bluuebunny/crossref_metadata_2025_split huggingface dataset:
Real-world clinical and psychosocial outcomes among people with mild or moderate haemophilia A treated on-demand in the Italian CHESS II cohort: a real-world data analysis
Authors: Castaman Giancarlo , Mancuso Maria Elisa , Di Minno Matteo Nicola Dario , Sannino Luigi , Tempre Rosaria , Bendinelli Sara , Blenkiron Tom , Burke Tom , Grazzi Enrico Ferri 
Published: May 2024

Abstract

Background
The burden of severe haemophilia A (HA) has been studied extensively owing to the higher bleeding frequency and associated treatment requirements, leaving a clear unmet need for research focused on the burden of mild and moderate HA.

Aims
This study sought to characterise the clinical and psychosocial burden of mild and moderate HA in the Italian cohort of the CHESS II study.

Methods
This was a retrospective analysis of clinical and psychosocial outcomes in a cohort of male adults (≥18 years old) with mild or moderate HA who participated in the cross-sectional CHESS II study (October 2019-November 2020). Treatment patterns, acute and chronic clinical outcomes and mental health indicators were collected via physician-completed forms. Psychosocial outcomes related to impact of HA on social activities, exercise, opportunities, and lifestyle were collected via a participant self-complete questionnaire. All results were reported descriptively.

Results
A total of 113 people with haemophilia A (PwHA) were included, 79 (70%) with moderate HA and 34 (30%) with mild HA, with mean age of 41.4 and 36.6 years, respectively. No one in the sample was receiving a prophylaxis at the time of data capture, with factor VIII use in the 12 months prior reported in 30% and 29% of moderate and mild PwHA, respectively. Ninety-one PwHA (81%) experienced ≥1 bleeding event in the preceding 12 months. People with moderate HA had higher mean annual bleed rate (2.9 vs. 1.1, respectively) and higher prevalence of chronic pain (74% vs. 35%), anxiety (20% vs. 12%), and/or depression (15% vs. 3%). Target joints were reported in 22% and 12% of moderate and mild PwHA, and problem joints in 51% and 12%, respectively. Of 113 participants, 44 (39%) completed the self-complete form (moderate HA, 57%; mild HA, 43%). Overall, 40% vs. 10% of those with moderate vs mild HA reported reducing or giving up social activities, 44% vs. 21% reducing or giving up exercise, 36% vs. 26% missing out on opportunities, and 48% vs. 26% reported HA impacted their lifestyle.

Conclusion
Moderate PwHA from the Italian CHESS II cohort appeared to have greater clinical morbidity and lifestyle impact than mild PwHA. Psychosocial outcomes were also worse among moderate PwHA, but significant burden was also observed among mild PwHA. These findings, and the absence of prophylactic treatment in the sample examined, highlight that improving management for potentially undertreated mild/moderate PwHA may aid the avoidance long-term clinical morbidity and negative psychosocial impact.
Living, Caring, Learning – Listening to patients with bleeding disorders and not being afraid to advocate
Authors: Witkop Michelle 
Published: May 2024

Abstract
Michelle brought experience of working with people with chronic pain to her role as a bleeding disorders nurse, before going on to develop a research department at the National Hemophilia Foundation (now the National Bleeding Disorders Foundation). Reflecting on the pain experienced by various individuals she cared for, she highlights the importance of listening carefully to what patients have to say and the value of patient-reported information. Michelle explains how understanding this led her into patient advocacy, and how nurses acting as advocates for their patients supports their care and thereby enable people with bleeding disorders to live the best life they can.
Living, Caring, Learning – Collaborating across borders to support a haemophilia patient with a rare complication
Authors: Githinji Cyrus 
Published: May 2024

