Steroid-sensitive Idiopathic Nephrotic Syndrome Research Articles

The Potential of Vitamin-D-Binding Protein as a Urinary Biomarker to Distinguish Steroid-Resistant from Steroid-Sensitive Idiopathic Nephrotic Syndrome in Iraqi Children

Objective: To determine the ability of uVDBP to discern SRNS from steroid-sensitive nephrotic syndrome (SSNS) in Iraqi children. Materials and Methods: This cross-sectional study enrolled children with SRNS (n=31) and SSNS (n=32) from the pediatric nephrology clinic of Babylon Hospital for Maternity and Pediatrics over three months. Patients' characteristics in terms of demographics, clinical data, and urinary investigations were collected. Quantitative analysis of uVDBP levels was undertaken via a commercially available ELISA kit. Results: The median uVDBP values were significantly higher (p-value<0.001) in the SRNS group (median=10.26, IQR=5.91 μg/mL) than in the SSNS group (median=0.953, IQR=4.12 μg/mL). A negative correlation was noted between uVDBP levels and estimated glomerular filtration rate (eGFR) (Spearman's rho coefficient= − 0.494, p=0.001). Nevertheless, the rise in uVDBP concentrations was still considerable in children with SRNS whose eGFR measurements were above 60 mL/min/1.73 m2. The study revealed a good discriminatory power for uVDBP as a predicting parameter to distinguish SRNS from SSNS (AUC= 0.909, p<0.0001. The optimal uVDBP cut-off value of 5.781 μg/mL was associated with a sensitivity of 0.839 and specificity of 0.844 to differentiate SRNS from SSNS. Conclusion: Considering its significant discriminatory strength, uVDBP can be considered as a potential marker to noninvasively distinguish children with SRNS from those with SSNS.

Predictive risk factors of steroid dependent nephrotic syndrome in children with idiopathic nephrotic syndrome

Introduction: Steroid dependent nephrotic syndrome (SDNS) is a difficult and troublesome presentation of idiopathic nephrotic syndrome (INS) in children, with complicated management and increased morbidity. Objectives: The aim of this study was to identify the predictive clinical and laboratory characteristics of patients with SDNS, for parents counseling, appropriate management and improving outcome. Patients and Methods: A total of 374 children with steroid sensitive INS were evaluated in two groups as steroid dependent (group 1=199) and non-steroid dependent (group 2= 175) INS. SDNS was defined as ≥2 relapses during steroid reducing treatment or 15 days after discontinuation of corticosteroids. Results: Mean age at presentation was significantly lower in children with SDNS than those without steroid dependency (P=0.022). Diagnostic age less than two years (P=0.016), total relapses (P<0.001), relapse/year (P<0.001), body mass index (BMI) (P=0.002) and serum cholesterol level (P=0.042) were significantly higher in children with SDNS, compared to those with low-frequent relapse. Mean relapse rate decreased significantly in SDNS with immunosuppressive treatment (P<0.001). Conclusion: Age younger than two years at diagnosis, high BMI, high relapse rate/year and hypercholesterolemia at remission are suggested as predictors of SDNS in children with INS.

Serum miRNA-30a-5p in Steroid Sensitive Idiopathic Nephrotic Syndrome in Indian Children: A Case-control Study

Introduction: Childhood Nephrotic Syndrome (NS) is a podocytopathy. Micro Ribonucleic Acid (miRNA), composed of 21-25 non coding nucleotides, regulates gene expression by inhibiting protein transcription by binding to complementary messenger RNA. The microRNA-30a is expressed in the human glomerular podocytes and collecting ducts. This microRNA protects the podocytes by targeting the Calcineurin-nuclear Factor of Activated T cells (NFATc) pathways. Serum microRNA30a-5p is a validated biomarker which is upregulated in NS. Aim: To determine the serum miRNA-30a-5p in steroid sensitive idiopathic NS in Indian children. Materials and Methods: This case-control study was conducted at the Department of Paediatrics, KS Hegde Medical Academy, Mangalore, Karnataka, India, from from January 2018 to June 2019. Thirty children with NS and age and gender matched controls were recruited. Relative expression of microRNA-30a5p was analysed by Real-Time quantitative Polymerase Chain Reaction (RT-qPCR). Estimations were done both in cases and controls at enrollment and also at four weeks when in remission in cases. The fold change was calculated as a power of cycle threshold. Statistical tests Kolmogorov-Smirnov test was used to establish the normality using Statistical Package for Social Sciences (SPSS) version 22.0. Results: There was upregulation of microRNA-30a-5p expression among children with NS with a significant fold change (~184) at enrollment. The levels declined, but remained above baseline (~6) after four weeks of treatment when compared to controls. The mean differences in delta threshold cycle and threshold cycle between the three groups were significant (p<0.001). There was no correlation with the biochemical parameters. Conclusion: The present study concludes that serum microRNA-30a-5p expression is upregulated in children with steroid sensitive NS in Indian children.

