作者: C. John Sperati , Alison R. Moliterno
DOI: 10.1016/J.HOC.2015.02.002
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摘要: Thrombotic microangiopathies (TMA) such as atypical hemolytic uremic syndrome (aHUS) have evolved from rare, fulminant childhood afflictions to uncommon diseases with acute and chronic phases involving both children adults. Breakthroughs in complement coagulation regulation allowed redefinition of specific entities despite substantial phenotypic mimicry. Reconciliation phenotypes delivery life saving therapies require a multidisciplinary team experts. The purpose this review is describe advances the molecular pathophysiology aHUS share 2014 experience Johns Hopkins TMA Registry applying diagnostic assays, reporting disease associations, genetic testing.