bluuebunny/crossref_metadata_2025_split huggingface dataset:
SOAL MODEL PISA KONTEN SPACE & SHAPE UNTUK MENUNJANG KEMAMPUAN BERPIKIR MATEMATIS PESERTA DIDIK
Authors: Satiti Wisnu Siwi , Lathifah Afifatul , Nasrulloh M Farid 
Published: September 2024

In order to develop students' mathematical abilities, a learning should not only emphasize mastery of the material, but mathematical activities should also support the development of students' ability to think mathematically. This due to mathematical thinking is important for a student's academic success and is also an ability needed in the role of each individual in society. One of the mathematical activities that support students' mathematical thinking skills is PISA like mathematics problems. One of the mathematical content in PISA is about space and shape. However, several previous studies have shown that many Indonesian students have difficulty in solving PISA like mathematics problems of space and shape content Therefore, in this study, PISA like mathematics problems of space and shape content was developed for students at the SMP/MTs level. The product is used as a mathematical activity in learning at school as an effort to support students' mathematical thinking. This study uses the Research and Development (R&D) method with the ADDIE model. The product trial results show that the product developed is valid and appropriate to support students' mathematical thinking. The final product received a response with "Good" criteria from students.
Perubahan Peran Suami dan Isteri dalam Keluarga di Desa Molinese Kecamatan Lainea Kabupaten Konawe Selatan
Authors: Al Kausar Muhamad , Desrianti Desrianti 
Published: September 2024

The purpose of this research is to find out how the roles of husbands and wives in the family and what the implications of changes in the roles of husbands and wives are for the family. The selection of informants in this study used the purposive sampling technique . This research uses the Theory of Social Change by William F. Ogburn. The data collection technique used in this research is field work using two methods, namely observation and in-depth interviews. This research is qualitative research using ethnographic research methods. The results of this study show that, role change in Molinese Village has slowly occurred in the 2000s and above and there are several families who experience it because of the lack of income in the family so that it affects the economy. However, with the entry of the oil palm company in this village in 2007, immediately the wives flocked to work as employees of the oil palm plantation as well as the husbands, but over time many husbands stopped working because of the wage system that was considered uneven and they chose to do gardening and some did not have a permanent job. Meanwhile, the wives are still working as employees of the oil palm plantation or PT MERBAU, until now. Some of the implications of changes in the role of husbands and wives on the family are the lack of harmony in the household, not having a tire, and the lack of a permanent job.
Elevated remnant cholesterol as a potential predictor for cardiovascular events in rheumatoid arthritis patients
Authors: Chang Ching-Kun , Li Yi-Chen , Chen Po-Ku , Chang Shih-Hsin , Chen Der-Yuan 
Published: September 2024

ObjectiveThe risk of cardiovascular disease (CVD) in patients with rheumatoid arthritis (RA) remains inadequately defined. Consequently, this study aims to evaluate the predictive value of remnant cholesterol (RC) for assessing CVD risk in RA patients.MethodsPlasma RC levels were measured in 114 RA patients and 41 healthy controls, calculated as total cholesterol minus HDL-C and LDL-C. These levels were further analyzed using 1H-NMR lipid/metabolomics. Meanwhile, the 28-joint Disease Activity Score (DAS28) assessed RA activity.ResultsRC levels were significantly elevated in RA patients (19.0 mg/dl, p &lt; 0.001) compared to healthy controls (14.5 mg/dl). Furthermore, RC levels were significantly elevated at 37.4 mg/dl in patients who experienced cardiovascular event (CVE) compared to 17.4 mg/dl in those without CVE (p &lt; 0.001). To enhance the precision and reliability of RC measurements, RC concentrations were further validated using 1H-NMR spectroscopy. Additionally, a positive correlation was observed between RC levels and DAS28. Multivariate analysis identified RC as a significant predictor of CVE (odds ratio = 1.82, p = 0.013). ROC curve analysis revealed superior predictive capability of RC for CVE (AUC = 0.919, p &lt; 0.001) compared to LDL-C (AUC = 0.669, p = 0.018), with a high sensitivity of 94.7% and a specificity of 82.1%.ConclusionElevated RC levels demonstrate greater accuracy in predicting CVE occurrence in RA patients compared to traditional measures such as LDL-C. These findings suggest that elevated RC levels may serve as a novel predictor for occurrence of CVE in RA patients, facilitating early intervention strategies based on the risk stratification.
Correction of osteopetrosis in the neonate oc/oc murine model after lentiviral vector gene therapy and non-genotoxic conditioning
Authors: Penna Sara , Zecchillo Alessandra , Di Verniere Martina , Fontana Elena , Iannello Valeria , Palagano Eleonora , Mantero Stefano , Cappelleri Andrea , Rizzoli Elena , Santi Ludovica , Crisafulli Laura , Filibian Marta , Forlino Antonella , Basso-Ricci Luca , Scala Serena , Scanziani Eugenio , Schinke Thorsten , Ficara Francesca , Sobacchi Cristina , Villa Anna , Capo Valentina 
Published: September 2024

