Positive Clinical Dynamics Research Articles

Коррекция метаболических нарушений при диабетической нефропатии у детей и подростков

The article presents data on the use of the drug ursodeoxycholic acid (Ursosan) for hepatomegaly and fatty hepatosis in children with diabetic nephropathy. 40 children were examined. Material treated by variational statistics Statistica for Windows v. 6.0. Use of preparations of ursodeoxycholic acid in addition to the basic treatment results in a significant improvement of clinical symptoms and lipid metabolism in children, as well as a favorable course of the main disease. It was noted positive clinical dynamics when using this drug.

Efficiency of a low-intensity laser in the treatment of genital herpes infections

90 women at the age of 30,9±0,4 years with clinical laboratory signs of herpetic defeat of urogenital system took part in research lasting 12 months. Therapy included a preparation Valaciclovir on 500 mg, complex therapy was carried out with use of intra vascular laser radiation of blood (wavelength of 365 nm, power of the radiation of 2 mW, an influence exposition the laser of 8 min, the number of sessions 15) along with application of a preparation Valaciclovir. Results of the study indicate a positive clinical dynamics and immunomodulatory action of intravascular laser irradiation of blood that can be recommended as a method of complex treatment of herpetic lesions of the reproductive system.

ИММУНОЛОГИЧЕСКИЕ МЕХАНИЗМЫ –КАК ДИАГНОСТИЧЕСКИЕ КРИТЕРИИ ЛЕЧЕНИЯ ПОЛИНЕВРОПАТИИ У ДЕТЕЙ

The study examined 35 children with acute polyradiculoneuritis; In the course of molecular genetic research, antiganglioside antibodies were detected in the blood serum, which is a criterion for the differential diagnosis of acute polyradiculoneuritis. Against the background of traditional treatment, taking into account the data of immunological studies, the cyclophosphamide preparation was included in the treatment complex. Positive clinical and neurological dynamics confirmed the correctness of this treatment method

Endovascular Mechanical Thromboembolus Fragmentation in the Treatment of Critical Pulmonary Thromboembolism in Patients with Acute Hemorrhagic Stroke.

The aim of the study was to evaluate the effectiveness of endovascular mechanical thromboembolus fragmentation in the treatment of critical pulmonary thromboembolism in patients with acute hemorrhagic stroke.Materials and MethodsThe study included 47 patients with acute hemorrhagic stroke complicated by massive high-risk pulmonary thromboembolism with critical manifestations of the right ventricular failure. All patients were divided into two groups depending on the treatment method: the examined group (n=17) undergone endovascular mechanical thromboembolus fragmentation and the control group (n=30) received only basic intensive therapy.ResultsThromboembolus fragmentation was performed on patients of the examined group in order to transfer embolism of the trunk and main branches to the smaller branches of the pulmonary artery. The technical success of the procedure (destruction of the central thromboembolus) was achieved in 100% of cases. 14 patients (82.4%) showed positive clinical dynamics: improvement of general condition, reduction of pulmonary artery pressure, decreased volume of pulmonary bed damage according to CT angiography. Three patients (17.6%) died in the early postoperative period. Twenty five patients from the control group died, hospital mortality rate was 83.3%. There were no deaths in the examined group after 6–9 months of follow-up, signs of pulmonary hypertension persisted in 11 patients (64.7%).ConclusionEndovascular mechanical thromboembolus fragmentation in the treatment of critical pulmonary thromboembolism in patients with acute hemorrhagic stroke results in fast and safe decrease in pulmonary artery pressure. Fragmentation of central thromboembolus and its displacement into the peripheral vascular bed with a modified pigtail catheter is a minimally invasive surgical procedure which may be used as an alternative to surgical embolectomy in cases of an extremely high risk of surgery and absolute contraindications to thrombolytic therapy.

