bluuebunny/crossref_metadata_2025_split huggingface dataset:
Impact of the COVID-19 pandemic on well-being and quality of life of patients with alpha-1-antitrypsin deficiency
Authors: Werdecker Catherina , Bals Robert 
Published: October 2023

Abstract
Background
Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder characterized by mutations in the SERPINA1 gene, primarily affecting the lungs and liver. The COVID-19 pandemic has raised questions about the susceptibility of individuals with AATD to COVID-19 and whether patients with rare lung disease might experience increased stress-related symptoms and mental health challenges. This study aims to investigate the impact of the COVID-19 pandemic on the quality of life of individuals living with AATD.

Methods
The study enrolled participants from the German registry for individuals with AATD. Questionnaires were sent to the 1250 participants, and a total of 358 patients were included in the analysis. The primary objective was to examine the influence of sociodemographic and disease-related factors on the occurrence of stress-related symptoms. This was accomplished through correlation and regression analyses. We also investigated the role of baseline quality of life (QoL), as measured by the St. George’s Respiratory Questionnaire (SGRQ), as a mediator of this relationship.

Results
Stress-related symptoms were predicted by young age, female gender, psychological disorders, and a history of exacerbations of lung disease, as determined by multiple regression analysis. QoL as measured by the SGRQ mediated the relationship between poor lung function, stress, and a decline in overall well-being.

Conclusion
The presented data demonstrate that the COVID-19 pandemic significantly affects the psychological well-being of patients with rare diseases, leading to increased levels of anxiety and stress. Disease-related factors can exacerbate stress manifestations, especially when compounded by sociodemographic and contextual factors. Thus, our study emphasizes the crucial role of taking these factors into account when managing individuals with AATD in pandemic situations.
Cell-specific NFIA upregulation promotes epileptogenesis by TRPV4-mediated astrocyte reactivity
Authors: Kong Shuo , Chen Tao-xiang , Jia Xiang-lei , Cheng Xue-lei , Zeng Meng-liu , Liang Jing-yi , He Xiao-hua , Yin Jun , Han Song , Liu Wan-hong , Fan Yuan-teng , Zhou Ting , Liu Yu-min , Peng Bi-wen 
Published: October 2023

Abstract
Background
The astrocytes in the central nervous system (CNS) exhibit morphological and functional diversity in brain region-specific pattern. Functional alterations of reactive astrocytes are commonly present in human temporal lobe epilepsy (TLE) cases, meanwhile the neuroinflammation mediated by reactive astrocytes may advance the development of hippocampal epilepsy in animal models. Nuclear factor I-A (NFIA) may regulate astrocyte diversity in the adult brain. However, whether NFIA endows the astrocytes with regional specificity to be involved in epileptogenesis remains elusive.

Methods
Here, we utilize an interference RNA targeting NFIA to explore the characteristics of NFIA expression and its role in astrocyte reactivity in a 4-aminopyridine (4-AP)-induced seizure model in vivo and in vitro. Combined with the employment of a HA-tagged plasmid overexpressing NFIA, we further investigate the precise mechanisms how NIFA facilitates epileptogenesis.

Results
4-AP-induced NFIA upregulation in hippocampal region is astrocyte-specific, and primarily promotes detrimental actions of reactive astrocyte. In line with this phenomenon, both NFIA and vanilloid transient receptor potential 4 (TRPV4) are upregulated in hippocampal astrocytes in human samples from the TLE surgical patients and mouse samples with intraperitoneal 4-AP. NFIA directly regulates mouse astrocytic TRPV4 expression while the quantity and the functional activity of TRPV4 are required for 4-AP-induced astrocyte reactivity and release of proinflammatory cytokines in the charge of NFIA upregulation. NFIA deficiency efficiently inhibits 4-AP-induced TRPV4 upregulation, weakens astrocytic calcium activity and specific astrocyte reactivity, thereby mitigating aberrant neuronal discharges and neuronal damage, and suppressing epileptic seizure.

