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BackgroundGenetic variants in calsequestrin-2 (CASQ2) cause an autosomal recessive form of catecholaminergic polymorphic ventricular tachycardia (CPVT), although isolated reports have identified arrhythmic phenotypes among heterozygotes. Improved insight into the inheritance patterns, arrhythmic risks, and molecular mechanisms of CASQ2-CPVT was sought through an international multicenter collaboration.MethodsGenotype-phenotype segregation in CASQ2-CPVT families was assessed, and the impact of genotype on arrhythmic risk was evaluated using Cox regression models. Putative dominant CASQ2 missense variants and the established recessive CASQ2-p.R33Q variant were evaluated using oligomerization assays and their locations mapped to a recent CASQ2 filament structure.ResultsA total of 112 individuals, including 36 CPVT probands (24 homozygotes/compound heterozygotes and 12 heterozygotes) and 76 family members possessing at least 1 presumed pathogenic CASQ2 variant, were identified. Among CASQ2 homozygotes and compound heterozygotes, clinical penetrance was 97.1% and 26 of 34 (76.5%) individuals had experienced a potentially fatal arrhythmic event with a median age of onset of 7 years (95% CI, 6-11). Fifty-one of 66 CASQ2 heterozygous family members had undergone clinical evaluation, and 17 of 51 (33.3%) met diagnostic criteria for CPVT. Relative to CASQ2 heterozygotes, CASQ2 homozygote/compound heterozygote genotype status in probands was associated with a 3.2-fold (95% CI, 1.3-8.0; P=0.013) increased hazard of a composite of cardiac syncope, aborted cardiac arrest, and sudden cardiac death, but a 38.8-fold (95% CI, 5.6-269.1; P

This essay offers a reorientation of our views on the interrelationships of language and thought as a field of constantly reprogrammable energy, and provides an argument as to why we believe this new metaphor (i.e., language as a field of energy) matters in language pedagogy, in classrooms at all levels, as well as within teacher education and teacher professional development. We define language as a field of energy in the following way: as language operating as a "region" in which a force (in this case, words and their rhetorical functions) operates to bring about some influence resulting in an effect or having an impact on one's own behaviors, on the behaviors of others, as well as having the capacity to influence emotions, and/or the course of events. Following a brief introduction in which we state our purpose, we present the case for the above argument in the context of current language and literacy education. In doing so, we delineate language-thought-perceived reality relationships through a synthesis of a selective representation of the primary thinkers in mainstream language scholarship, as well as other fields, such as philosophy, social anthropology, linguistics, discourse analysis, and to coin a new field, "linguistic metaphysics." While we focus our discussion on language, we are mindful of its obviously intimate, symbiotic relationship with thought. We conclude with suggestions as to how this concept, "language as a field of energy," can be pursued in educational settings.

What happens to a dream deferred? Does it dry up like a raisin in the sun? --LANGSTON HUGHES, FROM 'HARLEM' In January 2022, one day after the holiday to commemorate [...]

The disproportionate impact of environmental degradation on First Nations peoples and on other communities of color is not new. Indigenous peoples, Black people, and other marginalized communities experience the consequences of environmental degradation disproportionately (Taylor, 2014; Washington, 2019), telling us that environmental justice and social justice are not unrelated topics. One way that educators can help students to understand how this is the case is by creating opportunities for them to examine literature that addresses environmental and conservation topics through a lens of intersectional environmentalism. In this article, the authors define intersectional environmentalism in terms of critical multicultural analysis and examine how this critical perspective builds on assumptions associated with ecofeminism, a branch of feminism that argues that the same ideology that authorizes environmental degradation is also behind the oppression of women, people of color, people experiencing poverty, and the Earth itself. In doing so, they identify three questions that together compose a critical framework for examining how texts for the young that address environmental and conservation topics depict the relationship between environmental justice and social justice. They next apply the framework to Eliot Schrefer's (2012) "Endangered," a work of fiction that is read by many middle-grade readers, to demonstrate how examining the text through a lens of intersectional environmentalism opens up new possibilities for understanding it. To conclude, they examine the implications for educators of asking students to critique literary texts from a perspective of intersectional environmentalism.

This article draws on data from a qualitative research study that asked how six Latinos living in a southern state used literacy to enact, defend, and transform their identities as transnational youths in response to evolving social conditions they and their families encountered in the United States. In sharing the experiences of three participants, the authors demonstrate how the participants maintained their affiliation with the Latino community in part by using reading and writing to engage in community activism and highlight social and political issues facing Latino immigrants in the region where they lived. The authors conclude by discussing the implications of their research for English language arts teachers.

This paper reports findings from a study of two beginning English teachers and the relationship between their sense of agency and their respective contexts. The qualitative study followed two teachers through their first year of teaching. How the perception of agency and the role of being a teacher evolved was determined to be related to the organizational behavior and expectations that both teachers experienced in their respective contexts. This study suggests that organizational context matters in the continuing development and maintenance of agency of beginning teachers and the type of agency that teachers might develop is influenced by organizational expectations.

