bluuebunny/crossref_metadata_2025 huggingface dataset:
Toward Access Justice in the Academy: Centring Episodic Disability to Revision Research Methodologies
Authors: Croft Lacey , Harrison Elisabeth , Grant-Young Josh , McGillivray Kelly , Sebring Jennifer C. H. , Rice Carla
Published: January 2024

This article explores questions of disability access justice within the academy through the lens of an online digital/multimedia storytelling (DS) research workshop conducted during the COVID-19 pandemic. Our investigation uncovers how the shift from in-person to online DS methodologies created newfound opportunities for participation, particularly for individuals with episodic disabilities (EDs). Through an analysis of three co-author participants’ multimedia/digital stories and their reflective insights, we investigate the interplay between research methodologies and the broader context of disability access within the academy. Participants’ stories of inventive adaptations unfold against a backdrop of experiences in traditional academic settings that privilege normative ways of working and seldom accommodate diverse rhythms and access needs, including of students, faculty and staff. In light of these narratives, we advocate for an ongoing commitment to access-centered practices in research and work beyond crisis situations. The article concludes that academic research enterprises can accommodate a wider spectrum of participants—particularly those with episodic disabilities—and enhance research outcomes by recognizing and anticipating diverse bodyminds within the design of research methodologies and techniques.
Long term outcomes in the critically ill: Investigating the association between frailty and survival. LONG a SWARM regional observational study
Authors: Garland Adam , Womersley James , Clark Thomas
Published: January 2024

Background:

Frailty is common and is present in up to 30% of patients admitted to the ICU. We do not yet understand the true impact of ICU admission on this population, certainly in terms of longer-term outcomes.

Method:

Random selection of patients admitted to each general ICU within the SWARM network between October 1st 2012 and January 31st 2013. To be eligible, patients had to be over the age of 18 and admitted to the ICU with either a medical diagnosis or following emergency gastrointestinal/vascular surgery.

Results:

1888 patients were eligible for random selection. 560 patients were selected for the final analysis. Three hundred and eighteen patients had an estimated frailty score between 1 and 3 (classified as not frail, 56.8%), whilst 112 had a score of 4 (classified as vulnerable, 20%) and 130 patients had a score of between 5 and 7 (classified as frail, 23.2%). The median survival for patients with an estimated frailty score of four (vulnerable) was lower than those with an estimated score of 5 or greater (331.5 vs 594 days, p < 0.0001). One-year survival was 79% (75, 84), whilst 5-year survival was 69% (65, 75). Hazard ratios for ‘vulnerable’ and ‘frail’ sub-groups were estimated as 2.6 and 2.4 respectively.

Conclusion:

There is significant evidence to suggest a strong causal association between frailty and survival after ICU admission. ‘Vulnerable’ status may well be associated with similar effect on long-term survival to that of ‘frailty’.
Special issue on innovative approaches in the implementation of technology in sports science and engineering
Authors: Oviedo-Caro Miguel Ángel , Sanchez-Trigo Horacio
Published: January 2024

The technological advances of recent decades are having a great impact on society, modifying traditional ways of life by transforming social relationships, transactions, and jobs, among others. The physical activity and sport sectors are not immune to the technological transformation of society, and its adaptation is also taking place, resulting in great technological innovations. The implementation of technology in sports is playing an ever-increasing role by advances in modeling and materials, the design of sports equipment, the monitoring and control of physical and physiological performance, and the improvement of the daily performance of athletes, coaches, and referees.
Visualizing urban slum population across the globe
Authors: Ren Feiran , Zhang Yuheng , Zhou Qi
Published: January 2024

Monitoring the percentage of urban residents dwelling in slums (referred to as the urban slum population percentage) is crucial for enhancing living conditions in cities. Despite its importance, there is a notable lack of research that maps this indicator on a global scale. Addressing this gap, our study aims to graphically represent the urban slum population percentage alongside the absolute numbers of the urban slum population worldwide. This analysis was conducted by synthesizing statistical data from a variety of sources. Our findings indicate that countries with a high urban slum population percentage are predominantly situated in Africa, with India having the most substantial absolute slum-dwelling population. Our research offers a comprehensive global viewpoint, underscoring the imperative to improve urban living conditions.
Impact of Digital Marketing on Micro, Small, and Medium Enterprises (MSMEs)
Authors: Sareen Pankaj , Rani Pallvi
Published: January 2024

The micro, small, and medium enterprises (MSMEs) in India produce a varied collection of goods. The rapid changes in technology and consumer preferences have a huge impact on marketing techniques. In contrast to large industries, MSMEs do not have any proper tools for marketing so it is one of the weakest areas wherein small organizations face problems. This article explores the digital marketing prospects of MSMEs. These industries are major sources of employment, growth, and economy so digital marketing of SMEs is important. The various benefits of digital marketing with different tools available are highlighted in this article. This article will also help small industries in creating effecting websites so that they can take advantage of digital marketing. Web analytic tools for analysing website traffic are also discussed in this article so that companies can monitor the effect of digital marketing.
Evaluating histone modification analysis of individual preimplantation embryos
Authors: Zeng Yiren , Hoshino Yoichiro , Susami Kazuki , Honda Shinnosuke , Minami Naojiro , Ikeda Shuntaro
Published: January 2024

Background

We previously reported a modification of the CUT&Tag method (NTU-CAT) that allows genome-wide histone modification analysis in individual preimplantation embryos. In the present study, NTU-CAT was further simplified by taking advantage of the Well-of-the-Well (WOW) system, which enables the processing of multiple embryos in a shorter time with less reagent and cell loss during the procedure (WOW-CUT&Tag, WOW-CAT).

