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Pathogenesis of minimal change nephrotic syndrome: an immunological concept

Authors
 Seong Heon Kim  ;  Se Jin Park  ;  Kyoung Hee Han  ;  Andreas Kronbichler  ;  Moin A. Saleem  ;  Jun Oh  ;  Beom Jin Lim  ;  Jae Il Shin 
Citation
 Korean Journal of Pediatrics, Vol.59(5) : 205-211, 2016 
Journal Title
 Korean Journal of Pediatrics 
ISSN
 1738-1061 
Issue Date
2016
Keywords
B cell ; CD80 ; Minimal change nephrotic syndrome ; Pathogenesis ; T cell
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.
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DOI
10.3345/kjp.2016.59.5.205
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
신재일(Shin, Jae Il) ORCID logo https://orcid.org/0000-0003-2326-1820
임범진(Lim, Beom Jin) ORCID logo https://orcid.org/0000-0003-2856-0133
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/152076
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