Results 51 to 60 of about 98,806 (295)

Inhibition of the mechano-enzymatic amyloidogenesis of transthyretin: role of ligand affinity, binding cooperativity and occupancy of the inner channel

Scientific Reports, 2017
Dissociation of the native transthyretin (TTR) tetramer is widely accepted as the critical step in TTR amyloid fibrillogenesis. It is modelled by exposure of the protein to non-physiological low pH in vitro and is inhibited by small molecule compounds ...
Guglielmo Verona, P. Patrizia Mangione, Sara Raimondi, Sofia Giorgetti, Giulia Faravelli, Riccardo Porcari, Alessandra Corazza, Julian D. Gillmore, Philip N. Hawkins, Mark B. Pepys, Graham W. Taylor, Vittorio Bellotti   +11 more
doaj   +1 more source

The Amyloidosis Forum: a public private partnership to advance drug development in AL amyloidosis

Orphanet Journal of Rare Diseases, 2020
Background Immunoglobulin light chain (AL) amyloidosis is a rare, multi-systemic disorder characterized by two disease processes: an underlying plasma cell dyscrasia that provides the source of pathologic light chains, and the resulting organ dysfunction
The Inaugural Amyloidosis Forum Panelists, Isabelle Lousada   +1 more
doaj   +1 more source

Transthyretin amyloid cardiomyopathy: Literature review and red‐flag symptom clusters for each cardiology specialty

ESC Heart Failure, Volume 12, Issue 2, Page 955-967, April 2025.
Abstract Wild‐type transthyretin amyloid cardiomyopathy (ATTRwt‐CM) is a progressive and infiltrative cardiac disorder that may cause fatal consequences if left untreated. The estimated survival time from diagnosis is approximately 3–6 years. Because of the non‐specificity of initial symptom manifestation and insufficient awareness among treating ...
Yasuhiro Izumiya, Toru Kubo, Jin Endo, Seiji Takashio, Masatoshi Minamisawa, Jun Hamada, Tomonori Ishii, Hajime Abe, Hiroaki Konishi, Kenichi Tsujita   +9 more
wiley   +1 more source

Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis

Haematologica
Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing Renal Response or Renal Progression following chemotherapy after 6 months.
Muhammad Umaid Rauf, Steven Law, Marisa Santostefano, Philip N. Hawkins, Aviva Petrie, Francesco Cappelli, Federico Perfetto, Yousuf Razvi, Aldostefano Porcari, Sriram Ravichandran, Adam Ioannou, Joshua Bomsztyk, Alessia Argirò, Costanza Gaudio, Elisabetta Antonioli, Alessandro Barilaro, Marco Delsante, Vittorio Di Maso, Maria G. Chiappini, Olabisi Ogunbiyi, Oliver C. Cohen, Ana Martinez-Naharro, Carol Whelan, Helen J. Lachmann, Ashutosh D. Wechalekar, Federico Alberici, Marianna Fontana, Marco Allinovi, Julian D. Gillmore   +28 more
doaj   +1 more source

Prognostic role of high‐sensitivity cardiac troponin T in patients with cardiac sarcoidosis: insights from ILLUMINATE‐CS

ESC Heart Failure, Volume 12, Issue 2, Page 869-878, April 2025.
Abstract Aims The prognostic role of high‐sensitivity cardiac troponin T (hs‐cTnT) as a biomarker in patients with cardiac sarcoidosis (CS) has yet to be fully determined, especially when compared with B‐type natriuretic peptide (BNP). Methods and Results In this post‐hoc analysis of the ILLUMINATE‐CS (ILLUstration of the Management and prognosIs of ...
Yuichi Baba, Toru Kubo, Takeru Nabeta, Yuya Matsue, Takeshi Kitai, Yoshihisa Naruse, Tatsunori Taniguchi, Hidekazu Tanaka, Takahiro Okumura, Kenji Yoshioka, Hiroaki Kitaoka   +10 more
wiley   +1 more source

