Skip to main content
Erschienen in: Pediatric Nephrology 7/2011

01.07.2011 | Original Article

Clinical course and outcome of children with steroid-sensitive nephrotic syndrome

verfasst von: Seyed Taher Esfahani, Abbas Madani, Fahimeh Asgharian, Neamatollah Ataei, Azadeh Roohi, Mastaneh Moghtaderi, Parvin Rahimzadeh, Mohammad-Hasan Moradinejad

Erschienen in: Pediatric Nephrology | Ausgabe 7/2011

Einloggen, um Zugang zu erhalten

Abstract

We conducted a retrospective study on children with primary nephrotic syndrome (NS) to evaluate the clinical course and outcome of children with steroid-sensitive NS (SSNS). The medical records of 226 children, median 3.46 years (min 1.00, max 15.08) who referred to our clinics with SSNS between January 1978 and September 2005 were reviewed and entered into the study. Minimum duration of follow-up was 5 years and maximum 20 years (median 7.25 years). Of 226 patients who were treated with corticosteroids, 38 (16.8%) had no relapse but the remaining 188 (83.2%) patients experienced several relapses of which 128 patients (56.6%) required additional immunosuppressive agents for the remission. Of these, 122 (95%) were treated with levamisole, 22 (17%) with cyclosporine, 36 (28%) with cyclophosphamide, and ten (7.8 %) treated with mycophenolate mofetil. Several patients had to switch from one medication to others due to lack of response. On the last follow-up visit, 64(28.3%) patients were still under treatment, some patients had taken all of the above-mentioned drugs but still had multiple recurrences. Only 103 (45.5%) patients were in remission off the drug more than 3 years. This study shows that nearly one-third of pediatric patients with SSNS experience frequent relapses despite the combination of multiple immunosuppressive medications, which may continue until adulthood.
Literatur
1.
Zurück zum Zitat Hodson EM, Willis NS, Craig JC (2008) Non-corticosteroid treatment for nephrotic syndrome in children. Cochran Database Syst Rev 23(1):CDOO2290 Hodson EM, Willis NS, Craig JC (2008) Non-corticosteroid treatment for nephrotic syndrome in children. Cochran Database Syst Rev 23(1):CDOO2290
2.
Zurück zum Zitat Report of the International Study of Kidney Disease in Children (1984) Minimal change nephrotic syndrome in children: deaths during the first 5 to 15 years' observation. Pediatrics 73(4):497–501 Report of the International Study of Kidney Disease in Children (1984) Minimal change nephrotic syndrome in children: deaths during the first 5 to 15 years' observation. Pediatrics 73(4):497–501
3.
Zurück zum Zitat Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS (1985) Long-term outcome for children with minimal-change nephrotic syndrome. Lancet 16(1(8425)):368–370CrossRef Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS (1985) Long-term outcome for children with minimal-change nephrotic syndrome. Lancet 16(1(8425)):368–370CrossRef
4.
Zurück zum Zitat Koskimies O, Vilska J, Rapola J, Hallman N (1982) Long-term outcome of primary nephrotic syndrome. Arch Dis Child 57:544–548CrossRef Koskimies O, Vilska J, Rapola J, Hallman N (1982) Long-term outcome of primary nephrotic syndrome. Arch Dis Child 57:544–548CrossRef
5.
Zurück zum Zitat Ruth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ (2005) Children with steroid-sensitive nephrotic syndrome come of age: long–term outcome. J Pediatr 147(2):202–207CrossRef Ruth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ (2005) Children with steroid-sensitive nephrotic syndrome come of age: long–term outcome. J Pediatr 147(2):202–207CrossRef
6.
