bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prevalence of morbidities across the lifespan for adults with spinal muscular atrophy: a retrospective cohort study
Authors: G. Whitney Daniel , E. Neil Knierbein Erin , K. Daunter Alecia 
Published: August 2023

Abstract
Background
Recently approved treatments for spinal muscular atrophy (SMA) may shift clinical care priorities to secondary complications associated with SMA-related aging. To date, there is little knowledge about the natural history of morbidities across the adult lifespan for SMA. The objective of this study was to identify the prevalence and odds ratio (OR) of various morbidities among adults with vs. without SMA prior to SMA-related treatment.

Methods
This was a retrospective cohort study that accessed Medicare fee-for-service and commercial claims data from 01/01/2008-12/22/2016. Data from adults ≥ 18 years old with SMA and without SMA matched (1:200 case:control) on demographics, region, and study entry year were included. The prevalence of 30 morbidities across physiologic systems (e.g., cardiovascular, metabolic, musculoskeletal, urinary) and mental health disorders was examined. Age- and sex-adjusted OR was estimated using logistic regression for each morbidity and effect modification by age and sex was tested.

Results
There were 2,427 adults with SMA (mean [SD] age, 59.7 [17.4] years; 49.0% female) and 484,528 matched adults without SMA. Adults with vs. without SMA had a higher prevalence and adjusted OR of all 30 morbidities, ranging from OR = 1.61 (95% CI = 1.45–1.80) for hypothyroidism to OR = 7.80 (95% CI = 7.10–8.57) for fluid/electrolyte disorders. There was effect modification by age for 24 morbidities. The OR was highest for the youngest age group (18–40 years; OR range, 2.38 to 117.7; all P < 0.05) and declined with older age groups, but still remained significantly elevated in the oldest age group (≥ 75 years; OR range, 1.30 to 5.96; all P < 0.05).

Conclusions
The limitations of this study are that evidence of morbidities were limited to diagnostic claims and information on SMA type and symptoms or onset were not available. In conclusion, adults with SMA had a higher and earlier prevalence of a variety of morbidities across physiological systems and mental health disorders.
Initiatives to promote access to medicines after publication of the Brazilian Policy on the Comprehensive Care of People with Rare Diseases
Authors: Cunico Cássia , Vicente Geison , Leite Silvana Nair 
Published: August 2023

Abstract
Background
Rare diseases affect a small number of people compared to prevalent diseases. The vast majority of these diseases are of genetic origin, have no cure, are chronic and can lead to death. Although the right to access medicines is included in the constitutionally guaranteed right to health in Brazil, problems in the supply of medicines for rare diseases are reported in the country. This study aimed to describe and analyse the initiatives to promote access to medicines for treating rare diseases in the Unified Health System, Brazil, after the publication of the Brazilian Policy on the Comprehensive Care of People with Rare Diseases. Based on the model published by the WHO Regional Office for Europe, which described access to medicines in prelaunch, perilaunch and postlaunch policies, the initiatives referring to each category were summarized based on documentary research searched in online databases from January 2014 to December 2020.

Results
Different actions and policy interventions were identified, which went through the expansion of resources for research and development, health regulations, incorporation of new drugs, review and publication of clinical guidelines, and expansion of the network of care facilities by the Ministry of Health. On the other hand, aspects related to care policies, pricing methods, technological development, and development of pharmaceutical service processes were not implemented.

Conclusions
Although it is impossible to determine the explicit motivation of such actions concerning the Policy, its publication certainly was a landmark in Brazilian society, allowing greater recognition of the needs of rare disease patients and the specificities of treatment’. However, this study suggests that the steps that make up the life cycle of medicines are not linked, lacking articulation and integration of the care network, and consequently, there is no evidence that rare disease policy publication has generated a broad impact on the promotion of access to medicines to treat rare diseases in Brazil.
Neuropeptide Y directly reduced apoptosis of granulosa cells, and the expression of NPY and its receptors in PCOS subjects
Authors: Urata Yoko , Salehi Reza , Wyse Brandon A. , Jahangiri Sahar , Librach Clifford L. , Tzeng Chii-Ruey , Osuga Yutaka , Tsang Benjamin 
Published: August 2023

Abstract
Background
Most women with anovulatory infertility show polycystic ovarian syndrome (PCOS), and androgen excess is known as a key factor involved in pathogenicity of PCOS. However, the mechanism of follicular developmental arrest in PCOS is not completely understood. The reproductive function of Neuropeptide Y (NPY) in the ovary during folliculogenesis was previously reported; NPY function in apoptosis and proliferation of granulosa cells (GCs) is follicular-stage dependent. The objective of this study was to investigate the role of NPY in ovarian follicular development and the pathogenesis of PCOS.

