cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.14319310
title: Brazilian Dialysis Census: analysis of data from the 2009-2018 decade

description: ABSTRACT Introduction: National data on chronic dialysis treatment are essential for the development of health policies that aim to improve patient treatment. Objective: To present data from the Brazilian Society of Nephrology on patients with chronic dialysis for kidney disease in July 2018, making a comparative analysis of the past 10 years. Methods: Data collection from dialysis units, with filling in an online questionnaire for 2018. Data from 2009, 2013 and 2018 were compared. Results: 288 (36.6%) centers answered the questionnaire. In July 2018, the estimated total number of patients on dialysis was 133,464. Estimates of the prevalence and incidence rates of patients undergoing dialysis treatment per million of the population (pmp) were 640 and 204, respectively, with average annual increases of 23.5 pmp and 6 pmp for prevalence and incidence, respectively. The annual gross mortality rate was 19.5%. Of the prevalent patients, 92.3% were on hemodialysis and 7.7% on peritoneal dialysis, with 29,545 (22.1%) on the waiting list for transplantation. Median bicarbonate concentration in the hemodialysis bath was 32 mEq/L. Venous catheters were used as access in 23.6% of the hemodialysis patients. The prevalence rate of positive serology for hepatitis C showed a progressive reduction (3.2%). Conclusion: The absolute number of patients and rates of incidence and prevalence in dialysis in the country increased substantially in the period, although there are considerable differences in rates by state. There has been a persistent increase in the use of venous catheters as an access for dialysis; and reduction in the number of patients with positive serology for hepatitis C.
DOI: 10.6084/m9.figshare.14319310.v1
title: Brazilian Dialysis Census: analysis of data from the 2009-2018 decade

description: ABSTRACT Introduction: National data on chronic dialysis treatment are essential for the development of health policies that aim to improve patient treatment. Objective: To present data from the Brazilian Society of Nephrology on patients with chronic dialysis for kidney disease in July 2018, making a comparative analysis of the past 10 years. Methods: Data collection from dialysis units, with filling in an online questionnaire for 2018. Data from 2009, 2013 and 2018 were compared. Results: 288 (36.6%) centers answered the questionnaire. In July 2018, the estimated total number of patients on dialysis was 133,464. Estimates of the prevalence and incidence rates of patients undergoing dialysis treatment per million of the population (pmp) were 640 and 204, respectively, with average annual increases of 23.5 pmp and 6 pmp for prevalence and incidence, respectively. The annual gross mortality rate was 19.5%. Of the prevalent patients, 92.3% were on hemodialysis and 7.7% on peritoneal dialysis, with 29,545 (22.1%) on the waiting list for transplantation. Median bicarbonate concentration in the hemodialysis bath was 32 mEq/L. Venous catheters were used as access in 23.6% of the hemodialysis patients. The prevalence rate of positive serology for hepatitis C showed a progressive reduction (3.2%). Conclusion: The absolute number of patients and rates of incidence and prevalence in dialysis in the country increased substantially in the period, although there are considerable differences in rates by state. There has been a persistent increase in the use of venous catheters as an access for dialysis; and reduction in the number of patients with positive serology for hepatitis C.
DOI: 10.6084/m9.figshare.14319312.v1
title: Rare inherited kidney diseases: an evolving field in Nephrology

description: Abstract There are more than 150 different rare genetic kidney diseases. They can be classified according to diagnostic findings as (i) disorders of growth and structure, (ii) glomerular diseases, (iii) tubular, and (iv) metabolic diseases. In recent years, there has been a shift of paradigm in this field. Molecular testing has become more accessible, our understanding of the underlying pathophysiologic mechanisms of these diseases has evolved, and new therapeutic strategies have become more available. Therefore, the role of nephrologists has progressively shifted from a mere spectator to an active player, part of a multidisciplinary team in the diagnosis and treatment of these disorders. This article provides an overview of the recent advances in rare hereditary kidney disorders by discussing the genetic aspects, clinical manifestations, diagnostic, and therapeutic approaches of some of these disorders, named familial focal and segmental glomerulosclerosis, tuberous sclerosis complex, Fabry nephropathy, and MYH-9 related disorder.
DOI: 10.6084/m9.figshare.14319312
title: Rare inherited kidney diseases: an evolving field in Nephrology

