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 language | English |
|---|---|
| Pages (from-to) | 205-211 |
| Number of pages | 7 |
| Journal | Korean Journal of Pediatrics |
| Volume | 59 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 2016 May |
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All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Pediatrics
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}
Pathogenesis of minimal change nephrotic syndrome : An immunological concept. / Kim, Seong Heon; Park, Se Jin; Han, Kyoung Hee; Kronbichler, Andreas; Saleem, Moin A.; Oh, Jun; Lim, Beom Jin; Shin, Jae Il.
In: Korean Journal of Pediatrics, Vol. 59, No. 5, 05.2016, p. 205-211.Research output: Contribution to journal › Review article
TY - JOUR
T1 - Pathogenesis of minimal change nephrotic syndrome
T2 - An immunological concept
AU - Kim, Seong Heon
AU - Park, Se Jin
AU - Han, Kyoung Hee
AU - Kronbichler, Andreas
AU - Saleem, Moin A.
AU - Oh, Jun
AU - Lim, Beom Jin
AU - Shin, Jae Il
PY - 2016/5
Y1 - 2016/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84968655425&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84968655425&partnerID=8YFLogxK
U2 - 10.3345/kjp.2016.59.5.205
DO - 10.3345/kjp.2016.59.5.205
M3 - Review article
AN - SCOPUS:84968655425
VL - 59
SP - 205
EP - 211
JO - Korean Journal of Pediatrics
JF - Korean Journal of Pediatrics
SN - 1783-1061
IS - 5
ER -