bluuebunny/crossref_metadata_2025 huggingface dataset:
The Auxin Response Factor ARF27 is required for maize root morphogenesis
Authors: Dash Linkan , McReynolds Maxwell R. , Draves Melissa A. , Khangura Rajdeep S. , Muench Rebekah L. , Khor Jasper , Callwood Jodi B. , Cowling Craig L. , Mejia Ludvin , Lang Michelle G. , Dilkes Brian P. , Walley Justin W. , Kelley Dior R.
Published: August 2023

Crop root systems are central to nutrition acquisition and water usage. Root hairs and lateral roots contribute to fine-scale patterning of root systems and can offer many advantages for improving root function without drastically impacting overall system architecture. Key genetic regulators underpinning root hair morphogenesis have been well characterized in the model plant Arabidopsis but are less understood in maize. Here, we identify a novel determinant of root hair morphogenesis and auxin responses in maize, AUXIN RESPONSE FACTOR27 (ARF27), using both reverse and quantitative genetic approaches. ARF27 is enriched in maize primary root tissues at both the transcript and protein level. Loss ofARF27leads to short primary roots and reduced root hair formation, while lateral root density is unaltered. Inarf27roots, auxin-responsive gene expression is dysregulated, which is consistent with the predicted function of this transcription factor. Moreover, a genome wide association study (GWAS) to uncover genetic determinants of auxin-dependent root traits identifiedARF27as a candidate gene. Furthermore, auxin hypersensitive maize genotypes exhibit altered crown root length and surface area in field-grown plants. A gene regulatory network (GRN) was reconstructed and an ARF27 subnetwork was integrated with DAP-seq and GWAS data to identify ARF27 target genes. The ARF27 GRN includes known maize root development genes, such asROOTLESS CONCERNING CROWN ROOTS (RTCS), ROOTHAIRLESS 3 (RTH3)andRTH6. Altogether this work identifies a novel genetic driver of auxin-mediated root morphogenesis in maize that can inform agricultural strategies for improved crop performance.
Rapid genome diagnosis of alveolar capillary dysplasia leading to treatment in a child with respiratory and cardiac failure
Authors: Tower Dana R. , Day Ronald W. , Marrone Tighe , Palmquist Rachel , Nadauld Lincoln D. , Bonkowsky Joshua L. , Malone Jenkins Sabrina
Published: August 2023

Alveolar capillary dysplasia (ACD) is a fatal disorder that typically presents in the neonatal period with refractory hypoxemia and pulmonary hypertension. Lung biopsy is traditionally required to establish the diagnosis. We report a 22-mo-old male who presented with anemia, severe pulmonary hypertension, and right heart failure. He had a complicated hospital course resulting in cardiac arrest and requirement for extracorporeal membrane oxygenation. Computed tomography of the chest showed a heterogenous pattern of interlobular septal thickening and pulmonary edema. The etiology of his condition was unknown, lung biopsy was contraindicated because of his medical fragility, and discussions were held to move to palliative care. Rapid whole-genome sequencing (rWGS) was performed. In 2 d it resulted, revealing a novelFOXF1gene pathogenic variant that led to the presumptive diagnosis of atypical ACD. Cases of atypical ACD have been reported with survival in patients using medical therapy or lung transplantation. Based on the rWGS diagnosis and more favorable potential of atypical ACD, aggressive medical treatment was pursued. The patient was discharged home after 67 d in the hospital; he is currently doing well more than 30 mo after his initial presentation with only one subsequent hospitalization and no requirement for lung transplantation. Our case reveals the potential for use of rWGS in a critically ill child in which the diagnosis is unknown. rWGS and other advanced genetic tests can guide clinical management and expand our understanding of atypical ACD and other conditions.
Toward a fully coherent tender and hard X-ray free-electron laser via cascaded EEHG in fourth-generation synchrotron light sources
Authors: Yang X. , Yu L. H. , Smaluk V. , Shaftan T. , Huang X.
Published: August 2023

Free-electron-laser-based beamlines utilize fully coherent laser pulses with extremely narrow bandwidth allowing direct use of X-rays without monochromators. This could be very beneficial for all users of current and future fourth-generation diffraction-limited synchrotron light sources (DL-SLSs) who need narrowband full-coherence high-brightness X-ray pulses. Based on our previous finding, i.e. that separating the two stages of echo-enabled harmonic generation (EEHG) with a few extra bending-magnet sections provides an effective way to increase the momentum compaction of chicane 1, one can simultaneously achieve adequate prebunching at extremely high harmonics as well as keep the energy modulation to the ideal minimum. This could open the door for cascaded EEHG, toward fully coherent tender and hard X-ray wavelengths. Built on our compact design of a twin-pulse seeding electron beam with an adjustable delay and timing jitter at the level of a few femtoseconds, a cascaded EEHG can be implemented, which includes two EEHG beamlines, where the radiation pulse generated by the first beamline with harmonic h
1 could be used as the input seed laser pulse to the second beamline with harmonic h
2. Hence, the second radiator could potentially reach very high harmonics [h = h
1(20)h
2(25–100)] from 500 to 2000, corresponding to tender and hard X-ray wavelengths. It is demonstrated that the cascaded EEHG scheme is compatible with almost any current or planned fourth-generation DL-SLS, with significant benefits for space-limited storage rings in particular. The main advantage is that this scheme requires almost no change of the storage-ring lattice and is fully compatible with other beamlines. Current proposals for rings with much longer straight sections would add self-amplified spontaneous emission as another viable option for storage-ring-based free-electron lasers.
Impact of faith on food marketing and consumer behaviour: a review
Authors: Santovito Savino , Campo Raffaele , Rosato Pierfelice , Khuc Long Dai
Published: August 2023

