cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.9276140
title: Brazilian chronic dialysis survey 2017

description: Abstract Introduction: Having national data on chronic dialysis is essential in treatment planning. Objective: To present data of the survey from the Brazilian Society of Nephrology on patients with chronic kidney disease on dialysis in July 2017. Methods: Data was collected from dialysis units in Brazil. The data collection was done using a questionnaire completed online by the dialysis units. Results: Two hundred and ninety-one centers (38.4%) answered the questionnaire. In July 2017, the estimated total number of dialysis patients was 126,583. National estimates of prevalence and incidence rates of dialysis patients per million population (pmp) were 610 (range: 473 in the North region and 710 in the Midwest) and 194, respectively. The incidence rate of new dialysis patients with diagnosis of diabetic nephropathy was 77 pmp. The annual gross mortality rate was 19.9%. Of the prevalent patients, 93.1% were on hemodialysis and 6.9% on peritoneal dialysis, with 31,226 (24%) on the waiting list for renal transplantation. Venous catheter was used as access in 22.6% of patients on hemodialysis. The prevalence rate of positive serology for hepatitis C continued with a tendency to decrease (3.3%). Conclusion: The absolute number of patients and rates of incidence and prevalence on dialysis continued to increase; the mortality rate tended to rise. There were obvious regional and state discrepancies in these rates.
DOI: 10.6084/m9.figshare.9276140.v1
title: Brazilian chronic dialysis survey 2017

description: Abstract Introduction: Having national data on chronic dialysis is essential in treatment planning. Objective: To present data of the survey from the Brazilian Society of Nephrology on patients with chronic kidney disease on dialysis in July 2017. Methods: Data was collected from dialysis units in Brazil. The data collection was done using a questionnaire completed online by the dialysis units. Results: Two hundred and ninety-one centers (38.4%) answered the questionnaire. In July 2017, the estimated total number of dialysis patients was 126,583. National estimates of prevalence and incidence rates of dialysis patients per million population (pmp) were 610 (range: 473 in the North region and 710 in the Midwest) and 194, respectively. The incidence rate of new dialysis patients with diagnosis of diabetic nephropathy was 77 pmp. The annual gross mortality rate was 19.9%. Of the prevalent patients, 93.1% were on hemodialysis and 6.9% on peritoneal dialysis, with 31,226 (24%) on the waiting list for renal transplantation. Venous catheter was used as access in 22.6% of patients on hemodialysis. The prevalence rate of positive serology for hepatitis C continued with a tendency to decrease (3.3%). Conclusion: The absolute number of patients and rates of incidence and prevalence on dialysis continued to increase; the mortality rate tended to rise. There were obvious regional and state discrepancies in these rates.
DOI: 10.6084/m9.figshare.9276734
title: Long-term effects of delayed graft function duration on function and survival of deceased donor kidney transplants

description: Abstract Introduction: Delayed graft function (DGF) is a frequent complication after deceased donor kidney transplantation with an impact on the prognosis of the transplant. Despite this, long-term impact of DGF on graft function after deceased donor kidney transplantation has not been properly evaluated. Objective: The main objective of this study was to evaluate risk factors for DGF and the impact of its occurrence and length on graft survival and function. Methods: A retrospective cohort study was performed in 517 kidney transplant recipients who received a deceased donor organ between January 2008 and December 2013. Results: The incidence of DGF was 69.3% and it was independently associated with donor's final serum creatinine and age, cold ischemia time, use of antibody induction therapy and recipient's diabetes mellitus. The occurrence of DGF was also associated with a higher incidence of Banff ≥ 1A grade acute rejection (P = 0.017), lower graft function up to six years after transplantation and lower death-censored graft survival at 1 and 5 years (P &lt; 0.05). DGF period longer than 14 days was associated with higher incidence of death-censored graft loss (P = 0.038) and poorer graft function (P &lt; 0.001). No differences were found in patient survival. Conclusions: The occurrence of DGF has a long-lasting detrimental impact on graft function and survival and this impact is even more pronounced when DGF lasts longer than two weeks.
DOI: 10.6084/m9.figshare.9276734.v1
title: Long-term effects of delayed graft function duration on function and survival of deceased donor kidney transplants

