Scielo RSS <![CDATA[Portuguese Journal of Nephrology & Hypertension]]> http://scielo.pt/rss.php?pid=0872-016920160004&lang=en vol. 30 num. 4 lang. en <![CDATA[SciELO Logo]]> http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt <![CDATA[<b>C3 glomerulopathies</b>: <b>A new category encompassing rare complement mediated glomerulonephritis</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400001&lng=en&nrm=iso&tlng=en <![CDATA[<b>BK virus nephropathy in kidney transplantation</b>: <b>A literature review following a clinical case</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400002&lng=en&nrm=iso&tlng=en 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 <![CDATA[<b>Multiple myeloma and high cut-off haemodialysis</b>: <b>On the right track for better outcomes?</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400003&lng=en&nrm=iso&tlng=en Background: Acute kidney injury secondary to cast nephropathy is a common complication of multiple myeloma. Extracorporeal light chain elimination by high cut-off haemodialysis has been described as an adjuvant to effective chemotherapy to limit free light chain toxicity. The purpose of this study was to evaluate the impact of high cut-off haemodialysis and bortezomib-based chemotherapy on renal function recovery and overall survival in a cohort of patients with multiple myeloma and dialysis-dependent acute kidney injury. Methods: We did a historical cohort study of patients with multiple myeloma and dialysis-dependent acute kidney injury presenting to our Centre between the 1st January 1999 and 31st March 2013. Results: Forty-six patients were included, with a median age of 68 (56-73 Y) years old. Twenty-four per cent recovered renal function. Patients submitted to high cut-off haemodialysis had a significantly higher probability of renal function recovery (OR = 11.5; 95% IC: 1.0 to 126.5). Seventy-two per cent of the patients died. The median survival rate was 20 months and overall 1-year survival rate was 58.3%. Male sex was associated with worse overall survival (HR = 4.9; 95% CI: 2.0-12.3). Renal function recovery decreased the risk of death (HR = 0.24; 95% CI: 0.07-0.80) as compared with those who remained on dialysis. The use of HCOH had no influence on the risk of death. Conclusions: Adding high cut-off haemodialysis to the novel anti-myeloma agents was independently associated to better renal outcomes in patients with multiple myeloma and dialysis-dependent acute kidney injury. However, our confidence in these results is hampered by the observational nature of the study, and by the small sample size and imprecise estimates of effect. Randomized controlled trials addressing this issue are urgently needed <![CDATA[<b>An Automated Peritoneal Dialysis regimen is not detrimental to Residual Renal Function</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400004&lng=en&nrm=iso&tlng=en Background Residual Renal Function (RRF) preservation is related to survival in Peritoneal Dialysis (PD) patients. The effect of different PD modalities on RRF is unclear. Objectives: To analyse the evolution of RRF function in patients in PD, maintained with automated peritoneal dialysis (APD) and continuous ambulatory peritoneal dialysis (CAPD), and study other possible factors related to RRF decline. Methods: A single-centre retrospective study with 104 incident PD patients (48 CAPD and 56 APD). Patients with no RRF function at PD beginning were excluded. The mean age was 52.1±16 years, 70% were male and 16% diabetics. Thirteen patients used low glucose degradation product (GDP) solutions and 57 patients were using icodextrin. The use of diuretics, angiotensinconverting enzyme inhibitors and angiotensin II receptor blockers was also analysed. RRF and diuresis were analysed every 3 months and peritoneal transport every 6 months, forl a total of 48 months. The mean follow-up was 29.3±19 months. Results: CAPD patients were older, had higher prevalence of diabetes, used less icodextrin and more low GDP solutions and were followed for a longer time. No significant differences in RRF were observed in either modality in basal or during the follow-up: RRF at 24 months with 3.67±3.5ml/min in CAPD patients and 3.82±2.5ml/min in APD patients; or in diuresis: 883±807ml/day and 1333±905ml/day respectively. Neither group showed significant differences in peritoneal transport parameters over time. The use of icodextrin was related to a higher diuresis preservation at 24 months: 1519±1035ml/day in patients using icodextrin and 767±633ml/day in patients without icodextrin (p=0.01). No differences were found in RRF evolution