Conclusions
Our results uncover the critical role of NFIA in astrocyte reactivity and illustrate how epileptogenic brain injury initiates cell-specific signaling pathway to dictate the astrocyte responses.
Gut microbiota and autism spectrum disorders: where do we stand?
Authors: Zyoud Sa’ed H. , Shakhshir Muna , Abushanab Amani S. , Koni Amer , Shahwan Moyad , Jairoun Ammar A. , Abu Taha Adham , Al-Jabi Samah W. 
Published: October 2023

Abstract
Background
Children with autism spectrum disorder (ASD) often have digestive problems and microbial imbalances in their guts, suggesting that these conditions may play a role in the development of the disorder. Scopus-based research on the gut microbiota and ASD was examined in this bibliometric analysis to shed light on the current state of research and identify potential hotspots for future work in this area.

Methods
We searched documents from the Scopus database and reference citation analysis to collect published data on the gut microbiota and ASD from 2003 to 2022. The downloaded document records were exported to VOSviewer v.1.6.19 to examine and visualize the collaboration between countries and determine the research hotspots.

Results
The search yielded 958 articles specifically dedicated to gut microbiota and ASD. The number of publications in this field increased rapidly after 2013, with a peak in 2022. The United States (n = 267; 27.87%) was the most active country, followed by China (n = 171; 17.85%) and Italy (n = 96; 10.02). International collaboration was observed, with the USA playing a central role. University College Cork, Ireland, was the most productive institution (n = 24; 2.51%). The National Natural Science Foundation of China was the most active funding agency (n = 76; 7.93%). Nutrients journal had the highest number of publications (n = 28; 2.92%). The articles related to gut microbiota and ASD were highly cited, with an h-index of 108. The research themes identified focused on the modulation of gut microbiota as a potential therapy for children with ASD and gut-brain axis dysfunction in ASD.

Conclusions
In recent years, the study of gut microbiota and its association with ASD has garnered considerable interest as an emergent field of study. The results of this study substantially enhance our current understanding of the knowledge landscape in this field and illuminate potential avenues for future research. It is essential to emphasize the significance of devoting more resources to the newest and most promising research areas, such as investigating the potential therapeutic benefits of modulating the intestinal microbiota in children with ASD. This research has enormous potential and merits intensified focus and investigation.
Effects of blood-flow restricted exercise versus conventional resistance training in musculoskeletal disorders—a systematic review and meta-analysis
Authors: Jørgensen Stian Langgård , Kierkegaard-Brøchner Signe , Bohn Marie Bagger , Høgsholt Mathias , Aagaard Per , Mechlenburg Inger 
Published: October 2023

Abstract
Objective
To compare the effect of low-load blood flow restricted resistance training (BFR-RT) versus high-load resistance training (HL-RT) on muscle strength, muscle mass, physical function, patient-reported outcomes, and adherence to training in clinical musculoskeletal populations.

Data sources
Web of Science, Cochrane Central, Medline, Embase, SportDiscus was searched on the 30th May 2022.

Review methods
This study was conducted as a systematic review and meta-analysis. Randomized Controlled Trials (RCTs) were included if they (i) included patients, (ii) comprised of a BFR-RT intervention protocol and a group who performed HL-RT (≥ 70%1RM) for at least eight exercise sessions, and (iii) involved at least 1 exercise that targeted the lower limbs. The Cochrane Risk of Bias tool was used to evaluate the risk of bias. The meta-analyses were performed using a random effects model with an adjustment to the confidence interval.

Results
Seven RCTs comprising 303 participants (BFR-RT: n = 151; HL-RT: n = 152) were identified. HL-RT and BFR-RT showed similar gains in dynamic (1-10RM) knee extensor strength and leg press strength, quadriceps cross sectional area, sit-to-stand performance, and patient reported pain and function. There was a moderate effect favoring BFR-RT for increasing maximal isometric knee extensor strength. The grading of certainty in evidence was low-to-very low for all outcome variables.

