cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.25188423
title: Renal outcome in multiple myeloma patients with cast nephropathy: a retrospective analysis of potential predictive values on clinical and renal outcome

description: Cast nephropathy (CN) is the leading cause of acute kidney injury (AKI) in multiple myeloma (MM). Since it is sparsely documented why some patients with CN do achieve a renal response while others do not, we describe a single-center cohort of patients with multiple myeloma and biopsy-confirmed CN to evaluate potential markers of renal response. The data was collected as a retrospective, single-center analysis of CN-patients treated at the Medical University Vienna between 01/01/2004 and 01/01/2022. Baseline parameters and clinical outcome was compared between renal responders and non-responders. Among 28 patients with CN, <i>n</i> = 23 were assessed for renal response (14 responders; 9 non-responders). Renal responders were younger (median age: 61 years; 77 years, <i>p</i> = 0.039), showed higher overall survival (153months; 58months, <i>p</i> = 0.044) and achieved hematologic response (≥PR) to first-line therapy (<i>p</i> = 0.029), and complete hematologic response (CR) at any time (<i>p</i> = 0.025) significantly more often. Further, we could show that rapid initiation of anti-myeloma therapy after initial presentation correlated significantly with renal response (median 9 days; 27 days, <i>p</i> = 0.016). Analyses of kidney biopsy specimens revealed that patients with a high IF/TA score showed end stage renal disease (dialysis ≥ 3 months) significantly more often (<i>p</i> = &lt;0.001). In summary, our data suggests, that a rapid start with systemic hematologic treatment in patients with MM and CN is crucial and achieving an early hematologic response is important for renal recovery. Moreover, achieving a deep hematologic response and subsequent renal recovery improves clinical outcome as reflected by an overall survival benefit.
DOI: 10.6084/m9.figshare.25188423.v1
title: Renal outcome in multiple myeloma patients with cast nephropathy: a retrospective analysis of potential predictive values on clinical and renal outcome

description: Cast nephropathy (CN) is the leading cause of acute kidney injury (AKI) in multiple myeloma (MM). Since it is sparsely documented why some patients with CN do achieve a renal response while others do not, we describe a single-center cohort of patients with multiple myeloma and biopsy-confirmed CN to evaluate potential markers of renal response. The data was collected as a retrospective, single-center analysis of CN-patients treated at the Medical University Vienna between 01/01/2004 and 01/01/2022. Baseline parameters and clinical outcome was compared between renal responders and non-responders. Among 28 patients with CN, <i>n</i> = 23 were assessed for renal response (14 responders; 9 non-responders). Renal responders were younger (median age: 61 years; 77 years, <i>p</i> = 0.039), showed higher overall survival (153months; 58months, <i>p</i> = 0.044) and achieved hematologic response (≥PR) to first-line therapy (<i>p</i> = 0.029), and complete hematologic response (CR) at any time (<i>p</i> = 0.025) significantly more often. Further, we could show that rapid initiation of anti-myeloma therapy after initial presentation correlated significantly with renal response (median 9 days; 27 days, <i>p</i> = 0.016). Analyses of kidney biopsy specimens revealed that patients with a high IF/TA score showed end stage renal disease (dialysis ≥ 3 months) significantly more often (<i>p</i> = &lt;0.001). In summary, our data suggests, that a rapid start with systemic hematologic treatment in patients with MM and CN is crucial and achieving an early hematologic response is important for renal recovery. Moreover, achieving a deep hematologic response and subsequent renal recovery improves clinical outcome as reflected by an overall survival benefit.
DOI: 10.6084/m9.figshare.27919314
title: Association between autoimmune diseases and myelodysplastic syndrome:a Mendelian randomization study