Methods
To simulate the PCOS phenotype using a rat model, 21-day old Sprague Dawley rats were implanted with dihydrotestosterone (DHT) capsule (83 µg/day) and euthanized after 28 days. mRNA and protein content of NPY and its receptors were assessed in GCs from DHT treated rats using RT-qPCR and Western blot, respectively. Proliferation and apoptosis of GCs was assessed using Ki67- and TUNEL assays. Finally, NPY levels were measured in human follicular fluid (FF) from matched PCOS and non-PCOS patients using ELISA.

Results
GCs from DHT treated rats (PCOS-GCs) contained significantly less NPY protein and Npy mRNA by 0.16- and 0.56-fold, respectively, and more NPY receptor type 2 and 5 protein by 2.21- and 3.17-fold, respectively, when compared to sham control. Addition of recombinant NPY to PCOS-GCs culture did not alter Ki67-positive but significantly decreased TUNEL-positive cells by 0.65-fold, but not to baseline levels. There was no significant difference in NPY levels in FF between PCOS and non-PCOS subjects.

Conclusions
These results indicate that DHT modulates expression of NPY and its receptors, NPY decreases DHT-induced GCs apoptosis. That alterations in NPY’s function might be involved in follicular developmental failure of PCOS.
Brain tissue oxygen monitoring in traumatic brain injury: part I—To what extent does PbtO2 reflect global cerebral physiology?
Authors: Svedung Wettervik Teodor , Beqiri Erta , Bögli Stefan Yu , Placek Michal , Guilfoyle Mathew R. , Helmy Adel , Lavinio Andrea , O’Leary Ronan , Hutchinson Peter J. , Smielewski Peter 
Published: August 2023

Abstract
Background
The primary aim was to explore the association of global cerebral physiological variables including intracranial pressure (ICP), cerebrovascular reactivity (PRx), cerebral perfusion pressure (CPP), and deviation from the PRx-based optimal CPP value (∆CPPopt; actual CPP-CPPopt) in relation to brain tissue oxygenation (pbtO2) in traumatic brain injury (TBI).

Methods
A total of 425 TBI patients with ICP- and pbtO2 monitoring for at least 12 h, who had been treated at the neurocritical care unit, Addenbrooke’s Hospital, Cambridge, UK, between 2002 and 2022 were included. Generalized additive models (GAMs) and linear mixed effect models were used to explore the association of ICP, PRx, CPP, and CPPopt in relation to pbtO2. PbtO2 < 20 mmHg, ICP > 20 mmHg, PRx > 0.30, CPP < 60 mmHg, and ∆CPPopt < − 5 mmHg were considered as cerebral insults.

Results
PbtO2 < 20 mmHg occurred in median during 17% of the monitoring time and in less than 5% in combination with ICP > 20 mmHg, PRx > 0.30, CPP < 60 mmHg, or ∆CPPopt < − 5 mmHg. In GAM analyses, pbtO2 remained around 25 mmHg over a large range of ICP ([0;50] mmHg) and PRx [− 1;1], but deteriorated below 20 mmHg for extremely low CPP below 30 mmHg and ∆CPPopt below − 30 mmHg. In linear mixed effect models, ICP, CPP, PRx, and ∆CPPopt were significantly associated with pbtO2, but the fixed effects could only explain a very small extent of the pbtO2 variation.