Conclusion
This systematic review and meta-analysis extends our current knowledge about BFR-RT and HL-RT as equally effective exercise methods for inducing gains in maximal muscle strength in healthy populations, by now also comprising patients suffering from various clinical musculoskeletal conditions. The certainty in the estimates was low-to-very low, prompting the inclusion of future higher-quality trials.

Trial registration
PROSPERO ID (CRD42022337173). Registered June 18th 2022.
Structural Behavior of Construction Joints in a Composite Rigid-Frame Bridge
Authors: Cho Sanghyeon , Choi Jungho , Lee Heeyoung , Chung Wonseok 
Published: October 2023

AbstractComposite rigid-frame bridges with steel girders have excellent structural performance, but behavioral inconsistency appears at the connection between the steel girder and concrete pier. In addition, composite rigid-frame bridges are challenging to construct owing to the need to connect different materials at the pier. Therefore, this study developed and demonstrated a composite rigid-frame bridge with a construction joint and anchors. The structural performance and composite behavior of the proposed construction joint were investigated by evaluating a direct connection between the girder and concrete prior to casting (Joint A), a plain construction joint (Joint B), and a construction joint with anchors (Joint C). Joints B and C exhibited 16.5% and 46.5% higher ultimate capacities, respectively, than did Joint A. Finite element analysis of Joint C was conducted, and its results were verified against the experimental results. Further, parameter analysis was performed to determine the effects of the steel girder strength and anchor diameter. The results indicated that the proposed construction joint with anchors exhibited excellent structural performance and composite behavior.
Exploring the clinical and genetical spectrum of ADPKD in Chile to assess ProPKD score as a risk prediction tool
Authors: Bayyad Esperanza , Plaza Anita , Klenner Jaime , Downey Patricio , Salas Paulina , Maragaño Daniela , Herrera Patricio , Lehmann Paula , Quiroz Lily , Zavala María Jesus , Orostica Karen , Flores Claudio , Ardiles Leopoldo , Maturana Jorge , Krall Paola 
Published: October 2023

Abstract
Background
Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited condition associated primarily with PKD1 and PKD2 genes. However, ADPKD patients in Latin America have had limited access to comprehensive care. The ProPKD score predicts the likelihood of kidney failure before the age of 60. This study aimed to describe the clinical and genetic characteristics of Chilean ADPKD patients and assess the ProPKD score.

Methods
We enrolled 40 ADPKD probands and 122 relatives from different centers. Genetic analysis of PKD1 and PKD2 genes was performed by combining direct and next-generation sequencing. Pathogenicity was determined using bioinformatic tools. ProPKD scores were calculated based on clinical and genetic data.

Results
ADPKD probands were diagnosed at a median age of 35 years. Pathogenic, likely pathogenic, or uncertain significance variants were identified in 38/40 pedigrees, with 89% involving PKD1 and 11% involving PKD2 variants. Among the identified variants, 62% were novel. Patients with PKD1 truncating variants had a more severe disease course, reaching kidney failure by a median age of 48.5 years. ProPKD scores were assessed in 72 individuals, stratifying them into high-, intermediate-, or low-risk categories and the median ages for kidney failure were 45, 49, and 52 years, respectively (log-rank p = 0.001).

Conclusion
This study provides valuable insights into the clinical and genetic profiles of ADPKD patients in Chile. ADPKD poses a significant public health concern, warranting improvements in diagnosis and treatment. The use of the ProPKD score to predict disease progression should be further explored to enhance patient care and management.
Exhibiting Education
Authors: Cavanagh Sheila T. 
Published: October 2023

Abstract
Exhibition research, design, and creation offer students significant experience in a wide range of intellectual and practical pursuits. This essay presents these components as they are found in the Emory University joint undergraduate/graduate course Digging into the Archives and Creating an Exhibition. The students learn how to navigate archives; ways to collaborate successfully with library and museum exhibition teams (and each other); skills in design and presentation; public programming; and strategies for identifying and reaching broad and diverse audiences. This discussion of the course goals, structure, and outcomes details how such undertakings can enhance student learning in both undergraduate and graduate contexts, while building a range of transferrable skills.
Partial Labyrinth Removal Restricted to the Superior Semicircular Canal in Focal Combined Transpetrosal Approach: Description and Illustrative Cases
Authors: Vidal Claudio H. F. , Figueiredo Eberval G. , Hazin Gabriela F. , Hahn Yoav , Leal Mariana C. , Coimbra Caetano J. 
Published: October 2023

