bluuebunny/crossref_metadata_2025_split huggingface dataset:
Efficacy confirmation study of aceneuramic acid administration for GNE myopathy in Japan
Authors: Mori-Yoshimura Madoka , Suzuki Naoki , Katsuno Masahisa , Takahashi Masanori P. , Yamashita Satoshi , Oya Yasushi , Hashizume Atsushi , Yamada Shinichiro , Nakamori Masayuki , Izumi Rumiko , Kato Masaaki , Warita Hitoshi , Tateyama Maki , Kuroda Hiroshi , Asada Ryuta , Yamaguchi Takuhiro , Nishino Ichizo , Aoki Masashi 
Published: August 2023

Abstract
Background
A rare muscle disease, GNE myopathy is caused by mutations in the GNE gene involved in sialic acid biosynthesis. Our recent phase II/III study has indicated that oral administration of aceneuramic acid to patients slows disease progression.

Methods
We conducted a phase III, randomized, placebo-controlled, double-blind, parallel-group, multicenter study. Participants were assigned to receive an extended-release formulation of aceneuramic acid (SA-ER) or placebo. Changes in muscle strength and function over 48 weeks were compared between treatment groups using change in the upper extremity composite (UEC) score from baseline to Week 48 as the primary endpoint and the investigator-assessed efficacy rate as the key secondary endpoint. For safety, adverse events, vital signs, body weight, electrocardiogram, and clinical laboratory results were monitored.

Results
A total of 14 patients were enrolled and given SA-ER (n = 10) or placebo (n = 4) tablets orally. Decrease in least square mean (LSM) change in UEC score at Week 48 with SA-ER (− 0.115 kg) was numerically smaller as compared with placebo (− 2.625 kg), with LSM difference (95% confidence interval) of 2.510 (− 1.720 to 6.740) kg. In addition, efficacy was higher with SA-ER as compared with placebo. No clinically significant adverse events or other safety concerns were observed.

Conclusions
The present study reproducibly showed a trend towards slowing of loss of muscle strength and function with orally administered SA-ER, indicating supplementation with sialic acid might be a promising replacement therapy for GNE myopathy.
Trial registration number: ClinicalTrials.gov (NCT04671472).
Can the ultrasound microcystic pattern accurately predict borderline ovarian tumors?
Authors: Liu Danyi , Lyu Guorong , Lai Hongwei , Li Liya , Gan Yaduan , Yang Shuping 
Published: August 2023

Abstract
Objective
To investigate whether the ultrasound microcystic pattern (MCP) can accurately predict borderline ovarian tumors (BOTs).

Methods
A retrospective collection of 393 patients who met the inclusion criteria was used as the study population. Indicators that could well identify BOT in different pathological types of tumors were derived by multivariate unordered logistic regression analysis. Finally, the correlation between ultrasound MCP and pathological features was analyzed.

Results
(1) MCP was present in 55 of 393 ovarian tumors, including 34 BOTs (34/68, 50.0%), 16 malignant tumors (16/88, 18.2%), and 5 benign tumors (5/237, 2.1%). (2) Univariate screening showed significant differences (P < 0.05) in patient age, CA-125 level, ascites, > 10 cyst locules, a solid component, blood flow, and MCP among BOTs, benign ovarian tumors, and malignant ovarian tumors. (3) Multivariate unordered logistic regression analysis showed that the blood flow, > 10 cyst locules, and MCP were significant factors in identifying BOTs (P < 0.05). (4) The pathology of ovarian tumors with MCP showed "bubble"- or "fork"- like loose tissue structures.

Conclusion
MCP can be observed in different pathological types of ovarian tumors and can be used as a novel sonographic marker to differentiate between BOTs, benign tumors and malignant tumors. MCP may arise as a result of anechoic cystic fluid filling the loose tissue gap.
The effects of esketamine and treatment expectation in acute major depressive disorder (Expect): study protocol for a pharmacological fMRI study using a balanced placebo design
Authors: Falkenberg Irina , Bitsch Florian , Liu Wei , Matsingos Alexandros , Noor Laila , Vogelbacher Christoph , Yildiz Cüneyt , Kircher Tilo 
Published: August 2023

