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The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessively inherited disease that has undergone extensive phenotypic expansion since being first described in patients with fevers, recurrent strokes, livedo racemosa, and polyarteritis nodosa in 2014. It is now recognized that patients may develop multisystem disease that spans multiple medical subspecialties. Here, we describe the findings from a large single center longitudinal cohort of 60 patients, the broad phenotypic presentation, as well as highlight the cohort's experience with hematopoietic cell transplantation and COVID-19. Disease manifestations could be separated into three major phenotypes: inflammatory/vascular, immune dysregulatory, and hematologic, however, most patients presented with significant overlap between these three phenotype groups. The cardinal features of the inflammatory/vascular group included cutaneous manifestations and stroke. Evidence of immune dysregulation was commonly observed, including hypogammaglobulinemia, absent to low class-switched memory B cells, and inadequate response to vaccination. Despite these findings, infectious complications were exceedingly rare in this cohort. Hematologic findings including pure red cell aplasia (PRCA), immune-mediated neutropenia, and pancytopenia were observed in half of patients. We significantly extended our experience using anti-TNF agents, with no strokes observed in 2026 patient months on TNF inhibitors. Meanwhile, hematologic and immune features had a more varied response to anti-TNF therapy. Six patients received a total of 10 allogeneic hematopoietic cell transplant (HCT) procedures, with secondary graft failure necessitating repeat HCTs in three patients, as well as unplanned donor cell infusions to avoid graft rejection. All transplanted patients had been on anti-TNF agents prior to HCT and received varying degrees of reduced-intensity or non-myeloablative conditioning. All transplanted patients are still alive and have discontinued anti-TNF therapy. The long-term follow up afforded by this large single-center study underscores the clinical heterogeneity of DADA2 and the potential for phenotypes to evolve in any individual patient.
Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients With VEXAS
(2021)
BACKGROUND
Biallelic loss-of-function variants in NCF1 lead to reactive oxygen species deficiency and chronic granulomatous disease (CGD). Heterozygosity for the p.Arg90His variant in NCF1 has been associated with susceptibility to systemic lupus erythematosus, rheumatoid arthritis, and Sjögren's syndrome in adult patients. This study demonstrates the association of the homozygous p.Arg90His variant with interferonopathy with features of autoinflammation and autoimmunity in a pediatric patient.
CASE PRESENTATION
A 5-year old female of Indian ancestry with early-onset recurrent fever and headache, and persistently elevated antinuclear, anti-Ro, and anti-La antibodies was found to carry the homozygous p.Arg90His variant in NCF1 through exome sequencing. Her unaffected parents and three other siblings were carriers for the mutant allele. Because the presence of two NCF1 pseudogenes, this variant was confirmed by independent genotyping methods. Her intracellular neutrophil oxidative burst and NCF1 expression levels were normal, and no clinical features of CGD were apparent. Gene expression analysis in peripheral blood detected an interferon gene expression signature, which was further supported by cytokine analyses of supernatants of cultured patient's cells. These findings suggested that her inflammatory disease is at least in part mediated by type I interferons. While her fever episodes responded well to systemic steroids, treatment with the JAK inhibitor tofacitinib resulted in decreased serum ferritin levels and reduced frequency of fevers.
CONCLUSION
Homozygosity for p.Arg90His in NCF1 should be considered contributory in young patients with an atypical systemic inflammatory antecedent phenotype that may evolve into autoimmunity later in life. The complex genomic organization of NCF1 poses a difficulty for high-throughput genotyping techniques and variants in this gene should be carefully evaluated when using the next generation and Sanger sequencing technologies. The p.Arg90His variant is found at a variable allele frequency in different populations, and is higher in people of South East Asian ancestry. In complex genetic diseases such as SLE, other rare and common susceptibility alleles might be necessary for the full disease expressivity.
