[非典型溶血性尿毒症综合征的诊断线索:1例报告]。

IF 1 4区 医学 Q4 PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH Anales Del Sistema Sanitario De Navarra Pub Date : 2022-08-17 DOI:10.23938/ASSN.1006
M Luquin Irigoyen, C Armendariz Brugos, M Vallejo Ruiz
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引用次数: 0

摘要

非典型溶血性尿毒症综合征(aHUS)是一种以非免疫性溶血性贫血、血小板减少症和肾功能衰竭为特征的临床疾病,其病变是由系统性血栓性微血管病变介导的。这是一种罕见的病理,其起源是补体系统失调,由于其基因突变,导致C5的不受控制的激活和膜攻击复合物的形成。-正确的诊断使我们能够开出基于Eculizumab(一种C5抑制剂)的治疗处方。我们报告的情况下,怀孕患者aHUS的目的是强调早期鉴别诊断的重要性,以建立早期和有效的治疗这种病理。更新了aHUS的病理生理学、诊断和遗传学研究,以及治疗管理。
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[Diagnostic clues in atypical hemolytic uremic syndrome: a case report].

Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to mutations in its genes that lead to uncontrolled activation of C5 and the formation of the membrane attack complex.- Its correct diagnosis allows us to prescribe the treatment based on Eculizumab, a C5 inhibitor. We report the case of a pregnant patient with aHUS, with the aim of highlighting the importance of early differential diagnosis to establish an early and effective treatment of this pathology. The pathophysiology, diagnosis and genetic study are updated, as well as the therapeutic management of aHUS.

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来源期刊
Anales Del Sistema Sanitario De Navarra
Anales Del Sistema Sanitario De Navarra 医学-公共卫生、环境卫生与职业卫生
CiteScore
1.30
自引率
30.00%
发文量
88
审稿时长
>12 weeks
期刊介绍: La revista Anales del Sistema Sanitario de Navarra es una revista de contenido médico sanitario de carácter generalista. En ella tienen cabida artículos referidos a temas de salud/enfermedad en general, salud pública, administración y gestión sanitaria y Atención Primaria de salud.
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