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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/29664336/left-ventricular-myocardial-deformation-on-cine-mr-images-relationship-to-severity-of-disease-and-prognosis-in-amyloid-light-chain-amyloidosis
#1
Ke Wan, Jiayu Sun, Dan Yang, Hong Liu, Jie Wang, Wei Cheng, Qing Zhang, Zhi Zeng, Tianjing Zhang, Andreas Greiser, Marie-Pierre Jolly, Yuchi Han, Yucheng Chen
Purpose To measure left ventricular (LV) myocardial strain with cine magnetic resonance (MR) imaging and a deformable registration algorithm (DRA) and to assess the prognostic value of myocardial strain in patients with light-chain (AL) amyloidosis. Materials and Methods In this prospective study, 78 consecutive patients with AL amyloidosis who underwent contrast material-enhanced cardiac MR imaging were enrolled at West China Hospital. LV myocardial strains and late gadolinium enhancement (LGE) were evaluated...
April 17, 2018: Radiology
https://www.readbyqxmd.com/read/29664151/the-receptor-for-advanced-glycation-endproducts-is-a-mediator-of-toxicity-by-iapp-and-other-proteotoxic-aggregates-establishing-and-exploiting-common-ground-for-novel-amyloidosis-therapies
#2
REVIEW
Andisheh Abedini, Julia Derk, Ann Marie Schmidt
Proteotoxicity plays a key role in many devastating human disorders, including Alzheimer's, Huntington's and Parkinson's diseases; type 2 diabetes; systemic amyloidosis; and cardiac dysfunction, to name a few. The cellular mechanisms of proteotoxicity in these disorders have been the focus of considerable research, but their role in prevalent and morbid disorders, such as diabetes, is less appreciated. There is a large body of literature on the impact of glucotoxicity and lipotoxicity on insulin-producing pancreatic β-cells, and there is increasing recognition that proteotoxicty plays a key role...
April 17, 2018: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/29663861/amyloid-cardiomyopathy-a-hidden-heart-failure-cause-that-is-often-misdiagnosed
#3
Yale Tung-Chen, Miguel-Ángel Arnau
BACKGROUND: Congestive heart failure (CHF) is a very common condition, especially in the elderly, characterized by dyspnea, orthopnea, nocturnal paroxysmal dyspnea, and peripheral edema. CLINICAL PRESENTATION AND INTERVENTION: We report the case of a 76-year-old with CHF symptoms for the last 3 months. The ECG and transthoracic echocardiogram were suggestive of CHF due to amyloid cardiomyopathy. After cardiac MRI, a positive Congo red staining of subcutaneous fat aspiration and a negative genetic testing for mutant transthyretin, senile amyloid cardiomyopathy (ATTRw) was expected...
April 17, 2018: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29658384/complications-of-adult-onset-still-s-disease-and-their-management
#4
Stéphane Mitrovic, Bruno Fautrel
Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder in which management and treatment have considerably progressed over the past decade. Despite wide use of interleukin (IL)-1 or IL-6 inhibitors, serious complications remain possible. Areas covered: A comprehensive literature search in MEDLINE via Pubmed was performed to review AOSD's severe and sometimes life-threatening complications: reactive hemophagocytic lymphohystiocytosis, coagulation disorders, fulminant hepatitis, cardiac or pulmonary complications and amyloid A amyloidosis...
April 16, 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29657310/cardiac-amyloidosis-myocardial-biopsy-as-a-tool-in-chemotherapy-implementation-and-scd-prevention
#5
Małgorzata Cichoń, Katarzyna Mizia-Stec, Romuald Wojnicz, Piotr Kukla, Magdalena Drożdż
No abstract text is available yet for this article.
April 15, 2018: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29651545/extracardiac-18-f-florbetapir-imaging-in-patients-with-systemic-amyloidosis-more-than-hearts-and-minds
#6
T Wagner, J Page, M Burniston, A Skillen, J C Ross, R Manwani, D McCool, P N Hawkins, Ashutosh D Wechalekar
PURPOSE: 18 F-Florbetapir has been reported to show cardiac uptake in patients with systemic light-chain amyloidosis (AL). This study systematically assessed uptake of 18 F-florbetapir in patients with proven systemic amyloidosis at sites outside the heart. METHODS: Seventeen patients with proven cardiac amyloidosis underwent 18 F-florbetapir PET/CT imaging, 15 with AL and 2 with transthyretin amyloidosis (ATTR). Three patients had repeat scans. All patients had protocolized assessment at the UK National Amyloidosis Centre including imaging with 123 I-serum amyloid P component (SAP)...
