Glomerular permeability activity: Prevalence and prognostic value in pediatric patients with idiopathic nephrotic syndrome

2004 ◽  
Vol 44 (4) ◽  
pp. 604-610 ◽  
Author(s):  
Howard Trachtman ◽  
Laurence A. Greenbaum ◽  
Ellen T. McCarthy ◽  
Mukut Sharma ◽  
Bernard G. Gauthier ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Prognostic Value ◽  
Pediatric Patients ◽  
Idiopathic Nephrotic Syndrome ◽  
Glomerular Permeability

ALBUMIN VERSUS FRESH FROZEN PLASMA IN MANAGING DIURETIC RESISTANT EDEMA IN CHILDREN WITH IDIOPATHIC NEPHROTIC SYNDROME

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Vishwajeet Singh ◽  
P K Berwal ◽  
T C Saini ◽  
Narender Mishra
Keyword(s):  
Nephrotic Syndrome ◽  
Pediatric Patients ◽  
Idiopathic Nephrotic Syndrome ◽  
Dry Weight ◽  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Double Number ◽  
Group A ◽  
Group B ◽  
Frozen Plasma

Background: This study was carried out to compare the efficacy and outcome of albumin with fresh frozen plasma (FFP) in the treatment of diuretic resistant edema in nephrotic syndrome Methods: Sixty patients with idiopathic NS were enrolled in this prospective analytic study. Patients with moderate to severe edema with serum albumin <1.5 gm/dl were given albumin and FFP dividing into two groups. Group-A, received intravenous albumin- 1 gm/kg/day and Group-B intravenous FFP 15ml/kg/day. Total number of albumin and FFP infusion were determined by edema reduction. Results: Diagnosis of NS and biochemical parameters were same in both groups. Dry weight was achieved in Group-A in 6.62± 3.15 days and in Group-B 6.65 ± 3.18 days. In GroupA the number of albumin infusion required was 1.42±0.62 and Group-B FFP infusion required was 3.11± 1.05 (p=0.0001). No significant complications were observed in both the groups. Conclusion: FFP cost half than albumin and same duration required to reduce edema but with double number of infusion and it is safe in pediatric patients with NS presenting with moderate to severe edema. Keywords: NS, Oedema, FFP, Albumin

scholarly journals Apheresis Therapy for Steroid-Resistant Idiopathic Nephrotic Syndrome: Report on a Case Series

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Hamza Naciri Bennani ◽  
Thomas Jouve ◽  
Johan Noble ◽  
Lionel Rostaing ◽  
Paolo Malvezzi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Case Series ◽  
Calcineurin Inhibitors ◽  
Multidrug Resistant ◽  
Glomerular Permeability ◽  
Steroid Resistant ◽  
Double Filtration Plasmapheresis ◽  
Double Filtration

Idiopathic nephrotic syndrome (INS) represents 15%–30% of adulthood glomerulopathies. Corticosteroids have been the main treatment for decades and are effective in 70% of minimal-change disease patients and ~30% of focal segmental glomerulosclerosis patients. Multidrug-resistant (steroids, calcineurin-inhibitors, cyclophosphamide, mycophenolate-mofetil, rituximab) idiopathic nephrotic syndrome is a major therapeutic challenge in nephrology. Apheresis (double-filtration plasmapheresis or semi specific immunoadsorption) could act by eliminating the circulating factor (apolipoproteinA1b, solubleCD40L, suPAR) increasing glomerular permeability seen in INS. The aim of the study was to report the outcome of three patients with multidrug-resistant INS treated successfully with apheresis.

Role of the immune system in the pathogenesis of idiopathic nephrotic syndrome

2004 ◽  
Vol 107 (2) ◽  
pp. 125-136 ◽  
Author(s):  
José G. van den BERG ◽  
Jan J. WEENING
Keyword(s):  
Nephrotic Syndrome ◽  
Immune System ◽  
Direct Evidence ◽  
Idiopathic Nephrotic Syndrome ◽  
Minimal Change ◽  
Focal And Segmental Glomerulosclerosis ◽  
Glomerular Permeability ◽  
Glomerular Capillary ◽  
Glomerular Barrier

Idiopathic NS (nephrotic syndrome) is characterized by massive proteinuria, due to a leak in the glomerular barrier to proteins. Genetic defects that affect the function and the composition of the glomerular capillary wall, in particular of the visceral epithelial cells, have recently been recognized as the cause of familial forms of NS. MCNS (minimal change NS) and FSGS (focal and segmental glomerulosclerosis) are common non-familial forms of NS in which the causative defect has not yet been identified. Several studies have shown that non-familial NS is associated with the presence of circulating permeability factors and with complex disturbances in the immune system. Thus far, there is no direct evidence that these factors directly alter glomerular permeability to proteins, and some of these factors may be a consequence, rather than a cause, of NS. In this review, we will briefly highlight the mechanisms that underlie proteinuria in general and focus on the immunological disturbances associated with idiopathic NS, with attention to potential mechanisms whereby the immune system may directly act on the glomerular capillary filter.

scholarly journals Persistent Abnormalities of Fatty Acids Profile in Children With Idiopathic Nephrotic Syndrome in Stable Remission

2021 ◽  
Vol 8 ◽  
Author(s):  
Stefano Turolo ◽  
Alberto C. Edefonti ◽  
William Morello ◽  
Marie-Louise Syren ◽  
Valentina De Cosmi ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Arachidonic Acid ◽  
Nephrotic Syndrome ◽  
Pediatric Patients ◽  
Idiopathic Nephrotic Syndrome ◽  
Cell Communication ◽  
Fatty Acids Profile ◽  
Filtration Barrier ◽  
Omega 6 ◽  
Steroid Sensitive Nephrotic Syndrome

Steroid-sensitive nephrotic syndrome is an immunological disorder mediated by still poorly defined circulating factor(s) that target the podocyte and damage the filtration barrier. Fatty acids (FA) have several biological roles and, in particular, are strictly involved in cell to cell communication, inflammatory processes and regulation of lymphocyte pools. Studies of FAs during INS have been mainly focused on biochemical changes during the phase of proteinuria; while no information is available about FA profile in patients with idiopathic nephrotic syndrome (INS) on stable remission. Aim of this study is to assess differences in blood FA profile between pediatric patients with INS during the phase of stable remission. Blood fatty acid profile of 47 pediatric patients on stable remission and 47 matched healthy controls were evaluated with gas chromatography. Patients with INS on stable remission had significantly higher levels of PUFA and omega-6 than controls (40.17 vs. 37.91% and 36.95 vs. 34.79%), lower levels of SFA and MUFA. Considering the single fatty acids, levels of omega-6 18:2n6 linoleic acid and omega-6 20:4n6 arachidonic acid were significantly higher in patients with INS than in controls (23.01 vs. 21.55%, p-value 0.003 and 10.37 vs. 9.65%, p-value 0.01). Moreover, patients with INS showed lower levels of SFA 14:0 (0.74 vs. 0.92%) and 18:0 (10.74 vs. 11.74%) and MUFA 18:1n9 oleic acid (18.50 vs. 19.83%). To the best of our knowledge this is the first study assessing FAs profile in children with INS in stable remission. In a population of 47 patients, we were able to demonstrate a higher blood level of linoleic and arachidonic acid, and consequently of omega-6 and PUFA, compared to controls. Persistently higher than normal levels of either linoleic or arachidonic acid, could be viewed as candidate biomarker for a state of risk of relapse in children with idiopathic nephrotic syndrome.

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