cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.4626259
title: PowerPoint Slides for: Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis

description: <b><i>Background:</i></b> Fibrillary glomerulonephritis is characterized by randomly arranged fibrils, approximately 20 nm in diameter by electron microscopy. Patients present with proteinuria, hematuria and kidney insufficiency, and about half of the reported patients progress to end-stage kidney disease within 4 years. The dependence of patient characteristics and outcomes on race has not been explored. In this study, we describe a cohort of patients with fibrillary glomerulonephritis and compare their clinical characteristics and outcomes with those of patients previously described. <b><i>Methods:</i></b> The University of North Carolina (UNC) Nephropathology Database was used to retrospectively identify patients diagnosed with fibrillary glomerulonephritis between 1985 and 2015. Of these patients, those treated at UNC were selected. Their demographic and clinical characteristics - including signs and symptoms, comorbidities, laboratory values, treatments and outcomes - were compared with those of patients described earlier. <b><i>Results:</i></b> Among the 287 patients identified, 42 were treated at the UNC Kidney Center. When compared to earlier cohorts, a higher frequency of black race, hepatitis C virus (HCV) infection and use of hemodialysis were noted in both black and HCV-positive patients. Autoimmune diseases, infections and malignancies were frequently observed, present in over half of all cases. <b><i>Conclusion:</i></b> According to this study, fibrillary glomerulonephritis represents a secondary glomerular disease process (associated with autoimmune disease, infection or malignancy) in many cases and hence screening is essential. As the screening for comorbidities increased over time, more underlying causes were identified. We noted a high frequency of HCV among black patients, suggesting a possible causative association. Treatment of underlying disease is essential for patients for the best outcome.
DOI: 10.6084/m9.figshare.4626220
title: PowerPoint Slides for: Rationale and Design of a Clinical Trial Investigating Tolvaptan Safety and Efficacy in Autosomal Dominant Polycystic Kidney Disease

description: <b><i>Background:</i></b> In TEMPO 3:4, the vasopressin V2-receptor antagonist tolvaptan slowed kidney growth and function decline in autosomal dominant polycystic kidney disease (ADPKD) patients with relatively preserved kidney function. <b><i>Methods:</i></b> Prospective, phase 3b, multi-center, randomized-withdrawal, placebo-controlled, double-blind trial of tolvaptan in ADPKD patients with late stage 2 to early stage 4 chronic kidney disease (CKD). The primary endpoint was estimated glomerular filtration rate (eGFR) change from pre-treatment baseline to post-treatment follow-up. Secondary endpoints included annualized eGFR slope, incidence of ADPKD complications, and overall and hepatic safety profiles. Participants were 18-55 year-old ADPKD patients with baseline eGFR ≥25 and ≤65 mL/min/1.73 m<sup>2</sup> or 56-65 year-old with eGFR ≥25 and ≤44 mL/min/1.73 m<sup>2</sup> and evidence of eGFR decline &gt;2.0 mL/min/1.73 m<sup>2</sup> per year. Daily split doses of tolvaptan were titrated to tolerance (30/15, 45/15, 60/30, or 90/30 mg) and maintained for 12 months, after an 8-week pre-randomization period to screen out subjects unable to tolerate at least 60/30 mg for 3 weeks. <b><i>Results:</i></b> Of 1,495 subjects who entered the tolvaptan titration period, 125 (8.4%) discontinued the study before randomization. One thousand three hundred seventy subjects (684 tolvaptan, 686 placebo) from 213 centers across 21 countries were randomized. Baseline demographics were well balanced across treatment arms. Information collected during the study included eGFR, survey scores (PKD history and outcome), adverse events, vital signs, hematology, urinalysis, and serum chemistry tests. <b><i>Conclusion:</i></b> Replicating Evidence of Preserved Renal Function: An Investigation of Tolvaptan Safety and Efficacy (REPRISE) determines whether tolvaptan administered over 1 year exhibits disease-modifying properties in ADPKD patients with late stage 2 to early stage 4 CKD, which provides an important therapeutic advancement for this difficult-to-treat disease.
DOI: 10.6084/m9.figshare.4626259.v1
title: PowerPoint Slides for: Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis

description: <b><i>Background:</i></b> Fibrillary glomerulonephritis is characterized by randomly arranged fibrils, approximately 20 nm in diameter by electron microscopy. Patients present with proteinuria, hematuria and kidney insufficiency, and about half of the reported patients progress to end-stage kidney disease within 4 years. The dependence of patient characteristics and outcomes on race has not been explored. In this study, we describe a cohort of patients with fibrillary glomerulonephritis and compare their clinical characteristics and outcomes with those of patients previously described. <b><i>Methods:</i></b> The University of North Carolina (UNC) Nephropathology Database was used to retrospectively identify patients diagnosed with fibrillary glomerulonephritis between 1985 and 2015. Of these patients, those treated at UNC were selected. Their demographic and clinical characteristics - including signs and symptoms, comorbidities, laboratory values, treatments and outcomes - were compared with those of patients described earlier. <b><i>Results:</i></b> Among the 287 patients identified, 42 were treated at the UNC Kidney Center. When compared to earlier cohorts, a higher frequency of black race, hepatitis C virus (HCV) infection and use of hemodialysis were noted in both black and HCV-positive patients. Autoimmune diseases, infections and malignancies were frequently observed, present in over half of all cases. <b><i>Conclusion:</i></b> According to this study, fibrillary glomerulonephritis represents a secondary glomerular disease process (associated with autoimmune disease, infection or malignancy) in many cases and hence screening is essential. As the screening for comorbidities increased over time, more underlying causes were identified. We noted a high frequency of HCV among black patients, suggesting a possible causative association. Treatment of underlying disease is essential for patients for the best outcome.
DOI: 10.6084/m9.figshare.4626193
title: Supplementary Material for: Antibacterial Defense of Human Airway Epithelial Cells from Chronic Obstructive Pulmonary Disease Patients Induced by Acute Exposure to Nontypeable Haemophilus influenzae: Modulation by Cigarette Smoke

description: Antimicrobial proteins and peptides (AMPs) are a central component of the antibacterial activity of airway epithelial cells. It has been proposed that a decrease in antibacterial lung defense contributes to an increased susceptibility to microbial infection in smokers and patients with chronic obstructive pulmonary disease (COPD). However, whether reduced AMP expression in the epithelium contributes to this lower defense is largely unknown. We investigated the bacterial killing activity and expression of AMPs by air-liquid interface-cultured primary bronchial epithelial cells from COPD patients and non-COPD (ex-)smokers that were stimulated with nontypeable <i>Haemophilus influenzae </i>(NTHi). In addition, the effect of cigarette smoke on AMP expression and the activation of signaling pathways was determined. COPD cell cultures displayed reduced antibacterial activity, whereas smoke exposure suppressed the NTHi-induced expression of AMPs and further increased IL-8 expression in COPD and non-COPD cultures. Moreover, smoke exposure impaired NTHi-induced activation of NF-κB, but not MAP-kinase signaling. Our findings demonstrate that the antibacterial activity of cultured airway epithelial cells induced by acute bacterial exposure was reduced in COPD and suppressed by cigarette smoke, whereas inflammatory responses persisted. These findings help to explain the imbalance between protective antibacterial and destructive inflammatory innate immune responses in COPD.
DOI: 10.6084/m9.figshare.4626175
title: Supplementary Material for: Comparison of 55° Wide-Field Spectral Domain Optical Coherence Tomography and Conventional 30° Optical Coherence Tomography for the Assessment of Diabetic Macular Edema

description: <b><i>Purpose:</i></b> To compare conventional 30° spectral domain optical coherence tomography (SD-OCT) with 55° wide-field SD-OCT for the assessment of diabetic macular edema (DME). <b><i>Methods:</i></b> This study included 50 DME patients. Both 55° and 30° SD-OCT was conducted. Two readers evaluated scans according to a standardized grading protocol. Intergrader agreement as well as agreement between 30° and 55° SD-OCT were assessed. <b><i>Results:</i></b> Intergrader agreement (κ) was strong and ranged from 0.79 to 1.0. Perfect interdevice agreement (κ = 1.0) was found for the detection of intra- and subretinal fluid. Excellent agreement (κ ≥ 0.9) was found for the presence of epiretinal membrane (κ = 0.92) and cotton-wool spots (κ = 0.92). A strong agreement was found for the presence of hard exudates (κ = 0.89) and microaneurysms (κ = 0.81). A moderate correlation was found for ellipsoid zone integrity (κ = 0.69) and configuration of the vitreomacular interface (VMI) (κ = 0.69). A weak agreement was found for retinal pigment epithelium atrophy (κ = 0.51) and external limiting membrane integrity (κ = 0.35). <b><i>Conclusion:</i></b> Wide-field OCT imaging may be beneficial for evaluating DME, particularly for assessing the VMI and the integrity of hyperreflective bands.
DOI: 10.6084/m9.figshare.4626184
title: Supplementary Material for: Selective Biological Responses of Phagocytes and Lungs to Purified Histones

