Bilateral Glaucoma as Possible Additional Feature for PGAP3-Associated Hyperphosphatasia.

Hyperphosphatasia with mental disorder (HPMRS) is a rare autosomal recessive disease caused by gene mutations in enzymes involved in the synthesis and remodeling of lipids. Seven-month-old boy diagnosed with bilateral glaucoma had a cleft palate, facial dysmorphism, hypertelorism, a broad nasal bridge, and large fleshy earlobes. A brain MRI scan also revealed brain abnormalities. The observed phenotype in a seven-month-old boy is in agreement with the phenotypic features of HPRMS type-4. Whole exome sequencing revealed a possible pathogenic variant of PGAP3 in a homozygous state (c.320C > T, p.Ser107Leu) which supported the diagnosis of HPRMS type-4. We report an unusual presentation for HPMRS and suggest adding this syndrome to the list of differential diagnoses of syndromic congenital glaucoma.

Albuterol as an adjuvant in acute anticholinesterase pesticide poisoning: a randomized, placebo-controlled clinical trial.

Acute anticholinesterase pesticide poisoning is a serious clinical problem, particularly in developing countries. Atropine is the most acceptable treatment for acute anticholinesterase poisoning. However, it only stops fluid production. Albuterol is a beta-2 receptor agonist that can increase fluid removal and speed the return of effective oxygen exchange. This study aims to evaluate the safety and efficacy of nebulized albuterol as an adjuvant therapy in patients with acute anticholinesterase poisoning. This stratified block randomized, single-blinded, placebo-controlled, parallel-group clinical trial was conducted between November 2020 and October 2021. It enrolled 80 patients with acute anticholinesterase pesticide poisoning who were admitted to Tanta University Poison Control Center. Patients were allocated into two groups (40 patients each). The strata were based on the severity of poisoning (moderate and severe). Patients in group I received 10 mg of nebulized albuterol. Group II received an equivalent volume of nebulized normal saline. Additionally, standard treatment was provided to both groups. Outcomes included oxygenation, mortality, need for endotracheal intubation and mechanical ventilation, hospital stay duration, time to atropinization, and total doses of atropine and oxime. We found insignificant differences in sociodemographics, exposure characteristics, clinical manifestations, or routine laboratory tests between the studied groups. The median values of oxygen saturation by pulse oximetry were 99% in the albuterol moderate toxicity group and 98% in the control moderate toxicity group. Albuterol significantly improved oxygen saturation in moderate intoxicated patients (P = 0.039). Therefore, nebulized albuterol is a safe drug. Moreover, it may improve oxygenation in acute anticholinesterase pesticide poisoning.

Tetraalkylammonium salts (TAS) in solar energy applications - A review on in vitro and in vivo toxicity.

Tetraalkylammonium salt (TAS) is an organic salt widely employed as a precursor, additive or electrolyte in solar cell applications, such as perovskite or dye-sensitized solar cells. Notably, Perovskite solar cells (PSCs) have garnered acclaim for their exceptional efficiency. However, PSCs have been associated with environmental and health concerns due to the presence of lead (Pb) content, the use of hazardous solvents, and the incorporation of TAS in their fabrication processes, which significantly contributes to environmental and human health toxicity. As a response, there is a growing trend towards transitioning to safer and biobased materials in PSC fabrication to address these concerns. However, the potential health hazards associated with TAS necessitate a thorough evaluation, considering the widespread use of this substance. Nevertheless, the overexploitation of TAS could potentially increase the disposal of TAS in the ecosystem, thus, posing a major health risk and severe pollution. Therefore, this review article presents a comprehensive discussion on the in vitro and in vivo toxicity assays of TAS as a potential material in solar energy applications, including cytotoxicity, genotoxicity, in vivo dermal, and systemic toxicity. In addition, this review emphasizes the toxicity of TAS compounds, particularly the linear tetraalkyl chain structures, and summarizes essential findings from past studies as a point of reference for the development of non-toxic and environmentally friendly TAS derivatives in future studies. The effects of the TAS alkyl chain length, polar head and hydrophobicity, cation and anion, and other properties are also included in this review.