Abstract
Cyrus, a haemophilia nurse in Kenya, reflects on his experience of treating a patient with a rare complication of haemophilia in a setting with limited access to treatment. The patient developed a debilitating abdominal pseudotumor following an inadequately treated traumatic bleed. After consulting with experts in other countries, surgery was deemed too great a risk. Initiating FVIII prophylaxis with the aim of shrinking the pseudotumour was identified as the most appropriate approach to treatment, however supply issues made it difficult to maintain a prophylactic regimen. When the pseudotumour continued to grow, posing a risk to adjacent organs, an international partnership enabled the initiation of emicizumab prophylaxis through a clinical trial. It was hoped that emicizumab would prevent further bleeding into the pseudotumour and allow it to shrink over time. The patient continued to experience breakthrough bleeding and was withdrawn from the trial after 2.5 years. By this time the supply of FVIII to Kenya through the through the WFH Humanitarian Aid Program had increased and the patient was able to resume FVIII prophylaxis. The pseudotumour is now stable and he continues to be cared for by the same nursing team. Reflecting on this case, Cyrus highlights the value of sharing expertise across borders and working in partnership to try to ensure the best treatment outcome for a patient with a rare complication in a resource-constrained setting. He also reflects on being compassionate in his practice and the importance of a holistic approach to patient support.
Living, Caring, Learning – Setting boundaries for the wellbeing of everyone in bleeding disorders care
Authors: Harrison Cathy 
Published: May 2024

Abstract
Cathy, a haemophilia nurse specialist in the UK, reflects on a challenging patient with a rare inherited platelet disorder. The patient did not attend regular clinic appointments, and when presenting at clinic due to severe bleeding or feeling unwell was often aggressive and abusive towards staff. The care team were concerned that he was putting his health at risk and followed protocols for vulnerable adults to try and ensure he was accessing the care he needed. This involved bringing in expertise outside of the multidisciplinary team (MDT), including non-medical services. Steps were also taken to ensure staff safety and wellbeing through in-house psychological support and the agreement of strategies for dealing with difficult behaviour. Ultimately, it was necessary to find an alternative care pathway for the patient. While acknowledging her disappointment in this outcome, Cathy reflects on how the processes followed have reinforced the development of individualised care plans for all patients with complex needs, and the importance of access to specialist services beyond the MDT. She also highlights considerations around the safety and wellbeing of the care team, the role of discussion and ‘debriefing’, and the value of staff access to psychological support.
Insufficiency of quality of life as the treatment endpoint for balloon pulmonary angioplasty in inoperable chronic thromboembolic pulmonary hypertension
Authors: Gong Juanni , Ding Yuan , Wang Jianfeng , Wang Wei , Huang Qiang , Miao Ran , Kuang Tuguang , Yang Suqiao , Li Jifeng , Jiao Xiaojing , Yang Yuanhua 
Published: May 2024

Abstract

Background and Objectives
The ability of a quality of life (QoL) to guide balloon pulmonary angioplasty (BPA) among patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) has not been fully investigated. This study explored the relationship between QoL scores and hemodynamics in CTEPH patients after BPA and examined whether QoL could be applied as a treatment endpoint.

Materials and Methods
This cohort study included patients with inoperable CTEPH who had undergone at least four sessions of BPA. The patients’ demographic and clinical data as well as hemodynamic parameters and scores from the RAND 36-item short-form QoL questionnaire were recorded and compared before and after BPA.

Results
After BPA treatments, clinical characteristics, hemodynamic parameters, as well as QoL score improved significantly. A physical component summary (PCS) score of 35 or 46 can be used as the cutoff value for predicting better World Health Organization functional classification (WHO FC). Patients who had a higher PCS would have longer 6-min walk distance (6MWD), lower pulmonary vascular resistance (PVR), and better cardiac output (CO) both before and after BPA. However, 19 patients (55.9%) with a higher PCS score after BPA did not achieve the goal of mean pulmonary arterial pressure (mPAP) ≤30 mmHg. During the follow-up period, a significant reduction of PVR was observed, but the PCS score improved a little.

