Canpolat Nur
Department of Pediatrics, Division of Pediatric Nephrology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Turk Pediatri Ars. 2015 Jun 1;50(2):73-82. doi: 10.5152/tpa.2015.2297. eCollection 2015 Jun.
Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by the triad of thrombotic microangiopathy, thrombocytopenia, and acute kidney injury. Hemolytic uremic syndrome represents a heterogeneous group of disorders with variable etiologies that result in differences in presentation, management and outcome. In recent years, better understanding of the HUS, especially those due to genetic mutations in the alternative complement pathway have provided an update on the terminology, classification, and treatment of the disease. This review will provide the updated classification of the disease and the current diagnostic and therapeutic approaches on the complement-mediated HUS in addition to STEC-HUS which is the most common cause of the HUS in childhood.
溶血尿毒综合征(HUS)是一种以血栓性微血管病、血小板减少和急性肾损伤三联征为特征的临床综合征。溶血尿毒综合征代表了一组病因各异的异质性疾病,这些病因导致了临床表现、治疗和预后的差异。近年来,对HUS的深入了解,尤其是对因替代补体途径基因突变所致HUS的了解,为该疾病的术语、分类和治疗带来了新进展。本综述将提供该疾病的最新分类,以及补体介导的HUS和儿童期HUS最常见病因——产志贺毒素大肠杆菌相关溶血尿毒综合征(STEC-HUS)的当前诊断和治疗方法。