Results 21 to 30 of about 148,376 (307)

Plasmin activity promotes amyloid deposition in a transgenic model of human transthyretin amyloidosis

Nature Communications, 2021
ATTR amyloidosis causes heart failure through the accumulation of misfolded transthyretin in cardiac muscle. Here the authors report a mouse model of ATTR amyloidosis and demonstrate the involvement of protease activity in ATTR amyloid deposition.
Ivana Slamova, Rozita Adib, Stephan Ellmerich, Michal R. Golos, Janet A. Gilbertson, Nicola Botcher, Diana Canetti, Graham W. Taylor, Nigel Rendell, Glenys A. Tennent, Guglielmo Verona, Riccardo Porcari, P. Patrizia Mangione, Julian D. Gillmore, Mark B. Pepys, Vittorio Bellotti, Philip N. Hawkins, Raya Al-Shawi, J. Paul Simons   +18 more
doaj   +1 more source

A 15-year consolidated overview of data in over 6000 patients from the Transthyretin Amyloidosis Outcomes Survey (THAOS)

Orphanet Journal of Rare Diseases, 2023
Background Transthyretin amyloidosis (ATTR amyloidosis) is a progressive, multisystemic, life-threatening disease resulting from the deposition of variant or wild-type (ATTRwt amyloidosis) transthyretin amyloid fibrils in various tissues and organs ...
L. Gentile, Teresa Coelho, A. Dispenzieri, I. Conceição, M. Waddington-Cruz, A. Kristen, J. Wixner, Igor Diemberger, J. González-Moreno, E. Cariou, M. Maurer, V. Planté-Bordeneuve, P. García-Pavía, I. Tournev, J. González-Costello, Alejandra Gonzalez Duarte, M. Grogan, Anna Mazzeo, Doug Chapman, Pritam Gupta, Oliver Glass, L. Amass   +21 more
semanticscholar   +1 more source

A prospective study of nutritional status in immunoglobulin light chain amyloidosis

Haematologica, 2013
Weight loss is common in systemic immunoglobulin light chain amyloidosis but there are limited data on the impact of nutritional status on outcome.
Prayman T. Sattianayagam, Thirusha Lane, Zoe Fox, Aviva Petrie, Simon D.J. Gibbs, Jennifer H. Pinney, Signe S. Risom, Dorota M. Rowczenio, Ashutosh D. Wechalekar, Helen J. Lachmann, Janet A. Gilbertson, Philip N. Hawkins, Julian D. Gillmore   +12 more
doaj   +1 more source

Amyloidosis [PDF]

Annals of Clinical Biochemistry: International Journal of Laboratory Medicine, 2012
The term amyloid describes the deposition in the extracellular space of certain proteins in a highly characteristic, insoluble fibrillar form. Amyloidosis describes the various clinical syndromes that occur as a result of damage by amyloid deposits in tissues and organs throughout the body.
Jennifer H, Pinney, Philip N, Hawkins
openaire   +2 more sources

Outcomes of renal transplantation in patients with AL amyloidosis: an international collaboration through The International Kidney and Monoclonal Gammopathy Research Group

Blood Cancer Journal, 2022
Effective systemic therapies suppress toxic light chain production leading to an increased proportion of patients with light chain (AL) amyloidosis who survive longer albeit with end-stage renal disease.
Andrea Havasi, Cihan Heybeli, Nelson Leung, Avital Angel-Korman, Vaishali Sanchorawala, Oliver Cohen, Ashutosh Wechalekar, Frank Bridoux, Insara Jaffer, Victoria Gutgarts, Hani Hassoun, Maya Levinson, Cara Rosenbaum, Paolo Milani, Giovanni Palladini, Giampaolo Merlini, Ute Hegenbart, Stefan Schönland, Kaya Veelken, Alexander Pogrebinsky, Gheorghe Doros, Heather Landau   +21 more
doaj   +1 more source

Patisiran, an RNAi Therapeutic, for Hereditary Transthyretin Amyloidosis

New England Journal of Medicine, 2018
BACKGROUND Patisiran, an investigational RNA interference therapeutic agent, specifically inhibits hepatic synthesis of transthyretin. METHODS In this phase 3 trial, we randomly assigned patients with hereditary transthyretin amyloidosis with ...
D. Adams, A. González-Duarte, W. O'Riordan, Chih-Chao Yang, M. Ueda, A. Kristen, I. Tournev, H. Schmidt, T. Coelho, J. Berk, Kon-Ping Lin, G. Vita, S. Attarian, V. Planté-Bordeneuve, M. Mezei, J. Campistol, J. Buades, T. Brannagan, Byoung-Joon Kim, Jeeyoung Oh, Y. Parman, Y. Sekijima, P. Hawkins, S. Solomon, M. Polydefkis, P. Dyck, P. Gandhi, S. Goyal, Jihong Chen, A. Strahs, S. Nochur, M. Sweetser, P. Garg, A. Vaishnaw, J. Gollob, O. Suhr   +35 more
semanticscholar   +1 more source

Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies

European Journal of Heart Failure, 2022
An algorithm for non‐invasive diagnosis of amyloid transthyretin cardiac amyloidosis (ATTR‐CA) and novel disease‐modifying therapies have prompted an active search for CA. We examined the prevalence of CA in different settings based on literature data.
A. Aimo, M. Merlo, A. Porcari, G. Georgiopoulos, L. Pagura, G. Vergaro, G. Sinagra, M. Emdin, C. Rapezzi   +8 more
semanticscholar   +1 more source

Navigating the Complex Web of Prescribing Amyloidosis Therapeutics: A Primer

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2022
Advancement in the diagnosis and treatment of transthyretin amyloid cardiomyopathy has made great strides in recent years. Novel therapeutics for transthyretin amyloidosis such as tafamidis, patisiran, and inotersen have shown significant benefits in a ...
Hongya Chen, Pranav Chandrashekar, Katherine Fischer, Dayna Carlson, Urja Narayan, Jack Chen, Ahmad Masri   +6 more
doaj   +1 more source

A nationwide assessment of hepatocellular adenoma resection: Indications and pathological discordance

Hepatology Communications, EarlyView., 2022
Abstract Hepatocellular adenomas (HCAs) are benign liver tumors associated with bleeding or malignant transformation. Data on the indication for surgery are scarce. We analyzed indications and outcome of patients operated for HCAs < 50 mm compared to HCAs ≥ 50 mm. Changes in final postoperative diagnosis were assessed.
Martijn P. D. Haring, Arthur K. E. Elfrink, Christiaan A. J. Oudmaijer, Paul C. M. Andel, Alicia Furumaya, Nenke de Jong, Colin J. J. M. Willems, Thijs Huits, Julie M. L. Sijmons, Eric J. T. Belt, Koop Bosscha, Esther C. J. Consten, Mariëlle M. E. Coolsen, Peter van Duijvendijk, Joris I. Erdmann, Paul Gobardhan, Robbert J. de Haas, Tjarda van Heek, Hwai‐Ding Lam, Wouter K. G. Leclercq, Mike S. L. Liem, Hendrik A. Marsman, Gijs A. Patijn, Türkan Terkivatan, Babs M. Zonderhuis, Izaak Quintus Molenaar, Wouter W. te Riele, Jeroen Hagendoorn, Alexander F. M. Schaapherder, Jan N. M. IJzermans, Carlijn I. Buis, Joost M. Klaase, Koert P. de Jong, Vincent E. de Meijer, the Dutch Benign Liver Tumor Group, R. B. Takkenberg, J. Verheij, O. M. van Delden, J. I. Erdmann, B. V. van Rosmalen, A. Furumaya, G. Kazemier, D. Ramsoekh, J. N. M. IJzermans, R. A. de Man, M. D. Doukas, M. G. Thomeer, A. J. Klompenhouwer, A. F. M. Schaapherder, M. E. Tushuizen, M. J. Coenraad, A. S. L. P. Crobach, M. C. Burgmans, S. Feshtali, M. M. E. Coolsen, M. Kramer, J. C. Beckervordersandforth, I. V. Samarska, C. van der Leij, R. Miclea, J. H. W. de Wilt, P. B. van den Boezem, E. T. T. L. Tjwa, I. Munsterman, S. Vos, S. van Koeverden, V. E. de Meijer, F. J. C. Cuperus, R. J. de Haas, E. W. Duiker, M. P. D. Haring   +70 more
wiley   +1 more source

Clinical Amyloid Typing by Proteomics: Performance Evaluation and Data Sharing between Two Centres

Molecules, 2021
Amyloidosis is a relatively rare human disease caused by the deposition of abnormal protein fibres in the extracellular space of various tissues, impairing their normal function. Proteomic analysis of patients’ biopsies, developed by Dogan and colleagues
Diana Canetti, Francesca Brambilla, Nigel B. Rendell, Paola Nocerino, Janet A. Gilbertson, Dario Di Silvestre, Andrea Bergamaschi, Francesca Lavatelli, Giampaolo Merlini, Julian D. Gillmore, Vittorio Bellotti, Pierluigi Mauri, Graham W. Taylor   +12 more
doaj   +1 more source