bluuebunny/crossref_metadata_2025_split huggingface dataset:
Variability of disease activity in patients with hereditary angioedema type 1/2: longitudinal data from the Icatibant Outcome Survey
Authors: Maurer M. , Caballero T. , Aberer W. , Zanichelli A. , Bouillet L. , Bygum A. , Grumach A.S. , Botha J. , Andresen I. , Longhurst H.J. ,   for the IOS Study Group
Published: September 2021

AbstractBackgroundHereditary angioedema due to C1 inhibitor deficiency (HAE‐1/2) is a chronic and debilitating disease. The unpredictable clinical course represents a significant patient burden.ObjectiveTo analyse longitudinal registry data from the Icatibant Outcome Survey (IOS) in order to characterize temporal changes in disease activity in patients with HAE‐1/2.MethodsIcatibant Outcome Survey (NCT01034969) is an international observational registry monitoring the clinical outcomes of patients eligible for icatibant treatment. The current analyses are based on data collected between July 2009 and July 2019. Retrospective data for attacks recorded in the 12 months prior to IOS enrolment and for each 12‐month period up to 7 years were analysed.ResultsIncluded patients reported angioedema attacks without long‐term prophylaxis (LTP; n = 315) and with LTP (n = 292) use at the time of attack onset. Androgens were the most frequently used LTP option (80.8%). At the population level, regardless of LTP use, most patients (52–80%) reporting <5 attacks in Year 1 continued experiencing this rate; similarly, many patients (25–76%) who reported high attack frequency continued reporting ≥10 attacks/year. However, year on year, 31–51% of patients experienced notable changes (increase/decrease of ≥5 attacks) in annual attack frequency. Of patients who reported an absolute change of ≥10 attacks from Year 1 to 2, 17–50% continued to experience a change of this magnitude in subsequent years.ConclusionAt the population level, attack frequency was generally consistent over 7 years. At the small group level, 28.8–34.5% of patients reported a change in attack frequency of ≥5 attacks from Year 1 to Year 2; up to half of these patients continued to experience this magnitude of variation in disease activity in later years, reflecting high intra‐patient variability.
Children with familial hypercholesterolemia display changes in LDL and HDL function: A cross‐sectional study
Authors: Christensen Jacob J. , Narverud Ingunn , Ruuth Maija , Heier Martin , Jauhiainen Matti , Ulven Stine M. , Bogsrud Martin P. , Kovanen Petri T. , Halvorsen Bente , Oda Michael N. , Wium Cecilie , Retterstøl Kjetil , Öörni Katariina , Holven Kirsten B. 
Published: September 2021

AbstractBackgroundThe functional status of lipoprotein particles contributes to atherogenesis. The tendency of plasma low‐density lipoprotein (LDL) particles to aggregate and the ability of igh‐density lipoprotein (HDL) particles to induce and mediate reverse cholesterol transport associate with high and low risk for cardiovascular disease in adult patients, respectively. However, it is unknown whether children with familial hypercholesterolemia (FH) display lipoprotein function alterations.HypothesisWe hypothesized that FH children had disrupted lipoprotein functions.MethodsWe analyzed LDL aggregation susceptibility and HDL‐apoA‐I exchange (HAE), and activity of four proteins that regulate lipoprotein metabolism (cholesteryl ester transfer protein, lecithin–cholesterol acyltransferase, phospholipid transfer protein, and paraoxonase‐1) in plasma samples derived from children with FH (n = 47) and from normocholesterolemic children (n = 56). Variation in lipoprotein functions was further explored using an nuclear magnetic resonance‐based metabolomics profiling approach.ResultsLDL aggregation was higher, and HAE was lower in FH children than in normocholesterolemic children. LDL aggregation associated positively with LDL cholesterol (LDL‐C) and negatively with triglycerides, and HAE/apoA‐I associated negatively with LDL‐C. Generally, the metabolomic profile for LDL aggregation was opposite of that of HAE/apoA‐I.ConclusionsFH children displayed increased atherogenicity of LDL and disrupted HDL function. These newly observed functional alterations in LDL and HDL add further understanding of the risk for atherosclerotic cardiovascular disease in FH children.
Super‐resolution reconstruction of T2‐weighted thick‐slice neonatal brain MRI scans
Authors: Askin Incebacak N. Ceren , Sui Yao , Gui Levy Laura , Merlini Laura , Sa de Almeida Joana , Courvoisier Sebastien , Wallace Tess E. , Klauser Antoine , Afacan Onur , Warfield Simon K. , Hüppi Petra , Lazeyras Francois 
Published: September 2021

