揭示一例大量心包积液和心包填塞的心脏淀粉样变的诊断

IF 0.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Research in Cardiovascular Medicine Pub Date : 2021-04-01 DOI:10.4103/rcm.rcm_3_21
Pardha Sivakoti, G. Harsha, Praveen Nagula, Srinivas Ravi
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引用次数: 0

摘要

淀粉样变性是一种临床疾病,由细胞外沉积的不溶性原纤维与β -褶片状结构引起。蛋白质错误折叠异常导致淀粉样蛋白原纤维,可表现为原发性、继发性、家族性或老年性淀粉样变性。淀粉样蛋白沉积可发生在多个器官(如心、肝、肾、皮肤、眼睛、肺和神经系统),导致多种临床表现。心脏受累是一种进行性疾病,由于充血性心力衰竭和心律失常导致早期死亡。我们报告一个罕见的心脏淀粉样变病例,表现为复发性心包积液,并具有限制性心肌病的特征。
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Unveiling the diagnosis of cardiac amyloidosis in a patient with large pericardial effusion and tamponade
Amyloidosis is a clinical disorder caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, familial, or senile amyloidosis. Amyloid deposition can occur in multiple organs (e.g. heart, liver, kidney, skin, eyes, lungs, and nervous system), resulting in a variety of clinical manifestations. Cardiac involvement is a progressive disorder resulting in early death due to congestive heart failure and arrhythmias. We report a rare case of cardiac amyloidosis presenting as recurrent pericardial effusion with features of restrictive cardiomyopathy.
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来源期刊
Research in Cardiovascular Medicine
Research in Cardiovascular Medicine CARDIAC & CARDIOVASCULAR SYSTEMS-
自引率
0.00%
发文量
13
审稿时长
17 weeks
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