description: Abstract Introduction: Delayed graft function (DGF) is a frequent complication after deceased donor kidney transplantation with an impact on the prognosis of the transplant. Despite this, long-term impact of DGF on graft function after deceased donor kidney transplantation has not been properly evaluated. Objective: The main objective of this study was to evaluate risk factors for DGF and the impact of its occurrence and length on graft survival and function. Methods: A retrospective cohort study was performed in 517 kidney transplant recipients who received a deceased donor organ between January 2008 and December 2013. Results: The incidence of DGF was 69.3% and it was independently associated with donor's final serum creatinine and age, cold ischemia time, use of antibody induction therapy and recipient's diabetes mellitus. The occurrence of DGF was also associated with a higher incidence of Banff ≥ 1A grade acute rejection (P = 0.017), lower graft function up to six years after transplantation and lower death-censored graft survival at 1 and 5 years (P &lt; 0.05). DGF period longer than 14 days was associated with higher incidence of death-censored graft loss (P = 0.038) and poorer graft function (P &lt; 0.001). No differences were found in patient survival. Conclusions: The occurrence of DGF has a long-lasting detrimental impact on graft function and survival and this impact is even more pronounced when DGF lasts longer than two weeks.
DOI: 10.6084/m9.figshare.9276626
title: Resistant and refractory hypertension: two sides of the same disease?

description: Abstract Refractory hypertension (RfH) is an extreme phenotype of resistant hypertension (RH), being considered an uncontrolled blood pressure besides the use of 5 or more antihypertensive medications, including a long-acting thiazide diuretic and a mineralocorticoid antagonist. RH is common, with 10-20% of the general hypertensives, and its associated with renin angiotensin aldosterone system hyperactivity and excess fluid retention. RfH comprises 5-8% of the RH and seems to be influenced by increased sympathetic activity. RH patients are older and more obese than general hypertensives. It is strongly associated with diabetes, obstructive sleep apnea, and hyperaldosteronism status. RfH is more frequent in women, younger patients and Afro-americans compared to RFs. Both are associated with increased albuminuria, left ventricular hypertrophy, chronic kidney diseases, stroke, and cardiovascular diseases. The magnitude of the white-coat effect seems to be higher among RH patients. Intensification of diuretic therapy is indicated in RH, while in RfH, therapy failure imposes new treatment alternatives such as the use of sympatholytic therapies. In conclusion, both RH and RfH constitute challenges in clinical practice and should be addressed as distinct clinical entities by trained professionals who are capable to identify comorbidities and provide specific, diversified, and individualized treatment.
DOI: 10.6084/m9.figshare.9276626.v1
title: Resistant and refractory hypertension: two sides of the same disease?

description: Abstract Refractory hypertension (RfH) is an extreme phenotype of resistant hypertension (RH), being considered an uncontrolled blood pressure besides the use of 5 or more antihypertensive medications, including a long-acting thiazide diuretic and a mineralocorticoid antagonist. RH is common, with 10-20% of the general hypertensives, and its associated with renin angiotensin aldosterone system hyperactivity and excess fluid retention. RfH comprises 5-8% of the RH and seems to be influenced by increased sympathetic activity. RH patients are older and more obese than general hypertensives. It is strongly associated with diabetes, obstructive sleep apnea, and hyperaldosteronism status. RfH is more frequent in women, younger patients and Afro-americans compared to RFs. Both are associated with increased albuminuria, left ventricular hypertrophy, chronic kidney diseases, stroke, and cardiovascular diseases. The magnitude of the white-coat effect seems to be higher among RH patients. Intensification of diuretic therapy is indicated in RH, while in RfH, therapy failure imposes new treatment alternatives such as the use of sympatholytic therapies. In conclusion, both RH and RfH constitute challenges in clinical practice and should be addressed as distinct clinical entities by trained professionals who are capable to identify comorbidities and provide specific, diversified, and individualized treatment.
DOI: 10.6084/m9.figshare.19964118.v1
title: Pediatric lupus nephritis

description: Abstract Involvement of the kidneys by lupus nephritis (LN) is one of the most severe clinical manifestations seen in individuals with systemic lupus erythematosus (SLE). LN is more frequent and severe in pediatric patients and has been associated with higher morbidity and mortality rates. This narrative review aimed to describe the general aspects of LN and its particularities when affecting children and adolescents, while focusing on the disease's etiopathogenesis, clinical manifestations, renal tissue alterations, and treatment options.
DOI: 10.6084/m9.figshare.19964118
title: Pediatric lupus nephritis

description: Abstract Involvement of the kidneys by lupus nephritis (LN) is one of the most severe clinical manifestations seen in individuals with systemic lupus erythematosus (SLE). LN is more frequent and severe in pediatric patients and has been associated with higher morbidity and mortality rates. This narrative review aimed to describe the general aspects of LN and its particularities when affecting children and adolescents, while focusing on the disease's etiopathogenesis, clinical manifestations, renal tissue alterations, and treatment options.
DOI: 10.6084/m9.figshare.9276002
title: Acute Kidney Injury Epidemiology in pediatrics

