Baseline characteristics and evolution of Brazilian patients with atypical hemolytic uremic syndrome: first report of the Brazilian aHUS Registry.

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.

Dengue infection in kidney transplant recipients: clinical course and its impact on renal function / Infecção por dengue em receptores de transplante renal: curso clínico e seu impacto na função renal

Abstract Introduction: Kidney transplant recipients (KTR) are at increased risk for dengue virus (DENV) infection. The aim of this study was to outline the clinical presentation and laboratory profile of DENV infection in KTR and its impact on renal function. Methods: This was a retrospective study of KTR diagnosed with DENV infection. Adult patients who visited Santa Casa de Belo Horizonte Nephrology Center between April and September 2019 were included. Patients who did not sign the Informed Consent were excluded. Data were collected from the database and medical records. The study was approved by the local Institutional Ethics Committee and the Informed Consent was obtained. Results: Nineteen KTR were evaluated. The main signs and symptoms were myalgia, headache/retro-orbital pain, fever, and gastrointestinal symptoms. Two patients had acute cholecystitis without calculus, three experienced pleural and/or pericardial effusion, and one developed acute myocarditis. All patients were under immunosuppression with prednisone, tacrolimus, and mycophenolate, and most were not receiving induction therapy. Temporary suspension/reduction of immunosuppression was required in 58% of patients and leukopenia was the most common reason. Thrombocytopenia was common and 58% of patients developed acute kidney injury. All patients recovered renal function. Conclusions: DENV infection in KTR patients seems to follow a similar course as in the general population. Although there was no control group, we suspect that immunosuppression, preexisting kidney disease or type of donor was not a determining factor in most patients. Transient renal dysfunction was common but reversible. No patient experienced death or graft loss.

Resumo Introdução: Receptores de transplante renal (RTR) apresentam maior risco de infecção pelo vírus da dengue (DENV). O objetivo deste estudo foi delinear apresentação clínica e perfil laboratorial da infecção por DENV em RTR e seu impacto na função renal. Métodos: Este foi um estudo retrospectivo de RTR diagnosticados com infecção por DENV. Incluiu-se pacientes adultos que estiveram no Centro Nefrológico da Santa Casa de Belo Horizonte entre Abril/Setembro, 2019. Foram excluídos pacientes que não assinaram o Termo de Consentimento Livre e Esclarecido. Dados foram coletados do banco de dados e registros médicos. O estudo foi aprovado pelo Comitê de Ética Institucional local e obteve-se o Consentimento Livre e Esclarecido. Resultados: Avaliou-se dezenove RTR. Principais sinais e sintomas foram mialgia, cefaleia/dor retro-orbital, febre, sintomas gastrointestinais. Dois pacientes apresentaram colecistite aguda sem cálculo, três sofreram derrame pleural e/ou pericárdico, um desenvolveu miocardite aguda. Todos os pacientes estavam sob imunossupressão com prednisona, tacrolimus, micofenolato. A maioria não estava recebendo terapia de indução. Suspensão/redução temporária da imunossupressão foi necessária em 58% dos pacientes e leucopenia foi a razão mais comum. Trombocitopenia foi comum e 58% dos pacientes desenvolveram lesão renal aguda. Todos recuperaram a função renal. Conclusões: A infecção por DENV em pacientes RTR parece seguir um curso semelhante ao da população em geral. Embora não houvesse grupo controle, suspeitamos que imunossupressão, doença renal preexistente ou tipo de doador não foram fator determinante na maioria dos pacientes. Disfunção renal transitória foi comum, mas reversível. Nenhum paciente veio ao óbito ou sofreu perda do enxerto.

Dengue infection in kidney transplant recipients: clinical course and its impact on renal function.

