bluuebunny/crossref_metadata_2025 huggingface dataset:
The fiftieth anniversary of physics at UFSM: a gender analysis of graduates
Authors: Riffel Rogemar André , Dorneles Lucio Strazzabosco
Published: July 2018

The Federal University of Santa Maria (UFSM - Universidade Federal de Santa Maria) has trained more than 600 undergraduateand 200 Ph.D. students, becoming a reference in physics training at the Rio Grande do Sul central region. In this work we presenta gender analysis of the UFSM graduated students from both levels. The number of graduated students of the female gender hasbeen rising with time and is significatively bigger than the number observed nationally in both levels. The mean values are35 %, 37 %, 28 %, 42 % e 39 % for the courses physics teaching (daytime), physics teaching (nightime), physics, master anddoctorate, respectively. We also observe an increase in the participation of female students in graduate courses, when comparing to undergraduate courses, unlike what is observed in the national average numbers. Considering only the last 5 years the number of graduated students reaches 50 % in the physics teaching (daytime) and master courses. Although this study is of a local nature, it reinforces the need for the dissemination of data regarding the gender of graduates by all universities, allowing to conduct general gender studies.
Cultura de colaboración en un centro educativo: aportaciones desde el asesoramiento
Authors: Monge López Carlos , Domingo Sevilla Jesús , Torrego Seijo Juan Carlos
Published: July 2018

Los procesos de cambio orientados a la
mejora de la inclusión escolar suponen
un elemento necesario en los centros edu-
cativos. En este sentido, el aprendizaje
cooperativo se manifiesta en algunas ins-
tituciones educativas como una verdadera
innovación que responde a esta temática.
Para ello pueden utilizarse estrategias de
asesoramiento colaborativo, consideran-
do que la cooperación del alumnado ha
de ir vinculada a la colaboración del pro-
fesorado y con otros agentes de la comuni-
dad escolar. Una de estas estrategias viene
definida por una serie adaptable de fases:
(a) creación de condiciones para abordar
la implantación del proyecto cooperativo
y evaluación inicial de la red de enseñan-
za; (b) planificación y preparación para
el desarrollo; (c) desarrollo y seguimiento
del plan; (d) evaluación y propuestas de
mejora. El objetivo principal de esta in-
vestigación es identificar y comprender
las implicaciones esenciales de esta propuesta formativa. Para ello se desarrolla
un estudio de caso en un centro educativo
de la Comunidad de Madrid, recabando la
información a partir de entrevistas, grupos
de discusión, análisis documental, obser-
vación y cuestionarios. Los resultados des-
tacan el impacto en tres grandes ámbitos:
en la organización escolar, en el desarro-
llo profesional del profesorado y en los
aprendizajes del alumnado.
Entornos Personales de Aprendizaje para la comprensión y desarrollo de la Competencia Digital: análisis de los estudiantes universitarios en España
Authors: Prendes Espinosa M.ª Paz , Solano Fernández Isabel María , Serrano Sánchez José Luis , González Calatayud Víctor , Román García M.ª del Mar
Published: July 2018

Tanto en el contexto internacional como
nacional, se observa que cada vez es más
importante la concreción de las compe-
tencias digitales en el contexto educativo,
referidas tanto a los docentes como a los
discentes. Si nos fijamos de modo más es-
pecífico en los estudiantes, la competen-
cia digital es un amplio constructo teórico
que entre sus dimensiones sustenta la ne-
cesidad de analizar y potenciar el Entorno
Personal de Aprendizaje (PLE), constructo

que sustenta el Proyecto CAPPLE y que
nos permite comprender de modo global
cómo los estudiantes utilizan su compe-
tencia digital en sus procesos académicos
de aprendizaje. En este artículo mostra-
mos los principales resultados del Proyec-
to CAPPLE, un estudio exploratorio en el
que se ha utilizado un cuestionario ad
hoc para determinar las estrategias y he-
rramientas tecnológicas que los estudian-
tes universitarios de último curso (n=2054)
utilizan en los procesos de aprendizaje y
comunicación. El cuestionario ha sido va-
lidado con un triple procedimiento y un
alpha de Cronbach de 0.944. Las eviden-
cias obtenidas nos permiten desmitificar el
concepto de nativo digital, puesto que los
estudiantes de último curso de grado han
integrado las tecnologías para comunicar-
se en sus espacios personales y de ocio,
pero no tanto para su trabajo académico
en la universidad.
Innovación pedagógica en la formación del profesorado apoyada por videos en red
Authors: Colás Bravo Pilar , De Pablos Pons Juan , Conde Jiménez Jesús , Reyes de Cozar Salvador
Published: July 2018

