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Cardiac amyloid

Paolo Milani, Giampaolo Merlini, Giovanni Palladini
Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of dialysis is predicted by the severity of renal involvement, defined by the baseline proteinuria and glomerular filtration rate, and by the response to therapy...
2018: Mediterranean Journal of Hematology and Infectious Diseases
Patrick Krumm, Stefanie Mangold, Sergios Gatidis, Konstantin Nikolaou, Felix Nensa, Fabian Bamberg, Christian la Fougère
Combined PET/MRI is a novel imaging method integrating the advances of functional and morphological MR imaging with PET applications that include assessment of myocardial viability, perfusion, metabolism of inflammatory tissue and tumors, as well as amyloid deposition imaging. As such, PET/MRI is a promising tool to detect and characterize ischemic and non-ischemic cardiomyopathies. To date, the greatest benefit may be expected for diagnostic evaluation of systemic diseases and cardiac masses that remain unclear in cardiac MRI, as well as for clinical and scientific studies in the setting of ischemic cardiomyopathies...
March 10, 2018: Japanese Journal of Radiology
Broes Martens, Michel De Pauw, Jan L De Bleecker
Familial amyloid polyneuropathy (FAP) is a most often length-dependent axonal neuropathy, often part of a multisystem disorder also affecting other organs, such as cardiac, gastrointestinal, genitourinary, renal, meningeal and eye tissue. It is most frequently the result of a mutation in the TTR gene, most commonly a p.Val50Met mutation. TTR-FAP is a rare autosomal dominant heritable disabling, heterogeneous disease in which early diagnosis is of pivotal importance when attempting treatment. This paper discusses the course of four Belgian FAP patients with different TTR mutations (p...
March 9, 2018: Acta Neurologica Belgica
Wengen Chen, Van-Khue Ton, Vasken Dilsizian
PURPOSE OF REVIEW: The two most common types of cardiac amyloidosis are caused by fibril deposits of immunoglobulin light chains (AL) and transthyretin (TTR), each with distinct prognosis and clinical management. Cardiac amyloidosis is under-recognized among heart failure patients with preserved ejection fraction (HFpEF). Bone-seeking tracers like99m Tc-PYP and99m Tc-DPD have long been used to identify cardiac amyloidosis, and more recently, to differentiate TTR from AL cardiac amyloidosis in symptomatic patients...
March 8, 2018: Current Cardiology Reports
Paul R Scully, Gorka Bastarrika, James C Moon, Thomas A Treibel
PURPOSE OF REVIEW: This review article discusses the evolution of extracellular volume (ECV) quantification using both cardiovascular magnetic resonance (CMR) and computed tomography (CT). RECENT FINDINGS: Visualizing diffuse myocardial fibrosis is challenging and until recently, was restricted to the domain of the pathologist. CMR and CT both use extravascular, extracellular contrast agents, permitting ECV measurement. The evidence base around ECV quantification by CMR is growing rapidly and just starting in CT...
March 6, 2018: Current Cardiology Reports
Eve Piekarski, Renata Chequer, Vincent Algalarrondo, Ludivine Eliahou, Besma Mahida, Jonathan Vigne, David Adams, Michel S Slama, Dominique Le Guludec, Francois Rouzet
PURPOSE: Cardiac involvement in familial transthyretin (TTR) amyloidosis is of major prognostic value, and the development of early-diagnostic tools that could trigger the use of new disease-modifying treatments is crucial. The aim of our study was to compare the respective contributions of99m Tc-diphosphonate scintigraphy (DPD, detecting amyloid deposits) and123 I-MIBG (MIBG, assessing cardiac sympathetic denervation) in patients with genetically proven TTR mutation referred for the assessment of cardiac involvement...
March 6, 2018: European Journal of Nuclear Medicine and Molecular Imaging
Jeffrey M C Lau, Demetrios A Raptis, Richard Laforest, Felix Nensa, Jie Zheng, Robert J Gropler, Pamela K Woodard
Simultaneous acquisition positron emission tomography-magnetic resonance imaging (PET-MRI) has the ability to combine anatomic information derived from cardiac MRI with quantitative capabilities of cardiac PET and MRI and the promise of molecular imaging by specific PET tracers. This combination of cardiac PET and MRI delivers a robust and comprehensive clinical examination. It has the potential to assess various cardiovascular conditions, including assessment of myocardial ischemia, infarction, and function, as well as specific characterization of inflammatory and infiltrative heart diseases such as cardiac sarcoid and amyloid...
