Pathological aspects of membranoproliferative glomerulonephritis (MPGN) and haemolytic uraemic syndrome (HUS) thrombocytic thrombopenic purpura (TTP)
Pathological aspects of membranoproliferative glomerulonephritis (MPGN) and haemolytic uraemic syndrome (HUS) thrombocytic thrombopenic purpura (TTP)
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DOI：
10.1160/th07-12-0761
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发表时间：
2009-02-01
影响因子：
6.7
通讯作者：
Amann, Kerstin
中科院分区：
文献类型：
--
作者：
Benz, Kerstin;Amann, Kerstin
本文介绍了膜增生性肾小球肾炎（MPGN）、溶血性尿毒综合征（HUS）和血栓性血小板减少性紫癜三种类型的流行病学、发病机制和典型形态学特征。(TTP)将在光学显微镜、免疫组织学或免疫荧光和电子显微镜水平上进行审查。特别是，这些疾病的发病机制的差异进行了讨论。最近对三种类型的MPGN、典型和非典型HUS以及TTP的发病机制的重要分子和遗传学见解，即补体系统调节异常、C3和H因子（补体激活替代途径的主要调节蛋白）的不同分子缺陷，以及血管性血友病因子（VWF）切割蛋白酶（即ADAMTS 13）的缺乏，得到了强调。最后，将特别强调肾小球和血管形态的差异，在三种类型的MPGN和血栓性微血管病（TMA），这是特征性的形态学改变的肾脏在HUS和TTP，分别。
In this paper, epidemiology, pathogenesis and typical morphological aspects of all three types of membranoproliferative glomerulonephritis (MPGN), of the haemolytic uraemic syndrome (HUS) as well as of thrombotic thrombopenic purpura. (TTP) will be reviewed on the light microscopical, immunohistological or immunofluorescence and electron microscopical level. In particular, differences in the pathogenesis of these diseases are discussed. Important recent molecular and genetic insights into the pathogenesis of the three types of MPGN, of typical and atypical HUS and of TTP, i.e. dysregulation of the complement system, distinct molecular defects in C3 and factor H, the major regulatory protein of the alternative pathway of complement activation, and deficiency of a von Willebrand factor (VWF)-cleaving protease, i.e. ADAMTS 13, are highlighted. Finally, particular emphasis will be put on differences in glomerular and vascular morphology in the three types of MPGN and in thrombotic microangiopathy (TMA),which is the characteristic morphological alteration of the kidney in HUS and TTP, respectively.