Body fat predicts urinary tract infection in kidney transplant recipients: a prospective cohort study.

BACKGROUND: The association between obesity and infectious diseases is increasingly reported in the literature. There are scarce studies on the association between obesity and urinary tract infection after kidney transplantation (KTx). These studies defined obesity based on body mass index, and their results were conflicting. The present study aimed to evaluate this association using bioelectrical impedance analysis for body composition evaluation, and obesity definition. METHODS: A single-center cohort study was conducted. Demographic, clinical, anthropometric, and laboratory data were collected at KTx admission, and bioelectrical impedance analysis was performed to measure the visceral fat area, waist circumference, and total fat mass. The occurrence of urinary tract infection (symptomatic bacteriuria and/or histological evidence of pyelonephritis) was evaluated within three months after KTx. RESULTS: Seventy-seven patients were included in the cohort, and 67 were included in the final analysis. Urinary tract infection was diagnosed in 23.9% of the transplanted patients. Waist circumference (HR: 1.053; 95% CI 1.005-1.104; p = 0.032), visceral fat area (HR: 1.015; 95% CI 1.003-1.027; p = 0.014), and total fat mass (HR: 1.075; 95% CI 1.008-1.146; p = 0.028) were associated with urinary tract infection occurrence after KTx, using Cox regression models. Patients with high waist circumference (above 102 cm for men and above 88 cm for women) had a 4.7 times higher risk of a urinary tract infection than those with normal waist circumference (HR: 4.726; 95% CI 1.267-17.630; p = 0.021). Kaplan-Meier curves showed that patients with high waist circumference, high visceral fat area, and high total fat mass had more urinary tract infections (Log-rank test p = 0.014, p = 0.020, and p = 0.018, respectively). Body mass index was not able to predict urinary tract infection in the study sample. CONCLUSIONS: Waist circumference, visceral fat area, and total fat mass, assessed by bioelectrical impedance analysis, were predictors of urinary tract infection risk within the first three months after KTx.

Medicina e espiritualidade: o perfil de estudantes e médicos de uma escola médica brasileira / Medicine and spirituality: the profile of students and physicians at a Brazilian medical school

Resumo Introdução: Pesquisas científicas indicam que a espiritualidade desempenha um papel importante na vida da maioria dos pacientes. Além disso, atividades e crenças religiosas podem, de acordo com algumas pesquisa, estar relacionadas à melhor saúde e qualidade de vida1. Objetivo: Este estudo teve como objetivos avaliar o nível de espiritualidade de estudantes de Medicina e de médicos já formados, e analisar o ensino da interface "medicina e espiritualidade" na escola médica. Método: Realizamos um estudo transversal descritivo por meio da aplicação de questionários a estudantes de Medicina e médicos de uma escola médica pública brasileira. Resultado: Avaliaram-se 234 participantes. A maioria acredita em uma força superior. A espiritualidade foi maior entre médicos já formados e entre pessoas do sexo feminino. A maioria acredita que a formação universitária não prepara o médico para abordar o tema com os pacientes. Apesar dessa limitação, a maioria já abordou a espiritualidade com seus pacientes. Conclusão: Médicos e estudantes de Medicina consideram importante contemplar, de maneira ecumênica e respeitosa, aspectos espirituais dos pacientes. Apesar disso, consideram que não receberam preparo suficiente na escola médica para essa abordagem.

Abstract Introduction: Scientific research indicates that spirituality plays an important role in the daily life of most patients. Moreover, there are studies indicating that religious activities and beliefs may be related to better health and quality of life1. Objective: to evaluate the level of spirituality of medical students and graduated physicians, in addition to analyzing the teaching of the "Medicine and Spirituality" interface in medical school. Methodology: A descriptive cross-sectional study was carried out by applying questionnaires to medical students and doctors at a Brazilian public medical school. Results: a total of 234 participants were evaluated. Most believe in a higher power. Spirituality was higher among doctors who had already graduated and among females. Most believe that university education does not prepare doctors to address the topic with the patients. Despite this limitation, most have already discussed spirituality with their patients. Conclusion: Doctors and medical students consider it important to contemplate spiritual aspects of patients using an ecumenical and respectful approach. Nevertheless, they consider they were not sufficiently prepared in medical school for this approach.

Cost-effectiveness analysis of cinacalcet vs. paricalcitol in the treatment of hyperparathyroidism secondary to chronic kidney disease / Análise de custo-efetividade do cinacalcete vs. paricalcitol no tratamento do hiperparatireoidismo secundário à doença renal crônica

ABSTRACT Introduction For the reduction of PTH levels, two classes of drugs are available in the Brazilian market: non-selective and selective vitamin D receptor activators and calcimimetics. Among the mentioned drugs, the SUS provides oral calcitriol, paricalcitol and cinacalcet. Objectives: Develop cost-effectiveness (CE) and budgetary impact (BI) analysis of cinacalcet versus paricalcitol for patients on dialysis with SHPT, from the perspective of SUS. Methodology: A decision tree model was constructed for CE analysis, which considered the outcome of avoided parathyroidectomy and a time horizon of 1 year. As for the BI analysis, two scenarios were considered, one of which was measured demand and other epidemiological, based on data from the Brazilian Society of Nephrology (BSN). Results: The CE analysis showed that the use of cinacalcet results in one-off savings of R$1,394.64 per year and an incremental effectiveness of 0.08, in relation to avoided parathyroidectomy. The incremental CE ratio (ICER) was - R$ 17,653.67 per avoided parathyroidectomy for cinacalcet, as it was more effective and cheaper compared to paricalcitol. As for the BI analysis, it was estimated that the incremental BI with the expansion of the use of cinacalcet in the SUS will be between - R$ 1,640,864.62 and R$ 166,368.50 in the first year, considering the main and the epidemiological scenarios. At the end of 5 years after the expansion of use, an BI was estimated between - R$ 10,740,743.86 and - R$ 1,191,339.37; considering the same scenarios. Conclusion: Cinacalcet was dominant to avoid parathyroidectomies, being cost-effective.