description: <b>Background</b>: The relationship between different types of autoimmune diseases and myelodysplastic syndrome (MDS) is inconclusive. Therefore, we employed Mendelian randomization (MR) to examine whether genetically predicted susceptibility to ten autoimmune diseases is associated with the risk of MDS. <b>Methods:</b> Single nucleotide polymorphisms (SNPs) significantly associated with 10 autoimmune diseases were extracted from the summary statistics of European genome-wide association studies (GWAS). A two-sample MR analysis was performed using summary-level statistics sourced from GWAS datasets. Inverse-variance weighting (IVW), MR–Egger, and weighted median (WM) were further supported by several sensitivity analyses. <b>Results:</b> Four autoimmune diseases showed genetical predisposition to MDS: rheumatoid arthritis (OR = 1.186,95% CI = 1.028-1.367, <i>P</i> = 0.019), multiple sclerosis (OR = 1.247, 95% CI = 1.013-1.534, <i>P</i> = 0.037), myasthenia gravis (OR = 1.326,95% CI = 1.010-1.742, <i>P</i> = 0.042), and Hashimoto thyroiditis(OR = 1.519,95% CI = 1.008-2.290, <i>P</i> = 0.046). Nevertheless, no similar causal relationship was found between the remaining seven autoimmune diseases and MDS. The accuracy and robustness of these findings were confirmed by sensitivity tests. <b>Conclusions:</b> We are the first to use MR analysis to explore the relationship between autoimmune diseases and MDS. The mechanism needs to be further explored. We employed Mendelian randomization to explore the associations between autoimmune diseases and myelodysplastic syndrome. We found that rheumatoid arthritis, multiple sclerosis, myasthenia gravis and Hashimoto thyroiditis showed a genetical predisposition to myelodysplastic syndrome. The mechanism needs to be further explored.
DOI: 10.6084/m9.figshare.27919314.v1
title: Association between autoimmune diseases and myelodysplastic syndrome:a Mendelian randomization study

description: <b>Background</b>: The relationship between different types of autoimmune diseases and myelodysplastic syndrome (MDS) is inconclusive. Therefore, we employed Mendelian randomization (MR) to examine whether genetically predicted susceptibility to ten autoimmune diseases is associated with the risk of MDS. <b>Methods:</b> Single nucleotide polymorphisms (SNPs) significantly associated with 10 autoimmune diseases were extracted from the summary statistics of European genome-wide association studies (GWAS). A two-sample MR analysis was performed using summary-level statistics sourced from GWAS datasets. Inverse-variance weighting (IVW), MR–Egger, and weighted median (WM) were further supported by several sensitivity analyses. <b>Results:</b> Four autoimmune diseases showed genetical predisposition to MDS: rheumatoid arthritis (OR = 1.186,95% CI = 1.028-1.367, <i>P</i> = 0.019), multiple sclerosis (OR = 1.247, 95% CI = 1.013-1.534, <i>P</i> = 0.037), myasthenia gravis (OR = 1.326,95% CI = 1.010-1.742, <i>P</i> = 0.042), and Hashimoto thyroiditis(OR = 1.519,95% CI = 1.008-2.290, <i>P</i> = 0.046). Nevertheless, no similar causal relationship was found between the remaining seven autoimmune diseases and MDS. The accuracy and robustness of these findings were confirmed by sensitivity tests. <b>Conclusions:</b> We are the first to use MR analysis to explore the relationship between autoimmune diseases and MDS. The mechanism needs to be further explored. We employed Mendelian randomization to explore the associations between autoimmune diseases and myelodysplastic syndrome. We found that rheumatoid arthritis, multiple sclerosis, myasthenia gravis and Hashimoto thyroiditis showed a genetical predisposition to myelodysplastic syndrome. The mechanism needs to be further explored.
DOI: 10.6084/m9.figshare.25848621
title: Attitude towards blood donation and its associated factors, types of blood donation, willingness, and feeling towards blood donation among potential blood donors in Ethiopia: systematic review and meta-analysis, observational study

description: Blood donation is crucial for certain populations, such as pregnant mothers, anemic patients, traumatized patients, and individuals undergoing surgery. The imbalance between the number of blood donors and the demand for blood in Ethiopia is a serious public health concern. Having a favorable attitude towards blood donation could aid in correcting this imbalance. Therefore, this study aimed to assess the proportion of favorable attitudes, types of blood donation, willingness, and feelings towards blood donation in Ethiopia. Several databases were searched to retrieve the available articles. Heterogeneity and publication bias were assessed using the Galbraith plot with Cochrane I<sup>2</sup> statistics and funnel plot with Egger's test, respectively. Subgroup analysis was done to identify the cause of the substantial heterogeneity. The pooled prevalence of favorable attitudes about blood donation was 65.28% (60.10–70.47). A higher prevalence was reported among studies conducted after 2020, in Northern Ethiopia and among health care professionals: 72.66%, 68.45%, and 69.41%, respectively. The percentages of people who had good feelings, willing to donate, and encouraged others to donate are 83.99%, 74.23%, and 77.96%, respectively. Conversely, 42.84% of participants believe that risk will happen following donation. There was an association between knowledge and attitude towards blood donation (AOR = 1.76; 95% CI: 1.48–2.99). The findings of this study may imply the preparation of a blood donation campaign that helps the community. Concerned bodies from governmental and non-governmental organizations may arrange and design community education, which may increase the number of voluntary donors.
DOI: 10.6084/m9.figshare.25848621.v1
title: Attitude towards blood donation and its associated factors, types of blood donation, willingness, and feeling towards blood donation among potential blood donors in Ethiopia: systematic review and meta-analysis, observational study

