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Pathogenesis of minimal change nephrotic syndrome: an immunological concept
- Source :
- Korean Journal of Pediatrics, Vol 59, Iss 5, Pp 205-211 (2016)
- Publication Year :
- 2016
- Publisher :
- Korean Pediatric Society, 2016.
-
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.
- Subjects :
- Minimal change nephrotic syndrome
Pathogenesis
T cell
B cell
CD80
Pediatrics
RJ1-570
Subjects
Details
- Language :
- English
- ISSN :
- 17381061 and 20927258
- Volume :
- 59
- Issue :
- 5
- Database :
- Directory of Open Access Journals
- Journal :
- Korean Journal of Pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.514946e712ed4be6b1c8851c43b5ea28
- Document Type :
- article
- Full Text :
- https://doi.org/10.3345/kjp.2016.59.5.205