野生型TTR淀粉样变性(ATTRwt)是一种年龄相关性淀粉样变性,这种类型的淀粉样变性中的TTR具有正常的氨基酸序列,并且这种疾病主要影响老年人,原因不明。 What is the frequency and prognosis of cardiac amyloidosis 04 虽然几种类型的淀粉样蛋白可能影响心脏,但临床上看到的大多...
美国一项调查研究结果显示,未校正的AL型心脏淀粉样变(cardiac amyloidosis,AL-CA)的患病率从2007年的15.5例/百万人显著增长至2015年的40.5例/百万人(约为1/25 000),年变化率(annual percentage change,APC)为12%(P<0.001),经过年龄、性别校正的AL-CA患病率从20.1例/百万人显著增长至50.1例/百万人(约为1/20 ...
leading to cardiac structural abnormality and functional decline. AL cardiac amyloidosis is the most common subtype of cardiac amyloidosis. The onset of cardiac amyloidosis is obscure with diverse clinical manifestations, resulting
Cardiac deposition of amyloid fibrils is common, and the severity of cardiac involvement remains the primary driver of prognosis. Improvements in chemotherapy/immunotherapy have prompted a reassessment of the role of advanced cardiac therapies previously considered contraindicated in most patients, including ...
Cardiac amyloidosis has a unique strain pattern, in which longitudinal strain in the basal left ventricular wall segments is significantly impaired, whereas apical strain is relatively preserved.7 Because of this relatively specific finding, longitudinal strain imaging may be a potential tool to assist ...
Systemic AL amyloidosis (ALA) is a clonal plasma cell (PC) disease characterized by deposition of amyloid fibrils in different organs and tissues. Traditionally, the prognosis of ALA is poor and is primarily defined by cardiac involvement. The modern prognostic models are based on cardiac markers ...
ATTR amyloidosis has better prognosis th... T Kotecha,A Martinez-Naharro,TA Treibel,... - 《Journal of the American College of Cardiology》 被引量: 3发表: 2018年 T1 Mapping in Cardiac Amyloidosis In summary, T1 mapping techniques such as that reported by Karamitsos et al. (10) have ...
Intracardiac thrombosis and embolism in patients with cardiac amyloidosis. Circulation. 2007;116:2420–2426. 9. Soma K, Takizawa M, Uozumi H, et al. A case of ST elevated myocardial infarction resulting from obstructive intramural coronary amyloidosis. Int Heart J. 2010;51:134–1...
"The report from Sperry et al in this issue of theJournalmarks the beginning of a change in that dire prognosis." SUGGESTED for you AL amyloidosis results from a plasma-cell dyscrasia that causes unchecked production of a monoclonal immunoglobulin light chain. This excess of light chains aggreg...
The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men... SW Dubrey,K Cha,J Anderson,... - QJM 被引...