Scielo RSS http://scielo.pt/rss.php?pid=0872-016920190002&lang=pt vol. 33 num. 2 lang. pt http://scielo.pt/img/en/fbpelogp.gif http://scielo.pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200001&lng=pt&nrm=iso&tlng=pt http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200002&lng=pt&nrm=iso&tlng=pt Chronic kidney disease is increasing in prevalence, especially among older people. The development of integrated strategies between primary health care and hospital care is crucial to curb the growth of this pathology, already considered a public health problem. Telemedicine approaches the different levels of care and creates a great confidence in managing CKD in the community, with a beneficial and real patient-centered care. Other strategies that lead to empowerment of the primary care medical doctors should also be considered. This manuscript summarizes some of these aspects and lists the benefits of its implementation in Portugal. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200003&lng=pt&nrm=iso&tlng=pt Introduction and Aim:Hepatitis C virus screening using anti-HCV antibody is recommended for end-stage renal disease and chronic hemodialysis patients. Since the anti-HCV antibody test is not able to differentiate a concurrent infection from a resolved past one, a more reliable and cost-effective screening test is needed. Materials and Methods: Prospective study of end-stage renal disease and chronic hemodialysis patients from a hospital unit who were screened by anti-HCV antibody and total ARCHITECT® HCV antigen assay. Results: Cohen's κ test was run to determine if there was agreement between HCV Ribonucleic Acid and HCV core antigen assays on 7 individuals with positive anti-HCV antibody. The two tests had a perfect measurement agreement. Conclusions: HCV core antigen testing could be used as a sensitive method for HCV infection screening in this group of patients based on its accuracy, simplicity and low expense http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200004&lng=pt&nrm=iso&tlng=pt Introduction and aims: The complement system may play a part in the pathogenesis of amyloid disorders. The primary aim of the study was to analyse the relationship between transthyretin renal amyloidosis (ATTR) and the complement system. The secondary aim was to find potential clinical and morphologic features in ATTR amyloidosis associated with complement activation. Methods: We performed a retrospective cohort observational study in patients with renal amyloidosis submitted to kidney biopsy and complement measurements from January 2005 to June 2016. We compared clinical and laboratory results in different types of amyloidosis using the Kruskal-Wallis or Fisher's exact test. We performed a subgroup analysis in ATTR amyloidosis patients, comparing patients with normal and low complement serum levels in terms of clinical, laboratory, and morphologic characteristics, using the Mann-Whitney test or Fisher's exact test. Results: We included 42 patients in the analysis: 15 patients had ATTR amyloid deposits, 16 AA amyloid deposits and 11 AL amyloid deposits (all AL λ). A total of ten patients presented low C3 (6 ATTR; 3 AL; 1 AA). None of the patients had low C4 serum levels. Of the 15 Caucasian patients with renal ATTR amyloidosis, 6 (40%) presented low C3. Those patients had a higher sclerotic glomeruli number and 5 out of 6 had C3 deposits in immunofluorescence. Discussion: In our study, 40% of patients with renal ATTR amyloidosis showed complement consumption or activation, which led us to suspect that ATTR deposits can lead to systemic activation of the alternative complement pathway. It remains to be elucidated if this activation injures peripheral nerves and the kidney. Conclusions: C3 determinations can prove to be a useful tool for predicting renal involvement and glomerulosclerosis in TTR amyloidosis patients. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200005&lng=pt&nrm=iso&tlng=pt Introduction and aims: The complement system may play a part in the pathogenesis of amyloid disorders. The primary aim of the study was to analyse the relationship between transthyretin renal amyloidosis (ATTR) and the complement system. The secondary aim was to find potential clinical and morphologic features in ATTR amyloidosis associated with complement activation. Methods: We performed a retrospective cohort observational study in patients with renal amyloidosis submitted to kidney biopsy and complement measurements from January 2005 to June 2016. We compared clinical and laboratory results in different types of amyloidosis using the Kruskal-Wallis or Fisher's exact test. We performed a subgroup analysis in ATTR amyloidosis patients, comparing patients with normal and low complement serum levels in terms of clinical, laboratory, and morphologic characteristics, using the Mann-Whitney test or Fisher's exact test. Results: We included 42 patients in the analysis: 15 patients had ATTR amyloid deposits, 16 AA amyloid deposits and 11 AL amyloid deposits (all AL λ). A total of ten patients presented low C3 (6 ATTR; 3 AL; 1 AA). None of the patients had low C4 serum levels. Of the 15 Caucasian patients with renal ATTR amyloidosis, 6 (40%) presented low C3. Those patients had a higher sclerotic glomeruli number and 5 out of 6 had C3 deposits in immunofluorescence. Discussion: In our study, 40% of patients with renal ATTR amyloidosis showed complement consumption or activation, which led us to suspect that ATTR deposits can lead to systemic activation of the alternative complement pathway. It remains to be elucidated if this activation injures peripheral nerves and the kidney. Conclusions: C3 determinations can prove to be a useful tool for predicting renal involvement and glomerulosclerosis in TTR amyloidosis patients. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200006&lng=pt&nrm=iso&tlng=pt Chronic kidney disease is highly prevalent in patients with diabetes mellitus. There are many specific aspects in the treatment of diabetes that have to be taken into account when there is concomitant nephropathy. All antihyperglycemic drugs can be used in earlier stages of chronic kidney disease. With worsening nephropathy, most will require dose adjustments (some eventually suspension) and increased monitoring of adverse events and kidney function. New treatment options that are safe and effective are now available for more advanced stages of disease. Moreover, findings from large clinical trials suggest that some drugs, namely GLP-1 receptor agonists and SGLT2 inhibitors, might potentially have kidney and cardiovascular protective effects, although clinical significance and putative mechanisms are not yet fully understood. The aim of this review is to provide an updated overview of relevant data to guide clinical practice in the use of antihyperglycemic agents in chronic kidney disease patients, including older drugs and also the most recently available treatment options. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200007&lng=pt&nrm=iso&tlng=pt Cardiovascular diseases (CVD) remain the leading cause of morbimortality globally. Despite substantial improvement in outcomes, alarming increases in obesity, diabetes mellitus and other risk factors have been noted in recent years. Despite the majority of CVD being preventable and primary prevention being cost-effective, preventive approaches are poorly implemented in the population at large as well as in individual patients. The pillar of prevention is lifestyle changes (diet, weight, smoking and exercise) followed by, when appropriate, targeting of the main cardiovascular risk factors with pharmacotherapy: lipid lowering therapy, blood-pressure treatment and blood glucose control. Low-dose aspirin as “one-dose-fits-all strategy” remains controversial as primary prevention due to the increased bleeding risk. New scoring systems or further application of imaging techniques (for example, coronary calcium score) are necessary for better risk stratification. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200008&lng=pt&nrm=iso&tlng=pt The passenger lymphocyte syndrome may cause hemolytic anemia after bone marrow and solid organ transplantation when there is minor erythrocyte antigen incompatibility in the donor/recipient pair. Anemia is usually self-limiting, frequently related to ABO antibodies and rarely to Rh mismatches. We report a rare case of passenger B lymphocyte syndrome mediated by Rh antibodies occurring after a donor living renal transplantation and discuss its clinical severity at presentation, the therapeutic decisions and their outcome. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200009&lng=pt&nrm=iso&tlng=pt We report a case of myeloma-like cast nephropathy in a 27-year-old male patient with human immunodeficiency virus (HIV) infection for 2 years. He was on highly active anti-retroviral therapy (HAART) and presented with high grade fever, nausea and vomiting for 2 weeks. On investigations, he was found to have acute kidney injury (AKI). He was started on hemodialysis. Renal biopsy revealed acute tubulointerstitial nephritis with myeloma-like casts in a few tubular lumens. Detailed workup for multiple myeloma was negative. No evidence of monoclonal gammopathy was found. Finally, he was diagnosed as a case of myeloma-like cast nephropathy. With supportive treatment and modification in HAART therapy, his renal functions improved and he became dialysis free in 2 weeks. He was continued on HAART therapy, but he soon discontinued this against medical advice. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200010&lng=pt&nrm=iso&tlng=pt IgA dominant glomerulonephritis associated to Staphylococcus infection is a rare clinical entity that has been described mainly in case reports. Biopsy features can resemble other disease entities mainly IgA nephropathy and Henoch-Schonlein purpura nephritis. Treatment of IgA dominant glomerulonephritis associated to staphylococcal infection is based on antibiotics for the underlying infection, controlling hypertension and edema and may resort to concomitant use of steroids in selected cases. Prognosis markers such as hypertension, diabetes and interstitial fibrosis may influence treatment as they are associated with poor renal outcomes. We report a case of a 63-year-old man with known hypertension, pre-diabetes and recent history of methicillin‐sensitive Staphylococcus aureus bacteremia associated to prostatitis, who presented with a one-month history of edema, arthralgia and foamy urine. Over this period he progressed to anasarca and nephrotic range proteinuria with concomitant rise in creatinine levels being documented. The renal biopsy showed segmental endocapillary proliferation and IgA segmental dominant staining associated to C3 and lambda in minor distribution. On completion of two months of steroid therapy the patient partially recovered his renal function and proteinuria. After nine months of tapering steroids, he presented with acute inflammatory arthritis supporting an inflammatory background disease. To our knowledge this case describes an unusual entity such as IgA dominant glomerulonephritis associated to staphylococcal infection co-presenting with an associated reactive arthritis. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200011&lng=pt&nrm=iso&tlng=pt Nephronophthisis is an autosomal recessive cystic kidney disease characterized by reduced concentrating ability of the kidney, chronic tubulointerstitial nephritis, cystic renal disease and progression to end-stage renal disease. In 10-20% of cases it is associated with a variety of other ciliopathy phenotypes. A 10-year-old caucasian female patient presented to our hospital with a history of uncontrolled vomiting and signs of moderate dehydration. Past medical history was significant for reduced visual acuity and moderate intellectual disability with a minor cerebellar dysplasia. She also complained of polyuria, polydipsia and enuresis for several years. Laboratory screening revealed a low urine specific gravity and renal insufficiency that were maintained after intravenous rehydration. Based on the clinical picture, a ciliopathy was considered, particularly nephronophthisis with neurological manifestation and confirmed by genetic molecular analysis. In conclusion, this case report reinforces the diagnostic challenge of nephronophthisis because of its unspecific presentation and significant overlap with other ciliopathy phenotypes. Therefore, in the presence of a urinary concentration defect and renal impairment, with or without other extra-renal manifestations, nephronophthisis should be considered in the differential diagnosis to allow a prompt diagnosis and therapeutic intervention. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200012&lng=pt&nrm=iso&tlng=pt Nephronophthisis is an autosomal recessive cystic kidney disease characterized by reduced concentrating ability of the kidney, chronic tubulointerstitial nephritis, cystic renal disease and progression to end-stage renal disease. In 10-20% of cases it is associated with a variety of other ciliopathy phenotypes. A 10-year-old caucasian female patient presented to our hospital with a history of uncontrolled vomiting and signs of moderate dehydration. Past medical history was significant for reduced visual acuity and moderate intellectual disability with a minor cerebellar dysplasia. She also complained of polyuria, polydipsia and enuresis for several years. Laboratory screening revealed a low urine specific gravity and renal insufficiency that were maintained after intravenous rehydration. Based on the clinical picture, a ciliopathy was considered, particularly nephronophthisis with neurological manifestation and confirmed by genetic molecular analysis. In conclusion, this case report reinforces the diagnostic challenge of nephronophthisis because of its unspecific presentation and significant overlap with other ciliopathy phenotypes. Therefore, in the presence of a urinary concentration defect and renal impairment, with or without other extra-renal manifestations, nephronophthisis should be considered in the differential diagnosis to allow a prompt diagnosis and therapeutic intervention. http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000200013&lng=pt&nrm=iso&tlng=pt Nephronophthisis is an autosomal recessive cystic kidney disease characterized by reduced concentrating ability of the kidney, chronic tubulointerstitial nephritis, cystic renal disease and progression to end-stage renal disease. In 10-20% of cases it is associated with a variety of other ciliopathy phenotypes. A 10-year-old caucasian female patient presented to our hospital with a history of uncontrolled vomiting and signs of moderate dehydration. Past medical history was significant for reduced visual acuity and moderate intellectual disability with a minor cerebellar dysplasia. She also complained of polyuria, polydipsia and enuresis for several years. Laboratory screening revealed a low urine specific gravity and renal insufficiency that were maintained after intravenous rehydration. Based on the clinical picture, a ciliopathy was considered, particularly nephronophthisis with neurological manifestation and confirmed by genetic molecular analysis. In conclusion, this case report reinforces the diagnostic challenge of nephronophthisis because of its unspecific presentation and significant overlap with other ciliopathy phenotypes. Therefore, in the presence of a urinary concentration defect and renal impairment, with or without other extra-renal manifestations, nephronophthisis should be considered in the differential diagnosis to allow a prompt diagnosis and therapeutic intervention.