Conclusions
PbtO2 below 20 mmHg was relatively frequent and often occurred in the absence of disturbances in ICP, PRx, CPP, and ∆CPPopt. There were significant, but weak associations between the global cerebral physiological variables and pbtO2, suggesting that hypoxic pbtO2 is often a complex and independent pathophysiological event. Thus, other variables may be more crucial to explain pbtO2 and, likewise, pbtO2 may not be a suitable outcome measure to determine whether global cerebral blood flow optimization such as CPPopt therapy is successful.
Comparative polar and lipid plasma metabolomics differentiate KSHV infection and disease states
Authors: Privatt Sara R. , Braga Camila Pereira , Johnson Alicia , Lidenge Salum J. , Berry Luke , Ngowi John R. , Ngalamika Owen , Chapple Andrew G. , Mwaiselage Julius , Wood Charles , West John T. , Adamec Jiri 
Published: August 2023

Abstract
Background
Kaposi sarcoma (KS) is a neoplastic disease etiologically associated with infection by the Kaposi sarcoma-associated herpesvirus (KSHV). KS manifests primarily as cutaneous lesions in individuals due to either age (classical KS), HIV infection (epidemic KS), or tissue rejection preventatives in transplantation (iatrogenic KS) but can also occur in individuals, predominantly in sub-Saharan Africa (SSA), lacking any obvious immune suppression (endemic KS). The high endemicity of KSHV and human immunodeficiency virus-1 (HIV) co-infection in Africa results in KS being one of the top 5 cancers there. As with most viral cancers, infection with KSHV alone is insufficient to induce tumorigenesis. Indeed, KSHV infection of primary human endothelial cell cultures, even at high levels, is rarely associated with long-term culture, transformation, or growth deregulation, yet infection in vivo is sustained for life. Investigations of immune mediators that distinguish KSHV infection, KSHV/HIV co-infection, and symptomatic KS disease have yet to reveal consistent correlates of protection against or progression to KS. In addition to viral infection, it is plausible that pathogenesis also requires an immunological and metabolic environment permissive to the abnormal endothelial cell growth evident in KS tumors. In this study, we explored whether plasma metabolomes could differentiate asymptomatic KSHV-infected individuals with or without HIV co-infection and symptomatic KS from each other.

Methods
To investigate how metabolic changes may correlate with co-infections and tumorigenesis, plasma samples derived from KSHV seropositive sub-Saharan African subjects in three groups, (A) asymptomatic (lacking neoplastic disease) with KSHV infection only, (B) asymptomatic co-infected with KSHV and HIV, and (C) symptomatic with clinically diagnosed KS, were subjected to analysis of lipid and polar metabolite profiles

Results
Polar and nonpolar plasma metabolic differentials were evident in both comparisons. Integration of the metabolic findings with our previously reported KS transcriptomics data suggests dysregulation of amino acid/urea cycle and purine metabolic pathways, in concert with viral infection in KS disease progression.

Conclusions
This study is, to our knowledge, the first to report human plasma metabolic differentials between in vivo KSHV infection and co-infection with HIV, as well as differentials between co-infection and epidemic KS.
Large intracranial supratentorial tuberculoma imitating as a malignant lesion: a case report
Authors: Vyas Shashin N. , Shekhawat Jitender 
Published: August 2023

Abstract
Background
CNS TB roughly correlates to 1–10% of all intracranial tumors. The spectrum of CNS-TB is wide and can present as tuberculous meningitis, tubercular abscess, tuberculoma, or hypertrophic pachymeningitis. Tuberculomas are usually located at the corticomedullary junction and periventricular region, as expected for hematogenous dissemination. They are mostly infratentorial in children and supratentorial in adults. They may present months to years after infection.

Observations
Here we have a 55-year-old female who presented with a history of headaches, sudden loss of consciousness, and seizures. Initial imaging showed a right parietal mass lesion appearing to be malignant on initial imaging which had a significant mass effect and midline shift. The patient was operated on for the same and histopathology showed chronic granulomatous lesion and tissue staining s/o tuberculosis.

