Kabuki syndrome (KS) is commonly caused by mutations in the histone-modifying enzyme lysine methyltransferase 2D (KMT2D). Immune dysfunction is frequently observed in individuals with KS, but the ...role of KMT2D in immune system function has not been identified.
We sought to understand the mechanisms driving KS-associated immune deficiency (hypogammaglobulinemia low IgA, splenomegaly, and diminished immunization responses).
We performed a comprehensive evaluation of humoral immunity and secondary lymphoid tissues in an established KS (Kmt2d+/βGeo) mouse model and validated select findings in a patient with KS.
Compared with wild-type littermates, Kmt2d+/βGeo mice demonstrated deficiencies in multiple B-cell lineages and reduced serum IgA and elevated IgM levels across multiple ages. The bone marrow, spleen, and intestine of Kmt2d+/βGeo mice contained diminished numbers of IgA-secreting cells, while elevated germinal center B cells were found in the mesenteric lymph node and Peyer patches. Kmt2d+/βGeo mice have decreased size and numbers of Peyer patches, a finding confirmed in human samples. We identified deficiency of Itgb7 RNA and protein expression, a gene encoding an adhesion protein that mediates intestinal homing, and we demonstrated KMT2D-dependent control of ITGB7 expression in a human cell line.
Kmt2d haploinsufficiency has broad deleterious effects on B-cell differentiation, specifically hampering gut lymphocyte homing and IgA+ plasma cell differentiation. Intestinal lymphoid defects caused by ITGB7 deficiency have not previously been recognized in KS, and these results provide new mechanistic insights into the pathogenesis of KS-associated immune deficiency.
Although oral food challenge (OFC) is an important clinical procedure for diagnosing food allergy, there is a paucity of literature on the outcome of the procedure and specifically the patients on ...whom the procedure is performed from the aspects of their age, sex, race/ethnicity, health insurance status, and serum specific IgE to the food tested.
We aimed to review results of OFC and determine the impact of patient age, sex, race/ethnicity, insurance status, private or public, and food specific serum IgE on the outcome of OFC.
A retrospective chart review was performed of patients undergoing OFCs at a children's hospital outpatient allergy clinic over a two-year period. The outcome of OFC was allergic or non-allergic based on determination and documentation by the treating physician. A logistic regression model was built to determine the association between the OFC outcomes, age, and symptoms at the time of OFC. A Chi-square analysis was performed to check for any significant relationship between the OFC outcome and age when stratified by insurance status.
Five hundred and eight children underwent 641 OFCs. Twenty nine percent of OFCs had an allergic outcome with the most commonly challenged foods being peanuts, eggs, and milk. Patient age and gender, when stratified by insurance status, did not have a significant effect on OFC outcomes. Serum IgE to peanuts and egg was significantly different between allergic OFC and non-allergic outcome. Vomiting and urticaria/angioedema correlated with an allergic OFC outcome.
OFCs confirm the food allergy diagnosis in about one-third of patients tested, and they should continue to be used when possible for an accurate diagnosis. Age, sex, and insurance status do not have a significant association with the outcome of OFC and cannot be added as predictive factors.
The electrolyte is one of the most important components of the vanadium redox flow battery and its properties will affect cell performance and behavior in addition to the overall battery cost. ...Vanadium exists in several oxidation states with significantly different half‐cell potentials that can produce practical cell voltages. It is thus possible to use the same element in both half‐cells and thereby eliminate problems of cross‐contamination inherent in all other flow battery chemistries. Electrolyte properties vary with supporting electrolyte composition, state‐of‐charge, and temperature and this will impact on the characteristics, behavior, and performance of the vanadium battery in practical applications. This Review provides a broad overview of the physical properties and characteristics of the vanadium battery electrolyte under different conditions, together with a description of some of the processing methods that have been developed to produce vanadium electrolytes for vanadium redox flow battery applications.
How do your electrons flow? The properties of the vanadium redox flow battery electrolyte vary with supporting electrolyte composition, state‐of‐charge, and temperature; these all have an impact on the characteristics, behavior, and performance of the battery in practical applications. This Review provides a broad overview of the physical properties and characteristics of the vanadium electrolyte under different conditions.
Pesticides are one of the main organic pollutants as they are highly toxic and extensively used worldwide. The reclamation of wastewater containing pesticides is of utmost importance. For this ...purpose, GO-doped metal ferrites (GO-Fe
O
and GO-CoFe
O
) were prepared and characterized using scanning electron microscopy, X-ray diffraction and Fourier transform infrared spectroscopic techniques. Photocatalytic potentials of catalysts were investigated against acetamiprid's degradation. A detailed review of the parametric study revealed that efficiency of overall Fenton's process relies on the combined effects of contributing factors, i.e., pH, initial oxidant concentration, catalyst dose, contact time, and acetamiprid load. ~97 and ~90% degradation of the acetamiprid was achieved by GO-CoFe
O
and GO-Fe
O
, respectively during the first hour under UV radiations at optimized reaction conditions. At optimized conditions (i.e., pH:3, H
O
: 14.5 mM (for Fe
O
, GO-Fe
O
and GO-CoFe
O
) and 21.75 mM (for CoFe
O
), catalysts: 100 mgL
, time: 60min) the catalysts exhibited excellent performance, with high degradation rate, magnetic power, easy recovery at the end, and efficient reusability (up to 5 cycles without any considerable loss in catalytic activity). A high magnetic character offers its easy separation from aqueous systems using an external magnet. Moreover, the combined effects of experimental variables were assessed simultaneously and justified using response surface methodology (RSM).
