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Urea oxidation reaction (UOR) and hydrogen evolution reaction (HER) are the key processes for implementing urinated water electrolysis and hydrogen green production, respectively. This contribution investigates the modification of commercial nickel foam (NF) with a nickel phosphate (NiPO/NF) heterostructure layer via anodizing in phosphate solution at various potentials (5, 10 and 15 V) as a simple and efficient route to boost the urea-assisted water electrolysis and hydrogen production in alkaline medium. The morphology and composition physicochemical characterisation of the phosphate layer exhibit aggregates of crystalline nanoparticles with interstitial mesoporous and macroporous networks with a mole composition ratio of 9.42: 1.0: 8.14 for Ni: P: O respectively. The electrochemical measurements revealed the NiPO/NF anodized at 10 V exhibits a superior electroactive surface area of 255 cm2, a substantially higher urea oxidation current compared to pristine NF, achieving 20 and 500 mA/cm2 at 1.35 and 1.6 V vs. RHE respectively and retained 100 % of activity during the urea electrolysis for more than 3 h. The electrochemical impedance analysis confirmed the alkaline urea oxidation reaction proceeded via indirect (EC) and direct mechanism and the CO2 intermediates adsorption–desorption became the predominant reaction at more positive potential. The NiPO/NF anode employed in an H-shape can deliver up to ±400 mA/cm2 for UOR/HER at a bias potential of 1.85 V and 8-fold (2.0 mmol/min) much higher hydrogen production rate compared to the pristine NF anode (0.25 mmol/min). Combining commercial nickel foam modification via anodizing and alkaline urea electrolysis at ambient conditions offers a unique and innovative solution for both large-scale hydrogen green production as well as remedy of the urinated wastewater for a more sustainable future.

With the recent growth of global populations, main roads in cities have witnessed an evident increase in the number of vehicles. This has led to unprecedented challenges for authorities in managing the traffic of ambulance vehicles to provide medical services in emergency cases. Despite the high technologies associated with medical tracks and advanced traffic management systems, there is still a current delay in ambulances’ attendance in times of emergency to provide patients with vital aid. Therefore, it is indispensable to introduce a new emergency service system that enables the ambulance to reach the patient in the least congested and shortest paths. However, designing an efficient algorithm to plan the best route for an ambulance is still a global goal and a challenge that needs to be solved. This article introduces an Internet of Things emergency services system based on a real-time node rank index (NR-index) algorithm to find the best route for the ambulance to reach the patient and provide the required medical services in emergency cases. The proposed system design copes with the dynamic traffic conditions to guarantee the shortest transport time. For this purpose, a vehicular ad hoc network is employed to collect accurate real-time traffic data. In this article, we suggest two parameters to compromise distance and congestion level. The first is the distance between the patient and the surrounding ambulance vehicles, and the second determines the congestion level to avoid the path with high congestion traffic. The system employs a developed real-time NR-index algorithm to select a suitable ambulance vehicle to respond to emergency cases at a low travel cost with the fastest journey. Finally, our system makes it easier for ambulance vehicles to use the best route and avoid heavy traffic. This allows them to make their way to the patient quickly and increases the chance of saving lives. The simulation results show significant improvements in terms of average travel time, average travel speed, and normalized routing load.

Renal involvement of systemic lupus erythematosus needs aggressive treatment. Despite the development of multiple international guidelines, differences in practices exist. This study aimed to explore the current practices of pediatric rheumatologists and nephrologists for the diagnosis, management, and monitoring of lupus nephritis (LN) in Saudi Arabia through a survey. Among the 61 respondents, 54.1% were pediatric nephrologists and 49.9% were pediatric rheumatologists. Predominantly, the participating physicians received training either nationally (57%) or in North America (45%). Most of the respondents (77%) did not have a combined rheumatology-nephrology clinic, primarily because of space or time limitations (75%), or a lack of the other specialty (13%). In terms of the decision to request a renal biopsy, the most common factors were nephrotic-range proteinuria (85%) and a lower level of proteinuria associated with hypocomplementemia or elevated anti-double-stranded (ds) DNA (73%). There was marginal agreement over monitoring the disease’s activity and treatment response; Complements 3 and 4, anti-dsDNA, protein-creatinine ratio, and estimated glomerular filtration rate were the most popular parameters. The main reason for repeating a renal biopsy was a new renal manifestation that was inconsistent with the previous biopsy. There was considerable variability in the induction therapies used to initiate and taper corticosteroids and conventional immunosuppressive drugs. Most respondents (91%) used angiotensin-converting enzyme agents to control proteinuria. Considerable agreement exists among Saudi physicians managing children with LN but significant variations exist regarding the therapeutic strategies. Additional endeavors are needed to establish a unified national clinical approach for managing LN in children.

