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ABSTRACT Introduction: The Brazilian Dialysis Survey (BDS) is an important source of national data about people on chronic dialysis that contributes to the formulation of health policies regarding kidney failure. Objective: To report the 2021 epidemiological data from the BDS of the Brazilian Society of Nephrology (BSN). Methods: A survey was carried out in Brazilian chronic dialysis centers using an online questionnaire covering clinical and epidemiological aspects of patients in chronic dialysis, data on dialysis therapy, characteristics of dialysis centers, and the impact of the COVID-19 pandemic of 2021. Results: Thirty percent (n = 252) of the centers answered the questionnaire. In July 2021, the estimated total number of patients on dialysis was 148,363. The estimated prevalence and incidence rates of patients per million population (pmp) were 696 and 224, respectively. Of the prevalent patients, 94.2% were on hemodialysis (HD) (1.8% of these on hemodiafiltration), and 5.8% on peritoneal dialysis (PD); 21% were on the transplant waiting list. The incidence rate of confirmed COVID-19 between January and July 2021 was 1,236/10,000 dialysis patients, and the case-fatality rate reached 25.5%. Up to July 2021, 88.6% of patients had received at least one dose of the anti-SARS-CoV-2 vaccine. The estimated overall and COVID-19 crude annual mortality rates were 22.3% and 5.3%, respectively. Conclusion: The absolute number and the prevalence rate of patients on chronic dialysis continue to increase. Most dialysis patients were vaccinated against COVID-19 during the year. The COVID-19 pandemic was associated to the overall mortality rate.

RESUMO Introdução: O Censo Brasileiro de Diálise (CBD) constitui importante fonte de dados nacionais sobre pessoas em diálise crônica que contribui para a formulação de políticas de saúde. Objetivo: Relatar dados epidemiológicos de 2021 do CBD da Sociedade Brasileira de Nefrologia (SBN). Métodos: Realizou-se pesquisa em centros brasileiros de diálise crônica utilizando questionário online abrangendo aspectos clínicos e epidemiológicos de pacientes em diálise crônica, dados sobre terapia dialítica, características dos centros de diálise e o impacto da pandemia COVID-19 em 2021. Resultados: Trinta por cento (n = 252) dos centros responderam ao questionário. Em Julho de 2021, o número total estimado de pacientes em diálise foi 148.363. As taxas estimadas de prevalência e incidência de pacientes por milhão da população (pmp) foram 696 e 224, respectivamente. Dos pacientes prevalentes, 94,2% estavam em hemodiálise (HD) (1,8% destes em hemodiafiltração), e 5,8% em diálise peritoneal (DP); 21% estavam na lista de espera para transplante. A taxa de incidência de COVID-19 confirmada entre Janeiro e Julho de 2021 foi 1.236/10.000 pacientes em diálise, e a taxa de letalidade atingiu 25,5%. Até Julho de 2021, 88,6% dos pacientes haviam recebido pelo menos uma dose da vacina anti-SARS-CoV-2. As taxas estimadas de mortalidade bruta anual geral e por COVID-19 foram de 22,3% e 5,3%, respectivamente. Conclusão: O número absoluto e a taxa de prevalência de pacientes em diálise crônica continuam aumentando. A maioria dos pacientes em diálise foram vacinados contra COVID-19 durante o ano. A pandemia COVID-19 contribuiu com a taxa de mortalidade geral.

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INTRODUCTION: The Brazilian Dialysis Survey (BDS) is an important source of national data about people on chronic dialysis that contributes to the formulation of health policies regarding kidney failure. OBJECTIVE: To report the 2021 epidemiological data from the BDS of the Brazilian Society of Nephrology (BSN). METHODS: A survey was carried out in Brazilian chronic dialysis centers using an online questionnaire covering clinical and epidemiological aspects of patients in chronic dialysis, data on dialysis therapy, characteristics of dialysis centers, and the impact of the COVID-19 pandemic of 2021. RESULTS: Thirty percent (n = 252) of the centers answered the questionnaire. In July 2021, the estimated total number of patients on dialysis was 148,363. The estimated prevalence and incidence rates of patients per million population (pmp) were 696 and 224, respectively. Of the prevalent patients, 94.2% were on hemodialysis (HD) (1.8% of these on hemodiafiltration), and 5.8% on peritoneal dialysis (PD); 21% were on the transplant waiting list. The incidence rate of confirmed COVID-19 between January and July 2021 was 1,236/10,000 dialysis patients, and the case-fatality rate reached 25.5%. Up to July 2021, 88.6% of patients had received at least one dose of the anti-SARS-CoV-2 vaccine. The estimated overall and COVID-19 crude annual mortality rates were 22.3% and 5.3%, respectively. CONCLUSION: The absolute number and the prevalence rate of patients on chronic dialysis continue to increase. Most dialysis patients were vaccinated against COVID-19 during the year. The COVID-19 pandemic was associated to the overall mortality rate.

