Experience of using lumacaftor/ivacaftor in children with cystic fibrosis in the Astrakhan region

Background. Targeted therapy in patients with cystic fibrosis, which aims to restore the function of the cystic fibrosis transmembrane conductance regulator protein, is currently revolutionary in the treatment of the disease. The first drug available in Russia for the treatment of patients with this genetic disease is lumacaftor/ivacaftor (Orkambi®), which was registered on December 2, 2020.The aim. To study the efficacy of lumacaftor/ivacaftor during 12 weeks of administration in children in Astrakhan region.Materials and methods. This article presents the clinical experience of Orkambi medicine use in 3 children with F508del/F508del genotype and a severe course of cystic fibrosis for 12 weeks. According to the study design, such indices as body mass index, indexes of external respiratory function, character of respiratory tract microbiota, level of chloride in sweat fluid and pancreatic elastase in feces, dynamics of biochemical blood indexes were evaluated. The study was performed within the time periods specified in the study protocol, namely, before the start of therapy, 2, 4, and 12 weeks of treatment.Results. Two patients had a decrease in sweat test values by 11 and 19 mmol/l from the baseline, a significant increase in forced vital capacity of lungs. One patient had an adverse event in the form of hepatobiliary disorders manifested by increased liver transaminase activity, which was the reason for discontinuing the drug.Conclusions. The short-term experience of using pathogenetic therapy with lumacaftor/ ivacaftor in children in the Astrakhan region demonstrated both the expected effect on chlorine channel function and the possibility of side effects, including severe ones, that can lead to withdrawal of the drug

Screening of powdery mildew resistant barley accessions from Ethiopia for tolerance to abiotic stressors

Background. Analyzing the adaptive potential of cultivated barley with the aim of using new genotypes selected and developed in the process of work is a priority trend in scientific research. The Ethiopian barleys adapted to a variety of soil and climate conditions are characterized by many valuable biological and agronomic traits. Of particular value are genotypes that combine resistance to harmful organisms and environmental stressors.Materials and methods. The collection accessions of Ethiopian barley possessing powdery mildew resistance were studied for tolerance to adverse edaphic factors (chloride salinity and high content of toxic aluminum ions in the soil). Resistance to abiotic stressors was assessed in laboratory experiments. In the study of salt tolerance, a ‘roll-based’ assessment technique was used, which takes into account the inhibition of root growth under salt (NaCl) stress conditions, compared with the reference conditions without salinization. Aluminum sensitivity of barley accessions was diagnosed in the early stages of plant development using the root test.Results and conclusions. A polymorphism of Ethiopian barleys for resistance to adverse edaphic factors was revealed. Twentyone new sources of barley resistance to toxic aluminum ions were identified, of which accessions k-8552 and k-22933 were classified as highly resistant. Accessions k-17554, k-19975, k-20029, k-20048, k-22752, k-23450 and k-25009 proved resistant to soil salinization. Barley accessions k-17554, k-22752 and k-25009 were characterized by complex resistance to powdery mildew, toxic aluminum ions, and chloride soil salinity

Физическая работоспособность студентов транспортного профиля

For the English abstract and full text of the article please see the attached PDF-File (English version follows Russian version). ABSTRACT The article is devoted to the study of physical performance in the railway college. The authors propose a computer program written by them for this purpose, the novelty of which is that it determines, through control measurements and test checks, physical performance of a student in relation to his future specialization. Moreover, during the entire training period (3-4 years), it is possible to make adjustments to the plan of physical education with the student depending on his indicators and thereby contribute both to overall professional training and development of those individual qualities that will help to strengthen the physical potential of the graduate of the industry educational institution. Keywords: railway transport, college, physical performance, students, physical efficiency, fitness, medical groups, computer program, tests.Текст аннотации на англ. языке и полный текст статьи на англ. языке находится в прилагаемом файле ПДФ (англ. версия следует после русской версии). Статья посвящена исследованию физической работоспособности в железнодорожном колледже. Авторы предлагают написанную ими для этих целей компьютерную программу, новизна которой в том, что она определяет с помощью контрольных замеров и тестовой проверки физическую работоспособность студента применительно к его будущей специальности. Причём на протяжении всего времени обучения (3-4 года) есть возможность вносить коррективы в план физкультурной работы с учащимся в зависимости от его показателей и тем самым способствовать как общей профессиональной подготовке, так и развитию тех индивидуальных качеств, которые помогут укрепить физический потенциал выпускника отраслевого учебного заведения