Results

WOW-CAT allowed histone modification profiling from not only a single blastocyst but also from a portion of it. WOW-CAT generated similar H3K4me3 profiles as NTU-CAT, but they were closer to the profiles produced by chromatin immunoprecipitation-sequencing, such as a valley-like trend and relatively lower false positive rates, indicating that WOW-CAT may attenuate the bias of Tn5 transposase to cut open chromatin regions. Simultaneous WOW-CAT of two halves of single blastocysts was conducted to analyze two different histone modifications (H3K4me3 and H3K27ac) within the same embryo. Furthermore, trophectoderm cells were biopsied and subjected to WOW-CAT in anticipation of preimplantation diagnosis of histone modifications. WOW-CAT allowed the monitoring of epigenetic modifications in the main body of the embryo. For example, analysis of H3K4me3 modifications of XIST and DDX3Y in trophectoderm biopsies could be used to sex embryos in combination with quantitative PCR, but without the need for deep sequencing.

Conclusions

These results suggest the applicability of WOW-CAT for flexible epigenetic analysis of individual embryos in preimplantation epigenetic diagnosis.
Impact of intraoperative fluid management on postoperative complications in patients undergoing minimally invasive esophagectomy for esophageal cancer: a retrospective single-center study
Authors: Takahashi Misaki , Toyama Hiroaki , Takahashi Kazuhiro , Kaiho Yu , Ejima Yutaka , Yamauchi Masanori
Published: January 2024

Background

Esophagectomy is a high-risk procedure that can involve serious postoperative complications. There has been an increase in the number of minimally invasive esophagectomies (MIEs) being performed. However, the relationship between intraoperative management and postoperative complications in MIE remains unclear.

Methods

After the institutional review board approval, we enrolled 300 patients who underwent MIE at Tohoku University Hospital between April 2016 and March 2021. The relationships among patient characteristics, intraoperative and perioperative factors, and postoperative complications were retrospectively analyzed. The primary outcome was the relationship between intraoperative fluid volume and anastomotic leakage, and the secondary outcomes included the associations between other perioperative factors and postoperative complications.

Results

Among 300 patients, 28 were excluded because of missing data; accordingly, 272 patients were included in the final analysis. The median [interquartile range] operative duration was 599 [545–682] minutes; total intraoperative infusion volume was 3,747 [3,038–4,399] mL; total infusion volume per body weight per hour was 5.48 [4.42–6.73] mL/kg/h; and fluid balance was + 2,648 [2,015–3,263] mL. The postoperative complications included anastomotic leakage in 68 (25%) patients, recurrent nerve palsy in 91 (33%) patients, pneumonia in 62 (23%) patients, cardiac arrhythmia in 13 (5%) patients, acute kidney injury in 5 (2%) patients, and heart failure in 5 (2%) patients. The Cochrane-Armitage trend test indicated significantly increased anastomotic leakage among patients with a relatively high total infusion volume (P = 0.0085). Moreover, anastomotic leakage was associated with male sex but not with peak serum lactate levels. Patients with a longer anesthesia duration or recurrent nerve palsy had a significantly higher incidence of postoperative pneumonia than those without. Further, the incidence of postoperative pneumonia was not associated with the operative duration, total infusion volume, or fluid balance. The operative duration and blood loss were related to the total infusion volume. Acute kidney injury was not associated with the total infusion volume or serum lactate levels.

Conclusions

Among patients who underwent MIE, the total infusion volume was positively correlated with the incidence of anastomotic leakage. Further, postoperative pneumonia was associated with recurrent nerve palsy but not total infusion volume or fluid balance.
Multisite spontaneous hematomas and bleeding in critically ill Chinese patients with COVID-19: two case reports and a literature review
Authors: Sha Sha , Qi Sun , Qindong Shi
Published: January 2024

Background

Anticoagulation is recommended as a standardized therapy for COVID-19 patients according to the WHO guidelines. However, bleeding events have also been reported. Hemorrhage or hematoma was observed in sites including the retroperitoneum, brain, alimentary tract, muscles, and soft tissues. Reduction or suspension of anticoagulants is a common intervention. Transfusion, endoscopic hemostasis, and vascular interventional therapy have been used to improve the condition.

Case presentation

In this article, we present two cases of concurrent multisite hematomas and bleeding at other sites in patients with SARS-CoV-2 infection. Both patients were treated with heparins and experienced bleeding after the anticoagulation therapy. Both patients were older with more than two comorbidities, and critical COVID-19. Laboratory tests revealed a considerable decrease in hemoglobin levels and alterations in the coagulation system. In the first patient, the main intervention was embolization using angiography. However, we only adjusted the anticoagulation strategy in the second case. The first patient recovered and was discharged; however, the second died of other causes. This study provides a retrospective review of typical hemorrhagic cases during anticoagulation in COVID-19 patients over the course of four years. A relatively comprehensive search was performed in Pubmed by constructing MeSH subject terms on limiting the search period and specific contents. It summarizes and synthesizes the research related to heparins and other novel anticoagulants in the context of COVID-19 from the onset of the pandemic to the present disseminated phase. This study aimed to offer valuable insights and reference points for developing anticoagulation treatment strategies for patients with COVID-19.