Mycobacteriosis of the lungs: difficulties of diagnosis and treatment (literature review)

This review presents the literature data on classification of non-tuberculosis mycobacteria, prevalence, epidemio­logy, clinical picture. The analysis of the research results reveals the prevalence of mycobacteriosis in the world, the main ways of transmission. Some data of clinical recommendations of the British thoracic society (BTS) 2017 on the algorithm of examination and diagnosis of mycobacteriosis are presented. Treatment of mycobacteriosis is a difficult task, the effectiveness of therapy remains extremely low. According to the British researchers, the proportion of patients with mycobacteriosis caused by MAC, M. malmoense, M. xenopi, who received various regimens of therapy and completely completed the course of treatment with positive clinical and laboratory dynamics, as well as did not have relapses within a year after treatment, is only 28%. According to other studies involving patients with specific types of NTMB, the effectiveness of therapy ranges from 30–50% for diseases caused by M. abscessus, 50–70% for MAS and about 80–90% for M. malmoense and M. Kansasii.

Assessment of the efficiency of sialosis treatment in patients with type 2 diabetes mellitus

Was to determine changes in the levels of cytokines in the oral fluid in patients with type 2 diabetes and sialosis, depending on the level of glycemia and the duration of type 2 diabetes, as well as in the dynamics of basic and complex treatment. Forty patients with type 2 diabetes were enrolled in the study and divided into two groups according to disease duration: group 1 comprised 15 patients having diabetes for ≤5 years, group 2 - 25 suffering for more than 5 years. The study revealed changes in pancreatic cancer immunological parameters in patients with sialosis and type 2 diabetes. Long course of diabetes led to an increase in the level of IL-2 and a decrease in IFN-γ in oral fluid. The level of IL-1β was the highest in the group of patients with a duration of diabetes ≤5 years. According to the data presented, the content of IL-1β, IL-2 in patients with diabetes is higher, and INF-γ is lower than in healthy individuals. In patients with sialosis on the background of type 2 diabetes before treatment there is a decrease in the level of IFN-γ in the oral fluid and its increase to 7.3±0.2 pg/ml after treatment. At the same time, when enzyme therapy was included in the treatment regimen, the increase in the level of IFN-γ in the main group was more pronounced and averaged 8.3±0.1 pg/ml, which was 1.2 times higher than the initial level (p<0,05). The inclusion of enzyme therapy in the complex therapy of patients with sialosis against the background of diabets, unlike traditional methods of therapy, made it possible to provide faster positive clinical dynamics, namely, under the influence of this drug, the pain stopped in a short time, salivation increased and the feeling of dryness disappeared. Along with a faster relief of clinical symptoms, normalization of the levels of IL-1β, IL-2 and an increase in IFN-γ by the end of treatment were noted.

Clinical-hemodynamic efficiency of fixed combined hypotensin therapy for patients with heavy arterial hypertension

The efficacy of treatment was evaluated by the fixed combination drug «Exforge-H» in 40 patients suffering from grade 3 arterial hypertension. All patients underwent general clinical and laboratory and instrumental examination (electrocardiography, echocardiography, 24-hour blood pressure monitoring) before and after 6 months of treatment. It was established that treatment with «Exforge-H» statistically reliably reduces the level of blood pressure, normalizes the variability and daily blood pressure profile. So, two weeks after the start of treatment, the systolic and diastolic pressure levels during clinical measurement decreased by 32,1±2,7 and 30,4±1,9 mm Hg. Art. respectively. After 3 months of treatment, 60% of patients showed achievement of the target level of blood pressure, and after 6 months - 96%. High hypotensive efficacy of treatment was also demonstrated during the daily monitoring of blood pressure in all its indicators. A significant decrease in systolic and diastolic pressure was observed during the day and night hours. The amplitudes of systolic and diastolic pressure variability have also been improved. At the same time, the variability of systolic pressure during the day was reduced by 46%, diastolic - by 49%, and at night by 59,3 and 52,8%, respectively, indicating a normalization of blood pressure during the day. All this was also accompanied by a decrease in the clinical manifestations of the disease. So, after 6 months of treatment, 84% of patients had positive clinical dynamics: a decrease in cardiac and cerebral symptoms, as well as emotional lability, which positively correlated with a decrease in the level of clinical blood pressure. The cardioprotective effect of the drug is expressed in the form of regression of left ventricular hypertrophy. In general, the use of the three-component fixed drug «Exforge-H» (valsartan - 80 mg, amlodipine - 5 mg and hydrochlorothiazide - 12,5 mg) is manifested by an increased antihypertensive effect due to the influence of its constituents side effects and the development of metabolic changes.