Lessons
So a diagnostic option for infective etiology should be kept even for an intracranial supratentorial mass lesion.
Deciphering the functional diversity of the gut microbiota of the black soldier fly (Hermetia illucens): recent advances and future challenges
Authors: Eke Maurielle , Tougeron Kévin , Hamidovic Alisa , Tinkeu Leonard S. Ngamo , Hance Thierry , Renoz François 
Published: August 2023

AbstractBioconversion using insects is a promising strategy to convert organic waste (catering leftovers, harvest waste, food processing byproducts, etc.) into biomass that can be used for multiple applications, turned into high added-value products, and address environmental, societal and economic concerns. Due to its ability to feed on a tremendous variety of organic wastes, the black soldier fly (Hermetia illucens) has recently emerged as a promising insect for bioconversion of organic wastes on an industrial scale. A growing number of studies have highlighted the pivotal role of the gut microbiota in the performance and health of this insect species. This review aims to provide a critical overview of current knowledge regarding the functional diversity of the gut microbiota ofH. illucens, highlighting its importance for bioconversion, food safety and the development of new biotechnological tools. After providing an overview of the different strategies that have been used to outline the microbial communities ofH. illucens, we discuss the diversity of these gut microbes and the beneficial services they can provide to their insect host. Emphasis is placed on technical strategies and aspects of host biology that require special attention in the near future of research. We also argue that the singular digestive capabilities and complex gut microbiota ofH. illucensmake this insect species a valuable model for addressing fundamental questions regarding the interactions that insects have evolved with microorganisms. By proposing new avenues of research, this review aims to stimulate research on the microbiota of a promising insect to address the challenges of bioconversion, but also fundamental questions regarding bacterial symbiosis in insects.
Mind the gap: forest soils as a hidden hub for global micro- and nanoplastic pollution
Authors: Weber Collin J. , Rillig Matthias C. , Bigalke Moritz 
Published: August 2023

AbstractGlobal plastic pollution has become a major concern because of its effects on environmental and human health. A major fraction of environmental plastics is likely stored temporarily within terrestrial soils. However, even though forests represent the third most common type of land cover on Earth, almost nothing is known about plastics in forest soils. The atmospheric transport of micro- and nanoplastics provides ample opportunity for forest canopies to intercept plastic particles. These plastic particles, together with local plastic sources like litter and items used in forest management, eventually reach forest soils. In this paper we discuss the potential role of forest soils as a hub within global plastic cycles; transport processes from the atmosphere to the soil; and the integration of plastics into forest material cycles. Taken together, plastic in forests could have a major impact on sensitive ecosystems, economically important functions and global environmental plastic budgets. We also develop a roadmap for further investigation into plastics in forest soil systems.
An innovative medical waste management system in a smart city using XAI and vehicle routing optimization
Authors: Boudanga Zineb , benhadou Siham , Medromi Hicham 
Published: August 2023

Background: The management of medical waste is a complex task that necessitates effective strategies to mitigate health risks, comply with regulations, and minimize environmental impact. In this study, a novel approach based on collaboration and technological advancements is proposed. Methods: By utilizing colored bags with identification tags, smart containers with sensors, object recognition sensors, air and soil control sensors, vehicles with Global Positioning System (GPS) and temperature humidity sensors, and outsourced waste treatment, the system optimizes waste sorting, storage, and treatment operations. Additionally, the incorporation of explainable artificial intelligence (XAI) technology, leveraging scikit-learn, xgboost, catboost, lightgbm, and skorch, provides real-time insights and data analytics, facilitating informed decision-making and process optimization. Results: The integration of these cutting-edge technologies forms the foundation of an efficient and intelligent medical waste management system. Furthermore, the article highlights the use of genetic algorithms (GA) to solve vehicle routing models, optimizing waste collection routes and minimizing transportation time to treatment centers. Conclusions: Overall, the combination of advanced technologies, optimization algorithms, and XAI contributes to improved waste management practices, ultimately benefiting both public health and the environment.
The economic impact of living with a rare disease for children and their families: a scoping review protocol
Authors: Buckle Niamh , Doyle Orla , Kodate Naonori , Somanadhan Suja 
Published: August 2023