This research study explores the role of social annotation in supporting dialogic teaching in secondary English language arts. Grounded in Bakhtin's dialogism and building upon research into online discussion, this study describes how a high school English teacher and her students used a digital annotation tool to read and talk about texts. Analyzing student annotations based on discourse features associated with comprehension and high‐level thinking, the study examines the extent to which social annotation supports quality dialogue. Findings highlight the need for open‐ended prompts and teacher scaffolding of online discussions, and authors suggest that dialogue enhances comprehension of texts when students go beyond reporting on others' thoughts and instead share their own ideas, connections, and questions about the text. [ABSTRACT FROM AUTHOR]

This longitudinal study examined how the approach leaders in two schools took to implementing the Common Core State Standards shaped the way that two first-year teachers constructed meaning related to being a teacher. Instructional leadership constructs and threat rigidity theory were used to analyze qualitative data gathered over a nine-month period. Findings indicate that the way schools as organizations respond to external mandates can influence the way that beginning teachers conceptualize, and approach, their work in the classroom.

Building on scholarship that emphasizes a relationship between critical literacy and place-conscious pedagogy, this article describes a community inquiry project that asked undergraduates in an introductory literacy studies course to use oral histories that they collected from people in the Arkansas Ozark region to engage in placemaking and reclaim narratives about literacy in the area where they lived. The Literacy in Ozark Lives project challenged the students to examine the oral histories with an eye toward identifying people and institutions that sponsored their interviewees' experiences with reading and writing, and explaining how social and economic transformations in the region shaped the purpose of their interviewees' literacy. To conclude the project, the students produced digital video essays intended to complicate problematic cultural models that promote a deficit view of people and literacy in the U.S. South, particularly the Arkansas Ozark region.

This study examines the relationship between two first-year teachers and how organizational behavior influences their socialization when implementing an external mandate. The primary interests of this qualitative study are the organization behavior related to socializing newcomers, the manner in which new teaching roles and behaviors are realized, and the potential tension that can exist between what teachers believe their role should be and the role expectations of the organization. The findings suggest that how schools react to external mandates can influence the manner in which new teachers are socialized into their new role, potentially resulting in less desirable teaching practices.

Until recently, the standard of care for aortic valve disease had been surgical aortic valve replacement (SAVR), with reported low mortality rates (1%-2%). (1-3) Advances in catheter techniques have led [...], Background: Transcatheter aortic valve implantation (TAVI) is evolving rapidly and is increasingly being adopted in the treatment of aortic valve disease. The goal of this study was to examine regional differences in surgical aortic valve replacement (SAVR) and TAVI across Atlantic Canada. Methods: We identified all patients who underwent SAVR or TAVI between Jan. 1, 2010, and Dec. 31, 2014, in New Brunswick, Nova Scotia and Newfoundland and Labrador. Data obtained included patient demographic characteristics and surgical procedure details. We performed univariate descriptive analyses and calculated crude and age- and sex-adjusted incidence rates. Results: A total of 3042 patients underwent SAVR or TAVI during the study period, 1491 in Nova Scotia, 1042 in New Brunswick and 509 in Newfoundland and Labrador. Patient demographic characteristics were similar across regions. A much higher proportion of patients in Newfoundland and Labrador (43.6%) than in Nova Scotia (4.2%) or New Brunswick (13.6%) received a mechanical versus a bioprosthetic valve. Rates of TAVI increased over the study period, with New Brunswick adopting their program before Nova Scotia (144 v. 74 procedures). Adjusted rates of all AVR procedures remained stable in Nova Scotia (40-50 per 100 000 people). Adjusted rates were lower in New Brunswick and Newfoundland and Labrador than in Nova Scotia; they increased slowly in New Brunswick over the study period. Conclusion: Despite geographical proximity and similar patient demographic characteristics, there existed regional differences in the management of aortic valve disease within Atlantic Canada. Further study is required to determine whether the observed differences in age- and sex-adjusted rates of AVR may be explained by geographical disease-related differences, varying practice patterns or barriers in access to care. Contexte : Le remplacement valvulaire aortique par catheter, une methode en pleine evolution, est de plus en plus utilise pour le traitement des valvulopathies aortiques. Cette etude visait a examiner les differences regionales quant au remplacement valvulaire aortique par catheter ou par chirurgie dans les provinces de lAtlantique. Methodes : Nous avons recense tous les patients ayant subi un remplacement valvulaire aortique entre le 1 (er) janvier 2010 et le 31 decembre 2014 au Nouveau-Brunswick, en Nouvelle-Ecosse et a Terre-Neuve-et-Labrador. Nous avons recueilli des donnees sur les caracteristiques demographiques des patients et les interventions chirurgicales, puis nous avons realise une analyse descriptive univariee et avons calcule les taux d'incidence bruts et corriges selon l'age et le sexe. Resultats : En tout, 3042 patients ont subi un remplacement valvulaire aortique par catheter ou par chirurgie pendant la periode a l'etude : 1491 en Nouvelle-Ecosse, 1042 au Nouveau-Brunswick et 509 a Terre-Neuve-et-Labrador. Les caracteristiques demographiques des patients etaient semblables d'une region a l'autre. La proportion des patients recevant une prothese mecanique plutot qu'une bioprothese etait beaucoup plus elevee a Terre-Neuve-et-Labrador (43,6 %) qu'en Nouvelle-Ecosse (4,2 %) ou au Nouveau-Brunswick (13,6 %). Les taux de remplacement par catheter ont augmente au cours de la periode a l'etude; le Nouveau-Brunswick a adopte un programme a ce sujet avant la Nouvelle-Ecosse (144 c. 74 interventions). Les taux corriges pour tous les remplacements etaient stables en Nouvelle-Ecosse (40-50 par 100 000 habitants); ils etaient plus faibles au Nouveau-Brunswick et a Terre-Neuve-et-Labrador, mais ont augmente lentement au Nouveau-Brunswick pendant la pe riode a l'etude. Conclusion : Malgre la proximite geographique des provinces de lAtlantique et les caracteristiques demographiques semblables des patients, il existait des differences dans la prise en charge des valvulopathies aortiques. D'autres etudes seront necessaires pour determiner si les variations dans les taux de remplacement corriges selon l'age et le sexe pourraient s'expliquer par des differences geographiques dans le nombre de cas, des differences dans les pratiques ou des obstacles a l'acces aux soins.

