Idiopathic Nephrotic Syndrome Patients Research Articles

Idiopathic Nephrotic Syndrome in Children in Chad: Epidemiology and Clinical Outcomes.

Nephrotic syndrome (NS) remains the most common presentation of glomerular diseases in children. Moreover, NS is primarily idiopathic, accounting for 90% of cases, with an average onset age between 2 and 10 years. The objective of our study was to describe the characteristics and outcomes of NS in children from three major hospitals in one of the world's poorest countries, Chad. This observational, cross-sectional, descriptive, and multicenter study took place over a period of 36 months (1 January 2019-31 December 2021) and was carried out in three hospitals in N'Djamena, Chad. Children aged 1-15 years presenting with NS were included in the study. Out of 16,776 children hospitalized or followed up with in outpatient clinics, 24 cases of NS were identified, yielding a prevalence of 0.14%. The median age at presentation was 6.16 years (1-10). Nineteen children were male (sex ratio 3.8). Eight cases were classified as impure NS (33.3%). Edema was present in all patients, while oliguria was present in 29.16% (n = 7), and arterial hypertension was present in 20.83% (n = 5) of cases. Mean proteinuria, albuminemia, and total proteins were 2.86g/L, 19.13g/L and 30.41g/L, respectively. The median serum creatinine was 87.3 µmol/L (75-1375 µmol/L). Three patients experienced acute renal failure upon admission. Four patients had secondary NS. All idiopathic NS patients (n = 20) who had received corticosteroid therapy had a 90% response rate to steroids. Non-responsive or relapsed patients underwent kidney biopsy (n = 7), revealing focal segmental glomerulosclerosis (FSGS; n = 4) as the most common histological lesion, followed by minimal change disease (n = 2) and membranoproliferative glomerulonephritis (n = 1). The median length of hospitalization stay was 10.67 (5-27) days. None of the patients with idiopathic NS died. At the last follow-up, sixteen patients (80%) achieved long-term complete remission with normal renal function; however, four of those had subsequent relapses. One patient with secondary NS died. In Chad, childhood idiopathic nephrotic syndrome predominantly affects young males; steroid sensitivity is as high as 95%, and in the long-term, 80% of patients achieve remission with normal renal function.

Demographic Charecterstics of Children with First Attack Idiopathic Nephrotic Syndrome with Relapse- A Hospital-Based Observational Study

Background: Idiopathic nephrotic syndrome (INS) is a common childhood illness with or without relapses. So, the objectives of this study is to find out such children who are prone to develop frequent relapse and the demographic characteristics responsible for relapse. This retrospective study was conducted in the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, from September 2016 to August 2017.
 Methods: A total of 75 patients of idiopathic nephrotic syndrome (INS) with the initial attack, aged 1–18 years, were enrolled in this study. All patients were treated with prednisolone 60 mg/m2/day, single morning dose for 6 weeks, followed by 40 mg/m2 every alternate day for another 6 weeks and were analyzed and followed up for a minimum period of six months to identify the risk factors related to relapses.
 Results: Among them, 50 (66.7%) were males, 25 (33.3%) were females, with a male: female ratio of 2:1. 10 (13.3%) children had no relapse, 18 (24.0%) had infrequent relapse, and 40 (53.3%) had frequent relapse. Children responding between 2 and 4 weeks after the start of treatment had a higher chance of relapse (P = 0.002) than those who responded less than 1 week. Early age at onset, male gender, rural inhabitants, low socioeconomic status and atopy, though statistically not significant, are strongly associated with frequent relapses.
 Conclusion: Young age at diagnosis of INS, male gender, low serum albumin, and infections were predictive risk factors of multiple relapses. So, physicians should be vigilant to monitor these patients closely and counsel the families of nephrotic children regarding the prediction of subsequent relapses and outcomes.

A spectrum of novel anti-vascular endothelial cells autoantibodies in idiopathic nephrotic syndrome patients.

Idiopathic nephrotic syndrome (INS) is a common renal disease characterized by disruption of the glomerular filtration barrier. In a previous study, we screened and identified podocyte autoantibodies in nephrotic syndrome patients and proposed the concept of autoimmune podocytopathy. However, circulating podocyte autoantibodies cannot reach podocytes unless glomerular endothelial cells have been damaged. Therefore, we speculate that INS patients may also have autoantibodies against vascular endothelial cells. Sera from INS patients were used as primary antibodies to screen and identify endothelial autoantibodies by hybridization with vascular endothelial cell proteins separated by two-dimensional electrophoresis. The clinical application value and pathogenicity of these autoantibodies were further verified by clinical study and in vivo and in vitro experiments. Nine kinds of autoantibodies against vascular endothelial cells were screened in patients with INS, which can cause endothelial cell damage. In addition, 89% of these patients were positive for at least one autoantibody.

