Pattern of Glomerular Disease in Patients with Nephrotic Syndrome- A Single Centre South Indian Study.

Background: The prevalence of primary and secondary glomerular diseases presenting as nephrotic syndrome (NS) varies according to the demography, renal biopsy practice and geographic location. To determine the morphological patterns of glomerular lesions in renal biopsies from patients manifesting with NS, in our local (South Indian) population. Methods: The study was conducted in a tertiary care hospital in South India between 2008 and 2014 on adults and children presenting with NS. Renal biopsies were performed in all patients and subjected to light microscopic and immunofluorescence studies. Results: A total of 264 cases of NS were identified of which 80.7% were adults and 19.3% were children. The mean age for the adults was 42.2 years with a male: female ratio of 1.6:1 and the mean age for children was 12.1 with a male: female ratio of 1.8:1. The most common cause of NS in adults was minimal change disease (MCD) (42.7%) followed by membranous nephropathy (MN) (24.4%) and focal segmental glomerulo-sclerosis (FSGS) (17.8%). In children MCD (88%) was the single most common cause of NS followed by FSGS (5.9%) and MN (3.9%). Compared to MCD and MN, a higher incidence of microscopic haematuria and renal insufficiency was present in FSGS. Conclusion: A wide range of primary and secondary glomerular disorders can present as NS, the prevalence of which varies according to the geographic area and demography. Though the frequencies of MCD, MN, and FSGS in our study were different from other studies conducted in India and other countries, these three glomerular disorders are the three major causes of NS in many geographic areas across the world.

Glomérulo esclerosis focal y segmentaria. Trasplante renal y plasmaféresis / Focal segmental glomerulo sclerosis renal transplant and plasmapheresis

La Glomérulo Esclerosis Focal y Segmentaria (GEFS) primaria es una entidad que se define histológicamente por el depósito de material hialino en los glomérulos renales. Su origen permanece aún desconocido y es una de las causas más importantes del Síndrome Nefrótico (SN) Corticorresistente. La GEFS es la causa diagnostica de base del 8,3 por ciento de los casos de Insuficiencia Renal Crónica (lRC), del 14 por ciento de pacientes en plan de diálisis y del 13,5 por ciento de los receptores de Trasplante Renal. Además, luego del trasplante renal, esta enfermedad tiene una tasa elevada de recaída sobre el injerto. Sobre la fisiopatología de esta enfermedad, las últimas líneas teóricas hacen hincapié en la función de los podocitos, estos datos surgieron del conocimiento de alteraciones genéticas que implican a estas células. La hipótesis de que un "factor plasmático circulante" aumenta la permeabilidad de los glomérulos a la albumina está apoyada en numerosos estudios clínicos y experimentales. Esta es la base fisiopatológica del tratamiento donde se encuentra ubicada como uno de los pilares la Plasmaféresis, acompañando a las nuevas modalidades de Inmunosupresión. Esta revisión está focalizada en los mecanismos fisiopatológicos involucrados en esta enfermedad y a describir las terapéuticas utilizadas en su tratamiento.

Primary focal segmental glomerulosclerosis (FSGS) is an entity defined in histological terms by the hyaline material depot that is found in renal glomerulus. Its origin still remains unknown and it's one of the most important causes of corticosteroid-resistant nephrotic syndrome. FSGS is the main diagnostic cause of: 8.3 per cent of chronic renal failure (CRF) cases, 14 per cent of dialysis patients and 13.5 per cent of people receiving a kidney transplant. Besides, after the renal transplantation, this disease has a high rate relapse over the graft. Concerning the pathophysiology of FSGS, the latest theoretical research emphasizes on the podocytes function. These data came out from the knowledge of the genetic disorder that these cells implicate. The hypothesis that a "plasmatic circulating factor" increases the permeability of the glomerulus for albumin is supported by both several clinical and experimental research. The plasmatic circulating factor is the pathophysiological base for the treatment whit plasmapheresis along whit immunosuppression procedures. This present revision is focused on the pathophysiological mechanisms involved in, and des­cribes the therapies used on its treatment.

Implication of Angiotensin Converting Enzyme Gene Polymorphism on Renal Progression of Primary Nephrotic Syndrome in Children / 대한신장학회잡지

Human angiotensin converting enzymcC4CE) gene displays an insertion/deletion polymorphism in 16 intron, and three genotypes are determined by presence or absence of a 287-bp fragment of DNA; II, ID and DD genotype. DD genotype has been suggested as a risk factor of various cardiovascular diseases and chronic nephropathies such as IgA nephropathy and diabetic nephropathy. This study was designed to investigate if the ACE polymor-phism is related to the clinical and pathologic findings of minimal change nephrotic syndrome(MCNS) and focal segmental glomerulosclerosis(FSGS) in children. Ninety children with primary nephrotic syndrome(MCNS and steroid responsive nephrotic syndrome : 68 cases, FSGS; 22 cases) and 97 healthy normal controls were examined. The genotype for the polymorphism was determined by PCR method. The distribution of ACE genotypes in primary nephrotic syndrome(II 28.6%, ID 53.8%, DD 17.6%) was not different from that in controls(II 39.2%, ID 41.2%, DD 29.6%). The IJ genotype was more frequent in FSGS(II 64.7%, ID 23.5%, I)D 11.8%) than in MCNS and steroid responsive nephrotic syndrome(I 20.3%, ID 60.8%, DD 18.9%, p<0.03). The ACE genotypes were not associated either with frequency of relapse in MCNS or steroid responsive nephrotic syndrome or with presence of hypertension, responsiveness to steroid therapy and progression of renal dysfunction in FSGS. We concluded that deletion polymorphism of ACE gene is not associated with increased risk for renal progression in children with primary nephrotic syndrome.