Congenital disorder of glycosilation PMM2-CDG

Congenital glycosylation disorders represent a group of genetically determined diseases which violate the synthesis and addition of glycans to glycoproteins and glycolipids, and also the synthesis of glycosylphosphatidyl inositol. The most common defects are the defects of protein N-glycosylation. Jaken syndrome, a congenital disorder of PMM2-CDG glycosylation, is the most commonly diagnosed type (about 800 cases worldwide). However, there are only a few descriptions of clinical cases in the Russian literature. The article presents a clinical observation of a child with this type of congenital glycosylation disorder due to a defect in phosphomannomtase 2 (PMM2 gene). The diagnose was based on the combination of clinical, laboratory and instrumental data: a characteristic phenotype, hyperinsulinism, delayed physical and psychomotor development, neurological manifestations, coagulopathy, liver damage, exudative enteropathy, abnormal forms of transferrin, PMM2 gene mutations associated with Jaken’s syndrome. For the first time the authors described positive clinical and laboratory dynamics due to the inclusion of D-mannose to the therapy for this type of congenital glycosylation disorder.

Dynamics of proinflammatory cytokine serum levels in patients with acute inflammatory diseases of pelvic organs in the early stages of conservative treatment

Dynamics of serum proinflammatory cytokines such as interleukins (IL)-1, -2 and -6, tumor necrosis factor alpha (TNFα) was examined in patients of reproductive age suffering from acute inflammatory diseases of the pelvic organs. Our study was carried out with female patients at hospital admission prior to therapy applied with conventional methods of treatment and improved treatment by using vaginal low-frequency laser in a constant continuous magnetic field. The data obtained were compared with serum cytokine level from 20 healthy female volunteers of reproductive age consulted on better contraception methods. It was found that cytokine profile of in patients with acute inflammatory processes in pelvic organs was characterized by a high level of proinflammatory cytokines. It was shown that patients receiving conventional treatment contained decreased level of serum IL-1, IL-2, IL-6 and TNFα displaying slight dynamics, which did not reach it in control group. This may contribute to ongoing inflammatory process, despite the positive clinical dynamics. In turn, imbalance of immune responses leads to a persistently impaired fertility in women and need to perform subsequent comprehensive rehabilitation measures. Moreover, patients applied with intravaginal low-frequency laser radiation in a constant magnetic field were found to contain serum TNFα 100 pg/ml observed in 59.7% of cases, IL-6 level was lower than 20 pg/ml (prevalent in control group) found in 54.2% of cases. Serum IL-2 level was decreased by 3.5-fold compared to baseline, whereas for IL-1β it was higher than 100 pg/ml in as few as 23.6% patients. Such temporal pattern of inflammatory markers with rapid significant decrease of serum proinflammatory cytokines in patients with preformed pathogenic factors can reduce probability of connective tissue formation and activate their own repair as well as regenerative events. The results obtained allow to wider use combined physical interventional factors for therapy of patients with acute inflammatory diseases of the pelvic organs.

Clinical case of specific generalized lymphadenitis complicated by miliary tuberculosis of lungs