NURTuRE-INS: A Biobank of United Kingdom Idiopathic Nephrotic Syndrome Patients to Enable Research and Disease Stratification

NURTuRE-INS: A Biobank of United Kingdom Idiopathic Nephrotic Syndrome Patients to Enable Research and Disease Stratification

B-Cell Dysregulation in Idiopathic Nephrotic Syndrome: What We Know and What We Need to Discover.

The therapeutic efficacy of B-cell depletion by anti-CD20 treatment in pediatric and, more recently, in adult idiopathic nephrotic syndrome patients suggests a key role of B cells in the pathogenesis of the disease. However, their exact role is still unclear. B cells are able to secrete a large variety of antibodies that can protect against infections. However, B-cell dysregulation is well-established in a variety of autoimmune diseases. In parallel with their ability to produce antibodies, pathogenic B cells display altered effector functions by expressing activating surface molecules, which can strongly modify the immune homeostasis, or by producing specific cytokines, which can directly affect either podocyte structure and functions or modulate T-cell homeostasis. Herein, we report the most relevant clinical and experimental evidences of a pathogenic role of B cells in idiopathic nephrotic syndrome. We further highlight similarities and differences between children and adults affected by non-genetic forms of the disease and discuss what needs to be investigated in order to define the exact mechanisms underlying the pathogenic role of B cells and to identify more tailored therapeutic approaches.

Hypomethylation of NLRP3 gene promoter discriminates glucocorticoid-resistant from glucocorticoid-sensitive idiopathic nephrotic syndrome patients.

To assess whether NLRP3 gene promoter methylation was able to discriminate glucocorticoid (GC)‐resistant from GC‐sensitive idiopathic nephrotic syndrome (INS), patients with minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS), we measured the methylation level of NLRP3 promoter in DNA from peripheral blood cells of 10 adult patients with GC‐resistant FSGS already in hemodialysis and 18 patients with GC‐sensitive INS (13 MCD/5 FSGS) and in 21 pediatric patients with INS with MCD/FSGS before starting any treatment. Association of NLRP3 inflammasome with GC resistance was recapitulated in vitro in monocytic cell lines (THP‐1 and U937). In both adults and pediatric patients, NLRP3 promoter methylation was significantly reduced in GC‐resistant compared with GC‐sensitive patients. Indeed, NLRP3 methylation distinguished GC‐resistant and GC‐sensitive patients (area under the receiver operating characteristic curve [AUROC] 86.7% in adults, p = 0.00019, and 73.5% in children, p = 0.00097). NLRP3 knock‐down augmented sensitivity to GCs in THP‐1 cells, whereas NLRP3 inflammasome activation lowered GC receptor concentration, increasing GC resistance in U937 cells. Our results uncovered a new biological mechanism by which patients with INS may acquire GC resistance, that could be used in future as a novel noninvasive diagnostic tool. Study Highlights WHAT IS THE CURRENT KNOWLEDGE ON THE TOPIC? ☑ Approximately 80% of patients with idiopathic nephrotic syndrome (INS) respond to glucocorticoids, with the remaining 20% being steroid‐resistant. WHAT QUESTION DID THIS STUDY ADDRESS? ☑ Whether NLRP3 gene promoter methylation was able to discriminate glucocorticoid‐resistant from glucocorticoid (GC)‐sensitive INS. WHAT DOES THIS STUDY ADD TO OUR KNOWLEDGE? ☑ In both adults and children, NLRP3 promoter methylation was significantly reduced in leukocytes of patients with GC‐resistant compared with GC‐sensitive INS. NLRP3 inflammasome activation lowered GC receptor concentration and augmented GC resistance, whereas NLRP3 knockdown increased sensitivity to GCs in cell lines representative of monocytes (U937 and THP1). HOW MIGHT THIS CHANGE CLINICAL PHARMACOLOGY OR TRANSLATIONAL SCIENCE? ☑ Our findings uncovered a new biological mechanism whereby patients with INS may develop resistance to GCs that could be used in the future as a novel noninvasive diagnostic tool.

