Pathogenesis of minimal change nephrotic syndrome: An immunological concept

Seong Heon Kim, Se Jin Park, Kyoung Hee Han, Andreas Kronbichler, Moin A. Saleem, Jun Oh, Beom Jin Lim, Jae Il Shin

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11 Citations (Scopus)

Abstract

Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested. Recently, a “two-hit” theory was proposed that included the induction of CD80 (B7-1) and regulatory T-cell (Treg) dysfunction, with or without impaired autoregulatory functions of the podocyte. In contrast to the well-established involvement of T cells, the role of B cells has not been clearly identified. However, B-cell biology has recently gained more attention, because rituximab (a monoclonal antibody directed against CD20-bearing cells) demonstrated a very good therapeutic response in the treatment of childhood and adult MCNS. Here, we discuss recent insights into the pathogenesis of MCNS in children.

Original languageEnglish
Pages (from-to)205-211
Number of pages7
JournalKorean Journal of Pediatrics
Volume59
Issue number5
DOIs
Publication statusPublished - 2016 May

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All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Pediatrics

Cite this

Kim, S. H., Park, S. J., Han, K. H., Kronbichler, A., Saleem, M. A., Oh, J., Lim, B. J., & Shin, J. I. (2016). Pathogenesis of minimal change nephrotic syndrome: An immunological concept. Korean Journal of Pediatrics, 59(5), 205-211. https://doi.org/10.3345/kjp.2016.59.5.205