Conclusions

Anticoagulation is a crucial treatment option for patients with COVID-19. The difference in anticoagulant effects is related to the severity of COVID-19. Nafamostat can reduce thrombosis in the extracorporeal circuits in critically ill patients with COVID-19. The efficacy and safety of novel anticoagulants require further clinical data. Routine bedside assessments and real-time laboratory monitoring are essential for early identification of bleeding events during anticoagulant therapy and administering intervention.
Efficacy of treatment patterns based on concurrent chemoradiotherapy in patients with stage IIB cervical squamous cell carcinoma
Authors: Pan Xin-Bin , Lu Yan , Wei You-Sheng , Yao De-Sheng
Published: January 2024

Purpose

To assess survival of treatment patterns based on concurrent chemoradiotherapy (CCRT) in patients with stage IIB cervical squamous cell carcinoma (CSCC).

Materials and methods

Patients with stage IIB CSCC receiving CCRT were investigated from June 2012 to June 2019 in Guangxi Medical University Cancer Hospital. Baseline characteristics and treatment patterns were described. Survival between treatment patterns were compared using Kaplan-Meier methods.

Results

A total of 232 patients were included: 39.7% of patients received CCRT alone, 6.5% of patients received neoadjuvant chemotherapy (NACT) + CCRT, 45.6% of patients received CCRT + adjuvant chemotherapy (AC), and 8.2% of patients received NACT + CCRT + AC. CCRT + AC showed similar overall survival (OS; hazard ratio [HR] = 0.95, 95% confidence interval [CI]: 0.41–2.17; P = 0.894) and locoregional-free survival (LRFS; HR = 2.39, 95% CI: 0.45–12.63; P = 0.303) compared with CCRT. However, CCRT + AC had a worse distant metastasis-free survival (DMFS; HR = 5.39, 95% CI: 1.14–25.57; P = 0.034). After propensity score matching, CCRT + AC had comparable OS (HR = 0.89, 95% CI: 0.29–2.70; P = 0.833), LRFS (HR = 3.26, 95% CI: 0.30-35.38; P = 0.331), and DMFS (HR = 4.80, 95% CI: 0.55–42.26; P = 0.157) compared to CCRT.

Conclusion

AC did not improve survival in patients with stage IIB CSCC receiving CCRT.
Paclitaxel liposome (Lipusu) based chemotherapy combined with immunotherapy for advanced non-small cell lung cancer: a multicenter, retrospective real-world study
Authors: Li Ran , Liang Hongge , Li Jun , Shao Zhenyu , Yang Donghong , Bao Jing , Wang Keqiang , Xi Wen , Gao Zhancheng , Guo Renhua , Mu Xinlin
Published: January 2024

Background

Paclitaxel liposome (Lipusu) is known to be effective in non-small cell lung cancer (NSCLC) as first-line treatment. This study aimed to evaluate the effectiveness and safety of paclitaxel liposome based chemotherapy plus PD-1/PD-L1 inhibitor in patients with advanced NSCLC.

Methods

In this multicenter, retrospective, real-world study, patients with advanced NSCLC who were administered paclitaxel liposome based chemotherapy plus PD-1/PD-L1 inhibitor in three centers (Peking University People’s Hospital as the lead center) in China between 2016 and 2022 were included. Progression-free survival (PFS), overall survival (OS), objective response rate, disease control rate, and adverse events (AEs) were evaluated.

Results

A total of 49 patients were included, with 33 (67.3%) receiving paclitaxel liposome based chemotherapy plus PD-1/PD-L1 inhibitor as first-line treatment. There were 34 patients (69.4%) diagnosed with squamous cell carcinoma and 15 (30.6%) with adenocarcinoma. The median follow-up was 20.5 (range: 3.1–41.1) months. The median PFS and OS of all patients were 9.7 months (95% confidence interval [CI], 7.0-12.4) and 30.5 months (95% CI, not evaluable-not evaluable), respectively. Patients with squamous cell carcinoma and adenocarcinoma had median PFS of 11 months (95%CI, 6.5–15.5) and 9.3 months (95%CI, 7.0-12.4), respectively. The median PFS was 9.9 months (95%CI, 7.1–12.7) in patients who received the combined regimen as first-line treatment. Treatment-related AEs of any grade were observed in 25 (51.0%) patients, and AEs of grade 3 or worse were observed in nine patients (18.4%). The most common treatment-related AEs were myelosuppression (14.3%) and fever (10.2%).

Conclusions

Paclitaxel liposome based chemotherapy plus PD-1/PD-L1 inhibitor prolonged the PFS in advanced NSCLC with acceptable safety, which was worthy of clinical application.
Digital protractor as an intraoperative guide to cup anteversion in total hip arthroplasty
Authors: Yi Ning , Yuan Hang , Xu Nanwei , Liu Ruiping , Wang Yuji , Zhuang Chao
Published: January 2024

Introduction

To explore if digital protractor could guide the anteversion of acetabular cup during primary THA and make it consistent with preoperative.

Methods

We retrospectively reviewed 172 cases of primary THA with direct anterior approach (DAA) over 2 years. The anteversion of acetabular cup were measured from computed tomography (CT) scan preoperative and de-identified plain radiographs postoperative by two blinded investigators who were not involved in the surgery. The effect of the digital protractor on the anteversion was determined using regression analysis.

