Scielo RSS http://scielo.pt/rss.php?pid=0872-016920220001&lang=p vol. 36 num. 1 lang. p http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100003&lng=p&nrm=iso&tlng=p ABSTRACT Introduction: The severe acute respiratory syndrome coronavirus 2 pandemic caused a negative impact on transplantation worldwide. One of the complexities is the management of the immunosuppression and anti-viral medication in transplant recipients. Herein we describe our experience managing five patients with coronavirus disease 2019 (COVID-19) immediately after kidney transplant. Description: we describe a series of five transplant recipients. Two were submitted to a low immunological risk induction immunosuppression: one developed severe disease, the other developed critical disease. The remaining three were submitted to a high immunologic risk immunosuppression: one developed mild disease, but approximately two months after diagnosis developed moderate COVID-19; another developed severe COVID-19; the remaining patient developed critical illness and perished. Both patients with critical COVID-19 developed bacterial and fungal superinfections in the Intensive Care Unit. All eligible patients were submitted to remdesivir and dexamethasone. The anti-metabolite was reduced or suspended. Despite worsening kidney function during the acute phase, kidney replacement therapy was only needed in one patient and the average serum creatinine at the time of discharge was lower that the pre-COVID-19 nadir. Discussion: There is still no definitive answer on whether solid organ transplant recipients carry a higher risk for morbimortality. Risk factors for the general population can also affect their outcome. The prolonged viral shedding in recipients may explain the irregular evolution of the patient who developed COVID-19 months after the initial diagnosis. The reduction of immunosuppression can be problematic in early post-transplant, but no apparent signs of rejection were noted. Literature addressing this specific context is scarce and studies are needed to clarify the optimal management of these patients. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100007&lng=p&nrm=iso&tlng=p ABSTRACT Membranoproliferative glomerulonephritis is a rare chronic glomerulonephritis in pediatric age. It may be a primary disorder or secondary to other chronic diseases. Diagnose of membranoproliferative glomerulonephritis is based on specific kidney biopsy findings. The prognostic factors, the outcome and the most suitable treatment regimen are incompletely known in children. We report a case of a 3-year-old boy that presented with nephritic/nephrotic syndrome and hypocomplementemia. After 10 days under corticosteroid therapy, he maintained worsening of renal function and a renal biopsy specimen revealed a membranoproliferative glomerulonephritis. Autoimmune disease and infections were initially excluded, but further investigation revealed positive Polymerase Chain Reaction for cytomegalovirus in blood and urine. Cytomegalovirus genome at biopsy was not identified. He completed six months of treatment with valganciclovir and corticosteroids with total remission of proteinuria and undetected viral loads. With this case, we would like to draw attention to a type of nephropathy that is rare in childhood. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100011&lng=p&nrm=iso&tlng=p ABSTRACT A 56-year-old male with a poorly controlled human immunodeficiency virus infection presents to the emergency room due to anorexia and weight loss. The patient was emaciated, hypertensive and his laboratory tests showed thrombocytopenia, anaemia, an elevated lactate dehydrogenase and an acute kidney injury. The patient was admitted to the Infecciology ward and by the fourth day, as the kidney function did not improve, a Nephrology consultation was requested. We collected a full auto-immune and serological panel and a urinalysis to rule out glomerular pathologies. Two days later, the patient developed a hypertensive emergency associated with an acute respiratory distress, responsive to furosemide and anti-hypertensive medications. The complementary study showed a high LDH, unmeasurable haptoglobin, thrombocytopenia and schizocytes on peripheral blood smear. These findings suggested a thrombotic microangiopathy associated with human immunodeficiency virus infection and so, he restarted antiretroviral therapy. The kidney function deteriorated and, despite starting haemodialysis, the patient died shortly after. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100015&lng=p&nrm=iso&tlng=p ABSTRACT Primary inherited epidermal growth factor receptor defects have recently been described in severe inflammatory skin disease and diarrhoea case reports. We describe two case reports of female preterm newborns of Roma consanguineous parents who presented both with alopecia and erythroderma/ichthyosis, in addition to nephromegaly at birth in case 1. Later, they both developed hypomagnesaemia and other severe hydroelectrolyte disturbances, recurrent life-threatening sepsis and failure to thrive. Exome sequencing identified a homozygous mutation in the epidermal growth factor receptor, rarely described. Despite optimization of medical supportive care, the prognosis was poor and both patients died before the first year of life. There are few similar cases of epidermal growth factor receptor homozygous mutation reported so far. Our manuscript describes the genetics, clinical presentation, and complex treatment of our two patients, aiming to contribute to new advances in the management of this condition. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100020&lng=p&nrm=iso&tlng=p ABSTRACT We admitted a 21‑year‑old female to the Nephrology ward due to proteinuria and generalized oedema, which started soon after consuming clonixin 300mg thrice a day for two weeks. Her initial blood work showed nephrotic‑range proteinuria, hypoalbuminaemia, hypercholesterolaemia and normal kidney function. We conducted a full auto‑immune and serological study: there were no signs of infection, complemente consumption or underlying auto‑immune diseases. Suspecting minimal change disease, we started prednisolone 1mg/kg/day, with good clinical response and a reduction of urinary protein losses. By the third day, a kidney biopsy was performed: the glomeruli were morphologically normal and no immune deposits were found, which suggested minimal change disease. After seven days, the patient was discharged from the hospital with a corticoid taper until her Nephrology consultation. In the following weeks she maintained clinical and laboratorial remission and prednisolone was stopped after six months. Two months after, the patient noticed a growing bilateral cervical mass and went to the emergency room. The diagnostic workup was inconclusive, so an ultrasound‑guided biopsy was requested. The biopsy revealed lymphoid cell proliferation, positive for CD30, CD15, MUM‑1 and BCl6. These findings confirmed a Hodgkin’s Lymphoma diagnosis and the patient started radiotherapy and chemotherapy (ABVD‑doxorubicin, bleomycin, vinblastin, dacarbazine). In the following months her clinical status improved and the cervical mass had a significant size reduction. The kidney disease remained in remission, without therapeutic adjustments. A post‑treatment PET scan was requested, showing only a residual mediastinal mass. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100031&lng=p&nrm=iso&tlng=p ABSTRACT COVID-19 is a pandemic and life-threatening respiratory disease. Chronic kidney disease is a probable risk factor for more severe COVID-19, but outcomes in the peritoneal dialysis population are scarce. We analyzed our peritoneal dialysis center COVID-19 cases from March 2020 to January 2021, before full vaccination with the Pfizer BNT 162b2 mRNA vaccine in February 2021. There were 13 cases of COVID-19 out of 96 patients on peritoneal dialysis (cumulative incidence 13,5 cases per 1000 patients-month). Nine were considered mild (76,9%), two moderate (15,4%) and one severe/critical (7,7%). There was one asymptomatic case. The most common presenting signs and symptoms were myalgia, cough, fever, asthenia, hypotension, and loss of smell and/or taste. Only one patient required oxygen in the ICU. There was a hospitalization rate of 30,8% (three mild and one severe/critical) and a median time of hospitalization until discharge, or death, of 6 days. The most common reason for hospitalization was hypotension and asthenia, without respiratory failure (three mild out of four hospitalizations). One patient died (7,7%) and 12 patients recovered well (92,3%). Of eleven patients presenting with COVID-19 symptoms, nine reported persisting symptoms for over one month (81,8%). In conclusion, COVID-19 in patients under peritoneal dialysis had a relatively benign course with symptoms mainly unrelated to the respiratory tract. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100035&lng=p&nrm=iso&tlng=p ABSTRACT Introduction: Erythropoietin-stimulating agents hyporesponsiveness is common among hemodialysis patients. The aim was to analyze factors predictive of Erythropoietin-stimulating agent resistance and its association with mortality. Subjects and methods: Retrospective analysis of prevalent hemodialysis patients. Dose-response effect of Erythropoietin-stimulating agent therapy was evaluated using Erythropoietin-stimulating agent resistance index (ERI). Patients were classified in groups (ERI≤10 and ERI>10). All-cause mortality was assessed using standard survival methods. Results: Among 59 patients, 24 had ERI>10. Patients with ERI >10 had more central venous catheters, higher C-reactive protein levels, lower body mass index, serum iron, transferrin saturation, albumin and intact parathormone levels. Hyporesponsive patients had an increased risk of one-year mortality. Discussion and conclusion: Our study confirmed that malnutrition, inflammation, and iron deficiency are the main causes of Erythropoietin-stimulating agent hyporesponsiveness, and intact parathormone levels and central venous catheter use may also play a role. Erythropoietin-stimulating agent resistance appear to be associated with an increased mortality risk among prevalent hemodialysis patients. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100040&lng=p&nrm=iso&tlng=p ABSTRACT Introduction: Chronic kidney disease has significant morbidity and mortality worldwide. Various studies have demonstrated that incidente patients experience a higher mortality rate within the first 3 months of dialysis. Methods: A single-center retrospective case-control study (1:3) was performed to determine early (<90 days) mortality rate and associated risk factors in incident hemodialysis patients from January 2013 to December 2018. We compared variables between survivors and non-survivors at 90 days after initiation of hemodialysis. Multivariate logistic regression was used to calculate the adjusted odds ratio with 95% confidence intervals for the variables associated with early mortality and a predictive model was developed. Results: From a total of 626 incident hemodialysis patients, 48 (7.7%) died before 90 days of treatment. Non-survivors were older [OR 1.07 (1.03-1.11)], had higher rates of non-recovering acute kidney injury [OR 7.91 (3.63-17.24)], emergency start of hemodialysis [OR 4.31 (2.15-8.62)], congestive heart failure [OR 5.68 (2.81-11.48)], ischemic cardiomyopathy [OR 4.50 (2.25-8.99)], chronic obstructive pulmonary disease [OR 3.60 (1.44-8.95)], Charlson comorbidity index [OR 1.47 (1.27-1.70)] and dependence of assistance in daily living activities [OR 3.46 (1.76-6.82)]. Patients were less likely to have 90-day mortality if they had nephrologist appointments at least 90 days prior to end-stage renal disease [OR 0.25, 95% CI (0.13-0.50); p<0.001] or a higher serum albumin [OR 0.34, 95% CI (0.19-0.62); p<0.001]. Multivariate analysis risk factors independently associated with early mortality were older age [aOR 1.06 (1-01-1.10), p=0.022], acute kidney injury as cause of end-stage renal disease [aOR 12.62 (4.50-35.40), p<0.001], congestive heart failure [aOR 3.79 (1.58-9.11), p=0.003], and Charlson comorbidity index [aOR 1.30 (1.09-1.56), p=0.005]. The model showed very good discriminative ability [AUROC (95% CI) 0.88 (0.83-0.94)]. Conclusion: Early mortality occurred in 7.7% of our population. Our model could be used to identify patients at higher risk of death during the first 90 days of hemodialysis and aid informed decision-making regarding end-stage renal disease treatment options. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100046&lng=p&nrm=iso&tlng=p ABSTRACT Introduction: Social disparities in chronic kidney disease are a reality. Low socioeconomic status is associated with increased incidence of chronic kidney disease and overall worse outcomes. In Portugal, similarly to many European countries, a National Health System was established to provide equity in healthcare access, but its impact in specialized chronic kidney disease care is unclear. This study aims to compare the effects of economic insufficiency in referral and overall specialized kidney care in a Portuguese center. Subjects and Methods: Retrospective cohort study evaluating maintenance hemodialysis patients of a Public Portuguese Nephrology Center from 2017 through 2021. Medical records were compared for presence of low-income status, with primary outcome as baseline estimated glomerular filtration rate at Nephrology referral and secondary outcomes as presence of kidney replacement therapy options appointment, timely vascular access assessment and time to dialysis from referral. Results: A total of 212 participants were evaluated, 96 with low-income status. This group presented higher serum creatinine and lower estimated glomerular filtration rate at referral (2.35 vs. 3.29 mg/dl, p<0.001; 27.63 vs. 18.47 ml/min/1.73 m2, p<0.001; respectively). Low-income status associated with absence of kidney replacement therapy options appointment (OR 2.7, 95% CI: 1.44-5.08; p=0.003) and late vascular access evaluation (OR 2.77, 95% CI: 1.55-4.98, p=0.001). Dialysis-free survival analysis revealed shorter time to dialysis in the low-income status group (15.77 vs. 20.71 months, p<0.001) with a higher cumulative incidence in dialysis at 24 months (HR: 2.11, 95% CI: 1.39-3.21, p < 0.001), a difference that was not verified after adjusting for estimated glomerular filtration rate at referral (HR: 1.16, 95 % CI: 0.74 - 1.80, p = 0.53). Conclusion: Low-Income chronic kidney disease Portuguese patients are at risk for late Nephrology referral, an established factor for adverse outcomes. Shorter time to dialysis appears significative in reducing adequate kidney care access, which can be attenuated through education of primary care providers and general population for chronic kidney disease. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100050&lng=p&nrm=iso&tlng=p ABSTRACT Dialysis-associated immune dysfunction makes chronic kidney disease patients both susceptible to severe Coronavirus disease 19 (Covid19) and to a weaker response to vaccination. Previously infected patients are thought to sustain stronger and more durable humoral responses than vaccinated patients. Four months after a two dose-regimen of the Pfizer/BioNTech SARS-CoV-2 mRNA vaccine (BNT162b2), we evaluated the SARS-CoV-2 spike immunoglobin G (IgG-S1) antibody levels in previously infected peritoneal dialysis patients and compared them with infection naïve PD patients. A total of 79 peritoneal dialysis patients were analyzed, of which 11 had a previous history of Covid19. We have verified that the median titer of the IgG-S1 in previously infected patients (14310 AU/mL) was significantly superior to that in infection naïve patients (760,05 AU/mL) (p < 0,001). Previous Covid19 was the only significant predictor of IgG-S1 levels in a multivariate linear regression model (p < 0,001). These results may impact vaccination strategies for peritoneal dialysis patients regarding the future administration of BNT162b2 booster doses. In conclusion, previously infected peritoneal dialysis patients who have completed a two-dose regimen of the BNT162b2 may be well suited without a third, booster dose for longer than infection naïve peritoneal dialysis patients. This strategy could make additional doses available around the world. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100053&lng=p&nrm=iso&tlng=p ABSTRACT Point-of-care ultrasound aims to enhance physical examination findings with real-time information from the ultrasound. Peritoneal dialysis catheter-related infections are a frequent complication of peritoneal dialysis and a major predisposing factor to peritonitis. The diagnosis of peritoneal dialysis catheter-related infections is made with physical examination, but physical findings usually lack sensitivity or specificity. This is a single-center observational study that aimed to evaluate the accuracy of point-of-care ultrasound for the diagnosis of peritoneal dialysis catheter-related infections. Sixteen peritoneal dialysis patients with peritoneal dialysis catheter-related infections clinical suspicion were enrolled in this study, as well as nine controls. Peritoneal dialysis catheter-related infection was defined as a purulent discharge with or without inflammatory signs in the peritoneal dialysis catheter exit-site or subcutaneous tunnel, and a positive microbiological culture collected from the exit-site. Point-of-care ultrasound was considered positive when an anechoic collection in relation to the external cuff or catheter tunnel was identified. Physical examination findings were coded using the Exit-site Scoring System. A total of 13 peritoneal dialysis catheter-related infections were diagnosed. The mean Exit-site Scoring System score for the suspected cases of peritoneal dialysis catheter-related infections was 2,82 ± 1,22, and the most commonly encountered sign was purulent discharge. All of the cases of peritoneal dialysis catheter-related infections had a positive point-of-care ultrasound. In contrast, none of the controls had a positive point-of-care ultrasound (p < 0,001). The area under the receiver operating characteristic curve of physical examination for the diagnosis of peritoneal dialysis catheter-related infections was 0,6 (95% CI 0,37-0,84). In contrast, point-of-care ultrasound had an AUROC of 0,91 (95% CI: 0,73-1) for the diagnosis of peritoneal dialysis catheter-related infections. Nephrologist-performed point-of-care ultrasound is feasible and superior to physical examination for the diagnosis of peritoneal dialysis catheter-related infections. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692022000100057&lng=p&nrm=iso&tlng=p ABSTRACT Atrial fibrillation is highly prevalent in patients with end‑stage kidney disease. For patients with atrial fibrillation but normal kidney function, the net clinical benefit of oral anticoagulation with vitamin K antagonists and non‑vitamin K oral anticoagulants in preventing stroke against the hemorrhagic risk is well‑known. However, dialysis patients present unique risk factors that increase ischemic stroke and bleeding events that are not included in the traditional risk scores. These points, in addition to the pharmacokinetic issues related to the use of oral anticoagulants in end‑stage kidney disease, raise the question of the safety of these drugs, making it difficult to predict whether the benefit outweighs the risk. Data on this topic in chronic kidney disease stages 4, 5, and 5D patients is sparse and the clinical guidelines are also inconsistente in which is the best approach for these patients. This review gives an overview of the state of the art on this topic, specifically the recommendations of the newest guidelines, then discusses the various risk scores and their pitfalls, and finally summarizes the current knowledge on the possible benefits of Non‑vitamin K oral anticoagulants versus Vitamin K antagonists in dialysis patients.