bluuebunny/crossref_metadata_2025 huggingface dataset:
LAMA2‐related muscular dystrophy: Natural history of a large pediatric cohort
Authors: Zambon Alberto A. , Ridout Deborah , Main Marion , Mein Rachael , Phadke Rahul , Muntoni Francesco , Sarkozy Anna
Published: September 2020

Objective

To characterize natural history of Laminin‐α2 related muscular dystrophies (LAMA2‐RD) to help anticipating complications and identifying reliable outcome measures for clinical trial design and powering.

Methods

We conducted a retrospective, single‐center, cross‐sectional and longitudinal study on 46 LAMA2‐RD pediatric patients (37 families). Patients were seen at the Dubowitz Neuromuscular Centre, London between 1985 and 2019. Data were collected by case note reviews. Time‐to‐event analysis was performed to estimate median age at complications occurrence.

Results

Forty two patients had complete deficiency of Laminin‐α2 (CD) and four had partial deficiency (PD). Median age at first and last assessment was 2 years and 12.1 years, respectively. Median follow‐up length was 7.8 years (range 0‐18 years). Seven CD patients died at median age 12 years. One CD and two PD subjects achieved independent ambulation. We observed a linear increase in elbow flexor contractures in CD subjects. Thirty‐two CD and one PD patient developed scoliosis, nine underwent spinal surgery. Twenty‐two CD required nocturnal noninvasive ventilation (median age 11.7 years). CD subjects showed a 2.9% linear annual decline in forced vital capacity % predicted. Nineteen CD and one PD patient required gastrostomy insertion for failure to thrive and/or unsafe swallow (median age 10.9 years). Four CD patients had partial seizures. Mild left cardiac ventricular dysfunction and rhythm disturbances were identified in seven CD patients.

Interpretation

This retrospective longitudinal study provides long‐term natural history of LAMA2‐RD. This will help management and identification of key milestones of disease progression that could be considered for future therapeutic intervention.
Shatterproof partners to help consumers identify appropriate level of care
Authors: Enos Gary
Published: September 2020

Three prominent organizations have partnered on an initiative designed to give consumers more insight into the addiction treatment modality that could be most appropriate for them, before they make what could be a costly choice of a care provider. Leaders of the initiative indicate that the consumer tool could help balance the scales in a consumer search process that too often leads individuals to the highest‐intensity level of care.
3D Vascular Replicas Composed of Elastomer–Hydrogel Skin Multilayers for Simulation of Endovascular Intervention
Authors: Lim Jongkyeong , Kim Joonwon
Published: September 2020

Multilayered 3D vascular replicas incorporating the complementary advantages of elastomers and hydrogels can serve as a training platform to realistically simulate endovascular intervention, the preferred therapeutic procedure for cardio‐cerebrovascular disease. However, the fabrication process is challenging because of the difficulty in uniformly coating a thin hydrogel layer only on the inner surface of tortuous 3D vascular replicas composed of an elastomer monolayer. This study proposes an effective strategy to design and produce high‐fidelity 3D vascular replicas composed of elastomer–hydrogel multilayers by coating hydrogel polymers with robust interfaces only on the inner surface of the elastomer monolayer. The thin hydrogel layer can impart soft surface elasticity, lubricity, and superaerophobicity to the elastomer monolayer with high bulk elasticity, with deionized water alone as the circulating liquid, without the aid of additional lubricants. Owing to the complementary properties of the elastomers and hydrogels, multilayered 3D vascular replicas facilitate the control of medical devices and internal circulation of fluids, enabling realistic simulations of endovascular intervention under optical images in addition to X‐ray angiography. Furthermore, through practice courses, neurosurgeons demonstrated that the multilayered 3D vascular replicas are a reasonable platform for developing hand–eye coordination with pre‐procedure simulations and case‐specific training on demanding surgical sites.
Li Intercalation Effects on Interface Resistances of High‐Speed and Low‐Power WSe2 Field‐Effect Transistors
Authors: Shin Yong Seon , Lee Kiyoung , Duong Dinh Loc , Kim Jun Seok , Kang Won Tae , Kim Ji Eun , Won Ui Yeon , Lee Ilmin , Lee Hyangsook , Heo Jinseong , Lee Young Hee , Yu Woo Jong
Published: September 2020

Van der Waals (vdW) layered materials are promising channel materials for next‐generation field‐effect transistors (FETs). However, in vdW layered‐material FETs, the Schottky barrier and tunnel barrier at the vdW interfaces significantly reduce the electron injection efficiencies at electrical contacts, thereby limiting the development of high‐speed and low‐power FETs. This study demonstrates that intercalated lithium (Li) ions at vdW interfaces lower the Schottky and tunnel barriers at the electrical contacts of multilayer tungsten diselenide FETs owing to the low potential energy of Li and the mild electron doping from intercalation, thus decreasing the resistance at the vdW interfaces and enhancing the current flow and field‐effect mobility. Compared with before‐Li intercalation, the multilayer WSe2 planar FET demonstrates ≈1000 times higher current (IDS = 17.8 μA) at VGS = 50 V, ≈110 times higher maximum field‐effect mobility (μFE = 97.67 cm2 V−1 s−1), and ≈1000 times higher on/off current ratio (on/off ratio = ≈107). Furthermore, in multilayer WSe2 vertical FETs with vdW heterostructures having a structural strength of approximately the same as the current density, the intercalated Li ions at the graphene/WSe2 vdW interfaces additionally improves the current density and the vertical mobility by ≈20 times (1.00 A cm−2) and 10 times (2.52 × 10−4 cm2 V−1 s−1), respectively. This study establishes a method to reduce the resistance at vdW interfaces for developing high‐speed and low‐power multilayer vdW material FETs.
Blue Photosensitizer with Copper(II/I) Redox Mediator for Efficient and Stable Dye‐Sensitized Solar Cells
Authors: Ren Yameng , Flores‐Díaz Natalie , Zhang Dan , Cao Yiming , Decoppet Jean‐David , Fish George Cameron , Moser Jacques‐E. , Zakeeruddin Shaik Mohammed , Wang Peng , Hagfeldt Anders , Grätzel Michael
Published: September 2020

