Longitudinal case study and phenotypic multimodal characterization of McArdle disease-linked retinopathy: insight into pathomechanisms

2020 ◽  
Vol 41 (1) ◽  
pp. 73-78
Author(s):  
Veronika Vaclavik ◽  
Francine Naderi ◽  
André Schaller ◽  
Pascal Escher
2009 ◽  
Vol 81 ◽  
pp. 121-132
Author(s):  
Marianne Spoelman ◽  
Marjolijn Verspoor

Within Dynamic Systems Theory (DST), it is assumed that differences in the degree of variability can provide insight into the process of L2 development. This longitudinal case study investigates intra-individual variability in Finnish learner language, focusing on the development of accuracy and complexity. The study involves 54 writing samples, written by a Dutch student who learned Finnish as a foreign language. Finnish, a synthetic language of the agglutinating type, is very different from Indo-European languages and well known for its complex morphology. This complex morphology was investigated for accuracy in form and use. Word-, Noun Phrase-, clause- and sentence constructions were examined for complexity. The purpose of the study was to explain the fluctuations of intra-individual variability and complex relations between variables and to detect both supportive and competitive relationships between growers in order to provide valuable insights into the dynamic processes involved in L2 development.


Popular Music ◽  
2014 ◽  
Vol 34 (1) ◽  
pp. 67-93 ◽  
Author(s):  
Mark Slater

AbstractMiddlewood Sessions produced a kind of popular music that infuses the timbral aesthetics of jazz and orchestral music with the driving rhythms of dance music. This studio project, lasting for almost eight years, provided a rich resource for gaining insight into the increasingly prevalent context of the domestic project studio via a longitudinal case study approach. At the heart of this research is the desire to understand how people collaborate as part of a studio project, how people use technologies to make music and how all of this unfolds over time. To tackle the question of how to understand the shattered, scattered nature of creative practices, and in extending existing creativity research, I propose three ways of thinking about time: nests, arcs and cycles. While explicating this theoretical framework, something of the specific and idiographic nature of the case study, as an example of contemporary music production, is recounted.


2017 ◽  
Vol 4 (1) ◽  
pp. 218-237
Author(s):  
Sina-Mareen Köhler

This contribution presents results of a longitudinal qualitative study of young adults with different career plans and transition pathways. The central question of this study focuses on the relevance of vocational orientation programs at regular schools for young people’s career plans and transitions. The first part deals with the organization and research about vocational orientation programs. The second part begins by giving an insight into the empirical design of the longitudinal study. It then proceeds to discuss how the methodological perspective of reconstructive research can provide deeper understanding of student’s perspective. Narrative interviews are used as the basis to investigate how the socialization contexts are relevant and interconnected. Through the deeper understanding of student’s perspectives and the role of different socialization agents, it is possible to highlight the relevance of vocational orientation programs at schools. The findings could prove useful for improving vocational orientation programs at schools. Currently, such programs are disconnected from students’ everyday life and show little regard for their perspectives.


2016 ◽  
Vol 141 (1) ◽  
pp. 167-202
Author(s):  
Mark Slater

ABSTRACTVia a longitudinal case study of a studio project (Middlewood Sessions, 2004–12), this research explores processes of music-making in the increasingly prevalent context of the project studio to give an insight into contemporary music-making practices. Predicated upon technologies of decreasing size but increasing processing power, project studios represent a diversification of musical creativity in terms of the persons and locations of music production. Increasingly mobile technologies lead to increasingly mobile practices of music production, which presents a challenge to the seemingly simple question: where is the project studio? In response, I propose an ontology of project-studio music-making that sets out what conditions have to be met for location, as an active proposition, to take place.


1996 ◽  
Vol 27 (2) ◽  
pp. 194-214 ◽  
Author(s):  
Carolyn A. Maher ◽  
Amy M. Martino

This longitudinal case study presents a sequence of episodes that document the mathematical thinking of one child, Stephanie, over a 5-year period. Her development of the idea of mathematical justification spans grades 1–5. Stephanie worked on several combinatorics tasks in small-group, whole-class, and individual interview settings. The documented events indicate Stephanie's progress in classifying, organizing, and reorganizing data. The study provides some significant insight into the process by which Stephanie learned to make proofs, within a setting that encouraged the development of her ideas.


2015 ◽  
Vol 39 (4) ◽  
pp. 462 ◽  
Author(s):  
Michelle Gray ◽  
Jennifer Rowe ◽  
Margaret Barnes

Objective The aim of the present study was to investigate how midwives are responding to the changed re-registration requirements; specifically the Recency of Practice (RoP) Standard. Methods A qualitative longitudinal case study used conversational interviews conducted annually at two time phases after the introduction of national registration. Results Findings reveal that confusion has created challenges in demonstration of the RoP standard. This confusion was evident at individual and organisational levels. Conclusions Professional bodies need to support staff in this transition by providing clearer guidance that exemplifies the Nursing and Midwifery Board of Australia expectations. What is known about the topic? Impact subsequent to Australian legislative and regulatory changes affecting midwifery and nursing registration has not been examined. What does this paper add? The findings of this study provide an insight into midwives’ responses to the changed re-registration standard in Australia. What are the implications for practitioners? There appears to be a problem in the way tensions and challenges are being met; misinterpretation of the requirements has generated questions about the relationship between skills and work areas and demonstration of RoP. This may influence individual career planning and have broader workforce planning implications.