February 27, 2018: Journal of Thoracic Imaging
O Bourogianni, E Papadaki, E Foukarakis, S Koukouraki
BACKGROUND: Three types of amyloid are responsible for cardiac amyloidosis. Differentiation of the subtype is critical for the disease progression and the therapeutic decision. RESULTS: Myocardial scintigraphy using Tc-PYP is able to differentiate the cardiac amyloid subtype with high sensitivity and specificity. The myocardial uptake of PYP is higher in patients with TTR amyloidosis. CONCLUSION: Non-invasive tests for the detection of cardiac amyloidosis, like myocardial scintigraphy with bone seeking tracers, can play a major role in the diagnosis progression and therapeutic management of patients with cardiac amyloidosis...
February 23, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Yanping Zhu, Xiaoyang Shan, Farzaneh Safarpour, Nancy Erro Go, Nancy Li, Alice Shan, Mina Huang, Matthew Deen, Viktor Holicek, Roger Ashmus, Zarina Madden, Sharon Gorski, Michael Silverman, David J Vocadlo
The glycosylation of nucleocytoplasmic proteins with O-linked N-acetylglucosamine residues (O-GlcNAc) is conserved among metazoans and is particularly abundant within brain. O-GlcNAc is involved in diverse cellular processes ranging from the regulation of gene expression to stress response. Moreover, O-GlcNAc is implicated in various diseases including cancers, diabetes, cardiac dysfunction, and neurodegenerative diseases. Pharmacological inhibition of O-GlcNAcase (OGA), the sole enzyme that removes O-GlcNAc, reproducibly slows neurodegeneration in various Alzheimer Disease (AD) mouse models manifesting either tau or amyloid pathology...
February 20, 2018: ACS Chemical Neuroscience
Jacquelyn L S Hanson, Marios Arvanitis, Clarissa M Koch, John L Berk, Frederick L Ruberg, Tatiana Prokaeva, Lawreen H Connors
BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt), an underappreciated cause of heart failure in older adults, is challenging to diagnose and monitor in the absence of validated, disease-specific biomarkers. We examined the prognostic use and survival association of serum TTR (transthyretin) concentration in ATTRwt. METHODS AND RESULTS: Patients with biopsy-proven ATTRwt were retrospectively identified. Serum TTR, cardiac biomarkers, and echocardiographic parameters were assessed at baseline and follow-up evaluations...
February 2018: Circulation. Heart Failure
Pavla Flodrova, Patrik Flodr, Tomas Pika, Jiri Vymetal, Dusan Holub, Petr Dzubak, Marian Hajduch, Vlastimil Scudla
Amyloidosis is a heterogeneous group of diseases characterised by extracellular accumulation of amyloid in various tissues and organs of the body, leading to alteration and destruction of tissues. Heart involvement is the most important prognostic factor in patients with systemic amyloidosis and the diagnosis and typing of amyloid must be made properly. The clinical picture shows congestive heart failure with predominant right-sided heart failure symptoms in fully developed disease, various types of arrhythmias and characteristic electrocardiography and echocardiography findings...
February 12, 2018: Pathology
C Fielder Camm, Jaimal Kothari, Victoria Stnoble, Nikant K Sabharwal
No abstract text is available yet for this article.
February 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
L Li, X J Duan, Y Sun, Y Lu, H Y Xu, Q Z Wang, H Y Wang
Objective: To evaluate the sensitivity and specificity of immunohistochemistry (IHC) in the classification of cardiac amyloidosis on endomyocardial biopsy (EMB) and heart allograft. Methods: Twenty cardiac tissues from 19 patients at Fuwai Hospital from January, 1990 to April, 2017 with histopathologic features of amyloidosis and Congo red staining positivity were included. IHC was performed with monoclonal antibodies against AA amyloid and polyclonal antibodies against transthyretin (ATTR), λ-light chain (AL-λ), κ-light chain (AL-κ), ApoAⅠ, ApoAⅡ, ApoA Ⅳ and β(2)-microglobin...
