cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.30517841.v1
title: Impact of early minimal residual disease assessment on the outcomes of patients with systemic light chain amyloidosis

description: Systemic light chain (AL) amyloidosis is associated with a high mortality rate, particularly in the first year after diagnosis. Minimal residual disease (MRD) assessment plays a key role in the prognosis of AL amyloidosis but is typically performed later in the treatment process, thereby limiting its ability to guide early interventions. This retrospective study included 53 newly diagnosed AL amyloidosis patients who underwent MRD assessment <i>via</i> flow cytometry after two chemotherapy cycles. The aim of this study was to assess the effect of early MRD negativity on organ response kinetics and prognosis. After two chemotherapy cycles, 22.6% (12/53) of patients achieved early MRD negativity. Daratumumab-treated patients had a significantly greater early MRD negativity rate (41.46%/0%, <i>p</i> = 0.019). Early MRD-negative patients had a significantly longer event-free survival (EFS) (not reached vs. 37.48 months, <i>p</i> = 0.029) than MRD-positive patients. In terms of organ response, early MRD-negative patients with cardiac or renal involvement had higher complete response (CR) rates and significantly shorter times to cardiac and renal CR (cardiac CR = 5.6 months vs. 28.4 months, <i>p</i> = 0.009; renal CR = 12.2 months vs. 41.0 months; <i>p</i> &lt; 0.001). Early MRD negativity predicts faster/deeper organ responses in AL amyloidosis patients and is associated with improved prognosis. These findings offer new possibilities for integrating early MRD assessment into clinical practice. Early MRD negativity in AL amyloidosis patients leads to faster, deeper organ responses and significantly better event-free survival.Daratumumab-based induction therapy enhances early MRD negativity rates and improves prognosis, especially in cardiac and renal responses. Early MRD negativity in AL amyloidosis patients leads to faster, deeper organ responses and significantly better event-free survival. Daratumumab-based induction therapy enhances early MRD negativity rates and improves prognosis, especially in cardiac and renal responses.
DOI: 10.6084/m9.figshare.29413757
title: The role of systemic inflammation in the formation and rupture of intracranial aneurysms in moyamoya disease: a retrospective cohort study

description: Intracranial aneurysms (IAs) and their rupture are serious complications in patients with Moyamoya disease (MMD). However, specific risk factors for IA formation and rupture in MMD remain underexplored. We conducted a retrospective analysis of 450 MMD patients, evaluating the prevalence and characteristics of IAs. We analysed peripheral blood markers, including white blood cell count, neutrophil count, lymphocyte count, monocyte count, platelet count, and calculated SII-related biomarkers (SII, NLR, PLR, MLR, GLR). Among the 450 MMD patients, 57 (12.67%) had a total of 73 IAs. Unilateral MMD patients had a significantly higher risk of IAs and multiple IAs compared to bilateral MMD patients (OR 1.97, 95% CI [1.02 − 3.71], <i>p</i> = 0.042; OR 5.76, 95% CI [1.16 − 23.55], <i>p</i> = 0.032). Aspirin use was associated with a lower incidence of IAs (OR 0.34, 95% CI [0.17 − 0.70], <i>p</i> = 0.002). The SII, NLR, GLR, MLR, and PLR were significantly elevated in patients with IAs compared to those without. ROC analysis showed AUC values of 0.610 for SII (<i>p</i> = 0.007), 0.593 for PLR (<i>p</i> = 0.024), 0.659 for NLR (<i>p</i> &lt; 0.001), 0.665 for GLR (<i>p</i> &lt; 0.001), and 0.658 for MLR (<i>p</i> &lt; 0.001). In patients with ruptured IAs, SII, NLR, GLR, and MLR levels were significantly higher than in those with unruptured IAs, with AUC values for predicting IA rupture of 0.720 for SII (<i>p</i> = 0.002), 0.745 for NLR (<i>p</i> &lt; 0.001), 0.727 for GLR (<i>p</i> = 0.001), and 0.717 for MLR (<i>p</i> = 0.002). This study highlights the significant role of systemic inflammation in the formation and rupture of IAs in MMD patients. Elevated levels of SII and its constituent markers (NLR, PLR, MLR and GLR) are associated with an increased risk of IA development and rupture. Aspirin could reduce the risk of IA formation and rupture in MMD patients without increasing cerebral haemorrhage risk. Inflammation significantly contributes to IA formation and rupture in MMD.The SII and related biomarkers are elevated in MMD patients with IAs and are associated with an increased risk of IA rupture.Monitoring the SII and related biomarkers can provide valuable insights into IA risk and improve clinical management strategies. Inflammation significantly contributes to IA formation and rupture in MMD. The SII and related biomarkers are elevated in MMD patients with IAs and are associated with an increased risk of IA rupture. Monitoring the SII and related biomarkers can provide valuable insights into IA risk and improve clinical management strategies.
DOI: 10.6084/m9.figshare.29413757.v1
title: The role of systemic inflammation in the formation and rupture of intracranial aneurysms in moyamoya disease: a retrospective cohort study

