STUDY OF CLINICAL CASES OF MYOCHE’S MYOPATHY AND DIFFERENTIAL DIAGNOSIS WITH OTHER TYPES OF ADVERSE MYOPATHIES

Markesbery-Griggs myopathy, Miyoshi type (MM) is a rare type of myopathy, a form muscular dystrophy with the main involvement of the lower girdle and distal parts of the legs. Due to complexity of genetic testing, the diagnosis is mainly made on the neurological examination of the patient, which adds value to this case report. The childhood or adolescence onset of the disease is characterized initially by the calf muscles` wasting, accompanied by the severe elevation of the serum creatine kinase, as well as a slowly progressive ascending course. The disease refers to dysferlinopathies with various mutations in the DYSF gene. The dysferlin protein is localized in the plasma membrane and in the T-tubule system of skeletal muscles. Physiologically, skeletal muscles are constantly exposed to micromembrane lesions. Depending on the severity, these damages are restored using various complexes. One of the main reparative complexes is the dysferlin-dependent mechanism. Mutations can lead to a defect in the membrane repair, causing the influx of Ca 2+ into the cell, which leads to a cell`s destruction. There are three genetically identifiable types of Miyoshi myopathy: MMD1, MMD2, MMD3. The main clinical signs of the disease are the muscle weakness and atrophy, with predominant involvement of the distal parts of the lower limbs, especially in the gastrocnemius and plantar muscles. The MM causes tip toe walking disturbances and difficulties in climbing the stairs. Progression of the disease and further atrophy leads to the wasting of the lower girdle muscles, mainly gluteal ones. Peculiarity of these myopathies is the absence of cardiomyopathy, due to the immunity of cardiomyocytes to a deficiency of the protein dysferelin. Diagnosis is made on the basis of muscle biopsy and molecular genetic testing. The gold standard is immunoblotting or immunohistochemistry. One of treatment methods is the use of improperly folded dysferlin (treatment with a proteasome inhibitor MG-132) in fibroblasts with restoration of membrane sealing. The aim of this case report is to present an example of a possible clinical diagnosis of MM in a young man, in the absence of opportunities for molecular genetic testing.

CASE STUDY: MANAGEMENT OF PATIENT WITH SHOULDER PAIN BY GENERAL PRACTITIONER

Shoulder pain is one of the most common presentations of musculoskeletal pain with population prevalence of between 7 and 26 %. The most common pathology that affects the shoulder joint is rotator cuff syndrome. The most important aspects in the management of patient with shoulder pain by general practitioner are to ensure an accurate diagnosis and to start the appropriate treatment on time. The main direction of rotator cuff syndrome treatment includes a period of rest from the exacerbating activity and taking nonsteroidal anti-inflammatory drugs. In some cases the use of the NSAIDs is not possible, so we decided to suggest an alternative non-pharmacological method of treatment. We treated a patient with rotator cuff syndrome by injections of autologous plasma into the affected muscles and projections of entheses that were involved in the pathological process. We obtained positive results. The shoulder pain had been reduced, mobility and movement had been improved after a course of autologous plasma injections. In our opinion, it is expedient to consider such treatment of the rotator cuff syndrome both as an additional method and separately in case of impossibility of prescribing the main therapy.

THE EFFECT OF CYTOREDUCTIVE SURGERY AND INTRAPERITONEAL HYPERTHERMIC CHEMOPERFUSION (HIPEC) ON THE CLINICAL OUTCOME OF TREATMENT OF PATIENTS WITH ADVANCED GASTRIC CANCER

Introduction. For gastric cancer (GC), metastatic peritoneal carcinomatosis (PC) is a typical process. PC and its consequences usually lead to the death of 20-40 % of patients with GC. For carefully selected patients, intraperitoneal hyperthermic chemoperfusion may be considered. The aim of the study was to investigate the effect of cytoreductive surgery in combination with HIPEC on the clinical course of the postoperative period, the frequency of postoperative complications and side effects of chemotherapeutic drugs in the treatment of advanced GC. Materials and methods. The results of treatment of 34 patients with advanced GC were considered. The patients were divided into two groups: IA group — 24 patients with advanced GC, who first underwent complete primary cytoreductive surgery (CRS) with adjuvant chemotherapy (ACT) according to the FLOT scheme and IB group — 10 patients with advanced GC treated by CRS + ACT + HIPEC. Results. Comparing the number of patients with postoperative complications in groups IA (CRS + ACT) and IB (CRS + HIPEC + ACT) using the exact bilateral Fisher’s test p> 0. 05. Comparing the frequency of side effects of chemotherapeutic treatment in the FLOT mode in groups IA (CRS + ACT) and IB (CRS + HIPEC + ACT), the values ??of χ2 = 20. 750, χ2Cr = 28. 568 (p = 0. 0622) were obtained. Conclusions. The usage of HIPEC in combination with complete CRS and ACT is a promising method of treatment of this group of patients. As a result of this study, it was proved that there is no statistically significant effect on the clinical course of the postoperative period due to the increase in the frequency of postoperative complications or side effects of chemotherapeutic drugs.

