[Progressive fibrosing lung disease. Discussion aspects of the problem: A review].

The authors of the article prove the need to include a new name for the disease - "Progressive Fibrosing Lung Disease" into clinical practice. Recognition of the fact that some lung diseases end in a fibrosing process, which does not have any significant differences depending on the initial disease that led to fibrosis, will expand the indications for earlier prescription of antifibrotic drugs, which will undoubtedly improve the prognosis in this extremely severe category of patients.

[Pulmonary epithelioid hemangioendothelioma]. / Epitelioidnaya gemangioendotelioma legkikh.

The article describes the clinical, radiological and pathological features of epithelioid hemangioendothelioma (EHE) in 27 adult patients, mainly female. In all cases, with the exception of one, there was a benign course of the disease over many years with a tendency to stabilize growth, the morphological sign of which was the development of widespread sclerotic changes. With the help of immunohistochemical method, the endothelial nature of EHE cells and its relatively low proliferative potential were confirmed. Clinical and morphological features of EHE raise the question of the essence of proliferation of endothelial cells with the formation of tumor-like nodes. There is every reason to consider EHE as a pseudotumor of the type of nodose hyperplasia in the nosological group of dyshormonal hyperplasia, similar to benign leiomyoma of the uterus with lung damage, as we have previously proposed.

[Idiopathic interstitial pneumonias].

The article presents the evolution of views on one of the current problems of present pulmonology idiopathic interstitial pneumonias. On the basis of many years of experience in diagnosis and treatment of patients of IIPs in the clinic of pulmonology of Interstitial and Orphan Lung Diseases Research Institute of Pavlov First Saint Petersburg State Medical University, the authors formulated a new understanding of this pathology and proposed to unite all IIPs under the term idiopathic fibrosing pulmonary disease. Using the concept of idiopathic fibrosing pulmonary disease will make it possible to substantively address the issues of early diagnosis, determine the criteria for the activity of the pathological process, and there by develop an evidence base for the adequate prescription of antifibrotic drugs and corticosteroids.

DIAPHRAGM KINETICS IN PULMONARY FIBROSIS PATIENTS AND GASTROESOPHAGEAL REFLUX.

72 patients with pulmonary fibrosis were examined with ultrasound method. Some of them have also gastroesophageal reflux. Changes in amplitude of diaphragm motion during intensified breathing were informative. Diaphragm level in patients with gastroesophageal reflux was lower than without refiux.

[Computed tomography in evaluating the development of different types of pulmonary fibrosis in patients with interstitial lung diseases].

OBJECTIVE: to assess the favorable and unfavorable types of lung tissue fibrotic changes in patients with interstitial lung diseases (ILD) detected by high-resolution computed tomography (HRCT). MATERIAL AND METHODS. The results of examinations were analyzed in 385 patients: 181 with respiratory organ sarcoidosis, 130 with fibrosing alveolitis, 36 with histiocytosis X, and 38 with lymphangiolciomyomatosis. All the patients underwent HRCT; the data were compared with the results of comprehensive functional study of external respiration (CFSER), histological examination (in 70.1%), and the pattern of the disease. RESULTS: Comparison of the clinical and functional course of ILD with the types of lung tissue fibrotic changes detected by HRCT and morphological examination showed that the favorable types of pulmonary fibrosis included stringy central and peripheral interstitial fibrotic changes and the atelectatic type of fibrosis, the occurrence of which failed to affect the development of obvious perfusion and diffusion disorders and to give rise to respiratory failure. The unfavorable types of pulmonary fibrosis included the peripheral pulmonary interstitial fibrotic changes (acinar fibrosis, honeycomb lung), which led to restrictive changes and perfusion disorders, which were accompanied by significant respiratory failure, decreases in quality of life and survival, as well as fibrotic changes in the walls of long-lasting air-containing cysts and a fibrotic Aevity mass that resulted Ind complications (pulmonary hemorrhage, pneumothorax, and pneumomediastinum). CONCLUSION: The type of pulmonary fibrosis development is one of the major prognostic criteria for he course of ILD. HRCT makes it possible to assess its clinical picture as a whole and to reveal the type of development of fibrotic changes, their extent, the degree of involvement of the tracheobronchial tree in the process (formation of different types of bronchiectasis), concomitant extrapulmonary changes that may be clinically and functionally relevant (chest bone frame deformation, varying pleural changes, vascular disorders). Estimation of prognosis of the disease is most effective in comparing clinical, morphological, HRCT, and CFSER data.

[Immunogenicity and safety of a prototype chemical anthrax vaccine in laboratory animal models].