description: Blood donation is crucial for certain populations, such as pregnant mothers, anemic patients, traumatized patients, and individuals undergoing surgery. The imbalance between the number of blood donors and the demand for blood in Ethiopia is a serious public health concern. Having a favorable attitude towards blood donation could aid in correcting this imbalance. Therefore, this study aimed to assess the proportion of favorable attitudes, types of blood donation, willingness, and feelings towards blood donation in Ethiopia. Several databases were searched to retrieve the available articles. Heterogeneity and publication bias were assessed using the Galbraith plot with Cochrane I<sup>2</sup> statistics and funnel plot with Egger's test, respectively. Subgroup analysis was done to identify the cause of the substantial heterogeneity. The pooled prevalence of favorable attitudes about blood donation was 65.28% (60.10–70.47). A higher prevalence was reported among studies conducted after 2020, in Northern Ethiopia and among health care professionals: 72.66%, 68.45%, and 69.41%, respectively. The percentages of people who had good feelings, willing to donate, and encouraged others to donate are 83.99%, 74.23%, and 77.96%, respectively. Conversely, 42.84% of participants believe that risk will happen following donation. There was an association between knowledge and attitude towards blood donation (AOR = 1.76; 95% CI: 1.48–2.99). The findings of this study may imply the preparation of a blood donation campaign that helps the community. Concerned bodies from governmental and non-governmental organizations may arrange and design community education, which may increase the number of voluntary donors.
DOI: 10.6084/m9.figshare.26318857
title: Role of metabolites in mediating the effect of triacylglycerol on aplastic anemia

description: Observational studies have found a link between lipid metabolism disorders and aplastic anemia (AA). However, due to confounding variables and reverse causation, it is difficult to conclude such a causal link. The precise mechanism and potential implications of lipid metabolism disorder in AA remain unclear, necessitating further studies in this area. This study aimed to examine the causal relationship between 38 different subtypes of triacylglycerols and AA using two-sample Mendelian randomization (MR). Additionally, two-step MR analyses were conducted to investigate the mediating effects of vitamin A to oleoyl-linoleoyl-glycerol (18:1–18:2) ratio. MR analysis showed that triacylglycerol (53:3) levels were positively associated with the risk of AA [inverse variance weighting (IVW): odds ratio (OR) = 1.131,95% confidence interval (CI):1.029-1.243, <i>P </i>= 0.011; Bayesian weighted MR (BWMR): OR = 1.137,95% CI:1.031-1.254, <i>P </i>= 0.010]. Triacylglycerol (53:3) level showed no inverse causality with AA (IVW:<i>P </i>= 0.834; BWMR:<i>P </i>= 0.349). Mediation analyses showed that increasing the vitamin A to oleoyl-linoleoyl-glycerol (18:1–18:2) ratio can decrease the risk of AA. This study revealed the association between vitamin A to oleoyl-linoleoyl-glycerol (18:1–18:2) ratio, triacylglycerol (53:3) levels and AA, and indicated that lowering triacylglycerol (53:3) levels can reduce the risk of AA.
DOI: 10.6084/m9.figshare.26318857.v1
title: Role of metabolites in mediating the effect of triacylglycerol on aplastic anemia

description: Observational studies have found a link between lipid metabolism disorders and aplastic anemia (AA). However, due to confounding variables and reverse causation, it is difficult to conclude such a causal link. The precise mechanism and potential implications of lipid metabolism disorder in AA remain unclear, necessitating further studies in this area. This study aimed to examine the causal relationship between 38 different subtypes of triacylglycerols and AA using two-sample Mendelian randomization (MR). Additionally, two-step MR analyses were conducted to investigate the mediating effects of vitamin A to oleoyl-linoleoyl-glycerol (18:1–18:2) ratio. MR analysis showed that triacylglycerol (53:3) levels were positively associated with the risk of AA [inverse variance weighting (IVW): odds ratio (OR) = 1.131,95% confidence interval (CI):1.029-1.243, <i>P </i>= 0.011; Bayesian weighted MR (BWMR): OR = 1.137,95% CI:1.031-1.254, <i>P </i>= 0.010]. Triacylglycerol (53:3) level showed no inverse causality with AA (IVW:<i>P </i>= 0.834; BWMR:<i>P </i>= 0.349). Mediation analyses showed that increasing the vitamin A to oleoyl-linoleoyl-glycerol (18:1–18:2) ratio can decrease the risk of AA. This study revealed the association between vitamin A to oleoyl-linoleoyl-glycerol (18:1–18:2) ratio, triacylglycerol (53:3) levels and AA, and indicated that lowering triacylglycerol (53:3) levels can reduce the risk of AA.
DOI: 10.6084/m9.figshare.26206784
title: A retrospective study of prognostic factors and treatment outcome in advanced-stage Mycosis Fungoides and Sezary Syndrome

