Controlled Compact High Voltage Power Lines

Nowadays modern overhead transmission lines (OHL) constructions having several significant differences from conventional ones are being used in power grids more and more widely. Implementation of compact overhead lines equipped with FACTS devices, including phase angle regulator settings (compact controlled OHL), appears to be one of the most effective ways of power grid development. Compact controlled AC HV OHL represent a new generation of power transmission lines embodying recent advanced achievements in design solutions, including towers and insulation, together with interconnection schemes and control systems. Results of comprehensive research and development in relation to 110–500kV compact controlled power transmission lines together with theoretical basis, substantiation, and methodological approaches to their practical application are presented in the present paper

The influence of walking in an exoskeleton on rehabilitation of patients with spinal cord injury consequences

Objective: to substantiate the expediency of including training walking in an exoskeleton in the complex rehabilitation of patients with severe motor dysfunctions of the lower limbs due to spinal cord injury (SCI).Methods: 120 patients (84 men and 36 women, age 37.1 ± 8.40 years) with the consequences of SCI at the level of thoracic and lumbar spine who were in the hospital at the second stage of medical rehabilitation were examined. The duration of injury ranged from 1 year to 19 years (77.8 ± 4.05 months). All patients were divided into two groups (80 and 40 patients), comparable by sex, age, duration and severity of injury. Training walking in exoskeleton have been added in the group I rehabilitation program. ASIA, SCIM III scales and SF-36 quality of life questionnaire were used to evaluate the effectiveness of the rehabilitation measures.Results: there was an increase in the total score on SCIM III by 3.20 ± 0.50 (p = 0.05) in group I, which indicates an increase in the level of functioning and daily activity of patients. Positive dynamics was revealed when assessing the quality of life using SF-36 questionnaire: sum index reflecting the psychological component of health increased from 56,0 to 59,5 points (p = 0,05). Group II also showed positive dynamics of the analyzed indicators, but the changes by the end of the rehabilitation course were not statistically significant.Conclusion: the use of exoskeleton training walking at the second stage of medical rehabilitation increases the effectiveness of rehabilitation measures and improves the quality of life of patients with severe motor dysfunctions of the lower limbs due to spinal cord injury (SCI)

FEATURES OF HEART RATE REGULATION IN ADOLESCENTS WITH ESSENTIAL HYPERTENSION

Cardiointervalography is an important method of analyzing the regulation of heart rate variability, which allows evaluating the balance of the entire spectrum of humoral-metabolic and vegetative effects, both in condition of physiological rest and in various functional tests. It is noted that in hypertensive patients, in most cases, there is a chronic predominance of ergotrophic metabolic reactions that reflect the system tension in the regulatory mechanisms. The aim of our study was to conduct a comparative analysis of the features of the mechanisms of heart rate regulation in adolescents with confirmed essential hypertension and their healthy age mates. Material and methods. The study involved 50 adolescents (12–16 years of age) – 26 boys and 24 girls with labile essential hypertension (EHT) and stage 1 HT on the background of the standard medical correction of blood pressure (enalapril 0,58 mg/kg per day). Each participant of the study and control group underwent recording of at least 512 cardio cycles in the state of physiological rest in a sitting position, as well as registration of blood pressure. Results and discussion. We have found that for adolescents with EHT a significantly lower (by 15 %) average duration of the heart cycle is typical in combination with a decrease in the variation range, which indicates some rigidity of the heart rate regulatory mechanisms. The relative decrease in the proportion of high-frequency spectrum components in comparison with the low-frequency and very low-frequency ranges in adolescents with hypertension also indicated a tendency to predominance of sympathicotonic effects with the predominant involvement of the structures of the diencephalic region and the brain stem. At the same time blood pressure inversely correlated with the index of the variation range of cardiointervals, reflecting the lower adaptive potential of the heart rate regulation mechanisms. Conclusion. We have found regular changes in the regulation of heart rate in adolescents with hypertension, that can be taken as markers of its progressive development and used by clinicians to develop more personalized approaches to medication treatment

Comparative assessment of energy metabolism, body composition and metabolic features in children with hypothalamic and simple obesity

Background: Hypothalamic obesity often develops after surgical treatment of craniopharyngioma and is characterized by rapid weight gain, high frequency of metabolic disorders, body composition specificity and resistance to standard lifestyle modification approaches and medication therapy of obesity. Recent studies show that one of the mechanisms, explaining weight gain in these children is decrease in resting energy expenditure (REE).Aims: To compare REE, body composition parameters, and the frequency of metabolic disorders in children with hypothalamic and simple obesity.Materials and methods: The study included 60 obese children aged 7 to 17 years, divided into two groups. The study group included 20 children with hypothalamic obesity, developed after craniopharyngioma treatment. The control group consisted of 40 children with simple obesity. Body composition, REE, and metabolic disorders were associated in all children.Results: Children with hypothalamic obesity showed a significant decrease of resting energy expenditure. The average decrease in REE was 13.1%, but in single patients it reached 33.4%. The percentage of fat mass in hypothalamic and simple obesity does not differ significantly (39.7% [36.2; 42.6] vs 38.8 % [35.9; 43.2]; p=0.69). Screening for metabolic disorders revealed a high prevalence of metabolic disorders in hypothalamic obesity: impaired glucose tolerance - in 10%; dyslipidemia - 55%, insulin resistance-50%, non-alcoholic fatty liver disease - 60 %.Conclusions: Children with hypothalamic obesity showed a significant decrease of resting energy expenditure. When planning a diet in this group of patients it is preferable to use indirect calorimetry. Hypothalamic obesity even at an early age is associated with a high frequency of metabolic disturbance. Hypothalamic obesity in children is not associated with more pronounced hyperinsulinemia and insulin resistance compared to the simple obesity

