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Paper Information

Journal:   TEHRAN UNIVERSITY MEDICAL JOURNAL (TUMJ)   2003 , Volume 61 , Number 1; Page(s) 71 To 79.
 
Paper: 

CLINICOPATHOLOGICAL STUDY AND RESULTS OF TREATMENT IN 502 CHILDREN WITH PRIMARY NEPHROTIC SYNDROME (1981-2000)

 
 
Author(s):  MADANI ABAS*, ATAEI N., FALAK ALAFLAKI B.
 
* 
 
Abstract: 

Introduction: Primary nephrotic syndrome (PNS) accounts for approximately 90% of nephrosis in childhood. There are well defined therapeutic protocols for childhood nephrosic syndrome to minimizing side effects of corticosteroid and repeated relapses. The aim of this study was to assess the clinicopathological aspects and results of treatment in children with PNS.
Methods and Materials: We evaluated 502 patients with PNS from 1981-2000. Among these children 5 (1%) achieved spontaneous remission, and 313 children were initial responder. One hundred eighty four patients received at least 1 kidney biopsy (78 prior and 106 after initiation of treatment).
Results: Of these patients histology showed minimal change nephrosic syndrome (MCNS) in 67 (36.4%), focal segmental glomerular sclerosis (FSGS) in 41 (22.3%), diffuse mesangial proliferation (DMP) in 28 (15.2%), membranoproliferative glomerulonephritis (MPGN) in 21 (11.4%), focal proliferative glomerulonephritis (FPGN) in 12 (6.5%), congenital nephrosic syndrome (CNS) in 8 (4.3%) and membranous nephropathy (MN) in 7 (3.8%). Of 103 children with frequently relapsing steroid sensitive and steroid dependent idiopathic nephrosis, levamisol induced prolong remission in 33 (31.7%) patients. Cyclophosphamid and cyclosporine induced remission in 49 (50%) of 98 and 28 (41.3%) of 68 patients respectively.
Conclusion: Our observation suggests that patients with steroid-dependent nephrosic syndrome or MCNS had better response to cyclophosphamide or cyclosporine than patients with steroid-resistant nephrosic syndrome or FSGS (P<0.05). Additionally , our experience indicate that levamisole treatment is of considerable clinical benefit in patients with frequent relapsers and steroid dependent nephrosic syndrome.

 
Keyword(s): PRIMARY NEPHROTIC SYNDROME, CLINICOPATHOLOGICAL STUDY - TREATMENT - OUTCOME
 
References: 
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