Scielo RSS http://scielo.pt/rss.php?pid=0872-016920230001&lang=pt vol. 37 num. 1 lang. pt http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100003&lng=pt&nrm=iso&tlng=pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100006&lng=pt&nrm=iso&tlng=pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100009&lng=pt&nrm=iso&tlng=pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100011&lng=pt&nrm=iso&tlng=pt ABSTRACT Introduction: Elderly patients are the most frequent patients admitted to dialysis units. Relatively little is known about standards on endof-life (EOL) care among dialysis patients. From international literature, these patients are exposed to more aggressive treatments at EOL than patients with cancer or other chronic diseases and palliative referral is infrequent. This study aims to describe some end-of-life (EOL) practices in patients undergoing hemodialysis in NephroCare clinics in several cities in Portugal. Material and Methods: We designed a prospective, multicenter, observational, cross-sectional study conducted between October 1st, 2020 and September 30th, 2021 in six hemodialysis clinics including 1265 patients (pts). Results: During the year in analysis, 158 pts died (12.4%). Mean age was 76 years (25% of pts were older than 85 years) and dialysis vintage was 84 (2-963) months. Regarding comorbidities, 50% were diabetic, 40% had congestive heart failure, 25% had cancer and 17% had dementia. Mean age-adapted Charlson index was 13 ± 3. For 82 pts (51.9%), nephrologists would not be surprised if they died in the next 6 months. Seventy-three had hospital admissions in the previous three months. Four pts withdrew dialysis. Of those who died, only two had advance care directives, 18 had an opioid prescription in the last month of life and 17 were referred to palliative intervention mostly because of oncologic disorders. Seventy four percent of these pts died at the hospital and 23% died at home/nursing home. Not a single patient died under hospice care. Conclusion: Most of our patients commonly die in hospital, undergo invasive procedures, spend much of their remaining life fulfilling a demanding dialysis schedule and are admitted to the hospital wards without formal palliative intervention or discussion of goals of care, even when death is an expected outcome. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100017&lng=pt&nrm=iso&tlng=pt ABSTRACT Introduction: Hemodialysis catheter dysfunction compromises dialysis adequacy and may limit catheter survival. Strategies to prevent hemodialysis catheter dysfunction include catheter locks, generally either with heparin or citrate. Methods: Our hemodialysis unit converted to locking all central venous hemodialysis catheters with sodium citrate 4% instead of heparina 5000 U/mL. A retrospective analysis compared the outcomes of the 6 months prior and after the conversion. The compared outcomes were incidence of catheter thrombosis, catheter-related infection, flow-related catheter exchange rate, dialysis efficiency measured by Kt/V and catheter dysfunction. Results: Twenty-three patients were included. Between the two audit periods, the rate of catheter thrombosis was 0 (0 per 1000 catheter days) and 7 (2.78 per 1000 catheter days) during the heparin and citrate period respectively (p=0.109). The number of catheter exchanges due to catheter dysfunction was 0 (0 per 1000 catheter days) during the heparin period and just 1 (0.39 per 1000 catheter days) during the citrate period (p=0.317). Dialysis adequacy measured by KT/V was 1.54 (0.39) in the heparin group and 1.54 (0.35) in the citrate group (p=0.465). Catheter dysfunction was recorded in 146 sessions (12.3%) in the heparin period and in 159 sessions (14,7%) in the citrate period (p=0.234). There were no catheter related infections in either group. Conclusion: There was a 58% cost reduction associated with catheter-locking therapy between the heparin period and the citrate period. This cost reduction included the costs associated with alteplase use. Besides the financial advantages in switching to citrate locking, there are other potential advantages, namely the minimization of heparin related side effects or limitations. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100021&lng=pt&nrm=iso&tlng=pt ABSTRACT Introduction: Sleep disorders affect up to 70% of peritoneal dialysis (PD) patients and appear to have significant negative effects on their quality of life (QoL). We compared life and sleep quality between patients on automated PD (APD) and continuous ambulatory PD (CAPD). Material and Methods: Cross-sectional study in chronic PD patients followed in our Dialysis Unit in February 2022. We evaluated sleep quality with the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS) and QoL with EUROHIS-QOL-8. Results: Fifty-two patients, mostly men (65.4%, n=32), with a mean age of 52.7±14.2 years and PD vintage of 24.3±17.1 months. Our sample had similar distribution between APD