RESUMO Introdução: Para a redução dos níveis do paratormônio (PTH) estão disponíveis no mercado brasileiro duas classes de medicamentos: ativadores do receptor da vitamina D (não seletivos e seletivos) e calcimiméticos. Dentre os medicamentos supracitados, o SUS disponibiliza calcitriol oral, paricalcitol e cinacalcete. Objetivos: Desenvolver análise de custo-efetividade (CE) e de impacto orçamentário (IO) do cinacalcete versus paricalcitol para pacientes em diálise com HPTS, na perspectiva do SUS. Metodologia: Foi construído um modelo de árvore de decisão para a análise de CE, que considerou o desfecho paratireoidectomia evitada e um horizonte temporal de 1 ano. Quanto à análise de IO, foram considerados dois cenários, um de demanda aferida e outro de abordagem epidemiológica, baseado nos dados da Sociedade Brasileira de Nefrologia (SBN). Resultados: A análise de CE mostrou que o uso de cinacalcete resulta em economia de R$ 1.394,64 ao ano e efetividade incremental de 0,08, em relação a paratireoidectomia evitada. A razão de CE incremental (RCEI) foi de - R$ 17.653,67 por paratireoidectomia evitada para o cinacalcete, já que se mostrou mais efetivo e mais barato comparado ao paricalcitol. Estimou-se que o IO incremental com a ampliação do uso do cinacalcete no SUS estará entre - R$ 1.640.864,62 e R$ 166.368,50 no primeiro ano, considerando os cenários principal e epidemiológico baseado nos dados da SBN. Já ao final de 5 anos após a ampliação do uso, estimou-se um impacto incremental entre - R$ 10.740.743,86 e - R$ 1.191.339,37; considerando os mesmos cenários. Conclusão: Cinacalcete foi dominante para evitar paratireoidectomias, sendo custo-efetivo.

Exploring the COVID-19 Pandemic's Impact on the Health Technology Assessment Process of the National Commission for the Incorporation of Technologies Into the Brazilian Health System.

OBJECTIVES: This study aimed to evaluate the impact of the COVID-19 pandemic on Brazilian health technology assessment processes based on public reports from the National Committee for Health Technology Incorporation (CONITEC). METHODS: This descriptive study analyzed CONITEC's official reports on Brazil available on its website between 2018 and 2021 that aimed to propose recommendations for technologies to be incorporated into its public healthcare system. We used descriptive statistics covering the number of technologies and number of reports about drugs per year, objective, type of technology, demanding sector, and outcome before 2018 to 2019 and during the COVID-19 pandemic (2020-2021). Furthermore, we used logistic regression to explore any association between the final decision labeled as "incorporated" and the emergence of the COVID-19 pandemic. RESULTS: A total of 278 reports were analyzed. Approximately 85% (136 of 278), 79% (220 of 278), and 45% of the reports (125 of 278) were about drugs, for incorporation, and requested by the government, respectively. Moreover, 74 of 130 (57%) and 56 of 148 decisions (38%) were "incorporated" before and during the pandemic, respectively. No significant association was noted between incorporated decisions and the arrival of the COVID-19 pandemic for all technologies (odds ratio 1.43; 95% CI 0.84-2.46; P = .192) and for drugs (odds ratio 1.43; 95% confidence interval 0.81-2.53; P = .223) while adjusting for the type of technology and demandant. CONCLUSIONS: The COVID-19 pandemic has brought many challenges, but it does not seem to have had a significant impact on the health technology assessment approval decisions of CONITEC in Brazil.

Cost-effectiveness analysis of cinacalcet vs. paricalcitol in the treatment of hyperparathyroidism secondary to chronic kidney disease.

INTRODUCTION: For the reduction of PTH levels, two classes of drugs are available in the Brazilian market: non-selective and selective vitamin D receptor activators and calcimimetics. Among the mentioned drugs, the SUS provides oral calcitriol, paricalcitol and cinacalcet. OBJECTIVES: Develop cost-effectiveness (CE) and budgetary impact (BI) analysis of cinacalcet versus paricalcitol for patients on dialysis with SHPT, from the perspective of SUS. METHODOLOGY: A decision tree model was constructed for CE analysis, which considered the outcome of avoided parathyroidectomy and a time horizon of 1 year. As for the BI analysis, two scenarios were considered, one of which was measured demand and other epidemiological, based on data from the Brazilian Society of Nephrology (BSN). RESULTS: The CE analysis showed that the use of cinacalcet results in one-off savings of R$1,394.64 per year and an incremental effectiveness of 0.08, in relation to avoided parathyroidectomy. The incremental CE ratio (ICER) was - R$ 17,653.67 per avoided parathyroidectomy for cinacalcet, as it was more effective and cheaper compared to paricalcitol. As for the BI analysis, it was estimated that the incremental BI with the expansion of the use of cinacalcet in the SUS will be between - R$ 1,640,864.62 and R$ 166,368.50 in the first year, considering the main and the epidemiological scenarios. At the end of 5 years after the expansion of use, an BI was estimated between - R$ 10,740,743.86 and - R$ 1,191,339.37; considering the same scenarios. CONCLUSION: Cinacalcet was dominant to avoid parathyroidectomies, being cost-effective.