description: Abstract There are more than 150 different rare genetic kidney diseases. They can be classified according to diagnostic findings as (i) disorders of growth and structure, (ii) glomerular diseases, (iii) tubular, and (iv) metabolic diseases. In recent years, there has been a shift of paradigm in this field. Molecular testing has become more accessible, our understanding of the underlying pathophysiologic mechanisms of these diseases has evolved, and new therapeutic strategies have become more available. Therefore, the role of nephrologists has progressively shifted from a mere spectator to an active player, part of a multidisciplinary team in the diagnosis and treatment of these disorders. This article provides an overview of the recent advances in rare hereditary kidney disorders by discussing the genetic aspects, clinical manifestations, diagnostic, and therapeutic approaches of some of these disorders, named familial focal and segmental glomerulosclerosis, tuberous sclerosis complex, Fabry nephropathy, and MYH-9 related disorder.
DOI: 10.6084/m9.figshare.14319313
title: Usefulness of Kidney Donor Profile Index (KDPI) to predict graft survival in a South Brazilian Cohort

description: Abstract Introduction: Kidney Donor Profile Index (KDPI) has been incorporated in the United States to improve the kidney transplant allocation system. Objectives: To evaluate deceased kidney donors’ profile using KDPI and compare to the previous United Network for Organ Sharing (UNOS) definition of expanded criteria donors (ECD) and assess the KDPI applicability to predict five-year graft survival and renal function in our sample. Methods: Retrospective cohort of 589 kidney transplants from deceased donors performed from January 2009 to May 2013 with follow-up until May 2018. Results: In 589 kidney transplants, 36.6% of donors were classified as ECD and 28.8% had KDPI ≥ 85%. Mean KDPI was 63.1 (95%CI: 60.8-65.3). There was an overlap of standard and ECD in KDPI between 60 and 95 and a significantly lower death-censored graft survival in KDPI ≥ 85% (78.6%); KDPI 0-20: 89.8%, KDPI 21-59: 91.6%, and KDPI 60-84: 83.0%; p = 0.006. The AUC-ROC was 0.577 (95%CI: 0.514-0.641; p = 0.027). Renal function at 5 years was significantly lower according to the incremental KDPI (p &lt; 0.002). KDPI (HR 1.011; 95%CI 1.001-1.020; p = 0.008), donor-specific antibodies (HR 2.77; 95%CI 1.69-4.54; p &lt; 0.001), acute rejection episode (HR 1.73; 95%CI 1.04-2.86; p = 0.034) were independent and significant risk factors for death-censored graft loss at 5 years. Conclusion: In our study, 36.6% were classified as ECD and 28.8% had KDPI ≥ 85%. KDPI score showed a moderate power to predict graft survival at 5 years. Renal function was significantly lower in patients with higher KDPI.
DOI: 10.6084/m9.figshare.14319313.v1
title: Usefulness of Kidney Donor Profile Index (KDPI) to predict graft survival in a South Brazilian Cohort

description: Abstract Introduction: Kidney Donor Profile Index (KDPI) has been incorporated in the United States to improve the kidney transplant allocation system. Objectives: To evaluate deceased kidney donors’ profile using KDPI and compare to the previous United Network for Organ Sharing (UNOS) definition of expanded criteria donors (ECD) and assess the KDPI applicability to predict five-year graft survival and renal function in our sample. Methods: Retrospective cohort of 589 kidney transplants from deceased donors performed from January 2009 to May 2013 with follow-up until May 2018. Results: In 589 kidney transplants, 36.6% of donors were classified as ECD and 28.8% had KDPI ≥ 85%. Mean KDPI was 63.1 (95%CI: 60.8-65.3). There was an overlap of standard and ECD in KDPI between 60 and 95 and a significantly lower death-censored graft survival in KDPI ≥ 85% (78.6%); KDPI 0-20: 89.8%, KDPI 21-59: 91.6%, and KDPI 60-84: 83.0%; p = 0.006. The AUC-ROC was 0.577 (95%CI: 0.514-0.641; p = 0.027). Renal function at 5 years was significantly lower according to the incremental KDPI (p &lt; 0.002). KDPI (HR 1.011; 95%CI 1.001-1.020; p = 0.008), donor-specific antibodies (HR 2.77; 95%CI 1.69-4.54; p &lt; 0.001), acute rejection episode (HR 1.73; 95%CI 1.04-2.86; p = 0.034) were independent and significant risk factors for death-censored graft loss at 5 years. Conclusion: In our study, 36.6% were classified as ECD and 28.8% had KDPI ≥ 85%. KDPI score showed a moderate power to predict graft survival at 5 years. Renal function was significantly lower in patients with higher KDPI.
DOI: 10.6084/m9.figshare.14319321
title: Vitamin D Toxicity