Generation of transgene-free canker-resistant Citrus sinensis cv. Hamlin in the T0 generation through Cas12a/CBE co-editing.

Citrus canker disease affects citrus production. This disease is caused by Xanthomonas citri subsp. citri (Xcc). Previous studies confirmed that during Xcc infection, PthA4, a transcriptional activator like effector (TALE), is translocated from the pathogen to host plant cells. PthA4 binds to the effector binding elements (EBEs) in the promoter region of canker susceptibility gene LOB1 (EBEPthA4-LOBP) to activate its expression and subsequently cause canker symptoms. Previously, the Cas12a/CBE co-editing method was employed to disrupt EBEPthA4-LOBP of pummelo, which is highly homozygous. However, most commercial citrus cultivars are heterozygous hybrids and more difficult to generate homozygous/biallelic mutants. Here, we employed Cas12a/CBE co-editing method to edit EBEPthA4-LOBP of Hamlin (Citrus sinensis), a commercial heterozygous hybrid citrus cultivar grown worldwide. Binary vector GFP-p1380N-ttLbCas12a:LOBP1-mPBE:ALS2:ALS1 was constructed and shown to be functional via Xcc-facilitated agroinfiltration in Hamlin leaves. This construct allows the selection of transgene-free regenerants via GFP, edits ALS to generate chlorsulfuron-resistant regenerants as a selection marker for genome editing resulting from transient expression of the T-DNA via nCas9-mPBE:ALS2:ALS1, and edits gene(s) of interest (i.e., EBEPthA4-LOBP in this study) through ttLbCas12a, thus creating transgene-free citrus. Totally, 77 plantlets were produced. Among them, 8 plantlets were transgenic plants (#HamGFP1 - #HamGFP8), 4 plantlets were transgene-free (#HamNoGFP1 - #HamNoGFP4), and the rest were wild type. Among 4 transgene-free plantlets, three lines (#HamNoGFP1, #HamNoGFP2 and #HamNoGFP3) contained biallelic mutations in EBEpthA4, and one line (#HamNoGFP4) had homozygous mutations in EBEpthA4. We achieved 5.2% transgene-free homozygous/biallelic mutation efficiency for EBEPthA4-LOBP in C. sinensis cv. Hamlin, compared to 1.9% mutation efficiency for pummelo in a previous study. Importantly, the four transgene-free plantlets and 3 transgenic plantlets that survived were resistant against citrus canker. Taken together, Cas12a/CBE co-editing method has been successfully used to generate transgene-free canker-resistant C. sinensis cv. Hamlin in the T0 generation via biallelic/homozygous editing of EBEpthA4 of the canker susceptibility gene LOB1.

Astrocyte expression of aging-associated markers positively correlates with neurodegeneration in the frontal lobe of the rhesus macaque brain.

