Idea jakuckiej religii i obchody święta Ysyach w Republice Sacha

Od początku istnienia Republiki Sacha podejmowano tam wiele działań na rzecz zachowania odrębności kulturowej Jakutów i wzmocnienia ich tożsamości. Służyć temu miały między innymi przyznanie tradycyjnemu świętu Ysyakh statusu święta narodowego oraz próby tworzenia na bazie dawnych wierzeń jakuckiej religii narodowej. Jej podstawą jest tzw. doktryna aĭyy, opracowana przez przedstawicieli jakuckiej inteligencji narodowej. W artykule pokazuję w jakich sferach funkcjonowania dzisiejszego Ysyakhu widoczne są jej wpływy oraz jakie jest znaczenie święta dla jej propagowania

Simple methods for identification of Radiative properties of highly-porous ceria ceramics in the range of semi-transparency

Purpose: The aim of this paper is to present advanced experimental-numerical methods for identification of spectral absorption and scattering properties of highly porous ceria ceramics in the range of semi-transparency at room and elevated temperatures. Design/methodology/approach: At room temperature, a period of quasi-steady oscillations of the sample surface temperature generated in response to recurrent laser heating at fixed values of the maximum and minimum temperature of the irradiated surface is measured along with the normal-hemispherical reflectance. Radiative properties are then identified using a combined heat transfer model. At elevated temperatures, an analytical solution proposed in an earlier study for zirconia ceramics is used to retrieve spectral absorption coefficient of ceria ceramics from the measured normal emittance. Findings and Originality/value: This method can be used to obtain small absorption coefficient of ceria ceramics at room temperature. The required measurements of both the normal-hemispherical reflectance and the period of quasi-steady oscillations of the irradiated surface temperature of the ceramics sample between fixed values of the maximum and minimum temperatures can be readily conducted using thermal laboratory equipment. Another method has been suggested for identification of the spectral absorption coefficient of ceria ceramics at elevated temperatures. This method is based on a relation between the measured normal emittance of an isothermal sample and the absorption coefficientThe financial support by the Russian Foundation for Basic Research, grant no. 16-08-00157-a (L.A. Dombrovsky), and by the Australian Research Council, grant no. FT140101213 (W. Lipiński), is gratefully acknowledged

Numerical modelling of radiative heat transfer in a polydispersion of ceramic particles under direct high-flux solar irradiation

The effects of polydispersity on radiative and interfacial convective heat transfer are investigated in particle–gas two-phase media for solar particle receiver applications. Non-grey radiative transfer is analysed using the collision-based Monte Carlo ray-tracing method. The Mie theory is employed to calculate radiative properties of particles. The finite volume method and the explicit Euler time integration scheme are used to solve the transient energy equations for the particle and gas phases. Three alternative approaches to modelling particle properties and thermal conditions are employed: (i) a novel discrete size model, in which particle groups within discrete size intervals are assigned individual properties and temperatures locally; (ii) a lumped size model, in which integral properties and a single temperature are assigned to the particle phase locally; and (iii) a monodisperse size model, in which properties are evaluated for the Sauter mean diameter of the polydispersion and a single temperature is assigned to the particle phase locally. Strongly size-dependent radiation absorption and interfacial convective heat transfer are predicted with the discrete size model for alumina particles. Particles smaller than 27.4μm located near the aperture absorb the solar irradiation and transfer heat to the gas phase most effectively. The angular spread of the incident solar radiation is found to have a negligible effect on the overall absorption, although the most uniform thermal conditions occur for the solar irradiation with the smallest confinement angle. The overall absorptance of alumina particles is higher by 3.4% and 2.7% than that of iron (III) oxide and mullite particles, respectively. The lumped and monodisperse size models allow for reduction of the computational time at the expense of lower accuracy and limited information about particle properties and thermal conditions. © 2021 The Author(s