description: Abstract We performed a search in the MEDLINE database using the MeSH term: "Acute Kidney Injury", selecting the subtopic "Epidemiology", and applying age and year of publication filters. We also searched for the terms: "acute renal failure" and "epidemiology" "acute tubular necrosis" and "epidemiology" in the title and summary fields with the same filters. In a second search, we searched in the LILACS database, with the terms: "acute renal injury", or "acute renal failure" or "acute kidney injury" and the age filter. All abstracts were evaluated by the authors and the articles considered most relevant, were examined in their entirety. Acute Kidney Injury (AKI) -related mortality ranged from 3-63% in the studies included in this review. AKI etiology has marked regional differences, with sepsis being the main cause in developed countries. In developing countries, primary renal diseases and hypovolemia are still a common cause of AKI.
DOI: 10.6084/m9.figshare.9276002.v1
title: Acute Kidney Injury Epidemiology in pediatrics

description: Abstract We performed a search in the MEDLINE database using the MeSH term: "Acute Kidney Injury", selecting the subtopic "Epidemiology", and applying age and year of publication filters. We also searched for the terms: "acute renal failure" and "epidemiology" "acute tubular necrosis" and "epidemiology" in the title and summary fields with the same filters. In a second search, we searched in the LILACS database, with the terms: "acute renal injury", or "acute renal failure" or "acute kidney injury" and the age filter. All abstracts were evaluated by the authors and the articles considered most relevant, were examined in their entirety. Acute Kidney Injury (AKI) -related mortality ranged from 3-63% in the studies included in this review. AKI etiology has marked regional differences, with sepsis being the main cause in developed countries. In developing countries, primary renal diseases and hypovolemia are still a common cause of AKI.
DOI: 10.6084/m9.figshare.9276350
title: Chronic kidney disease patients who smoke have higher serum phosphorus

description: ABSTRACT Introduction: Mineral and bone metabolism disorders in chronic kidney disease (CKD-MBD) constitute a syndrome defined by changes in calcium, phosphorus (P), vitamin D and parathormone, fibroblast growth factor 23 (FGF-23) and its specific cofactor, Klotho. CKD-MBD, as well as smoking, are associated with an increased risk of cardiovascular disease. However, it is not known whether or not smoking impacts the cardiovascular risk in CKD- MBD. Objective: To analyze the relationship between smoking and CKD-MBD markers. Methods: We evaluated 92 patients divided into: 1) Control Group: non-smokers without CKD; 2) CKD group in stages III and IV under conservative treatment (20 non-smokers and 17 smokers); 3) CKD group on dialysis (21 non-smokers and 19 smokers). Clinical, demographic, and biochemical markers were compared between the groups. Results: FGF-23 and Klotho levels were not different between smokers and non-smokers. Patients in the CKD group on conservative treatment had higher serum P than non-smokers (p = 0.026) even after adjusted for renal function (p = 0.079), gender (p = 0.145) and age (p = 0.986). Conclusion: Smoking confers a higher cardiovascular risk to CKD patients under conservative treatment as it is associated with higher levels of P. Further studies are needed to confirm and better elucidate this finding.
DOI: 10.6084/m9.figshare.9276350.v1
title: Chronic kidney disease patients who smoke have higher serum phosphorus

description: ABSTRACT Introduction: Mineral and bone metabolism disorders in chronic kidney disease (CKD-MBD) constitute a syndrome defined by changes in calcium, phosphorus (P), vitamin D and parathormone, fibroblast growth factor 23 (FGF-23) and its specific cofactor, Klotho. CKD-MBD, as well as smoking, are associated with an increased risk of cardiovascular disease. However, it is not known whether or not smoking impacts the cardiovascular risk in CKD- MBD. Objective: To analyze the relationship between smoking and CKD-MBD markers. Methods: We evaluated 92 patients divided into: 1) Control Group: non-smokers without CKD; 2) CKD group in stages III and IV under conservative treatment (20 non-smokers and 17 smokers); 3) CKD group on dialysis (21 non-smokers and 19 smokers). Clinical, demographic, and biochemical markers were compared between the groups. Results: FGF-23 and Klotho levels were not different between smokers and non-smokers. Patients in the CKD group on conservative treatment had higher serum P than non-smokers (p = 0.026) even after adjusted for renal function (p = 0.079), gender (p = 0.145) and age (p = 0.986). Conclusion: Smoking confers a higher cardiovascular risk to CKD patients under conservative treatment as it is associated with higher levels of P. Further studies are needed to confirm and better elucidate this finding.
DOI: 10.6084/m9.figshare.9276590
title: Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