Zurück zum Zitat Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P, Lesavre P, Chauveau D, Knebelmann B, Broyer M, Grünfeld JP, Niaudet P (2003) Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis 41(3):550–557CrossRef Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P, Lesavre P, Chauveau D, Knebelmann B, Broyer M, Grünfeld JP, Niaudet P (2003) Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis 41(3):550–557CrossRef
7.
Zurück zum Zitat Arneil GC (1956) Treatment of nephrosis with prednisolone. Lancet 1:409–411CrossRef Arneil GC (1956) Treatment of nephrosis with prednisolone. Lancet 1:409–411CrossRef
8.
Zurück zum Zitat (1981) International study of kidney Disease in children. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisolone, J Pediatr 98:561–564 (1981) International study of kidney Disease in children. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisolone, J Pediatr 98:561–564
9.
Zurück zum Zitat (1979) Alternate–day versus intermittent prednisolone in frequently relapsing nephrotic syndrome. A report of Arbetsgemeinschaft für Pädiatrische Nephrologie: Lancet 1(8113):401–403 (1979) Alternate–day versus intermittent prednisolone in frequently relapsing nephrotic syndrome. A report of Arbetsgemeinschaft für Pädiatrische Nephrologie: Lancet 1(8113):401–403
10.
Zurück zum Zitat Bagga A, Hari P, Srivastava RN (1999) Prolonged versus standard prednisolone therapy for initial episode of nephrotic syndrome. Pediatr Nephrol 13:824–827CrossRef Bagga A, Hari P, Srivastava RN (1999) Prolonged versus standard prednisolone therapy for initial episode of nephrotic syndrome. Pediatr Nephrol 13:824–827CrossRef
11.
Zurück zum Zitat Brodehl J (1991) The treatment of minimal change nephritic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr 150:380–387CrossRef Brodehl J (1991) The treatment of minimal change nephritic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr 150:380–387CrossRef
12.
Zurück zum Zitat Ehrich JH, Brodehl J (1993) Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children; Arbeitsgemeinschaft für Pädiatrische Nephrologie. Eur J Pediatr 152(4):357–361CrossRef Ehrich JH, Brodehl J (1993) Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children; Arbeitsgemeinschaft für Pädiatrische Nephrologie. Eur J Pediatr 152(4):357–361CrossRef
13.
Zurück zum Zitat Ueda N, Chihara M, Kawaguchi S, Niinomi Y, Nonoda T, Matsumoto J, Ohnishi M, Yasaki T (1988) Intermittent versus long-term tapering prednisolone for initial therapy in children with idiopathic nephrotic syndrome. J Pediatr 112(1):122–216CrossRef Ueda N, Chihara M, Kawaguchi S, Niinomi Y, Nonoda T, Matsumoto J, Ohnishi M, Yasaki T (1988) Intermittent versus long-term tapering prednisolone for initial therapy in children with idiopathic nephrotic syndrome. J Pediatr 112(1):122–216CrossRef
14.
Zurück zum Zitat Ksiázek J, Wyszyńska T (1995) Short versus long initial prednisone treatment in steroid-sensitive nephrotic syndrome in children. Acta Paediatr 84(8):889–893CrossRef Ksiázek J, Wyszyńska T (1995) Short versus long initial prednisone treatment in steroid-sensitive nephrotic syndrome in children. Acta Paediatr 84(8):889–893CrossRef
15.
Zurück zum Zitat Jayantha UK (2004) Comparison of ISKDC regime with a 7 month regime in the first attack of nephrotic syndrome (Abstract). Pediatr Nephrol 19(9):C81 Jayantha UK (2004) Comparison of ISKDC regime with a 7 month regime in the first attack of nephrotic syndrome (Abstract). Pediatr Nephrol 19(9):C81
16.
Zurück zum Zitat Hodson EM., Willis NS, Craig IC (2007) Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 17(4):CD001533 Hodson EM., Willis NS, Craig IC (2007) Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 17(4):CD001533