Comparison of Lipid Profile in Different Types of Steroid Sensitive Idiopathic Relapsing Nephrotic Syndrome in Children during Active Disease and Remission

Introduction: Nephrotic syndrome is a disease of relapse and remission. Relapse rate is more than 80%. Hyperlipidemia and hypoalbuminemia are important characteristic of nephrotic syndrome. Hyperlipidemia persist even after remission of disease in frequent relapse nephrotic syndrome possibly due to frequent attack of disease and frequent use of steroid. Hyperlipidemia causes premature atherosclerosis, progressive renal injury leading to chronic renal failure, cardiac complications (myocardial infarction, hypertension), cerebrovascular disease and frequent relapse of nephrotic syndrome. Objectives: The aim of study was to see the lipid profile and comparison of lipid profile among different types of steroid sensitive idiopathic relapsing nephrotic syndrome during active disease and in remission. Materials and Methods: A cross sectional study included 120 ( 40 in each group) children aged 2-16 years with steroid sensitive idiopathic relapsing nephrotic syndrome patients who were admitted or attended in out patients department (OPD) in paediatric nephrology department Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, during December 2014 to December 2015. They were clinically examined and fasting lipid profile was done in each case during active disease and after one month of urinary remission. The study population were divided into three groups- Infrequent relapse nephrotic syndrome (IFRNS), frequent relapse nephrotic syndrome ( FRNS ) and steroid dependent nephrotic syndrome (SDNS) based on clinical response. Results: Total patients were 120 (40 in each group). The study showed a male predominance with a male to female ratio 2.24:1, male patients were 69%, female 31%. In all cases, there were increased mean total cholesterol, low density lipoprotein ( LDL ) , triglyceride (TG) and high density lipoprotein (HDL) was normal during active disease, more raised in FRNS and SDNS.There was significant decrease in the mean level of total cholesterol, LDL and triglyceride during remission ( p <0.001). Cholesterol became normal but triglyceride and LDL remained elevated even after one month of urinary remission in FRNS and SDNS. Conclusion: Hyperlipidemia persist during remission of steroid sensitive relapsing nephrotic syndrome. Children with FRNS and SDNS should be addressed with lipid lowering medication, healthy foods and healthy life style. Multicenter prospective studies with larger sample are needed for validating the findings of the present study. Medicine Today 2022 Vol.34(2): 111-116

FC067: Long-Term Outcome of Childhood Onset Idiopathic Nephrotic Syndrome

Abstract BACKGROUND AND AIMS Little is known about the long-term outcome during adulthood of childhood onset idiopathic nephrotic syndrome (INS). We aimed to determine which patients require long-term follow up after transition, to identify risk factors of relapse and to analyze treatment strategies. METHOD In this monocentric retrospective study, we included all patients admitted in our adult nephrology department with INS diagnosed during childhood. Patients who reached kidney failure during childhood were excluded. Data regarding the outcome at adult age were obtained through clinical database and medical charts. RESULTS Eighty-two patients (male/female = 2/1) were included, with a median age at diagnosis of 3.9 years (1.2–16.5). Sixty-eight patients had steroid-sensitive INS, including 52 with steroid-dependent nephrotic syndrome or frequently relapsing nephrotic syndrome. Fourteen patients had steroid-resistant nephrotic syndrome. A total of 89% of patients received corticosteroid sparing treatment during childhood. Median follow-up during adulthood was 6.2 (0.3–25) years. A total of 71% of patients experienced at least one relapse during adulthood. The total number of relapses during childhood and the number of relapses per year during childhood, reflecting disease activity, were significantly higher in patients who experienced relapses during adulthood than in patients who did not (Figure 1). The risk of relapse during adulthood was also significantly associated with the need for immunosuppressive regimen at the time of the transition visit (P = 0.002). To promote the successful transition of young people, we propose to organize a transition visit where the adolescent/young adult is seen jointly by pediatric and adult nephrologists, as it was done for 68% of patients in this study. The relapse rate was significantly lower (50%) in the subgroup of patients who had such a transition visit. We also found that relapse during the first two-years of adulthood follow-up was significantly associated with the risk of further relapse, highlighting the need for a close follow-up during the transition period. A total of 45% of patients received corticosteroid sparing treatment during adulthood, mainly mycophenolate mofetil (N = 23), calcineurin inhibitors (N = 21) and rituximab (N = 12). The main complications were high blood pressure (20/82, 26%) and osteopenia (19/26, 73% when bone densitometry was performed). Only one thrombo-embolic event and three severe infections were reported. At last follow-up, median eGFR was 87.1 (23.4–150.8) mL/min/1.73 m². CONCLUSION The incidence of relapses in adulthood is high in patients with active disease during childhood. A long-term follow-up is mandatory in these patients. Whereas renal function remained normal in most patients, high blood pressure and osteopenia were frequent and should be carefully monitored during adulthood. Transition visit should be carefully coordinated to prevent the risk of nonadherence.

