Treatment of AL cardiac amyloidosis with intravenous high-dose melphalan and stem cell transplantationangiotensin IInitric oxide synthase uncouplingnox expressioncGMP-dependent protein kinasevasodilator-stimulated phosphoproteinLipid-lowering agents have been shown to reduce morbidity and mortality associated with ...
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 ...
摘要: Treatment with the small interfering RNA patisiran preserves functional capacity and improves health status and quality of life in patients with transthyretin cardiac amyloidosis, according to data from the 12-month double-blind period of the ongoing APOLLO-B trial....
The different types of amyloidosis include: AL amyloidosis (immunoglobulin light chain amyloidosis).This is the most common type and used to be called primary amyloidosis. AL stands for "amyloid light chains," which is the type of protein responsible for the condition. There’s no known cause,...
Systemic amyloidoses are rare and proteiform diseases, caused by extracellular accumulation of insoluble misfolded fibrillar proteins. Prognosis is dictated by cardiac involvement, which is especially frequent in light chain (AL) and in transthyretin variants (ATTR, both mutated, (ATTRm), and wild-...
Cardiac amyloidosis is a restrictive cardiomyopathy that results from the deposition of misfolded light chain or transthyretin proteins, most commonly, in ... KW Zhang,KE Stockerl-Goldstein,DJ Lenihan - 《Jacc Basic to Translational Science》 被引量: 0发表: 2019年 ...
If patients develop severe complications from amyloidosis, those conditions will need to be treated. For example, dialysis may be necessary if kidney failure develops, and cardiac medications may improve heart function and reduce retained fluid if cardiac disease becomes a problem. ...
The arrhythmic manifestations of cardiac amyloidosis can range from conduction-system disease and bradyarrhythmias to atrial fibrillation and sudden cardiac death. Bradyarrhythmias and conduction system disease may occur secondarily to amyloid infiltration, but the timing of pacemaker implantation remains ...
Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment. Cardiac involvement is associated with symptoms of heart failure, and dictates the clinical course of the disease. Cardiac amyloidosis can be diagnosed non... OK Siddiqi,FL Ruberg - 《Trends in Cardiovascular Medicine》 被引...
"The heart is the most important abnormality in amyloidosis because if the heart is affected, that is the complete driver of how the patient will do and what their prognosis is," says Dr. Grogan. "That's why cardiac amyloidosis is so important to recognize and treat as early as possible....