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[心脏孤立性原发性淀粉样变性终生诊断病例]

[Case of lifetime diagnosis of isolated primary amyloidosis of the heart].

作者信息

Demko I V, Pelinovskaya L I, Kraposhina A Yu, Selin V E, Ryazanov A E, Verigo Ya I, Mosina V A

机构信息

Krasnoyarsk state medical university by prof. Voino-Yasenetsky V.F., Krasnoyarsk.

Regional clinical hospital, Krasnoyarsk.

出版信息

Kardiologiia. 2022 Jan 31;62(1):106-108. doi: 10.18087/cardio.2022.1.n1049.

Abstract

The article presents a case of intravital diagnosis of a rare heart disease, isolated primary amyloidosis. The clinical onset of the disease was heart failure (HF) that was resistant to treatment; chemotherapy was ineffective and was poorly tolerated by the patient. The diagnostics was based on a combination of refractory HF and changes in echocardiography data (atrial dilatation, small size of the left ventricular cavity, interventricular septal hypertrophy with the presence of hyperechoic inclusions of the "granular fluorescence" type), and changes detected by contrast-enhanced cardiac magnetic resonance imaging. The diagnosis of amyloidosis was confirmed by results of pathohistological examination of the material obtained during autopsy.

摘要

本文介绍了一例罕见心脏病——孤立性原发性淀粉样变性的活体诊断病例。该疾病的临床起病表现为抗治疗的心力衰竭(HF);化疗无效且患者耐受性差。诊断基于难治性HF、超声心动图数据变化(心房扩张、左心室腔小、室间隔肥厚并伴有“颗粒状荧光”型高回声内含物)以及对比增强心脏磁共振成像检测到的变化。淀粉样变性的诊断通过尸检获取材料的病理组织学检查结果得以证实。

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