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Journal of the Korean Society of Pediatric Nephrology 2003;7(1): 67-72.
알포트 증후군 형제에서 동시에 발병된 급성 연쇄상 구균 감염 후 사구체 신염 2례
신혜경, 김지희, 홍영숙, 이주원, 김순겸, 원남희, 정해일, 유기환
1고려대학교 의과대학 소아과학교실
2고려대학교 의과대학 소아과학교실
3고려대학교 의과대학 소아과학교실
4고려대학교 의과대학 소아과학교실
5고려대학교 의과대학 소아과학교실
6고려대학교 의과대학 병리학교실
7서울대학교 의과대학 소아과학교실
8고려대학교 의과대학 소아과학교실
Alport Syndrome Associated with Poststreptococcal Glomerulonephritis in Brothers
Hye-Kyoung Shin, Ji-Hee Kim, Young-Sook Hong, Joo-Won Lee, Soon-Kyum Kim, Nam-Hee Won, Hae-Il Cheong, Kee-Hwan Yoo
1Department of Pediatrics, College of Medicine, Korea University
2Department of Pediatrics, College of Medicine, Korea University
3Department of Pediatrics, College of Medicine, Korea University
4Department of Pediatrics, College of Medicine, Korea University
5Department of Pediatrics, College of Medicine, Korea University
6Department of Pathology, College of Medicine, Korea University
7Department of Pediatrics, Seoul National University College of Medicine
8Department of Pediatrics, College of Medicine, Korea University
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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.
Key words: Alport syndrome | Acute poststreptococcal glomerulonephritis
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