bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mast cell leukemia: clinical and molecular features and survival outcomes of patients in the ECNM Registry
Authors: Kennedy Vanessa E. , Perkins Cecelia , Reiter Andreas , Jawhar Mohamad , Lübke Johannes , Kluin-Nelemans Hanneke C. , Shomali William , Langford Cheryl , Abuel Justin , Hermine Olivier , Niedoszytko Marek , Gorska Aleksandra , Mital Andrzej , Bonadonna Patrizia , Zanotti Roberta , Tanasi Ilaria , Mattsson Mattias , Hagglund Hans , Triggiani Massimo , Yavuz Akif Selim , Panse Jens , Christen Deborah , Heizmann Marc , Shoumariyeh Khalid , Müller Sabine , Elena Chiara , Malcovati Luca , Fiorelli Nicolas , Wortmann Friederike , Vucinic Vladan , Brockow Knut , Fokoloros Christos , Papageorgiou Sotirios G. , Breynaert Christine , Bullens Dominique , Doubek Michael , Ilerhaus Anja , Angelova-Fischer Irena , Solomianyi Oleksii , Várkonyi Judit , Sabato Vito , Rüfer Axel , Schug Tanja Daniela , Hermans Maud A. W. , Fortina Anna Belloni , Caroppo Francesca , Bumbea Horia , Gulen Theo , Hartmann Karin , Elberink Hanneke Oude , Schwaab Juliana , Arock Michel , Valent Peter , Sperr Wolfgang R. , Gotlib Jason 
Published: September 2022

Abstract
Mast cell leukemia (MCL) is a rare subtype of systemic mastocytosis defined by ≥20% mast cells (MC) on a bone marrow aspirate. We evaluated 92 patients with MCL from the European Competence Network on Mastocytosis registry. Thirty-one (34%) patients had a diagnosis of MCL with an associated hematologic neoplasm (MCL-AHN). Chronic MCL (lack of C-findings) comprised 14% of patients, and only 4.5% had “leukemic MCL” (≥10% circulating MCs). KIT D816V was found in 62/85 (73%) evaluable patients; 9 (11%) individuals exhibited alternative KIT mutations, and no KIT variants were detected in 14 (17%) subjects. Ten evaluable patients (17%) had an abnormal karyotype and the poor-risk SRSF2, ASXL1, and RUNX1 (S/A/R) mutations were identified in 16/36 (44%) patients who underwent next-generation sequencing. Midostaurin was the most common therapy administered to 65% of patients and 45% as first-line therapy. The median overall survival (OS) was 1.6 years. In multivariate analysis (S/A/R mutations excluded owing to low event rates), a diagnosis of MCL-AHN (hazard ratio [HR], 4.7; 95% confidence interval [CI], 1.7-13.0; P = .001) and abnormal karyotype (HR, 5.6; 95% CI, 1.4-13.3; P = .02) were associated with inferior OS; KIT D816V positivity (HR, 0.33; 95% CI, 0.11-0.98; P = .04) and midostaurin treatment (HR, 0.32; 95% CI, 0.08-0.72; P = .008) were associated with superior OS. These data provide the most comprehensive snapshot of the clinicopathologic, molecular, and treatment landscape of MCL to date, and should help further inform subtyping and prognostication of MCL.
KDELR2-KIF20A axis facilitates bladder cancer growth and metastasis by enhancing Golgi-mediated secretion
Authors: Meng Xiangui , Li Weiquan , Yuan Hongwei , Dong Wei , Xiao Wen , Zhang Xiaoping 
Published: September 2022

Abstract
Background
Bladder cancer (BCa) is a fatal form of cancer worldwide associated with a poor prognosis. Identifying novel drivers of growth and metastasis hold therapeutic potential for the disease. Transport homeostasis between the endoplasmic reticulum and Golgi and the secretion of matrix metalloproteinases (MMPs) mediated by Golgi have been reported to be closely associated with tumor progression. However, to date, mechanistic studies remain limited.

Results
Here, we identified KDELR2 as a potential risk factor with prognostic value in patients with BCa, especially those harbouring the KDELR2 amplification. In addition, we found that KDELR2 is a regulator of BCa cell proliferation and tumorigenicity based on bioinformatic analysis with functional studies. Mechanistically, we revealed that KDELR2 could regulate the expression of KIF20A, thus stimulating the expression of MMP2, MMP9 and MKI67. Functionally, the overexpression of KDELR2 and KIF20A markedly promoted proliferation, migration, and invasion in vitro and enhanced tumor growth in vivo, while knockdown of KDELR2 and KIF20A exerted the opposite effects. And the overexpression of KDELR2 also enhanced lymph node metastasis in vivo.

