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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/29449366/use-of-serum-transthyretin-as-a-prognostic-indicator-and-predictor-of-outcome-in-cardiac-amyloid-disease-associated-with-wild-type-transthyretin
#1
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
https://www.readbyqxmd.com/read/29448998/cardiac-amyloidosis-from-clinical-suspicion-to-morphological-diagnosis
#2
REVIEW
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
https://www.readbyqxmd.com/read/29429161/-classification-of-cardiac-amyloidosis-an-immunohistochemical-analysis
#3
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
https://www.readbyqxmd.com/read/29407121/lung-uptake-during-99mtc-hydroxymethylene-diphosphonate-scintigraphy-in-patient-with-ttr-cardiac-amyloidosis-an-underestimated-phenomenon
#4
Francesco Cappelli, Chiara Gallini, Egidio Natalino Costanzo, Francesca Tutino, Alfonso Ciaccio, Luca Vaggelli, Simone Bartolini, Sofia Morini, Raffaele Martone, Paola Angelotti, Sabrina Frusconi, Carlo Di Mario, Federico Perfetto
BACKGROUND: Full body scintigraphy using bone tracers plays an important role in defining the type of amyloidosis and in diagnosing the heart involvement (cardiac amyloidosis, CA). No study has been conducted to explore lung retention (LR) in CA and its correlation to heart retention (HR).We evaluated LR in patients undergoing 99mTc-HMDP scintigraphy during evaluation for suspected CA. METHODS AND RESULTS: We enrolled 93 suspected CA patients. Patients underwent a complete diagnostic work up...
March 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29405655/a-case-of-cardiac-sarcoidosis-mimicking-cardiac-amyloidosis-on-cardiovascular-magnetic-resonance
#5
Kazunori Takemura, Ryuta Nakamura, Kazuhito Shimazu, Youichiro Sugimoto, Tetsuro Takase, Minamimoto Ryogo, Michiaki Hiroe
A 52-year-old male visited our hospital with abnormal electrocardiogram and exertional fatigue. The electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block, and inverted T waves in Leads II, III, aVF, V3, and V4. Echocardiography showed biventricular wall thickening involving granular sparkling of the interventricular septum. Late gadolinium enhancement on cardiovascular magnetic resonance (CMR) was found at the circumferential right ventricular wall and patchy regions of the left ventricle...
February 5, 2018: ESC Heart Failure
https://www.readbyqxmd.com/read/29401357/diagnosis-pathogenesis-and-outcome-in-leucocyte-chemotactic-factor-2-alect2-amyloidosis
#6
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
https://www.readbyqxmd.com/read/29394116/normal-scores-of-deep-breathing-tests-beware-of-dysrhythmia-in-transthyretin-amyloidosis
#7
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
https://www.readbyqxmd.com/read/29386197/growth-differentiation-factor-15-is-a-new-biomarker-for-survival-and-renal-outcomes-in-light-chain-al-amyloidosis
#8
Efstathios Kastritis, Ioannis Papassotiriou, Giampaolo Merlini, Paolo Milani, Evangelos Terpos, Marco Basset, Athanasios Akalestos, Francesca Russo, Erasmia Psimenou, Filia Apostolakou, Maria Roussou, Maria Gavriatopoulou, Evangelos Eleutherakis Papaiakovou, Despina Fotiou, Dimitrios C Ziogas, Elektra Papadopoulou, Constantinos Pamboucas, Meletios A Dimopoulos, Giovanni Palladini
Growth differentiation factor-15 (GDF-15) improves prognostication in patients with cardiovascular disorders in addition to conventional cardiac markers (NT-proBNP, troponins) and has shown prognostic value in patients with renal diseases. In patients with AL amyloidosis cardiac involvement is the major determinant of prognosis and cardiac markers define prognosis, while biomarkers of renal involvement stratify renal risk. We explored the prognostic importance of serum levels of GDF-15 in patients with AL amyloidosis, in two independent cohorts...
January 31, 2018: Blood
https://www.readbyqxmd.com/read/29369708/reduced-left-atrial-myocardial-deformation-irrespective-of-cavity-size-a-potential-cause-for-atrial-arrhythmia-in-hereditary-transthyretin-amyloidosis
#9
Michael Y Henein, Ole B Suhr, Sandra Arvidsson, Björn Pilebro, Per Westermark, Rolf Hörnsten, Per Lindqvist
BACKGROUND: Cardiac amyloidosis (CA) is a myocardial disease and commonly under-diagnosed condition. In CA patients, atrial fibrillation might occur in the absence of left atrial (LA) enlargement. OBJECTIVES: The aim of this study is to assess LA size and function, and its relationship with atrial arrhythmia in patients with hereditary transthyretin amyloidosis (ATTR). METHODS: Forty-six patients with confirmed ATTR amyloidosis on abdominal biopsy were studied...
January 25, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29351628/-a-new-staging-system-for-cardiac-transthyretin-amyloidosis-is-it-already-on-the-verge-of-obsolescence
#10
Avinainder Singh, Rodney H Falk
No abstract text is available yet for this article.
