Scielo RSS http://scielo.pt/rss.php?pid=0872-016920210001&lang=es vol. 35 num. 1 lang. es http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100003&lng=es&nrm=iso&tlng=es http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100006&lng=es&nrm=iso&tlng=es http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100011&lng=es&nrm=iso&tlng=es ABSTRACT Hematological malignancies (HM) confer a high risk of acute kidney injury (AKI), which is associated with elevated morbi-mortality. The aim of this study was to identify the prognostic factors for in-hospital mortality and one-year mortality in this population. We conducted a single center, retrospective, observational cohort study of 101 in-hospital patients with AKI and HM between January 2015 and December 2019. Multiple myeloma was present in 30.7% of the patients, followed by non-Hodgkin lymphoma (NHL) in 27.7%. Renal support therapy (RST) was needed in 60.4% of the cases. Independent predictors for in-hospital mortality were invasive mechanical ventilation (IMV) (OR 49.53; 95% CI:9.17 - 267.57; P<0.001) and infection during in-hospital stay (IDHS) (OR 5.09; 95% CI:1.18 - 21.89; P=0.029). Predictors for one-year mortality were NHL (HR 2.88; 95% CI:1.54 - 5.39; P=0.001), tumor progression (HR 2.36; 95% CI:1.29 - 4.32; P=0.006) and IMV (HR 6.38; 95% CI:3.50 - 11.64; P<0.001). Higher albumin levels at AKI diagnosis conferred a better prognosis (HR 0.57; 95% CI:0.35 - 0.91; P=0.020). Our model showed that patients with HM and AKI who were submitted to IMV and had IDHS had a probability of in-hospital death of 96%. Albumin at the time of AKI influenced one-year mortality. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100018&lng=es&nrm=iso&tlng=es ABSTRACT Background: Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) have been introduced as useful inflammatory markers to predict the outcome of a wide spectrum of diseases, such as malignancies and cardiovascular pathologies. Limited evidence is available for their role in end-stage renal disease and dialysis patients. The aim of this study was to evaluate NLR and PLR as predictors of mortality in peritoneal dialysis (PD) patients. Methods: In this retrospective study 122 incident PD patients between 2004 and 2019 were included. Demographic, clinical and laboratory data were collected. Relationships between NLR, PLR and high-sensitivity C-reactive protein (hs-CRP) were evaluated by Spearman correlation test. Univariable and multivariable Cox regression analysis were performed to determine the association of NLR and PLR with all-cause mortality. Results: Mean levels of NLR and PLR were 3.99±2.6 and 195.5±101.7, respectively. Both NLR and PLR were significantly and positively correlated with serum hs-CRP levels (r=0.340, p<0.001 and r=0.360, p<0.001, respectively). The overall mortality rate was 18.9% after a mean follow-up of 30.2±24.0 months. On multivariable modeling, we found that higher NLR (HR=1.662, 95%CI 1.117-2.472) and higher PLR (HR=1.010, 95%CI 1.004-1.015), in addition to lower residual renal function and higher Charlson comorbidity index were significant independent predictors of poor survival, when adjusted for nutritional status. Discussion: In this study, NLR and PLR were validated as inflammatory markers and predicted survival in our PD patients. Our results suggest that NLR might be a better indicator of mortality than PLR. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100022&lng=es&nrm=iso&tlng=es ABSTRACT Background: There is an ongoing increasing focus on person‑centered individualized dialysis prescription. For this model, incremental peritoneal dialysis has been established as a strategy to start peritoneal dialysis, with an intention to increase the dose of peritoneal dialysis, as a consequence of renal clearance declines. In spite of being broadly accepted, the evidence of non‑inferiority of the incremental approach in comparison with full dose peritoneal dialysis is weak. To disclose the possibility and safety of an incremental approach in incident patients, we assessed patients and technique survival (main outcomes) between incremental and full dose peritoneal dialysis. We also compared the effects in the clinical parameters of adequacy, urinary output and peritonitis incidence in both approaches. Methods: Following a retrospective design, we undertook an observational study in our center over 20 years. We investigated 106 patients divided into two groups, according to initial peritoneal dialysis strategy (incremental or full dose). We used