IntroductionAutosomal recessive osteopetrosis (ARO) is a rare genetic disease, characterized by increased bone density due to defective osteoclast function. Most of the cases are due to TCIRG1 gene mutation, leading to severe bone phenotype and death in the first years of life. The standard therapy is the hematopoietic stem cell transplantation (HSCT), but its success is limited by several constraints. Conversely, gene therapy (GT) could minimize the immune-mediated complications of allogeneic HSCT and offer a prompt treatment to these patients.MethodsThe Tcirg1-defective oc/oc mouse model displays a short lifespan and high bone density, closely mirroring the human condition. In this work, we exploited the oc/oc neonate mice to optimize the critical steps for a successful therapy.ResultsFirst, we showed that lentiviral vector GT can revert the osteopetrotic bone phenotype, allowing long-term survival and reducing extramedullary haematopoiesis. Then, we demonstrated that plerixafor-induced mobilization can further increase the high number of HSPCs circulating in peripheral blood, facilitating the collection of adequate numbers of cells for therapeutic purposes. Finally, pre-transplant non-genotoxic conditioning allowed the stable engraftment of HSPCs, albeit at lower level than conventional total body irradiation, and led to long-term survival and correction of bone phenotype, in the absence of acute toxicity.ConclusionThese results will pave the way to the implementation of an effective GT protocol, reducing the transplant-related complication risks in the very young and severely affected ARO patients.
De novo and inherited micro-CNV at 16p13.11 in 21 Chinese patients with defective cardiac left-right patterning
Authors: Yu Kun , Chen Weicheng , Chen Yan , Shen Libing , Wu Boxuan , Zhang Yuan , Zhou Xiangyu 
Published: September 2024

ObjectiveCopy number changes at Chromosomal 16p13.11 have been implicated in a variety of human diseases including congenital cardiac abnormalities. The clinical correlation of copy number variants (CNVs) in this region with developmental abnormalities remains controversial as most of the patients inherit the duplication from an unaffected parent.MethodsWe performed CNV analysis on 164 patients with defective left-right (LR) patterning based on whole genome-exome sequencing (WG-ES) followed by multiplex ligation-dependent probe amplification (MLPA) validation. Most cases were accompanied with complex congenital heart disease (CHD).ResultsCNVs at 16p13.11 were identified in a total of 21 cases, accounting for 12.80% (21/164) evaluated cases. We observed a marked overrepresentation of chromosome 16p13.11 duplications in cases when compared with healthy controls according to literature reports (15/164, 9.14% versus 0.09% in controls). Notably, in two independent family trios, de novo 16p13.11 micro-duplications were identified in two patients with laterality defects and CHD. Moreover, 16p13.11 micro-duplication was segregated with the disease in a family trio containing 2 affected individuals. Notably, five coding genes, NOMO1, PKD1P3, NPIPA1, PDXDC1, and NTAN1, were potentially affected by micro-CNV at 16p13.11 in these patients.ConclusionOur study provides new family-trio based evidences to support 16p13.11 micro-duplications predispose individuals to defective cardiac left-right patterning and laterality disorder.
Cost-effectiveness of first-line enfortumab vedotin in addition to pembrolizumab for metastatic urothelial carcinoma in the United States
Authors: Li Andong , Wu Meiyu , Xie Ouyang , Xiang Heng , Meng Kehui , Tan Chongqing , Wang Long , Wan Xiaomin 
Published: September 2024

