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Journal of the Korean Society of Pediatric Nephrology 2013;17(1): 1-5. doi: https://doi.org/10.3339/jkspn.2013.17.1.1
C3 신염의 병리
김용진
영남대학교 의과대학 병리학교실
Pathology of C3 Glomerulonephritis
Yong-Jin Kim
Department of Pathology, Yeungnam University College of Medicine, Daegu, Korea
Corresponding Author: Yong-Jin Kim ,Tel: 053-620-3331, Fax: 053-656-1429, Email: yyjjkim@ynu.ac.kr
Received: March 15, 2013;  Accepted: March 25, 2013.
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons. org/licenses/bync/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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ABSTRACT
C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term ‘C3 glomerulopathy’ is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.
Key words: C3 glomerulonephritis | C3 glomerulonephropathy | alternative complement pathway | pathology
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