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Toward European harmonization of national myasthenia gravis registries: modified Delphi procedure-based expert consensus on collectable data.
BACKGROUND: Myasthenia gravis (MG) is a rare autoimmune disorder. Several new treatment concepts have emerged in recent years, but access to these treatments varies due to differing national reimbursement regulations, leading to disparities across Europe. This highlights the need for high-quality data collection by stakeholders to establish MG registries. A European MG registry could help bridge the treatment access gap across different countries, offering critical data to support regulatory decisions, foster international collaborations, and enhance clinical and epidemiological research. Several national MG registries already exist or are in development. To avoid duplication and ensure harmonization in data collection, a modified Delphi procedure was implemented to identify essential data elements for inclusion in national registries. RESULTS: Following a literature review, consultations with patient associations and pharmaceutical companies, and input from multiple European MG experts, 100 data elements were identified. Of these, 62 reached consensus for inclusion and classification, while only 1 item was agreed for exclusion. 30 items failed to reach the ≥ 80% agreement threshold and were excluded. Among the 62 accepted items, 21 were classified as mandatory data elements, 32 optional, and 9 items pertained to the informed consent form. CONCLUSIONS: Through a modified Delphi procedure, consensus was successfully achieved. This consensus-based approach represents a crucial step toward harmonizing MG registries across Europe. The resulting dataset will facilitate the sharing of knowledge and enhance European collaborations. Furthermore, the harmonized data may assist in regulatory or reimbursement decisions regarding novel therapies, as well as address treatment access disparities between European countries.
First-trimester nutrition insights from the United Arab Emirate Birth Cohort Study (UAE-BCS): assessment of dietary intake, micronutrient profiles, and folic acid supplementation in Emirati Women.
Maternal health and nutrition in early pregnancy play a vital role in the growth and development of the foetus. During this time, macro and micronutrients contribute to nutritional programming, which helps form the foundations of the foetus's life course health outcomes. This study aimed to investigate dietary habits, macro and micronutrient intake, micronutrient status, and folic acid supplement adherence among Emirati pregnant women in their first trimester. Data were collected according to the UAE-BCS study protocol, which was set up to investigate maternal nutrition, health, child growth, and developmental outcomes within the first 1000 days. Pregnant Emirati women with singleton pregnancies within their first trimester of pregnancy (between 8 and 12 weeks of gestation) were enrolled. The 24-hour food recall method was administered to collect dietary intake. The maternal mean average age was 29 years. Participants had high adherence to supplementation during pregnancy compared to preconception. The mean energy intake was 1345kcal, and 56% of participants consumed saturated fats above the acceptable macronutrient distribution ranges (AMDR), while 94% consumed below AMDR for total fibre. The consumption of micronutrients was below the recommended dietary allowance (RDA). Biochemical results show a high prevalence of low haemoglobin (74%) and deficiencies in vitamin D (39%) and vitamin E (96%). There is a need for research into dietary patterns and influences in pregnant women in the UAE. Furthermore, investigations of knowledge practices and attitudes towards supplementation and the factors contributing to folic acid supplement use are needed to inform government strategies and interventions.
Communication Between Anaesthesia Providers for Clinical and Professional Purposes: A Scoping Review
Background: Anaesthesia providers in all contexts need to be able to communicate with colleagues to meet a variety of clinical and professional needs, including physical help, advice and support as well as learning, supervision and mentorship. Such communication can be regarded as a ‘social resource’ which underpins anaesthesia providers’ practice, but which has not itself been extensively studied. The objective of this scoping review is to provide an overview of the literature related to communication among anaesthesia providers to meet clinical and professional goals, focusing on the modalities, contexts and purposes or outcomes of such communication, as well as which providers are involved. Methods: We conducted a scoping review using the JBI methodology to examine the current literature available, searching the Cochrane Database of Systematic Reviews and Cochrane Central Register of Controlled Trials, Medline, Embase, CINAHL and Google Scholar. Papers were eligible for inclusion where they primarily addressed the subject of communication between trained anaesthesia providers for any clinical or professional purpose (excluding purely social interactions). Data were charted for the location and cadre of providers represented, means of communication and the situation, purposes and outcomes of communication. Results: 3872 records were identified for screening, and 225 papers were ultimately included. Communication was reported both as a variable influencing a wide range of clinical and nonclinical outcomes and as an outcome in itself which might be modified by other factors. It was also considered in a smaller group of studies as a resource with varying availability to anaesthesia providers. Physician providers were well represented in included documents, but nurse anaesthetists, clinical officers and other nonphysician, nonnurse anaesthetists were far less commonly included. The majority of identified studies on communication between anaesthesia providers originated from and related to high-income countries. Conclusion: Communication between anaesthesia providers affects all aspects of their practice and has implications for both patient outcomes and workforce capacity. More research is necessary to understand how the availability of communication as a resource affects patient care and health worker well-being, particularly in low- and middle-income contexts and among nonphysician anaesthesia providers.
