bluuebunny/crossref_metadata_2025 huggingface dataset:
Transition radiation detectors
Authors: Kolanoski Hermann , Wermes Norbert
Published: September 2020

The rearrangement of the electromagnetic field of a charged particle at a transition between media with different electric permittivity leads to the emission of electromagnetic radiation, so-called transition radiation. The chapter begins with the description of the characteristics of the radiation at a boundary, such as angular distribution, energy spectrum, dependence on the Lorentz factor γ and photon yield. Then it is shown that a sufficient photon yield can only be achieved with a large number of transitions which is usually accomplished with stacks of thin foils. The interference phenomena and their dependence on the coherence conditions, parametrised by the ‘formation length’ are explained in detail. The explanation includes also threshold and saturation effects on the measurement of the Lorentz factor γ. Finally, typical transition radiation detectors are presented.
In the Air
Authors: Oppitz-Trotman George
Published: September 2020

Few plays insist more adamantly upon a connection between travel and moral loss than The Tragical History of Doctor Faustus. Here it is argued that the play written by Christopher Marlowe was performed by the English Comedians in Frankfurt-am-Main as early as Autumn 1592, and that it remained in their repertoire throughout the 1590s—before an English play-text was published (in 1604), indeed before its first recorded English performance (in 1594). The play’s unique ability to reconcile a wide array of comic materials to a powerful moral lesson recommended it to itinerant theatre groups. Yet the tonal unevenness of the extant English editions has been an evergreen concern for editors and critics. Having established that the play was performed abroad and thus adapted for many different audiences and scenarios, this chapter suggests that the confusing middle of the extant text(s) represents the modularity of its structure in the 1590s. Marlowe’s ultimate source had been published in Frankfurt in 1587, and merchants at the city’s fair had practical interest and expertise in contracts of all kinds. The performance of the English play there raises many exciting new questions not only of literary interpretation but also of knowledge transfer in early modern Europe.
Introduction
Authors: Rennie David A.
Published: September 2020

This chapter addresses the war’s multifaceted effect, not only on different areas of society but in terms of the competing interpretations that existed within various social groups. David Rennie suggests that authors, too, could demonstrate shifting, sophisticated, and even contradictory reactions to the war in their fictional and non-fictional outputs. The machinations of the publishing industry, advertising, Hollywood, and authors’ artistic and personal development meant that writers’ reactions to the war were complex, provisional, and subject to change in relation to intrapersonal and interpersonal variables. Rennie also proposes, contrary to the findings of Paul Fussell, that American writers did draw on native historical and literary examples to express contrast—but also some elements of continuity—between modern war and nineteenth-century notions of heroism.
The Fallacy of Macroeconomic Indicators
Authors: Lawreniuk Sabina , Parsons Laurie
Published: September 2020

Chapter 2 undertakes an holistic analysis of Cambodia’s recent growth and contemporary endowments, in order to highlight the shortcomings of traditional economic indicators of inequality in the context of translocal livelihoods. It argues that traditional measures such as the Gini coefficient and the Palma and Thiel indices share a static conception of wealth which fails to reflect the translocal livelihoods of contemporary Cambodians. To exemplify this point, the chapter subsequently explores how widely noted inequalities in physical capital are produced and sustained by mobile livelihoods. Finally, inequities in the educational and health dimensions of human capital are examined in order to demonstrate the inadequacy of economic measures at capturing the embodied and intergenerational nature of translocal inequality.
Introduction
Authors: Teukolsky Rachel
Published: September 2020

The introduction explores both Victorian and contemporary theories of visual culture, while developing the book’s own interdisciplinary methodology. Visual culture studies, media history, art history, literary history, and cultural history number among the book’s disciplines. The chapters move across media to study novels and poems alongside photographs and illustrations. Weaving together both visual and textual strands, the book presents a revisionist, multidisciplinary approach to “culture” as it was lived and experienced in the nineteenth century. Academic divides between the disciplines today have obscured the cross-media connections studied in the book. The book’s approach captures the historical reality of the nineteenth century’s turbulent media moment, when the bounds of high art and mass culture were not yet fixed, and words and images mingled indiscriminately in the cultural field.
Sensation
Authors: Teukolsky Rachel
Published: September 2020

Photography was a quintessential new visual technology of the nineteenth century. Chapter 4 studies cartes de visite, or small photographic portraits. These collectible photographs became both popular and controversial during the so-called “sensation” craze of the 1860s. Scholars have largely focused on sensation novels, known for their lurid crime plotlines and outrageous villainesses. Yet sensation was more than merely a literary aesthetic: it was a multimedia phenomenon encompassing both novels and photographs. It responded to new forms of spectacular female celebrity, as seen in the wild popularity of photo portraits of actresses, opera divas, prostitutes, even Queen Victoria. The carte-de-visite medium, circulating women’s portrait photographs in millions of paper copies, perfectly encapsulated sensation’s dialectic between embodiment and mediation, and between individual celebrity and the democratized mass. These themes drive the plots of sensation novels, especially Wilkie Collins’s The Woman in White and M. E. Braddon’s Lady Audley’s Secret.
Conclusion
Authors: Teukolsky Rachel
Published: September 2020