Results

The mean anteversion for the THAs in digital protractor group was 15.5°and 21.4°in control group (P < 0.01). The mean anteversion bias for the THAs in digital protractor group was 1.59° and 6.63° in control group (P < 0.01).Regression analysis identified a 10.7% difference in anteversion due to the use of digital protractor (P < 0.01), and THAs performed without digital protractor were six times more likely to result in anteversion of > 25°. The correlation coefficient for the interobserver reliability of the measurement of the two investigators was 0.94.

Conclusion

The digital protractor is a practical tool in the DAA for THA to determine the anteversion of the acetabular prosthesis.
Time-Controlled Adaptive Ventilation (TCAV): a personalized strategy for lung protection
Authors: Al-Khalisy Hassan , Nieman Gary F. , Kollisch-Singule Michaela , Andrews Penny , Camporota Luigi , Shiber Joseph , Manougian Toni , Satalin Joshua , Blair Sarah , Ghosh Auyon , Herrmann Jacob , Kaczka David W. , Gaver Donald P. , Bates Jason H. T. , Habashi Nader M.
Published: January 2024

Acute respiratory distress syndrome (ARDS) alters the dynamics of lung inflation during mechanical ventilation. Repetitive alveolar collapse and expansion (RACE) predisposes the lung to ventilator-induced lung injury (VILI). Two broad approaches are currently used to minimize VILI: (1) low tidal volume (LVT) with low-moderate positive end-expiratory pressure (PEEP); and (2) open lung approach (OLA). The LVT approach attempts to protect already open lung tissue from overdistension, while simultaneously resting collapsed tissue by excluding it from the cycle of mechanical ventilation. By contrast, the OLA attempts to reinflate potentially recruitable lung, usually over a period of seconds to minutes using higher PEEP used to prevent progressive loss of end-expiratory lung volume (EELV) and RACE. However, even with these protective strategies, clinical studies have shown that ARDS-related mortality remains unacceptably high with a scarcity of effective interventions over the last two decades. One of the main limitations these varied interventions demonstrate to benefit is the observed clinical and pathologic heterogeneity in ARDS. We have developed an alternative ventilation strategy known as the Time Controlled Adaptive Ventilation (TCAV) method of applying the Airway Pressure Release Ventilation (APRV) mode, which takes advantage of the heterogeneous time- and pressure-dependent collapse and reopening of lung units. The TCAV method is a closed-loop system where the expiratory duration personalizes VT and EELV. Personalization of TCAV is informed and tuned with changes in respiratory system compliance (CRS) measured by the slope of the expiratory flow curve during passive exhalation. Two potentially beneficial features of TCAV are: (i) the expiratory duration is personalized to a given patient’s lung physiology, which promotes alveolar stabilization by halting the progressive collapse of alveoli, thereby minimizing the time for the reopened lung to collapse again in the next expiration, and (ii) an extended inspiratory phase at a fixed inflation pressure after alveolar stabilization gradually reopens a small amount of tissue with each breath. Subsequently, densely collapsed regions are slowly ratcheted open over a period of hours, or even days. Thus, TCAV has the potential to minimize VILI, reducing ARDS-related morbidity and mortality.

Graphical Abstract
Increased regulatory activity of intestinal innate lymphoid cells type 3 (ILC3) prevents experimental autoimmune encephalomyelitis severity
Authors: Lazarević Milica , Stegnjaić Goran , Jevtić Bojan , Despotović Sanja , Ignjatović Đurđica , Stanisavljević Suzana , Nikolovski Neda , Momčilović Miljana , Fraser Graeme L. , Dimitrijević Mirjana , Miljković Đorđe
Published: January 2024

Experimental autoimmune encephalomyelitis (EAE) induced in inbred rodents, i.e., genetically identical animals kept under identical environmental conditions, shows variable clinical outcomes. We investigated such variations of EAE in Dark Agouti rats immunized with spinal cord homogenate and identified four groups: lethal, severe, moderate, and mild, at day 28 post immunization. Higher numbers of CD4+ T cells, helper T cells type 1 (Th1) and 17 (Th17) in particular, were detected in the spinal cord of the severe group in comparison with the moderate group. In addition, increased proportion of Th1 and Th17 cells, and heightened levels of interferon (IFN)-γ and interleukin (IL)-6 were detected in the small intestine lamina propria of the severe group. A selective agonist of free fatty acid receptor type 2 (Ffar2) applied orally in the inductive phase of EAE shifted the distribution of the disease outcomes towards milder forms. This effect was paralleled with potentiation of intestinal innate lymphoid cells type 3 (ILC3) regulatory properties, and diminished Th1 and Th17 cell response in the lymph nodes draining the site of immunization. Our results suggest that different clinical outcomes in DA rats are under determinative influence of intestinal ILC3 activity during the inductive phase of EAE.
Repeated cold stress, an animal model for fibromyalgia, elicits proprioceptor-induced chronic pain with microglial activation in mice
Authors: Wakatsuki Koji , Kiryu-Seo Sumiko , Yasui Masaya , Yokota Hiroki , Kida Haruku , Konishi Hiroyuki , Kiyama Hiroshi
Published: January 2024

Background

Fibromyalgia is characterized by chronic pain, fatigue, and other somatic symptoms. We have recently revealed that proprioceptor hyperactivation induces chronic pain in a rat model of myalgic encephalomyelitis. The present study explores whether similar proprioceptor-induced pain is elicited in a mouse model of fibromyalgia.