multivariate multinomial model to assess predictors of peritonitis. The main outcomes were studies using a competing risk model. Results: One year after peritoneal dialysis start, our data disclosed statistically significant differences of phosphatemia and solute removal between full dose and incremental approach, favoring the latter. In an incremental approach, two or more peritoneal infections and drop out to hemodialysis were less usual. Conclusions: Incremental peritoneal prescription seems to be a good choice to start peritoneal dialysis. Potential benefits and, above all, safety reinforce the adoption of incremental peritoneal dialysis in incident patients as a strategy of individualized care, in compliance with new guidelines. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100029&lng=es&nrm=iso&tlng=es ABSTRACT Fabry disease (FD) is a rare systemic disease, with a large spectrum of disease severity. A GLA gene mutation in X‑chromosome leads to progressive accumulation of globotriaosylceramide (Gb3) in various organs. We screened all patients under dialysis from a single center for GLA gene changes. Enzymatic activity of alpha galactosidase A (α‑Gal A) and concentration of lyso‑Gb3 were determined in dried blood spots. Genetic study was performed in male patients with low α‑Gal A activity and in all female subjects. For all positive patients, a complete family study was performed. A total of 72 dialysis patients were screened. Sequence analysis was carried out in 53 patients (25 males). Heterozygous variants of the GLA gene were found in 4 patients (7.5%): c.937G>T (D313Y) in exon 6; c.352C>T (R118C) in exon 2; c.870G>C (M290I) in exon 6 and c.580A>G (T194A) in exon 4. Family screening was performed in a total of 17 subjects, with a GLA genetic variant prevalence of 58.8%. Unlike p.D313Y and p.R118C, well‑known non‑pathogenic polymorphisms, p.M290I is a controversial poorly described mutation. Reports about its phenotypic expression are crucial for a better understanding of its behavior. The recognition of the novel mutation p.T194A is importante for better knowledge of FD and its spectrum of clinical manifestations. These affected patients are expected to develop a classic and life‑threatening FD phenotype and an early diagnosis is essential for their treatment success. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100035&lng=es&nrm=iso&tlng=es ABSTRACT Metabolic acidosis is a usual complication of progressive chronic kidney disease and is associated with morbidity and mortality. The correction of metabolic acidosis is a main goal of dialysis. In prevalent hemodialysis patients, acid-base homeostasis depends on many factors, mainly net acid production, amount of alkali given by the dialysate bath and duration of the interdialytic period. At present, the main concern in prevalent hemodialysis patients is not over patients with metabolic acidosis, but rather about the growing numbers of patients with metabolic alkalosis. Several large cohort studies have shown significant associations between high predialysis bicarbonate, as well as blood pH, and morbidity and mortality risk. Based on recent guidelines, we should keep predialysis serum bicarbonate concentrations at 24-26 mEq/L, although this is opinion-based and there is no consensus on the issue. Furthermore, there should be specific focus on the patient´s nutritional status and reversible comorbidities. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100039&lng=es&nrm=iso&tlng=es ABSTRACT Nephrotic syndrome in systemic lupus erythematosus patients with histological evidence of minimal change disease, mesangial proliferation or focal and segmental glomerulosclerosis on light microscopy, represents a distinct clinical entity - lupus podocytopathy. This entity is characterized by a diffuse foot process effacement on electron microscopy and by absence of subepithelial or subendothelial immune‑complex deposition. We report the case of a 49‑year‑old woman admitted on suspicion of lupus nephritis flare, characterized by nephrotic syndrome and acute kidney injury, whose renal biopsy revealed histological features of lupus podocytopathy. Six months after discharge, under prednisolone and azathioprine, she presented 300 mg/day proteinuria, normal kidney function, without hematuria. A review of the pathogenesis, clinical features, diagnostic criteria, treatment and prognosis of lupus podocytopathy is provided. This case highlights the mounting evidence that lupus podocytopathy encompasses distinct clinical and morphological features, that should be included in the upcoming pathological classification of lupus nephritis. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100043&lng=es&nrm=iso&tlng=es ABSTRACT Introduction: Ammonia is a resultant molecule from the protein metabolism that can be neurotoxic when present in high concentrations, causing brain edema and encephalopathy. Extracorporeal blood purification technique (EBPT) can play a role in reducing both ammonia and urea blood levels. However, there are no specific guidelines for its use in hyperammonemia, due to its low incidence and scarce literature on this subject, particularly in adults. Case report: The authors describe a case of a 22‑year‑old previously healthy woman. She presented with polyuria, polydipsia, anorexia, vomiting and significant (>10%) weight loss over a month. Laboratory data revealed hypoglycemia and a high anion gap metabolic acidosis with severe acidemia with normal lactate, serum creatinine of 1.12 mg/dl and 260 (reference value 26‑74)umol/l of ammonia. Urine analysis showed ketone bodies. She evolved with progressive encephalopathy and neurological deterioration. The hypothesis of hyperammonemic encephalopathy secondary to a hereditary metabolic disease was suspected. Medical supportive therapy was initiated. An EBPT for ammonia removal and acid‑base correction was initiated using continuous venovenous hemodiafiltration (CVVHDF) with a drastic neurologic improvement. A fatty acid beta‑oxidation deficit was discovered. The patient was discharged with no neurological changes, with a strict diet free of fat, animal protein and high‑protein vegetables, plus supplementation with benzoate, carnitine, riboflavin, maltodextrine and essential amino acids. Discussion and conclusion: In adults, hyperammonemia related to metabolic disorders is rare, and in the presence of encephalopathy, ECDT should be considered. Ammonia is easily dialysable either by intermittent or continuous techniques. CVVHDF provides a continuous clearance with less rebound effect. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100047&lng=es&nrm=iso&tlng=es ABSTRACT Systemic lupus erythematosus and ANCA-associated vasculitis overlap syndrome is rare and complex. Moreover, the mechanisms that explain the interaction between these two conditions are still unclear. The authors describe the case of a patient who had myeloperoxidase ANCA-associated vasculitis as the initial diagnosis, with biopsy-proven pauci-immune crescentic glomerulonephritis, attaining complete remission after immunosuppression. Five years later, Systemic Lupus Erythematosus was diagnosed and a second kidney biopsy showed a pattern of lupus nephritis class III. While on immunosuppression treatment, the patient developed a progressive elevation of liver enzymes and was later diagnosed with primary biliary cholangitis. It seems that in this case, the overlap of Systemic Lupus Erythematosus and ANCA-associated vasculitis may be part of a poliautoimmune syndrome suggested by association with a third autoimmune disease. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051&lng=es&nrm=iso&tlng=es ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organs. When the kidney is involved it is generally termed lupus nephritis (LN), and is a major contributor to the morbidity and mortality associated with SLE. The classic pattern of lupus nephritis in a renal biopsy is an immune complex mediated glomerulonephritis. The presence of crescents, in diffuse proliferative lupus nephritis, and serologic positivity for antineutrophil cytoplasmatic antibodies (ANCA), more often antimyeloperoxidase than anti-proteinase 3, has been linked with specific clinicopathological features, poor treatment response and a worse kidney survival. Clinical case: We present the case of a 19-year-old male, without relevant past medical history, who presented severe headaches, hypertension and peripheral edema. The blood analysis revealed hemoglobin (Hb) 7.6 g/dL, creatinine (Cr) 2.64 mg/dL, blood urea nitrogen (BUN) 101 mg/dL, and urine analysis, hematuria and nephrotic proteinuria. The autoimmunity panel results were consistent with SLE paired with positivity for ANCA-proteinase 3 antibody. A renal biopsy revealed crescentic glomerulonephritis with fibrinoid necrosis and Bowman capsule rupture. The patient was diagnosed with class IV LN. The initiation treatment consisted of cyclophosphamide (CIPH) and prednisolone (PDN). At the 6-month follow-up, CIPH was stopped and mycophenolate mofetil (MMF) initiated as