Purpose

The aim of this manuscript is to study the current scientific literature on food marketing and religion, by giving an overview of current knowledge and the possible future research opportunities once found the gaps.

Design/methodology/approach

This a classic literature review carried on by considering marketing, consumer behaviour and management journals as well as religion-focused ones.

Findings

Current literature highlights how faith impacts on food marketing and religious consumers choices (and not only). Most of the papers focus on Islam. Considering the globalised economy, the authors’ findings highlight also the relevance of religious certifications for less/not religious people, because of certified food is considered safe and healthy.

Originality/value

The paper joins and reviews two research fields whose connection is growing, so it is relevant to know the current status of scientific literature, which is updated compared to recent literature.
Theoretical analysis and numerical simulation of axial tensile behavior of pipe joints based on bi-linear cohesive bond-slip model
Authors: Yuan Hong , Han Jun , Lu Huaqiang , Li Junhui , Zeng Lan
Published: August 2023

Purpose

Due to its inexpensive production costs, low stress concentration and maintenance-friendliness, the adhesive bonded pipe joint is frequently utilized for pipe connection. However, further theoretical analysis is needed to understand the debonding failure mechanism of such bonded pipe joints under axial tension.

Design/methodology/approach

In this study, based on the bi-linear cohesive zone model, the integrated closed-form solutions were derived by considering the axial stiffness ratio and failure stage to determine the relative interfacial slip, interfacial shear stress and relationship of tension–displacement in the bonded pipe joint.

Findings

Additionally, solutions for the critical bonded length and the ultimate load capacity were put forth. Besides, the numerical study was conducted to verify the theoretical solutions regarding the load–displacement relationship. The interfacial shear stress distribution at different failure stages was presented to understand the interfacial shear stress transmission and debonding process. The effect of bonded length on the ultimate load and ductility of pipe joints was also discussed.

Originality/value

The findings in this study can give a reference for the design of bonded pipe joints in their actual engineering applications.
Health professions’ perspective on emergency remote education
Authors: Ribeiro Cleide Gisele , Ramos Plinio dos Santos , Bechara Raimundo Nonato , de Oliveira Juliano Machado , de Almeida Erika Bicalho , Miguel Soraida Sozzi , Ricardo Djalma Rabelo , de Oliveira Rodrigo Guerra
Published: August 2023

Purpose

The COVID-19 pandemic has created a significant disruption in the educational systems worldwide. Some institutions opted for emergency remote education due to the need to cancel in-person activities. The aims of this paper were to evaluate the use of asynchronous methodology in health sciences education, determine whether asynchronous methodology was sacrificing overall student satisfaction, and investigate whether satisfaction improved as the program develops.

Design/methodology/approach

Initially, there was phase 1 that corresponded to four weeks of activities. Each professor produced a video lesson, and after each video lesson, a weekly educational activity was made available. Next, phase 2 was implemented using the same methodology, however lasting six weeks. Three questionnaires were developed, and a Likert scale was administered to verify the students’ level of satisfaction. Data were analyzed using frequency distributions, mean values, standard deviation and confidence interval. The normality of the sum data (total of the questionnaires) was tested using the Kolmogorov–Smirnov test.

Findings

Although the students pointed out that the asynchronous methodology facilitated access to the content and considered this methodology satisfactory, they expressed a reduced level of satisfaction regarding emergency remote education in general when data from the first weeks were compared to those of the previous weeks. It is clear that students became increasingly discouraged and tired over time, which motivated the institution to shift into a combination of synchronous and asynchronous methodology to improve student learning.

Originality/value

Teaching in the field of health care encompasses difficult competencies that sometimes are impossible to be learned remotely, so there is a need to examine and evaluate properly the remote education in this area. With careful planning, educational institutions can evaluate their experiences during the pandemic, allowing those involved to highlight strengths and identify weaknesses to better prepare for future needs to improve remote education.
Transgenerational control intention and employment practices of family firms in China
Authors: Chen Jiawen , Li Pengfei , Liu Linlin
Published: August 2023

Purpose

This study aims to examine the employment practices of family firms in emerging markets. Drawing from the social exchange theory, the authors propose that transgenerational control intention enhances the motivation for family owners to engage in favorable employment practices as inducement for future contribution of employees.

Design/methodology/approach

Multilevel regression models were applied to test the hypotheses with a sample of 3033 Chinese private family firms.

Findings

The results show that the employment practices of family firms are positively associated with transgenerational control intention, and the effect of transgenerational control intention is contingent on regional social trust.

