Medicni perspektivi, 2021;26(3)
2021 Vol. XXVI N 3
Key words: bone implants, functional-protective coatings, corundum ceramics, bone healing, osteoinduction, osteoclasts, peri-implant regeneration
Abstract. Histological and histochemical assessment of short-term events in peri-implant bone for osteoinductivity evaluation of functional-protective implant coatings. Loskutov O.Ye., Shponka I.S., Bondarenko O.O., Bondarenko N.S., Bozhko A.G. Utilization of functional-protective coatings for implants based on corundum ceramics seems promising from the point of view of stability, bioinertness, and low cost. In order to study the histological criteria for evaluating the osteoinductive properties of functional protective coatings, 6 types of coatings were studied on an experimental animal model: 90 Wistar rats were implanted with 6 test types of implants with various combinations of surface treatment (sand-blasting, surface treatment with a plasma torch with simultaneous application of aluminum oxide, powdered titanium, etc.) with an exposure of 1, 2 and 4 weeks. After euthanasia, a histological examination of decalcified bone with Masson-Goldner trichrome staining and TRAP-histochemical reaction for osteoclasts was performed. The obtained results demonstrated significantly higher osteoinductive properties of functional protective coatings with a more pronounced roughness (Ra>10 μm) compared to an untreated titanium surface after 2 (p<0.01) and 4 (p<0.05) weeks of implantation. The corundum ceramic coating prevented the formation of implant wear particles, hence contributed to the stabilization of the newly formed bone. Therefore, the use of functional protective implant coatings based on corundum ceramics can increase the survival rate of conventional titanium implants, since the combination of factors such as surface roughness, mechanical stability, and chemical inertness of coatings with corundum ceramics provides better osteoinductive properties of implant materials.
Key words: atrial fibrillation, risk factors, risk factor modification, American Heart Association
Abstract. Risk factors of atrial fibrillation and their modification: a new look at a long-term problem. Babkina T.M., Smyrnova H.S., Hladka L.Yu., Polishchuk O.V. Atrial fibrillation (AF) is one of the most common arrhythmias diagnosed in clinical practice. Today, AF is associated with an increased risk of mortality, acute brain disorders, and heart failure. The results of many studies suggest that the incidence of AF is directly related to such risk factors (RF) as age, obesity, hypertension, diabetes mellitus, obstructive sleep apnea, alcohol, tobacco smoking etc. Recently published studies have described the positive impact of lifestyle changes on the incidence of AF. Therefore, it is believed that early detection of RF could prevent or delay the onset of AF. New data suggest the need to review the management of patients with AF. In 2020, the American Heart Association has presented a scientific statement that highlighted the relationship between modified RF and AF, discussed the implementation of strategies for effective modification of RF, as well as identified areas for new research. The data allow us to conclude that the correction of RF helps to reduce the incidence of AF. Future research is needed to establish the effects and generalizability of systematic lifestyle and RF modification for AF: to study the role of pericardial/epicardial fat accumulation in risk and progression of AF; determination of the most effective weight loss techniques for long-term success in reducing AF; effects of high-intensity/short-duration interval training on left atrium structural and electric remodeling; the degree of glycemic control for AF.
Key words: pesticides, working conditions, occupational risk, type of spraying, ecotoxicological risk, dispersion, biological efficiency
Abstract. Hygienic evaluation of the most common methods of agricultural crops treatment with chemical protection products (literature review). Borysenko А.А., Antonenko А.М., Shpak B.I., Omelchuk S.Т., Bardov V.G. Global strategies, including application of chemical plant protection products, are important in the cultivation of safe corps and preservation of human health. A promising area of preventive medicine, agronomy, agroecology and agroengineering is the optimization of methods of pesticide formulations application. The aim of the work was a hygienic assessment of the most common and latest methods of crops treatment with chemical pesticides. Results. The biological effectiveness of different types of pesticides is achieved by different application rates of the working solution. The most common method of pesticide application is spraying, which ensures the application of pesticides in the drop-liquid state and is characterized by low consumption of active substance per unit area, variable-controlled distribution on the treatment surface, provides good adhesion and retention on facilities, allows the use of combined formulations. One of the most effective ways to minimize the negative impact of chemical plant protection products and achieve economic success is a rational approach to the choice of pesticide application, as it takes into account the hazard class of pesticides, the presence of water protection zones, the sensitivity of target crops, etc. Conclusion. Rational application of pesticides includes minimizing the overall effect of pesticides on human health and the environment and achieving high-targeted biological efficiency. Adherence to the methodology of choosing the type of pesticide application and selection of the type of spray is a key point in optimizing the rational use of chemical plant protection products, which requires a detailed study from the standpoint of both efficiency and safety. Control over compliance with the recommendations should be included in sanitary-hygienic and sanitary-ecological monitoring.
Key words: children, eating behavior, liver steatosis, nonalcoholic steatohepatitis
Abstract. Features of eating behavior in children with different forms of non-alcoholic fatty liver disease. Stepanov Yu.M., Zavhorodnia N.Yu., Zavhorodnia O.Yu. Aim – to study the features of the eating behavior (EB) in children with different forms of nonalcoholic fatty liver disease (NAFLD) - simple steatosis (SS) and nonalcoholic steatohepatitis (NASH), to compare the obtained data with the eating behavior of children without liver steatosis. 120 patients aged 7 to 16 years were examined, the average age of the patients was (12.00 ± 2.56) years. The presence and degree of liver steatosis was determined by transient elastography using a FibroScan®502Touch with an assessment of CAP (controlled attenuation parameter). According to the CAP, gender-specific alanine aminotransferase levels and body mass index, patients were divided into 4 groups: 1 group consisted of 26 patients with NASH, 2 group – of 44 patients with SS, 3 group – of 35 patients with obesity without liver steatosis, 4 group (control) – of 15 patients with normal weight without metabolic disorders. The groups were homogeneous in age. Assessment of EB was carried out using the pediatric modification of the Dutch eating behavior questionnaire (DEBQ-C). It was found that most patients with SS (75,0%) and NASH (87,5%) demonstrated an emotional type of EB. The restrictive type of EB was predominant in obese children without hepatic steatosis (73,7%). The total score of the restrictive type of EB in children of the 3 group was significantly higher compared with the data of the 4 group (p<0,05). The external type of EB occurred in 25% of children with SS and was not observed in children with NASH. Thus, children with NAFLD and obesity are characterized by an increased incidence of various types of unhealthy eating behavior compared to children with normal weight, which requires appropriate correction and involvement of psychologists in a team of specialists involved in the health care of the obese children.