Introduction: As the population over the age of 65 increases, rates of neurodegenerative disorders and dementias will rise - necessitating further research into the cellular and molecular mechanisms that contribute to brain aging. With the critical importance of astrocytes to neuronal health and functioning, we hypothesized that alterations in astrocyte expression of aging-associated markers p16INK4a (p16) and sirtuin 1 (SIRT1) with age would correlate with increased rates of neurodegeneration, as measured by FluoroJade C (FJC) staining. Methods: To test this hypothesis, 19 rhesus macaques at the Tulane National Primate Research Center were selected based on the following criteria: archival FFPE CNS tissue available to use, no noted neuropathology, and an age range of 5-30 years. Tissues were cut at 5 µm and stained for GFAP, p16, SIRT1, and FJC, followed by whole-slide imaging and HALO® image analysis for percentage of marker-positive cells and relative intensity of each stain. Results: We found the percentage of p16+ cells increases with age in total cells and astrocytes of the frontal (p = 0.0021, p = 0.0012 respectively) and temporal (p = 0.0226, p = 0.0203 respectively) lobes, as well as the relative intensity of p16 staining (frontal lobe: p = 0.0060; temporal lobe: p = 0.0269). For SIRT1, we found no correlation with age except for an increase in the relative intensity of SIRT1 in the temporal lobe (p = 0.0033). There was an increase in neurodegeneration, as measured by the percentage of FJC+ cells in the frontal lobe with age (p = 0.0057), as well as in the relative intensity of FJC staining in the frontal (p = 0.0030) and parietal (p = 0.0481) lobes. Importantly, increased p16 and SIRT1 expression in astrocytes correlated with increasing neurodegeneration in the frontal lobe (p = 0.0009, p = 0.0095 respectively). Discussion: Together, these data suggest that age-associated alterations in astrocytes contribute to neurodegeneration and provide a target for mechanistic studies in the future.

Repositioning of anti-infective compounds against monkeypox virus core cysteine proteinase: a molecular dynamics study.

Monkeypox virus (MPXV) core cysteine proteinase (CCP) is one of the major drug targets used to examine the inhibitory action of chemical moieties. In this study, an in silico technique was applied to screen 1395 anti-infective compounds to find out the potential molecules against the MPXV-CCP. The top five hits were selected after screening and processed for exhaustive docking based on the docked score of ≤ -9.5 kcal/mol. Later, the top three hits based on the exhaustive-docking score and interaction profile were selected to perform MD simulations. The overall RMSD suggested that two compounds, SC75741 and ammonium glycyrrhizinate, showed a highly stable complex with a standard deviation of 0.18 and 0.23 nm, respectively. Later, the MM/GBSA binding free energies of complexes showed significant binding strength with ΔGTOTAL from -21.59 to -15 kcal/mol. This report reported the potential inhibitory activity of SC75741 and ammonium glycyrrhizinate against MPXV-CCP by competitively inhibiting the binding of the native substrate.

Sedentary behavior in mice induces metabolic inflexibility by suppressing skeletal muscle pyruvate metabolism.

Carbohydrates and lipids provide the majority of substrates to fuel mitochondrial oxidative phosphorylation (OXPHOS). Metabolic inflexibility, defined as an impaired ability to switch between these fuels, is implicated in a number of metabolic diseases. Here we explore the mechanism by which physical inactivity promotes metabolic inflexibility in skeletal muscle. We developed a mouse model of sedentariness, small mouse cage (SMC) that, unlike other classic models of disuse in mice, faithfully recapitulated metabolic responses that occur in humans. Bioenergetic phenotyping of skeletal muscle mitochondria displayed metabolic inflexibility induced by physical inactivity, demonstrated by a reduction in pyruvate-stimulated respiration (JO2) in absence of a change in palmitate-stimulated JO2. Pyruvate resistance in these mitochondria was likely driven by a decrease in phosphatidylethanolamine (PE) abundance in the mitochondrial membrane. Reduction in mitochondrial PE by heterozygous deletion of phosphatidylserine decarboxylase (PSD) was sufficient to induce metabolic inflexibility measured at the whole-body level, as well as at the level of skeletal muscle mitochondria. Low mitochondrial PE in C2C12 myotubes was sufficient to increase glucose flux towards lactate. We further implicate that resistance to pyruvate metabolism is due to attenuated mitochondrial entry via mitochondrial pyruvate carrier (MPC). These findings suggest a mechanism by which mitochondrial PE directly regulates MPC activity to modulate metabolic flexibility in mice.

Machine learning-based prognostication of mortality in stroke patients.