description: Abstract Fortification of food products with vitamin D was central to the eradication of rickets in the early parts of the 20th century in the United States. In the subsequent almost 100 years since, accumulating evidence has linked vitamin D deficiency to a variety of outcomes, and this has paralleled greater public interest and awareness of the health benefits of vitamin D. Supplements containing vitamin D are now widely available in both industrialized and developing countries, and many are in the form of unregulated formulations sold to the public with little guidance for safe administration. Together, this has contributed to a transition whereby a dramatic global increase in cases of vitamin D toxicity has been reported. Clinicians are now faced with the challenge of managing this condition that can present on a spectrum from asymptomatic to acute life-threatening complications. This article considers contemporary data on vitamin D toxicity, and diagnostic and management strategies relevant to clinical practice.
DOI: 10.6084/m9.figshare.14319321.v1
title: Vitamin D Toxicity

description: Abstract Fortification of food products with vitamin D was central to the eradication of rickets in the early parts of the 20th century in the United States. In the subsequent almost 100 years since, accumulating evidence has linked vitamin D deficiency to a variety of outcomes, and this has paralleled greater public interest and awareness of the health benefits of vitamin D. Supplements containing vitamin D are now widely available in both industrialized and developing countries, and many are in the form of unregulated formulations sold to the public with little guidance for safe administration. Together, this has contributed to a transition whereby a dramatic global increase in cases of vitamin D toxicity has been reported. Clinicians are now faced with the challenge of managing this condition that can present on a spectrum from asymptomatic to acute life-threatening complications. This article considers contemporary data on vitamin D toxicity, and diagnostic and management strategies relevant to clinical practice.
DOI: 10.6084/m9.figshare.14319325.v1
title: Anca negative pauci-immune crescentic glomerulonephritis and mixed connective tissue disease: a case study

description: Abstract One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.
DOI: 10.6084/m9.figshare.14319325
title: Anca negative pauci-immune crescentic glomerulonephritis and mixed connective tissue disease: a case study

description: Abstract One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.
DOI: 10.6084/m9.figshare.12137373
title: Data_FluidizedGranularFlows_ParticleDrivenGravityCurrent_BougouinEtAl

description: Experimental data of gravity currents generated by both fluidized granular and salt water flows entering water.<br>- Temporal evolution of fluidized granular flows (spreadsheet 1) and salt water flows (spreadsheet 2) entering fresh water, by varying the mass flux per width and the volume per width of the flow, and the maximum water depth.<br>- Experimental data (spreadsheet 3) of the mean height and the bulk particle concentration of gravity currents generated by fluidized granular and salt water flows.
DOI: 10.6084/m9.figshare.12137373.v2
title: Data_FluidizedGranularFlows_ParticleDrivenGravityCurrent_BougouinEtAl

description: Experimental data of gravity currents generated by both fluidized granular and salt water flows entering water.<br>- Temporal evolution of fluidized granular flows (spreadsheet 1) and salt water flows (spreadsheet 2) entering fresh water, by varying the mass flux per width and the volume per width of the flow, and the maximum water depth.<br>- Experimental data (spreadsheet 3) of the mean height and the bulk particle concentration of gravity currents generated by fluidized granular and salt water flows.
DOI: 10.6084/m9.figshare.12137373.v1
title: Data_FluidizedGranularCollapse_TurbidityCurrent_BougouinEtAl