AbstractBackground and PurposeSuper‐resolutionreconstruction (SRR) can be used to reconstruct 3‐dimensional (3D) high‐resolution (HR) volume from several 2‐dimensional (2D) low‐resolution (LR) stacks of MRI slices. The purpose is to compare lengthy 2D T2‐weighted HR image acquisition of neonatal subjects with 3D SRR from several LR stacks in terms of image quality for clinical and morphometric assessments.MethodsLR brain images were acquired from neonatal subjects to reconstruct isotropic 3D HR volumes by using SRR algorithm. Quality assessments were done by an experienced pediatric radiologist using scoring criteria adapted to newborn anatomical landmarks. The Wilcoxon signed‐rank test was used to compare scoring results between HR and SRR images. For quantitative assessments, morphology‐based segmentation was performed on both HR and SRR images and Dice coefficients between the results were computed. Additionally, simple linear regression was performed to compare the tissue volumes.ResultsNo statistical difference was found between HR and SRR structural scores using Wilcoxon signed‐rank test (p = .63, Z = .48). Regarding segmentation results, R2 values for the volumes of gray matter, white matter, cerebrospinal fluid, basal ganglia, cerebellum, and total brain volume including brain stem ranged between .95 and .99. Dice coefficients between the segmented regions from HR and SRR ranged between .83 ± .04 and .96 ± .01.ConclusionQualitative and quantitative assessments showed that 3D SRR of several LR images produces images that are of comparable quality to standard 2D HR image acquisition for healthy neonatal imaging without loss of anatomical details with similar edge definition allowing the detection of fine anatomical structures and permitting comparable morphometric measurement.
Exploration of an effective method to determine the relative sweetness of natural alternative sweeteners: Comparison of two‐alternative forced‐choice test and generalized labeled magnitude scale
Authors: Lee Soo‐Hyun , Kim Min‐Ji , Hong Jae‐Hee 
Published: September 2021

AbstractThe relative sweetness (RS) of natural alternative sweeteners is influenced by their distinct flavors and temporal profiles but also by the determination method. This study investigated the reported RS of natural alternative sweeteners using two methods—the two‐alternative forced‐choice (2‐AFC) test and generalized labeled magnitude scale (g‐LMS). The 2‐AFC test and g‐LMS were applied to determine the RS of rebaudioside A, luo han guo extract, and xylo‐oligosaccharide to 5% sucrose. The RS values were verified by comparison to those obtained from descriptive analysis (DA). The 2‐AFC test produced RS values more similar to those of the DA, whereas the g‐LMS yielded much higher RS values. The results suggest that the 2‐AFC test, which compares the integrated perception of samples directly to that of a reference, yields a more accurate RS value than the g‐LMS, which asks panelists to rate sweetness separately from other sensations that might influence perception.Practical applicationsReplacing sugar with alternative sweeteners is one sugar reduction strategy widely used in the food industry. It is important to accurately identify RS values because an accurate replacement ratio based on the RS is necessary for successful sugar replacement that does not change the original sensory profile. However, the unique sensory characteristics and temporal profiles of natural alternative sweeteners can make it difficult to determine RS values accurately. This study compared the performances of the 2‐AFC test and g‐LMS, which use different evaluation strategies, to provide researchers with insights on the efficacy of the methods. It is expected that the outcomes of this study will aid in the research and development of sugar replacement strategies in both academia and industry.
Moving toward responsible value creation: Business model challenges faced by organizations producing responsible health innovations
Authors: Lehoux Pascale , Silva Hudson P. , Denis Jean‐Louis , Miller Fiona A. , Pozelli Sabio Renata , Mendell Marguerite 
Published: September 2021