Objectives: Predicting stroke mortality is crucial for personalized care. This study aims to design and evaluate a machine learning model to predict one-year mortality after a stroke. Materials and methods: Data from the National Multiethnic Stroke Registry was utilized. Eight machine learning (ML) models were trained and evaluated using various metrics. SHapley Additive exPlanations (SHAP) analysis was used to identify the influential predictors. Results: The final analysis included 9840 patients diagnosed with stroke were included in the study. The XGBoost algorithm exhibited optimal performance with high accuracy (94.5%) and AUC (87.3%). Core predictors encompassed National Institutes of Health Stroke Scale (NIHSS) at admission, age, hospital length of stay, mode of arrival, heart rate, and blood pressure. Increased NIHSS, age, and longer stay correlated with higher mortality. Ambulance arrival and lower diastolic blood pressure and lower body mass index predicted poorer outcomes. Conclusions: This model's predictive capacity emphasizes the significance of NIHSS, age, hospital stay, arrival mode, heart rate, blood pressure, and BMI in stroke mortality prediction. Specific findings suggest avenues for data quality enhancement, registry expansion, and real-world validation. The study underscores machine learning's potential for early mortality prediction, improving risk assessment, and personalized care. The potential transformation of care delivery through robust ML predictive tools for Stroke outcomes could revolutionize patient care, allowing for personalized plans and improved preventive strategies for stroke patients. However, it is imperative to conduct prospective validation to evaluate its practical clinical effectiveness and ensure its successful adoption across various healthcare environments.

The impact of weekly semaglutide, a glucagon-like peptide-1 agonist, on kidney outcomes in adults with type 2 diabetes mellitus.

Background: Type 2 diabetes mellitus (T2DM) is a chronic metabolic disorder associated with kidney complications. This study aims to investigate the effects of weekly subcutaneous semaglutide, a GLP-1 agonist, on kidney outcomes. Methods: This retrospective cohort study was conducted in nephrology and endocrinology clinics at KFAFH from March 2022 to February 2023. The sample size was determined based on hospital records, and randomly selected patients who met the inclusion criteria were included. The inclusion criteria included adults with T2DM who were on weekly subcutaneous semaglutide for 6 months or longer. Patients with type 1 diabetes mellitus, pregnant or gestational diabetes patients, individuals who added other antidiabetic medications during the study period, and participants who refused to be involved were excluded from the study. Results: The study included participants aged between 42 and 85 years, with a mean age of 65.38 years, and the majority (58.7%) were males. There was a significant weight and BMI reduction observed in all patients, with P values <0.001 for both. The average weight reduction was 2.97 kg (SD = 2.34, 95% CI 1.65-3.30), and the average Body (BMI) reduction was 1.16 (SD = 0.91, 95% CI 1.03-1.29). A vast majority of participants (98.5%) reported a weight loss of at least 1 kg, and 13.8% of participants experienced a change in BMI category from higher to lower. No patients reported an increase in the BMI category. There was a significant reduction in glycohemoglobin (HbA1c) measurements from 9.18 pretreatment to 8.13 posttreatment, with an average reduction of 1.05 units (SD = 1.84, 95% CI 0.79-1.31). The majority of participants (70.9%) reported a reduction in HbA1c of at least 0.1 unit. Although there was a slight decrease in estimated glomerular filtration rate (eGFR) values on average (1.35 units), the change was not statistically significant (P = 0.059). More than half of the participants (51.5%) reported an increase in eGFR, 45.4% reported a decrease, and 3.1% reported no change. There was a statistically significant reduction in urinary albumin-to-creatinine ratio (UACR) values from a median of 5.97 pretreatment to a median of 5.60 post-treatment. The median decrease was 0.72 units, with one-third (33.3%) reporting an increase and two-thirds (66.7%) reporting a decrease in values. The correlation analysis revealed no significant association between the total quantity of semaglutide taken and the magnitude of changes. Conclusion: Our study on the impact of weekly semaglutide in adults with T2DM reveals positive effects on kidney outcomes, including weight loss, glycemic control and improved urine albumin creatine ratio, and a reduced risk of nephropathy. These findings highlight the potential of semaglutide as a safe and effective treatment option for improving renal health in individuals with T2DM.