Dye‐sensitized solar cells (DSCs) based on copper(II/I) redox mediators have recently achieved notable performance progress through tailoring the ligand structure of the copper complexes, exploiting cosensitization, and introducing an advanced device structure. In order to further improve the power conversion efficiency (PCE), it is imperative to develop wide spectral‐response photosensitizers compatible with the copper(II/I) redox mediators while attaining a high Voc. Herein, a blue photosensitizer coded R7 is reported, which is purposely designed for highly efficient DSCs with copper‐based electrolytes. R7 features a strong electron‐donating segment of 9,19‐dihydrobenzo[1′,10′ ]phenanthro[3′,4′:4,5]thieno[3,2‐b]benzo[1,10]phenanthro[3,4‐d]thiophene conjugated with a bulky auxiliary donor N‐(2,4′‐bis(hexyloxy)‐[1,1′‐biphenyl]‐4‐yl)‐2,4′‐bis(hexyloxy)‐N‐methyl‐[1,1′‐biphenyl]‐4‐amine and the electron acceptor 4‐(7‐ethynylbenzo[c][1,2,5]thiadiazol‐4‐yl)benzoic acid. The blue dye R7 with bulkier auxiliary donor moiety in the DSC largely outperforms the reference dye R6, which has a smaller bis(4‐(hexyloxy)phenyl)amine donor unit. Transient absorption spectroscopy and electrochemical impedance spectroscopy measurements show that the R7 based DSC has a higher charge separation yield, a higher charge collection efficiency, and lower charge recombination with the copper electrolyte than the R6 based counterpart. A cosensitized system of R7 and Y123 for the DSC presents an outstanding PCE of 12.7% and retains 90% of its initial value after 1000 h of light soaking under 1 sun at 45 °C.
Molecular‐Scale Strategies to Achieve High Efficiency and Low Efficiency Roll‐off in Simplified Solution‐Processed Organic Light‐Emitting Diodes
Authors: Kim Young‐Hoon , Han Tae‐Hee , Lee Changsoo , Kim Yun‐Hi , Yang Yang , Lee Tae‐Woo
Published: September 2020

Solution‐processed small‐molecule organic light‐emitting diodes (OLEDs) are regarded as next‐generation flat‐panel displays and solid‐state lighting sources due to low material loss and a simple device fabrication process. However, they still suffer from low device efficiency and severe efficiency roll‐off. Here, molecular‐scale strategies are proposed to achieve highly efficient solution‐processed small‐molecule OLEDs with reduced efficiency roll‐off. By combining experiments with ab initio and molecular dynamics simulations, it is shown that an acetylacetonate group in a phosphorescent dopants lowers the dipole moment and molecular interaction energy of dopants, reducing dopant aggregation and increasing charge carrier transport. Furthermore, a charge‐balance assistant molecule is incorporated in the mixed‐host emitting layer to increase the balance of charge carrier transport and to broaden the exciton recombination zone in the center of the emitting layer. The resulting OLEDs have a current efficiency (CE) of 103.7 cd A−1, which is the highest yet reported in solution‐processed OLEDs, and low efficiency roll‐off (CE = 99.68 cd A−1 at a luminance LEL = 100 cd m−2, and CE = 75.00 cd A−1 at LEL = 1000 cd m−2) even with the simplified device architecture. It is expected that this strategy will advance the feasibility of commercialization of low‐cost high‐efficiency OLEDs.
Lateral Artificial Synapses on Hybrid Perovskite Platelets with Modulated Neuroplasticity
Authors: Gong Jiangdong , Yu Haiyang , Zhou Xin , Wei Huanhuan , Ma Mingxue , Han Hong , Zhang Shuo , Ni Yao , Li Yuelong , Xu Wentao
Published: September 2020

Polycrystalline organometal halide perovskite films have been recently exploited as the active layer in artificial synapses, demonstrating the basic functional emulation of biological synapses. However, for the implementation of neuromorphic computing and bioinspired intelligent systems, full synapse‐like functionality with a simple structure and extremely low energy consumption are of crucial importance. Here, a modified thickness‐confined surfactant‐assistant self‐assembly strategy is proposed to synthesize CH3NH3PbBr3 single‐crystalline thin platelets (SCTPs) and a two‐terminal lateral‐structured synaptic device with ultralow operating current down to sub‐pA is fabricated. Essential synaptic behaviors are realized, including paired‐pulse facilitation, spike‐dependent plasticity, transition from sensory memory to short‐term memory and potentiation/depression. Furthermore, the activity‐dependent plasticity is also demonstrated on the SCTP‐based artificial synapse, which may enable nociceptors to detect intense external harm. These results provide a new protocol for designing lateral‐structured synaptic devices based on hybrid perovskite SCTPs and future neuromorphic bioelectronics.
Bioactive Self‐Pumping Composite Wound Dressings with Micropore Array Modified Janus Membrane for Enhanced Diabetic Wound Healing
Authors: Bao Feng , Pei Ge , Wu Zhongcao , Zhuang Hui , Zhang Zhaowenbin , Huan Zhiguang , Wu Chengtie , Chang Jiang
Published: September 2020