El propósito de esta aportación es ofrecer
una visión de conjunto sobre las posibi-
lidades pedagógicas de las redes socia-
les y los videos en red para un cambio
paradigmático en la formación inicial y
permanente del profesorado. Este trabajo
se nutre, tanto de resultados de investiga-
ciones empíricas, como de elaboraciones
teóricas, procedentes de publicaciones
actuales sobre esta temática. La metodo-
logía tiene un carácter eminentemente
documental, basada en bases de datos in-
ternacionales (ERIC, WOS, SCOPUS). Los
hallazgos muestran que es en los últimos
cinco años cuando se inician investigacio-
nes y proyectos de innovación, orienta-
dos a explorar, experimentar y contrastar
los beneficios de estas tecnologías en los
aprendizajes y en sus aplicaciones en el
aula. Por otra parte, se observa en el pano-
rama internacional aportaciones conceptuales referidas a nuevas exigencias forma-
tivas del profesorado para adaptarse a la
sociedad actual. Sin embargo, son todavía
escasas las reflexiones sobre cómo las re-
des sociales y la inclusión del video en
las mismas pueden contribuir a la trans-
formación de los actuales modelos y para-
digmas de formación del profesorado. Los
resultados que se aportan van referidos a:
a) la conceptualización de nuevos retos
que debe asumir la formación docente
en el nuevo entorno digital y que deben
ser contemplados en el diseño de nuevos
modelos formativos del profesorado, b) la
sistematización de líneas y enfoques de
investigación sobre los usos pedagógicos
y funciones formativas de los videos en
las redes sociales en la formación del pro-
fesorado y c) la identificación del poten-
cial pedagógico que propician las redes
sociales y la inclusión del video: apren-
dizaje abierto, interacción comunicativa,
agencialidad de los sujetos y construcción
colaborativa del conocimiento. Estos as-
pectos se consideran clave para sustentar
un nuevo modelo de formación del profe-
sorado. Pero también para facilitar la cons-
trucción de una nueva identidad y cultura
profesional docente.

 
Termes complexes et langues de spécialité en sciences humaines et sociales : que nous apprennent les textes intégraux ?
Authors: Jacquey Evelyne , Kister Laurence , Marcon Mario , Barreaux Sabine
Published: July 2018

À partir d’un corpus d’articles scientifiques de trois disciplines de sciences humaines (archéologie, linguistique et psychologie), l’étude que nous présentons a pour objectif d’examiner la composition des candidats termes polylexicaux de forme N_Adj (langage humain, niveau individuel, origine sociale, structure syllabique). Nous nous concentrons sur les occurrences de candidats termes considérées comme relevant d’un usage disciplinaire par les annotateurs experts de chaque discipline. Nous nous intéressons, en particulier, aux classements possibles des noms et des adjectifs dans trois lexiques en interaction dans l’écrit scientifique : le lexique scientifique transdisciplinaire, les ressources terminologiques et le lexique de la langue générale. Les candidats termes analysés ont au moins un composant, le nom ou l’adjectif, qui appartient à au moins deux des trois disciplines du corpus. À l’issue de cette étude, nous constatons le rôle primordial du lexique disciplinaire et ses interactions fréquentes avec le lexique scientifique transdisciplinaire dans la formation des termes polylexicaux de forme N_Adj.
The Incidence and Prevalence of Adult Primary Sjögren's Syndrome in New York County
Authors: Izmirly Peter M. , Buyon Jill P. , Wan Isabella , Belmont H. Michael , Sahl Sara , Salmon Jane E. , Askanase Anca , Bathon Joan M. , Geraldino‐Pardilla Laura , Ali Yousaf , Ginzler Ellen M. , Putterman Chaim , Gordon Caroline , Helmick Charles G. , Parton Hilary
Published: July 2018

Objective

Extant epidemiologic data of primary Sjögren's syndrome (SS) remains limited, particularly for racial/ethnic populations in the US. The Manhattan Lupus Surveillance Program (MLSP) is a population‐based retrospective registry of cases of systemic lupus erythematosus and related diseases, including primary SS in Manhattan, New York. The MLSP was used to provide estimates of the incidence and prevalence of primary SS across major racial/ethnic populations.

Methods

MLSP cases were identified from hospitals, rheumatologists, and population databases. Three case definitions were used for primary SS, including physician diagnosis, rheumatologist diagnosis, and modified primary SS criteria. Rates among Manhattan residents were age‐adjusted, and capture‐recapture analyses were conducted to assess underascertainment of cases.

Results

By physician diagnosis, age‐adjusted overall incidence and prevalence rates of primary SS among adult Manhattan residents were 3.5 and 13.1 per 100,000 person‐years, respectively. Capture‐recapture adjustment increased incidence and prevalence rates (4.1 and 14.2 per 100,000 person‐years, respectively). Based on physician diagnosis, incidence and prevalence rates were approximately 6 times higher among women than men (P < 0.001). Incidence of primary SS was statistically higher among non‐Latina Asian women (10.5) and non‐Latina white women (6.2) compared with Latina women (3.2). Incidence was also higher among non‐Latina Asian women compared with non‐Latina black women (3.3). Prevalence of primary SS did not differ by race/ethnicity. Similar trends were observed when more restrictive case definitions were applied.

Conclusion

Data from the MLSP revealed disparities among Manhattan residents in primary SS incidence and prevalence by sex and differences in primary SS incidence by race/ethnicity among women. These data also provided epidemiologic estimates for the major racial/ethnic populations in the US.
Role of Hip Injury and Giving Way in Pain Exacerbation in Hip Osteoarthritis: An Internet‐Based Case–Crossover Study
Authors: Fu Kai , Makovey Joanna , Metcalf Ben , Bennell Kim , Zhang Yuqing , Asher Rebecca , Robbins Sarah , Deveza Leticia , Hunter David J.
Published: July 2018

Objective

To evaluate the association between hip injury/giving way and hip pain exacerbations in patients with symptomatic hip osteoarthritis (OA).

Methods

We conducted an internet‐based case–crossover study to assess hip injury and giving way for hip pain exacerbation. Eligible participants with symptomatic hip OA were followed up for 90 days and asked to complete online questionnaires at baseline and 10‐day intervals (control periods). They also logged on to the study web site to complete questionnaires for an episode of a hip pain exacerbation (case periods) defined as an increase of 2 points in pain intensity compared with the baseline rating on a numeric rating scale (range 0–10). The relationship of hip injury and giving way to the risk of pain exacerbation was examined using conditional logistic regression.

