bluuebunny/crossref_metadata_2025_split huggingface dataset:
Initiation of Haploidentical Stem Cell Transplantation With Post‐Transplant Cyclophosphamide in Children: A Low–Middle‐Income Country Institutional Experience
Authors: Bukhari Syed Ibrahim , Saeed Javeria , Fadoo Zehra , Belgaumi Asim Fakhruddin , Allani Naureen , Altaf Sadaf 
Published: August 2024

ABSTRACTBackgroundHaploidentical hematopoietic stem cell transplant (HSCT) is a curative treatment especially for countries where bone marrow registries are nonexistent. We present our experience with haploidentical HSCT in pediatric patients.MethodsRetrospective data collected and analyzed for patients ≤18 years, from January 2017 to December 2022.ResultsThe cohort consisted of 20 patients with median age at transplant of 61.5 (IQR: 124) months. Fourteen (70%) were malignant and 6 (30%) were benign diseases. Donors were father in majority (9/20; 45%). Stem cell source was peripheral blood 8, marrow 8, and combined 4. c‐specific antibodies were positive in 6 (30%). Median CD34 cell dose infused: 9.35 × 106/kg. Median engraftment time: 15 (IQR: 17) days. Acute and chronic graft‐versus‐host disease (GVHD) occurred in 12/20 (60%) and 5/20 (25%), respectively. Complications included infection/sepsis (14/20; 70%), cytomegalovirus reactivation (14/20; 70%), sinusoidal obstruction syndrome (1/20; 5%), primary graft failure (PGF) (6/20; 30%), and secondary graft failure (4/20; 20%). PGF was more common in benign conditions (p = 0.003) and less prevalent in cases with aGVHD (p = 0.007). aGVHD was more common in malignant conditions (p = 0.007). Overall survival (OS), relapse‐free survival (RFS), and treatment‐related mortality (TRM) were 40%, 50%, and 35%, respectively. Median time of survival and relapse were 8 (IQR: 15) and 9 (IQR: 13) months, respectively.ConclusionOS was comparable to that of other low–middle‐income countries. GVHD was a major challenge, along with sepsis and CMV infection. Half of the leukemias relapsed. Graft failure was a major concern in nonmalignant diseases.
Long‐Term Amino Acid Homeostasis, Neurodevelopmental and Growth Profiles Following Liver Transplantation in Maple Syrup Urine Disease
Authors: Vasudevan Anu K. , Shanmugam Naresh , Rammohan Ashwin , Balakrishnan Umamaheswari , Menon Jagadeesh , Iqbal Mohammed Asrar Ul Huq , Mohammed Gehan Mohammed Osman , Kaliamoorthy Ilankumaran , Rela Mohamed 
Published: August 2024

ABSTRACTIntroductionMaple syrup urine disease (MSUD) is caused by the deficiency of branched‐chain keto acid dehydrogenase (BCKAD) and, it is well described that BCKAD contributed by an allograft following liver transplantation (LT) phenotypically normalizes this inborn error of metabolism (IEM). There is, however, a paucity of data especially with regards to the neurodevelopmental aspects and catch‐up growth profiles after LT in a resource‐challenged setting. We present our series of children under 6 years of age who underwent LT for MSUD particularly focusing on their amino acid homeostasis, neurodevelopmental and somatic growth profiles.MethodsOf 580 consecutive pediatric LT (PLT) performed between January 2011 and December 2022, all children who underwent LT for MSUD were included for analysis. Data accrued included peri‐LT details, pre‐ and post‐LT metabolic profile, neurodevelopmental assessment, somatic growth evaluation, and long‐term outcomes.ResultsSix children underwent LT for MSUD with a median age and weight at LT of 20.5 (IQR: 8–60) months and 10.1 (IQR: 6.7–15.8) kg, respectively. One explanted liver was used as a domino graft for Arginase deficiency. Median follow‐up period was 52.5 (IQR: 27–94) months. None had vascular or biliary complications. Following LT, all children were started on an unrestricted protein diet and had normalization of BCAA levels. Post‐LT height and weight improved by 1 SD but did not achieve the normal profile. None of the children had neuro‐deterioration and have achieved new milestones.ConclusionThis is the first‐report presenting the growth aspects, amino acid and neurodevelopmental profiles of children who underwent LT for MSUD within the socio‐economic‐cultural idiosyncrasies and constraints prevalent in our part of the world.
Navigating financial cycles: Economic growth, bureaucratic autonomy, and regulatory governance in emerging markets
Authors: Coban M. Kerem , Apaydin Fulya 
Published: August 2024

