Steroid-responsive Idiopathic Nephrotic Syndrome Research Articles

Elevated baseline adiponectin level predicting an increased risk of disease activity in idiopathic nephrotic syndrome and chronic kidney disease in children

Adiponectin, an adipocyte-derived protein, is present abundantly in circulation and has anti-atherogenic and anti-inflammatory effects, so we aimed to assess the role of adiponectin in idiopathic nephrotic syndrome and chronic kidney disease in children. Overall of 290 children, 95 patients with the steroid-responsive idiopathic nephrotic syndrome (50 in remission and 45 in relapse). Another 95 patients with chronic kidney disease (50 on hemodialysis and 45 on conservative therapies) and 100 apparently healthy matched children were enrolled into the study. Serum adiponectin levels, blood chemistry (protein/albumin, serum creatinine, blood urea and electrolytes levels) and 24-hour proteinuria w studied as well. There was highly significant increasing differences in adiponectin level between the relapsed idiopathic nephrotic syndrome (26.93±6.79 µg/mL) compared to remission (10.96±3.33 µg/mL) and control group (4.89±1.01 µg/mL) and between remission in comparing to control group (P<0.001). Also there was significant negative correlation between adiponectin level and serum albumin level in the relapse group (r=-0.60, P≤0.05), and highly positive correlation between adiponectin level and 24-hour proteinuria in relapse (r=0.98, P≤0.001) and remission group (r=0.92, P≤0.001). Regarding children with chronic kidney disease, There were high significant increasing differences in adiponectin levels in conservative (13.88±4.27 µg/mL) and dialysis (15.09±6.14 µg/mL) groups compared to controls (6.05±5.83 µg/mL) with P<0.001. Furthermore, there was a significant positive correlation between adiponectin and serum creatinine and blood urea levels in both conservative and dialysis groups with P<0.05. Adiponectin can be considered as an important useful biomarker for chronic kidney diseases in children.

Final height of adults with childhood-onset steroid-responsive idiopathic nephrotic syndrome

The aim of this study was to evaluate the final stature of adults with childhood-onset steroid-responsive idiopathic nephrotic syndrome (INS) and the influence of disease-related issues on the achievement of their target heights. We analyzed 60 (41 male) patients and/or their records, with a minimum age of 19 years or at a Tanner's pubertal stage 4 for boys or status postmenarche for girls, and normal glomerular filtration rate. Mean age at first and last consultation was 5.3+/-2.4 years and 20.5+/-3.1 years, respectively. Mean follow-up period was 15.10 years. Mean cumulative dose of prednisone was 1254+/-831.40 mg/kg. Mean initial and final height Z scores (HtZ) were, respectively, -0.60+/-1.0 and -0.64+/-0.92 (p=0.72). The final HtZ showed a significant correlation only with the initial HtZ and the target HtZ (THZ). Six patients achieved a final HtZ below -2, which in male patients correlated strongly to the initial HtZ and THZ. A strong correlation was demonstrated between final HtZ, initial HtZ, and THZ. INS-related issues did not prevent the final stature to reach the predicted target height.

Growth in steroid-responsive nephrotic syndrome: a study of 85 pediatric patients.

The statural growth of 85 patients with steroid-responsive idiopathic nephrotic syndrome, attending the Pediatric Nephrology Unit, Children's Institute, Hospital das Clínicas School of Medicine, University of São Paulo, with a minimum follow-up of 3 years, was evaluated. Analysis of the patient population as a group did not show any significant alterations in the height Z score and the mean height percentile between the first (-0.59 and 33.9, respectively) and last consultation (-0.57 and 34.8, respectively). Analysis of each individual patient allowed the definition of two subgroups. Subgroup A, which achieved growth improvement, was composed of 47 children-initial Z score and mean initial height percentile of -0.91 and 24.0, respectively; final Z score and mean height percentile of -0.30 and 40.7, respectively ( P=0.00). Subgroup B, which showed growth retardation, was composed of 38 children-initial Z score and mean initial height percentile of -0.19 and 46.2, respectively; final Z score and mean height percentile of -0,9 and 27.5, respectively ( P=0.00). The following factors were significantly different when both subgroups were compared: (1) total duration of prednisone therapy and total prednisone dose were greater in subgroup B; (2) the final chronological age of patients using prednisone was higher in subgroup B; (3) the pubertal growth spurt in subgroup B showed attenuation and retardation.

