bluuebunny/crossref_metadata_2025_split huggingface dataset:
Severe pancytopenia at the presentation of Imerslund-Gräsbeck syndrome in a 23-month-old Italian boy
Authors: Di Sario Francesca , Piloni Francesca , Gasparini Francesco , Serpetti Eleonora , Bruschi Barbara , Coccia Paola , Lionetti Maria Elena , Gatti Simona 
Published: September 2024

Abstract
Background
Imerslund-Gräsbeck syndrome (IGS) is a rare autosomal recessive disorder characterized by megaloblastic anemia due to selective cobalamin malabsorption and benign proteinuria. IGS is caused by a disfunction of the cubam receptor, which mediates the reabsorption of cobalamin in the ileum and the reuptake of albumin in renal proximal tubules.

Case presentation
We describe the case of a 23-month-old-italian infant presenting with severe pancytopenia and failure to thrive in whom the diagnosis of IGS was made and vitamin B12 replacement therapy was resolutive. Genetic analysis (NGS with CNV analysis including 214 genes involved in bone marrow failure and anemia), showed the presence of two pathogenetic variants in the AMN gene (c-208–2 A > G and c.1006 + 34_1007-31del). These variants have been previously described in the literature, but their combination has never been reported.

Conclusions
Imerslund-Gräsbeck syndrome should be considered in the differential diagnosis of children with severe pancytopenia even in those without neurological involvement. This case emphasizes the importance of an early diagnosis and prompt treatment, to prevent irreversible neurological injury.
Should my child be given antibiotics? A systematic review of parental decision making in rural and remote locations
Authors: Marsh Stephanie A. , Parsafar Sara , Byrne Mitchell K. 
Published: September 2024

Abstract
Background
The emergence and growth in antibiotic resistant bacteria is a critical public health problem exacerbated by the misuse of antibiotics. Children frequently succumb to illness and are often treated with antibiotic medicines which may be used improperly by the parent. There is limited evidence of the factors influencing parental decision-making about the use of antibiotics in low-resource contexts. The aim of this systematic review was to understand and describe how parents living in rural and remote locations make choices about their children’s antibiotic use.

Method
The CINAHL, Web of Science, Medline, Scopus and Academic Search Premier databases were systematically searched from 31 January until 28 June in 2023. No date restrictions were applied and additional search methods were utilised to identify further studies that met inclusion criteria. Eligibility criteria included studies which reported on factors contributing to parental decisions about their children’s use of antibiotics in rural and remote settings. The Joanna Briggs Institute Critical Appraisal Checklists were employed to evaluate studies. Characteristics and findings were extracted from studies, and data was synthesised descriptively and presented in summary tables.

Results
A total of 3827 articles were screened and 25 worldwide studies comprising of quantitative, qualitative and prospective designs were included in the review. Studies that reported the number of rural caregivers consisted of 12 143 participants. Data analysis produced six broad themes representing the mechanisms that influenced parents in their access and use of antibiotics: the child’s symptoms; external advice and influences; parent-related determinants; barriers to healthcare; access to antibiotics; and socio-demographic characteristics.

Conclusions
A number of factors that influence parents’ prudent use of antibiotics in rural contexts were identified. In seeking to enhance appropriate use of antibiotics by parents in rural and remote settings, these determinants can serve to inform interventions. However, the identified studies all relied upon parental self-reports and not all studies reviewed reported survey validation. Further research incorporating validated measures and intervention strategies is required.

Registration details
Should my child be given antibiotics? A systematic review of parental decision making in rural and remote locations; CRD42023382169; 29 January 2023 (date of registration). Available from PROSPERO.
Clinical efficacy and safety of faecal microbiota transplantation in the treatment of irritable bowel syndrome: a systematic review, meta-analysis and trial sequential analysis
Authors: Lo Shao-Wei , Hung Tsung-Hsuan , Lin Yen-Tsen , Lee Chun-Shen , Chen Chiung-Yu , Fang Ching-Ju , Lai Pei-Chun 
Published: September 2024

Abstract
Background
The aim of this study is to evaluate the efficacy and safety of faecal microbiota transplantation (FMT) for the treatment of irritable bowel syndrome (IBS).