Jagged2 targeting in lung cancer activates anti-tumor immunity via Notch-induced functional reprogramming of tumor-associated macrophages.

Signaling through Notch receptors intrinsically regulates tumor cell development and growth. Here, we studied the role of the Notch ligand Jagged2 on immune evasion in non-small cell lung cancer (NSCLC). Higher expression of JAG2 in NSCLC negatively correlated with survival. In NSCLC pre-clinical models, deletion of Jag2, but not Jag1, in cancer cells attenuated tumor growth and activated protective anti-tumor T cell responses. Jag2-/- lung tumors exhibited higher frequencies of macrophages that expressed immunostimulatory mediators and triggered T cell-dependent anti-tumor immunity. Mechanistically, Jag2 ablation promoted Nr4a-mediated induction of Notch ligands DLL1/4 on cancer cells. DLL1/4-initiated Notch1/2 signaling in macrophages induced the expression of transcription factor IRF4 and macrophage immunostimulatory functionality. IRF4 expression was required for the anti-tumor effects of Jag2 deletion in lung tumors. Antibody targeting of Jagged2 inhibited tumor growth and activated IRF4-driven macrophage-mediated anti-tumor immunity. Thus, Jagged2 orchestrates immunosuppressive systems in NSCLC that can be overcome to incite macrophage-mediated anti-tumor immunity.

A Cross-Sectional Study of Anesthesia Safety in Wad Medani, Sudan: A Pre-war Status Indicating a Post-war Crisis.

BACKGROUND: As the surgical burden grows, increasing patient safety during anesthesia and surgery becomes a major global public health priority. Anesthesia can be safely administered in higher-income countries, yet it is more challenging in third-world countries. This study focuses on Sudan, a third-world country, and its unmet anesthetic needs before the current war and how these needs might compromise the post-war status. AIM: The aim of this study is to compare Sudan's outstanding anesthesia requirements to the World Health Organization's safe anesthesia practice standards in terms of workforce, medications, equipment, and anesthesia conduct. METHODS: This study was carried out in four hospitals (Wad Medani Teaching Hospital, Wad Medani Maternity Hospital, Gezira Centre for Renal and Urological Surgeries, and the National Centre for Pediatric Surgeries) in Wad Medani, two of which were referral and two were state-run. Each hospital from every category was identified using a convenience sampling technique. The World Health Organization-World Federation of Societies of Anesthesiologists International Standard and earlier regional African publications were used to determine the minimum predicted safe anesthesia needs. RESULTS: The results of our study demonstrate that overall, the hospitals surveyed fulfilled the minimum standards set by the World Health Organization and the World Federation of Societies of Anesthesiologists (WHO-WFSA) for safe anesthesia practice by 73% with no significant difference in the safety of anesthesia practice between state and referral hospitals. CONCLUSIONS: The state of safe anesthesia care in Wad Medani hospitals surveyed fell well short of the expected minimal criteria due to important requirements such as patient monitoring indicators, the inaccessibility of life-saving facilities such as defibrillators, and difficult intubation instruments. More importantly, the conduct of anesthesia was far below the standard.

Study of High-Transverse-Momentum Higgs Boson Production in Association with a Vector Boson in the qqbb Final State with the ATLAS Detector.

This Letter presents the first study of Higgs boson production in association with a vector boson (V=W or Z) in the fully hadronic qqbb final state using data recorded by the ATLAS detector at the LHC in proton-proton collisions at sqrt[s]=13 TeV and corresponding to an integrated luminosity of 137 fb^{-1}. The vector bosons and Higgs bosons are each reconstructed as large-radius jets and tagged using jet substructure techniques. Dedicated tagging algorithms exploiting b-tagging properties are used to identify jets consistent with Higgs bosons decaying into bb[over ¯]. Dominant backgrounds from multijet production are determined directly from the data, and a likelihood fit to the jet mass distribution of Higgs boson candidates is used to extract the number of signal events. The VH production cross section is measured inclusively and differentially in several ranges of Higgs boson transverse momentum: 250-450, 450-650, and greater than 650 GeV. The inclusive signal yield relative to the standard model expectation is observed to be µ=1.4_{-0.9}^{+1.0} and the corresponding cross section is 3.1±1.3(stat)_{-1.4}^{+1.8}(syst) pb.