Abstract
Background
Major depressive disorder (MDD) is a highly prevalent (8–15%), severely disabling disorder and is associated with enormous socioeconomic impact. Antidepressant medication for the treatment of MDD has proven effective in RCTs; however, placebo response is also substantial. Given the potential benefits of modulating the placebo response in patient care and pharmacological research, understanding the mechanisms underlying placebo response is of high clinical relevance. The placebo response is mediated by treatment expectation, i.e. an individual’s belief about whether and how much they will improve as a consequence of their treatment. The mechanisms and moderators of treatment expectation effects in MDD are poorly understood. Initial brain imaging studies on placebo responses in MDD point towards the relevance of the lateral prefrontal cortex and the rostral anterior cingulate cortex (rACC). In this project, we will investigate the neural mechanisms underlying the antidepressant effects of treatment expectation associated with the fast-acting antidepressant esketamine in patients with MDD. Esketamine is an NMDA receptor antagonist inducing antidepressant effects within hours.

Methods
We will employ a fully balanced placebo design with the factors “treatment” (i.v. esketamine / placebo) and verbally induced “expectation” (high / low) combined with fMRI (resting state, emotion and reward processing paradigms) to investigate the psychological and neural mechanisms underlying the antidepressant effects of expectation, and how these interact with the pharmacological effects of esketamine.

Discussion
The insights gained by this project promise fundamental implications for clinical treatment and future drug trials. Unraveling the mechanisms underlying expectation effects on antidepressant treatment may inform (1) strategies to modulate these effects and thus improve assay sensitivity in RCTs and (2) novel treatment regiments aiming to maximize the synergistic effects of expectation and pharmacological treatment in the clinical care of patients with MDD.

Trial registration
This trial has been prospectively registered with the EU Clinical Trials Register: EudraCT-No.: 2020–000784-23 (November 17, 2020).
Rapid assessment of 3-dimensional intra-tumor heterogeneity through cycling temperature capillary electrophoresis
Authors: Połeć Anna , Ekstrøm Per Olaf , Fougner Christian , Sørlie Therese , Norum Jens Henrik 
Published: August 2023

Abstract
Objective
Tumors are heterogeneous three-dimensional masses populated by numerous cell types, including distinct sub-clones of cancerous cells. Various sub-clones within the same tumor mass may respond differently to cancer treatment, and intra-tumor heterogeneity contributes to acquired therapeutic resistance. Thus, one tissue biopsy will in most cases not be representative of the entire genetic landscape of a tumor mass. In this study, we aimed to establish an easily accessible, low cost method to address intra-tumor heterogeneity in three dimensions, for a limited number of DNA alterations.

Results
This study includes analyses of the three-dimensional (3D) distribution of DNA mutations in human colon cancer and mouse mammary gland tumor tissue samples. We used laser capture microdissection for the unbiased collection of tissue in several XY-planes throughout the tumor masses. Cycling temperature capillary electrophoresis was used to determine mutant allele frequency. High-resolution distribution maps of KRAS and Trp53 mutations were generated for each XY-plane in human and mouse tumor samples, respectively. To provide a holistic interpretation of the mutation distribution, we generated interactive 3D heatmaps giving an easily interpretable understanding of the spatial distribution of the analyzed mutations. The method described herein provides an accessible way of describing intra-tumor heterogeneity for a limited number of mutations.
Comparison of four DNA extraction methods for 16s rRNA microbiota profiling of human faecal samples
Authors: Sinclair James , West Nicholas P , Cox Amanda J 
Published: August 2023

Abstract
Objective
Growth in large population-based studies assessing contributions of the gut microbiota to health and disease requires high-throughput sample processing and analysis methods. This study assessed the impact that modifications to a commercially available magnetic bead based, semi-automated DNA extraction kit had on determination of microbial composition, relative to an established in-house method involving a combination of mechanical and chemical lysis. DNA was extracted from faecal samples from healthy adults (n = 12; 34–69 years), microbial composition was determined by V3-V4 16s rRNA sequencing and compared between extraction methods.

