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.

Comparison of trisodium citrate and heparin as catheter-locking solution in hemodialysis patients.

INTRODUCTION: The high incidence of patients beginning dialysis treatment with venous catheters, as well as the growing number of patients with permanent catheter access, has increased catheter- related blood infections and their consequences. Thus the search for substances with anticoagulant properties that also prevent catheter contamination is necessary. OBJECTIVE: This study aimed at comparing heparin and trisodium citrate used as long-term catheter locking solutions regarding the occurrence of pyrogenic reaction, bacteremia, infection- related hospitalizations, thrombosis, and death. METHODS: Retrospective study on the infection data from the Infection and Adverse Event Prevention Control Program registry, which included all hemodialysis patients using long-term catheters from April, 2006 to March, 2008. During the first 365 days, catheters were locked with heparin (Heparin group) and, during the following 365 days, with 46.7% trisodium citrate (Citrate group). Sixty-five patients were included in the study using 92 catheters. The groups were compared regarding the occurrence of pyrogenic reaction, bacteremia, hospitalization, catheter thrombosis, and death. RESULTS: The catheter-related bacteremia episodes were significantly lower and hospitalization time was significantly shorter in the Citrate group when compared with those in the Heparin group. A tendency towards a lower occurrence of access site infection-related hospitalization was observed in the Citrate group (p = 0.055), and no difference was observed in catheter thrombosis leading to dysfunction between groups. Bacteremia-free and hospitalization-free times were longer in the Citrate group. The occurrence of bacteremia was associated with the presence of diabetes and heparin use. In multivariate analysis, being in the Heparin group was the only factor associated with bacteremia. CONCLUSION: The use of 46.7% citrate solution effectively reduced bacteremia episodes and hospitalization in chronic kidney disease patients on hemodialysis with long-term catheters.

Comparação entre citrato trissódico e heparina como solução para selo de cateter em pacientes em hemodiálise / Comparison of trisodium citrate and heparin as catheter-locking solution in hemodialysis patients

INTRODUÇÃO: A elevada incidência de pacientes iniciando ou sendo mantidos em tratamento dialítico através de cateteres venosos tem aumentado o número de infecções da corrente sanguínea relacionadas aos cateteres e suas consequências, obrigando a busca de substâncias que tenham propriedades anticoagulantes e que também impeçam a contaminação dos mesmos. OBJETIVOS: Comparar a utilização de heparina e citrato trissódico como selo de cateteres de longa permanência quanto à ocorrência de pirogenia, bacteremia, internações hospitalares relacionadas à infecção, trombose e óbito. MÉTODOS: Estudo retrospectivo por meio de dados do registro de infecção do Programa de Controle e Prevenção de Infecções e Eventos Adversos, onde foram incluídos todos os pacientes que entre abril de 2006 e março de 2008 utilizaram cateteres de longa permanência. Nos primeiros 365 dias, o selo do cateter foi feito com heparina (Grupo Heparina) e nos 365 dias seguintes foi feito com citrato trissódico a 46,7 por cento (Grupo Citrato). Sessenta e cinco pacientes fizeram parte do estudo utilizando noventa e dois cateteres. Os grupos foram comparados para ocorrência de pirogenia, bacteremia, hospitalização, trombose de cateter e óbito. RESULTADOS: A ocorrência de bacteremia relacionada ao cateter e o tempo de hospitalização foram significantemente menores no Grupo Citrato. Houve uma tendência a menor ocorrência de hospitalização relacionada à infecção de acesso no Grupo Citrato (p = 0.055), e não houve diferença quanto à trombose de cateteres levando a disfunção. O tempo livre de bacteremia e de hospitalização foram maiores no Grupo Citrato. A ocorrência de bacteremia esteve associada a ser diabético e a pertencer ao Grupo Heparina. Na análise multivariada, apenas pertencer ao Grupo Heparina esteve associada à sua ocorrência.

INTRODUCTION: The high incidence of patients beginning dialysis treatment with venous catheters, as well as the growing number of patients with permanent catheter access, has increased catheter- related blood infections and their consequences. Thus the search for substances with anticoagulant properties that also prevent catheter contamination is necessary. OBJECTIVE: This study aimed at comparing heparin and trisodium citrate used as long-term catheter locking solutions regarding the occurrence of pyrogenic reaction, bacteremia, infection- related hospitalizations, thrombosis, and death. METHODS: Retrospective study on the infection data from the Infection and Adverse Event Prevention Control Program registry, which included all hemodialysis patients using long-term catheters from April, 2006 to March, 2008. During the first 365 days, catheters were locked with heparin (Heparin group) and, during the following 365 days, with 46.7 percent trisodium citrate (Citrate group). Sixty-five patients were included in the study using 92 catheters. The groups were compared regarding the occurrence of pyrogenic reaction, bacteremia, hospitalization, catheter thrombosis, and death. RESULTS: The catheter-related bacteremia episodes were significantly lower and hospitalization time was significantly shorter in the Citrate group when compared with those in the Heparin group. A tendency towards a lower occurrence of access site infection-related hospitalization was observed in the Citrate group (p = 0.055), and no difference was observed in catheter thrombosis leading to dysfunction between groups. Bacteremia-free and hospitalization-free times were longer in the Citrate group. The occurrence of bacteremia was associated with the presence of diabetes and heparin use. In multivariate analysis, being in the Heparin group was the only factor associated with bacteremia.

Avaliaçäo e terapêutica nutricional com auxílio de microcomputador / Nutritional evaluation and therapeutics with microcomputers support

Um programa para microcomputadores foi escrito com o intuito de auxiliar médicos a realizar avaliaçäo nutricional e planejar um suporte nutricional adequado bem como realizar avaliaçäo desta terapêutica. É apresentada uma descriçäo das partes que compöem o programa e a seqüência em que o mesmo é processado. Nenhum conhecimento prévio de computaçäo ou operaçäo em microcomputadores é exigido do usuário