in either group. Conclusions: We conclude that DP modality does not influence either the RRF function or diuresis outcomes or peritoneal transport parameters. The initial use of icodextrin was related to better diuresis preservation without changes in RRF <![CDATA[<b>Exit-site fungal infections</b>: <b>experience of a Peritoneal Dialysis Unit</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400005&lng=en&nrm=iso&tlng=en Introduction: Exit-site infections are an important complication of peritoneal dialysis; however, very little is known about fungi-related exit-site infections. The literature is very sparse and there are virtually no studies that report it. Objective: To evaluate in retrospect the risk factors associated with exit-site fungal infection and its development. Methods: The study included all diagnosed episodes of exit- site fungal infections in patients undergoing peritoneal dialysis in a hospital unit between 2011 and 2014, analyzed in relation to demographic, clinical and analytical variables. Results: The studied group included 26 patients, 70% of which were female, with a median age of 54 years old; average length in PD treatment three years. The majority of patients (70%) were undergoing manual peritoneal dialysis. About 30 episodes of exit-site fungal infections were diagnosed during the follow-up period, which corresponded to 5.6% of total exit-site infections diagnosed. About 23% of patients suffered from diabetes mellitus and 13% had undergone immunosuppressive therapy in the six months previous to the episode. Two thirds of patients (n=20) had undergone antibiotherapy in the previous three months, the majority (65%) for the treatment of peritoneal dialysis-related infection and 46% of these patients underwent concomitant antifungal prophylaxis with fluconazole. The exit-site fungal infections were predominantly caused by Candida parapsilosis (67%). Eighty per cent of patients (n=24) improved with antifungal therapy (fluconazole or itraconazole), and the remaining patients underwent peritoneal catheter replacement (n=3) or removal (n=3) due to persistent infection. Exit-site fungal infections were the cause of technique failure in 2 of these patients. Conclusion: Exit-site fungal infections are an unusual but important complication of peritoneal dialysis. In this study, the most relevant risk factor associated with these infections was previous antibiotherapy and the majority of patients evolving favourably under medical treatment <![CDATA[<b>Cancer and End-Stage Kidney Disease</b>: <b>A Death Sentence?</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400006&lng=en&nrm=iso&tlng=en Background: End-stage kidney disease is associated with considerable morbidity and mortality, a feature that is shared with malignant neoplasms. Hence, patients with the cumulative effect of these two diseases frequently give rise to the question of whether dialysis should be implemented. The primary goal of this study was to characterize the clinical progression and evaluate the outcome of a group of oncology patients on chronic haemodialysis and also to identify the characteristics associated with prolonged survival. Methods: Retrospective analysis of all patients on the chronic haemodialysis programme in an oncology hospital-based haemodialysis centre between January 1991 and September 2014. Results: 141 patients were treated during this period. The main aetiologies for end-stage kidney disease were multiple myeloma (24.8%) and chronic interstitial disease (22.7%), while the most common tumours were genitourinary cancer (47.5%) and multiple myeloma (24.8%). Multiple tumours were present in 22.0% of patients and 19.2% harboured metastatic disease. Overall, 66.7% of patients died during this period; 7.8% were transferred to other centres as a result of clinical stability; 4.3% recovered renal function; 1.4% received a kidney transplant and 19.9% were still alive at the end of the study. Overall survival was 58.8% at 2 years and 34.8% at 5 years. Multiple myeloma (HR=5.950; 95% CI: 2.512-14.092) and gastrointestinal cancers (HR=3.277; 95% CI: 1.176-9.134) were associated with increased likelihood of death. Conclusions: Survival among patients with often locally advanced or metastatic oncological disease on chronic haemodialysis was unexpectedly high, with 1/3 still alive at 5 years. Accordingly, decision-making in the cancer <![CDATA[<b>A rare malignancy in the post-transplant period</b>: <b>Myeloma cast nephropathy</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400007&lng=en&nrm=iso&tlng=en Myeloma-associated renal disorders are