Global prevalence of alkhumra hemorrhagic fever virus infection: The first meta-analysis and systematic review.

Alkhumra hemorrhagic fever virus (AHFV) has spread beyond the Middle East. However, the actual global prevalence of the virus is yet unknown. This systematic review and meta-analysis, thus, followed the standard reporting guidelines to provide comprehensive details on the prevalence of Alkhumra virus infection globally. The pooled prevalence of AHFV globally was estimated at 1.3% (95% CI: 0.3-6.3), with higher prevalence in humans (3.4%, 95% CI: 0.4-25.0) compared to animals (0.7%, 95% CI: 0.3-1.8). The prevalence in ticks and camels were 0.7% and 0.2%, respectively. Overall, there was a high prevalence rate in Asia (2.6%) compared to Africa (0.5%), and a distinctly higher prevalence in Saudi Arabia (4.6%) compared to other parts of the world (<1%). Lower surveillance rate in humans was observed in recent years. These findings will aid public health preparedness, surveillance, and development of preventive measures due to AHFV's potential for outbreaks and severe health consequences.

Gastrointestinal decontamination using oil-based solutions in patients with acute aluminum phosphide poisoning: a systematic review and meta-analysis.

Some studies suggested that gastrointestinal (GIT) decontamination with oil may improve the prognosis of patients who ingested aluminum phosphide (AlP). The aim of this study is to compare the efficacy and safety of gastric lavage with oil-based solutions to any method of gastric decontamination not using oils in patients presenting with acute AlP poisoning. The literature was searched for English-published randomized controlled trials (RCTs) from inception to 16 September 2023. The searched electronic databases included MEDLINE/PubMed, Cochrane Library, Web of Science, Egyptian Knowledge Bank, Scopus, and Google Scholar. Data were extracted and pooled by calculating the risk ratio (RR) for categorical outcomes and standardized mean difference (SMD) for numerical outcomes, with 95% confidence intervals (CI). Seven RCTs were included. Paraffin oil was significantly associated with a lower risk of mortality (RR = 0.59 [95% CI: 0.45, 0.76], p < .001), intubation (RR = 0.59 [95% CI: 0.46, 0.76], p < .001) and vasopressor need (RR = 0.71 [95% CI: 0.56, 0.91], p = .006). Survival time was significantly prolonged with paraffin oil (SMD = 0.72 [95% CI: 0.32, 1.13], p < .001). Coconut oil was significantly associated with prolonged survival time (SMD = 0.83 [95% CI: 0.06, 1.59], p = .03) as well as decreased risk of requiring intubation (RR = 0.78 [95% CI: 0.62, 0.99], p = .04). Oil-based GIT decontamination using paraffin oil showed benefits over conventional lavage regarding the incidence of in-hospital mortality and endotracheal intubation, and survival time. Coconut oil showed some benefits in terms of the intubation incidence and survival time. Decontamination using paraffin oil is recommended. Future clinical trials are warranted with larger sample sizes and focusing on cost-benefit and safety.

First-in-human stage III/IV melanoma​ clinical trial of immune priming agent IFx-Hu2.0.