Conclusions
Collectively, our findings clarified a hitherto unexplored mechanism of KDELR2-KIF20A axis in increasing Golgi-mediated secretion of MMPs to drive tumor progression in BCa.
Overexpression of cotton GhNAC072 gene enhances drought and salt stress tolerance in transgenic Arabidopsis
Authors: Mehari Teame Gereziher , Hou Yuqing , Xu Yanchao , Umer Muhammad Jawad , Shiraku Margaret Linyerera , Wang Yuhong , Wang Heng , Peng Renhai , Wei Yangyang , Cai Xiaoyan , Zhou Zhongli , Liu Fang 
Published: September 2022

Abstract
Background
Crops face several environmental stresses (biotic and abiotic), thus resulting in severe yield losses. Around the globe abiotic stresses are the main contributors of plant damages, primarily drought and salinity. Many genes and transcription factors are involved in abiotic and biotic stress responses. NAC TF (Transcription Factors) improves tolerance to stresses by controlling the physiological and enzyme activities of crops.

Results
In current research, GhNAC072 a highly upregulated TF in RNA-Seq was identified as a hub gene in the co-expression network analysis (WGCNA). This gene was transformed to Arabidopsis thaliana to confirm its potential role in drought and salt stress tolerance. Significant variations were observed in the morpho-physiological traits with high relative leaf water contents, chlorophyll contents, higher germination and longer root lengths of the overexpressed lines and low excised leaf loss and ion leakage as compared to the wildtype plants. Besides, overexpressed lines have higher amounts of antioxidants and low oxidant enzyme activities than the wildtype during the period of stress exposure.

Conclusions
In summary, the above analysis showed that GhNAC072 might be the true candidate involved in boosting tolerance mechanisms under drought and salinity stress.
Metformin reverse minocycline to inhibit minocycline-resistant Acinetobacter baumannii by destroy the outer membrane and enhance membrane potential in vitro
Authors: Guo Tingting , Sun Xiaoli , Yang Jie , Yang Liying , Li Mengying , Wang Yuhang , Jiao Hongmei , Li Guocai 
Published: September 2022

Abstract
Background
Acinetobacter baumannii (A. baumannii) is an opportunistic pathogen and has emerged as one of the most troublesome pathogens. Drug resistance in A. baumannii has been reported on a global scale. Minocycline was found to be active against multi-drug resistant A. baumannii and was approved by the FDA for the infections caused by sensitive strains of A. baumannii. However, the emergence of minocycline resistance and its toxic effects still need to be addressed. Therefore, this study aimed to evaluate the synergistic effects of metformin combined with minocycline on minocycline-resistant A. baumannii.

Results
The effect of metformin on the antibacterial activity of minocycline was determined by checkerboard and time-killing assay. Further, it was observed by biofilm formation assay that metformin combination with minocycline can inhibit the formation of biofilm. Outer membrane integrity, membrane permeability, membrane potential and reactive oxygen species (ROS) were monitored to explore the underlying synergistic mechanisms of metformin on minocycline. And the results shown that metformin can destroy the outer membrane of A. baumannii, enhance its membrane potential, but does not affect the membrane permeability and ROS.

Conclusion
These findings suggested that the combination of metformin and minocycline has the potential for rejuvenating the activity of minocycline against minocycline-resistant A. baumannii.
Alleviation of iron deficiency in pear by ammonium nitrate and nitric oxide
Authors: Liu Jianlong , Wang Jinzhu , Wang Zidong , Li Min , Liang Chenglin , Yang Yingjie , Li Dingli , Wang Ran 
Published: September 2022

Abstract
Background
Iron is essential for the growth and development of trace elements in plants, and iron deficiency can lead to leaf chlorosis. Ammonium and nitrate are the major forms of nitrogen present in soils. Ammonium nitrate alleviates the chlorosis of leaves caused by iron deficiency, but the mechanism is not clear in pear.

Results
Ammonium nitrate induced the increase of nitric oxide (NO) under iron deficiency. We further analyzed the effect of NO by exogenous NO treatment. The results showed that ammonium nitrate and NO increased the activity of ferric chelate reductase. NO induced the expression of multiple IRT genes and promoted the transmembrane transport of irons. Ammonium nitrate and NO promoted the activity of nitrogen assimilation-related enzymes and the nitrogen absorption capacity, and they also increased glutamine synthetase activity. Finally, ammonium nitrate and NO increased chlorophyll synthesis, with subsequent increase in the photosynthetic capacity of plants and accumulation of biomass.

