Immunosuppressive therapy in children with steroid-resistant, frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome: a single center experience

Introduction: Immunosuppressive agents are recommended for the management of children with steroidresistant (SRNS), frequently-relapsing (FRNS), and steroid-dependent idiopathic nephrotic syndrome (SDNS). This study evaluated the efficacy of immunosuppressive agents in these cases. Methods: This is a retrospective analysis of the records of 130 pediatric cases recruited from a tertiary-care center over a period of two years. They were divided into two groups: 51 patients with SRNS (Group I) and 79 cases with SDNS and FRNS (Group II). They were treated with immunosuppressive agents in addition to steroids, either as double- or triple-combination therapy. Complete or partial remission was considered a good response. Results: In group I, the proportions of good response to cyclophosphamide, cyclosporine A, and mycophenolate mofetil were 48.6, 60, and 80%, respectively (p = 0.162). In group II, the resistance rate was significantly higher with levamisole than with cyclophosphamide and azathioprine (p = 0.046). Leukopenia was reported infrequently after the administration of cyclophosphamide or azathioprine. The most serious adverse reaction was to cyclosporine A, which induced nephrotoxicity (6.4%), while no adverse effects related to levamisole were reported. Histopathological diagnoses were available in only 39 patients. Conclusion: The high potency of cyclosporine with steroids recommends its use in patients with idiopathic SRNS with a normal glomerular filtration rate. Its efficacy is augmented when combined with mycophenolate mofetil. Cyclophosphamide, orally or as intravenous boluses, together with alternate-day steroids, could be a good option outside the peripubertal age. The outcomes of FRNS and SDNS could be improved by encouraging compliance with the use of levamisole

Prevalence of Viral Infection among Egyptian Children with End Stage Renal Disease

Abstract: Background: Viral infections are frequent in hemodialysis patients, notably those due to hepatitis C virus (HCV), hepatitis B virus (HBV), hepatitis G virus (HGV) cytomegalovirus (CM V) and human immunodeficiency virus (HIV). Objective: The aim of this work is to study the prevalence of viral infections among Egyptian children with end stage renal disease whether on conservative management or on hemodialysis and to identify the possible associations between viral infections and some clinical parameters. Subjects: This cross-section study included 50 patients with end stage renal disease. They were divided into two groups; the first group consisted of 20 patients on conservative management. The second group consisted of 30 patients on regular hemodialysis. Inclusion criteria: Children below 18 years, both gender, end stage renal disease patients whether on conservative management or on hemodialysis. Exclusion criteria: Patients on immunosuppressant treatment for any particular disease. M ethods: All patients were subjected to full history, thorough clinical examination and laboratory investigations in the form of complete blood count, renal function tests, and serum electrolytes. Abdominal ultrasound and echocardiography were also done. In addition virological screen was assessed (conventional PCR for HCV, HGV and CMV and ELISA for HBV, HCV and HIV). Results: The comparative study between patients on conservative management (group I) and patients on hemodialysis (group II) showed no statistical significant difference between the two groups as regard to gender and age. 60% of the examined patients in each group were male. The mean age in group I was 11.48 ± 4.06 year while in group II it was 10.27 ± 3.20 year (P=0.253). However, great statistical differences was found between both groups in the mean onset of chronic renal failure it was 5.93 ±2.67 year in group I and 3.40 ± 1.30 year in group II (P=0.002). Also significant statistical differences were found between both groups as regard to the presence of anemia, (P=0.000) and history of blood transfusions (P =0.002). 100% of hemodialysis patients had anemia and 86.7% of them received blood. Hypertension was present in 35% of group I and in 63.3% of group II patients (P= 0.049). Stunted growth was found in 50% of patients on conservative management and in 83.3% of patients on hemodialysis (P=0.012). Laboratory studies showed no statistical significant differences between both groups as regard to hemoglobin, hematocrit, ALT, AST, calcium, phosphorus and alkaline phosphatase enzyme. Also no statistical significant difference was found in blood urea between both groups. W hereas there was a significant statistical difference in creatinine level between the two groups. The mean creatinine level in group I patients = 3.49 ± 1.72 mg/dl while in hemodialysis patients =7.16 ± 1.41 mg/dl (P=0.000). Abdominal ultrasound showed that the common cause of chronic renal failure (CRF) in patients on conservative management was obstructive uropathies (60% of cases). W hile congenital malformations were the commonest cause of CRF in hemodialysis patients (60%). Echocardiography showed that 50% and 10% of hemodialysis patients had left ventricular hypertrophy and pericardial effusion respectively. The virological studies showed that the commonest viral infection in both groups was HCV. It was detected by PCR in 35% of group I and in 50% of group II patients as single infection or as coinfection with other viruses. Cytomegalovirus was present in 20% of group I and in 10% of group II patients. HGV was only present in hemodialysis patients (13.3% of cases). HCV antibodies were detected by ELISA test in 15% of patient on conservative management and in 43.3% of patients on hemodialysis therapy P =0.035. No antibodies for HBV and HIV were detected in our patients. Significant association was found between viral infections and patient's age (P= 0.043). Also significant association was found between viral infections and duration of hemodialysis (P=0.015). But no significant associations were found between viral infections and both frequency of dialysis settings (P=0.485) and patient's gender (p=0.361). Conclusion and Recommendations: Viral infections are frequent in hemodialysis patients. Aust. J. Basic & Appl. Sci., 3(4): 3479-3491, 2009 3480 Strict infection control measures in dialysis units may help in decreasing the risk of infection. Both PCR and ELISA tests are required to maximize HCV diagnostic sensitivity. W e also recommended more researches to explore the prevalence of viral infections among children with end stage renal disease in the different nephrology departments and renal dialysis units

Egyptian pediatric clinical practice adapted guidelines: evidence-based [2] steroid-resistant nephrotic syndrome (SRNS) 2022

Abstract Background Nephrotic syndrome is one of the most common chronic kidney diseases in children. Steroid sensitive type (SSNS) constitutes about 85–90%, whereas steroid-resistant type (SRNS) only 15–20% (Mickinney et al. Pediatr Nephrol 16:1040-1044, 2001). While MCD is the most common histopathology in SS type, children with SRNS have MCD, mesangial proliferative glomerulonephritis, or focal and segmental glomerulosclerosis (FSGS) (International Study Kidney Disease in children, Kidney Int 20;765-771, 1981). SRNS is defined as those who do not show remission after 6 weeks and standard dose of oral steroids ± 3 IV MPD doses (Trautmann et al. Pediatr Nephrol 35:1529-1561, 2020). Objectives These national adapted guidelines aim to frame evidence-based recommendations adopted or adapted from the IPNA 2020, KDIGO 2021, and Japanese 2014 de novo guidelines for diagnosis and management of nephrotic children to be presented in two manuscripts: (1) steroid sensitive (SSNS) and (2) steroid-resistant nephrotic syndrome (SRNS). Methodology Formulation of key questions was followed with a review of literature guided by our appraised guidelines using AGREE plus appraisal tool. Virtual monthly meetings all through the year 2021 were activated  for reviewing and validation of final adaptation evidence-based draft, considering all comments of external reviewers including KDIGO assigned reviewer. Discussion Rationale behind the selection of adopted statements and tailoring of others to suit our local facilities, expertise, and our local disease profile was discussed in the text with reasons. Conclusion The provided guidelines aim to optimize patient care and outcome and suggest research areas lacking validated research recommendations