bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Outcomes and Genetic Analyses of Restrictive Cardiomyopathy in Children
Authors: Ishida Hidekazu , Narita Jun , Ishii Ryo , Suginobe Hidehiro , Tsuru Hirofumi , Wang Renjie , Yoshihara Chika , Ueyama Atsuko , Ueda Kazutoshi , Hirose Masaki , Hashimoto Kazuhisa , Nagano Hiroki , Kogaki Shigetoyo , Kuramoto Yuki , Miyashita Yohei , Asano Yoshihiro , Ozono Keiichi 
Published: June 2023

BACKGROUND:
Restrictive cardiomyopathy in children is rare and outcomes are very poor. However, little information is available concerning genotype-outcome correlations.

METHODS:
We analyzed the clinical characteristics and genetic testing, including whole exome sequencing, of 28 pediatric restrictive cardiomyopathy patients who were diagnosed from 1998 to 2021 at Osaka University Hospital in Japan.

RESULTS:

The median age at diagnosis (interquartile range) was 6 (2.25–8.5) years. Eighteen patients received heart transplantations and 5 patients were on the waiting list. One patient died while waiting for transplantation. Pathologic or likely-pathogenic variants were identified in 14 of the 28 (50%) patients, including heterozygous
TNNI3
missense variants in 8 patients.
TNNT2
,
MYL2
, and
FLNC
missense variants were also identified. No significant differences in clinical manifestations and hemodynamic parameters between positive and negative pathogenic variants were detected. However, 2- and 5-year survival rates were significantly lower in patients with pathogenic variants (50% and 22%) compared with survival in patients without pathogenic variants (62% and 54%;
P
=0.0496, log-rank test). No significant differences were detected in the ratio of patients diagnosed at nationwide school heart disease screening program between positive and negative pathogenic variants. Patients diagnosed by school screening showed better transplant-free survival compared with patients diagnosed by heart failure symptoms (
P
=0.0027 in log-rank test).

CONCLUSIONS:

In this study, 50% of pediatric restrictive cardiomyopathy patients had pathogenic or likely-pathogenic gene variants, and
TNNI3
missense variants were the most frequent. Patients with pathogenic variants showed significantly lower transplant-free survival compared with patients without pathogenic variants.
Existence and the Problem of Aḥwāl: The Quiddity and Ontological Status of Existence in Avicenna and His Islamic Reception
Authors: Zamboni Francesco Omar 
Published: June 2023

Abstract
The Avicennian distinction between quiddity and existence opens the way to several derivative issues concerning the quiddity of existence (what existence is) and the ontological status of existence (whether and how existence is). This paper presents a fine-grained account of the positions and arguments developed by post-Avicennian authors on these matters, showing how the debates on states (aḥwāl) and grounding (taʿlīl) feed into the picture. The discussions on the quiddity of existence revolve around the features of its knowability and its connection to a ground (ʿilla), or lack thereof. As for the ontological status of existence, the standard idea of a clash between realism (existence is an extramental existent) and conceptualism (existence is a purely mental existent) calls for further refinement. First, realism itself encompasses two distinct positions when it comes to the relation between the second-order existence of existence and existence itself (sameness, additionality). Second, the tradition presents other doctrines not easily classifiable within the realism-conceptualism framework (the existential non-assertability of existence, the non-existence of existence).
Spatiotemporal evolution of ecological entropy in an ecologically vulnerable area
Authors: Hou Jingwei , Tan Yonghong , Hou Bo , Pang Xinyan 
Published: June 2023

