Results 21 to 30 of about 162,440 (399)

AL Amyloidosis [PDF]

Orphanet Journal of Rare Diseases, 2012
Abstract Definition of the disease AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone.
Desport, Estelle, Bridoux, Franck, Sirac, Christophe, Delbes, Sébastien, Bender, Sébastien, Fernandez, Béatrice, Quellard, Nathalie, Lacombe, Corinne, Goujon, Jean-Michel, Lavergne, David, Abraham, Julie, Touchard, Guy, Fermand, Jean-Paul, Jaccard, Arnaud   +13 more
openaire   +5 more sources

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

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

Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage

ESC Heart Failure, 2020
Aims Cardiac transthyretin amyloidosis (ATTR‐CM) is a progressive and fatal condition. Prognosis can be determined at diagnosis according to the National Amyloidosis Centre (NAC) transthyretin amyloidosis (ATTR) stage.
Steven Law, Aviva Petrie, Liza Chacko, Oliver C. Cohen, Sriram Ravichandran, Janet A. Gilbertson, Dorota Rowczenio, Ashutosh Wechalekar, Ana Martinez‐Naharro, Helen J. Lachmann, Carol J. Whelan, David F. Hutt, Philip N. Hawkins, Marianna Fontana, Julian D. Gillmore   +14 more
doaj   +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

Amyloidosis [PDF]

Postgraduate Medical Journal, 2001
Abstract Amyloidosis is not a single disease but a series of diseases in which there is extracellular deposition of a protein which, although it may be derived from different and unrelated sources, folds into a β pleated sheet. There have recently been significant advances in elucidating the pathogenesis and in the ...
M F, Khan, R H, Falk
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

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

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

Systemic amyloidosis in England: an epidemiological study. [PDF]

, 2013
Epidemiological studies of systemic amyloidosis are scarce and the burden of disease in England has not previously been estimated. In 1999, the National Health Service commissioned the National Amyloidosis Centre (NAC) to provide a national clinical ...
Banypersad, SM, Dungu, J, Gibbs, SD, Gillmore, JD, Hawkins, PN, Lachmann, HJ, Pinney, JH, Smith, CJ, Taube, JB, Venner, CP, Wechalekar, AD, Whelan, CJ   +11 more
core   +1 more source