Results
Diversity metrics did not differ between extraction methods. Differences in the relative abundance of key phyla, including a significantly lower abundance of the Firmicutes (p = 0.004) and higher relative abundance of the Bacteroidetes (p = 0.005) and Proteobacteria (p = 0.008) phyla were noted where the DNA extraction did not include additional chemical and mechanical lysis. Principal coordinate analysis of family and genera level data also suggested a potential for sample pre-processing to impact microbial composition. Observations of the potential for skewed microbial composition profiles from samples prepared using a semi-automated DNA extraction kit without additional sample pre-processing highlights a need for consideration of standardisation of methodological approaches to increase the comparability of microbial compositional data.
Clinical impact and misdiagnosis of functional ophthalmological symptoms: a case report
Authors: Pozuelo Moyano Beatriz , Duquenne Catherine , Favrat Bernard , Francois-Xavier Borruat , Kokkinakis Ioannis , Tzartzas Konstantinos 
Published: August 2023

Abstract
Background
There is a high prevalence of somatoform disorders and medically unexplained symptoms. When it comes to deciding whether a patient is able to work, it is essential to differentiate a somatoform disorder from a factitious disorder. The case presented demonstrates the impact on disability benefits and the subsequent psychosocial repercussions of misdiagnosing between a factitious disorder and a somatoform disorder.

Case presentation
A 42-year-old Caucasian woman worked as a 100% fiduciary accountant until the age of 32 when she was placed on medical leave due to persistent trigeminal neuralgia. Afterward, she developed total blindness, not explained by a physiological process, accompanied by distress in a crucial emotional context. We evaluated the patient for a revision of a disability income after a diagnosis of factitious disorder with severe consequences such as disability income suspension and family conflict. Our psychiatric examination concluded the diagnoses of pain disorders related to psychological factors and a dissociative neurological symptom disorder with visual disturbance.

Conclusions
Blindness not explained by a physiological process may accompany trauma and psychological distress. Differentiating this pathology from factitious disorder or simulation is essential from an insurance medicine point of view, but also for its treatment.
Metachronous ureteral metastasis of a gastric adenocarcinoma: a case report and review of literature
Authors: Alla Meriem Ait , Chekrine Tarik , Bouchbika Zineb , Benchakroun Nadia , Jouhadi Hassan , Tawfiq Nezha , Chaouki Houda , Marnissi Farida , Karkouri Mehdi , Sahraoui Souha 
Published: August 2023

Abstract
Background
Ureteral metastasis from gastric cancers are rare and can be a cause of ureteral obstruction. There have been few published case reports in the literature. In this paper, we report an additional case and a review of the literature of all the previous reported cases.

Case presentation
A 67 years old North African women who was treated four years before for a gastric adenocarcinoma, presented with abdominal pain. Imaging and endoscopy showed a mural stenosis of the left ureter, without any other abnormality. Histopathology confirmed the gastric origin of the metastasis. A palliative chemotherapy was foreseen, but due to the deterioration of the general condition of the patient, she received palliative care. We have also reviewed the literature and reported the previously published cases of ureteral metastasis from gastric cancer.

Conclusions
It is worth recalling that in a context of neoplasia and with the presence of signs of ureteral obstruction, it is important to keep in mind the possibility of a ureteral metastasis.
Production of omega-5 gliadin monoclonal antibodies for allergenic evaluation of WDEIA-causing wheat varieties
Authors: Sim Jae-Ryeong , Lee Jong-Yeol , Kim Sewon 
Published: August 2023