Methods
We searched four databases for randomised controlled trials (RCTs) that compared FMT with a control intervention in patients with IBS. The revised Cochrane risk-of-bias (RoB) tool was chosen for appraisal. Meta-analysis with trial sequential analysis (TSA) was conducted. Grading of Recommendations Assessment Development and Evaluation (GRADE) methodology was used to assess the certainty of evidence (CoE).

Results
We included 12 RCTs with a total of 615 participants. Meta-analyses showed no significant difference between the FMT and control groups in terms of clinical responses (relative risk [RR] = 1.44, 95% confidence interval [CI] 0.88–2.33) and changes in IBS Severity Scoring System (IBS-SSS) scores (standardised mean difference [SMD] =  − 0.31, 95% CI  − 0.72 to 0.09) and IBS Quality of Life (IBS-QOL) scores (SMD = 0.30, 95% CI  − 0.09 to 0.69). Subgroup analysis revealed that in studies with low RoB and using endoscopy, nasojejunal tube and rectal enema delivery, FMT led to a significant improvement in clinical responses and changes in IBS-SSS and IBS-QOL scores. TSA suggested that the current evidence is inconclusive and that the CoE is very low.

Conclusion
This study suggests that patients with IBS may benefit from FMT especially when it is administered via endoscopy, nasojejunal tube or rectal enema. However, the certainty of evidence is very low. Further research is needed to confirm the efficacy and safety of FMT for IBS treatment.
Trial Registration: PROSPERO registration number CRD42020211002.
Immune-related [18F]FDG PET findings in patients undergoing checkpoint inhibitors treatment: correlation with clinical adverse events and prognostic implications
Authors: Santo Giulia , Cucè Maria , Restuccia Antonino , Del Giudice Teresa , Tassone Pierfrancesco , Cicone Francesco , Tagliaferri Pierosandro , Cascini Giuseppe Lucio 
Published: September 2024

Abstract
Background
Direct comparisons between [18F]FDG PET/CT findings and clinical occurrence of immune-related adverse events (irAEs) based on independent assessments of clinical and imaging features in patients receiving immune checkpoint inhibitors (ICIs) are missing. Our aim was to estimate sites, frequency, and timing of immune-related PET findings during ICIs treatment in patients with melanoma and NSCLC, and to assess their correlation with clinical irAEs. Prognostic implications of immune-related events were also investigated.

Methods
Fifty-one patients with melanoma (47%) or NSCLC (53%) undergoing multiple PET examinations during anti-PD1/PDL1 treatment were retrospectively included. Clinical irAEs were graded according to CTCAE v.5.0. Abnormal PET findings suggestive of immune activation were described by two readers blinded to the clinical data. Progression-free survival (PFS) and overall survival (OS) were analyzed with the Kaplan-Meier method in patients stratified according to the presence of irAEs, immune-related PET findings or both.

Results
Twenty-one patients showed clinical irAEs only (n = 6), immune-related PET findings only (n = 6), or both (n = 9). In patients whose imaging findings corresponded to clinical irAEs (n = 7), a positive correlation between SUVmax and the severity of the clinical event was observed (rs=0.763, p = 0.046). Clinical irAEs occurred more frequently in patients without macroscopic disease than in metastatic patients (55% vs. 23%, p = 0.039). Patients who developed clinical irAEs had a significantly longer PFS than patients who remained clinically asymptomatic, both in the overall cohort (p = 0.011) and in the subgroup of (n = 35) patients with metastatic disease (p = 0.019). The occurrence of immune-related PET findings significantly stratified PFS in the overall cohort (p = 0.040), and slightly missed statistical significance in patients with metastatic disease (p = 0.08). The best stratification of PFS was achieved when all patients who developed immune-related events, either clinically relevant or detected by PET only, were grouped together both in the overall cohort (p = 0.002) and in patients with metastatic disease (p = 0.004). In the whole sample, OS was longer in patients who developed any immune-related events (p = 0.032).

