bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evaluation and Management of Deficiency of Adenosine Deaminase 2
Authors: Lee Pui Y. , Davidson Brad A. , Abraham Roshini S. , Alter Blanche , Arostegui Juan I. , Bell Katherine , Belot Alexandre , Bergerson Jenna R. E. , Bernard Timothy J. , Brogan Paul A. , Berkun Yackov , Deuitch Natalie T. , Dimitrova Dimana , Georgin-Lavialle Sophie A. , Gattorno Marco , Grimbacher Bodo , Hashem Hasan , Hershfield Michael S. , Ichord Rebecca N. , Izawa Kazushi , Kanakry Jennifer A. , Khubchandani Raju P. , Klouwer Femke C.C. , Luton Evan A. , Man Ada W. , Meyts Isabelle , Van Montfrans Joris M. , Ozen Seza , Saarela Janna , Santo Gustavo C. , Sharma Aman , Soldatos Ariane , Sparks Rachel , Torgerson Troy R. , Uriarte Ignacio Leandro , Youngstein Taryn A. B. , Zhou Qing , Aksentijevich Ivona , Kastner Daniel L. , Chambers Eugene P. , Ombrello Amanda K. ,   DADA2 Foundation, Makley Mary K. , Hayner Kristen L. , Kling Bridget E. , Cowsert Lex M. , Williams Julie S. 
Published: May 2023

ImportanceDeficiency of adenosine deaminase 2 (DADA2) is a recessively inherited disease characterized by systemic vasculitis, early-onset stroke, bone marrow failure, and/or immunodeficiency affecting both children and adults. DADA2 is among the more common monogenic autoinflammatory diseases, with an estimate of more than 35 000 cases worldwide, but currently, there are no guidelines for diagnostic evaluation or management.ObjectiveTo review the available evidence and develop multidisciplinary consensus statements for the evaluation and management of DADA2.Evidence ReviewThe DADA2 Consensus Committee developed research questions based on data collected from the International Meetings on DADA2 organized by the DADA2 Foundation in 2016, 2018, and 2020. A comprehensive literature review was performed for articles published prior to 2022. Thirty-two consensus statements were generated using a modified Delphi process, and evidence was graded using the Oxford Center for Evidence-Based Medicine Levels of Evidence.FindingsThe DADA2 Consensus Committee, comprising 3 patient representatives and 35 international experts from 18 countries, developed consensus statements for (1) diagnostic testing, (2) screening, (3) clinical and laboratory evaluation, and (4) management of DADA2 based on disease phenotype. Additional consensus statements related to the evaluation and treatment of individuals with DADA2 who are presymptomatic and carriers were generated. Areas with insufficient evidence were identified, and questions for future research were outlined.Conclusions and RelevanceDADA2 is a potentially fatal disease that requires early diagnosis and treatment. By summarizing key evidence and expert opinions, these consensus statements provide a framework to facilitate diagnostic evaluation and management of DADA2.
Smoking Cessation and Changes in Anxiety and Depression in Adults With and Without Psychiatric Disorders
Authors: Wu Angela Difeng , Gao Min , Aveyard Paul , Taylor Gemma 
Published: May 2023

ImportanceAlthough many people report a desire to quit smoking, concerns about mental health worsening after quitting are often raised by clinicians and people who smoke.ObjectiveTo assess changes in mental health following smoking cessation using 3 confirmatory coprimary analytical approaches.Design, Setting, and ParticipantsThis cohort study was conducted using data from a large, randomized clinical trial, the Evaluating Adverse Events in a Global Smoking Cessation Study. Analytical approaches included multivariable Tobit regression, propensity score adjustment, and instrumental variable regressions conducted from August to October 2022. Missing data were imputed for sensitivity analysis. The trial occurred in 16 countries at 140 centers between 2011 and 2015. Only data from participants who completed the trial collected in the US were available for this secondary analysis. Participants included adults with or without a psychiatric disorder who smoked.ExposureSmoking abstinence between weeks 9 through 24.Main Outcomes and MeasuresAnxiety and depression scores were measured using the Hospital Anxiety and Depression Scale at 24 weeks, where a lower score indicates better mental health (range, 0-21).ResultsOf the 4260 participants included (mean [SD] age, 46.5 [12.4] years; 2485 women [58.3%]; 3044 White individuals [71.5%]), 2359 (55.4%) had a history of mental illness. The mean (SD) baseline Hospital Anxiety and Depression Scale score was 4.25 (3.68) (median [IQR], 3 [1-6]) for anxiety and 2.44 (2.91) (median [IQR], 1 [0-4]) for depression. After adjustment for demographics and baseline variables, smoking cessation was associated with a decrease in scores for both anxiety (−0.40 point; 95% CI, −0.58 to −0.22 point) and depression (−0.47 point; 95% CI, −0.61 to −0.33 point) compared with continuing smoking. Similarly, propensity score–adjusted models indicated that smoking cessation was associated with reduced scores for anxiety (β = −0.32; 95% CI, −0.53 to −0.11) and depression (β = −0.42; 95% CI, −0.60 to −0.24). Instrumental variable analysis was underpowered, and estimates were imprecise. Findings were robust to planned sensitivity and subgroup analyses, with larger effect sizes in people with a history of mental illness.Conclusions and RelevanceIn this cohort study of people with and without psychiatric disorders, smoking cessation, sustained for at least 15 weeks, was associated with improved mental health outcomes in observational analyses, but the instrumental variable analysis provided inconclusive evidence. Findings like these may reassure people who smoke and their clinicians that smoking cessation likely will not worsen and may improve mental health.
Standard Restrictions vs Expedited Activity After Pelvic Organ Prolapse Surgery
Authors: O’Shea Michele , Siddiqui Nazema Y. , Truong Tracy , Erkanli Alaattin , Barber Matthew D. 
Published: May 2023