Patterns, triggers, and predictors of relapses among children with steroid-sensitive idiopathic nephrotic syndrome at the University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria

Patterns, triggers, and predictors of relapses among children with steroid-sensitive idiopathic nephrotic syndrome at the University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria

Short Versus Long Steroid Therapy in Nephrotic Syndrome

Source: Pasini A, Bertulli C, Casadio L, et al. Childhood idiopathic nephrotic syndrome: does the initial steroid treatment modify the outcome? A multicentre, prospective cohort study. Front. Pediatr. 2021;9:627636; doi.10.3389/fped.2021.627636Investigators from multiple institutions in Italy conducted a prospective study comparing relapses in children with idiopathic nephrotic syndrome (INS) treated with different steroid regimens, with treatment based on time to remission (TTR). Study patients were children >6 months to <18 years old with a first episode of INS, defined as proteinuria >40 mg/m2/hr or urine protein/creatinine ration (uPr/UCr) >2 mg/mg, and serum albumin <2.5 g/dL who were treated at 49 different pediatric units in Italy between 2011 and 2016. Children with congenital or secondary nephrotic syndrome were excluded. TTR was defined as first day of negative/trace protein on daily dipstick assessment after initiation of treatment. Study patients with a TTR of ≤10 days received prednisone, 60 mg/m2/day for 4 weeks, while those with a TTR >10 days were treated with the same prednisone dose for 6 weeks. Both groups had the same steroid-tapering dose regimen. Study patients who did not achieve remission within 8 weeks were classified as steroid-resistant and excluded.Study participants were followed for 24 months, with relapses defined as 3 days of ≥2+ protein on urine dipstick confirmed by a uPr/UCr >2 mg/mg. Primary outcomes included any relapse, time to first relapse and number of relapses. These outcomes were compared between those with a TTR ≤10 days (group A) and those with a TTR >10 days (group B) using chi-square or non-parametric (Wilcoxon, Krukal-Wallis) tests. A Kaplan Meier analysis was used to compare relapse-free survival in patients in groups A and B.A total of 184 children were enrolled in the study, but 21 steroid-resistant and 20 non-compliant patients were excluded from the analyses. Among the remaining 143 participants, 100 (70%) had a TTR ≤10 days (group A) and 43 had a longer TTR (group B). Children in group A were significantly older (mean ages 4.73 and 4.14 years, respectively; P = 0.01) and had a lower mean uPr/UCr than those in group B. A relapse occurred in 118 (78%) participants, including 77% of those in group A and 79% of group B patients (P = 0.91). There was also no difference in number of relapses between participants in group A and B (mean number 1.8 and 2, respectively; P = 0.43). Mean time to first relapse was longer for children in group A than group B (206 vs 167 days; P = 0.05), but relapse-free survival was not significantly different between groups.The authors conclude that in children with INS, different steroid regimens, based on TTR, were not associated with risk of relapse.Dr Sanchez-Kazi has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.Nephrotic syndrome is one of the most common glomerular disorders in children.1 Kidney Disease Improving Global Outcomes (KDIGO) guidelines recommend treatment of the initial episode with 60 mg/m2 or 2 mg/kg daily for 4–6 weeks (maximum 60 mg) followed by alternate day prednisone at 40 mg/m2 or 1.5 mg/kg x 2–5 months with tapering of the dose.2 The current investigators assessed whether induction therapy of 4 weeks vs 6 weeks with the same duration of weaning will alter the frequency of relapses and development of frequently relapsing INS.There may be an inherent fault in the trial since the investigators used TTR as the randomization criterion. The results of previous studies demonstrated that TTR is not a significant factor in the prediction of the number of relapses in steroid sensitive INS.1 It would have been better if the randomization was done based on age. As reported in the current study, the younger age group had a higher number of relapses despite longer induction. Similar findings were reported in a meta-analysis of European children less than 4 years of age.1,3 Multiple randomized controlled studies in different countries also have shown that the standard therapy and further extension of the standard therapy did not alter the risk of frequent relapses in steroid sensitive INS (See AAP Grand Rounds. 2020;43:32.)4-7Shorter steroid therapy was not only cost-effective but associated with fewer adverse effects on growth and behavior due to lower cumulative dose.5–6 In the future, biomarkers may be useful in risk stratification of patients with steroid-sensitive INS and modifying the frequency of relapses.Duration and dose of steroid induction therapy do not modify the number of relapses in steroid-sensitive INS in children.