INTRODUCTION: Kidney transplant recipients (KTR) are at increased risk for dengue virus (DENV) infection. The aim of this study was to outline the clinical presentation and laboratory profile of DENV infection in KTR and its impact on renal function. METHODS: This was a retrospective study of KTR diagnosed with DENV infection. Adult patients who visited Santa Casa de Belo Horizonte Nephrology Center between April and September 2019 were included. Patients who did not sign the Informed Consent were excluded. Data were collected from the database and medical records. The study was approved by the local Institutional Ethics Committee and the Informed Consent was obtained. RESULTS: Nineteen KTR were evaluated. The main signs and symptoms were myalgia, headache/retro-orbital pain, fever, and gastrointestinal symptoms. Two patients had acute cholecystitis without calculus, three experienced pleural and/or pericardial effusion, and one developed acute myocarditis. All patients were under immunosuppression with prednisone, tacrolimus, and mycophenolate, and most were not receiving induction therapy. Temporary suspension/reduction of immunosuppression was required in 58% of patients and leukopenia was the most common reason. Thrombocytopenia was common and 58% of patients developed acute kidney injury. All patients recovered renal function. CONCLUSIONS: DENV infection in KTR patients seems to follow a similar course as in the general population. Although there was no control group, we suspect that immunosuppression, preexisting kidney disease or type of donor was not a determining factor in most patients. Transient renal dysfunction was common but reversible. No patient experienced death or graft loss.

Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.

BACKGROUND: Atypical Hemolytic Uremic Syndrome (aHUS) is an ultra-rare disease that potentially leads to kidney graft failure due to ongoing Thrombotic Microangiopathy (TMA). The aim was evaluating the frequency of TMA after kidney transplantation in patients with aHUS in a Brazilian cohort stratified by the use of the specific complement-inhibitor eculizumab. METHODS: This was a multicenter retrospective cohort study including kidney transplant patients diagnosed with aHUS. We collected data from 118 transplant centers in Brazil concerning aHUS transplanted patients between 01/01/2007 and 12/31/2019. Patients were stratified into three groups: no use of eculizumab (No Eculizumab Group), use of eculizumab for treatment of after transplantation TMA (Therapeutic Group), and use of eculizumab for prophylaxis of aHUS recurrence (Prophylactic Group). RESULTS: Thirty-eight patients with aHUS who received kidney transplantation were enrolled in the study. Patients' mean age was 30 years (24-40), and the majority of participants was women (63% of cases). In the No Eculizumab Group (n = 11), there was a 91% graft loss due to the TMA. The hazard ratio of TMA graft loss was 0.07 [0.01-0.55], p = 0.012 in the eculizumab Prophylactic Group and 0.04 [0.00-0.28], p = 0.002 in the eculizumab Therapeutic Group. CONCLUSION: The TMA graft loss in the absence of a specific complement-inhibitor was higher among the Brazilian cohort of kidney transplant patients. This finding reinforces the need of eculizumab use for treatment of aHUS kidney transplant patients. Cost optimization analysis and the early access to C5 inhibitors are suggested, especially in low-medium income countries.

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.

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.

Mudança de atitudes dos estudantes durante o curso de medicina: um estudo de coorte / Changes in medical students' attitudes during the medical course: a cohort study

INTRODUÇÃO: Devido ao possível potencial preditivo das atitudes apresentadas pelos estudantes durante o curso de graduação em Medicina, o diagnóstico e o acompanhamento de aspectos atitudinais necessários ao exercício profissional poderiam propiciar mudanças no processo de formação da identidade profissional para efetivação de uma prática médica renovada. MÉTODO: Estudo de coorte, descritivo e quantitativo, por meio da aplicação, em dois momentos, de uma escala de atitudes (tipo Likert com alpha de Cronbach = 0,87), em que se analisam cinco aspectos (ambiência, conhecimento, crença, ética e social) segundo as Diretrizes Curriculares Nacionais do Curso de Graduação em Medicina. Os sujeitos foram 202 estudantes de Medicina (52,85% mulheres e 47,15% homens), da primeira à oitava fase, com média de idade de 21,45 anos (Q inf: 21 e Q sup: 22,50); 91,50% provenientes da escola particular; 97,52% solteiros; de família de bom padrão de escolaridade e econômico; 91,54% da Região Sul, sendo 48,77% do próprio Estado; 86,63% não exerciam atividades extracurriculares; e 86,07% tinham como meta ingressar na residência médica após a graduação. RESULTADO: Identificados dois grupos, com provável homogeneidade de comportamento, com tendência atitudinal positiva, mais frequente no sexo feminino, demonstrando provável conflito na dimensão crença. CONCLUSÕES: Os estudantes apresentaram alguns aspectos atitudinais positivos relevantes para a prática médica, não havendo diferenças substanciais considerando-se as fases e o período estudado. Foram percebidos possíveis conflitos atitudinais quando analisados os itens de determinados aspectos, permitindo uma reflexão para possível associação com as questões educacionais e fornecendo subsídios para estudos futuros.