Abstract
The spatiotemporal evolution of ecological entropy (EE) reflects the differences and correlations among the different elements (roads, rivers, land, etc.) and regions in an ecosystem. In this study, an index system for evaluating the EE in an ecologically vulnerable area in China from 2005 to 2020 is constructed according to the pressure–state–response model. Models of the EE and its gradient are constructed, and they are evaluated using GIS and remote sensing. The results show that except for the utilization rate of solid waste, the index values of EE have increased dramatically in the past 16 years. Investment in waste pollution control, GDP per capita, vegetation index in July, and utilization rate of solid waste make large contributions to the EE. The EEs on the edge of the ecologically vulnerable area increased from 2005 to 2020, indicating that the ecological environment in these areas has deteriorated year by year. The regions with the largest outflow of EE indicate that the EEs of these regions had a large impact on those of their surrounding regions. The regions with the largest inflow of EE indicate that the ecological security of these regions is relatively stable to the surrounding regions. Large EE gradients existed between adjacent regions show large rate of change and inflow and outflow of EE. The results contribute to alleviating the high entropy, reversing the ecological imbalance, enhancing the ecological benefits, and improving the environmental quality in an ecological entropy system. The present results offer decision support for ecological evaluation, protection, restoration, compensation, and security.
Racial/Ethnic Differences in the Predictive Utility of Psychosocial Determinants of COVID-19 Vaccination Intentions
Authors: Pierce Jace D. , Segundo Joahana , Derrick Jaye L. 
Published: June 2023

Purpose To test whether the impact of subjective norms, race/ethnicity-specific descriptive norms, vaccine conspiracy beliefs, and prosocial concern on COVID-19 vaccination intentions differs by race/ethnicity for young adults. Design Cross-sectional study. Setting May-August 2021 in Texas. Subjects Racially/ethnically diverse unvaccinated college students (N = 314). Measures COVID-19 vaccination intentions, theory-driven constructs (eg, perceived susceptibility), vaccine conspiracy beliefs, prosocial concern, and social norms. Analysis Block-sequential multiple Tobit regression. Results Results revealed three significant two-way interactions between race/ethnicity and (1) subjective norms, F (5, 251) = 2.28, P < .05; (2) COVID-19 vaccine conspiracy beliefs, F (5, 251) = 2.88, P < .05; and (3) prosocial concern, F (5, 251) = 2.61, P < .05. There was a positive association between subjective norms and intentions for European and African Americans, a positive association between prosocial concerns and intentions for European and multiracial/multiethnic Americans, and a negative association between conspiracy beliefs and intention for Hispanics. The interaction between race/ethnicity and race/ethnicity-specific descriptive norms was not significant, F (5, 251) = 1.09, P = .37. Conclusion Although based on a relatively small sample, our findings suggest the importance of culturally tailoring COVID-19 vaccination messages to correct conspiracy beliefs, signaling a positive subjective norm, and enhancing prosocial concerns for specific racial-ethnic groups.
The marginalization of policy integration: Dynamics of integrated policymaking in the periphery of bureaucracy
Authors: Joosse Hans , van Buuren Arwin 
Published: June 2023

Governmental organizations face the challenge to integrate different sectoral policy perspectives and interests to deal effectively with the wicked issues of late-modern societies. This article delves into attempts of governmental organizations to realize such integrated policies and unravels the intra-organizational dynamics of collaborating on policy integration. Based on two in-depth case studies from The Netherlands, one focusing on public transport for specific target groups in the city of Rotterdam and the other focusing on a national policy program for criminal youth groups, we show how attempts at policy integration take place in the periphery of bureaucracies by temporary program teams. After the abolishment of the program team, the integrated policy easily volatilizes because of the lack of foothold and ownership in the line organization. Ironically, policy integration becomes a differentiated activity in the margins of public organizations rather than a joint exercise of sectoral organizational units. We present three explanatory hypotheses of this dynamic of marginalization. First, from a system-psychodynamic perspective, the line organization and the program team maintain distance from each other to reduce the tensions that are inherent in policy integration. Second, from an institutional perspective, the line organization marginalizes policy integration to protect their sectoral and vested interests. Third, from an innovation perspective, however, innovative policy integration does need the margins of organizations to protect itself against conservative reflexes from bureaucracies at risk of becoming marginalized and alienated. To facilitate a productive dynamic of policy integration in governmental organizations, this article concludes with providing the components for such an organizational design.
Stratification of Risk Factors of Lung Cancer-Associated Venous Thromboembolism and Determining the Critical Point for Preemptive Intervention: A Systematic Review With Meta-analysis
Authors: Nwagha Theresa , Nweke Martins 
Published: June 2023