POS-130 EXPERIENCE WITH LEVAMISOLE IN CHILDREN WITH FREQUENTLY RELAPSING AND STEROID DEPENDENT NEPHROTIC SYNDROME HAVING FOLLOW UP AT TIKUR ANBESSA SPECIALIZED HOSPITAL, ADDIS ABABA, ETHIOPIA

Nephrotic syndrome is characterized by nephrotic range proteinuria, and hypoalbuminemia,most commonly associated with hyperlipidemia and edema. Without treatment,nephrotic syndrome in children is associated with high risk of death,most commonly from infections. Corticosteroids are the treatment of choice for idiopathic nephrotic syndrome (INS). Although about 80-90% of children with INS respond to corticosteroids, 20-30% become steroid dependent. Chronic corticosteroid therapy in patients with steroid dependent or frequently relapsing NS (FRNS or SDNS) is associated with numerous side effects. Levamisole is an anti helminthic agent with immunomodulatory effect. Levamisole has been considered the least toxic and least expensive steroid sparing drug for preventing relapses of steroid sensitive idiopathic nephrotic syndrome (SSINS). Though FDA doesn’t approve it for this purpose many researches done so far have shown that Levamisole is effective in treatment of children with FR/SDNS. We are using Levamisole in our Hospital but we have no data supporting or negating the use of levamisole as a good choice for treatment of children with SDNS/ FRNS to date. To determine the outcome of pediatric FR/SDNS treated with levamisole among children who had follow up at TASH pedaitric renal clinic. Methods: Retrospective cohort study on clinical records of patients with FR/SDNS having follow up at Tikur Anbessa Specialized Hospital from January 2016 to January 2019 . Charts of 80 patients reviewed and using exclusion criteria 33 patients were excluded from the study. Patient charts were retrieved using patients MRN and type of their disease from registration book at pediatrics renal clinic having follow up at our Hospital during the study period .The collected data was cleaned, Coded and analyzed using SPSS Version 25. Inclusion and Exclusion Criteria: Inclusion criteria: All patients who had FR/SD idiopathic nephrotic syndrome age between 1yr and 18 years attending follow up at TASH pediatric renal clinic. Exclusion criteria: All patients with steroid resistant nephrotic syndrome. All patients of Congenital or Infantile nephrotic syndrome. All patients with CKD. Nephrotic syndrome secondary to systemic illness Among 47 patients with FR/SDNS 32 were males and 15 were females, 21 (44.5%) were FRNS and 26 (55.5%) were SDNS. The duration of Nephrotic Syndrome was 8.75 3.7 yrs, the number of relapses before Levamisole treatment was 3, relapse after Levamisole was 0.7. The duration of Levamisole treatment was 19.9 during levamisole treatment. Levamisole is associated with significant reduction in relapses of FRNS/SDNS.It also significantly reduces adverse effects associated with corticosteroid treatment. The medication is not available in hospital pharmacies and treating those patients who have indications is difficult and the drug should be available at Hospital pharmacies.

Predictive Value of Serum Interleukins in Children with Idiopathic Nephrotic Syndrome.

