bluuebunny/crossref_metadata_2025_split huggingface dataset:
Children with multiple endocrine neoplasia type 2B: Not tall and marfanoid, but short with normal body proportions
Authors: van den Broek Medard F. M. , van Santen Hanneke M. , Valk Gerlof D. , Verrijn Stuart Annemarie A. 
Published: June 2021

AbstractObjectiveMultiple endocrine neoplasia 2B (MEN2B) is characterised by early‐onset medullary thyroid carcinoma (MTC), pheochromocytoma and several nonendocrine manifestations. Unfortunately, MEN2B is often diagnosed late, after the development of clinically significant MTC. Marfanoid habitus is considered an important related feature, which may lead to the assumption that patients with MEN2B have tall stature. Here, we describe the longitudinal growth and body proportions of eight MEN2B patients during childhood.DesignIt is a retrospective case series.MethodsPatients were under the care of a Dutch MEN expertise centre. Growth patterns were assessed and interpreted in relation to body mass index (BMI), age at diagnosis and at thyroidectomy, extensiveness of disease manifestations and parental height.ResultsSeven patients were short during childhood, of whom four showed growth below target height range (THR) and three at the lowest margin of THR. Only one patient grew well within THR. All patients who attained final height (n = 4) ended within THR, despite short stature during childhood. Arm span/height ratio was not increased and upper segment/lower segment ratio was not reduced in any patient. Short stature in childhood in this study did not seem to be associated with age at diagnosis, age at thyroidectomy, extensiveness of MTC, endocrine deficiencies or BMI.ConclusionsThis study shows that children with MEN2B may well present with short rather than tall stature. Thereafter, final height within THR was attained in those who already reached adulthood, but none had tall stature. Finally, body proportions were normal in all children and adults in this case series, not underlining a ‘marfanoid’ body habitus.
Predictors of prolonged hypotension requiring vasopressor support after resection of pheochromocytoma and paraganglioma
Authors: Shu Qian , Lan Ling , Zhang Yuelun , Yu Chunhua , Huang Yuguang 
Published: June 2021

AbstractObjectiveProlonged hypotension is a common complication after resection of pheochromocytoma (PCC) or paraganglioma (PGL). The objective of our study was to identify preoperative or intraoperative clinical factors that can predict prolonged hypotension after PCC/PGL resection.Patients and MethodsA total of 414 patients who underwent resection of PCC or PGL at our institution between January 2013 and January 2020 were included. Patients were divided into two groups according to whether or not vasopressor support was required postoperatively. Associations between preoperative and intraoperative variables and prolonged hypotension were evaluated.ResultsTwo hundred and one (48.6%) patients had postoperative hypotension that required vasopressor support with a median duration of 20 h. Multivariable analysis demonstrated that increased 24‐h urinary norepinephrine (NE) levels (odds ratio [OR] = 1.091, 95% confidence interval [CI]: 1.052–1.132, p < .001), longer operative time (OR = 1.008, CI: 1.004–1.011, p < .001) and lower preoperative phenoxybenzamine dose (OR = 0.336, CI: 0.150–0.753, p = .008) were predictors of prolonged hypotension. Moreover, operative time, body mass index, 24‐h urinary level of NE and preoperative phenoxybenzamine dose were significantly correlated with the duration of postoperative vasopressor support.ConclusionsIncreased urine NE level, longer operative time and lower preoperative phenoxybenzamine dose were predictors of prolonged hypotension requiring vasopressor support. Clinicians can identify these factors to manage their patients better and prevent severe complications.
Genotypic and phenotypic analysis in 51 Chinese patients with primary distal renal tubular acidosis
Authors: Guo Wencong , Song Qijing , Zhang Ruixiao , Xin Qing , Liu Zhiying , Lang Yanhua , Zhao Xiangzhong , Shao Leping 
Published: June 2021