BIOCHEMISTRY OF SHORT-TERM AND LONG-TERM MEMORY

Memory is provided by changes in synapses in neural circuits: short-term memory - by functional changes in a separate sensory neuron and a separate motor neuron, long-term memory - by structural changes (regrowth of new synapses).During the formation of short-term memory in synapses, cAMP, protein kinase A, are used, which act inside the cell and transmit a signal that cause the release of large amounts of the neurotransmitter glutamate. Two independent mechanisms are involved in the formation of long-term memory: - one triggers a long-term strengthening of synaptic connections, directing protein kinase A to the nucleus, which activates the CREB protein, thereby turning on the structural genes encoding proteins necessary for the growth of new synaptic connections; - the other reinforces the already formed memory, supporting the newly formed synaptic endings, which requires local synthesis of proteins.

BEZOAR IN THE PRACTICE OF GENERAL PRACTITIONER AND PEDIATRICIAN: CLINICAL CASE – RAPUNZEL SYNDROME

Introduction. Bezoar is a cluster of foreign, inedible or difficult to digest objects in the digestive tract (especially in the stomach). Depending on the composition, there are: phytobezoars, trichobezoars, pharmacobezoars, lactobezoars. Patients may not have any symptoms for years, and their appearance is associated with an bezoar size enlargment, then there is abdominal pain, bloating, nausea and vomiting, a feeling of rapid satiety, anorexia and weight loss. The small bowel obstruction is the often cause of acute surgical pathology in the case of bezoar. It is important to pay attention to the peculiarities of the patient’s behavior, halitosis, spotted alopecia. The purpose of this publication was to raise the awareness of pediatricians and general practitioners in the diagnosis and management of patients with bezoars. The article presents a review of the literature and the clinical case of trichobezoar (Rapunzel syndrome) in a 14-year-old girl. Conclusions. Bezoar, regardless of its nature, disrupts the function of the gastrointestinal tract and can cause acute surgical complications patients of all ages. General practitionersn and pediatricians, taking into account the risk factors and clinical symptoms, may suspect bezoar formation in the early stages. In pediatric practice at the stage of primary diagnostic search, ultrasound can be recommended as a less invasive, but quite informative method. The “golden standard” for verification and treatment of uncomplicated forms of bezoar is the endoscopic method, regardless of age. Phytobezoars are less common in our region, but the processes of globalization, migration and increase in the diet of exotic fruits and vegetables may actualize this pathology. Adolescents, mostly girls who have long-term dyspeptic symptoms, alopecia, weight loss, especially if it occurs against the background of difficult social circumstances or pre-existing psychiatric disorders, are at risk of developing trichobezoar, so they need careful examination and consultation with a psychiatrist.

FEATURES OF THE COURSE OF COMMUNITY-ACQUIRED PNEUMONIA IN CHILDREN WITH CEREBRAL PALSY

Сerebral palsy (CP) occupies a significant place in the structure of neurological pathology of childhood, which is connected with comorbid pathology, such as pneumonia, which is characterized by a long and atypical course, and associates with frequent development of complications. Aims. The aim was to determinate the main features of the course and identify microbiological agents of community-acquired pneumonia(CAP) in children with cerebral palsy. Methods. The cases of CAP in children with of cerebral palsy was retrospectively studied. Children with CAP have been treated in the Children's City Clinical Hospital No. 3 of the Odessa between 2018 to 2020. The main typical clinical signs were analyzed. The microbiological agents were investigated by the microbiological examination of sputum. Results. CAP in children with cerebral palsy has its own specific features. Onset of the disease is characterized by a significant severity of the general condition, rapid progression of intoxication in children with cerebral palsy. Resistant agents and Candida albicans were identified in children with cerebral palsy. Conclusions. The studied features will help pediatricians in the assessment of CAP in patients with cerebral palsy, and it will be useful in the management of antibiotic therapy in this patients.

PROSPECTS FOR INTRODUCTION OF THE THREE-COMPONENT COORDINATION COMPOUND OF HERMANIUM OXYETHYLIDENED DIPHOSPHONATE WITH NICOTIC ACID IN THE BOUNDARIES WITH IN STUDYING OF THEIR PHARMACOKINETICS