Cost-effectiveness analysis of intravenous paricalcitol vs. oral calcitriol in the treatment of hyperparathyroidism secondary to chronic kidney disease / Análise de custo-efetividade do paricalcitol intravenoso vs. calcitriol oral no tratamento do hiperparatireoidismo secundário à doença renal crônica

Abstract Introduction: Hyperparathyroidism (SHPT) secondary to chronic kidney disease (CKD) is characterized by high levels of parathyroid hormone (PTH), hyperplasia of the parathyroid glands and cardiovascular disease. Selective and non-selective and selective vitamin D-receptor activators, calcimimetics, are available in the Brazilian market to reduce PTH levels. Objectives: To develop a cost-effectiveness (C/E) and budgetary impact (BI) analysis of intravenous paricalcitol vs. oral calcitriol for patients on dialysis with SHPT, from the perspective of the Brazilian Public Health Care System (SUS). Methodology: We built a decision-tree model to analyze C/E, which considered the outcome of avoided death and a time horizon of 1 year. As for the BI analysis, two scenarios were considered, one of demand and one of epidemiological approach, based on data from the Brazilian Society of Nephrology. Results: The analysis showed that the C/E ratio was R$ 1,213.68 per year, and an incremental effectiveness of 0.032, referring to avoided death. The incremental C/E ratio was R$37,927.50 per death averted by paricalcitol. It was estimated that the incremental BI with the expansion of paricalcitol use will be between R$1,600,202.28 and R$4,128,565.65 in the first year, considering the main and epidemiological scenarios. At the end of 5 years after the expansion of its use, an incremental BI was estimated between R$ 48,596,855.50 and R$ 62,90,555.73. Conclusion: Intravenous paricalcitol has superior efficacy and similar safety to oral calcitriol, reducing the overall mortality of dialysis patients, although it implies a higher cost.

Resumo Introdução: O hiperparatireoidismo secundário (HPTS) à doença crônica renal (DRC) é caracterizado por elevados níveis de paratormônio (PTH), hiperplasia das glândulas paratireoides e doença cardiovascular. Para a redução dos níveis do PTH, estão disponíveis no mercado brasileiro os ativadores não seletivos e seletivos do receptor da vitamina D e os calcimiméticos. Objetivos: Desenvolver análise de custo-efetividade (C/E) e de impacto orçamentário (IO) do paricalcitol intravenoso vs. calcitriol oral para pacientes em diálise com HPTS, na perspectiva do Sistema Único de Saúde. Metodologia: Foi construído um modelo de árvore de decisão para a análise de C/E, que considerou o desfecho morte evitada e um horizonte temporal de 1 ano. Quanto à análise de IO, foram considerados dois cenários, sendo um de demanda aferida e um de abordagem epidemiológica, baseado nos dados da Sociedade Brasileira de Nefrologia. Resultados: A análise mostrou que a relação de C/E foi de R$ 1.213,68 ao ano, e uma efetividade incremental de 0,032, referente à morte evitada. A razão de C/E incremental foi de R$ 37.927,50 por morte evitada para o paricalcitol. Estimou-se que o IO incremental com a ampliação do uso do paricalcitol estará entre R$ 1.600.202,28 e R$ 4.128.565,65 no primeiro ano, considerando os cenários principal e o epidemiológico. Já no fim de 5 anos após a ampliação do uso, estimou-se IO incremental entre R$ 48.596.855,50 e R$ 62.90.555,73. Conclusão: O paricalcitol intravenoso tem eficácia superior e segurança semelhante ao comparador calcitriol oral, diminuindo a mortalidade geral dos pacientes em diálise, embora implique maior custo.

Cost-effectiveness analysis of intravenous paricalcitol vs. oral calcitriol in the treatment of hyperparathyroidism secondary to chronic kidney disease.

INTRODUCTION: Hyperparathyroidism (SHPT) secondary to chronic kidney disease (CKD) is characterized by high levels of parathyroid hormone (PTH), hyperplasia of the parathyroid glands and cardiovascular disease. Selective and non-selective and selective vitamin D-receptor activators, calcimimetics, are available in the Brazilian market to reduce PTH levels. OBJECTIVES: To develop a cost-effectiveness (C/E) and budgetary impact (BI) analysis of intravenous paricalcitol vs. oral calcitriol for patients on dialysis with SHPT, from the perspective of the Brazilian Public Health Care System (SUS). METHODOLOGY: We built a decision-tree model to analyze C/E, which considered the outcome of avoided death and a time horizon of 1 year. As for the BI analysis, two scenarios were considered, one of demand and one of epidemiological approach, based on data from the Brazilian Society of Nephrology. RESULTS: The analysis showed that the C/E ratio was R$ 1,213.68 per year, and an incremental effectiveness of 0.032, referring to avoided death. The incremental C/E ratio was R$37,927.50 per death averted by paricalcitol. It was estimated that the incremental BI with the expansion of paricalcitol use will be between R$1,600,202.28 and R$4,128,565.65 in the first year, considering the main and epidemiological scenarios. At the end of 5 years after the expansion of its use, an incremental BI was estimated between R$ 48,596,855.50 and R$ 62,90,555.73. CONCLUSION: Intravenous paricalcitol has superior efficacy and similar safety to oral calcitriol, reducing the overall mortality of dialysis patients, although it implies a higher cost.