AIM: Evaluation of immune stimulating and toxic effects of a vaccine prototype protein components. MATERIALS AND METHODS: Linear mice, guinea pigs and rabbits were immunized subcutaneously once or twice by recombinant protective antigen (rPA), S-layer protein (EA1) or their complex. Innate immunity structure activation was registered by changes in Toll-like receptor (TLR) expression. Adaptive immune response parameters were determined by established methods. Toxicity of the preparations was determined using flow cytofluorometry and densitomorphometry. RESULTS: The ability of rPA and EA1 to activate structures of innate immunity - TLR 2 and 6 - was established. Features of anti-PA antibody titer dynamics for each of the animal species was determined, a comparison with antibody formation during immunization with Bacillus anthracis STI- 1 was carried out. 2 immunizations ofbiomodels with a complex preparation combined with an adjuvant provides protection from infection by a test-strain that is comparable with protectivity of a live vaccine. Evidences regarding damaging effect of rPA and EAI on cells and tissues of macro organism were not detected throughout the study. CONCLUSION: Aprototype of a chemical anthrax vaccine under development has high immunogenicity and its protein components are not toxic for laboratory animals based on the results of complex testing.

The effects of N-acetyl-cysteine and acetyl-L-carnitine on neural survival, neuroinflammation and regeneration following spinal cord injury.

Traumatic spinal cord injury induces a long-standing inflammatory response in the spinal cord tissue, leading to a progressive apoptotic death of spinal cord neurons and glial cells. We have recently demonstrated that immediate treatment with the antioxidants N-acetyl-cysteine (NAC) and acetyl-l-carnitine (ALC) attenuates neuroinflammation, induces axonal sprouting, and reduces the death of motoneurons in the vicinity of the trauma zone 4weeks after initial trauma. The objective of the current study was to investigate the effects of long-term antioxidant treatment on the survival of descending rubrospinal neurons after spinal cord injury in rats. It also examines the short- and long-term effects of treatment on apoptosis, inflammation, and regeneration in the spinal cord trauma zone. Spinal cord hemisection performed at the level C3 induced a significant loss of rubrospinal neurons 8 weeks after injury. At 2 weeks, an increase in the expression of the apoptosis-associated markers BCL-2-associated X protein (BAX) and caspase 3, as well as the microglial cell markers OX42 and ectodermal dysplasia 1 (ED1), was seen in the trauma zone. After 8 weeks, an increase in immunostaining for OX42 and the serotonin marker 5HT was detected in the same area. Antioxidant therapy reduced the loss of rubrospinal neurons by approximately 50%. Treatment also decreased the expression of BAX, caspase 3, OX42 and ED1 after 2 weeks. After 8 weeks, treatment decreased immunoreactivity for OX42, whereas it was increased for 5HT. In conclusion, this study provides further insight in the effects of treatment with NAC and ALC on descending pathways, as well as short- and long-term effects on the spinal cord trauma zone.

[Computed tomography in the assessment of causes of restrictive lung disorders in patients with pulmonary histiocytosis X].

OBJECTIVE: To estimate the capabilities of computed tomography (CT) in revealing the anatomic causes of restrictive lung changes in patients with pulmonary histiocytosis X. SUBJECT AND METHODS: The results of examining 36 patients with pulmonary histiocytosis X, who underwent comprehensive functional study of external respiration (CRSER), CT, and high-performance CT (HPCT), were analyzed. RESULTS: According to the results of CRSER, the authors identified a group of patients with restrictive ventilation disorders, which included 7 men and 1 woman. The most common cause of restrictive disorders was generalized fibrous changes in lung tissue, which fails to result in its expansion. Multiple cysts were another cause of restrictive disorders in 2 patients. Fusion of individual cysts into large ones and the formation of a great number of paradoxically ventilated cysts were the third cause of restrictive disorders in pulmonary histiocytosis X, which was identified during HPCT. CONCLUSION: Comprehensive morphofunctional examination involving CRSER and high-resolution CT can reveal the unfavorable course of pulmonary histiocytosis X with the restrictive type of lesion.

[Differential diagnosis of lymphoid infiltrates in the lungs].

Clinico-morphological and immuno-histological data on 15 cases of marked lymphocytic lung tissue infiltration are presented. The nature of B- and T-lymphocyte distribution, their proliferative activity as well as patterns of distribution of cells synthesizing light chains of immunoglobulins are described. In 12 cases, lymphocytic interstitial pneumonia was diagnosed; it featured distinct boundaries between zones of B- and T-lymphocytes of proliferating and resting cells. However, non-Hodgkin's lymphoma (MALT) detected in 3 cases lacked such a pattern. That might be interpreted as a marker of the particular pathology.