本文選題：淀粉樣變性 + 心臟　； 參考：《蘭州大學(xué)》2017年碩士論文

【摘要】：目的:探討系統(tǒng)性淀粉性變性累及心臟的臨床特點(diǎn)、診治思路及疾病預(yù)后。方法:分析1例系統(tǒng)性淀粉樣變性累及心臟的患者的病例資料,進(jìn)行病例報(bào)告和相關(guān)文獻(xiàn)學(xué)習(xí)。病例:患者因反復(fù)心衰加重入院,伴肝腎功能異常,多次發(fā)生暈厥。完善心臟彩超提示雙房增大,室間隔運(yùn)動(dòng)幅度減低,室間隔、左室后壁略增厚,左室舒張功能明顯減低,收縮功能略減低。心電圖提示低電壓、心律失常。干預(yù)及結(jié)果:間斷利尿治療心衰,聯(lián)合護(hù)腎、糾正貧血等治療,胸悶、氣短好轉(zhuǎn)出院。結(jié)論:系統(tǒng)性淀粉樣變性是一組可累及多個(gè)系統(tǒng)的異質(zhì)性疾病,累及心臟時(shí)可呈現(xiàn)下列特征:超聲心動(dòng)圖的特征:左室舒張功能減退、心室壁增厚(可對(duì)稱或不對(duì)稱,對(duì)稱均質(zhì)性增厚更有診斷價(jià)值)及伴毛玻璃樣變化,心電圖的特征:標(biāo)準(zhǔn)肢體導(dǎo)聯(lián)低電壓及胸前導(dǎo)聯(lián)R波遞增不良,限制性心肌病癥狀體征,體位性低血壓,暈厥,各種心律失常。系統(tǒng)性粉樣淀粉樣變可累及任何組織器官,臨床表現(xiàn)復(fù)雜多樣,無(wú)特異性,易被誤診,早期診斷困難。明確心臟發(fā)生淀粉樣變的金標(biāo)準(zhǔn)是心內(nèi)膜心肌活檢。系統(tǒng)性淀粉樣變性整體預(yù)后欠佳,然累及心臟且出現(xiàn)心衰表現(xiàn)后,預(yù)后更差,中位生存期常常小于6個(gè)月�？偟闹委熢瓌t是基礎(chǔ)及對(duì)癥治療,前者包括化療及移植。
[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.
【學(xué)位授予單位】：蘭州大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R597.2

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本文編號(hào)：1807631