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心脏淀粉样变——诊断挑战与临床情况不明。

Cardiac Amyloidosis-Challenging Diagnosis and Unclear Clinical Picture.

机构信息

Student Scientific Society at the Department of Internal, Autoimmune and Metabolic Diseases, School of Medicine, Medical University of Silesia, 40-752 Katowice, Poland.

Department of Internal, Autoimmune and Metabolic Diseases, School of Medicine, Medical University of Silesia, 40-752 Katowice, Poland.

出版信息

Medicina (Kaunas). 2021 May 6;57(5):450. doi: 10.3390/medicina57050450.

Abstract

Cardiac amyloidosis (CA) is a rare systemic disease determined by the extracellular deposition of amyloid protein in the heart. The protein can accumulate in any part of the heart: myocardium, vessels, endocardium, valves, epicardium and parietal pericardium. The types of CA include the following types: light chain (AL), amyloidosis AA (Amyloid A) and transthyretin (ATTR). The detection of specific subtypes remains of great importance to implement the targeted treatment. We present the case of a 65-year-old woman, who was admitted with severe deterioration of exercise capacity, a bilateral reduction of physiological vesicular murmur, ascites and edema of lower extremities. CA was suspected due to echocardiographic examination results, which led to further examination and final diagnosis. The aim of this study is to improve the disease awareness among clinicians and shorten the delay between the first symptoms and the diagnosis establishment resulting in a better outcome.

摘要

心脏淀粉样变性(CA)是一种罕见的全身性疾病,由心脏细胞外沉积淀粉样蛋白引起。该蛋白可在心脏的任何部位积聚:心肌、血管、心内膜、瓣膜、心外膜和壁层心包。CA 包括以下类型:轻链(AL)、淀粉样变性 AA(Amyloid A)和转甲状腺素(ATTR)。检测特定亚型对实施靶向治疗仍然非常重要。我们介绍了一位 65 岁女性的病例,她因运动能力严重恶化、双肺生理性肺泡呼吸音减弱、腹水和下肢水肿而入院。由于超声心动图检查结果,怀疑为 CA,进一步检查后最终确诊。本研究旨在提高临床医生对该病的认识,缩短从首发症状到诊断确立的时间延迟,从而获得更好的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a59/8148212/df119e3fe762/medicina-57-00450-g001.jpg

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