ABSTRACT
Amyloidoses are a group of disorders in which soluble proteins aggregate and deposit extracellularly in tissues as insoluble fibrils, causing organ dysfunction. Clinical management depends on the subtype of the protein deposited and the affected organs. Systemic amyloidosis may stem from anomalous proteins, such as immunoglobulin light chains or serum amyloid proteins in chronic inflammation or may arise from hereditary disorders. Hereditary amyloidosis consists of a group of rare conditions that do not respond to chemotherapy, hence the identification of the amyloid subtype is essential for diagnosis, prognosis, and treatment. The kidney is the organ most frequently involved in systemic amyloidosis. Renal amyloidosis is characterized by acellular pathologic Congo red-positive deposition of amyloid fibrils in glomeruli, vessels, and/or interstitium. This disease manifests with heavy proteinuria, nephrotic syndrome, and progression to end-stage kidney failure. In some situations, it is not possible to identify the amyloid subtype using immunodetection methods, so the diagnosis remains indeterminate. In cases where hereditary amyloidosis is suspected or cannot be excluded, genetic testing should be considered. Of note, laser microdissection/mass spectrometry is currently the gold standard for accurate diagnosis of amyloidosis, especially in inconclusive cases. This article reviews the clinical manifestations and the current diagnostic landscape of renal amyloidosis.
ABSTRACT
Nephrotic syndrome is the most common clinical presentation of glomerular disease in elderly patients, and renal biopsy is an important diagnostic resource. The aim of this study was to describe nephrotic syndrome among elderly patients in Brazil, focusing on tubulointerstitial and vascular involvement. This was a retrospective study of patients over 65 years of age with nephrotic syndrome who underwent renal biopsy between January 2012 and December 2019. Of the 123 renal biopsies that occurred during the study period, 44 (35.8%) were performed for the investigation of nephrotic syndrome. Among those 44 cases, the main etiologies were membranous nephropathy in 13 cases (29.5%), amyloidosis in ten (22.7%), non-collapsing focal segmental glomerulosclerosis (FSGS) in four (9.1%), and collapsing FSGS in four (9.1%). Patients with minimal change disease (MCD) had the lowest degree of interstitial fibrosis compared with the other glomerulopathies, and histological signs of acute tubular necrosis (ATN) were less common among those with amyloidosis than among those with membranous nephropathy, FSGS, or MCD (P=0.0077). Of the patients with ATN, the frequency of acute kidney injury (AKI) was highest in those with MCD (P<0.001). All patients had some degree of vascular involvement, regardless of the type of glomerulopathy. In conclusion, the second most common cause of nephrotic syndrome in this population was amyloidosis, and acute interstitial tubule involvement was more marked in MCD. Vascular involvement is something that cannot be dissociated from the age of the patient and is not only due to the underlying glomerulopathy.
ABSTRACT
Arthrospira (Spirulina) platensis was cultivated in laboratory under controlled conditions (30ºC, photoperiod of 12 hours light/dark provided by fluorescent lamps at a light intensity of 140 µmol photons.m-2.s-1 and constant bubbling air) in three different culture media: (1) Paoletti medium (control), (2) Paoletti supplemented with 1 g.L-1 NaCl (salinated water) and (3) Paoletti medium prepared with desalinator wastewater. The effects of these treatments on growth, protein content and amino acid profile were measured. Maximum cell concentrations observed in Paoletti medium, Paoletti supplemented with salinated water or with desalinator wastewater were 2.587, 3.545 and 4.954 g.L-1, respectively. Biomass in medium 3 presented the highest protein content (56.17 percent), while biomass in medium 2 presented 48.59 percent protein. All essential amino acids, except lysine and tryptophan, were found in concentrations higher than those requiried by FAO.
Arthrospira (Spirulina) platensis foi cultivada em laboratório sob condições controladas (30ºC, intensidade luminosa de 140 µmol fótons.m-2.s-1, 12 horas claro/escuro e insuflação constante de ar atmosférico), em três meios de cultivo: (1) meio de Paoletti (controle), (2) meio de Paoletti suplementado com 1,0 g.L-1 de NaCl (água salinizada) e (3) meio de Paoletti preparado com rejeito de dessalinizador. Foi verificado o efeito destes tratamentos no crescimento, teor de proteínas e aminoácidos. As concentrações celulares máximas obtidas foram de 2,587; 3,545 e 4,954 g.L-1 no meio controle, meio de Paoletti suplementado com água salinizada ou com rejeito de dessalinizador, respectivamente. Com relação às concentrações protéicas, estas foram maiores na biomassa cultivada no meio 3, com 56,17 por cento, enquanto que a biomassa cultivada no meio 2 apresentou 48,59 por cento. A maioria dos aminoácidos essenciais encontrou-se acima dos limites requeridos pela FAO, com exceção apenas de lisina e triptofano.