Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children View Full Text


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Article Info

DATE

2015-09-03

AUTHORS

Aya Inaba, Yuko Hamasaki, Kenji Ishikura, Riku Hamada, Tomoyuki Sakai, Hiroshi Hataya, Fumiyo Komaki, Tetsuji Kaneko, Masaaki Mori, Masataka Honda

ABSTRACT

BackgroundSeveral recent studies have shown improved short-term outcome of steroid-resistant nephrotic syndrome (SRNS) in children; however, only a few studies have evaluated the long-term outcome. The aims of our study were to obtain detailed data and analyze the long-term outcome of children with SRNS.MethodsSixty-nine children with idiopathic SRNS were enrolled and divided into two groups based on initial histopathological patterns: focal segmental glomerulosclerosis (FSGS) and minimal change (MC)/diffuse mesangial proliferation (DMP). The effects of initial treatment with the immunosuppressant of choice (cyclosporine or cyclophosphamide) on renal survival, remission, and incidence of complications were analyzed in both groups (4 subgroups).ResultsThe renal survival rate was significantly different among the four different subgroups based on different combinations of initial histopathological pattern (FSGS vs. MC/DMP) and initial immunosuppressant used for treating SRNS (cyclosporine vs. cyclophosphamide) (P = 0.013), with renal survival in the FSGS (cyclophosphamide) subgroup being especially low (54.6 %). Disease- and/or treatment-associated complications were relatively low; however, hypertension at last examination was observed in a considerable number of patients (31.9 %).ConclusionsOur results suggest that a recently developed therapeutic regimen with cyclosporine considerably improves both the initial remission rate and the long-term renal survival rate of children with idiopathic SRNS. More... »

PAGES

425-434

References to SciGraph publications

  • 2006-01-05. Differential risk of remission and ESRD in childhood FSGS in PEDIATRIC NEPHROLOGY
  • 1990-07. Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents in PEDIATRIC NEPHROLOGY
  • 2009-08-28. Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome in PEDIATRIC NEPHROLOGY
  • 2008-09-01. Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome—a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie in PEDIATRIC NEPHROLOGY
  • 2008-09-01. Efficacy of intravenous pulse cyclophosphamide treatment versus combination of intravenous dexamethasone and oral cyclophosphamide treatment in steroid-resistant nephrotic syndrome in PEDIATRIC NEPHROLOGY
  • 2003-03-21. Intravenous cyclophosphamide in steroid-resistant nephrotic syndrome in PEDIATRIC NEPHROLOGY
  • 2015-02-06. Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy in CLINICAL AND EXPERIMENTAL NEPHROLOGY
  • 2006-10-21. Primary focal segmental glomerular sclerosis in children: clinical course and prognosis in PEDIATRIC NEPHROLOGY
  • 2013-06-19. Chronic cyclosporine-induced nephrotoxicity in children with steroid-resistant nephrotic syndrome in PEDIATRIC NEPHROLOGY
  • 2009-08-01. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study in PEDIATRIC NEPHROLOGY
  • 2004-08-18. Efficacy of methylprednisolone pulse therapy in steroid-resistant nephrotic syndrome in PEDIATRIC NEPHROLOGY
  • 2001-11. Treatment of focal glomerulosclerosis with pulse steroids and oral cyclophosphamide in PEDIATRIC NEPHROLOGY
  • 2013-01-13. Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis in PEDIATRIC NEPHROLOGY
  • 2001-08. Primary focal segmental glomerulosclerosis in children: prognostic factors in PEDIATRIC NEPHROLOGY
  • 2006-05-30. Predictive factors of chronic kidney disease in primary focal segmental glomerulosclerosis in PEDIATRIC NEPHROLOGY
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/s00467-015-3174-7

    DOI

    http://dx.doi.org/10.1007/s00467-015-3174-7

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1033405510

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/26335197


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    40 schema:description BackgroundSeveral recent studies have shown improved short-term outcome of steroid-resistant nephrotic syndrome (SRNS) in children; however, only a few studies have evaluated the long-term outcome. The aims of our study were to obtain detailed data and analyze the long-term outcome of children with SRNS.MethodsSixty-nine children with idiopathic SRNS were enrolled and divided into two groups based on initial histopathological patterns: focal segmental glomerulosclerosis (FSGS) and minimal change (MC)/diffuse mesangial proliferation (DMP). The effects of initial treatment with the immunosuppressant of choice (cyclosporine or cyclophosphamide) on renal survival, remission, and incidence of complications were analyzed in both groups (4 subgroups).ResultsThe renal survival rate was significantly different among the four different subgroups based on different combinations of initial histopathological pattern (FSGS vs. MC/DMP) and initial immunosuppressant used for treating SRNS (cyclosporine vs. cyclophosphamide) (P = 0.013), with renal survival in the FSGS (cyclophosphamide) subgroup being especially low (54.6 %). Disease- and/or treatment-associated complications were relatively low; however, hypertension at last examination was observed in a considerable number of patients (31.9 %).ConclusionsOur results suggest that a recently developed therapeutic regimen with cyclosporine considerably improves both the initial remission rate and the long-term renal survival rate of children with idiopathic SRNS.
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