Thyroid Function in Children with Idiopathic Nephrotic Syndrome

Background: The underlying abnormality in nephrotic syndrome is an increase inpermeability of the glomerular membrane. Urinary loses of binding proteins such asThyroxine Binding Globulin (TBG) albumin results in a reduction in serumthyroxine(T4) and sometimes in total T3 levels. The study was done to assess thyroidhormone status of idiopathic nephrotic syndrome patients at diagnosis, comparethe thyroid function after treatment with steroid, correlate with serum albumin andcompare the thyroid function with control group.
 Materials and methods: It was an analytic type of cross sectional study, done inPaediatric ward, Chittagong Medical College Hospital, Chattogram from 01.05.11 to30.11.11. Total 58 patients in two groups were included. In group A 32 cases ofidiopathic nephrotic syndrome patients and in group B 26 control cases of same ageand sex were taken. In group A thyroid function was done at initial diagnosis and inhypothyroid patients in this group again thyroid function was done after treatmentwith steroid. Data was collected through interviewing with pre-designedquestionnaire, physical examination and investigation techniques.
 Results: Thyroid hormone level of nephrotic children were significantly lower thanthat of control group. Nephrotic children with thyroid hypofunction were foundeuthyroid after initial treatment with steroid that was statistically significant andthere was positive correlation between serum albumin and thyroid hormone level.
 Conclusion: Children suffering from nephrotic syndrome had significant thyroidhypofunction because of low serum albumin level and after treatment with steroidthey became euthyroid.
 Chatt Maa Shi Hosp Med Coll J; Vol.19 (1); January 2020; Page 51-54

P1817DOES EPIGENETIC FACTORS LIKE HDAC2 PLAYS ROLE IN STEROID RESISTANCE VIA REGULATION OF P-GP AND MRP-1 IN CHILDHOOD IDIOPATHIC NEPHROTIC SYNDROME?

Abstract Background and Aims The action of glucocorticoids is to switch off activated inflammatory genes. The activated glucocorticoid receptors (GR) interact with co-repressor molecules to impair NFκB-associated co-activator activity, reducing histone acetylation, chromatin remodelling. Reduction in histone acetylation occurs via recruitment of histone deacetylase (HDAC) 2 to the activated inflammatory gene complex by activated GR, resulting in efficacious suppression of activated inflammatory genes within the nucleus. To evaluate the effect HDAC 2 on P-gp and MRP-1 expression and function. Method Total of 78 subjects were considered in the study out of which 50 were steroid sensitive nephrotic syndrome (SSNS), and 28 were steroid resistant nephrotic syndrome (SRNS) patients. mRNA expression was analyzed on peripheral blood mononuclear cells (PBMCs) in SRNS patients (mean age 8.43±3.8 years), SSNS patients (mean age 7.54±3.5 years). PBMCs were treated with 1µM of Theophylline (HDAC2 stimulator) and 0.8µM of Trichostatin A (HDAC2 inhibitor) for a period of 48 hours. Quantitative PCR was performed using light cycler LC480 using SYBR green PCR technology with SYBR premix relative gene expression levels were calculated and normalized to the corresponding levels of the housekeeping gene (GAPDH). Results Expression of P-gp (4.79±0.970 v/s 2.13±0.72, p<0.0001) and MRP-1 (3.99 ±0.8 v/s 1.99 ±0.91, p<0.0001) on PBMCs was increased in SRNS as compared to that of SSNS. HDAC2 mRNA levels were significantly decreased in SRNS patients as compared to that of SSNS patients (2.97 ± 0.95 v/s 6.02 ± 1.13, p<0.0001). Theophylline(HDAC stimulator) for a period of 48 hours decreased mRNA levels of P-gp and MRP-1 in PBMCs of SRNS with maximal induction at 1µM (fold change 2.65 and 2.21, *p<0.0001) However HDAC2 mRNA expression increased significantly (fold change5.67, *p<0.0001). In SSNS patients P-gp and MRP-1 mRNA expression decreased at1µM (fold change 1.25, 1.24, *p<0.0001) while the mRNA expression was increased (fold change 6.93, *p<0.0001). TSA(HDAC inhibitor) for a period of 48 hours increased mRNA levels of P-gp and MRP-1 in PBMCs of SRNS with maximal induction at 0.8µM (fold change 7.51, 7.31, *p<0.0001) and significantly decreased the level of HDAC2 (fold change1.50, *p<0.0001) similarly in SSNS patients P-gp and MRP-1 mRNA expression increased at 0.8µM (fold change 3.49, 3.35, *p<0.0001) and HDAC2 decreased (fold change2.53, *p<0.0001) at 0.8µM. The functional activity of P-gp and MRP-1 was significantly higher in SRNS group as compared to SSNS group (p<0.001), whereas enzymatic activity of HDAC2 was increased in SSNS group as compared to SRNS group (p<0.001) Conclusion As we observed that HDAC2 regulates P-gp and MRP-1 efflux pumps, Inducer of HDAC2 may be a probable treatment stratergy for patients of Idiopathic Nephrotic Syndrome.