AbstractThe aim of this study was to analyze the genetic variants of 51 Chinese patients with distal renal tubular acidosis (dRTA) and explore the correlation between their genotype and phenotype. Eight variants of SLC4A1, 19 variants of ATP6V0A4, and 16 variants of ATP6V1B1 have been identified, and of which 14 were novel ones. Eleven patients with autosomal dominant dRTA, and four patients with autosomal recessive dRTA were caused by genetic defects in SLC4A1; 18 and nine patients with recessive dRTA were resulted by defects in ATP6V0A4 and ATP6V1B1 respectively; no causal gene was identified in seven patients. Mutation frequency of SLC4A1 in Chinese populations was more common than Europeans. The incidence of deafness in ATP6V0A4 and ATP6V1B1 groups was 16.7% and 54.5%, respectively. The frequency of CKD in adults, children and infants was 100%, 51%, and 3%, separately. Our study will further expand the mutation spectrum of primary dRTA and provide valuable references to genetic counseling of Chinese populations.
Large‐sample‐size assessment of socioeconomic predictors of community‐level resource management occurrence
Authors: Brewer Tom David , Andrew Neil , Gruber Bernd , Kool Johnathan 
Published: June 2021

AbstractCommunity‐level resource management efforts are cornerstones in ensuring sustainable use of natural resources. Yet, understanding how community characteristics influence management practices remains contested. With a sample size of ≥725 communities, we assessed the effects of key community (i.e., socioeconomic) characteristics (human population size and density, market integration, and modernization) on the probability of occurrence of fisheries management practices, including gear, species, and spatial restrictions. The study was based in Solomon Islands, a Pacific Island country with a population that is highly dependent on coastal fisheries. People primarily dwell in small communities adjacent to the coastline dispersed across 6 island provinces and numerous smaller islands. We used nationally collected data in binomial logistic regression models to examine the likelihood of management occurrence, given socioeconomic context of communities. In contrast to prevailing views, we identified a positive and statistically significant association between both human population size and market integration and all 3 management practices. Human population density, however, had a statistically significant negative association and modernization a varied and limited association with occurrence of all management practices. Our method offers a way to remotely predict the occurrence of resource management practices based on key socioeconomic characteristics. It could be used to improve understanding of why some communities conduct natural resource management activities when statistical patterns suggest they are not likely to and thus improve understanding of how some communities of people beat the odds despite limited market access and high population density.
Emergency resection or diverting stoma followed by elective resection in patients with colonic obstruction due to locally advanced cancer: a national cohort study
Authors: Rosander Emma , Holm Torbjörn , Sjövall Annika , Hjern Fredrik , Weibull Caroline E. , Nordenvall Caroline 
Published: June 2021

AbstractAimThe aim was to assess long‐term prognosis after emergency resection versus primary diverting stoma followed by elective tumour resection.MethodA national‐register‐based cohort study with retrospective analysis of prospectively collected data was performed. All Swedish patients with non‐metastatic obstructive locally advanced colon cancer treated with emergency resection or diverting stoma, followed by an elective resection, between 2007 and 2017 were included. The Kaplan–Meier method and Cox proportional hazards model were used to compare all‐cause mortality between patients with emergency resection and elective right‐ and left‐sided resection. The multivariable model was adjusted for year of diagnosis, age at diagnosis, sex, Charlson Comorbidity Index, American Society of Anesthesiologists class, tumour location and pN stage.ResultsIn all, 751 patients with a tumour in the right colon and 700 patients with a tumour in the left colon were included. Emergency resection was more common in patients with right‐sided colon tumours (681/751) than in patients with left‐sided colon tumours (483/700). The 5‐year overall survival in patients with right‐sided tumours was 25% after emergency resection and 46% after diverting stoma followed by elective resection (log‐rank test P = 0.001). The corresponding numbers for patients with left‐sided colon tumours were 40% and 64% (P < 0.001). Emergency resection was independently associated with increased all‐cause mortality in patients with left‐sided tumour (hazard ratio 1.63, 95% CI 1.21–2.19) but not in patients with right‐sided tumour (hazard ratio 1.21, 95% CI 0.80–1.81).ConclusionDiverting stoma followed by elective resection is associated with improved survival compared with emergency resection in patients with left‐sided colonic obstruction due to locally advanced tumours.
Effectiveness of interventions to shift drivers of roving banditry and reduce illegal fishing by Vietnamese blue boats
Authors: Wilcox Chris , Bergseth Brock J 
Published: June 2021