April 12, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29627865/advances-in-treatment-of-cardiac-amyloid
#7
REVIEW
Cherie N Dahm, R Frank Cornell, Daniel J Lenihan
Systemic amyloidosis represents a complex group of diseases with a common feature characterized by misfolded autologous proteins depositing into tissues or organs throughout the body. Light chain amyloidosis (AL) and transthyretin (TTR) amyloid are the two most prevalent forms of this disease that commonly results in cardiac amyloidosis. In both of these conditions, the myocardium is a frequent site of infiltration and end-organ involvement often with devastating consequences. With cardiac amyloidosis becoming an increasingly identified disease that has previously been under-recognized, the purpose of this comprehensive review is to focus on the diagnosis and treatment of these two types of cardiac amyloidosis including a contemporary update on currently available therapies being investigated in clinical trials...
April 7, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29622862/the-effect-and-safety-of-bortezomib-in-the-treatment-of-al-amyloidosis-a-systematic-review-and-meta-analysis
#8
Fengjuan Jiang, Jin Chen, Hui Liu, Lijuan Li, Wenli Lu, Rong Fu
Bortezomib began to be used in the treatment of light chain (AL) amyloidosis in recent years. We performed the first meta-analysis of randomized clinical trials and clinical controlled trials to evaluate the effect and safety of bortezomib treatment for AL amyloidosis. We conducted a search (until July 2016) in electronic databases (PubMed databases and the Cochrane Central Register of Controlled Trials bases from the year 2003). There were 205 records we searched and eight studies was included (n = 617 persons)...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29615436/ttr-transthyretin-stabilizers-are-associated-with-improved-survival-in-patients-with-ttr-cardiac-amyloidosis
#9
Hannah Rosenblum, Adam Castano, Julissa Alvarez, Jeff Goldsmith, Stephen Helmke, Mathew S Maurer
BACKGROUND: TTR (transthyretin) cardiac amyloidosis is caused by dissociation of TTR into monomers, which misassemble into amyloid fibrils. TTR stabilizers act at the dimer-dimer interface to prevent dissociation. We investigated differences in survival among patients with TTR cardiac amyloidosis on stabilizer medications compared with those not on stabilizers. METHODS AND RESULTS: A retrospective study of patients with TTR cardiac amyloidosis presenting to a single center was conducted...
April 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29589647/a-case-of-primary-systemic-amyloidosis
#10
Anja Trajber Horvat, Katarina Trčko, Vesna Jurčić, Pij Bogomir Marko
Primary systemic amyloidosis is characterized by the deposition of insoluble monoclonal immunoglobulin light chains in various tissues and is usually associated with an underlying plasma cell dyscrasia. In the early stage of the disease, dermatological findings can be the only manifestation, as opposed to organ involvement in the later stages. A dermatologist can diagnose amyloidosis early with a skin biopsy stained with Congo red dye and other appropriate investigations. This case report describes a female patient with primary systemic amyloidosis confirmed histologically from a skin biopsy...
March 2018: Acta Dermatovenerologica Alpina, Panonica, et Adriatica
https://www.readbyqxmd.com/read/29589208/significance-of-11-c-pib-pet-ct-in-cardiac-amyloidosis-compared-with-99m-tc-aprotinin-scintigraphy-a-pilot-study
#11
Ryogo Minamimoto, Toru Awaya, Kentaro Iwama, Masatoshi Hotta, Kazuhiko Nakajima, Risen Hirai, Osamu Okazaki, Yukio Hiroi
BACKGROUND: This study was to investigate the significance of11 C-Pittsburgh B (PIB) PET/CT in patients with suspected cardiac amyloidosis compared with99m Tc-aprotinin scintigraphy. METHODS: Thirteen consecutive patients with suspected cardiac amyloidosis were considered for enrolment in this prospective pilot study. Participants were scheduled to undergo a series of11 C-PIB PET/CT and99m Tc-aprotinin within a 2-month period. Finally, we evaluated nine cases who underwent both imaging modalities, and compared imaging results with clinical and pathological results and prognosis...
March 27, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/29583159/-heart-transplantation-in-al-amyloidosis
#12
Pia Dahlberg, Sven-Erik Bartfay, Kristjan Karason, Ulf-Henrik Mellqvist, Entela Bollano
Amyloidosis is a disease complex characterized by the deposition of protein fibrils in various tissues, which leads to structural and functional derangement of the affected organ. There are different types of amyloidosis categorized on the basis of the type of protein fibrils deposited. Cardiac involvement has been predominantly noted in amyloid light chain (AL) amyloidosis and is the major prognostic determinant and influences the therapeutic strategy.  In AL amyloidosis, heart transplantation is generally not recommended because of a high risk of recurrence in the transplanted heart and poor survival rate...