AbstractResponsible Research and Innovation (RRI) seeks to steer innovation toward important societal challenges and, by doing so, calls for entrepreneurial activities that create economic, social, and environmental value. Nonetheless, little is known about the way different types of organization can produce responsible products and services and the challenges they face when implementing new business models remain largely uncharted. By linking the RRI and the business model literatures, the aim of this article is to generate a better understanding of the challenges underlying responsible value creation. To do so, we approach the business model as a dynamic construct that crosses organizational boundaries and develop an empirically grounded multilevel model that links entrepreneurs' practices (micro‐level), organizational management (meso‐level), and innovation system dynamics (macro‐level). Our multiple case studies include for‐profit and not‐for‐profit Canadian and Brazilian organizations (n = 16) engaged in the production of responsible health innovations and explore the following research questions: “What business model challenges do these organizations face in their attempt to produce responsible innovations? How do these challenges affect the implementation of their business model and capacity to achieve responsible value creation?” Our findings focus on cross‐case commonalities that clarify how specific business model components are dynamically adapted in response to eight micro‐, meso‐, and macro‐level business model challenges, while the organizations' capacity to adequately align these components remains precarious. Our study provides innovation management scholars with an empirically grounded model that brings conceptual clarity to responsible value creation. This groundwork may foster cumulative knowledge growth on the way RRI‐oriented organizations can orchestrate their activities toward responsible value creation, which simultaneously requires individual entrepreneurial skills, organizational capacities, and the support of other innovation stakeholders.
Rural‐urban differences in HPV testing for cervical cancer screening
Authors: Locklar Lindsay R. B. , Do D. Phuong 
Published: September 2021

AbstractPurposeRural preventable cancer disparities are often attributed in part to lower screening rates secondary to compromised health care access. When considering higher cervical cancer incidence and mortality, existing analyses primarily consider differences in Pap testing rather than the preferred method of HPV testing, which is more sensitive in identifying severe cases of cervical dysplasia.MethodsLogistic regression using data from the 2016 and 2018 Behavioral Risk Factor Surveillance System was used to examine urban and rural rates of cervical cancer screening according to national guidelines. Propensity score weighting was used to account for baseline sociodemographic differences between rural and urban populations in the 2016 landline sample.FindingsIn 2016 and 2018, rural women were less likely than urban women to have current cervical cancer screening. This disparity was explained by sociodemographic variables in 2016. Among women with current cervical cancer screening, rural women were significantly less likely than urban women to undergo HPV testing in both 2016 and 2018.ConclusionRural women with current cervical cancer screening were significantly less likely than their urban counterparts to have HPV testing. It is possible that updates to preventive care guidelines may be slower to reach rural providers, rural patients may be unaware that HPV testing was completed, or rural practice configuration may complicate the integration of HPV testing into clinical practice. Failure to undergo HPV testing may lead to delayed cervical dysplasia diagnosis, missed opportunities for early intervention, and contribute to rural/urban disparities in cervical cancer incidence and mortality.
Integration of multiple volatile cues into plant defense responses
Authors: Hu Lingfei 
Published: September 2021

SummaryThe ability to predict future risks is essential for many organisms, including plants. Plants can gather information about potential future herbivory by detecting volatiles that are emitted by herbivore‐attacked neighbors. Several individual volatiles have been identified as active danger cues. Recent work has also shown that plants may integrate multiple volatiles into their defense responses. Here, I discuss how the integration of multiple volatiles can increase the capacity of plants to predict future herbivore attack. I propose that integration of multiple volatile cues does not occur at the perception stage, but may through downstream early defense signaling and then be further consolidated by hormonal crosstalk. Exploring plant volatile cue integration can facilitate our understanding and utilization of chemical information transfer.
Shifting seasonal patterns of water availability: ecosystem responses to an unappreciated dimension of climate change
Authors: Hajek Olivia L. , Knapp Alan K. 
Published: September 2021