The book’s conclusion examines the early cinema of the 1890s, to look at the ways it invoked and transformed earlier Victorian visual traditions. The earliest films were shown at fairgrounds and public entertainment venues, and thus differ from the more parlor-oriented objects studied in the book. Yet early film also extended Victorian pictorial traditions. Comic strips anticipated sequential visual storytelling, which was expanded in narrative stereoviews. All of the images examined in Picture World became subjects for the earliest films, from magic portrait albums coming alive to “phantom rides” alongside picturesque landscapes. The eye-tricking pleasures of early cinema extended the phantasmagoric worlds of earlier mass visual phenomena.
Sharp Words and Violent Gestures in Piers Plowman
Authors: Zeeman Nicolette
Published: September 2020

This chapter and the next investigate medieval theories about the possibility that the physical decline and suffering of the body might have some power to curb sin. These theories are connected to a tradition of ‘medical-stoic’ thought which assumes the mutual influence of the body and soul on each other. In the later Middle Ages, such ideas shape much medieval pastoral thought, and are reflected in allegorical writings and visual imagery. Although this does not necessarily mean that theologians thought that bodily suffering could substitute for moral effort, some thinkers may have considered that, by making sin difficult, bodily suffering might push the beleaguered human being in a moral direction. This means that these theories sit in an uneasy potential tension with intentionalist ethics. This chapter focuses in particular on the physiological decline associated with old age. It argues that in Piers Plowman Langland both countenances and questions ideas about the impact of the incapacitation of old age; but it also notes that by the paradiastolic last scene of the poem the poet casts doubt on the idea that age’s decrepitude makes people do better. At the same time, the chapter notes that the end of the poem is characterized by forms of exhaustion, and by even the spectre of the narrator’s death. It concludes by asking if the incapacity of old age has provided Langland with a means of closing his long poem.
What’s Wrong with Rights in Ethics?
Authors: Biggar Nigel
Published: September 2020

Chapter 6 observed the tendency of contemporary rights-talk to push all other moral considerations off the table—an observation adumbrated in Chapter 5, where contemporary defences of natural rights were seen to lack awareness that the exercise of such rights might be subject to moral obligations and even contingent upon duties of virtue. Chapter 1 observed the complaint of sceptics that natural, moral rights are often not distinguished from legal ones, with the consequence that the stability and security of the latter are smuggled into natural morality or ethics. Such smuggling is part of a cultural inclination to assimilate morality to law and replace conscience with procedures, in order to abolish the possibility of a failure of conscience. It expresses an aversion to risk and denial of tragedy, whose cost is a practical, moral rigidity that ranges from the imprudent to the absurd. This chapter displays the problem as it appears in ethics, by analysing David Rodin’s War and Self-Defense. Rodin’s attempt to justify killing in terms of a fundamental (natural) moral ‘right to life’, which can only be forfeited through culpable wrongdoing, fails. As he himself inadvertently acknowledges, that right is contingent on a range of moral factors external to the right-holder. Whether it exists at all depends on the situation as a whole and can only be determined at the end of a process of moral deliberation, not posited at the beginning as fundamental. Talk about a (natural) moral right, connoting stability and security, misleads.
First Woman
Authors: Fleming James Rodger
Published: September 2020

This book, based on the life and work of Joanne (Gerould) Simpson (1923–2010), charts the history of women in meteorology and the history of tropical meteorology in the context of her long and productive career as pioneer scientist, project leader, and mentor. In 1943 women had no status in meteorology, tropical weather was largely aer incognita, and Joanne Gerould, a new graduate student at the University of Chicago, had just set her sights on understanding the behavior of clouds. Establishing her career in an era of overwhelming marginalization of women in science was no easy matter, and Joanne (who published under three married names and raised three children) had to fight every step of the way. Under the mentorship of Herbert Riehl, she received a PhD degree from Chicago in 1949. Later, while working at Woods Hole, she collaborated with Riehl on their revolutionary and controversial “hot tower” hypothesis that cumulonimbus clouds were the driving force in the tropical atmosphere, providing energy to power the Hadley circulation, the trade winds, and by implication, the global circulation. The mechanism of hot towers alludes to the incessant battle between buoyancy and entrainment in tropical convection, valorizing those clouds that successfully break through the trade wind inversion to soar to the top of the troposphere. The metaphor of hot towers points to the incessant battles Joanne waged between her sky-high aspirations and the dark psychological and institutional forces dragging her down. Yet she prevailed, reaching the pinnacle of personal and professional accomplishment, especially in her years at NASA, as she conditioned the atmosphere for further breakthroughs for women in science. She is best remembered as a pioneer woman scientist, the best tropical scientist of her generation.
Breaking Through
Authors: Fleming James Rodger
Published: September 2020

This concluding chapter features the testimonies of Joanne’s colleagues while it reprises and re-evaluates her accomplishments.
Balance laws for mass, forces, and moments
Authors: Anand Lallit , Govindjee Sanjay
Published: September 2020