Clinical and Genetic Evaluation of Familial Steroid-Responsive Nephrotic Syndrome in Childhood

Steroid-responsive idiopathic nephrotic syndrome (SSINS) is the most common form of nephrotic syndrome in childhood. This article reports a cohort of familial SSINS with disease onset in childhood. The clinical course in terms of age at onset, symptoms during the initial phase, renal morphology, and outcome was evaluated. Furthermore, linkage to NPHS2, the gene for autosomal-recessive steroid-resistant INS on chromosome 1, was examined. Two families with haplotypes consistent with linkage to NPHS2 were evaluated for mutations in the NPHS2 gene. Familial SSINS (32 patients from 15 families, minimal change NS in 12 of 12 biopsies) was found to be a clinically homogeneous entity. Interfamilial and intrafamilial variability with respect to the age at disease onset was low, indicating a strong genetic influence on disease onset. By linkage studies and mutational analysis, familial SSINS was found to be genetically distinct from NPHS2. This is the first report of a large cohort of familial SSINS. Exclusion of linkage to NPHS2 makes likely the existence of a distinct gene locus for SSINS.

Decline of Vaccine-Induced Antipneumococcal Antibody in Children With Nephrotic Syndrome

Twenty-five children with steroid-responsive idiopathic nephrotic syndrome were studied for persistence of antipneumococcal capsular polysaccharide antibody during relapse of their disease and at 1, 6, and 12 months after vaccination with the 14-valent pneumococcal vaccine. Nonrelapsers (group I) were compared to those who had at least one relapse but whose sera were obtained during remission (group II). Group II had a more rapid decline in total anticapsular antibody per month than group I (5.3% v 2.4%). Analysis by individual anticapsular types showed that differences between groups approached significance only for type 4 (P = .07). Rates of decline of antibody against pneumococcal capsular polysaccharide varied among types. One year after vaccination, 50% of patients had less than 300 ngAbN/mL against types 4, 6A, 7F, 8, and 19F. Sera obtained from seven patients during relapse had geometric mean antibody concentrations less than 300 ngAbN/mL against those same types; two of these types have been reported to cause disease in vaccinated patients with nephrosis. Decline of antibody against pneumococcal capsular polysaccharide following vaccination varies by capsular type. Type-specific analysis should be used when monitoring serum antibody levels in these patients after vaccination.

Response to Pneumococcal Vaccination in Children With Nephrotic Syndrome

Serologic responses to a 14-valent pneumococcal vaccine were measured in 20 children with steroid-responsive idiopathic nephrotic syndrome. All patients were free of proteinuria and receiving either daily (five patients) or alternate-day (15 patients) prednisone in a dosage of 1-2 mg/kg/day at the time of vaccination. Patients on alternate-day steroids received the vaccine on a day prednisone was not given. The mean fold rise in antibody titer was found to be normal in these children when antibody levels measured 3-6 wk post-vaccination were compared to prevaccination levels. This serologic response has correlated well with a 3-yr follow-up of the patients, none of whom has developed peritonitis secondary to any pneumococcal types in the vaccine. Also described are three patients who developed pneumococcal peritonitis during this period despite prior vaccination; in two of these patients, the pneumococcal type was not included in the vaccine (types 6b and 10a) and in one patient the organism was not typed. It is concluded that in children with nephrotic syndrome pneumococcal vaccination confers good protection against types included in the vaccine despite the concomitant administration of steroids. However, the patients who developed peritonitis secondary to other pneumococcal types remind us that pneumococcus must still be considered as an etiologic agent for peritonitis in nephrotic children who have been vaccinated.

Serum hemolytic factor D values in children with steroid-responsive idiopathic nephrotic syndrome

Serum hemolytic factor D activity, an important component of the ACP, was measured in patients with SR-INS. The mean serum factor D hemolytic activity of patients with SR-INS in relapse was significantly reduced compared to the mean of the control group. Twenty-one of 27 SR-INS patients in relapse (78%) had reduced serum factor D activity. In contrast, the mean serum factor D hemolytic activity of SR-INS patients in remission was not significantly different from that in the control group. Factor D hemolytic activity was also reduced in other types of renal disease with the nephrotic syndrome. Serum factor D values were highly correlated with the serum albumin concentration. Although hemolytic factor D activity could not be detected in the urine, the low molecular weight and the significant correlation with serum albumin concentration suggest that urinary loss is responsible for the low serum levels of factor D. Deficient serum values of factor D may contribute to the increased susceptibility of SR-INS patients in relapse to bacterial infections with organisms which activate the ACP.