Results

Of 252 patients recruited into the study, we included 133 patients (53%) who provided data from both case and control periods. Hip injury during the last 7 days increased the risk of hip pain exacerbation (odds ratio [OR] 2.74 [95% confidence interval (95% CI) 1.62–4.62]). The hip giving way during the last 2 days was associated with an increased risk of hip pain exacerbation (OR 2.10 [95% CI 1.30–3.39]) and showed a significant relationship between the number of hip giving way events and the risk of hip pain exacerbations (P < 0.001).

Conclusion

Hip injury and episodes of the hip giving way were significantly related to pain exacerbation in patients with symptomatic hip OA. Methods to prevent exposure to injury may help to reduce the burden of pain in patients with hip OA.
Time of Disease‐Modifying Antirheumatic Drug Start in Juvenile Idiopathic Arthritis and the Likelihood of a Drug‐Free Remission in Young Adulthood
Authors: Minden Kirsten , Horneff Gerd , Niewerth Martina , Seipelt Eva , Aringer Martin , Aries Peer , Foeldvari Ivan , Haas Johannes‐Peter , Klein Ariane , Tatsis Stefanie , Tenbrock Klaus , Zink Angela , Klotsche Jens
Published: July 2018

Objective

To study juvenile idiopathic arthritis (JIA) long‐term outcomes in relation to the time of initiation of biologic disease‐modifying antirheumatic drug (bDMARD).

Methods

Outcomes of JIA patients prospectively followed by the Biologika in der Kinderrheumatologie (BiKeR) and Juvenile Arthritis Methotrexate/Biologics Long‐Term Observation (JuMBO) registers were analyzed with regard to drug‐free remission and inactive disease, functional status and quality of life, and surgery. To analyze the influence of early bDMARD therapy on outcomes, patients were assigned to 3 groups based on the time from symptom onset to bDMARD start (G1: ≤2 years, G2: >2 to ≤5 years, and G3: >5 years). Propensity score–adjusted outcome differences were analyzed by multinomial logistic regression analyses among the groups.

Results

A total of 701 JIA patients were observed for mean ± SD 9.1 ± 3.7 years. At the last follow‐up (disease duration mean ± SD 14.3 ± 6.1 years), 11.7% of patients were in drug‐free remission, and 40.0% had inactive disease. More than half of the patients reported no functional limitation, while 5% had undergone arthroplasty, and 3% had eye surgery. At the 10‐year time point, patients in G1 (n = 108) were significantly more likely to be in drug‐free remission than those patients who began treatment later (G2, n = 199; G3, n = 259), with 18.5%, 10.1%, and 4.9%, respectively. Patients in G1 had significantly lower disease activity (clinical Juvenile Arthritis Disease Activity Score in 10 joints = 4.9), a better overall well‐being (18.2% patient global assessment score = 0), and higher functional status (59.2% Health Assessment Questionnaire score = 0), compared to patients in G3 (7.1, 8.4%, and 43.7%, respectively). G1 patients required arthroplasty significantly less frequently than G3 patients and had significantly lower disease activity over time than patients in both G2 and G3.

Conclusion

Early DMARD treatment is associated with better disease control and outcomes, which supports the concept of a “window of opportunity” for JIA.
Two‐Dimensional Antimonene‐Based Photonic Nanomedicine for Cancer Theranostics
Authors: Tao Wei , Ji Xiaoyuan , Zhu Xianbing , Li Li , Wang Junqing , Zhang Ye , Saw Phei Er , Li Wenliang , Kong Na , Islam Mohammad Ariful , Gan Tian , Zeng Xiaowei , Zhang Han , Mahmoudi Morteza , Tearney Guillermo J. , Farokhzad Omid C.
Published: July 2018

Antimonene (AM) is a recently described two‐dimensional (2D) elemental layered material. In this study, a novel photonic drug‐delivery platform based on 2D PEGylated AM nanosheets (NSs) is developed. The platform's multiple advantages include: i) excellent photothermal properties, ii) high drug‐loading capacity, iii) spatiotemporally controlled drug release triggered by near‐infrared (NIR) light and moderate acidic pH, iv) superior accumulation at tumor sites, v) deep tumor penetration by both extrinsic stimuli (i.e., NIR light) and intrinsic stimuli (i.e., pH), vi) excellent multimodal‐imaging properties, and vii) significant inhibition of tumor growth with no observable side effects and potential degradability, thus addressing several key limitations of cancer nanomedicines. The intracellular fate of the prepared NSs is also revealed for the first time, providing deep insights that improve cellular‐level understanding of the nano–bio interactions of AM‐based NSs and other emerging 2D nanomaterials. To the best of knowledge, this is the first report on 2D AM‐based photonic drug‐delivery platforms, possibly marking an exciting jumping‐off point for research into the application of 2D AM nanomaterials in cancer theranostics.
11.2% All‐Polymer Tandem Solar Cells with Simultaneously Improved Efficiency and Stability
Authors: Zhang Kai , Xia Ruoxi , Fan Baobing , Liu Xiang , Wang Zhenfeng , Dong Sheng , Yip Hin‐Lap , Ying Lei , Huang Fei , Cao Yong
Published: July 2018