Methods

Repeated cold stress (RCS) was used as a fibromyalgia model. Pain behavior was examined using the von Frey test, and neuronal activation was examined immunohistochemically as activating transcription factor (ATF)3 expression. The Atf3:BAC transgenic mouse, in which mitochondria in hyperactivated neurons are specifically labeled by green fluorescent protein, was used to trace the activated neuronal circuit. PLX3397 (pexidartinib) was used for microglial suppression.

Results

RCS elicited long-lasting pain in mice. ATF3, a marker of cellular hyperactivity and injury, was expressed in the lumbar dorsal root ganglion (DRG) 2 days after RCS initiation; the majority of ATF3-expressing DRG neurons were tropomyosin receptor kinase C- and/or vesicular glutamate transporter 1-positive proprioceptors. Microglial activation and increased numbers of microglia were observed in the medial part of the nucleus proprius 5 days after RCS initiation, and in the dorsal region of the ventral horn 7 days after RCS. In the ventral horn, only a subset of motor neurons was positive for ATF3; these neurons were surrounded by activated microglia. A retrograde tracer study revealed that ATF3-positive motor neurons projected to the intrinsic muscles of the foot (IMF). Using Atf3:BAC transgenic mice, we traced hyperactivated neuronal circuits along the reflex arc. Green fluorescent protein labeling was observed in proprioceptive DRG neurons and their processes originating from the IMF, as well as in motor neurons projecting to the IMF. Microglial activation was observed along this reflex arc, and PLX3397-induced microglial ablation significantly suppressed pain behavior.

Conclusion

Proprioceptor hyperactivation leads to local microglial activation along the reflex arc; this prolonged microglial activation may be responsible for chronic pain in the present model. Proprioceptor-induced microglial activation might be the common cause of chronic pain in both the fibromyalgia and myalgic encephalomyelitis models, although the experimental models are different.
Efficacy of direct-to-operating room trauma resuscitation: a systematic review
Authors: Seo Dongmin , Heo Inhae , Choi Donghwan , Jung Kyoungwon , Jung Hohyung
Published: January 2024

Background

Hemorrhage control is a time-critical task, and recent studies have demonstrated that a shorter time to definitive care is positively associated with patient survival and functional outcomes. The concept of direct transport to the operating room was proposed in the 1960s to reduce treatment time. Some trauma centers have developed protocols for direct-to-operating room resuscitation (DOR) programs. Moreover, few studies have reported the clinical outcomes of DOR in patients with trauma; however, their clinical effect in improving the efficiency and quality of care remains unclear. In this systematic review, we aimed to consolidate all published studies reporting the effect of DOR on severe trauma and evaluate its utility.

Methods

The PubMed, EMBASE, and Cochrane databases were searched from inception to April 2023, to identify all articles published in English that reported the effect of direct-to-operating room trauma resuscitation for severe trauma. The articles were reviewed as references of interest.

Results

We reviewed six studies reporting the clinical effect of operating room trauma resuscitation. A total of 3232 patients were identified. Five studies compared the actual mortality with the predicted mortality using the trauma score and injury severity score, while one study compared mortality using propensity matching. Four studies reported that the actual survival rate for overall injuries was better than the predicted survival rate, whereas two studies reported no difference. Some studies performed subgroup analyses. Two studies showed that the survival rate for penetrating injuries was better than the predicted survival rate, and one showed that the survival rate for blunt injuries was better than the predicted survival rate. Five studies reported the time to surgical intervention, which was within 30 min. Two studies time-compared surgical intervention, which was shorter in patients who underwent DOR.

Conclusion

Implementing DOR is likely to have a beneficial effect on mortality and can facilitate rapid intervention in patients with severe shock. Future studies, possibly clinical trials, are needed to ensure a proper comparison of the efficiency.
A review and recommendations for oral chaperone therapy in adult patients with Fabry disease
Authors: Nowicki Michał , Bazan-Socha Stanisława , Błażejewska-Hyżorek Beata , Kłopotowski Mariusz M. , Komar Monika , Kusztal Mariusz A. , Liberek Tomasz , Małyszko Jolanta , Mizia-Stec Katarzyna , Oko-Sarnowska Zofia , Pawlaczyk Krzysztof , Podolec Piotr , Sławek Jarosław ,   on behalf of the Polish Fabry Disease Collaborative Group
Published: January 2024

Fabry disease (FD) is a rare, X-linked lysosomal storage disorder affecting both males and females caused by genetic abnormalities in the gene encoding the enzyme α-galactosidase A. FD-affected patients represent a highly variable clinical course with first symptoms already appearing in young age. The disease causes a progressive multiple organ dysfunction affecting mostly the heart, kidneys and nervous system, eventually leading to premature death. Disease-specific management of FD includes enzyme replacement therapy with agalsidase α and β or pharmacological oral chaperone migalastat. Migalastat is a low-molecular-mass iminosugar, that reversibly binds to active site of amenable enzyme variants, stabilizing their molecular structure and improving trafficking to the lysosome. Migalastat was approved in the EU in 2016 and is an effective therapy in the estimated 35–50% of all patients with FD with amenableGLAgene variants. This position statement is the first comprehensive review in Central and Eastern Europe of the current role of migalastat in the treatment of FD. The statement provides an overview of the pharmacology of migalastat and summarizes the current evidence from the clinical trial program regarding the safety and efficacy of the drug and its effects on organs typically involved in FD. The position paper also includes a practical guide for clinicians on the optimal selection of patients with FD who will benefit from migalastat treatment, recommendations on the optimal selection of diagnostic tests and the use of tools to identify patients with amenableGLAmutations. Areas for future migalastat clinical research have also been identified.
Spatial multi-omics: novel tools to study the complexity of cardiovascular diseases
Authors: Kiessling Paul , Kuppe Christoph
Published: January 2024