Tafamidis Reduces Death and Hospitalization for Acute Heart Failure in Octogenarian Patients With Transthyretin Cardiac Amyloidosis: A Propensity Score–Weighted Cohort Study

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Background Transthyretin cardiac amyloidosis results from the deposition of misfolded amyloid fibrils in cardiac tissue, leading to progressive heart failure.
Louise Zmuda, Amira Zaroui, Mounira Kharoubi, Emmanuelle Boutin, Frédéric Roca, Silvia Oghina, Emmanuel Teiger, Marie Laurent, Florence Canoui‐Poitrine, Thibaud Damy, Amaury Broussier   +10 more
doaj   +1 more source

Prevalence of transthyretin cardiac amyloidosis in undifferentiated heart failure with preserved ejection fraction

ESC Heart Failure, Volume 12, Issue 2, Page 1176-1182, April 2025.
Abstract Aims Transthyretin amyloid cardiomyopathy (ATTR‐CM) is an increasinglyrecognized cause of heart failure with preserved ejection fraction (HFpEF), which may be diagnosed non‐invasively using 99mTc 3,3‐diphosphono‐1,2‐propanodicarboxylic acid (DPD) scintigraphy‐based diagnostic criteria.
L. Healy, G. Giblin, A. Gray, N. Starr, L. Murphy, D. O'Sullivan, E. Kavanagh, C. Howley, C. Tracey, E. Morrin, A. McDaid, A. Clarke, J.O. O'Neill, E. Joyce, M. O'Connell, N. G. Mahon   +15 more
wiley   +1 more source

Real‐world characteristics and treatment of cardiac transthyretin amyloidosis: A multicentre, observational study

ESC Heart Failure, Volume 12, Issue 2, Page 1203-1216, April 2025.
The 366 patients diagnosed with transthyretin amyloidosis cardiomyopathy (ATTR‐CM) were analyzed regarding their clinical characteristics in the first year after approval of tafamidis 61 mg for ATTR‐CM in Germany. Nearly two‐thirds of the patients were in an advanced disease stage and 64% met the key criteria of the “Transthyretin Amyloidosis ...
Richard J. Nies, Svenja Ney, Ingrid Kindermann, Yvonne Bewarder, Angela Zimmer, Fabian Knebel, Katrin Hahn, Sebastian Spethmann, Peter Luedike, Lars Michel, Tienush Rassaf, Maria Papathanasiou, Stefan Störk, Vladimir Cejka, Amin Polzin, Fabian Voss, Malte Kelm, Bernhard Unsöld, Christine Meindl, Michael Paulus, Ali Yilmaz, Bishwas Chamling, Caroline Morbach, Roman Pfister   +23 more
wiley   +1 more source

Epidemiology of hospitalized heart failure in France based on national data over 10 years, 2012–2022

ESC Heart Failure, Volume 12, Issue 2, Page 1283-1294, April 2025.
Abstract Aims We aim to describe the incidence of HF hospitalization in France in the post‐pandemic era, the prevalence of HF cases and patients' characteristics, management and outcomes while focusing on sex, age and socio‐economic differences and to analyse time‐trends between 2012 and 2022.
Valérie Olié, Richard Isnard, Françoise Pousset, Clémence Grave, Jacques Blacher, Amélie Gabet   +5 more
wiley   +1 more source

Reliability and validity of the Transthyretin Amyloidosis – Quality of Life (ATTR-QOL) Questionnaire impact scales

Journal of Patient-Reported Outcomes
Background The diversity of disease phenotypes associated with transthyretin (ATTR) amyloidosis poses challenges for measurement of health outcomes. The Transthyretin Amyloidosis – Quality of Life (ATTR-QOL) Questionnaire is a disease-specific patient ...
Andrew Lovley, Kristen Hsu, Kaitlin LaGasse, Isabelle Lousada, Kristen L. McCausland, Michelle K. Carty, Sabrina Rebello, Jakob B. Bjorner   +7 more
doaj   +1 more source