Conclusions
QoL is a useful tool for assessing the exercise endurance of patients with inoperable CTEPH treated with BPA, but is insufficient to serve as a treatment endpoint for BPA.
Development and status quo of digestive endoscopy in China: An analysis based on the national census in 2013 and 2020
Authors: Jiao Yunfei , Cheng Zhiyuan , Gao Ye , Wang Tianjiao , Xin Lei , Lin Han , Cai Mengxi , Ma Xudong , Li Zhaoshen , Wang Luowei 
Published: May 2024

Abstract

Background and Objectives
Technique and practice of digestive endoscopy are undergoing speedy development all over the world. This study aimed to evaluate its status quo and development in China.

Methods
All hospitals performing digestive endoscopy in mainland China participated in the national census in 2013 and 2020. Retrospective data of hospitals, endoscopists, volumes, and qualities were collected via an online structured questionnaire, and its accuracy and rationality were verified by logical tests and manual reviews. Data from other countries were used to compare with that of China.

Results
From 2012 to 2019, the number of hospitals performing digestive endoscopy increased from 6,128 to 7,470 (1.22-fold), in which primary healthcare played a minor role. The median hospitals per 100,000 inhabitants per provincial region increased from 0.49 (IQR, 0.39-0.57) to 0.55 (IQR, 0.49-0.63). The endoscopists increased from 26,203 to 39,638 (1.51-fold), but their average workload even expanded. Overall volume increased from 28.8 million to 44.5 million (1.55-fold), and most types of endoscopic procedures recorded a high growth rate. Contrastingly, the specific utilization rates were low and paled in comparison with some developed countries. Nationwide, regional utilization rates showed a significant correlation with GDP per capita (P <0.001). Overall qualities of digestive endoscopy were excellent, but certain results of quality indicators posed a huge challenge, such as the detection rates of adenoma and early cancers.

Conclusions
Impressive progress has been made in digestive endoscopy with rapidly expanding economy in China. However, primary healthcare, utilization rates, and income-related inequality of regional services were needed to be improved to promote public health better.
Latest advances on new promising molecular-based therapeutic approaches for Huntington’s disease
Authors: Cheng Yangfan , Zhang Sirui , Shang Huifang 
Published: May 2024

Abstract
Huntington’s disease (HD) is a devastating, autosomal-dominant inherited, neurodegenerative disorder characterized by progressive motor deficits, cognitive impairments, and neuropsychiatric symptoms. It is caused by excessive cytosine-adenine-guanine (CAG) trinucleotide repeats within the huntingtin gene (HTT). Presently, therapeutic interventions capable of altering the trajectory of HD are lacking, while medications for abnormal movement and psychiatric symptoms are limited. Numerous pre-clinical and clinical studies have been conducted and are currently underway to test the efficacy of therapeutic approaches targeting some of these mechanisms with varying degrees of success. In this review, we update the latest advances on new promising molecular-based therapeutic strategies for this disorder, including DNA-targeting techniques such as zinc-finger proteins, transcription activator-like effector nucleases, and CRISPR/Cas9; post-transcriptional huntingtin-lowering approaches such as RNAi, antisense oligonucleotides, and small-molecule splicing modulators; and novel methods to clear the mHTT protein, such as proteolysis-targeting chimeras. We mainly focus on the ongoing clinical trials and the latest pre-clinical studies to explore the progress of emerging potential HD therapeutics.
Eco-Innovation and Higher Education in the European Union: Exploring the Green Interconnection
Authors: Bucur Louisa-Maria 
Published: May 2024

Abstract
Currently, the emphasis is placed on sustainability, requiring economies to continuously innovate, demonstrating their competence for eco-innovation. This form of innovation is a key concept in today’s world, providing innovative and sustainable solutions to environmental challenges, expecting a high level of education. Thus, this paper aims to explore the relationship between eco-innovation and higher education within the member states of the European Union, concentraiting on the quantitative analysis of the impact of higher education on eco-innovation development in these countries. Applying regression analysis, the eco-innovation index and the level of education in EU countries will be inspected to establish any significant correlation between these two variables. Following the analysis, the collected data involved in the regression method will reveal possible interrelationships and significant influences between the level of education and the degree of eco-innovation development in the member countries of the EU. Conclusively, the research proposes to highlight the influence of higher education in the development of eco-innovation within the European Union. The suggested quantitative analysis could lead to a better understanding of the association between higher education and eco-innovation, revealing the potential that the education sector has in promoting sustainability and a green future for European Union.
The Convergence of Corporate Sustainability and Social Responsibility in Modern Business Contexts: A Bibliometric Analysis
Authors: Gorski Andra-Teodora , Dumitraşcu Dănuț Dumitru 
Published: May 2024

