The Spectrum of Histopathological Lesions in Children Presenting with Steroid-Resistant Nephrotic Syndrome at a Single Center in Pakistan

Muhammed Mubarak,Javed I Kazi,Seema Hashmi,Shaheera Shakeel,Ali Lanewala

doi:10.1100/2012/681802

Abstract

Steroid-resistant nephrotic syndrome (SRNS) is a common problem in pediatric nephrology practice. There is currently little information in the literature on the spectrum of histopathologic lesions in children presenting with SRNS in Pakistan. This study was designed to determine the histopathologic lesions in children presenting with SRNS at our center. The study was conducted at the Histopathology Department, Sindh Institute of Urology and Transplantation (SIUT) from January 2009 to August 2011. All children (≤16 years) presenting with SRNS, in whom renal biopsies were performed, were included. Their demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy forms. The results were analyzed by SPSS version 10.0. A total of 147 children were included. Of these, 91 (61.9%) were males and 56 (38.1%) females, with male-to-female ratio of 1.6 : 1. The mean age was 7.03 ± 4.0 years (range: 6 months–16 years). The histopathological lesions seen on renal biopsies comprised of focal segmental glomerulosclerosis (FSGS) (38.5%), followed by minimal change disease (MCD) (23.2%), IgM nephropathy (IgMN) (13.6%), idiopathic mesangial proliferative GN (10.2%), membranous GN (8.2%), and mesangiocapillary GN (4.8%). Our results indicate that FSGS is the predominant lesion in children with SRNS, followed by MCD and IgMN.

Highlights

Steroid-resistant nephrotic syndrome (SRNS) is a common problem in pediatric nephrology practice and one that poses significant therapeutic challenge for pediatric nephrologists [1,2,3,4]
The spectrum of histopathological lesions seen on renal biopsies was wide and comprised of focal segmental glomerulosclerosis (FSGS) (38.7%), followed by minimal change disease (MCD) (23.1%), IgM nephropathy (13.6%), idiopathic mesangial proliferative GN (MesPGN) (10.2%), membranous GN (MN) (8.2%), mesangiocapillary or membranoproliferative GN (MPGN) (4.8%), and a number of rare lesions (Table 2)
These results demonstrate the predominance of FSGS in children with SRNS

Summary

Introduction

Steroid-resistant nephrotic syndrome (SRNS) is a common problem in pediatric nephrology practice and one that poses significant therapeutic challenge for pediatric nephrologists [1,2,3,4]. We have found earlier a steroidresistant pattern of INS in 30% of our children who underwent renal biopsy [14]. There are very few studies in the literature on the histopathological spectrum of glomerulopathies underlying SRNS in children [9,10,11,12]. There is currently little information about the histopathologic spectrum of lesions in children presenting with SRNS in Pakistan [15, 16]. There are data in the literature that suggest that the pattern of histopathology underlying INS is changing in adults and in children [17,18,19,20]. It became imperative to determine the true pattern of the prevailing glomerulopathies underlying SRNS in a large cohort of children who presented to our center

Methods

Results

Conclusion