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[检测抗21-羟化酶自身抗体在原发性自身免疫性肾上腺皮质功能减退症诊断中的应用]

[Assessment of autoantibodies against 21-hydroxylase in the diagnosis of primary autoimmune adrenal insufficiency].

作者信息

Sozaeva Leila S, Makazan Nadezhda V, Nikankina Larisa V, Malysheva Natalya M, Kuvaldina Ekaterina V, Kareva Maria A, Orlova Elizaveta M, Peterkova Valentina A

机构信息

Endocrinology Research centre.

Endocrinology research centre.

出版信息

Probl Endokrinol (Mosk). 2020 Jun 10;65(6):466-473. doi: 10.14341/probl12106.

Abstract

Primary adrenal insufficiency is manifested by a deficiency of adrenal cortex hormones and can lead to a life-threatening condition. Early diagnosis is key to patient survival. Auto-antibodies to one of the adrenal steroidogenesis enzymes, 21-hydroxylase, are an immunological marker of autoimmune adrenal insufficiency. On the one hand, the study of antibodies to 21-hydroxylase is a method that helps establish the etiology of the disease – the autoimmune genesis of adrenal gland damage. On the other hand, the determination of autoantibodies to 21-hydroxylase is the only prognostic factor of the risk of adrenal insufficiency, which makes it possible to prevent the development of acute adrenal crisis. The article provides a brief literature review on autoantibodies to 21-hydroxylase and the pathogenesis of autoimmune adrenal insufficiency, and a series of clinical cases that illustrates the significant role of autoantibodies to 21-hydroxylase in diagnosis of adrenal insufficiency.

摘要

原发性肾上腺皮质功能减退症表现为肾上腺皮质激素缺乏,可导致危及生命的状况。早期诊断是患者存活的关键。针对肾上腺类固醇生成酶之一21-羟化酶的自身抗体是自身免疫性肾上腺皮质功能减退症的免疫标志物。一方面,对21-羟化酶抗体的研究是一种有助于确定疾病病因——肾上腺损伤的自身免疫起源的方法。另一方面,测定21-羟化酶自身抗体是肾上腺皮质功能减退风险的唯一预后因素,这使得预防急性肾上腺危象的发生成为可能。本文对21-羟化酶自身抗体及自身免疫性肾上腺皮质功能减退症的发病机制进行了简要文献综述,并通过一系列临床病例说明了21-羟化酶自身抗体在肾上腺皮质功能减退症诊断中的重要作用。

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