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Pathogenesis of childhood idiopathic nephrotic syndrome: a paradigm shift from T-cells to podocytes
Kazunari Kaneko, Shoji Tsuji, Takahisa Kimata, Tetsuya Kitao, Sohsaku Yamanouchi, Shogo Kato
Osaka, Japan
Author Affiliations: Department of Pediatrics, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010, Japan (Kaneko K, Tsuji S, Kimata T, Kitao T, Yamanouchi S, Kato S)
Corresponding Author: Kazunari Kaneko, MD, Professor and Chair, Department of Pediatrics, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010, Japan (Tel: +81-72-804-0101 ext. 2560; Fax: +81-72-804-2758; Email: kanekok@hirakata.kmu.ac.jp)
doi: 10.1007/s12519-015-0003-9
Background: Nephrotic syndrome is the most common cause of kidney disease in children, but its pathogenesis remains unclear. This article reviews the novel aspects of the mechanisms underlying massive proteinuria in minimal-change disease, which is the most common form of childhood nephrotic syndrome.
Data sources: This article integrates the findings of a PubMed database search for English language articles published in the past 40 years (from September 1974 to February 2014) using the key words "pathogenesis", "minimal change nephrotic syndrome" or "idiopathic nephrotic syndrome".
Results: Unknown humoral factors associated with T-cell dysfunction have been thought to play an important role in the pathogenesis of minimal-change disease. However, recent findings are changing this paradigm, i.e., visceral glomerular epithelial cells (podocytes) may be involved via expression of molecules such as CD80 and angiopoietin-like 4.
Conclusions: Recent evidence suggests that minimal-change disease results from interactions between humoral factors and dysfunctional podocytes. In addition to immunosuppressant drugs that target lymphocytes, a biological agent such as an antibody against the abnormal molecule(s) expressed by podocytes may provide novel drug treatment for minimal-change disease.
World J Pediatr 2015;11(1):21-28
Key words: angiopoietin-like 4;
CD80;
cytokine;
minimal change nephrotic syndrome;
podocyte
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