This chapter discusses the law of balance of mass, as well as the laws of balance of forces and moments. The important related concept of stress is this then presented as formalized by Cauchy in terms of a central theorem of Continuum Mechanics, which asserts that satisfaction of global balance of forces and moments is equivalent to the existence of a symmetric tensor field in the deformed body called the Cauchy stress, such that the traction vector acting across each oriented surface element at a point in the body is given by the Cauchy stress tensor operating linearly on the outward unit normal to the surface at that point. In addition, the stress tensor must satisfy a partial differential equation, known as the equation of motion, which asserts that the divergence of the stress tensor plus a body force per unit volume, is equal to the mass density times the acceleration.
Balance of energy and entropy imbalance
Authors: Anand Lallit , Govindjee Sanjay
Published: September 2020

This chapter discusses the first and second laws of thermodynamics. The first law represents a balance between the rate of change of the internal energy plus the rate of change of kinetic energy of a part of the body, and the rate at which energy in the form of heat is transferred to the part plus the mechanical power expended upon it. A part also possesses entropy, and the second law is the statement that the rate at which the net entropy of a part changes is greater than or at a minimum equal to the entropy flow into the part, resulting in a free energy imbalance known as the Clausius-Duhem inequality.
Linear thermoelasticity
Authors: Anand Lallit , Govindjee Sanjay
Published: September 2020

This chapter presents a theory for the coupled thermal and mechanical response of solids under circumstances in which the deformations are small and elastic, and the temperature changes from a reference temperature are small --- a framework known as the theory of linear thermoelasticity. The basic equations of the fully-coupled linear theory of anisotropic thermoelasticity are derived. These equations are then specialized for the case of isotropic materials. Finally, as a further specialization a weakly-coupled theory in which the temperature affects the mechanical response, but the deformation does not affect the thermal response, are discussed; this is a specialization which is of importance for many engineering applications, a few of which are illustrated in the examples.
A small deformation chemoelasticity theory for energy storage materials
Authors: Anand Lallit , Govindjee Sanjay
Published: September 2020

This chapter presents the specialization of chemoelasticity to energy storage materials (batteries). The theory presented is quasi-linear in that the constitutive relations are modelled as kinematically linear but nonlinearly dependent on the concentration of the chemical species. This permits the proper modeling of large changes in the concentration of the chemical species, but restricted to the small deformation regime. The model is also specialized to the case of isotropic response.
Limits to elastic response. Yielding and failure
Authors: Anand Lallit , Govindjee Sanjay
Published: September 2020

This chapter presents conditions for determining the limits of elastic behaviour for isotropic materials. The stress invariants of equivalent pressure, equivalent shear stress, and equivalent tensile stress are defined. These are then used to define common yield conditions, viz. the pressure-independent Mises and Tresca yield conditions, as well as the pressure-dependent Coulomb-Mohr and the Drucker-Prager yield conditions. Rankine’s failure criterion for brittle materials in tension, that is failure in a brittle material will initiate when the maximum principal stress at a point in the body reaches a critical value, is also discussed.
Linear elastic fracture mechanics
Authors: Anand Lallit , Govindjee Sanjay
Published: September 2020

This chapter introduces the basics of linear elastic fracture mechanics. It starts by recalling the asymptotic elastic crack tip solutions and the concept of stress intensity factors for Mode-I, Mode-II, and Mode-III loading. The concept of critical stress intensity factor is next introduced as a model for fracture under small scale yielding conditions. In this context the limits of linear elastic facture mechanics are discussed. Further, methods and requirements for fracture toughness testing are discussed.
Sirolimus for patients with connective tissue disease-related refractory thrombocytopenia: a single-arm, open-label clinical trial
Authors: Wu Chanyuan , Wang Qian , Xu Dong , Li Mengtao , Zeng Xiaofeng
Published: September 2020

Objectives

Connective tissue disease-related thrombocytopenia (CTD-TP) is a problematic disorder in clinical practice. Because the first-line therapy of glucocorticoid and/or immunosuppressants is not effective for refractory cases, alternative treatment approaches are urgently needed. The present study investigated the efficacy and safety of sirolimus in refractory CTD-TP patients.

Methods

This single-centre, single-arm, phase II study enrolled 20 refractory CTD-TP patients between September 2017 and September 2018 (registered on ClinicalTrials.gov: NCT03688191). Oral sirolimus administration was dose-adjusted to maintain a therapeutic range of 6–15 ng/ml for 6 months. The primary endpoints were partial and complete remission rates at 6 months.

Results

Twelve (60%) patients achieved the primary end point with a 50% complete remission rate after 6 months. Among the 14 SLE patients, the overall response rate was 71.4%, with a complete remission rate of 64.3%. None of the primary Sjögren's syndrome cases responded to sirolimus. There was no significant difference in baseline clinical characteristics or lymphocyte subpopulations between responders and non-responders. No severe side effect was detected during the study.

Conclusion

Sirolimus is an effective and safe treatment option for refractory CTD-TP patients.