Pro-inflammatory cytokines have been suggested in the pathogenesis of idiopathic nephrotic syndrome (INS), with conflicting results. This study was performed to identify alteration of different serum interleukins (ILs) in children with INS, and their predictive value in response to steroid treatment. Three groups of children (27; steroid-sensitive INS, 21; steroid-resistant INS, and 19 healthy controls) with normal serum C3, negative serologic tests of hepatitis B virus (HBV), hepatitis C virus (HCV), human immune deficiency virus (HIV), and parasitic infections were included in this study. Serum concentrations of IL-1β, IL-2, IL-6, IL-8, IL-13, and IL-18 were measured, using quantitative colorimetric sandwich ELISA kits. Children with secondary nephrotic syndrome, inflammations, systemic disorders, and chronic kidney disease were excluded. The serum concentration of all ILs; except IL-13 and IL-18; was significantly higher in children with INS, compared with the healthy controls. Serum IL-2 had the highest sensitivity of (95.24%) in patients with INS. All of the serum ILs had acceptable accuracy in children with INS, compared with the control group. The serum concentration of IL-1β, IL-6, and IL-8 was significantly higher in children with steroid-sensitive nephrotic syndrome (SSNS), compared with steroid-resistant nephrotic syndrome (SRNS). All of these ILs had acceptable accuracy for the prediction of steroid response in patients with INS. Our findings suggested the pathogenic role of pro-inflammatory cytokines in children with INS, of which IL-1β, IL-6, and IL-8 were accurate biomarkers for the prediction of steroid response in these patients.

Predictive Value of Platelet Aggregation Responses and Coagulation Parameters for Thromboembolic Risk in Children with Idiopathic Nephrotic Syndrome.

Introduction: Idiopathicnephrotic syndrome (INS) is the most common renal disease among children. INS present with proteinuria, hyperlipidemia, edema, hypoalbuminemia, and hypercoagulability. A serious consequence of INS is arterial and/or venous thromboembolism e.g., deep vein thrombosis (DVT), pulmonary emboli and renal vein thrombosis. This hypercoagulability may be due to urinary loss of small proteins, specifically antithrombin (AT) III, protein C and S. INS children have been observed to develop thrombocytosis and platelet hyper aggregation leading to increased thromboembolic risk. Aim of the Study: To evaluate the coagulation profile and results of platelet functions during active nephrosis in children with steroid sensitive and steroid resistant INS. Methods: Thirty-six children with INS were studied.Twenty-four steroid sensitive and 12 steroid resistant patients were evaluated for platelet function and coagulation parameters during active nephrosis (either at presentation or relapse). The study also included 15 healthy age and sex matched children as control.Doppler imaging was done to confirm thromboembolic events. Results: Thrombo-embolic complications were observed in 14/36 patients, diagnosed both clinically and/or by doppler.Prolonged PTT was observed during active nephrosis, compared to control. Hypoalbuminemia and hyperlipidemia contributed to hypercoagulability. Mean platelet aggregation to ADP and Ristocetin were significantly elevated among both groups (108.3±10.8% and 104.8±13.2% respectively). Levels of ATIII were significantly reduced, further adds to thrombotic risk. Conclusion: Thromboembolic complications are not uncommon in nephrotic children. Platelet hyperaggregability positively correlated with VTE in INS children and can be a beneficial predictor of thrombosis during active nephrosis especially those past history of VTE.

Interleukin-4 and Interleukin-13 Gene Polymorphisms in Children With Idiopathic Nephrotic Syndrome.

Background: Idiopathic Nephrotic syndrome (INS) is an immune-mediated disease in which a number of cytokines, including IL-4 and IL-13, have been implicated in the pathogenesis. Cytokine gene polymorphisms might affect their levels and activity. Therefore, may affect INS susceptibility and response to treatment. The aim of the study was to determine the association of IL-4 and IL-13 gene polymorphisms and INS susceptibility and their effects on steroid responsiveness in children.Methods: The polymorphisms in IL-4 and IL-13 genes were detected by PCR-RFLP in 155 INS patients and 64 controls.Results: A total of 132 steroid-sensitive (SS) and 23 steroid resistance (SR) INS patients; mean age 7.3 ± 4.0 years, were included. Male: Female ratio was 2:1. No significant statistical differences were detected in the frequency of CC, CT, and TT genotypes of IL-4 gene compared to controls (P = 0.57, 0.61, and 1.00, respectively). There was no significant difference in the T and C-allele frequencies, in SS and SR subgroups. Analysis of IL-13 gene polymorphism also did not show significant statistical differences in the frequency of QQ, RQ, and RR genotypes compared to controls (P = 0.74, 1.00, and 0.68, respectively). No significant difference was found in the Q and R-allele frequency. However, the heterozygous RQ genotype of the IL13 gene was significantly higher in SS INS patients compared to the SR INS cases (P = 0.04).Conclusion: Our findings did not show an association between IL-4 and IL-13 gene polymorphisms and INS susceptibility. However, IL-13 RQ genotype was expressed more in children with INS who are steroid sensitive.

