Korean J Pediatr.
2016 May;59(5):205-211. 10.3345/kjp.2016.59.5.205.
Pathogenesis of minimal change nephrotic syndrome: an immunological concept
- Affiliations
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- 1Department of Pediatrics, Pusan National University Children's Hospital, Yangsan, Korea.
- 2Department of Pediatrics, Daewoo General Hospital, Ajou University School of Medicine, Geoje, Korea.
- 3Department of Pediatrics, Jeju National University School of Medicine, Jeju, Korea.
- 4Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Innsbruck, Austria.
- 5Children's and Academic Renal Unit, Dorothy Hodgkin Building-University of Bristol, Bristol, UK.
- 6Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
- 7Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
- 8Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea. shinji@yuhs.ac
- KMID: 2164983
- DOI: http://doi.org/10.3345/kjp.2016.59.5.205
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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