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  1. 1

    Severe Tubulointerstitial Nephritis and Uveitis: A Pediatric Case Report

    Publication
    by Miguel, Pedro J.
    Other Authors: Costa-Reis, Patrícia; Leal, Inês; Boavida, João; Esteves da Silva, José
    Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare oculorenal inflammatory condition of unknown patho‐ genesis. It is thought to be an immune‐mediated process that can have multiple triggers, most notably infections or drugs; other toxic agents, such as hair‐dyes or paint thinners, have been associated with kidney injury, but their role as triggers of TINU is unknown. Usually, a complete recovery of renal function occurs, while the uveitis can be relapsing and challenging to control. We report a teenage girl with frequent contact with hair‐dyes and paint thinners, who initially presented with non‐oliguric acute kidney injury (AKI) of unknown etiology, requiring renal replacement therapy. Acute tubulointerstitial nephritis (TIN) was identified by kidney biopsy. Drugs, infectious and auto‐immune causes were excluded. Contact with hair‐dyes and paint thinners was stopped. The patient was treated with oral prednisolone, with complete recovery of renal function. Four months after the first symptoms, during corticosteroid taper, the patient developed eye redness, pain, photophobia and decreased visual acuity. Anterior bilateral uveitis and TINU syndrome were diagnosed. Eye inflammation was resistant to topical steroids associated with systemic steroids and methotrexate and resolved after adding adalimumab. Two years after initial presentation, the patient has normal renal function, normal urinary sediment and no proteinuria, normal blood pressure and absence of ocular inflammation. No relapses occurred under treatment with methotrexate and adalimumab.
  2. 2

    Membranous Nephropathy as a Manifestation of IgG4‐Related Disease (IgG4‐RD): A Case Report

    Publication
    by Quaresma, Marta
    Other Authors: Stock da Cunha, Teresa; Ferreira , Joel; Góis, Mário; Azevedo, Ana
    IgG4-related disease (IgG4-RD) is an immune-mediated, inflammatory condition that can affect multiple organs, marked by lymphoplasmocytic infiltration and IgG4-positive plasma cells. Elevated serum IgG4 is common but not essential. Sole renal manifestation is rare, usually appearing as tubulointerstitial nephritis (TIN) or, less commonly, membranous nephropathy (MN). We present a case of an 87-year-old man with a medical history of hypertension, dyslipidemia, ischemic cardiomyopathy, and COPD. The patient presented with nephrotic syndrome (proteinuria/creatininuria ratio (P/C ratio) of 11g/g) and acute kidney injury (creatinine 4mg/dl). Urinalysis indicated proteinuria and haematuria without dysmorphic erythrocytes. Investigation revealed normochromic normocytic anaemia, hypoalbuminemia, and no evidence of monoclonal gammopathy or hypocomplementemia. Serologies for HIV, hepatitis B, and hepatitis C were negative. Antinuclear, anti-neutrophil, and anti-phospholipase A2 antibodies (anti-PLA2r) were also negative. Treatment including anti-proteinuric and anticoagulation therapies was initiated. Kidney biopsy displayed membranous nephropathy and interstitial nephritis; immunofluorescence showed parietal granular deposits of IgG-κ and IgG-λ; immunohistochemistry revealed lymphocytes, plasma cells (with more than 10 IgG4-positive cells per high-power field), and IgG4 deposits on the glomerular basement membrane. Serum IgG subclass levels detected a slight elevation of IgG4. Other organ involvements hinting at IgG4-RD were ruled out. Assuming IgG4-RD with renal involvement therapy with prednisolone (40mg/day) was started and after one month the patient demonstrated a significant improvement in kidney function, anaemia, hypoalbuminemia, and proteinuria. Although renal manifestation as the first and/or only manifestation of IgG4-RD is rare, should be suspected in renal cases showing MN with or without TIN, especially when anti-PLA2R is negative.
  3. 3

    Revision of the Portuguese Rules for the Selection of the Donor‐Recipient Pair in Kidney Transplantation

