No 6 (2018)

Hypermobility of the joints is a very common condition (from 6 to 57% in different populations), in which the amplitude of active and/ or passive movements in the joints exceeds the conventional average statistical rate. Hypermobility of the joints is one of the frequent manifestations of both undifferentiated and differentiated (syndromic) forms of connective tissue dysplasia. It can be a harbinger of systemic connective tissue diseases in combination with musculoskeletal pain syndrome.

In case of hypermobility joints syndrome, a high incidence of valvular and arrhythmic manifestations of heart connective tissue dysplasia is shown, their features and spectrum are studied. Aim of the research. To estimate the prevalence and spectrum of valvular manifestations of cardiac connective tissue dysplasia in hypermobility joints syndrome patients and their possible association with the features of the orthopedic status. Material and methods. 214 patients with hypermobility joints syndrome were examined in comparison with the control group (n = 40). X-ray and X-ray investigations of the spine, thorax, MRI of the spine, and joints were performed according to indications. For the verification of dysplastic valve syndromes, heart rhythm disturbances EchoCG, resting ECG and 24-hour ECG monitoring were used. Results. Polyfocal and combined skeletal dysplasias in hypermobility patients' phenotype are worked out in details. The prevalence and nosological characteristics of thorax and spine deformations, increasing the risk of unfavorable course of cardiac dysplastic syndromes with the formation of structural hemodynamic disorders and complications, have been fixed. Conclusion. The presence in the phenotype of hypermobility persons of polyfocal and associated skeletal dysplasia, predisposing to clinically significant deformations with a possible cardiac and large vascular trunks disposition classifies them as a risk group for the development and adverse course of cardiac dysplastic syndromes.

Aim of the study. To study the effect of connective tissue dysplasia (CTD) symptoms' influence at gastric acidity disorder in patients with acid-dependent diseases. Material and methods. 151 patients were included in the randomized controlled comparative one-stage study. All participants had general clinical examination, determination of CTD features, esophagogastroduodenoscopy, abdominal organs ultrasound, 24-hour pH-metry. Statistical processing of obtained data was carried out using the methods of parametric and nonparametric analysis. Classification of objects based on the results of 24-hour pH-metry was carried out using the program Deductor Studio Academic 5.3 using neural network algorithms by constructing Kohonen maps. Analysis of the effect of CTD signs at the formation of gastric acidity varieties was carried out using the technology of data mining - the construction of decision trees. Results. A unique set of signs of CTD influencing the development of gastric acidity variants is revealed, the strength and direction of this influence are determined. Hypoacid variant with high probability develops with gastroptosis, with the probability of medium strength (23%) - with deformation of the chest. The biliary variant develops with the probability of medium strength (27%) with duodenal-gastric reflux and gall-bladder deformity (30%). Reflux variant develops with a probability of medium strength (18%) with a decrease in body weight below 66.5 kg. The hyperacid variant develops with a probability of moderate strength with a decrease in body weight below 66.5 kg (15%) and without deformation of the chest (12%). The normoacid variant of gastric acidity develops with the probability of medium strength (21%) in the absence of dolichostenomelia. Conclusion. The obtained data on the diagnosis of gastric acidity variants can be used in the treatment of patients with acid-dependent diseases and signs of CTD.

