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心脏压塞作为2型自身免疫性多内分泌腺综合征的首发表现:一例病例报告

Cardiac tamponade as the initial presentation of autoimmune polyglandular syndrome Type 2: a case report.

作者信息

Glick Laura R, Hodson Daniel Z, Sharma Shreyak, Savarimuthu Stella

机构信息

Department of Internal Medicine, Yale School of Medicine, New Haven, CT, USA.

Section of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, Yale School of Medicine, New Haven, CT, USA.

出版信息

Eur Heart J Case Rep. 2022 Apr 8;6(5):ytac145. doi: 10.1093/ehjcr/ytac145. eCollection 2022 May.

Abstract

BACKGROUND

Cardiac tamponade is a rare but serious manifestation of autoimmune polyglandular syndrome Type 2 (APS 2). Patients often present with symptoms of thyroid dysfunction and adrenal insufficiency, but the insidious onset of the disease may lead to delayed diagnosis, which can progress rapidly to haemodynamic instability requiring urgent intervention.

CASE SUMMARY

A 39-year-old previously healthy male was admitted with cardiac tamponade complicated by cardiac arrest requiring emergent pericardiocentesis. An extensive work up revealed primary adrenal insufficiency and Hashimoto's thyroiditis. His positive autoantibodies to thyroid peroxidase and 21-hydroxylase combined with rapid improvement with initiation of corticosteroids and levothyroxine confirmed a diagnosis of APS 2.

DISCUSSION

Although this disease is often difficult to diagnose given its vague symptoms, it should be considered in the differential diagnosis for young patients presenting with pericardial effusion or cardiac tamponade of unknown origin. Early diagnosis and management are critical and often result in rapid improvement after appropriate treatment.

摘要

背景

心脏压塞是2型自身免疫性多腺体综合征(APS 2)的一种罕见但严重的表现形式。患者常出现甲状腺功能障碍和肾上腺功能不全的症状,但该病隐匿起病可能导致诊断延迟,进而迅速发展为血流动力学不稳定,需要紧急干预。

病例摘要

一名39岁既往健康男性因心脏压塞合并心脏骤停入院,需紧急进行心包穿刺术。全面检查发现原发性肾上腺功能不全和桥本甲状腺炎。他针对甲状腺过氧化物酶和21-羟化酶的自身抗体呈阳性,且在开始使用皮质类固醇和左甲状腺素后迅速好转,确诊为APS 2。

讨论

尽管鉴于其症状模糊,这种疾病通常难以诊断,但对于出现不明原因心包积液或心脏压塞的年轻患者,在鉴别诊断时应考虑该病。早期诊断和管理至关重要,适当治疗后通常会迅速好转。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d22/9071554/e01400146130/ytac145f1.jpg

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