Carbene-activated stannylenes to access selective C(sp3)–H bond scission at the steric limit
The ubiquity of N-heterocyclic carbenes (NHCs) in diverse areas of chemical research typically arises from their potent stabilising capabilities and role as innocent spectators to stabilise otherwise non-bottleable compounds and complexes. This has, until now, been particularly true for NHC-stabilised stannylenes, with no exceptions reported thus far. Herein, we demonstrate that the combination of heteroleptic terphenyl-/amido-based stannylenes and tetra-alkyl substituted NHCs renders the corresponding NHC-ligated stannylenes highly reactive, yet isolable. In solution, this induces sterically controlled inter- and intramolecular C(sp3)–H bond scissions, resulting in the selective formation of stannylene metallocycles that depend on both the NHC source and the meta-terphenyl ligand coordinated to tin.
Flash Communication: Cyanophosphide Transfer Reactions
Cyanophosphides of the general form [RPCN]− can be viewed as cyanide adducts of phosphinidenes and are phosphorus species in the (+1)-oxidation state. We have recently reported on the stable cyanophosphide [DippTerPCN]K (DippTer = 2,6-Dipp2C6H3, Dipp = 2,6-iPrC6H3) and now investigate its reactions with ECln (E = Ge, n = 2; E = P, As, n = 3) in either salt metathesis or base-assisted dehydrohalogenation reactions. In the case of GeCl2, salt metathesis with [DippTerPCN]K afforded the dimer of a chlorogermylene [DippTerP(CN)GeCl]2. When only 0.5 equiv of GeCl2 was used, the diphosphanylgermylene [DippTerP(CN)]2Ge was generated in solution. With ECl3 (E = P, As), facile cyanophosphide transfer was achieved from DippTerP(H)CN in NEt3-assisted dehydrohalogenation, giving diphosphanes or arsaphosphanes of the type DippTerP(CN)ECl2 (E = P, As), respectively.
One-Step HF-Free Synthesis of Alkali Metal Fluorides from Fluorspar.
Alkali metal fluorides (MF) are commodity chemicals currently synthesized from naturally occurring fluorite (fluorspar, CaF2) in two steps: conversion of acid grade fluorspar (AGF) into highly hazardous hydrogen fluoride (HF) followed by neutralization with alkali metal hydroxides/carbonates. Herein, we report a one-step mechanochemical reaction that converts AGF into alkali metal fluorides under basic conditions, bypassing HF. The method consists of reacting AGF with alkali metal (hydr)oxides and titanium dioxide (TiO2) under mechanical energy for MF formation and in situ sequestration of calcium (hydr)oxide byproducts as calcium titanate (CaTiO3). Ca2+ sequestration prevents reversible CaF2 formation upon aqueous extraction, thus enabling the isolation of alkali metal fluorides. We also demonstrate that alkali metal titanates (M2TiO3) are suitable reagents for both CaF2 activation and Ca2+ sequestration, with K2TiO3 being optimal for KF synthesis.
Enantioconvergent nucleophilic substitution via synergistic phase-transfer catalysis.