Abstract Introduction Plasminogen (PLG) deficiency is an ultrarare disease. The reported manifestations in literature were linked to pseudomembrane formation and mucosal surfaces inflammation. Recently, PLG, its activators and its receptors have gained more attention in inflammation regulatory processes, including the release of proinflammatory signaling molecules, and thus its role is believed to have clinical implications beyond what has been known. Case Report We present a child with recurrent fever who, although managed initially as familial Mediterranean fever, later on, developed a constellation of findings that were not explained by a classified autoinflammatory disease. Genetic testing revealed a novel homozygous PLG mutation (PLG: c.466G>A: p.D156N) and a likely benign heterozygous MEFV gene variant. We propose that the PLG mutation is responsible for the clinical manifestations, which may or may not be exacerbated by the coexistence of the MEFV variant. A relationship between the PLG pathway, inflammation, and FMF severity has been addressed recently in several studies. Conclusion This report highlights the recently recognized role of the PLG pathway in inflammatory diseases and describes a potentially new presentation of PLG pathogenesis. Further studies are needed to confirm this finding and allow for a more definitive conclusion.

Recently the urea and urinated wastewater electrolysis process has shown promising technology for hydrogen fuel green production in addition to denitrification of wastewater. However, to make the process economically valuable, the electrocatalysts nanoarchitecture, nanometer size, shape, facets, and composition need to be engineered to boost the urea oxidation reaction (UOR). This work demonstrates a simple and novel approach to the synthesis of mesoporous nickel phosphate nanoparticles (meso-NiPO) via chemical deposition from a surfactant liquid crystal template. Typically, the nickel ions dissolved in the aqueous domain of the hexagonal liquid crystalline phase of the Brij®78 template were chemically reacted with the sodium phosphate solution to precipitate the mesoporous nickel phosphate nanoarchitecture after washing up the surfactant. The physicochemical characterizations show the meso-NiPO exhibits an amorphous highly mesoporous structure with a higher specific surface area (43.50 m2/g) compared to the bare nickel phosphate (bare-NiPO, 4.26 m2/g) prepared in the absence of surfactant. The electrochemical performance of meso-NiPO electrocatalyst for the urea oxidation reaction in alkaline solution exhibits superior activity including a lower oxidation onset potential (0.30 V vs. Ag/AgCl), charge transfer resistance (3.35 O) and mass activity of 700.7 mA/cm2 mg at the oxidation potential of 0.6 V vs. Ag/AgCl. Moreover, the meso-NiPO reveals long-term stability, and 97.5% of the steady-state oxidation current was maintained after the 3-hour urea electrolysis test. Using an H-shape urea electrolyzer, the corresponding cathodic hydrogen production rate reached 415 µmol/h and a Faradic efficiency of 96.8 % at an applied bias of 2.0 V. The electroactivity high performance of the mesoporous nickel phosphate is ascribed to the high specific surface area and mesoporous architecture that provide efficient charge transfer, as well as mass transport of the electroactive species. The chemical deposition from a surfactant liquid crystal template has the advantages of a one-pot template, applicable to the synthesis of a wide range of nanomaterials with various compositions and nanoarchitectures at room temperature for application in electrochemical energy production and storage systems.

Objectives To validate the PEDiatric Behçet's Disease classification criteria (PEDBD) with an evidence-based approach. Methods A total of 210 pediatric patients [70 Behçet's disease (BD), 40 periodic fever, aphthous stomatitis, pharyngitis, adenitis, 35 familial Mediterranean fever, 26 hyper-IgD syndrome, 22 TNF-receptor associated periodic fever syndrome, 17 undefined recurrent fevers] were randomly selected from the Eurofever Registry. A set of 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients. Using the table consensus as gold standard (agreement ≥ 80%), the PEDBD, ISG and ICBD criteria were applied to BD patients and to confounding diseases with other autoinflammatory conditions in order to define their sensitivity, specificity and accuracy. Results At the end of the third round, a consensus was reached in 139/210 patients (66.2%). The patients with a consensus ≥80% were classified as confirmed BD (n  = 24), and those with an agreement of 60–79% as probable BD (n  = 10). When comparing these patients with the confounding diseases group, an older age at disease onset, the presence of oral and genital ulcers, skin papulo-pustular lesions, a positive pathergy test and posterior uveitis were BD distinctive elements. The ISG, ICBD and PEDBD criteria were applied to confirmed BD and to the confounding disease group, showing a sensitivity of 0.50, 0.79 and 0.58, a specificity of 1.00, 0.97 and 0.99, and an accuracy of 0.91, 0.94 and 0.92, respectively. Conclusions The PEDBD criteria were very specific, while the ICBD were more sensitive. The complexity of childhood BD suggests larger prospective international cohorts to further evaluate the performance of the criteria. [ABSTRACT FROM AUTHOR]