Conclusion
Ammonium nitrate indirectly alleviates the symptoms of plant yellowing by promoting the increase of NO, which increases the response of iron transporters. Both substances increase the nitrogen accumulation in plants. This study demonstrates a new option for minimizing Fe deficiency by regulating the balance between nutrients.
The contribution of functional cognition screening during acute illness hospitalization of older adults in predicting participation in daily life after discharge
Authors: Arieli Maya , Agmon Maayan , Gil Efrat , Kizony Rachel 
Published: September 2022

Abstract
Background
Cognitive assessment in acutely hospitalized older adults is mainly limited to neuropsychological screening measures of global cognition. Performance-based assessments of functional cognition better indicate functioning in real-life situations. However, their predictive validity has been less studied in acute hospital settings. The aim of this study was to explore the unique contribution of functional cognition screening during acute illness hospitalization in predicting participation of older adults one and three months after discharge beyond traditional neuropsychological measures.

Methods
This prospective longitudinal study included 84 older adults ≥ 65 years hospitalized in internal medicine wards due to acute illness, followed by home visits at one month and telephone interviews at three months (n = 77). Participation in instrumental activities of daily living, social and leisure activities was measured by the Activity Card Sort. In-hospital factors included cognitive status (telephone version of the Mini-Mental State Examination, Color Trails Test), functional cognition screening (medication sorting task from the alternative Executive Function Performance Test), emotional status (Hospital Anxiety and Depression scale), functional decline during hospitalization (modified Barthel index), length of hospital stay, the severity of the acute illness, symptoms severity and comorbidities.

Results
Functional cognition outperformed the neuropsychological measures in predicting participation declines in a sample of relatively high-functioning older adults. According to a hierarchical multiple linear regression analysis, the overall model explained 28.4% of the variance in participation after one month and 19.5% after three months. Age and gender explained 18.6% of the variance after one month and 13.5% after three months. The medication sorting task explained an additional 5.5% of the variance of participation after one month and 5.1% after three months, beyond age and gender. Length of stay and the Color Trails Test were not significant contributors to the change in participation.

Conclusions
By incorporating functional cognition into acute settings, healthcare professionals would be able to better detect older adults with mild executive dysfunctions who are at risk for participation declines. Early identification of executive dysfunctions can improve continuity of care and planning of tailored post-discharge rehabilitation services, especially for high-functioning older adults, a mostly overlooked population in acute settings. The results support the use of functional cognition screening measure of medication management ability in acute settings.
Association between multimorbidity trajectories and incident disability among mid to older age adults: China Health and Retirement Longitudinal Study
Authors: Shi Zaixing , Zhang Zeyun , Shi Kanglin , Yu Bohan , Jiang Zhongquan , Yang Li , Lin Jianlin , Fang Ya 
Published: September 2022

Abstract
Background
Although multimorbidity is a risk factor for disability, the relationship between the accumulative patterns of multimorbidity and disability remains poorly understood. The objective of this study was to identify the latent groups of multimorbidity trajectories among mid to older age adults and to examine their associations with incident disability.

Methods
We included 5,548 participants aged ≥ 45 years who participated in the China Health and Retirement Longitudinal Study from 2011 to 2018 and had no multimorbidity (≥ 2 chronic conditions) at baseline. The group-based multi-trajectory modeling was used to identify distinct trajectory groups of multimorbidity based on the latent dimensions underlying 13 chronic conditions. The association between multimorbidity trajectories and incident disability was analyzed using the generalized estimating equation model adjusting for potential confounders.

Results
Of the 5,548 participants included in the current analysis, 2,407 (43.39%) developed multimorbidity during the follow-up. Among participants with new-onset multimorbidity, four trajectory groups were identified according to the combination of newly diagnosed diseases: “Cardiometabolic” (N = 821, 34.11%), “Digestive-arthritic” (N = 753, 31.28%), “Cardiometabolic/Brain” (N = 618, 25.68%), and “Respiratory” (N = 215, 8.93%). Compared to participants who did not develop multimorbidity, the risk of incident disability was most significantly increased in the “Cardiometabolic/Brain” trajectory group (OR = 2.05, 95% CI: 1.55–2.70), followed by the “Cardiometabolic” (OR = 1.96, 95% CI: 1.52 –2.53) and “Digestive-arthritic” (OR = 1.70, 95% CI: 1.31–2.20) trajectory groups.