IFx-Hu2.0 was designed to encode part of the Emm55 protein contained within a plasmid in a formulation intended for transfection into mammalian cells. IFx-Hu2.0 promotes both adaptive and innate immune responses in animal studies. Furthermore, previous studies have demonstrated safety/efficacy in equine, canine, and murine species. We present the first-in-human study of IFx-Hu2.0, administered by intralesional injection into melanoma tumors of seven patients with stage III/IV unresectable melanoma. No dose-limiting toxicities attributable to IFx-Hu2.0 were observed. Grade 1/2 injection site reactions were observed in five of seven patients. IgG and IgM responses were seen in the peripheral blood to Emm55 peptides and known melanoma antigens, suggesting that IFx-Hu2.0 acts as an individualized "in-situ vaccine." Three of four patients previously refractory to anti-PD1 experienced clinical benefit upon subsequent anti-PD1-based treatment. Therefore, this approach is feasible, and clinical/correlative outcomes warrant further investigation for treating metastatic melanoma patients as an immune priming agent.

Association of ABO, Rh-D and Kell blood groups with transfusion transmitted infections among blood donors from the Asir Region, Saudi Arabia: A retrospective observational study.

OBJECTIVES: To evaluate the association between transfusion-transmitted infections (TTIs) and ABO, Rh-D, and Kell blood systems among blood donors. METHODS: This was a retrospective study of 10,095 donors who visited the Blood Bank at Asir Hospital, Abha, Saudi Arabia. Data including demographic information, ABO, Rh-D, and Kell blood groups, and serological and molecular test results of TTIs (the TTIs were obtained from each donor's records). Chi-squared and Fisher's exact tests were employed to establish possible associations between blood groups and TTIs. RESULTS: The prevalence rate of TTIs among donors was 6.3%, with HBcAb (70%) being the most prevalent biomarker among positive donors. Donors with the O blood group were at a higher risk of contracting TTIs. Significant associations were observed between HIV and blood group A (χ2=6.30, p=0.01), HBsAg and group AB (χ2=17.3193, p=0.00003), malaria and group A (χ2=5.0567, p=0.02), and HBV-DNA and group AB (χ2=12.3163, p=0.0004). Also, Kell blood group was significantly associated with HIV (χ2=14.5, p=0.0001), HBcAb (χ2=78.51, p<0.0001), and syphilis (χ2=25.225, p<0.00001). CONCLUSION: ABO and Kell blood groups are associated with TTI markers. These findings highlight the need for improved strategies and approaches in screening and managing blood donations to minimize the risk of TTIs.

Nursing privilege: A concept analysis.

AIM: The study aimed to provide a comprehensive concept analysis of nursing privileges by elucidating its meaning and implications within the healthcare context. DESIGN: A concept analysis paper. METHODS: A comprehensive literature review was conducted from nursing and healthcare databases, professional nursing organizations, and regulatory bodies. Documents reviewed include research studies, policy documents and professional guidelines. The study employed Walker and Avant's eight-step method of concept analysis. This involved identifying the uses of the concept, its underlying attributes and referents, and constructing model, borderline, related and contrary cases. The antecedents, consequences and empirical referents of nursing privileges were also determined. RESULTS: The analysis uncovered vital attributes defining nursing privileges, encompassing professional authority, autonomy, access to resources, information, influence, decision-making power, respect and recognition. Additionally, antecedents and consequences of nursing privilege were identified, spanning development and resource access, as well as professional satisfaction and enhanced patient care. PATIENT OR PUBLIC CONTRIBUTION: No patient or public contribution.

Measurement of the Centrality Dependence of the Dijet Yield in p+Pb Collisions at sqrt[s_{NN}]=8.16 TeV with the ATLAS Detector.

ATLAS measured the centrality dependence of the dijet yield using 165 nb^{-1} of p+Pb data collected at sqrt[s_{NN}]=8.16 TeV in 2016. The event centrality, which reflects the p+Pb impact parameter, is characterized by the total transverse energy registered in the Pb-going side of the forward calorimeter. The central-to-peripheral ratio of the scaled dijet yields, R_{CP}, is evaluated, and the results are presented as a function of variables that reflect the kinematics of the initial hard parton scattering process. The R_{CP} shows a scaling with the Bjorken x of the parton originating from the proton, x_{p}, while no such trend is observed as a function of x_{Pb}. This analysis provides unique input to understanding the role of small proton spatial configurations in p+Pb collisions by covering parton momentum fractions from the valence region down to x_{p}∼10^{-3} and x_{Pb}∼4×10^{-4}.

