Synthesis and Characterizations of Titanium Tungstosilicate and Tungstophosphate Mesoporous Materials

The work reports a development approach for the synthesis of novel multi-components mesoporous materials of titanium tungstate (meso-TiW) titanium tungstosilicate (meso-TiWSi) and tungstophosphate (meso-TiWP) mixed oxides that have high surface area and ordered mesoporous structures at nanometer length scale. Using the solvent evaporation-induced self-assembly (EISA) new oxides of bi- and tri-component of meso-TiW, meso-TiWSi and meso-TiWP oxides with different compositions and porosity were achieved. The physicochemical properties of the mesoporous oxides were characterized by X-ray diffraction, BET surface area analyzer, scanning, and transmission electron microscopes. Subject to the oxide composition, the obtained meso-TiW, meso-TiWSi and meso-TiWP exhibits high surface area, ordered 2D hexagonal mesostructured with order channels extended over a large area. The produced meso-TiW, meso-TiWSi, and meso-TiWP adsorbents exhibit good adsorption efficiency for the removal of Pb(II), Cd(II) and Hg(II) ions from water solution due to the presence of high surface area and accessibility of surface active sites. The adsorption efficiency of these mesoporous oxide reaches up to 95% and is found to be dependent contact time and adsorbents dose. The synthesis strategy is particularly advantageous for the production of new complex (multi-component) inorganic mesoporous materials that might have an application in the field of environmental, catalysis or energy storage and production

Photoelectrochemical performance of strontium titanium oxynitride photo-activated with cobalt phosphate nanoparticles for oxidation of alkaline water

Photoelectrochemical (PEC) solar water splitting is favourable for transforming solar energy into sustainable hydrogen fuel using semiconductor electrodes. Perovskite-type oxynitrides are attractive photocatalysts for this application due to their visible light absorption features and stability. Herein, strontium titanium oxynitride (STON) containing anion vacancies of SrTi(O,N)3−δ was prepared via solid phase synthesis and assembled as a photoelectrode by electrophoretic deposition, and their morphological and optical properties and PEC performance for alkaline water oxidation are investigated. Further, cobalt-phosphate (CoPi)-based co-catalyst was photo-deposited over the surface of the STON electrode to boost the PEC efficiency. A photocurrent density of ~138 μA/cm at 1.25 V versus RHE was achieved for CoPi/STON electrodes in presence of a sulfite hole scavenger which is approximately a four-fold enhancement compared to the pristine electrode. The observed PEC enrichment is mainly due to the improved kinetics of oxygen evolution because of the CoPi co-catalyst and the reduced surface recombination of the photogenerated carriers. Moreover, the CoPi modification over perovskite-type oxynitrides provides a new dimension for developing efficient and highly stable photoanodes in solar-assisted water-splitting reactions

Structure and electrochemical activity of nickel aluminium fluoride nanosheets during urea electro-oxidation in an alkaline solution

An electrocatalyst of potassium nickel aluminium hexafluoride (KNiAlF6) nanosheets has been prepared using solid-phase synthesis at 900 °C. X-ray diffraction, scanning electron microscopy, and conductivity studies confirmed the formation of KNiAlF6 nanosheets having a cubic defect pyrochlore structure with an average thickness of 60–70 nm and conductivity of 1.297 × 103 S m−1. The electrochemical catalytic activity of the KNiAlF6 nanosheets was investigated for urea oxidation in alkaline solution. The results show that the KNiAlF6 nanosheets exhibit a mass activity of ∼395 mA cm−2 mg−1 at 1.65 V vs. HRE, a reaction activation energy of 4.02 kJ mol−1, Tafel slope of 22 mV dec−1 and an oxidation onset potential of ∼1.35 V vs. HRE which is a significant enhancement for urea oxidation when compared with both bulk Ni(OH)2 and nickel hydroxide-based catalysts published in the literature. Chronoamperometry and impedance analysis of the KNiAlF6 nanosheets reveal lower charge transfer resistance and long-term stability during the prolonged urea electro-oxidation process, particularly at 60 °C. After an extended urea electrolysis process, the structure and morphology of the KNiAlF6 nanosheets were significantly changed due to partial transformation to Ni(OH)2 but the electrochemical activity was sustained. The enhanced electrochemical surface area and the replacement of nickel in the lattice by aluminium make KNiAlF6 nanosheets highly active electrocatalysts for urea oxidation in alkaline solution