Background and objectiveThe EV-302 trial found that the combination of enfortumab vedotin (EV) with pembrolizumab significantly improved survival for patients with metastatic urothelial carcinoma (mUC). However, given the high cost of the drugs, there is a need to assess its value by considering both efficacy and cost. This study assessed the cost-effectiveness of EV plus pembrolizumab as a first-line treatment for patients with mUC from the perspective of U.S. payers.MethodsA Markov model was developed to compare the lifetime costs and effectiveness of EV in combination with pembrolizumab with chemotherapy in the treatment of mUC patients from U.S. payer perspective. Life-years (LYs), quality-adjusted LYs (QALYs), and lifetime costs were estimated. One-way, two-way and probabilistic sensitivity analyses were conducted to evaluate model uncertainty. Additionally, subgroup analyses were performed.ResultsCompared to chemotherapy, the combination of EV and pembrolizumab provided an additional 2.10 LYs and 1.72 QALYs, at an incremental cost of $962,240.8 per patient. The incremental cost-effectiveness ratio (ICER) is $558,973 per QALY. Subgroup analysis indicated that patients ineligible for cisplatin treatment had a lower ICER compared to those who were eligible for cisplatin.ConclusionsFrom the perspective of US payers, at a willingness-to-pay threshold of $150,000 per QALY, the combination of EV and pembrolizumab is estimated to not be cost-effective compared to traditional chemotherapy in the first-line treatment of mUC patients.
Case reports: Intraoperative migratory retinal venous thrombus in proliferative diabetic retinopathy
Authors: Lyu Danni , Liu Huan , Fang Yijiong , Wang Yao 
Published: September 2024

PurposeThis study aimed to study the characteristics, possible causes, and clinical implications of intraoperative migratory retinal venous thrombus in proliferative diabetic retinopathy (PDR).CasesTwo middle-aged Chinese patients with diabetes mellitus presented with blurred vision and were diagnosed with PDR and tractional retinal detachment (TRD). An interesting phenomenon was observed during pars plana vitrectomy in both patients. Movement of tiny white thrombi and interruption of blood flow were observed in a branch of the central retinal vein when the vein was pulled at the time of fibrovascular membrane delamination and disappeared with the elimination of retinal traction after finishing the process of delamination. Laboratory studies revealed abnormal erythrocyte sedimentation rate, fibrinogen, D-dimer, international normalized ratio, and IgA anti-β2-glycoprotein I in one patient and elevated fibrinogen and IgA anticardiolipin in the other. Follow-up examinations at 1 week, 1, 3, and 6 months postoperatively showed good prognosis. Fluorescein fundus angiography at 1 month postoperatively showed neither embolus sign nor prolonged venous filling time in both patients.DiscussionLocal blood stasis of the retinal vein persistently dragged by the fibrovascular membrane may result in thrombogenesis, and traction of the retina during the delamination process may lead to the movement of thrombi. On the other hand, endothelial injury and disordered local blood stasis during delamination may also activate the biological coagulation process and instant thrombus formation. As well, antiphospholipid antibodies may also be a risk factor of ocular thrombogenesis.ConclusionThis study provides the first videos recording migratory thrombus in terminal vessels, which indicates that fibrovascular membrane in PDR can lead to thrombogenesis due to dragging and hemostasis of the involved retinal vein. PDR patients with fibrovascular membranes may benefit from early relief of vascular traction through fibrovascular membrane delamination.
Case report: Development of clonal hematologic disorders from inherited bone marrow failure
Authors: Cermak Jaroslav 
Published: September 2024

