bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effectiveness and safety of everolimus treatment in patients with tuberous sclerosis complex in real-world clinical practice
Authors: Cockerell Ine , Christensen Jakob , Hoei-Hansen Christina E. , Holst Lotte , Grenaa Frederiksen Mikkel , Issa-Epe Aart Imran , Nedregaard Bård , Solhoff Ragnar , Heimdal Ketil , Johannessen Landmark Cecilie , Lund Caroline , Nærland Terje 
Published: December 2023

Abstract
Background
The randomised double-blinded placebo-controlled EXIST-1–3 studies have showed everolimus effective with adverse effects reported as acceptable in treatment of symptoms in patients with tuberous sclerosis complex (TSC), although evidence of outcomes in clinical practice remains limited. This study aimed to investigate, in clinical practice, the effectiveness and safety of everolimus for epilepsy, renal angiomyolipoma (rAML), and subependymal giant cell astrocytoma (SEGA) in patients with TSC.

Results
The study included 64 patients with TSC (median age: 19, range 0.9–54 years) receiving everolimus treatment (Norway: n = 35; Denmark: n = 29). Among 45 patients with epilepsy, 14 (31%) were responders experiencing ≥ 50% reduction in seizure frequency in the last 3 months of treatment compared with the last 3 months before treatment. Nineteen (42%) patients changed their anti-seizure medications (ASMs). Responders were more common among patients < 18 years (46%) than among patients ≥ 18 years (14%, p = 0.03). In 29 patients with rAML, everolimus reduced (≥ 30% decrease) and stabilized (< 20% increase, ≤ 30% decrease) longest diameter of rAML in 38% and 59%, respectively, after a mean treatment duration of 37 months. SEGA volume was reduced in three patients by 71%, 43%, and 48% after 39, 34, and 82 months. Adverse effects were reported in 61 of 64 patients (95%) after a median treatment duration of 31 months (range 0–106), with oral ulceration/stomatitis (63%) and upper respiratory tract infections (38%) being the most common. The most common laboratory abnormalities were increased cholesterol (41%), anaemia (30%), and leucopoenia (25%). Grade 3–4 adverse effects were reported in 36% of cases, and life-threatening conditions were reported in two patients. Nine patients discontinued everolimus treatment.

Conclusions
Seizure reduction in this study sample was consistent with results from EXIST, but might be lower than expected, given that changes in concomitant ASMs are part of clinical practice. Seizure reduction was associated with younger age. As with EXIST, everolimus reduced or stabilised rAML size in most patients. SEGA volume was reduced in all three patients. Close follow-up is needed for this group, especially for children and patients who may not be able to report adverse effects.
Prognostic value of capillary refill time in adult patients: a systematic review with meta-analysis
Authors: Jacquet-Lagrèze Matthias , Pernollet Aymeric , Kattan Eduardo , Ait-Oufella Hafid , Chesnel Delphine , Ruste Martin , Schweizer Rémi , Allaouchiche Bernard , Hernandez Glenn , Fellahi Jean-Luc 
Published: December 2023

Abstract
Purpose
Acute circulatory failure leads to tissue hypoperfusion. Capillary refill time (CRT) has been widely studied, but its predictive value remains debated. We conducted a meta-analysis to assess the ability of CRT to predict death or adverse events in a context at risk or confirmed acute circulatory failure in adults.

Method
MEDLINE, EMBASE, and Google scholar databases were screened for relevant studies. The pooled area under the ROC curve (AUC ROC), sensitivity, specificity, threshold, and diagnostic odds ratio using a random-effects model were determined. The primary analysis was the ability of abnormal CRT to predict death in patients with acute circulatory failure. Secondary analysis included the ability of CRT to predict death or adverse events in patients at risk or with confirmed acute circulatory failure, the comparison with lactate, and the identification of explanatory factors associated with better accuracy.

Results
A total of 60,656 patients in 23 studies were included. Concerning the primary analysis, the pooled AUC ROC of 13 studies was 0.66 (95%CI [0.59; 0.76]), and pooled sensitivity was 54% (95%CI [43; 64]). The pooled specificity was 72% (95%CI [55; 84]). The pooled diagnostic odds ratio was 3.4 (95%CI [1.4; 8.3]). Concerning the secondary analysis, the pooled AUC ROC of 23 studies was 0.69 (95%CI [0.65; 0.74]). The prognostic value of CRT compared to lactate was not significantly different. High-quality CRT was associated with a greater accuracy.

