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- 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.
- Transitions of care management in CKD: critical thinking and improving strategiesPublication . Correia, I.; Rodrigues, A.Chronic kidney disease (CKD) has a high clinical and socioeconomic impact and is often associated with multimorbidity. Improved treatment has allowed an increase in patient survival, but patient life expectancy remains limited. The disease course has a continuum of lesion, stage and treatment transitions. The focus is often placed on treatment modality, disregarding the course of a CKD patient’s disease. In addition, patient management in transitions of modalities of renal replacement therapy (RRT) can also be a vector for improving clinical outcomes. The transition between different types of CKD treatment and the transition of care from paediatric to adult team are critical processes throughout the life of a CKD patient. In the therapeutic transition, there is the need to identify better predictors of success in allocating patients with stage 5 CKD to their first dialytic modality in. There is a risk of early mortality in the induction period of dialysis, particularly of the elderly in extracorporeal dialysis regimens. Doubt remains in decision making about the ideal timing to establish the transition to renal replacement therapy and its most appropriate type. Transfer between dialytic modalities also calls for opportune and integrated policies protecting vascular resources. Renal transplantation is considered the optimal renal replacement therapy; however, transplant failure or the side effects of immunosuppression are threats to consider, which may redirect these patients back to dialysis and involves a re‑evaluation of the patient’s status. Also, end‑of‑life care and decision making between initiating renal replacement therapy or maintaining conservative management are a challenge in the elderly. This review identifies the main challenges in these transitional processes, raising awareness of areas in need of improvement in patient care. The aim should be to achieve a more comprehensive and appropriate health management than a limited focus on CKD modality treatment.
- Portuguese consensus document statement in diagnostic and management of atypical hemolytic uremic syndromePublication . Azevedo, A.; Faria, B.; Teixeira, C.; Carvalho, F.; Neto, G.; Santos, J.; Santos, M.; Oliveira, N.; Fidalgo, T.; Calado, J.Among thrombotic microangiopathies (TMA), the hemolytic uremic syndrome associated with dysregulation of the alternative complement pathway (aHUS) is one of the most challenging diseases a nephrologist can face. By the end of the XXth century, the complement’s role was unraveled with the discovery that mutations in the factor H coding gene were responsible for aHUS. But it was the acknowledgment that pharmacological C5-9 blockage provided a cure for aHUS that fostered the interest of the nephrology community in the genetics, pathophysiology and therapeutics of, not only of aHUS, but TMA in general. The molecular genetics of aHUS is technically demanding and, as such, in Portugal (alike many other European countries) a single laboratory emerged as a national reference center. The fact that all samples are evaluated in a single center provides a unique opportunity for data collection and a forum for discussion for all those interested in the field: immunologists, molecular geneticists, pathologists and nephrologists. The current consensus document emerged from such a discussion forum and was sponsored by the Portuguese Society of Nephrology. The goal is more to portray the Portuguese picture regarding the diagnostic approach and therapeutic options than to extensively review the state of the art of the subject. The accompanying documents that are published as supplementary data are in line with that goal. They range from the informed consent and clinical form to be sent together with the biological samples for genetic testing, to the appendix regarding the actual sampling and storing conditions. The document is also intended to set an example for future documents and independente discussion forums on other kidney diseases for which emerging diagnostic and/or therapeutic strategies are reaching clinical practice.
- 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.
- Infectious risk of elderly patients on peritoneal dialysis: Experience of a portuguese centerPublication . Belino, C.; Barreto, P.; Santos, M.; Gomes, A.; Fonseca, Isabel; Fernandes, J.Background: The goal of this study was to compare the risk of peritoneal dialysis‑related infections in younger and older patients and to identify risk factors for infection in elderly patients. Methods: We performed a longitudinal retrospective study on a population of Portuguese peritoneal dialysis patients treated at the same center between January 2005 and December 2015. Clinical and laboratory data were collected from medical reports. Two groups of patients were compared: non elderly (< 65 years) and elderly (≥ 65 years). Results: Among 100 patients, there were 73 non elderly (median age 52 years) and 27 elderly (median age 74 years). Elderly patients were not associated with higher PD‑infection rates or with less time to PD‑related infections. Cerebrovascular disease was the only significant adverse predictor of peritonitis in elderly (crude HR 3.8; 95% CI 1.10 to 13.34; P = 0.035) and those with higher levels of serum albumin were less likely to develop peritonitis (crude HR 0.47 per each g/dl of increase; 95% CI 0.24 to 0.89; P = 0.023). Conclusions: In our study, elderly patients did not present a greater risk for peritonitis or catheter‑related infections.
- Peritoneal Dialysis in the elderly: challenge acceptedPublication . Lança, A.; Carvalho, M.; Rodrigues, A.At present, mostly in Western countries, age is no longer an absolute limitation for renal replacement therapy (RRT); however, some elderly patients are still not considered for peritoneal dialysis (PD), mainly due to late referral, social isolation, low functional capacity or lower life expectancy. In this review, we address the challenges posed by older patients on PD programs, focusing on a SWOT (strengths, weaknesses, opportunities and threats) analysis and illustrate how PD may have successful outcomes in this population, worldwide and in Portugal. Finally, we will enumerate strategies to overcome the barriers to this technique. From January to December 2017, we conducted a systematic review of the literature using MEDLINE, the Cochrane Library and Web of Knowledge. Studies on PD and HD were included. All searches were limited to English and Portuguese and were augmented by review of bibliographic references from the studies included. Findings concerning modality superiority and better outcome in older people are still scarce and controversial, however according to several well-established PD programs worldwide, including assisted PD, elderly patients presented similar technique survival, hospitalization rates and frequency of peritonitis as compared to younger PD patients and HD patients. As expected, older patients had higher mortality though, especially in patients with more comorbidities. On the other hand, PD was associated with less cognitive loss and showed benefit in perceived quality of life. In Portugal, the low utilization of PD and the patients’ age discrepancy between both modalities explains the limited literature and the discrepante results (some studies show lower peritonitis rate, superior technique and patient survival and others presente higher hospitalization episodes and mortality rates); however, it appears to be a good (cost-effective) option for elderly patients. Overall, PD is an equally suitable modality for elderly patients in the long term.
- 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.
- 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.
- 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.
- Kidney transplantation in a patient with preformed and exclusively anti-HLA-Cw donor specific antibodyPublication . Santos, S.; Castro, A.; Campos, A.; Pedroso, S.; Dias, L.; Castro-Henriques, A.We report a patient who had received a first kidney transplant and had preformed DSA anti-HLA-Cw, developing AMR C4d+ soon after transplant. Classically anti-HLA-Cw are considered less immunogenic and are not considered in many organ allocation systems or immunologic risk stratification algorithms, including in Portugal. However, data from literature confirms that their presence is as deleterious as DSA anti-HLA A/B/DR/DQ. Thus we should take HLA-C typing and respective antibody identification into account in sensitized patients, in order to access risk stratification and establish the need for correct induction or desensitization therapies.