The multifaceted presentation of chronic recurrent multifocal osteomyelitis: A series of 486 cases from the Eurofever international registry

Objectives. Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry. Methods. A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved. Results. Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3%) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88%), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, 2% urticarial rash) and gastrointestinal symptoms (8%). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response. Conclusion. This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations

International cohort study for pediatric Behçet’s Disease: update 2011

PubMe

Development and Implementation of the AIDA International Registry for Patients With Undifferentiated Systemic AutoInflammatory Diseases

Objective: This 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). Methods: This 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. Results: The 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. Conclusions: The 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/

In silico validation of the autoinflammatory disease damage index

Introduction Autoinflammatory diseases can cause irreversible tissue damage due to systemic inflammation. Recently, the Autoinflammatory Disease Damage Index (ADDI) was developed. The ADDI is the first instrument to quantify damage in familial Mediterranean fever, cryopyrin-associated periodic syndromes, mevalonate kinase deficiency and tumour necrosis factor receptor-associated periodic syndrome. The aim of this study was to validate this tool for its intended use in a clinical/research setting. Methods The ADDI was scored on paper clinical cases by at least three physicians per case, independently of each other. Face and content validity were assessed by requesting comments on the ADDI. Reliability was tested by calculating the intraclass correlation coefficient (ICC) using an â observer-nested-within-subject\u27 design. Construct validity was determined by correlating the ADDI score to the Physician Global Assessment (PGA) of damage and disease activity. Redundancy of individual items was determined with Cronbach\u27s alpha. Results The ADDI was validated on a total of 110 paper clinical cases by 37 experts in autoinflammatory diseases. This yielded an ICC of 0.84 (95% CI 0.78 to 0.89). The ADDI score correlated strongly with PGA-damage (r=0.92, 95% CI 0.88 to 0.95) and was not strongly influenced by disease activity (r=0.395, 95% CI 0.21 to 0.55). After comments from disease experts, some item definitions were refined. The interitem correlation in all different categories was lower than 0.7, indicating that there was no redundancy between individual damage items. Conclusion The ADDI is a reliable and valid instrument to quantify damage in individual patients and can be used to compare disease outcomes in clinical studies

Autoimmune Neuromuscular Disorders in Childhood

Autoimmune neuromuscular disorders in childhood include Guillain-Barré syndrome and its variants, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), juvenile myasthenia gravis (JMG), and juvenile dermatomyositis (JDM), along with other disorders rarely seen in childhood. In general, these diseases have not been studied as extensively as they have been in adults. Thus, treatment protocols for these diseases in pediatrics are often based on adult practice, but despite the similarities in disease processes, the most widely used treatments have different effects in children. For example, some of the side effects of chronic steroid use, including linear growth deceleration, bone demineralization, and chronic weight issues, are more consequential in children than in adults. Although steroids remain a cornerstone of therapy in JDM and are useful in many cases of CIDP and JMG, other immunomodulatory therapies with similar efficacy may be used more frequently in some children to avoid these long-term sequelae. Steroids are less expensive than most other therapies, but chronic steroid therapy in childhood may lead to significant and costly medical complications. Another example is plasma exchange. This treatment modality presents challenges in pediatrics, as younger children require central venous access for this therapy. However, in older children and adolescents, plasma exchange is often feasible via peripheral venous access, making this treatment more accessible than might be expected in this age group. Intravenous immunoglobulin also is beneficial in several of these disorders, but its high cost may present barriers to its use in the future. Newer steroid-sparing immunomodulatory agents, such as azathioprine, tacrolimus, mycophenolate mofetil, and rituximab, have not been studied extensively in children. They show promising results from case reports and retrospective cohort studies, but there is a need for comparative studies looking at their relative efficacy, tolerability, and long-term adverse effects (including secondary malignancy) in children

Development of the autoinflammatory disease damage index (ADDI)

OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. METHODS: We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. RESULTS: More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. CONCLUSIONS: An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process