Conclusion
CRT poorly predicted death and adverse events in patients at risk or established acute circulatory failure. Its accuracy is greater when high-quality CRT measurement is performed.
17-year follow-up of association between telomere length and all-cause mortality, cardiovascular mortality in individuals with metabolic syndrome: results from the NHANES database prospective cohort study
Authors: Xiong Lijiao , Yang Guangyan , Guo Tianting , Zeng Zhaohao , Liao Tingfeng , Li Yanchun , Li Ying , Chen Fujuan , Yang Shu , Kang Lin , Liang Zhen 
Published: December 2023

Abstract
Background
The relationship between leukocyte telomere length (LTL) and mortality risk in individuals with metabolic syndrome (MetS) remains poorly understood. This study aimed to investigate the association between telomere length and long-term all-cause mortality, and cardiovascular disease (CVD) mortality, in individuals with MetS in the United States.

Methods
A total of 1980 participants with MetS aged 18 years or older from the National Health and Nutrition Examination Survey (NHANES) prospective cohort study (1999–2002) were included in this cohort study. Medical records review was used to identify the cause of deaths as of December 2018. We employed Kaplan-Meier curves, fitted curves, and Cox proportional hazards regression models to estimate hazard ratios (HRs) for all-cause and CVD mortality, stratified by tertiles of LTL.

Results
Over a median follow-up of 17.75 years of participants with metabolic syndrome, 819 deaths occurred, including 231 cardiovascular deaths. After adjusting for multiple covariates, participants with shorter telomere length had a significantly higher risk of all-cause mortality (HR, 1.33; 95% CI, 1.11–1.6) and CVD mortality (HR, 1.36; 95% CI, 0.96–1.93) compared with those in the highest tertile of telomere length. All-cause mortality (P < 0.001) and cardiovascular disease mortality (P = 0.028) followed a similar pattern across tertiles of telomere length.

Conclusion
In individuals with MetS, shorter telomere length is associated with increased risks of death from cardiovascular disease and all causes. The underlying mechanisms and clinical implications of these findings require additional investigation.
Validation of non-invasive indicators in the screening of metabolic dysfunction-associated fatty liver disease: a cross-sectional study among Uighurs in rural Xinjiang
Authors: Guo Yanbo , Hu Yunhua , Yang Jing , Ma Rulin , Zhang Xianghui , Guo Heng , Wang Xinping , Li Yu , Peng Xinyu , Zhang Shijie , He Jia , Guo Shuxia 
Published: December 2023

Abstract
Background
Metabolic dysfunction-associated fatty liver disease (MAFLD) is the most common chronic liver disease in China. Our study aimed to evaluate the screening value of the fatty liver index (FLI), hepatic steatosis index (HSI), lipid accumulation product (LAP), visceral adiposity index (VAI), and Zhejiang University index (ZJU), as well as other single indicators for MAFLD. We aimed to find the optimal screening tool and its appropriate cut-off values for rural Uyghur adults.

Methods
We completed a survey of 14,321 Uyghur adults in 51 groups in Kashgar, Xinjiang, in 2016 using a typical sampling method, with 12,794 patients ultimately included in statistical analyses. Fatty liver was diagnosed using ultrasonography.

Results
The prevalence of fatty liver disease (FLD) and MAFLD was 16.73% and 16.55%, respectively, and the FLI, HSI, LAP, VAI, and ZJU were all independently associated with an increased risk of MAFLD. The areas under the receiver operating characteristic curves (AUCs) of the FLI for diagnosing MAFLD in men and women were 0.853 and 0.847, respectively. The AUCs of the body mass index (BMI) for diagnosing MAFLD in men and women were 0.850 and 0.852, respectively. Compared with other metabolic-related markers, FLI had the largest AUC. In men, the optimal cut-off values of FLI and BMI for diagnosing MAFLD were 45 (sensitivity 84.83%, specificity 69.57%) and 27.4 (sensitivity 78.47%, specificity 76.30%), respectively. In women, the optimal cut-off values of FLI and BMI for diagnosing MAFLD were 45 (sensitivity 80.11%, specificity 74.23%) and 28.0 (sensitivity 79.56%, specificity 75.41%), respectively. In men and women, an FLI score of < 30 ruled out MAFLD, while a score of ≥ 50 was a basis for diagnosis.