Trial registration

https://clinicaltrials.gov, NCT03688191.
Personality Traits as Markers of Psychosis Risk in Kenya: Assessment of Temperament and Character
Authors: Mamah Daniel , Cloninger C Robert , Mutiso Victoria N , Gitonga Isaiah , Tele Albert , Ndetei David M
Published: September 2020

Specific personality traits have been proposed as a schizophrenia-related endophenotype and confirmed in siblings at risk for psychosis. The relationship of temperament and character with psychosis has not been previously investigated in Africa. The study was conducted in Kenya, and involved participants at clinical high-risk (CHR) for psychosis (n = 268) and controls (n = 251), aged 15–25 years. CHR status was estimated using the Structured Interview of Psychosis-Risk Syndromes (SIPS) and the Washington Early Psychosis Center Affectivity and Psychosis (WERCAP) Screen. Student’s t-tests were used to assess group differences on the Temperament and Character Inventory (TCI). Neurocognitive functioning, stress severity, and substance use were correlated with the TCI, correcting for psychosis severity. CHR participants were more impulsive (ie, higher novelty seeking [NS]) and asocial (ie, lower reward dependence) than controls. They were also more schizotypal (ie, high self-transcendence [ST] and lower self-directedness [SD] and cooperativeness [CO] than controls). CO was related to logical reasoning, abstraction, and verbal memory. Stress severity correlated with high HA and schizotypal character traits. Lifetime tobacco use was related to NS, and lifetime marijuana use to high NS, low SD and high ST. Temperament and character of Kenyan CHR youth is similar to that observed in schizophrenia. Psychosis risk in Kenya is associated with impulsive, asocial, and schizotypal traits. CHR adolescents and young adults with schizophrenia-specific personality traits may be most at risk for developing a psychotic disorder and to require early intervention to improve outcomes.
The Origins of Coca: Museum Genomics Reveals Multiple Independent Domestications from ProgenitorErythroxylum gracilipes
Authors: White Dawson M , Huang Jen-Pan , Jara-Muñoz Orlando Adolfo , MadriñáN Santiago , Ree Richard H , Mason-Gamer Roberta J
Published: September 2020

Coca is the natural source of cocaine as well as a sacred and medicinal plant farmed by South American Amerindians and mestizos. The coca crop comprises four closely related varieties classified into two species (Amazonian and Huánuco varieties within Erythroxylum coca Lam., and Colombian and Trujillo varieties within Erythroxylum novogranatense (D. Morris) Hieron.) but our understanding of the domestication and evolutionary history of these taxa is nominal. In this study, we use genomic data from natural history collections to estimate the geographic origins and genetic diversity of this economically and culturally important crop in the context of its wild relatives. Our phylogeographic analyses clearly demonstrate the four varieties of coca comprise two or three exclusive groups nested within the diverse lineages of the widespread, wild species Erythroxylum gracilipes; establishing a new and robust hypothesis of domestication wherein coca originated two or three times from this wild progenitor. The Colombian and Trujillo coca varieties are descended from a single, ancient domestication event in northwestern South America. Huánuco coca was domesticated more recently, possibly in southeastern Peru. Amazonian coca either shares a common domesticated ancestor with Huánuco coca, or it was the product of a third and most recent independent domestication event in the western Amazon basin. This chronology of coca domestication reveals different Holocene peoples in South America were able to independently transform the same natural resource to serve their needs; in this case, a workaday stimulant. [Erythroxylum; Erythroxylaceae; Holocene; Museomics; Neotropics; phylogeography; plant domestication; target-sequence capture.]
Genetic parameters and trends for growth traits in Blanco Orejinegro cattle
Authors: Ramírez Toro Edison J , Burgos Paz William O , Elzo Mauricio A , Martínez Sarmiento Rodrigo A , Cerón-Muñoz Mario F
Published: September 2020

Since 1940, efforts have been made to preserve the Blanco Orejinegro (BON) cattle breed by maintaining gene banks. Nine years ago, a BON genetic improvement program was implemented to increase genetic gain based on control of productivity and the use of performance tests, polygenic and genomic evaluations, and selection indices. The objective of this study was to estimate genetic parameters and trends for growth traits by using polygenic (PM) and genomic–polygenic (GPM) models. Productive information from the years 1980 to 2019 was used. The following data were used: 7,304 birth weight (BW) records, 1,281 records of body weight adjusted to 120 days (W120), 4,791 records of weight adjusted to 240 days (W240), 3,339 records of weight adjusted to 480 days (W480), and 1,364 records of weight adjusted to 720 days (W720). The relationship matrix included 13,612 pure animals belonging to the Colombian Corporation for Agricultural Research - AGROSAVIA and the Producer Network program. A total of 1,224 individuals were genotyped with chips of different densities and all were imputed up to 50,932 SNPs. Polygenic and genomic-polygenic models were used to estimate genetic parameters and correlations between the genetic values estimated by each model. A generalized additive model with smoothing was used to estimate trends of genetic values from 1980 to 2019. Heritabilities between 0.29 and 0.40, genetic correlations between 0.13 and 0.94, and phenotypic correlations between 0.23 and 0.72 were observed. Close-to-zero genetic growth was observed for BW and W120. Genetic trends for the other traits were positive, with higher growth in the last 7 years. The heritabilities observed in this population indicate that the traits measured would respond to selection. Greater genetic progress can be achieved in W240, W480, and W720 by continuing with genetic evaluations and developing performance tests and strategies to make genetic material readily available to producers.
Shade tree species affect gas exchange and hydraulic conductivity of cacao cultivars in an agroforestry system
Authors: Ávila-Lovera Eleinis , Blanco Héctor , Móvil Olga , Santiago Louis S , Tezara Wilmer
Published: September 2020