    Publication
    by Lima, Bruno A.
    Other Authors: Alves, Helena
    The process of reviewing and updating the rules for the selection of donor‐recipient pairs in transplantation is underway, 16 years after the implementation of the current rules. Shortly after the implementation of the 2007 rules, some voices questioned the effectiveness of their results. Therefore, in addition to technical and scientific advances, the errors identified in the past should also serve as lessons learned for this new process underway. We hope that the current review will lead to simpler, more transparent and fairer allocation rules for such a precious commodity as deceased donor kidneys for transplantation.
  4. 4

    Conservative Care Management in Chronic Kidney Disease: A Value for the Money?

    Publication
    by Farinha, Ana
    Other Authors: Barata Coelho, Pedro; Teixeira Rodrigues, António
    End stage kidney disease (ESKD) is a potentially fatal disease that can be treated by one of three modalities: dialysis (hemodialysis or peritoneal dialysis) and kidney transplantation. Either of these options represents a heavy burden on health systems. Kidney transplantation has its own financial framework. Dialysis care, provided by public or private institutions is funded, since 2008, by a prospective bundle payment system called preço compreensivo (“comprehensive price”). In 2011, Direção Geral de Saúde (Directorate General of Health) proposed in “Norma 17” a fourth modality to handle ESKD: conservative care management (or kidney palliative care). This option focuses treatments on improving quality of life (and in some cases, time) in frail patients or patients who opt not to proceed to invasive treatment op‐ tions. “Norma 17” regulated the clinical framework for this proposal but its socioeconomic evaluation and subsequent reimbursement has not been established. This has become a barrier to its application by public and private providers. This work intends to reflect the individual, social and economic benefits of the implementation of the conservative care option so healthcare decision makers may support their decisions. The authors reflect on the limitations to analyze costs and results in end‐of‐life care and propose outcomes to compare dialysis and conservative care in ESKD.
  5. 5

    Does Prior Abdominal Surgery Influence Technique Survival of Peritoneal Dialysis Patients?

    Publication
    by Matos Silva, Rita
    Other Authors: Fragoso, Pedro; Reis, Marina; Cerqueira, Sofia; Romãozinho, Catarina; Borges, Andreia et al.
    Introduction: Prior abdominal surgery may result in peritoneal membrane adhesions and fibrosis. Its impact on peritoneal membrane function and peritoneal dialysis technique survival has not been adequately investigated. The aim of our study was to investigate the effect of prior abdominal surgical procedures on peritoneal dialysis technique survival. Methods: We performed a retrospective, longitudinal study that included 155 peritoneal dialysis patients followed at our unit from September 2017 to September 2022. Two groups were created according to previous abdominal surgery status. The primary outcome was technique survival, defined as time to hemodialysis transition. Secondary outcomes were dialytic efficacy and risk of peritonitis. Results: Sixty‐eight patients (43.9%) had a previous history of abdominal surgery of whom 25 patients were found to have peritoneal adhesions at the time of catheter implantation. Prior abdominal surgical procedures or peritoneal adhesions did not impact technique survival (p‐value=0.498; p‐value=0.447, respectively). Although prior abdominal surgery did not increase the risk of peritonitis (OR 1.01; p‐value=0.988), patients with intra‐abdominal adhesions presented a trend for increased peritonitis risk during follow‐up (OR 2.324; p‐value=0.059), even after adjusting for patient’s comor‐ bidities and time in dialysis. Diabetes (HR 2.14; p‐value=0.027) and baseline Kt/V (HR 0.23; p‐value < 0.001) were the only variables with prognostic impact on technique survival in multivariate survival analysis. Conclusion: According to our results, prior abdominal surgical procedures do not appear to compromise technique survival in patients on peritoneal dialysis.
  6. 6

