Gülhan Bora, Özaltın Fatih
Division of Pediatric Nephrology, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey.
Nephrogenetics Laboratory, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey;Division of Pediatric Nephrology, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey.
Turk Arch Pediatr. 2021 Sep;56(5):415-422. doi: 10.5152/TurkArchPediatr.2021.21128.
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA), mainly affecting the kidney. The disease is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. It is one of the most common causes of acute kidney injury in children. Under this umbrella, there are several different disorders: Shiga toxin-associated HUS, Streptococcus pneumoniae-associated HUS, and HUS associated with complement dysregulation. Several drugs and conditions may also cause HUS. There are many different classification systems, which have been developed during the history of the disease. In recent years, clinical and experimental studies have revealed abnormalities in different pathways beyond complement system. Besides, therapeutic options that are based on the pathophysiology have been available for HUS patients.
溶血性尿毒症综合征(HUS)是一种血栓性微血管病(TMA),主要影响肾脏。该疾病的特征为微血管病性溶血性贫血、血小板减少和急性肾损伤。它是儿童急性肾损伤最常见的病因之一。在这一范畴内,有几种不同的病症:志贺毒素相关的HUS、肺炎链球菌相关的HUS以及与补体调节异常相关的HUS。几种药物和病症也可能导致HUS。在该疾病的发展历程中已形成了许多不同的分类系统。近年来,临床和实验研究揭示了补体系统之外不同途径的异常。此外,基于病理生理学的治疗方案已可供HUS患者使用。