Shade tolerance is a widespread strategy of rainforest understory plants. Many understory species have green young stems that may assimilate CO2 and contribute to whole-plant carbon balance. Cacao commonly grows in the shaded understory and recent emphasis has been placed on diversifying the types of trees used to shade cacao plants to achieve additional ecosystem services. We studied three agricultural cacao cultivars growing in the shade of four timber species (Cedrela odorata L., Cordia thaisiana Agostini, Swietenia macrophylla King and Tabebuia rosea (Bertol) A.D.C.) in an agroforestry system to (i) evaluate the timber species for their effect on the physiological performance of three cacao cultivars; (ii) assess the role of green stems on the carbon economy of cacao; and (iii) examine coordination between stem hydraulic conductivity and stem photosynthesis in cacao. Green young stem photosynthetic CO2 assimilation rate was positive and double leaf CO2 assimilation rate, indicating a positive contribution of green stems to the carbon economy of cacao; however, green stem area is smaller than leaf area and its relative contribution is low. Timber species showed a significant effect on leaf gas exchange traits and on stomatal conductance of cacao, and stem water-use efficiency varied among cultivars. There were no significant differences in leaf-specific hydraulic conductivity among cacao cultivars, but sapwood-specific hydraulic conductivity varied significantly among cultivars and there was an interactive effect of cacao cultivar × timber species. Hydraulic efficiency was coordinated with stem-stomatal conductance, but not with leaf-stomatal conductance or any measure of photosynthesis. We conclude that different shade regimes determined by timber species and the interaction with cacao cultivar had an important effect on most of the physiological traits and growth variables of three cacao cultivars growing in an agroforestry system. Results suggested that C. odorata is the best timber species to provide partial shade for cacao cultivars in the Barlovento region in Venezuela, regardless of cultivar origin.
Defining ‘Family Members’ of EU Citizens and the Circumstances under Which They Can Rely on EU Law
Authors: Milios Georgios
Published: September 2020

The present article deals with the legal concept of family members of EU citizens in EU and national legislation. In particular, it examines the legal definition of family in Directive 2004/38, as well as the way the Court of Justice of the EU has interpreted the relevant provisions of the Directive. Not least, the present article focuses on the circumstances under which these persons may qualify as family members according to the case law of the CJEU. As for the research at national level, the present study examines the way the same issues are regulated in national implementing legislation of Spain. The article concludes that although the definition of family in Directive 2004/38 is still focused on a formal and traditional family model, the CJEU has quite progressively adopted a more expansive interpretation in relation to this concept. Regardless of this finding, this article argues that a more de facto approach is still necessary in order for the applicable rules to become more compatible with the reality of personal relations nowadays but also with the international human rights standards.
Antifungal Activity of Plant-Derived Essential Oils on Pathogens of Pulse Crops
Authors: Parikh Lipi , Agindotan Bright O. , Burrows Mary E.
Published: September 2020

Pulse crops such as chickpeas, lentils, and dry peas are grown widely for human and animal consumption. Major yield- and quality-limiting constraints include diseases caused by fungi and oomycetes. The environmental and health concerns of synthetic fungicides used for disease management, emergence of fungicide-resistant pathogens, and demand for organic pulse crop products necessitate the search for effective alternatives. Safe and environmentally friendly plant-derived essential oils (EOs) have been reported effective against some pathogenic fungi. Growth on EO-amended growth medium and an inverted Petri plate assay were used to determine the effects of 38 oils and their volatiles on mycelial growth and spore germination of important pathogenic fungi and oomycetes: Aphanomyces euteiches, Botrytis cinerea, Colletotrichum lentis, Didymella pisi, D. rabiei, D. lentis, Fusarium avenaceum, Stemphylium beticola, Sclerotinia sclerotiorum, and Pythium sylvaticum. Palmarosa, oregano, clove, cinnamon, lemongrass, citronella, and thyme oils incorporated in media inhibited mycelial growth of all the pathogens by 100% at 1:1,000 to 1:4,000 dilution. In addition, thyme oil (1:500 dilution) showed complete inhibition of conidial germination (0% germination) of F. avenaceum and D. pisi. All seven EO volatiles inhibited mycelial growth of all pathogens by 50 to 100% except for B. cinerea and S. sclerotiorum. EO effects on mycelial growth were fungistatic, fungicidal, or both and varied by EO. EOs show potential for management of major crop diseases in organic and conventional production systems.
Managing patients with sickle cell disease in primary care
Authors: Hehre Robert J.
Published: September 2020

Sickle cell disease (SCD) is a group of disorders affecting the hemoglobin in erythrocytes. SCD is associated with significant morbidity and mortality and occurs most commonly among people of African ancestry. In 2014, the National Heart, Lung, and Blood Institute updated its guidelines for the management of SCD. These guidelines were implemented to provide evidence-based recommendations to assist primary care clinicians in the proper management of patients with SCD. This article reviews the current practice guidelines for SCD, with attention to health maintenance and hydroxyurea.
Primary immune regulatory disorders: a growing universe of immune dysregulation
Authors: Chan Alice Y. , Torgerson Troy R.
Published: September 2020

Purpose of review

Primary immune regulatory disorders (PIRD) are a growing subset of diseases referred to as inborn errors of immunity. Unlike classical primary immune deficiency disorders that typically present with severe, recurrent, or unusual infections, the clinical manifestations of PIRD are dominated by immune-mediated diseases (autoimmunity, autoinflammation/hyperinflammation, lymphoproliferation, malignancy, and severe atopy). This review introduces the concept of PIRD including clinical phenotypes, treatments, and new PIRD-associated gene defects.