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OBJECTIVE: We aimed to evaluate the prevalence and sociodemographic determinants of predialysis dietitian follow-up in a large cohort of Brazilian dialysis patients. METHODS: We retrospectively evaluated data from all incident adult dialysis patients included in the Brazilian Dialysis Registry from January 2011 to September 2021. Predialysis dietitian follow-up was classified as present when a period more than 6 months of dietitian care was reported. Gender, age, skin color, education, body mass index, chronic kidney disease etiology, first chronic dialysis program, healthcare provider, and geographic regions were tested in logistic regression models. RESULTS: Ten thousand three hundred and eighty two patients met the inclusion criteria and 1,254 (12.1%) reported predialysis dietitian follow-up, most of them referred by a nephrologist (94.2%). The independent determinants of dietitian follow-up were older age, white skin color, higher education level, not having diabetes, living in North/Northeast and South (compared to Southeast), and having a nonpublic healthcare provider. When considered only patients under a predialysis care of a nephrologist, higher education, hemodialysis as the first dialysis modality, and living in the North/Northeast and South regions (compared with Southeast) were associated with dietitian follow-up. CONCLUSION: Predialysis dietitian follow-up for more than 6 months in a country where the public health system is the main dialysis provider is still very low. The nephrologist is pivotal for referral to dietitians but socioeconomic factors also seem to play a role in this regard.

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Abstract Introduction: National data on chronic dialysis treatment are essential to support the development of health policies aimed at improving the treatment for thousands of people. Objective: To report epidemiological data from the 2020 Brazilian Dialysis Survey, sponsored by the Brazilian Society of Nephrology. Methods: A survey was carried out in Brazilian chronic dialysis centers using an online questionnaire for the year, covering clinical and epidemiological aspects of patients in a chronic dialysis program, data on dialysis therapy, characteristics of dialysis units and the impact of the COVID-19 pandemic. Results: 235 (28%) of the centers responded to the questionnaire. In July 2020, the estimated total number of patients on dialysis was 144,779. The estimated prevalence and incidence rates of patients per million population (pmp) were 684 and 209, respectively. Of the prevalent patients, 92.6% were on hemodialysis (HD) and 7.4% were on peritoneal dialysis (PD); 23% were on the transplant waiting list. A central venous catheter was used by a quarter of patients on HD. The incidence rate of confirmed COVID-19 between February and July 2020 was 684/10,000 dialysis patients, and the lethality rate was 25.7%. The estimated overall mortality and COVID-19 crude annual mortality rates were 24.5 and 4.2%, respectively. Conclusion: The absolute number of patients on chronic dialysis and prevalence rate continued to increase. The low use of PD as dialysis therapy was maintained and the use of long-term catheters for HD increased. The COVID-19 pandemic contributed to the increase in the overall mortality rate.

Resumo Introdução: Dados nacionais sobre tratamento dialítico crônico são essenciais para apoiar o desenvolvimento de políticas de saúde destinadas a melhorar o tratamento de milhares de pessoas. Objetivo: Relatar dados epidemiológicos do Censo Brasileiro de Diálise 2020, patrocinado pela Sociedade Brasileira de Nefrologia. Métodos: Realizou-se pesquisa em centros brasileiros de diálise crônica utilizando questionário online relativo ao ano, abrangendo aspectos clínicos e epidemiológicos de pacientes em programa de diálise crônica, dados sobre terapia dialítica, características das unidades de diálise e impacto da pandemia COVID-19. Resultados: 235 (28%) centros responderam ao questionário. Em julho de 2020, o número total estimado de pacientes em diálise foi 144.779. As taxas estimadas de prevalência e incidência de pacientes por milhão da população (pmp) foram 684 e 209, respectivamente. Dos pacientes prevalentes, 92,6% estavam em hemodiálise (HD) e 7,4% em diálise peritoneal (DP); 23% estavam na lista de espera para transplante. Cateter venoso central foi utilizado por um quarto dos pacientes em HD. A taxa de incidência de COVID-19 confirmada entre fevereiro e julho de 2020 foi 684/10.000 pacientes em diálise, a taxa de letalidade foi 25,7%. As taxas estimadas de mortalidade geral e mortalidade bruta anual por COVID-19 foram 24,5 e 4,2%, respectivamente. Conclusão: O número absoluto de pacientes em diálise crônica e a taxa de prevalência continuaram a aumentar. O baixo uso de DP como terapia de diálise foi mantido e o uso de cateteres de longa permanência para HD aumentou. A pandemia COVID-19 contribuiu para o aumento da taxa de mortalidade geral.