Key words: artificial bladder, electromyographic, perineum, pelvic floor, bladder cancer
Abstract. Electromyographic features of the perineum and pelvic floor in patients with an artificial bladder. Savchuk R.V., Kostyev F.I., Golovko S.V., Dekhtiar Y.M., Zalyva K.A. Aim – to assess the electromyographic features of the pelvic floor muscles and the sphincter apparatus in patients who underwent radical cystprostatectomy with the formation of an artificial bladder.The main study group consisted of 57 patients with muscle invasive bladder cancer who underwent a standard radical cystprostatectomy with ileocystoplasty. The study of the pelvic floor muscular bioelectric activity with computed electromyography (EMG) of the sphincter apparatus of the pelvic organs was carried out on a 2-channel computer electromyograph "NeuroTrac ™ MyoPlus4". The obtained results of the study of the bioelectric activity of the pelvic floor muscles showed a decrease in the amplitude of contractions in the Work Average mode by 42.1% (p≤0.001) for the perineal electrode, and by 35.7% (p≤0.05) for the rectal electrode, compared with the control group, which indicates a low contractility of the muscular diaphragm of the pelvis and may cause incontinence in patients with an artificial bladder. The average deviation over the entire duration of the session in Work mode in the group of patients with neobladder in channels A and B was 2.3 (p≤0.05) and 1.9 (p≤0.05) times higher, respectively, compared with control group. These data indicate an imbalance in the muscle tone of the pelvic floor in patients after extensive reconstructive intervention on the pelvic organs and can potentiate urodynamic disorders in the study group of patients. The average amplitude (Rest Average) of the activity of biopotentials in the resting state of the pelvic floor muscles along channels A and B is higher by 42.4% and 47.6% (p≤0.05), in comparison with the control group, which indicates insufficient relaxation and rest of striated muscles and sphincter. Despite the change in bioelectric potentials from the rectal electrode in the study group of patients, there were no signs of functional insufficiency of the anal sphincter, in contrast to the severity of urinary incontinence, which correlated and corresponded to the results of bioelectrical changes obtained through channels A and B, up to oscillations and loss of complete control. The EMG analysis of the pelvic floor muscles revealed characteristic changes in the biopotentials of the pelvic sphincters and indicated their relationship with the clinical features of the rehabilitation of this group of patients and the prospect of including the biofeedback method. It is a derivative form of the electromyographic signal in the treatment of incontinence in patients with neobladder.
Key words: atopic dermatitis, children, phenotypes, risk, cutaneous T-cell attracting chemokine
Abstract. The role of cutaneous T-cell attracting chemokine in the development of different phenotypes of atopic dermatitis in children. Dytiatkovsky V.O., Abaturov O.Ye., Naumenko N.V., Alifirenko O.O., Filatova I.A., Taran S.M. The goal of this study was to detect the risk of developing different atopic dermatitis (AD) phenotypes in children (isolated or combined with other comorbid atopic diseases (AtD)) depending on serum concentrations of cutaneous T-cell attracting chemokine (CTACK)/CCL27. The main group comprised 39 children aged 3 to 18 years old suffering from different AD phenotypes – isolated (18 patients) and combined with comorbid AtD – AR/ARC and/or bronchial asthma (21 patients). The control group comprised 47 children aged 3 to 18 years old, suffering from diseases of the gastrointestinal tract (GIT). Serum CTACK/CCL 27 concentrations were detected in all children. In the full main group, the average level of CTACK/CCL27 was significantly higher compared to the patients of the control group: 4403.6 pg/ml (95% CI: 3726.2; 5148.7, p<0.001) and 3495.9 pg/ml (95% CI: 3197.8; 4186.8, p<0.001), respectively. Mean serum CTACK/CCL27 levels in patients of the main group with different AD phenotypes were higher than those in the full main group: with isolated AD – 4549.4 pg/ml (LQ; HQ: 3923.5; 5175.2, p<0.05), with AD associated with other AtD – 5116.6 pg/ml (LQ, HQ: 4062.8; 6170.5, p<0.05). In phenotypes of overall and isolated AD, the cut-off value of serum CTACK/ CCL27 is 3586.5 pg/ml (76.9% and 77.8%, respectively, and 38.3% in the control group). The risk of development at this concentration is 5.37 (95% CI: 2.05; 14.07, p<0.001) for the total AD phenotype and 5.64 (95% 1.56; 20.32, p<0.05) for the isolated AD phenotype. In AD phenotype combined with comorbid AtD, the cut-off value of serum CTACK/CCL27 is 4308.8 pg/ml (66.7% of the main and 21.3% in the control group). The risk of developing this AD phenotype at this concentration is 7.40 (95% CI: 2.30; 23.76, p<0.001). Serum CTACK/CCL27 levels are the reliable biomarker of the risk for developing different AD phenotypes in children. In the serum level of CTACK/CCL27=3658.5 pg/ml, the significant risk of developing total AD phenotype is 5.37, and isolated – AD=5.64. In the serum concentration of CTACK/CCL27=4308.8 pg/ml, the significant risk of developing AD phenotype combined with comorbid AtD is 7.40.
Key words: systemic autoimmune diseases, Epstein-Barr virus, antiviral therapy
Abstract. Effectiveness of treatment of patients with systemic autoimmune diseases on the background of reactivation of persistent Epstein-Barr virus infection. Lishchuk-Yakymovych K.O., Haiduchok I.H., Ischeykin K.E., Chopyak V.V. The article presents the study of effectiveness of inosine pranobex (IP) in patients with systemic autoimmune diseases (SAD) on the background of reactivation of persistent Epstein-Barr (EBV) infection. Among 380 patients with SAD (systemic lupus erythematosus, systemic vasculitides, rheumatoid arthritis, psoriasis), in 144 patients (37.9%) the reactivation of persistent EBV infection was detected through virus DNA identification using polymerase chain reaction (PCR) in three biological matrices (blood, saliva, scraping from the lesion site). 48 patients were receiving inosine pranobex at a dose of 50 mg/kg per day for three months. Treatment efficacy was controlled by studying the levels of expression of miR-146а, miR-155, miR EBV (BART-13 and BART-15), TLR9, the quantity of lymphocytes populations and subpopulations. After treatment, PCR results showed a decrease in viral replication in 66.7% of cases. The use of IP contributed to a significant decrease in the level of IgM, IgG specific antibodies, an increase in the level of expression of anti-inflammatory miR-146a, a decrease in the level of expression of pro-inflammatory miR-155 which may signify the strengthening of antiviral control. The study data demonstrated the decrease in the expression of miR EBV (BART-13 and BART-15) and TLR9 on the immunocompetent cells that can also be attributed to the criteria for IP effectiveness. The effectiveness of IP was also proved by the stabilization of cell mechanisms, namely the tendency to normalizing T and B cell populations, decrease in the number of natural killer cells and activated cells (CD25+, CD3+ HLA DR+). On the other hand, the number of lymphocytes with suppressor activity (CD4+25+) remained significantly high mitigating autoimmune aggression. The results of the study show that the use of IP for treating the acute phase of EBV infection contributed to the decrease of repliсative activity of the virus; suppressing the aggressiveness of autoimmune reactions. The decrease in the expression of miR EBV (BART-13 and BART-15) can be recommended as a criterion for the IP effectiveness; the decrease in the expression of TLR9 on immunocompetent cells –as a criterion for suppressing autoimmune reactions.