Molecular indicators reflecting the state of stress-limiting systems of whole blood mononuclear leukocytes (MNCs) in patients undergoing community-acquired pneumonia (CAP) were studied. Purpose of the study. Study of the character of the dependence between the level of phosphorylation of terminal p38 proteinkinase, reflecting the state of the signaling pathway and the content in the cell of components that regulate the processes of proliferation, differentiation and cell death in CAP convalescents. Material and methods. We examined 30 male patients with mild course of bacterial CAP on the 15-17th day of the disease, before their discharging from the hospital. The control group consisted of 15 healthy young people taken from blood donors. Cellular content of MAPK / SAPK and JAK / STAT components of signaling pathways, the content of p53 protein and cytochrome C were evaluated. Also in cell supernatants, the level of molecules controlling apoptosis and proliferation, the members of tumor necrosis factor family (soluble forms of DR5 and DcR3 receptors, ligands TRAL, TWEAK, BAFF, LIGHT), as well as the concentration of antioxidants were investigated. The level of these indicators was investigated depending on the activity of p38 protein kinase, assessed by the level of its phosphorylation. Results. In patients undergoing CAP, on the phone of an increase of the proteinkinase p38 level, takes place an increase of the content of p53 protein in MNC, as well as an increase in the phosphorylation of the inhibitor of the transcription factor of the NF-kB. A statistically significant increase in phosphorylation of protein RB, the soluble form of DR5, TRAIL and BAFf was also detected. These changes were accompanied by a decrease in the level of phosphorylation of JAK2, STAT3 and CREB, as well as the concentrations of TWEAK, LIGHT and cytochrome C. No significant changes in the content of DcR3, STAT5A and AOC were found in groups with different p38 activities. A linear regression analysis revealed a statistically significant positive correlation of p38 activity with the level of phosphorylation of the retinoblastoma protein, STAT3, IkB, JNK and negative with the level of JAK2 phosphorylation. Conclusion. The activity of the MAPK/SAPK signaling pathway in the OLS of SAP convalescents is antagonistic to the JAK/STAT signaling pathway, weakening its activity with a decrease in the sensitivity of cells to the corresponding cytokines. At the same time, an increase of p38 phosphorylation is associated with an increase in IkB phosphorylation, determining the transcription of genes controlling apoptosis, proliferation and cell differentiation. Increased expression of the BAFF gene and increased production of cytokines promotes the activation of B-lymphocytes, while the secretion of TRAIL enhances apoptosis of sensitive cells. Thus, p38 activation in MNCs may contribute to an increase of body resistance and antitumor protection. Thus, the proapoptotic function of p38 could be realized by stimulating p53 production, expression on DR5 cells and their TRAIL production, while the proliferation and differentiation of B lymphocytes can be enhanced by stimulating BAFF production, which is obviously determined by its effect on nuclear transcription through modulation of the functional IkB activity .

Ehlers-Danlos syndromes (SED) as a manifestation of hereditary disorders of the connective tissue are distinguished by the multisystem damage and the variability of symptoms. The diagnostic criteria developed in 1998, which include the description of 6 types of SED, were successfully used for two decades. However, the results of clinical observation, biochemical and molecular studies are supplemented with new knowledge about the syndrome. New international classification of 2017 contains the diagnostic criteria of 13 types of SED, also hyper-mobile type features are revised there.

Do Russian doctors often make mistakes? And the American? Chinese, German, Brazilian? Unfortunately, there is no answer to this question - in many countries there is no official statistical data of medical errors (and sometimes no even a legislative definition!). It would seem that the more advanced the medicine becomes, the less should be medical errors there. In fact, as experts say, everything is not so simple. Diagnostic and therapeutic equipment is becoming more and more difficult to operate, and, therefore, requires more strict control and highly skilled, experienced personnel, whose training takes years. The development of diagnostic and treatment facilities leads to the identification of new diseases, a significant increase in the amount of medical knowledge, therefore, the work of a physician requires a constant increase in the level of qualification and is becoming more and more highly specialized. At the same time, if a patient has a difficult combination of diseases, sometimes it is very difficult for a "narrow" specialist to make a correct diagnosis and find the optimal treatment - you need advice from senior colleagues or a coordinated opinion of the council of physicians. However, sometimes doctors prefer to prescribe more and more new examinations and consultations of even more narrow specialists. In Russian realities, the situation is complicated by the interrelated problems of the severe shortage of medical personnel, poor wages, too much amount of work for medical personnel, non-stop updating of standards and procedures for providing medical care, which creates a lot of formal (bureaucratic) obstacles, etc. What is the price of medical errors? The popular opinion of enthusiastic media lovers is: hundreds of patients are dying by fault of the doctors. And if they survive, then, as a rule, they become disabled. Fortunately, this myth is hopelessly distant from reality. But there is a very real problem which is considered not so much by patients, frightened by lots of negative information as by doctors themselves, - the growth of unnecessary («idle») costs for health care servicesboth in developed and developing countries due to the implementation of expensive new high-tech methods of diagnosis and treatment, as well as due to the progressive population aging. In this case, according to the statistics, the increase of costs is not accompanied by an adequate increase of life duration. The analysis has shown, that almost a third of the funds allocated for health care are wasted for redundant, ineffective, and sometimes even dangerous interventions. How to get rid of this «ballast», to provide safe, high-quality and affordable medical care? Anton Chablin, chief editor of «We will be healthy» medical club (Stavropol), spoke on that topic with the doctor of medical sciences, physician and cardiologist - Olga BOEVA.

Acute respiratory viral infections (ARVI), including influenza, have the most severe course in patients with associated somatic diseases and require a certain approach to therapy. The data review of Ergoferon use in treatment and prevention of ARVI and influenza in patients with different associated diseases, including patients from the risk groups, has been presented. The results show that Ergoferon possesses a good efficiency and safety ratio and can be recommended for the treatment and prevention of ARVI and influenza in patients with comorbid diseases.