Adolescent A., aged 15 years and 10 months, was admitted to the Department of Oncohematology of the Regional Children’s Clinical Hospital in Ivano­Frankivsk urgently on 23rd of March, 2018; he was in a difficult clinical condition with generalized and conglomerate increase of many groups of peripheral lymphatic nodes with suspicion of lymphogranulomatosis. After a comprehensive examination and an open biopsy of the anterior­cervical lymph node rightward, on March 27th, 2018, granulomatous specific lymphadenitis of tubercular aetiology was confirmed pathohistologically. On April 5th, 2018, the patient was transferred for the treatment to the Children’s Department of the RPC (Regional Phthisiopulmonology Centre) with the FDPTB (First Diagnosed Primary Tuberculosis) (April 6th, 2018) of the lymph nodes, the generalized form, Destruction — MBT (Mycobacterium tuberculosis) — M– C (Culture)– Resistance 0 Histology+ (March 27th, 2018) Category 1 Cohort 2 (2018). He received antimycobacterial therapy H0.3, R0.3, Z1.5, Lfx0.5. In connection with the maintenance of the elevated level of several OncoMarkers in peripheral blood, he was distantly consulted by leading specialists of the Kyiv State Institution «National Institute of Phthisiology and Pulmonology named after F.H. Yanovsky of the AMS of Ukraine» on May 10th, 2018. Diagnosis of specific lymph nodes’ damage is confirmed, it is recommended to strengthen the treatment scheme with kanamycin. Having received the 90 doses of medicines, were noted: positive clinical dynamics, reduction of the size and conglomerate of peripheral lymph nodes and their absence in the retroperitoneal space according to the ultrasound study. On July 13th, 2018, the patient was discharged from the hospital for the outpatient treatment. After completing of the 120 doses of antimycobacterial medicines, on August 2nd, 2018, the miliary pulmonary tuberculosis was detected on the control MSCT (MultiSpiral Computed Tomography), and therefore the patient was rehospitalized with FTPTB (Failure of Treatment of Primary Tuberculosis) (August 6th, 2018) of the lymph nodes, generalized form Destruction– MBT– M– C– Resistance 0 Histology + (March 27th, 2018), complicated by miliary dissemination Category 2 Cohort 3 (2018). Treatment was continued according to the scheme 2H0.3, R0.45, Z1.5. E0.8, S0.75 with corticosteroid therapy with prednisolone of 20 mg with gradual decrease of the dose. On the control MSCT on October 4th, 2018, it was noted — positive dynamics — partial resorption of focal dissemination in both lungs, a significant reduction of the lymph nodes’ size of the mediastinum to normal. Subsequently, according to the MSCT on January 31, 2019, in both lungs throughout the course, an increasing focal (miliar) dissemination. February 20, 2019 a review of histological preparations of the biopsy of the lymph nodes of the neck, held consultation in the Kyiv State Institution «National Institute of Phthisiology and Pulmonology named after F.H. Yanovsky of the NAMS of Ukraine». The diagnosis of the specific nature of lymph nodes and lung lesions has been re­confirmed. On the 26th of February, 2019 according to the decision of the Central Medical Advisory Commission of Drug­Resistant TB of the Ivano­Frankivsk Regional Phthisiopulmonology Centre in connection with the negative dynamics of a specific process in the lungs, it is recommended to register a clinical incidence in 4 categories with the diagnosis: RMDR­TB (26.02.2019) pulmonary (miliary) Destruction– MBT– M– MG– C– Resistance 0 Histology + (March 27th, 2018), Category 4 (FTPTB) Cohort 1 (2019). The patient is prescribed treatment according to the scheme Cm0.6, Mfx0.4, Cs0.5, Pt0.5, Z1.6. Thus, the specific lymph nodes’ damage can clinically occur as a tumorous process. An important part in differential diagnosis is pathohistological study of the biopsy material of the lymph nodes. Further clinical observations, dynamic ultrasound study and MSCT­examination are necessary for controlling the course of the disease and correcting the treatment.

Analysis of four-year management of the waiting list for liver transplantation in Rostov region: prospects for reducing mortality of candidates listed for liver transplantation