Abstract
Global challenges such as climate change, the Covid-19 pandemic, and geopolitical instability have driven organizations to embed sustainability into their strategies and operations, moving away from the profit-driven mindset to a broader emphasis on societal value. Sustainability, today a widespread concept in academia and business, requires balancing social, environmental, and economic responsibilities to achieve long-term success. Embracing the Triple Bottom Line (TBL) model, organizations aim for profit while also positively impacting society and the environment. However, there is a prevailing tendency to emphasize rights over responsibilities, a pattern that needs to shift in order to promote sustainability. This paper contributes to the body of knowledge on corporate sustainability by exploring its relationship with social responsibility. A bibliometric analysis was conducted on a sample of 9,378 documents processed using VOSviewer. The study examines the interplay between sustainability, social responsibility, and corporate sustainability, mapping out key concepts and visualizing their networks. Furthermore, the links to other key themes such as performance, management, innovation, strategy, governance, supply chain management, leadership, stakeholders, and others were also explored. Social-responsibility disclosure was also found to be an emergent topic in terms of organizational accountability and transparency.
Asymmetric Effects of Local and Global Business Cycle Variations on the Sectoral Industrial Production in Singapore
Authors: Iqbal Javed 
Published: May 2024

Abstract
This paper investigates the sensitivities of Singapore’s sectoral industrial production to local and global business cycle variations using the auto-regressive distributed lag (ARDL) model in the nonlinear and asymmetric cointegration framework. By employing monthly time series data from Jan 1983 to Dec 2022 the study corroborates the commonly held view that durable industries are pro-cyclic to thelocal business cycle. However, the nature of cyclic sensitivity is different if viewed from a global perspective. Industries including pharmaceutical, computer, and motor vehicles industries flourish in both the local and global business cycle booms. Almost all industries having long-run linkages with global industrial production are also affected by global production growth in the short run. However, consistent with earlier studies for Southeast Asian countries, very few industries exhibit short-run asymmetries in their relationship with local and global business cycles. We found that incorporating long-run information also improves the forecasting ability of sectoral industrial production growth in Singapore.
Digital Transformation: A quantitative analysis of Romanian SMEs
Authors: Mihu Cantemir , Herciu Mihaela 
Published: May 2024

Abstract
With the expansion and omnipresence of digital technologies in most aspects of human life, organizations have felt the need to implement changes. Thus, digital transformation has become imperative, with organizations having to adapt to the new standards imposed by a digitalized world. This paper analyses the digital transformation in Romanian SMEs using a methodology based on quantitative analysis of data collected through a questionnaire. The aim is to understand how these companies’ approach and implement digital transformation, as well as the impact of this process on management strategies and business models. The analysis of the quantitative research results will focus on the demographic profile of the companies, the stage of the digital transformation process, the impact on business performance, investments in digital transformation and predictive models. This work makes a significant contribution to understanding and promoting digital transformation among SMEs, providing a specific and relevant insight into the Romanian SME context.
Are Women Recognized in the Digital Economy? Experiences of Developed Economies
Authors: Ristanović Vladimir , Šostar Marko , Hak Mario 
Published: May 2024

