bluuebunny/crossref_metadata_2025_split huggingface dataset:
True Restriction in Diffusion-Weighted Imaging in a Mistreated Patient With Phenylketonuria
Authors: Palaiodimou Lina , Zompola Christina , Lachanis Stefanos , Mylona Varvara , Theodorou Aikaterini , Papagiannopoulou Georgia , Kotsali-Peteinelli Vasiliki , Foska Aikaterini , Kasti Arezina , Skouma Anastasia , Tsivgoulis Georgios 
Published: December 2020

Introduction:
Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism and causes neurological manifestations because of excessive accumulation of phenylalanine (PHE). It can also affect adult patients who discontinue their treatment, even if they had been under adequate metabolic control during childhood. For that reason, it is recommended that PKU treatment should be continued throughout life and target PHE levels for adult patients should range between 120 and 600 μmol/L.

Case Report:
The authors present an adult patient with PKU who discontinued treatment and developed cognitive dysfunction because of high blood levels of PHE. Brain magnetic resonance imaging (MRI) of the patient was characteristic for PKU, presenting periventricular and callosal white matter hyperintensities in T2 and fluid-attenuated inversion recovery sequences, which were additionally associated with true restriction in diffusion-weighted imaging sequence, a far less recognized PKU neuroimaging feature.

Discussion:
Cognitive dysfunction and psychiatric disorders can be present in adult patients with PKU who discontinue treatment and have poor PHE metabolic control. The presence of white matter hyperintensities in T2 and fluid-attenuated inversion recovery MRI-sequences is a well-described neuroimaging feature of PKU, but diffusion-weighted imaging sequence may also be reliable in detecting brain lesions in patients with PKU. PKU lesions should be considered in the differential diagnosis of true diffusion restriction in brain MRI of patients with PKU history or those who might have escaped newborn screening diagnosis but present neurocognitive dysfunction. Appropriate treatment for the management of PKU should be initiated for the reversal of the clinical and neuroimaging findings.
Evaluation of Drug Abuse by Hair Analysis and Self-Reported Use Among MSM Under PrEP: Results From a French Substudy of the ANRS-IPERGAY Trial
Authors: Chas Julie , Bauer Rebecca , Larabi Islam Amine , Peytavin Gilles , Roux Perrine , Cua Eric , Cotte Laurent , Pasquet Armelle , Capitant Catherine , Meyer Laurence , Raffi Francois , Spire Bruno , Pialoux Gilles , Molina Jean-Michel , Alvarez Jean-Claude 
Published: December 2020

Background:
We used the Agence nationale de Recherches sur le sida et les hépatites virales (ANRS)-IPERGAY trial to qualitatively and quantitatively measure drug use among men who have sex with men under preexposure prophylaxis using 2 different methods, to better understand and collectively respond to risky practices.

Method:
We included 69 volunteers of the ANRS-IPERGAY trial. We measured drug use by 2 methods: (1) drug detection by hair analysis and (2) reported drug use by self-reported drug consumption.

Results:
New psychoactive substances (NPS) and conventional drugs were detected in 53 of the 69 (77%) volunteers by hair analysis and in 39 of the 69 (57%) volunteers by questionnaires. On the 219 hair segments analyzed, the most commonly used drugs were cocaine in 47 of the 69 (68%), 3,4-methylenedioxymethamphetamine/ecstasy in 31 of the 69 (45%), and NPS in 27 of the 69 (39%). On the 1061 collected questionnaires, the most commonly used drugs were cocaine in 31 of the 69 (45%), 3,4-methylenedioxymethamphetamine/ecstasy in 29 of the 69 (42%), and NPS in 16 of the 69 (23%). Hair analysis detects more conventional drugs and/or NPS use (P < 0.05). Drug use identified by hair was significantly associated with a higher number of sexual partners in the past 2 months (P ≤ 0.001), more often casual partners (P ≤ 0.001), condomless anal sex (P ≤ 0.005), hardcore sexual practices (P ≤ 0.001), a higher number of sexually transmitted infections, and chemsex (P ≤ 0.05).

Conclusions:
Self-report drug use by questionnaires remains the reference tool for harm reduction at the individual level because of its feasibility and low cost. However, hair analysis is more sensitive, objectively assessing consumption, and interesting to understand uses and to be able to collectively respond to risky practices with adapted messages.
Expansion of Phenotypic and Genotypic Spectrum in Yao Syndrome
Authors: Yao Qingping , Kontzias Apostolos 
Published: December 2020

Objectives
Yao syndrome (YAOS; OMIM 617321) was formerly termed nucleotide-binding oligomerization domain-containing protein 2 (NOD2)–associated autoinflammatory disease. This study sought to report novel findings related to this disease.