Effects of Plasma-Lyte® and 0. 9% saline in renal function after deceased-donor kidney transplant: a randomized controlled trial

Abstract Background The influence of different crystalloid solutions infused during deceased-donor kidney transplant on the incidence of delayed graft function remains unclear. We investigated the influence of Plasma-Lyte® vs. 0.9% saline on the incidence of delayed graft function in deceased-donor kidney transplant recipients. Methods We conducted a single-blind randomized controlled trial of 104 patients aged 18 to 65 years who underwent deceased-donor kidney transplant under general anesthesia. Patients were randomly assigned to receive either Plasma-Lyte® (n = 52) or 0.9% saline (n = 52), at the same infusion volume, for intraoperative fluid replacement. The primary outcome was the occurrence of delayed graft function. Secondary outcomes included metabolic and electrolytic changes at the end of surgery. Results Two patients in the Plasma-Lyte® group and one in the 0.9% saline group died postoperatively and were not included for analysis. The incidence of delayed graft function in Plasma-Lyte® and 0.9% saline groups were 60.0% (95% Confidence Interval [95% CI 46.2-72.4]) and 74.5% (95% CI 61.1-84.4), respectively (p= 0.140). Mean (standard deviation) values of immediate postoperative pH and serum chloride levels in Plasma-Lyte® and 0.9% saline groups were 7.306 (0.071) and 7.273 (0.061) (p= 0.013), and 99.6 (4.2) mEq.L-1 and 103.3 (5.6) mEq.L-1, respectively (p< 0.001). All other postoperative metabolic and electrolyte variables were not statistically different at the immediate postoperative period (p> 0.05). Conclusion In deceased-donor kidney transplant recipients, the incidence of delayed graft function is not influenced by Plasma-Lyte® or 0.9% saline used for intraoperative fluid replacement.

Migratory pattern of the coronavirus disease 2019 and high fatality rates among kidney transplant recipients: report from the Brazilian Multicenter Cohort Study / Padrão migratório da doença coronavírus 2019 e altas taxas de mortalidade entre receptores de transplante renal: relatório do Estudo de Coorte Multicêntrico Brasileiro

Abstract Introduction: The unprecedented coronavirus disease 2019 (COVID-19) pandemic has affected kidney transplant (KT) recipients, with worldwide fatality rates around 25%. Considering the well-known Brazilian socio-demographic disparities, this report describes for the first time the main outcomes of COVID-19 in KT recipients according to Brazilian geographic regions. Methods: This multicenter national retrospective analysis included data from KT recipients with confirmed COVID-19 between March and November 2020. Results: Thirty-five of the 81 centers (57% of KT activity in Brazil) reported 1,680 patients with COVID-19. The Northeast was the first to reach the peak in the number of infections. The Southeast, due to its population density, contributed with the largest number of patients. Patients had a median age of 52 years, 76% had hypertension and 34% diabetes, 75% were recipients of a deceased donor, and the time interval between diagnosis and transplantation was 5.9 years. In 53% of patients, immunosuppression was adjusted, and clinical support varied according to geographic region. Hospitalization was required for 65% of the patients, 35% of them needed intensive care, 25% mechanical ventilation, and 23% renal replacement therapy. The 90-day overall fatality was 21%, being 23% in the Southeast, 16% in the Northeast, and 19% in the Central-west and South regions. Conclusion: The migratory pattern of the pandemic among KT recipients followed that of the general population and the outcomes were influenced by regional features. COVID-19 in KT recipients was associated with high utilization of health-care resources and higher fatality rates than those reported in the general population.

Resumo Introdução: A pandemia da COVID-19 afetou receptores de transplante renal (TR), com taxas de mortalidade mundial em torno de 25%. Considerando as notórias disparidades sócio-demográficas brasileiras, este relatório descreve pela primeira vez principais características e desfechos da doença em receptores de TR, segundo as regiões geográficas. Métodos: Esta análise retrospectiva multicêntrica nacional incluiu dados de receptores de TR com COVID-19 confirmada entre Março/Novembro de 2020. Resultados: Trinta e cinco dos 81 centros (57% da atividade de transplante renal brasileira) relataram 1.680 pacientes com COVID-19. O Nordeste foi o primeiro a atingir o pico no número de infecções. O Sudeste, por sua densidade populacional, contribuiu com maior número de pacientes. Pacientes tinham em média 52 anos, 76% apresentavam hipertensão e 34% diabetes, 75% receptores de doador falecido e o tempo entre diagnóstico e transplante foi de 5,9 anos. Em 53% dos pacientes, os imunossupressores foram ajustados, e o tratamento variou segundo a região. Hospitalização foi necessária para 65% dos pacientes, 35% necessitaram de cuidados intensivos, 25% ventilação mecânica, e 23% terapia renal substitutiva. A mortalidade geral em 90 dias foi 21%, sendo 23% no Sudeste, 16% no Nordeste, e 19% nas regiões Centro-Oeste e Sul. Conclusão: O padrão migratório da pandemia entre os receptores de TR seguiu o da população em geral e os desfechos foram influenciados por características regionais. A COVID-19 em receptores de TR foi associada à alta utilização de recursos de saúde e taxas de mortalidade mais altas do que as relatadas na população em geral.

Recommendations for the diagnosis and management of Fabry disease in pediatric patients: a document from the Rare Diseases Committee of the Brazilian Society of Nephrology (Comdora-SBN) / Recomendações para o diagnóstico e manejo de pacientes pediátricos com doença de Fabry: documento do comitê de doenças raras da Sociedade Brasileira de Nefrologia (Comdora-SBN)

Fabry disease (FD) is a genetic disease, with X-chromosome linked inheritance, due to variants in the GLA gene that encodes the α-galactosidase A (α-GAL) enzyme. The purpose of the present study was to create a consensus aiming to standardize the recommendations regarding the renal involvement of FD with guidelines on the diagnosis, screening, and treatment of pediatric patients. This consensus is an initiative of the Rare Diseases Committee (Comdora) of the Brazilian Society of Nephrology (SBN). Randomized controlled clinical studies and studies with real-life data added to the authors' experience were considered for this review. The result of this consensus was to help manage patient and physician expectations regarding treatment outcomes. Thus, this consensus document recommends the investigation of the pediatric family members of an index case, as well as cases with suggestive clinical signs. From the diagnosis, assess all possible FD impairments and grade through scales. From an extensive review of the literature including pediatric protocols and particularly evaluating pediatric cases from general studies, it can be concluded that the benefits of early treatment are great, especially in terms of neuropathic pain and renal impairment parameters and outweigh the possible adverse effects that were mainly manifested by infusion reactions.

