Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children.
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.
Department of Pediatrics, Yokohama City University Medical Center, Kanagawa, Japan.
Aya
A
Inaba
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.
Yuko
Y
Hamasaki
Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.|Department of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Kenji
K
Ishikura
Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Riku
R
Hamada
Department of Pediatrics, Shiga University of Medical Science, Shiga, Japan.
Tomoyuki
T
Sakai
Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Hiroshi
H
Hataya
Community Health Welfare Division, Kawasaki Saiwai Ward Office Health and Welfare Center, Kanagawa, Japan.
Fumiyo
F
Komaki
Department of Clinical Research, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.|Teikyo Academic Research Center, Teikyo University, Tokyo, Japan.
Tetsuji
T
Kaneko
Department of Pediatrics, Yokohama City University Medical Center, Kanagawa, Japan.
Masaaki
M
Mori
Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Masataka
M
Honda
D000293:Adolescent; D049109:Cell Proliferation / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D005920:Glomerular Mesangium / Q000187:drug effects* / Q000473:pathology; D005923:Glomerulosclerosis, Focal Segmental / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009402:Nephrosis, Lipoid / Q000175:diagnosis / Q000188:drug therapy*; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000175:diagnosis / Q000188:drug therapy; D012074:Remission Induction; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003520:Cyclophosphamide
Children; Diffuse mesangial proliferation; Focal segmental glomerulosclerosis; Immunosuppressant; Long-term outcome; Minimal change; Steroid-resistant nephrotic syndrome