description: <b>Background</b>: Mycosis fungoides (MF) and Sezary Syndrome (SS) comprise over half of all Cutaneous T-cell lymphoma diagnoses. Current risk stratification is largely based on TNMB staging, few research investigated the prognostic value of clinical exams. Current systemic therapy for advanced disease includes immunomodulatory drugs, chemotherapy, and HADC inhibitors. Few clinical trials or retrospective research compared the efficacy of different drugs. <b>Method</b>: Here, we performed a retrospective analysis of prognostic factors and treatment outcomes of 92 patients diagnosed with MF/SS at the Peking Union Medical College Hospital from 2013-2023. <b>Results</b>: Cox regression analysis identified that age ≥ 50 years, WBC ≥ 8 × 10<sup>9</sup>/L, serum LDH ≥ 250U/L, β2-MG ≥ 4.50 mg/L, and stage IV were associated with reduced overall survival, age ≥ 50 years, serum LDH ≥ 250U/L and stage IV were associated with reduced progression free survival. Kaplan-Meier analysis established that immunomodulatory therapy was associated with longer progression free survival. <b>Conclusion</b>: These results suggested new factors in predicting prognosis and selecting appropriate treatments in patients with advanced MF/SS.
DOI: 10.6084/m9.figshare.26206784.v1
title: A retrospective study of prognostic factors and treatment outcome in advanced-stage Mycosis Fungoides and Sezary Syndrome

description: <b>Background</b>: Mycosis fungoides (MF) and Sezary Syndrome (SS) comprise over half of all Cutaneous T-cell lymphoma diagnoses. Current risk stratification is largely based on TNMB staging, few research investigated the prognostic value of clinical exams. Current systemic therapy for advanced disease includes immunomodulatory drugs, chemotherapy, and HADC inhibitors. Few clinical trials or retrospective research compared the efficacy of different drugs. <b>Method</b>: Here, we performed a retrospective analysis of prognostic factors and treatment outcomes of 92 patients diagnosed with MF/SS at the Peking Union Medical College Hospital from 2013-2023. <b>Results</b>: Cox regression analysis identified that age ≥ 50 years, WBC ≥ 8 × 10<sup>9</sup>/L, serum LDH ≥ 250U/L, β2-MG ≥ 4.50 mg/L, and stage IV were associated with reduced overall survival, age ≥ 50 years, serum LDH ≥ 250U/L and stage IV were associated with reduced progression free survival. Kaplan-Meier analysis established that immunomodulatory therapy was associated with longer progression free survival. <b>Conclusion</b>: These results suggested new factors in predicting prognosis and selecting appropriate treatments in patients with advanced MF/SS.
DOI: 10.6084/m9.figshare.25333833
title: Hub genes and associated drugs for multiple myeloma with 1q21+: identified by bioinformatic analysis

description: While 1q21+ was common genetic alteration and found to have adverse effect on prognosis, the underlying genes remain unclear. Identification of related genes may provide additional help for rational intervention. The microarray dataset GSE2658 associated with MM was downloaded from the Gene Expression Omnibus (GEO) database. The differentially expressed genes (DEGs) were obtained, and Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analyses were performed to annotate their functions. The hub genes were derived from the combined results of up-regulated DEGs and weighted gene coexpression network analysis (WGCNA). The receiver operating characteristic (ROC) curves of hub genes were plotted to evaluate correlation with 1q21+. Survival analysis and drug-gene interaction of hub genes were performed separately to find the prognostic value and potential targeted drugs. A total of 55 DEGs were identified. GO and KEGG pathway analyses suggested that the DEGs were related to several pathways of cell proliferation. <i>NVL, IL6R, DUSP23</i> were proven to be highly correlated with 1q21+ and have adverse effects on prognosis. <i>IL6R, DUSP23</i> were matched to known interaction-drug. This study revealed potential roles of hub genes in the pathogenesis and progression of MM with 1q21+, further investigations are needed to elucidate the mechanisms.
DOI: 10.6084/m9.figshare.25333833.v1
title: Hub genes and associated drugs for multiple myeloma with 1q21+: identified by bioinformatic analysis