AbstractIn allergic individuals, ingestion of wheat can lead to wheat-dependent exercise-induced anaphylaxis (WDEIA). Many studies have been conducted to find WDEIA allergen–deficient wheat, including by generating omega-5 gliadin antibodies. However, the reported antibodies have not been specific enough to detect omega-5 gliadins encoded on the 1B chromosome. In this study, we generated monoclonal antibodies against the major allergens causing WDEIA, omega-5 gliadins. Using these antibodies (mono-O5B-1C10), we assessed accumulation of omega-5 gliadins in wild-type and nullisomic-tetrasomic (NT) lines of the wheat (Triticum aestivum) varieties Chinese Spring (CS) by one- and two-dimensional gel electrophoresis, followed by Coomassie blue staining or immunoblotting with mono-O5B-1C10. We also tested mono-O5B-1C10 for major omega-5 gliadins in various wheat germplasms. Our results thus demonstrate the specificity of mono-O5B-1C10 for major omega-5 gliadins and potentially useful for identifying of omega-5 gliadin–deficient wheat varieties that should not cause WDEIA.
First record of rainbow shrimp, exotic species Mierspenaeopsis sculptilis (Heller, 1862), in the Brazilian coastal amazon, validated by DNA barcode
Authors: Ferreira Charles Samuel Moraes , de Mesquita David Carvalho , de Freitas Lutz Ítalo Antônio , Veneza Ivana Barbosa , Martins Thaís Sousa , Santana Paula da Conceição Praxedes , Miranda Josy Alessandra Barreto , de Sousa Jefferson Miranda , Matos Suane Cristina do Nascimento , Holanda Francisco Carlos Alberto Fonteles , da Cunha Sampaio Maria Iracilda , Evangelista-Gomes Grazielle Fernanda 
Published: August 2023

Abstract
Background
This is the first record of the alien shrimp Mierspenaeopsis sculptilis in Brazil. The invasion was detected within Marine Extractive Reserves based on eight specimens accidentally caught by local fishermen using trawlnets focused on fisheries of native species. These specimens were transported to the Laboratory of Applied Genetics and morphologically identified as Mierspenaeopsis sculptilis (rainbow shrimp). The taxonomic status of analyzed samples was confirmed by DNA barcoding using a 627-bp fragment of the Cytochrome C Oxidase Subunit I (COI) gene.

Results
A single haplotype was recovered from the eight specimens, being identical to a haplotype reported in India, where this species naturally occurs, and in Mozambique, where the rainbow shrimp is considered an invasive species. The present analyses indicated a putative invasive route (i.e., India-Mozambique-Brazil) mediated by shipping trade.

Conclusions
This study presents the first record of Mierspenaeopsis sculptilis in Brazil, in areas of extractive reserves on the Amazon coast. Notably exotic species can cause imbalance in the ecosystem, harming native species. In view of this, the registration of new invasions is essential as they contribute to the implementation of control plans.
Validation of the media fill method for Cytokine-Induced killer cells manufacturing process
Authors: Ferrero Ivana , Castiglia Sara , Adamini Aloe , Mandese Alessandra , Sismondi Francesca , Rustichelli Deborah , Leone Marco , Pinnetta Giuseppe , Giordanengo Lucia , Mareschi Katia , Fagioli Franca 
Published: August 2023

Abstract
Background
Media fills are used to demonstrate the adequacy of the aseptic conditions of a pharmaceutical production process, according to good manufacturing practice (GMP), using a growth medium in place of the product solution. For advanced therapy medicinal product (ATMP), where the final product consists of viable cells, the media fill is still a challenge, but represents the starting point for process validation.

Methods
The aim of this paper is to describe the media fill test procedure in the context of ATMP manufacturing, in particular of Cytokine-Induced Killer (CIK) cell expansion process under GMP conditions, including quality control tests and environmental monitoring. The media fill test has been designed to cover all the critical steps of the process, including worst cases and deviations.

Results
From July 2019 to August 2022, we performed 16 media fill tests. During these years, the media fill protocol has been gradually improved and the worst cases were designed to be closer to reality and occurring cases. Although some deviations occurred, all the media fills performed were compliant.

Conclusions
A good media fill design combined with a robust environmental monitoring program provides a high degree of assurance of the microbial safety of ATMPs.
Suzuki–Miyaura cross-couplings for alkyl boron reagent: recent developments—a review
Authors: Ahmed Adnan , Mushtaq Irfan , Chinnam Sampath 
Published: August 2023

AbstractIn the history of catalysis and organic synthesis, boric chemistry has been developed into one of the most varied and practical disciplines. Several useful Suzuki–Miyaura cross-coupling (SMC) reactions as well as hydroborylation reactions are regarded the essential tools inside the chemical synthesis toolkit accompanied by researchers of the industry or the academia. Novel synthesis of the less electrically and sterically ongoing carbon–boron synthetic reagents is carried out to ensure a unique coupling reaction utilizing metals along with these reagents which draws considerable interest in accelerating the discovery of creative uses for otherwise difficult organic adducts in many disciplines. This article details the noteworthy advancements in the use of traditional metal-catalyzed carbon–carbon coupling processes with cutting-edge coupling partners such as carbon–boron reagents often the beta-alkyl Suzuki–Miyaura coupling since 2001. The current review covers alkylboranes, organotrifluoroborates, 9-BBN, alkylboronic acids and boronic esters as useful reagents in SMCs that will help synthetic chemists in developing new compounds.
Graphical Abstract
“The Agriculture Ministry of the Whole World”
Authors: Williams Elizabeth R. 
Published: August 2023

