Long-Term Study in Children with Steroid-Resistant Nephrotic Syndrome Progressing to End-Stage Renal Disease

Abstract

Introduction:Steroid-resistant nephrotic syndrome (SRNS) in children is a glomerular disease who often fails to respond to immunosuppressive treatment and is a leading cause for progression to end-stage renal disease (ESRD) and dialysis. Some risk factors, that appear to be common in patients with SRNS and progression to ESRD, have been identified and reported: focal and segmental glomeruloslerosis on kidney biopsy, high range persistent proteinuria, microscopic hematuria, hypertension, episodes of acute kidney injury (AKI) and resistance to immunosuppressive agents. The challenge is to identify these risk factors and improve patients' management, because children with ESRD have many associated complications and a high rate of morbidity and mortality. Objective: The aim of our study is to observe the incidence of SRNS in our patients and identify the presence of common risk factors in those progressing to ESRD with requirement for dialysis or kidney transplant. Material and methods:We studied a total number of 125 pacients who were diagnosed with nephrotic syndrome in the Department of Pediatric Nephrology of "M. S. Curie" Emergency Clinical Hospital for Children, Bucharest, Romania, from January 2013 to December 2020. Twenty six patients diagnosed with SRNS were included in our study; all of them underwent clinical examination and laboratory tests and were regularly monitored to assess the progression of kidney disease to ESRD. Discussion and results:Steroid-resistant nephrotic syndrome is associated with an increased risk for developing ESRD with requirement for dialysis and transplant. Resistance to immunosuppressive agents was associated with ESRD in our patients. Focal segmental glomerulosclerosis (FSGS), the most common histopathologic lesion, had no value for progression to ESRD in our study. Hematuria, persistent high value proteinuria, hypertension and episodes of AKI were found in our patients with SRNS and progressed to ESRD.

FIGURE 1.

Nephrotic syndrome defined by the response to steroid therapy in steroid-sensitive nephrotic syndrome (SSNS), steroid-dependent nephrotic syndrome (SDNS) and steroid-resistant nephrotic syndrome (SRNS)

FIGURE 2.

Renal histopathologic features in nephrotic syndrome: minimal changes disease (MCD); focal and segmental glomerulosclerosis (FSGS); membranous nephropathy (MN); lupus membranous nephropathy (LMN)

FIGURE 3.

Minimal changes disease: A. optical microscopy; B. electron microscopy

FIGURE 4.

Membranous nephropathy: A. immunofluorescence (Ig G); B. electron microscopy

FIGURE 5.

Focal and segmental glomerulosclerosis: A. optical microscopy; B: electron microscopy

FIGURE 6.

Patients’ distribution according to their glomerular filtration rate (eGFR)