Scielo RSS http://scielo.pt/rss.php?pid=0872-016920220002&lang=pt vol. 36 num. 2 lang. pt http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200071&lng=pt&nrm=iso&tlng=pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200077&lng=pt&nrm=iso&tlng=pt ABSTRACT Renal cortical necrosis (RCN) is a rare cause of acute kidney injury (AKI) in developed countries. Drugs, especially non-steroidal antiinflammatory drugs, (NSAIDs) are very rarely described in the literature to cause cortical necrosis. It is characterized by confluent necrosis of the entire cortex apart from a thin rim of viable tissue in the subcapsular, juxtaglomerular areas, and medulla. Although the pathogenesis of the disease remains unclear, the final common pathway is permanent occlusion of afferent arterioles and interlobular arteries in the cortical vasculature. NSAIDs are widely prescribed in general clinical practice. Despite being readily available, a subset of individuals is susceptible to serious renal toxicity and caution should be exercised when these drugs are used. We present the case of a young adult who presented with renal cortical necrosis with irreversible renal failure. Unfortunately, it occurred as a result of inappropriate use of over-the-counter NSAIDs in the setting of the pandemic outbreak of COVID-19. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200080&lng=pt&nrm=iso&tlng=pt ABSTRACT Hemolytic uremic syndrome (HUS) triggered by influenza virus (iHUS) is rare. Influenza A infections have been described to trigger atypical HUS (aHUS) in individuals with an underlying genetic complement dysregulation. To date there are only few reports of Influenza B as a trigger of aHUS, all identified cases associated with mutations in the MCP or C3 gene, occasionally combined with other mutations. aHUS patients should be screened for all known disease-associated genes and screening should not be stopped after finding a mutation, to identify other genetic susceptibility factors influencing gene phenotype, particularly in patients with MCP or CFI mutations. Complement blockade using a monoclonal anti-C5 antibody, eculizumab, has greatly improved the outcome in recent years for certain groups of HUS. The decision on whether to treat or not with eculizumab should be made based on clinical and laboratorial evolution as well as molecular studies results. Influenza A and B are preventable through vaccination and strategies should be addressed for patients with complemente gene mutations identified. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200084&lng=pt&nrm=iso&tlng=pt ABSTRACT Membranous nephropathy may present in any age group. While it is one of the most common causes of adult nephrotic syndrome, it is a rare histologic entity in the paediatric population. In adults it is more commonly classified as primary or idiopathic. In contrast, among children and adolescents, it is more frequently secondary to systemic disorders or drug exposures. One of the potentially implicated drugs is penicillamine, which has long been used as first-line therapy for symptomatic children with Wilson’s disease. We report a case of an adolescente treated with penicillamine for Wilson’s disease, who presented with overt nephrotic syndrome due to membranous nephropathy. As most of our knowledge about children and adolescents with membranous nephropathy has been based on extrapolations from adult studies, paediatric case reports may provide useful insights. We also aimed to emphasize the importance of proteinuria close monitoring in patients under treatment with penicillamine to allow for timely consideration of the drug withdrawal. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200089&lng=pt&nrm=iso&tlng=pt ABSTRACT Antineutrophil cytoplasmic antibodies (ANCA) are useful as markers for systemic vasculitis. PR3-ANCA antibodies have been also identified in association with chronic inflammatory and infectious conditions, other autoimmune diseases, malignancy, and certain drugs. The association of PR3 and minimal change disease (MCD) without vasculitis is not an expected finding. We report a case of MCD with PR3-ANCA positive title and no histopathological findings of vasculitis. This reports to an 86-year-old Caucasian woman without relevant past medical history and a normal renal function one month before presentation with sudden-onset nephrotic syndrome and rapidly progressive renal failure. Renal ultrasound was normal. Autoimmune screening using enzyme-linked immunosorbent assay (ELISA) revealed PR3-ANCA high titers (238.7UQ). Renal biopsy did not show vasculitis or crescentic glomerulonephritis. The glomeruli were normal. Autoimmune, inflammatory, infectious and malignant diseases were excluded. MCD was assumed and she started prednisolone with clinical and analytical improvement. ANCA-associated glomerular disease can coexist with a variety of other glomerular diseases including membranous nephropathy, lupus nephritis, IgA nephropathy, and bacterial infection-related glomerulonephritis. This association could be an incidental finding, but there may be an association with MCD not previously reported. Our case emphasizes the importance of performing renal biopsies before embarking on a full-scale immunosuppression therapy based on ANCA title alone. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200093&lng=pt&nrm=iso&tlng=pt ABSTRACT Older to older living kidney transplantation is an acceptable alternative to avoid dialysis, improve quality of life and maintain favorable long-term outcomes of patients with end-stage kidney disease. However, the management of these patients is complex and the acceptance of older donors and recipients to kidney transplantation remains controversial. The older recipients usually have several comorbidities that may complicate the postoperative course and increase the risk of end-stage kidney disease. We present a clinical case of living kidney transplantation between an older donor and recipient, both over 70 years old, who evolved to a stage 3 chronic kidney disease, without proteinuria, hypertension, or other complications, three years after transplantation. Our case reinforces favorable outcomes in older living transplantation, both for recipient and donor, as has been demonstrated in several published studies. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200101&lng=pt&nrm=iso&tlng=pt 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. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000200115&lng=pt&nrm=iso&tlng=pt ABSTRACT Fluid status is crucial for dialysis patients. Volume overload is a major cardiovascular risk factor. Nevertheless, volume depletion is associated with myocardial stunning and cerebral ischaemia. For decades, many diagnostic tools have been proposed for increased accuracy in evaluation of volemia, considering that clinical assessment is unreliable. Lung ultrasound and bioimpedance analysis emerged as the most useful tools, although randomized clinical studies are lacking to implement their universal use. Early management of hydration may improve clinical outcomes, as it allows personalized dialysis prescriptions and nutritional support.