17.
Zurück zum Zitat Abeyagunawardena A, Dillon MJ, Rees L, Van’t Hoff W, Trompeter RS (2003) The use of steroid-sparing agents in steroid-sensitive nephrotic syndrome. Pediatr Nephrol 18(9):919–924CrossRef Abeyagunawardena A, Dillon MJ, Rees L, Van’t Hoff W, Trompeter RS (2003) The use of steroid-sparing agents in steroid-sensitive nephrotic syndrome. Pediatr Nephrol 18(9):919–924CrossRef
18.
Zurück zum Zitat Boyer O, Moulder JK, Grandin L, Somers MJ (2008) Short and long-term efficacy of levamisole as adjunctive therapy in childhood nephrotic syndrome. Pediatr Nephrol 23(4):575–580CrossRef Boyer O, Moulder JK, Grandin L, Somers MJ (2008) Short and long-term efficacy of levamisole as adjunctive therapy in childhood nephrotic syndrome. Pediatr Nephrol 23(4):575–580CrossRef
19.
Zurück zum Zitat Durkan AM, Hodson EM, Willis NS, Craig JC (2001) Immunosuppressive agents in childhood nephrotic syndrome: a meta- analysis of randomized controlled trials. Kidney Int 59(5):1919–1927CrossRef Durkan AM, Hodson EM, Willis NS, Craig JC (2001) Immunosuppressive agents in childhood nephrotic syndrome: a meta- analysis of randomized controlled trials. Kidney Int 59(5):1919–1927CrossRef
20.
Zurück zum Zitat Sumegi V, Haszon I, Ivanyi B, Bereczki C, Papp F, Turi S (2004) long-term effects of levamisole treatment in childhood nephrotic syndrome. Pediatr Nephrol 19(12):1354–1360CrossRef Sumegi V, Haszon I, Ivanyi B, Bereczki C, Papp F, Turi S (2004) long-term effects of levamisole treatment in childhood nephrotic syndrome. Pediatr Nephrol 19(12):1354–1360CrossRef
21.
Zurück zum Zitat Ginevri F, Trivelli A, Ciardi MR, Chiggeri CM, Parfumo F, Gusmano R (1996) Protracted levamisole in children with frequent relapse nephrotic syndrome. Pediatr Nephrol 10(4):550PubMed Ginevri F, Trivelli A, Ciardi MR, Chiggeri CM, Parfumo F, Gusmano R (1996) Protracted levamisole in children with frequent relapse nephrotic syndrome. Pediatr Nephrol 10(4):550PubMed
22.
Zurück zum Zitat El-Hosseini A, El-Basuony F, Mahmoud I, Sheashaa H, Sabry A, Hassan R, Taha N, Hassan N, Seyed-Ahmad N, Sobh M (2005) Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: a single- center experience. Nephrol Dial Transplant 20(11):2433–2438CrossRef El-Hosseini A, El-Basuony F, Mahmoud I, Sheashaa H, Sabry A, Hassan R, Taha N, Hassan N, Seyed-Ahmad N, Sobh M (2005) Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: a single- center experience. Nephrol Dial Transplant 20(11):2433–2438CrossRef
23.
Zurück zum Zitat Kemper MJ, Kuwertz-Broeking E, Bulla M, Mueller-Wiefel DE, Neuhaus TJ (2004) Recurrence of severe steroid dependency in cyclosporin A-treated childhood idiopathic nephrotic syndrome. Nephrol Dial Transplant 19(5):1136–1141CrossRef Kemper MJ, Kuwertz-Broeking E, Bulla M, Mueller-Wiefel DE, Neuhaus TJ (2004) Recurrence of severe steroid dependency in cyclosporin A-treated childhood idiopathic nephrotic syndrome. Nephrol Dial Transplant 19(5):1136–1141CrossRef
24.
Zurück zum Zitat Ishikura K, Ikeda M, Hattori S, Yoshikawa N, Sasaki S, Iijima K, Nakanishi K, Yata N, Honda M (2008) Effective and safe treatment with cyclosporine in nephrotic children: a prospective, randomized multicenter trial. Kidney Int 73(10):1167–1173CrossRef Ishikura K, Ikeda M, Hattori S, Yoshikawa N, Sasaki S, Iijima K, Nakanishi K, Yata N, Honda M (2008) Effective and safe treatment with cyclosporine in nephrotic children: a prospective, randomized multicenter trial. Kidney Int 73(10):1167–1173CrossRef
25.
