Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Assessment of myocardial uptake of Tc-99m-pyrophosphate (Tc-99m PYP) is pivotal in distinguishing transthyretin-associated cardiac amyloidosis (ATTR) from light chain amyloid (AL).
|
31172386 |
2020 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Cardiac manifestations and prognostic implications of hereditary transthyretin amyloidosis associated with transthyretin Ala97Ser.
|
31521469 |
2020 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Here, the role of APOE was investigated with regard to the efficacy of Patisiran, the first LNP-siRNA recently approved for clinical use in patients having transthyretin amyloidosis (ATTR amyloidosis).
|
31648569 |
2020 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
First-in-Human Study of AG10, a Novel, Oral, Specific, Selective, and Potent Transthyretin Stabilizer for the Treatment of Transthyretin Amyloidosis: A Phase 1 Safety, Tolerability, Pharmacokinetic, and Pharmacodynamic Study in Healthy Adult Volunteers.
|
31172685 |
2020 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Out of 343 patients (age 60 ± 13 years), 251 (73%) carried a likely/pathogenic gene variant, including 12 (3.5%) in the CA-associated genes TTR (n = 11) and ApoAI (n = 1).
|
31371117 |
2020 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Cardiac amyloidosis or amyloid cardiomyopathy (ACM), commonly resulting from extracellular deposition of amyloid fibrils consisted of misfolded immunoglobulin light chain (AL) or transthyretin (TTR) protein, is an underestimated cause of heart failure and cardiac arrhythmias.
|
31375251 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
The aims of this study were to characterize RV involvement in patients with CA and to identify parameters that may help in the differential diagnosis between ALCA and transthyretin-derived CA subtypes.
|
31078369 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Inotersen, a 2'-O-methyoxyethyl-modified antisense oligonucleotide, which acts by reducing the production of transthyretin, was recently demonstrated to improve disease course and quality of life in early hereditary transthyretin amyloidosis polyneuropathy in a 15-month Phase III study.
|
31118583 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Transthyretin amyloidosis (ATTR amyloidosis) is a rare disease characterised by extracellular deposition of amyloid fibrils composed by transthyretin.
|
30683924 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Transthyretin (TTR) is a major amyloidogenic protein associated with hereditary (ATTRm) and nonhereditary (ATTRwt) intractable systemic transthyretin amyloidosis.
|
31167790 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Transthyretin amyloidosis (ATTR amyloidosis) is a fatal systemic disease caused by amyloid deposits of misfolded transthyretin, leading to familial amyloid polyneuropathy and/or cardiomyopathy, or a rare oculoleptomeningeal amyloidosis.
|
31001136 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
A series of noninvasive technique enables the diagnosis of hereditary transthyretin-related cardiac amyloidosis.
|
31348283 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
The homotetrameric plasma protein transthyretin (TTR), is responsible for a series of debilitating and often fatal disorders in humans known as transthyretin amyloidosis.
|
31835306 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Transthyretin amyloidosis (ATTR) is a rare, progressive, life-threatening, hereditary disorder caused by mutations in the transthyretin gene.
|
31517333 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Transthyretin amyloidosis with polyneuropathy (ATTR-PN), a rare and progressive hereditary disorder, results from mutations in the gene coding for the transthyretin (TTR) protein that destabilize the protein's tetrameric structure.
|
31098895 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review.
|
31171094 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Hereditary transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized progressive cardiomyopathy with heterogenous clinical manifestations that lead to its misdiagnosis and poor prognosis.
|
31718691 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Stabilization of cardiac function with diflunisal in transthyretin (ATTR) cardiac amyloidosis.
|
31805416 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
We investigated differences in survival among patients with ATTR cardiac amyloidosis by nutritional status as defined by modified BMI (mBMI) and by inflammatory state as defined by serum uric acid.<b>Methods and results:</b> This study was a retrospective analysis of patients diagnosed with ATTR cardiac amyloidosis at a single tertiary medical centre.
|
31825676 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The rate of diagnosis with hATTR-CM among TTR V122I carriers with heart failure was measured.
|
31821430 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
In this study, we measured plasma neurofilament light chain (pNfL) concentration in 73 patients with ATTR and found that pNfL was significantly raised in ATTRm patients with peripheral neuropathy compared to healthy controls.
|
31583784 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
Out of > 1000 patients with cardiac amyloidosis (AL or ATTR) admitted to our centre between September 1998 and January 2016, a cohort of 120 patients with a complete cardiac assessment at diagnosis, including right heart catheterization, echocardiography and biomarkers, was analysed retrospectively in this study.
|
31630214 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
[I-123]MIBG is mainly useful in patients with hereditary and wild-type ATTR cardiac amyloidosis, not in AA and AL amyloidosis.
|
28887775 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Amyloid Cardiomyopathy in the Rare Transthyretin Tyr78Phe Mutation.
|
30604309 |
2019 |
Senile cardiac amyloidosis
|
0.400 |
Biomarker
|
disease |
BEFREE |
AG10 is a selective, oral TTR stabilizer under development for transthyretin amyloidosis cardiomyopathy (ATTR-CM) that mimics a protective TTR mutation.
|
30885685 |
2019 |