Purpose: analysis of various clinical results in patients registered in the liver transplantation waiting list (LTWL).Materials and methods: the study was carried at the Center of Surgery and Donor Coordination of the Rostov Regional Clinical Hospital using clinical, laboratory and instrumental data of 198 patients from the LTWL. 99 men and 99 women were enrolled into this study. The men age ranged from 21 to 70 years (47.8 ± 10.4 years), women age - from 18 to 66 years (49.2 ± 10.9 years). At the time of analysis of the LTWL, the average follow-up period was 14.8 ± 11.2 months. All patients were examined according to the list required for inclusion in the LTWL.Results: depending on the outcome, 198 patients from TLWL were grouped into 4 groups. The first group (delisting group) — 19 patients (9.6 %) with clinical and laboratory indicators that allowed them to be excluded from WL. The second group — 67 patients (33.8 %) who had positive clinical dynamics following therapy. The third group — 39 patients (19.7 %) who underwent liver transplantation. The fourth group — 73 patients (36.9 %) who had negative dynamics following therapy, including patients with a fatal outcome. While keeping LTWL for 4 years, 61 (30.81 %) of 198 listed patients died. The majority (40 patients) died of bleeding from varicose veins and OPPN, 17 patients died of hepatic coma and SPB. Each group represents the distribution of patients according to the MELD-Na scale, the severity of portal hypertension and hepatic encephalopathy.Conclusion: the following factors are indispensable for successful work of the transplant center: systematic work with the territories in order to expand the donor base; defining the patient priority criteria in the LTWL in order to reduce the death rate in the list; detailed examination of the patient before entering the list; forming the observation base; systematic patient observation during the pre- and postoperative period, at the rehabilitation stage, as well as at long-term periods in order to develop an effective algorithm of management of the recipient of a solid organ.

P4527Cardiac resynchronization therapy in patients with non-ischemic cardiomyopathy and severe heart failure may influence the development of unstable ventricular tachycardia

Abstract Objective The aim of the study was to determine the effect of cardiac resynchronization therapy (CRT) on the development of unstable ventricular tachycardia in patients with non-ischemic cardiomyopathy (CMP) and to analyze the potential of radionuclide methods in predicting life-threatening arrhythmias in the background of CRT. Materials and methods The study included 100 patients with non-ischemic cardiomyopathy aged from 32 to 75 years (55±12 years). Patients had III functional class of heart failure (HF); left ventricular ejection fraction (LV) (EF) 30.1±3.8%; 6 minutes walk at a distance of 290.5±64.3 m; and the final diastolic volume of the left ventricle (EDV) is 220.7±50.9 ml. Before implantation of the device with CRT and after 1 year, all patients underwent a 24-hour ECG monitoring. Patients were divided into two groups depending on the presence or absence of paroxysms of unstable ventricular tachycardia. The 1st group included 55 patients (55%) with registered paroxysms of unstable ventricular tachycardia with adequate doses of antiarrhythmic drugs. Group 2 consisted of 45 patients (45%) without reported episodes. Prior to CRT, myocardial metabolism defect (MMD) was evaluated in all patients using radionuclide methods. Results Control tests were performed 1 year after the onset of CRT and showed positive clinical dynamics: the left ventricular EF increased from 30.1±3.8% to 42.8±4.8% (p≤0.001); functional class decreased from III to II; The 6-minute walking distance increased from 290.5±64.3 m to 377.2±45.3 m (p≤0.001); and LV EDV decreased from 220.7±50.9 ml to 197.9±47.8 ml (p≤0.005). During 1 year of observation in the first group: 48 patients (80%) had no episodes of unstable ventricular tachycardia; In 7 patients (20%), episodes of unstable ventricular tachycardia were recorded. Evaluation of the effect of increasing EF and reducing EDV on the development of VT paroxysms was performed after the distribution of patients with CRT into subgroups based on the presence or absence of VT episodes. The data showed that in patients whose EF increased by 14%, and EDV decreased by 35 ml during 1 year of CRT, there were no VT episodes, even if VT paroxysms were recorded before CRT. Patients who had VT paroxysms showed an increase in EF of only 9% and a decrease in EDV of only 13 ml. Phase 2 of the study was to assess the impact of MMD changes. In the presence of CRT in patients with less than 15% MMD, there were no VT episodes. VT paroxysms were reported in patients whose MMD exceeded 15%. Conclusions (1) Effective CRT in patents with non-ischemic cardiomyopathy significantly reduces the number of VT episodes. (2) Improving fatty acid metabolism (LVD MMD less than 15%) in patients with non-ischemic cardiomyopathy reduces the incidence of VT episodes against the background of CRT.