Key words: epidural steroid injections, degenerative changes, spine
Abstract. The role of epidural steroid injections in the treatment of pain in patients with degenerative changes in the lumbar spine. Fishchenko Ia.V., Roy I.V., Kravchuk L.D. Epidural steroid injections of the lumbar spine are a common interventional procedure that is used to alleviate radicular pain resulting from degenerative changes in the spine. Although several studies have compared epidural steroid injections with placebo with favorable outcomes, randomized controlled trials in this direction are needed. The purpose of the study was to evaluate the effectiveness of the use of epidural steroid injections in the treatment of pain in patients with degenerative lesions of the lumbar spine. During the study, 262 patients with degenerative lesions of the spine at one or two levels of the vertebral-motor segment (VMS) were selected. Epidurally transforaminally under fluoroscopic control all patients received steroid injections at the appropriate level (s) of VMS on the basis of the rehabilitation department of the Institute of Traumatology and Orthopedics of the National Academy of Medical Sciences of Ukraine during 2017-2019. Of the 262 patients who received epidural steroid injections, 204 were able to reduce pain and avoid surgery within one year. However, 58 patients experienced only slight pain relief and were recommended surgical treatment. In our study, patients with negative results were offered surgery after 1.98 ESI procedures with an interval of 3.7 months. In the group of operated patients, the preliminary use of steroid injections did not bring relief by the results of Oswestry Disability Index (ODI) and Visual Analog scale (VAS), however, the condition of these patients improved significantly after surgery (p<0.05). The use of epidural injections is possible as a first-line therapy in patients with moderate functional limitations, which can subsequently be directed to surgery in the absence of a positive result.
Key words: seropositive myasthenia gravis, decrement test, electromyography of a single muscle fiber
Abstract. The prognostic value of diagnostic tools in patients with seropositive myasthenia gravis: a retrospective study of 31 cases. Gryb V.A., Tretyakova A.I., Titov I.I., Chudovska L.D., Nasonova T.I., Slobodin T.M., Doroshenko O.O. To confirm the diagnosis of myasthenia gravis (MG), in addition to clinical observation of the muscle weakness dynamics, pharmacological and functional tests, Computed tomography/Magnetic resonance imaging (CT/MRI) of the mediastinum, detection of antibodies to acetylcholine receptors (AChR) and to muscle specific tyrosine kinase (MuSK)/; electrophysiological tests are used: rhythmic nerve stimulation (RNS)/decrement test and electromyography of a single muscle fiber (single-fiber EMG (SF-EMG)/jitter). The aim of our study is to determine the possible relationship between the level of antibodies to AChR and the decrement test value, to verify a correlation between SF-EMG and the severity of MG seropositive to AChR. To evaluate the effectiveness of pathogenetic treatment and prediction of the duration of remission according to the results of the study. A total of 31 patients with myasthenia gravis seropositive to AChR were examined, among whom there were 19 (61.3%) women aged 19 to 74 years. The pattern of muscle weakness was evaluated by a score of the International Clinical Classification of Severity of Myasthenia gravis scale (MGFA). The presence of respiratory failure and its degree was assessed by spirography. The number of antibodies to AChR was determined using enzyme-linked immunosorbent assay (ELISA). The criteria for inclusion in the study were the confirmed diagnosis of seropositive myasthenia gravis with an AChR antibody level of more than 0.5 nmol/L. All patients underwent electrophysiological studies. Patients were examined three times: 1) at the time of visiting the doctor (in the hospital or on an outpatient basis); 2) in 16 and 3) in 24 weeks from the start of the study. Depending on the clinical condition of the patients the following treatment was prescribed: pyridostigmine, methylprednisolone, azathioprine. Statistical analyses were performed using the statistical computing environment R (R Core Team). In the dynamics of observation, a decrease in the level of antibodies to AChR and an improvement in the condition of patients according to the MGFA classification were generally observed but no correlation was found between the severity of MG and the level of antibodies to AChR during the first visit (Kruskal-Wallis test: H (4, N=31)=2.23 p=0.69); during the second visit (Kruskal-Wallis test: H (5, N=31)=9.44 p=0.09), as well as during the third visit (Kruskal-Wallis test: H (2, N=30)=2.74 p=0.25). A correlation was found between the concentration of antibodies to AChR and a thymectomy in the clinical history (Kruskal-Wallistest: H (1, N=7)=3.153752 p=0.07): over time, the level of antibodies decreased. Decrement test deviations were detected in 23 (74.2%) of the 31 patients, SF-EMG – in 20 (95%) of 21 patients. Abnormal jitter was recorded in 100% of cases with a generalized form of myasthenia gravis. During all three visits, a correlation was determined between the highest decrement test of symptomatic muscle and MG severity according to MGFA (r=0.39; p=0.042), (r=0.35; p=0.048), (r=0.41; p=0.039); and also between the jitter value and MG severity (r=0.54; p=0.032) (r=0.35; p=0.048), (r=0.61; p=0.034 respectively). Analysis of the contingency tables using the exact Fisher test provided information on the best method of treating patients. We consider that the most effective prognostic test that may affect the choice of further treatment is SF-EMG. However, due to the difficulties of using this method in routine practice, rhythmic nerve stimulation (RNS) test in clinically weak muscles should be recommended. It is not recommended to monitor antibodies to AChR in order to predict the course of the disease, it is better to use this test only for the diagnosis of seropositive myasthenia gravis.
