SNEF - Artigos publicados em revistas não indexadas na Medline
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- Acute kidney injury with active urinary sediment analysis, a positive ANCA test and hypocomplememtemia: A tough situationPublication . Campos, A.; Vizcaíno, J.; Coelho, A.; Freitas, C.; Rocha, G.
- Acute tubulointersticial nephritis with uveitis: A report of two casesPublication . Silva, F.; Correia, S.; Castro, A.; Moreira, C.; Santos, S.; Malheiro, J.; Santos, J.; Martins, L.; Cabrita, A.Tubulointersticial nephritis and uveitis syndrome is an idiopathic and rare cause of acute kidney injury that should not overlooked, because it usually requires specific therapeutic interventions. We report two distinct cases: a young and an elder female. Both cases presented with unspecific constitutional symptoms but had different onset of renal and ocular involvement. Both were treated with topical and systemic corticoids and although there was a good initial response in both cases, an early relapse after steroids taper was observed in the younger patient and a persistent renal dysfunction in the older one. A high clinical suspicion and understanding of this disease is necessary for an adequate management and treatment of these patients. Recent data associates a worse renal prognosis when the disease appears in advanced age. In both of our cases the outcome was good but we had a short follow-up. The histological presentation of this disease in our older patient was similar to that reported in the literature, with a high percentage of fibrosis and chronicity of renal tissue that can contribute to the higher grade of renal dysfunction in this type of patients.
- Anderson-Fabry disease: Ten-year outcome of enzyme replacement therapy in a renal transplant patientPublication . Santos, S.; Campos, A.; Beirao, I.Anderson‑Fabry disease (AFd) is a rare disorder characterised by the deficiency or absence of lysosomal enzymatic alpha‑galactosidase A activity (α‑Gal A) that leads to progressive and systemic accumulation of glycosphingolipids. The clinical manifestations are variable but kidney disease usually manifests before the fourth decade of life and chronic renal failure rapidly progresses to end‑stage renal disease (ESRD), requiring dialysis and kidney transplantation (KT). In patients with a definite diagnosis, enzyme replacement therapy (ERT) is recommended as soon as there are early clinical signs of kidney, heart or brain involvement. We present a case of a kidney transplant patient who was diagnosed with AFd nine years after KT, confirming the difficulty that may exist in na early diagnosis of this disease even among high‑risk groups. At this stage, in addition to renal damage, the patient already had advanced disease and established organ injury, including ocular, pulmonary, cerebrovascular and cardiac. He started agalsidase beta (Fabrazyme®) intravenously every two weeks at a dose of 1 mg/kg body weight. During ten years of treatment no major adverse events were reported and our experience indicates that ERT is a safe and effective treatment for extra‑renal Fabry manifestations in KT patients
- Are we building too many arteriovenous fistulas? A single-center experiencePublication . Leal-Moreira, C.; Teixeira, V.; Bessa, L.; Queirós, J.; Silva, F.; Cabrita, A.Introduction: Arteriovenous fistula has been associated with improved morbimortality in hemodialysis patients. This has resulted in the “fistula First, catheter last” initiative. Nonetheless, the survival benefit of arteriovenous fistula has been questioned. Methods: We conducted a retrospective observational study of all patients with non-end stage renal disease referred for first vascular access building between January 2014 and December 2015 in our hospital center. Our main goal was to evaluate the clinical impact and burden of building fistula in predialysis patients. Results: During this period, of 178 first arteriovenous accesses placed, 87 patients remained in predialysis and 91 patients started a chronic hemodialysis program. Median follow-up time by a nephrologist was 3.9 (2.5, 9.7) years. The mean age was 65.8±14.7 years, with 50.6% (n=90) of male patients. A higher rate of thrombosis in the predialysis group (26% vs 13%, p=0.037) was observed, but vascular access survival did not differ significantly (55% vs 67%, p=0.12). Mean vascular access placing was higher in the predialysis group (1.4±0.7 vs 1.2±0.4, p=0.006) and less interventions were requested (0.2±0.5 vs 0.3±0.6, p=0.10). Median time from vascular access placement to hemodialysis start was 22 (13, 41) months. At hemodialysis initiation, 10 (10.9%) patients used a central venous catheter; 80 (87.9%) patients an arteriovenous fistula, and one patient a graft. A total of 227 vascular accesses were built; 121 (53.3%) in predialysis vs 106 (46.7%) in incident hemodialysis patients. In a multivariate model, the presence of a functional arteriovenous fistula at hemodialysis start was only associated with a trend to survival benefit (HR 0.38, 95% CI 0.14-1.00, p=0.05). Conclusions: Our results stress the need for an individual approach and for future tools to assess the risk of death and progression to end-stage renal disease, therefore helping reduce the number of unutilized vascular accesses and rising cost of interventions.
- Arterio-arterial graft – an option for hemodialysis patients with exhaustion of venous patrimonyPublication . Castro, A.; Almeida, P.; Silva, F.; Rego, D.; Tavares, J.; Santos, J.; Silva, F.; Queirós, J.; Cabrita, A.; Almeida, R.Introduction: Vascular access (VA) for hemodialysis (HD) is the lifeline for End Stage Renal Disease (ESRD) patients. Long-term HD patients often have exhaustion of their venous patrimony for an autologous VA construction and, sometimes, even for a central venous catheter (CVC) placement. Case report: We describe the case of a 43-year-old woman with ESRD due to lupus nephritis, on maintenance HD since 2009. She also had secondary antiphospholipid syndrome and was chronically anticoagulated. Nevertheless, the patient had multiorgan thrombotic events (without sequelae) and several episodes of irreversible thrombosis of arteriovenous fistulas. Her HD course was also marked by multiple severe CVC infections, at diferente locations; a hemoperitoneum during cholecystectomy, and an immediate thrombosis of the renal artery of a kidney transplant. She was admitted to our hospital after an irreversible dysfunction of a right jugular CVC, with documentation of thrombosis of the superior and inferior vena cava. Exhaustion of the venous patrimony for HD was assumed and it was decided to make an arterio-arterial graft (AAG) of early cannulation. The first cannulation of the AAG was performed two days after surgical intervention, with no complications. The patient performed a twelve hour per week HD treatment with good efficiency. Conclusion: AAG is an alternative for HD patients who have exhausted all their venous patrimony and it can be considered prior to the placement of a CVC as their sole remaining vascular access.