maintenance therapy, combined with PDN. Although microscopic hematuria and C3 consumption were still present (a new biopsy was pondered but the patient refused any further invasive diagnostic measures), C1q levels decreased from 26.6 to 19.3 U/ml (negative if < 20 U/ml) and anti-dsDNA was also negative with progressive declination of the degree of proteinuria. The creatinine levels returned to normal. Conclusion: The authors emphasize the importance of this class IV LN, influenced by the association with anti-proteinase 3, due to the implications in the histopathological pattern and in therapy selection. In this specific case kidney function, proteinuria and lupus activity had an important decrease without significant complications with the chosen treatment. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100055&lng=es&nrm=iso&tlng=es ABSTRACT Introduction: Antineutrophil cytoplasmic autoantibodies (ANCAs) are crucial in the pathogenesis of ANCA-associated glomerulonephritis. Although highly specific for this disease, these antibodies can appear in healthy individuals or occur in other settings such as malignance, other inflammatory disease, or drugs. Renal biopsy remains essential for diagnosis and as a prognostic tool. Failing to perform a biopsy risks an incomplete evaluation or misdiagnosis. Clinical case: A 60-year-old male known to have had previous normal kidney function was admitted with generalized pitting edema, nonpruritic erythematous rash, and decreased urine output. He had been recently started on clozapine for depression. Laboratory tests revealed nephrotic syndrome with massive proteinuria (11g/day), hematuria and elevated serum creatinine that reached a maximum of 4.9 mg/dl. The only positive finding was a high titer of myeloperoxidase (MPO) ANCA antibody (111 UI/ml). Clozapine was discontinued, and he was started on conservative management of the nephrotic syndrome. The rash disappeared within one week. Kidney biopsy showed cellular variant focal segmental glomerulosclerosis (FSGS) with mild acute tubular injury. The patient was treated with oral prednisolone with complete remission. Repeat MPO-ANCA was negative after one month. Discussion: We present a challenging case that highlights the importance of kidney biopsy for accurate diagnosis with major impact on treatment and prognosis. This case also underlines the presentation and evolution of FSGS cellular variant, a rare and poorly understood disease. The relationship between skin rash with high MPO titer and the administration of clozapine with clinical remission after its discontinuation favors the hypothesis of drug associated ANCA-vasculitis. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100059&lng=es&nrm=iso&tlng=es ABSTRACT Introduction: Antineutrophil cytoplasmic autoantibodies (ANCAs) are crucial in the pathogenesis of ANCA-associated glomerulonephritis. Although highly specific for this disease, these antibodies can appear in healthy individuals or occur in other settings such as malignance, other inflammatory disease, or drugs. Renal biopsy remains essential for diagnosis and as a prognostic tool. Failing to perform a biopsy risks an incomplete evaluation or misdiagnosis. Clinical case: A 60-year-old male known to have had previous normal kidney function was admitted with generalized pitting edema, nonpruritic erythematous rash, and decreased urine output. He had been recently started on clozapine for depression. Laboratory tests revealed nephrotic syndrome with massive proteinuria (11g/day), hematuria and elevated serum creatinine that reached a maximum of 4.9 mg/dl. The only positive finding was a high titer of myeloperoxidase (MPO) ANCA antibody (111 UI/ml). Clozapine was discontinued, and he was started on conservative management of the nephrotic syndrome. The rash disappeared within one week. Kidney biopsy showed cellular variant focal segmental glomerulosclerosis (FSGS) with mild acute tubular injury. The patient was treated with oral prednisolone with complete remission. Repeat MPO-ANCA was negative after one month. Discussion: We present a challenging case that highlights the importance of kidney biopsy for accurate diagnosis with major impact on treatment and prognosis. This case also underlines the presentation and evolution of FSGS cellular variant, a rare and poorly understood disease. The relationship between skin rash with high MPO titer and the administration of clozapine with clinical remission after its discontinuation favors the hypothesis of drug associated ANCA-vasculitis.