Neurodevelopmental disorder with dysmorphic facies and distal limb anomalies (NEDDFL), defined primarily by developmental delay/intellectual disability, speech delay, postnatal microcephaly, and dysmorphic features, is a syndrome resulting from heterozygous variants in the dosage-sensitive bromodomain PHD finger chromatin remodeler transcription factor BPTF gene. To date, only 11 individuals with NEDDFL due to de novo BPTF variants have been described. To expand the NEDDFL phenotypic spectrum, we describe the clinical features in 25 novel individuals with 20 distinct, clinically relevant variants in BPTF, including four individuals with inherited changes in BPTF. In addition to the previously described features, individuals in this cohort exhibited mild brain abnormalities, seizures, scoliosis, and a variety of ophthalmologic complications. These results further support the broad and multi-faceted complications due to haploinsufficiency of BPTF.
This article provides insights into the modalities of business-model change and innovation. On the basis of an analysis of empirical data of small and medium enterprises, a transition from wine production centrism to its expanded use in hospitality and tourism is explored. Previous research on wine tourism and hospitality predominantly focuses on a destination perspective, neglecting the organizational winery perspective. The article deploys a mixed methods approach, combining netnography and a content analysis for data collection with grounded research and clustering for theory building. The sample size included 885 German wineries. Data stemmed from two distinct sources (websites and a secondary publication in form of a wine guide) and has been analyzed through a two-step clustering algorithm as well as a Principal Component Analysis (PCA). The two-step clustering algorithm resulted in nine different business models while the PCA analysis grouped the variables into the following two categories: basic winery business model (BM) and BM extension into hospitality and tourism, thereby validating the difference between the two constructs. The results point to the diverse nature of business model extensions of wineries in tourism and hospitality, depending on their organizational type and size. This study offers a classification of small and medium sized enterprise’s strategic business model expansion, and explores the expansion of the wine industry through wine hospitality and tourism services, starting with the winery organizational perspective, which has not been done before.
The purpose of this paper is to examine the impact that various types of business model extensions (hospitality and tourism, online sales platforms, and sustainability) have on the winery business. The research is based on company data and online observations of N = 886 German wineries and deploys a content analysis, netnography, and structural equation modeling (SEM) in order to test the hypothesis on business model extensions of wineries, which have been set forth in the previous literature. The findings indicate that business model extensions related to online sales platforms have a positive impact on winery business size. These results mean that developing online sales platforms enlarges the winery BM (business model) size and type (manager-run, state-owned, or cooperatives). The paper presents in detail the impact of winery BM extensions on winery BM model type and size, thereby contributing to the literature on business model innovation.
This study set out to uncover brand positioning configurations by presenting state-of-the-art brand management literature and applying a novel, mixed-methods approach to examine the under-researched wine industry transformation towards open innovation in branding. German winery brands were analyzed using a multimethod approach leaning on a novel netnographic methodology and multiple sources. The sample included 572 wineries from all 13 German wine regions with website text data and online review text data from each winery. The study identified nine prime words used to describe both brand identity as well as wine brand image. It revealed word–price clusters of brand identity and image. The results offer insights into communication and pricing opportunities for wine brand identity as well as image, thereby contributing to open brand innovation.
The article improves understanding on leveraging new technology for DT (digital transformation) of grape harvest in SME wineries. It provides evidence on technologies used and workplace types deployed in grape harvesting, as well as strategic paths in deploying new technology, thereby contributing to the literature on networked sensing and seizing capabilities in the wine industry 4.0. The research approach is explorative and qualitative drawing on 31 interviews with wine industry 4.0 experts and managers, mostly owners of SMEs: wineries, wine software and wine machinery enterprises. Resulting findings serve as a roadmap for digital transformation of grape harvest process in SME wineries explaining technologies and work roles necessary for DWT (digital workplace transformation), as well as strategic paths of deployment of novel grape harvest technology. Previous research on the wine industry 4.0 has focused on BMI, while this research expands the focus to include a wider concept of technology adoption strategy as well as DWT. The research identifies two types of factors impacting the strategic deployment of grape harvest technology: pull factors, also termed servitization factors, as well as push factors, termed also digital transformation factors.