Conclusions
The growing burden of multimorbidity, especially the comorbid of cardiometabolic and brain diseases, may be associated with a significantly increased risk of disability for mid to older age adults. These findings improve our understanding of multimorbidity patterns that affect the independence of living and inform the development of strategies for the primary prevention of disability.
Determinants of quality of life among people with dementia: evidence from a South Asian population
Authors: Jayakody Surangi , Arambepola Carukshi 
Published: September 2022

Abstract
Background
Dementia has become a public health priority along with population aging worldwide. Owing to its chronic progressive nature in the absence of a cure, maintaining the best possible quality of life (QOL) has become the desired outcome for people with dementia (PWD).

Aim
The current study aimed to determine the factors associated with ‘good QOL’ in people with dementia in the Sri Lankan setting.

Methods
An unmatched case–control study was conducted to identify the factors associated with ‘good QoL. Cases in the study included dementia patients having ‘good’ QOL, whereas controls were those having ‘poor’ or ‘average’ QOL. Both cases and controls were selected from the same patient base at a premier tertiary care state hospital in Colombo District, Sri Lanka. They were identified using the validated DEMQOL (Dementia Specific Quality of Life) tool, adhering to strict eligibility criteria. An interviewer-administered questionnaire was used to assess the associated factors. Bivariate analysis followed by logistic regression modelling determined the associated factors for ‘good QOL’ adjusted for confounders using odds ratio (OR) and 95% confidence interval (CI).

Results
The study sample consisted of 64 cases and 208 controls. After adjusting for confounders, education up to GCE O/Level and above (OR = 4.02; 95% CI = 2.97, 12.0), ever employed (OR = 3.21; 95% CI = 1.59, 11.06), good social functioning (OR = 4.14; 95% CI = 3.39, 16.46), mild functional impairment (OR = 1.77; 95% CI = 1.13, 9.67), little or no caregiver burden (OR = 2.96; 95% CI = 1.86, 10.94), absence of apathy (OR = 2.22; 95% CI = 1.27, 12.48) and absence of irritability (OR = 2.17; 95% CI = 1.72, 10.34) were found to be significantly associated with ‘good QOL’. 60% of the variance of ‘good’ QOL among PWD was explained by the factors in the final model.

Conclusions and recommendations
The identified determinants of ‘good QOL’ clearly show how the QOL improvement interventions need to be planned. Accordingly, such programmes should be primarily focused on strategies to improve PWDs' ADL (Activities of Daily living), reduce and manage neuropsychiatric symptoms effectively and to promote activities enhancing social functioning, and plan programmes to address caregiver burden.
Relationship between active Helicobacter pylori infection and risk factors of cardiovascular diseases, a cross-sectional hospital-based study in a Sub-Saharan setting
Authors: Tali Lionel Danny Nguefak , Faujo Ghislaine Florice Nintewoue , Konang Justine Laure Nguieguia , Dzoyem Jean Paul , Kouitcheu Laure Brigitte Mabeku 
Published: September 2022

AbstractBackgroundChronic inflammation has been reported as one of the novel coronary heart disease (CHD) risk factors. Knowing thatHelicobacter pylori(H. pylori) provokes a local inflammation, the relationship betweenH. pyloriinfection and cardiovascular disease (CVD) has received considerable attention. However, the attempt to demonstrate the association betweenH. pyloriand specific cardiovascular disease risk factors is always a challenging issue due to the conflicting reports in the literatures.MethodsWe performed a cross-sectional study of 363 consecutive dyspeptic subjects in three reference health facilities in Cameroon from October 2020 to October 2021. Each participation gave a written consent and the study was approved by the local Ethical Committee. Check-up for cardiovascular disease (CVD) risk factors such as dyslipidemia-related parameters, obesity-related parameter, high blood pressure as well asH. pyloridetection was done for each participant. Data was analyzed using SSPS statistical package.ResultsHelicobacter pyloriinfection was significantly associated with higher total cholesterol level (OR: 2.3324, p = 0.0002) and higher LDL cholesterol level (OR: 2.3096, p = 0.0006). The crude OR ofH. pyloristatus on the prevalence of high body mass index (BMI) was 1.0813 (p = 0.7300) and the adjusted OR for confounding factors was 1.1785 (p = 0.5095). The strength of the association betweenH. pyloriinfection and blood pressure, shows an OR of 1.3807 (p = 0.2991), 1.0060 (p = 0.9855) and 1.4646 (p = 0.2694) for diastolic pressure, hypertension and high heart rate respectively, while that of systolic pressure was 0.8135 (p = 0.4952).H. pyloriinfection is associated with dyslipidemia in our milieu.
Endovascular recanalization of acute ischemic stroke patients exhibiting large vessel occlusion after pulmonary lobectomy: case series
Authors: Ji Renjie , Xu Ziqi , Chen Hanfeng , Luo Benyan 
Published: September 2022

Abstract
Objective
We analyzed the outcomes of patients suffering acute ischemic stroke (AIS) with large vessel occlusion (LVO) soon after pulmonary lobectomy.