ImportanceRestrictions on postoperative activity following pelvic organ prolapse (POP) surgery are not evidence based. Nonetheless, many pelvic surgeons place lifting and activity restrictions on patients following surgery.ObjectiveTo evaluate whether expedited activity results in noninferior anatomic and symptomatic outcomes compared with standard activity restrictions after POP surgery.Design, Setting, and ParticipantsThis randomized noninferiority clinical trial included patients undergoing vaginal or laparoscopic apical reconstructive surgery for POP between July 1, 2020, and October 31, 2021, at a single academic tertiary referral center in Durham, North Carolina. Anatomic outcomes were assessed by masked examiners, and subjective outcomes were assessed via validated surveys, both completed at 3 months postoperatively. Patients meeting minimum physical activity criteria with at least stage II bothersome POP were eligible. A total of 218 patients were approached, of whom 123 were randomly assigned and 107 had complete outcome data and were included in the analysis.InterventionsPatients were randomly assigned to receive standard restrictions vs expedited postoperative activity instructions.Main Outcomes and MeasuresThe anatomic coprimary outcome was maximum anatomic POP support loss (SLmax), which is the most distal point of pelvic organ support loss according to the Pelvic Organ Prolapse Quantification System (noninferiority margin, 1.0 cm). The symptomatic coprimary outcome was the Pelvic Organ Prolapse Distress Inventory (POPDI) symptom score (noninferiority margin, 34.3 points). Differences between outcomes were assessed using linear regression models controlling for baseline SLmax and POPDI, respectively.ResultsOf 123 participants randomized, 107 had complete 3-month outcome data and were included in the analysis. Mean (SD) age was 62.8 (10.1) years. At 3 months, mean (SD) SLmax was −1.7 (1.4) cm in the expedited group and −1.5 (1.4) cm in the standard group (P = .44). After adjusting for baseline SLmax, the mean maximum support loss was 0.18 cm higher within the vaginal canal in the expedited group (95% CI, −0.68 to 0.33 cm). The coprimary outcome of POPDI score was a mean (SD) 23.7 (41.8) points in the expedited group vs 25.7 (39.3) points in the standard group (P = .80). After adjusting for baseline scores, mean POPDI scores were 5.79 points lower in the expedited group (95% CI, −20.41 to 8.84).Conclusions and RelevanceThe findings demonstrate that expedited activity after prolapse surgery results in noninferior anatomic and symptomatic prolapse outcomes. It is reasonable to instruct patients undergoing minimally invasive prolapse surgery to resume physical activities ad lib postoperatively.Trial RegistrationClinicalTrials.gov Identifier: NCT04329715
Peptide Hydrogels as Immunomaterials and Their Use in Cancer Immunotherapy Delivery
Authors: Falcone Natashya , Ermis Menekse , Tamay Dilara Goksu , Mecwan Marvin , Monirizad Mahsa , Mathes Tess Grett , Jucaud Vadim , Choroomi Auveen , de Barros Natan Roberto , Zhu Yangzhi , Vrana Nihal Engin , Kraatz Heinz‐Bernhard , Kim Han‐Jun , Khademhosseini Ali 
Published: May 2023