IntroductionInherited bone marrow failure (IBMF) syndromes are caused by mutations forming pathologic germline variants resulting in the production of defective hematopoietic stem cells (HSC) and in congenital failure in the production of one or more blood lineages. An acquisition of subsequent somatic mutations is determining further course of the disease. Nevertheless, a certain number of patients with IBMF may escape correct diagnosis in childhood, especially those with mild cytopenia and minimal clinical features without non-hematologic symptoms. These patients usually present in the third decade of life with unexplained cytopenia or myelodysplastic syndrome (MDS).Methods and resultsWe report 2 patients with IBMF who were correctly diagnosed between 20 and 40 years of age when they were referred with progressive MDS with adverse prognostic factors that affected their outcome.DiscussionIBMF syndromes should be excluded in all patients below 40 years of age with unexplained cytopenia. Early hematopoietic stem cell transplantation (HSCT) is the treatment of choice in these patients.
Tumor lysis syndrome signal with the combination of encorafenib and binimetinib for malignant melanoma: a pharmacovigilance study using data from the FAERS database
Authors: Xia Shuang , Xu Jing-Wen , Yan Kang-Xin , Noguchi Yoshihiro , Sarangdhar Mayur , Yan Miao 
Published: September 2024

ObjectiveTo investigate the potential association between tumor lysis syndrome (TLS) and drugs for the treatment of malignant melanoma (MM).MethodsReports of TLS recorded in the FDA Adverse Event Reporting System (FAERS) (January 2004–2023q3) were identified. Demographic and clinical characteristics were described, and disproportionality signals were assessed through the Reporting Odds Ratio (ROR) and Information Component (IC). The latency of TLS with anticancer drugs was described based on parametric models. Subgroup analysis was conducted to explore the differences of TLS signals in different age and sex.ResultsWe found 5 (1.49%), 59 (17.61%), 79 (23.58%), 19 (5.67%), 13 (3.88%), 13 (3.88%), 33 (9.85%), 49 (14.63%), 16 (4.78%) TLS reports with pembrolizumab, nivolumab, ipilimumab, dabrafenib, vemurafenib, dacarbazine, “encorafenib and binimetinib”, “nivolumab and ipilimumab”, “dabrafenib and trametinib”, respectively. The combination of encorafenib and binimetinib showed the strongest signal of TLS (IC025 = 3.98). The median days of latency of TLS with combination of encorafenib and binimetinib is 2 days, which was much shorter than nivolumab (22.0 days) and ipilimumab (21.5 days). TLS cases associated with drugs for MM were predominantly recorded in females and aged 25–65 years. After excluding confounding factors such as pre-existing diseases and co-treated drugs, the disproportionate signal of TLS with “encorafenib and binimetinib” remained strong.ConclusionsStronger disproportionate signal of TLS was detected in MM patients using the combination of encorafenib and binimetinib than other drugs. Further research is needed to investigate the underlying mechanisms and identify patient-related predisposing factors to support safe prescribing of the combination of encorafenib and binimetinib.
Slovenian validation of the Capacity to Love Inventory: associations with clinical measures and mindfulness
Authors: Glavač Timotej , Poštuvan Vita , Schmeckenbecher Jim , Kapusta Nestor D. 
Published: September 2024

AimThe main purpose of the present study was to validate the Slovenian version of the 41- item Capacity to Love Inventory (CTL-I). Based on psychoanalytic theory, limitations to capacity to love are expected to be associated with personality dysfunction and disintegration as well as fundamental mental capacities such as self-reflection and self-awareness.MethodTo examine these assumptions, a sample of 552 Slovenian non-clinical individuals were recruited through academic networks. The construct validity of the CTL-I was assessed using a confirmatory factor analysis and convergent validity of the CTL-I and its subscales was established against IPO-16, PID-5 BF, MAAS.ResultsOur findings show that the Slovenian version of the CTL-I replicated the six-factor structure, exhibiting good model fit as well as satisfactory internal consistency of all subscales. In line with expectations, capacity to love was found to be inversely associated with dysfunctional personality traits and structural personality disturbances. Accordingly, higher dispositional mindfulness was coherently associated with all domains of CTL-I.ConclusionThe results add to the growing evidence for the cross-cultural validity and sound psychometric properties of CTL-I, presented here in the Slovenian version. Our findings also point to the significance of dispositional mindfulness both in relation to capacity to love as well as mental health.
Internal blood lead exposure levels in permanent residents of Jiangxi Province and its effects on routine hematological and biochemical indices
Authors: He Wenxin , Fu Junjie , Fu Ruiyi , Song Xiaoguang , Huang Siyue , Wang Yujue , Lu Keke , Wu Hao 
Published: September 2024