A doença de Fabry (DF) é uma doença genética, com herança ligada ao cromossomo X, devido a variantes no gene GLA que codifica a enzima α-galactosidase A (α-GAL). O propósito do presente estudo foi criar um consenso objetivando padronizar as recomendações em relação ao acometimento renal da DF com orientações sobre o diagnóstico, rastreamento, e tratamento de pacientes pediátricos. Esse consenso é uma iniciativa do Comitê de Doenças Raras (Comdora) da Sociedade Brasileira de Nefrologia (SBN). Foram considerados para esta revisão estudos clínicos controlados randomizados e estudos com dados de vida real somados à experiência dos autores. O resultado desse consenso foi auxiliar no gerenciamento das expectativas de pacientes e médicos quanto aos resultados do tratamento. Assim, este documento de consenso recomenda a investigação dos familiares pediátricos de um caso índice, assim como de casos com clínica sugestiva. A partir do diagnóstico, avaliar todos os possíveis acometimentos da DF e graduar através de escalas. A partir de uma revisão extensa da literatura incluindo protocolos pediátricos e avaliando particularmente os casos pediátricos de estudos gerais, pode-se concluir que os benefícios do tratamento precoce são grandes, principalmente quanto aos parâmetros de dor neuropática e do acometimento renal, e suplantam os possíveis adversos que foram sobretudo manifestados por reações infusionais.

Brazilian consensus recommendations for the diagnosis, screening, and treatment of individuals with fabry disease: Committee for Rare Diseases - Brazilian Society of Nephrology/2021 / Consenso brasileiro de doença de fabry: recomendações de diagnóstico, triagem e tratamento. Comitê de doenças raras (Comdora) - SBN/2021

Abstract Fabry disease (FD) is an X-linked inherited disorder caused by mutations in the GLA gene encoding enzyme alpha-galactosidase A (α-Gal A). The purpose of this study was to produce a consensus statement to standardize the recommendations concerning kidney involvement in FD and provide advice on the diagnosis, screening, and treatment of adult and pediatric patients. This consensus document was organized from an initiative led by the Committee for Rare Diseases (Comdora) of the Brazilian Society of Nephrology (SBN). The review considered randomized clinical trials, real-world data studies, and the expertise of its authors. The purpose of this consensus statement is to help manage patient and physician expectations concerning the outcomes of treatment. Our recommendations must be interpreted within the context of available evidence. The decisions pertaining to each individual case must be made with the involvement of patients and their families and take into account not only the potential cost of treatment, but also concurrent conditions and personal preferences. The Comdora intends to update these recommendations regularly so as to reflect recent literature evidence, real-world data, and appreciate the professional experience of those involved. This consensus document establishes clear criteria for the diagnosis of FD and for when to start or stop specific therapies or adjuvant measures, to thus advise the medical community and standardize clinical practice.

Resumo A doença de Fabry (DF) é uma doença genética, com herança ligada ao cromossomo X, que ocorre devido a variantes no gene GLA que codifica a enzima α-galactosidase A (α-GAL). O propósito do presente estudo foi criar um consenso objetivando padronizar as recomendações em relação ao acometimento renal da DF com orientações sobre o diagnóstico, rastreamento e tratamento de pacientes adultos e pediátricos. Esse consenso é uma iniciativa do Comitê de Doenças Raras (Comdora) da Sociedade Brasileira de Nefrologia (SBN). Foram considerados para esta revisão estudos clínicos controlados randomizados e estudos com dados de vida real somado à experiência dos autores. O resultado desse consenso foi auxiliar no gerenciamento das expectativas de pacientes e médicos quanto aos resultados do tratamento. Nossas recomendações devem ser interpretadas no contexto das evidências e ressaltando que as decisões finais devem ser tomadas individualmente, em uma decisão conjunta com o paciente e familiares, considerando os custos envolvidos, não apenas financeiros, doenças concomitantes e preferências pessoais. O Comdora pretende atualizar essas recomendações regularmente, e assim seguir novas evidências na literatura, considerar dados de vida real e valorizar a experiência profissional dos envolvidos. Esse consenso estabelece critérios claros para o diagnóstico da DF, início e interrupção de terapia específica e de medidas adjuntas, orientando a comunidade médica e uniformizando condutas.

Recommendations for the diagnosis and management of Fabry disease in pediatric patients: a document from the Rare Diseases Committee of the Brazilian Society of Nephrology (Comdora-SBN).

Fabry disease (FD) is a genetic disease, with X-chromosome linked inheritance, due to variants in the GLA gene that encodes the α-galactosidase A (α-GAL) enzyme. The purpose of the present study was to create a consensus aiming to standardize the recommendations regarding the renal involvement of FD with guidelines on the diagnosis, screening, and treatment of pediatric patients. This consensus is an initiative of the Rare Diseases Committee (Comdora) of the Brazilian Society of Nephrology (SBN). Randomized controlled clinical studies and studies with real-life data added to the authors' experience were considered for this review. The result of this consensus was to help manage patient and physician expectations regarding treatment outcomes. Thus, this consensus document recommends the investigation of the pediatric family members of an index case, as well as cases with suggestive clinical signs. From the diagnosis, assess all possible FD impairments and grade through scales. From an extensive review of the literature including pediatric protocols and particularly evaluating pediatric cases from general studies, it can be concluded that the benefits of early treatment are great, especially in terms of neuropathic pain and renal impairment parameters and outweigh the possible adverse effects that were mainly manifested by infusion reactions.

Brazilian consensus recommendations for the diagnosis, screening, and treatment of individuals with fabry disease: Committee for Rare Diseases - Brazilian Society of Nephrology/2021.