Recent findings

The number of genetic defects associated with PIRD is rapidly growing. The identified genes often encode proteins that play critical roles in regulating the immune response to various triggers. Understanding the molecular mechanisms underlying PIRD has shed light on the clinical phenotypes and has helped to identify targeted therapies. In some cases, hematopoietic cell transplant (HCT) has been successfully employed as a cure.

It is important to recognize the broad clinical manifestations of PIRD as patients may have symptoms atypical of classical ‘immunodeficiency’. Because of their diverse immune dysregulation problems, they are often primarily managed by other subspecialists. Immunologists can help connect the diverse immune-mediated pathologies to a gene defect. This, in turn, can play a significant role in directing clinical management, selecting effective therapy, and deciding on appropriateness of HCT.
Building the Next Generation of Researchers: Mentored Training in Dissemination and Implementation Science
Authors: Brownson Ross C. , Jacob Rebekah R. , Carothers Bobbi J. , Chambers David A. , Colditz Graham A. , Emmons Karen M. , Haire-Joshu Debra , Kerner Jon F. , Padek Margaret , Pfund Christine , Sales Anne
Published: September 2020

Problem

Dissemination and implementation (D&I) science provides the tools needed to close the gap between known intervention strategies and their effective application. The authors report on the Mentored Training for Dissemination and Implementation Research in Cancer (MT-DIRC) program—a D&I training program for postdoctoral or early-career cancer prevention and control scholars.

Approach

MT-DIRC was a 2-year training institute in which fellows attended 2 annual Summer Institutes and other conferences and received didactic, group, and individual instruction; individualized mentoring; and other supports (e.g., pilot funding). A quasi-experimental design compared changes in 3 areas: mentoring, skills, and network composition. To evaluate mentoring and D&I skills, data from fellows on their mentors’ mentoring competencies, their perspectives on the importance of and satisfaction with mentoring priority areas, and their self-rated skills in D&I competency domains were collected. Network composition data were collected from faculty and fellows for 3 core social network domains: contact, mentoring, and collaboration. Paired t tests (mentoring), linear mixed models (skills), and descriptive analyses (network composition) were performed.

Outcomes

Mentors were rated as highly competent across all mentoring competencies, and each mentoring priority area showed reductions in gaps between satisfaction and importance between the 6 and 18 months post-first Summer Institute. Fellows’ self-rated skills in D&I competencies improved significantly in all domains over time (range: 42.5%–52.9% increase from baseline to 18 months post-first Summer Institute). Mentorship and collaboration networks grew over time, with the highest number of collaboration network ties for scholarly manuscripts (n = 199) in 2018 and for research projects (n = 160) in 2019.

Next Steps

Building on study findings and existing literature, mentored training of scholars is an important approach for building D&I skills and networks, and thus to better applying the vast amount of available intervention evidence to benefit cancer control.
Percutaneous, Intra-articular, Chevron Osteotomy (PeICO) for the Treatment of Hallux Valgus
Authors: Del Vecchio Jorge J. , Ghioldi Mauricio E. , Uzair Anuar E. , Chemes Lucas N. , Dealbera Eric D. , Dalmau-Pastor Miki
Published: September 2020

Percutaneous (also called minimally invasive surgery) surgery has advanced and, nowadays, is a valid option for the treatment of hallux valgus deformities. This fact is strongly supported by different studies. Early results of the third-generation procedures seem very promising with at least comparable deformity correction and less postoperative stiffness and pain compared with some open osteotomies. Percutaneous, intra-articular, chevron osteotomy is one of this type of procedures and presents some advantages when comparing with other third-generation surgeries. Percutaneous, intra-articular, chevron osteotomy accurately reproduces the open chevron technique with all its known advantages. Our recommendation is that no surgeon should begin doing percutaneous surgery until they have gone through specific cadaveric training.

Level of Evidence: Diagnostic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Prehab and Rehab for Femoroacetabular Impingement (FAI): Clinical Pearls
Authors: Horschig Aaron
Published: September 2020

Current literature supports the benefits of nonoperative conservative treatment with physical therapy along with activity modification for femoroacetabular impingement. Despite this fact, there is a gap in the literature detailing appropriate physical therapy management of this injury. The goal of this article is to present conservative physical therapy treatment options for femoroacetabular impingement. Improving joint mobility through posterior and lateral joint glide of the femur, strengthening the surrounding hip musculature, and re-educating functional movement to limit excessive adduction/internal rotation during hip flexion can be helpful for decreasing symptoms and allowing patients to return to their prior level of function.
Appropriate therapy for patients with stable ischemic heart disease: a review of literature and the implication of the International Study of Comparative Effectiveness with Medical and Invasive Approaches trial
Authors: Guo Ming Hao , Nantsios Alex , Ruel Marc
Published: September 2020

Purpose of review

The aim of this review is to review the current literature on the management of stable ischemic heart disease in light of findings from the International Study of Comparative Effectiveness with Medical and Invasive Approaches (ISCHEMIA) trial.