Effectiveness of Cardiometabolic Therapy in the Treatment of Acute Coronary Syndrome without ST-segment Elevation in Perimenopausal Women

Acute forms of coronary heart disease (CHD) are one of the most pressing problems in the structure of morbidity and mortality, especially among perimenopausal women. Of particular interest are approaches to the medical correction of acute myocardial ischemia that occurs on the background of non-obstructive lesions or intact coronary arteries. The objective: to study the effectiveness of a fixed combination of meldonium with γ-butyrobetaine (γ-BB) as part of the optimal pharmacotherapy of GKSbelST. Materials and methods. 81 women with percutaneous and postmenopausal GKSbelST at the age of 39–69 years (mean age – 50.26 ± 1.24 years) were examined. Patients were evaluated major circadian blood pressure monitoring parameters (MMAD) was determined levels of female sex hormones (estradiol, follicle-stimulating hormone (FSH) and luteinizing hormone (LH)), circulating endothelial dysfunction markers (endothelial NO-synthase (eNOS), endothelin-1 (ET-1)), indicators of systemic inflammation (PSA) and POL (malonic dialdehyde (MDA), ceruloplasmin (CP)), the main indicators of lipid, carbohydrate and purine metabolism at the beginning of the study on the first day of hospitalization and after 2 weeks hospital treatment. Patients were divided into two groups. The 1-st group (n = 29) included women (average age – 51.26 ± 1.82 years), to whom a fixed combination of γ-BB dihydrate (60 mg) and meldonium dihydrate (180 mg) was added to the standard OMT (Kapikor, “Olainfarm”, Latvia) 2 capsules 2 times a day; in the 2nd group (n = 52) – women with GKSbelST women (average age – 49.64 ± 1.12 years), who received only the standard OMT complex. Results. On the first day of hospitalization of patients with GKSbelST, there was a decrease in eNOS and an increase in ET-1, MDA, CB, and SRP compared to practically healthy individuals, with ABPM, an increase in BP variability (VAD), an increase in BP (HV AD), time index (YV ) at night and insufficient nightly decrease in blood pressure. Compared with women of the 2 nd group, two-week treatment of patients of the 1st group significantly accelerates the restoration of endothelial function. The content of eNOS increased in the 1st group by 22.13% (from 191.76 ± 7.67 to 234.82 ± 17.26 pg / ml; p 0.05), levels of E T-1 decreased (by 23.45% in the 1st group (from 15 , 31 ± 0.60 to 11.71 ± 0.68 pg / ml; p 0.05). Conclusion. Adding to the GKSbelST treatment in women of a fixed combination of meldonium with γ-BB significantly speeds up the restoration of endothelial function and contributes to a decrease in peripheral resistance, balances vascular tone, stabilizes systemic and regional blood flow, which is manifested by the rapid positive dynamics of pressure loads, improved clinical course of NS and reduced hospitalization. Women who took the meldonium with γ-BB combination, in addition to the standard OMT, recorded significant positive changes in the content of female sex hormones in the blood, in particular estradiol (by 20.8%), which may be a consequence of the beneficial effect of meldonium with γ-BB on the microcirculation ovary.

Сучасна немедикаментозна терапія клімактеричного синдрому

The objective: to learn the effectiveness of non-medicated methods of treatment of climacteric syndrome with the use of HBO and metered physical activities. Materials and methods: 60 women with severe menopausal symptoms were examined. The patients were divided into two groups: • the main group – 35 women, who used terencuri or HBO for the treatment; • the comparative group – 25 women, who used hormone therapy. Results. The treatment of climacteric syndrome with the use of non-hormonal methods led to the significant decrease (P&lt;0.05) in the majority of clinical symptoms. The most positive clinical dynamics was noticed in the main group, which is confirmed by a decrease of such symptoms as: hot flashes, sleep disorders, psychological and urinal disorders. Conclusions: the result of the study confirms that the inclusion of HBO and metered physical activities in the symptomatic therapy is an effective way of treatment of the climacteric syndrome. Key words: climacteric syndrome, symptoms, hyperbaric oxygenation and terencuri.

