Results 31 to 40 of about 148,376 (307)

Guidelines and new directions in the therapy and monitoring of ATTRv amyloidosis

Amyloid: Journal of Protein Folding Disorders, 2022
The recent approval of three drugs for the treatment of amyloid transthyretin (ATTR) amyloidosis, both hereditary and wild-type, has opened a new era in the care of these diseases. ATTR amyloidosis is embedded in its pathophysiology, and the drugs target
Y. Ando, D. Adams, M. Benson, J. Berk, V. Planté-Bordeneuve, T. Coelho, I. Conceição, B. Ericzon, L. Obici, Claudio Rapezz, Y. Sekijima, M. Ueda, G. Palladini, G. Merlini   +13 more
semanticscholar   +1 more source

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Blood Cancer Journal, 2021
The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival
Andrew Staron, Luke Zheng, Gheorghe Doros, Lawreen H. Connors, Lisa M. Mendelson, Tracy Joshi, Vaishali Sanchorawala   +6 more
doaj   +1 more source

Transthyretin cardiac amyloidosis

Cardiovascular Research, 2022
Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) and mortality worldwide. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have shifted ATTR-CA from a rare and ...
A. Porcari, M. Fontana, J. Gillmore
semanticscholar   +1 more source

Proteomic Analysis for the Diagnosis of Fibrinogen Aα-chain Amyloidosis

Kidney International Reports, 2019
Introduction: Hereditary fibrinogen Aα-chain (AFib) amyloidosis is a relatively uncommon renal disease associated with a small number of pathogenic fibrinogen Aα (FibA) variants; wild-type FibA normally does not result in amyloid deposition.
Graham W. Taylor, Janet A. Gilbertson, Rabya Sayed, Angel Blanco, Nigel B. Rendell, Dorota Rowczenio, Tamer Rezk, P. Patrizia Mangione, Diana Canetti, Paul Bass, Philip N. Hawkins, Julian D. Gillmore   +11 more
doaj   +1 more source

Electromechanical dissociation of left atrium in patients with Cardiac Amyloidosis by Magnetic Resonance: Prognostic and clinical correlates

International Journal of Cardiology: Heart & Vasculature, 2020
Objectives: Left atrial (LA) function is an important marker of hemodynamic status in cardiac amyloidosis (CA), and its characterization may provide relevant prognostic information.
Giovanni Donato Aquaro, Sofia Morini, Chrysanthos Grigoratos, Giulia Taborchi, Gianluca Di Bella, Raffaele Martone, Elisa Vignini, Michele Emdin, Iacopo Olivotto, Federico Perfetto, Francesco Cappelli   +10 more
doaj   +1 more source

Comparative Outcomes of a Transthyretin Amyloid Cardiomyopathy Cohort Versus Patients With Heart Failure With Preserved Ejection Fraction Enrolled in the TOPCAT Trial

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2023
Background Transthyretin cardiac amyloidosis (ATTR‐CM), found in 6% to 15% of cohorts with heart failure with preserved ejection fraction, has long been considered a rare disease with poor prognosis.
Morris M. Kim, Mark Prasad, Yunwoo Burton, Clinton M. Kolseth, Yuanzi Zhao, Pranav Chandrashekar, Babak Nazer, Ahmad Masri   +7 more
doaj   +1 more source

High-precision plasma β-amyloid 42/40 predicts current and future brain amyloidosis

Neurology, 2019
Objective We examined whether plasma β-amyloid (Aβ)42/Aβ40, as measured by a high-precision assay, accurately diagnosed brain amyloidosis using amyloid PET or CSF p-tau181/Aβ42 as reference standards.
S. Schindler, James G. Bollinger, Vitaliy Ovod, K. Mawuenyega, Yan Li, B. Gordon, D. Holtzman, J. Morris, T. Benzinger, C. Xiong, A. Fagan, R. Bateman   +11 more
semanticscholar   +1 more source

Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis.

Circulation: Heart Failure, 2019
Cardiomyopathy is a manifestation of transthyretin amyloidosis (ATTR), which is an underrecognized systemic disease whereby the transthyretin protein misfolds to form fibrils that deposit in various tissues and organs.
M. Maurer, S. Bokhari, T. Damy, S. Dorbala, B. Drachman, M. Fontana, M. Grogan, A. Kristen, I. Lousada, J. Nativi-Nicolau, Candida Cristina Quarta, C. Rapezzi, F. Ruberg, R. Witteles, G. Merlini   +14 more
semanticscholar   +1 more source

Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee

Amyloid: Journal of Protein Folding Disorders, 2020
The ISA Nomenclature Committee met electronically before and directly after the XVII ISA International Symposium on Amyloidosis, which, unfortunately, had to be virtual in September 2020 due to the ongoing COVID-19 pandemic instead of a planned meeting ...
M. Benson, J. Buxbaum, D. Eisenberg, G. Merlini, M. Saraiva, Y. Sekijima, J. Sipe, P. Westermark   +7 more
semanticscholar   +1 more source

Global epidemiology of amyloid light-chain amyloidosis

Orphanet Journal of Rare Diseases, 2022
Background Amyloid light-chain (AL) amyloidosis is an ultra-rare disease associated with significant morbidity and mortality. Few studies have examined the global epidemiology of this condition.
Nishant Kumar, Nicole Zhang, D. Cherepanov, D. Romanus, M. Hughes, D. Faller   +5 more
semanticscholar   +1 more source