Key words: arterial hypertension, hemorrhagic stroke, 24-hours ambulatory blood pressure monitoring, ultrasonography of the large cervical arteries, hemodynamic load
Abstract. Hemodynamic load on the intracranial arterial system in subjects who have suffered a hemorrhagic stroke as a complication of essential arterial hypertension. Netiazhenko V.Z., Tkachyshyn O.V. The aim of the investigation was to compare a hemodynamic load on the intracranial arterial system, assessed by the indices of 24-hours ambulatory blood pressure monitoring and ultrasonography of the large cervical arteries between the group of patients with essential arterial hypertension in ≥6 months after a hemorrhagic stroke and a group of patients with essential arterial hypertension without complications. The first one was the main group (n=94; age – 54.4±0.9 years), М±m), the second one – the comparison group (n=104; age – 53.7±0.9 years). The indices of 24-hours ambulatory blood pressure monitoring in the main group and the comparison group were the following: the mean daytime systolic blood pressure was 109.6±1.6 and 121.1±1.1 mm Hg, the minimal one was 74.4±2.0 mm Hg and 82.3±12.5 mm Hg, and the maximal one was 168.2±1.9 and 161.9±1.7 mm Hg, p<0.05. The daytime systolic blood pressure sigma (17.9±0.6) and the average real variability of systolic blood pressure (11.31±2.52 mm Hg) were bigger in the main group (p<0.01). The daytime index of hyperbaric load of systolic blood pressure was bigger in the main group: it was 403.6±25.9 against 231.7±12.1 mm Hg × h in the comparison group (p<0.01). The maximal pulse pressure for a 24-hours interval was 74.2±2.0 and 66.4±0.9 mm Hg, respectively (p<0.01). The indices of ultrasonography of the large cervical arteries in the right and left vessels of the main group were the following: the Gosling's pulsatility index was 1.578±0.059 and 1.552±0.042 for the common carotid artery, 1.210±0.044 and 1.102±0.037 for the internal carotid artery, 1.191±0.030 and 1.150±0.023 for the vertebral artery. The above-mentioned indices were bigger in the main group than in the comparison one (p<0.01). The diameters of all the large cervical arteries were bigger in the main group (p<0.01). Therefore, according to the above-mentioned indices which are associated with a bigger risk of cerebrovascular events, a bigger hemodynamic load on the intracranial arterial system was found in the main group than in the comparison one, despite the smaller mean systolic blood pressure indices.
Key words: laparoscopic hernia repair, incisional ventral hernia, open hernia repair, body mass index
Abstract. Comparative analysis of surgical interventions for postoperative ventral hernia in obese patients. Kravchenko B.S., Klimenko A.V., Klimenko V.N., Sergееva L.N. The problem of surgical treatment of postoperative ventral hernias remains relevant today. Currently, a large number of methods of surgical interventions for hernias of the anterior abdominal wall have been proposed, the variety of which requires systematization and analysis of the immediate and distant results of treatment. Goal: to analyze the effectiveness of laparoscopic hernia repair in patients with postoperative ventral hernia with a body mass index of 30 kg/m2. The results of treatment of 29 patients with postoperative ventral hernia were analyzed. Men – 8 (27.5%), women – 21 (72.5%). In 16 (55.2%) patients (main group) laparoscopic intraperitoneal onlay mesh – IPOM was performed, in 13 (44.8%) patients (comparison group) open hernia repair with fixation of the mesh graft in the Sublay position was performed. The analysis of the duration of the surgical intervention indicates that with open hernia repair, there is a significant increase in the duration of the surgical intervention, p˂0.0001. Comparison of the duration of operations in both groups depending on the body mass index also indicates the dependence of the duration of surgery on this indicator. Thus, in the comparison group, there was a tendency to an increase in the duration of the operation with an increase in the body weight of patients with a significant difference in the duration of the operation between the main group and the comparison. The duration of a patient's stay in the hospital and the occurrence of respiratory complications correlates with the level of postoperative pain and the duration of surgery. Postoperative complications in the form of fluid accumulations in the wound area of the abdominal wall depend on the method of surgery and the location of the mesh graft. The use of laparoscopic – IPOM in obese patients significantly improves the results of surgery by reducing the level of postoperative pain, shortening the duration of surgery, and the absence of complications in the early and late postoperative period. When choosing the method of surgical intervention in patients with postoperative ventral hernia, laparoscopic IPOM should be preferred.
Key words: bronchopulmonary dysplasia, premature, neonates, newborns, respiratory support, prophylaxis
Abstract. Treatment and prophylaxis of moderate and severe bronchopulmonary dysplasia in premature neonates. Bolonska A.V., Sorokina O.Yu. Bronchopulmonary dysplasia in premature neonates leads to physical and mental developmental disorders and behavioral problems and associated with frequent rehospitalizations and long hospital stay. Study objective: to study the predictors of bronchopulmonary dysplasia development in premature neonates in structure of intensive care. Study design: A retrospective cohort analysis was performed in 127 children recruited from two NICU of Dnipro between January 2016 to March 2020. Inclusion criteria: preterm neonates 28-32 gestation weeks with respiratory distress syndrome (RDS). Results demonstrated that every day of mechanical ventilation, supplemental oxygen with FiO2 more than 30% and cardiac drugs usage increased risk of bronchopulmonary dysplasia development by 15-20%. In conclusion, finding out predictors of bronchopulmonary dysplasia helps to improve and prudently use usual treatment regimens in premature neonates and decrease the frequency of moderate and severe bronchopulmonary dysplasia.
Key words: acute abdominal pathology, dehydration, volume depletion, fluid compartments
Abstract. Formation of initial changes in hemodynamics and fluid compartments in high surgical risk patients under the influence of acute abdominal pathology. Kravets O.V., Yekhalov V.V., Krishtafor D.A., Zozulia O.O., Volkov O.O., Vlasov O.O. Among the main factors of pathological changes that accompany acute abdominal pathology are the inflammatory process of the peritoneum and fluid deficiency due to its pathological losses. The aim of our study was to analyze the initial state of fluid compartments of the body and hemodynamics in high surgical risk patients with acute surgical abdominal pathology. There were examined 157 patients with acute abdominal pathology who underwent emergency laparotomy. The presence and severity of fluid deficiency were determined clinically by tissue hydrophilicity test by P.I. Shelestiuk, biochemically – by assessing the levels of hematocrit, hemoglobin, erythrocytes, blood electrolytes, vasopressin (antidiuretic hormone (ADH)) and brain natriuretic propeptide (proBNP), as well as the mean erythrocyte volume and plasma osmolarity. Variables of fluid compartments of the body and central hemodynamics were studied using the non-invasive bioimpedancemetry. Based on the values of oxygen concentration in arterial and venous blood, total oxygen consumption (VO2) and delivery of oxygen (DO2), oxygen extraction ratio (O2ER) were calculated. The detected changes indicate intravascular fluid deficiency and concomitant hemoconcentration with normal electrolytes levels and plasma osmolarity. In patients with high surgical risk and moderate dehydration according to P.I. Shelestiuk, urgent surgical pathology of the abdominal cavity reduces extracellular fluid volume by 19.1% (p=0.019) of the reference by reducing the volume of the interstitium and intravascular fluid respectively by 20.7% (p=0.002) and 16.3% (p=0.001) of regional values, which forms in patients a state of "volume depletion" of moderate severity. This is accompanied by an increase in the ADH concentration by 16.7% (p=0.041) above reference and normal proBNP levels. Stroke volume decreases by 28.8% (p=0.021) against tachycardia (increase in heart rate by 39.7% (p=0.001) above normal) and vascular spasm (increase in systemic vascular resistance by 86.9% (p=0.001) above reference), which supports the normodynamic type of blood circulation (cardiac index – 3.2 (0.4) l/min/m2) with the decrease in stroke index and peripheral perfusion index by 41.3% (p=0.002) and 55.2% (p=0.002) from reference, respectively. DO2 decreases by 11.1% (p=0.011) from reference with VO2 increased by 16.3% (p=0.004) above reference, which leads to a decrease in oxygen utilization by 7.2% (p=0.041) from reference.