AbstractPolitical decisions over economic growth policies influence the degree of bureaucratic autonomy and regulatory governance dynamics. Yet, our understanding of these processes in the Global South is somewhat limited. The article studies the post‐Global Financial Crisis period and relies on elite interviews and secondary sources from Turkey. It problematizes how an economic growth model dependent on foreign capital inflows, which are contingent on global financial cycles, influences the trajectory of bureaucratic autonomy. Specifically, we argue that dependence on foreign capital flows for economic growth creates an unstable macroeconomic policy environment: while the expansionary episode of the global financial cycle masks conflicts between the incumbent and bureaucracy, the contractionary episode threatens the political survival of the incumbent. In the case of Turkey, this has incentivized the ruling coalition to resort to executive aggrandizement to control monetary policy and banking regulation, which resulted in a dramatic decay of the autonomy of the regulatory agencies since 2013.
Does it matter how we measure poverty? Correlates of income and multidimensional poverty in Sri Lanka
Authors: Deyshappriya N. P. Ravindra , Feeny Simon 
Published: August 2024

AbstractWhile it is widely accepted that poverty is multidimensional, debates over how it is best measured continue. This paper examines whether different measurements of poverty provide different implications from a policy perspective. Using probit models applied to household level data it examines the correlates of both income poverty and multidimensional poverty in the context of Sri Lanka. Results suggest that the two measures of poverty share many of the same correlates. Age of the household head, education, marital status, employment, access to agricultural land and the receipt of remittances are important across the two poverty measures. However, there are important differences with respect to the signs of the coefficients on household size and ethnicity variables. Further, the magnitude of the associations of independent variables with poverty vary considerably across the two measures. Policy recommendations are provided.
Effect of post‐discharge online kangaroo care training on breastfeeding self‐efficacy in mothers with preterm infants: A randomised controlled study
Authors: Kaynak Serap , Ergün Sibel 
Published: August 2024

AbstractBackgroundPreterm newborns face many health problems due to their incomplete intrauterine development and the immaturity of their systems. One of these problems concerns nutrition. This study aimed to determine the effect of online kangaroo care (KC) training provided post‐discharge on breastfeeding self‐efficacy in mothers with preterm infants.MethodsThis research was conducted as an experimental study with a randomised control group. Two groups were formed: kangaroo care and control. The sample of the study consisted of 68 mothers of preterm infants. The mothers in the KC group were given online KC training after discharge from the hospital. The mothers were asked to perform KC regularly, at least once a day, for 20 min, 7 days a week. The Breastfeeding Self‐Efficacy Scale was administered to the mothers before and after training.ResultsThe results of the study revealed that the difference between the pre‐test and post‐test breastfeeding self‐efficacy scores was statistically significantly higher among the mothers who received online KC training compared with the controls (p < 0.001).ConclusionAccording to the results of our research carried out in this context, the mothers who received online KC training had increased breastfeeding self‐efficacy compared to those in the control group.
NrCAM activates the NF‐κB signalling pathway by competitively binding to SUMO‐1 and promotes Th17 cell differentiation in Graves' disease
Authors: Huang Fengjiao , Zhang Lijuan , Zhou Yingying , Zhao Shuiying , Wang Jiao 
Published: August 2024