Spatial multi-omic studies have emerged as a promising approach to comprehensively analyze cells in tissues, enabling the joint analysis of multiple data modalities like transcriptome, epigenome, proteome, and metabolome in parallel or even the same tissue section. This review focuses on the recent advancements in spatial multi-omics technologies, including novel data modalities and computational approaches. We discuss the advancements in low-resolution and high-resolution spatial multi-omics methods which can resolve up to 10,000 of individual molecules at subcellular level. By applying and integrating these techniques, researchers have recently gained valuable insights into the molecular circuits and mechanisms which govern cell biology along the cardiovascular disease spectrum. We provide an overview of current data analysis approaches, with a focus on data integration of multi-omic datasets, highlighting strengths and weaknesses of various computational pipelines. These tools play a crucial role in analyzing and interpreting spatial multi-omics datasets, facilitating the discovery of new findings, and enhancing translational cardiovascular research. Despite nontrivial challenges, such as the need for standardization of experimental setups, data analysis, and improved computational tools, the application of spatial multi-omics holds tremendous potential in revolutionizing our understanding of human disease processes and the identification of novel biomarkers and therapeutic targets. Exciting opportunities lie ahead for the spatial multi-omics field and will likely contribute to the advancement of personalized medicine for cardiovascular diseases.
Crowdfunding innovative but risky new ventures: the importance of less ambiguous tone
Authors: Liu Ye , Zhang Ke , Xue Weili , Zhou Ziyu
Published: January 2024

Crowdfunding provides a novel and potential way for innovative but risky new ventures to fund their new product development (NPD) projects. To help potential investors evaluate the projects and enhance the credibility of disclosure, founders are struggling with how to phrase the project description. The rapidly growing cleantech crowdfunding projects provide an ideal context to study this issue. We collected information on cleantech crowdfunding projects and matched non-cleantech crowdfunding projects from Kickstarter. The sample period extends from January 2013 to October 2018. Using signaling research as a theoretical lens and a dictionary-based, computerized text mining method, we found that founders of high-quality cleantech crowdfunding projects could create a reliable signal of quality by providing a project description with a less ambiguous tone and thus boost the success of crowdfunding. Moreover, the signaling effectiveness of a less ambiguous tone is more pronounced in cleantech crowdfunding than in matched non-cleantech crowdfunding, suggesting that the marginal benefit of using a less ambiguous tone is larger when the industry information environment is noisier. Further evidence shows that the signaling effectiveness of a less ambiguous tone in cleantech crowdfunding could be strengthened by backers’ endorsements. Our findings imply that tone ambiguity in project descriptions is related to founders’ information-concealing behavior. Potential investors could search ambiguous words in project descriptions and just allocate their limited attention into projects with a low percentage of ambiguous words to avoid information overload. Founders of high-quality projects could boost crowdfunding success by using a less ambiguous tone to describe their projects. The marginal effect is larger when there is greater uncertainty about project prospects.
Impact of flipped classroom on EFL learners’ self- regulated learning and higher-order thinking skills during the Covid19 pandemic
Authors: Samadi Fatemeh , Jafarigohar Manoocher , Saeedi Masood , Ganji Mansoor , Khodabandeh Farzaneh
Published: January 2024

The advent of innovative online tools has ushered in new possibilities for enhancing the quality of language learning. This study delved into the influence of the flipped classroom approach on augmenting the cognitive and meta-cognitive competence of EFL learners. Two distinct participant groups were enlisted for the study's sequential phases, necessitating varying sample sizes to compare the effects of the flipped classroom against traditional instructional modes on the improvement of EFL learners' self-regulated learning strategies and higher-order thinking. Homogeneous groups of language learners were essential for addressing the research inquiries, and participants were selected from two private language institutes in Arak, Iran. Multiple data sources, including a language proficiency test, the Self-Regulation Questionnaire, a flipped classroom attitude questionnaire, and the Higher-Order Thinking Skills Test, were employed to gather the necessary data. The results demonstrated a significant impact of flipped classrooms on learners' utilization of cognitive self-regulated learning strategies, indicating that the flipped classroom approach fosters students' development as self-directed learners. Concerning the influence of the flipped classroom on enhancing EFL learners' higher-order thinking skills, it was deduced that the flipped classroom significantly affected the learners' engagement in evaluating, analyzing, and creating. The implications of these findings underscore the potential of the flipped classroom approach to positively shape EFL learners' self-regulated learning and higher-order thinking skills, advocating for its incorporation into language education practices.
Efficacy of the use of perfluorocarbon as a temporary tamponade agent in severe ocular trauma and/or complex retinopexy: a scoping review
Authors: Pérez Sara Margarita Pérez , Arias Valentina Cárdenas , Ramírez Daniela Jaramillo , Martínez Camilo Eduardo , Tamayo Martínez Nathalie
Published: January 2024

Background

Perfluorocarbon (PFC)possesses unique chemical properties that favor the pigment epithelium’s adhesion and allows the drainage of subretinal fluid through retinal holes present in retinal detachment cases. However, PFC as a temporary tamponade agent has been limited due to its high potential for toxicity.

