ABSTRACT Introduction: Elderly patients are the most frequent patients admitted to dialysis units. Relatively little is known about standards on endof-life (EOL) care among dialysis patients. From international literature, these patients are exposed to more aggressive treatments at EOL than patients with cancer or other chronic diseases and palliative referral is infrequent. This study aims to describe some end-of-life (EOL) practices in patients undergoing hemodialysis in NephroCare clinics in several cities in Portugal. Material and Methods: We designed a prospective, multicenter, observational, cross-sectional study conducted between October 1st, 2020 and September 30th, 2021 in six hemodialysis clinics including 1265 patients (pts). Results: During the year in analysis, 158 pts died (12.4%). Mean age was 76 years (25% of pts were older than 85 years) and dialysis vintage was 84 (2-963) months. Regarding comorbidities, 50% were diabetic, 40% had congestive heart failure, 25% had cancer and 17% had dementia. Mean age-adapted Charlson index was 13 ± 3. For 82 pts (51.9%), nephrologists would not be surprised if they died in the next 6 months. Seventy-three had hospital admissions in the previous three months. Four pts withdrew dialysis. Of those who died, only two had advance care directives, 18 had an opioid prescription in the last month of life and 17 were referred to palliative intervention mostly because of oncologic disorders. Seventy four percent of these pts died at the hospital and 23% died at home/nursing home. Not a single patient died under hospice care. Conclusion: Most of our patients commonly die in hospital, undergo invasive procedures, spend much of their remaining life fulfilling a demanding dialysis schedule and are admitted to the hospital wards without formal palliative intervention or discussion of goals of care, even when death is an expected outcome.

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.

Chronic kidney disease (CKD) is a burdensome disease that has been successfully managed with dialysis. But even if dialysis can prolong patients' survival in certain circumstances, it also brings a high burden of physical and psychosocial symptoms, poor outcomes, and high costs of care. Fragile patients may not benefit from starting dialysis. In this way, Palliative Care (PC) should be integrated into CKD care to control symptoms' burden, to discuss the shared decision to start or forgo dialysis when stage 5 CKD approaches, or to select conservative management of end-stage renal disease (ESRD) or withdrawal dialysis. As patients attended in private hemodialysis units get older and more fragile, incorporation of PC is associated with the highest quality care standards provided to our ESRD patients. That being so, we designed a project based on the World Health Organization (WHO) recommendations to implement palliative interventions in NephroCare Hemodialysis Units. In our project, we consider the following five steps: (i) assessment of palliative needs in dialysis patients; (ii) team education; (iii) contact and referral to specialized teams; (iv) registration (v) and trial of the pilot project. We also reflect on the barriers to implementation of this project. This article aims to raise awareness of the project and use it as a model or a first step to other projects in the actual area to be implemented in other health units.

Resumo O distúrbio mineral e ósseo é uma característica comum da doença renal crônica. A síndrome da face leonina é uma complicação rara do hiperparatireoidismo grave em pacientes com doença renal terminal, que tem sido menos relatada devido aos avanços na diálise e tratamento médico na última década. O reconhecimento precoce da deformidade facial característica é crucial para estimular o tratamento precoce e prevenir a desfiguração severa. Os autores apresentam um caso raro de hiperparatireoidismo grave, apresentando síndrome da face leonina e fraturas ósseas.

Abstract Mineral bone disorder is a common feature of chronic kidney disease. Lion face syndrome is rare complication of severe hyperparathyroidism in end-stage renal disease patients, which has been less commonly reported due to dialysis and medical treatment advances in the last decade. The early recognition of the characteristic facial deformity is crucial to prompt management and prevent severe disfigurement. The authors present a rare case of severe hyperparathyroidism presenting with lion face syndrome and bone fractures.

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

O micofenolato de mofetil (MMF) genérico é uma alternativa mais barata e possivelmente tão eficaz quanto a molécula original na imunossupressão de manutenção em receptores de transplantes de órgãos sólidos, nomeadamente transplante renal. Para ser aprovada, cada nova formulação genérica tem que demonstrar bioequivalência ao fármaco original em voluntários saudáveis. No entanto, a farmacocinética do MMF não é necessariamente igual em voluntários saudáveis e receptores de transplantes. Perante a imposição da conversão dos doentes para MMF genérico os autores avaliaram e compararam exposição ao ácido micofenólico (AMF) com o fármaco original e com o genérico em 14 doentes convertidos ao genérico na sua unidade de transplante renal. A exposição ao AMF, avaliada pela AUC0-12h estimada a partir de 3 determinações séricas em tempos pré-definidos, foi ligeiramente mais elevada com a formulação genérica (40.49 versus 34.96mcg.h/mL, p = 0,041). Não se verificou variação significativa da TFGe, hemoglobina nem contagem de leucócitos após a conversão para o fármaco genérico. Apenas um dos doentes convertidos para MMF genérico desenvolveu efeitos adversos do foro gastrointestinal de novo que resolveram após reintrodução da molécula original. Em conclusão, na nossa amostra não se verificou variação clinicamente significativa na exposição ao AMF, função do enxerto renal nem na incidência de efeitos adversos com a formulação genérica, a curto prazo

Generic mycophenolate mofetil (MMF) is a cheaper and possibly equally effective option as the original molecule for maintenance immunosuppression in solid organ transplant recipients. In order to be approved, each generic formulation has to prove bioequivalence to the reference product in healthy volunteers. However, MMF pharmacokinetics may be different between healthy volunteers and transplant recipients. Faced with the introduction of the generic formulation in the pharmacy of their kidney transplant unit, the authors studied and compared the exposure to mycophenolic acid (MPA) before and after the switch in 14 kidney transplant recipients converted to the generic molecule. The exposure to MPA as evaluated by the AUC0-12h estimated by a limited sample strategy was slightly higher with the generic formulation (40.49 versus 34.96mcg.h/mL, p = 0.041). There was no significant variation in the eGFR, haemoglobin and leucocyte count after the switch. Only one of the patients converted to the generic formulation developed de novo adverse gastrointestinal effects, which resolved after restart of the original formulation. In conclusion, in our cohort there was no clinically significant variation in the exposure to MPA, kidney graft function or incidence of adverse events with the generic formulation on a short follow-up period

Histoplasmosis is a clinically relevant fungal infection in immunosuppressed patients, in whom it may present as a serious disease. We describe the case of a renal transplant recipient who presented with malaise, dry cough, severe weight loss, abdominal pain and watery diarrhoea. The laboratory workup revealed anaemia and elevated C reactive protein. Chest X-ray showed bilateral reticulonodular infiltrates, and computed tomography scan of the chest and abdomen revealed diffuse thickening of the inter- and intralobular septs, suggestive of lymphangitic infiltration. The abdomen had multiple lymphadenopathies along the mesenteric vessels. Endoscopic evaluation disclosed disseminated ulcers throughout the entire gastrointestinal tract, and biopsies confirmed the diagnosis of histoplasmosis. Despite adequate treatment with liposomal amphotericin B, the patient had a fatal outcome. We describe this case because of the rarity of gastrointestinal presentation in the context of disseminated histoplasmosis, and we also speculate about the transmission through the allograft. It highlights the importance of considering this once geographically limited disease in challenging cases, even in non-endemic areas