AbstractThis study aimed to explore the molecular mechanism of neuronal cell adhesion molecule (NrCAM) by regulating Th17 cell differentiation in the pathogenesis of Graves' disease (GD). Naïve CD4+ T cells were isolated from peripheral blood mononuclear cells of GD patients and healthy control (HC) subjects. During the differentiation of CD4+ T cells into Th17 cells, NrCAM level in GD group was improved. Interference with NrCAM in CD4+ T cells of GD patients decreased the percentage of Th17 cells. NrCAM overexpression in CD4+ T cells of HC subjects increased the percentage of Th17 cells and upregulated p‐IκBα, p50, p65, c‐Rel protein expressions, and NF‐κB inhibitor BAY11‐7082 partially reversed NrCAM effect. NrCAM overexpression promoted the degradation of IκBα, and overexpression of small ubiquitin‐related modifier 1 (SUMO‐1) inhibited IκBα degradation. NrCAM overexpression reduced IκBα binding to SUMO‐1. During Th17 cell differentiation in HC group, NrCAM overexpression increased IL‐21 levels and secretion, and IL‐21 neutralizing antibody reversed this effect. IL‐21 level was decreased after p65 interference in CD4+ T cells of HC subjects. p65 interacts with IL‐21 promoter region. In conclusion, NrCAM binds to SUMO‐1 and increases phosphorylation of IκBα, leading to activation of NF‐κB pathway, which promotes Th17 cell differentiation.
Muscle memory in humans: evidence for myonuclear permanence and long‐term transcriptional regulation after strength training
Authors: Cumming Kristoffer Toldnes , Reitzner Stefan Markus , Hanslien Marit , Skilnand Kenneth , Seynnes Olivier R. , Horwath Oscar , Psilander Niklas , Sundberg Carl Johan , Raastad Truls 
Published: August 2024

AbstractThe objective of this work was to investigate myonuclear permanence and transcriptional regulation as mechanisms for cellular muscle memory after strength training in humans. Twelve untrained men and women performed 10 weeks of unilateral elbow‐flexor strength training followed by 16 weeks of de‐training. Thereafter, 10 weeks’ re‐training was conducted with both arms: the previously trained arm and the contralateral untrained control arm. Muscle biopsies were taken from the trained arm before and after both training periods and from the control arm before and after re‐training. Muscle biopsies were analysed for fibre cross‐sectional area (fCSA), myonuclei and global transcriptomics (RNA sequencing). During the first training period, myonuclei increased in type 1 (13 ± 17%) and type 2 (33 ± 23%) fibres together with a 30 ± 43% non‐significant increase in mixed fibre fCSA (P = 0.069). Following de‐training, fCSA decreased in both fibre types, whereas myonuclei were maintained, resulting in 33% higher myonuclear number in previously trained vs. control muscle in type 2 fibres. Furthermore, in the previously trained muscle, three differentially expressed genes (DEGs; EGR1, MYL5 and COL1A1) were observed. Following re‐training, the previously trained muscle showed larger type 2 fCSA compared to the control (P = 0.035). However, delta change in type 2 fCSA was not different between muscles. Gene expression was more dramatically changed in the control arm (1338 DEGs) than in the previously trained arm (822 DEGs). The sustained higher number of myonuclei in the previously trained muscle confirms myonuclear accretion and permanence in humans. Nevertheless, because of the unclear effect on the subsequent hypertrophy with re‐training, the physiological benefit remains to be determined.
imageKey points
Muscle memory is a cellular mechanism that describes the capacity of skeletal muscle fibres to respond differently to training stimuli if the stimuli have been previously encountered.
This study overcomes past methodological limitations related to the choice of muscles and analytical procedures.
We show that myonuclear number is increased after strength training and maintained during de‐training.
Increased myonuclear number and differentially expressed genes related to muscle performance and development in the previously trained muscle did not translate into a clearly superior responses during re‐training. Because of the unclear effect on the subsequent hypertrophy and muscle strength gain with re‐training, the physiological benefit remains to be determined.
Investigating the Impact of Steam Enhancement on Combustion in a Swirl-Assisted Jet-Stabilized Gas Turbine Combustor
Authors: Izadi Saeed , Kislat Oliver , Zanger Jan , Seliger-Ost Hannah , Kutne Peter , Aigner Manfred 
Published: August 2024

