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LANCET:溶血性尿毒症综合征

2017-03-01 MedSci MedSci原创

溶血性尿毒症综合征是一种主要影响肾脏的血栓性微血管疾病的,其特征为血小板减少,机械性溶血性贫血以及急性肾损伤的三联征。该术语包括几种病症:志贺毒素诱导以及肺炎球菌诱导的溶血性尿毒症综合征;与补体调节异常或二酰基甘油激酶激酶突变相关的溶血性尿毒症综合征;与钴胺素C缺陷有关的溶血性尿毒症综合征;以及异质性继发的溶血性尿毒性综合征的原因(感染、药物、癌症和全身性疾病)。在过去二十年,相关实验、遗传和临床

溶血性尿毒症综合征是一种主要影响肾脏的血栓性微血管疾病的,其特征为血小板减少,机械性溶血性贫血以及急性肾损伤的三联征。

该术语包括几种病症:志贺毒素诱导以及肺炎球菌诱导的溶血性尿毒症综合征;与补体调节异常或二酰基甘油激酶激酶突变相关的溶血性尿毒症综合征;与钴胺素C缺陷有关的溶血性尿毒症综合征;以及异质性继发的溶血性尿毒性综合征的原因(感染、药物、癌症和全身性疾病)。

在过去二十年,相关实验、遗传和临床研究已经帮助理解这些形式各异的溶血性尿毒症综合征的病理生理学,并且在诊断方法上取得了巨大的改善。

此外,对于由于补体调节异常引起的非典型溶血性尿毒症综合征的患者,已经提供了针对发病机制的治疗。

但是,对于其他几种疾病类型,包括志贺毒素诱导的溶血性尿毒症综合征,相关治疗仍然缺乏。

原始出处:
Fakhouri F, Zuber J, et al. Haemolytic uraemic syndrome. Lancet. 2017 Feb 23. pii: S0140-6736(17)30062-4. doi: 10.1016/S0140-6736(17)30062-4. 

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    2017-05-13 howi
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    2017-03-02 1ddf0692m34(暂无匿称)

    深度好文

    0

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