Cardio-facial-cutaneous syndrome is a rare genetic disorder that typically presents with a combination of CHDs, distinctive facial features, and cutaneous abnormalities. Cardio-facial-cutaneous syndrome is usually caused by a genetic change in the BRAF gene but can also be due to genetic change in the MAP2K1, MAP2K2, or KRAS genes. It is an autosomal dominant condition, but most cases are not inherited, due to new genetic change that occurs in the formation of the egg or sperm or shortly after fertilisation. It has been seen previously in the literature where the patient required a mitral valve replacement because of insufficiency. In this case report, we describe a patient with cardio-facial-cutaneous syndrome who also presented with mitral valve prolapse. This rare association expands the spectrum of cardiovascular manifestations in cardio-facial-cutaneous syndrome and highlights the importance of comprehensive cardiovascular evaluation in these patients.

Behavioural activation (BA) is recommended for the treatment of depression but most research focuses on working age adults and there is a dearth of literature concerning the delivery of BA with people with co-occurring depression and mild cognitive impairment (MCI). This case study outlines a BA intervention with a male in his late 60s with depression and MCI and describes appropriate adaptations that were useful. Treatment consisted of psychoeducation of depression and BA, formulation, activity monitoring and scheduling, tackling self-critical thoughts and rumination, and relapse planning. The 12-session BA treatment resulted in a decrease in both depressive symptoms and psychological distress as well as an increase in the individual’s engagement with meaningful activities. This case study adds to the literature and strengthens the argument for the use of BA in the treatment of depression in older adults with MCI. Adaptations, conclusions and limitations are discussed.

Key learning aims

1. (1) To gain an understanding of the use of behavioural activation (BA) in the treatment of depression in older adults with mild cognitive impairment (MCI).

2. (2) To illustrate treatment of depression using BA with an older adult utilising the current evidence base.

3. (3) To outline adaptations that can be made to BA to help deliver this treatment with an older adult who has MCI.

A structurally transforming multi-mode product can realize a changing set of functions across its modes, replacing multiple related products while offering increased cost, space, and time efficiency. However, there is a lack of connected methods that address the additional design complexities due to the product’s physical transformations and the resulting structural component-sharing between modes. A framework, grounded in standard design practice and built upon existing methods, is proposed to help navigate the two most impacted design stages: 1. Problem Definition and 2. Conceptual Design. The Problem Definition stage in this new framework involves identifying the external factor that determines the product’s modes and defining the functional requirements for the modes and transformation methods. The Conceptual Design stage involves iteratively linking conceptualized forms of each mode to adjacent modes through conceptualized transformation methods. The framework is demonstrated in a case study involving the design of a structurally transforming multi-mode piece of children’s furniture that transforms between a cradle, floor seat and a multipurpose toddler step stool. The proposed framework is a promising step toward systematically, cohesively, and comprehensively addressing design challenges during the development of a wide variety of structurally transforming multi-mode products, therefore facilitating better, more effective product design.

Despite the phenomenon of hybrid emerging market state-owned enterprises (EM-SOEs) in international markets, the complexity inherent in their ownership structure, their paradoxes in institutional and market economies, and concerns about their proximity to the state make it challenging for EM-SOEs to fulfill sustainable development mandates as part of their internationalization strategies. In this study, we propose a biomimicry perspective to investigate how hybrid EM-SOEs with plural institutional demands manage their stakeholder relationships and undertake their corporate sustainability practices in the host market. Shifting from the firm-centric perspective, the biomimicry perspective offers a systems' explanation to spur new insights based on a single in-depth case study in the power generation industry in Indonesia. The findings highlight the importance of a long-term, value-driven strategic approach, emphasizing the cultivation of symbiotic relationships for delivering innovative products and processes to provide renewable energy supply in local markets. Additionally, legitimation practices are observed across three key dimensions embedded in the internal and external systems – governments, local elites, and local grassroots citizens, involving idiosyncratic local stakeholders whose impacts are rather mixed for EM-SOEs' international operations in Indonesia.

Most existing literature treats family culture as a static and deterministic factor with a double-edged effect on the competitive advantage (CA) of family firms while overlooking its dynamic nature. Moreover, limited literature addresses how to solve this double-edged problem. This study fills these gaps by examining how family culture can generate sustainable CA from the lens of the affordance perspective. A three-stage process model is developed based on a longitudinal case study of Baiyun, a Chinese family firm with over a 100-year family history. This model suggests that family firms should intentionally adopt appropriate sensemaking and sensegiving strategies tailored to different stakeholders and dynamic entrepreneurship situations to effectively leverage the natural and designed affordances of family culture. By cultivating efficiency, emotional, and value identifications among internal and external stakeholders, these sensegiving strategies facilitate family collaboration, continuous trust, strategic focus, and extensive ecological synergy, which serve as the key sources of CA. Ultimately, the model emphasizes that the key to managing the double-edged effect lies in the four sensemaking strategies taken by family firm leaders. This study sheds light on the dynamic interplay between family culture, environment, strategies, and CA, offering actionable insights for family firms.