and CAPD; we found no difference between groups regarding demographic data, PD-related quality parameters or prevalence of previous sleep disturbances. Mean QoL index was 69.9%±9.3% and similar between APD and CAPD patients; EUROHIS-QOL-8 negatively correlated with the scores on ESS (p=0.004) and PSQI (p=0.011). Regarding sleep quality, the mean ESS was 8.8±4.2 and PSQI was 7.6±3.6. APD patients had similar scores on ESS, but higher prevalence of severe daytime sleepiness (ESS≥18) (p=0.04) and higher scores on PSQI (p=0.002). Within the PSQI, sleep’ latency (p=0.003), duration (p=0.002) and efficiency (p=0.013) were the most affected parameters. Higher PSQI scores also correlated with worse blood pressure control; a cut-off value for PSQI of 6 was found to increase the risk of uncontrolled hypertension. Conclusion: In our series, APD patients had worse global sleep quality (PSQI) when compared to ACPD patients. This difference was mainly due to worse sleep latency, duration and efficiency. The assessment of the quality of life showed no difference between groups, however there was a clear link between quality of life and sleep quality. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100025&lng=pt&nrm=iso&tlng=pt ABSTRACT Dysfunction of the arteriovenous fistula for hemodialysis is a challenge for the nephrologist and the vascular surgeon. Although experience is limited, the use of the forearm basilic vein as outflow of the radiocephalic fistula has been considered. We report the case of a 60-year-old male, with cardiovascular comorbidities and a history of ischemic complications induced by the hemodialysis access. Drainage problems were present in the radiocephalic fistula and the forearm basilic vein was used in the vascular access repair. The procedure was successful, and the patient maintains a functional fistula, with secondary patency of 32 months, with no cardiac complications or hand access-induced distal ischaemia syndrome. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100028&lng=pt&nrm=iso&tlng=pt ABSTRACT Peritonitis is one of the main complications of peritoneal dialysis and accounts for considerable mortality and hospitalization. It is usually treated with intraperitoneal antibiotherapy, as it is the best way to achieve high concentrations at the site of infection. We report a case of an 86-year-old female patient with end-stage renal disease under continuous ambulatory peritoneal dialysis, who was admitted to our hospital with complaints of intense diffused abdominal pain. The effluent culture yielded Staphylococcus hominis only sensible to vancomycin and linezolid. She was diagnosed with peritonitis secondary to Staphylococcus hominis infection and treated with oral linezolid without cateter removal. Adequate linezolid peritoneal fluid concentration (between 3 and 20 μg/mL) was achieved and the antibiotic therapy was maintained for 21 days with clinical and analytical improvement. According to our knowledge, this was the first Staphylococcus peritonitis in a peritoneal dialysis patient, successfully treated with oral linezolid and has had linezolid levels monitored in the peritoneal dialysis effluent. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100031&lng=pt&nrm=iso&tlng=pt ABSTRACT Xanthogranulomatous pyelonephritis is an unusual variant of chronic pyelonephritis in which there is massive destruction of the renal parenchyma by granulomatous tissue. The pathophysiological mechanism is not yet fully understood, and the diagnosis is challenging. This is a rare and sporadic diagnosis in the pediatric population. Here, we report a case of a 2-year-old girl with a one-year history of urinary tract infections and recurrent cloudy urine with traces of sand. Several urinalyses demonstrated pyuria without bacteria growth, and she was admitted to the pediatric nephrology unit. Imaging tests were performed and a xanthogranulomatous pyelonephritis was diagnosed. After confirmation of functional exclusion of the right kidney, a successful right nephrectomy was performed, and the histology confirmed the diagnosis. This case highlights the importance of complementary tests, in a child with signs and symptoms of urinary tract dysfunction, to exclude any pathology requiring therapeutic intervention. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100036&lng=pt&nrm=iso&tlng=pt ABSTRACT A 43-year-old male patient followed for 27 years in a Nephrology consultation with negative autoimmune diagnostic workup and a histologically proven membranous nephropathy (MN), interpreted as a primary MN. During the follow-up he had multiple relapses, requiring continuous immunosuppression. Eventually, end-stage renal disease developed and the patient started hemodialysis (HD) in January 2018. Soon after initiating HD, he was admitted to the ward due to persistent fever of unknown origin, refractory to antipyretic and antibiotic therapy, but responsive to prednisolone (40 mg/day). Again, the diagnostic workup was negative and the patient was discharged with a corticosteroid taper over 16 weeks. In a posterior admission, after prednisolone suspension, the patient presented to the emergency room with persistente fever, involuntary weight loss, pruriginous macular rash and inflammatory joint pain. As the anti-nuclear antibody and anti-dsDNA came up positive and there was complement consumption, a diagnosis of a systemic lupus flare was established. His stay was complicated by sudden respiratory distress associated with hemoptysis and hemoglobin drop, with a confirmed diffuse alveolar hemorrhage. Daily plasma Exchange and intensive immunosuppression was necessary and the patient evolved into complete clinical remission with partial serological response. Usually, patients with systemic lupus erythematosus (SLE) develop clinical and serological remission upon initiating HD, reducing the number of flares and need for medication. This case depicts a lupus flare after starting HD, on a previously serologically negative patient, complicated with a rare type of lung parenchyma involvement, which is associated with a high mortality and required intensive immunosuppression for disease control. One can speculate whether or not the initial glomerulopathy was the first manifestation of SLE, previously controlled due to the continuous use of immunosuppression. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100040&lng=pt&nrm=iso&tlng=pt ABSTRACT Focal segmental glomerulosclerosis (FSGS) is a kidney histologic lesion that may be caused by multiple aetiologies and pathophysiological mechanisms, with podocyte injury and depletion as the common denominator. FSGS may be subdivided into different subclasses: primary, secondary, genetic and unknown forms. Notwithstanding the overlapping clinical and histological characteristics across the different forms of FSGS, their management and response to treatment are strikingly different. Genetic FSGS may be suggested by the appearance of nephrotic syndrome during childhood, but it may also present in adulthood, where the diagnosis is rather challenging due to widely variable clinical and histological phenotypes. Herein we present the case of a 34-year-old female with a family history of chronic kidney disease of undetermined aetiology, referred for a Nephrology consultation due to haematoproteinuria and de novo arterial hypertension. Complementary evaluation revealed a urinary protein/creatinine ratio of 4.3 g/g and albumin/creatinine ratio of 3.9 g/g with hypoalbuminaemia. Kidney biopsy revealed lesions of FSGS, associated with extensive foot process effacement. The constellation of findings and family history of kidney disease raised the suspicion of a genetic cause, therefore genetic testing was performed. Two variants in the NPHS2 gene [c.686G>A, p.(Arg229Gln) and c.855_856del, p(Arg286Thrfs*17)] were found in compound heterozygosity, compatible with the diagnosis of genetic FSGS. This case highlights the importance of a detailed evaluation of patients with FSGS lesions in order to identify the FSGS form, given its therapeutic and prognostic impact, including after kidney transplantation. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692023000100045&lng=pt&nrm=iso&tlng=pt ABSTRACT Focal segmental glomerulosclerosis (FSGS) is a kidney histologic lesion that may be caused by multiple aetiologies and pathophysiological mechanisms, with podocyte injury and depletion as the common denominator. FSGS may be subdivided into different subclasses: primary, secondary, genetic and unknown forms. Notwithstanding the overlapping clinical and histological characteristics across the different forms of FSGS, their management and response to treatment are strikingly different. Genetic FSGS may be suggested by the appearance of nephrotic syndrome during childhood, but it may also present in adulthood, where the diagnosis is rather challenging due to widely variable clinical and histological phenotypes. Herein we present the case of a 34-year-old female with a family history of chronic kidney disease of undetermined aetiology, referred for a Nephrology consultation due to haematoproteinuria and de novo arterial hypertension. Complementary evaluation revealed a urinary protein/creatinine ratio of 4.3 g/g and albumin/creatinine ratio of 3.9 g/g with hypoalbuminaemia. Kidney biopsy revealed lesions of FSGS, associated with extensive foot process effacement. The constellation of findings and family history of kidney disease raised the suspicion of a genetic cause, therefore genetic testing was performed. Two variants in the NPHS2 gene [c.686G>A, p.(Arg229Gln) and c.855_856del, p(Arg286Thrfs*17)] were found in compound heterozygosity, compatible with the diagnosis of genetic FSGS. This case highlights the importance of a detailed evaluation of patients with FSGS lesions in order to identify the FSGS form, given its therapeutic and prognostic impact, including after kidney transplantation.