description: Histones invoke strong proinflammatory responses in many different organs and cells. We assessed biological responses to purified or recombinant histones, using human and murine phagocytes and mouse lungs. H1 had the strongest ability in vitro to induce cell swelling independent of requirements for toll-like receptors (TLRs) 2 or 4. These responses were also associated with lactate dehydrogenase release. H3 and H2B were the strongest inducers of [Ca<sup>2+</sup>]i elevations in phagocytes. Cytokine and chemokine release from mouse and human phagocytes was predominately a function of H2A and H2B. Double TLR2 and TLR4 knockout (KO) mice had dramatically reduced cytokine release induced in macrophages exposed to individual histones. In contrast, macrophages from single TLR-KO mice showed few inhibitory effects on cytokine production. Using the NLRP3 inflammasome protocol, release of mature IL-1β was predominantly a feature of H1. Acute lung injury following the airway delivery of histones suggested that H1, H2A, and H2B were linked to alveolar leak of albumin and the buildup of polymorphonuclear neutrophils as well as the release of chemokines and cytokines into bronchoalveolar fluids. These results demonstrate distinct biological roles for individual histones in the context of inflammation biology and the requirement of both TLR2 and TLR4.
DOI: 10.6084/m9.figshare.4626193.v1
title: Supplementary Material for: Antibacterial Defense of Human Airway Epithelial Cells from Chronic Obstructive Pulmonary Disease Patients Induced by Acute Exposure to Nontypeable Haemophilus influenzae: Modulation by Cigarette Smoke

description: Antimicrobial proteins and peptides (AMPs) are a central component of the antibacterial activity of airway epithelial cells. It has been proposed that a decrease in antibacterial lung defense contributes to an increased susceptibility to microbial infection in smokers and patients with chronic obstructive pulmonary disease (COPD). However, whether reduced AMP expression in the epithelium contributes to this lower defense is largely unknown. We investigated the bacterial killing activity and expression of AMPs by air-liquid interface-cultured primary bronchial epithelial cells from COPD patients and non-COPD (ex-)smokers that were stimulated with nontypeable <i>Haemophilus influenzae </i>(NTHi). In addition, the effect of cigarette smoke on AMP expression and the activation of signaling pathways was determined. COPD cell cultures displayed reduced antibacterial activity, whereas smoke exposure suppressed the NTHi-induced expression of AMPs and further increased IL-8 expression in COPD and non-COPD cultures. Moreover, smoke exposure impaired NTHi-induced activation of NF-κB, but not MAP-kinase signaling. Our findings demonstrate that the antibacterial activity of cultured airway epithelial cells induced by acute bacterial exposure was reduced in COPD and suppressed by cigarette smoke, whereas inflammatory responses persisted. These findings help to explain the imbalance between protective antibacterial and destructive inflammatory innate immune responses in COPD.
DOI: 10.6084/m9.figshare.4626166
title: List of 2016 albums

description: List of 2016 albums filtered per music genres<br>
DOI: 10.6084/m9.figshare.4626184.v1
title: Supplementary Material for: Selective Biological Responses of Phagocytes and Lungs to Purified Histones

description: Histones invoke strong proinflammatory responses in many different organs and cells. We assessed biological responses to purified or recombinant histones, using human and murine phagocytes and mouse lungs. H1 had the strongest ability in vitro to induce cell swelling independent of requirements for toll-like receptors (TLRs) 2 or 4. These responses were also associated with lactate dehydrogenase release. H3 and H2B were the strongest inducers of [Ca<sup>2+</sup>]i elevations in phagocytes. Cytokine and chemokine release from mouse and human phagocytes was predominately a function of H2A and H2B. Double TLR2 and TLR4 knockout (KO) mice had dramatically reduced cytokine release induced in macrophages exposed to individual histones. In contrast, macrophages from single TLR-KO mice showed few inhibitory effects on cytokine production. Using the NLRP3 inflammasome protocol, release of mature IL-1β was predominantly a feature of H1. Acute lung injury following the airway delivery of histones suggested that H1, H2A, and H2B were linked to alveolar leak of albumin and the buildup of polymorphonuclear neutrophils as well as the release of chemokines and cytokines into bronchoalveolar fluids. These results demonstrate distinct biological roles for individual histones in the context of inflammation biology and the requirement of both TLR2 and TLR4.
DOI: 10.6084/m9.figshare.4626163
title: Supplementary Material for: Prognostic Value of the Delivery Dialysis Dose on Twice-Weekly Hemodialysis Patients