Conclusion
FLI and BMI had good screening ability for MAFLD and were superior to HSI, LAP, VAI, and ZJU in both sexes.
Women empowerment in agriculture and its effect on household food security: evidence from Gamo Zone of Southern Ethiopia
Authors: Jemaneh Sileshi Abebe , Shibeshi Elias Mekonnen 
Published: December 2023

Abstract
Background
Women empowerment and food security are supposed to be two mutually reinforcing goals of the 2030 Sustainable Development Agenda of the United Nations. However, the measurement of women’s empowerment and its relationship with household food security in the agricultural context is too limited, especially in Ethiopia. The main objective of this study is to examine the effect of women’s empowerment in agriculture on household food security in the Gamo zone of Ethiopia.

Methods
Cross-sectional data were collected from 385 dual-adult households, selected through a multistage random sampling procedure from two districts—Chencha and Kucha of Gamo zone in Ethiopia. The data were collected on the sample households’ demographic, socioeconomic, women empowerment, food consumption and livelihood activities—crop production, livestock holding and off-farm participation by interviewing both primary male and female decision-makers from each household. Household food security was measured using Calorie Availability, Household Food Consumption and Dietary Diversity Score, while women’s empowerment in agriculture was measured using the Abbreviated Women Empowerment in Agriculture Index. Ordinary Least Square, Two-Stage Least Square, Poisson and Instrumental Variable Poisson regression were used as analytical models.

Results
Our study shows that increases in women’s empowerment are positively and significantly associated with all food security indicators—adult equivalent calorie availability, household-level food consumption and dietary diversity. The result also shows that food security is positively related to the proportion of adult household members, age and education of primary female, landholding size, crop diversification, food crop production, the number of dairy cows owned and off-farm participation. However, food security is negatively related to household size.

Conclusion
The implication of the finding is that stakeholders should give due attention to promoting women’s empowerment and eliminating the gender gap in agriculture through appropriate gender mainstreaming intervention in dual-adult households. Food security programmes could also gain from prioritizing female education and promoting agricultural diversification (crop and livestock) and off-farm income diversification strategies as valuable investments to improve household food security. This study is the first application of the Abbreviated Women’s Empowerment in Agriculture Index in Ethiopia as a predictor to household food security, and contributes to the women’s empowerment–household food security nexus literature in developing countries.
Construct validity of the German version of the Emotion Reactivity Scale
Authors: Lüönd Antonia M. , Thoma Martina F. , Spiller Tobias R. , Weilenmann Sonja , Jansson Billy , Pfaltz Monique C. 
Published: December 2023

Abstract
Background
Emotional reactivity is an important construct to consider when studying mental disorders. This study was conducted to translate and assess the factor structure, construct validity and internal consistency of a German version of the Emotion Reactivity Scale (ERS), which is an originally English questionnaire assessing three components of emotional reactivity: sensitivity, intensity and persistence of emotions.

Methods
The German ERS and a range of questionnaires used to assess convergent and discriminant validity were completed by 334 German speaking Swiss participants.

Results
Confirmatory factor analysis showed strong support for a bi-factor model, with evaluation indices pointing to a unidimensional construct rather than to domain specific factors. The questionnaire showed good reliability and the factor structure was similar across gender. The ERS showed convergent validity with general psychopathology, behavioral inhibition, negative affect, orienting sensitivity, depressive symptoms and symptoms of disordered eating, and discriminant validity with behavioral activation and alcohol consumption.

