Abstract
Objectives:To determine the effectiveness of levamisole in maintaining remission of proteinuria in children with frequent relapsing and steroid dependent nephrotic syndrome (FR/SDNS).Methods:This observational study on 81 children with FR /SDNS was carried out from June 2007 - June 2017 at The Kidney Center-Postgraduate Training Institute, Karachi-Pakistan. Levamisole (leva) along with low dose prednisolone on alternate day (AD) was used after induction of remission with daily oral prednisolone in children with FR/ SDNS for 6-36 months. Patients with steroid resistance were excluded. Data was analyzed using descriptive statistics.Results:Eighty-one patients with FR (66) or SD (15) received levamisole treatment. Mean age at diagnosis was 3.72 ±2.33 years. Levamisole was used on AD in 59.25% and daily in 40.74% of cases. Twenty-four could not complete six months and were excluded, 57 patients completed treatment duration of 15.68±9.93 months and 51 post-leva follow-up of 11.70±11.23 months. Mean leva-dose was 1.73±0.67 mg/kg/ patient. Mean cumulative prednisolone dose per patient before, on-leva and post-leva was 3389.81±2785.22, 2471.97±2024.98 and 661.37± 905.37 mg respectively. Mean relapse rate per year before leva, on -leva and post -leva was 3.30 ±0.50,0.98± 1.1and 0.79±1.27 respectively. Levamisole was effective in 90% of patients. During post-leva follow up, 76.4% patients, maintained remission, whereas 23.5% behaved as FR/SD and require further immunosuppressive therapy.Conclusions:Levamisole was effective in maintaining remission in 90% while on treatment, whereas it maintained remission after discontinuation in 76.4% cases. Levamisole may be used as first steroid sparing agent before other immunosuppressive therapies in children with FR/SDNS. Further studies are required for optimal duration and dosage schedule.
Highlights
Majority of children with nephrotic syndrome are steroid sensitive minimal change disease(MCD), but after initial response to standard corticosteroid therapy, more than 80% experience relapses.[1]
We in this study report our experience of levamisole as the first-line alternative steroid sparing agent in FRNS and SDNS
Based on subsequent number and frequency of relapses, patients were categorized as frequent relapses (FR) if >2 relapses in 6 months or > 4 in a 12 months’ period and SDNS if child develops two consecutive relapses on steroid therapy or within 14 days of switching to alternate day (AD) prednisolone.[12]
Summary
Majority of children with nephrotic syndrome are steroid sensitive minimal change disease(MCD), but after initial response to standard corticosteroid therapy, more than 80% experience relapses.[1] Among the steroid sensitive children, 40-60 % experience frequent relapses (FR) and 30-50% of MCD become steroid dependent (SDNS).[1] Patients with FR and SD who require repeated. Pak J Med Sci September - October 2020 Vol 36 No 6 www.pjms.org.pk 1193 courses of steroid are at risk of long -term steroid toxicities and serious infections, may affect the qualities of life.[2] In children with FRNS, steroids can be stopped intermittently but not in SDNS and patients with SD are more vulnerable to steroid toxicity and require aggressive immunosuppressive protocols.[1,3]. Management strategies for children with FR and SDNS, include prolonged use of low dose oral prednisolone (OP) on alternate day (AD), levamisole along with AD low dose OP, cyclophosphamide (CPM) for 2-3 months, mycophenolate mofetil (MMF) and calcineurin inhibitors (CNIs) like cyclosporine or tacrolimus.[4,5,6] Rituximab (anti-CD20) has been used in developed countries for difficult cases of FRNS /SDNS.[7]
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