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Background: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease. Therefore, studies involving large samples are scarce, making registries powerful tools to evaluate cases. We present herein the first analysis of the Brazilian aHUS Registry (BRaHUS). Methods: Analysis of clinical, laboratory, genetic and treatment data from patients inserted in the BRaHUS, from 2017 to 2020, as an initiative of the Rare Diseases Committee of the Brazilian Society of Nephrology. Results: The cohort consisted of 75 patients (40 adults and 35 pediatric). There was a predominance of women (56%), median age at diagnosis of 20.7 years and a positive family history in 8% of cases. Renal involvement was observed in all cases and 37% had low C3 levels. In the <2 years of age group, males were predominant. Children presented lower levels of hemoglobin (P = .01) and platelets (P = .003), and higher levels of lactate dehydrogenase (LDH) (P = .004) than adults. Genetic analysis performed in 44% of patients revealed pathogenic variants in 66.6% of them, mainly in CFH and the CFHR1-3 deletion. Plasmapheresis was performed more often in adults (P = .005) and 97.3% of patients were treated with eculizumab and its earlier administration was associated with dialysis-free after 3 months (P = .08). Conclusions: The cohort of BRaHUS was predominantly composed of female young adults, with renal involvement in all cases. Pediatric patients had lower hemoglobin and platelet levels and higher LDH levels than adults, and the most common genetic variants were identified in CFH and the CFHR1-3 deletion with no preference of age, a peculiar pattern of Brazilian patients.

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Abstract Infection by the hepatitis C virus is more prevalent in patients on dialysis than in the general population in Brazil, and has been associated with worse outcomes. Current therapy for hepatitis C is highly effective, safe, and widely available in Brazil, with coverage provided to dialysis patients with chronic kidney disease, which makes the elimination of hepatitis C a viable target. The Brazilian Society of Nephrology, the Brazilian Society of Hepatology, and the Brazilian Liver Institute developed the "Brazilian Registry for the Elimination of Hepatitis C in Dialysis Units". This project aims to identify, treat, and monitor the response to treatment of patients on chronic dialysis infected with the hepatitis C virus in Brazil. This article presents the issue and invites Brazilian nephrologists to rally around the achievement of a significant goal.

Resumo A infecção pelo vírus da hepatite C é mais prevalente em pacientes em diálise do que na população geral no Brasil e implica um pior prognóstico. O tratamento atual para hepatite C é altamente eficaz, seguro e disponível no país, inclusive para a população de pacientes crônicos em diálise, o que torna a eliminação do vírus da hepatite C uma meta viável. A Sociedade Brasileira de Nefrologia, a Sociedade Brasileira de Hepatologia e o Instituto Brasileiro do Fígado desenvolveram o "Registro Brasileiro para Eliminação da Hepatite C nas Unidades de Diálise". O projeto visa identificar pacientes em diálise crônica com vírus da hepatite C no Brasil, além de tratar e monitorar a resposta virológica após o tratamento. Este breve artigo apresenta o problema e convida os nefrologistas brasileiros a unirem forças nesse objetivo comum.

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INTRODUCTION: National data on chronic dialysis treatment are essential to support the development of health policies aimed at improving the treatment for thousands of people. OBJECTIVE: To report epidemiological data from the 2020 Brazilian Dialysis Survey, sponsored by the Brazilian Society of Nephrology. METHODS: A survey was carried out in Brazilian chronic dialysis centers using an online questionnaire for the year, covering clinical and epidemiological aspects of patients in a chronic dialysis program, data on dialysis therapy, characteristics of dialysis units and the impact of the COVID-19 pandemic. RESULTS: 235 (28%) of the centers responded to the questionnaire. In July 2020, the estimated total number of patients on dialysis was 144,779. The estimated prevalence and incidence rates of patients per million population (pmp) were 684 and 209, respectively. Of the prevalent patients, 92.6% were on hemodialysis (HD) and 7.4% were on peritoneal dialysis (PD); 23% were on the transplant waiting list. A central venous catheter was used by a quarter of patients on HD. The incidence rate of confirmed COVID-19 between February and July 2020 was 684/10,000 dialysis patients, and the lethality rate was 25.7%. The estimated overall mortality and COVID-19 crude annual mortality rates were 24.5 and 4.2%, respectively. CONCLUSION: The absolute number of patients on chronic dialysis and prevalence rate continued to increase. The low use of PD as dialysis therapy was maintained and the use of long-term catheters for HD increased. The COVID-19 pandemic contributed to the increase in the overall mortality rate.

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Infection by the hepatitis C virus is more prevalent in patients on dialysis than in the general population in Brazil, and has been associated with worse outcomes. Current therapy for hepatitis C is highly effective, safe, and widely available in Brazil, with coverage provided to dialysis patients with chronic kidney disease, which makes the elimination of hepatitis C a viable target. The Brazilian Society of Nephrology, the Brazilian Society of Hepatology, and the Brazilian Liver Institute developed the "Brazilian Registry for the Elimination of Hepatitis C in Dialysis Units". This project aims to identify, treat, and monitor the response to treatment of patients on chronic dialysis infected with the hepatitis C virus in Brazil. This article presents the issue and invites Brazilian nephrologists to rally around the achievement of a significant goal.