Conclusion
Patients with melanoma or NSCLC under ICI treatment can develop clinical irAEs, immune-related PET findings, or both. The occurrence of immune-related events has a prognostic impact. Combining clinical information with PET assessment improved outcome stratification.
Impact of ante-mortem fluoxetine administration on estimation of post-mortem interval and insect activity in rabbit carcasses
Authors: Saleh Fatema M. , Badawy Assem H. , Badawy Rawda M. , Rahman Asmaa Abdel , Adly Eslam 
Published: September 2024

Abstract
Background
Increasing the number of drug-related deaths has affected medico-legal death investigations. Drugs within a corpse have a great impact on the insects’ development rate which in turn will affect the rate of post-mortem decomposition and the estimation of the post-mortem interval. This explains the importance of the application of forensic entomotoxicology, which studies the impact of drugs and toxins on the development and succession patterns of insects. The current study aimed to determine the impact of fluoxetine, one of the selective serotonin reuptake inhibitors (SSRIs), on post-mortem decomposition, insects’ attraction, and its pattern of succession on carcasses. Sixteen healthy male and female Oryctolagus cuniculus rabbits were chosen to be included in this study. They were divided into a treated group of eight rabbits received oral fluoxetine for 28 days and a control group of eight rabbits received oral distilled water for 28 days. After oral administration of 10 mg/kg/day of fluoxetine and distilled water for 28 days, rabbits were sacrificed, and carcasses were transmitted to the roof of Research and Training Centre on Vectors of Diseases at faculty of Sciences, Ain Shams University, for following up the post-mortem decomposition process and insect’s attraction to carcasses for 60 days. Carcasses were put in two outdoor sites that differ in temperature to assess the effect of temperature on decomposition process.

Results
Fluoxetine administration has accelerated the rate of post-mortem decomposition in the treated carcasses by 3–9 days compared to the control ones and affected the numbers and species of attracted insects, while it had no effect on the insects’ succession patterns. Exposure of carcasses to direct sunlight has accelerated the rate of decomposition in comparison to that of carcasses put in shade in the overall period of decomposition by about 14 to 16 days.

Conclusions
Fluoxetine has an important and effective role in post-mortem decomposition and estimation of post-mortem interval (PMI) and has a great impact on attracted insects to the treated carcasses. Temperature has a great effect on the rate of decomposition of carcasses. Higher temperature accelerates the rate of post-mortem decomposition.
A systematic review of sofosbuvir/velpatasvir/voxilaprevir in HCV patients previously treated with direct-acting antivirals
Authors: Rustam Rehan , Qaisar Aqsa 
Published: September 2024

AbstractThe importance of sofosbuvir/velpatasvir/voxilaprevir (SOF/VEL/VOX) remains crucial in managing chronic HCV infection among patients who have experienced treatment failure and relapse after prior use of direct-acting antivirals (DAAs), as evidenced by high SVR12. However, limited real-world data exists on safety and efficacy. Therefore, the study’s goal was to conduct a qualitative systematic review to assess SOF/VEL/VOX’s effectiveness and safety. Thorough searches spanned PubMed, Embase, and Scopus, from 2015 to August 1st, 2023. The outcomes assessed were SVR12 and treatment-related adverse events (AEs). We identified and analyzed 12 studies in which SVR12 of the per-protocol (PP) population was 96.7% and of the intention-to-treat (ITT) population was 92.6% showing excellent efficacy of SOF/VEL/VOX. SVR12 rates notably differed among patients: those without GT3 infection (94.20%) and without cirrhosis (97.60%) experienced higher rates compared to patients having GT3 infection (87.40%) and cirrhotic patients (94.20%). Treatment-related AEs were also recorded. To summarize, our study presents evidence that SOF/VEL/VOX serves as an extremely safe and efficacious therapy for HCV-infected patients, previously treated with DAAs.
The Moral Hazards of Technical Debt in Large Language Models: Why Moving Fast and Breaking Things Is Bad
Authors: Vee Annette 
Published: September 2024

Abstract
Companies such as OpenAI and other tech start-ups often pass on “technical debt” to consumers—that is, they roll out undertested software so that users can discover errors and problems. The breakneck pace of our current AI “arms race” implicitly encourages this practice and has resulted in consumer-facing large language models (LLMs) that have problems with bias and truth and unclear social implications. Yet, once the models are out, they are rarely retracted. The result of passing on the technical debt of LLMs to users is a “moral hazard,” where companies are incentivized to take greater risks because they do not bear their full cost. The concepts of technical debt and moral hazards help to explain the dangers of LLMs to society and underscore a need for a critical approach to AI to balance the ledger of AI risks.
Harriet Tubman's Deep Voice
Authors: Wallace Maurice , Peeler Matthew 
Published: September 2024