Abstract
Data and debates about the role of women in the digital world are gaining increasing importance in the study of gender and the digital economy. This paper explores the complex relationship of the role of women in the digital economy over the last two decades. The analysis used a review of the literature of scientific works and documents of official international institutions. All available sources indicate progress in recent years, but challenges remain. Several samples were created, and quantitative analysis assessed trends over time. The general conclusions suggest that the role of women in the digital economy is increasingly pronounced and significant. The results of the study confirm global expectations that promote the role of women in the digital economy (glass ceilings), where women work in key positions (women on boards) and participate in decision-making (glass cliffs). The regression econometric model shows that the role of women in the digital economy is increasingly important to recognize women who use Internet networks to a greater extent (business, social), those with above basic overall digital skills, as well as ICT experts. The paper ends with a list of potential recommendations for the modern framework of the digital economy.
Developing Inclusive Models of Value Chain Financing
Authors: Săvescu Roxana Florența 
Published: May 2024

Abstract
This paper presents a framework for designing external financial intervention mechanisms in the form of partnership agreements between financial institutions and different economic participants involved in the farm machinery supply chain. The scope of these partnership agreements is to facilitate small farmers’ access to loans when purchasing agricultural equipment. The research methodology and research results are drawn from desk and field research conducted on the emerging agricultural market. Several inclusive conceptual models of financing mechanisms based on business relationships between financial institutions, small farmers and either the suppliers of agricultural equipment or buyers of agricultural outputs are presented.
Entry Mode in the Presence of International Outsourcing
Authors: Lingga Doriani , Simanjuntak Damiana 
Published: May 2024

Abstract
International outsourcing affects firms’ decisions in various ways. We develop a model to analyze the entry mode behavior of a foreign firm on entering a domestic market when international outsourcing takes place. Being vertically integrated, the foreign firm can either produce in-house input or (partially) outsource it from an outside input supplier. Such practices can be widely observed in real life, in which a firm may outsource some input despite being able to produce the input by itself for various strategic advantages. Depending on its strategy of obtaining input, we consider two entry modes, export and FDI, and derive conditions under which the foreign firm chooses one entry mode over the other. Among the findings, we present a case in which an increase in tariff may raise the likelihood of the foreign firm choosing export. The reason is that the tariff raises the input price and, hence, the rival’s marginal cost. Therefore, such a case prevails when the tariff is high enough to weaken the domestic firm but not too high that it harms the foreign firm. We feature several other interesting cases highlighting the roles of entry costs and in-house input production costs.
Exploring the Relationship Between Google Trends and Cryptocurrency Metrics
Authors: Orăştean Ramona , Mărginean Silvia Cristina , Sava Raluca 
Published: May 2024

Abstract
Bitcoin and Ethereum are the two largest cryptocurrencies in the world by market capitalization and trading volume and the most popular despite high price fluctuations. This paper analyzes the relationship between Bitcoin and Ethereum metrics and the internet search interest on cryptocurrencies. As the literature shows, Google searches signal investor attention and Google Trends has proven useful for nowcasting economic and financial indicators. We aim to find the impact of Google Trends on Bitcoin and Ethereum prices, trading volumes and market capitalization since 2015 and discuss the potential correlations and patterns that may exist between these metrics and Google search interest. Through correlation and time-series analysis, we provide insights into the dynamics of this relationship and its implications for understanding cryptocurrency market behavior. The interest in cryptocurrencies tracked by Google Trends is a good indicator of measuring the social interest in the cryptocurrency market that drives a price movement. On the other hand, the price fluctuations of Bitcoin and Ethereum generate media and social attention and increase the interest in these cryptocurrencies. We also observe a positive effect of Google Trends values on trading volumes. The findings could help investors to understand the cryptocurrencies dynamics and build their trading strategies and could be of special interest to policymakers.
Innovationspolitik für die Transformation zur Klimaneutralität
Authors: Peterson Sonja , Semrau Finn Ole 
Published: May 2024

Abstract
Rapid deployment and developments in green innovations are crucial for achieving climate neutrality. While market-based policy instruments are the backbone of climate policy, governmental support for research and development (R&D) complements an efficient policy mix that supports green innovations at all stages of the innovation process. Nonetheless, international R&D support has declined over the last decade. In an international comparison, Japan is at the forefront of green R&D support. Germany is also a leader in R&D support, but its market-based environmental policy stringency remains low, which undermines the importance of an adequate climate policy mix.