description: ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.
DOI: 10.6084/m9.figshare.9276590.v1
title: Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

description: ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.
DOI: 10.6084/m9.figshare.9276632
title: Renal histological findings in a patient with acute renal injury associated with purpura fulminans: a case report

description: ABSTRACT Introduction: Purpura fulminans (PF) is a rapid progressive thrombotic disease in which hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occurs. It can potentially cause acute kidney injury (AKI). However, there is no description in the medical literature of renal histological findings of PF. Case report: A 20-year-old female patient, previously healthy, was admitted to the emergency department (ED) with odynophagia, fever, generalized myalgia and anuria, which evolved with the appearance of purpuric plaques on the face and limbs. She required dialysis on admission. Laboratorial tests showed anemia, leukocytosis, thrombocytopenia, and elevation of lactic dehydrogenase (LDH). The purpuric lesions became bullous with ruptures and then necrotic and erosive, reaching the dermis, subcutaneous tissue and musculature, until bone exposure. There was no improvement with initial antibiotic therapy aimed at the treatment of meningococcemia. Thrombotic microangiopathy (TMA) and PF were then suspected. The patient remained in daily dialysis, requiring plasmapheresis. After sustained improvement of the thrombocytopenia, she underwent renal biopsy, which was not compatible with TMA, characterizing possible PF. A complete recovery of the renal function was achieved and cutaneous sequels were treated with grafts. Conclusion: When thrombotic and hemorrhagic phenomena overlap, obtaining a renal biopsy can be difficult. However, in the presented case, the biopsy allowed the exclusion of AKI caused by TMA, presenting for the first time, histological findings compatible with PF.
DOI: 10.6084/m9.figshare.9276632.v1
title: Renal histological findings in a patient with acute renal injury associated with purpura fulminans: a case report

description: ABSTRACT Introduction: Purpura fulminans (PF) is a rapid progressive thrombotic disease in which hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occurs. It can potentially cause acute kidney injury (AKI). However, there is no description in the medical literature of renal histological findings of PF. Case report: A 20-year-old female patient, previously healthy, was admitted to the emergency department (ED) with odynophagia, fever, generalized myalgia and anuria, which evolved with the appearance of purpuric plaques on the face and limbs. She required dialysis on admission. Laboratorial tests showed anemia, leukocytosis, thrombocytopenia, and elevation of lactic dehydrogenase (LDH). The purpuric lesions became bullous with ruptures and then necrotic and erosive, reaching the dermis, subcutaneous tissue and musculature, until bone exposure. There was no improvement with initial antibiotic therapy aimed at the treatment of meningococcemia. Thrombotic microangiopathy (TMA) and PF were then suspected. The patient remained in daily dialysis, requiring plasmapheresis. After sustained improvement of the thrombocytopenia, she underwent renal biopsy, which was not compatible with TMA, characterizing possible PF. A complete recovery of the renal function was achieved and cutaneous sequels were treated with grafts. Conclusion: When thrombotic and hemorrhagic phenomena overlap, obtaining a renal biopsy can be difficult. However, in the presented case, the biopsy allowed the exclusion of AKI caused by TMA, presenting for the first time, histological findings compatible with PF.
DOI: 10.6084/m9.figshare.9276485
title: Uremic lion face syndrome

description: Abstract Mineral bone disorder is a common feature of chronic kidney disease. Lion face syndrome is rare complication of severe hyperparathyroidism in end-stage renal disease patients, which has been less commonly reported due to dialysis and medical treatment advances in the last decade. The early recognition of the characteristic facial deformity is crucial to prompt management and prevent severe disfigurement. The authors present a rare case of severe hyperparathyroidism presenting with lion face syndrome and bone fractures.
DOI: 10.6084/m9.figshare.9276485.v1
title: Uremic lion face syndrome

description: Abstract Mineral bone disorder is a common feature of chronic kidney disease. Lion face syndrome is rare complication of severe hyperparathyroidism in end-stage renal disease patients, which has been less commonly reported due to dialysis and medical treatment advances in the last decade. The early recognition of the characteristic facial deformity is crucial to prompt management and prevent severe disfigurement. The authors present a rare case of severe hyperparathyroidism presenting with lion face syndrome and bone fractures.