All‐polymer solar cells (all‐PSCs) that contain both p‐type and n‐type polymeric materials blended together as light‐absorption layers have attracted much attention, since the blend of a polymeric donor and acceptor should present superior photochemical, thermal, and mechanical stability to those of small molecular‐based organic solar cells. In this work, the interfacial stability is studied by using highly stable all‐polymer solar cell as a platform. It is found that the thermally deposited metal electrode atoms can diffuse into the active layer during device storage, which consequently greatly decreases the power conversion efficiency. Fortunately, the diffusion of metal atoms can be slowed down and even blocked by using thicker interlayer materials, high‐glass‐transition‐temperature interlayer materials, or a tandem device structure. Learning from this, homojunction tandem all‐PSCs are successfully developed that simultaneously exhibit a record power conversion efficiency over 11% and remarkable stability with efficiency retaining 93% of the initial value after thermally aging at 80 °C for 1000 h.
2D MoSe2 Transistor with Polymer‐Brush/Channel Interface
Authors: Jeong Yeonsu , Park Ji Hoon , Ahn Jongtae , Lim June Yeong , Kim Eunkyoung , Im Seongil
Published: July 2018

Toward any practical applications of 2D transition metal dichalcogenide (TMD) transistors, two issues are often met. First, threshold voltage (Vth) of usual TMD 2D field effect transistors (FETs) is located quite away from 0 V, making the device already on and less practical. Second, a large hysteresis exists during transistor operation. Here, hysteresis‐minimized n‐TMD FETs are fabricated using polymer‐brush/channel interface, to extend the 2D FET study to a sensor application. Gated by piezoelectric P(VDF‐TrFE) touch pad that allows dipole switching and instantaneous voltages of +5 and ‐5 V, n‐MoSe2 FET operates well in its ON/OFF drain current behavior, due to its properly small Vth of −5 V. However, n‐MoS2 FET with a relatively high Vth of more than −8 V cannot be suitably switched off. To minimize the gate hysteresis, ultrathin polystyrene‐brush layer is applied between TMD channel and 50 nm thin Al2O3 dielectric. n‐Channel MoS2 and MoSe2 FETs exhibit a minimum voltage hysteresis of 1 and 0.5 V, respectively. It is regarded that Vth and hysteresis issues are uneasy to resolve but still possible to circumvent by using proper TMD channel and channel/dielectric interface materials.
Simultaneous Observation of the Electrical and Luminous Characteristics of Light‐Emitting Electrochemical Cells by Using a Displacement Current Measurement Technique
Authors: Noguchi Yutaka , Higeta Tetsuya , Yonekawa Fumihiro
Published: July 2018

An extended displacement current measurement (DCM) is performed to analyze the operation mechanisms of a poly(p‐phenylenevinylene) derivative (Super Yellow)‐based light‐emitting electrochemical cell (LEC). The characteristics of the actual current, displacement current, and electroluminescence (EL) intensity during the relaxation processes of electrochemical doping are simultaneously measured. The transient characteristics indicate correlations between the conductance, capacitance, and EL efficiency. The conductance and capacitance decrease with proceeding doping relaxation, whereas the EL efficiency increases. The EL efficiency is dominated by the electrochemical doping state, rather than the actual current density. Moreover, the EL efficiency deteriorates with increasing direct current (DC) voltage applied for electrochemical doping. Since the DCM reveals that the electrical properties of the device obey the trap charge limited current regime in the intrinsic region and are almost independent of the applied DC voltage, efficiency deterioration is not responsible for the intrinsic region but for the doped region. The results suggest that the self‐absorption in the doped region causes efficiency loss, otherwise the emitting zone is formed close to the doped region. The extended DCM enables us to comprehensively analyze the operation mechanisms of LECs on the basis of the simultaneous observations of electrical and luminous characteristics, including their transient changes.
Polarization‐Sensitive and Broadband Photodetection Based on a Mixed‐Dimensionality TiS3/Si p–n Junction
Authors: Niu Yue , Frisenda Riccardo , Flores Eduardo , Ares Jose R. , Jiao Weicheng , Perez de Lara David , Sánchez Carlos , Wang Rongguo , Ferrer Isabel J. , Castellanos‐Gomez Andres
Published: July 2018

The capability to detect the polarization state of light is crucial in many day‐life applications and scientific disciplines. Novel anisotropic 2D materials such as TiS3 combine polarization sensitivity, given by the in‐plane optical anisotropy, with excellent electrical properties. Here, the fabrication of a monolithic polarization‐sensitive broadband photodetector based on a mixed‐dimensionality TiS3/Si p–n junction is demonstrated. The fabricated devices show broadband responsivity up to 1050 nm, a strong sensitivity to linearly polarized illumination with difference between the two orthogonal polarization states up to 350%, and a good detectivity and fast response time. The discussed devices can be used as building blocks to fabricate more complex polarization‐sensitive systems such as polarimeters.
Emergence of Dynamical Dissipative Structures in a Bistable Spin Crossover Solid Under Light: Experience and Theory
Authors: Boukheddaden Kamel , Sy Mouhamadou , Varret François
Published: July 2018

In this article, the basic spatiotemporal theory elucidating the physical conditions leading to the emergence of dissipative structures and pattern formation in spin‐crossover solids under light causing their photo‐heating is outlined. In this context, the spatiotemporal properties of the spin‐crossover (SC) transition are described as a reaction diffusion process. The time evolution of the spin state (the high spin fraction) is coupled to the spatiotemporal properties of temperature inside the lattice, accounting for: (i) the crystal photo‐heating, (ii) the coupling to thermal bath, and (iii) the thermal diffusion. It is demonstrated that around well‐defined unstable regions, this system leads to the emergence of nonstationary Turing patterns, dynamic dissipative structures, and autocatalytic oscillations of which an experimental example is provided on the SC single crystal ([{Fe(NCSe)(py)2}2(m‐bpypz)]). These results pave the way to a new fertilized field in the quest of nonlinear dynamics and morphogenesis in molecular switchable solids undergoing first‐order phase transitions.
Mechanisms of sepsis‐induced immunosuppression and immunological modification therapies for sepsis
Authors: Ono Satoshi , Tsujimoto Hironori , Hiraki Shuichi , Aosasa Suefumi
Published: July 2018