When teachers work with students to construct a metalanguage that they can draw on to describe and analyze graphic novels, and then invite students to apply that metalanguage in the service of composing multimodal texts of their own, teachers broaden students' analytical frameworks. In the process of doing so, teachers empower students. In this article, Connors states that reading and producing graphic novels with the intention of mapping their multimodal design can heighten students' appreciation for the form's complexity.

This article recounts what happened when one of the authors, a pre-service teacher, introduced a digital multimodal composition project in her 9th-grade inclusion English classes to support junior high students as they read the novel "To Kill a Mockingbird". Rather than regard new literacies as competing with print literacy for attention, the authors argue that teachers serve students best when they design assignments that blend old and new literacies in ways that allow them to coexist and inform one another. (Contains 1 figure.)

Literacy educators might advocate using graphic novels to develop students' visual literacy skills, but teachers who lack a vocabulary for engaging in close analysis of visual texts may be reluctant to teach them. Recognizing this, teacher educators should equip preservice teachers with a vocabulary for analyzing visual texts. This article foregrounds three analytic concepts preservice teachers can use to examine the role issues of design play in eliciting their response to images--specifically, those in graphic novels. To demonstrate how the concepts function, the author applies them to his own reading of panels excerpted from a critically acclaimed graphic novel for adolescents. (Contains 6 figures.)

This article reports the author's experiences using graphic novels with pre-service teachers in a young adult literature course. Drawing on critical response papers two students composed after reading "Pride of Baghdad," a graphic novel by Brian K. Vaughan and Niko Henrichon, the author argues that when readers possess the background knowledge needed to approximate the role of the implied reader--that is, the imaginary audience for whom authors envision themselves writing--they are capable of engaging with graphic novels in ways that readers who lack experience with the form, or who question its literary merit, are not.

This article reports a study of a university supervisor and a preservice English language arts teacher as they worked collaboratively within two different field experience sites to develop a conceptual understanding of instructional scaffolding. An analysis of classroom observations and mentoring conversations was conducted to examine how the supervisor supported the preservice teacher's transfer of tools for instruction from a university program to the high school classroom. Findings indicate the significant role of the teacher education program's conceptual base in fostering this transfer. (Contains 2 tables and 3 notes.)

Through their study of classroom talk about and around literary text, the authors discovered that their application of Rosenblatt's (1938/1995, 1978) "aesthetic" stance to elementary (primarily Grades 4-6) students' affective responses to literary text uniformly lacked the simultaneous articulation of "the real impact between the book and the mind of the reader" (p. 72), that is, what Rosenblatt (1978) and Bogdan (1992) had in mind when they conceptualized "aesthetic" response. The authors' solution was to search for a descriptor that would adequately capture what they saw in these responses, that is, responses that were indeed affective (personal, emotional) and experiential. They found that Jakobson's (1987) expressive function of language could more appropriately and consistently describe such responses. They are aware that the concept "expressive" carries with it some sizable baggage related to how it has been used and perceived in the domain of writing instruction (most notably promoted by Elbow, 1973). This article, then, focuses on a conceptual discussion that provides an argument for utilizing what the authors have termed "expressive response" to account for readers' personal connections to the texts they have read in terms of "memories and free associations" (Rosenblatt, 1978, p. 151). This then leads to an exploration of the possibility of a "third" stance that can account for the kinds of responses that any reader might make at different times and for different reasons as well as Rosenblatt's (1938/1995, 1978) aesthetic response. The descriptor "aesthetic response" may have been asked to do work for which it was never intended. (Contains 3 figures.)