AbstractIllegal fishing via roving banditry, or fishing illegally in other countries’ territorial waters, continues to threaten the security, sustainability, and biodiversity of global marine resources. Yet, little is known about the behavioral drivers of banditry, and whether interventions can shift these. We address this critical knowledge gap by quantitatively surveying 82 fishers in two known port havens (Da Nang and Sa Ky, central Vietnam) for roving banditry by Vietnamese “blue boats,” before and after interventions aimed at reducing illegal fishing. We present three key findings: (1) displacement from the South China Sea and degraded local resources were primary behavioral drivers, (2) interventions increased perceptions of risk and shame if apprehended, and (3) the source of bail money (i.e., family vs. business) was highly predictive of whether fishers saw punishment as an adequate deterrent to illegal fishing. Lastly, we discuss the implications of these findings for regional policy and management strategies.
COVID‐19 in heart transplant recipients during February–August 2020: A systematic review
Authors: Diaz‐Arocutipa Carlos , Carvallo‐Castañeda Darla , Luis‐Ybañez Odalis , Pariona Marcos , Rivas‐Lasarte Mercedes , Álvarez‐García Jesús 
Published: June 2021

AbstractThe coronavirus disease 2019 (COVID‐19) pandemic represents a major concern in immunosuppressed patients such as heart transplant recipients. Therefore, we performed a systematic review to summarize the clinical features, treatment, and outcomes of heart transplant recipients with COVID‐19. We searched electronic databases from inception to January 11, 2021. Thirty‐nine articles (22 case reports and 17 cohorts) involving 415 patients were included. The mean age was 59.9 ± 15.7 years and 77% of patients were men. In cohort studies including outpatients and inpatients, the hospitalization rate was 77%. The most common symptoms were fever (70%) and cough (67%). Inflammatory biomarkers (C‐reactive protein and procalcitonin) were above the normal range. Forty‐eight percent of patients presented with severe or critical COVID‐19. Hydroxychloroquine (54%), azithromycin (14%), and lopinavir/ritonavir (14%) were the most commonly used drugs. Forty‐nine percent of patients discontinued the baseline regimen of antimetabolites. In contrast, 59% and 73% continued the same regimen of calcineurin inhibitors and corticosteroids, respectively. Short‐term mortality among cohorts limited to inpatients was 25%. Our review suggests that heart transplant recipients with COVID‐19 exhibited similar demographic and clinical features to the general population. However, the prognosis was poor in these patients.
Efficacy and safety of dulaglutide 3.0 and 4.5 mg in patients aged younger than 65 and 65 years or older: Post hoc analysis of the AWARD‐11 trial
Authors: Frias Juan P. , Bonora Enzo , Nevárez Ruiz Luis , Hsia Stanley H. , Jung Heike , Raha Sohini , Cox David A. , Bethel M. Angelyn , Konig Manige 
Published: June 2021

AbstractAimTo evaluate the efficacy and safety of dulaglutide 3.0 and 4.5 mg versus 1.5 mg when used as an add‐on to metformin in subgroups defined by age (<65 and ≥65 years).Materials and MethodsOf 1842 patients included in this post hoc analysis, 438 were aged 65 years or older and 1404 were younger than 65 years. The intent‐to‐treat (ITT) population, while on treatment without rescue medication, was used for all efficacy analyses; the ITT population without rescue medication was used for hypoglycaemia analyses; all other safety analyses used the ITT population.ResultsPatients aged 65 years or older and those younger than 65 years had a mean age of 69.5 and 53.2 years, respectively. In each age subgroup, the reduction from baseline in HbA1c and body weight (BW), and the proportion of patients achieving a composite endpoint of HbA1c of less than 7% (<53 mmol/mol) with no weight gain and no documented symptomatic or severe hypoglycaemia, were larger for dulaglutide 3.0 and 4.5 mg compared with dulaglutide 1.5 mg, but the treatment‐by‐age interactions were not significant. The safety profile for the additional dulaglutide doses was consistent with that of dulaglutide 1.5 mg and was similar between the age subgroups.ConclusionDulaglutide doses of 3.0 or 4.5 mg provided clinically relevant, dose‐related improvements in HbA1c and BW with no significant treatment‐by‐age interactions, and with a similar safety profile across age subgroups.
Fiscal space and the procyclicality of fiscal policy: The case for making hay while the sun shines
Authors: Ahmad Asif , McManus Richard , Ozkan F. Gulcin 
Published: June 2021