Mottled mice and non-mammalian models of Menkes disease

Menkes disease is a multi-systemic copper metabolism disorder caused by mutations in the X-linked ATP7A gene and characterised by progressive neurodegeneration and severe connective tissue defects. The ATP7A protein is a Copper (Cu)-transporting ATPase expressed in all tissues and plays a critical role in the maintenance of copper homeostasis in cells of the whole body. ATP7A participates in copper absorption in the small intestine and in copper transport to the CNS across the blood-brain-barrier and blood–cerebrospinal fluid- barrier. Cu is essential for synaptogenesis and axonal development. In cells, ATP7A participates in the incorporation of copper into Cu-dependent enzymes during the course of its maturation in the secretory pathway. There is a high degree of homology (>80% ) between the human ATP7A and murine Atp7a genes. Mice with mutations in the Atp7a gene, called mottled mutants, are well-established and excellent models of Menkes disease. Mottled mutants closely recapitulate the Menkes phenotype and are invaluable for studying Cu-metabolism. They provide useful models for exploring and testing new forms of therapy in Menkes disease. Recently, non-mammalian models of Menkes disease, Drosophila melanogaster and Danio rerio mutants were used in experiments which would be technically difficult to carry out in mammals

Steroid-eluting epicardial pacing in children

Background: Permanent cardiac pacing is a known method of the treatment for children with bradycardia. Epicardial pacing is required in small children and in children with some congenital heart defects. Steroid-eluting leads (SEL) have been introduced to reduce implant site fibrosis and to retain permanent low-pacing threshold values. Our aim was to evaluate the pacing characteristic and follow-up of children with epicardial steroid-eluting pacing. Material and methods: We implanted steroid-eluting epicardial pacing systems in 53 children (age at implantation: 2 days - 17.5 years, mean 4.6 years), of whom 37 (70%) had congenital heart disease and 22 of these had already had cardiac surgery. These children formed group I. Group II was the control group and consisted of 29 children (age at implantation: 10 days - 13 years, mean 6.5 years) with non-steroid epicardial pacing systems; 15 patients (51%) had diagnosed congenital heart disease, and 9 of these had had cardiac surgery. The pacing threshold (PT) was obtained during implantation, before discharge, 1-3 and 6 months following implantation and then every 6 months. Results: In group I the mean PT during the implantation procedure was 1.6 V/0.4 ms and decreased significantly before discharge. In group II the PT during the implantation procedure was low and increased before discharge. In group I the PT of the atrial leads was low and stable during the follow-up period, while the PT of the ventricular leads slowly increased and four years following implantation was similar to that of group II. Conclusions: In children with permanent epicardial pacing the ventricular PT was significantly lower when steroid-eluting leads had been used then when these had not been used, but during the follow-up period the PT slowly increased, while the pacing threshold of atrial steroid-eluting leads remained stable

The effect of decomposing biomass of the grasses Festuca arundinacea, F. ovina, and F. rubra on the species composition and quality of lawns

The aim of the study was to assess the impact of cut vegetative shoots of chosen lawn grass cultivars of Festuca being left on the lawn sward surface on the species composition, sodding and appearance, and over-wintering of the lawn. The influence of decomposing biomass was studied in a field experiment between 2008 and 2014. Each cultivar was sown as a monoculture on microplots with an area of 1 m2. The control consisted of sites from which the cut sward had been removed immediately after cutting. The results obtained may indicate an allelopathic effect of the cut sward of the cultivars left on the lawn surfaces. The following had the most negative effects on the species composition of the lawn sward (from greater to lesser negative impact): F. ovina ‘Espro’, F. rubra ‘Areta’, F. arundinacea ‘Asterix’, and F. ovina ‘Pintor’. With the exception of ‘Espro’, these cultivars also limited the presence of dicotyledonous plants in the lawn sward. The cover of dicotyledonous plants and other unsown grasses was also recorded on the sites with F. rubra ‘Olivia’ and ‘Nimba’. The greatest negative influence on the sodding of the lawn swards was demonstrated by the latter F. rubra cultivar, whereas F. ovina ‘Espro’ had the greatest negative influence on the appearance. However, no differences were found in assessments of over-wintering of the cultivars at the study sites. However, taking into account the scale of these impacts on the characteristics evaluated, the cultivars of Festuca species tested can be recommended for extensive use, where a cut sward can be left on the surface of the lawn. Some caution in this respect is recommended when it comes to F. ovina ‘Espro’ and F. rubra ‘Areta’ and ‘Nimba’

Transient Elastography for Detection of Liver Fibrosis in Children With Autosomal Recessive Polycystic Kidney Disease

