Identification of possible non-stationary effects in a new type of vortex furnace

The article presents the results of an experimental study of pressure and velocity pulsations in the model of improved vortex furnace with distributed air supply and vertically oriented nozzles of the secondary blast. Investigation of aerodynamic characteristics of a swirling flow with different regime parameters was conducted in an isothermal laboratory model (in 1:25 scale) of vortex furnace using laser Doppler measuring system and pressure pulsations analyzer. The obtained results have revealed a number of features of the flow structure, and the spectral analysis of pressure and velocity pulsations allows to speak about the absence of large-scale unsteady vortical structures in the studied design

Thermography of flame during diesel fuel combustion with steam gasification

The paper represents a study concerning the combustion of liquid hydrocarbon fuel in a perspective burner device with the controlled forced supply of overheated steam into the combustion zone, using diesel fuel. The thermal imaging measurements are conducted for the outer flame of the burner device in the wide range of regime parameters (flow rate and temperature of steam). A thermal imaging camera (FLIR, JADE J530SB) is used in the experiments. The effective emissivity coefficient of flame is obtained versus the flow rate of steam supplied. The steam parameters are found to influence on the temperature in the outer flame of the burner device

Новая мутация в гене TRIP4, ассоциированная с фенотипом врожденной мышечной дистрофии типа Давиньон–Шове (клинический случай)

Congenital muscular dystrophies are heterogeneous groups of neuromuscular diseases leading to hypotonia, progressive muscle weakness and dystrophic or structural signs in muscle biopsy. At the present time, 34 genes associated with congenital muscular dystrophy have been described. The clinical case of a rare form of congenital muscular dystrophia associated with a homozygous mutation in the TRIP4 gene in a patient with respiratory failure requiring respiratory support, neurological symptoms, muscular hypotonia, and multiple congenital malformations of skeletal system is presented for the first time in Russia. The undescribed pathogenic homozygous variant of the nucleotide sequence in the TRIP4 gene (chr15:64686179, c.136C>T, p.Arg46Ter, 2 exon, NM_016213.4) was detected by whole exome sequencing. The mutation in the TRIP4 gene was validated by Sanger sequencing in a child and its origin was investigated. The mother and father of the girl are carriers of the heterozygous variant in the TRIP4 gene. Identification of the genetic cause of a rare form of neuromuscular disease is important for determining the tactics of patient management and medical and genetic counseling of the family, as well as clarifying the pathogenesis of a rare pathology. Врожденные мышечные дистрофии и врожденные миопатии представляют собой гетерогенную группу нервно-мышечных заболеваний, приводящих к гипотонии, прогрессирующей мышечной слабости и дистрофическим или структурным признакам при мышечной биопсии. В настоящее время описано 34 гена, связанных с врожденной мышечной дистрофией. Впервые в России представляется клинический случай редкой формы врожденной мышечной дистрофии, обусловленной гомозиготной мутацией в гене TRIP4, у пациента с дыхательной недостаточностью, требующей респираторной поддержки, неврологической симптоматикой, мышечной гипотонией, множественными врожденными пороками развития опорно-двигательной системы. В результате проведенного полноэкзомного секвенирования выявлен ранее не описанный патогенный вариант нуклеотидной последовательности в гене TRIP4 в гомозиготном состоянии, приводящий к остановке синтеза полнофункционального белка (chr15:64686179, c.136C>T, p.Arg46Ter, 2 й экзон, NM_016213.4). Мутация в гене TRIP4 была валидирована методом секвенирования по Сэнгеру у ребенка, и исследовано ее происхождение. Мать и отец девочки являются носителями гетерозиготного варианта в гене TRIP4. Выявление генетической причины редкой формы нервно-мышечного заболевания важно для определения тактики ведения пациента и медико-генетического консультирования семьи, а также уточнения патогенеза редкой патологии

Identification of possible non-stationary effects in a new type of vortex furnace

The article presents the results of an experimental study of pressure and velocity pulsations in the model of improved vortex furnace with distributed air supply and vertically oriented nozzles of the secondary blast. Investigation of aerodynamic characteristics of a swirling flow with different regime parameters was conducted in an isothermal laboratory model (in 1:25 scale) of vortex furnace using laser Doppler measuring system and pressure pulsations analyzer. The obtained results have revealed a number of features of the flow structure, and the spectral analysis of pressure and velocity pulsations allows to speak about the absence of large-scale unsteady vortical structures in the studied design