Timing and predictive factors of developing chronic kidney disease in childhood-onset idiopathic nephrotic syndrome: an Asian experience.

Idiopathic nephrotic syndrome (INS) is the commonest type of nephrotic syndrome in children, and a majority of cases have favourable outcomes. A small proportion of INS cases progress to chronic kidney disease (CKD). We investigated the time to CKD and predictive risk factors associated with progression of CKD in these children. A retrospective review of medical records was done to investigate the demographic variables, and biochemical and histological changes in children with INS aged 12 months to 18 years between 2001 and 2016 at Hospital Universiti Sains Malaysia. The median renal survival time for progression to CKD stage III or higher was determined using survival curve analysis. Multiple Cox regression analysis was used to identify predictive factors for CKD. The total number of participants was 112 (boys: n = 71; girls: n = 41) and a majority had steroid-sensitive INS. Only about 10% of INS progressed to CKD Stage III or higher, with an overall median renal survival time of 19 years. Median renal survival time in steroid-resistant nephrotic syndrome (SRNS) was 13 years. Focal segmental glomerulosclerosis was predominant in SRNS. The predictors of progression to CKD were steroid resistance (adjusted hazard ratio [HR] [95% confidence interval (CI)] 23.8 [2.8-200.9]) and the presence of hypertension at presentation (adjusted HR [95% CI] 8.1 [1.2-55.7]). The median renal survival time in our study was comparable to other studies. SRNS and the presence of hypertension at presentation were the main predictors for developing CKD in our population.

Oral health conditions in children with idiopathic nephrotic syndrome: a cross-sectional study

BackgroundNephrotic syndrome is one of the chronic illnesses in the pediatric age group. The aim of this study was to assess the oral health of patients with steroid-sensitive idiopathic nephrotic syndrome (iNS).MethodsA case-control study was performed on iNS patients and healthy from May 2018 to April 2019. Dental caries was assessed by the World Health Organization criteria, developmental defects of enamel by the mDDE index, oral hygiene by the OHI-S and API, and gingival condition by the GI. Oral health behavior was recorded using a standardized questionnaire including tooth brushing, fluoride prevention, dietary habits and utilization of dental care. Additionally, Streptococcus mutans (SM) and Lactobacillus spp. (LB) bacteria in saliva were assessed using the CRT bacteria test. Statistical analysis comparing oral health parameters was carried by Pearson’s chi-squared, Fisher’s exact, Shapiro-Wilk verified by Student’s t or Mann-Whitney U tests.ResultsThe study included 94 participants of both sexes at the age of 4 to 17 years (47 cases and 47 controls) who were treated in Clinic of Pediatric Nephrology or outpatients’ dental clinic in Wroclaw, Poland. The iNS patients compared to the controls revealed some lower caries experience (83.0% vs 95.7%) and number of caries affected primary and/or permanent teeth (4.6 ± 3.5 vs 6.0 ± 4.1), a significantly lower number of filled primary and/or permanent teeth (1.1 ± 1.6 vs 3.5 ± 3.0, P < 0.001) and higher incidence of enamel hypoplasia (31.9% vs 4.3%, P < 0.001). The numbers of the iNS patients with high level of SM and LB were similar to the controls. The iNS patients had a higher OHI-S score (1.89 ± 1.59 vs 1.05 ± 1.02, P < 0.010) and a higher GI score (0.7 ± 1.0 vs 0.3 ± 0.6, P = 0.050). Moreover, they less frequently brushed their teeth twice a day (78.8% vs 93.6%, P = 0.026) and more frequently consumed three or more snacks daily (53.2% vs 23.4%, P = 0.002).ConclusionsThe iNS patients despite the poor oral hygiene revealed lower caries experience but smaller number of restored caries-affected teeth, more severe gingivitis and more frequently teeth affected by enamel hypoplasia.