description: While 1q21+ was common genetic alteration and found to have adverse effect on prognosis, the underlying genes remain unclear. Identification of related genes may provide additional help for rational intervention. The microarray dataset GSE2658 associated with MM was downloaded from the Gene Expression Omnibus (GEO) database. The differentially expressed genes (DEGs) were obtained, and Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analyses were performed to annotate their functions. The hub genes were derived from the combined results of up-regulated DEGs and weighted gene coexpression network analysis (WGCNA). The receiver operating characteristic (ROC) curves of hub genes were plotted to evaluate correlation with 1q21+. Survival analysis and drug-gene interaction of hub genes were performed separately to find the prognostic value and potential targeted drugs. A total of 55 DEGs were identified. GO and KEGG pathway analyses suggested that the DEGs were related to several pathways of cell proliferation. <i>NVL, IL6R, DUSP23</i> were proven to be highly correlated with 1q21+ and have adverse effects on prognosis. <i>IL6R, DUSP23</i> were matched to known interaction-drug. This study revealed potential roles of hub genes in the pathogenesis and progression of MM with 1q21+, further investigations are needed to elucidate the mechanisms.
DOI: 10.6084/m9.figshare.25450653
title: Expert consensus on the management of pharmacodynamic breakthrough-hemolysis in treated paroxysmal nocturnal hemoglobinuria

description: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, non-malignant hematologic disease characterized by complement-mediated hemolysis (with or without hemoglobinuria), fatigue, increased susceptibility to thrombosis, and bone marrow dysfunction. The development of complement inhibitors has transformed outcomes for patients with PNH, but patients may still experience pharmacodynamic breakthrough hemolysis (BTH), which can be caused by exposure to a complement amplifying condition (CAC), such as vaccination, infection, or surgery. A 13-member expert panel used a validated methodology (a RAND/UCLA modified Delphi panel) to develop consensus on how to classify pharmacodynamic BTH in patients with complement-inhibitor treated PNH. Physicians reviewed literature, rated the appropriateness of over 400 scenarios, and discussed the ratings at an in-person meeting. After the meeting, the panel agreed on 77% of scenarios. Here, we present the group’s agreed-upon recommendations on how to manage BTH caused by a CAC, as well as provide a severity classification system for BTH and strategies to mitigate risk of BTH in special circumstances (e.g. vaccination, planned or unplanned surgery, and pregnancy). In general, as severity of BTH increased, experts agreed more interventions to manage the BTH were appropriate. These recommendations are based on clinical experience and opinion. Without clear data from randomized trials to guide the management of BTH, expert opinion can be useful to support patient care.
DOI: 10.6084/m9.figshare.25450653.v1
title: Expert consensus on the management of pharmacodynamic breakthrough-hemolysis in treated paroxysmal nocturnal hemoglobinuria

description: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, non-malignant hematologic disease characterized by complement-mediated hemolysis (with or without hemoglobinuria), fatigue, increased susceptibility to thrombosis, and bone marrow dysfunction. The development of complement inhibitors has transformed outcomes for patients with PNH, but patients may still experience pharmacodynamic breakthrough hemolysis (BTH), which can be caused by exposure to a complement amplifying condition (CAC), such as vaccination, infection, or surgery. A 13-member expert panel used a validated methodology (a RAND/UCLA modified Delphi panel) to develop consensus on how to classify pharmacodynamic BTH in patients with complement-inhibitor treated PNH. Physicians reviewed literature, rated the appropriateness of over 400 scenarios, and discussed the ratings at an in-person meeting. After the meeting, the panel agreed on 77% of scenarios. Here, we present the group’s agreed-upon recommendations on how to manage BTH caused by a CAC, as well as provide a severity classification system for BTH and strategies to mitigate risk of BTH in special circumstances (e.g. vaccination, planned or unplanned surgery, and pregnancy). In general, as severity of BTH increased, experts agreed more interventions to manage the BTH were appropriate. These recommendations are based on clinical experience and opinion. Without clear data from randomized trials to guide the management of BTH, expert opinion can be useful to support patient care.