Originality/value

This study highlights the role of transgenerational control intention of family owners in motivating favorable employment in family firms. The study adds nuance to the variances in employment behaviors of family firms as well as the family owner-employee exchange relationship in emerging markets.
Wine international market structure and competitiveness
Authors: Thomé Karim Marini , Paiva Vitoria Angie Leal , Gois Tafarel Carvalho de
Published: August 2023

Purpose

This paper aims to analyse the wine market in relation to international competitiveness and international market structure.

Design/methodology/approach

To describe the international market structure, this paper uses Herfindahl–Hirschman Index and Net Export Index to measure export competitiveness revealed symmetric comparative advantage (RSCA). Finally, survival function analyses were developed using the Kaplan–Meier product-limit estimator to characterise the stability and duration of the competitiveness in the international wine market of each country and after they were grouped into Old and New World wine-exporting countries, and Wilcoxon and the Log-rank tests were used to compare the survivor functions.

Findings

The findings have revealed that the import market structure has remained unconcentrated, whereas the export market structure is moderately concentrated. Concerning trade characteristics, France, Italy, Spain, Australia, Chile, New Zealand, Portugal, Argentina, South Africa and Georgia are exporters. Austria is a trader (re-exporter), and the USA, Germany, the UK and the Netherlands are importers with strong domestic consumption. Regarding the RSCA, the New and Old World wine-exporting countries have high scores, specifically France, Italy, Spain, Australia, Chile, New Zealand, Portugal, Argentina, South Africa and Georgia. However, the advantages have weakened for most of the countries analysed. Only a few Old World wine-exporting counties (France, Italy, Spain, Portugal and Georgia) have demonstrated stable comparative advantages over time. However, when grouped into Old World and New World, their survivor functions present little statistical differentiation during the period.

Originality/value

The originality of the paper is that it applies the industrial organisation and comparative advantage approaches to the wine international market, highlighting the top global players. The paper also makes valuable contributions to the wine literature by analysing the duration and stability of comparative advantage in the worldwide wine trade at a country level and comparing them grouped into Old and New World wine-exporting countries.
Creativity and commerce: a shifting balance for specialty foods and beverages
Authors: Andersen Kristina Vaarst , Münchow Morten
Published: August 2023

Purpose

Producers of specialty products that apply nonindustrial production techniques to deliver high quality and authenticity to niche markets define themselves in opposition to industrial production. This forces them to strike a delicate balance between, on the one hand, emphasizing the symbolic value related to quality, creativity and distance from traditional industrial production, and on the other hand, ensuring profitability through commercial orientation. This paper aims to explore how new and established firms benefit from a different balance between a strategic orientation toward creativity and the symbolic value of specialty products and commercialization.

Design/methodology/approach

Based on survey data from 99 specialty coffee roasters, this study uses statistical regression models to analyze how new versus established firms benefit from a strategic orientation toward creativity versus commercialization.

Findings

The authors find that firms benefit from shifting the balance from a strategic orientation on creativity in new firms to a strategic orientation on commercialization in more established firms. This shift increases the likelihood that firms’ passion and creativity lead to commercial success.

Originality/value

The findings demonstrate the importance of strategic orientation and emphasize that business owners need to revisit and adjust their strategic orientation as their businesses develop.
The relationship between the role of management accountants, advanced manufacturing technologies, cost system sophistication and performance: a path model
Authors: Banhmeid Badr , Aljabr Abdulrahman
Published: August 2023

Purpose

This paper aims to test a contingency-based path model that concurrently links the role of management accountants (MA) and advanced manufacturing technologies (AMTs) to cost system sophistication (CSS), as well as linking the latter to improvements in organisational performance through improving cost management and product planning decisions.

Design/methodology/approach

This paper used the questionnaire survey strategy to collect data from 373 medium and large manufacturing business units based in the UK, then subjected the data to structural equation modelling analysis to test a contingency-based path model.

Findings

The results show that the role of MAs and AMTs positively influence CSS. Moreover, it was found that the latter is positively associated with improvements in cost management decisions which, in turn, lead to improvements in organisational performance. However, no support was found for the association between the level of CSS and improvements in product planning decisions, although the latter was found to be positively associated with organisational performance. These results confirm the theory and empirical findings regarding the role that MAs and AMTs play in designing the cost accounting system, and support the argument that adopting sophisticated cost systems does not lead directly to improvements in organisational performance, unless the benefits of such systems, in terms of improved decision-making and cost applications, are used.

Originality/value

This research contributes to the literature by testing a contingency-based path model that incorporates hitherto underexamined contextual factors, namely, the role of MAs and AMTs; examining the effect of CSS on a critical output, organisational performance and the mechanisms of this effect; and considering the complexity of the business environments through the concurrent testing of the relationships involved in the research model.
Achieving acoustic comfort in the library: a sound masking initiative
Authors: Manna Debabrata , De Sarkar Tanmay
Published: August 2023

Purpose

The purpose of this paper is to highlight the sources of noise generation in a library and suggest the implementation of a sound masking system to provide acoustic comfort, maintain speech privacy and create an environment more engaging for the users.