Interferon gamma: is it a co-player in the pathogenesis of idiopathic nephrotic syndrome?

Introduction: Idiopathic nephrotic syndrome (INS), the most common form of NS in childhood, was considered 4 decades ago as a systemic disorder of T cells, mediated through its released cytokines. To date, the exact incriminated cytokine or immunological mediator is not properly defined. Interferon-gamma (IFN-gamma), a pro-inflammatory cytokine, is thought to have a role in the provocation of the T cell-mediated INS relapse, through the promotion of T helper1 (Th1) differentiation and suppression of regulatory T cells (Treg). Aim of the study: To evaluate the immunopathogenic role of IFN-gamma in children with steroid-sensitive idiopathic nephrotic syndrome (SSNS) through monitoring the changes in its levels with disease course. Methods: This study included twenty-five newly diagnosed children with SSINS. They were all given full dose prednisolone, evaluated at initial diagnosis and at full remission as regards the serum level of IFN-gamma. Results: Serum levels of IFN-gamma were lowermost at the time of diagnosis and increased with remission on corticosteroids. Conclusions: This study points to a role for the lower serum IFN-gamma at diagnosis, in the immunopathogenesis of INS than at remission and the rise in its serum level might be a marker of remission induction, however, this awaits confirmation in larger-scale studies. Studies on renal biopsy specimens are needed to determine the exact renal in situ levels and effects of IFN-gamma

Extended initial course of prednisolone did not improve clinical outcomes in steroid-sensitive nephrotic syndrome

Study design: Double-blind, parallel-group, randomised, placebo-controlled trial. Setting and patients: 237 patients with steroid-sensitive idiopathic nephrotic syndrome aged 1–14 years recruited from 125 UK tertiary nephrology centres and district general...

Influence of prednisone therapy on physical development of children with nephrotic syndrome

THE AIM: to compare the parameters of physical development (PD) in children with idiopathic nephrotic syndrome (INS), depending on the treatment with prednisone.PATIENTS AND METHODS. The effect of treatment with prednisone on PD was analyzed in 60 children with INS aged from 2 to 17 years. The children were divided into 2 groups: 30 children who did not receive prednisone, and 30 children who received it during the last 6 months before the study (1st and 2nd group, respectively). The groups compared the anamnestic parameters and the risk factors of children in terms of length, weight, and body mass index.RESULTS. When comparing the characteristics of the risk factors of children of the above 2 groups, differences in body mass and BMI were established. In children who received prednisone for the last 6 months, body weight and BMI were significantly exceeded compared to WHO standards and similar patients who did not receive prednisone for the last 6 months. We have established a reliable association of the Z-BMI criterion with the cumulative dose of prednisone in the last 6 months: r = 0.49, p &lt;0.05. At the same time, no reliable association of body weight with a cumulative dose of prednisone, which the child received before 6 months, has been identified. When analyzing the effectiveness of different doses of prednisone therapy for stopping relapses in children with steroid-sensitive INS, it was found that the onset and duration of remission did not significantly differ when taking standard (60mg/m2/day or 2 mg/kg/day) and half as much (1mg/kg/day) doses of prednisone.CONCLUSION. The relationship of the body mass of children with INS and the cumulative dose of prednisone in the last 6 months has been established. When treating a recurrent steroid-sensitive non-relapsing INS, a decrease in the daily dose of prednisone from 2 mg/kg/day to 1 mg/kg/day is possible in adolescents who are afraid of steroid obesity or who have had severe complications during previous courses of prednisone therapy.

Pediatric Patients With Steroid-Sensitive Nephrotic Syndrome Have Higher Expression of T Regulatory Lymphocytes in Comparison to Steroid-Resistant Disease.

