ABSTRACT Introduction: Dysproteinemia is associated with kidney damage and treatment of underlying conditions is crucial to minimize kidney damage. The aim of this study was to describe and analyze the characteristics and outcome of the patients diagnosed with dysproteinemia-associated kidney disease with initial renal presentation. Methods: We performed a retrospective observational study of the patients admitted with initial dysproteinemia-associated kidney disease in the Department of Nephrology and Renal Transplantation at Centro Hospitalar Universitário Lisboa Norte in Lisbon over five years. Results: Thirty patients were diagnosed with dysproteinemia-associated kidney disease: twenty-six (86.7%) with multiple myeloma, one of these with concomitant amyloidosis; three (10.0%) with gammopathy of renal significance and one (3.0%) with Waldenström macroglobulinemia. Mean age was 71.5 (± 12.44) years and half patients were male. Anemia, hypercalcemia, hyperphosphatemia, and hypoalbuminemia was found in twenty-three (76.7%), ten (30.0%), fourteen (46.7%) and seventeen (56.7%) patients, respectively. Bence Jones proteinuria was found in twenty-one (70.0%) patients and thirteen (43.3%) patients presented with creatinine >6 mg/dL. Twelve (40.0%) patients were submitted to kidney biopsy and light chain cast nephropathy was the most common finding. Twenty-nine (96.7%) patients were treated with chemotherapy. Twenty-two (72.3%) patients required renal replacement therapy and six of these (27.3%) patients were submitted to high cut-off dialyzers. Four (13.3%) patients had full recovery of renal function, sixteen (53.3%) remained dialysis-dependent at hospital discharge. At one-year follow-up, eleven (36.7%) patients remained dialysis-dependent and six (20.0%) had died. Conclusion: Dysproteinemia-associated kidney disease can present heterogeneously, and diagnostic suspicion is crucial to start prompt directed therapy to improve kidney and overall outcomes. Renal replacement therapy requirement and dependence at discharge appear to be related to higher mortality in these patients. In the future, routine screening of dysproteinemia may be particularly relevant in older patients to prevent irreversible kidney damage.

Chronic kidney disease (CKD) is increasing worldwide and studies estimate that around 6% of women of childbearing age suffer from kidney disease. Preconception counselling of women with CKD and their management during pregnancy requires a multidisciplinary team, with both nephrologists and obstetricians experienced in advising and taking care of women across the CKD spectrum, including dialysis patients and women with a kidney transplant. Here, the authors describe a model of a Nephro-Obstetric outpatient clinic, created in 2011, detailing their current clinical experience in the management of this complex population of women.

Chronic kidney disease (CKD) is associated to significant infection incidence and severity, the cause of death of, approximately, 20% of end-stage renal disease patients. Close to 80% of all infections in these patients can be related to organ or tissue other than vascular access, reflecting immune system dysfunction related to several mechanisms. Strongyloides stercoralis is an endemic nematode found especially in tropical and subtropical areas. The clinical manifestations of stercoralis infection vary according to the acuity of infection and the host response, from asymptomatic to the disseminated presentation, frequently lethal. Risk factors for severe presentations are related to immunosuppression states. The authors report a case of a patient with advanced CKD of unknown etiology, who developed an exuberant eczema, respiratory symptoms and severe hypereosinophilia. The etiological study revealed a disseminated form of Strongyloides stercoralis infection with myocardia involvement, diagnosed solely by serologic testing, despite microbiological tests to identify the infective agent. In this case, the patient has evolved favorably, with remission of symptoms and cardiac features, after adequate anti-helminthic treatment. The importance of parasitic colonization and its potential harm becomes clinically more relevant when patients become immunosuppressed, as in CKD progression or in the immunosuppressive therapy setting, such as solid organ transplant rejection therapy. A brief literature review related to strongyloidiasis and immunosuppression in the CKD setting is also presented

Resumo Introdução: A colocação de cateteres intra-auriculares (IAC) tem surgido como uma técnica life-saving nos doentes em hemodiálise (HD) com exaustão de acessos vasculares. Objetivo: Analisar as complicações decorrentes da colocação de IAC, assim como a sobrevivência dos doentes e do acessos vascular após este procedimento. Métodos: Os autores analisaram retrospetivamente sete doentes com exaustão de acessos vasculares para HD, sem alternativa imediata de terapêutica substitutiva renal, submetidos a colocação de IAC entre Janeiro de 2004 e Dezembro de 2015. Resultados: Os sete doentes foram submetidos à colocação de doze IAC. A hemorragia (6/7) e as infeções (3/7) foram as principais complicações no pós-operatório imediato. Dois (2/7, 29%) doentes faleceram por complicações precoces e 5/7 tiveram alta com cateter funcionante. A complicação tardia mais frequente foi a exteriorização acidental do cateter em todos os doentes, seguida da trombose e infeção relacionada com o cateter, na mesma proporção (2/5). Durante o seguimento, dois dos cinco doentes faleceram por complicações associadas com o acesso vascular. Após a falência do IAC, um doente foi transferido para diálise peritoneal e outro foi submetido a transplantação renal. Apenas um doente permanece em HD após o terceiro IAC, com uma sobrevivência de 50 meses. A sobrevivência média dos doentes após colocação de IAC foi de 19 ± 25 (0-60) meses e a patência média do IAC foi de 8 ± 11 (0-34) meses. Conclusão: A colocação de um IAC para HD esteve associado a riscos significativos e mortalidade elevada. Contudo, quando as terapêuticas de substituição renal alternativas estão esgotadas, ou como uma ponte para outras modalidades, esta opção deve ser considerada.

Abstract Introduction: Intra-atrial catheter (IAC) placement through an open surgical approach has emerged as a life-saving technique in hemodialysis (HD) patients with vascular access exhaustion. Objective: To assess the complications of IAC placement, as well as patient and vascular access survival after this procedure. Methods: The authors retrospectively analyzed all seven patients with vascular capital exhaustion, without immediate alternative renal replacement therapy (RRT), who underwent IAC placement between January 2004 and December 2015 at a single center. Results: Seven patients were submitted to twelve IAC placements. Bleeding (6/7) and infections (3/7) were the main complications in the early postoperative period. Two (2/7, 29%) patients died from early complications and 5/7 were discharged with a properly functioning IAC. The most frequent late complication was catheter accidental dislodgement in all remaining five patients, followed by catheter thrombosis and catheter-related infections in the same proportion (2/5). During follow-up, two of five patients died from vascular accesses complications. After IAC failure, one patient was transferred to peritoneal dialysis and a kidney transplant was performed in the other. Only one patient remains on HD after the third IAC, with a survival of 50 months. The mean patient survival after IAC placement was 19 ± 25 (0-60) months and the mean IAC patency was 8 ± 11 (0-34) months. Conclusion: Placing an IAC to perform HD is associated to significant risks and high mortality. However, when alternative RRT are exhausted, or as a bridge to others modalities, this option should be considered.

The increasing prevalence of end-stage chronic kidney disease associated with advances in treatment is expected to lead to improved survival rates of chronic haemodialysis patients in Portugal. Establishing and maintaining vascular access patency is becoming a challenging issue in these patients. We present a case report of a multiple vascular access failure patient to whom a novel vascular access device was implanted which allowed successful haemodialysis, despite the presence of central venous stenosis and/or occlusion. To our knowledge the present case report describes the first utilization of Hemodialysis Reliable Outflow (HeRO) Graft device in Portugal. We present the case as well as a brief description of the device and available literature. This case report also underlines the importance of adopting a multidisciplinary approach in order to overcome complex and life-threatening clinical challenges