Design/methodology/approach

Analyzing the existing literature and exploring the existing practices as observed in different libraries, the study gives an overview of the sound masking initiatives in libraries.

Findings

With practical examples of libraries, the study demonstrates how a sound masking system has been implemented to invoke better acoustic design in the library. The expansion of various activities in the library and a gradual shift from individual attention to a collaborative approach necessitates a strong focus on the acoustic design architecture of the library. The study showcases how the libraries adopt sound masking with the introduction of acoustic panels, dual panel partitions, sound-absorbent false ceilings, sound insulation, sound isolation and noise-dampening measures, installing furniture with sound containment features, adopting vibration control mechanism, mounting of white noise machines, etc., keeping the aesthetic quotient of the library alive.

Originality/value

The study attempts to show the current practices of the adoption of the sound masking system in libraries and promotes collaborative reading with the creation of an acoustic design-influenced library environment to control noise and reverberation and provide a comfortable reading environment.
The ambiguity and limitations of measuring firm-level multinationality: a theoretical and empirical exploration
Authors: Vaněk Patrik
Published: August 2023

Purpose

This paper aims to explore the ambiguity and limitations of measuring firm-level multinationality (FLM) using theoretical and empirical comparisons of existing methods. The paper puts forward a list of five key aspects that collectively serve as a tool for researchers to select the most appropriate method for future research and as a basis for the future development of methods.

Design/methodology/approach

Firstly, the author reviews existing methods of measuring FLM and consolidates findings into five key aspects. Secondly, the author uses the aspects to compare existing methods theoretically, and subsequently, the author groups them into three distinct streams. Thirdly, the author compares existing methods across a sample of the 35 largest European MNEs by sales in 2020 to identify and demonstrate the ambiguity and limitations of these methods.

Findings

The author identifies the five key aspects of measuring FLM: framework, aggregation, segmentation, metrics and indicators. Using empirical comparison, the author empirically confirms the limitations highlighted in the literature and shows the differences and inconsistencies among methods, which cause confusion rather than clarity in the extant literature. Additionally, the author emphasises that three distinct streams further drive the debate on the regional/global nature and present further limitations of methods not mentioned in the literature to date.

Originality/value

This paper provides the most comprehensive review of the existing literature on FLM, resulting in five novel aspects of measuring FLM. The analysis of a sample of 35 European firms demonstrates and identifies the ambiguity and limitations of FLM-measuring methods.
Trustworthy brand signals, price fairness and organic food restaurant brand loyalty
Authors: Konuk Faruk Anıl
Published: August 2023

Purpose

This research aims to examine the influence of brand signals (transparency, innovativeness and reputation) on brand trust and price fairness toward organic food restaurant brand.

Design/methodology/approach

The empirical data were gathered with the survey instrument from respondents who had previously visited organic food restaurant. The formulated hypotheses were analyzed with structural equation modeling.

Findings

The collected data demonstrated the positive effect of brand signals on brand trust and price fairness. Trust in organic food restaurant brand was found to positively impact both price fairness and brand loyalty. Additionally, the results provided evidence of the positive linkage between price fairness and brand loyalty. Among brand signals, brand transparency exerted the greatest impact on brand trust and price fairness. The findings also indicated the significant indirect linkage of brand signals on restaurant brand loyalty through brand trust and price fairness.

Originality/value

Through the lens of signaling theory and equity theory, this study provides novel insights into how brand signals contribute to brand trust, price fairness and organic food restaurant brand loyalty. The managerial implications for implementing brand strategies for organic food restaurants were discussed.
Factors that enhance and limit youth empowerment, according to social educators
Authors: Llena Berñe Asunción , Planas-Lladó Anna , Vila-Mumbrú Carles , Valdivia-Vizarreta Paloma
Published: August 2023

Purpose

This study aims to identify the contextual and relational factors that enhance and limit the empowerment of young people from the perspective of social education professionals.

Design/methodology/approach

Bronfenbrenner's bioecological model made it possible to locate the narratives of the educators in the territory. These narratives include field diaries, i.e. hybrid narratives that include visual, written and spoken materials, and focus groups with 11 educators from different fields of action and related to youth empowerment projects.

Findings

According to these educators, the most important factors for empowering young people are their immediate environment, and the issues that affect them most. For these factors to be empowering, young people need to be accompanied, with support based on connectedness, horizontality and the creation of safe spaces and learning experiences. Both the microsystem and the mesosystem form the immediate reality for their action. Aware of this, educators do the work of connecting with the exosystem.

Practical implications

It is evident why communities are spaces with opportunities for youth empowerment, and the authors observe the need for more transversal and less welfare-based social and youth policies that generate empowerment instead of dependency.

Social implications

This methodology evidenced the environmental structures of educators and the dissimilar levels to explore and understand the work of educators and the complex interrelationships, which play an important role in empowerment processes.