Fabry disease (FD) is an X-linked inherited disorder caused by mutations in the GLA gene encoding enzyme alpha-galactosidase A (α-Gal A). The purpose of this study was to produce a consensus statement to standardize the recommendations concerning kidney involvement in FD and provide advice on the diagnosis, screening, and treatment of adult and pediatric patients. This consensus document was organized from an initiative led by the Committee for Rare Diseases (Comdora) of the Brazilian Society of Nephrology (SBN). The review considered randomized clinical trials, real-world data studies, and the expertise of its authors. The purpose of this consensus statement is to help manage patient and physician expectations concerning the outcomes of treatment. Our recommendations must be interpreted within the context of available evidence. The decisions pertaining to each individual case must be made with the involvement of patients and their families and take into account not only the potential cost of treatment, but also concurrent conditions and personal preferences. The Comdora intends to update these recommendations regularly so as to reflect recent literature evidence, real-world data, and appreciate the professional experience of those involved. This consensus document establishes clear criteria for the diagnosis of FD and for when to start or stop specific therapies or adjuvant measures, to thus advise the medical community and standardize clinical practice.

Migratory pattern of the coronavirus disease 2019 and high fatality rates among kidney transplant recipients: report from the Brazilian Multicenter Cohort Study.

INTRODUCTION: The unprecedented coronavirus disease 2019 (COVID-19) pandemic has affected kidney transplant (KT) recipients, with worldwide fatality rates around 25%. Considering the well-known Brazilian socio-demographic disparities, this report describes for the first time the main outcomes of COVID-19 in KT recipients according to Brazilian geographic regions. METHODS: This multicenter national retrospective analysis included data from KT recipients with confirmed COVID-19 between March and November 2020. RESULTS: Thirty-five of the 81 centers (57% of KT activity in Brazil) reported 1,680 patients with COVID-19. The Northeast was the first to reach the peak in the number of infections. The Southeast, due to its population density, contributed with the largest number of patients. Patients had a median age of 52 years, 76% had hypertension and 34% diabetes, 75% were recipients of a deceased donor, and the time interval between diagnosis and transplantation was 5.9 years. In 53% of patients, immunosuppression was adjusted, and clinical support varied according to geographic region. Hospitalization was required for 65% of the patients, 35% of them needed intensive care, 25% mechanical ventilation, and 23% renal replacement therapy. The 90-day overall fatality was 21%, being 23% in the Southeast, 16% in the Northeast, and 19% in the Central-west and South regions. CONCLUSION: The migratory pattern of the pandemic among KT recipients followed that of the general population and the outcomes were influenced by regional features. COVID-19 in KT recipients was associated with high utilization of health-care resources and higher fatality rates than those reported in the general population.

High mortality among kidney transplant recipients diagnosed with coronavirus disease 2019: Results from the Brazilian multicenter cohort study.

BACKGROUND: Kidney transplant (KT) recipients are considered a high-risk group for unfavorable outcomes in the course of coronavirus disease 2019 (COVID-19). AIM: To describe the clinical aspects and outcomes of COVID-19 among KT recipients. METHODS: This multicenter cohort study enrolled 1,680 KT recipients diagnosed with COVID-19 between March and November 2020, from 35 Brazilian centers. The main outcome was the 90-day cumulative incidence of death, for the entire cohort and according to acute kidney injury (AKI) and renal replacement therapy (RRT) requirement. Fatality rates were analyzed according to hospitalization, intensive care unit (ICU) admission, and mechanical ventilation (MV) requirement. Multivariable analysis was performed by logistic regression for the probability of hospitalization and death. RESULTS: The median age of the recipients was 51.3 years, 60.4% were men and 11.4% were Afro-Brazilian. Comorbidities were reported in 1,489 (88.6%), and the interval between transplantation and infection was 5.9 years. The most frequent symptoms were cough (54%), myalgia (40%), dyspnea (37%), and diarrhea (31%), whereas the clinical signs were fever (61%) and hypoxemia (13%). Hospitalization was required in 65.1%, and immunosuppressive drugs adjustments were made in 74.4% of in-hospital patients. ICU admission was required in 34.6% and MV in 24.9%. In the multivariable modeling, the variables related with the probability of hospitalization were age, hypertension, previous cardiovascular disease, recent use of high dose of steroid, and fever, dyspnea, diarrhea, and nausea or vomiting as COVID-19 symptoms. On the other hand, the variables that reduced the probability of hospitalization were time of COVID-19 symptoms, and nasal congestion, headache, arthralgia and anosmia as COVID-19 symptoms. The overall 90-day cumulative incidence of death was 21.0%. The fatality rates were 31.6%, 58.2%, and 75.5% in those who were hospitalized, admitted to the ICU, and required MV, respectively. At the time of infection, 23.2% had AKI and 23.4% required RRT in the follow-up. The cumulative incidence of death was significantly higher among recipients with AKI (36.0% vs. 19.1%, P < 0.0001) and in those who required RRT (70.8% vs. 10.1%, P < 0.0001). The variables related with the probability of death within 90 days after COVID-19 were age, time after transplantation, presence of hypertension, previous cardiovascular disease, use of tacrolimus and mycophenolate, recent use of high dose of steroids, and dyspnea as COVID-19 symptom. On the other hand, the variables that reduced the risk of death were time of symptoms, and headache and anosmia as COVID-19 symptoms. CONCLUSION: The patients diagnosed with COVID-19 were long-term KT recipients and most of them had some comorbidities. One in every five patients died, and the rate of death was significantly higher in those with AKI, mainly when RRT was required.