2021 ◽  
Vol 11 (7) ◽  
pp. 3231
Author(s):  
Mehrzad Lavassani ◽  
Johan Åkerberg ◽  
Mats Björkman

The network infrastructures in the future industrial networks need to accommodate, manage and guarantee performance to meet the converged Internet technology (IT) and operational technology (OT) traffics requirements. The pace of IT–OT networks development has been slow despite their considered benefits in optimizing the performance and enhancing information flows. The hindering factors vary from general challenges in performance management of the diverse traffic for green-field configuration to lack of outlines for evolving from brown-fields to the converged network. Focusing on the brown-field, this study provides additional insight into a brown-field characteristic to set a baseline that enables the subsequent step development towards the future’s expected converged networks. The case study highlights differences between real-world network behavior and the common assumptions for analyzing the network traffic covered in the literature. Considering the unsatisfactory performance of the existing methods for characterization of brown-field traffic, a performance and dynamics mixture measurement is proposed. The proposed method takes both IT and OT traffic into consideration and reduces the complexity, and consequently improves the flexibility, of performance and configuration management of the brown-field.


Author(s):  
Vera de Paula ◽  
Maiara Rosa ◽  
Henrique Rozenfeld

The mass properties of aircraft directly influence their performance and costs, and are particularly subject to high uncertainties in the early phases of the development process. As aircraft systems become more detailed, their mass properties are iteratively updated. Those updates, in turn, may lead to rework on aircraft systems. To avoid excessive iterations, aircraft manufacturers employ the mass properties management (MPM) process during their development processes. However, even when this approach is adopted, the continuous cycle of increasing weight and redesigning aircraft structures represents a significant challenge, which may lead to the cancellation of programs. One of the causes of this problem is inefficient integration between MPM and the aircraft development process. We propose a concept to significantly enhance the integration between MPM and aircraft development processes, suggesting feasible practices to support its implementation. The research methodology combines a review of the literature, an exploratory case study, a three-year longitudinal case study, and verification by experts. The results describe a concept for integrating aircraft MPM and development, supported by 16 practices. They also include a characterization of the MPM process based on literature and practices, which lists 17 characteristics divided into four categories: Goals/Strategy, Activities/Information, Resources/Tools, and Organization/Roles and Responsibilities.


Agronomy ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1538
Author(s):  
Valentina Fanelli ◽  
Vincenzo Roseti ◽  
Michele Antonio Savoia ◽  
Monica Marilena Miazzi ◽  
Pasquale Venerito ◽  
...  

Calabria is a region located in Southern Italy and it is characterized by a long tradition of viticulture practices and favorable pedoclimatic conditions for grapevine cultivation. Nevertheless, less than 2% of cultivated land is dedicated to grapevine growing in Calabria. The characterization of local grapevine accessions is crucial to valorize the local and peculiar Italian products and boost the Calabrian winemaking sector. With this purpose, we performed a deep characterization of two widespread Calabrian grapevine varieties—Magliocco Dolce and Brettio Nero, of which very little is known. In particular, a genetic and morphological analysis, a berry physico-chemical and polyphenolic compositions assessment, and oenological evaluation of monovarietal wines were carried out. Our results allowed us to demonstrate that Magliocco Dolce and Brettio Nero are unique and distinct varieties with peculiar morphological and chemical characteristics and show the suitability of these two varieties in high-quality wine production. Moreover, the obtained molecular profiles will be useful for authentication and traceability purposes.


2021 ◽  
pp. 017084062110532
Author(s):  
Jonathan Staggs ◽  
April Wright ◽  
Lee Jarvis

We shed new light on the processes through which institutions are created and changed by investigating the question how does institutional entrepreneuring unfold in an already organized world. We conducted a longitudinal case study of the field of scientific research production in Australia, which changed over three decades through entrepreneuring processes associated with the creation of a new ‘Smart State’ place in the city of Brisbane in Queensland. A new place is a form of organizing human activity that has materiality and meaning at a specific geographic location. Our findings showed how field change was interwoven with place creation through four processes of entrepreneuring: structural emancipation, dissociating and reimagining place meanings, bricolaging of place forms, and co-evolving place identities. These entrepreneuring processes constituted the field as a flow of ‘becoming’ that spilled over into temporary and provisional settlements in local places. Our findings make important contributions through: (1) deepening understanding of how organizational fields change through multilevel, distributed, cascading and often unreflexive processes of entrepreneuring processes in an already organized world, (2) bringing attention to a relationship between institutions and place, in which place is both the medium and outcome of institutional entrepreneuring, and (3) providing new insight into embedded agency by illustrating how institutions in ‘becoming’ continually (re)produce the resources and possibilities for agency within gradual institutional change over time.


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