Originality/value

This research presents a new perspective that allows traditional qualitative reflection to be embedded in the bioecological model. All of this sheds light on relational ecosystems with young people and proposes youth policies, in this case, oriented towards empowerment.
Exploring the link between home country attributes and firms' internationalisation: evidence from GEDI and WEF data
Authors: Ndofirepi Takawira Munyaradzi
Published: August 2023

Purpose

This study aims to examine the degree to which a selection of home country factors affects the proclivity of firms to internationalise. The study also proposes and tests a conceptual model that fuses institutional and resource-based theories to improve our understanding of firm internationalisation.

Design/methodology/approach

The study uses cross-sectional, national-level secondary data from the 2018 Global Entrepreneurship Development Institute and World Economic Forum data sets on global entrepreneurship and competitiveness indices for 137 countries. The data is analysed using correlation and hierarchical regression analysis to test the hypotheses.

Findings

The results indicate that national income, institutions, trade openness and availability of risk capital positively influenced firm internationalisation, while home-country networking had an inverse effect. However, home country infrastructure had no statistically significant effect on firm internationalisation.

Research limitations/implications

The findings highlight the importance of considering home country attributes in understanding the internationalisation of firms.

Originality/value

This study contributes to the body of knowledge by providing empirical evidence of the role of local factors on the internationalisation of entrepreneurial ventures. It also tests a novel conceptual model that integrates institutional and resource-based theories to explain the nuances of the internationalisation of business ventures globally.
Family bargaining over parental leave: A collective household model with endogenous gender power
Authors: Sakamoto Ryo
Published: August 2023

Although existing studies have suggested that men's leave‐taking positively affects children's development and their involvement in childcare and household chores in the long run, the underlying mechanism remains unclear. To shed light on this ambiguity, this study develops a dynamic collective household model and analyses household decisions on parental leave‐sharing between spouses, resource allocation, home production, and labour supply. We demonstrate that gender equality in bargaining positions within families before leave‐taking is crucial in explaining the positive impacts of men's leave‐taking. Specifically, if women's intrahousehold bargaining power before taking parental leave is sufficiently low, their husbands are unlikely to take leave, and women's positions may deteriorate after leave. Consequently, households may allocate fewer resources to children, and men may not actively participate in childcare and household chores. Our model suggests that implementing parental leave policies for both genders may not be sufficient; simultaneous measures to increase women's bargaining power are necessary to encourage men to take parental leave and maximize the policies' benefits.
Authenticity and Identity in Adolescent Decision‐Making
Authors: Auckland Cressida
Published: August 2023

While much criticism has surrounded the seemingly incoherent law governing decision‐making for adolescents, relatively little work has sought to address the question of whether and when adolescents will be acting autonomously in decisions to refuse treatment, particularly in cases where their choices are motivated by religious beliefs inculcated from a young age. This is important, not just because it goes to the heart of when an adolescent ought to be able to exercise a right to self‐determination, but also because an analysis of the case law indicates that judges are taking account of concerns about the authenticity of the values or beliefs which motivate a given decision when deciding cases. This paper will examine how different accounts of autonomy and authentic choice map onto the psychological development of adolescents, and thus the extent to which adolescent's decisions to refuse treatment on religious grounds can be regarded as truly authentic. It will conclude by considering how such cases ought to be approached by the courts, suggesting a modified Mental Capacity Act 2005 test for assessing adolescent capacity, and offering an empirically‐grounded justification for overriding the choices of even capacitous adolescents.
Developing a transgender and non‐binary inclusive obstetrics and gynaecology undergraduate medical curriculum in Aotearoa/New Zealand: Where are we at, and where do we need to be?
Authors: Jones Lilian , Carroll Rona , Parker George , Steers Denise , Ormandy Judy , Filoche Sara
Published: August 2023

Internationally, undergraduate medical education is not currently enabling early career doctors to meet the needs of trans and gender diverse (TGD) people as healthcare consumers. This review outlines inclusion of TGD education in undergraduate medical education more broadly to contextualise curriculum development needs in obstetrics, gynaecology and reproductive medicine in Aotearoa/New Zealand. Limited, and lack of integrated content, teaching capability and current absence of TGD health knowledge as graduate outcomes, compounded by pedagogy (biomedical/binary framing) and more appropriate learning resources are indicators for curricula, and workforce, development.
How to optimize outcome of patients undergoing HLA‐matched related haematopoietic stem cell transplantation in acquired and inherited bone marrow failure syndromes
Authors: Giardino Stefano , Pierri Filomena , Faraci Maura
Published: August 2023

Up‐front allogeneic haematopoietic stem cell transplantation (allo‐HSCT) after a reduced intensity conditioning regimen is the standard treatment in children with acquired severe aplastic anaemia (aSAA) and inherited bone marrow failure syndromes (iBMFs) in the presence of a healthy matched related donor (MRD). The paper by Alsultan et al. report the safety and efficacy of MRD HSCT conditioned with low‐dose cyclophosphamide, fludarabine and thymoglobulin in both aSAA and non‐Fanconi iBMFs, strengthening the concept of the pivotal role of immunosuppressive approach in allo‐HSCT for specific subgroups of non‐malignant diseases requiring a reduced risk of toxicities, offering the opportunity to discuss the essential points for achieving patients' long‐term survival after MRD HSCT in BMF.