description: Intracranial aneurysms (IAs) and their rupture are serious complications in patients with Moyamoya disease (MMD). However, specific risk factors for IA formation and rupture in MMD remain underexplored. We conducted a retrospective analysis of 450 MMD patients, evaluating the prevalence and characteristics of IAs. We analysed peripheral blood markers, including white blood cell count, neutrophil count, lymphocyte count, monocyte count, platelet count, and calculated SII-related biomarkers (SII, NLR, PLR, MLR, GLR). Among the 450 MMD patients, 57 (12.67%) had a total of 73 IAs. Unilateral MMD patients had a significantly higher risk of IAs and multiple IAs compared to bilateral MMD patients (OR 1.97, 95% CI [1.02 − 3.71], <i>p</i> = 0.042; OR 5.76, 95% CI [1.16 − 23.55], <i>p</i> = 0.032). Aspirin use was associated with a lower incidence of IAs (OR 0.34, 95% CI [0.17 − 0.70], <i>p</i> = 0.002). The SII, NLR, GLR, MLR, and PLR were significantly elevated in patients with IAs compared to those without. ROC analysis showed AUC values of 0.610 for SII (<i>p</i> = 0.007), 0.593 for PLR (<i>p</i> = 0.024), 0.659 for NLR (<i>p</i> &lt; 0.001), 0.665 for GLR (<i>p</i> &lt; 0.001), and 0.658 for MLR (<i>p</i> &lt; 0.001). In patients with ruptured IAs, SII, NLR, GLR, and MLR levels were significantly higher than in those with unruptured IAs, with AUC values for predicting IA rupture of 0.720 for SII (<i>p</i> = 0.002), 0.745 for NLR (<i>p</i> &lt; 0.001), 0.727 for GLR (<i>p</i> = 0.001), and 0.717 for MLR (<i>p</i> = 0.002). This study highlights the significant role of systemic inflammation in the formation and rupture of IAs in MMD patients. Elevated levels of SII and its constituent markers (NLR, PLR, MLR and GLR) are associated with an increased risk of IA development and rupture. Aspirin could reduce the risk of IA formation and rupture in MMD patients without increasing cerebral haemorrhage risk. Inflammation significantly contributes to IA formation and rupture in MMD.The SII and related biomarkers are elevated in MMD patients with IAs and are associated with an increased risk of IA rupture.Monitoring the SII and related biomarkers can provide valuable insights into IA risk and improve clinical management strategies. Inflammation significantly contributes to IA formation and rupture in MMD. The SII and related biomarkers are elevated in MMD patients with IAs and are associated with an increased risk of IA rupture. Monitoring the SII and related biomarkers can provide valuable insights into IA risk and improve clinical management strategies.
DOI: 10.6084/m9.figshare.29430156
title: 1,25-Dihydroxy vitamin D<sub>3</sub> inhibits LPS-mediated inflammatory responses in endometriosis

description: Endometriosis, a chronic immune-mediated inflammatory disease, remains elusive in its pathogenesis. Given vitamin D (VD)’s pivotal role in modulating innate and adaptive immune responses, we sought to elucidate how VD modulates inflammatory responses in endometriosis. We isolated primary human ectopic endometrial stromal cells (EESCs) from ectopic endometrium of ovarian endometrioma, alongside Ishikawa cells, and subjected them to treatment with lipopolysaccharide (LPS), a potent inducer of inflammation, alongside varying concentrations of 1,25-dihydroxyvitamin D<sub>3</sub> (1,25(OH)<sub>2</sub>D<sub>3</sub>), the biologically active form of VD, and its analog TEI-9647 (25-dehydro-1α-hydroxyvitamin D<sub>3</sub>-26,23-lactone). Our results revealed that 1,25(OH)<sub>2</sub>D<sub>3</sub> significantly reversed LPS-induced cell proliferation, migration, and inflammatory factor production in EESCs and Ishikawa cells, and induced apoptosis. Additionally, 1,25(OH)<sub>2</sub>D<sub>3</sub> inhibited the expression and nuclear translocation of phosphorylated p65 in LPS-activated EESCs and Ishikawa cells. Furthermore, 1,25(OH)<sub>2</sub>D<sub>3</sub> counteracted LPS-induced suppression of VD receptor (VDR)/IκBα and enhancement of Toll-like receptor 4 (TLR4)/pyrin domain (PYD)-containing protein 3 (NLRP3) activation, while the addition of TEI-9647 reversed VD’s regulatory effects on the NF-κB pathway. <i>In vivo</i> experimental results showed that 1,25(OH)<sub>2</sub>D<sub>3</sub> significantly inhibited lesion growth, suppressed NF-κB pathway activation, and corresponding inflammatory phenotypes in a rat model of endometriosis. Collectively, these results underscore the potential of 1,25(OH)<sub>2</sub>D<sub>3</sub> as a therapeutic target for endometriosis <i>via</i> VDR-dependent endometrial homeostasis regulation, suppressing LPS-mediated inflammatory responses and NF-κB signaling pathway through VDR activation and IκBα stabilization.
DOI: 10.6084/m9.figshare.29430156.v1
title: 1,25-Dihydroxy vitamin D<sub>3</sub> inhibits LPS-mediated inflammatory responses in endometriosis

