TITLE:
Histopathology Review of Idiopathic Steroid Resistant Nephrotic Syndrome and Outcome in Children in North-West of Iran
AUTHORS:
Mahtab Rahbar
KEYWORDS:
Idiopathic Nephrotic Syndrome, Steroid-Resistance, Histopathology, Outcome, Child
JOURNAL NAME:
Open Journal of Nephrology,
Vol.6 No.4,
December
28,
2016
ABSTRACT: Introduction: There is currently little information in the
literature on the spectrum of histopathologic
patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic
distribution of different subtypes’ glomerular morphologic patterns in iSRNS
and the clinical and biochemical parameters at the time of diagnosis and
outcome of patients after immunosuppressive
therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone
therapy (60 mg/m2/day) referred to
nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic,
clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy
reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients
separately. This study investigated prognostic
effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59
(29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ±
4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of
102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children
with iSRNS secondary to MCD are more likely to achieve remission and have
better long term prognostic value (P 0.00). Focal segmental
glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive
therapy (P 0.04). Conclusions: This study defines the
true spectrum of clinicohistopathology
patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the
underlying histopathology patterns which have been earned by adequate renal
biopsy.