Treatment of chronic wounds such as diabetic wounds with large volumes of biofluids is a significant clinical challenge due to ischemia caused by localized edema. To alleviate edema and stimulate angiogenesis, a four‐layer composite dressing is designed with a micropore array modified Janus membrane for self‐pumping and bioactive ion backflow, a superabsorbent layer for high capacity water absorption, and a bioactive layer containing bioglass for stimulating angiogenesis. The modified Janus membrane not only allows wound exudates transport from wound bed to the dressing, but also enables controlled backflow of bioactive ion containing fluid to the wound bed for stimulating angiogenesis. The in vivo experiment confirms the function of composite dressing in reducing wound edema, stimulating blood vessel formation and promoting diabetic wound healing. These results demonstrate that the novel composite dressing is a promising wound healing biomaterial for promoting chronic wound healing, and the unique characteristics of the micropore arrayed Janus membrane may extend its applications in biomedicine.
Influence of Heavy Atom Effect on the Photophysics of Coinage Metal Carbene‐Metal‐Amide Emitters
Authors: Feng Jiale , Reponen Antti‐Pekka M. , Romanov Alexander S. , Linnolahti Mikko , Bochmann Manfred , Greenham Neil C. , Penfold Thomas , Credgington Dan
Published: September 2020

The effect of the heavy metal atom on the photophysics of carbene‐metal‐amide (CMA) photoemitters is explored, where the metal bridge is either Au, Ag, or Cu. Spectroscopic investigations reveal the coupling mechanism responsible for communication between the singlet and triplet manifolds. The photophysical properties do not reflect expected trends based upon the heavy atom effect, as both direct coupling between charge‐transfer states and spin‐vibronic coupling via a ligand‐centered triplet state are present. Direct coupling is weakest for CMA(Ag), increasing the importance of the spin‐vibronic pathway and rendering its properties more sensitive to inter‐state energy gaps than for the Au and Cu‐bridged analogues. The measured activation energy correlates with the expected exchange energy of the charge‐transfer state, which is also closely related to the length of the bonds joining the carbene and amide ligands, and decreases in the order CMA(Cu) > CMA(Au) > CMA(Ag). These findings reveal that reducing interference between charge‐transfer and ligand‐centers excited, and minimizing exchange energy, are required for developing efficient luminescent CMA complexes.
Metal Soap Membranes for Gas Separation
Authors: Liu Qi , Babu Deepu J. , Hao Jian , Vahdat Mohammad Tohidi , Campi Davide , Agrawal Kumar Varoon
Published: September 2020

Metal soaps or metal alkanoates are metal–organic complexes held together with metal cations and the functional groups of hydrocarbon chains. They can be synthesized at a high yield by simply mixing the metal and organic sources, forming crystalline frameworks with diverse topology, and have been studied in the past because of their rich polymorphism‐like liquid crystals. Their ability to melt while retaining the crystalline properties upon cooling is unique among nanoporous materials and is especially attractive for membrane fabrication. Herein, metal soaps as a new class of material for molecular separation are reported. Three metal soaps, Ca(SO4C12H25)2, Zn(COOC6H13)2, and Cu(COOC9H19)2, hosting lamellar structure with molecular‐sized channels are synthesized. They are processed in thin, intergrown, polycrystalline films on porous substrates by two scalable methods, interfacial crystallization and melting with an extremely small processing time (a minute to an hour). The resulting crystalline films are oriented with the alkyl chains perpendicular to the porous substrate which favors molecular transport. The prepared membranes demonstrate attractive gas separation behavior, e.g., 300‐nm‐thick Ca(SO4C12H25)2 membrane prepared in a minute using interfacial crystallization yields H2 permeance of 6.1 × 10−7 mol m−2 s−1 Pa−1 with H2/CO2 selectivity of 10.5.
Nanoarchitectured Design of Vertical‐Standing Arrays for Supercapacitors: Progress, Challenges, and Perspectives
Authors: Qi Junlei , Yan Yaotian , Cai Yifei , Cao Jian , Feng Jicai
Published: September 2020

Vertical‐standing arrays have aroused great enthusiasm as electrode materials for supercapacitors in recent years owing to their structural and compositional characteristics. Although significant efforts have been made in the construction of vertical‐standing arrays with tailored compositions and architectures, an in‐depth understanding of the relevant structure–activity relationships has not yet been reviewed in detail. Herein, recent important progress in controllably synthesizing vertical‐standing arrays as well as their application as supercapacitors is reviewed. Afterward, promising strategies to improve the electrochemical performance of vertical‐standing arrays are discussed. Finally, the challenges and possible directions for developing vertical‐standing arrays with outstanding performance are outlined. This review provides important guidelines for designing and regulating vertical‐standing arrays and constructing desirable electrode materials for future electrochemical energy storage.
Advanced Materials and Systems for Biodegradable, Transient Electronics
Authors: Han Won Bae , Lee Joong Hoon , Shin Jeong‐Woong , Hwang Suk‐Won
Published: September 2020