Surgical injury can be a life‐threatening complication, not only due to the injury itself, but also due to immune responses to the injury and subsequent development of infections, which readily result in sepsis. Sepsis remains the leading cause of death in most intensive care units. Unfavorable outcomes of several high‐profile trials in the treatment of sepsis have led researchers to state that sepsis studies need a new direction. The immune response that occurs during sepsis is characterized by a cytokine‐mediated hyper‐inflammatory phase, which most patients survive, and a subsequent immunosuppressive phase. Therefore, therapies that improve host immunity might increase the survival of patients with sepsis. Many mechanisms are responsible for sepsis‐induced immunosuppression, including apoptosis of immune cells, increased regulatory T cells and expression of programmed cell death 1 on CD4+ T cells, and cellular exhaustion. Immunomodulatory molecules that were recently identified include interleukin‐7, interleukin‐15, and anti‐programmed cell death 1. Recent studies suggest that immunoadjuvant therapy is the next major advance in sepsis treatment.
Programming mammalian gene expression with the antibiotic simocyclinone D8 and the flavonoid luteolin
Authors: Bojar Daniel , Fussenegger Martin
Published: July 2018

Precise control of gene expression with small‐molecular or physical inducers has been a central aim of synthetic biology in recent decades, and has led, for example, to dramatic improvements in the production of protein therapeutics. However, the number of inputs for inducible promoters and gene circuits is still limited, and new orthogonal inducers are needed to support the construction of more complex programmable systems in the fields of bioengineering and biocomputing. Here, two new gene switches inducible by the antibiotic simocyclinone D8 (SD8) and the flavonoid luteolin in mammalian cells are presented. Streptomyces antibioticus Tü 6040 has evolved the DNA gyrase inhibitor SD8, which, through the bacterial TetR‐like transcriptional repressor SimR, also regulates the multidrug efflux pump SimX. Taking advantage of SimR, as well as the luteolin‐binding TetR‐like transcriptional repressor EmrR from Sinorhizobium meliloti, we optimized the amounts of transfected genes and DNA operator sites to engineer highly effective, orthogonal transcriptional OFF‐ as well as ON‐switches triggered by SD8 and/or luteolin. We confirmed that SD8 and luteolin are not cytotoxic at the concentrations required for switching, and demonstrated the functionality of these gene switches in a range of biotechnologically relevant cell lines. These switches were combined to generate OR and AND Boolean logic gates, and we confirmed their modularity by the addition of the vanillic acid‐responsive transcriptional repressor VanR to generate a three‐input AND gate. These additions to the panoply of inducers available for synthetic biology are expected to facilitate advances in the fields of biocomputing, biopharmaceutical manufacturing, and biomedicine. © 2018 The Authors. AIChE Journal published by Wiley Periodicals, Inc. on behalf of American Institute of Chemical Engineers. AIChE J, 64: 4237–4246, 2018
Immunoglobulin light chain amyloidosis: 2018 Update on diagnosis, prognosis, and treatment
Authors: Gertz Morie A.
Published: July 2018

Disease Overview

Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical multiple myeloma.”

Diagnosis

Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow, salivary gland, or subcutaneous fat aspirate in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy.

Prognosis

N‐terminal pro‐brain natriuretic peptide (NT‐proBNP), serum troponin T, and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months.

Therapy

All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include systolic blood pressure >90 mm Hg, troponin T < 0.06 ng/mL, age < 70 years, and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered melphalan‐dexamethasone or cyclophosphamide‐bortezomib‐dexamethasone. Daratumumab appears to be highly active in AL amyloidosis. Antibodies designed to dissolve existing amyloid deposits are under study.

Future Challenges

Delayed diagnosis remains a major obstacle to initiating effective therapy.

Educational Objectives

Upon completion of this educational activity, participants will be better able to:

Master recognition of clinical presentations that should raise suspicion of amyloidosis.

Understand simple techniques for confirming the diagnosis and providing material to classify the protein subunit.

Recognize that a tissue diagnosis of amyloidosis does not indicate whether the amyloid is systemic or of immunoglobulin light chain origin.

Understand the roles of the newly introduced chemotherapeutic and investigational antibody regimens for the therapy of light chain amyloidosis.
Holoprosencephaly from conception to adulthood
Authors: Weiss Karin , Kruszka Paul S. , Levey Eric , Muenke Max
Published: July 2018

Holoprosencephaly (HPE) consists of a spectrum of malformations related to incomplete separation of the prosencephalon. There is a wide clinical variability depending on the HPE subtype seen on imaging. Early postnatal lethality is common, however a significant fraction of newborns diagnosed with HPE will survive into childhood and even adulthood. Here we will review the clinical management of HPE during different ages from the prenatal period to adulthood.
Heralded Universal Quantum Gate and Entangler Assisted by Imperfect Double‐Sided Quantum‐Dot‐Microcavity Systems
Authors: Wei Hai‐Rui , Chen Ning‐Yang , Liu Ji‐Zhen
Published: July 2018

