ABSTRACT Urinary tract infection is an important cause of hospitalization, morbidity and mortality, being responsible for an important fraction of health expenses. The main risk factors are urine flow obstruction, urinary catheters, immunosuppression and diabetes mellitus. We present a case of pyelonephritis that culminated in nephrectomy, finally unravelling an unexpected diagnosis. A 52-year-old male presented in the emergency department with two weeks of pain in the left iliac fossa that irradiated to the lumbar region and cloudy urine. Lab work revealed severe anaemia (4.9 g/dL), inflammatory markers elevation, leucoerythrocyturia, serum creatinine of 4.33 mg/dL and metabolic acidosis. Computed tomography scan showed a swollen left kidney with a staghorn calculus and intraparenchymal hypodensities. After a failed trial of large spectrum antibiotics, he was submitted to left radical nephrectomy with significant clinical improvement. Gross examination showed a markedly dilated pyelocalicial tree, filled with several staghorn calculi and purulent secretions with associated parenchymal atrophy. Histopathologic evaluation revealed exuberant chronic pyelonephritis with an acute inflammatory infiltrate that extended into the perinephric fat and multiple Schistosoma ova, predominantly calcified. The authors describe the case of a severe pyelonephritis with exuberant clinical and imagiological features and rare microbiological findings, suggesting previous genitourinary Schistosomiasis that predisposed to nephrolithiasis and chronic pyelonephritis.

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.

Resumo Introdução: O uso de Rituximab (RTX) em doenças glomerulares (DG) aumentou nos últimos anos, embora ainda utilizado apenas em uma pequena fração de pacientes. Métodos: Conduzimos em nosso centro, de 2010-2020, um estudo retrospectivo de único centro de pacientes adultos com nefropatia membranosa (NM), glomeruloesclerose segmentar focal (GESF), nefrite lúpica (NL) e vasculite tratada com RTX como terapia de primeira ou segunda linha. Resultados: Identificamos 19 pacientes; 36,8% tinham NM; 25,0% cada apresentava GESF, NL e vasculite. RTX foi terapia de primeira linha em 26,3% dos pacientes e em 73,7% foi terapia de segunda linha. O tempo médio de acompanhamento foi 7,7 ± 7,2 anos. Em NM, 2 pacientes (28,6%) tiveram remissão completa (RC), 2 pacientes (28,6%) remissão parcial (RP), e 3 pacientes (42,9%) não tiveram resposta (NR). Na GESF, 2 pacientes (50,0%) apresentaram RC, 1 paciente (25,0%) não teve resposta e, 1 paciente, deterioração renal. Dois pacientes (50,0%) apresentaram NL classe IV com RC após RTX, 1 paciente com NL classe IIIC/V não teve resposta, e 1 paciente com NL classe II apresentou deterioração renal. Na vasculite, 3 pacientes (75,0%) apresentaram RC e 1 paciente RP. Reações à infusão ocorreram em 2 pacientes (10,5%) e um paciente apresentou múltiplas complicações infecciosas. Conclusões: A eficácia do RTX em tratar diferentes tipos de DG imunomediada tem sido demonstrada com diferentes taxas de resposta, mas com perfil geral seguro. Em nossa série de casos, os resultados também são encorajadores. Estudos longitudinais são necessários para compreender melhor o efeito do RTX na DG.

Abstract Introduction: The use of Rituximab (RTX) in glomerular diseases (GD) has increased in the past years, although it is still only used in a small fraction of patients. Methods: A single center retrospective study of adult patients with membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), lupus nephritis (LN), and vasculitis treated with RTX as first or second-line therapy was conducted at our center from 2010 to 2020. Results: We identified 19 patients; 36.8% had MN and 25.0% each had FSGS, LN, and vasculitis. RTX was first-line therapy in 26.3% of patients and in 73.7% it was second-line therapy. Mean follow-up time was 7.7 ± 7.2 years. In MN, 2 patients (28.6%) had complete remission (CR), 2 patients (28.6%) had partial remission (PR), and 3 patients (42.9%) had no response (NR). In FSGS, 2 patients (50.0%) presented CR, 1 patient (25.0%) had no response, and 1 patient had renal deterioration. Two patients (50.0%) had a LN class IV with a CR after RTX, 1 patient with LN class IIIC/V had no response, and 1 patient with LN class II had renal deterioration. In vasculitis, 3 patients (75.0%) presented CR and 1 patient had PR. Infusion reactions were present in 2 patients (10.5%) and one patient had multiple infectious complications. Conclusions: The efficacy of RTX in treating different types of immune-mediated GD has been demonstrated with different response rates, but an overall safe profile. In our case series, the results are also encouraging. Longitudinal studies are needed to better understand the effect of RTX in GD.

Vascular access (VA) dysfunction is an important cause of morbidity and mortality in the hemodialysis (HD) population. Regardless of the increasing prevalence of HD patients with complex VA, the definitions of VA failure (VAF) or end-stage VA failure (ES-VAF) are still being discussed and there is no current guideline that provides recommendation for solving the problem of complicated vascular access. The aim of this study was to review the management approach for resuming the continuity of RRT in patients with problematic vascular accesses, based on our center experience. In a retrospective analysis of 30 patients with VAF or ES-VAF over an 11-year period, twenty patients were transferred to peritoneal dialysis (PD), seven patients remained in HD after the placement of a non-standard site VA and three patients had a renal transplant (RT). In half of these patients, the initial therapeutic option eventually failed, leading to multiple reinterventions and RRT changes. Mean survival was 47 ± 32.7 months. Ten patients died, the majority within the first year of VAF diagnosis. The management of end-stage renal disease patients includes vessel preservation, the combination of RRTs to extend life expectancy and VA care to maintain long-term function. Our approach stratifies patients according to exhausted VA sites in order to plan for RT, transition to PD or alternative VA site management. This allows for better management of the VAF patient and highlights the need to individualize patient care.