description: Experimental data of gravity currents generated by both fluidized granular and salt water flows entering water.<br>- Temporal evolution of fluidized granular flows (spreadsheet 1) and salt water flows (spreadsheet 2) entering fresh water, by varying the mass flux per width and the volume per width of the flow, and the maximum water depth.<br>- Experimental data (spreadsheet 3) of the mean height and the bulk particle concentration of gravity currents generated by fluidized granular and salt water flows.
DOI: 10.6084/m9.figshare.14319331
title: Resistant starch supplementation effects on plasma indole 3-acetic acid and aryl hydrocarbon receptor mRNA expression in hemodialysis patients: Randomized, double blind and controlled clinical trial

description: ABSTRACT Introduction: Gut microbiota imbalance is linked to high uremic toxins production such as indole-3-acetic acid (IAA) in chronic kidney disease patients. This toxin can activate the aryl hydrocarbon receptor (AhR), a ligand-activated transcription factor involved with inflammation. Strategies to restore gut microbiota balance can be associated with reduced production of IAA and its deleterious effects. This study aimed to evaluate prebiotic resistant starch (RS) supplementation effects on IAA plasma levels and AhR mRNA expression in CKD patients on hemodialysis (HD). Methods: This randomized, double-blind and placebo-controlled clinical trial evaluated forty-two stable HD patients allocated in RS (n=22) or placebo (n=20) groups. Patients received, alternately, cookies and sachets containing 16 g/day of RS (Hi-Maize 260®) or manioc flour for four weeks. Fasting pre-dialysis blood samples were collected and IAA plasma levels measured by high performance liquid chromatography. Peripheral blood mononuclear cells were isolated and processed for AhR and nuclear factor kappa B (NF-κB) mRNA expression analyzes by quantitative real-time PCR. Anthropometric and biochemical parameters, as well as food intake were also evaluated. Results: Thirty-one patients completed the study, 15 in the RS group and 16 in the placebo group. Although there was no significant alteration in IAA plasma levels, neither in AhR mRNA expression and NF-κB mRNA expression after RS supplementation, a positive correlation (r=0.48; p=0.03) was observed between IAA plasma levels and AhR expression at baseline. Conclusion: Even though prebiotic RS supplementation did not influence IAA levels or AhR expression, their positive association reinforces a possible interaction between them.
DOI: 10.6084/m9.figshare.14319331.v1
title: Resistant starch supplementation effects on plasma indole 3-acetic acid and aryl hydrocarbon receptor mRNA expression in hemodialysis patients: Randomized, double blind and controlled clinical trial

description: ABSTRACT Introduction: Gut microbiota imbalance is linked to high uremic toxins production such as indole-3-acetic acid (IAA) in chronic kidney disease patients. This toxin can activate the aryl hydrocarbon receptor (AhR), a ligand-activated transcription factor involved with inflammation. Strategies to restore gut microbiota balance can be associated with reduced production of IAA and its deleterious effects. This study aimed to evaluate prebiotic resistant starch (RS) supplementation effects on IAA plasma levels and AhR mRNA expression in CKD patients on hemodialysis (HD). Methods: This randomized, double-blind and placebo-controlled clinical trial evaluated forty-two stable HD patients allocated in RS (n=22) or placebo (n=20) groups. Patients received, alternately, cookies and sachets containing 16 g/day of RS (Hi-Maize 260®) or manioc flour for four weeks. Fasting pre-dialysis blood samples were collected and IAA plasma levels measured by high performance liquid chromatography. Peripheral blood mononuclear cells were isolated and processed for AhR and nuclear factor kappa B (NF-κB) mRNA expression analyzes by quantitative real-time PCR. Anthropometric and biochemical parameters, as well as food intake were also evaluated. Results: Thirty-one patients completed the study, 15 in the RS group and 16 in the placebo group. Although there was no significant alteration in IAA plasma levels, neither in AhR mRNA expression and NF-κB mRNA expression after RS supplementation, a positive correlation (r=0.48; p=0.03) was observed between IAA plasma levels and AhR expression at baseline. Conclusion: Even though prebiotic RS supplementation did not influence IAA levels or AhR expression, their positive association reinforces a possible interaction between them.
DOI: 10.6084/m9.figshare.14319333
title: Urine hepcidin, netrin-1, neutrophil gelatinase-associated lipocalin and C-C motif chemokine ligand 2 levels in multicystic dysplastic kidney