Conclusions
Findings support the construct validity of the German ERS and suggest that it assesses a unidimensional construct with high internal consistency. Accounting for the unidimensional nature of the scale and aiming for efficient assessment tools, future research could, based on these findings, develop and evaluate the psychometric properties of a short version of the ERS.
Optimizing scenarios of a deep geothermal aquifer storage in the southern Upper Rhine Graben
Authors: Stober Ingrid , Jägle Martin , Kohl Thomas 
Published: December 2023

AbstractBased on a newly developed geological 3D reservoir model for the demonstration site of the ‘Freiburger Bucht’ in the Upper Rhine Graben (SW Germany), geothermal development and realization concepts of an aquifer thermal energy storage (ATES) in the Buntsandstein aquifer were elaborated and energetically evaluated by numerical modeling. The thermal–hydraulic coupled modeling was performed with the FE-software OpenGeoSys and COMSOL. For this purpose, the geological model was converted into a numerical model and calibrated by local and regional, hydrogeological and geothermal measured values. A detailed study based on two-phase storage-heating cycles per year with constant injection temperature on the ‘hot side’ of the ATES, different volumetric flow rates, and temperature spreads was performed to quantify possible storage capacities, energies, and efficiencies. The calculated efficiency of the cyclic storage operation in this study, averaged over 10 storage heating cycles, are between 50 and 85%, depending on flow rate and temperature spread. The efficiency of the individual storage heating cycles increases from year to year in all scenarios considered, as the ‘hot side’ of the storage heats up in the long term. To increase ATES’ efficiency, also horizontal wells were integrated into the numerical model and the results were compared with those of inclined wells.
Factors that increase the rate of periprosthetic dislocation after reverse shoulder arthroplasty
Authors: Reddy Chethan , Venishetty Nikit , Jones Hunter , Mounasamy Varatharaj , Sambandam Senthil 
Published: December 2023

Abstract
Introduction
Reverse shoulder arthroplasty (RSA) is considered one of the greatest technological innovations in shoulder reconstruction surgery, as evidenced by the fact its growth rate of usage is greatest among all shoulder arthroplasties. However, like all arthroplasties, a post-surgical complication often arises. One of these complications, periprosthetic dislocation (PPD), requires revision and poses, therefore, a burden on both patients and healthcare providers. While PPD is understood to be a complication of RSA, it is unclear to what extent certain risk factors and co-morbidities predispose patients to post-RSA PPD. The purpose of this study was to identify and evaluate the impact of specific risk factors and co-morbidities that contribute to the development of PPD following RSA.

Methods
In this retrospective study, we used the Nationwide Inpatient Sample (NIS) database from 2016–2019 to analyze the prevalence and impact of various risk factors and co-morbidities on the incidence of PPD following RSA. A univariate and subsequent multivariate logistic regression model was made to provide a descriptive association between variables that impact the rates of PPD after RSA.

Results
The NIS database identified 59,925 patients, 1,000 of whom experienced a PPD while the remaining 58,825 were placed in the non-PPD group (controls). The PPD group consisted predominantly of females (53.10%) and Caucasians (86.30%). There was a higher incidence of tobacco-related disorders (P = 0.003), obesity (P < 0.001), morbid obesity (P < 0.001), liver cirrhosis (P < 0.001), and Parkinson’s disease (PD) (P < 0.001) in PPD patients compared to controls. Young patients had a 1.89-fold increased odds (OR: 1.89, 95% CI [1.58, 2.26], P < 0.001), patients with tobacco-related disorders had decreased odds (OR: 0.80, 95% CI [0.67, 0.97], P = 0.02), morbidly obese patients had 1.50 times the odds (OR: 1.50, 95% CI [1.14, 1.97]), liver cirrhosis patients had 2.67-fold increased odds (OR: 2.67, 95% CI [1.55, 4.60], P < 0.001), and Parkinson’s disease patients had 2.66 times the odds (OR: 2.66, 95% CI [1.78, 3.96], P < 0.001) to develop PPD following RSA compared to patients who did not have the corresponding condition.

Conclusions
Patients with specific risk factors and co-morbidities are predisposed to developing PPD after RSA. Risk factors that were found to be associated with a higher incidence of PPD are gender (female), race (Caucasian), and age (young patients). Analysis revealed the history of tobacco-related disorder, obesity, morbid obesity, liver cirrhosis, and Parkinson’s disease increased the odds of developing PPD following RSA. These findings can inform both healthcare providers and patients to improve RSA surgical outcomes and tailor post-surgery recovery programs to fit the patient’s needs.
Analyzing safety and effectiveness of Mavacamten in comparison with placebo for managing hypertrophic cardiomyopathy: a systemic review and meta-analysis
Authors: Rangwala Hussain Sohail , Fatima Hareer , Ali Mirha , Ahmed Syed Taha , Rangwala Burhanuddin Sohail , Abbas Syed Raza 
Published: December 2023

Abstract
Background
Hypertrophic cardiomyopathy (HCM) is a hereditary myocardial disorder, often due to sarcomere gene mutations, characterized by the left ventricular hypertrophy. Current treatments offer symptomatic relief but lack specificity. Mavacamten, an allosteric inhibitor, has shown significant improvements in HCM patients in trials, reducing the requirement for invasive treatments. This meta-analysis assesses Mavacamten’s efficacy and safety as a targeted HCM intervention.

