J Korean Soc Pediatr Nephrol.
2003 Apr;7(1):67-72.
Alport Syndrome Associated with Poststreptococcal Glomerulonephritis in Brothers
- Affiliations
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- 1Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea. guroped@korea.ac.kr
- 2Department of Pathology, College of Medicine, Korea University, Seoul, Korea.
- 3Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
Abstract
- Alport syndrome is the most common type of hereditary nephritis, and acute poststreptococcal glomerulonephritis(APSGN) is a common disease in children. We experienced the clinical and pathologic findings of Alport syndrome and APSGN in brothers of one family. Both patients presented with heavy gross hematuria and proteinuria. ASO titer was elevated in both cases, and the C3 level was reduced in one of the cases. In renal pathology, both showed characteristics of Alport syndrome as well as the glomerular changes of APSGN with hump-like subepithelial deposits by electron microscopy. These clinical observation indicated that the patients had APSGN superimposed on Alport syndrome, and that the episode of APSGN might exacerbate the clinical course of Alport syndrome.
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