BackgroundLead exposure levels are closely linked to human health and can cause damage to multiple organ systems, including the blood system and liver. However, due to insufficient evidence, the effects of lead exposure on hematological and biochemical indices have not been fully established.ObjectiveThis study aims to explore the blood lead levels of permanent residents in Jiangxi Province and analyze the factors affecting blood lead levels and the impact of blood lead levels on hematological and biochemical indices.MethodsWe conducted a cross-sectional study including questionnaires, health examinations, and blood sample examinations on 720 randomly selected permanent residents (3–79 years) in Jiangxi Province in 2018. The blood lead levels were measured using inductively coupled plasma mass spectrometry. Routine hematological and biochemical tests were determined by qualified medical institutions using automated hematology analyzers and biochemistry analyzers.ResultsThe geometric mean of blood lead concentration in permanent residents of Jiangxi Province was 20.45 μg/L. Gender, age, annual household income, smoking, and hypertension were the influencing factors for blood lead levels. For each 1 μg/L increase in blood lead, the risks of elevated red blood cell count (from low to high), platelet volume distribution width, alkaline phosphatase (from low to high), and cholesterol increased by 2.4, 1.6, 3.6, and 2.3%, respectively, whereas the risks of elevation of direct bilirubin and total bilirubin both decreased by 1.7%.ConclusionThe blood lead level in permanent residents of Jiangxi Province is higher than the national average. Higher blood lead levels were found in men than in women; blood lead levels were positively correlated with age but negatively correlated with annual household income; smoking and hypertension are risk factors for elevated blood lead; and blood lead levels affect routine hematological and biochemical markers such as red blood cell count, platelet volume distribution width, direct bilirubin, total bilirubin, alkaline phosphatase, and cholesterol.
Early detection of students’ mental health issues from a traditional daily health observation scheme in Japanese schools and its digitalization
Authors: Nishimura Tomoko , Wakuta Manabu , Osuka Yuko , Tsukui Nobuaki , Hirata Ikue , Takahashi Michio , Adachi Masaki , Katayama Taiichi , Aizaki Kyoko , Sumiya Motofumi , Kawakami Sayaka , Iwabuchi Toshiki , Senju Atsushi 
Published: September 2024

ObjectiveThe implementation of school-based mental health screening offers promise for early detection of mental health issues in children; however, various barriers hinder its widespread adoption. This study aimed to investigate the predictive value of digital data obtained from an established daily health observation scheme in Japanese schools to identify later mental health issues in children.MethodsData for the analysis were obtained from 2,433 students enrolled in five public schools. The data acquisition period spanned 76 school days, from September 1, 2022, to December 23, 2022, and student absences were recorded during this period. Depressive and anxiety symptoms were assessed in January 2023. The students’ daily physical and emotional health status was recorded as “daily health issue” scores and group-based trajectory modeling was employed to classify the long-term trends in these scores. Additionally, rolling z-scores were utilized to capture variability in daily health issue scores, with z-scores above +1 considered unusual responses.ResultsAfter 4 months of daily health observations, students’ response trends were classified into five trajectory groups. The group experiencing the highest number of daily health issues (Group 5; 5.4% of the sample) exhibited more subsequent depressive and anxiety symptoms compared to the group with fewer issues (Group 1; 47.5%) (incident rate ratio [IRR] = 5.17; 95% confidence interval [CI]: 3.82, 6.99). Group 5 also demonstrated significantly more days of absence than Group 1 (IRR = 2.14, 95% CI: 1.19, 3.85). The average daily health issue scores for the entire period were associated with both depressive/anxiety symptoms and the number of days absent from school (IRR = 1.59, 95% CI: 1.45, 1.73; IRR = 1.18, 95% CI: 1.04, 1.35, respectively). Furthermore, a higher number of unusual responses during the entire period was also associated with more depressive/anxiety symptoms (IRR = 1.10, 95% CI: 1.07, 1.12).ConclusionThe current study is the first to demonstrate the predictive capability of a traditional daily health observation scheme to identify mental health issues in children. This study highlights the scheme’s potential to screen and safeguard children’s mental health, emphasizing the importance of digitalization and collaboration with various stakeholders.