Methods
A medical records review analysis of a case series was conducted, and all patients fulfilled the diagnostic criteria for YAOS and underwent comprehensive diagnostic workups, including molecular genotyping of blood specimens for periodic fever syndromes and NOD2-associated disease.

Results
A total of 11 patients with YAOS were analyzed, and all were Whites with a median age of 25.9 years at disease onset. All patients shared the similar autoinflammatory phenotype of YAOS. Among the 11 patients, we identified 7 patients who had the known phenotype of YAOS, as well as recurring and brief eyelid swelling with or without eyelid discoloration or conjunctivitis. Molecular analysis of blood cells using periodic fever gene panel has identified the presence of NOD2 variants in all 11 patients. Apart from the known YAOS-associated common NOD2 genotype, 5 novel and unknown significance NOD2 variants were identified in patients who presented with typical phenotype of YAOS.

Conclusions
This study provides novel clinical and molecular data for YAOS and supports the expansion of the phenotypic and genotypic spectrum of the disease.
The use of islands and cluster-randomized trials to investigate vector control interventions: a case study on the Bijagós archipelago, Guinea-Bissau
Authors: Jones Robert T. , Pretorius Elizabeth , Ant Thomas H. , Bradley John , Last Anna , Logan James G. 
Published: December 2020

Vector-borne diseases threaten the health of populations around the world. While key interventions continue to provide protection from vectors, there remains a need to develop and test new vector control tools. Cluster-randomized trials, in which the intervention or control is randomly allocated to clusters, are commonly selected for such evaluations, but their design must carefully consider cluster size and cluster separation, as well as the movement of people and vectors, to ensure sufficient statistical power and avoid contamination of results. Island settings present an opportunity to conduct these studies. Here, we explore the benefits and challenges of conducting intervention studies on islands and introduce the Bijagós archipelago of Guinea-Bissau as a potential study site for interventions intended to control vector-borne diseases.This article is part of the theme issue ‘Novel control strategies for mosquito-borne diseases'.
A multi-innovation with forgetting factor based EKF-SLAM method for mobile robots
Authors: Zhou Zhen , Wang Dongqing , Xu Boyang 
Published: December 2020

Purpose
The purpose of this paper is to explore a multi-innovation with forgetting factor-based EKF-SLAM (FMI-EKF-SLAM) algorithm to solve the error increasing problem, caused by the Extended Kalman filtering (EKF) violating the local linear assumption in simultaneous localization and mapping (SLAM) for mobile robots because of strong nonlinearity.

Design/methodology/approach
A multi-innovation with forgetting factor-based EKF-SLAM (FMI-EKF-SLAM) algorithm is investigated. At each filtering step, the FMI-EKF-SLAM algorithm expands the single innovation at current step to an extended multi-innovation containing current and previous steps and introduces the forgetting factor to reduce the effect of old innovations.

Findings
The simulation results show that the explored FMI-EKF-SLAM method reduces the state estimation errors, obtains the ideal filtering effect and achieves higher accuracy in positioning and mapping.

Originality/value
The method proposed in this paper improves the positioning accuracy of SLAM and improves the EKF, so that the EKF has higher accuracy and wider application range.
How does entrepreneurial leadership affect innovation work behavior? The mediating role of individual and team creativity self-efficacy
Authors: Bagheri Afsaneh , Akbari Morteza , Artang Armin 
Published: December 2020

PurposeThis paper explored the ways through which entrepreneurial leadership practices of chief executive officers (CEOs) enhance their employees' innovation at workplace in knowledge-based firms. Building on social cognitive theory and resource-based view of firms, this paper argues that entrepreneurial leadership fosters employees' innovation work behavior by enhancing their individual and team creativity self-efficacy.Design/methodology/approachThe sample was selected from knowledge-based firms in Iran using the simple random sampling method. Two mediation models were tested using data from 41 CEOs and 207 employees in two separate phases.FindingsThe analysis supported that CEOs' entrepreneurial leadership improves their employees' innovation work behavior through enhancing their individual and team creativity self-efficacy.Originality/valueThe research contributes motivational and enabling mechanisms at both individual and team levels that entrepreneurial leaders use to improve employees' innovation work behavior in the context of knowledge-based firms. The findings may assist managers and business leaders in effectively leading innovation process.
Narcissistic leadership and behavioral cynicism in the hotel industry: the role of employee silence and negative workplace gossiping
Authors: Aboramadan Mohammed , Turkmenoglu Mehmet Ali , Dahleez Khalid Abed , Cicek Berat 
Published: December 2020

Purpose
Building on leader-member exchange and social cognitive theories, this paper aims to propose a model of the influence of narcissistic leadership on hotel employees’ behavioral cynicism through the mediating roles of employee silence and negative work-related gossiping on this relationship.