Background: Rare diseases are an often chronic, progressive and life-limiting group of conditions affecting more than 30 million people in Europe. These diseases are associated with significant direct and indirect costs to a spectrum of stakeholders, ranging from individuals and their families to society overall. Further quantitative research on the economic cost for children and their families living with a rare disease is required as there is little known on this topic. This scoping review aims to document the extent and type of evidence on the economic impacts of living with a rare disease for children and their families. Methods: This scoping review will follow the PRISMA-ScR and Joanna Briggs Institute guidelines and follow the six-stage methodology for scoping reviews: (1) identifying the research question, (2) identifying relevant studies, (3) study selection, (4) charting the data, (5) collating, summarising and reporting results and (6) knowledge user consultation. Key inclusion criteria have been developed according to the Population-Concept-Context (PCC) framework. The databases EconLit, ABI/Inform, MEDLINE, PubMed, CINAHL, and Scopus will be searched for possible articles for inclusion. Two independent reviewers will screen titles and abstracts of potential articles using a dual review process to ensure all relevant studies are included. All included articles will be assessed using a validated quality appraisal tool. A panel of patient and public involvement representatives experiencing rare diseases and knowledge users will validate the review results. Conclusions: This scoping review will map the current literature on the economic impact of paediatric rare diseases to understand how these impacts affect children living with rare diseases and their families. This evidence has the potential to influence policy and future research in this area and will support the future development of a cost-minimal intervention prototype to address the economic impact for families as part of this doctoral project.
Mind the Gap: Gender Disparity in Online Learning Platform Interactions
Authors: Wang Zhihan (Helen) , Li Jun , Wu Di (Andrew) 
Published: August 2023

Problem definition: Education technology innovations, such as massive open online course (MOOC) platforms, could potentially enable a more inclusive learning environment by delivering education to traditionally disadvantaged learners, like women. However, inclusivity does not necessarily translate into equal treatment on the platform. We investigate whether female and male learners benefit equally from forum discussions—typically the only form of interaction available—in online learning platforms. Methodology/results: Utilizing a large-scale, interaction-level data set on 174 courses on Coursera, we uncover an economically sizable and statistically significant disparity between male and female learners in receiving responses to their posts in MOOC discussion forums. On average, female learners’ questions are 3.11 percentage points less likely to receive responses from teaching staff than male learners’ questions, which equals 15.2% of the female group average. We also find significant gender disparity in staff response quality and sentiments. We investigate possible mechanisms behind the gender disparity using new techniques, including textual analysis tools. We show that the disparity is not because of content differences in male and female learners’ posts, nor is it attributable to their linguistic styles or the reputation of the posters. Instead, our results are most consistent with a male-driven gender homophily mechanism; although female staff members are gender neutral in their interactions with learners, male staff members systemically prefer responding to posts from male learners. We additionally show that receiving staff response leads to significant improvement in course passing rates, particularly for female learners. Therefore, the unequal access to information through course forums unfavorably hinders female learners’ performance. Managerial implications: Our results provide operational and organizational suggestions to platforms and content providers, including degendering user identifiers, implementing a content-focused post recommendation system, incorporating a gender-neutral user reputation system, promoting the recruiting of female teaching staff, and providing staff training that highlights the importance of gender-neutral interactions. Supplemental Material: The online appendix is available at https://doi.org/10.1287/msom.2022.0426 .
Linezolid-induced pancreatitis associated with lactic acidosis and relative hypoglycemia: A rare case report
Authors: Kim Gi Eun , Ansari Soubiya , Ali Ateeque , Adams Ehab , Eltahir Mugahid 
Published: August 2023

Background: Linezolid is known to cause side effects, including nausea, diarrhea, and headaches of short duration. As extended use of linezolid is becoming more common, additional rare side effects should be considered. Case Presentation: A 68-year-old man hospitalized for osteomyelitis developed severe abdominal pain and altered mental status following five weeks of linezolid therapy. Laboratory studies showed very high lipase levels, lactic acidosis not responding to resuscitation, and relative hypoglycemia. All common causes of pancreatitis were ruled out, and a trial of linezolid withdrawal was done resulting in drastic improvement in the patient's clinical status. Conclusions: For patients on extended course of linezolid who develop abdominal pain, drug-induced pancreatitis should be considered as a side effect, and a trial of withdrawal of linezolid should be undertaken.