Background: Several biomarkers or risk factors have been identified and several prediction models exist. The major limitations inherent in these models include cost-ineffectiveness and lack of systematic stratification of risk factors resulting in the inclusion of clinically insignificant biomarkers in the models. This review aimed to systematically stratify the risk factors of lung cancer-associated venous thromboembolism (VTE) and determine the critical point for preemptive intervention. Methods: This systematic review was structured as per the Preferred Reporting Item for Systematic Reviews and Meta-analyses. We searched MEDLINE, PubMed, Cochrane Library, CINAHL, Academic Search Complete, and PsycINFO from the onset to June 2022. We included studies that reported the risk factors of lung cancer-associated VTE and corresponding risk estimates, irrespective of treatment status but studies were excluded if patients were on anti-VTE medications. We employed random effects models of meta-analysis and computed risk stability index and risk weight (Rw) to achieve the review objectives. The review protocol is registered with PROSPERO (CRD42022336476). Results: The clinically significant risk factors of VTE in lung cancer patients were D-dimer (odds ratio [OR] = 5.510, 95% CI = 2.6-11.7; Rw = 5.0), albumin (OR = 2.2, 95% CI = 1.0-4.8; Rw = 1.79), leukocyte (OR = 2.48, 95% CI = 1.9-3.2; Rw = 1.77), histological type (OR = 1.69 , 95% CI = 1.2-2.4; Rw = 1.3), age (OR = 1.56; Rw = 0.99), and hemoglobin (OR = 1.85, 95% CI = 1.3-2.6; Rw = 0.92). Based on the distribution of Rw across risk factors, the critical point (upper third of the upper quartile class) was 4.5 and may mark the point at which preemptive intervention should be commenced. Conclusions: Targeted screening for VTE in lung cancer patients could be patient-specific and should be based on a combination of the most significant risk factors required to meet the critical point, provided that such a combination is affordable as illustrated in the ALBAH model. Registration: The review protocol is registered with PROSPERO (ID: CRD42022336476).
Practice patterns in diagnostics, staging, and management strategies of gallbladder cancer among Nordic tertiary centers
Authors: Takala Sini , Lassen Kristoffer , Søreide Kjetil , Sparrelid Ernesto , Angelsen Jon-Helge , Bringeland Erling A. , Eilard Malin S. , Hemmingsson Oskar , Isaksson Bengt , Karjula Heikki , Lammi Jukka-Pekka , Larsen Peter N. , Lavonius Maija , Lindell Gert , Mortensen Frank V. , Mortensen Kim , Nordin Arno , Pless Torsten , Sandström Per , Sandvik Oddvar , Vaalavuo Yrjö , Villard Christina , Sallinen Ville 
Published: June 2023

Background and objective: Gallbladder cancer (GBC) is a rare malignancy in the Nordic countries and no common Nordic treatment guidelines exist. This study aimed to characterize the current diagnostic and treatment strategies in the Nordic countries and disclose differences in these strategies. Methods: This was a survey study with a cross-sectional questionnaire of all 19 university hospitals providing curative-intent surgery for GBC in Sweden, Norway, Denmark, and Finland. Results: In all Nordic countries except Sweden, neoadjuvant/downstaging chemotherapy was used in GBC patients. In T1b and T2, majority of the centers (15–18/19) performed extended cholecystectomy. In T3, majority of the centers (13/19) performed cholecystectomy with resection of segments 4b and 5. In T4, majority of the centers (12–14/19) chose palliative/oncological care. The centers in Sweden extended lymphadenectomy beyond the hepatoduodenal ligament, whereas all other Nordic centers usually limited lymphadenectomy to the hepatoduodenal ligament. All Nordic centers except those in Norway used adjuvant chemotherapy routinely for GBC. There were no major differences between the Nordic centers in diagnostics and follow-up. Conclusions: The surgical and oncological treatment strategies of GBC vary considerably between the Nordic centers and countries.
‘GITA’ Cannot wait…
Authors: Krishnan Ganesh 
Published: June 2023