Catalytic enantioconvergent nucleophilic substitution reactions of alkyl halides are highly valuable transformations, but they are notoriously difficult to implement. Specifically, nucleophilic fluorination is a renowned challenge, especially when inexpensive alkali metal fluorides are used as fluorinating reagents due to their low solubility, high hygroscopicity and Brønsted basicity. Here we report a solution by developing the concept of synergistic hydrogen bonding phase-transfer catalysis. Key to our strategy is the combination of a chiral bis-urea hydrogen bond donor (HBD) and an onium salt-two phase-transfer catalysts essential for the solubilization of potassium fluoride-as a well-characterized ternary HBD-onium fluoride complex. Mechanistic investigations indicate that this chiral ternary complex is capable of enantiodiscrimination of racemic benzylic bromides and α-bromoketones, and upon fluoride delivery affords fluorinated products in high yields and enantioselectivities. This work provides a foundation for enantioconvergent fluorination chemistry enabled through the combination of a HBD catalyst with a co-catalyst specifically curated to meet the requirement of the electrophile.
Phosphate-enabled mechanochemical PFAS destruction for fluoride reuse.
Perfluoroalkyl and polyfluoroalkyl substances (PFASs) are persistent, bioaccumulative and anthropogenic pollutants that have attracted the attention of the public and private sectors because of their adverse impact on human health1. Although various technologies have been deployed to degrade PFASs with a focus on non-polymeric functionalized compounds (perfluorooctanoic acid and perfluorooctanesulfonic acid)2-4, a general PFAS destruction method coupled with fluorine recovery for upcycling is highly desirable. Here we disclose a protocol that converts multiple classes of PFAS, including the fluoroplastics polytetrafluoroethylene and polyvinylidene fluoride, into high-value fluorochemicals. To achieve this, PFASs were reacted with potassium phosphate salts under solvent-free mechanochemical conditions, a mineralization process enabling fluorine recovery as KF and K2PO3F for fluorination chemistry. The phosphate salts can be recovered for reuse, implying no detrimental impact on the phosphorus cycle. Therefore, PFASs are not only destructible but can now contribute to a sustainable circular fluorine economy.
The Clinical Trial Landscape in Autoimmune Encephalitis: Challenges and Opportunities.
Autoimmune encephalitis (AE) is an important cause of neurologic morbidity and mortality. Treatment algorithms are primarily based on observational studies, retrospective series, and expert opinion. Despite clinical improvement with empiric therapy, recovery is often incomplete with a substantial burden of residual neurologic deficits and recurring symptoms. There is a pressing need for higher quality evidence-based therapies. However, designing and conducting clinical trials for patients with rare diseases such as AE has specific challenges, including slow recruitment, suboptimal outcome measures, and inclusivity vs exclusivity of the various disease subtypes. The anticipated knowledge gained from AE clinical trials emphasizes the need to overcome these challenges and support the development of the next generation of clinical trials. Yet, given these challenges, alternative approaches may be required. In this article, we review past and present clinical trials in AE with a focus on studies enrolling patients with neural surface antibodies. We discuss the potential challenges and opportunities inherent to clinical trials in rare diseases and provide an outlook for the field.
Increases in Arctic Extreme Climatic Events Are Linked to Negative Fitness Effects on the Local Biota.
The Arctic harbours uniquely adapted biodiversity and plays an important role in climate regulation. Strong warming trends in the terrestrial Arctic have been linked to an increase in aboveground biomass (Arctic greening) and community-wide shifts such as the northwards-expansion of boreal species (borealization). Whilst considerable efforts have been made to understand the effects of warming trends in average temperatures on Arctic biota, far fewer studies have focused on trends in extreme climate events and their biotic effects, which have been suggested to be particularly impactful during the Arctic winter months. Here, we present an analysis of trends in two ecologically relevant winter extreme events-extreme winter warming and rain-on-snow-followed by a meta-analysis on the evidence base for their effects on Arctic biota. We show a strong increase in extreme winter warming across the entire Arctic and high variability in rain-on-snow trends, with some regions recently experiencing rain-on-snow for the first time whilst others seeing a decrease in these events. Ultimately, both extreme events show significant changes in their characteristics and patterns of emergence. Our meta-analysis, encompassing 178 effect sizes across 17 studies and 49 species, demonstrates that extreme winter warming and rain-on-snow induce negative impacts on Arctic biota, with certain taxonomic groups-notably angiosperms and chordates (mostly vertebrates)-exhibiting higher sensitivity than others. Our study provides evidence for both emerging trends in Arctic winter extreme climate events and significant negative biotic effects of such events-which calls for attention to winter weather variability under climate change in the conservation of Arctic biodiversity, whilst highlighting important knowledge gaps.