Key words: components of anxiety, neurotic disorders, sleep disorders, organic anxiety disorder
Abstract. Clinical features of sleep disturbances in anxiety disorders of neurotic and organic genesis. Liashchenko Yu.V., Yuryeva L.M. There is a bi-directional link between dyssomnia and psychological diseases. Sleep disorders can influence the severity of the underlying disease, complicate the process of treatment, and increase the risk of the recurrence further. Besides, there is an obvious dependence of the sleep disorder severity from the pathological anxiety. The aim of the research: to study clinical features and relationship of sleep disorders and anxiety in patients with anxiety disorders of neurotic and organic genesis. To achieve the objective, 120 patients with anxiety-depressive disorders, who were divided into 2 groups depending on the genesis of the disease were studied. The first group included patients with anxiety-depressive disorders, anxiety phobic disorder and generalized anxiety disorder. The second group included patients with emotionally labile and organic anxiety disorders. The research was done with the help of clinical-psychopathological method added with psychometric scales and with the method of statistical processing. According to the results, it was detected that the level of insomnia was reliably higher in patients with anxiety disorders of organic genesis than in the group of patients with neurotic disorders. After the analysis of the components of the pathological anxiety it was determined that the components of emotional discomfort and the assessment of the prospects of anxiety dominated in the group of neurotic genesis, but the asthenic and phobic components were more expressed in the group of patients with anxiety disorders of organic level. In addition, according to the results of the correlation analyses, it was determined the link of the degree of expression and the severity of insomnia. Understanding of the semantic character of the pathological anxiety and clinical display of sleeping disorders will give the possibility to create the relevant differential rehabilitation programs of the treatment of the patients with anxiety-depressive disorders with sleep disorders.
Key words: urogenital infections, clinic, diagnostics
Abstract. Nosomorphosis of diseases caused by urogenital infections. Diudiun A.D., Polion M.Y., Polion N.N. One of the real threats to the health of the next generation are diseases caused predominantly by sexually transmitted infections (STIs). The frequency of these diseases is constantly increasing among the population of Ukraine and other countries. The aim of our work was to establish the features of the clinical manifestations of diseases caused by urogenital infections (UGI) at the present stage. Under our supervision, there were 577 patients with STIs, 83% of the patients were at the age of the highest socio-economic and sexual activity. A complex of methods for examining the patients included: physical and general clinical studies, laboratory research to identify STIs and conditionally pathogenic microorganisms. A comprehensive complex examination made it possible to identify urogenital chlamydia in 395 (68.5%), trichomoniasis – in 381 (66.03%), ureaplasmosis – in 240 (41.6%), microorganisms associated with bacterial vaginosis – in 80 (13.9%) and urogenital candidiasis – in 75 (13.0%). Monoinfection was diagnosed only in 61 (10.6%) of patients. Among the patients examined, the inflammatory process of the genitourinary organs was supported by two infectious agents in 487 (84.4%), three or more – in 52 (9.0%) of the observed patients. The affection of the urogenital system with STIs occurs with several infectious agents. Clinical manifestations of diseases caused by STIs are asymptomatic or low-symptomatic. The asymptomatic course of diseases caused by STIs leads to a chronic long-term course of the pathological process with the formation of multi-focal lesions and residual phenomena, including proliferative neoplasms.
Key words: hydrocephalus, children, cerebral oximetry, hemodynamics, anesthesia
Abstract. Features of noninvasive cerebral oximetry and central hemodynamics in young children with hydrocephalus. Pavlysh O.S., Snisar V.I. Hydrocephalus is one of the most common diseases of the nervous system in young children. Features of structural and morphological changes of the brain in children with hydrocephalus are the predominance of signs of periventricular ischemia of brain tissue due to cerebral circulatory disorders. Despite the existence of a large number of methods for assessing cerebral hemodynamics, in the modern literature there is only limited information about the oxygen status of the brain when using different types of anesthesia in children. The aim of the study was to assess the dynamics of noninvasive cerebral oximetry and central hemodynamics in young children with hydrocephalus during ventriculoperitoneal shunting. The research included 59 young children with acquired hydrocephalus who underwent ventriculoperitoneal shunting. 34 children underwent total intravenous anesthesia with propofol, 25 children – total inhalation anesthesia with sevoflurane. Intraoperative control of vital functions of the patient was performed: systolic blood pressure, diastolic blood pressure, mean arterial pressure, heart rate, regional saturation, carbon dioxide level on exhalation, sevoflurane concentration on inspiration and exhalation, non-invasive cerebral indicators. Children with acquired hydrocephalus had cerebral oximetry within normal regional level. The use of sevoflurane leads to increased cerebral oxygenation by inhibiting cerebral metabolic needs for oxygen and vasodilation of blood vessels with increased cerebral blood flow. Total intravenous anesthesia does not change the rate of intraoperative cerebral oxygenation, leads to hemodynamic changes in the form of decreased stroke volume, which may indicate that propofol reduces the level of oxygen consumption by the brain with decreased cerebral blood flow against the background of hemodynamic inhibition.
Key words: growth hormone deficiency, neglect, psychological abuse, physical violence, school bullying
Abstract. Domestic and school violence in short stature children with growth hormone deficiency. Aryayev M.L., Senkivska L.I. The work is devoted to assessing the frequency of family (neglect, aggressive methods of punishment) and school (bullying) violence among short stature children with growth hormone deficiency (GHD) in comparison with children with normal growth.There were 94 children with GHD in the main group at the age of 7.2±0.4 years. The control group included 310 healthy children aged 7.1±0.3 years. Signs of domestic violence (neglect, aggressive methods of punishment) and violence at school (school bullying) were identified by questioning children and parents. School bullying was assessed using The Olweus Bully/Victim Questionnaire, adapted in Ukrainian and Russian. To check the statistical hypothesis on differences of relative frequencies, in two independent samples, the criteria of хі-square (χ2) was used. No differences were found in the frequency of neglect in the main group (8.5%) compared to the control group (9.5%), p>0.05. An equally low frequency of the use of non-aggressive methods of upbringing without physical and psychological punishment was found both in the main and in the control groups: 31.9% compared to 39.6% (p=0.25).There was no increased physical aggressiveness towards children with GHD: the frequency of physical punishment was 29.8% and did not differ from the control group by 35.3% (p=0.4). Psychological methods of punishment were used more often (38.3%) in the main group in comparison with the control group (25.1%); p=0.04.There was an increased frequency of school bullying in relation to children with GHD (27.2%: 18.1-36.3) compared with the control group (17.7%: 13.4-22.0), p=0.04. The frequency of school bullying in boys of the main group was 2 times higher than in boys of the control group. School bullying victims in the main group, compared with the control group, were more likely to feel depressed (30.8% versus 12.7%; p=0.05), less often had good friends (42.3% versus to 78.2%; p=0.001) and felt loneliness more often (26.9% in relation to 9.1%; p=0.04).