Commentary on: Alsultan et al. Human leucocyte antigen‐matched related haematopoietic stem cell transplantation using low‐dose cyclophosphamide, fludarabine and thymoglobulin in children with severe aplastic anaemia. Br J Haematol 2023;203:255‐263.
Myelin protein zero mutation‐related hereditary neuropathies: Neuropathological insight from a new nerve biopsy cohort
Authors: Bremer Juliane , Meinhardt Axel , Katona Istvan , Senderek Jan , Kämmerer‐Gassler Elke K. , Roos Andreas , Ferbert Andreas , Schröder J. Michael , Nikolin Stefan , Nolte Kay , Sellhaus Bernd , Popzhelyazkova Klimentina , Tacke Frank , Schara‐Schmidt Ulrike , Neuen‐Jacob Eva , de Groote Chantal Ceuterick , de Jonghe Peter , Timmerman Vincent , Baets Jonathan , Weis Joachim
Published: August 2023

Myelin protein zero (MPZ/P0) is a major structural protein of peripheral nerve myelin. Disease‐associated variants in the MPZ gene cause a wide phenotypic spectrum of inherited peripheral neuropathies. Previous nerve biopsy studies showed evidence for subtype‐specific morphological features. Here, we aimed at enhancing the understanding of these subtype‐specific features and pathophysiological aspects of MPZ neuropathies. We examined archival material from two Central European centers and systematically determined genetic, clinical, and neuropathological features of 21 patients with MPZ mutations compared to 16 controls. Cases were grouped based on nerve conduction data into congenital hypomyelinating neuropathy (CHN; n = 2), demyelinating Charcot–Marie‐Tooth (CMT type 1; n = 11), intermediate (CMTi; n = 3), and axonal CMT (type 2; n = 5). Six cases had combined muscle and nerve biopsies and one underwent autopsy. We detected four MPZ gene variants not previously described in patients with neuropathy. Light and electron microscopy of nerve biopsies confirmed fewer myelinated fibers, more onion bulbs and reduced regeneration in demyelinating CMT1 compared to CMT2/CMTi. In addition, we observed significantly more denervated Schwann cells, more collagen pockets, fewer unmyelinated axons per Schwann cell unit and a higher density of Schwann cell nuclei in CMT1 compared to CMT2/CMTi. CHN was characterized by basal lamina onion bulb formation, a further increase in Schwann cell density and hypomyelination. Most late onset axonal neuropathy patients showed microangiopathy. In the autopsy case, we observed prominent neuromatous hyperinnervation of the spinal meninges. In four of the six muscle biopsies, we found marked structural mitochondrial abnormalities. These results show that MPZ alterations not only affect myelinated nerve fibers, leading to either primarily demyelinating or axonal changes, but also affect non‐myelinated nerve fibers. The autopsy case offers insight into spinal nerve root pathology in MPZ neuropathy. Finally, our data suggest a peculiar association of MPZ mutations with mitochondrial alterations in muscle.
The cryobiopsy in interstitial lung diseases guided by probe‐based confocal laser endomicroscopy is feasible
Authors: Zheng Yu , Zhang Liyan , Lou Yueyan , Fan Bijun , Cui Yongqi , Wu Xueling , Tan Xiaoming
Published: August 2023

Background

Transbronchial lung cryobiopsy (TBLB) is routinely used to diagnose the interstitial lung disease (ILD). These results are consistent with those of surgical lung biopsy. Fluoroscopy is also used to confirm the final position of the cryoprobe; however, it can increase radiation exposure for both patients and medical care personnel. Probe‐based confocal laser endomicroscopy (pCLE) is a novel optical imaging technique that allows real‐time imaging at the cellular level in vivo. pCLE technology can also be used to identify malignancy, acute rejection in lung transplantation, amiodarone lung, and pulmonary alveolar proteinosis and visualize elastin fibres in the alveolar compartment.

Objectives

The aim of this study is to investigate the ability of pCLE to distinguish fibrotic pulmonary issues from normal lung disease and the safety and feasibility of CLE‐guided bronchoscopy and transbronchial lung cryobiopsy (TBLC) in patients with interstitial lung disease (ILD).

Methods

pCLE images from 17 ILD patients were obtained during TBLB. These images were then compared with histology results to assess the correspondence rate.

Results

pCLE imaging of the alveolar structures was performed. Key characteristics were visible, which could potentially influence the diagnostic rate (fibrotic areas) and the complication rate (blood vessel and pleura).

Conclusion

pCLE may reduce complications and increase the diagnostic yield. It is a potential guidance tool for cryobiopsy in the patients with ILD without fluoroscopy.
Exploration of the minimal clinically important difference value of the 3‐min simulated pedal motion in patients with chronic obstructive pulmonary disease: A self‐controlled prospective clinical trial
Authors: Zhang Xiaoting , Liu Ni , Yang Feng , Su Guansheng , Hu Jieying , Chen Rongchang , Zheng Zeguang
Published: August 2023

Background

To help elderly patients with severe or very severe chronic obstructive pulmonary disease (COPD) with pulmonary rehabilitation, we have developed Zheng's supine rehabilitation exercise (ZSRE). Currently, none of the terminal or critically ill patients with severe exercise limitation can complete the 6‐min walking distance (6MWD) and cardiopulmonary exercise testing (CPET).