Transient electronics refers to an emerging class of advanced technology, defined by an ability to chemically or physically dissolve, disintegrate, and degrade in actively or passively controlled fashions to leave environmentally and physiologically harmless by‐products in environments, particularly in bio‐fluids or aqueous solutions. The unusual properties that are opposite to operational modes in conventional electronics for a nearly infinite time frame offer unprecedented opportunities in research areas of eco‐friendly electronics, temporary biomedical implants, data‐secure hardware systems, and others. This review highlights the developments of transient electronics, including materials, manufacturing strategies, electronic components, and transient kinetics, along with various potential applications.
Vanadium‐Based Materials as Positive Electrode for Aqueous Zinc‐Ion Batteries
Authors: Fan Lin , Ru Yue , Xue Huaiguo , Pang Huan , Xu Qiang
Published: September 2020

With the increasing dependence on high power equipment, there is an urgent need to develop advanced and sustainable energy systems. As one of the main energy storage devices, battery research should make great efforts to implement the sustainable development strategies on ecological, clean and environmentally friendly energy. Currently, aqueous zinc ion batteries (ZIBs) have gained much attention owing to their cheapness, abundant resources, high safety, and ecological friendliness. Nevertheless, ZIBs also have shortcomings with respect to the positive electrode. Therefore, the development of satisfactory positive electrode materials with structural stability and high capacity has become the focus of attention. In recent years, many vanadium‐based compounds have been strongly upheld for their advantages of safety and high theoretical capacity. In this paper, different energy storage mechanisms of vanadium‐based positive electrodes are summarized. Typical structures, such as layered and tunnel types, are particularly emphasized. Moreover, the comparison and analysis of electrochemical results of vanadium‐based compounds, including vanadium oxide and metal vanadate are focused. Finally, the strategies for improving aqueous ZIBs and development prospects are also discussed.
Estimating COVID‐19 Impacts on the Broiler Industry
Authors: Maples Joshua G. , Thompson Jada M. , Anderson John D. , Anderson David P.
Published: September 2020

The COVID‐19 pandemic created massive disruptions in meat supply chains including the US poultry industry. Detrimental impacts to chicken demand due to restaurant closures led to supply adjustments affecting broiler producers. The integrated structure of the broiler industry creates significant challenges in estimating grower losses. Farmers who raise broiler chickens do not own the birds and price drops are not directly felt in the same manner as in other commodities. This article examines the broiler industry and presents methodology to better understand the farm‐level impacts of COVID‐19. Further, this work is useful for policy response considerations for broiler growers.
A New Pathway for Liquid–Solid Triboelectric Nanogenerator Using Streaming Flow by a Novel Direct Charge Transfer
Authors: Nam Gyu Hyeon , Ahn Jee Hwan , Lee Gang Hee , Vo Cong Phat , Ahn Kyoung Kwan
Published: September 2020

Herein, a new triboelectric energy‐harvesting method by a direct transfer‐triboelectric nanogenerator (DT‐TENG) in a streaming flow is proposed. Previously, most of the fluid‐based‐triboelectric nanogenerators (Flu‐TENGs) generate the electricity by electrostatic induction, which is one of the indirect charge transfer methods. To enhance the triboelectric effect of liquid from the surface of dielectric material, negative dielectric surface treatment is focused. The significance of a novel direct charge transfer method to generate high output power of Flu‐TENG is first reported herein. From the experiment, the maximum power of DT‐TENG reaches up to 28.4 μW. The findings not only enhance the output power in the streaming flow but also provide a paradigm‐shifting technique to harvest mechanical energy in the flow electrification.
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk‐stratification and management
Authors: Tefferi Ayalew , Barbui Tiziano
Published: September 2020

Disease overview

Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms (MPN) respectively characterized by clonal erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus and risk of leukemic or fibrotic transformation.

Diagnosis

Bone marrow morphology remains the cornerstone of diagnosis. In addition, the presence of JAK2 mutation is expected in PV while approximately 90% of patients with ET express mutually exclusive JAK2, CALR or MPL mutations (so called driver mutations). In ET, it is most important to exclude the possibility of prefibrotic myelofibrosis.

Survival

Median survivals are approximately 15 years for PV and 18 years for ET; the corresponding values for patients age 40 or younger were 37 and 35 years. Certain mutations (mostly spliceosome) and abnormal karyotype might compromise survival in PV and ET. Life‐expectancy in ET is inferior to the control population. Driver mutations have not been shown to affect survival in ET but risk of thrombosis is higher in JAK2 mutated cases. Leukemic transformation rates at 10 years are estimated at <1% for ET and 3% for PV.

Thrombosis risk

In PV, two risk categories are considered: high (age > 60 years or thrombosis history present) and low (absence of both risk factors). In ET, four risk categories are considered: very low (age ≤ 60 years, no thrombosis history, JAK2 wild‐type), low (same as very low but JAK2 mutation present), intermediate (age > 60 years, no thrombosis history, JAK2 wild‐type) and high (thrombosis history present or age > 60 years with JAK2 mutation).

Risk‐adapted therapy

The main goal of therapy in both PV and ET is to prevent thrombohemorrhagic complications. All patients with PV require phlebotomy to keep hematocrit below 45% and once‐daily or twice‐daily aspirin (81 mg), in the absence of contraindications. Very low risk ET might not require therapy while aspirin therapy is advised for low risk disease. Cytoreductive therapy is recommended for high‐risk ET and PV, but it is not mandatory for intermediate‐risk ET. First‐line drug of choice for cytoreductive therapy, in both ET and PV, is hydroxyurea and second‐line drugs of choice are interferon‐α and busulfan. We do not recommend treatment with ruxolutinib in PV, unless in the presence of severe and protracted pruritus or marked splenomegaly that is not responding to the aforementioned drugs.