Concordance analysis between dosed serum bicarbonate and that calculated by gas analysis in chronic renal patients / Análise de concordância entre o bicarbonato sérico dosado e o calculado pela gasometria em pacientes renais crônicos

Abstract Introduction: The control of metabolic acidosis in dialysis patients focuses on the supply of bicarbonate during the dialysis session, and it is not standard in all hemodialysis to assess serum bicarbonate concentrations. Bicarbonate expressed in blood gas analysis is the most sensitive standard of analysis and it is measured indirectly, using the Henderson-Hasselbalch equation. There are no studies in this population evaluating the concordance between the calculated bicarbonate with the direct method of biochemical analysis. The aim of this study was to analyze the concordance between the measured and calculated serum bicarbonate levels using blood gas analysis. Methods: We analyzed blood samples from chronic kidney patients undergoing hemodialysis, using the same sample of bicarbonate analysis by biochemistry and gasometry. The concordance was assessed using the Bland-Altman method. Results: 51 samples were analyzed. The analysis revealed a high correlation (r = 0.73) and a mean difference (bias) of 1.15 ± 3 mmol/L. The median time between collection and examination was 241 minutes. Discussion: We can conclude that the biochemical bicarbonate analysis compared to that calculated from blood gas analysis in chronic renal patients was consistent. For greater concordance between the data, it is important that the time between the collection of the samples and the referral to the laboratory for carrying out the dosages does not exceed four hours. The serum bicarbonate dosage can result in cost savings when compared to that of bicarbonate in blood gas analysis.

Resumo Introdução: O controle da acidose metabólica em pacientes dialíticos está voltado, principalmente, para o suprimento de bicarbonato durante a sessão de diálise, não sendo padrão em todas as hemodiálises avaliar as concentrações séricas do bicarbonato. O bicarbonato expresso na gasometria é considerado o padrão mais sensível de análise e é medido indiretamente por meio da equação de Henderson-Hasselbalch. Não há estudos nessa população avaliando a concordância do bicarbonato calculado com o método direto de análise bioquímica. O objetivo deste estudo é analisar a concordância entre o bicarbonato sérico medido e o calculado por meio da gasometria. Métodos: Foram analisadas amostras de sangue de pacientes renais crônicos em hemodiálise sendo feito na mesma amostra de análise do bicarbonato pela bioquímica e análise pela gasometria. A concordância foi avaliada pelo método de Bland-Altman. Resultados: Foram analisados um total de 51 amostras. A análise de correlação revelou alta correlação (r = 0.73) e a diferença média (bias) de 1.15 ± 3 mmol/L. O tempo mediano entre a realização da coleta e do exame foi de 241 minutos. Discussão: Podemos concluir que a realização da dosagem bioquímica do bicarbonato comparada com a calculada a partir da gasometria em pacientes renais crônicos foi concordante. Para maior concordância entre os dados, é importante que o tempo entre a coleta das amostras e o encaminhamento ao laboratório para a realização das dosagens não exceda quatro horas. A dosagem do bicarbonato sérico pode resultar numa economia de custos comparada à do bicarbonato da gasometria.

COMDORA-SBN recommendations for patients with rare kidney diseases in relation to the Covid-19 pandemic.

During the Covid-19 pandemic, the issue is how to maintain adequate care for people with other diseases. In this document, the SBN Rare Diseases Committee (COMDORA) gives some guidelines on the care of patients with rare kidney diseases. These patients should follow the recommendations for the general population, bearing in mind that, as they have chronic kidney disease, they are included in the risk group for more serious outcomes if they develop Covid-19. Non-essential decision-making procedures should be postponed. In stable cases under appropriate treatment, we must choose to contact our patients remotely, using teleconsultations and home exam collections (if possible). In the presence of a symptom or sign of decompensation of the underlying disease, or infection with Sars-cov-2, advise the patient to seek medical assistance. The patient should not be waiting to get worse. Changes to the prescription should only be made on a scientific basis. Dosage suspension or change is not recommended, even in cases in which the patient needs to go to a center to receive his medication; in this case, the infusion center must follow the recommendations of the Ministry of Health. If the patient develops Covid-19 and uses any drugs, check the need for dose adjustment of the routine medications. Avoid the use of antimetabolics and anti-CD20 in patients with Covid-19, as they reduce viral clearance and predispose to bacterial infections. Contact between the patient and the medical team is essential; changes are recommended only with specialized medical guidance.

Concordance analysis between dosed serum bicarbonate and that calculated by gas analysis in chronic renal patients.

INTRODUCTION: The control of metabolic acidosis in dialysis patients focuses on the supply of bicarbonate during the dialysis session, and it is not standard in all hemodialysis to assess serum bicarbonate concentrations. Bicarbonate expressed in blood gas analysis is the most sensitive standard of analysis and it is measured indirectly, using the Henderson-Hasselbalch equation. There are no studies in this population evaluating the concordance between the calculated bicarbonate with the direct method of biochemical analysis. The aim of this study was to analyze the concordance between the measured and calculated serum bicarbonate levels using blood gas analysis. METHODS: We analyzed blood samples from chronic kidney patients undergoing hemodialysis, using the same sample of bicarbonate analysis by biochemistry and gasometry. The concordance was assessed using the Bland-Altman method. RESULTS: 51 samples were analyzed. The analysis revealed a high correlation (r = 0.73) and a mean difference (bias) of 1.15 ± 3 mmol/L. The median time between collection and examination was 241 minutes. DISCUSSION: We can conclude that the biochemical bicarbonate analysis compared to that calculated from blood gas analysis in chronic renal patients was consistent. For greater concordance between the data, it is important that the time between the collection of the samples and the referral to the laboratory for carrying out the dosages does not exceed four hours. The serum bicarbonate dosage can result in cost savings when compared to that of bicarbonate in blood gas analysis.

