Review of Efficiency Improvement Technologies of Wind Diesel Hybrid Systems for Decreasing Fuel Consumption

The article contains current information on the development of energy-efficient technologies of wind–diesel hybrid systems (WDHS) for decreasing organic fuel consumption. As a result of the review, three research directions are identified: WDHS design optimization, the main equipment and control system improvements. A comparison of their effectiveness is presented. The methods of selecting WDHS configuration, equipment capacities and location, the optimization algorithms and objective functions used are described and WDHS project feasibility calculation results are presented. The methods to improve energy efficiency of WDHS major units’ (diesel generator (DG) and wind turbine (WT)) are considered. The methods to decrease diesel fuel consumption using special devices and energy storage system are presented. Special attention is paid to WDHS operating modes’ control methods and strategies, as well as to algorithms providing the efficient system operation. As a result, recommendations for the design of both isolated and on-grid WDHS are formulated

Optimization of virtual power plant with a distributed generation

The article is devoted to the task of optimizing the structure of virtual power plants (VPP) with distributed generation sources. The solution of the problem is based on the structural topological and probabilistic-statistical analysis of the VPP structure. An algorithm for estimating the structure of VPP has been developed. The optimization criteria for VPP are proposed and investigated. The criteria allow to evaluate VPP structural properties and to take into account the inconstancy of generation from renewable energy sources (RES) in VPP composition

Thyristor Voltage Regulator Experimental Research

The article is devoted to the thyristor voltage regulator (TVR) development. The TVR purpose is to control power flows and regulate voltage in 6-20 kV distribution electrical networks (DEN). The principle of TVR operation is based on the plus EMF (or minus EMF) introduction into power line when the shared use of longitudinal (change of magnitude) and transverse (change of phase) voltage regulation. The description of the TVR prototype is given. The TVR prototype consists of a 0.4 kV thyristor switches, power transformers (shunt and serial) and a 6 kV switchgear. The TVR has a two-level control system (CS). The TVR prototype experimental research was conducted in four stages: check of power equipment, first level CS research, second level CS research, prototype tests as a whole. The connection diagrams (thyristor switches unit, transformer and measuring equipment) and contact connections reliability were checked when the power part was tested. A qualitative characteristic of the input and output signals was obtained when testing the first level CS. It is found that the thyristor control pulses are formed according to the developed algorithm. The correctness of control system algorithms, executed and transmitted commands, passed and received data was confirmed as a result of the second level CS tests. The TVR research results indicate that the prototype provides the smoothness and specified accuracy of voltage regulation in all modes. The control range of the output voltage relative to the input was ±10%. The discreteness of regulation did not exceed 1.5%. The range of change in the shift angle of the output voltage relative to the input was ±5°. Research confirmed the TVR ES operability and its readiness for trial operation

Economy Mode Setting Device for Wind-Diesel Power Plants

The article is devoted to the problem of reducing fuel consumption in a diesel generator set (DGS) as a part of a wind-diesel power plant (WDPP). The object of the research is a variable speed DGS. The goal is to develop the WDPP intelligent control system, providing an optimal shaft speed of an internal combustion engine (ICE). The basis of the intelligent control system is an economy mode setting device (EMSD), which controls the fuel supply to the ICE. The functional chart of EMSD has been presented. The main EMSD blocks contain a controller and an associative memory block. The associative memory block is a software model of an artificial neural network that determines the optimal shaft speed of the ICE. An algorithm for the WDPP intelligent control system has been developed and tested using the WDPP Simulink model. The EMSD prototype has been created, and its research has been conducted. Dependences of the change in specific and absolute fuel consumption on the load power have been obtained for two 4 kW DGS: with constant rotation speed and variable rotation speed DGS with EMSD. It has been established that the use of EMSD in the mode of low loads allow one to reduce fuel consumption by almost 30%. The error in determining the optimal engine speed using EMSD prototype is not more than 15%

Review of Efficiency Improvement Technologies of Wind Diesel Hybrid Systems for Decreasing Fuel Consumption

The article contains current information on the development of energy-efficient technologies of wind–diesel hybrid systems (WDHS) for decreasing organic fuel consumption. As a result of the review, three research directions are identified: WDHS design optimization, the main equipment and control system improvements. A comparison of their effectiveness is presented. The methods of selecting WDHS configuration, equipment capacities and location, the optimization algorithms and objective functions used are described and WDHS project feasibility calculation results are presented. The methods to improve energy efficiency of WDHS major units’ (diesel generator (DG) and wind turbine (WT)) are considered. The methods to decrease diesel fuel consumption using special devices and energy storage system are presented. Special attention is paid to WDHS operating modes’ control methods and strategies, as well as to algorithms providing the efficient system operation. As a result, recommendations for the design of both isolated and on-grid WDHS are formulated