The purpose of this study is to research the antecedents of the sustainable travel decision-making of European travelers and thereby identify important lessons for the transition towards sustainable travel and tourism. The study is based on data collected through a representative survey, conducted in five European countries, with a sample of n = 5024 respondents. The results of descriptive statistics, EFA (Exploratory Factor Analysis) and FA (Factor Analysis) are presented in order to explore sustainable travel decision-making through environmental (policy-related and personal) attitudes and travel mode decision priorities in the European context. Furthermore, the study provides new evidence regarding the under-researched phenomenon of the attitude–behavior gap by presenting a model for the sustainability-oriented decision-making of travelers, including attitudes and travel mode priorities as antecedents. The results confirm the existence of moral licensing in travel decision-making, thereby extending the relevance of this theory into travel and tourism, which has not been done before. The denial of environmental issues is also being researched as regards its interaction with positive environmental attitudes, environmental travel mode priorities and non-environmental travel priorities, thereby advancing our understanding of the interplay between these categories. The interplay between the four categories furthers our understanding of the perplexity of travelers in terms of sustainable travel decision-making.
The reported research examines the impact of product portfolio labeling strategies on brand reputation and equity. A netnographic approach allowed to observe winery portfolio labeling approaches and create a typology of winery labeling strategies. Expert evaluation served to assess the dependent variable brand equity by deploying a regression analysis. For the observed wine industry, being part of the food industry, creating consistent and recognizable brands has a direct relevance for reducing (sustainability-related) food information overload and thereby building sustainable brand equity. The results uncover the relative importance of each of the six identified labeling strategies as well as their impact on reputation and brand equity creation. The results point to the need to establish a consistent, strategically founded product communication. Such an approach, with a positive effect on reputation building can serve to build sustainable brand equity. “Stuck in the middle”-type strategies apparently diminish winery brand equity exploitation. The findings contribute to the knowledge on food labels in product communication strategies and their impact on organizational brand equity, thereby having high relevance for the implementation of environmental certification initiatives in an organizational context. The article deploys a novel research approach in an under-researched area to provide new insights for further research as well as implications for practice.
The article explores SME (Small and Medium Sized Enterprises) brand strategies as a means to position and successfully engage in competitive markets. A derived typology of brand strategy types deals with social profiling and sheds light on brand strategy internalization of two current managerial paradigms—sustainability and co-creation. N = 895 German SME wineries were examined, leaning on a netnographic analysis of predominantly websites and social media interactions. A two-step clustering method thereby identified eight winery SME brand strategy types. The importance of sustainability across the identified eight brand strategy types is significant. Co-creation turned out to be a key profiling trait characterizing one brand strategy type. The typology illustrates strategic richness, with brand strategies leaning predominantly on traditional values, on sustainability, on external reputation, or on more innovative customer centric concepts such as co-creation. Hereby, the typology and the identified brand levers invite to strategically design brand management, governance, and sustainability. Wineries which focus on traditional positioning and legitimacy were found to be cautious in deploying co-creation through social media. Winery brands that are characterized by engagement in digital co-creation apparently either tend to expand their scope or partially combine it with traditional values, making them the most diverse type identified. Sustainability obviously needs to be addressed by all brand strategies. Despite industry and country focus, the analyses illustrate the relevance of socially-oriented profiling and highlights that sustainability has reached a status of a fundamental business approach still allowing to differentiate thereon. Furthermore, the business models of the SMEs need to deliver communicated values.
Most people use disaster apps infrequently, primarily only in situations of turmoil, when they are physically or emotionally vulnerable. Personal data may be necessary to help them, data protections may be waived. In some circumstances, free movement and liberties may be curtailed for public protection, as was seen in the current COVID pandemic. Consuming and producing disaster data can deepen problems arising at the confluence of surveillance and disaster capitalism, where data has become a tool for solutionist instrumentarian power (Zuboff 2019, Klein 2008) and part of a destructive mode of one world worlding (Law 2015, Escobar 2020). The special use of disaster apps prompts us to ask what role consumer protection could play in safeguarding democratic liberties. Within this work, a set of current approaches are briefly reviewed and two case studies are presented of what we call appropriation or design against datafication. These combine document analysis and literature research with several months of online and field ethnographic observation. The first case study examines disaster app use in response to the 2010 Haiti earthquake, the second explores COVID Contact Tracing in Taiwan in 2020/21. Against this backdrop we ask, ‘how could and how should consumer protection respond to problems of surveillance disaster capitalism?’ Drawing on our work with the is IT ethical? Exchange, a co-designed community platform and knowledge exchange for disaster information sharing, and a Societal Readiness Assessment Framework that we are developing alongside it, we explore how co-design methodologies could help define answers.
