系统性淀粉样变性累及心脏1例病例报告及文献复习

发布时间：2018-04-26 20:59

本文选题：淀粉样变性 + 心脏　；参考：《兰州大学》2017年硕士论文

【摘要】：目的:探讨系统性淀粉性变性累及心脏的临床特点、诊治思路及疾病预后。方法:分析1例系统性淀粉样变性累及心脏的患者的病例资料,进行病例报告和相关文献学习。病例:患者因反复心衰加重入院,伴肝肾功能异常,多次发生晕厥。完善心脏彩超提示双房增大,室间隔运动幅度减低,室间隔、左室后壁略增厚,左室舒张功能明显减低,收缩功能略减低。心电图提示低电压、心律失常。干预及结果:间断利尿治疗心衰,联合护肾、纠正贫血等治疗,胸闷、气短好转出院。结论:系统性淀粉样变性是一组可累及多个系统的异质性疾病,累及心脏时可呈现下列特征:超声心动图的特征:左室舒张功能减退、心室壁增厚(可对称或不对称,对称均质性增厚更有诊断价值)及伴毛玻璃样变化,心电图的特征:标准肢体导联低电压及胸前导联R波递增不良,限制性心肌病症状体征,体位性低血压,晕厥,各种心律失常。系统性粉样淀粉样变可累及任何组织器官,临床表现复杂多样,无特异性,易被误诊,早期诊断困难。明确心脏发生淀粉样变的金标准是心内膜心肌活检。系统性淀粉样变性整体预后欠佳,然累及心脏且出现心衰表现后,预后更差,中位生存期常常小于6个月。总的治疗原则是基础及对症治疗,前者包括化疗及移植。
[Abstract]:Objective: to investigate the clinical features, diagnosis, treatment and prognosis of systemic amyloidosis involving the heart. Methods: a case of systemic amyloidosis involving the heart was analyzed. Case: the patient was admitted to hospital because of repeated heart failure, accompanied by abnormal liver and kidney function, and syncope occurred many times. The results of perfect echocardiography showed that the amplitude of interventricular septal motion decreased, the ventricular septum, left ventricular posterior wall thickened, left ventricular diastolic function decreased, and systolic function decreased slightly. Electrocardiogram indicates low voltage, arrhythmia. Intervention and results: intermittent diuretic treatment of heart failure, combined treatment of kidney care, anemia correction, chest tightness, shortness of breath improved and discharged. Conclusion: systemic amyloidosis is a group of heterogeneous diseases involving multiple systems. The following features can be observed when involving the heart: left ventricular diastolic dysfunction, ventricular wall thickening (symmetric or asymmetrical), echocardiography, left ventricular diastolic dysfunction, and ventricular wall thickening. Symmetrical homogeneity thickening is more valuable for diagnosis) and accompanied by glass-like changes, electrocardiogram characteristics: standard limb lead low voltage and chest lead R wave increase poor, restrictive cardiomyopathy symptoms and signs, postural hypotension, syncope, Various arrhythmias. Systemic amyloidosis can involve any tissues and organs. Its clinical manifestations are complex, non-specific, easy to be misdiagnosed and difficult to diagnose early. The gold standard for determining the incidence of amyloidosis in the heart is endomyocardial biopsy. The overall prognosis of systemic amyloidosis was poor, but the prognosis was even worse after heart involvement and heart failure, and the median survival time was often less than 6 months. The general principle of treatment is basic and symptomatic treatment, the former includes chemotherapy and transplantation.
【学位授予单位】：兰州大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R597.2

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