The purpose of this work is to experimentally study the pharmacokinetics of a three-component coordination compound of germanium oxyethylidene diphosphonate with nicotinic acid (MIGU-4), created on the basis of natural metabolites. The study of the pharmacokinetics of MIGU-4 was carried out for germanium and was based on the extraction-photometric method. The experiment was carried out on male Wistar rats weighing 130-170 g. MIGU-4 was injected intraperitoneally at the rate of 37.5 mg of germanium per 1 kg of body weight. Its amount in tissues was determined photometrically by the light absorption of germanium phenifluoronate. The results of the experimental study were processed statistically. Pharmacokinetic parameters calculated using partial models analyzing the pharmacokinetic parameters, it can be seen that MIGU-4 was rapidly distributed over the organs and tissues of experimental animals. The time of admission to the examined organs was less than 0,25 hours. The studied biologically active substance quickly enters the well-vascularized organs and is evenly distributed in them. The elimination half-life (T1|2) was 5 - 8 h, the largest in the lungs, the smallest in the liver. The average residence time of MIGU-4 in experimental animals ranged from 7 hours to 27 hours. Therefore, with intraperitoneal administration of MIGU-4 is rapidly excreted from the body. The results of pharmacokinetic studies have shown that MIGU-4 is promising for the creation of a safe and effective drug. The dynamics of the therapeutic effect is comparable to the maximum concentration of the substance in the organs and tissues of experimental animals.

COMPARATIVE CHARACTERISTICS OF THE TERMINAL LINK OF GLYCOLYSIS IN THE MUSCLES OF ADULT ANIMALS AND THEIR OFFSPRING

The organism of sexually mature individuals differs significantly from the organism of immature individuals in a number of anatomical, physiological and biochemical parameters. The aim of the work was to investigate the state of the terminal link of glycolysis in the muscles of sexually mature animals and their offspring. The isoenzyme spectrum of LDH in the myocardium of sexually mature animals is characterized by a high content of LDH1 and LDH2 isozymes rapidly migrating to the anode. The isoenzyme spectrum of LDH in skeletal muscles of mature animals is represented mainly by the LDH 5.A feature of the isozyme spectrum of LDH in the tissues of rat pups is that the content of LDH1 and LDH2 is significantly reduced in the myocardium. Their number is 1.2 and 1.13 times, respectively, less than in sexually mature animals. Against this background, the content of LDH3 increases slightly, the content of LDH4 exceeds twice, and that of LDH5 is more than 6 times the indicators of sexually mature animals. In skeletal muscles of rat pups, the dominant content of LDH5 and LDH4 increases and this occurs due to a decrease in the activity of LDH3 (more than 1.5 times), LDH2 (more than 2.3 times) and LDH1 (2.2 times) compared with sexually mature animals. The data obtained indicate that in the myocardium and skeletal muscles of rat pups there is a greater percentage of isoenzymes formed from M-subunits functioning under anaerobic conditions, and with age, as a result of epigenetic transformations, the content of H-subunits increases.

MODERN CONCEPT OF RENDERING AID TO WOMEN WITH OVARIAN APOPLEXY

Purpose. Analysis of rendering the medical aid to women with apoplexy of the ovary, determination of the basic clinical, laboratory and ultrasonic criteria, which influence the choice of therapeutic tactics, the evaluation of the immediate and long-term results of treatment of ovarian hemorrhages. Materials and methods. There was studied medical aid to 888 women with apoplexy of the ovary from 2012 to 2020. Depending on the volume of hemoperitoneum patients were divided into three groups: І group — 480 (54.0 %) patients whose volume of hemoperitoneum did not exceed 200 ml; ІІ group — 283 (31.8 %) women with intraperitoneal hemorrhage with volume from 200 to 500 ml; ІІІ group — 125 (14.1 %) patients with a volume of hemoperitoneum of more than 500 ml. The group І consisted of ІC group — 270 (30.4 %) women who were given conservative treatment of apoplexy of the ovary (AO), and ІL group — 210 (23.6 %) patients whose main diagnostic and treatment measure was laparoscopic intervention. Results of the study. The average age of the patients was (28.3±5.2). The pelvic pain was the leading clinical symptom in all patients. According to the data of the transvaginal US made in the saggital plane, a linear dependence was observed between the level of free liquid and the volume of hemoperitoneum (r=0.63, p<0.05). In 792 (89.2 %) patients the diagnosis of apoplexy of the ovary and intraperitoneal hemorrhage was made to surgical intervention — on the basis of clinical picture and US data. In the remaining cases — 92 (10.4 %) diagnostic laparoscopy was required for confirming the diagnosis. The average volume of hemoperitoneum discovered in patients with AO, in absence of the adhesive process was (273.5±21.3) ml, whereas in patients with the expressed adhesive process of the small pelvis organs it was (141.4±35.5) ml. The cause of AO was the corpus luteum or cyst of the corpus luteum in more than the half of the cases — 348 (56.3 %). Conclusions. The manifestation of the clinical symptoms in apoplexy of the ovaries depends on the volume of intraperitoneal hemorrhage. The data analysis of transvaginal echography allows to determine quantitatively, with a high degree of accuracy the volume of intraabdominal hemorrhage and the morphological state of the affected ovary. Laparoscopy is not only a “gold standard” in diagnosis and treatment of AO, but also contributes to the prevention of ovarian hemorrhages in future.