description: <b><i>Background:</i></b> Few studies have evaluated the prognostic value of dialysis dose in twice-weekly hemodialysis (HD). A single-pool Kt/V (spKt/V) over 1.70 may benefit patients receiving twice-weekly maintenance HD. <b><i>Methods:</i></b> This is a multicenter randomized controlled trial performed on 163 patients from 17 dialysis centers in Shanghai who were allocated to high- (<i>n</i> = 98) and standard-dose groups (<i>n</i> = 65) and followed through 96 weeks of study period. Therapeutic approaches were given to increase spKt/V to over 1.70 in the high-dose group. Data were collected every 12-24 weeks. The primary outcomes were all-cause mortality and major adverse cardio-cerebrovascular events (MACEs) occurrence, and secondary outcomes included residual kidney function (RKF) and health-related quality of life (HR-QOL). <b><i>Results:</i></b> The spKt/V in high-dose and standard-dose groups were 1.80 ± 0.23 and 1.55 ± 0.19, respectively, after an 8-week intervention (<i>p</i> &lt; 0.001). At the end of the study, SF-36 physical function and total score in high-dose group were 82 (69-90) and 74 (47-84), respectively, both of which were higher than those in the standard-dose group. Decline in urine volume was observed in both groups with no significant difference (<i>p</i> = 0.431). No difference was found in overall survival between the 2 groups (<i>p</i> = 0.580). The 1-year MACE-free survival for high-dose group was 84.49%, better than 76.72% for standard-dose group (<i>p</i> = 0.029). <b><i>Conclusions:</i></b> Higher spKt/V is also associated with MACE-free survival and better HR-QOL, especially in physical function aspect for twice-weekly dialysis patients. Increasing spKt/V over 1.70 in twice-weekly HD population does not cause loss of RKF.
DOI: 10.6084/m9.figshare.4626166.v1
title: List of 2016 albums

description: List of 2016 albums filtered per music genres<br>
DOI: 10.6084/m9.figshare.4626175.v1
title: Supplementary Material for: Comparison of 55° Wide-Field Spectral Domain Optical Coherence Tomography and Conventional 30° Optical Coherence Tomography for the Assessment of Diabetic Macular Edema

description: <b><i>Purpose:</i></b> To compare conventional 30° spectral domain optical coherence tomography (SD-OCT) with 55° wide-field SD-OCT for the assessment of diabetic macular edema (DME). <b><i>Methods:</i></b> This study included 50 DME patients. Both 55° and 30° SD-OCT was conducted. Two readers evaluated scans according to a standardized grading protocol. Intergrader agreement as well as agreement between 30° and 55° SD-OCT were assessed. <b><i>Results:</i></b> Intergrader agreement (κ) was strong and ranged from 0.79 to 1.0. Perfect interdevice agreement (κ = 1.0) was found for the detection of intra- and subretinal fluid. Excellent agreement (κ ≥ 0.9) was found for the presence of epiretinal membrane (κ = 0.92) and cotton-wool spots (κ = 0.92). A strong agreement was found for the presence of hard exudates (κ = 0.89) and microaneurysms (κ = 0.81). A moderate correlation was found for ellipsoid zone integrity (κ = 0.69) and configuration of the vitreomacular interface (VMI) (κ = 0.69). A weak agreement was found for retinal pigment epithelium atrophy (κ = 0.51) and external limiting membrane integrity (κ = 0.35). <b><i>Conclusion:</i></b> Wide-field OCT imaging may be beneficial for evaluating DME, particularly for assessing the VMI and the integrity of hyperreflective bands.
DOI: 10.6084/m9.figshare.4626163.v1
title: Supplementary Material for: Prognostic Value of the Delivery Dialysis Dose on Twice-Weekly Hemodialysis Patients