February 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Mohd Aizat Abdul Rahim, Zubaidah Haji Abdul Rahim, Wan Azman Wan Ahmad, Marina Mohd Bakri, Muhammad Dzafir Ismail, Onn Haji Hashim
An early intervention using biomarkers to predict acute myocardial infarction (AMI) will effectively reduce global heart attack incidence, particularly among high-risk patients with type 2 diabetes mellitus (T2DM). This study attempted to identify potential biomarkers by detecting changes in the levels of plasma proteins in T2DM patients following onset of AMI in comparison with those without AMI. Volunteer T2DM patients without AMI (control; n=10) and T2DM patients with AMI (n=10) were recruited. Plasma samples from these patients were evaluated via two-dimensional gel electrophoresis (2DE) to screen for proteins with level changes between the two groups...
February 8, 2018: Acta Pharmacologica Sinica
Tamer Rezk, Janet A Gilbertson, Dorota Rowczenio, Paul Bass, Helen J Lachmann, Ashutosh D Wechalekar, Marianna Fontana, Shameem Mahmood, Sajitha Sachchithanantham, Carol J Whelan, Jonathan Wong, Nigel Rendell, Graham W Taylor, Philip N Hawkins, Julian D Gillmore
Introduction: Renal biopsy series from North America suggest that leucocyte chemotactic factor 2 (ALECT2) amyloid is the third most common type of renal amyloid. We report the first case series from a European Centre of prevalence, clinical presentation and diagnostic findings in ALECT2 amyloidosis and report long-term patient and renal outcomes for the first time. Methods: We studied the clinical features, diagnostic investigations and the outcome of all patients with ALECT2 amyloidosis followed systematically at the UK National Amyloidosis Centre (NAC) between 1994 and 2015...
February 1, 2018: Nephrology, Dialysis, Transplantation
Urban Wiklund, Amir Kadkhodaee, Kennet Andersson, Ole B Suhr, Rolf Hörnsten
BACKGROUND: The heart rate (HR) response to paced deep breathing (DB) is a common test of cardiac autonomic function, where high heart rate variability (HRV) is considered to reflect normal autonomic function. We evaluated the DB test in patients with hereditary transthyretin amyloid (ATTRm) amyloidosis, where autonomic dysregulation and atrial arrhythmias are common. METHODS: Paced DB was performed during one minute (six breaths/min) in 165 recordings in adult ATTRm amyloidosis patients with the TTR Val30Met mutation, 42 hypertrophic cardiomyopathy (HCM) patients and 211 healthy subjects...
February 2, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
Rebecca Y Klinger, Olga G James, Salvador Borges-Neto, Tiffany Bisanar, Yi-Ju Li, Wenjing Qi, Miles Berger, Niccolò Terrando, Mark F Newman, P Murali Doraiswamy, Joseph P Mathew, Michael W Weiner, Paul Aisen, Michael Weiner, Paul Aisen, Ronald Petersen, Clifford R Jack, William Jagust, John Q Trojanowki, Arthur W Toga, Laurel Beckett, Robert C Green, Andrew J Saykin, John Morris, Leslie M Shaw, Zaven Khachaturian, Greg Sorensen, Maria Carrillo, Lew Kuller, Marc Raichle, Steven Paul, Peter Davies, Howard Fillit, Franz Hefti, David Holtzman, M Marcel Mesulam, William Potter, Peter Snyder, Adam Schwartz, Robert C Green, Tom Montine, Ronald Petersen, Paul Aisen, Ronald G Thomas, Michael Donohue, Sarah Walter, Devon