The effect of vitamin 25-(OH)-D3 on Glucocorticoid Receptor-β (GRβ) and 25-(OH)-D receptors in idiopathic nephrotic syndrome patients

Backgound: Idiopathic Nephrotic Syndrome (INS) is the most chronic glomerular disease in children. Glucocorticoid is the main therapy acts by binding to glucocorticoid receptor α (GRα), whereas it is reported that glucocorticoid receptor β (GRβ) is a main inhibitor of GRα. The interaction between glucocorticoid receptors and vitamin D can increase anti-inflammation at glucocorticoid receptors, thereby decreasing GRβ levels. This study was aimed to investigate the effect ssupplementation of vitamin D3 on GRβ and 25-(OH)-D level in children with INS and the correlation between both of parameters.
 Method: A randomized clinical trial, double blind, pre and post-test control group was conducted among 30 subjects with newly diagnosed as INS. Subjects were divided into 2 groups, group 1 (G1) were treated with prednisone and vitamin D3 whereas group 2 (G2) were only treated with prednisone. The level of GRβ and 25-(OH)-D level was measured by ELISA method. Data were analyzed using SPSS version 17 for Windows.
 Result: There was significant elevation of 25-(OH)-D plasma between G1 (31.46 ± 9.69 ng/mL) and G2 (13.43±17.25 ng/mL) (p<0.001), and significant decrease of GRβ levels between G1 (-13.71 ± 8.06 ng/mL) and G2 (-0.917 ± 3.76 ng/mL) (p<0.000). Pearson testing first group showed that vitamin D level was positively correlated with GRβ levels (r=0.458; p= 0.01).
 Conclusion: The effect supplementation of vitamin D3 can increase 25-(OH)-D level and decrease of GRβ levels significantly in children with idiopathic nephrotic syndrome who receive glucocorticoid therapy.

Evaluation of Serum Iron and Transferrin in Idiopathic Nephrotic Syndrome Patients Attending Al-Hussein Pediatric Nephrology Clinic

Background: Nephrotic syndrome is characterized by excessive urinary excretion of albumin and other intermediated-size plasma proteins such as transferrin.. Excessive urinary losses of transferrin can produce low serum transferrin level that may lead to low plasma iron concentration and consequently the development of iron resistant microcytic hypochromic anemia. The aim of the work to evaluate changes in serum iron and transferrin and the relationship between the serum and urinary transferrin in the active and remission state of nephrotic syndrome Patients and methods: The present work included fifty children with idiopathic nephrotic syndrome (INS), divided into 2 groups: group A (25 cases in activity) and group B (25 cases in remission). These two groups were age and sex matched with a mean age of (7.74 ± 2.45). All patients in this work were clinically evaluated together with routine laboratory investigations. Serum albumin and urinary alb. /creatinine were assessed. Levels of serum iron, serum ferritin, serum transferrin and urinary transferrin were measured. Results: This work shows that the level of hemoglobin, MCV, HCT were statistically significantly lower in the cases in active state than cases in remission. The mean serum iron, serum ferritin and serum transferrin were significantly lower in the cases in active state than in the cases in remission .The mean urinary transferrin was statistically significantly higher in the cases in active state than in the cases with remission. Conclusion: Urinary transferrin can be considered a reliable parameter for early prediction of iron deficiency anemia in children with active nephrotic syndrome.