AbstractUtilizing data from 133 countries over the period 1950–2014, we identify fiscal space as a key factor underlying the cyclicality of fiscal policies. We find that less fiscal space induces greater fiscal procyclicality and show that this relationship is nonlinear; countries in the bottom tail of the fiscal space distribution need to make significant improvements before they can perform countercyclical policy. Given the increasingly dominant role of fiscal action in downturns, as is highlighted during the Covid‐19 crisis, these findings clearly indicate the importance of building fiscal space in good times to provide capacity for countercyclical policy in bad times.
Factor product utilization and health outcomes in patients with haemophilia A and B on extended half‐life concentrates: A Canadian observational study of real‐world outcomes
Authors: Sun Haowei (Linda) , Yang Ming , Poon Man‐Chiu , Lee Adrienne , Robinson K. Sue , Sholzberg Michelle , Wu John , Iorio Alfonso , Blanchette Victor , Carcao Manuel , Klaassen Robert J. , Jackson Shannon 
Published: June 2021

AbstractIntroductionRecombinant factors VIII and IX Fc (rFVIIIFc/rFIXFc) became available in Canada in 2016 and were the only extended half‐life (EHL) factor concentrates available in Canada until 2018.ObjectivesWe aim to describe the change in product utilization in Canadians who switched to rFVIIIFc/rFIXFc.MethodsThis prospective and retrospective cohort study enrolled males aged ≥6 years with moderate or severe haemophilia who switched to rFVIIIFc/rFIXFc and those who remained on standard half‐life (SHL) between 2016 and 2018. Factor utilization and annualized bleeding rates (ABR) were collected at baseline, 1‐year and 2‐years. Due to low prospective enrolment (n = 25 switchers), prospective and retrospective data were pooled.Results125 switchers (93 rFVIIIFc, 32 rFIXFc) and 33 non‐switchers were included. The median age was 17 (rFVIIIFc) and 38 years (rFIXFc). Prior to switch, over 80% were on prophylaxis. There was a statistically significant reduction in the prescribed weekly prophylactic dose after the switch to rFVIIIFc/rFIXFc for all age groups, with a corresponding reduction (15‐16%) in actual annualized FIX utilization in switchers (combined adults and children) to rFIXFc, and a smaller non‐significant reduction in actual annualized FVIIII utilization (7%) in children who switched to rFVIIIFc. A significant reduction in the median ABR was only observed in children who switched to rFVIIIFc, but not in adults who switched to rFVIIIFc or rFIXFc.ConclusionSwitching from SHL to EHL products led to a small reduction in factor utilization, while preserving a low ABR in children and adults with haemophilia. Further patient‐reported outcomes data will further elucidate the role of EHL in the haemophilia landscape.
The effect of celebrity endorsement on omission neglect at different levels of skepticism
Authors: Lopes Evandro Luiz , Goulart‐da‐Silva Jussara 
Published: June 2021

AbstractThe main objective of this study was to identify the effect of omission neglect concerning advertisements endorsed by celebrities given different levels of skepticism of the evaluators. Through three experiments (n = 512 university students and use of Analysis of Variance [ANOVA]), in which we manipulated the volume of information on different products and services, we identified that the endorsement of celebrities (Study 1) congruent with the endorsed product (Study 2) makes it difficult to recognize the omission of information by making individuals with high cognitive contextual knowledge (experts) neglect the omission of information. Through moderation analysis, we identified that high skepticism regarding advertisements cancels the effect of the endorsement (Study 3), causing experts to recognize the existence of absent data and, thus, make less positive evaluations of the advertisements analyzed. At the end of the study, we indicate management recommendations to potentiate the effect of advertisements endorsed by celebrities.
Survival migrants’ intention to stay: The case of Venezuelans in Colombia
Authors: Wentzel Arnold , Herrera Medina Nelson Eduardo , Vásquez Rodríguez Karol Marcela , Pretorius Marinda 
Published: June 2021