description: Endometriosis, a chronic immune-mediated inflammatory disease, remains elusive in its pathogenesis. Given vitamin D (VD)’s pivotal role in modulating innate and adaptive immune responses, we sought to elucidate how VD modulates inflammatory responses in endometriosis. We isolated primary human ectopic endometrial stromal cells (EESCs) from ectopic endometrium of ovarian endometrioma, alongside Ishikawa cells, and subjected them to treatment with lipopolysaccharide (LPS), a potent inducer of inflammation, alongside varying concentrations of 1,25-dihydroxyvitamin D<sub>3</sub> (1,25(OH)<sub>2</sub>D<sub>3</sub>), the biologically active form of VD, and its analog TEI-9647 (25-dehydro-1α-hydroxyvitamin D<sub>3</sub>-26,23-lactone). Our results revealed that 1,25(OH)<sub>2</sub>D<sub>3</sub> significantly reversed LPS-induced cell proliferation, migration, and inflammatory factor production in EESCs and Ishikawa cells, and induced apoptosis. Additionally, 1,25(OH)<sub>2</sub>D<sub>3</sub> inhibited the expression and nuclear translocation of phosphorylated p65 in LPS-activated EESCs and Ishikawa cells. Furthermore, 1,25(OH)<sub>2</sub>D<sub>3</sub> counteracted LPS-induced suppression of VD receptor (VDR)/IκBα and enhancement of Toll-like receptor 4 (TLR4)/pyrin domain (PYD)-containing protein 3 (NLRP3) activation, while the addition of TEI-9647 reversed VD’s regulatory effects on the NF-κB pathway. <i>In vivo</i> experimental results showed that 1,25(OH)<sub>2</sub>D<sub>3</sub> significantly inhibited lesion growth, suppressed NF-κB pathway activation, and corresponding inflammatory phenotypes in a rat model of endometriosis. Collectively, these results underscore the potential of 1,25(OH)<sub>2</sub>D<sub>3</sub> as a therapeutic target for endometriosis <i>via</i> VDR-dependent endometrial homeostasis regulation, suppressing LPS-mediated inflammatory responses and NF-κB signaling pathway through VDR activation and IκBα stabilization.
DOI: 10.6084/m9.figshare.30273821
title: Predicting six-month mortality in adult hemophagocytic lymphohistiocytosis with machine learning: a prognostic approach utilizing laboratory data