BACKGROUND AND OBJECTIVES:
The labyrinthine structures obstruct the surgical view of the deep petroclival region in the transpetrosal approach. Historically, labyrinthectomy and removal of all 3 semicircular canals, with resultant deafness, was used in patients with ipsilateral functional hearing deficits to improve access. The advent and systematization of superior and posterior semicircular canal removal (transcrusal approach) with good rates of hearing preservation has allowed a redefinition of the possibility of partial labyrinthectomy in patients without previous hearing deficits. The present manuscript is intended to describe a technical refinement of partial labyrinthectomy during focal combined petrosectomy, offering a customization of the approach through the selective removal of the superior semicircular canal for specific types of tumors.

METHODS:
The use of the technique is demonstrated through surgical drawings, pictures, and videos. The rationale to indicate this new approach is discussed based on clinical cases.

RESULTS:
Three illustrative clinical cases (petroclival meningiomas) are demonstrated. Functional hearing on the approach side has been preserved in all of them.

CONCLUSION:
The focal combined transpetrosal approach associated with the superior semicircular canal resection has been a promising surgical technique in the treatment of selected petroclival tumors. It has the potential to further decrease the risks of postoperative auditory and vestibular dysfunctions associated with labyrinthectomies.
Post-radiotherapy osteomyelitis of the cervical spine in head and neck cancer patients
Authors: Tsur Nir , Segal Ella , Kurman Noga , Tzelnick Sharon , Wiesel Ory , Wilk Lior , Hamzany Yaniv , Bachar Gideon , Shoffel-Havakuk Hagit 
Published: October 2023

Objective To evaluate patient characteristics, risk factors, disease course, and management of cervical vertebral osteomyelitis in patients who had radiation for head and neck cancers. Methods A retrospective cohort study (case series) of patients diagnosed with post-radiation osteomyelitis of the cervical spine between 2012 and 2021. Data were collected from the patient’s medical files. Results Seven patients (71% male) with post-radiation cervical osteomyelitis were reviewed. The median patient age was 64 years. The mean interval between diagnosis of osteomyelitis and the first and last radiotherapy course was 8.3 and 4.0 years, respectively. A medical or surgical event preceded the diagnosis in four patients (57%) by a mean of 46.25 days. Common imaging findings were free air within the cervical structures and fluid collection. Four patients recovered from osteomyelitis during the follow-up within an average of 65 days. Conclusion: Post-radiation osteomyelitis is characterized by a subtle presentation, challenging diagnosis, prolonged treatment, and poor outcome. Clinicians should maintain a high index of suspicion for the long-term after radiotherapy. Multidisciplinary evaluation and management are warranted. Advances in knowledge: The study describes post-radiotherapy osteomyelitis of the cervical spine, a rare and devastating complication. Literature data regarding this complication are sparse.
The evolving landscape of health professions education in Pakistan: A critical discourse on CHPE regulation and standardization
Authors: Zaidi Syed Jaffar Abbas , Azim Syeda Rubaba 
Published: October 2023

doi: https://doi.org/10.12669/pjms.40.1.8783
How to cite this: Zaidi SJA, Azim SR. The evolving landscape of health professions education in Pakistan: A critical discourse on CHPE regulation and standardization. Pak J Med Sci. 2024;40(1):---------. doi: https://doi.org/10.12669/pjms.40.1.8783
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Safavid and Portuguese Christian Influence on Saint Stepanos Monastery in Julfa, Iran
Authors: Shayestehfar Mahnaz 
Published: October 2023