P76-S Therapeutic possibilities of transcranial magnetic stimulation in patients after traumatic brain injury (updated report)

Objectives The purpose of this study is to estimate the therapeutic potential of transcranial magnetic stimulation (TMS) in patients after traumatic brain injury (TBI) with impaired consciousness and motor activity. Methods Observation groups: (1)19 patients after TBI at the age of 16–50 with a prolonged (from 60 to 360 days) unconscious state in which the rhythmic TMS was performed; (2) Control group: 6 healthy men aged 22–28. Changes of neurological status, indicators of the memory and attention, and N100, N200 and P300 components of auditory ERP hour before and hour after stimulation, the dynamics of diagnostic TMS and bimodal (somatosensory and short latency auditory) EP’s were estimated. Results Therapeutic rTMS of the sagittal premotor cortex areas in both groups concurred with improvement of any attention and normalization of amplitude and latency of all ERP components, especially for P300. rTMS of the frontal lobes facilitated positive clinical dynamics, while rTMS of the parietal lobes did not prove any clear positive effects. Conclusions The obtained data proposes the availability of using rTMS for therapeutic purposes in patients with PUS. The most promising targets for stimulation at the moment seems to be the frontal and sagittal premotor areas of the brain. Further research is required to clarify the areas and parameters of stimulation. Supported by: RFFI N 16-29-08304 .

Paroxysmal nocturnal hemoglobinuria: the path to diagnosis

The aim of study was to describe the case of diagnosis of orphan disease — paroxysmal nocturnal hemoglobinuria in clinical practice of the therapist on an outpatient basis. Materials and methods . The patient Sh., 47 years, due to the gradual increase in the volume of the abdomen, the appearance of swelling of the legs, the increase in shortness of breath, yellowing of the skin, the appearance of rare but abundant nosebleeds appealed to 63polyclinic N. I. Pirogov City Clinical Hospital № 1. The patient was repeatedly hospitalized. During the diagnostic search, additional research methods were carried out: evaluation of laboratory data in dynamics; x-ray of the abdominal cavity; ultrasound examination of the abdominal cavity, kidneys and retroperitoneal space; computed tomography of the abdominal cavity; ultrasound examination of the pelvic organs; liver biopsy followed by microscopic examination of micro-drugs; samples for the diagnosis of autoimmune diseases; immunophenotyping of peripheral blood. The patient was consulted by a surgeon, hematologist. Results. In the course of difficult verification of such rare pathology, diagnostic and therapeutic concepts changed. The patient underwent symptomatic treatment: drainage of the abdominal cavity with evacuation of more than 14 liters of ascitic fluid; therapy with fresh frozen plasma; plasmapheresis. And only after positive clinical and laboratory dynamics during therapy with Eculizumab, immunophenotypic diagnosis was performed, that showed erythrocytes of type I (normal expression of CD59) — 1.2 %, type III (complete deficiency of CD59) — 49.5 %; monocytes with FLAER/CD14 deficiency — 83.4 %; granulocytes with FLAER/CD24 deficiency — 77.77 %. On the basis of these results, the diagnosis of paroxysmal nocturnal hemoglobinuria was established. Taking into account the severe course of the disease, according to vital indications, the patient continues therapy with Eculizumab at a maintenance dose of1200 mg every 14 (± 2) days. Conclusion . Timely highly qualified diagnosis, a modern approach in the treatment of this disease, provided a satisfactory outcome of the process in the patient with improved clinical condition and a favorable prognosis for health.