Efficient ways are presented to accomplish photonic controlled‐phase‐flip gate and entangler with the assistance of imperfect double‐sided quantum‐dot‐microcavity systems, but without ancillary qubits. Compact quantum circuits for implementing entanglement swapping between photon pairs and electron pairs are then designed. Unity fidelities of the schemes can be achieved, and physical imperfections in the construction processes are detected by single‐photon detectors. Also, the efficiencies of the schemes can be further improved by repeating the operation processes when the undesired performances are detected. The evaluations show that the schemes are possible with current experiment parameters.
Identifying the Structural Evolution of the Sodium Ion Battery Na2FePO4F Cathode
Authors: Li Qi , Liu Zigeng , Zheng Feng , Liu Rui , Lee Jeongjae , Xu Gui‐Liang , Zhong Guiming , Hou Xu , Fu Riqiang , Chen Zonghai , Amine Khalil , Mi Jinxiao , Wu Shunqing , Grey Clare P. , Yang Yong
Published: July 2018

Na2FePO4F is a promising cathode material for Na‐ion batteries owing to its relatively high discharge voltage and excellent cycling performance. Now, the long‐ and short‐range structural evolution of Na2FePO4F during cycling is studied by in situ high‐energy X‐ray diffraction (XRD), ex situ solid‐state nuclear magnetic resonance (NMR), and first‐principles DFT calculations. DFT calculations suggest that the intermediate phase, Na1.5FePO4F, adopts the space group of P21/c, which is a subgroup (P21/b11, No. 14) of Pbcn (No. 60), the space group of the starting phase, Na2FePO4F, and this space group provides a good fit to the experimental XRD and NMR results. The two crystallographically unique Na sites in the structure of Na2FePO4F behave differently during cycling, where the Na ions on the Na2 site are electrochemically active while those on the Na1 site are inert. This study determines the structural evolution and the electrochemical reaction mechanisms of Na2FePO4F in a Na‐ion battery.
Photolumineszierende Diamidophosphankomplexe der Erdalkalimetalle, des Aluminiums und des Zinks
Authors: Bestgen Sebastian , Schoo Christoph , Neumeier B. Lilli , Feuerstein Thomas J. , Zovko Christina , Köppe Ralf , Feldmann Claus , Roesky Peter W.
Published: July 2018

Die positiv geladenen und schwach polarisierbaren s‐Block‐Metalle tragen üblicherweise keine Phosphanliganden in ihren molekularen Verbindungen. Hier wird von ein‐ und zweikernigen Diamidophosphankomplexen der Erdalkalimetalle Mg, Ca und Sr berichtet, die aus gängigen Startverbindungen mit N,N‐Bis(2‐(diphenylphosphanyl)phenyl)ethan‐1,2‐diamin (PNHNHP) leicht erhalten werden können. Die Erdalkalimetallkomplexe [(PNNP)Mg]2 und [(PNNP)M(thf)3] (M=Ca, Sr) weisen M‐P‐Koordinationen sowie intensive Fluoreszenz sowohl in Lösung als auch im Festkörper auf. Weiterhin wurden die noch stärker lumineszierenden Verbindungen von Al und Zn – [(PNNP)Zn]2 und [(PNNP)AlCl] – dargestellt. Emissionslebensdauern im Nanosekundenbereich sowie hohe Photolumineszenz‐Quantenausbeuten von bis zu 93 % werden bei Raumtemperatur beobachtet.
FRET Monitoring of Intracellular Ketal Hydrolysis in Synthetic Nanoparticles
Authors: Nuhn Lutz , Van Herck Simon , Best Andreas , Deswarte Kim , Kokkinopoulou Maria , Lieberwirth Ingo , Koynov Kaloian , Lambrecht Bart N. , De Geest Bruno G.
Published: July 2018

Degradable synthetic crosslinking is a versatile strategy to harness nanomaterials against disassembly in a complex physiological medium prompted by dilution effects or competitive interaction. In particular, chemical bonds such as ketals that are stable at physiological conditions but are cleaved in response to disease‐mediated or intracellular conditions (e.g., a mildly acidic pH) are of great relevance for biomedical applications. Despite the range of spectroscopic or chromatographic analyses methods that allow chemical degradation in solution to be assessed, it is much less straightforward to interrogate synthetic nanomaterials for their degradation state when located inside a living organism. We demonstrate a method based on FRET analysis to monitor intracellular disassembly of block‐copolymer‐derived nanoparticles engineered with a FRET couple on separate polymer chains, which after self‐assembly are covalently crosslinked with a pH‐sensitive ketal‐containing crosslinker.
Self‐Assembled Functional DNA Superstructures as High‐Density and Versatile Recognition Elements for Printed Paper Sensors
Authors: Liu Meng , Zhang Qiang , Kannan Balamurali , Botton Gianluigi A. , Yang Jie , Soleymani Leyla , Brennan John D. , Li Yingfu
Published: July 2018

Micrometer‐sized functional nucleic acid (FNA) superstructures (denoted as 3D DNA) were examined as a unique class of biorecognition elements to produce highly functional bioactive paper surfaces. 3D DNA containing repeating sequences of either a DNA aptamer or DNAzyme was created from long‐chain products of rolling circle amplification followed by salt aging. The resulting 3D DNA retained its original spherical shape upon inkjet printing and adhered strongly to the paper surface via physisorption. 3D DNA paper sensors showed resistance to degradation by nucleases, suppressed nonspecific protein adsorption, and provided a much higher surface density of functional DNA relative to monomeric FNAs, making such species ideally suited for development of paper‐based biosensors.
A Rotaxane‐like Cage‐in‐Ring Structural Motif for a Metallosupramolecular Pd6L12 Aggregate
Authors: Käseborn Matthias , Holstein Julian J. , Clever Guido H. , Lützen Arne
Published: July 2018