Abstract
A newly developed gas turbine combustor system based on the swirl-assisted jet-stabilized concept using Jet A-1 and natural gas as reference fuel is tested under wet conditions to evaluate its combustion characteristics in the presence of steam. The effect of steam injection into the gas turbine combustor under both spray and superheated liquid fuel injection conditions is studied experimentally on an atmospheric test rig. The experiments are conducted at atmospheric pressure and an elevated combustion air temperature of 305 °C. To evaluate the effect of steam injection on combustion performance, the water-to-gas ratio (WGR) is varied from 0% to 32%. Even at very high WGR levels, the results show virtually no combustion thermoacoustic instability during operation. With increasing WGR = 0–16% and at stoichiometric condition, NOx reductions of −82% to −100% were observed during Jet A-1 and natural gas combustion, respectively. It is shown that the reduction of the combustion zone temperature due to the steam acting as a heat sink is the main cause of the NOx decrease. For both wet and dry conditions, CO levels remained fairly similar. Both flame length and flame height above the burner increased with increasing WGR. This is due to the reduced reactivity of the fuel–air mixture. The operating range of the burner remained fairly constant for Jet A-1 until WGR = 20% while it decreased significantly with increasing WGR for natural gas combustion. While the effect of the WGR on CO was modest, the greatest effect of the WGR was on the heat release zone intensity at a constant air to fuel ratio. In reducing the NOx levels of Jet A-1 and natural gas combustion, both thermal and chemical effects of steam injection were observed. However, steam acting as a heat sink and lowering the flame temperature, thereby reducing the thermal NO formation rate, was the dominant factor in NOx reduction.
Development of Detailed and Reduced Chemical Kinetic Models for Ammonia Gas Turbines
Authors: Rahman Ramees K. , Pierro Michael , Winquist Mark , Subbaraman Ganesan , Sun Wenting , Steele Robert , Vega John , Vasu Subith S. 
Published: August 2024

Abstract
The power generation sector has been recently moving toward decarbonization, and there is an increased interest in replacing conventional fossil fuels with fuels that produce reduced/zero carbon emissions. One such fuel is ammonia (NH3). However, ammonia is hard to ignite, has a low flame speed, and produces a significantly large amount of nitrogen oxide (NOx) emissions. Hence, using 100% ammonia as fuel in gas turbines requires significant modifications and the development of novel combustors. Blending hydrogen with ammonia, however, helps in having better control over the combustion properties. For example, a 70%/30% mixture of NH3/H2 mixture has a flame speed comparable to natural gas. Before utilizing hydrogen-blended ammonia in an actual gas turbine combustor, thorough simulation studies are required to evaluate its performance, possible hazards, and emissions. The literature lacks well-validated chemical kinetic models for the combustion of hydrogen-blended ammonia for undiluted mixtures at gas turbine-relevant conditions (∼20 bar). Most models available in the literature have been developed for ammonia extremely diluted in diluents such as argon or nitrogen. Hence, in this work, we develop a detailed chemical kinetic model for hydrogen-blended ammonia combustion and validate it with a wide range of experimental data for both dilute and undiluted mixtures relevant to gas turbine operating conditions. We outline the strengths and weaknesses of the current mechanism to aid future users of our chemical kinetic mechanism. The detailed chemical kinetic mechanism was reduced to a smaller version (32 species mechanism) without significant loss in accuracy using the directed relation graph with error propagation (DRGEP) and full species sensitivity analysis. The resultant mechanism can predict a wide range of experimental results with the least cumulative error and will be a valuable tool in computational fluid dynamics (CFD) simulations that will enable the development of gas turbines for zero-carbon power generation.
Impact of Diluents on Flame Stability With Blends of Natural Gas and Hydrogen
Authors: Rodriguez Camacho Javier , Le Dang , Blust James , O'Connor Jacqueline 
Published: August 2024

Abstract
Two potential decarbonization pathways for natural gas (NG)-fueled gas turbine engines include blending hydrogen (H2) into NG and postcombustion carbon capture. H2 blending changes several combustion properties, including flame speed and stretch sensitivity. The use of post-combustion carbon capture systems is typically facilitated by the implementation of exhaust gas recirculation (EGR), where exhaust gases are injected into the inlet of the engine, increasing carbon dioxide (CO2) concentration at the outlet and, hence, increasing the efficiency of carbon capture technologies. In this work, we explore the impact of H2 blending and EGR on the stability of a swirl-stabilized, central-piloted flame. Mixtures of NG and H2 are tested at a range of different diluent compositions, with oxygen varied from 21% to 15% by volume in the oxidizer. In all cases, a constant adiabatic flame temperature is maintained to mimic the operation of a gas turbine at a given turbine inlet temperature. A variable-length combustor is used for testing, where combustor length is varied to understand the dynamic stability characteristics of the system. Results show that EGR and H2 work in opposition to each other, where higher levels of EGR result in poor flame holding and higher levels of H2 result in better flame holding. Increasing H2 generally increases the amplitude of thermoacoustic instability at each condition, a result of the change in flame position in this particular combustor. Importantly, H2 can be added to NG to improve flame holding without significantly decreasing CO2 levels in the products, showing that H2 blending can be a method for counteracting combustor operability issues that arise from high levels of EGR necessary to improve the efficiency of typical carbon capture systems.
Axial Flow Fan Performance in a Forced Draught Air-Cooled Heat Exchanger for a Supercritical Carbon Dioxide Brayton Cycle
Authors: Boshoff Francois D. , van der Spuy Sybrand J. , Pretorius Johannes P. 
Published: August 2024