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BACKGROUND: Lipoprotein glomerulopathy (LPG) is a rare autosomal dominant disease caused by mutations in APOE, the gene which encodes apolipoprotein E. LPG mainly affects Asian individuals, however occasional cases have also been described in Americans and Europeans. Herein we report two unrelated Brazilian patients with LPG in whom genetic analyses revealed the APOE-Osaka/Kurashiki variant. CASE PRESENTATION - CASE 1: A 29-year-old Caucasian male sought medical attention with complaints of face swelling and foamy urine for the last 3 months. He denied a family history of kidney disease, consanguinity, or Asian ancestry. His tests showed proteinuria of 12.5 g/24 h, hematuria, serum creatinine 0.94 mg/dL, albumin 2.3 g/dl, total cholesterol 284 mg/dL, LDL 200 mg/dL, triglycerides 175 mg/dL, and negative screening for secondary causes of glomerulopathy. A kidney biopsy revealed intraluminal, laminated deposits of hyaline material in glomerular capillaries consistent with lipoprotein thrombi. These findings were confirmed by electron microscopy, establishing the diagnosis of LPG. His apolipoprotein E serum level was 72 mg/dL and genetic analysis revealed the APOE pathogenic variant c.527G > C, p.Arg176Pro in heterozygosis, known as the Osaka/Kurashiki mutation and positioned nearby the LDL receptor binding site. CASE 2: A 34-year-old Caucasian man sought medical assessment for renal dysfunction and hypertension. He reported intermittent episodes of lower-limb edema for 3 years and a family history of kidney disease, but denied Asian ancestry. Laboratorial tests showed BUN 99 mg/dL, creatinine 10.7 mg/dL, total cholesterol 155 mg/dL, LDL 79 mg/dL, triglycerides 277 mg/dL, albumin 3.1 g/dL, proteinuria 2.7 g/24 h, and negative screening for secondary causes of glomerulopathy. His kidney biopsy was consistent with advanced chronic nephropathy secondary to LPG. A genetic analysis also revealed the Osaka/Kurashiki variant. He was transplanted a year ago, displaying no signs of disease relapse. CONCLUSION: We report two unrelated cases of Brazilian patients with a diagnosis of lipoprotein glomerulopathy whose genetic assessment identified the APOE-Osaka/Kurashiki pathogenic variant, previously only described in eastern Asians. While this is the second report of LPG in Latin America, the identification of two unrelated cases by our medical team raises the possibility that LPG may be less rare in this part of the world than currently thought, and should definitely be considered when nephrotic syndrome is associated with suggestive kidney biopsy findings.

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PURPOSE OF REVIEW: In this narrative review, we describe general aspects, histological alterations, treatment, and implications of Fabry disease (FD) nephropathy. This information should be used to guide physicians and patients in a shared decision-making process. SOURCE OF INFORMATION: Original peer-reviewed articles, review articles, and opinion pieces were identified from PubMed and Google Scholar databases. Only sources in English were accessed. METHODS: We performed a focused narrative review assessing the main aspects of FD nephropathy. The literature was critically analyzed from a theoretical and contextual perspective, and thematic analysis was performed. KEY FINDINGS: FD nephropathy is related to the progressive accumulation of GL3, which occurs in all types of renal cells. It is more prominent in podocytes, which seem to play an important role in the pathogenesis of this nephropathy. A precise detection of renal disorders is of fundamental importance because the specific treatment of FD is usually delayed, making reversibility unlikely and leading to a worse prognosis. LIMITATIONS: As no formal tool was applied to assess the quality of the included studies, selection bias may have occurred. Nonetheless, we have attempted to provide a comprehensive review on the topic using current studies from experts in FD and extensive review of the literature.

OBJET DE LA REVUE: Dans cette revue narrative, nous discutons des aspects généraux, des modifications histologiques, du traitement et des implications de la néphropathie liée à la Maladie de Fabry. Des informations qui serviront à guider les médecins et les patients dans un processus commun de prise de décision. SOURCES: Les originaux d'articles évalués par les pairs, d'articles-synthèses et d'articles d'opinion ont été répertoriés dans les bases de données Pubmed et Google Scholar. Seuls les articles en anglais ont été consultés. MÉTHODOLOGIE: Nous avons procédé à une revue narrative ciblée examinant les principaux aspects de la néphropathie liés à la maladie de Fabry. La documentation a fait l'objet d'une critique rigoureuse du point de vue théorique et contextuel, et une analyse thématique a été effectuée. PRINCIPAUX RÉSULTATS: La néphropathie liée à la maladie de Fabry est associée à l'accumulation progressive de GL3, qui se produit dans tous les types de cellules rénales. Elle est plus présente dans les podocytes, qui semblent jouer un rôle important dans la pathogenèse de la néphropathie. Un dépistage précis des troubles rénaux est d'une importance capitale puisque le traitement spécifique de la maladie de Fabry est généralement retardé, ce qui rend la réversibilité peu probable et conduit à un pronostic plus défavorable. LIMITES: Des biais de sélection pourraient s'être introduits puisqu'aucun outil formel n'a été utilisé pour évaluer les études incluses. Nous avons néanmoins tenté de procéder à un examen complet du sujet grâce aux études actuelles menées par des experts de la maladie de Fabry et à une revue approfondie de la documentation.

