대한신장학회

Skip Navigation: Skip to contents

KOR
ENG
전체메뉴보기

간행물 검색

현재 페이지 경로

HOME
간행물
간행물 검색

논문분류	춘계학술대회 초록집
제목	Complementopathy in Atypical HUS/C3 Glomerulopathy
저자	Myung-Gyu Kim
출판정보	2022; 2022(1):
키워드
초록	Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The pathology of aHUS is thrombotic microangiopathy (TMA), in which there is extensive thrombosis in arterioles and capillaries; In addition, C3 glomerulopathy (C3G) is a rare set of glomerulonephritis with 2 patterns: C3 glomerulonephritis (C3GN) and dense deposit disease. The dysregulation of the alternative pathway of the complement activation system, either from acquired or genetic cause, is the underlying mechanism of both diseases. Recent studies of the genetic background of them have revealed significant genetic heterogeneity in several genes involved in complement regulation, including complement factor H, CFH-relate protein, complement factor I, complement factor B, complement component 3 and CD46 genes in the alternative complement pathway. Although clinical studies have provided a better understanding of disease pathogenesis, genetic factors are still largely unknown, and it is difficult to identify genetic predispositions in various clinical conditions. In addition, given the prognosis of the diseases remains poor, further research is needed to improve diagnosis and treatment protocols based on genetic predisposition and to develop new therapeutic agents.
원문(PDF)	PDF 원문보기

목록

위로가기