Gessert, Tamie Sather, Gus Jiminez, Archana B Balasubramanian, Jennifer Mason, Iris Sim, Laurel Beckett, Danielle Harvey, Michael Donohue, Clifford R Jack, Matthew Bernstein, Nick Fox, Paul Thompson, Norbert Schuff, Charles DeCArli, Bret Borowski, Jeff Gunter, Matt Senjem, Prashanthi Vemuri, David Jones, Kejal Kantarci, Chad Ward, William Jagust, Robert A Koeppe, Norm Foster, Eric M Reiman, Kewei Chen, Chet Mathis, Susan Landau, John C Morris, Louis Nigel J Cairns, Erin Franklin, Lisa Taylor-Reinwald, Leslie M Shaw, John Q Trojanowki, Virginia Lee, Magdalena Korecka, Michal Figurski, Arthur W Toga, Karen Crawford, Scott Neu, Andrew J Saykin, Tatiana M Foroud, Steven Potkin, Li Shen, Kelley Faber, Sungeun Kim, Kwangsik Nho, Michael W Weiner, Lean Thal, Zaven Khachaturian, Leon Thal, Neil Buckholtz, Michael W Weiner, Peter J Snyder, William Potter, Steven Paul, Marilyn Albert, Richard Frank, Zaven Khachaturian, John Hsiao, Jeffrey Kaye, Joseph Quinn, Lisa Silbert, Betty Lind, Raina Carter, Sara Dolen, Lon S Schneider, Sonia Pawluczyk, Mauricio Becerra, Liberty Teodoro, Bryan M Spann, James Brewer, Helen Vanderswag, Adam Fleisher, Judith L Heidebrink, Joanne L Lord, Ronald Petersen, Sara S Mason, Colleen S Albers, David Knopman, Kris Johnson, Rachelle S Doody, Javier Villanueva-Meyer, Valory Pavlik, Victoria Shibley, Munir Chowdhury, Susan Rountree, Mimi Dang, Yaakov Stern, Lawrence S Honig, Karen L Bell, Beau Ances, John C Morris, Maria Carroll, Mary L Creech, Erin Franklin, Mark A Mintun, Stacy Schneider, Angela Oliver, Daniel Marson, David Geldmacher, Marissa Natelson Love, Randall Griffith, David Clark, John Brockington, Erik Roberson, Hillel Grossman, Effie Mitsis, Raj C Shah, Leyla deToledo-Morrell, Ranjan Duara, Maria T Greig-Custo, Warren Barker, Marilyn Albert, Chiadi Onyike, Daniel D'Agostino, Stephanie Kielb, Martin Sadowski, Mohammed O Sheikh, Anaztasia Ulysse, Mrunalini Gaikwad, Jeffrey R Petrella, Terence Z Wong, Edward Coleman, Steven E Arnold, Jason H Karlawish, David A Wolk, Christopher M Clark, Charles D Smith, Greg Jicha, Peter Hardy, Partha Sinha, Elizabeth Oates, Gary Conrad, Oscar L Lopez, MaryAnn Oakley, Donna M Simpson, Anton P Porsteinsson, Bonnie S Goldstein, Kim Martin, Kelly M Makino, M Saleem Ismail, Connie Brand, Steven G Potkin, Adrian Preda, Dana Nguyen, Kyle Womack, Dana Mathews, Mary Quiceno, Allan I Levey, James J Lah, Janet S Cellar, Jeffrey M Burns, Russell H Swerdlow, William M Brooks, Liana Apostolova, Kathleen Tingus, Ellen Woo, Daniel H S Silverman, Po H Lu, George Bartzokis, Neill R Graff-Radford, Francine Parfitt, Kim Poki-Walker, Martin R Farlow, Ann Marie Hake, Brandy R Matthews, Jared R Brosch, Scott Herring, Christopher H van Dyck, Richard E Carson, Martha G MacAvoy, Pradeep Varma, Howard Chertkow, Howard Bergman, Chris Hosein, Sandra Black, Bojana Stefanovic, Curtis Caldwell, Ging-Yuek, Robin Hsiung, Benita Mudge, Vesna Sossi, Howard Feldman, Michele Assaly, Elizabeth Finger, Stephen Pasternack, Irina Rachisky, Dick Trost, Andrew Kertesz, Charles Bernick, Donna Munic, Marek-Marsel Mesulam, Emily Rogalski, Kristine Lipowski, Sandra Weintraub, Borna Bonakdarpour, Diana Kerwin, Chuang-Kuo Wu, Nancy Johnson, Carl Sadowsky, Teresa Villena, Raymond Scott Turner, Kathleen