Claire Dossier1, Jean-Daniel Delbet2, Olivia Boyer3, Patrick Daoud4, Bettina Mesples5, Beatrice Pellegrino6, Helène See7, Gregoire Benoist8, Anne Chace9, Anis Larakeb10, Julien Hogan11, Georges Deschênes11. 1. Department of Pediatric Nephrology, Hôpital Armand-Trousseau, APHP, Paris, France. claire.dossier@aphp.fr. 2. Department of Pediatric Nephrology, Hôpital Armand-Trousseau, APHP, Paris, France. 3. Department of Pediatric Nephrology, Hôpital Necker-Enfants-Malades, APHP, Paris, France. 4. Department of Pediatrics and Neonatology, Centre Hospitalier Intercommunal André-Grégoire, Montreuil, France. 5. Department of Pediatrics, Hôpital Louis-Mourier, APHP, Colombes, France. 6. Department of Pediatrics, CH Francois-Quesnay, Mantes-La-Jolie, France. 7. Department of Pediatrics, Centre Hospitalier Intercommunal Robert-Ballanger, Aulnay Sous Bois, France. 8. Department of Pediatrics, Hôpital Ambroise-Paré, APHP, Boulonge-Billancourt, France. 9. Department of Pediatrics, Centre Hospitalier Intercommunal de Villeneuve Saint-Georges, Villeneuve Saint-Georges, France. 10. Department of Pediatrics, Centre Hospitalier de Meaux, Meaux, France. 11. Department of Pediatric Nephrology, Hôpital Robert-Debré, APHP, 48 Bd Serurier, 75019, Paris, France.
Abstract
BACKGROUND: The optimal therapeutic regimen for children at onset of idiopathic nephrotic syndrome (INS) is still under debate. A better knowledge of the disease's course is necessary to design more appropriate and/or personalized treatment protocols. METHODS: We report the 5-year outcome of patients included from December 2007 to May 2010 in the prospective multicentric and multiethnic population-based NEPHROVIR study. Patients were treated at onset according to the French steroid protocol (3990 mg/m2, 18 weeks). Data were collected at 5 years or last follow-up. RESULTS: Out of the 188 children with nephrotic syndrome (121 boys, 67 girls; median age 4.1 years), 174 (93%) were steroid-sensitive. Six percent of steroid-sensitive patients required intravenous steroid pulses to get into remission. Relapse-free rate for steroid-sensitive patients was 21% (36/174) at last follow-up (median 72 months). A first relapse occurred in138 steroid sensitive patients (79%) with a median time of 8.3 months (IQ 3.4-11.3). Out of the 138 relapsers, 43 were frequent relapsers. Age at onset below 4 years was the only predictive factor of relapse, while gender, ethnicity, and delay to first remission were not. At 96 months of follow-up, 83% of frequent relapsers were still under steroids and/or immunosuppressive drugs. CONCLUSIONS: The treatment of the first flare deserves major improvements in order to reduce the prevalence of relapsers and the subsequent long-lasting exposure to steroids and immunosuppression.
BACKGROUND: The optimal therapeutic regimen for children at onset of idiopathic nephrotic syndrome (INS) is still under debate. A better knowledge of the disease's course is necessary to design more appropriate and/or personalized treatment protocols. METHODS: We report the 5-year outcome of patients included from December 2007 to May 2010 in the prospective multicentric and multiethnic population-based NEPHROVIR study. Patients were treated at onset according to the French steroid protocol (3990 mg/m2, 18 weeks). Data were collected at 5 years or last follow-up. RESULTS: Out of the 188 children with nephrotic syndrome (121 boys, 67 girls; median age 4.1 years), 174 (93%) were steroid-sensitive. Six percent of steroid-sensitive patients required intravenous steroid pulses to get into remission. Relapse-free rate for steroid-sensitive patients was 21% (36/174) at last follow-up (median 72 months). A first relapse occurred in138 steroid sensitive patients (79%) with a median time of 8.3 months (IQ 3.4-11.3). Out of the 138 relapsers, 43 were frequent relapsers. Age at onset below 4 years was the only predictive factor of relapse, while gender, ethnicity, and delay to first remission were not. At 96 months of follow-up, 83% of frequent relapsers were still under steroids and/or immunosuppressive drugs. CONCLUSIONS: The treatment of the first flare deserves major improvements in order to reduce the prevalence of relapsers and the subsequent long-lasting exposure to steroids and immunosuppression.
Authors: Mohan Shenoy; Nicholas D Plant; Malcolm A Lewis; Mark G Bradbury; Rachel Lennon; Nicholas J A Webb Journal: Pediatr Nephrol Date: 2010-01-27 Impact factor: 3.714