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溶血性尿毒症综合征

Hemolytic Uremic Syndrome.

作者信息

Cody Ellen M, Dixon Bradley P

机构信息

Department of Pediatrics, University of Colorado School of Medicine, 13123 East 16th Avenue, Box 158, Aurora, CO 80045, USA.

Departments of Pediatrics & Medicine, University of Colorado School of Medicine, 12631 E. 17th Avenue, Aurora, CO 80045, USA.

出版信息

Pediatr Clin North Am. 2019 Feb;66(1):235-246. doi: 10.1016/j.pcl.2018.09.011.

Abstract

Hemolytic uremic syndrome (HUS) is the clinical triad of thrombocytopenia, anemia, and acute kidney injury. Classically associated with enterocolitis from Shiga toxin-producing Escherichia coli, HUS is also associated with Streptococcus pneumoniae infections; genetic dysregulation of the alternative complement pathway or coagulation cascade; and, rarely, a hereditary disorder of cobalamin C metabolism. These share a common final pathway of a prothrombotic and proinflammatory state on the endothelial cell surface, with fibrin and platelet deposition. Much work has been done to distinguish between the different mechanisms of disease, thereby informing the optimal therapeutic interventions for each entity.

摘要

溶血性尿毒症综合征(HUS)是血小板减少、贫血和急性肾损伤的临床三联征。传统上,HUS与产志贺毒素大肠杆菌引起的小肠结肠炎相关,也与肺炎链球菌感染、替代补体途径或凝血级联反应的基因失调有关;此外,还与罕见的钴胺素C代谢遗传性疾病有关。这些情况在内皮细胞表面具有促血栓形成和促炎状态的共同最终途径,伴有纤维蛋白和血小板沉积。为区分不同的疾病机制已开展了大量工作,从而为每种疾病实体提供最佳治疗干预措施。

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