Key words: cystic fibrosis, chronic infection, Pseudomonas aeruginosa, airway microbiome, resistance
Abstract. Microbiological features of Pseudomonas aeruginosa in children with Cystic Fibrosis. Ishchenko O.V., Stepanskiy D.O. The purpose of the study was to determine the frequency rate of Pseudomonas aeruginosa infection among children with cystic fibrosis (CF) in Dnipro region and to provide microbiological characteristics of the obtained isolates. The study was conducting from January 2019 to December 2020. Children with genetically confirmed CF diagnosis were enrolled. The main research method was bacteriological with identification of microorganisms by biochemical properties; antimicrobial susceptibility was determined by disk-diffusion method. Biological material: mucus from a deep smear from the posterior pharyngeal wall, sputum and tracheobronchial lavage waters. The Leeds criteria were used to define persons with chronic infection. The study involved 21 children. We collected 183 respiratory samples with 49 isolates of P. aeruginosa. The most important co-existing pathogens were Staphylococcus aureus, Aspergillus spp. and Candida spp. In our study, P. aeruginosa was associated with Aspergillus spp. (χ2=20.952; df=1; p˂0.001). Mucoid isolates were found in 24.49% of cases. P. aeruginosa showed variable sensitivity to different groups of antimicrobial agents, but the highest resistance was to penicillins. Mucoid P. aeruginosa was more resistant to penicillins (p˂0.001) and cephalosporins (p=0.036). Infection P. aeruginosa is frequent among children with CF; there were three children with chronic bronchopulmonary infection P. aeruginosa in Dnipro region in the end of 2020. The likelihood of Aspergillus spp. infection was higher in the case of current P. aeruginosa infection. P. aeruginosa showed variable susceptibility to different groups of antimicrobial agents, but mucoid isolates were more resistant.
Key words: fracture of the mandible, delayed consolidation, osteogenesis, fibrin-enriched platelets, bone morphogenetic protein
Abstract. Therapy of delayed consolidation of mandible fractures. Idashkina N.H., Gudarian O.O., Samoilenko I.A., Shandyba S.I. The aim of the study is to improve the effectiveness of treatment for delayed consolidation of mandibular fractures by developing and clinical testing of integrated therapy using growth factors and bone morphogenetic protein-2. Clinical material is based on the study of 62 patients with the problem of mandibular fractures consolidation. The examinations of bone density were carried out on a cone-beamed computerized tomograph Planmeca Promax 3D (Finland) with an X-ray emitter of 85 kV 5-7 mA on the day 7 and 21 of the study. The evaluation of the density of jaw bone tissue was conducted by U. Lekholm and G. Zarb (1985) classifications, and by S. Mish (1990). Bone density of more than 850 units was considered as an intact dense bone, the values from 350 to 850 units - as a relatively intact, loose bone, and less than 350 Hounsfield units - local osteoporosis. Statistical processing of materials was carried out using standard methods of variation statistics using the STATISTIKA (ver. 6.1; Statsoft Inc., USA, No. AGAR 909E415822FA). Use in the therapy of delayed consolidation of mandible fractures of the developed complex, which includes strontium ranelate – a drug with osteotropic action, antioxidant Mexidol, immunomodulator of recombinant human interleukin-2, and a local introduction of fibrin-enriched platelets containing numerous growth factors along the fissure of mandible fracture, or recombinant bone morphogenetic protein-2 in more complicated cases. The improvement of reparative processes was in 58 patients. In any case there was no need to carry out additional surgical interventions for osteosynthesis or bone «grafting». The basic program on care of patients with a delayed consolidation of mandible fractures should affect all pathogenesis components of this complication: it should include osteotropic drugs, antioxidants and immunomodulators. Involvement of platelet-rich fibrin in the integrated therapy positively affects the quality of bone regenerate and treatment terms, ensuring the normalization of clinical and laboratory parameters up to the day 21. The best optimization of reparative osteogenesis in the complex therapy of delayed consolidation is facilitated by the additional injection of recombinant human bone morphogenetic protein-2. This tactic allowed to ensure its favorable course by the type of initially delayed adhesion in all patients (8 out of 8).
Key words: stomatogenic maxillary sinusitis, microbiota, iatrogenic sinusitis, the etiopathogenesis of sinusitis, differential diagnostics
Abstract. Microbial landscape and immune status in maxillary sinusitis of stomatogenic origin. Kovach I.V. , Varzhapetian S.D., Bunyatyan Kh.A., Reyzvikh O.E., Babenya A.A., Strogonova T.V. Oroanthral fistula (anastomosis) is an element preventing the restoration of homeostasis in the maxillary sinus due to the constant flow of microbes from the oral cavity. It is also contributes to frequent exacerbations of maxillary sinusitis. Saprophytic gram-positive cocci and fungi of the oral cavity are dominating representatives of the microbial flora in the maxillary sinus. As the result of research, we found that in the case of maxillary sinusitis with oroantral fistula fungi made up 25.0% of microbiota, gram-positive bacteria – 41.7%, gram-negative bacteria – 33.3%. Gram-positive cocci from the Staphylococcus genus (Staphylococcus aureus and Staphylococcus epidermidis) and fungi (Candida albicans) comprised the biggest proportion of microbial flora that 33.3% and 16.7%, respectively. Slightly decreased levels of monocytes in venous blood was noted in 69.2% of patients. The average value of total serum IgE in group with oroantal fistula was 226.2 (70.4) IU/ml, the result exceeded normal limits almost in 2.26 times. Large circulating immune complexes (CICs) were normal in all patients in the group with iatrogenic maxillary sinusitis. The average lavels of small size CICs was 170.2 (4.23) ОU, which is in 1.06 times higher the upper limit of the norm (160 ОU). Elevated levels of total Ig E in serum of patients with oroanthral fistula indicates allergic sensitization. The detection of increased levels of CICs with small and medium sizes in serum may indicate a susceptibility of this category of patients to the development of immunopathological reactions.