SummarySeasonal patterns of water availability can differ dramatically among ecosystems, with well‐known consequences for ecosystem structure and functioning. Less appreciated is that climate change can shift the seasonality of water availability (e.g. to wetter springs, drier summers), resulting in both subtle and profound ecological impacts. Here we (1) review evidence that the seasonal availability of water is being altered in ecosystems worldwide, (2) explore several mechanisms potentially driving these changes, and (3) highlight the breadth of ecological consequences resulting from shifts in the seasonality of water availability. We conclude that seasonal patterns of water availability are changing globally, but in regionally specific ways requiring more rigorous and nuanced assessments of ecosystem vulnerability as well as the ecological consequences.
Cannabis use disorder among atrial fibrillation admissions, 2008–2018
Authors: Chouairi Fouad , Miller P. Elliott , Guha Avirup , Clarke John‐Ross , Reinhardt Samuel W. , Ahmad Tariq , Freeman James V. , Desai Nihar R. , Friedman Daniel J. 
Published: September 2021

AbstractBackgroundDespite changes inthe legality of cannabis use and the increasing prevalence of cannabis use disorder (CUD), there is little data investigating the association between CUD and inpatient atrial fibrillation (AF) hospitalizations.MethodsUsing the National Inpatient Sample, we identified Atrial Fibrillation (AF) hospitalizations with and without a codiagnosis of CUD using International Classification of Diseases diagnosis codes and compared demographics, socioeconomics, comorbidities, outcomes, and trends between cohorts.ResultsBetween 2008 and 2018, we identified 5,155,789 admissions for AF of which 31,768 (0.6%) had a codiagnosis of CUD. The proportion of admissions with a history of CUD increased from 0.3% in 2008 to 1.0% in 2018 (p < .001). Hospital discharges of patients with CUD were significantly younger (53 vs. 72 years, p < .001), had a higher proportion of black race (CUD: 26.6% vs. 8.0%, p < .001), and had a higher proportion of income in the lowest income quartile than without a codiagnosis of CUD (CUD: 40.5% vs. 26.2%, p < .001).ConclusionsCUD is increasingly prevalent among AF hospitalizations, particularly among young patients. Codiagnosis of CUD in AF hospitalizations is also more common in underserved patients. As a result, it is important for future research to examine and understand the impact of CUD on this population, particularly in the light of changing legislation surrounding the legality of cannabis.
Pneumatosis cystoides intestinalis: A rare complication after hematopoietic stem cell transplantation
Authors: Arman Bilir Özlem , Demir Arzu Meltem , Akçabelen Yunus Murat , Ok Bozkaya İkbal , Gürlek Gökçebay Dilek , Güneş Altan , Özbek Namık Yaşar , Yaralı Neşe 
Published: September 2021

AbstractBackgroundPneumatosis cystoides intestinalis (PCI) is a disorder in which widespread air sacs are present in mucosa, submucosa, subserosa, and intraabdominal area of the intestinal wall. It has a heterogeneous clinical presentation as a rare complication of intestinal graft‐versus‐host disease (GVHD). Computed tomography is the preferred imaging method for the diagnosis. Since the air sacs could be ruptured spontaneously, the presence of free air in the peritoneal cavity does not confirm intestinal perforation. The conservative treatment approach is sufficient in cases that do not require urgent surgical intervention, such as perforation or obstruction.CaseHere, we present a 2.5‐year‐old patient diagnosed with primary hemophagocytic lymphohistiocytosis (pHLH), who underwent allogeneic hematopoietic stem cell transplantation from a matched unrelated donor (MUD) and developed PCI secondary to intestinal GVHD 14th months after HSCT.ConclusionsPneumatosis cystoides intestinalis, which is a rare complication, should be kept in mind, especially in patients with intestinal GVHD and receiving intensive immunosuppressive, octreotide, and steroid treatment after HSCT.
Barriers to Advance Care Planning Implementation in Health care: An Umbrella Review with Implications for Evidence‐Based Practice
Authors: Poveda‐Moral Silvia , Falcó‐Pegueroles Anna , Ballesteros‐Silva Mónica Patricia , Bosch‐Alcaraz Alejandro 
Published: September 2021