description: <b><i>Background:</i></b> Few studies have evaluated the prognostic value of dialysis dose in twice-weekly hemodialysis (HD). A single-pool Kt/V (spKt/V) over 1.70 may benefit patients receiving twice-weekly maintenance HD. <b><i>Methods:</i></b> This is a multicenter randomized controlled trial performed on 163 patients from 17 dialysis centers in Shanghai who were allocated to high- (<i>n</i> = 98) and standard-dose groups (<i>n</i> = 65) and followed through 96 weeks of study period. Therapeutic approaches were given to increase spKt/V to over 1.70 in the high-dose group. Data were collected every 12-24 weeks. The primary outcomes were all-cause mortality and major adverse cardio-cerebrovascular events (MACEs) occurrence, and secondary outcomes included residual kidney function (RKF) and health-related quality of life (HR-QOL). <b><i>Results:</i></b> The spKt/V in high-dose and standard-dose groups were 1.80 ± 0.23 and 1.55 ± 0.19, respectively, after an 8-week intervention (<i>p</i> &lt; 0.001). At the end of the study, SF-36 physical function and total score in high-dose group were 82 (69-90) and 74 (47-84), respectively, both of which were higher than those in the standard-dose group. Decline in urine volume was observed in both groups with no significant difference (<i>p</i> = 0.431). No difference was found in overall survival between the 2 groups (<i>p</i> = 0.580). The 1-year MACE-free survival for high-dose group was 84.49%, better than 76.72% for standard-dose group (<i>p</i> = 0.029). <b><i>Conclusions:</i></b> Higher spKt/V is also associated with MACE-free survival and better HR-QOL, especially in physical function aspect for twice-weekly dialysis patients. Increasing spKt/V over 1.70 in twice-weekly HD population does not cause loss of RKF.
DOI: 10.6084/m9.figshare.4626130
title: Online Appendix A4 - Supplementary results.pdf

description: <b>The “Inventor Balance” and the Functional Specialization </b><b>in Global Inventive Activities</b><b>Appendix A.4 Supplementary results</b>
DOI: 10.6084/m9.figshare.4626133
title: Supplementary Material for: Exome Sequencing Identifies a Novel DES Mutation (R227C) in a Chinese Dilated Cardiomyopathy Family

description: <b><i>Objectives:</i></b> Dilated cardiomyopathy (DCM) is a common disease in the clinic, and it is the leading cause of heart failure and sudden cardiac death. Previous studies have proven that genetic factors play a crucial role in the occurrence of DCM; more than 50 disease genes including <i>desmin </i>(<i>DES</i>) have been identified to be associated with DCM. At present, most<i> DES </i>mutations are reported in desmin-related myofibrilla myopathy patients, but variants leading to isolated DCM are rarely reported. <b><i>Methods:</i></b> We applied whole-exome sequencing and cardiomyopathy-related gene filtering strategies to discover the genetic factors in a Chinese DCM family. <b><i>Results:</i></b> A novel mutation (c.679 C&gt;T /p.R227C) in exon 3 of<i> DES</i> was identified and cosegregated with the affected members of a Chinese family with isolated DCM phenotypes (left ventricle and left atrial diameters). <b><i>Conclusion:</i></b> This mutation leads to a substitution of arginine by cysteine and it is predicted to be deleterious by bioinformatics programs. Our study not only contributes to the genetic diagnosis and counseling of families with DCM, but it also further proves that <i>DES </i>mutations may lead to isolated DCM and provides a new case for the study of the relationship between <i>DES</i> mutations and DCM.
DOI: 10.6084/m9.figshare.4626130.v1
title: Online Appendix A4 - Supplementary results.pdf

description: <b>The “Inventor Balance” and the Functional Specialization </b><b>in Global Inventive Activities</b><b>Appendix A.4 Supplementary results</b>
DOI: 10.6084/m9.figshare.4625986
title: Wilken_et_al_Dataset.gp

description: Representative protein sequences of the known Ascomycete mating proteins renamed in the review by Wilken et al. (Fungal Biology Reviews, 2017).<br>
DOI: 10.6084/m9.figshare.4625974
title: Supplementary Material for: Therapeutic Advances and New Directions for Triple-Negative Breast Cancer

description: Triple-negative breast cancer (TNBC) is a molecularly diverse grouping with poor prognosis for which chemotherapy remains the foundation of treatment. The molecular heterogeneity of the disease rationalizes its diverse biological behavior and differential response to treatment. Estimates of up to 20% of patients diagnosed have germline mutations in DNA-damage repair-pathway genes, namely BRCA1 and 2, and this can be used to select patients likely to respond to platinums and/or inhibitors of poly(ADP-ribose) polymerase (PARP). Similar strategies can be utilized in other subtypes of TNBC that have ‘BRCA-like’ tumor biology due to the presence of mutations in alternate DNA-damage repair genes. The diverse biological behavior of TNBC and its variable response to chemotherapy were largely decoded following genotyping studies that enabled the identification of distinct molecular subtypes, such that the biological and genetic heterogeneity of the disease could be understood. This subsequently enabled the identification of therapeutic ‘vulnerabilities’ for each subtype that encompass biological processes including proliferation, DNA repair, apoptosis, angiogenesis, immune modulation, and invasion and metastasis. To expedite the development of therapies for high-risk, early-stage breast cancer, we have adopted novel trial designs and re-defined endpoints as surrogates of clinical outcomes. The purpose of this review is to highlight the current standard and experimental treatment options for TNBC.