The author is a career banker with over 30 years of working experience. The job has given him an opportunity to interact extensively with people from all parts of India. One similarity that is profound, cutting across the landscape of this country, be it a farmer in a rural village in Gujarat, Executive Director of a Public Sector Undertaking in Delhi, a start-up entrepreneur in Bengaluru, or a motivational speaker in Chennai, the Indian DNA is dominated by Karma Theory and we Indians live the ‘Gita’ subconsciously. The article is an attempt to highlight the same for the students and the youth of our country. The work is subsequent to the current government’s announcement regarding the introduction of Gita as a part of the academic curriculum and the protests that followed against its implementation. The work brings out that Gita is a psychological treatise cutting across age, sex and affiliations. The work breaks the myth that Gita is post-retirement entertainment and establishes that it is a weapon that enables an individual to fight the daily battles of life. The Gita is the nerve centre of Indian culture and has influenced its civilisation. Gita meets the spiritual, emotional and cultural needs of a seeker and enhances resilience of the individual. So, hurry up, as Gita cannot wait. ‘GITA’ Cannot wait ….
Personalization and Learning Outcome in a Nursing Pathophysiology and Pharmacology Course: Canvas© Mastery Adoption Pilot Study
Authors: Hinkle Julie F. 
Published: June 2023

Introduction Personalized learning has been shown to improve learning outcomes. The aim of this pilot was to test a tool embedded in the Canvas© learning platform to improve personalization and collect data to see if the available level of personalization employed improved learning outcomes. Methods A nursing pathophysiology and pharmacology course was redesigned using the Canvas© Mastery Paths feature to provide personalized learning content to students. Post-class quiz grades were used to trigger Canvas© to conditionally release content review materials to students who did poorly on the quiz and provide a second quiz to test the efficacy of supplementary review materials. Data from the redesigned course was compared to the previous semester's course data. Results Use of Canvas© Master Paths to conditionally release supplementary material to poorly performing students resulted in significant improvement in course grades and certain activity scores correlated with improved course and ATI© quiz means. Conclusion The degree of personalization of course content available with Master Paths has the potential to have a positive impact on learning outcomes.
Subclonal TP53 mutations are frequent and predict resistance to radioimmunotherapy in follicular lymphoma
Authors: Burack W. Richard , Li Hongli , Adlowitz Diana , Spence Janice M. , Rimsza Lisa M. , Shadman Mazyar , Spier Catherine M. , Kaminski Mark S. , Leonard John P. , Leblanc Michael L. , Smith Sonali M. , Friedberg Jonathan W. 
Published: June 2023

Abstract
Although TP53 is commonly mutated in transformed follicular lymphoma, mutations are reported in &lt;5% of pretreatment follicular lymphoma (FL) specimens. We assayed archival follicular B-cell non-Hodgkin lymphoma specimens from a completed clinical trial, Southwest Oncology Group S0016, a phase 3 randomized intergroup trial of CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone) chemotherapy plus R-CHOP (rituximab-CHOP) compared with CHOP chemotherapy plus 131-iodine tositumomab (radioimmunotherapy [RIT]-CHOP). Subclonal TP53 mutations (median allele frequency 0.02) were found in 25% of diagnostic FL specimens and in 27% of a separate validation cohort. In the R-CHOP arm, pathogenic TP53 mutations were not associated with progression-free survival (PFS) (10-year PFS 43% vs 44%). In contrast, among patients with no detectable pathogenic TP53 mutation, RIT-CHOP was associated with a longer PFS than with R-CHOP (10-year PFS 67% vs 44%; hazard ratio = 0.49; P = .008). No relationship was detected between PFS and the extent of activation-induced cytidine deaminase (AICDA)–mediated heterogeneity. In summary, subclonal TP53 mutations are common in FL and are a distinct phenomenon from AICDA-mediated genetic heterogeneity. The absence of a detectable subclonal mutation in TP53 defined a population that particularly benefited from RIT.
What can we learn from biodegradation of natural polymers for regulation?
Authors: Hahn Stefan , Hennecke Dieter 
Published: June 2023