Design/methodology/approach
The model was examined using covariance-based structural equation modeling using data collected from 468 employees working in several different departments in Italian hotels.

Findings
The findings illustrate that narcissistic leadership positively affects behavioral cynicism. Furthermore, employee silence and negative work-related gossiping are shown to have a significant mediating effect on this relationship.

Practical implications
The study may be of use for hotel managers as it demonstrates how narcissism can be very damaging to their organizations and employees.

Originality/value
To date, this study is the first to examine negative work-related gossiping and employee silence as mediator variables in the relationship between narcissistic leadership and behavioral cynicism in the hotel industry. Further, this research makes a significant contribution to the hospitality literature as the topic of narcissistic leadership has not, to date, been adequately investigated in the sector.
The decision tree for longer-stay hotel guest: the relationship between hotel booking determinants and geographical distance
Authors: Lee Yejin , Kim Dae-Young 
Published: December 2020

Purpose
Using the decision tree model, this study aims to understand the online travelers booking behaviors on Expedia.com, by examining influential determinants of online hotel booking, especially for longer-stay travelers. The geographical distance is also considered in understanding the booking behaviors trisecting travel destinations (i.e. Americas, Europe and Asia).

Design/methodology/approach
The data were obtained from American Statistical Association DataFest and Expedia.com. Based on the US travelers who made hotel reservation on the website, the study used a machine learning algorithm, decision tree, to analyze the influential determinants on hotel booking considering the geographical distance between origin and destination.

Findings
The results of the findings demonstrate that the choice of package product is the prioritized determinant for longer-stay hotel guests. Several similarities and differences were found from the significant determinants of the decision tree, in accordance with the geographic distance among the Americas, Europe and Asia.

Research limitations/implications
This paper presents the extension to an existing machine learning environment, and especially to the decision tree model. The findings are anticipated to expand the understanding of online hotel booking and apprehend the influential determinants toward consumers’ decision-making process regarding the relationship between geographical distance and traveler’s hotel staying duration.

Originality/value
This research brings a meaningful understanding of the hospitality and tourism industry, especially to the realm of machine learning adapted to an online booking website. It provides a unique approach to comprehend and forecast consumer behavior with data mining.
Power distribution operational risk model driven by FMEA and ISM approach
Authors: Sangode Pallawi Baldeo , Metre Sujit G. 
Published: December 2020

PurposeThe purpose of this paper is to identify various risks in the power distribution supply chain and further to prioritize the risk variables and propose a model to the power distribution industry for managing the interruptions in its supply chain. To accomplish this objective, a case of a major power distribution company has been considered.Design/methodology/approachFailure mode and effects analysis (FMEA) analysis has been done to identify the potential failure modes, their severity, and occurrence and detection scores. Then an interpretive structural model (ISM) has been developed to identify and understand the interrelationships among these enablers followed by MICMAC analysis, to classify the risk variables in four quadrants based on their driving and dependency powers.FindingsThe results of this study exhibit that technical failure in the information and technology system, the use of improper equipment, poor maintenance and housekeeping in the internal operations are the major risk drivers. Exposure to live wires and commercial loss in power supply has strong dependence power.Research limitations/implicationsThis study is limited to a single power distribution company and not the whole power distribution sector.Practical implicationsThis study suggests the managers of the power distribution company develop an initial understanding of the drivers and the dependent powers on the supply chain risks.Social implicationsThrough prioritization, identification of drivers and the dependent risks, the losses in the power distribution supply chain can be minimized.Originality/valueVarious failures in the power distribution have been studied in the past, but they have not investigated the supply chain risks in the power distribution of a power distribution company.
Does female labour force participation contribute to better financial inclusion? Evidence from cross-country analysis
Authors: Balasubramanian Senthil Arasu , Kuppusamy Thenmozhi 
Published: December 2020