Abstract
An axial flow cooling fan has been designed for use in a concentrated solar power plant. The plant is based on a supercritical carbon dioxide (sCO2) Brayton cycle, and uses a forced draft air-cooled heat exchanger (ACHE) for cooling. The fan performance has been investigated using both computational fluid dynamics (CFD) and scaled fan tests. This paper presents a CFD model that integrates the fan with the heat exchanger. The objective is to establish a foundation for similar models and to contribute to the development of efficient ACHE units designed for sCO2 power cycles. The finned-tube bundle is simplified, with a Porous Media Model representing the pressure drop through the bundle. Pressure inlet and -outlet boundary conditions are used, meaning the air flowrate is solved based on the fan and tube bundle interaction. The flowrate predicted by the CFD model is 0.5% higher than the analytical prediction, and 3.6% lower than the design value, demonstrating that the assumptions used in the design procedure are reasonable. The plenum height is also found to affect the flowrate, with shorter plenums resulting in higher flow rates and fan efficiencies, and longer plenums resulting in more uniform cooling air flow.
Laboratory Tests in Cyber-Physical Mode for an Energy Management System Including Renewable Sources and Industrial Symbiosis
Authors: Ferrari Mario L. , Gini Lorenzo , Pascenti Matteo 
Published: August 2024

Abstract
The aim of this paper regards the laboratory validation of an energy management system (EMS) for an industrial site on the Eigerøy island (Norway). It will be the demonstration district in the ROBINSON project for a consequent concept replication. This activity in cyber-physical mode is an innovative approach to finalize the EMS tool with real measurement data with prime movers available at laboratory level, considering the necessary EMS robustness and flexibility for replication on other industrial islands. This EMS was designed and developed to minimize variable costs, producing on/off and set-point signals that, through a model predictive control (MPC) software, establish the system status. This smart grid includes renewable sources (e.g., solar panels, a wind turbine, and syngas) and traditional prime movers, such as a steam boiler for the industry needs. Moreover, an energy storage device is installed composed of an electrolyzer with a hydrogen pressure vessel. The main results reported in this work regard 26-h tests performed in cyber-physical mode thanks to the real-time interaction of hardware and software. So, a real microturbine and real photovoltaic (PV) panels were managed by the EMS in conjunction with software models for components not physically present in the laboratory. Although the optimization target was cost minimization, significant improvement was also obtained in terms of efficiency increase and CO2 emission decrease.
Numerical Analysis and Design of New Exhaust Section Downstream of Constant Volume Combustor
Authors: Gallis Panagiotis , Misul Daniela Anna , Boust Bastien , Bellenoue Marc , Salvadori Simone 
Published: August 2024

Abstract
Pressure gain combustors (PGC) exploit either their isochoric or detonative combustion increasing the theoretical thermal efficiency of a gas turbine cycle. On this basis, a constant-volume combustor (CVC) is developed operating with rotary valves while the chamber is fed with mixture of air and liquid iso-octane. This work describes the numerical design of a new exhaust section after the CVC. First, the design parametrization of the transition duct and the resulting design of experiments (DOE) of 81 samples are introduced. Every case is numerically tested and the election of the best sample is based on the pressure losses and the oscillations characterization. In the last part, the LS89-VKI vane is added at the aft part of the best transition duct and the ensemble exhaust system is analyzed with the help of transient CFD analysis. Every component is evaluated in terms of pressure losses and oscillations, while the operation of the vane is investigated in details. The results of the stator's performance are discussed and compared with steady experimental data. During a CVC period, the cycle average outlet flow angle remains close to the outlet metal angle denoting promising results for the future numerical analysis of the subsequent rotor.