Abstract
In 1905 the establishment of the International Institute of Agriculture (IIA), the forerunner to the Food and Agriculture Organization (FAO) of the United Nations, created a forum for bureaucrats and technocrats from around the globe to exchange information about the latest developments in agricultural practice and administration. Representatives from the Ottoman Empire were active participants in the institute's early activities. This article traces their contributions to the institute's formative debates that aimed to set international standards as well as the imperial-level projects that resulted from their participation. It argues that the institute, in addition to representing a space in which Ottoman officials could assert their expertise and perform their capacity to be a part of global standard-setting processes, provided an impetus for collating and comparing statistics from across the empire. The projects undertaken drew from existing provincial statistics-gathering institutions and served to reveal differences across provinces in a step toward greater empire-wide legibility. Focusing on the empire's Eastern Mediterranean provinces, the article demonstrates how these statistics' public circulation not only enabled Ottoman officials to identify regions they considered ripe for further agricultural development, but also supported French officials' justifications for imposing colonial rule post–World War I.
Introduction
Authors: Abusharaf Rogaia , Chandra Uday , Promodh Irene 
Published: August 2023

Abstract
This special section draws on the dynamic new field of Indian Ocean studies to rethink key concepts such as space and circulation (Jeremy Prestholdt), gender and kinship (Mahmood Kooria), and popular media and infrastructure (Bindu Menon Mannil). Through fresh interdisciplinary insights from the contributors' archives and fieldwork, the articles critically interrogate these concepts from a distinctive intellectual vantage point. Historians and anthropologists based in different parts of the globe come together in this special section to “think with the Indian Ocean.” The authors take a concept that is in wide currency in the humanities and social sciences and then reimagine it creatively in the contexts that they study. In doing so, they decenter familiar ways of seeing and knowing, offering a new decolonial lens to make sense of the circularities and connections that constitute our world.
Ethical issues of involving people with intellectual disabilities in genomic research: a scoping review protocol
Authors: Chepkirui Dorothy , Kipkemoi Patricia , Bitta Mary , Harris Eli , Musesengwa Rosemary , Kamuya Dorcas 
Published: August 2023

Background: Psychiatric genomic research is a growing field of research in Africa that is looking at epigenetics of psychiatric disorders; within which a specific focus is neurodevelopmental disorders including intellectual disability (ID). Conducting this type of research is important to identify etiologies and possible interventions or areas for further research. However, genomic research generally, and psychiatric genomic research, faces many social, ethical, cultural, and legal issues; research involving people with ID is particularly challenging. All research stakeholders - researchers, research review bodies, regulators, patient groups - generally agree that involving people with ID require several considerations, including extra protection. It is also recognized that not involving people with ID in research that is relevant to them means that opportunities to learn on specific issues including lived experiences are missed. In this scoping review, we aim to describe the range of ethical and social-cultural issues concerning involvement of people with intellectual disability in genomic research from existing literature. Methods: This scoping review will be conducted based on the Joanna Briggs Institute guidance for scoping review and reported using the PRISMA-ScR guidelines. Iterative review stages will include systematic search of six databases (Embase, Ovid Global Health, PubMed, Scopus, PsycInfo and Web of Science core collection), screening, charting and synthesis of the data. Forward and backward citation screening will also be done for the articles included in the final review. We will include peer reviewed journal articles, guidance documents and reports. Screening and selection of studies based on the eligibility criteria will be done independently by three reviewers; conflicts will be resolved through discussion with a third reviewer and other experts. Results: The results will be included in the scoping review publication. Conclusions: This scoping review will identify key areas of ethical tensions and possible solutions and inform opportunities for empirical ethics studies.