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.

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in Caucasian adults aged over 60 years old. Approximately two thirds of cases of membranous nephropathy (MN) in adults are primary (idiopathic), and the remaining are secondary forms which have been attributed to a variety of agents or conditions. Recent advances in pathophysiology and the availability of new laboratory diagnostic methods reinforce the importance of differentiating idiopathic from secondary MN, given the repercussions on therapy. We present the case of a 71-year-old Caucasian male referred to Nephrology due to nephrotic syndrome. He had a history of gastric adenocarcinoma and of parathyroid cancer, but was considered cured. Investigation excluded immunologic disease, dysproteinaemia, chronic infection and active malignancy. Kidney biopsy was performed and confirmed MN. Histological characterization of IgG subtypes was not available, nor was biopsy study for the reactivity for PLA2 receptor. The patient was started on conservative treatment, but, after a one-year follow-up, presented persistent nephrotic proteinuria (5g/day) and renal function decline (serum creatinine of 1.6mg/dL). Serological determination of phospholipase A2 receptor antibody was positive, but with borderline titer. Considering his history of malignancy as well as kidney function deterioration, Rituximab was prescribed. At one year of follow-up the patient has proteinuria of 500 mg/day, stable renal function (serum creatinine of 1.2 mg/dL) and negative serum anti-PLA2 receptor antibody. Despite the recent availability of serological determination of phospholipase A2 receptor antibody and histological characterization of IgG subtypes, in this case they did not allow us to safely distinguish between idiopathic and secondary MN. Therefore the past medical history played a significant part in the therapeutic option. Recent studies suggest that Rituximab has similar results to non-specific immunosuppression, promoting remission in patients with severe and persistent MN, with less adverse effects, and thus can be considered as first-line therapy

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

Os problemas de acesso vasculares sao uma causa importante de transferencia obrigatoria para dialise peritoneal. Surpreendentemente, pouco e conhecido sobre os efeitos destas transferencias na adequação da dialise peritoneal, sobrevida do paciente ou da tecnica. Analisamos retrospetivamente um grupo de 75 pacientes tratados na Unidade de Dialise Peritoneal do Centro Hospitalar Lisboa Norte - Hospital de Santa Maria no ano 2011. Os ‘problemas de acesso vascular’ foram definidos clinicamente. As caracteristicas e sobrevida dos pacientes foi comparada entre os doentes com (grupo 1, n = 14) e sem problemas de acesso vascular (grupo 2, n = 61). No grupo 1 havia um numero maior de doentes de raca negra e transferidos de hemodialise, com um tempo dispendido nesta tecnica consideravelmente superior. Estes doentes eram mais propensos a anuria, menor remocao total de fluidos/dia, menor depuracao de creatinina renal e total, e inferior Kt/V da ureia. A depuracao de creatinina peritoneal e o tipo de transportador de membrana peritoneal nao divergiu entre os grupos. As taxas de infeccao do orificio de saida e peritonite foram superiores no grupo 1, a albuminemia era menor nestes doentes. Nao se verificaram diferencas na sobrevida dos doentes ou tecnica (metodo >Kaplan-Meier). No modelo de regressao >Cox, apenas valores superiores de depuracao de creatinina total influenciaram de forma significativa e positiva a sobrevida da tecnica. Em conclusao, na Unidade a prevalencia de problemas de acesso vascular foi 18.7%, e isso justificou 78.6% das transferencias da Unidade de Hemodialise. Estes nao seriam os doentes ideais para dialise peritoneal. No entanto, os dados sugerem que a sobrevida (paciente e tecnica) de doentes com transferência obrigatoria para dialise peritoneal por problemas de acesso vascular e similar a de doentes sem problemas de acesso vascular. A depuracao de creatinina total surgiu como fator protetor independente da sobrevida da tecnica

Vascular access problems are an important cause of compulsory transfer to peritoneal dialysis. Surprisingly, little is known about the effect of these transfers on peritoneal dialysis adequacy, patient or technique survival. We have analysed retrospectively a cohort of 75 patients treated at the Peritoneal Dialysis Unit of Centro Hospitalar Lisboa Norte - Hospital de Santa Maria during the year 2011. ‘Vascular access problems’ were defined clinically. Patient characteristics, peritoneal dialysis features and survival were compared between patients with (group 1, n = 14) and without vascular access problems (group 2, n = 61). In group 1, significantly more patients were Black and had been transferred from haemodialysis, with a considerably longer time spent on this technique. These patients were more likely to be anuric, with an inferior daily total fluid removal, lower renal and total creatinine clearance, and poorer Kt/V for urea. Peritoneal clearance and peritoneal membrane transport type did not differ between groups. Group 1 had a considerably higher exit site infection and peritonitis rate, and a lower albumin level. No significant differences were observed in unadjusted patient or technique survival between the two study groups. In the Cox multiple regression model, only a higher total creatinine clearance significantly and positively influenced the technique survival. In conclusion, the prevalence of vascular access problems of the Peritoneal Dialysis Unit was 18.7% and it justified 78.6% of transfers from the Haemodialysis Unit. These were not the ideal patients for peritoneal dialysis. Nonetheless, our data suggest that the outcome (patient and technique survival) of patients with mandatory transfer to peritoneal dialysis because of vascular access problems is similar to that achieved in patients without vascular access problems. Total creatinine clearance appeared as an independent protective factor of technique survival