Abstract

BackgroundSteroid-resistant nephrotic syndrome (SRNS) is a burden in the country due to the progressive severity of chronic kidney disease (CKD). Calcineurin inhibitors (CNIs) or monoclonal antibodies are currently recommended for the treatment of this disease. In developing countries, steroid and cyclophosphamide (CPA) are available drugs used during the treatment. This study aims to provide a non-invasive modality that can be used to predict the response of SRNS children to CPA therapy. Subsequently, the proteinuria duration was shortened to reduce the risk of glomerular damage. The present study aims to determine whether there is a correlation between baseline serum TGFB and proteinuria in SRNS children six months after receiving CPA treatment. The author hypothesized that there would be a negative correlation between those variables.MethodA prospective-cohort-study was conducted at Hasan Sadikin General Hospital Bandung, Indonesia. A total of 88 SRNS children, aged 1 to 18 were accessed for serum TGF-β level before receiving CPA therapy for six months, and clinical signs were observed. Furthermore, after six months of CPA treatment, the subjects were divided into CPA responder and non-responder based on the presence of proteinuria, then the data were analyzed using multiple logistic regression to adjust age and gender.ResultsThere was a statistically significant relationship between TGF-β and the risk of non-response to CPA therapy, after accounting for age, gender, baseline GFR, baseline ureum, and baseline urinary protein, the adjusted-OR was 1.051 (95% CI 1.007, 1.097, p = 0.022).ConclusionThe high level of serum TGF-β obtained prior to CPA administration are reliable data for estimating adverse results on CPA therapy. Based on these results, a high baseline TGF-β level correlates with the poor response of CPA therapy.

Highlights

  • Nephrotic syndrome is the most common glomerular disease discovered in children, and this entity is divided into two categories based on its steroid response, including steroidresistant nephrotic syndrome (SRNS) and steroidsensitive nephrotic syndrome (SSNS)

  • A high baseline transforming growth factor-beta (TGF-b) level correlates with the poor response of CPA therapy

  • Serum was collected from 88 Steroid-resistant nephrotic syndrome (SRNS) children, 31 with focal-segmental glomerulosclerosis (FSGS), 8 with MPGN, 1 with Mes-GN, and 13 with minimal change disease (MCD), and the TGF-b serum was measured by using Enzyme-linked immunosorbent assay (ELISA) method

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Summary

Introduction

Nephrotic syndrome is the most common glomerular disease discovered in children, and this entity is divided into two categories based on its steroid response, including steroidresistant nephrotic syndrome (SRNS) and steroidsensitive nephrotic syndrome (SSNS). SRNS is associated to an increased risk of end-stage renal disease (ESRD) development. Steroid-resistant nephrotic syndrome (SRNS) is a burden in the country due to the progressive severity of chronic kidney disease (CKD). Steroid and cyclophosphamide (CPA) are available drugs used during the treatment. This study aims to provide a non-invasive modality that can be used to predict the response of SRNS children to CPA therapy. The present study aims to determine whether there is a correlation between baseline serum TGFB and proteinuria in SRNS children six months after receiving CPA treatment. The author hypothesized that there would be a negative correlation between those variables

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