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Nowadays vitamin D (25-OHD) deficiency is supposed to be a global epidemic condition. Expectedly, vitamin D measurement and intake exponentially increased in Brazil in this decade. Although the benefit of vitamin D to general health is still in debate, its indiscriminate use potentially may lead to enhance the incidence of vitamin D intoxication, which is considered a rare disorder. We report a case of a 70 year old diabetic male with chronic renal disease (blood creatinine of 1.6 mg/dL) who progressed suddenly to acute kidney injury (blood creatinine of 5.7 mg/dL) associated with hypercalcemia and high blood levels of vitamin D. Vitamin D and calcitriol were discontinued and hypercalcemia was managed by hydration followed by furosemide. Thereafter, disodium pamidronate was administered and the patient did not undergo on dialysis. It took approximately 14 months to normalize 25-OHD levels and blood creatinine returned to basal levels only after 24 months. The indicated labeling dosage was 2000 IU, but most likely the vitamin D manipulated preparation was higher as the vitamin D blood levels were very high. Although rare, vitamin D intoxication is becoming more frequent as the patients use frequently manipulated preparations that could be subject to errors in the manufacturing and labeling of the tablets or capsules. The present report alerts to the potential increase in the incidence of severe vitamin D intoxication due to the frequent use of this secosteroid as a nutritional supplement. At the same time, it is necessary to improve regulation on the nutrient supplement market.

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Abstract Nowadays vitamin D (25-OHD) deficiency is supposed to be a global epidemic condition. Expectedly, vitamin D measurement and intake exponentially increased in Brazil in this decade. Although the benefit of vitamin D to general health is still in debate, its indiscriminate use potentially may lead to enhance the incidence of vitamin D intoxication, which is considered a rare disorder. We report a case of a 70 year old diabetic male with chronic renal disease (blood creatinine of 1.6 mg/dL) who progressed suddenly to acute kidney injury (blood creatinine of 5.7 mg/dL) associated with hypercalcemia and high blood levels of vitamin D. Vitamin D and calcitriol were discontinued and hypercalcemia was managed by hydration followed by furosemide. Thereafter, disodium pamidronate was administered and the patient did not undergo on dialysis. It took approximately 14 months to normalize 25-OHD levels and blood creatinine returned to basal levels only after 24 months. The indicated labeling dosage was 2000 IU, but most likely the vitamin D manipulated preparation was higher as the vitamin D blood levels were very high. Although rare, vitamin D intoxication is becoming more frequent as the patients use frequently manipulated preparations that could be subject to errors in the manufacturing and labeling of the tablets or capsules. The present report alerts to the potential increase in the incidence of severe vitamin D intoxication due to the frequent use of this secosteroid as a nutritional supplement. At the same time, it is necessary to improve regulation on the nutrient supplement market.

Resumo Atualmente, muitos brasileiros têm utilizado vitamina D (25-OHD) como suplemento vitamínico para prevenção de diversas doenças crônicas, apesar da falta de dados científicos consistentes sobre o papel deste secosteroide na prevenção de doenças que não as do metabolismo mineral. A intoxicação por vitamina D é rara, mas devido ao seu uso indiscriminado tem ocorrido com maior frequência. Nesse relato, um homem diabético de 70 anos de idade com doença renal crônica (creatinina sérica de 1,6 mg/dL) passou a fazer uso de colecalciferol e calcitriol para recomposição dos níveis de 25-OHD, que eram de 16 ng/mL. O mesmo desenvolveu quadro de lesão renal aguda (creatinina = 5,7 mg/dL), após 45 dias. Este processo emergiu em paralelo ao surgimento de hipercalcemia e níveis circulantes elevados de vitamina D. Foram suspensas a administração de vitamina D e calcitriol, a hipercalcemia foi tratada com hidratação endovenosa, seguida de diurético de alça e posteriormente pamidronato. O paciente, que havia sido encaminhado para diálise, não necessitou desse tratamento. Os níveis de 25-OHD voltaram ao normal 14 meses após a sua suspensão, e os níveis de creatinina voltaram aos patamares anteriores 24 meses após esse evento. A dose prescrita de vitamina D correspondeu a 2000 UI/dia, a qual não é considerada inadequada segundo recomendações atuais. Existe, no entanto, na literatura controvérsia quanto à sensibilidade individual à vitamina D. Não pode ser descartado o uso inapropriado pelo paciente e nem eventual erro de manipulação. Embora raro, o quadro de intoxicação por vitamina D é grave e potencialmente pode levar a complicações clínicas irreversíveis.

