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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