Abstract
The authors suggest that national investment in the educational utility of automated software comes at an enormous cost, a price paid by the very students that technology aims to convert to history as a lively and accessible field. This “high-tech mimicry” pretends to incarnate the past but instead silences the inflections of time, gender, region, race, or other vocalic variables. Further, the perception that technology is objective or unbiased conceals the vulnerabilities of language models. The authors argue that the illusions of authenticity these bots produce does not bode well for teaching African American history.
The Origins of Generative AI in Transcription and Machine Translation, and Why That Matters
Authors: Stone Matthew , Goodlad Lauren M. E. , Sammons Mark 
Published: September 2024

Abstract
In this essay, written in dialogue with the introduction to this special issue, the authors offer a critical history of the development of large language models (LLMs). The essay's goal is to clearly explicate their functionalities and illuminate the effects of their “generative” capacities—particularly the troubling divergences between how these models came into being, how they are currently developed, and how they are marketed. The evolution of LLMs and of their deployment as chatbots was not rooted in the design of interactive systems or in robust frameworks for humanlike communication or information access. Instead LLMs—in particular, generative pretrained transformers (GPTs)—arose through the steady advance of statistical proxies for predicting the plausibility of automated transcriptions and translations. Buoyed by their increasing faith in scale and “data positivism,” researchers adapted these powerful models for the probabilistic scoring of text to chat interaction and other “generative” applications—even though the models generate convincingly humanlike output without any means of tracking its provenance or ensuring its veracity. The authors contrast this technical trajectory with other intellectual currents in AI research that aimed to create empowering tools to help users to accomplish explicit goals by augmenting their capabilities to think, act, and communicate, through mechanisms that were transparent and accountable. The comparison to this “road not taken” positions the weaknesses of LLMs, chatbots, and LLM-based digital assistants—including their well-known “misalignment” with helpful and safe human use—as a reflection of developers’ failure to conceptualize and pursue their ambitions for intelligent assistance as responsible to and engaged with a broader public.
Challenges and opportunities in preserving intangible cultural heritage: lessons from the Demoscene
Authors: Lichnerowicz Andrzej , Grzeszczuk Maciej , Skorupska Kinga 
Published: September 2024

The Demoscene is a pioneering digital culture and an art form. Its roots in Poland date back to the mid-1980s. In 2019, the Polish Demosceners, alongside their Finnish and German counterparts in the Art of Coding campaign, embarked on an initiative to advocate for the recognition of the Demoscene by UNESCO. This move highlighted the tension between the inherently decentralised and demarcating nature of the Demoscene and formal requirements of cultural heritage preservation. In this paper, we present a brief overview of the Demoscene, combining personal observations from the UNESCO enlisting process with insights gathered from a supportive study conducted among Demoscene community members. The results of this study are presented in the context of digital preservation challenges in various domains, offering a comparative perspective. The paper concludes with some insights into what information to store alongside the Demoscene productions themselves and outlines potential further work, addressing both current and emerging challenges faced by the Demoscene community.
The Lesser Poland version of Silesian folk costume Attitudes towards the Cieszyn folk costume displayed by regionalists east of the Biała River in the 21 st century
Authors: Król Tymoteusz 
Published: September 2024

The Cieszyn folk costume, also referred to as the Silesian or the Valachian costume, is an important element of the cultural heritage of Cieszyn Silesia. It has been analysed in many academic and popular publications, both in terms of its material aspect and its role within the society. Thus far, however, authors focused on the region of Cieszyn Silesia in its historical borders, while in the early 20th century the Cieszyn folk costume could also be seen in what is now the Bestwina, Wilamowice and Brzeszcze communes, historically belonging to the region of Lesser Poland. Nowadays, it is worn for church ceremonies, as well as by members of folk dance and song ensembles and Rural Housewives Associations from the area. In some localities, there have been initiatives to replace the Cieszyn folk costume with others, e.g. the so-called lachowski (Lachy) attire, which used to be worn in the region and is associated with the cultural heritage of western Lesser Poland. The aim of this article is to analyse the various attitudes towards the Cieszyn folk costume as part of the heritage of western Lesser Poland. Some see it as a source of pride, others, as unwanted heritage. The analysis is based on interviews with local activists coupled with field observation and examination of photographic documentation available on the Internet.
Aksjologiczny wymiar pracy w językowo-kulturowym obrazie świata mieszkańców tradycyjnej polskiej wsi (na materiale małopolskim)
Authors: Piechnik Anna 
Published: September 2024

