BACKGROUND: Conventional wisdom states that greater than 80% of children with nephrotic syndrome (NS) respond to steroid treatment, remain steroid-sensitive during subsequent relapses, and consequently have a favorable long-term prognosis. In contrast, steroid resistance is believed to be associated with a high risk of developing chronic renal failure. Recent reports suggest that the histologic pattern of NS in children may be changing, but whether the change is accompanied by a parallel change in steroid sensitivity is unknown. METHODS: Initial and subsequent steroid responsiveness was evaluated in all children aged 1 to 18 years who presented with newly diagnosed NS to the 2 pediatric nephrology referral centers in southeastern Louisiana between 1994 and 2003. NS was defined as presence of edema, heavy proteinuria, and serum albumin concentration below 2.5 g/dL. Steroid sensitivity (SS) was defined as total resolution of proteinuria and edema, and partial response to steroids (PR) was defined as loss of edema with continuing proteinuria. RESULTS: There were 210 new cases of NS. Forty-one patients (20%) had immune complex glomerulonephritis. Six patients were excluded because of incomplete data availability. Of the remaining 163 patients, 115 (71%) were SS and 23 (14%) achieved PR during the initial 4 weeks of treatment; 25 (15%) were steroid-resistant (SR). Follow-up data were available for 91 of the 115 initially SS patients; 19 subsequently became steroid-resistant. Thus, at least 45% of the patients with new-onset NS did not have typical childhood steroid-responsive NS. Initial steroid resistance was more likely in African American children and in children with older age at onset (11.5 vs. 4.6 years). Development of steroid resistance after initial SS was associated with shorter interval to the first relapse (2.2 vs. 5.4 months) and having the first relapse during the initial steroid treatment. CONCLUSION: Compared to previous reports, our results show a higher incidence of initial and subsequent steroid resistance, characteristics not consistent with typical minimal change NS with a benign prognosis. The results suggest that in the current era, NS in children may not be as benign as indicated by earlier studies.
BACKGROUND: Conventional wisdom states that greater than 80% of children with nephrotic syndrome (NS) respond to steroid treatment, remain steroid-sensitive during subsequent relapses, and consequently have a favorable long-term prognosis. In contrast, steroid resistance is believed to be associated with a high risk of developing chronic renal failure. Recent reports suggest that the histologic pattern of NS in children may be changing, but whether the change is accompanied by a parallel change in steroid sensitivity is unknown. METHODS: Initial and subsequent steroid responsiveness was evaluated in all children aged 1 to 18 years who presented with newly diagnosed NS to the 2 pediatric nephrology referral centers in southeastern Louisiana between 1994 and 2003. NS was defined as presence of edema, heavy proteinuria, and serum albumin concentration below 2.5 g/dL. Steroid sensitivity (SS) was defined as total resolution of proteinuria and edema, and partial response to steroids (PR) was defined as loss of edema with continuing proteinuria. RESULTS: There were 210 new cases of NS. Forty-one patients (20%) had immune complex glomerulonephritis. Six patients were excluded because of incomplete data availability. Of the remaining 163 patients, 115 (71%) were SS and 23 (14%) achieved PR during the initial 4 weeks of treatment; 25 (15%) were steroid-resistant (SR). Follow-up data were available for 91 of the 115 initially SS patients; 19 subsequently became steroid-resistant. Thus, at least 45% of the patients with new-onset NS did not have typical childhood steroid-responsive NS. Initial steroid resistance was more likely in African American children and in children with older age at onset (11.5 vs. 4.6 years). Development of steroid resistance after initial SS was associated with shorter interval to the first relapse (2.2 vs. 5.4 months) and having the first relapse during the initial steroid treatment. CONCLUSION: Compared to previous reports, our results show a higher incidence of initial and subsequent steroid resistance, characteristics not consistent with typical minimal change NS with a benign prognosis. The results suggest that in the current era, NS in children may not be as benign as indicated by earlier studies.
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
Authors: Mona Khurana; Avram Z Traum; Manuel Aivado; Meghan P Wells; Manuel Guerrero; Franck Grall; Towia A Libermann; Asher D Schachter Journal: Pediatr Nephrol Date: 2006-06-30 Impact factor: 3.714
Authors: Agnes Trautmann; Monica Bodria; Fatih Ozaltin; Alaleh Gheisari; Anette Melk; Marta Azocar; Ali Anarat; Salim Caliskan; Francesco Emma; Jutta Gellermann; Jun Oh; Esra Baskin; Joanna Ksiazek; Giuseppe Remuzzi; Ozlem Erdogan; Sema Akman; Jiri Dusek; Tinatin Davitaia; Ozan Özkaya; Fotios Papachristou; Agnieszka Firszt-Adamczyk; Tomasz Urasinski; Sara Testa; Rafael T Krmar; Lidia Hyla-Klekot; Andrea Pasini; Z Birsin Özcakar; Peter Sallay; Nilgun Cakar; Monica Galanti; Joelle Terzic; Bilal Aoun; Alberto Caldas Afonso; Hanna Szymanik-Grzelak; Beata S Lipska; Sven Schnaidt; Franz Schaefer Journal: Clin J Am Soc Nephrol Date: 2015-01-29 Impact factor: 8.237
Authors: Eugen Widmeier; Merlin Airik; Hannah Hugo; David Schapiro; Johannes Wedel; Chandra C Ghosh; Makiko Nakayama; Ronen Schneider; Agape M Awad; Anish Nag; Jang Cho; Markus Schueler; Catherine F Clarke; Rannar Airik; Friedhelm Hildebrandt Journal: J Am Soc Nephrol Date: 2019-02-08 Impact factor: 10.121