New treatment directions

Controlled studies are needed to confirm the clinical outcome value of twice‐daily vs once‐daily aspirin dosing and the therapeutic role of pegylated interferons and direct oral anticoagulants.
Asthma onset after exposure to fluorinated hydrocarbons in the presence of combustion
Authors: Lindström Irmeli , Ryhänen Anna‐Mari , Jungewelter Soile , Suojalehto Hille , Suuronen Katri
Published: September 2020

Fluorinated hydrocarbons, which can thermally degrade into toxic hydrofluoric acid, are widely used as, for example, cooling agents in refrigerators and air conditioning systems and as medical aerosol propellants. Hydrofluoric acid is a known causative agent of irritant‐induced asthma. We report on two patients with asthma initiation shortly after exposure to fluorinated hydrocarbon‐based cooling agents while welding or smoking cigarettes in a confined space. Both cases developed respiratory symptoms and headache and later demonstrated nonspecific bronchial hyperresponsiveness. In follow‐up, asthma was persistent and responded poorly to asthma medication. Exposure to the fluorinated hydrocarbons themselves is unlikely to have caused asthma due to their low toxicity. Instead, exposure to small amounts of hydrofluoric acid via the thermal degradation of the fluorinated hydrocarbons was considered the most likely cause of asthma onset. This is supported by the typical clinical picture of irritant‐induced asthma and acute symptoms resembling hydrofluoric acid poisoning. When fluorinated hydrocarbons are used in the presence of combustion, thermal degradation may lead to the formation of hydrofluoric acid. In confined spaces, this exposure may induce asthma via irritation. Welding, smoking, and other sources of combustion in confined spaces may be a risk in workplaces and other places in which fluorinated hydrocarbons are used.
Caput membranaceum: A novel clinical presentation of ZIC1 related skull malformation and craniosynostosis
Authors: Sasaki Erina , Byrne Angela T. , Murray Dylan J. , Reardon William
Published: September 2020

We report clinical and radiological features of a patient born with an isolated skull malformation of caput membranaceum and partial bicoronal craniosynostosis with a novel, de novo heterozygous missense variant in ZIC1 [NM_003412.3:c.1183C>G, p.(Pro395Ala)]. Caput membranaceum, or boneless skull, is a rare manifestation of skull ossification defect. It can result from an isolated, enlarged parietal foramina or it can present as part of skeletal dysplasia syndromes associated with poor mineralization such as hypophosphatasia, osteogenesis imperfecta type II, and Saethre‐Chotzen syndrome. Their causative genes are well described. ZIC1, Zinc Finger protein of the cerebellum 1 (OMIM #600470) belongs to ZIC family genes, each encoding a Cys2 His2‐type zinc finger domain‐containing transcription factors. Recent studies have shown that pathogenic variants in ZIC1 have deleterious effect in developing human central nerves system and skull bone. ZIC1 related clinical conditions are reported and include cerebellum malformation, Dandy‐Walker malformation, spinal dysraphism, microcephaly, and craniosynostosis with associated intellectual disability. To‐date, there is no report of pathogenic variant in ZIC1 causing isolated caput membranaceum. Our observation adds to the clinical spectrum of ZIC1 related skull malformation.
Effects of complement gene‐set polygenic risk score on brain volume and cortical measures in patients with psychotic disorders and healthy controls
Authors: Holland Jessica F. , Cosgrove Donna , Whitton Laura , Harold Denise , Corvin Aiden , Gill Michael , Mothersill David O. , Morris Derek W. , Donohoe Gary
Published: September 2020

Multiple genome‐wide association studies of schizophrenia have reported associations between genetic variants within the MHC region and disease risk, an association that has been partially accounted for by alleles of the complement component 4 (C4) gene. Following on previous findings of association between both C4 and other complement‐related variants and memory function, we tested the hypothesis that polygenic scores calculated based on identified schizophrenia risk alleles within the “complement” system would be broadly associated with memory function and associated brain structure. We tested this using a polygenic risk score (PRS) calculated for complement genes, but excluding C4 variants. Higher complement‐based PRS scores were observed to be associated with lower memory scores for the sample as a whole (N = 620, F change = 8.25; p = .004). A significant association between higher PRS and lower hippocampal volume was also observed (N = 216, R2 change = 0.016, p = .015). However, after correcting for further testing of association with the more general indices of cortical thickness, surface area or total brain volume, none of which were associated with complement, the association with hippocampal volume became non‐significant. A post‐hoc analysis of hippocampal subfields suggested an association between complement PRS and several hippocampal subfields, findings that appeared to be particularly driven by the patient sample. In conclusion, our study yielded suggestive evidence of association between complement‐based schizophrenia PRS and variation in memory function and hippocampal volume.
Novel marine‐based gold nanocatalyst in solvent‐free synthesis of polyhydroquinoline derivatives: Green and sustainable protocol
Authors: Sepahvand Mahsa , Buazar Foad , Sayahi Mohammad Hosein
Published: September 2020

We report an ecofriendly synthetic approach for the fabrication of biogenic gold nanoparticles (AuNPs) using electron‐rich sea cucumber extract as a bio‐reductant and stabilizing agent in reducing gold cations into AuNPs at the optimal conditions. The produced AuNPs are spherical in shape with an average particle size of 11 ± 1.5 nm in transmission electron microscopy (TEM) and exhibited a crystal structure of face‐centered cubic in X‐ray diffraction (XRD) analyses. Our results indicated that bioinspired AuNPs demonstrate superior catalytic activity in the safe and facile one‐pot synthesis of polyhydroquinoline derivatives under solvent‐free reaction conditions. This green route encompasses multiple benefits including highly recyclable bioinspired catalyst (5 cycles), short reaction times, convenient workout, high to excellent product yields (82%–97%), and nonhazardous conditions.
The influence of size and healing content on the performance of extrinsic self‐healing coatings
Authors: Malekkhouyan Roya , Neisiany Rasoul Esmaeely , Khorasani Saied Nouri , Das Oisik , Berto Filippo , Ramakrishna Seeram
Published: September 2020

