Best practice guidelines for idiopathic nephrotic syndrome: recommendations versus reality

Background The optimal therapeutic regimen for managing childhood idiopathic nephrotic syndrome (INS) is still under debate. We have evaluated the choice of steroid regimen and of symptomatic treatment adopted by pediatricians and pediatric nephrologists in a large number of centers as the first step towards establishing a shared protocol Methods This was a multicenter, retrospective study. A total of 231 children (132 admitted to pediatric units) aged 6 months to Results Median steroid dosing was 55 (range 27-75) mg/[m.sup.2]/ day. The overall median cumulative dose regimen for the first episode was 3,440 (1,904-6,035) mg/[m.sup.2], and the median duration of the therapeutic regimen was 21 (9-48) weeks. The total duration and cumulative steroid dose were significantly higher in patients treated by pediatricians than in those treated by pediatric nephrologists (p=0.001 and p=0.008). Among the patient cohort, 55, 64 and 22 % received albumin infusions, diuretics and acetyl salicylic acid treatment, respectively, but the laboratory and clinical data did not differ between children treated or not treated with symptomatic drugs. Albumin and diuretic use did not vary between patients in pediatric units and those in pediatric nephrology units. Conclusions This study shows major differences in steroid and symptomatic treatment of nephrotic syndrome by pediatricians and pediatric nephrologists. As these differences can influence the efficacy of the treatments and the appearance of side-effects, shared guidelines and their implementation through widespread educational activities are necessary. Keywords Idiopathic nephrotic syndrome * Steroid regimen * Albumin infusion * Thromboembolic prophylaxis Guidelines
Introduction Idiopathic nephrotic syndrome (INS) is a rare disease (2-7 cases/year per 100,000 children aged In the early phase of the disease, symptomatic treatment is also important as many severe [...]