The Monastery of Saint Stepanos, in Julfa, is located in northwesternIran, in the province of East Azerbaijan. Whilst the exactdate of construction is unknown, the building displays exceptionalarchitecture. It is believed that the monastery was built between thetenth and seventeenth centuries, and this doubt emphasises the needto investigate the nature of structure and decoration. The Monasteryis situated in a deep canyon beside the Araxes river, on the Iranianside of the border between Iran and the Nakhichevan AutonomousRepublic in Azerbaijan. It was originally built in the ninth century,and was rebuilt during the Safavid era after being damaged by warsand earthquakes. Considering that scholars and travel writers, such asthe French Traveller Taurine, consider the construction of this buildingto be from the Safavid period, probing the structural and decorativesimilarities of this building with Safavid period architecture can bea way of dating this outstanding historical monument. During theSafavid period, the Portuguese arrival on the island of Hormuz inthe Persian Gulf (1515–1622 AD) prepared the ground for the arrivaland activity of European Christians, who spread Christianity and itsculture. One of the earliest consequences of this was the constructionof the Monastery and other Christian architecture. The basic objectivesof the article are to study the influence of Portuguese Christianarchitecture on the construction of Saint Stepanos Monastery, and toinvestigate the influence of Safavid structural design on its architecture.The main questions, are what influence has the architecture of SaintStepanos Monastery had on Christian architecture in Portugal,and what influence did the architecture the Monastery have on thearchitecture of the Safavid period?This research for this article is descriptive and analytical based onhistorical information, available documents and existing images. Anexamination of the architectural similarities between Saint Stepanos
Monastery in Julfa and Christian architecture in Portugal is essentialfor a more accurate understanding of the Monastery.
Diagnosis and treatment of dehydration after stroke: a synthesis of existing evidence
Authors: Miller Colette , McLoughlin Alison SR , Benedetto Valerio , Christian Danielle L , Jones Stephanie P , Smith Eleanor , Watkins Caroline L 
Published: October 2023

Dehydration after stroke is associated with poor health outcomes, increased mortality and poses a significant economic burden to health services, yet research suggests that monitoring and assessment of hydration status is not routinely undertaken. This commentary critically appraises a systematic review which aimed to synthesise the existing evidence regarding diagnosis and treatment of dehydration after stroke. The review discusses common measures of dehydration, describes studies evaluating rehydration treatments, and highlights the link between dehydration and poorer health outcomes in both human and animal studies. The reviewers suggest that future research should focus on determining a single, validated, objective measure to clinically diagnose dehydration in stroke patients. Research designs should include clearly defined patient characteristics, type and severity of stroke, and type and time point of dehydration measurement, to enable comparison between studies. Management of hydration status is a crucial element of acute stroke care which should be routinely practiced. Commentary on Bahouth MN, Gottesman RF, Szanton SL. Primary ‘dehydration’ and acute stroke: a systematic research review. J Neurol. 2018;265(10):2167-2181. 10.1007/s00415-018-8799-6
The Effect of Global Value Chain Participation on Carbon Embodied in Forest Product Exports: A Quasi-Natural Experiment from the Financial Crisis Shock
Authors: Guo Ling , Xiao Liqiang , Wang Wenlan , Yin Jiachen , Huang Wanting 
Published: October 2023

Abstract
Reasonable assessment of the environmental benefits of integrating forest products into global value chains (GVCs) is important to promote sustainable development. Based on the forest product sector data for 41 countries from 2002 to 2014, this paper explores the impact of GVC participation on carbon embodied in exports using the 2008 financial crisis, a quasi-natural experiment of negative global value chain shocks. We found that deepening backward participation in forest product value chains led to more substantial increases in carbon emissions than did forward participation. Countries with large decreases in GVC participation reduced more carbon embodied in forest product exports after the financial crisis (relative to countries with small decreases) through a larger reduction in the scale of forest product exports, and a decrease in the growth rate of capital-intensive products as a result of the relative decline in capital investment. They increased the embodied carbon of exports through a decrease in the growth rate of skilled personnel. Strengthening the technology effect of GVCs with the guidance of skilled forestry personnel is a key way to decrease exported embodied carbon.