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INTRODUCTION: Some beneficial effects from long-term use of corticosteroids have been reported in patients with IgA nephropathy. OBJECTIVE: This retrospective study aimed to evaluate the outcome of proteinuria and renal function according to a protocol based on a 6-month course of steroid treatment. METHOD: Twelve patients were treated with 1 g/day intravenous methylprednisolone for 3 consecutive days at the beginning of months 1, 3, and 5 plus 0.5 mg/kg oral prednisone on alternate days for 6 months (treated group). The control group included 9 untreated patients. RESULTS: Proteinuria (median and 25th and 75th percentiles) at baseline in the treated group was 1861 mg/24h (1518; 2417 mg/24h) and was 703 mg/24h (245; 983) and 684 mg/24h (266; 1023) at the 6th (p < 0.05 vs. baseline) and 12th months (p < 0.05 vs. baseline), respectively. In the control group the proteinuria was 1900 mg/24h (1620; 3197) at baseline and was 2290 mg/24h (1500; 2975) and 1600 mg/24h (1180; 2395) at the 6th and 12th months, respectively (not significant vs. baseline). When compared with the control group, the treated group showed lower proteinuria (p < 0.05) during the follow-up and a higher number of patients in remission (p < 0.05) at the 6th and 12th months. Renal function did not change during the follow-up and the adverse effects were mild in most of the patients. CONCLUSION: The 6-month course of steroid treatment was effective in reducing proteinuria during the 12 months of the follow-up, and was well-tolerated by most of the patients.

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INTRODUCTION: Some beneficial effects from long-term use of corticosteroids have been reported in patients with IgA nephropathy. OBJECTIVE: This retrospective study aimed to evaluate the outcome of proteinuria and renal function according to a protocol based on a 6-month course of steroid treatment. METHOD: Twelve patients were treated with 1 g/day intravenous methylprednisolone for 3 consecutive days at the beginning of months 1, 3, and 5 plus 0.5 mg/kg oral prednisone on alternate days for 6 months (treated group). The control group included 9 untreated patients. RESULTS: Proteinuria (median and 25th and 75th percentiles) at baseline in the treated group was 1861 mg/24h (1518; 2417 mg/24h) and was 703 mg/24h (245; 983) and 684 mg/24h (266; 1023) at the 6th (p < 0.05 vs. baseline) and 12th months (p < 0.05 vs. baseline), respectively. In the control group the proteinuria was 1900 mg/24h (1620; 3197) at baseline and was 2290 mg/24h (1500; 2975) and 1600 mg/24h (1180; 2395) at the 6th and 12th months, respectively (not significant vs. baseline). When compared with the control group, the treated group showed lower proteinuria (p < 0.05) during the follow-up and a higher number of patients in remission (p < 0.05) at the 6th and 12th months. Renal function did not change during the follow-up and the adverse effects were mild in most of the patients. CONCLUSION: The 6-month course of steroid treatment was effective in reducing proteinuria during the 12 months of the follow-up, and was well-tolerated by most of the patients.

INTRODUÇÃO: Tem sido sugerido que tratamento mais prolongado com corticosteroides pode ser benéfico em pacientes com nefropatia da IgA primária. OBJETIVO: Neste estudo retrospectivo avaliamos os efeitos na proteinúria e na função renal após 12 meses do protocolo baseado no uso por 6 meses de corticosteroides. MÉTODO: Doze pacientes receberam pulsos de 1 g/dia de metilprednisolona intravenosa por 3 dias consecutivos no início dos meses 1, 3 e 5, seguidos por prednisona (0,5 mg/kg) por via oral em dias alternados após cada pulso durante 6 meses (grupo tratado). O grupo controle foi composto por nove pacientes não tratados. RESULTADOS: A proteinúria (mg/24h; mediana; 25º; 75º percentis) no período basal no grupo tratado foi de 1861 (1518; 2417) e de 703 (245; 983) e de 684 (266; 1023) nos 6º (p < 0,05 vs. basal) e 12º (p < 0,05 vs. basal) meses, respectivamente. No grupo controle, a proteinúria foi de 1900 (1620; 3197) no período basal e de 2290 (1500; 2975) e de 1600 (1180; 2395) nos 6º e 12º meses, respectivamente (não significantes vs. basal). Comparado com o grupo controle, o grupo tratado teve menor proteinúria (p < 0,05) e número maior de pacientes em remissão (p < 0,05) nos 6º e 12º meses. A função renal não teve alteração significante e eventos adversos foram de pequena intensidade na maioria dos pacientes. CONCLUSÃO: O protocolo terapêutico base-ado no uso por 6 meses de corticosteroides foi efetivo em reduzir a proteinúria durante os 12 meses de seguimento e foi bem tolerado pela maioria dos pacientes.

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A 19-year-old female with type 1 diabetes for four years, and a 73-year-old female with type 2 diabetes for twenty years developed sudden-onset nephrotic syndrome. Examination by light microscopy, immunofluorescence, and electron microscopy (in one case) identified minimal change disease (MCD) in both cases. There was a potential causative drug (meloxicam) for the 73-year-old patient. Both patients were treated with prednisone and responded with complete remission. The patient with type 1 diabetes showed complete remission without relapse, and the patient with type 2 diabetes had two relapses; complete remission was sustained after associated treatment with cyclophosphamide and prednisone. Both patients had two years of follow-up evaluation after remission. We discuss the outcomes of both patients and emphasize the role of kidney biopsy in diabetic patients with an atypical proteinuric clinical course, because patients with MCD clearly respond to corticotherapy alone or in conjunction with other immunosuppressive agents.

