BACKGROUND: To investigate the long-term outcome in children with frequently relapsing nephrotic syndrome (FRNS) we conducted a follow-up of a previous randomized controlled trial (RCT) 10 years after the initiation of the treatment protocol. METHODS: We previously conducted an RCT on the efficacy of cyclosporine for treating children with FRNS. After 2 years of treatment, a recommended a management protocol of steroids, and immunosuppressants was provided. RESULTS: Valid information was available for 46 of the 56 patients (82.1 %) enrolled in the original RCT. The median follow-up period was 10.3 years from the start of protocol treatment with cyclosporine. At last follow-up (mean age 18.7 years), only ten patients (21.7 %) showed disease-free remission (no relapse for at least 2 years). In contrast, 23 (50.0 %) continued to relapse frequently or were on immunosuppressants, eight patients (17.4 %) had infrequent relapses without immunosuppressants. Adverse effects attributable to treatment included short stature (6 patients), osteoporosis (six patients), obesity (4 patients), cataracts (3 patients) and hypertension (3 patients). No lethal event or renal dysfunction occurred during follow-up. CONCLUSIONS: This 10-year follow-up study shows that most children with FRNS experience relapses after 2 years ofcyclosporine treatment, in adolescence and into adulthood. Outcomes in terms of life expectancy and renal function are favorable.
RCT Entities:
BACKGROUND: To investigate the long-term outcome in children with frequently relapsing nephrotic syndrome (FRNS) we conducted a follow-up of a previous randomized controlled trial (RCT) 10 years after the initiation of the treatment protocol. METHODS: We previously conducted an RCT on the efficacy of cyclosporine for treating children with FRNS. After 2 years of treatment, a recommended a management protocol of steroids, and immunosuppressants was provided. RESULTS: Valid information was available for 46 of the 56 patients (82.1 %) enrolled in the original RCT. The median follow-up period was 10.3 years from the start of protocol treatment with cyclosporine. At last follow-up (mean age 18.7 years), only ten patients (21.7 %) showed disease-free remission (no relapse for at least 2 years). In contrast, 23 (50.0 %) continued to relapse frequently or were on immunosuppressants, eight patients (17.4 %) had infrequent relapses without immunosuppressants. Adverse effects attributable to treatment included short stature (6 patients), osteoporosis (six patients), obesity (4 patients), cataracts (3 patients) and hypertension (3 patients). No lethal event or renal dysfunction occurred during follow-up. CONCLUSIONS: This 10-year follow-up study shows that most children with FRNS experience relapses after 2 years of cyclosporine treatment, in adolescence and into adulthood. Outcomes in terms of life expectancy and renal function are favorable.
Authors: Markus J Kemper; Jutta Gellermann; Sandra Habbig; Rafael T Krmar; Katalin Dittrich; Therese Jungraithmayr; Lars Pape; Ludwig Patzer; Heiko Billing; Lutz Weber; Martin Pohl; Katrin Rosenthal; Anne Rosahl; Dirk E Mueller-Wiefel; Jörg Dötsch Journal: Nephrol Dial Transplant Date: 2011-11-09 Impact factor: 5.992
Authors: Debbie S Gipson; Susan F Massengill; Lynne Yao; Shashi Nagaraj; William E Smoyer; John D Mahan; Delbert Wigfall; Paul Miles; Leslie Powell; Jen-Jar Lin; Howard Trachtman; Larry A Greenbaum Journal: Pediatrics Date: 2009-07-27 Impact factor: 7.124