Aya Inaba1, Yuko Hamasaki2, Kenji Ishikura3,4, Riku Hamada3, Tomoyuki Sakai5, Hiroshi Hataya3, Fumiyo Komaki6, Tetsuji Kaneko7,8, Masaaki Mori1, Masataka Honda3. 1. Department of Pediatrics, Yokohama City University Medical Center, Kanagawa, Japan. 2. Department of Pediatric Nephrology, Toho University Faculty of Medicine, 6-11-1, Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan. yuhamasaki@med.toho-u.ac.jp. 3. Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan. 4. Department of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan. 5. Department of Pediatrics, Shiga University of Medical Science, Shiga, Japan. 6. Community Health Welfare Division, Kawasaki Saiwai Ward Office Health and Welfare Center, Kanagawa, Japan. 7. Department of Clinical Research, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan. 8. Teikyo Academic Research Center, Teikyo University, Tokyo, Japan.
Abstract
BACKGROUND: Several 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. METHODS: Sixty-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). RESULTS: The 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 %). CONCLUSIONS: Our 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.
BACKGROUND: Several 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. METHODS: Sixty-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). RESULTS: The 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 %). CONCLUSIONS: Our 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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