Main body

We conducted a scoping review regarding the use of PFC in vitreoretinal surgery as a temporary tamponade in subjects with severe ocular trauma or severe retinal detachment who received a therapeutic intervention (vitrectomy via posterior approach with the use of PFC as a temporary tamponade), compared to vitrectomy without the use of PFC as a temporary tamponade. Outcomes of interest were retinal reattachment, visual acuity (VA), postoperative complications and retinal toxicity. The search was performed in Medline, Medline In-Process & Other Non-Indexed Citations, Medline Daily Update, Embase databases. Reference lists from relevant review articles were also included. Two hundred thirty-eight studies were found, with no duplicate entries. In the first selection, 230 articles were eliminated; in the second selection, 6 additional articles were discarded. In total, 8 articles were obtained in this review. Two selected articles corresponded to animal studies and 6 to studies in humans. Regarding study design, 5 were case series, and 1 was a cohort study.

Conclusion

PFC as a short-term tamponade had high rates of reapplication, improved VA, and the most frequent adverse effects were reversible after PFC withdrawal. Nonetheless, the quality of the studies was poor. Studies with more rigorous methodologies are needed to determine visual and structural outcomes and potential risks of PFC use as a temporary tamponade in vitreoretinal surgery.
Autologous internal limiting membrane transplantation achieves anatomic closure and functional improvement in the treatment of large, persistent macular holes
Authors: Camenzind-Zuche Hanna , Janeschitz-Kriegl Lucas , Hasler Pascal W. , Prünte Christian
Published: January 2024

Purpose

To evaluate the clinical outcome of subretinal autologous internal limiting membrane (ILM) transplantation during pars-plana vitrectomy for persistent full-thickness macular hole (FTMH) repair.

Methods

Retrospective, consecutive case series of 13 eyes (13 patients) undergoing small-incision vitrectomy with ILM transplantation and air tamponade for large persistent FTMH after prior unsuccessful vitrectomy with posterior hyaloid detachment and ILM peeling.

Main outcome measurements

For all eyes, high-definition spectral domain optical coherence tomography scans (SD-OCT Spectralis, Heidelberg Engineering GmbH, Germany) of the macula were routinely performed before surgery, 1 and 4 weeks after surgery, and at the final follow-up visit. Additionally, age, gender, axial length, macular hole diameter, biomicroscopic fundus evaluation and best‐corrected visual acuity (BCVA) at baseline, 1 and 4 weeks after surgery, and at the final follow-up visit were analyzed.

Results

Anatomic closure was achieved in all 13 cases (100% success rate). Closure pattern was classified in accordance with to Rossi et al. (Graefe’s Arch Clin Exp Ophthalmol 258(12):2629–2638, 2020). Mean baseline BCVA logMAR was 0.93, mean postoperative BCVA logMAR was 0.66 with a mean postoperative follow-up period of 11.4 months. No re-opening occurred during the observation period.

Conclusions

Placing an autologous ILM-transplant in the subretinal space beneath the margin of the FTMH can support anatomic restauration and functional improvement in large, persistent FTMHs.
Unveiling the potential of Trichoderma harzianum against Heterodera cajani in pigeon pea: impact on cysts, egg and juveniles abundance
Authors: Maurya Amit Kumar , Kumar Harish , John Vinny , Pant Hemlata , Srivastava D. K. , Ahmed Nevin , Baakdah Fadi , Sami Rokayya , Baazeem Alaa , Elhakem Abeer , Alshahrani Mohammad Y. , Qumsani Alaa T. , Qari Sameer H.
Published: January 2024

Background

Pigeon pea is affected by the cyst nematode Heterodera cajani limiting the production. H. cajani can be managed by biological agents such as Trichoderma harzianum. Thus, the determination of this experiment was to estimate native strains of T. harzianum isolated from pigeon pea fields in India on population of cyst, eggs and juveniles of H. cajani. Pots experiment was conducted at the playhouse of Sam Higginbottom University of Science and Technology, Prayagraj (UP), India, during the year of 2018–2020. Earthen pots were filled with sterilized soil @ 10 kg/pot for each replicate of the treatments. Ten days before sowing of the pigeon pea seed, about 5000 spores of T. harzianum were amended in to the soil for proper colonization. Fifteen days after seed germination, 200 cysts/pot were inoculated near the root zone of pigeon pea plant. The pots were irrigated when required. Observations were recorded of cyst population, eggs and second juveniles of H. cajani/500gm of pigeon pea rhizospheric soil at 90 days after sowing.

Results

Among all the isolates of T. harzianum, the treatment (T 7) reduced of cyst population (12), eggs population (234) and juveniles’ population (153) as compared with all other treatments including control at 90 days after sowing of pigeon pea seeds in the year 2018–2019. Similarly, in 2019–2020 reduction in cyst population (11), eggs population (189) and juveniles population (160) was observed at 90 days after sowing in Trichoderma isolates (T 7) as compared to control.