Several inorganic additives are investigated as potential precipitation inhibitors for the positive half‐cell electrolyte of the vanadium redox flow battery (VRB) at elevated temperatures. ...Electrolyte stability tests on the more concentrated 2 m vanadium electrolyte show that of the additives tested, the best results are obtained with the addition of 1 % phosphoric acid that give induction times of 40, 22 and 18 days for 80, 90 and 95 % state of charge (SOC) solutions at 50 °C, compared with 5, 2 and 1 days respectively for the corresponding blank solution. Several additives are also evaluated at 45 °C in a vanadium redox flow cell operating with a 2 m vanadium electrolyte. In the case of the cell employing the blank solution, a sudden drop in capacity, followed by pump failure is observed after around 70 cycles, while the cells with the additives continue to cycle without failure for 120 and 150 cycles for ammonium sulphate and ammonium phosphate additives respectively. A similar stabilising effect has previously been observed with these additives at low temperatures in the negative half‐cell electrolyte of the VRB, confirming that these stabilising agents have the potential to extend the upper and lower operating temperature limits of a 2 m vanadium electrolyte in the VRB to 45 and 5 °C respectively, while also significantly enhancing its energy density without the need for potentially hazardous chloride additions.
That little bit extra: Precipitation inhibitors are evaluated in a vanadium redox flow cell at 45.0 °C. The cell capacity without additives drops dramatically after 70 cycles, while the cells with the additives continue to cycle without failure for 120 and 150 cycles for ammonium sulphate and ammonium phosphate additives respectively.
Fifty groundwater samples were collected from Al-Hasa to analyze the pH, electrical conductivity (EC, dS m
−1
), total dissolved solids (TDS), major anions (HCO
3
−
, CO
3
2−
, Cl
−
, SO
4
2−
, and ...NO
3
−
), major cations (Ca
2+
, Mg
2+
, Na
+
, and K
+
), and total hardness. The analyzed data plotted in the Piper, Gibbs, and Durov diagrams, and water quality index (WQI) were calculated to evaluate the groundwater geochemistry and its water quality. The results reveal that most of the investigated samples are Ca
2+
, Mg
2+
, SO
4
2−
, Cl
−
and Na
+
, and HCO
3
−
water types using the Piper diagram. Na
+
> Ca
2+
> Mg
2+
are the dominant cations, while Cl
−
> HCO
3
−
> SO
4
2−
> CO
3
2−
are the dominant anions. Sodium adsorption ratio (SAR) values varied from 0.79 to 10; however, the Kelly ratio (KR) ranged between 0.1 and 2.2. The permeability index (PI) showed that well water is suitable for irrigation purposes with 75% or more of maximum permeability. The US salinity diagram revealed that the water quality classes of studied waters were CIII-SI, CIII-SII, and CIV-SII, representing height hazards of salinity and medium- to low-sodium hazard. The water quality index (WQI) results indicated that total dissolved solids are out of the drinking water standard limits in Saudi Arabia. The WQI revealed that 38% of the studied wells were considered as poor water (class III), 52% are found as very poor water class (IV), and 10% are unsuitable water for drinking class (V).
The flow via needle has prominent applications in the modern world such as nano-wires, microstructure electric gadgets, microsensors, surgical instruments and biological treatments. The present ...investigation focuses on boundary layer heat, flow, and mass transfer of MHD tangent hyperbolic fluid (conveying tiny particles) via a thin needle under the impacts of activation energy, non-constant thermal conductivity, heat source, and nonlinear thermal radiation. In the description of the Buongiorno model, the significant features of Brownian motion and thermophoresis have been included. Adopting appropriate transformations to the given problem specified by the set of partial differential equations yields the dimensionless form of ordinary differential equations After that, these obtained ODEs are solved numerically via MATLAB bvp4c. A comparative result with previous findings is conducted. Physical parameters’ impact on flow rate, heat, and concentration is exhibited and explained in depth. The main findings of this study are that flow patterns reduce as the magnetic parameter and the Weissenberg number grow. Higher values of Brownian motion, heat source/sink, nonlinear radiation, and thermophoretic parameter improve the thermal profile. Moreover, the rate of heat transfer for the variable property case is significantly improved. Concentration profiles reduce as the thermophoresis parameter and chemical reaction parameter grow but improve as the activation energy and Brownian motion parameter rise. The percentage increase in Sherwood number is 35.07 and 5.44 when the thermophoresis takes input in the range 0 ≤
Nt
≤ 0.2 and activation energy parameters 0 ≤
E
≤ 0.2. The Weissenberg number and power-law index parameters are all designed to boost the Sherwood number.
In this work, the effect of a new quinoxaline derivative, 2-phenyl-3-(prop-2-yn-1-yloxy) quinoxaline (PYQX), was evaluated as a corrosion inhibitor for carbon steel (CS) in 1 M HCl electrolyte. ...Weight loss measurement, atomic absorption spectroscopy, potentiodynamic polarization, electrochemical impedance spectroscopy, scanning electron microscopy with energy dispersive spectroscopy, and UV-vis spectroscopy were employed to assess the inhibitory activity. The electronic properties of the interaction between the inhibitor molecule and the CS substrate were studied using molecular dynamics (MD) simulation, density functional theory (DFT), and Fukui functions. According to AC impedance experiments, the inhibitor under consideration showed a maximum level of 98.1 % inhibition efficiency at 1 mM and 30 °C. The Langmuir adsorption isotherm model explains the adsorption of PYQX on the CS surface. A slope of 1 denotes a strong molecule-substrate interaction, suggesting that the binding occurs at specific surface locations. To understand the functioning of the adsorption mechanism, various thermodynamic and activation parameters were evaluated. PDP tests demonstrated that PYQX functions as a mixed-type inhibitor. Computational correlations (DFT, MD, and Fukui indices) supported the experimental findings.
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