The Search for Biomarkers to Aid in Diagnosis, Differentiation, and Prognosis of Childhood Idiopathic Nephrotic Syndrome

Abstract

Identification of genes associated with childhood-onset nephrotic syndrome has significantly advanced our understanding of the pathogenesis of this complex disease over the past two decades, however the precise etiology in many cases remains unclear. At this time, we still rely on invasive kidney biopsy to determine the underlying cause of nephrotic syndrome in adults. In children, response to steroid therapy has been shown to be the best indicator of prognosis, and therefore all children are treated initially with corticosteroids. Because this strategy exposes a large number of children to the toxicities of steroids without providing any benefit, many researchers have sought to find a marker that could predict a patient's response to steroids at the time of diagnosis. Additionally, the identification of such a marker could provide prognostic information about a patient's response to medications, progression to end stage renal disease, and risk of disease recurrence following transplantation. Major advances have been made in understanding how genetic biomarkers can be used to predict a patient's response to therapies and disease course, especially after transplantation. Research attempting to identify urine- and serum-based biomarkers which could be used for the diagnosis, differentiation, and prognosis of nephrotic syndrome has become an area of emphasis. In this review, we explore the most exciting biomarkers and their potential clinical applications.

Effects of Huaiqihuang Granules adjuvant therapy in children with primary nephrotic syndrome

“…Serum IL-10 in the two groups of patients before treatment was significantly lower than that in the control group, suggesting that the activity of kidney disease is associated with immunoregulatory defects, and hormone can inhibit the secretion of IL-10. A recent study suggests that hormone can promote the synthesis of IL-10, while the latter participates in the immune function of CD4+, CD25+ regulatory T cells (Treg) [ 12 ]. In renal disease, the inhibition mechanism of Treg cells is damaged, IL -10 drops, causing the hyperactivity of T effector cells (Teff) and hypersecretion of inflammatory factors, which may participate in the pathogenesis of PNS.…”

Comprehensive Review of Steroid-Sensitive Nephrotic Syndrome Genetic Risk Loci and Transcriptional Regulation as a Possible Mechanistic Link to Disease Risk

“…Hillarey K. Stone, DO 1,6 ; Sreeja Parameswaran, PhD 2,6 ; Amy A. Eapen, MD 3,6 ; Xiaoting Chen, PhD 2,6 ; John B. Harley, PhD, MD 2,6,7 ; Prasad Devarajan, MD 1,6 ; Matthew T. Weirauch, PhD 2,4,5,6 ; Leah Kottyan, PhD 2,3,6 1. Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati,…”

The Unique Difference Between Serum Level of Soluble Urokinase Plasminogen Activator Receptor (suPAR) in Steroid-Resistant Nephrotic Syndrome Children Treated with an Alkylating Agent and Calcineurin Inhibitors

“…There are no studies on change in suPAR level with alkylating agent and CNIs in FSGS treatment. However, just recently, the usefulness of the soluble urokinase plasminogen activator receptor as a biomarker in clinically estimating and differentiating SRNS was corroborated (14,15,(20)(21)(22)(23)(24)(25)(26)(27). Some studies also reported that using suPAR level, it is possible to distinguish whether FSGS patients are responsive or resistant to therapy (23,28).…”