Among the several approaches for the protection of metallic structures from corrosion, covering with a polymeric coating has attracted more attention due to their convenient application, cost‐effective price, and the relatively benign environmental impact. However, the polymeric coatings are sensitive to mechanical/thermal shocks and aggressive environments, leading to damages in the coatings that affect their barrier performance. Self‐healing polymeric coatings have introduced remarkable development by extending the service life and reducing maintenance costs, leading to a significant boost in the reliability and durability of the conventional polymeric coatings. Among the different strategies to develop self‐polymeric coatings, encapsulating healing agent within micro/nanocapsules, micro/nanofibers, and microvascular systems and incorporating them within the conventional coatings have been widely acknowledged as the most applicable approach. However, several factors, such as the effect of the healing system's size and content, have a significant influence on healing performance. Therefore, this review aims to reveal the effects of healing system size and healing content on the self‐healing performance in polymeric coatings through the analysis of recently published articles.
WorldFlora: An R package for exact and fuzzy matching of plant names against the World Flora Online taxonomic backbone data
Authors: Kindt Roeland
Published: September 2020

Premise

The standardization of plant names is a critical step in various fields of biology, including biodiversity, biogeography, and vegetation research. The WorldFlora package is introduced here to help achieve this goal by matching lists of plant names with a static copy from World Flora Online (WFO), an ongoing global effort to complete an online flora of all known vascular plants and bryophytes by 2020.

Methods and Results

Based on direct and fuzzy matching, WorldFlora inserts matching cases from the WFO to a submitted data set containing taxonomic names. The results and success rates for selecting the expected best single matches are presented for four data sets, including two data sets used in recent comparisons of software tools for correcting taxon names.

Conclusions

WorldFlora offers a straightforward pipeline for semi‐automatic plant name checking. For the four data sets, the success rate of credible matches ranged from 94.7% to 99.9%.
From Ag2Zr(IO3)6 to LaZr(IO3)5F2: A Case of Constructing Wide‐band‐gap Birefringent Materials through Chemical Cosubstitution
Authors: Mao Feifei , Liu Jiadi , Hu Jinyu , Wu Hua
Published: September 2020

Two mixed‐metal zirconium iodates were prepared and studied as a case of chemical cosubstitution. The structure of Ag2Zr(IO3)6 (P21/c) features 0D [Zr(IO3)6]2− anion group and 1D [Ag(IO3)2]− anionic chain, with the [Zr(IO3)6]2− anion groups interconnected by Ag+ ions into 3D network; LaZr(IO3)5F2 (P21/n) features 0D [Zr(IO3)5F2]3− anion group and 2D [La(IO3)5]2− anionic layer, with the [Zr(IO3)5F2]3− groups interlinked by La3+ ions into 3D structure. Notably, LaZr(IO3)5F2 is the first zirconium iodate fluoride reported. Wide optical band gaps of 3.77 and 4.13 eV are given for Ag2Zr(IO3)6 and LaZr(IO3)5F2, respectively. Theoretical calculations confirmed that the weak d–d transition of Zr4+ in the band structure leads to a moderate band gap of Ag2Zr(IO3)6, and the introduction of F− into the zirconium iodate compound results in a large band gap of LaZr(IO3)5F2. Both of the compounds are birefringent materials with birefringences of 0.064 @1064 nm and 0.082 @1064 nm, respectively.
Unified Total Synthesis of (−)‐Enigmazole A and (−)‐15‐O‐Methylenigmazole A
Authors: Sakurai Keisuke , Sakamoto Keita , Sasaki Makoto , Fuwa Haruhiko
Published: September 2020

The total synthesis of cytotoxic marine phosphomacrolides, (−)‐enigmazole A and (−)‐15‐O‐methylenigmazole A, is described in detail. The 2,6‐cis‐substituted tetrahydropyran ring was efficiently elaborated by using a tandem olefin cross‐metathesis/intramolecular oxa‐Michael addition reaction. The 18‐membered macrolactone skeleton was forged via a Au‐catalyzed propargylic benzoate rearrangement/macrocyclic ring‐closing metathesis sequence. Late‐stage diversification of a common intermediate enabled unified total synthesis of (−)‐enigmazole A and (−)‐15‐O‐methylenigmazole A.
Exploring the Event‐Related Potentials' Time Course of Associative Recognition in Autism
Authors: Desaunay Pierre , Clochon Patrice , Doidy Franck , Lambrechts Anna , Wantzen Prany , Wallois Fabrice , Mahmoudzadeh Mahdi , Guile Jean‐Marc , Guénolé Fabian , Baleyte Jean‐Marc , Eustache Francis , Bowler Dermot M. , Guillery‐Girard Bérengère
Published: September 2020