AbstractSynthetic polymers are chemicals of emerging concern for the environment, which is mainly attributed to their persistence in environmental compartments. On the other hand, polymers exist in nature as well. They are regarded of no concern for the environment. The present article focusses on the environmental fate of natural polymers and the implications on the persistence assessment for synthetic polymers. Natural polymers vary widely in structure, function, and properties. Crystallinity, wettability and surface area are important influencing factors on the (bio)degradation kinetics. Chemical and enzymatic hydrolysis is the most important process for the degradation of natural polymers, which for particulate material takes place either by bulk or surface erosion. Some natural polymers are degraded rapidly, but in other cases, degradation of natural polymers takes very long until complete mineralization. These differences in biodegradability are a result of evolution as they have to fulfil specific functions in nature which might require a certain persistency. Consequently, many natural polymers would have to be considered persistent or very persistent (P or vP) based on the available studies using the standard assessment approach. At the same time, they are considered no concern to the environment for good reasons. The analysis emphasizes that mineralization alone is not a resilient persistence endpoint for polymers. This needs to be recognized when assessing synthetic polymers, many of which would fulfil the existing P/vP criteria as well. For such synthetic polymers, it would be important to establish meaningful endpoints and polymer specific criteria to enable an adequate persistence assessment using a weight-of-evidence approach.
The effect-site concentration of remifentanil blunting endotracheal intubation responses during anesthesia induction with etomidate: a dose-finding study
Authors: Jiang Zhencong , Xiao Jun , Wang Xiaoqing , Luo Tao 
Published: June 2023

Abstract
Background
Remifentanil can inhibit the hemodynamic responses caused by endotracheal intubation, but the effect-site concentration of it required to control intubation responses when combined with etomidate has not been demonstrated. The purpose of this study was to determine the effect-site concentration of remifentanil blunting tracheal intubation responses in 50% and 95% of patients (EC50 and EC95) during etomidate anesthesia.

Methods
American Society of Anesthesiologists physical status (ASA) I-II elective surgical patients receiving target-controlled infusion (TCI) of remifentanil, followed by etomidate and rocuronium for anesthesia were enrolled. The Belive Drive A2 monitor was used to calculate the MGRSSI (Maygreen Sedative state index) of hypnotic effect and the MGRNOX (Maygreen Nociception index) of nociception. The MGRSSI and the MGRNOX value were generated every 1 s. Mean arterial pressure (MAP) and heart rate (HR) were measured every minute, noninvasively. Using the modified Dixon’s up-and-down method, the concentration of remifentanil was determined based on the intubation response of the previous patient. The cardiovascular response during endotracheal intubation was defined as positive when MAP or HR is 20% higher than the pre-intubation value. A probit analysis was used for calculating EC50, EC95 and 95% confidence interval (CI).

Results
The EC50 and EC95 of remifentanil blunting tracheal intubation responses were found to be 7.731 ng/ml (95%CI: 7.212–8.278 ng/ml) and 8.701 ng/ml (95%CI: 8.199–11.834 ng/ml). There were statistically significant increases in HR, MGRSSI and MGRNOX value to tracheal intubation in the positive responses group compared to the negative group. The most common adverse event was postoperative nausea and vomiting, which occurred in 3 patients.

Conclusion
Remifentanil effect-site concentration of 7.731 ng/ml is effective in blunting sympathetic responses to tracheal intubation in 50% of patients when combined with etomidate anesthesia.

Trial registration
The trial was registered at the Chinese Clinical Trials Registry (www.chictr.org.cn, registration number: ChiCTR2100054565, date of registration: 20/12/2021).