Carbohydrate metabolism in children after complex treatment of medulloblastoma

BACKGROUND: Disorders of carbohydrate metabolism are frequent complications after complex treatment of cancer. Early detection of these disorders allows to modify lifestyle, if necessary, apply drug therapy and correct the identified changes in time, which will subsequently lead to a decrease in cardiovascular risks, an improvement in the quality and an increase in the life expectancy of this group of patients.AIM: To identify the frequency of occurrence and determine the optimal methods for early diagnosis of carbohydrate metabolism disorders in children after complex treatment of medulloblastoma.MATERIALS AND METHODS: 97 patients (64 boys) were examined after complex treatment of medulloblastoma (surgical treatment, craniospinal radiation therapy [CSI], chemotherapy). The median age at the time of the survey was 11.2  (5.29 -17.9) years. The average follow-up period after the end of all types of treatment is 4.7 (0.59 - 11.94) years. At the time of examination, all patients were compensated for hypothyroidism and hypocorticism (if any), and no one was on growth hormone therapy. All patients underwent a standard oral glucose tolerance test (OGTT) and determination of the level of glycated hemoglobin (HbA1c).RESULTS: None of the patients had a basal glycemic level higher than 5.5 mmol / L. Impaired glucose tolerance ( glycemia at the 120th minute ≥7.8 mmol / L <11.09 mmol / L) was detected in 10 out of 97 patients (10.3%). 7 patients with IGT had insulin resistance (according to MATSUDA index), while 1 of them had normal HOMA-IR index. Only one patient with IGT had an increase in the level of glycated hemoglobin up to 6.2%. Insulin resistance (using the MATSUDA index) was present in 30 patients, and 11 of them had a normal HOMA-IR index. Thus, 34 out of 97 patients (35.1%) had disorders of carbohydrate metabolism (IGT and / or insulin resistance). At the same time, the level of glycated hemoglobin reflected disturbances in only one case, and the basal level of glycemia was not informative in any case.CONCLUSION: Assessment of basal levels of glycemia and insulin, as well as glycated hemoglobin, is insufficient to exclude disorders of carbohydrate metabolism in patients after complex treatment of medulloblastoma. It is advisable to carry out a standard OGTT

Russian clinical practice guidelines «congenital adrenal hyperplasia»

Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases characterized by a defect in one of the enzymes or transport proteins involved in the cortisol synthesis in the adrenal cortex. The most common form of CAH, which occurs in more than 90% of cases, is a 21-hydroxylase enzyme deficiency. The latter is subdivided into nonclassical and classic (salt-losing and virilizing) forms. The prevalence of classic forms of 21-hydroxylase deficiency ranges from 1: 14,000 to 1:18,000 live births worldwide. According to the data of neonatal screening in the Russian Federation, the prevalence of the disease in some regions ranges from 1: 5000 to 1: 12000, in the country as a whole - 1: 9638 live newborns. The non-classical form of CAH occurs more often - from 1: 500 to 1: 1000 among the general population. In second place is the hypertensive form of CAH - a deficiency of 11β-hydroxylase, which, according to the literature, occurs in about 1 per 100,000 newborns. These clinical guidelines were compiled by a professional community of narrow specialists, approved by the expert council of the Ministry of Health of the Russian Federation, and updated the previous version published in 2016. The clinical guidelines are based on systematic reviews, meta-analyses and original articles, and scientific work on this issue in the Russian Federation and other countries. The purpose of this document is to provide clinicians with the most up-to-date, evidence-based guidelines for the CAH diagnosis and treatmen

Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1

Patients with biallelic loss-of-function variants of AIRE suffer from autoimmune polyendocrine syndrome type-1 (APS-1) and produce a broad range of autoantibodies (auto-Abs), including circulating auto-Abs neutralizing most type I interferons (IFNs). These auto-Abs were recently reported to account for at least 10% of cases of life-threatening COVID-19 pneumonia in the general population. We report 22 APS-1 patients from 21 kindreds in seven countries, aged between 8 and 48 yr and infected with SARS-CoV-2 since February 2020. The 21 patients tested had auto-Abs neutralizing IFN-α subtypes and/or IFN-ω; one had anti–IFN-β and another anti–IFN-ε, but none had anti–IFN-κ. Strikingly, 19 patients (86%) were hospitalized for COVID-19 pneumonia, including 15 (68%) admitted to an intensive care unit, 11 (50%) who required mechanical ventilation, and four (18%) who died. Ambulatory disease in three patients (14%) was possibly accounted for by prior or early specific interventions. Preexisting auto-Abs neutralizing type I IFNs in APS-1 patients confer a very high risk of life-threatening COVID-19 pneumonia at any age.publishedVersio