Key words: global warming, premature birth, infant mortality, adverse effects of pregnancy
Abstract. Prevalence of premature birth in conditions of global warming. Tsymbaliuk V.I., Vadziuk S.N., Tolokova T.I., Tabas P.S. The dynamics and likely associative link between global warming and the prevalence of preterm births in Ukraine over the years 2009-2018 was studied. to form modern ideas about the prognosis and prevention of this pathology. Data on medical care for pregnant women, mothers and parturients and adverse effects of pregnancy on preterm birth (form 21) for the period 2009-2018 were obtained from the municipal non-profit enterprise "Ternopil Regional Center of Public Health of Ternopil Regional Council". The correlation between the number of premature births per 100 births according to the average annual air temperature according to the Global Historical Climatology Network from the US Department of Ocean and Atmospheric Research in the climatically homogeneous regions of Ukraine was estimated. Predictive analysis of time series was performed by the method of integrated auto-regression of the moving average (ARIMA). The model error was estimated by calculating the absolute percentage error of the mean (MAPE). Statistical processing of materials was performed using programs Statistica 6.0 (StatSoft, USA) and open statistical package "R". The study meets modern requirements of moral and ethical standards regarding the provisions of legislative acts of Ukraine. Analysis of these reports of women's counseling shows an increase in the average number of preterm births from 2.88 per 100 births in the total number of births in Ukraine – 491445 in 2009 to 3.33, per 100 births in the total number of 309191 in 2018, which testifies to a significant increase in premature births in our country. Since 2009, there has been an annual, varying degree of increase in average annual air temperature in Ukraine. As a result of the correlation analysis, a significant strong direct correlation was established between the average level of premature birth and the average annual air temperature in Ukraine (r=0.84, p<0.05). Regression analysis revealed a significant increase in the number of premature births (per 100 births) in 1, 2, 5, 6, 7, 8 and 10 climatically homogeneous regions and a tendency to increase in, respectively, 3, 4, 9, 11 and 12 climatically homogeneous regions of Ukraine. Based on the analysis of data on the average annual air temperature in Ukraine for 2009-2018, the average annual air temperature in Ukraine is projected to increase by 0.3 ° С in 3 years (MAPE <10%, p<0.05). The annual number of premature births is expected to increase (cases per 100 births) in 3 years by 0.4 cases per 100 births (MAPE <10%, p <0.05). In the context of global warming, the number of negative consequences of pregnancy is increasing, namely idiopathic premature termination of pregnancy with the birth of premature infants. Strong correlations have been established between the annual number of premature births and the average annual air temperature in Ukraine. Regression models of preterm birth showed a significant increase in 1, 2, 5, 6, 7, 8, and 10 climatically homogeneous regions and a tendency to increase in 3, 4, 9, 11 and 12 climatically homogeneous regions. The annual number of premature births in Ukraine is projected to increase by 2023 by 20 cases per 100 births compared to 2018. The strategy for preventing premature births and related adverse effects of pregnancy should include the identification of global warming as a risk factor for increasing level of this pathology.
Key words: servicemen, informational model, daily nutrition ration, energy losses, nutrients
Abstract. Applying of informational model of nutrition ration calculation for compensation of energy losses of the Ukrainian Armed Forces servicemen. Deputat Yu.M., Gulich M.P., Savytskyi V.L., Ivanko O.M., Levit Yo.R., Bogomolets O.V. The article is devoted to the research of the problem of compensation of energy losses of servicemen doing qualification course of the UAF Special Operations Forces as well as development of the method of promp response of the officials of food service on changing of energy losses of servicemen on different phases of qualification course by corresponding correction of their daily nutrition ration. The aim of work was directed for development and substantiation of informational model of nutrition ration calculation for compensation of energy losses of the Ukrainian Armed Forces servicemen. Time and tabular method of energy losses calculations, calculations of ration composition, instrumental estimations, laboratory and statistics assessments were used as methods of research. Calculations of informational model were done in Microsoft Excel 2007 functional environment. The description of informational polyparametrical model of the computational system of nutrients composition and energy value of servicemen daily nutrition ration depending on revealed energy losses doing qualification course of UAF Special Operations Forces has been presented. The model is meant for operative calculations of nutrients composition and energy value for compensation of energy losses of corresponding servicemen doing different phases of qualification course. Informational model got approval and was implemented in the UAF Special Operations Forces activities.
Key words: thyroid gland, ecologically conditioned pathology, hypothyroidism, heavy metals, thyroid disruptors, radiаtion induced pathology, electromagnetic induced pathology
Abstract. Environmental determinants of thyroid pathology. Ryabukha O.I., Fedorenko V.I. Ecological situation of many countries, including Ukraine, is characterized by progressive anthropogenic and technogenic pollution, which causes growth in thyroid pathology, the share of which is significant in the structure of endocrine diseases. The main causes of thyroid disorders include iodine deficiency in the environment, exposure to a number of widely used chemicals (thyrodisruptors), heavy metal ions. A variety of physical environmental factors are important. A significant increase in thyroid cancer is frequently associated with local or general exposure to ionizing radiation. Prolonged exposure to electromagnetic fields can lead to disorders in the gland's homeostasis. The functional capacity of the gland is also impaired by unsanitary living conditions, some bacteria and viruses, and improper nutrition. High sensitivity of the gland to external impacts and high social significance of thyroid pathology give grounds to consider the morphofunctional condition of the thyroid gland as a marker of ecological well-being of the environment.
Key words: health care, patient safety culture, health care facilities, Cronbach's alpha factor
Abstract. Patient safety culture in health care facilities in Ukraine (message 1). Yavorovsky A.P., Rygan M.M., Naumenko A.N., Skaletsky Yu.N., Gichka S.G., Ivanko A.V., Brukhno R.P., Gorval A.K. Using a questionnaire from the United States Agency for Research and Quality in Health Care (AHRQ), the characteristics of patient safety (PS) culture in the staff of various health care facilities (HCF) in Ukraine were analyzed. In addition, the characteristics of PS culture were analyzed depending on the length of service and affiliation of the respondents to the medical or nursing staff, as well as the profile of therapeutic or surgical activities. It is established that the weakness of the PS culture of the staff of domestic HCF is "Reaction to mistakes" (less than 30% of positive responses), which indicates the predominance of culture of accusation (unfair culture) in Ukrainian HCF and as a consequence fears of the staff to disclose mistakes and accordingly, the lack of opportunity to learn from these mistakes.“Staffing” is identified as a weakness of the PS culture (less than 50% of positive responses) in most comparison groups. It is worth noting such a characteristic of the culture of BP, as the "Frequency of error messages" (less than 70% of positive responses). The Cronbach's alpha coefficient in all groups of respondents ranged from 0.62 to 0.78, which indicates the truth of the results of the study.