AbstractThis paper investigates the factors that determine survival migrants’ intention to stay in their host country, using a survey of Venezuelan migrants in Colombia and analysed by means of a binary regression model. A theoretical framework that explains the intention to stay was derived from the theory of return migration and confirmed the importance of both meso‐level and macro‐level factors in migrants’ intention to stay. A macroeconomic comparison confirmed that macro‐level factors played a role in the decision to migrate and stay. With respect to meso‐level factors, we found that survival migrants’ intentions deviate from those of voluntary migrants since ties to their home country were not significant influences on their intention to stay. In contrast, factors that strengthen or weaken structural integration and ties to the host country were often highly significant, as were factors that influenced survival migrants’ knowledge of the host country.
A set partitioning based goal programming model for the team formation problem
Authors: Daş Gülesin Sena , Altınkaynak Büşra , Göçken Tolunay , Türker Ahmet Kürşad 
Published: June 2021

AbstractIn this paper, we offer a multi‐objective set‐partitioning formulation for team formation problems using goal programming. Instead of selecting team members to teams, we select suitable teams from a set of teams. This set is generated using a heuristic algorithm that uses the social network of potential team members. We then utilize the proposed multi‐objective formulation to select the desired number of teams from this set that meets the skill requirements. Therefore, we ensure that selected teams include individuals with the required skills and effective communication with each other. Two real datasets are used to test the model. The results obtained with the proposed solution are compared with two well‐known approaches: weighted and lexicographic goal programming. Results reveal that weighted and lexicographic goal programming approaches generate almost identical solutions for the datasets tested. Our approach, on the other hand, mostly picks teams with lower communication costs. Even in some cases, better solutions are obtained with the proposed approach. Findings show that the developed solution approach is a promising approach to handle team formation problems.
Phase, domain, and microstructures in Sr2+ substituted low‐temperature sintering PZT‐based relaxor ferroelectrics
Authors: Chen Hao , Xing Jie , Xi Jingwen , Pu Tao , Liu Hong , Zhu Jianguo 
Published: June 2021

AbstractThe Ag‐Pd internal electrode of multilayer piezoelectric ceramics needs to be sintered below 1000°C, and lead wires and components need to be welded with lead‐free solder at 260°C. PNN–PMW–PZT–xSr piezoelectric ceramics with high Curie temperature (Tc > 260°C) were synthesized at a low sintering temperature (960°C) to meet the requirements of multilayer piezoelectric devices. The relationship between structures (phase, domain, and microstructures) and electrical properties (piezo/ferroelectric properties, and dielectric relaxation) in the Sr2+ substituted ceramics was investigated. Rietveld refinement and Raman spectra show that Sr2+ substitution can cause the phase change and increase the force constant of [BO6] octahedron. The piezoelectric response increases with increasing the content of the tetragonal phase (CTP) in the rhombohedral‐tetragonal (R‐T) coexisted ceramics. The ceramics with 0.6 mol% Sr2+ substitution have minimum activation energy for domain wall movement (Ea) of 0.0362 eV which favors the formation of nanometer‐sized domains, and possess excellent electrical properties (d33 = 623 pC/N, d33* =783 pm/V, Tc =295°C). The higher the CTP, the lower the Ea. The lower Ea favors the rotation of polarization direction and extension, and is beneficial to the generation of the nanometer‐size domains, resulting in high piezoelectric properties.
A Puzzle about Disability and Old Age
Authors: Lippert‐Rasmussen Kasper 
Published: June 2021

ABSTRACTIt is often claimed that (i) disability is bad for disabled people only when they live in an ableist social environment. However, few believe that (ii) having the physical and mental capacities that typify old age is worse than having the capacities of, say, a typical 30‐year‐old only because we live in an ageist social environment. This is a problem because it is plausible that (iii) claim (i) is correct if, and only if, claim (ii) is. The three claims form a trilemma. At least one must be rejected, however attractive each may seem. I submit, first, that how we think about disability‐related disadvantage constrains the way we should think of old‐age‐related disadvantages, and vice versa. Second, this constraint forces most of us to revise the way in which we think about either the disadvantages of disability, or old age, or both. Third, ultimately, it is more fruitful to discuss whether the disadvantages specifically connected with disability, or old age, or both, are bad in themselves rather than discussing whether the broad and quite varied set of disadvantages involved in disability and old age, respectively, are bad independently of the nature of the relevant social environment in which they exist.