Introduction: Congenital hepatic fibrosis (CHF) is invariably present in all patients with autosomal recessive polycystic kidney disease (ARPKD) but is usually clinically asymptomatic. The portal hypertension in the course of CHF develops and progresses over time, so an early detection of liver fibrosis remains crucial.Aim: The aim of the study was to evaluate a predictive value of transient elastography for evaluating liver disease progress in pediatric ARPKD patients.Material and Methods: The study group encompassed 21 pediatric patients with ARPKD and 20 healthy children (control group) from The Children's Memorial Health Institute in Warsaw, Poland. Liver fibrosis was determined by assessing the liver stiffness (LS) with transient elastography (FibroScan®, FS) using size-appropriate probes. In ARPKD group the laboratory findings, results of an abdominal ultrasound examination, and an endoscopic gastroduodenoscopy were also analyzed.Results: Compared with healthy controls, patients with ARPKD had significantly increased median LS values (22 vs. 4.25 kPa, p < 0.0001). Based on FS results, ARPKD group was divided into two subgroups: patients (n = 5) with LS results suggestive of no fibrosis or minimal fibrosis (LS < 6.9 kPa, METAVIR fibrosis stage 0–1) and patients (n = 16) with LS results suggestive of at least significant liver fibrosis (LS ≥ 6.9 kPa, METAVIR fibrosis stage 2–4). In the first subgroup (no fibrosis or minimal fibrosis), all patients had no signs of portal hypertension. In the subgroup with at least significant liver fibrosis, splenomegaly was observed in 87.5% of patients and thrombocytopenia in 69% of patients. An endoscopic gastroduodenoscopy was performed in 15 out of 21 ARPKD patients, nine patients (60%) had esophageal varices. All of these patients had LS results suggestive of at least significant liver fibrosis.Conclusions: TE by FibroScan can be used as an additional method for evaluating liver disease progress in pediatric ARPKD patients

Elektrody nasierdziowe uwalniające sterydy w stałej stymulacji serca u dzieci

Wstęp: Stała stymulacja serca jest uznaną metodą leczenia bradyarytmii u dzieci. U małych dzieci i w niektórych patologiach układu krążenia konieczne jest zastosowanie elektrod nasierdziowych. Przyjmuje się, że sterydy uwalniane na końcu elektrod nasierdziowych, zmniejszając miejscowe włóknienie, przyczyniają się do utrzymania niskiego progu stymulacji serca. Celem pracy była ocena przebiegu stymulacji serca u dzieci z EP z elektrodami uwalniającymi sterydy. Materiał i metody: Pierwszą badaną grupę stanowiło 53 dzieci z EP z elektrodami uwalniającymi sterydy, w wieku od 2 doby życia do 17,5 roku (śr. 4,6 roku) w momencie zabiegu. U 37 (70%) z nich rozpoznano wrodzoną wadę serca, 22 przebyło operację serca. Do grupy II - kontrolnej zakwalifikowano 29 dzieci z EP niesterydowymi wszczepionymi w wieku od 10 doby życia do 13 lat (śr. 6,5 roku). U 15 (51%) rozpoznano wrodzoną wadę serca, 9 leczono kardiochirurgicznie. Przebieg stymulacji analizowano retro- i prospektywnie. Progi stymulacji (PT) mierzono w czasie wszczepienia EP, przy wypisie, po 1–3 oraz po 6 miesiącach od zabiegu, a następnie co pół roku. Wyniki: U pacjentów z I grupy PT podczas wszczepienia EP wynosił średnio 1,6 V/0,4 ms i istotnie obniżał się już w pierwszych dniach po zabiegu, natomiast u pacjentów z grupy II początkowo niski PT, później narastał. W okresie obserwacji PT elektrod przedsionkowych uwalniających sterydy były stabilne, a komorowych powoli wzrastały i po 4 latach od zabiegu były porównywalne z PT elektrod niesterydowych. Wnioski: U dzieci z EP progi stymulacji komorowej są istotnie niższe po zastosowaniu elektrod uwalniających sterydy w porównaniu z elektrodami niesterydowymi, jednak powoli się podnoszą

Absolute measurement of the ^{1}S_{0} − ^{3}P_{0} clock transition in neutral ^{88}Sr over the 330 km-long stabilized fibre optic link

We report a stability below $7\times 10{}^{-17}$ of two independent optical lattice clocks operating with bosonic ${}^{88}$Sr isotope. The value (429228066418008.3(1.9)${}_{syst}$(0.9)${}_{stat}$~Hz) of the absolute frequency of the ${}^{1}S_{0}$ - ${}^{3}P_{0}$ transition was measured with an optical frequency comb referenced to the local representation of the UTC by the 330 km-long stabilized fibre optical link. The result was verified by series of measurements on two independent optical lattice clocks and agrees with recommendation of Bureau International des Poids et Mesures