본문 바로가기
HOME> 논문 > 논문 검색상세

논문 상세정보

Case reports in nephrology v.2013, 2013년, pp.1 - 4  

Acute Progression of Adult-Onset Atypical Hemolytic-Uremic Syndrome due to CFH Mutation: A Case Report

Posnik, Bartlomiej (Department of Nephrology, Transplantology and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland ) ; Sikorska, Dorota (Department of Nephrology, Transplantology and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland ) ; Hoppe, Krzysztof (Department of Nephrology, Transplantology and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland ) ; Schwermer, Krzysztof (Department of Nephrology, Transplantology and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland ) ; Pawlaczyk, Krzysztof (Department of Nephrology, Transplantology and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland ) ; Oko, Andrzej (Department of Nephrology, Transplantology and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland ) ;
  • 초록  

    Atypical hemolytic-uremic syndrome (aHUS), unlike typical HUS, is not due to bacteria but rather to an idiopathic or genetic cause that promotes dysregulation of the alternative complement pathway. It leads to hemolytic anemia, thrombocytopenia, and renal impairment. Although aHUS secondary to a genetic mutation is relatively rare, when occurring due to a mutation in Factor H (CFH), it usually presents with younger onset and has a more severe course, which in the majority ends with end-stage renal failure. Paradoxically to most available data, our case features acute aHUS due to a CFH mutation with late onset (38-year-old) and rapid progression to end-stage renal disease. Due to current data indicating a high risk of graft failure in such patients, the diagnosis of aHUS secondary to a genetic cause has disqualified our patient from a living (family) donor renal transplantation and left her with no other option but to begin permanent renal replacement therapy.


 활용도 분석

  • 상세보기

    amChart 영역
  • 원문보기

    amChart 영역

원문보기

무료다운로드
유료다운로드
  • 원문이 없습니다.

유료 다운로드의 경우 해당 사이트의 정책에 따라 신규 회원가입, 로그인, 유료 구매 등이 필요할 수 있습니다. 해당 사이트에서 발생하는 귀하의 모든 정보활동은 NDSL의 서비스 정책과 무관합니다.

원문복사신청을 하시면, 일부 해외 인쇄학술지의 경우 외국학술지지원센터(FRIC)에서
무료 원문복사 서비스를 제공합니다.

NDSL에서는 해당 원문을 복사서비스하고 있습니다. 위의 원문복사신청 또는 장바구니 담기를 통하여 원문복사서비스 이용이 가능합니다.

이 논문과 함께 출판된 논문 + 더보기