PurposeThe purpose of this paper is to analyse the impact of female labour force participation (FLFP) in the access and usage of formal financial services by women.Design/methodology/approachThe study uses cross-country data from 107 countries. The study uses multivariate regression (OLS) to explain the impact of FLFP on the financial inclusion variables. The study also accounted for different groups of country-level control variables. Instrumental variables regression is also used in the study to consider for endogeneity issues.FindingsThe results show that FLFP has significant influence on all of the financial inclusion variables used in the study. The role of financial literacy is prominent in determining women's access to sophisticated financial services such as debit card and credit card. Improving financial infrastructure of an economy facilitates greater access to formal account by womenPractical implicationsFrom policymakers’ perspective, women should be motivated to enter labour market for better financial inclusion.Social implicationsMore opportunities for women to enter formal employment encourages female participation in labour market and benefits women and the economy.Originality/valueThis paper is the first of its kind to study the influence of FLFP on indicators of financial inclusion of women. The study extended the scope of access to financial services by considering access to bank account, debit card and credit card. The study also analysed use of financial services through digital platforms by women.
Using Fintech in scaling up Islamic microfinance
Authors: Shaikh Salman Ahmed 
Published: December 2020

Purpose
This study aims to propose a hybrid microfinance model that integrates various Islamic commercial and social finance institutions through Fintech for efficient and impactful results. The microfinance model caters to the financial and social intermediation needs through a set of financial services and non-financial support.

Design/methodology/approach
The study uses both a mathematical model and an empirical estimation using micro panel data to establish the core problem in microfinance operations. Conclusions from the mathematical model and estimated results in the empirical analysis are used to suggest an institutional design which embeds technology in the delivery of Islamic microfinance in an integrated structure. For screening and incentive conditions, the study gives illustration through numerical examples.

Findings
The mathematical model highlights the need for financial sustainability, outreach, scale and complementariness of non-financial factors such as commitment, repayment incentives and skills enhancement multiplier. In light of this, the proposed Islamic microfinance model is outlined to create synergies by integrating a diversity of funding sources through social savings and impact investments. The programme also blends financial services with non-financial support to ensure engagement and commitment on a long-term basis. It uses Fintech in various demand and supply-side operations to show how technology embeddedness can help in achieving cost efficiencies and extend outreach.

Originality/value
It is the first study in integrated institutional design in Islamic microfinance literature that embeds Fintech in both demand side and supply side operations comprehensively. The proposed model is conducive for enhancing outreach, scale and impact in the Islamic microfinancial services.
The integration of risk and performance management: the role of boundary objects
Authors: Bracci Enrico , Gobbo Giorgia , Papi Luca 
Published: December 2020

PurposeThis paper investigates the role of boundary objects and boundary work in the integration of risk management (RM) and performance management (PM) systems. In particular, the paper combines theoretical insights with an empirical focus to examine how shared contexts are created through the boundary work performed by key actors across knowledge boundaries.Design/methodology/approachThe paper develops an exploratory qualitative case study from a local government context. The methodology is based on document analysis and semi-structured interviews.FindingsBoundary objects can act as knowledge integration mechanisms, allowing key actors to understand the meanings and uses of RM and PM practices. The paper shows how collaborative versus competitive boundary work exerted by key actors can explain the creation of shared contexts leading to integration between RM and PM.Originality/valueThe results contribute to the debate about the integration of RM with other managerial systems. Differently from previous research, the integration theme is addressed in the present work by looking specifically to the integration between RM and PM. In doing so, the role of both boundary objects and the boundary work performed by relevant actors to demarcate their legitimacy and autonomy over preferred practices is portrayed.
The technological impact on real estate investing: robots vs humans: new applications for organisational and portfolio strategies
Authors: Souza Lawrence A , Koroleva Olga , Worzala Elaine , Martin China , Becker Alicia , Derrick Nathaniel 
Published: December 2020

PurposeThe goal of this paper is to present a roadmap for real estate operating companies (REOCs) to transform themselves into tech-centric enterprises.Design/methodology/approachThis qualitative approach is based on the impact of technology on physical real estate assets and organisational structures as reviewed in industry and academic literature, professional experience and current property technology (PropTech) applications.FindingsNew technologies are rapidly changing how investors, tenants and managers use, invest and finance property. The revolutionary change for the industry will be in its organisational and industry structure, away from the traditional hierarchical-mechanistic form to a virtual open-agile-innovative organisational form.Research limitations/implicationsResearch limitations come from the lack of real estate companies utilising the hybrid flipped form of organisational structures.Practical implicationsDue to the current state of the economy, effects of the pandemic and rapid adoption of new technologies, real estate companies are likely to radically change the way they are organised, how they add value, innovate and their leadership/management style.Social implicationsThe revolution in real estate technologisation will not come from the application of these technologies but the rapid change in ideological thought and management leadership style and culture.Originality/valueThe introduction of artificial intelligence/machine learning (AI/ML), blockchain, virtual reality, tablets, cell phones, applications, 5G, etc. is putting pressure on real estate organisations to change. These changes are long overdue and the future, modern real estate company will take a hybrid PropTech form – a company focussed on delivering high-quality products and services to its clients in real time.