This paper gives an overview of how we can benefit from using container technology in our academic work. It aims to be a starting point for fellow researchers which also think about applying these technologies. Hence, we focus on decribing our own experiences and motivations instead of proving hard scientific facts.
Experience made with free and open source software (FOSS) in the public research is shared with the community. The motivation for using and publishing FOSS is to increase visibility, transparancy and feedback quality while at the same time lowering software licensing costs. Also, the idea of giving back and returning a value plays a role. The most frequently given counter arguments are discussed. In the end, it’s important to embed FOSS publishing into the company’s strategy for the exploitation of scientific research results. To help with this, a checklist of criteria to indicate FOSS publishing is suggested. On the backround of wireless sensor networks, some case studies of FOSS contribution are detailed. The emphasis is on checking the original motivation and the spirit of FOSS back with the reality. Finally, further potential of publishing FOSS in the context of scientific research is identified.
Cancer is one of the leading causes of death worldwide [183], with lung tumors being the most frequent cause of cancer deaths in men as well as one of the most common cancers diagnosed in woman [40]. As symptoms often arise in advanced stages, an early diagnosis is especially important to ensure the best and earliest possible treatment. In order to achieve this, Computed Tomography (CT) scans are frequently used for tumor detection and diagnosis. We will present examples of publicly available CT image data of lung cancer patients and discuss possible methods to realize an automatic system for automated cancer diagnosis. We will also look at the recent SPIE-AAPM Lung CT Challenge [10] data set in detail and describe possible methods and challenges for image segmentation and classification based on this data set.
One of the biggest challenges faced by many tech start-ups from developed markets is to have validated market-fit products/services and to see their solutions implemented. In several sectors, stringent regulations, and the law of handicap of head start at home can be hurdles that limit the development and even the survival potential of theses start-ups. Tech start-ups seeking implementation, learning, and legitimacy may have a solution in expanding into emerging markets. Emerging markets offer both business opportunities in sectors in need of new technologies as they are “fertile grounds” for developing and testing internationalisation business models. We present here a process designed to help tech start-ups to identify, access, shape and seize these opportunities and to overcome their own specificities and emerging markets specificities. The three phases of the proposed process cover entry node concept, partnership, and business, operating and revenue joint models’ development. DesignScience Research Paradigm is used for the design and evaluation of the process. To show the relevance of this process, a case study on the expansion in Morocco of a Dutch start-up active in e-health is used. The study shows the importance of the process for the embeddedness in a local relevant value network with a relevant adopter’s system, a key enabler to achieve time and cost-effective expansion in that specific business and institutional contexts. A pilot to assess the proposed models and evidence of benefits is under development. To boost their chances of growth tech start-ups from developed markets should consider expansion into emerging markets in their strategy. It would be beneficial that policy makers adopt a strategy by which to assist tech start-ups in accessing value networks in emerging markets. It is also important for policy makers from emerging markets to consider developing schemes to attract tech start-ups from developed markets.