AbstractPeptide‐based hydrogel biomaterials have emerged as an excellent strategy for immune system modulation. Peptide‐based hydrogels are supramolecular materials that self‐assemble into various nanostructures through various interactive forces (i.e., hydrogen bonding and hydrophobic interactions) and respond to microenvironmental stimuli (i.e., pH, temperature). While they have been reported in numerous biomedical applications, they have recently been deemed promising candidates to improve the efficacy of cancer immunotherapies and treatments. Immunotherapies seek to harness the body's immune system to preemptively protect against and treat various diseases, such as cancer. However, their low efficacy rates result in limited patient responses to treatment. Here, the immunomaterial's potential to improve these efficacy rates by either functioning as immune stimulators through direct immune system interactions and/or delivering a range of immune agents is highlighted. The chemical and physical properties of these peptide‐based materials that lead to immuno modulation and how one may design a system to achieve desired immune responses in a controllable manner are discussed. Works in the literature that reports peptide hydrogels as adjuvant systems and for the delivery of immunotherapies are highlighted. Finally, the future trends and possible developments based on peptide hydrogels for cancer immunotherapy applications are discussed.
Heterostructures Regulating Lithium Polysulfides for Advanced Lithium‐Sulfur Batteries
Authors: Wang Tao , He Jiarui , Zhu Zhi , Cheng Xin‐Bing , Zhu Jian , Lu Bingan , Wu Yuping 
Published: May 2023

AbstractSluggish reaction kinetics and severe shuttling effect of lithium polysulfides seriously hinder the development of lithium‐sulfur batteries. Heterostructures, due to unique properties, have congenital advantages that are difficult to be achieved by single‐component materials in regulating lithium polysulfides by efficient catalysis and strong adsorption to solve the problems of poor reaction kinetics and serious shuttling effect of lithium‐sulfur batteries. In this review, the principles of heterostructures expediting lithium polysulfides conversion and anchoring lithium polysulfides are detailedly analyzed, and the application of heterostructures as sulfur host, interlayer, and separator modifier to improve the performance of lithium‐sulfur batteries is systematically reviewed. Finally, the problems that need to be solved in the future study and application of heterostructures in lithium‐sulfur batteries are prospected. This review will provide a valuable reference for the development of heterostructures in advanced lithium‐sulfur batteries.
Highly Atom‐Economic and Efficient Electrochemical Selenylative Annulation of 2‐Alkynyl Biaryls
Authors: Mukherjee Nilanjana , Chatterjee Tanmay 
Published: May 2023

AbstractA catalyst‐ and oxidant‐free, highly efficient, scalable, and sustainable synthetic method is developed for the selenylative annulation of 2‐alkynyl biaryls or 2‐heteroaryl‐substituted alkynyl benzenes with readily available diselenides under electrochemical conditions to synthesize a wide variety of selanyl polycyclic aromatic hydrocarbons and polycyclic heteroaromatics in high to excellent yield up to 99% at room temperature in a short time (2‐5 h). The transformation required only electricity as a green reagent and produces hydrogen gas as the only innocuous byproduct. Notably, the green chemistry metrics of the protocol are found excellent. Mechanistic studies revealed a radical pathway being initiated by the in situ generation of the corresponding selenyl radical from diselenides under electrochemical conditions. Significantly, a direct comparison of the electrochemical approach with that of our previously developed iodine‐catalyzed chemical approach revealed that the electrochemical approach is not only catalyst‐ and oxidant‐free, but also more energy‐efficient, high‐yielding, sustainable, and practical.
A Fe Single Atom Seed‐Mediated Strategy Toward Fe3C/FeNC Catalysts with Outstanding Bifunctional ORR/OER Activities
Authors: Chang Jiangwei , Zhang Qi , Yu Jingkun , Jing Wen , Wang Siyang , Yin Guangchao , Waterhouse Geoffrey I. N. , Lu Siyu 
Published: May 2023