January 16, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29344917/non-cardiac-uptake-of-technetium-99m-pyrophosphate-in-transthyretin-cardiac-amyloidosis
#11
Brett W Sperry, Matthew H Gonzalez, Richard Brunken, Manuel D Cerqueira, Mazen Hanna, Wael A Jaber
BACKGROUND: Technetium-based bone scintigraphy is rapidly becoming the most common non-invasive imaging tool in the diagnosis of Transthyretin cardiac amyloidosis (ATTR). Skeletal muscle uptake has been described with technetium-99m-3,3-diphosphono-1,2-propanodicarboxylic acid (TcDPD), and may account for masking of bony uptake. We sought to investigate skeletal muscle uptake of technetium-99m-pyrophosphate (TcPYP) in patients with ATTR. METHODS AND RESULTS: This was a retrospective analysis of 57 patients diagnosed with ATTR who underwent TcPYP scintigraphy...
January 17, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/29343286/diagnosis-and-management-of-transthyretin-familial-amyloid-polyneuropathy-in-japan-red-flag-symptom-clusters-and-treatment-algorithm
#12
REVIEW
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
https://www.readbyqxmd.com/read/29321803/the-eyes-are-the-window-to-the-heart-one-case-of-cardiac-amyloidosis-with-eyelid-swelling-as-the-initial-symptom
#13
Ting-Zhi Deng, Bai-Qing Ou, Dao-Quan Peng
No abstract text is available yet for this article.
November 2017: Journal of Geriatric Cardiology: JGC
https://www.readbyqxmd.com/read/29308670/late-iodine-enhancement-and-myocardial-extracellular-volume-quantification-in-cardiac-amyloidosis-by-using-dual-energy-cardiac-computed-tomography-performed-on-a-dual-layer-spectral-detector-scanner
#14
Seitaro Oda, Takeshi Nakaura, Daisuke Utsunomiya, Kyoko Hirakawa, Seiji Takashio, Yasuhiro Izumiya, Kenichi Tsujita, Hiroyuki Hata, Yukio Ando, Yasuyuki Yamashita
No abstract text is available yet for this article.
January 8, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29305971/more-than-meets-the-eye-time-for-a-new-imaging-paradigm-to-test-for-cardiac-amyloidosis
#15
EDITORIAL
Mathew S Maurer, Frederick L Ruberg, Jonathan W Weinsaft
No abstract text is available yet for this article.
January 3, 2018: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/29305414/rapid-hematological-responses-improve-outcomes-in-patients-with-very-advanced-stage-iiib-cardiac-immunoglobulin-light-chain-amyloidosis
#16
Richa Manwani, Darren Foard, Shameem Mahmood, Sajitha Sachchithanantham, Thirusha Lane, Cristina Quarta, Taryn Youngstein, Tamer Rezk, Helen J Lachmann, Julian D Gillmore, Marianna Fontana, Carol Whelan, Philip N Hawkins, Ashutosh D Wechalekar
No abstract text is available yet for this article.
January 5, 2018: Haematologica
https://www.readbyqxmd.com/read/29303069/unfolding-cardiac-amyloidosis-from-pathophysiology-to-cure
#17
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
https://www.readbyqxmd.com/read/29298867/repeat-doses-of-antibody-to-serum-amyloid-p-component-clear-amyloid-deposits-in-patients-with-systemic-amyloidosis
#18
Duncan B Richards, Louise M Cookson, Sharon V Barton, Lia Liefaard, Thirusha Lane, David F Hutt, James M Ritter, Marianna Fontana, James C Moon, Julian D Gillmore, Ashutosh Wechalekar, Philip N Hawkins, Mark B Pepys
Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This residual SAP is a specific target for dezamizumab, a fully humanized monoclonal IgG1 anti-SAP antibody that triggers immunotherapeutic clearance of amyloid...
January 3, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29298704/the-prognostic-value-of-t1-mapping-and-late-gadolinium-enhancement-cardiovascular-magnetic-resonance-imaging-in-patients-with-light-chain-amyloidosis
#19
Lu Lin, Xiao Li, Jun Feng, Kai-Ni Shen, Zhuang Tian, Jian Sun, Yue-Ying Mao, Jian Cao, Zheng-Yu Jin, Jian Li, Joseph B Selvanayagam, Yi-Ning Wang
BACKGROUND: Cardiac impairment is associated with high morbidity and mortality in immunoglobulin light chain (AL) type amyloidosis, for which early identification and risk stratification is vital. For myocardial tissue characterization, late gadolinium enhancement (LGE) is a classic and most commonly performed cardiovascular magnetic resonance (CMR) parameter. T1 mapping with native T1 and extracellular volume (ECV) are recently developed quantitative parameters. We aimed to investigate the prognostic value of native T1, ECV and LGE in patients with AL amyloidosis...
January 3, 2018: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29288196/hydroxychloroquine-mediated-cardiotoxicity-with-a-false-positive-99mtechnetium-labeled-pyrophosphate-scan-for-transthyretin-related-cardiac-amyloidosis
#20
Ian C Y Chang, John P Bois, Melanie C Bois, Joseph J Maleszewski, Geoffrey B Johnson, Martha Grogan
No abstract text is available yet for this article.
January 2018: Circulation. Cardiovascular Imaging
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