Results 51 to 60 of about 148,376 (307)
Atrial amyloidosis: mechanisms and clinical manifestations
European Journal of Heart Failure, 2022 Cardiac amyloidosis (CA) is now recognized as an important cause of heart failure. Increased wall thickness and diastolic dysfunction of the left ventricle are the most easily detectable manifestations of CA, but amyloid accumulates in all cardiac ...G. Vergaro, A. Aimo, C. Rapezzi, V. Castiglione, I. Fabiani, A. Pucci, G. Buda, C. Passino, J. Lupón, A. Bayés‐Genís, M. Emdin, E. Braunwald +11 moresemanticscholar +1 more sourceClinical Practice Guideline for Evaluation and Management of Peripheral Nervous System Manifestations in Sjögren's Disease
Arthritis Care &Research, Accepted Article.Objectives
Sjögren's disease is an autoimmune disorder that can impact multiple organ systems, including the peripheral nervous system (PNS). PNS manifestations, which can exist concurrently, include mononeuropathies, polyneuropathies, and autonomic nervous system neuropathies. To help patients and providers in the decision‐making process, we developed Anahita Deboo, Robert Fox, Katherine M. Hammitt, Julie Frantsve‐Hawley, Matthew C. Baker, Stamatina Danielides, Eduardo De Sousa, Brent P. Goodman, Jennifer K. King, Steven Mandel, Ghaith Noaiseh, Pantelis P. Pavlakis, George Sarka, R. Hal Scofield, Arun Varadhachary, Daniel J. Wallace, Matt Makara, Nancy Carteron, Steven Carsons, In collaboration with the Consensus Expert Panel (CEP) members +19 morewiley +1 more sourceClinical and genetic profile of patients enrolled in the Transthyretin Amyloidosis Outcomes Survey (THAOS): 14-year update
Orphanet Journal of Rare Diseases, 2022 Background Transthyretin amyloidosis (ATTR amyloidosis) is a rare, life-threatening disease caused by the accumulation of variant or wild-type (ATTRwt amyloidosis) transthyretin amyloid fibrils in the heart, peripheral nerves, and other tissues and ...A. Dispenzieri, T. Coelho, I. Conceição, M. Waddington-Cruz, J. Wixner, A. Kristen, C. Rapezzi, V. Planté-Bordeneuve, J. González-Moreno, M. Maurer, M. Grogan, Doug Chapman, L. Amass, Pablo Garcia Ivaylo Jose Gonzalez Maria Alejandra Gonzal Pavia Tarnev Costello Briseno Schmidt Drachman Bar, P. G. Pavía, Ivaylo Tarnev, J. Costello, Maria Alejandra González-Duarte Briseño, H. Schmidt, B. Drachman, F. Barroso, T. Yamashita, O. Lairez, Y. Sekijima, G. Vita, E. Jeon, M. Hanna, D. Slosky, M. Luigetti, S. Lorusso, F. Beamud, D. Adams, H. Moelgaard, R. Press, C. Cirami, Hans Nienhuis, J. M. Plana, J. Inamo, D. Jacoby, M. Emdin, D. Quan, S. Hummel, R. Witteles, A. Dori, Sanjiv Shah, D. Lenihan, O. Azevedo, S. Murali, S. Živković, S. Low, J. Nativi-Nicolau, N. Fine, J. Tallaj, C. Tschoepe, R. Torrón, M. Polydefkis, G. Merlini, S. Bădeliță, Stephen S. Gottlieb, J. Tauras, E. B. Correia, H. Ventura, B. Gess, F. Darstein, Jeeyoung Oh, Tessa Marburger, J. Van Cleemput, V. Salutto, Y. Parman, C. Chao, N. Sarswat, Christopher Mueller, D. Steidley, J. Ralph, A. Warner, W. Cotts, J. Hoffman, M. Rugiero, S. Misawa, J. L. Blanco, L. G. Dávila, M. Sadeh, Jinju Luo, T. Kyriakides, Annabel K. Wang, H. Kaufmann, S. Živković +86 moresemanticscholar +1 more sourceAL 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 moreopenaire +4 more sourcesPotent Liver‐Tropic mRNA Lipid Nanoparticles: ApoE‐Mediated Delivery Through a Low‐Density Lipoprotein Receptor Independent Uptake Mechanism
Advanced Materials, EarlyView.Helper and ionizable lipids play a crucial role in determining ApoE binding and subsequent liver tropism and LDLR‐mediated uptake. Ionizable lipids primarily govern the LDLR‐independent uptake pathway. This complementary interplay between lipid components ultimately governs LNP delivery performance and therapeutic efficacy in the liver.Ashish Sarode, Christian Ortiz, Tadeh Derstepanian, Natalia Vargas‐Montoya, Priyal Patel, Nikita Khadse, Saikat Manna, Ryan Landis, Joseph Skaleski, Lianne Boeglin, Hongfeng Deng, Anusha Dias, Hong Wang, Debora Barreiros Petropolis, Barak Yahalom, Shrirang Karve, Frank DeRosa +16 morewiley +1 more sourceCardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience
Frontiers in Cardiovascular Medicine, 2023 AimsTransthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to cardiac transplantation (CT).Yousuf Razvi, Aldostefano Porcari, Aldostefano Porcari, Concetta Di Nora, Rishi K. Patel, Adam Ioannou, Muhammad U. Rauf, Ambra Masi, Steven Law, Liza Chacko, Tamer Rezk, Sriram Ravichandran, Janet Gilbertson, Dorota Rowczenio, Iona J. Blakeney, Nandita Kaza, David F. Hutt, Helen Lachmann, Ashutosh Wechalekar, William Moody, Sern Lim, Colin Chue, Carol Whelan, Lucia Venneri, Ana Martinez-Naharro, Marco Merlo, Gianfranco Sinagra, Ugolino Livi, Philip Hawkins, Marianna Fontana, Julian D. Gillmore +30 moredoaj +1 more sourceInotersen Treatment for Patients with Hereditary Transthyretin Amyloidosis
New England Journal of Medicine, 2018 BACKGROUND Hereditary transthyretin amyloidosis is caused by pathogenic single‐nucleotide variants in the gene encoding transthyretin (TTR) that induce transthyretin misfolding and systemic deposition of amyloid. Progressive amyloid accumulation leads to M. Benson, M. Waddington-Cruz, J. Berk, M. Polydefkis, P. Dyck, Annabel K. Wang, V. Planté-Bordeneuve, F. Barroso, G. Merlini, L. Obici, M. Scheinberg, T. Brannagan, W. Litchy, C. Whelan, B. Drachman, D. Adams, S. Heitner, I. Conceição, H. Schmidt, G. Vita, J. Campistol, J. Gámez, P. Gorevic, E. Gane, A. Shah, S. Solomon, B. Monia, S. Hughes, J. Kwoh, B. McEvoy, Shiangtung W. Jung, B. Baker, E. Ackermann, M. Gertz, T. Coelho +34 moresemanticscholar +1 more sourceNARFL Knockout Triggers Ferroptosis‐Driven Vascular Endothelial Dysfunction
Advanced Science, EarlyView.NARFL is vital for CIA and oxidative stress resistance. NARFL deletion in HPMEC cells, zebrafish, and mice is lethal and rescued by a Ferroptosis inhibitor. NARFL deficiency disrupted its interaction with CIA proteins, decreased aconitase activity, increased IRP1 activity, induced Fe overload, and led to ferroptosis and oxidative stress, resulting in ...Hui Hu, Jing Luo, Li Yu, Daoxi Qi, Boyu Li, Yating Chen, Chen Wang, Xiaokang Zhang, Wenzheng Guo, Qiyong Lou, Gang Zhai, Yonglin Ruan, Jianfei Huang, Shengchi Shi, Zhan Yin, Fang Zheng +15 morewiley +1 more sourceMultiorgan Dysfunction and Associated Prognosis in Transthyretin Cardiac Amyloidosis
Journal of the American Heart Association: Cardiovascular and Cerebrovascular DiseaseBackground Transthyretin cardiac amyloidosis (ATTR‐CA) is a progressive and ultimately fatal cardiomyopathy. Biomarkers reflecting multiorgan dysfunction are of increasing importance in patients with heart failure; however, their significance in ATTR‐CA ...Adam Ioannou, Christian Nitsche, Aldostefano Porcari, Rishi K. Patel, Yousuf Razvi, Muhammad U. Rauf, Ana Martinez‐Naharro, Lucia Venneri, Antonella Accietto, Lucrezia Netti, Francesco Bandera, Ruta Virsinskaite, Tushar Kotecha, Dan Knight, Aviva Petrie, Carol Whelan, Ashutosh Wechalekar, Helen Lachmann, Philip N. Hawkins, Julian D. Gillmore, Marianna Fontana +20 moredoaj +1 more sourceEvaluation of a new ELISA assay for monoclonal free‐light chain detection in patients with cardiac amyloidosis
eJHaem, 2022 The causal protein of amyloid light‐chain (AL) amyloidosis is a monoclonal immunoglobulin free light chain (mFLC), which must be quantified in the serum for patient diagnosis and monitoring.Hajer Abroud, Asma Beldi‐Ferchiou, Vincent Audard, François Lemonnier, Fabien Le Bras, Karim Belhadj, Anissa Moktefi, Elsa Poullot, Khalil El Karoui, Jehan Dupuis, Alizée Maarek, Louise Roulin, Marie‐Hélène Delfau‐Larue, Silvia Oghina, Mounira Kharoubi, Mélanie Bézard, Amira Zaroui, Thibaud Damy, Valérie Molinier‐Frenkel +18 moredoaj +1 more source