AbstractThe discovery of low‐cost and high‐performance bifunctional oxygen electrocatalysts is vital to the future commercialization of rechargeable zinc‐air batteries (ZABs). Herein, a Fe single atom seed‐mediated strategy is reported for the fabrication of Fe3C species closely surrounded by FeN4C active sites with strong electronic interactions built between them and more importantly, creating optimized coordination environment, via subtly adjusting their ratio, for favorable adsorption energies of oxygen intermediates formed during oxygen reduction reaction (ORR) and oxygen evolution reaction (OER). Concretely, the voltage difference (ΔE) between the ORR half‐wave and OER potential at a current density of 10 mA cm−2 for the compositionally‐optimized FeNC/Fe3C‐op electrocatalyst is only 0.668 V, endowing itself one of the best bifunctional OER/ORR benchmarks. As a demo, ZABs assembled with FeNC/Fe3C‐op as the air cathode deliver a remarkable specific capacity (818.1 mAh gZn−1) and a power density (1013.9 mWh gZn−1), along with excellent long‐term durability (>450 h). This work extends the methodology to modulate the activity of FeN4C atomic site, undoubtedly inspiring wide explorations on the precise design of bifunctional oxygen electrocatalysts.
Expert Panel Curation of 113 Primary Mitochondrial Disease Genes for the Leigh Syndrome Spectrum
Authors: McCormick Elizabeth M. , Keller Kierstin , Taylor Julie P. , Coffey Alison J. , Shen Lishuang , Krotoski Danuta , Harding Brian ,   NICHD ClinGen U24 Mitochondrial Disease Gene Curation Expert Panel, Gai Xiaowu , Falk Marni J. , Zolkipli‐Cunningham Zarazuela , Rahman Shamima 
Published: May 2023

ObjectivePrimary mitochondrial diseases (PMDs) are heterogeneous disorders caused by inherited mitochondrial dysfunction. Classically defined neuropathologically as subacute necrotizing encephalomyelopathy, Leigh syndrome spectrum (LSS) is the most frequent manifestation of PMD in children, but may also present in adults. A major challenge for accurate diagnosis of LSS in the genomic medicine era is establishing gene–disease relationships (GDRs) for this syndrome with >100 monogenic causes across both nuclear and mitochondrial genomes.MethodsThe Clinical Genome Resource (ClinGen) Mitochondrial Disease Gene Curation Expert Panel (GCEP), comprising 40 international PMD experts, met monthly for 4 years to review GDRs for LSS. The GCEP standardized gene curation for LSS by refining the phenotypic definition, modifying the ClinGen Gene–Disease Clinical Validity Curation Framework to improve interpretation for LSS, and establishing a scoring rubric for LSS.ResultsThe GDR with LSS across the nuclear and mitochondrial genomes was classified as definitive for 31 of 114 GDRs curated (27%), moderate for 38 (33%), limited for 43 (38%), and disputed for 2 (2%). Ninety genes were associated with autosomal recessive inheritance, 16 were maternally inherited, 5 were autosomal dominant, and 3 were X‐linked.InterpretationGDRs for LSS were established for genes across both nuclear and mitochondrial genomes. Establishing these GDRs will allow accurate variant interpretation, expedite genetic diagnosis of LSS, and facilitate precision medicine, multisystem organ surveillance, recurrence risk counseling, reproductive choice, natural history studies, and determination of eligibility for interventional clinical trials. ANN NEUROL 2023;94:696–712
Hidden Lives. Early Childhood Care as an Academic: The Slow Burn
Authors: Shenvi Ryan A. 
Published: May 2023

AbstractAs the inaugural entry in the new series Hidden Lives, this Viewpoint Article highlights challenges in early childhood care faced by academicians. Research centers must adapt to societal shifts in family structure, uncertainty around research funding, expanded job responsibilities and upheavals brought about by the pandemic. These problems represent opportunities for change at the technological, cultural and policy levels. It is crucial that we recognize those in need and help where we can.
Construction of Asymmetrical Dual Jahn–Teller Sites for Photocatalytic CO2 Reduction
Authors: Liu Huanhuan , Chen Yanxu , Li Huiyi , Wan Guanglin , Feng Yafei , Wang Wentao , Xiao Chong , Zhang Genqiang , Xie Yi 
Published: May 2023

AbstractPhotocatalytic CO2 reduction (PCR) expresses great attraction to convert useless greenhouse gas into valuable chemical feedstock. However, the weak interactions between catalytic sites and PCR intermediates constrains the PCR activity and selectivity. Herein, we proposed a new strategy to match the intermediates due to the maximum orbital overlap of catalytic sites and C1 intermediates by establishing dual Jahn–Teller (J–T) sites, in which, the strongly asymmetric J–T sites can break the nonpolar CO2 molecules and self‐adapt the different structure of C1 intermediates. Taking cobalt carbonate hydroxide as an example, the weakly symmetric dual cobalt (Co2) dual J–T sites, weakly asymmetric Fe&Co sites and strongly asymmetric Cu&Co sites were assembled. After illumination, the interaction between dual J–T sites and the CO2 molecules enhances J–T distortion, which further modulates the PCR activity and selectivity. As a result, the Cu&Co sites exhibited CO yield of 8137.9 μmol g−1, about 2.3‐fold and 4.2‐fold higher than that of the Fe&Co and Co2 sites within 5‐hour photoreaction, respectively. In addition, the selectivity achieved as high as 92.62 % than Fe&Co (88.67 %) and Co2 sites (55.33 %). This work provides a novel design concept for the construction of dual J–T sites to regulate the catalytic activity and selectivity.
Coupled Solar Battery with 6.9 % Efficiency
Authors: Jiao Lei , Zhang Xiang , Feng Yangyang , Lin Jing , Yuan Daqiang , Wang Yaobing 
Published: May 2023