Long-term results of chronic intrathecal baclofen treatment in patients with spasticity and secondary dystonia

To estimate the efficacy of intrathecal treatment with baclofen (ITB) in spasticity and dystonia. Two groups of patients have been operated: 19 patients with spinal spasticity and 33 patients with cerebral spasticity (cerebral palsy). After baclofen screening test, the Synchromed2 (Medtronic) was implanted. The level of muscle tone was assessed by the Ashworth scale. The locomotion was assessed by GMFM 88 and Arens scale. The dystonia was assessed by the BFM scale. Follow-up was performed 3, 6 and 12 months after the operation and yearly after that. The duration of the follow-up ranged from 2 to 9 years. ITB led to a significant decrease in leg spasticity in both groups of patients (p<0.001). The dynamics of spasticity was more significant in the first group compared to the second group (2.77 and 2.07 points, respectively (p<0.0031)). The dynamics of muscle tone in arms was lesser then in legs (p<0.0022). The positive clinical dynamics in patients with secondary dystonia was minimal (BFM from 65±17 to 60±19). Increasing of ITB daily dose was required to maintenance the clinical effect in most patients. The loss of ITB effect was noted in 5% of patients. ITB was more effective in patients with spinal spasticity. The influence of ITB on the secondary dystonia was lesser then on the spasticity. The dynamic of the muscle tone was more significant in legs than in arms. The loss of ITB effect can be observed in some cases after the operation despite the positive result of baclofen screening-test.

Task-Related Reorganization of Functional Connectivity in Early Detection of Consciousness in Patients With Severe Brain Injury

In recent years the number of patients with early postcomatosis long-term states of unconsciousness is increasing in the world. Accurate assessment of current functional state of patients in unconscious states is very important for correct treatment strategy and rehabilitation activities especially in cases when the difference between those states is not clinically obvious. To investigate features of resting-state wavelet synchrony in unconscious patients with severe brain injury and how TMS-therapy can affect resting state functional connectivity. We used stimulus-based EEG paradigm to detect changes of functional connectivity after TMS-therapy. Fifteen patients were recruited. They had the diagnosis of minimally conscious state (MCS) (male = 9, female = 2; n = 11), vegetative state/unresponsive wakefulness syndrome (VS/UWS) (male = 4, n = 4). We analyzed functional connectivity, values of wavelet synchrony in resting state and for responses on stimuli of patients before and after TMS-therapy. Of the 15 patients, eleven (eight MCS, two VS/UWS) had positive clinical dynamics including improvement of moving, emergence of glance fixation and gaze tracking, speech understanding and attempts of speech productions. The appearance of interhemispheric and diagonal connections is a favorable prognostic sign. TMStherapy makes it possible to include activate functional networks in resting state.

The excitotoxic impairment of brain in patients with discirculatory encephalopathyand therapeutic correction

In view of the proved fact of influence of excitotoxicity on progressive course of dyscirculatory encephalopa- thy, clinical, neurophysiological investigation of the patients in dynamics of treatment with cytoflavin and cortexin. The positive clinical dynamics and partial normalization of electroencephalographic patterns were found out. Correlation analysis have proved ambiguous connection between clinical signs, focal changes in brain and therapeutic effectiveness. Results of investigation have proved the appropriateness of dyscirculatory encephalopathy therapy with cytoflavin and cortexin with the aim of inhibition of excitotoxicity. Multiplevalued connection between obtained parameters allow to assume the necessity of future investigations on this problem.

Dynamics of the endotoxicosis indices in patients, suffering extended purulent peritonitis

Objective. To investigate the endotoxicosis indices dynamics in patients, suffering extended purulent peritonitis (EPP) during the treatment process.&#x0D; Маterials and methods. In the investigation 39 patients, suffering EPP in terminal stage, were included. Quantitative contents of endotoxin in the blood serum (the amoebacytes lysate Limulus) was determined in dynamics, using LAL-test, and the patient’s state severity was estimated, basing on clinical and laboratory indices. Hospital lethality have characterized the disease course.&#x0D; Results. In patients with positive clinical dynamics (Group I) the average quantitative indices of endotoxicosis have lowered trustworthily (р=0.0001). In the patients, who died (Group II), the endotoxicosis quantitative indices dynamics were trustworthily higher, comparing with average indices in patients of Group I (р &lt; 0.05).&#x0D; Conclusion. Еndotoxicosis, measuring more 5 еndotoxic units in 1 ml of the blood serum, during 3 postoperative days constitutes one of indications for reoperation. LAL-test in patients, suffering EPP, has statistically significant prognostic and diagnostic value, what substantiates recommendation for its application as indicator of the pathological process course.