Conclusions

The findings of this study are very relevant since seven isolates of native T. harzianum were promising to suppress the number of cysts in 79.31 and 83.07%, eggs in 22.00 and 38.83% and juveniles’ population in 17.74 and 20.39% of the nematode H. cajani, besides promoting the pigeon pea plant growth.
Management of status epilepticus in pregnancy: a clinician survey
Authors: Swor Dionne , Juneja Pallavi , Constantine Charlotte , Mann Catrin , Rosenow Felix , LaRoche Suzette
Published: January 2024

Background

Status epilepticus in pregnancy (SEP) is rare and life-threatening for both mother and fetus. There are well-established guidelines for the management of women with epilepsy during pregnancy; however, there is little evidence guiding the management of SEP, leading to uncertainty among treating physicians. Therefore, this survey aims to investigate the real-world practices of physicians treating SEP to explore management approaches for improvements in care.

Methods

An anonymous, electronic survey was created and distributed to neurointensivists and neurologists between September and December 2021.

Results

One hundred physicians initiated the survey and 95 completed it in full: 87 (87%, 87/100) identified neurology as their primary specialty, 31 had subspecialty training in neurocritical care, and 48 had subspecialty training in epilepsy and/or clinical neurophysiology. Over half of the survey respondents (67%, 67/100) reported having participated in the management of SEP, with 48.9% (49/98) having done so in the past year. Most survey respondents (73%, 73/100) reported that their management approach to SEP is different than that of non-pregnant patients. Survey respondents were more likely to involve epilepsy consultants when treating SEP (58.5%, 58/99) and the vast majority involved Obstetrics/Maternal Fetal Medicine consultants (90.8%, 89/98). Survey respondents showed a clear preference for levetiracetam (89.7%, 87/97) in the treatment of benzodiazepine refractory status epilepticus followed by lacosamide (61%, 60/98) if an additional second line agent was needed. Valproate and phenobarbital were unlikely to be used. There was less agreement for the management of refractory and super-refractory SEP.

Conclusions

Levetiracetam is the most frequently used anti-seizure medication (ASM) for benzodiazepine-refractory SEP. Survey participants tended to manage SEP differently than in non-pregnant patients including greater involvement of interdisciplinary teams as well as avoidance of ASMs associated with known teratogenicity.
Role of endoscopic ultrasound in gallbladder and biliary system diseases in patients with normal transabdominal ultrasonography
Authors: Hashim Ahmed Morad , Ahmed Ahmed Nabil , Esmail Yasser Mahmoud , Awad Abeer , Elfatah Yasmine Abd
Published: January 2024

Background

The clinical management of patients experiencing recurrent abdominal pain resembling biliary-type, but with negative findings on conventional transabdominal ultrasound (TUS), poses a challenge. In recent years, endoscopic ultrasound (EUS) has emerged as a valuable tool for diagnosing gallbladder diseases. This study aims to assess the role of EUS in the evaluation of gallbladder and biliary system diseases specifically in patients with normal transabdominal ultrasonography results.

Methods

This study was a cross-sectional analytic study that enrolled 150 patients with any complaints related to the gallbladder and biliary system necessitating transabdominal ultrasound and endosonography in the period between February 2021 and December 2021.

Results

The mean age of the studied patients was 46.1 ± 10.3 years; 46.7% were males and 53.3% were females. The main complaint was right abdominal pain in 78.0%; meanwhile, 74.7% had a picture of obstructive jaundice. EUS revealed gallbladder wall thickening in 15 patients (10%), mud and/or small stones inside the gallbladder in 73 patients (48.7%), positive GB mass and/or polyp findings in 30 patients (20%), and biliary lesions such as duct strictures and/or dilatations were detected in 48 patients (32%), while transabdominal ultrasound was normal in all those patients.

Conclusion

Endoscopic ultrasound (EUS) proves to be a valuable diagnostic modality in patients experiencing biliary-type abdominal pain, despite normal transabdominal ultrasonography results. It not only aids in the accurate diagnosis of underlying conditions but also has the potential to impact the management plan for these patients.
Vitamin D profile in autism spectrum disorder children and its relation to the disease severity
Authors: Mansour Amira , Amer Ayman , Sobh Ali , Zaki Maysaa , Abou-Elsaad Tamer
Published: January 2024

Background

The study aimed to investigate whether vitamin D deficiency is a common finding in autism spectrum disorder (ASD) children and whether such deficiency is related to ASD severity and language age or not.

Methods

A cross-sectional observational study was conducted on ASD children aged 2-6 years. The participants were 80 Egyptian children with ASD. All participants were assessed using DSM-V, the Childhood Autism Rating Scale (CARS), language assessment, and assessment of serum vitamin D using ADVIA Centaur Vit D assay.

Results

About 63.8% of ASD children have vitamin D insufficiency, 28.8 % have vitamin D deficiency, and 7.4% have normal serum levels. No correlation was found between serum vitamin D and language age (r = -0.085, P = 0.451), DSM 5 severity levels (r = 0.015,P= 0.894), and CARS scores (r= 0.075, P= 0.511).

Conclusion

ASD children have lower serum vitamin D levels, which may be one of the environmental factors contributing to ASD development in genetically susceptible individuals, and its correction may be helpful as adjuvant therapy for ASD.