    Clinical Presentation and Treatment of Childhood‐Onset Lupus Nephritis

    Publication
    by Silva, Rita M.
    Other Authors: Correia , Ana L.; Marques, Maria G.; Cordinhã, Carolina; Romãozinho, Catarina; Oliveira, Nuno A. et al.
    Introduction: Lupus nephritis (LN) in childhood usually presents after the age of 10 years and is more common in female children. Despite the availability of effective immunosuppressive therapies, treatment response in childhood‐onset LN remains suboptimal, with 50% to 78% patients in full remission at 24 months. Improvement in survivals have also plateaued in past decades, and up to 9% and 14% of children with childhood‐onset LN died or developed end‐stage kidney disease during the course of illness, respectively. In our study, we aim to evaluate clinical presentation of LN with childhood‐onset, the treatment regimen instituted and the transition to adulthood.   Methods: Retrospective study of patients with LN followed by pediatric nephrology with subsequent transition to adult nephrology since 1995 to 2020.   Results: Fourteen patients (female:78.6%) with a median age of 26.3 ± 5.2 years (range 21‐44) were included. The median age of LN diagnosis was 13.6 years. The most common manifestation was proteinuria (71.4%). Of non‐renal manifestations, polyarthritis (100.0%) and rash (57.1%) were the most common. All patients underwent kidney biopsy with a majority of diffuse LN (class IV) (n=11, 78.6%). Induction therapy consisted of corticosteroids combined with other immunosuppressants, primarily cyclophosphamide (CYC) (n=5, 35.3%) or mycophenolate (MMF) (n=9, 64.7%). Maintenance therapy consisted of corticosteroids combined with primarily CYC (n=6, 42.9%) with posterior transition to MMF, or MMF (n=5, 35.7%) or azathioprine (n=3, 21.4%). A complete remission occurred in all patients. At the end of follow‐up, 11 patients (78.6%) were with MMF and oral corticosteroids and 3 patients were with oral corticosteroids. None of these patients developed end‐stage kidney disease or died.   Conclusion: The diagnosis of LN in pediatric age has its particularities and constitutes a challenge for the nephrology community. Biological therapy currently shows promise in improving the prognosis of these patients.
  7. 7

    Mandibular and Rib Persistent Brown Tumors of Secondary Hyperparathyroidism

    Publication
    by Gonçalves, Francisco
    Other Authors: Pereira, Luciano; Oliveira, Ana; Beco, Ana
  8. 8

    Abstracts of 37th Congress of the Portuguese Society of Nephrology

    Publication
    by Nefrologia, Sociedade Portuguesa de
    Contains: - Oral Communications - Mini Orals - Posters
  9. 9

    Communication in Chronic Kidney Disease

    Publication
    by Piedade, Ana
    Other Authors: Domingues , Patrícia; Inácio , António; Mendes , Beatriz; Farinha , Ana
    In the 21st century, chronic kidney disease has become a leading cause of mortality and suffering. In Portugal, the prev‐ alence of chronic kidney disease was estimated at 20.9%, higher than the world and European average. End‐stage renal disease requiring dialysis is a significant condition with a high disease burden, morbidity, and mortality rate. Effective communication plays an important role in facilitating adaptation to the reality of living with a serious illness. Appro‐ priate decision‐making, quality of life and adaptation to the realities of the disease are significantly aided by effective communication. Good patient‐centered communication is associated with better adherence to treatment, pain control, satisfaction, emotional health, functional status, confidence, and physical outcomes. Nephrologists care for a clinically complex population that faces difficult decisions about treatment options and end‐of‐ ‐life care. In Nephrology, effective communication between patient and nephrologist is crucial. However, communication training within the nephrology fellowship is rare. We must routinely incorporate communication about treatment goals into our clinical care structures and processes.
  10. 10

    Cognitive Dysfunction in Chronic Kidney Disease Patients: Time to Prevent

    Publication
    by Ferreira, Ana Carina
    Mild cognitive impairment (MCI) is a neurocognitive deterioration of at least one of the following six cognitive domains in humans: complex attention, executive function, learning and memory, language, perceptual‐motor, and social cognition. Patients with chronic kidney disease are at an increased risk of developing this disorder, with serious implications for the patient, family, and society. In this article, we aim to increase awareness of the disorder and summarize the existing evidence on the topic.