Methods

In this study, we discuss the definition of the standardized 3‐min simulated pedal motion (3MSPM) test and its operational specifications. Also, we evaluate the minimal clinically important difference (MCID) value of the 3MSPM.

Results

The results showed that the mMRC score of COPD patients with acute exacerbation of dyspnea was progressively reduced from the second day of respiratory rehabilitation, and the difference between the first and seventh days was statistically significant (p < 0.000, χ2 = 176.664). 6MWD increased progressively, and the difference between 6MWD on day 1–7 was statistically significant (p = 0.024, F = 2.443). The difference between 3MSPM on day 1–7 was also statistically significant (p < 0.000, F = 4.481). Further analysis showed that 6MWD was negatively correlated with mMRC (p < 0.000, OR = −0.524). 3MSPM was positively correlated with 6MWD (p < 0.000, OR = 0.640) but negatively correlated with mMRC (p < 0.000, OR = −0.413). There is a linear regression relationship between 6MWD and 3MSPM, that is, 6MWD = 14.151 + 0.301 * 3MSPM, adjusted R2 = 0.401.

Conclusion

Based on the regression equation, 3MSPM can predict 6MWD, and it can be used as a simple exercise endurance method to evaluate patients with safety hazards in underground activities or who cannot complete the 6MWD test. The minimum clinically important difference value is increased by 23.
Botulism after intragastric botulinum toxin injections for weight reduction
Authors: Hennen Christina , Demir Seda , Dafsari Haidar Salimi , Wunderlich Gilbert , Böll Boris , Hüser Christoph , Barbe Michael Thomas , Fink Gereon Rudolf , Rueger Maria Adele
Published: August 2023

Although—considering the risk–benefit ratio—botulinum neurotoxin A (BoNT/A) is unequivocally recommended to treat severe neurological diseases such as dystonia, this has not yet been determined for its endoscopic intragastric injection aimed at weight reduction in obesity. However, severe adverse effects of intragastric BoNT/A had not yet been reported, prompting some European countries to endorse its (off‐label) use and treat patients transnationally. We here present three cases of botulism after intragastric BoNT/A injections for obesity treatment in a Turkish hospital. Patients presented with cranial nerve affection, bulbar symptoms, and descending paresis, and benefited from treatment with BoNT antitoxin and pyridostigmine. We assume that iatrogenic botulism was induced by overdosing in combination with toxin spread via the highly vascularized gastric tissue. Of note, within a few weeks, more than 80 cases of iatrogenic botulism were reported across Europe after identical intragastric BoNT/A injections. These cases demonstrate the risks of BoNT/A injections if they are not applied within the limits of evidence‐based medicine. There is a need for international guidelines to define the indication and a safe dosing scheme, especially in the context of medical tourism.
Interactions of Na2SO4 and yttrium‐silicate environmental barrier coatings at 825°C
Authors: Ardrey Kristyn , Heinrich Helge , Opila Elizabeth
Published: August 2023

Yttrium‐silicates (Y2Si2O7 and Y2SiO5) are candidate environmental barrier coating (EBC) materials for silicon carbide ceramic matrix composites (SiC‐CMCs). These materials’ high‐temperature, high‐velocity steam, and siliceous debris resistance are well studied. However, Na2SO4‐induced hot corrosion mechanisms are less understood. Free‐standing atmospheric plasma sprayed Y2Si2O7 and Y2SiO5 coupons were exposed to 2.5 mg/cm2 of Na2SO4 at 825°C in 0.1% SO2‐O2 (g). Scanning electron microscopy, X‐ray diffraction, transmission electron microscopy, and inductively coupled plasma‐optical emission spectrometry were used to identify a previously unknown damage mechanism. Water‐soluble Y and Na‐Y sulfates and oxysulfates formed in reaction with Na2SO4, causing significant damage to the yttrium‐silicate EBCs materials.
Phase transformation induced fatigue of a Ce‐TZP/Al2O3 composite
Authors: Cheng Yung‐Shin , Chen Yu‐Xin , Tuan Wei‐Hsing , Chen Jin‐Ren , Lai Po‐Liang
Published: August 2023

In this study, the stress–cycle number (S–N curve) of a composite consisting of ceria‐stabilized zirconia (Ce‐TZP) and alumina (Al2O3) was measured. First, the initial bending strength of the Ce‐TZP/Al2O3 composite was determined using a four‐point bending technique. Next, stress above 70% of the initial bending strength was applied on the composite for up to 1,000,000 cycles. Transformation bands were formed on the tensile surface in the first several cycles as the stress was higher than a critical value. The number and width of bands then changed marginally after their fomraiton. Fatigue failure occured at the band with the largest width, as a result of slow crack growth process. The transformation bands could be removed using a post‐fatigue heating treatment. After removal of these bands, the strength was consequently enhanced.
Clinical and histologic findings after use of a novel combined retinol, tripeptide, and glaucine containing cream in the treatment of photo‐aged skin
Authors: Rao Shikha , Goldberg David
Published: August 2023

Background

Neck skin is thinner and has a more delicate dermal layer than facial skin. The studied product was specifically formulated for the neck combining a hydrating delivery system with a trifunctional corrective technology composed of 0.2% pure retinol, 2.5% tripeptide concentrate, and 5.0% glaucine complex to help improvement in signs of aging.