Leukemia risk and the pattern of dose accumulation. Part 1: Characteristics of the study group of the Mayak Production Association personnel

Results of development of the database “Leukemia in the cohort of workers of the Mayak Production Association employed in 1948-1958” that was developed in the Laboratory of Radiation Epidemiology of Southern Urals Biophysics Institute were presented. The database contains hematological, clinical and dosimetric information for the workers of main plants of the first production facility of Soviet atomic industry employed in the period of production development – 1948-1958. The computer database contains information for two groups of Mayak Production Association workers: main group of workers with leukemia as the main cause of death (n=84) and group of personnel without hematological cancer pathology for comparison (n=300). The comparison group was composed in such a way to allow 3-4 controls of corresponding gender, age of exposure start, accumulated dose of external gamma-exposure to red bone marrow for each leukemia case. Methods of data collection and sources of medical data were described. Characteristics of software developed for the database was presented. The database containing a total of 19593 results of blood tests reflects dynamics of hematological values in atomic facility personnel due to prolonged radiation exposure. The range of accumulated absorbed doses of external gamma-exposure to red bone marrow in the group of personnel with leukemia diagnoses was 0.001-4.96 Gy; average dose for males was 1.24 Gy and 0.61 Gy – for females. Similar range is observed in the group of personnel without leukemia outcomes. Alongside differences in accumulation rates of external radiation exposure doses is evident: in the group of workers with hematological cancer pathology the average period of radiation exposure at production facility was 14.3 years for males and 12.1 years for females; in the comparison group – 20.4 and 13.9 years, respectively. Most leukemia cases were diagnosed in the workers of radiochemical facility (54.8%). Myeloid leukemia prevailed (47.6%) in the leukemia structure; among them acute myeloid leukemia made more than a half of the cases; lymphoid leukemia was diagnosed in 14.3% cases with prevailing role of chronic lymphoid leukemia. Acute and chronic monocytic leukemia were the rarest types of malignant neoplasms of hematopoietic tissue. Possible correlation between hematological values with individual doses of external gamma- and internal alpha-exposure (incorporated Pu-239) distributed over time was stated. The database allows calculating integrated leukocytic indices reflecting response of hemopoietic system to radiation exposure, tracking changes in hemopoiesis in proportion to accumulated dose to red bone marrow, analyzing specific characteristics of rehabilitation of hematological alterations after occupational contact to ionizing radiation is terminated. Information on acute and chronic diseases available for the workers in the database allows excluding non-specific response of hemopoietic system caused by associated pathology. A revision of leukemia risk estimated in the relation to dose rate for personnel exposed to chronic radiation could be regarded as one of perspective trends in using the database

Лейкомогенный риск и темп накопления радиационной дозы. Сообщение 1: Характеристика исследуемой группы работников производственного объединения «МАЯК»

