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Pathogenesis of minimal change nephrotic syndrome: an immunological concept

Authors
Kim, SH | Park, SJ  | Han, KH | Kronbichler, A | Saleem, MA | Oh, J | Lim, BJ | Shin, JI
Citation
Korean journal of pediatrics, 59(5). : 205-211, 2016
Journal Title
Korean journal of pediatrics
ISSN
1738-10612092-7258
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.
Keywords

DOI
10.3345/kjp.2016.59.5.205
PMID
27279884
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Pediatrics & Adolescent Medicine
Ajou Authors
박, 세진
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