Amyloidosis is a multisystemic disease, caused by deposits of misfolded protein fragments. There are two main types of amyloidosis, the light-chain (AL) and transthyretin-related amyloidosis, both of them, affect the heart. We present a case of a 59-year-old male who came to the emergency ...
Introduction and objectives Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases. Methods We conducted a single-centre, retrospective review of ...
Cardiac involvement in systemic amyloidosis (AL) occurs in ~50% of all AL patients. However once symptomatic heart failure develops, therapeutic options are limited thereby conferring a poor overall prognosis. The median survival is <6 months when AL patients are untreated for the underlying plasma...
Light chain toxicity is an important consideration because it influences treatment and patients will present sooner with significant illness. One study in mice demonstrated the effects of infusing light chain proteins from human patients diagnosed with cardiacamyloidosisand found the infusion resulted in ...
Objective To evaluate the relationship between left ventricular myocardial strain and prognosis in patients with light chain cardiac amyloidosis (AL-CA) by three-dimensional speckle tracking imaging (3D-STI). Methods A retrospective analysis was performed on the clinical data of 120 patients with AL-...
This cohort study assesses whether native T1 mapping can measure the cardiac light-chain amyloidosis treatment response and the association between
6) Cardiac amyloidosis 心肌淀粉样变性 1. objective Cardiac amyloidosis(CAL) is a rare disease and we are not familiar with its clinical features. 目的 心肌淀粉样变性 ( cardiac amyloidosis CAL)是临床上相对少见的疾病 ,对其临床特点认识不足。
Light-chain cardiac amyloidosis Cardiac amyloidosis is an underrecognized condition, in which delays to diagnosis have great implications on management options, prognosis, and morbidity. ... AK Mankad,I Sesay,KB Shah - 《Current Problems in Cancer》 被引量: 3发表: 2017年 加载更多来源...
Light-chain cardiac amyloidosis (AL-CA) is a fatal cardiomyopathy caused by the deposition of monoclonal immunoglobulin light-chain in the cardiac fibers, conduction system, valves, and other parts of the heart. Both Mayo 2004 and 2012 staging systems are widely used to assess the prognosis of ...
Amyloid fibrils derived from antibody light chains are key pathogenic agents in systemic AL amyloidosis. They can be deposited in multiple organs but cardiac amyloid is the major risk factor of mortality. Here we report the structure of a λ1 AL amyloid fibril from an explanted human heart at ...