Clinical course of asymptomatic nephrotic children without edema

Abstract

We retrospectively analyzed the clinical course of 93 consecutive children with nephrotic syndrome (NS) who had been followed-up at Saitama Children’s Medical Center for at least one year since initial presentation. Of the 93, eight were asymptomatic without edema (asymptomatic patients) and were identified by chance proteinuria in urinary screening program. A total of 80% (75/93) were classified as having steroid-sensitive NS (seven in asymptomatic patients and 68 in symptomatic patients). The mean number of days to complete remission after initial steroid therapy in asymptomatic patients was significantly shorter than that in symptomatic patients (6.0 vs 10.0 days, p<0.01). Furthermore, none of the seven asymptomatic patients with steroid-sensitive NS developed FR/SDNS during follow-up period. Although all of 17 symptomatic patients with steroid-resistant NS achieved complete remission after the initiation of immunosuppressive agents, WT-1 mutation was detected in one asymptomatic patient with steroid and cyclosporine-resistant NS. We conclude that genetic testing should be done for asymptomatic patients with steroid-resistant NS before the initiation of immunosuppressive agents.