AbstractSolar‐to‐electrochemical energy storage in solar batteries is an important solar utilization technology comparable to solar‐to‐electricity (solar cells) and solar‐to‐fuel (photocatalytic cells) conversion. Unlike the indirect approach of integrated solar flow batteries combining photoelectrodes with redox‐electrodes, coupled solar batteries enable direct solar energy storage, but are hampered by low efficiency due to rapid charge recombination of materials and misaligned energy levels between electrodes. Herein, we propose a design for a coupled solar battery that intercouples two photo‐coupled ion transfer (PCIT) reactions through electron‐ion transfer upon co‐photo‐pumping of photoelectrochemical storage cathode and anode. We used a representative covalent organic framework (COF) to achieve efficient charge separation and directional charge transfer between two band‐matched photoelectrochemical storage electrodes, with a photovoltage sufficient for COF dual‐redox reactions. By pumping these electrodes, the coupled solar battery stores solar energy via two synergistic PCIT reactions of electron‐proton‐relayed COF oxidation and reduction, and the stored solar energy is released as electrochemical energy during COF regeneration in discharge while interlocking the loops. A breakthrough in efficiency (6.9 %) was achieved, adaptive to a large‐area (56 cm2) tandem device. The presented photo‐intercoupled electron‐ion transfer (PIEIT) mechanism provides expandable paths toward practical solar‐to‐electrochemical energy storage.
Total Synthesis of Thiamyxins A–C and Thiamyxin E, a Potent Class of RNA‐Virus‐Inhibiting (Cyclo)depsipeptides
Authors: Bauer Kevin , Kazmaier Uli 
Published: May 2023

AbstractWe present the first total synthesis of the thiamyxins A–C and the now fully characterized thiamyxin E, an interesting class of thiazole‐ and thiazoline‐rich depsipeptides with diverse antiviral activity. The synthesis features a parallel closing of two methyl thiazoline units, with low epimerization of the very labile adjacent stereocenter. It also includes the three‐step synthesis of an uncommon hydroxy acid and the oxidation‐free elimination of a phenylselenide to form a dehydroalanine moiety. The exploitation of the acid‐labile stereocenter at the isoleucine moiety and the reopening of the macrolactones gave access to the four thiamyxins with good yields and diastereomeric purities from a single precursor. The modular total synthesis allows further testing of the biological activity and gives opportunities to explore the pharmacophore and antiviral target through derivatization.
Synergistic modification of short‐chain fluoro‐alcohol and siloxane end‐capping agents endowing an enhanced superhydrophobic waterborne polyurethane coating
Authors: Wang Bolin , Zhao Nana , Wu Jiawei , Pan Zhicheng , Zhao Cheng , Pan Mingwang 
Published: May 2023

AbstractWaterborne polyurethane (WPU) is an environmentally friendly product that can replace organic solvents with water. Still, its linear molecular chain structure and the presence of hydrophilic groups greatly affect water resistance and mechanical properties, limiting its wide application in the field of coatings. Herein, a series of fluorosilicone modified waterborne polyurethanes (FSiWPUs) were prepared by introducing tridecafluoro‐nonanol (TFN) and (3‐aminopropyl) triethoxysilane (APTES) into the ends of molecular chains of WPUs. These chains were constituted by different soft segments, including polybutylene adipate glycol, polycarbonate diol, or polytetramethylene ether glycol. Then fluorine element in TFN migrates and aggregates onto the surface during FSiWPU film‐forming to reduce the surface energy. Meanwhile, the hydrolytic condensation of APTES forms a Si–O–Si micro‐crosslinking structure. As a result, the two capping agents enable the modified FSiWPU to have better hydrophobicity, water resistance, and mechanical properties. The research results show that when the TFN:APTES molar ratio of capping agents introduced is 8:2, the static water contact angles of FSiWPUs with different soft sections reach 161.4°–165.3° and the maximum water absorption rate reduce to 8.9%–13.78%. Due to the simultaneous improvement of hydrophobic, water resistance, and mechanical properties, the modified FSiWPUs become the most promising candidates for superhydrophobic coatings.