Recent findings

Compared with optimal medical therapy, coronary artery bypass grafting (CABG) has been shown to improve survival and incidence of myocardial infarction in patients with stable ischemic heart disease whereas percutaneous coronary intervention (PCI) has not. The ISCHEMIA trial compared an initial conservative management strategy with optimal medical therapy with an initial invasive strategy with coronary angiogram followed by revascularization and found no difference in survival and myocardial infarction between the two groups. However, the trial was conducted under the assumption that patients would be referred to the most efficacious revascularization therapy; it was apparent that CABG was underutilized in many appropriate patients in the ISCHEMIA trial. The study highlighted the importance of optimizing medical therapy in stable ischemic heart disease (SIHD) patients but does not answer whether revascularization or conservative management is better for patients with SIHD.

Optimal medical therapy is an important element and should be included in the Heart Team discussion, and also applied after revascularization from PCI or CABG. Cardiac surgeons need to continue to advocate for the appropriate use of CABG based on the current available evidence.
Using a Likelihood Heuristic to Summarize Conflicting Literature on Predictors of Clinical Outcome Following Sport-Related Concussion
Authors: Büttner Fionn , Terry Douglas P. , Iverson Grant L.
Published: September 2020

Objective:

To introduce a new methodology for summarizing the results from systematic reviews–a likelihood heuristic–to the field of sport-related concussion.

Data Sources:

We applied the likelihood heuristic to the results of a systematic review published by Iverson et al (2017), containing 101 studies, on the predictors of worse clinical outcome following sport-related concussion.

Study Selection:

We re-examined 5 individual prognostic factors that are of clinical interest and for which there is conflicting literature (female sex = 44 studies, prior concussion history = 41 studies, loss of consciousness = 31 studies, post-traumatic amnesia = 25 studies, and retrograde amnesia = 10 studies).

Data Extraction:

For each prognostic factor, likelihood ratios were generated using the (1) number of significant and nonsignificant studies, (2) study power, (3) alpha level, and (4) prior probability that the alternative hypothesis was true.

Data Synthesis:

Assuming each study had 80% power and an alpha level of 5%, observing the reported number of conflicting studies for female sex, prior concussion history, and retrograde amnesia is substantially more likely if each prognostic factor is associated with worse clinical outcome following sport-related concussion. For loss of consciousness, the observed number of conflicting studies is more likely if loss of consciousness is not associated with worse clinical outcome following sport-related concussion. A secondary analysis incorporating potentially more realistic study parameters of statistical power (45%) and alpha level (25%) generates weaker likelihood evidence that the observed numbers of studies for each prognostic factor are associated with worse clinical outcome following sport-related concussion than they are not.

Conclusions:

Using a likelihood heuristic with 80% power and 5% alpha level, there is very strong likelihood evidence that female sex, prior concussion history, and retrograde amnesia are associated with worse clinical outcome following sport-related concussion. The strength of likelihood evidence that prognostic factors are associated with worse clinical outcome reduces when statistical power is lower and alpha level is inflated.
Keratoconus Patient Satisfaction and Care Burden with Corneal Gas-permeable and Scleral Lenses
Authors: Shorter Ellen , Schornack Muriel , Harthan Jennifer , Nau Amy , Fogt Jennifer , Cao Dingcai , Nau Cherie
Published: September 2020

SIGNIFICANCE

Scleral lenses (SLs) are increasingly being considered as the initial correction for patients with keratoconus. In this study, keratoconus patients report higher levels of comfort and visual satisfaction with SL compared with corneal gas-permeable lenses (GPs).

PURPOSE

This study aimed to compare patient satisfaction and care burden associated with GP and SL for the management of keratoconus.

METHODS

An electronic survey was distributed by the National Keratoconus Foundation from October 2016 to March 2017. Age at diagnosis, initial and current treatment, lens complications, access to care, lens handling time, and annual out-of-pocket treatment costs were collected. Vision, lens comfort, and ease of use satisfaction were rated from 1 to 5.

RESULTS

A total of 422 responses were received, including 75 bilateral GP and 76 bilateral SL wearers. Scleral lens wearers had greater satisfaction with vision (3.2 ± 1.1 [SL] vs. 2.6 ± 1.1 [GP]; P < .001) and comfort (3.3 ± 1.0 [SL] vs. 2.2 ± 1.2 [GP]; P < .001) but similar ease of use satisfaction in both groups (2.8 ± 1.1 [SL] vs. 2.7 ± 1.1 [GP]; P < .90). Both groups reported issues with cloudy or foggy vision (GP, 63%; SL, 58%) and contact lens discomfort (GP, 77%; SL, 67%). Although GP wearers reported more issues with lens movement or loss (40 [GP] vs. 18% [SL]), they had fewer difficulties with halos (53 vs. 72% [SL]) and lens handling (40%) compared with SL wearers (63%). Gas-permeable lens (48%) and SL (45%) wearers spend 6 to 10 minutes daily handling their lenses. Sixty percent of GP wearers reported annual out-of-pocket cost expenses less than U.S.$1000, whereas only 41% of SL wearers reported the same.