Ultrashort filaments of light in weakly-ionized, optically-transparent media

Modern laser sources nowadays deliver ultrashort light pulses reaching few cycles in duration, high energies beyond the Joule level and peak powers exceeding several terawatt (TW). When such pulses propagate through optically-transparent media, they first self-focus in space and grow in intensity, until they generate a tenuous plasma by photo-ionization. For free electron densities and beam intensities below their breakdown limits, these pulses evolve as self-guided objects, resulting from successive equilibria between the Kerr focusing process, the chromatic dispersion of the medium, and the defocusing action of the electron plasma. Discovered one decade ago, this self-channeling mechanism reveals a new physics, widely extending the frontiers of nonlinear optics. Implications include long-distance propagation of TW beams in the atmosphere, supercontinuum emission, pulse shortening as well as high-order harmonic generation. This review presents the landmarks of the 10-odd-year progress in this field. Particular emphasis is laid to the theoretical modeling of the propagation equations, whose physical ingredients are discussed from numerical simulations. Differences between femtosecond pulses propagating in gaseous or condensed materials are underlined. Attention is also paid to the multifilamentation instability of broad, powerful beams, breaking up the energy distribution into small-scale cells along the optical path. The robustness of the resulting filaments in adverse weathers, their large conical emission exploited for multipollutant remote sensing, nonlinear spectroscopy, and the possibility to guide electric discharges in air are finally addressed on the basis of experimental results.Comment: 50 pages, 38 figure

The integrated exploration of Raifa lake sediments and dendrochronological analysis of Raifa forestry pines

© 2006-2017 Asian Research Publishing Network (ARPN). All rights reserved.The article presents some results of comprehensive research on the properties of Raifa lake bottom sediments and dendrochronological study of Raifa forestry pines (Russia). A preliminary seismic acoustic investigations have been carried out, which allowed to select the sampling site (E 48 ° 43'40.6" N 55 ° 54'21.7"). The length of core sample was 32 cm. Laboratory studies of the core, including the study of the elemental composition, magneto-mineralogical and carcinologic analysis revealed the features of sedimentation mass formation

Optic chiasm in the species of order Clupeiformes, family Clupeidae: Optic chiasm of Spratelloides gracilis shows an opposite laterality to that of Etrumeus teres

In most teleost fishes, the optic nerves decussate completely as they project to the mesencephalic region. Examination of the decussation pattern of 25 species from 11 different orders in Pisces revealed that each species shows a specific chiasmic type. In 11 species out of the 25, laterality of the chiasmic pattern was not determined; in half of the individuals examined, the left optic nerve ran dorsally to the right optic nerve, while in the other half, the right optic nerve was dorsal. In eight other species the optic nerves from both eyes branched into several bundles at the chiasmic point, and intercalated to form a complicated decussation pattern. In the present study we report our findings that Spratelloides gracilis, of the order Clupeiformes, family Clupeidae, shows a particular laterality of decussation: the left optic nerve ran dorsally to the right (n = 200/202). In contrast, Etrumeus teres, of the same order and family, had a strong preference of the opposite (complementary) chiasmic pattern to that of S. gracilis (n = 59/59), revealing that these two species display opposite left–right optic chiasm patterning. As far as we investigated, other species of Clupeiformes have not shown left–right preference in the decussation pattern. We conclude that the opposite laterality of the optic chiasms of these two closely related species, S. gracilis and E. teres, enables investigation of species-specific laterality in fishes of symmetric shapes