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A 19-year-old female with type 1 diabetes for four years, and a 73-year-old female with type 2 diabetes for twenty years developed sudden-onset nephrotic syndrome. Examination by light microscopy, immunofluorescence, and electron microscopy (in one case) identified minimal change disease (MCD) in both cases. There was a potential causative drug (meloxicam) for the 73-year-old patient. Both patients were treated with prednisone and responded with complete remission. The patient with type 1 diabetes showed complete remission without relapse, and the patient with type 2 diabetes had two relapses; complete remission was sustained after associated treatment with cyclophosphamide and prednisone. Both patients had two years of follow-up evaluation after remission. We discuss the outcomes of both patients and emphasize the role of kidney biopsy in diabetic patients with an atypical proteinuric clinical course, because patients with MCD clearly respond to corticotherapy alone or in conjunction with other immunosuppressive agents.

Uma paciente de 19 anos de idade com diabetes tipo 1 durante quatro anos e uma paciente de 73 anos de idade com diabetes tipo 2 durante vinte anos desenvolveram quadro súbito de síndrome nefrótica. O exame histológico à microscopia de luz, imunofluorescência e microscopia eletrônica (em um caso) diagnosticou glomerulopatia de lesões mínimas (GLM) em ambos os casos. Na paciente de 73 anos de idade, houve uma associação com o uso de meloxicam. As duas pacientes foram tratadas com corticosteroides e responderam com remissão completa do quadro. A paciente de 19 anos com diabetes tipo 1 apresentou remissão completa sem recidivas, e a paciente de 73 anos com diabetes tipo 2 apresentou dois episódios de recidiva; a remissão completa foi conseguida após associação ao tratamento com ciclofosfamida. As duas pacientes foram seguidas dois anos após a remissão completa. Os casos descritos enfatizam o papel da biópsia renal em pacientes diabéticos com evolução atípica do aparecimento de proteinúria, pois pacientes com GLM respondem bem à corticoterapia como único tratamento ou associado a outro agente imunossupressor.

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INTRODUÇÃO: Tem ocorrido aumento da população de idosos e estes vêm envelhecendo em melhores condições clínicas do que no passado. Entretanto, a distribuição e evolução das doenças renais nos idosos ainda são pouco conhecidas, em parte devido à resistência em indicar biópsia renal. OBJETIVO: Avaliar a distribuição, a evolução e as características clínicas das nefropatias diagnosticadas por biópsia em pacientes idosos. PACIENTES E MÉTODOS: Foram avaliadas todas as biópsias renais percutâneas de rins nativos. Elas foram realizadas entre janeiro de 1990 e dezembro de 2006 em 71 pacientes com idade mínima de 60 anos (67,3 ± 6,5 anos), sendo 47 do gênero masculino e 24 do feminino. Os pacientes foram agrupados conforme a indicação clínica da biópsia. RESULTADOS: Síndrome nefrótica foi verificada em 35 pacientes (49,3 por cento) associada, na maioria dos casos, à nefropatia membranosa (17 casos), seguida por amiloidose e glomeruloesclerose segmentar e focal com 7 casos cada. Hipótese diagnóstica de injúria renal aguda (IRA), com 19 pacientes, teve como principais diagnósticos a necrose tubular aguda (6 casos) e a nefropatia do cilindro (3 casos). Dentre os 19 casos, apenas dois tiveram evolução satisfatória, enquanto os demais morreram precocemente ou evoluíram para doença renal avançada. Doze pacientes biopsiados por hematúria ou proteinúria assintomática tiveram diagnósticos variados, mas a maioria já apresentava nefropatia crônica relevante. Biópsia por síndrome nefrítica ocorreu em 5 casos, também com diagnósticos variados. CONCLUSÕES: Síndrome nefrótica foi a principal indicação de biópsia renal com a nefropatia membranosa como diagnóstico mais frequente. Entre os pacientes com IRA e hematúria ou proteinúria assintomática os diagnósticos foram variados com elevadas taxas de nefropatia crônica avançada.

INTRODUCTION: The elderly population is growing and aging in better clinical conditions than in the past. However, the distribution and course of kidney diseases in elderly patients are not well known partially due to reluctance to indicate renal biopsies in those patients. OBJECTIVE: To evaluate the distribution, clinical features, and outcomes of nephropathies diagnosed by biopsy in the elderly. PATIENTS AND METHODS: Seventyone patients (47 males, 24 females) aged 60 years or older (67.3 ± 6.5 years), undergoing biopsy from January 1990 to December 2006, were evaluated. They were grouped according to their clinical syndromes. RESULTS: Nephrotic syndrome was observed in 35 patients (49.3 percent), mainly associated with membranous nephropathy (17 patients), followed by amyloidosis and focal segmental glomerulosclerosis (seven patients each). Acute kidney injury (AKI) was diagnosed in 19 patients, and the main histopathological diagnoses were acute tubular necrosis (six patients) and cast nephropathy (three patients). Of those 19 patients, only two had a favorable course, while the others died early or progressed toward advanced chronic kidney disease. Twelve patients undergoing biopsy because of asymptomatic hematuria or proteinuria had different diagnoses, but most of them already had significant chronic nephropathy. In five patients with nephritic syndrome, the biopsies also showed several diagnoses. CONCLUSIONS: Nephrotic syndrome was the major indication for renal biopsy, and membranous nephropathy was the most frequent diagnosis. Among patients with AKI and asymptomatic hematuria or proteinuria, different diagnoses were found with high levels of advanced chronic nephropathy.