Results of development of the database “Leukemia in the cohort of workers of the Mayak Production Association employed in 1948-1958” that was developed in the Laboratory of Radiation Epidemiology of Southern Urals Biophysics Institute were presented. The database contains hematological, clinical and dosimetric information for the workers of main plants of the first production facility of Soviet atomic industry employed in the period of production development – 1948-1958. The computer database contains information for two groups of Mayak Production Association workers: main group of workers with leukemia as the main cause of death (n=84) and group of personnel without hematological cancer pathology for comparison (n=300). The comparison group was composed in such a way to allow 3-4 controls of corresponding gender, age of exposure start, accumulated dose of external gamma-exposure to red bone marrow for each leukemia case. Methods of data collection and sources of medical data were described. Characteristics of software developed for the database was presented. The database containing a total of 19593 results of blood tests reflects dynamics of hematological values in atomic facility personnel due to prolonged radiation exposure. The range of accumulated absorbed doses of external gamma-exposure to red bone marrow in the group of personnel with leukemia diagnoses was 0.001-4.96 Gy; average dose for males was 1.24 Gy and 0.61 Gy – for females. Similar range is observed in the group of personnel without leukemia outcomes. Alongside differences in accumulation rates of external radiation exposure doses is evident: in the group of workers with hematological cancer pathology the average period of radiation exposure at production facility was 14.3 years for males and 12.1 years for females; in the comparison group – 20.4 and 13.9 years, respectively. Most leukemia cases were diagnosed in the workers of radiochemical facility (54.8%). Myeloid leukemia prevailed (47.6%) in the leukemia structure; among them acute myeloid leukemia made more than a half of the cases; lymphoid leukemia was diagnosed in 14.3% cases with prevailing role of chronic lymphoid leukemia. Acute and chronic monocytic leukemia were the rarest types of malignant neoplasms of hematopoietic tissue. Possible correlation between hematological values with individual doses of external gamma- and internal alpha-exposure (incorporated Pu-239) distributed over time was stated. The database allows calculating integrated leukocytic indices reflecting response of hemopoietic system to radiation exposure, tracking changes in hemopoiesis in proportion to accumulated dose to red bone marrow, analyzing specific characteristics of rehabilitation of hematological alterations after occupational contact to ionizing radiation is terminated. Information on acute and chronic diseases available for the workers in the database allows excluding non-specific response of hemopoietic system caused by associated pathology. A revision of leukemia risk estimated in the relation to dose rate for personnel exposed to chronic radiation could be regarded as one of perspective trends in using the database.Представлены результаты разработки базы данных «Лейкозы в когорте персонала производственного объединения «Маяк» 1948–1958 гг. найма», созданной в лаборатории радиационной эпидемиологии Южно-Уральского института биофизики. База данных содержит гематологическую, клиническую и дозиметрическую информацию о работниках основных заводов первого промышленного объекта отечественной атомной отрасли, нанятых в период становления производства – 1948–1958 гг. В компьютерную базу данных вошли сведения о двух группах работников производственного объединения «Маяк»: основную группу работников, причиной смерти которых был лейкоз (n=84), и группу сравнения – персонал предприятия без онкогематологической патологии (n=300). Группа сравнения сформирована из расчета 3–4 контроля к каждому случаю лейкоза с сопоставлением по полу, возрасту начала облучения, накопленной дозе внешнего гамма-облучения на красный костный мозг. Описаны методика сбора данных и использованные источники медицинской информации. Дана характеристика специально созданного программного обеспечения. База данных, включающая в итоге 19 593 анализа крови, отражает динамику гематологических показателей персонала атомного комплекса в результате пролонгированного радиационного воздействия. Диапазон накопленных поглощенных доз внешнего гамма-облучения на красный костный мозг в группе персонала с диагностированными лейкозами составил 0,001–4,96 Гр со средней дозой среди мужчин – 1,24 Гр, среди женщин – 0,61 Гр. Группа персонала без исхода в виде лейкозов характеризовалась аналогичным диапазоном доз. Вместе с тем, наблюдались различия в темпе накопления внешних доз радиационного воздействия. Средний период радиационного воздействия на производстве в группе работников с онкогематологической патологией составил среди мужчин 14,3 года, среди женщин – 12,1 года; в группе сравнения – 20,4 и 13,9 лет соответственно. Наибольшее число диагностированных лейкозов отмечалось у персонала радиохимического производства (54,8%). В структуре лейкозов у работников преобладали миелолейкозы (47,6%), среди которых острые миелолейкозы составляли более половины; лимфолейкоз диагностирован в 14,3% случаев с превалированием хронических лимфолейкозов. Наиболее редкими формами злокачественных новообразований кроветворной ткани были острый и хронический моноцитарные лейкозы. Показана возможность соотнесения гематологических показателей с индивидуальными дозами внешнего гамма- и внутреннего альфа-облучения (инкорпорированным плутонием-239), распределенными по времени. База данных позволяет производить расчет интегральных лейкоцитарных индексов, отражающих реагирование системы кроветворения на радиационное воздействие, отслеживать изменения гемопоэза пропорционально накопленной дозе на красный костный мозг, анализировать особенности восстановления гематологических сдвигов после прекращения контакта персонала с ионизирующей радиацией. Наличие сведений об острых и хронических заболеваниях работников дает возможность исключить неспецифические реакции системы кроветворения, вызванные сопутствующей патологией. В качестве перспективного направления использования базы данных может рассматриваться пересмотр оценок лейкомогенного риска в зависимости от мощности дозы у персонала, подвергавшегося хроническому радиационному воздействию

Establishment of a Pilot Newborn Screening Program for Spinal Muscular Atrophy in Saint Petersburg