Johnson, Brigid Reynolds, Reisa A Sperling, Keith A Johnson, Gad Marshall, Jerome Yesavage, Joy L Taylor, Barton Lane, Allyson Rosen, Jared Tinklenberg, Marwan N Sabbagh, Christine M Belden, Sandra A Jacobson, Sherye A Sirrel, Neil Kowall, Ronald Killiany, Andrew E Budson, Alexander Norbash, Patricia Lynn Johnson, Thomas O Obisesan, Saba Wolday, Joanne Allard, Alan Lerner, Paula Ogrocki, Curtis Tatsuoka, Parianne Fatica, Evan Fletcher, Pauline Maillard, John Olichney, Charles DeCarli, Owen Carmichael, Smita Kittur, Michael Borrie, T-Y Lee, Dr Rob Bartha, Sterling Johnson, Sanjay Asthana, Cynthia M Carlsson, Steven G Potkin, Adrian Preda, Dana Nguyen, Pierre Tariot, Anna Burke, Ann Marie Milliken, Nadira Trncic, Adam Fleisher, Stephanie Reeder, Vernice Bates, Horacio Capote, Michelle Rainka, Douglas W Scharre, Maria Kataki, Brendan Kelley, Earl A Zimmerman, Dzintra Celmins, Alice D Brown, Godfrey D Pearlson, Karen Blank, Karen Anderson, Laura A Flashman, Marc Seltzer, Mary L Hynes, Robert B Santulli, Kaycee M Sink, Leslie Gordineer, Jeff D Williamson, Pradeep Garg, Franklin Watkins, Brian R Ott, Geoffrey Tremont, Lori A Daiello, Stephen Salloway, Paul Malloy, Stephen Correia, Howard J Rosen, Bruce L Miller, David Perry, Jacobo Mintzer, Kenneth Spicer, David Bachman, Elizabeth Finger, Stephen Pasternak, Irina Rachinsky, John Rogers, Andrew Kertesz, Dick Drost, Nunzio Pomara, Raymundo Hernando, Antero Sarrael, Susan K Schultz, Karen Ekstam Smith, Hristina Koleva, Ki Won Nam, Hyungsub Shim, Norman Relkin, Gloria Chiang, Michael Lin, Lisa Ravdin, Amanda Smith, Balebail Ashok Raj, Kristin Fargher, Michael W Weiner, Paul Aisen, Michael Weiner, Paul Aisen, Ronald Petersen, Robert C Green, Danielle Harvey, Clifford R Jack, William Jagust, John C Morris, Andrew J Saykin, Leslie M Shaw, Arthur W Toga, John Q Trojanowki, Thomas Neylan, Jordan Grafman, Jordan Grafman, Robert C Green, Tom Montine, Michael Weiner, Ronald Petersen, Paul Aisen, Ronald G Thomas, Michael Donohue, Devon Gessert, Tamie Sather, Melissa Davis, Rosemary Morrison, Gus Jiminez, Thomas Neylan, Jacqueline Hayes, Shannon Finely, Danielle Harvey, Michael Donohue, Clifford R Jack, Matthew Bernstein, Bret Borowski, Jeff Gunter, Matt Senjem, Kejal Kantarci, Chad Ward, William Jagust, Robert A Koeppe, Norm Foster, Eric M Reiman, Kewei Chen, Susan Landau, John C Morris, Nigel J Cairns, Erin Householder, Leslie M Shaw, John Q Trojanowki, Virginia Lee, Magdalena Korecka, Michal Figurski, Arthur W Toga, Karen Crawford, Scott Neu, Andrew J Saykin, Tatiana M Foroud, Steven Potkin, Li Shen, Kelley Faber, Sungeun Kim, Kwangsik Nho, Michael W Weiner, Karl Friedl, Lon S Schneider, Sonia Pawluczyk, Mauricio Becerra, James Brewer, Helen Vanderswag, Yaakov Stern, Lawrence S Honig, Karen L Bell, Debra Fleischman, Konstantinos Arfanakis, Raj C Shah, Ranjan Duara, Daniel Varon, Maria T Greig, Jeffrey R Petrella, Anton P Porsteinsson, Bonnie Goldstein, Kimberly S Martin, Steven G Potkin, Adrian Preda, Dana Nguyen, Jacobo Mintzer, Dino Massoglia, Olga Brawman-Mintzer, Carl Sadowsky, Walter Martinez, Teresa Villena, William Jagust, Susan Landau, Howard Rosen, David Perry, Raymond Scott Turner, Kelly Behan, Brigid Reynolds, Reisa A Sperling, Keith A Johnson, Gad Marshall, Marwan N