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INTRODUCTION: The elderly population is growing and aging in better clinical conditions than in the past. However, the distribution and course of kidney diseases in elderly patients are not well known partially due to reluctance to indicate renal biopsies in those patients. OBJECTIVE: To evaluate the distribution, clinical features, and outcomes of nephropathies diagnosed by biopsy in the elderly. PATIENTS AND METHODS: Seventy-one patients (47 males, 24 females) aged 60 years or older (67.3 ± 6.5 years), undergoing biopsy from January 1990 to December 2006, were evaluated. They were grouped according to their clinical syndromes. RESULTS: Nephrotic syndrome was observed in 35 patients (49.3%), mainly associated with membranous nephropathy (17 patients), followed by amyloidosis and focal segmental glomerulosclerosis (seven patients each). Acute kidney injury (AKI) was diagnosed in 19 patients, and the main histopathological diagnoses were acute tubular necrosis (six patients) and cast nephropathy (three patients). Of those 19 patients, only two had a favorable course, while the others died early or progressed toward advanced chronic kidney disease. Twelve patients undergoing biopsy because of asymptomatic hematuria or proteinuria had different diagnoses, but most of them already had significant chronic nephropathy. In five patients with nephritic syndrome, the biopsies also showed several diagnoses. CONCLUSIONS: Nephrotic syndrome was the major indication for renal biopsy, and membranous nephropathy was the most frequent diagnosis. Among patients with AKI and asymptomatic hematuria or proteinuria, different diagnoses were found with high levels of advanced chronic nephropathy.

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BACKGROUND: Interleukin-12 (IL12) participates in the pathophysiology of various experimental types of progressive glomerulonephritis, but its role in acute mesangial glomerulonephritis (AMG) induced by habu snake venom (HSV) has not been determined. This study aims to evaluate the effect of the absence of IL12 on AMG induced by HSV. METHODS: AMG was induced in IL12 knockout (IL12-/-) and C57Bl/6 (IL12+/+) mice by a single i.v. administration of HSV. Vehicle was used in control animals. Mice were studied after 3, 7, and 14 days (D3, D7, and D14). RESULTS: After treatment with HSV, IL12+/+ and -/- mice developed focal glomerular lesions, but groups of both lineages showed no statistical difference concerning albuminuria, serum creatinine, histopathology, number of cells by glomerular tuft, and glomerular tuft area. Compared to IL12+/+ mice, IL12-/- mice showed lower scores of glomerular desmin expression on D7 [1.55 (1.32; 1.65) vs. 1.12 (1.07; 1.22); p < 0.01] and D14 [1.60 (1.55; 1.75) vs. 1.20 (1.15; 1.20); p < 0.001], respectively, and lower scores of glomerular alpha-SMA expression on D14 [0.30 (0.21; 0.38) vs. 0.16 (0.26; 0.36); p < 0.001], respectively. CONCLUSION: The absence of IL12 reduced the activity of mesangial cells, but did not modify the course of HSV-induced AMG in mice.

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BACKGROUND/AIMS: The urinary concentration of the monocyte chemoattractant protein-1 (uMCP-1) chemokine is increased in several proteinuric and/or inflammatory renal diseases. In the present study, we evaluated the association between uMCP-1 and renal function, proteinuria, glomerular and interstitial macrophage infiltration, and renal fibrosis in patients with primary and secondary glomerulopathies diagnosed by renal biopsy. METHODS: Thirty-seven patients aged 32.6 +/- 7.7 years were studied. uMCP-1 was determined by ELISA. Renal macrophage expression (CD68 positive cells) is reported as number of macrophages/10(4) microm2 of the cortical tubulointerstitial (TI) area or of glomerular capillary tuft area. Cortical interstitial fibrosis was quantitated by PicroSirius red staining under polarized light by a computerized manner. RESULTS: The uMCP-1 ratio (pg/ml/urinary creatinine mg/ml) was positively correlated (Spearman coefficient) with proteinuria (r = 0.4629; p < 0.005) and number of macrophages in the cortical TI area (r = 0.64; p = 0.0005), and negatively correlated with creatinine clearance (r = -0.4877; p < 0.001). The uMCP-1 ratio was not significantly correlated with number of macrophages/glomerular capillary tuft area (r = 0.27; p = 0.19) or with percent cortical interstitial fibrosis (r = 0.08; p = 0.62). CONCLUSIONS: The uMCP-1 excretion is a biomarker of the inflammatory activity of the TI area, and does not reflect chronic interstitial damage.

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