- Biomarkers in Kidney Transplantation: Translating to clinical practicePublication . Fonseca, IsabelImproving long-term graft survival is a major challenge in kidney transplantation. Ischaemia-reperfusion injury is a critical early allograft insult that enhances the risk of delayed graft function, which is common in deceased-donor transplantation. Delayed graft function complicates the post-transplant management and has a negative impact on both short and long-term outcomes. The development of effective interventions to prevent and attenuate the injury caused by ischaemia-reperfusion is constricted by the limited ability of early detection of kidney damage. In recent years, clinical and translational research has focused on improvements in the diagnosis of acute kidney injury and provided prognostic information that is helpful in the post-transplant care. Numerous biomarkers in kidney transplantation have been evaluated in the past decade, but, so far, evidence to support their use in routine practice is limited. The purpose of this review is to examine the current status of three biomarkers for early diagnosis and prognosis of delayed graft function, namely urinary neutrophil gelatinase-associated lipocalin, oxidative stress and cystatin C. In addition, the concept of a biomarker is addressed, as well as the existing challenges and perspectives for developing a biomarker. This review discusses current literature and reflects the author’s own interpretation and experience.
- BK virus nephropathy in kidney transplantation - A literature review following a clinical casePublication . Barreto, P.; Almeida, M.; Dias, L.; Vieira, P.; Pedroso, S.; Martins, L.; Castro-Henriques, A.; Cabrita, A.Over the last 15 years, better immunosuppressive drugs have decreased acute rejection rates in kidney transplantation but have also led to an increase in the incidence and impact of BK virus nephropathy. The authors report the case of a 62 -year -old man submitted to a renal transplant of a deceased donor with an immunosuppression regimen free of rabbit anti -thymocyte globulin and tacrolimus, in whom BK nephropathy was diagnosed at seven weeks post -transplant. Intravenous human immunoglobulin (IVIG) was administered after immunosuppression reduction. Instituted treatment was successful. This clinical case highlights the importance of a high index of suspicion for an atypical presentation of BK nephropathy in renal transplant recipients and strengthens the need for other therapeutic interventions beyond the reduction of immunosuppression. It was the starting point for a review of BK virus nephropathy in kidney transplantation with a focus on risk factors, diagnosis and treatment.
- Clinical implications of anti-HLA antibodies testing in kidney transplantationPublication . Malheiro, J.; Tafulo, S.Alloantibodies against donor human leukocyte antigens (HLA), termed as donor‑specific antibodies (DSA), are one of the most important factors for both early and late kidney allograft dysfunction. In the past, these antibodies were mainly detected through cell‑based crossmatch tests. Recently, new techniques such as solid phase immunoassays (SPI) have revealed these antibodies in patient sera with a high degree of detail, previously unimaginable. They have allowed us to accurately determine recipients’ allosensitization status, improve pre‑transplant risk assessment with a potential donor and post‑transplant alloimmune monitoring. However, the high sensitivity of these new assays has also created areas of uncertainty about their clinical impact. In the pre‑transplant setting, the presence of preformed DSA has been associated with an increased risk of antibody‑mediated rejection (AMR) and subsequent allograft loss. Nevertheless, several studies have shown that not all DSA are deleterious. Hence, understanding the clinical correlations of DSA characteristics, namely strength, HLA class, complement‑fixing ability or IgG subclasses, is paramount for an adequate stratification of the immunological risk at transplant. Furthermore, given that the number of allosensitized patients on waiting lists is increasing, the added information from these new SPI is essential to improve their chance of being transplanted with an admissible immunological risk. After transplantation, the appearance of de novo DSA (dnDSA) has also been associated with a deleterious effect on kidney allograft survival. Moreover, it has been acknowledged that a majority of late allograft failures are caused by alloantibody‑driven injury. The current challenges, in this setting, are determining cost‑effective DSA screening protocols and understanding which patients could benefit from specific interventions. Furthermore, although therapeutic strategies to control antibody‑induced damage remain limited, the longitudinal surveillance of dnDSA emergence and the clinical correlations of their characteristics will play a crucial role in the improvement of late kidney allograft survival.
- Diagnosis of monoclonal gammopathy of renal significancePublication . Correia, S.; Santos, S.; Martins, L.; Santos, J.Monoclonal gammopathies are a heterogeneous group of disorders characterized by clonal proliferation of immunoglobulin produced by B-lymphocytes or plasma cell clone. The term monoclonal gammopathy of renal significance (MGRS) was introduced to distinguish monoclonal gammopathies that result in the development of kidney disease from those that are benign. Screening for monoclonal immunoglobulin and an appropriate hematologic workup are fundamental and sometimes a difficult challenge, with therapeutic and prognostic implications. Kidney biopsy is essential to determine the exact nature of the lesion and to evaluate the severity of renal disease. In this review we discuss the clinical and pathologic features of MGRS, highlighting the most diagnostic difficulties and current therapeutic options.