INTRODUCTION: During medical school, the students' values and attitudes are affected by knowledge, environment and training. Due to the possible predictive potential, __ the diagnoses and monitoring of the attitude aspects needed for the professional exercise could propitiate changes in the process of the professional identity formation for the implementation of a renewed medical practice. OBJECTIVES: To measure the attitudes and to evaluate the behavior changes during the graduation process. METHOD: A cohort study, descriptive and quantitative, in which it was utilized a questionnaire, that applied in two moments an attitude range (Miranda, 2006) analyzing five aspects: environment, knowledge, beliefs, ethics and society. The attitudes were categorized in positive, negative and conflicting. The subjects were 202 medical students (52.85% women and 47.15% men), from the first to the eighth periods, with a mean age of 21.45 years; 91.50% from private schools; 97.52% single; from good socio-cultural family standards; 91.54% from the South Region, being 48,77% from the State; 86.63% did not perform extracurricular activities; and 86.07% had as their goal to join the residency program after graduation. RESULT: Identified two groups, with a probable homogeneity of behavior, with positive attitudinal trend, more common in females, demonstrating a probable conflict in belief dimension. CONCLUSIONS: Students tended to positive attitudinal aspects___ relevant to the medical practice, and showed no substantial differences considering the phases and the periods studied. Were perceived attitudinal conflicts in certain areas, allowing for a reflection of a possible association with educational issues, and providing subsidies for future studies.

Construção de uma escala para avaliar atitudes de estudantes de medicina / The construction of a scale to measure medical students' attitudes

INTRODUÇÃO: Estudos têm mostrado que as atitudes influenciam o comportamento. O desenvolvimento e a manutenção de determinadas atitudes dos estudantes durante o curso médico podem influenciar a qualidade do cuidado médico dado aos pacientes no exercício da profissão. As Diretrizes Curriculares Nacionais do Curso de Graduação em Medicina reforçam a importância de aspectos atitudinais relevantes para a prática médica. OBJETIVO: Construção e validação de uma escala de atitude do tipo Likert, para avaliar atitudes de estudantes de Medicina considerando as Diretrizes Curriculares Nacionais, adaptadas à realidade sociocultural brasileira. MÉTODO: Na construção da escala foram considerados cinco aspectos: social, ambiência, crença, conhecimento e ética. Os sujeitos foram 202 estudantes do curso de graduação em Medicina, da primeira à oitava fase, sendo 52,85% mulheres e 47,15% homens. Estudo de coorte, descritivo e quantitativo. Os dados foram coletados por meio da aplicação de um questionário e de uma escala de atitudes (do tipo Likert) com 102 proposições. A escala foi aplicada em dois momentos. RESULTADOS: A confiabilidade da escala, medida pelo coeficiente α, de Cronbach, foi igual a 0,87. A escala foi validada (validade de construto) por cinco especialistas. Não houve diferença estatisticamente significante entre as oito fases analisadas. As atitudes observadas foram predominantemente positivas. CONCLUSÃO: O trabalho desenvolvido resultou na definição de um instrumento válido e confiável para medir as atitudes de estudantes de Medicina segundo as dimensões social, ambiência, crença, conhecimento e ética.

INTRODUCTION: Studies have shown that attitudes influence behavior. Developing and maintaining certain attitudes can influence the quality of health care delivered by medical students to their patients. The Brazilian National Guidelines for Medical Education emphasize the significance of the students' attitudes relevant for the medical practice. OBJECTIVES: This study aimed designing and validating an attitude scale to evaluate the impact of the medical curriculum on students' attitudes. METHODOLOGY: The subjects were 202 students (female = 52.85%, male = 47.15%), attending from the first to the eighth phase of the medical undergraduate course. The data for this descriptive and quantitative cohort study were collected using a questionnaire and an attitude measurement scale (5-point Likert scale) containing 102 propositions covering five different factors (social, environmental, belief, knowledge and ethics). RESULTS: Several analyses were undertaken to establish the internal validity of the instrument, including reliability (total = 0.87 and 0.89) and correlation studies. There were no statistically significant differences among the eight analyzed phases. The students showed predominantly positive attitudes. CONCLUSIONS: The final version of the scale was therefore regarded valid and reliable for the assessment of medical students' attitudes.