Advanced Search
REVIEW ARTICLE
Korean J Pediatr 2016 May;59(5) :205-211.
Published online 2016 May 15.       
Pathogenesis of minimal change nephrotic syndrome: an immunological concept
Seong Heon Kim1,*, Se Jin Park2,*, Kyoung Hee Han3, Andreas Kronbichler4, Moin A. Saleem5, Jun Oh6, Beom Jin Lim7, Jae Il Shin8
1Department of Pediatrics, Pusan National University Childrens 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
5Childrens 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 Childrens Hospital, Yonsei University College of Medicine, Seoul, Korea
Corresponding Author: Jae Il Shin ,Tel: +82-2-2228-2050, Fax: +82-2-393-9118, Email: shinji@yuhs.ac
* These authors contributed equally to this study and should be considered co-first authors.
Copyright © 2016 by The Korean Pediatric Society
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: Minimal change nephrotic syndrome | Pathogenesis | T cell | B cell | CD80
TOOLS
PDF Links  PDF Links
Full text via DOI  Full text via DOI
Download Citation  Download Citation
Supplementary Material  Supplementary Material
  E-Mail
  Print
Share:      
METRICS
1,480
View
183
Download
Genetics of hereditary nephrotic syndrome: a clinical review  2017 March;60(3)
Cystic Fibrosis of Pancreas and Nephrotic Syndrome: a rare association  2013 October;56(10)
A case of steroid-induced psychosis in a child having nephrotic syndrome with toxic epidermal necrolysis  2010 March;53(3)
Cyclosporin A Treatment of Minimal Change Nephrotic Syndrome and Focal Segmental Glomerulosclerosis  1993 December;36(12)
Register for e-submission
Register here to access the e-submission system of Korean J Pediatr for authors and reviewers.
Manuscript Submission
To submit a manuscript, please visit the Korean J Pediatr e-submission management system at http://submit.kjp.or.kr, read the Instructions for Authors, and log into the Korean J Pediatr e-submission system. For assistance with manuscript submission, please contact: kjpped@gmail.com.
Free archive
Anyone may access any past or current articles without logging in.
Korean Pediatric Society Office
#1606, Seocho World Officetel, 19 Seoun-ro, Seocho-gu, Seoul 137-070, Korea
TEL : +82-2-3473-7305    FAX : +82-2-3473-7307   E-mail: kjpped@gmail.com
BrowseCurrent IssueFor Authors and ReviewersAbout
Copyright© The Korean Pediatric Society. All right reserved.