Zurück zum Zitat Hino S, Takemura T, Okada M, Murakami K, Yagi K, Fukushima K, Yoshioka K (1998) Follow-up study of children with nephrotic syndrome treated with a long-term moderate dose of cyclosporine. Am J Kidney Dis 31(6):932–939CrossRef Hino S, Takemura T, Okada M, Murakami K, Yagi K, Fukushima K, Yoshioka K (1998) Follow-up study of children with nephrotic syndrome treated with a long-term moderate dose of cyclosporine. Am J Kidney Dis 31(6):932–939CrossRef
26.
Zurück zum Zitat Bagga A (2008) Management of steroid-sensitive nephrotic syndrome: revised guidelines. Indian Pediatr 45:203–214 Bagga A (2008) Management of steroid-sensitive nephrotic syndrome: revised guidelines. Indian Pediatr 45:203–214
27.
Zurück zum Zitat Iijima K, Hamahira K, Tanaka R, Kobayashi A, Nozu K, Nakamura H, Yoshikawa N (2002) Risk factors for cyclosporine-induced tubulointerstitial lesions in children with minimal change nephrotic syndrome. Kidney Int 61(5):1801–1805CrossRef Iijima K, Hamahira K, Tanaka R, Kobayashi A, Nozu K, Nakamura H, Yoshikawa N (2002) Risk factors for cyclosporine-induced tubulointerstitial lesions in children with minimal change nephrotic syndrome. Kidney Int 61(5):1801–1805CrossRef
28.
Zurück zum Zitat Magnasco A, Rossi A, Catarsi P, Gusmano R, Ginevri F, Perfumo F, Chiggeri GM (2008) Cyclosporine and organ specific toxicity: clinical aspects, pharmacogenetics and prospectives. Curr Clin Pharmacol 3(3):166–173CrossRef Magnasco A, Rossi A, Catarsi P, Gusmano R, Ginevri F, Perfumo F, Chiggeri GM (2008) Cyclosporine and organ specific toxicity: clinical aspects, pharmacogenetics and prospectives. Curr Clin Pharmacol 3(3):166–173CrossRef
29.
Zurück zum Zitat Barratt TM, Soothil JF (1970) controlled trials of cyclophosphamide in steroid-sensitive relapsing nephrotic syndrome of childhood. Lancet 2:479–482CrossRef Barratt TM, Soothil JF (1970) controlled trials of cyclophosphamide in steroid-sensitive relapsing nephrotic syndrome of childhood. Lancet 2:479–482CrossRef
30.
Zurück zum Zitat Kyrieleis HA, levtchenko EN, Wetzels JF (2007) Long–term outcome after cyclophosphamide treatment in children with steroid dependent and frequently relapsing minimal change nephrotic syndrome. Am J Kidney Dis 49:592–597CrossRef Kyrieleis HA, levtchenko EN, Wetzels JF (2007) Long–term outcome after cyclophosphamide treatment in children with steroid dependent and frequently relapsing minimal change nephrotic syndrome. Am J Kidney Dis 49:592–597CrossRef
31.
Zurück zum Zitat Bagga A, Hari P, Moudgil A, Jordan SC (2003) Mycophenolate mofetil and prednisolone therapy in children with steroid-dependent nephrotic syndrome. Am J Kidney Dis 42(6):1114–2110CrossRef Bagga A, Hari P, Moudgil A, Jordan SC (2003) Mycophenolate mofetil and prednisolone therapy in children with steroid-dependent nephrotic syndrome. Am J Kidney Dis 42(6):1114–2110CrossRef
32.
Zurück zum Zitat Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, Nauta J, Hop WC, van der Heijden AJ (2008) Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol 23(11):2013–2020CrossRef Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, Nauta J, Hop WC, van der Heijden AJ (2008) Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol 23(11):2013–2020CrossRef
Metadaten
Titel
Clinical course and outcome of children with steroid-sensitive nephrotic syndrome
verfasst von
Seyed Taher Esfahani
Abbas Madani
Fahimeh Asgharian
Neamatollah Ataei
Azadeh Roohi
Mastaneh Moghtaderi
Parvin Rahimzadeh
Mohammad-Hasan Moradinejad
Publikationsdatum
01.07.2011
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Nephrology / Ausgabe 7/2011
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-011-1837-6

Weitere Artikel der Ausgabe 7/2011

Pediatric Nephrology 7/2011 Zur Ausgabe

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.