Background and Aim: Idiopathic nephrotic syndrome (INS) is classified according to the response to drug therapy in steroid-sensitive (SS), steroid-dependent (SD), and steroid-resistant (SR) categories. Previous studies showed changes in inflammatory activity of subpopulations of lymphocytes in INS. This study aimed to compare SS and SR patients in regard to subpopulations of leukocytes, profile of regulatory lymphocytes, and migratory activity of lymphocyte subpopulations. Results obtained in INS patients were also compared to age and sex-matched healthy controls.Methods: This is a cross-sectional study including SS patients (n = 30), SR patients (n = 14), and controls (n = 10). Peripheral blood samples were withdrawn for ex-vivo leukocyte flow cytometry analysis.Results: Percentage of B-lymphocytes and natural killer (NK) cells were significantly reduced in SR patients when compared to controls, while the percentage of NKT cells were decreased in SS patients in comparison to controls. Percentages of CD4+ expressing FoxP3 and CTLA4 were significantly higher in SS patients in comparison to SR patients and controls. The expression of integrin CD18 on the surface of T lymphocytes (CD3+) was reduced in SS patients if compared to controls.Conclusion: This study found that SS INS patients have higher levels of regulatory T-lymphocytes and lower expression of adhesion molecules than SR patients.

Influenza vaccination among children with idiopathic nephrotic syndrome: an investigation of practices

BackgroundAnnual influenza vaccination is recommended for all children with idiopathic nephrotic syndrome (INS) in France. Consequently, the Social Security automatically sends prescriptions to all patients suffering from a chronic disease. The aim of this study was to evaluate the follow-up to these recommendations.MethodsWe conducted a monocentric retrospective investigation of practices. We included all children with steroid-sensitive INS in remission who attended our clinics from January 1st 2015 to January 1st 2017, resided in France and had a valid phone number. Data were collected from May 2017 to June 2017 through a phone interview and review of clinical charts.Results75 patients met the inclusion criteria. The parents of 57 children could be reached by phone and agreed to participate to the survey. 35/57 (61.4%) declared having received a prescription during the 2016–2017 campaign. Only 14 children (24.6%) were vaccinated. 17/43 (39.5%) parents of unvaccinated children had concerns about the safety of the vaccine, 16/43 (37.2%) were not aware of the recommendations, 5/43 (11.6%) had been recommended by their physician not to vaccinate their child, 3/43 (7%) forgot to have them vaccinated and 2/43 (4.6%) reported no reason. 13/43 (30%) unvaccinated children presented a relapse during the flu season - 2/13 during an influenza-like illness - whereas 1/14 (7%) immunized children presented a relapse during the six months of post-vaccination follow-up. Relapse rates were not increased in vaccinated children compared to unvaccinated children (p = 0.15), nor in the 6 months following vaccination compared to the 6 months prior (1/14 vs 5/14, p = 0.20).Conclusions1) < 2/3 patients were properly prescribed the recommended yearly influenza vaccination at our center 2) only 1/4 were vaccinated and most of their parents were misinformed. Physicians must be aware of this and should make every effort to better inform their patients on the risks of flu illness and the benefits and safety of the vaccination.

Initial treatment of steroid-sensitive idiopathic nephrotic syndrome in children with mycophenolate mofetil versus prednisone: protocol for a randomised, controlled, multicentre trial (INTENT study)

IntroductionIdiopathic nephrotic syndrome is the most common glomerular disease in childhood with an incidence of 1.8 cases per 100 000 children in Germany. The treatment of the first episode implies...

A randomized clinical trial indicates that levamisole increases the time to relapse in children with steroid-sensitive idiopathic nephrotic syndrome

Levamisole has been considered the least toxic and least expensive steroid-sparing drug for preventing relapses of steroid-sensitive idiopathic nephrotic syndrome (SSINS). However, evidence for this is limited as previous randomized clinical trials were found to have methodological limitations. Therefore, we conducted an international multicenter, placebo-controlled, double-blind, randomized clinical trial to reassess its usefulness in prevention of relapses in children with SSINS. The efficacy and safety of one year of levamisole treatment in children with SSINS and frequent relapses were evaluated. The primary analysis cohort consisted of 99 patients from 6 countries. Between 100 days and 12 months after the start of study medication, the time to relapse (primary endpoint) was significantly increased in the levamisole compared to the placebo group (hazard ratio 0.22 [95% confidence interval 0.11-0.43]). Significantly, after 12 months of treatment, six percent of placebo patients versus 26 percent of levamisole patients were still in remission. During this period, the most frequent serious adverse event (four of 50 patients) possibly related to levamisole was asymptomatic moderate neutropenia, which was reversible spontaneously or after treatment discontinuation. Thus, in children with SSINS and frequent relapses, levamisole prolonged the time to relapse and also prevented recurrence during one year of treatment compared to prednisone alone. However, regular blood controls are necessary for safety issues.