Methods
This study examined four randomized controlled trials comparing Mavacamten to placebo in HCM patients. Each trial had a unique primary endpoint, and secondary outcomes included improvements in NYHA-FC, eligibility for septal reduction therapy (SRT) or undergoing it, adverse events (serious and treatment-related), atrial fibrillation, and non-sustained ventricular tachycardia. Statistical analysis involved calculating risk ratios (RRs) and assessing heterogeneity.

Results
The four included studies showed minimal risk of bias and involved 503 patients with HCM (273 Mavacamten and 230 placebo). Mavacamten significantly increased the primary endpoint (RR 2.15, 95% CI 1.20–3.86, P = 0.01) and ≥ 1 NYHA-FC class (RR 2.21, 95% CI 1.48–3.3, P = 0.0001). Mavacamten group had lower rates of SRT compared to those receiving placebo (RR, 0.30, 95% CI 0.22–0.40; P < 0.00001). No significant differences existed in rates adverse events between the Mavacamten and placebo groups.

Conclusions
Our study suggests that Mavacamten may have therapeutic benefits for HCM patients, as indicated by its positive impact on certain endpoints. Further research with larger samples, longer follow-up, and comprehensive analysis is needed to understand Mavacamten’s safety and efficacy in HCM patients.
Do user-generated content and micro-celebrity posts encourage generation Z users to search online shopping behavior on social networking sites—the moderating role of sponsored ads
Authors: Shetu Sabakun Naher 
Published: December 2023

AbstractThe study investigates the impact of user-generated content (UGC) and micro-celebrity posts on the online purchasing behavior of Generation Z on social networking sites. In addition, the mediator function of the user’s search intent is investigated. Thus, sponsored ads employ moderation. To collect primary data, self-administered questionnaires and cross sectional studies were applied. The population is comprised of university students of Generation Z from Dhaka, Bangladesh. Systematic random sampling was used to select Generation Z members from six institutions in the city of Dhaka. Using convenience sampling, primary data were collected. The research yielded 565 standardized questionnaire samples. The study’s findings demonstrated that user-generated content’s direct, indirect, and mediation relationships were statistically significant. Moreover, the direct and indirect relationships of micro-celebrity posts were found to be statistically significant, but the mediation relationship was found to be insignificant. Additionally, users’ search intention has a strong correlation with online purchasing behavior. Thus, the moderation analysis of sponsored ads was deemed crucial. In Bangladesh, the stimulus-organism-response (S-O-R) paradigm is rarely applied to studies on the online purchasing preferences of Generation Z consumers. This study also examined its findings’ implications for future research and limitations.
Comprehensive index condition assessment of structural concrete based on surface wave techniques
Authors: Xu Xiang , Lan Jiasheng , Kırlangıç Ahmet Serhan , Polak Maria Anna 
Published: December 2023

AbstractTo reliably assess the condition of concrete structures, a surface wave-based comprehensive index has been developed. Considering various factors (e.g., aggregate types) influencing the surface wave-based characteristics, a relative index for concrete condition assessment has presented. To address the conflicts between individual indexes, the weight for each index is determined in accordance with its credibility. The effectiveness of the proposed methodology is verified by using test results from six concrete beams with different void volume ratios. Hereby three characteristics of surface waves (i.e., P- and R-wave velocities, and attenuation coefficient) are used to diagnose the concrete beams. It is found that the comprehensive index has the advantage in handling abnormal measurements of the individual indexes by reducing their weights to lower their influence on the final diagnosis. Comparison of the diagnoses based on the comprehensive index and individual indexes reveals that the comprehensive index performs much better than any other individual index. It matches greatly with the compressive strength of the concrete beams obtained from the destructive test. In conclusion, the proposed methodology presents a single comprehensive diagnostic feature with improved reliability on the fitness of the concrete structures for decision-makers.