Behavioral data on episodic recollection in autism spectrum disorders (ASD) point limited relational memory functioning. However, the involvement of successive memory processes in the profile of episodic memory in ASD needs more study. Here, we used event‐related potentials (ERP) to investigate the time course of episodic recollection with an associative recognition paradigm with picture pairs. Twenty‐two participants with ASD and 32 with typical development (TD), all right‐handed, were included. Behavioral results confirmed difficulties in correctly recognizing identical pairs in the ASD relative to TD group. We found an unexpected amplitude decrement on the P2 (220–270 msec) and FN400 (350–470 msec) potentials, suggesting diminished priming and familiarity effects in the ASD relative to TD group. However, ERP data revealed that the recognition of associative information relies on the same electrophysiological process (old/new effect in the 600–700‐msec late positive component) in ASD participants as in TD ones, with a parietal extension in the ASD group. These results suggest that the electrophysiological processes of associative recognition are qualitatively similar in individuals with and without ASD but may differ quantitatively. This difference may be driven by the reduced early processing of picture pairs that may in turn lead to their diminished integration into the semantic memory system, being partially compensated by a greater involvement of associative memory during the recollection process. Other studies would be useful to go further in identifying these cognitive processes involved in atypical recognition in ASD and their neural substrates. Autism Res 2020, 13: 1998‐2016. © 2020 International Society for Autism Research and Wiley Periodicals LLC

Lay Summary

We identified diminished performance on the associative recognition of picture pairs in adolescents and young adults with autism when compared to typical development. Electrophysiological data revealed qualitative similarities but quantitative differences between‐group, with diminished priming and familiarity processes partially compensated by an enhanced parietal recollection process.
Localized Sampling Enables Monitoring of Cell State via Inline Electrospray Ionization Mass Spectrometry
Authors: Chilmonczyk Mason A. , Doron Gilad , Kottke Peter A. , Culberson Austin L. , Leguineche Kelly , Guldberg Robert E. , Horwitz Edwin M. , Fedorov Andrei G.
Published: September 2020

Nascent advanced therapies, including regenerative medicine and cell and gene therapies, rely on the production of cells in bioreactors that are highly heterogeneous in both space and time. Unfortunately, advanced therapies have failed to reach a wide patient population due to unreliable manufacturing processes that result in batch variability and cost prohibitive production. This can be attributed largely to a void in existing process analytical technologies (PATs) capable of characterizing the secreted critical quality attribute (CQA) biomolecules that correlate with the final product quality. The Dynamic Sampling Platform (DSP) is a PAT for cell bioreactor monitoring that can be coupled to a suite of sensor techniques to provide real‐time feedback on spatial and temporal CQA content in situ. In this study, DSP is coupled with electrospray ionization mass spectrometry and direct‐from‐culture sampling to obtain measures of CQA content in bulk media and the cell microenvironment throughout the entire cell culture process (≈3 weeks). Post hoc analysis of this real‐time data reveals that sampling from the microenvironment enables cell state monitoring (e.g., confluence, differentiation). These results demonstrate that an effective PAT should incorporate both spatial and temporal resolution to serve as an effective input for feedback control in biomanufacturing.
Peripheral versus central mechanisms of the cannabinoid type 2 receptor agonist AM1710 in a mouse model of neuropathic pain
Authors: Wilkerson Jenny L. , Alberti Lauren B. , Kerwin Audra A. , Ledent Catherine A. , Thakur Ganesh A. , Makriyannis Alexandros , Milligan Erin D.
Published: September 2020

The CB2R agonist AM1710, examined in animal models of peripheral neuropathy, is effective in controlling aberrant light touch sensitivity, referred to as mechanical allodynia. However, nonspecific binding of AM1710 to CB1R, either peripherally or centrally, could be partially responsible for the analgesic effects of AM1710. Thus, we sought to determine in mice whether spinal (intrathecal; i.t.) or peripheral AM1710 administration could lead to anti‐allodynia by reducing the protein expression of spinal and dorsal root ganglia (DRG) proinflammatory cytokines and elevating the anti‐inflammatory cytokine interleukin‐10 (IL‐10) in the absence of CB1R. Macrophage cell cultures were examined to characterize AM1710‐mediated suppression of the proinflammatory cytokine tumor necrosis factor‐alpha (TNF‐α). Either i.p. or i.t. AM1710 reversed CCI‐induced mechanical allodynia to sham levels in CB1R (−/−), (+/−), (+/+) mice. CCI‐induced neuropathy decreased IL‐10 immunoreactivity (IR) in the dorsal root ganglia (DRG) and the dorsal horn of the spinal cord, with i.t. AM1710 restoring basal IL‐10 IR. CCI‐induced elevations in proinflammatory cytokine IR were decreased within the spinal cord only after i.t. AM1710 in all mouse genotypes. Meanwhile, within DRG tissue from neuropathic mice, proinflammatory cytokines were decreased following either i.p. or i.t. AM1710. Analysis of cultured supernatants revealed AM1710 decreased TNF‐alpha protein. We conclude that CB1R is dispensable for either peripheral or central anti‐allodynic actions of AM1710 in neuropathic mice. Cannabinoid CB2R agonists produce heightened spinal IL‐10 which may be clinically relevant to successfully treat neuropathic pain.
BUB1B promotes hepatocellular carcinoma progression via activation of the mTORC1 signaling pathway
Authors: Qiu Jiannan , Zhang Shaopeng , Wang Peng , Wang Hao , Sha Bowen , Peng Hao , Ju Zheng , Rao Jianhua , Lu Ling
Published: September 2020

Background and Aims

Accumulating studies identified that BUB1 mitotic checkpoint serine/threonine kinase B (BUB1B) is integrally involved in the initiation and development of tumors. Nevertheless, the precise biological role and underlying mechanisms of BUB1B in hepatocellular carcinoma (HCC) remain indistinct.