Praca zajmuje jedną z naczelnych pozycji w ludowym systemie aksjologicznym. W tradycyjnej społeczności wiejskiej najwyżej była ceniona praca fizyczna, a zwłaszcza szczególnie bliska jej członkom praca na roli. Wykonywanie pracy podlegało bardzo drobiazgowemu oglądowi i krytycznej ocenie. Najstarsze pokolenie mieszkańców polskiej wsi nadal jest nosicielem wspomnianego systemu wartości, a o poczesnym miejscu pracy w wiejskiej aksjosferze świadczy m.in. duże bogactwo funkcjonującego współcześnie nacechowanego słownictwa odnoszącego się do pracy. Obraz pracy, zwłaszcza pracy na roli, jest zróżnicowany pokoleniowo i niejednorodny. Materiałem językowym wykorzystanym w tekście jest przede wszystkim leksyka ekspresywna określająca człowieka ze względu na jego stosunek do pracy lub na sposób jej wykonywania. Analizie zostały poddane zarówno apelatywne ekspresywizmy osobowe i nieoficjalne antroponimy wzbogacone o parafrazy motywacyjne, jak i fragmenty historii mówionych zgromadzonych podczas badań terenowych w Małopolsce.
IgG4-Related Disease: Comprehensive Overview of Pathogenesis, Clinical Manifestations, and Diagnostic Challenges.
Authors: Bartoszek Lidia , Orłowska Dominika , Olszak Joanna , Zalewa Karolina , Kapłan Wojciech , Starownik Jakub , Gastoł Bartłomiej 
Published: September 2024

Introduction
IgG4-related disease (IgG4-RD) is a progressive and potentially life-threatening condition characterized by immune system activation and tissue fibrosis, affecting various organs such as the pancreas, kidneys, and lungs. Initially recognized in 2003, it often presents as mass-like lesions, which can mimic tumors. Despite advancements in understanding its pathology, epidemiological data are limited, and many patients remain undiagnosed due to unfamiliarity with the disease.
Aim of the study
The aim of this article wast to summarize the latest knowledge on the diagnosis and clinical manifestations of IgG4-related disease.
Materiał and methods
This review is based on articles from the PubMed and Google Scholar databases, covering the years 2007-2024, using the keywords: IgG4-related disease; autoimmune pancreatitis; retroperitoneal fibrosis; classification criteria.
Results
The disease's complex pathogenesis involves B and T cell activity, with genetic and environmental factors contributing. While effective treatments, like B cell depletion, exist, the disease's broad clinical manifestations and multi-organ involvement require a multidisciplinary approach for proper diagnosis and management.
Conclusion
Diagnosing IgG4-RD is challenging due to its varied symptoms, often mimicking other diseases. Key findings include high IgG4 levels and specific histopathological features. Further research is needed to understand its genetic factors, pathogenesis, and epidemiology.
benefits of triathlon disciplines in maintaining good health - a review of the literature
Authors: Zajkowska-Sierpniak Aleksandra , Cąkała Marlena , Kozioł Magdalena , Podgórniak Kamila , Szymchel Michał , Grzegorzak Filip 
Published: September 2024

Introduction:
A triathlon is a multisport event comprising three individual disciplines: swimming, cycling and running. The event is structured around two transition periods, during which competitors change from one mode of transportation to another. The multidisciplinary nature of triathlon places an exceptional physiological burden on athletes, with modifications to haemodynamic, biochemical, cardiorespiratory and metabolic parameters. Participation in triathlon has been linked with a number of benefits, including improved endurance, body toning and enhanced laboratory results. However, this is not an exhaustive list. Based on the evidence currently available, we will attempt to demonstrate that triathlon and its constituent activities represent an optimal approach for enhancing well-being.
Aim of the study:
The aim of this study is to demonstrate the impact of swimming, cycling and running on specific organ systems, with a particular emphasis on the components of a triathlon. In the course of our research, we have drawn upon the findings of numerous previous studies, as referenced in the bibliography. Every effort has been made to organize and standardize this knowledge.