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Olson, J.L. and Schwartz, M.M. (1998) The Nephrotic Syndrome: Minimal Change Disease, Focal Segmental Glomerulosclerosis, and Miscellaneous Causes. In: Jennette, J.C., Olson, J.L., Schwartz, M.M. and Silva, F.G., Eds., Heptinstall’s Pathology of the Kidney, Lippincott-Raven Publishers, Philadelphia, 187-257.

has been cited by the following article:

• 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.