March 26, 2018: Läkartidningen
https://www.readbyqxmd.com/read/29581546/depth-of-organ-response-in-al-amyloidosis-is-associated-with-improved-survival-grading-the-organ-response-criteria
#13
Eli Muchtar, Angela Dispenzieri, Nelson Leung, Martha Q Lacy, Francis K Buadi, David Dingli, Martha Grogan, Suzanne R Hayman, Prashant Kapoor, Yi Lisa Hwa, Amie Fonder, Miriam Hobbs, Rajshekhar Chakraborty, Wilson Gonsalves, Taxiarchis V Kourelis, Rahma Warsame, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Shaji K Kumar, Morie A Gertz
Organ recovery following anti-plasma cell therapy is associated with improved outcome in patients with AL amyloidosis. Current organ response criteria do not consider the depth of response. Newly diagnosed AL amyloidosis patients who had involvement of at least one evaluable organ and documentation of organ response were included. Cardiac response was seen in 182 patients. Time to cardiac response was 9.4 months. Renal response was seen in 278 patients, at a median time to response of 6 months. Hepatic response was seen in 70 patients, with a median time to response of 6...
February 20, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29574587/can-nuclear-imaging-techniques-predict-patient-outcome-and-guide-medical-management-in-hereditary-transthyretin-cardiac-amyloidosis
#14
REVIEW
Vincent Algalarrondo, Eve Piekarski, Ludivine Eliahou, Dominique Le Guludec, Michel S Slama, François Rouzet
PURPOSE OF REVIEW: Nuclear imaging recently gained a key role in the diagnosis and prognostic assessment of transthyretin (TTR)-related cardiac amyloidosis. This review aims at summarizing the state-of-the art regarding the implementation of nuclear imaging in the management of hereditary mutated TTR-cardiac amyloidosis (mTTR-CA). RECENT FINDINGS: Although cardiac uptake of bone tracers is acknowledged as a specific marker of TTR amyloid cardiac burden, recent studies validated the implementation of bone scan in the flow chart for non-invasive diagnosis and follow-up of CA in multicenter trials...
March 24, 2018: Current Cardiology Reports
https://www.readbyqxmd.com/read/29559999/recurrent-syncope-a-clue-in-amyloid-cardiomyopathy
#15
Julian A Marin-Acevedo, Catalina Sanchez-Alvarez, Ali A Alsaad, Ricardo J Pagán
Infiltrative cardiomyopathies include a variety of disorders that lead to myocardial thickening resulting in a constellation of clinical manifestations and eventually heart failure that could be the first clue to reach the diagnosis. Among the more described infiltrative diseases of the heart is amyloid cardiomyopathy. The disease usually presents with subtle, nonspecific symptoms. Herein, we illustrate a case of recurrent syncope as the initial presenting symptom for systemic amyloid with polyneuropathy and cardiomyopathy as a cause of syncope...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29550324/native-t1-and-extracellular-volume-in-transthyretin-amyloidosis
#16
Ana Martinez-Naharro, Tushar Kotecha, Karl Norrington, Michele Boldrini, Tamer Rezk, Candida Quarta, Thomas A Treibel, Carol J Whelan, Daniel S Knight, Peter Kellman, Frederick L Ruberg, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
OBJECTIVES: This study evaluated the prognostic potential of native myocardial T1 in cardiac transthyretin amyloidosis (ATTR) and compared native T1 with extracellular volume (ECV) in terms of diagnostic accuracy and prognosis. BACKGROUND: ATTR is an increasingly recognized cause of heart failure that has an overlapping clinical phenotype with hypertrophic cardiomyopathy (HCM). Native T1 mapping by cardiac magnetic resonance (CMR) is useful for diagnosis in cardiac amyloidosis but its prognostic potential has never been assessed...
March 12, 2018: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29531659/light-chain-amyloidosis
#17
REVIEW
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
https://www.readbyqxmd.com/read/29529882/response-atrial-impairment-in-transthyretin-cardiac-amyloidosis-an-early-marker-of-cardiac-involvement-and-a-prognostic-factor
#18
Michal Y Henein, Per Lindqvist
No abstract text is available yet for this article.
March 12, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29523040/atrial-impairment-in-transthyretin-cardiac-amyloidosis-an-early-marker-of-cardiac-involvement-and-a-prognostic-factor
#19
Gianluca Di Bella, Fabio Minutoli
No abstract text is available yet for this article.
March 9, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29520480/clinical-phenotyping-of-transthyretin-cardiac-amyloidosis-with-bone-seeking-radiotracers-in-heart-failure-with-preserved-ejection-fraction
#20
REVIEW
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
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