Research on entrepreneurial eco-systems is evolving with exhortations for empirical studies at regional and local levels to augment national surveys. The study, therefore, sought to explore the entrepreneurial eco-system of the Central Region, which is relatively well-endowed with natural resources but lags behind in economic advancement in Ghana. Through descriptive research design, quantitative data were collected using self-administered questionnaires from a convenience sample of 44 entrepreneurs under the presidential business support programme in the Central Region of Ghana, in 2019. Data were analysed, by conducting descriptive analysis such as means (M) and percentages and by exploratory factor analysis, with the IBM SPSS Version 25. Descriptive results of 37 valid responses showed that the respondents were satisfied, in varying degrees (M = 4.19-5.65), with 11 factors within the eco-system; the top three factors were demand, security and availability of raw materials. Respondents were, however, not satisfied with access to business development services, access to finance, rent charges and access to repairers of equipment and thus, pose as challenges to their entrepreneurial pursuits. Principal component analysis revealed inter-connectedness among the factors in the eco-system with strong loadings of measures of institutions and resource endowment under the two components of the solution. Based on the findings, it is concluded that the entrepreneurs surveyed were satisfied with more factors in the EES of the Central Region while they were dissatisfied with relatively few but critical factors in the EES, thereby posing as major challenges to their entrepreneurial activities. As an exploratory study, the findings suggest that the entrepreneurial eco-system of the Central Region of Ghana is, to some extent, supportive of entrepreneurial activities but has key challenges. To achieve maximum outcomes, policy interventions should collectively address, at a time, factors that interact strongly to influence entrepreneurship within the system.
Personal values and electronic waste disposal behaviours among households in Cape Coast Metropolis
(2021)
The study examined social values that accounted for electronic waste recycling and reuse behaviours. Via a crosscommunity survey of 193 of households in the Cape Coast Metropolis, a correlational design was employed in the study. Partial Least Squares-Structural equation modelling was used to analyse the data. Results from the analysis showed the influence of altruistic values (β = 0.275, p < 0.05) on reuse behaviour. Similarly, environmental awareness (β = 0.213, p<0.05) also showed significant influence on participation in recycling, whereas psychological ownership significantly influenced both reuse (β = 0.319, p < 0.05), and participation in recycling (β = 0.339, p < 0.05), The joint significance of altruistic values, environmental awareness and psychological ownership to explaining recycling participation was 21.3% (R2 = 0.213, p < 0.05) and that of reuse was 24.6% (R2 = 0.246, p < 0.05). The results of the study showed that individuals who are knowledgeable about the state of their environment were more likely to participate in recycling. On the other hand, individuals with altruistic values preferred giving unwanted electronic equipment to others for reuse. Altruistic values are particularly true of collectivist cultural orientation. Psychological ownership was significant in predicting both behaviours, however, the effect size on reuse was moderate. Psychological ownership due to waste aversion and frugality lead consumers to keep, and subsequently give to close relatives in their social network. It was recommended that individuals should be encouraged to patronize formal recycling services. as a way to show concern for the well-being of others by reducing pollution due to improper waste treatment. Again, like in developed economies, second-hand collection systems for unwanted electronic products can be developed, and made convenient for individuals with reusable items, who may be willing to donate or even resell.
The role of tourism entrepreneurship in rural development continues to be a subject of interest and debate among academia and practitioners. Theoretically, it is anticipated that tourism entrepreneurship will lead to livelihood diversification, enhancement and ultimately a revitalization of the rural economy. While tourism is posited as an accessible entrepreneurship pathway, there is a dearth of information regarding rural dwellers’ actual experiences with it, especially within the Ghanaian context. Using a case study approach and qualitative data from Wli; a rural tourism destination in Ghana, this paper delves into the opportunities and concerns associated with tourism entrepreneurship in rural areas. Data was obtained between November and December 2016 from 27 persons who were either tourism enterprise owners or employees. Findings from the study showed that entrepreneurial activities centred on the provision of accommodation, food and beverage, souvenir and guiding services. The nature of the activities enabled easy transfer of existing skills and knowledge. Further, entry into tourism entrepreneurship was perceived to be easy by the majority of study participants. These findings confirm the potential for tourism to be employed in boosting entrepreneurial activities in rural areas. Nevertheless, there were concerns regarding access to credit, institutional support, unhealthy competitions, low incomes, unguaranteed pensions, and seasonality and skewness of demand. These concerns threatened the growth and sustainability of tourism entrepreneurship within the community. From a policy perspective, there is a need for institutional recognition and support for tourism entrepreneurial intentions and activities in rural areas. Practice-wise, credit facilities need to be designed specifically for tourism-related rural enterprises. Further, periodic skills and knowledge augmentation programmes must be initiated to help expand the skill sets for the rural entrepreneurs. Finally, there is a need for the formation of traderelated networks to provide a platform for knowledge and experience sharing among the entrepreneurs.