Sabbagh, Sandra A Jacobson, Sherye A Sirrel, Thomas O Obisesan, Saba Wolday, Joanne Allard, Sterling C Johnson, J Jay Fruehling, Sandra Harding, Elaine R Peskind, Eric C Petrie, Gail Li, Jerome A Yesavage, Joy L Taylor, Ansgar J Furst, Steven Chao, Norman Relkin, Gloria Chiang, Lisa Ravdin, James A Blumenthal, Jorn A Karhausen, Miklos D Kertai, Mihai V Podgoreanu, Mark Stafford-Smith, Madhav Swaminathan, David S Warner, Bonita L Funk, Narai Balajonda, Rachele Brassard, Mary Cooter, Yanne Toulgoat-Dubois, Peter Waweru, Michael A Babyak, James A Blumenthal, Jeffrey N Browndyke, Kathleen A Welsh-Bohmer, Michael H Sketch, Ellen R Bennett, Carmelo Graffagnino, Daniel T Laskowitz, Warren J Strittmatter, Kevin Collins, Greg Smigla, Ian Shearer, Thomas A D'Amico, Mani A Daneshmand, R Jeffrey G Gaca, Donald D Glower, Jack Haney, R David Harpole, Mathew G Hartwig, G Chad Hughes, Jacob A Klapper, Shu S Lin, Andrew J Lodge, Carmelo A Milano, Ryan P Plichta, Jacob N Schroeder, Peter K Smith, Betty C Tong
BACKGROUND: Amyloid deposition is a potential contributor to postoperative cognitive dysfunction. The authors hypothesized that 6-week global cortical amyloid burden, determined by F-florbetapir positron emission tomography, would be greater in those patients manifesting cognitive dysfunction at 6 weeks postoperatively. METHODS: Amyloid deposition was evaluated in cardiac surgical patients at 6 weeks (n = 40) and 1 yr (n = 12); neurocognitive function was assessed at baseline (n = 40), 6 weeks (n = 37), 1 yr (n = 13), and 3 yr (n = 9)...
February 1, 2018: Anesthesiology
Sirinart Kumfu, Savitree T Charununtakorn, Thidarat Jaiwongkam, Nipon Chattipakorn, Siriporn C Chattipakorn
 Cardiac ischemia-reperfusion (I/R) injury has been shown to impair brain function. Humanin analogue (HNG) given prior to cardiac ischemia has been shown to attenuate both heart and brain mitochondrial dysfunction caused by cardiac I/R injury. In a clinical setting, patients received medical treatment for acute myocardial infarction either during or after the onset of myocardial ischemia; thus, in this study, we tested the hypothesis that the administration of HNG during cardiac I/R injury has therapeutic potential for brain protection...
2018: Journal of Alzheimer's Disease: JAD
Yoshiki Sekijima, Mitsuharu Ueda, Haruki Koike, Sonoko Misawa, Tomonori Ishii, Yukio Ando
Hereditary ATTR (ATTRm) amyloidosis (also called transthyretin-type familial amyloid polyneuropathy [ATTR-FAP]) is an autosomal-dominant, adult-onset, rare systemic disorder predominantly characterized by irreversible, progressive, and persistent peripheral nerve damage. TTR gene mutations (e.g. replacement of valine with methionine at position 30 [Val30Met (p.Val50Met)]) lead to destabilization and dissociation of TTR tetramers into variant TTR monomers, which form amyloid fibrils that deposit in peripheral nerves and various organs, giving rise to peripheral and autonomic neuropathy and several non-disease specific symptoms...
January 17, 2018: Orphanet Journal of Rare Diseases
Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P Rainer
Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated...
January 4, 2018: Current Medicinal Chemistry
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