Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Case of Familial IgA Nephropathy

가족성 IgA 신병증 1례

  • Kim, Yu-Jin (Department of Pediatrics, College of Medicine, Chungbuk National University) ;
  • Kim, Wun-Kon (Department of Pediatrics, College of Medicine, Chungbuk National University) ;
  • Yoon, Shin-Ae (Department of Pediatrics, College of Medicine, Chungbuk National University) ;
  • Lee, Jin-Seok (Department of Pediatrics, College of Medicine, Chungbuk National University) ;
  • Ha, Tae-Sun (Department of Pediatrics, College of Medicine, Chungbuk National University)
  • 김유진 (충북대학교 의과대학 소아과학교실) ;
  • 김운곤 (충북대학교 의과대학 소아과학교실) ;
  • 윤신애 (충북대학교 의과대학 소아과학교실) ;
  • 이진석 (충북대학교 의과대학 소아과학교실) ;
  • 하태선 (충북대학교 의과대학 소아과학교실)
  • Received : 2011.03.21
  • Accepted : 2011.04.21
  • Published : 2011.04.30
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Abstract

IgA nephropathy is the most common form of primary glomerulonephritis and chronic glomerular disease worldwide including Korea. Familial gathering of IgA nephropathy suggests that genetic factors contribute to the development of this disease. Although there have been many reports on familial IgA nephropathy with genetic analysis and their pedigrees, there has been few reports in Korea. We reported a partial familial IgA nephropathy pedigree with a brief review of the literatures.

IgA 신병증은 전 세계적으로 가장 흔한 원발성 사구체 질환으로 성인의 경우 30% 정도가 만성신부전으로 진행하며 소아의 경우 10% 정도가 만성신부전으로 진행한다. IgA 신병증은 전신증상이 없이 현미경적 혈뇨와 간헐적인 육안적 혈뇨를 특징으로 하며 심한 경우 단백뇨를 동반한다. IgA 신병증의 원인이나 발병기전은 정확히 알려져 있지 않으며. 국외에서는 IgA 신병증이 유전되는 가계에 대한 보고와 함께 관련 유전자 연구가 활발하지만 국내에서는 이에 대한 증례가 매우 드물다. 저자들은 국내에서 가족 내유전된 IgA 신병증에 대한 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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