ABSTRACTBackgroundAdvance care planning (ACP) refers to a process of discussions between professionals, patients, and their families, which allows the patient to define and communicate their care and treatment preferences. Understanding the barriers to advance care planning is the first step on the way to overcoming them and to improving person‐centred care and attention.AimsTo identify the barriers perceived by professionals, patients, and family members when implementing ACP in a clinical context and to analyse the methodological quality of the evidence.MethodsAn umbrella review guided by the Joanna Briggs Institute and a systematic review in accordance with PRISMA 2015 were utilized. Data were obtained from MEDLINE, Cochrane Library, The Joanna Briggs Institute, CINAHL, Scopus, and EMBASE in November 2018.ResultsFourteen systematic reviews were included. The main barriers reported by professionals were lack of knowledge and skills to carry out ACP, a certain fear of starting conversations about ACP, and a lack of time for discussions. Patients and family members considered that the main barriers were fear of discussing their relative’s end of life, lack of ability to carry out ACP, and not knowing who was responsible for initiating conversations about ACP.Linking Evidence to ActionThis review has examined the barriers presented by health professionals, patients, and family members, so that future lines of research can develop preventive or decisive measures that encourage the implementation of ACP in health care.
Model Based Design of a Low Cost and Compliant Low Profile Prosthetic Foot
Authors: Bartlett Harrison L. , King Shane T. , Goldfarb Michael , Lawson Brian E. 
Published: September 2021

Abstract
This paper describes the design of a simple and low-cost compliant low-profile prosthetic foot based on a cantilevered beam of uniform strength. The prosthetic foot is developed such that the maximum stress experienced by the beam is distributed approximately evenly across the length of the beam. Due to this stress distribution, the prosthetic foot exhibits compliant behavior not achievable through standard design approaches (e.g., designs based on simple cantilevered beams). Additionally, due to its simplicity and use of flat structural members, the foot can be manufactured at low cost. An analytical model of the compliant behavior of the beam is developed that facilitates rapid design changes to vary foot size and stiffness. A characteristic prototype was designed and constructed to be used in both a benchtop quasi-static loading test as well as a dynamic walking test for validation. The model predicted the rotational stiffness of the prototype with 5% error. Furthermore, the prototype foot was tested alongside two commercially available prosthetic feet (a low profile foot and an energy storage and release foot) in level walking experiments with a single study participant. The prototype foot displayed the lowest stiffness of the three feet (6.0, 7.1, and 10.4 Nm/deg for the prototype foot, the commercial low profile foot, and the energy storage and release foot, respectively). This foot design approach and accompanying model may allow for compliant feet to be developed for individuals with long residual limbs.
Development and Validation of a Finite Element Model of Wear in UHMWPE Liner Using Experimental Data From Hip Simulator Studies
Authors: Kottan Nihal , Gowtham N. H. , Basu Bikramjit 
Published: September 2021

Abstract
The wear of acetabular liner is one of the key factors determining osseointegration and long-term performance of total hip joint replacement implants. The experimental measurements of wear in total hip replacement components are time and cost-intensive. While addressing this aspect, a finite element model of a hip joint bearing consisting of zirconia-toughened alumina femoral head and ultrahigh molecular weight polyethylene liner was developed to predict the dynamic wear response of the liner. The Archard–Lancaster equation, consisting of surface contact pressure, wear rate, and sliding distance, was employed to predict the wear of the acetabular liner. The contact pressure and wear at the articulating surface were found to decrease over time. A new computational method involving three-dimensional point clouds from the finite element analyzed results were used to construct wear maps. The model was able to predict the linear wear, over 2 × 106 cycles with relative errors ranging from 9% to 36% when compared to the published results. The increasing error percentage occurring primarily from the use of a constant wear rate was reduced to a maximum of 17% by introducing a correction factor. The volumetric rate was predicted with a maximum relative error of 7% with the implementation of the correction factor. When the model was implemented to study acetabular liners of diameters ranging from 28 to 36 mm, the linear wear was seen to decrease with an increase in femoral head diameter, which is in agreement with the clinical data. This study emphasizes the need to develop more such FEA-based computational studies to reliably predict and correlate with experimentally measured temporal evolution of wear of load-bearing articulating joints.