Spinal muscular atrophy 5q (SMA) is one of the most common neuromuscular inherited diseases and is the most common genetic cause of infant mortality. SMA is associated with homozygous deletion of exon 7 in the SMN1 gene. Recently developed drugs can improve the motor functions of infants with SMA when they are treated in the pre-symptomatic stage. With aim of providing an early diagnosis, newborn screening (NBS) for SMA using a real-time PCR assay with dried blood spots (DBS) was performed from January 2022 through November 2022 in Saint Petersburg, which is a representative Russian megapolis. Here, 36,140 newborns were screened by the GenomeX real-time PCR-based screening test, and three genotypes were identified: homozygous deletion carriers (4 newborns), heterozygous carriers (772 newborns), and wild-type individuals (35,364 newborns). The disease status of all four newborns that screened positive for the homozygous SMN1 deletion was confirmed by alternate methods. Two of the newborns had two copies of SMN2, and two of the newborns had three copies. We determined the incidence of spinal muscular atrophy in Saint Petersburg to be 1 in 9035 and the SMA carrier frequency to be 1 in 47. In conclusion, providing timely information regarding SMN1, confirmation of disease status, and SMN2 copy number as part of the SMA newborn-screening algorithm can significantly improve clinical follow-up, testing of family members, and treatment of patients with SMA

Clinical and Genetic Characteristics of Pediatric Patients with Hypophosphatasia in the Russian Population

(1) Hypophosphatasia (HPP) is a rare inherited disease caused by mutations (pathogenic variants) in the ALPL gene which encodes tissue-nonspecific alkaline phosphatase (TNSALP). HPP is characterized by impaired bone mineral metabolism due to the low enzymatic activity of TNSALP. Knowledge about the structure of the gene and the features and functions of various ALPL gene variants, taking into account population specificity, gives an understanding of the hereditary nature of the disease, and contributes to the diagnosis, prevention, and treatment of the disease. The purpose of the study was to describe the spectrum and analyze the functional features of the ALPL gene variants, considering various HPP subtypes and clinical symptoms in Russian children. (2) From 2014–2021, the study included the blood samples obtained from 1612 patients with reduced alkaline phosphatase activity. The patients underwent an examination with an assessment of their clinical symptoms and biochemical levels of TNSALP. DNA was isolated from dried blood spots (DBSs) or blood from the patients to search for mutations in the exons of the ALPL gene using Sanger sequencing. The PCR products were sequenced using a reagent BigDye Terminator 3.1 kit (Applied Biosystems). Statistical analysis was performed using the GraphPad Prism 8.01 software. (3) The most common clinical symptoms in Russian patients with HPP and two of its variants (n = 22) were bone disorders (75%), hypomyotonia (50%), and respiratory failure (50%). The heterozygous carriage of the causal variants of the ALPL gene was detected in 225 patients. A total of 2 variants were found in 27 patients. In this group (n = 27), we identified 28 unique variants of the ALPL gene, of which 75.0% were missense, 17.9% were frameshift, 3.6% were splicing variants, and 3.6% were duplications. A total of 39.3% (11/28) of the variants were pathogenic, with two variants being probably pathogenic, and 15 variants had unknown clinical significance (VUS). Among the VUS group, 28.6% of the variants (7/28) were discovered by us for the first time. The most common variants were c.571G > A (p.Glu191Lys) and c.1171del (Arg391Valfs*12), with frequencies of 48.2% (13/28) and 11% (3/28), respectively. It was found that the frequency of nonsense variants of the ALPL gene was higher (p < 0.0001) in patients with the perinatal form compared to the infantile and childhood forms of HPP. Additionally, the number of homozygotes in patients with the perinatal form exceeded (p < 0.01) the frequencies of these genotypes in children with infantile and childhood forms of HPP. On the contrary, the frequencies of the compound-heterozygous and heterozygous genotypes were higher (p < 0.01) in patients with infantile childhood HPP than in perinatal HPP. In the perinatal form, residual TNSALP activity was lower (p < 0.0005) in comparison to the infantile and childhood (p < 0.05) forms of HPP. At the same time, patients with the heterozygous and compound-heterozygous genotypes (mainly missense variants) of the ALPL gene had greater residual activity (of the TNSALP protein) regarding those homozygous patients who were carriers of the nonsense variants (deletions and duplications) of the ALPL gene. Residual TNSALP activity was lower (p < 0.0001) in patients with pathogenic variants encoding the amino acids from the active site and the calcium and crown domains in comparison with the nonspecific region of the protein