This work examines the cooperative effect between Zr doping and oxygen vacancy engineering in anodized TiO2 nanotubes (TNTs) for enhanced oxygen reduction reactions (ORRs). Zr dopant and annealing conditions significantly affected the electrocatalytic characteristics of grown TNTs. Zr doping results in Zr4+ substituted for Ti4+ species, which indirectly creates oxygen vacancy donors that enhance charge transfer kinetics and reduce carrier recombination in TNT bulk. Moreover, oxygen vacancies promote the creation of unsaturated Ti3+(Zr3+) sites at the surface, which also boosts the ORR interfacial process. Annealing at reductive atmospheres (e.g., H2, vacuum) resulted in a larger increase in oxygen vacancies, which greatly enhanced the ORR activity. In comparison to bare TNTs, Zr doping and vacuum treatment (Zr:TNT–Vac) significantly improved the conductivity and activity of ORRs in alkaline media. The finding also provides selective hydrogen peroxide production by the electrochemical reduction of oxygen.

The article discusses a case report of a 12-year-old Saudi boy with ISG15 deficiency, a rare genetic disorder associated with autoimmune manifestations. The boy presented with recurrent fever, respiratory symptoms, and musculoskeletal issues, which were managed with corticosteroids and tofacitinib. Genetic testing revealed a homozygous variant in the ISG15 gene, leading to the diagnosis of ISG15 deficiency. The patient's symptoms improved with treatment, highlighting the autoimmune features of ISG15 deficiency and its impact on the patient's health. The article emphasizes the complexity of the disease and the need for further research to understand its manifestations fully. [Extracted from the article]

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystemic disease. Monitoring disease activity thoughtout the disease course is important for effective management and assessment of disease outcome. Objective: To assess whether the pan-immune inflammation value (PIIV) at diagnosis could predict organ involvement and disease activity in childhood SLE (cSLE) patients after 12 months of disease onst. Methods: This is an observational retrospective multicenter study that comprised cSLE patients seen and followed at the participating centers between January 2010 and December 2022. All patients met the EULAR/ACR-19 criteria, were immunosuppressive drug-naïve at the time of SLE diagnosis and had a minimal follow-up period of 12 months. The data included clinical and laboratory findings and disease activity using the SLEDAI-2K. Receiver operating characteristic (ROC) curves were employed to determine the optimal cut-off value of PIIV and assess its predictive potential for disease activity, and organ involvement. Results: A total of 125 patients (104 female) with a median age of 16.0 (IQR 5.6) years, a median age at disease onset of 10.9 (IQR 3.0) years, and a median disease duration of 4.8 (IQR 5.3) years were included. The most frequent involved organs at diagnosis were hematological (89.6%), musculoskeletal (68.8%), mucocutaneous (63.2%), and renal (58.4%). However, at a 12-month follow-up visit, the most frequent involved organs were renal (40.0%), hematological (39.2%), musculoskeletal (15.2%), and mucocutaneous (10.4%). The median PIIV at diagnosis was 139 (IQR 229.6), while the median SLEDAI was 12 (IQR 6.5) and 3.5 (IQR 7.0) at diagnosis and 12 months, respectively. An optimal PIIV cut-off of 250 was found to be a predicative for disease activity, with a sensitivity of 45% and a specificity of 86%. The study revealed that the PIIV successfully predicted four systems in our cohort of patients. Conclusion: Our work suggests the PIIV might be a reasonable predictor for organ involvement and disease activity in newly diagnosed cSLE, though further research, particularly larger studies, is required to validate these findings, especially regarding organ involvement. [ABSTRACT FROM AUTHOR]

