had hereditary diseases of the lungs. The most frequent were pulmonary mucoviscidosis in adults, obstructive pulmonary affections with deficiency of alpha 1-inhibitor of proteases and Zivert Kartagener syndrome. Schemes for diagnosis of such diseases and dispensarization of the patients and probant families are described. Early diagnosis and medicogenetic consultations in the probant families are important for prophylaxis of development of severe forms of hereditary diseases of the lungs.

[Etiologic significance of hereditary deficiency of proteinase alpha1-inhibitor in the formation of respiratory diseases]. / Etiologicheskoe znachenie nasledstvennogo defitsita alpha1-ingibitora proteaz v formirovanii zabolevanii organov dykhaniia.

A study was made of the content of protease alpha 1-inhibitor and of the phenotyping of protease alpha 1-inhibitor subtypes in 666 patients with different chronic non-specific pulmonary diseases. It is concluded that pronounced deficiency of protease alpha 1-inhibitor is of importance in the formation of primary emphysema, chronic obstructive bronchitis and bronchial asthma. The clinical characteristics of obstructive pulmonary diseases marked by protease alpha 1-inhibitor deficiency have been investigated.

[Prognostic criteria of suppurative-destructive complications in patients with a severe course of acute pneumonia]. / Kriterii prognoza gnoino-destruktivnykh oslozhnenii u bol'nykh s tiazhelym techeniem ostroi pnevmonii.

54 patients with acute pneumonia were examined. There were 36 men and 18 women aged 16 to 72 years. Use was made of the clinico-roentgenological, bronchological and laboratory methods including biochemistry and immunoassays to assess the gravity of the patients' condition. The system protease inhibitors, the concentration of immunoglobulins in blood serum, circulating immune complexes, functional activity of neutrophils and monocytes of peripheral blood were under study. The major part of the patients (45 persons) did not develop destructive lesions, in 9 patients, pneumonia was complicated by suppurative-destructive lesions. In both patients' groups, the disease (in 90.3% of cases) ran its course with the signs of active virus infection. The risk factor for the destruction development was the rise of the laboratory gravity index to over 5-6 marks, a tendency towards the decrease of the phagocytic activity of monocytes and immunoglobulin M, the presence of unbalance in the system protease inhibitors at the expense of inadequate reduction of the antiproteolytic potential.

[External respiratory function in patients with primary pulmonary emphysema etiologically related to alpha 1-antitrypsin deficiency]. / Funktsiia vneshnego dykhaniia u bol'nykh pervichnoi émfizemoi legkikh, étiologicheski sviazannoi s defitsitom al'fa 1-antitripsina.

To improve the diagnosis of primary lung emphysema etiologically linked with alpha-trypsin deficiency, the authors provide the results of a detailed clinico-functional study of 6 patients with an appreciable deficiency of this enzyme. According to the clinical and functional signs, all the patients were diagnosed to have a typical, pronounced lung emphysema. Therefore, the measurement of the alpha-antitrypsin content plays the crucial part in differential diagnosis of primary (hereditary determined) and secondary (obstructive) emphysema.