Ab Initio Investigation of the Structural, Elastic, Dynamic, Electronic, and Magnetic Properties of Cubic Perovskite CeCrO3.

We presented the results of various aspects related to structural, elastic, electronic, dynamic, and magnetic parameters of cubic perovskite CeCrO3 by means of the full-potential linearized augmented plane wave (FP-LAPW) approach. The calculation of the unit cell volume against the total energy curve confirms that CeCrO3 exhibits higher energetic stability in the ferromagnetic (FM) order. Calculated structural aspects at equilibrium demonstrate excellent similarity to present information, lending credibility to our results. Moreover, monocrystalline elastic constants have been analyzed numerically. These constants provide insights into several related properties, including elastic anisotropy, mechanical stability, and several polycrystalline elastic aspects. Furthermore, the phonon dispersion curves obtained from our calculations reveal the existence of soft modes, which suggests the potential metastability of CeCrO3. Through an analysis of the energy band dispersions, the half-metallic nature of this material is confirmed, such as Eg = 3.00 and 3.13 eV for the HM state within generalized gradient approximations Perdew-Burke-Ernzerhof (GGA-PBE) and Tran-Blaha modified Becke-Johnson (TB-mBJ) calculations, respectively, as well as the FM total magnetic moment of 4.000 µB. Partial density of states (PDOS) aided in identifying the electronic states that contribute to the energy bands. Finally, the computed total magnetic moment aligns fit the theoretical findings available in the literature.

Whole-Exome Sequencing Identifies DYNC2H1 Mutations as a Cause of Jeune Asphyxiating Thoracic Dystrophy Without Extra-Skeletal Organ Involvement.

Jeune syndrome, or asphyxiating thoracic dystrophy (JATD), is a rare autosomal recessive skeletal dysplasia with heterogeneous genetic and clinical phenotypes, which primarily affects cartilage and bone development. Herein, we report a patient with a lethal form of SRTD3 without polydactyly (JATD), which led to severe restrictive lung disease and fatal respiratory failure. A full-term boy was born to a 30-year-old mother who was known to have hypothyroidism and was on thyroxine. The parents were first-degree cousins and had one healthy older son. Fetal ultrasound showed a cephalic fetus, normal amniotic fluid and a fundal placenta. All long bones and ribs were below the 1% percentile. The femur was bowed with no fractures or signs of significant demineralization at time of imaging. Head and abdominal circumference were within normal range. An echocardiogram on the 2nd day of life showed severe pulmonary hypertension (PHTN). Nitric oxide was started due to the presence of persistent hypoxia and severe PHTN. The patient continued to require high cardiorespiratory support, but the medical condition worsened, and respiratory failure persisted. The patient died of severe respiratory failure at 16 days of life due to respiratory insufficiency secondary to a severely restricted thoracic cage. Whole-exome sequencing (WES) revealed a homozygous mutation in the DYNC2H1 (NM_001377.3) gene, namely, the c.9041G>T NP_001368.2: p.(Arg3014Ile) missense variant, which results in the substitution of the arginine codon at amino acid position 3014 with an isoleucine codon. The phenotyping of the patient's JATD and the detection of a homozygous variant in the DYNC2H1 gene confirmed the diagnosis of short-rib thoracic dysplasia-3 without polydactyly. In summary, the patient had isolated skeletal anomalies without polydactyly or other organ involvement. Additionally, the infant had severe PHTN on top of the respiratory failure, which eventually caused death. Considerably more work will need to be done to determine the clinical spectrum of JATD and understand its genetic heterogeneity.