A BODIPY‐based bis(3‐pyridyl) ligand undergoes self‐assembly upon coordination to tetravalent palladium(II) cations to form a Pd6L12 metallosupramolecular assembly with an unprecedented structural motif that resembles a rotaxane‐like cage‐in‐ring arrangement. In this assembly the ligand adopts two different conformations—a C‐shaped one to form a Pd2L4 cage which is located in the center of a Pd4L8 ring consisting of ligands in a W‐shaped conformation. This assembly is not mechanically interlocked in the sense of catenation but it is stabilized only by attractive π‐stacking between the peripheral BODIPY chromophores and the ligands’ skeleton as well as attractive van der Waals interactions between the long alkoxy chains. As a result, the co‐arrangement of the two components leads to a very efficient space filling. The overall structure can be described as a rotaxane‐like assembly with a metallosupramolecular cage forming the axle in a metallosupramolecular ring. This unique structural motif could be characterized via ESI mass spectrometry, NMR spectroscopy, and X‐ray crystallography.
Tailormade Polysaccharides with Defined Branching Patterns: Enzymatic Polymerization of Arabinoxylan Oligosaccharides
Authors: Senf Deborah , Ruprecht Colin , Kishani Saina , Matic Aleksandar , Toriz Guillermo , Gatenholm Paul , Wågberg Lars , Pfrengle Fabian
Published: July 2018

The heterogeneous nature of non‐cellulosic polysaccharides, such as arabinoxylan, makes it difficult to correlate molecular structure with macroscopic properties. To study the impact of specific structural features of the polysaccharides on crystallinity or affinity to other cell wall components, collections of polysaccharides with defined repeating units are required. Herein, a chemoenzymatic approach to artificial arabinoxylan polysaccharides with systematically altered branching patterns is described. The polysaccharides were obtained by glycosynthase‐catalyzed polymerization of glycosyl fluorides derived from arabinoxylan oligosaccharides. X‐ray diffraction and adsorption experiments on cellulosic surfaces revealed that the physicochemical properties of the synthetic polysaccharides strongly depend on the specific nature of their substitution patterns. The artificial polysaccharides allow structure–property relationship studies that are not accessible by other means.
Light‐Triggered Release of Trapped Charges in Molecular Assemblies
Authors: Balgley Renata , Algavi Yadid M. , Elool Dov Neta , Lahav Michal , van der Boom Milko E.
Published: July 2018

We demonstrate the mediation of charge transport and release in thin films and devices by shifting the redox properties of layers of metal complexes by light. The nanoscale surface arrangement of both photo‐ and electrochemically‐active components is essential for the function of the thin films. Layers of well‐defined ruthenium complexes on indium‐tin‐oxide electrodes provide electron‐transport channels that allow the electrochemical addressing of layers of isostructural cobalt complexes. These cobalt complexes are electrochemically inactive when assembled directly on transparent metal‐oxide electrodes. The interlayer of ruthenium complexes on such electrodes allows irreversible oxidation of the cobalt complexes. However, shifting the redox properties of the ruthenium complexes by excitation with light opens up an electron‐transport channel to reduce the cobalt complexes; hence releasing the trapped positive charges.
Wet‐spinning assembly of continuous and macroscopic graphene oxide/polyacrylonitrile reinforced composite fibers with enhanced mechanical properties and thermal stability
Authors: Zhao Renhai , Tian Mingwei , Qu Lijun , Zhao Yuling , Chen Shaojuan , Zhu Shifeng , Han Guangting
Published: July 2018

Polyacrylonitrile (PAN) composite microfibers with different contents of graphene oxide (GO) were fabricated via wet‐spinning route in this work. Based on nonsolvent‐induced phase separation theory, N,N‐dimethyl formamide/water mixture system was employed as coagulation bath, nonsolvent (water) diffused into PAN spinning solution and led to a quick PAN fiber solidification. Nematic liquid crystal state of GO dispersions and GO/PAN spinning solutions were determined via polarized optical microscopy images, and the morphology and structure of the composite fibers were characterized via scanning electron microscope, Transmission electron microscopy, Fourier transform infrared spectra, and X‐ray diffraction. 1 wt % GO/PAN composite fibers exhibited outstanding mechanical properties, 40% enhancement in tensile strength and 34% enhancement in Young's modulus compared with pure PAN fiber. The results of dynamic mechanical analysis indicated that the composite fiber with 1 wt % GO performed the best thermal mechanical property with 5.5 GPa and 0.139 in storage modulus and loss tangent, respectively. In addition, thermogravimetric analysis showed that thermal stability of the composite fibers enhanced with the increasing GO contents. GO/PAN composite fibers can be as the candidate of carbon fiber precursor, high performance fibers, and textiles applications. © 2018 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2019, 136, 46950.
Disease‐Modifying Osteoarthritis Treatment With Interleukin‐1 Receptor Antagonist Gene Therapy in Small and Large Animal Models
Authors: Nixon Alan J. , Grol Matthew W. , Lang Hayley M. , Ruan Merry Z. C. , Stone Adrianne , Begum Laila , Chen Yuqing , Dawson Brian , Gannon Francis , Plutizki Stanislav , Lee Brendan H. L. , Guse Kilian
Published: July 2018

Objective

Gene therapy holds great promise for the treatment of osteoarthritis (OA) because a single intraarticular injection can lead to long‐term expression of therapeutic proteins within the joint. This study was undertaken to investigate the use of a helper‐dependent adenovirus (HDAd)–mediated intraarticular gene therapy approach for long‐term expression of interleukin‐1 receptor antagonist (IL‐1Ra) as sustained symptomatic and disease‐modifying therapy for OA.