description: ABSTRACT Introduction: Glomerular hyperfiltration may lead to proteinuria and chronic kidney disease in unilateral multicystic dysplastic kidney (MCDK). We aimed to investigate the urine neutrophil-gelatinase-associated lipocalin (NGAL), netrin-1, hepcidin, and C-C motif chemokine ligand-2 (MCP-1/CCL-2) levels in patients with MCDK. Methods: Thirty-two patients and 25 controls were included. The urine hepcidin, netrin-1, NGAL, and MCP-1/CCL-2 levels were determined by ELISA. Results: The patients had higher serum creatinine (Cr) levels, urine albumin, and netrin-1/Cr ratio with lower GFR. There were positive correlations between urine protein/Cr, MCP-1/CCL-2/Cr, and netrin-1 with NGAL (r = 0.397, p = 0.031; r = 0.437, p = 0.041, r = 0.323, p = 0.042, respectively). Urine netrin-1/Cr was positively correlated with MCP-1/CCL-2/Cr (r = 0.356, p = 0.045). There were positive associations between the presence of proteinuria and netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr [Odds ratio (OR): 1.423, p = 0.037, OR: 1.553, p = 0.033, OR: 2.112, p = 0.027, respectively)]. ROC curve analysis showed that netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr had high predictive values for determining proteinuria p = 0.027, p = 0.041, p = 0.035, respectively). Urine hepcidin/Cr was negatively correlated with tubular phosphorus reabsorption and was positively correlated with urine NGAL/Cr (r = -0.418, p = 0.019; r = 0.682, p = 0.000; respectively). Conclusions: MCP-1/CCL-2 may play a role in the development of proteinuria in MCDK. Netrin-1 may be a protective factor against proteinuria-induced renal injury. Urine hepcidin/Cr may reflect proximal tubule damage in MCDK. Urine NGAL/Cr may be a predictor of tubule damage by proteinuria.
DOI: 10.6084/m9.figshare.14319333.v1
title: Urine hepcidin, netrin-1, neutrophil gelatinase-associated lipocalin and C-C motif chemokine ligand 2 levels in multicystic dysplastic kidney

description: ABSTRACT Introduction: Glomerular hyperfiltration may lead to proteinuria and chronic kidney disease in unilateral multicystic dysplastic kidney (MCDK). We aimed to investigate the urine neutrophil-gelatinase-associated lipocalin (NGAL), netrin-1, hepcidin, and C-C motif chemokine ligand-2 (MCP-1/CCL-2) levels in patients with MCDK. Methods: Thirty-two patients and 25 controls were included. The urine hepcidin, netrin-1, NGAL, and MCP-1/CCL-2 levels were determined by ELISA. Results: The patients had higher serum creatinine (Cr) levels, urine albumin, and netrin-1/Cr ratio with lower GFR. There were positive correlations between urine protein/Cr, MCP-1/CCL-2/Cr, and netrin-1 with NGAL (r = 0.397, p = 0.031; r = 0.437, p = 0.041, r = 0.323, p = 0.042, respectively). Urine netrin-1/Cr was positively correlated with MCP-1/CCL-2/Cr (r = 0.356, p = 0.045). There were positive associations between the presence of proteinuria and netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr [Odds ratio (OR): 1.423, p = 0.037, OR: 1.553, p = 0.033, OR: 2.112, p = 0.027, respectively)]. ROC curve analysis showed that netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr had high predictive values for determining proteinuria p = 0.027, p = 0.041, p = 0.035, respectively). Urine hepcidin/Cr was negatively correlated with tubular phosphorus reabsorption and was positively correlated with urine NGAL/Cr (r = -0.418, p = 0.019; r = 0.682, p = 0.000; respectively). Conclusions: MCP-1/CCL-2 may play a role in the development of proteinuria in MCDK. Netrin-1 may be a protective factor against proteinuria-induced renal injury. Urine hepcidin/Cr may reflect proximal tubule damage in MCDK. Urine NGAL/Cr may be a predictor of tubule damage by proteinuria.