Key words: children, allergic and non-allergic skin diseases, atopic dermatitis, incidence, prevalence, environment, pollutants
Abstract. Allergic and non-allergic skin diseases in children of Ukraine: a retrospective study of the prevalence and incidence over the past 24 years. Volosovets O.P., Bolbot Yu.K., Beketova G.V., Berezenko V.S., Umanets T.R., Rechkina O.O., Міtiuriaeva-Korniyko I.O., Volosovets T.М., Pochinok T.V. Bacterial, allergic, parasitic and fungal skin lesions are much more common among children than among adult patients. The aim of this study was to analyze data on the prevalence and incidence of diseases of skin and subcutaneous tissue with allergic and non-allergic origin among children of Ukraine in period from 1994 to 2017. Our main purpose was to determine the possible impact of different adverse environmental factors on children`s growth, including radiation, connected with the Chernobyl disaster in 1986. Over the past 24 years Ukraine has witnessed a 10.3% increase in prevalence of diseases of skin and subcutaneous tissue among children and 8.6% raise of incidence rate with a predominance of detection of this pathology in children affected by the Chernobyl accident and children from areas of radiological control. We have found a direct influence of moderate strength on the connection between population-weighted effective dose of total human exposure (in millizieverts) in different regions of the country in the period of 1997-2011 and the incidence and prevalence of skin diseases among children aged 0-14 years, including 2011 (p<0.05).This fact may indicate the impact of this disaster on the occurrence and pathogenesis of skin diseases and its consequences among children. During the same period, due to the influence of a number of environmental factors, there was a significant transformation in the structure of skin diseases in children. We have witnessed double increasing of the proportion of allergic lesions of the skin and its elements. The prevalence of diseases of the skin and its elements in general has reached 28.7% against the corresponding reduction of skin infections.
A CASE FROM PRACTICE
Key words: сovid-19, endotheliocytes, thrombotic complications, thrombovasculitis
Abstract. Postcovid syndrome: regarding the risks of developing thrombotic complications in patients with ischemic limb lesions (clinical cases). Vysochyna I.L., Nikolaenko-Kamyshova T.P., Biesiedin O.M. The problem of postcovidal syndrome is currently relevant, since in the stabilization of vital functions, the processes of immunological inflammation with significant disorders of endothelium functions continue; this causes tissue ischemization, especially in the presence of changes at the level of microcirculation in patients with angiopathy and vasculopathy. It is noted that in COVID-19 comorbidity with hypertension, coronary heart disease, diabetes mellitus, systemic connective tissue diseases the risks, course, prognosis and development of vascular complications significantly increase. The severity of the inflammatory process and predicting the likelihood of complications is accompanied by changes in laboratory indicators, namely: in the general blood test – leukocytosis, an increase in the level of neitrophilic granulocytes, a decrease in the content of hemoglobin and platelets; in biochemical indicators – a decrease in the level of the general protein, an increase in the indicators of alaninetransferase, aspartaminotransferase, C-reactive protein, lactatdehydrogenase, ferritin; coagulation changes – increase in the level of fibrinogen, D-dimer, reduction of activated partial thromboplastic time. On the basis of the considered clinical cases, taking into account modern ideas about the peculiarities of the development of thrombotic complications in COVID-19 infection in patients with ischemic lesions of the extremities, the need to prescribe low molecular weight heparin as a selection drug for the treatment of thrombophilic conditions has been proven. Detailed study of histological material is important for establishing changes at the level of microcirculation and ischemization of tissues, this will allow to develop algorithms for the treatment and prevention of complications. Solving these issues, not only in the presence of COVID-19, but also in any stressful cases, requires careful clarification of the mechanisms for the development of complications and a timely adequate integrated approach to treatment to improve and stabilize the condition of patients.
Key words: limb-girdle muscular dystrophy, CAPN3 gene, Gower’s sign, proximal muscle weakness
Abstract. Аutosomal recessive limb-girdle muscular dystrophy type 2A: two cases in Ukraine with different age of onset. Antipkin Yu.H., Kyrylova L.H., Miroshnykov O.O., Yuzva O.O., Orzheshkovskyi V.V., Nechay A.F. The paper reports on two cases of young women from Ukraine with autosomal recessive limb-girdle muscular dystrophy type 2A with different age of symptoms onset and an absence of any family history presented with gradual onset of proximal muscle weakness in four limbs and thinning of shoulders, arms and thighs. Both patients had elevated creatine phosphokinase level and c.550delA mutations in CAPN3 gene. Sequence analysis and deletion/duplication testing of the 159 genes from skeletal muscles disease testing panel of 5-year-old girl identified deletion of exon 8 (heterozygous) and c.550delA (p.Thr184Argfs*36) mutation (heterozygous), were in CAPN3 gene. Magnetic Resonance Imaging of soft tissue of the proximal lower extremities was performed which showed signs of symmetrical atrophic changes in the major adductor muscle, the long and short adductor muscles, the semitendinosus muscle of the thigh, as a manifestations of autosomal recessive limb-girdle muscular dystrophy type 2A. Homozygous, pathogenic variant of the defect in the CAPN3 gene c.550del (p.Thr184Argfs * 36) was identified in a 25-year-old woman. Type 2A is the most common form of limb-girdle muscular dystrophy, accounting for about 30% of cases. The autosomal recessive limb-girdle muscular dystrophy type 2A is on caused by mutations in the CAPN3 gene, and it is characterized by selective atrophy and weakness of proximal limb and girdle muscles. The age of onset of muscle weakness is extremely variable; the most common being between 8 and 15 years, although it can range between 2 and 50 years. The diagnosis can be suspected by findings on a muscle biopsy or when a doctor experienced in muscular dystrophy examines you. A serum creatine kinase blood test may also show raised levels which indicate a problem in the muscles. The diagnosis has to be confirmed by means of identifying a mutation in the CAPN3 gene which is done on a deoxyribonucleic acid sample from a blood test. To date there are no specific treatments for limb-girdle muscular dystrophy, however careful management of the symptoms of the condition can improve a person’s quality of life. Joint contractures (tightening) can occur in limb-girdle muscular dystrophy and therefore regular physiotherapy is recommended.
Key words: juvenile systemic scleroderma, dilated cardiomyopathy, children
Abstract. Heart injury in juvenile systemic sclerodermy (clinical case). Omelchenko L.I., Mukvich O.N., Belska O.A., Dudka I.V., Klymyshyn Y.I., Rudenko N.M., Kalashnikova R.V., Ismakaieva D.L., Vdovina N.M., Liudvik T.A. Juvenile systemic sclerosis (JSS) has many clinical manifestations that differ from adults. Early diagnosis is problematic. The course of the disease and the severity of the prognosis depend on the involvement of internal organs in the process, first of all, the heart, lungs, kidneys. Cardiac pathology is a frequent and prognostically unfavorable target of the scleroderma process in adults, but it is rarely diagnosed in children. The aim of the work was to study the features of the clinical manifestations of systemic sclerosis in a child with severe heart disease. A polymorphism of the clinical symptoms of severe heart damage with the development of dilated cardiomyopathy in a one-year-old child with systemic sclerosis is presented. The features of the case are the early debut of systemic sclerosis in a child with a burdened hereditary history of autoimmune pathology (psoriasis in the father and grandmother), rapid progression of the autoimmune process, severe heart damage by the type of non-compact (dilated) cardiomyopathy, positive clinical dynamics when using pathogenetic therapy. Early detection of cardiovascular lesions using modern diagnostic methods, timely implementation of adequate therapy in a multidisciplinary team and regular cardiovascular screening can improve the prognosis, quality of life and reduce mortality.
IN MEMORY OF THE SCIENTIST