Objectives

To evaluate cosmetic and histologic changes 3 months after treatment using immunostains for Type I collagen, Type III collagen, and glycosaminoglycan (GAGS). In addition, overall clinical improvement in photoaged skin was measured by both Griffith's photonumeric photoaging scale, photographic improvement, and questionnaires.

Methods

This study was an open‐label, blinded clinical trial evaluating a combined retinol, tripeptide, and glaucine containing cream in the treatment of photo‐aged skin. The study enrolled a total of 20 healthy male or female subjects, who applied the product for 3 months to their face and neck.

Results

Clinical as well histologic changes were consistent with improvement in all 20 subjects.

Conclusion

Use of a combined retinol, tripeptide, and glaucine containing cream led to both clinical and histologic improvement of phototoaging.
Accuracy of torque controllers on healing abutments in implant dentistry—an in vitro study
Authors: Goyal Varun , Delgado‐Ruiz Rafael , Kurtz Kenneth S. , Romanos Georgios E.
Published: August 2023

Purpose

To evaluate the accuracy of three torque‐control devices used on healing abutments in implant prosthodontics.

Materials and Methods

In this in vitro experiment, three torque controllers, Ankylos friction‐based, Straumann spring‐based, and Anthogyr pre‐calibrated torque control devices were used to tighten the healing abutments of Ankylos and Straumann dental implants to a pre‐determined value of 15 Ncm. A digital torque meter (DT), Cedar DID‐4A, was used to assess removal torque as a surrogate for the accuracy of the torque controllers to apply a tightening force of 15 Ncm on healing abutments.  One‐way analysis of variance (ANOVA) was used to identify differences between each torque controller and the digital torquemeter, a p < 0.05 was considered significant.

Results

The torque required to remove the healing abutments was 16.05 ± 0.66 Ncm for the Ankylos ratchet, 12.61 ± 0.90 Ncm for the Straumann ratchet, and 14.37 ± 1.08 Ncm for the Anthogyr torque‐control device. Significant statistical differences were observed between Ankylos and the control digital torquemeter (p = 1.84E−8; F = 50.3); Anthogyr and control digital torquemeter (p = 0.01; F = 6.79); and Straumann and control digital torquemeter (p = 0.01; F = 141.15).

Conclusion

Friction‐based (Ankylos), spring‐based (Straumann), and pre‐calibrated (Anthogyr) torque control devices present over‐torque and under‐torque values when used over healing abutments of Ankylos and Straumann implant systems.
Taraxacum sectum sp. nov., a new member of T. sect. Erythrosperma (Asteraceae) from the Baltic island Öland, Sweden
Authors: Wendt Göran , Øllgaard Hans
Published: August 2023

Taraxacum sectum is described as a new member of T. sect. Erythrosperma from the Baltic island of Öland, Sweden, where it has been found at ca 15 sites in alvar habitats as well as in somewhat disturbed dry and sandy grasslands. The new species is similar to T. saphycraspedum, T. marginatum and T. taeniatum but differs from all of these by a combination of characters: pale greenish yellow styles, ± vertically erect leaves, strongly recurved outer phyllaries, hairy scapes and conspicuously light grayish hairy basal parts.
The experience of eating for older nursing home residents with dysphagia: A qualitative descriptive study
Authors: Hsu Wen‐Ching , Tsai Hsiu‐Hsin , Weng Li‐Cheng , Wang Yi‐Wen
Published: August 2023

Background

Understanding the experience of eating for nursing home residents with dysphagia is essential for developing effective and holistic compensatory intervention programmes for older adults with dysphagia. However, there is a lack of studies on the experience of eating for older adults with dysphagia, especially for individuals in Asian cultures.

Objectives

This study aimed to understand the experience of eating for older nursing home residents who have difficulty swallowing (dysphagia), which is often a problem for this population.

Methods

This qualitative descriptive study recruited older nursing home residents with dysphagia from facilities in central Taiwan. Residents were recruited by purposive sampling. Data were collected through individual in‐depth semistructured face‐to‐face interviews. Interviews were audio‐recorded and transcribed interview data were analysed with content analysis.

Results

A total of 20 residents participated in the study; the mean age was 78.7 years (standard deviation = 8.54 years); male and female residents were equally represented. The main core theme describing the experience of eating for nursing home residents with dysphagia was irregular coughing, which often occurred spontaneously. Three subthemes described how residents responded: making adjustments to eating and swallowing, receiving assistance from NH staff and fear of eating.

Conclusions

Our findings can serve as an evidence‐based reference for clinical care aimed at nursing home residents with dysphagia. Support programmes that provide safe swallowing skills and emotional support for managing dysphagia are recommended.

Implications for practice

Nursing home residents with dysphagia should receive interventions focussed on self‐supporting care, training in swallowing skills and emotional support.