The two main large-scale features of Arctic sea-ice drift are the Beaufort Gyre and the Transpolar Drift Stream. They exhibit strong intraseasonal and interannual variability. Winter 2016/17 showed increased cyclone activity, leading to the collapse of the Beaufort Sea high and the reversal of the Beaufort Gyre. Winter 2020/21 displayed decreased cyclone activity and intense anticyclonic ice transport in the Beaufort Gyre. Here we show that the European Centre for Medium-Range Weather Forecasts’s (ECMWF) extended-range (46 days) retrospective forecasts were able to predict the ice motion during these cases. The initial contrasts in sea level pressure, surface winds and ice drift were well captured, and their temporal evolution—including the reversal of the usual drift direction—well reproduced by the forecasts initialized about a week before the event. Sea-ice thickness in the forecast exhibited initial errors even greater than 1 m that persisted throughout the forecast and negatively affected the ice speed forecast. Despite these shortcomings, the dynamic forecast outperformed the persistence and climatology forecast and represented the observed relation between surface winds and ice drift well. The benefit of dynamic forecasts is especially clear in cases that differ from climatology, like the one we focus on.

The world is currently undergoing a technological transformation with numerous innovative concepts emerging. This shift is driven by remarkable advancements in artificial intelligence and the urgent need for decarbonisation. With this comes a growing demand for skilled engineers who can actively contribute at any stage within the life cycle of a product. This can be the generation of new concepts at low Technology Readiness Levels or contributing actively to their development and operational safety. This paper explores the integration of a 1-day practical activity to reinforce theoretical concepts learned within a classroom-based environment. Small groups of students were given the opportunity of engaging with a small helicopter engine (Rolls-Royce Gnome engine) through the disassembly and reassembly of the exhaust and power turbine section while following the manufacturer’s manual and ensuring industrial norms for safe practice. This hands-on activity included an introduction to tooling, a Gnome familiarisation activity, and an introduction to inspection techniques. Based on the feedback recorded, the students experienced a notable improvement in their basic understanding by effectively reinforcing knowledge acquired within the classroom through active engagement with an actual gas turbine engine.

This study explores the alignment between automotive design innovation and consumer acceptance, particularly in the context of two significant trends: electrification and digitalization. We probed the acceptance of replacing rear window with wide-angle camera We surveyed 1,546 potential customers from China and Sweden to assess their openness to such a radical design. Findings suggest a consensus on its futuristic appeal but diverge in adoption willingness. The study offers insights to bridge the design preference gap, positioning customer acceptance as key for car design strategy.

In the field of individualized medical implants for bone replacesment, additive manufacturing offers far-reaching advantages for bridging bone defects and supporting the production of natural form and function. The article uses the example of a large, customized cranial implant to show the challenges of manufacturing with osteoinductive bone cements. The process is shown, starting with planning and design, through to functional integration using adapted manufacturing strategies to create defined porosity.

This study explores Machine Learning (ML) integration for household refrigerator efficiency. The ML approach allows to optimize defrost cycles, offering energy savings without complexity or cost escalation. The paper initially presents a State-of-the-Art of ML potential to improve functionality and efficiency of refrigerators. Since frost is the cause of significant energy losses, a ML-based Virtual Sensor was developed to predict frost formation on the evaporator also in low -level refrigerators. The results show the environmental significance of ML in enhancing appliance efficiency.

This paper explores the implementation of Industry 4.0 technologies in Small Enterprises (SEs), focusing on the unique challenges they face. It presents four case studies demonstrating how low-cost, low-effort solutions can enhance efficiency and productivity in small companies. The solutions, developed in a local makerspace, address specific manufacturing challenges that lack affordable or existing off-the-shelf solutions. The paper highlights the value of iterative prototyping in implementing Industry 4.0 solutions and discusses how this approach can help SEs overcome adaptation challenges.

Various industries use computer simulation for verifying product properties in early phases of development. Traditionally, such properties include the stability of mechanical structures or the efficiency of aircraft turbines. More recently, research also focuses on the mountability of industrial products using virtual assembly. While research on virtual assembly already started in the mid-1990s, the applicability in different industries remains largely unclear today. To advance the state-of-the-art, in this paper we present learnings from developing a virtual assembly environment for cooling cabinets.

Supporting product developers in innovating is an important task of design research. An invention and a valid need situation described through a product profile are necessary elements of innovation. But how can we derive recommendations on how to develop “innovative” products if the success of a product can only be measured retrospectively? Retrospective case studies are one approach to investigate relationships between context factors, variations in systems and product profiles, and innovation success. To investigate these, we use product profiles in case studies across system generations.