Urinary Neutrophil Gelatinase- Associated Lipocalin in Children with Idiopathic Nephrotic Syndrome: A Biomarker to Differentiate Between Steroid Sensitive and Resistant Forms of Disease

Background: Objectives of the study were to find out the ability of urinary neutrophil gelatinase- associated lipocalin (NGAL) as a biomarker to differentiate between steroid resistant nephrotic syndrome (SRNS) and steroid sensitive nephrotic syndrome (SSNS), and also to observe variation in levels, if any, between focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) in SRNS patients.Methods: The study included 63 patients of idiopathic nephrotic syndrome (19 SSNS in relapse, 19 SSNS in remission and 25 SRNS), aged 2.5-16 years, along with 17 controls. Urinary NGAL was measured by ELISA and the values were normalised with urinary creatinine.Results: Median urinary NGAL/creatinine level was significantly raised in SRNS as compared to SSNS relapse, SSNS remission and controls (p 18.3 ng/mg had sensitivity of 84%, specificity of 97.4% and area under the curve of 0.935 (p< 0.001, 95% confidence interval 0.871-0.998) to differentiate SRNS from SSNS patients. Median urinary NGAL/creatinine value was significantly raised in children with FSGS as compared to MCD (p=0.003) in SRNS patients. It had significant positive correlations with duration of illness (r=0.342, p=0.006), urine protein creatinine ratio (r=0.594, p< 0.001) and negative correlation with serum albumin (r=0.470, p<0.001).Conclusion: Urinary NGAL/creatinine level correlated with activity of the disease and it can distinguish not only SRNS from SSNS but also FSGS and MCD histopathological sub-types of SRNS in children.

FCGR2A single nucleotide polymorphism confers susceptibility to childhood-onset idiopathic nephrotic syndrome

Childhood-onset idiopathic nephrotic syndrome affects 1.15–3.4 children/100,000 children/year in Western Countries. Immune-mediated mechanisms, particularly T cell-mediated, are thought to play a key pathogenic role. The genetic basis of the disease is still poorly understood. We tested the association between single nucleotide polymorphisms (SNPs) of four genes encoding Fc gamma receptors (FCGR2A, FCGR2B, FCGR3A, FCGR3B) and idiopathic nephrotic syndrome in a case-control study of paediatric patients. Children with idiopathic nephrotic syndrome (aged 1–16 years) were included. FCGR2A rs1801274 and FCGR3A rs396991 SNPs were genotyped using real-time PCR with the TaqMan method, while FCGR2B rs1050501 and FCGR3B NA1/NA2 were genotyped using Sanger sequencing. Fisher’s exact test was used to explore genetic association. We enrolled 103 idiopathic nephrotic syndrome patients and 181 healthy controls. A significant association was found between idiopathic nephrotic syndrome and FCGR2A rs1801274 SNP (both with the T allele and the TT genotype, p value=0.0009, OR 1.81, 95% CI 1.27–2.59 and p value=0.0007, OR 2.39, 95% CI 1.44–3.99, respectively). No associations were found for the remaining SNPs. Fc gamma receptors might modulate response to rituximab; since 60 of the enrolled patients were treated with rituximab, we also tested the association between the studied SNPs and rituximab efficacy in this patient subgroup, but found only a weak association with FCGR2A CC genotype (p value=0.03). The FCGR2A rs1801274 SNP in the gene encoding the activating receptor CD32A confers susceptibility to idiopathic nephrotic syndrome.

Combined supplementation with α-tocopherol and vitamin C improves the blood pressure of pediatric idiopathic nephrotic syndrome patients

Summary The purpose of the present study was to investigate the effects of combined supplementation with α-tocopherol and vitamin C on blood pressures and levels of ox-LDL and NOx in with pediatric idiopathic nephrotic syndrome patients. A total of thirty-six pediatric idiopathic nephrotic syndrome patients was involved in the study, which was assigned randomly into two groups, the control group of pediatric idiopathic nephrotic syndrome patients who received a standard therapy and placebo and the group of pediatric idiopathic nephrotic syndrome patients who received a standard therapy plus α-tocopherol and vitamin C. α-tocopherol and vitamin C were administered at a dose of 10–15 mg/kg/day for 12 weeks. An analysis of lipid profile and NOx was performed by means of a spectrophotometer. An analysis of ox-LDL was performed by using the enzyme-linked immunosorbent assay technique. There was a significant decrease in the number of prehypertensive and hypertensive patients in the supplementation group relative to the control group (p 0.50). In conclusion, the combined supplementation with α-tocopherol and vitamin C may improve blood pressure in pediatric idiopathic nephrotic syndrome patients. Thus, combination of α-tocopherol and vitamin C can be used in the clinical management of pediatric idiopathic nephrotic syndrome.

Trace elements in children suffering from idiopathic nephrotic syndrome.