description: Hemophagocytic lymphohistiocytosis (HLH) is associated with high mortality rates. This study was conducted to develop and validate a predictive model for adult HLH patients at high risk of six months mortality using machine learning (ML) algorithms. The study utilized univariate analysis and LASSO regression, incorporating eleven ML algorithms, to perform a comprehensive analysis of both admission and discharge variables. Model performance was assessed using metrics such as AUC, F1 score, Kaplan Meier (KM) curves, calibration curves and decision curve analysis (DCA). A total of 136 patients meeting the HLH-2004 criteria were included in this study. Among them, 59 patients received chemotherapy or other cytotoxic drugs, while the remaining 77 patients underwent symptomatic treatment. The median age was 52 years among nonsurvivors and 48 years among survivors, with 47 (42.6%) males in the nonsurvivor group and 31 (47.6%) in the survivor group. Age and nine discharge variables were identified as the most significant features for model construction. Random forest (RF) algorithm demonstrated superior predictive capabilities, achieving an AUC of 1.00, accuracy of 0.98 and F1 score of 0.98 in the training cohort, and an AUC of 0.89, accuracy of 0.85 and F1 score of 0.85 in the validation cohort. The top five predictors were all discharge variables (ferritin, white blood cell (WBC), albumin (ALB), platelet (PLT) and direct bilirubin (DB)). The predictive model developed in this study provides a valuable tool for clinicians to early identify high-risk HLH patients, thereby enabling more targeted and effective interventions. Hemophagocytic lymphohistiocytosis (HLH) is associated with high mortality rates.Discharge variables offer more reliable prognostic information for adult HLH.This study provides a valuable tool for clinicians to early identify high-risk HLH patients, thereby enabling more targeted and effective interventions Hemophagocytic lymphohistiocytosis (HLH) is associated with high mortality rates. Discharge variables offer more reliable prognostic information for adult HLH. This study provides a valuable tool for clinicians to early identify high-risk HLH patients, thereby enabling more targeted and effective interventions
DOI: 10.6084/m9.figshare.30273821.v1
title: Predicting six-month mortality in adult hemophagocytic lymphohistiocytosis with machine learning: a prognostic approach utilizing laboratory data

description: Hemophagocytic lymphohistiocytosis (HLH) is associated with high mortality rates. This study was conducted to develop and validate a predictive model for adult HLH patients at high risk of six months mortality using machine learning (ML) algorithms. The study utilized univariate analysis and LASSO regression, incorporating eleven ML algorithms, to perform a comprehensive analysis of both admission and discharge variables. Model performance was assessed using metrics such as AUC, F1 score, Kaplan Meier (KM) curves, calibration curves and decision curve analysis (DCA). A total of 136 patients meeting the HLH-2004 criteria were included in this study. Among them, 59 patients received chemotherapy or other cytotoxic drugs, while the remaining 77 patients underwent symptomatic treatment. The median age was 52 years among nonsurvivors and 48 years among survivors, with 47 (42.6%) males in the nonsurvivor group and 31 (47.6%) in the survivor group. Age and nine discharge variables were identified as the most significant features for model construction. Random forest (RF) algorithm demonstrated superior predictive capabilities, achieving an AUC of 1.00, accuracy of 0.98 and F1 score of 0.98 in the training cohort, and an AUC of 0.89, accuracy of 0.85 and F1 score of 0.85 in the validation cohort. The top five predictors were all discharge variables (ferritin, white blood cell (WBC), albumin (ALB), platelet (PLT) and direct bilirubin (DB)). The predictive model developed in this study provides a valuable tool for clinicians to early identify high-risk HLH patients, thereby enabling more targeted and effective interventions. Hemophagocytic lymphohistiocytosis (HLH) is associated with high mortality rates.Discharge variables offer more reliable prognostic information for adult HLH.This study provides a valuable tool for clinicians to early identify high-risk HLH patients, thereby enabling more targeted and effective interventions Hemophagocytic lymphohistiocytosis (HLH) is associated with high mortality rates. Discharge variables offer more reliable prognostic information for adult HLH. This study provides a valuable tool for clinicians to early identify high-risk HLH patients, thereby enabling more targeted and effective interventions
DOI: 10.6084/m9.figshare.30928091
title: Diagnostic value of CEACAM6 and HE4 in pleural fluid for malignant pleural effusion

description: This study aimed to assess the diagnostic performance of carcinoembryonic antigen-related adhesion molecule 6 (CEACAM6) and human epididymis protein 4 (HE4) in pleural fluid for the detection of malignant pleural effusion (MPE). In this prospective study, pleural levels of CEACAM6 and HE4 were measured in two independent cohorts. The test cohort included 182 patients with exudative pleural effusions (123 malignant and 59 benign), and the validation cohort comprised 117 patients with exudative pleural effusions (65 malignant and 52 benign). Receiver operating characteristic (ROC) curves were used to assess the diagnostic performance of CEACAM6 and HE4 for MPE. Both CEACAM6 and HE4 levels were significantly elevated in MPE compared to benign pleural effusion (BPE) in both cohorts (<i>p</i> &lt; .001). In the test cohort, CEACAM6 and HE4 demonstrated areas under the curve (AUC) values of 0.862 and 0.826, respectively. The combination of CEACAM6 and HE4 yielded a higher AUC of 0.938 compared to either marker alone. In the validation cohort, both CEACAM6 (AUC = 0.811) and HE4 (AUC = 0.721), along with their combination (AUC = 0.834), exhibited strong diagnostic performance for MPE. Notably, in cytology-negative cases, the combination of CEACAM6 and HE4 also demonstrated a favourable diagnostic efficacy, with an AUC of 0.800. The addition of CEA to the CEACAM6/HE4 combination further improved the AUC to 0.819 (<i>p</i> = .04). Pleural CEACAM6 and HE4 are promising biomarkers for distinguishing MPE from BPE. Their combination improves diagnostic accuracy, offering a valuable tool for MPE diagnosis, especially in challenging cases with cytology-negative pleural effusion.
DOI: 10.6084/m9.figshare.30928091.v1
title: Diagnostic value of CEACAM6 and HE4 in pleural fluid for malignant pleural effusion