Methods
We retrospectively reviewed the clinical records of patients who underwent pulmonary lobectomy to treat primary lung cancer. We retrieved clinical characteristics and the incidence of AIS with LVO. The clinical courses of patients who experienced AIS were reviewed.

Results
In 10 (0.3%) of 3406 patients, AIS with LVO developed soon (within 3 days) after pulmonary lobectomy. The lung resection site was on the left in eight patients (80%). All patients underwent thrombectomy and achieved complete recanalization (Thrombolysis in Cerebral Infarction [TICI] 3). The average time between symptom onset and recanalization was 165.5 min. Nine (90%) patients exhibited favorable outcomes (modified Rankin scale [mRS] score ≤ 2) at the 3-month follow-up.

Conclusion
Endovascular therapy effectively treats AIS with LVO that develops after lung surgery, and direct aspiration is a promising strategy. A large, multicenter study is warranted to further confirm these findings.
A case of novel DYT6 dystonia variant with serious complications after deep brain stimulation therapy: a case report
Authors: Grofik M. , Cibulka M. , Olekšáková J. , Turčanová Koprušáková M. , Galanda T. , Necpál J. , Jungová P. , Kurča E. , Winkelmann J , Zech M. , Jech R. 
Published: September 2022

Abstract
Background
DYT6 dystonia belongs to a group of isolated, genetically determined, generalized dystonia associated with mutations in the THAP1 gene.

Case presentation
We present the case of a young patient with DYT6 dystonia associated with a newly discovered c14G>A (p.Cys5Tyr) mutation in the THAP1 gene. We describe the clinical phenotype of this new mutation, effect of pallidal deep brain stimulation (DBS), which was accompanied by two rare postimplantation complications: an early intracerebral hemorrhage and delayed epileptic seizures. Among the published case reports of patients with DYT6 dystonia, the mentioned complications have not been described so far.

Conclusions
DBS in the case of DYT6 dystonia is a challenge to thoroughly consider possible therapeutic benefits and potential risks associated with surgery. Genetic heterogeneity of the disease may also play an important role in predicting the development of the clinical phenotype as well as the effect of treatment including DBS. Therefore, it is beneficial to analyze the genetic and clinical relationships of DYT6 dystonia.
Intrinsic immune evasion patterns predict temozolomide sensitivity and immunotherapy response in lower-grade gliomas
Authors: Tu Zewei , Ji Qiankun , Han Qing , Long Xiaoyan , Li Jingying , Wu Lei , Huang Kai , Zhu Xingen 
Published: September 2022

Abstract
Background
Although intrinsic immune-evasion is important in cancer proliferation, metastasis and response to treatment, it is unclear whether intrinsic immune-evasion patterns of gliomas can aid in predicting clinical prognosis and determining treatment.

Methods
A total of 182 immune-evasion genes intrinsic to cancer were subjected to consensus clustering to identify immune-evasion patterns in 1421 patients with lower-grade glioma (LGG). The levels of each cancer hallmark were determined by the Gene Set Variant Analysis (GSVA) method, and immune cell infiltrations were quantified using two algorithms, the single-sample Gene Set Enrichment Analysis (ssGSEA) and the Cell-type Identification By Estimating Relative Subsets Of RNA Transcripts (CIBERSORT) methods. IEVscore was determined by a method that combined univariate Cox regression analysis, least absolute shrinkage and selection operator (LASSO) regression and principal component analysis (PCA).

Results
Transcriptional and genomic analysis showed that most immune evasion genes (IEVGs) were upregulated in LGGs, with aberrant expression driven by alterations in copy number variants (CNV). Based on the mRNA expression profiles of cancer-intrinsic IEVGs could be divided into three LGG subgroups with distinct prognosis, clinicopathological features and immune infiltrations. A combined scoring scheme designed to assess the immune-evasion levels of LGGs divided these 1421 patients into two subgroups that differed in IEVscores. LGG patients with low-IEVscore had a better prognosis, would be more likely to benefit from immune check-point inhibitors and would be more susceptible to temozolomide (TMZ) chemotherapy.

Conclusion
Intrinsic immune evasion in the tumor microenvironment (TME) has a crucial effect on glioma formation. Quantitatively assessing the IEV scores of individual LGG patients could enhance knowledge about the intra-glioma microenvironment and lead to the development of individualized therapeutic strategies for patients with LGG.