Methods

In mouse models of OA, efficacy of HDAd–IL‐1Ra was evaluated by histologic analysis, micro–computed tomography (micro‐CT), and hot plate analysis. In a horse OA model, safety and efficacy of HDAd–IL‐1Ra were evaluated by blood chemistry, analyses of synovial fluid, synovial membrane, and cartilage, and gross pathology and lameness assessments.

Results

In skeletally immature mice, HDAd–IL‐1Ra prevented development of cartilage damage, osteophytes, and synovitis. In skeletally immature and mature mice, treatment with HDAd–interleukin‐1 receptor antagonist post–OA induction resulted in improved—albeit not significantly—cartilage status assessed histologically and significantly increased cartilage volume, cartilage surface, and bone surface covered by cartilage as assessed by micro‐CT. Fewer osteophytes were observed in HDAd–IL‐1Ra–treated skeletally immature mice. Synovitis was not affected in skeletally immature or mature mice. HDAd–IL‐1Ra protected against disease‐induced thermal hyperalgesia in skeletally mature mice. In the horse OA model, HDAd–IL‐1Ra therapy significantly improved lameness parameters, indicating efficient symptomatic treatment. Moreover, macroscopically and histologically assessed cartilage and synovial membrane parameters were significantly improved, suggesting disease‐modifying efficacy.

Conclusion

These data from OA models in small and large animals demonstrated safe symptomatic and disease‐modifying treatment with an HDAd‐expressing IL‐1Ra. Furthermore, this study establishes HDAd as a vector for joint gene therapy.
Predictability of the 2017 North Atlantic hurricane season
Authors: Camp Joanne , Scaife Adam A. , Heming Julian
Published: July 2018

The 2017 North Atlantic hurricane season was one of the most active in recent history. The season recorded a total of 17 named storms, of which 10 became hurricanes and 6 became major hurricanes, and an Accumulated Cyclone Energy (ACE) index of 226. The number of major hurricanes was joint second highest since 1970, and the ACE index was third highest. We show that the Met Office Atlantic seasonal tropical cyclone forecast, initialised on May 15, 2017, predicted an increased risk of above‐average activity. The forecast also captured aspects of the spatial distribution of the tropical cyclones, such as the high frequency of tracks which crossed the northeast Caribbean in September 2017, at more than 3 months lead time. We show that the high levels of seasonal activity, particularly in terms of major hurricanes and ACE index, were likely a result of a combination of low wind shear in the western Atlantic, above‐average tropical Atlantic sea surface temperatures (SSTs) and a transition to cooler SSTs in the tropical Pacific due to a developing La Niña event, all of which appear to be predictable at seasonal lead times. This study provides evidence for seasonal predictability of the intense 2017 Atlantic hurricane season, including advanced warning of an increased risk of tropical cyclones transiting the northeast Caribbean.
Seasonal Variation of Disinfection Byproduct Precursors in a Large Water Supply
Authors: Zhao Ran , Reckhow David A. , Becker William C. , Schindler Steven
Published: July 2018

Disinfection byproduct (DBP) precursors originate in large reservoirs from at least three types of sources: (1) watershed or allochthonous, (2) algal or autochthonous, and (3) bottom sediments or benthic. The properties of the bulk natural organic matter (NOM) and the DBP precursor content of that NOM are unique to each source. Knowledge about the relative importance of these three sources would be valuable in understanding the natural variability in DBP precursor occurrence and in managing source waters for the purpose of minimizing DBP precursors. In this study, we used temporal and spatial water quality data from a drinking water reservoir to shed light on autochthonous and benthic sources of NOM and DBP precursors. The Cannonsville Reservoir (New York) was chosen for this work because of its well‐documented water quality. To assist in the data analysis, we developed a series of two‐dimensional contour plots that bring clarity to benthic and near‐surface (algal) processes on water quality. Combining conventional water quality parameters with DBP precursor analysis can facilitate a more comprehensive understanding of limnological factors that define DBP precursor levels. These data were compared with known properties of NOM and related natural biochemical parameters. From this analysis, we conclude that algal‐dominated NOM contributes disproportionately to dihaloacetonitrile formation, whereas watershed‐dominated NOM is especially rich in trihaloacetic acid and trihalomethane precursors as well as ultraviolet‐absorbing substances. The sediment‐dominated precursor behaved like a mix of the other two but most resembled the watershed NOM.
Gas fermentation of C1 feedstocks: commercialization status and future prospects
Authors: Teixeira Leonardo V. , Moutinho Liza F. , Romão‐Dumaresq Aline S.
Published: July 2018

The increasing emissions of carbon dioxide, methane and carbon oxide (collectively referred as C1 compounds) are likely to configure a major contribution to global warming and other environmental issues. The implementation of carbon capture and storage (CCS) is considered a crucial strategy to prevent global warming, but the overall costs of currently available CCS technologies are still prohibitive for its large‐scale deployment. Using microorganisms capable of assimilating C1 compounds for producing value‐added products could be an important driver for mitigating emissions and minimizing their deleterious consequences, while simultaneously deriving additional economic benefits from these compounds. This review summarizes the main microorganisms and metabolic routes being investigated, with special focus on both the products targeted and the current industrial initiatives. There are a number of companies investing in these routes and in some instances commercial deployment was identified. Despite the variety of commercially‐appealing products, genetic manipulation of microorganisms to maximize yields and the design of technologies capable of efficiently using the gaseous feedstocks are major challenges yet to be overcome to fully unlock the potential of C1 microbiological routes. © 2018 Society of Chemical Industry and John Wiley & Sons, Ltd