Developing highly efficient and stable electrocatalysts for urea electro-oxidation reactions (UORs) will improve wastewater treatment and energy conversion. A low-cost cobalt sulfide-anchored nickel sulfide electrode (CoS/Ni3S2@CP) was synthesized by electrodeposition in DMSO solutions and found to be highly effective and long-lasting. The morphology and composition of catalyst surfaces were examined using comprehensive physicochemical and electrochemical characterization. Specifically, CoS/Ni3S2@CP electrodes require a potential of 1.52 volts for a 50 mA/cm2 current, confirming CoS in the heterointerface CoS/Ni3S2@CP catalyst. Further, the optimized CoS/Ni3S2@CP catalyst shows a decrease of 100 mV in the onset potential (1.32 VRHE) for UORs compared to bare Ni3S2@CP catalysts (1.42 VRHE), demonstrating much greater performance of UORs. As compared to Ni3S2@CP, CoS/Ni3S2@CP exhibits twofold greater UOR efficiency as a result of a larger electroactive surface area. The results obtained indicate that the synthetic CoS/Ni3S2@CP catalyst may be a favorable electrode material for managing urea-rich wastewater and generating H2. [ABSTRACT FROM AUTHOR]

ObjectiveThe aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination.MethodsThis Registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients' management. The Registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, thus enhancing international collaboration and data sharing for research purposes. The Registry is practical enough to be easily modified to meet future needs regarding VEXAS syndrome.ResultsTo date (April 22nd, 2022), 113 Centers from 23 Countries in 4 continents have been involved; 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) are currently able to access the registry for data entry (or data sharing) and collection. The Registry includes 4,952 fields organized into 18 instruments designed to fully describe patient's details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access.ConclusionThis international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease, with the final goal to obtain real-world evidence for daily clinical practice, especially in relation to the comprehension of this disease about the natural history and the possible therapeutic approaches. This Project can be found on https://clinicaltrials.gov NCT05200715.

ObjectiveThis paper points out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients affected by Undifferentiated Systemic AutoInflammatory Diseases (USAIDs).MethodsThis is an electronic registry employed for real-world data collection about demographics, clinical, laboratory, instrumental and socioeconomic data of USAIDs patients. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is designed to obtain standardized information for real-life research. The instrument is endowed with flexibility, and it could change over time according to the scientific acquisitions and potentially communicate with other similar tools; this platform ensures security, data quality and data governance.ResultsThe focus of the AIDA project is connecting physicians and researchers from all over the world to shed a new light on heterogeneous rare diseases. Since its birth, 110 centers from 23 countries and 4 continents have joined the AIDA project. Fifty-four centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 179 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry is collecting baseline and follow-up data using 3,769 fields organized into 23 instruments, which include demographics, history, symptoms, trigger/risk factors, therapies, and healthcare information access for USAIDs patients.ConclusionsThe development of the AIDA International Registry for USAIDs patients will facilitate the online collection of real standardized data, connecting a worldwide group of researchers: the Registry constitutes an international multicentre observational groundwork aimed at increasing the patient cohort of USAIDs in order to improve our knowledge of this peculiar cluster of autoinflammatory diseases. NCT 05200715 available at https://clinicaltrials.gov/.

Abstract Introduction Systemic lupus erythematosus (SLE) is a multi-organ inflammatory disease associated with autoimmune diseases. The aim of the study is to assessed the frequency of celiac disease (CD) in adults and children with SLE (aSLE and cSLE, respectively) and compare them with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) patients; the study also explored the clinical impact of CD serologic markers on SLE disease activity and severity. Methods This was a cross-sectional study. Patients with SLE who had regular follow-up in rheumatology clinics were evaluated for laboratory and clinical variables using serology and the SLE Disease Activity Index (SLEDAI). To assess the occurrence of CD serology in cSLE and aSLE and the clinical impact of CD serologic markers on SLE, patients were tested for antigliadin (AGA), anti-endomysium (EmA) and anti-tissue transglutaminase (tTG) antibodies. RA and JIA patients were included for comparison. Duodenal biopsy was conducted in patients who exhibited CD markers. Results The CD marker was found in 29 (11.6%) of the 250 patients. AGA was present in seven aSLE patients and tTG in two (11.1%). Among cSLE patients, the autoantibody was present in 17.6% (AGA in four, tTG in two, and EmA in three). For RA patients, five had AGA and tTG and one had EmA, with an overall positivity of 9.7%. Five JIA patients had AGA (four with EmA and five with tTG) with overall positivity of 10.9%; the serum IgA level was normal in all patients except one. Duodenal endoscopic biopsy was performed in patients with positive CD markers (two declined). Histologic confirmation of CD was reported in one RA and one JIA patient but in none of the SLE patients. There was no correlation between the presence of CD markers and autoantibodies in SLE. Conclusion CD antibodies did not influence SLE activity. Thus, SLE patients may not need to be screened for CD antibodies.