Trace elements play a significant role in several metabolic processes and often circulate in the blood binding to protein. The purpose of this study was to determine the status of selenium, zinc, and boron in idiopathic nephrotic syndrome patients in active and remission phases. Fourteen patients and fourteen healthy age-matched controls were included in the study. The selenium, zinc and boron level in plasma and urine were measured by the inductively coupled plasma mass spectrometry. The plasma levels of zinc and selenium were significantly lower in both active and remission patients (for all p=0.0001). The plasma boron level was significantly lower only in patients in active phase (p=0.0002 vs control). The concentrations of urinary boron and selenium were significantly higher during active phase compared with remission (p=0.0003 and 0.0001, respectively). Supplementation with zinc, selenium and boron may be justified in patients suffering with this disease.

Toll Like Receptor 4 (Tlr4) And p65 Nuclear Factor Kappa B (Nf-Kb) Expression In Monocyte Cell Of Children With Steroid Resistant Nephrotic Syndrome

This research aimed to prove the difference of TLR4 and p65NF-kB subunit expression between children with steroid-sensitive nephrotic syndrome (SSNS) and those with steroid-resistent nephrotic syndrome (SRNS) and to find out the correlation between expression of TLR4 and p65NF-kB on children with SRNS. Twenty three patients of idiopathic nephrotic syndrome patients (INS) were enrolled in this study and classified into two groups—those with SSNS (11 patients) and SRNS (12 patients). Peripheral Blood Mononuclear Cells (PBMC) isolation was performed, taken from 5 ml patients’ blood samples, followed by monocyte isolation of PMBC using monocyte cultu re. Examination of TLR4 and p65NF-kB expression from monocyte's cells culture was performed using flow cytometry. The result showed that the differences of TLR4 expression between SSNS and SRNS was significant (p=0.012), The differences of p65 NF-kB expression between SSNS and SRNS showed an insignificant correlation (p=0.880). Pearson correlation test between the expression of TLR4 and p65NF-kB showed no significant correlation (p=0.472), while Spearman correlation test to see the relationship between expression of TLR4 and SRNS produced correlation value of 0.512 and its importance p value was 0.013 (p<0.05). There is a significant difference of TLR4 expression between SRNS and SSNS and there is also a significant correlation between expression of TLR4 and SRNS, that proved TLR4 has an important role in steroid resistance of nephrotic syndrome.

In vitro decrease of glomerular heparan sulfate by lymphocytes from idiopathic nephrotic syndrome patients

Lymphocytes are involved in the physiopathologic mechanism of idiopathic nephrotic syndrome (INS). We have recently demonstrated that plasma from patients with INS decreases human glomerular epithelial cell (GEC) glycosaminoglycans (GAGs), particularly heparan sulfates (HS) in vitro. In this study we investigate the effect of peripheral blood lymphocytes (PBL) from INS patients on glomerular cell GAG and HS. Human GECs were cultured with total peripheral blood mononuclear cells (PBMCs), PBL, and monocytes from patients and controls. The amounts of GAG and HS were assessed using a cationic membrane after metabolic labeling. In coculture with GECs, mononuclear cells from controls decreased total epithelial cell GAG (-30% with PBMC, P < 0.05; -25% with PBL, P < 0.02; -19% with monocytes, P < 0.05). Particularly HSs were decreased (-36% with PBMC, P < 0.05; -27% with PBL, P < 0.02; and -19% with monocytes, P < 0.05). When GECs were in coculture with PBL from INS patients, the decrease in GAG and HS was significantly greater in comparison to control PBL (-10%, P < 0.02; -10%, P < 0.02, respectively, for GAG and HS). Moreover, supernatants of stimulated PBMCs from patients decreased also GAG and HS in comparison with controls (-13%, P < 0.02; -15%, P < 0.02, respectively, for GAG and HS). These data provide direct evidence that PBLs from INS patients are able to decrease GEC HS as previously shown with plasma from patients. This might be instrumental in the onset of albuminuria.

In vitro decrease of glomerular heparan sulfate by lymphocytes from idiopathic nephrotic syndrome patients

In vitro decrease of glomerular heparan sulfate by lymphocytes from idiopathic nephrotic syndrome patients

Ig Synthesis of Lymphocytes from Idiopathic Nephrotic Syndrome Patients

Ig Synthesis of Lymphocytes from Idiopathic Nephrotic Syndrome Patients