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儿童溶血尿毒综合征。

Hemolytic-Uremic Syndrome in Children.

机构信息

Pediatric Nephrology, Necker Enfants Malades Hospital, Université Paris Cité, France; Néphrologie Pédiatrique, Hôpital Necker, 149 Rue de Sèvres, Paris 75015, France.

Pediatric Nephrology, Necker Enfants Malades Hospital, Université Paris Cité, France.

出版信息

Pediatr Clin North Am. 2022 Dec;69(6):1181-1197. doi: 10.1016/j.pcl.2022.07.006. Epub 2022 Oct 29.

Abstract

Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Most cases are caused by Shiga-toxin-producing bacteria, especially Escherichia coli. Transmission occurs through ground beef and unpasteurized milk. STEC-HUS is the main cause of acute renal failure in children. Management remains supportive. Immediate outcome is most often. Atypical HUS represents about 5% of cases, has a relapsing course with more than half of the patients progressing to end-stage kidney failure. Most cases are due to variants in complement regulators of the alternative pathway. Complement inhibitors, such as eculizumab, have considerably improved the prognosis.

摘要

溶血尿毒综合征的特征为微血管性溶血性贫血、血小板减少和急性肾衰竭三联征。大多数病例由产志贺毒素的细菌引起,尤其是大肠杆菌。传播途径为食用未煮熟的碎牛肉和未经巴氏消毒的牛奶。产志贺毒素大肠埃希菌溶血尿毒综合征是儿童急性肾衰竭的主要病因。治疗仍以支持治疗为主。大多数患者的即时预后良好。非典型溶血尿毒综合征约占病例的 5%,呈反复发作病程,超过一半的患者进展至终末期肾衰竭。大多数病例是由于补体旁路调节因子的变异引起的。补体抑制剂,如依库珠单抗,显著改善了预后。

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