rare events among renal transplants and can occur as recurrent or de novo disease. We describe an unusual case of renal allograft dysfunction due to myeloma cast nephropathy occurring 19 years after having received a renal transplant in a patient with no prior history of multiple myeloma or monoclonal gammopathy preceding transplantation. Our patient was treated with five cycles of chemotheraphy (bortezomib, melphalan and steroids), which resulted in short-term improvement in allograft dysfunction and complete haematological remission. The longer patient and graft survival after renal transplantation make post-transplant lymphoproliferative disease more frequent. Multiple myeloma after kidney transplant is rare and an elevated index of suspicion is necessary to make a timely diagnosis of this serious disease. Further work is needed to identify the best treatment options for these patients <![CDATA[<b>Acute kidney injury due to Q fever</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400008&lng=en&nrm=iso&tlng=en Coxiella burnetii (C. burnetii) causes a zoonotic disease - Q fever. This bacterium is highly resistant to harsh environmental conditions and causes an uncharacteristic clinical syndrome. Q fever may be acute or chronic and renal manifestations of the disease are more common in the chronic forms. It is reported a case of a 83 -year old woman, with previous normal renal function and a medical history of arterial hypertension and osteoarthritis. She presented with leg oedema, acute kidney injury (serum creatinine 4.14 mg/dl), and an urine protein-to-creatinine ratio of 9.14 gr/gr. A diagnosis of acute kidney injury with nephrotic syndrome was admitted. The lab work revealed a decrease in serum complement levels (C3, C4), and elevated serum levels of β2 -microglobulin and IgM anticardiolipin. Renal ultrasound showed bilateral cysts, so a renal biopsy could not be performed. During hospital stay, renal function worsened with oliguria and the patient needed transient haemodialysis. The renal function gradually recovered but the nephrotic syndrome (ratio 18gr/gr) persisted, with a thrombotic complication (deep vein thrombosis and pulmonary emboli). She presented a fever of unknown origin (FUO) and was treated with several antibiotic courses, eventually becoming afebrile. She was discharged with a serum creatinine 0,89mg/dL and a proteinuria of 18g/24H. By then, we had obtained the result of a positive serology for C. burnetii, so doxycycline was started and proteinuria (0,3gr/gr) remitted. Currently, the patient remains with high levels of C. burnetii antibodies and is still under treatment with doxycycline. The authors report a case of acute Q fever manifested by recurrent fever and acute kidney injury with nephrotic syndrome. This case illustrates a rare aetiology of nephrotic syndrome with acute kidney injury <![CDATA[<b>Type II mixed cryoglobulinaemia due to hepatitis C virus infection</b>: <b>The role of new direct-acting antivirals in a kidney transplant recipient</b>]]> http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692016000400009&lng=en&nrm=iso&tlng=en Hepatitis C virus (HCV) infection remains prevalent in chronic kidney disease (CKD) patients. In a posttransplant patient, it can increase the risk for several complications such as transplant glomerulopathy and cryoglobulinaemia. We describe a 69 year-old woman with chronic kidney failure secondary to autosomal dominant polycystic kidney disease (ADPKD) on haemodialysis since January 1989. She had HCV infection (genotype 1b) diagnosed within the dialysis period. Liver biopsy revealed signs of chronic hepatitis. Ten years later, she underwent a deceased donor kidney transplant. She maintained normal kidney allograft function and subnephrotic proteinuria. In July 2015, she was hospitalized with necrotic ulcers in both legs. Laboratory findings revealed type II cryoglobulinaemia and low complement levels. HCV viraemia was high. Cutaneous biopsy showed a leukocytoclastic vasculitis. It was decided to treat cryoglobulinaemia and HCV infection with new direct-acting antivirals agents (DAAs) sofosbuvir/ledipasvir during 12 weeks. Skin ulcers improved and HCV RNA was undetectable after 4 weeks of treatment. Patient showed good tolerance for drug regimen. Proteinuria increased to nephrotic range after DAAs initiation. Donor-specific antibodies class I and II were negative and a kidney allograft biopsy demonstrated features of focal segmental glomerulosclerosis. Exacerbation of proteinuria in our patient could be a possible adverse effect of DAAs therapy, rarely reported in other cases