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[溶血性尿毒症综合征(HUS)的治疗进展]

[Advances in the treatment of hemolytic uremic syndrome (HUS)].

作者信息

Ito K, Shiraga H

机构信息

Department of Pediatric Nephrology, Tokyo Women's Medical College.

出版信息

Nihon Rinsho. 1997 Mar;55(3):715-20.

PMID:9086786
Abstract

The hemolytic uremic syndrome (HUS) is the end result of a variety of etiologic agents that can induce endothelial cell injury and thrombotic microangiopathy (TMA) mostly within the kidney. The typical, post-diarrheal verocytotoxin associated HUS (D + HUS) is the major cause of acute renal failure in children worldwide. In the course of HUS treatment, fluid overload is usually the result of overhydration in the context of oliguria or anuria which cause edema, hypertension, worsening of neurologic signs and cardiac failure. Appropriate and timely use of dialysis has dramatically reduced complications of renal failure and extra-renal complications are now the main causes of mortality and morbidity in D + HUS. The reasons for treatment by infusion of fresh frozen plasma and/or plasmapheresis for D + HUS are theoretical and their therapeutic effects are inconclusive. We believe that plasma administration for regular D + HUS has no value and is potentially harmful. Until new strategies become available in clinical practice, the general consensus for the moment is that careful supportive management with patience is still the most appropriate form of D + HUS therapy.

摘要

溶血尿毒综合征(HUS)是多种病因导致的最终结果,这些病因大多可在肾脏内诱发内皮细胞损伤和血栓性微血管病(TMA)。典型的、腹泻后与产志贺毒素相关的HUS(D + HUS)是全球儿童急性肾衰竭的主要原因。在HUS治疗过程中,液体超负荷通常是少尿或无尿情况下补液过度的结果,可导致水肿、高血压、神经症状恶化和心力衰竭。适当及时地使用透析已显著减少了肾衰竭的并发症,肾外并发症现已成为D + HUS患者死亡和发病的主要原因。对D + HUS输注新鲜冷冻血浆和/或进行血浆置换治疗的原因是基于理论,其治疗效果尚无定论。我们认为,对常规D + HUS进行血浆输注没有价值且可能有害。在临床实践中出现新的治疗策略之前,目前的普遍共识是,耐心进行仔细的支持性治疗仍然是D + HUS最恰当的治疗方式。

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