CONCLUSIONS

Scleral lens wearers with keratoconus report greater satisfaction with vision and comfort than do GP wearers, although both groups reported cloudy vision and lens discomfort.
Differences between Inferior and Superior Bulbar Conjunctiva Goblet Cells in Scleral Lens Wearers: A Pilot Study
Authors: Macedo-de-Araújo Rute J. , Serramito-Blanco Maria , van der Worp Eef , Carracedo Gonzalo , González-Méijome José M.
Published: September 2020

SIGNIFICANCE

Scleral lenses (SLs) rest on the scleroconjunctival region, which could result in a mechanical impact in the bulbar conjunctiva that can hypothetically modify some properties of conjunctival cells.

PURPOSE

The purpose of this study was to evaluate the differences in goblet cell density (GCD) and mucin cloud amplitude (MCA) between superior and inferior bulbar conjunctiva in SL wearers.

METHODS

A total of 26 eyes wearing SL were randomly selected from 26 subjects (11 females) with different grades of keratoconus enrolled in a prospective clinical series. Superior and inferior conjunctival impression cytologies were performed and therefore analyzed with scanning laser confocal microscopy to evaluate GCD and MCA. All subjects filled out the Ocular Surface Disease Index (OSDI) questionnaire.

RESULTS

The mean ± standard deviation OSDI score was 23.62 ± 15.12. Although a higher density of goblet cells was observed in the samples taken in the superior conjunctiva (74.70 ± 57.55 cells/mm2) than on the inferior conjunctiva (55.91 ± 34.80 cells/mm2), there were no statistically significant differences between them (P = .14, Wilcoxon). Regarding MCA, no differences were found between superior (21.81 ± 3.30 μm) and inferior (20.72 ± 2.95 μm) samples (P = .201, Wilcoxon). No statistically significant differences were found in GCD and MCA regarding the time of SL wear.

CONCLUSIONS

There were no differences in GCD and MCA in the samples taken in the superior and inferior conjunctival areas. Also, it seems that the SL wearing time does not affect the density and secretion of goblet cells. Prospective studies need to be conducted in larger samples to confirm those outcomes.
Pediatric Sepsis in Community Emergency Care Settings: Guideline Concordance and Outcomes
Authors: Greenwald Emily , Olds Elizabeth , Leonard Jan , Deakyne Davies Sara J. , Brant Julia , Scott Halden F.
Published: September 2020

Objectives

Bundled pediatric sepsis care has been associated with improved outcomes in tertiary pediatric emergency departments. Sepsis care at nontertiary sites where most children seek emergency care is not well described. We sought to describe the rate of guideline-concordant care, and we hypothesized that guideline-concordant care in community pediatric emergency care settings would be associated with decreased hospital length of stay (LOS).

Method

This retrospective cohort study of children with severe sepsis presenting to pediatric community emergency and urgent care sites included children 60 days to 17 years with severe sepsis. The primary predictor was concordance with the American College of Critical Care Medicine 2017 pediatric sepsis resuscitation bundle, including timely recognition, vascular access, intravenous fluids, antibiotics, vasoactive agents as needed.

Results

From January 1, 2015, to December 31, 2017, 90 patients with severe sepsis met inclusion criteria; 22 (24%) received guideline-concordant care. Children receiving concordant care had a median hospital LOS of 95.3 hours (50.9–163.8 hours), with nonconcordant care, LOS was 88.3 hours (57.3–193.2 hours). In adjusted analysis, guideline-concordant care was not associated with hospital LOS (incident rate ratio, 0.99 [0.64–1.52]). The elements that drove overall concordance were timely recognition, achieved in only half of cases, vascular access, and timely antibiotics.

Conclusions

Emergency care for pediatric sepsis in the community settings studied was concordant with guidelines in only 24% of the cases. Future study is needed to evaluate additional drivers of outcomes and ways to improve sepsis care in community emergency care settings.
The Role of Targeted Therapy in the Management of High-Risk Resected Kidney Cancer
Authors: Brown Landon C. , Zhang Tian , George Daniel J.
Published: September 2020

The primary treatment for localized renal cell carcinoma (RCC) is surgical resection with curative intent. Despite this, many patients, especially those with high-risk features, will develop recurrent or metastatic disease. Antiangiogenic therapies targeting vascular endothelial growth factor have been a mainstay of treatment of advanced RCC for more than 10 years. Evidence supporting the use of these therapies in the adjuvant setting is mixed, although one clinical trial, S-TRAC, has shown improvements in disease-free survival with 1 year of adjuvant sunitinib among patients with clear cell histology and high-risk features, leading to the first US Food and Drug Administration approval of an adjuvant therapy for high-risk RCC patients. Further investigation into combination therapies with immunotherapy, neoadjuvant approaches, and patient selection will be key to determining optimal adjuvant therapy regimens to improve outcomes and increase cure rates for patients with localized RCC.