description: This study aimed to assess the diagnostic performance of carcinoembryonic antigen-related adhesion molecule 6 (CEACAM6) and human epididymis protein 4 (HE4) in pleural fluid for the detection of malignant pleural effusion (MPE). In this prospective study, pleural levels of CEACAM6 and HE4 were measured in two independent cohorts. The test cohort included 182 patients with exudative pleural effusions (123 malignant and 59 benign), and the validation cohort comprised 117 patients with exudative pleural effusions (65 malignant and 52 benign). Receiver operating characteristic (ROC) curves were used to assess the diagnostic performance of CEACAM6 and HE4 for MPE. Both CEACAM6 and HE4 levels were significantly elevated in MPE compared to benign pleural effusion (BPE) in both cohorts (<i>p</i> &lt; .001). In the test cohort, CEACAM6 and HE4 demonstrated areas under the curve (AUC) values of 0.862 and 0.826, respectively. The combination of CEACAM6 and HE4 yielded a higher AUC of 0.938 compared to either marker alone. In the validation cohort, both CEACAM6 (AUC = 0.811) and HE4 (AUC = 0.721), along with their combination (AUC = 0.834), exhibited strong diagnostic performance for MPE. Notably, in cytology-negative cases, the combination of CEACAM6 and HE4 also demonstrated a favourable diagnostic efficacy, with an AUC of 0.800. The addition of CEA to the CEACAM6/HE4 combination further improved the AUC to 0.819 (<i>p</i> = .04). Pleural CEACAM6 and HE4 are promising biomarkers for distinguishing MPE from BPE. Their combination improves diagnostic accuracy, offering a valuable tool for MPE diagnosis, especially in challenging cases with cytology-negative pleural effusion.
DOI: 10.6084/m9.figshare.30928094.v1
title: Impact of sodium-glucose cotransporter-2 inhibitors on aging biomarkers and plasma ceramide levels in type 2 diabetes: beyond glycemic control

description: Aging is a complex biological process marked by the decline of physiological functions and heightened susceptibility to chronic illnesses, notably cardiometabolic disorders. Ceramides (Cer) are lipid derivatives linked to aging and metabolic diseases. Sodium-Glucose Cotransporter-2 inhibitors (SGLT2i), widely used in managing type 2 diabetes, have an unclear impact on aging biomarkers and Cer profiles. This study explored the association between SGLT2i use, plasma Cer levels (CerC16:0, CerC18:0, CerC22:0, CerC24:0, and CerC24:1), and aging biomarkers—Human Insulin-Like Growth Factor 1 (IGF-1), mammalian target of rapamycin (mTOR), 5-Methylcytosine (5MC), and Human H2AFX (Histone H2AX) in patients with type 2 diabetes mellitus (T2DM). In this retrospective study, 95 participants were divided into three groups: patients on SGLT2i (<i>n</i> = 34), patients on non-SGLT2i anti-diabetic treatments (<i>n</i> = 36), and healthy controls (<i>n</i> = 25). Plasma Cer and aging biomarkers were quantified using Liquid Chromatography with tandem mass spectrometry (LC–MS–MS) and ELISA, respectively. Principal component analysis (PCA) assessed group-based clustering, while ANCOVA evaluated group differences with confounder adjustment. SGLT2i-treated patients showed significantly lower CerC16:0, CerC22:0, and CerC24:1 levels (<i>p</i> &lt; 0.01) and decreased 5MC and H2AX (<i>p</i> &lt; 0.05) compared to non-SGLT2i patients. IGF-1 was significantly elevated in the SGLT2i group (<i>p</i> &lt; 0.01), suggesting a possible protective effect on metabolic health. PCA distinguished control from diabetic groups but revealed overlap between SGLT2i and non-SGLT2i groups. Beyond glucose control, SGLT2i may improve plasma Cer and aging markers in diabetic patients, supporting their broader therapeutic potential in aging and age-related diseases. Further large-scale studies are warranted to confirm these effects and underlying mechanisms.