COMDORA-SBN recommendations for patients with rare kidney diseases in relation to the Covid-19 pandemic / Recomendações do COMDORA-SBN a pacientes portadores de doenças renais raras em relação à pandemia de Covid-19

ABSTRACT During the Covid-19 pandemic, the issue is how to maintain adequate care for people with other diseases. In this document, the SBN Rare Diseases Committee (COMDORA) gives some guidelines on the care of patients with rare kidney diseases. These patients should follow the recommendations for the general population, bearing in mind that, as they have chronic kidney disease, they are included in the risk group for more serious outcomes if they develop Covid-19. Non-essential decision-making procedures should be postponed. In stable cases under appropriate treatment, we must choose to contact our patients remotely, using teleconsultations and home exam collections (if possible). In the presence of a symptom or sign of decompensation of the underlying disease, or infection with Sars-cov-2, advise the patient to seek medical assistance. The patient should not be waiting to get worse. Changes to the prescription should only be made on a scientific basis. Dosage suspension or change is not recommended, even in cases in which the patient needs to go to a center to receive his medication; in this case, the infusion center must follow the recommendations of the Ministry of Health. If the patient develops Covid-19 and uses any drugs, check the need for dose adjustment of the routine medications. Avoid the use of antimetabolics and anti-CD20 in patients with Covid-19, as they reduce viral clearance and predispose to bacterial infections. Contact between the patient and the medical team is essential; changes are recommended only with specialized medical guidance.

RESUMO Durante a pandemia da Covid-19, fica a questão de como manter o atendimento adequado aos portadores de outras doenças. O Comitê de Doenças Raras (COMDORA) da SBN neste documento dá algumas orientações ao atendimento de pacientes com doenças renais raras. Estes pacientes devem seguir as recomendações destinadas à população geral tendo em mente que, por serem portadores de doença renal crônica, estão incluídos no grupo de risco para desfechos mais graves, caso venham a desenvolver a Covid-19. Procedimentos não essenciais para tomada de decisão devem ser adiados. Deve-se optar por contatos a distância, como teleconsultas, e coletas de exames domiciliares (se possível) nos casos estáveis sob tratamento adequado. Na presença de sintoma ou sinal de descompensação da doença de base ou infecção pelo Sars-cov-2, orientar o paciente a procurar a equipe médica. O paciente não deve ficar esperando o quadro agravar-se. Alterações na prescrição só devem ser feitas com embasamento científico. Não se recomenda a suspensão ou alteração posológica, mesmo nos casos em que o paciente necessita ir a um centro para receber sua medicação; neste caso o centro de infusão deve seguir as recomendações do Ministério da Saúde. Caso o paciente desenvolva a Covid-19 e faça uso de alguma droga, verificar a necessidade de ajuste nas doses dos medicamentos rotineiros. Evitar o uso de antimetabólicos e antiCD20 nos pacientes com a Covid-9, por reduzirem o clareamento viral e predisporem a infecções bacterianas. O contato entre paciente e equipe médica é essencial; alterações são recomendadas apenas com orientação médica especializada.

Kidney transplantation with donors in severe acute kidney injury. Should we use these organs? Retrospective Case Series / Transplante renal com doadores em lesão renal aguda severa. Devemos utilizar esses órgãos? Série de casos retrospectiva

ABSTRACT Introduction: The number of incident and prevalent patients on dialysis has increased, as well as the number of candidates for renal transplantation in Brazil, without a proportional increase in the number of organ donors. The use of expanded kidneys, as to renal function, may be an alternative to increase the supply of organs. Objective: to discuss the feasibility of using expanded kidneys for renal function, which are in severe acute renal injury. Methods: All cases of renal transplantation of deceased donors performed at the Hospital das Clínicas de Botucatu of UNESP, from January 2010 to June 2018, totaling 732 cases were evaluated. Cases with final donor creatinine greater than 6 mg/dL were selected. Results: four patients were selected, of whom all donors were in severe acute kidney injury (AKI). These donors presented rhabdomyolysis as a probable cause of severe AKI, were young, with no comorbidities and had decreased urinary volume in the last 24 hours. The clinical evolution of all the recipients was satisfactory, with a glomerular filtration rate after transplantation ranging from 48 to 98 mL/min/1.73 m2. Conclusion: this series of cases shows the possibility of using renal donors in severe AKI, provided the following are respected: donor age, rhabdomyolysis as the cause of AKI, and implantation-favorable biopsy findings. Additional studies with better designs, larger numbers of patients and longer follow-up times are needed.

RESUMO Introdução: O número de pacientes incidentes e prevalentes em diálise tem aumentado, assim como o número de candidatos ao transplante renal no Brasil, sem um aumento proporcional do número de doadores de órgãos. O uso de rins expandidos, quanto à função renal, pode ser uma alternativa para aumentar a oferta de órgãos. Objetivo: discutir a viabilidade do uso de rins expandidos quanto à função renal, que estejam em lesão renal aguda severa. Métodos: foram avaliados todos os casos de transplante renal de doador falecido realizados no Hospital das Clínicas de Botucatu da UNESP, de janeiro de 2010 a junho de 2018, totalizando 732 casos. Selecionou-se os casos com creatinina final do doador maior do que 6 mg/dL. Resultados: quatro pacientes foram selecionados, dos quais todos os doadores estavam em lesão renal aguda (LRA) severa. Esses doadores apresentavam rabdomiólise como provável causa de LRA severa, eram jovens, sem comorbidades e apresentavam diminuição de volume urinário nas últimas 24 horas. A evolução clínica de todos os receptores foi satisfatória, com taxa de filtração glomerular após o transplante variando entre 48 a 98 mL/min/1,73m2. Conclusão: essa série de casos mostra a possibilidade de utilização de doadores renais em LRA severa, desde que respeitadas as condições seguintes: idade do doador, rabdomiólise como causa de LRA e achados de biópsia favoráveis à implantação. Estudos adicionais com melhores desenhos, maior número de pacientes e maiores tempos de seguimento são necessários.