Method

To figure out the role of BUB1B in HCC, we first assessed its expression using The Cancer Genome Atlas (TCGA) and Gene Expression Profiling Interactive Analysis (GEPIA) databases. We then verified BUB1B expression in HCC tissues, nontumor tissues, and HCC cell lines through western blotting, quantitative reverse transcription‐polymerase chain reaction, and immunohistochemistry. To explore the specific function of BUB1B in HCC in vivo and in vitro, we performed the flow cytometry, Cell Counting Kit‐8, 5‐ethynyl‐2′‐deoxyuridine incorporation, colony formation, Transwell, wound‐healing, subcutaneous tumor growth, and metastasis assays. Additionally, we identified the BUB1B‐regulated pathways involved in HCC by using gene set enrichment analysis.

Results

Our data displayed that higher BUB1B expression was detected in HCC tissues and HCC cell lines. The overexpression of BUB1B was positively correlated with adverse clinicopathological characteristics. Survival analyses showed that lower recurrence‐free and overall survival rates were correlated with the overexpression of BUB1B in patients with HCC. Moreover, the malignancy of HCC was facilitated by BUB1B both in vivo and in vitro. Lastly, the results were confirmed by western blots, which showed that BUB1B upregulated mTORC1 signaling pathway in HCC. Meanwhile, the oncogenic effect of BUB1B will be impaired when the mTORC1 signaling pathway was inhibited by rapamycin.

Conclusion

We highlighted that BUB1B played an oncogenic role in HCC and was identified as a possible clinical prognostic factor and a potential novel therapeutic target for HCC.
Utility of a multigene testing for preoperative evaluation of indeterminate thyroid nodules: A prospective blinded single center study in China
Authors: Song Yuntao , Xu Guohui , Ma Tonghui , Zhu Yanli , Yu Hao , Yu Wenbin , Wei Wei , Wang Tianxiao , Zhang Bin
Published: September 2020

Background

Thyroid nodules are highly prevalent, with fine‐needle aspiration (FNA) commonly used as the standard preoperative tool for their diagnosis. However, the method classifies some of the samples as indeterminate, leading to unnecessary surgery. In this study, we evaluated the value of next‐generation sequencing (NGS) for cancer diagnosis in indeterminate thyroid nodules.

Materials and methods

We performed a prospective, blinded cohort study on 189 patients, with 196 Bethesda III/IV nodules. Specifically, we analyzed DNA mutations and RNA fusions across the FNA samples using NGS, then reviewed follow‐up reports from 84 nodules following definitive surgery, to determine the assay performance.

Results

Enough DNA and RNA were obtained in 188 nodules, revealing mutations or fusions in 34.6% of them. The most frequently mutated genes were RAS, followed by BRAF V600E. Based on surgical pathology, 39% (33/84) and 4.8% (4/84) of the nodules were malignant and intermediate, respectively. According to the risk stratification criteria, 28 cases were categorized High‐Risk group, all of the resected nodules (n = 20) were malignant. Twenty‐four thyroid nodules were in the Low‐Risk group, 28.6% (4/14) surgically removed nodules were malignant. In the Benign‐Like category, 18.0% (9/50) were malignant. Five out of 13 nodules with benign mutations were resected, including SPOP, EZH1, and ZNF148, all of them were benign. If genetic alterations annotated with High‐Risk or Low‐Risk was considered as positive, and negative if Benign‐Like. Multigene testing revealed sensitivity, specificity, positive predictive values (PPV), and negative predictive value (NPV) of 73%, 80%, 71%, and 82%, respectively. In addition, if four intermediate nodules were counted as malignant, the PPV and NPV were 71% and 74%.

Conclusion

Our results allow for further stratification of Bethesda III/IV thyroid nodules based on the risk of their malignancy. SPOP, EZH1, and ZNF148 mutations may be used as benign markers.
Giant cell glioblastoma in 6‐year‐old kid: Report of an unusual case
Authors: Shrestha Suraj , Homagain Sushan , Raut Akash , Sedhain Gopal , Bhatta Suraj , Shrivastav Shreya
Published: September 2020

Pediatric giant cell glioblastoma, a highly malignant and lethal tumor, can only be distinguished from glioblastoma multiforme histologically. Though it is said to have a better prognosis, adequate evidence in favor is lacking. Early diagnosis with gross total resection and adjuvant chemotherapy might increase the survival period.
Symmetrical peripheral gangrene: A rare clinical entity
Authors: Macheka Kudzai T. , Masamha Tasimbanashe , Mungani Hopewell , Kumirayi Linda
Published: September 2020

Symmetrical peripheral gangrene (SPG) may present initially with septicemia. Prompt identification and management of the underlying cause of SPG is central for the best outcome. Good management incorporates delaying early surgical intervention, suitable antibiotic therapy, judicious debridement, and skin grafting.
A challenging management of hemophilia B patient with inhibitors undergoing major orthopedic surgeries in a resource‐constrained country
Authors: Matrane Wafaa , Nsiri Afak , Rafai Mohamed , Midmani Fatima , Boughaza Nada , Cherkaoui Siham , Qachouh Meryem , Khoubila Nisrine
Published: September 2020

In this paper, we report a life‐threatening condition and relate our experience in managing a hemophilia B patient who required three surgical procedures, highlighting the difficulties we encountered in our setting and propose some tangible.
When a patient walks in with a knife in his back: A scene from the movies
Authors: Shah Tuhin , Shakya Arjana
Published: September 2020

Stab injury is a relatively common assault mode especially in developing countries. Having a preset protocol to follow with multidisciplinary departmental involvement whenever necessary helps with better management and outcome. It is important to rule out injury to the major neurovascular structures in such a presentation both clinically and by investigations.