Results 91 to 100 of about 44,633 (211)

Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry

Swiss Medical Weekly
AIMS OF THE STUDY: Systemic amyloidoses are rare protein-folding diseases with heterogeneous, often nonspecific clinical presentations. To better understand systemic amyloidoses and to apply state-of-the-art diagnostic pathways and treatment, the ...
Sofie Brouwers, Raphael Heimgartner, Natallia Laptseva, Adriano Aguzzi, Niklas F. Ehl, Thomas Fehr, Felicitas Hitz, Hans H. Jung, Joel Kälin, Markus G. Manz, Beat Müllhaupt, Frank Ruschitzka, Harald Seeger, Georg Stussi, Markus Zweier, Andreas J. Flammer, Bernhard Gerber, Rahel Schwotzer   +17 more
doaj   +1 more source

Technetium-99m pyrophosphate cardiac SPECT in endomyocardial biopsy negative cardiac amyloidosis

Radiology Case Reports, 2018
Cardiac amyloidosis is an under-appreciated cause of heart failure. Establishing a diagnosis is important because traditional heart failure treatment regimens can worsen left ventricular failure in this disease. Endomyocardial biopsy is the gold standard
Martin Krupa, MD, Rina Nguyen, BS, Jonathan Revels, DO, Lester S. Johnson, MD, PhD, FACR   +3 more
doaj   +1 more source

State-of-the-art radionuclide imaging in cardiac transthyretin amyloidosis

, 2019
Cardiac amyloidosis, once considered untreatable, is now gaining well-deserved attention due to advances in imaging and the recent approval of targeted breakthrough therapies.
Singh, Vasvi, Kijewski, Marie, Di Carli, Marcelo F., Dorbala, Sharmila, Rapezzi, Claudio, Falk, Rodney   +5 more
core   +1 more source

Peptide probes detect misfolded transthyretin oligomers in plasma of hereditary amyloidosis patients

, 2017
Peptide probes detect misfolded transthyretin oligomers in amyloidosis patient plasma.
Sergio Teruya, John M. Kelly, Joseph D. Schonhoft, Christopher G. Parker, Evan T. Powers, Benjamin F. Cravatt, Lars Plate, Daniel Boland, Yvonne S. Eisele, Mathew Maurer, Teresa Coelho, Jeffery W. Kelly, Marta Novais, Stephen Helmke, Yoshiki Sekijima, Cecilia Monteiro, John Berk   +16 more
core   +1 more source

Cryo-EM structure of an ATTRwt amyloid fibril from systemic non-hereditary transthyretin amyloidosis

, 2022
Wild type transthyretin-derived amyloid (ATTRwt) is the major component of non-hereditary transthyretin amyloidosis. Its accumulation in the heart of elderly patients is life threatening.
Schönland, Stefan, Steinebrei, Maximilian, Hegenbart, Ute, Schmidt, Matthias, Baur, Julian, Röcken, Christoph, Gottwald, Juliane   +6 more
core   +1 more source

Treatment of transthyretin cardiac amyloidosis

Current Opinion in Cardiology
Purpose of review Tafamidis is currently the only approved disease-modifying treatment for ATTR-CM. However, there have been important developments in the treatment of ATTR-CM, as the results of two phase 3 trials were published and several other trials are in their final stages.
Bampatsias, Dimitrios, Wardhere, Abdirahman, Maurer, Mathew S.   +2 more
openaire   +2 more sources

Clinical outcomes for 2788 patients with transthyretin amyloidosis: Tafamidis meglumine early access program in France

International audienceEarly access experience in France with tafamidis meglumine, a selective transthyretin stabilizer for transthyretin-related amyloidosis cardiomyopathy (ATTR-CM), following transthyretin-related amyloidosis (ATTR) polyneuropathy ...
Legallois, Damien, Lequeux, Benoit, Fievez, Stephane, Quinault, Aurore, Hippocrate, Aurelie, Eicher, Jean-Christophe, Josse, Constant, Damy, Thibaud, Réant, Patricia, Rudant, Jeremie, Bauer, Fabrice, Noirot Cosson, Charlotte, Lairez, Olivier, Dubois, Margaux, Habib, Gilbert, Squara, Pierre-Alexandre, Inamo, Jocelyn, Jeanneteau, Julien, Bartoli, Mathilde, Kharoubi, Mounira   +19 more
core   +1 more source

Cryo-EM structure of a transthyretin-derived amyloid fibril from a patient with hereditary ATTR amyloidosis

, 2019
ATTR amyloidosis is one of the worldwide most abundant forms of systemic amyloidosis. The disease is caused by the misfolding of transthyretin protein and the formation of amyloid deposits at different sites within the body.
Wiese, Sebastian, Engler, Jonas, Zacharias, Martin, Schmidt, Matthias, Fändrich, Marcus, Agarwal, Shubhangi, Fritz, Günter, Westermark, Per, Adak, Volkan, Wiese, Sebastian,, Fändrich, Marcus,, Fritz, Günter,, Schmidt, Matthias,, Agarwal, Shubhangi,, Engler, Jonas,, Westermark, Per,, Adak, Volkan,, Zacharias, Martin,   +17 more
core   +1 more source

Emerging Therapeutics for the Treatment of Light Chain and Transthyretin Amyloidosis

JACC: Basic to Translational Science, 2019
Summary: Cardiac amyloidosis is a restrictive cardiomyopathy that results from the deposition of misfolded light chain or transthyretin proteins, most commonly, in cardiac tissue.
Kathleen W. Zhang, MD, Keith E. Stockerl-Goldstein, MD, Daniel J. Lenihan, MD   +2 more
doaj   +1 more source

Erythropoietin in Familial Amyloidosis ATTR V30M

, 2013
Familial amyloidosis ATTR V30M is an hereditary disorder, the most frequent type of transthyretin related amyloidosis. The main manifestation of the disease is a sensory-motor and autonomic polyneuropathy.
Costa, P.P., Beirão, I.
core   +1 more source