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Cardiac amyloidosis

Sabha Bhatti, Srikanth Vallurupalli, Stephanie Ambach, Adam Magier, Evan Watts, Vien Truong, Abdul Hakeem, Wojciech Mazur
Cardiac MRI is frequently used in the diagnosis of cardiac amyloidosis. Feature tracking is a novel method of analyzing myocardial strain at the myocardial borders. We investigated myocardial deformation mechanics of both the right and left ventricles in patients with multiple myeloma with suspected cardiac amyloidosis. Comprehensive strain analysis was performed in 43 patients with multiple myeloma and suspected cardiac amyloidosis. MRI strain by feature tracking was measured using 2D cardiac performance analysis MR software (Tomtec, Germany)...
October 14, 2016: International Journal of Cardiovascular Imaging
Cyril Garrouste, Dany Anglicheau, Nassim Kamar, Claire Bachelier, Joseph Rivalan, Bruno Pereira, Sophie Caillard, Julien Aniort, Philippe Gatault, Martin Soubrier, Johnny Sayegh, Charlotte Colosio, Anthony Buisson, Eric Thervet, Nicolas Bouvier, Anne Elisabeth Heng
Anti-tumor necrosis factor-α (TNFα) therapy has improved the prognosis of many chronic inflammatory diseases. It appears to be well-tolerated by liver-transplant patients. However, their use and their safety in kidney-transplant patients have yet to be determined.In this retrospective study, we identified 16 adult kidney-transplant patients aged 46.5 years (34-51.8) who received anti-TNFα therapy from 7 kidney transplantation centers. The indications for this treatment included: chronic inflammatory bowel disease (n = 8), inflammatory arthritis (n = 5), AA amyloidosis (n = 1), psoriasis (n = 1), and microscopic polyangiitis (n = 1)...
October 2016: Medicine (Baltimore)
Ulrich Tebbe, Karin Bramlage, Fiete John, Dirk Härtel, Ralf Felgendreher, Kathrin Machalke, Reinhard Kandolf, Peter Bramlage
BACKGROUND: In patients with cardiomyopathy of unknown origin, endomyocardial biopsy provides the possibility of improved diagnosis and tailored treatment. Specific guidance has been developed based on cardiovascular centre of excellence experience but it is unknown if the benefits also extend into the tertiary care hospital setting. METHODS: Endomyocardial biopsies was performed in patients with cardiomyopathy of unknown origin. The outcomes were mirrored against the current ESC recommendations...
October 10, 2016: BMC Research Notes
Eli Muchtar, Daniele Derudas, Michelle Mauermann, Teerin Liewluck, Angela Dispenzieri, Shaji K Kumar, David Dingli, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Nelson Leung, Rajshekhar Chakraborty, Wilson Gonsalves, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz
OBJECTIVE: To characterize the natural history of immunoglobulin light chain amyloidosis-associated myopathy and to provide guidelines for recognition. PATIENTS AND METHODS: Fifty-one patients with systemic immunoglobulin light chain amyloidosis and biopsy-confirmed muscle amyloid deposition diagnosed between January 1, 1995, and December 31, 2015, were included in this study. RESULTS: Common presenting symptoms were muscle weakness in 49 patients (96%), dysphagia in 23 (45%), myalgia in 17 (33%), macroglossia in 17 (33%), jaw claudication in 13 (25%), and hoarseness in 9 (18%)...
October 2016: Mayo Clinic Proceedings
Angela Dispenzieri, Giampaolo Merlini
Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy...
2016: Cancer Treatment and Research
S Rosengren, U-H Mellqvist, H Nahi, K Forsberg, S Lenhoff, O Strömberg, L Ahlberg, O Linder, K Carlson
High-dose melphalan and autologous stem cell transplantation (HDM/ASCT) is widely used in immunoglobulin light chain (AL) amyloidosis, but the benefit is debated mainly because of the high treatment-related mortality (24% in a randomised study comparing HDM/ASCT with oral melphalan/dexamethasone). We report here on the long-term outcome of all patients treated with HDM/ASCT for AL amyloidosis in Sweden between 1994 and 2009. Seventy-two patients were treated at eight Swedish centres. Median follow-up was 67...
October 3, 2016: Bone Marrow Transplantation
F Legou, V Tacher, T Damy, V Planté-Bordeneuve, S Rappeneau, N Benhaiem, J Rosso, E Itti, A Luciani, H Kobeiter, A Rahmouni, J-F Deux
PURPOSE: To detect if a difference of T2 ratio, defined as the signal intensity (SI) of the myocardium divided by the SI of the skeletal muscle on T2-weigthed cardiac magnetic resonance (CMR) imaging, exists between patients with systemic amyloidosis, by comparison to control subjects. To determine if a relationship exists between T2 ratio and the overall mortality. MATERIALS AND METHODS: CMR imaging examinations of 73 consecutive patients (48 men, 25 women; mean age, 63 years±15[SD]) with amyloidosis and suspicion of CA and 27 control subjects were retrospectively analyzed after institutional review board approval...
September 28, 2016: Diagnostic and Interventional Imaging
David J Hur, Demetrius L Dicks, Steffen Huber, Hamid R Mojibian, Judith L Meadows, Stuart E Seropian, Lauren A Baldassarre
No abstract text is available yet for this article.
October 2016: Circulation. Cardiovascular Imaging
Francesca Lavatelli, Giampaolo Merlini
INTRODUCTION: More than ten distinct forms of amyloidoses that can involve the heart have been described, classified according to which protein originates the deposits. Cardiac amyloid infiltration translates into progressive and often life-threatening cardiomyopathy, but disease severity, prognosis and treatment drastically differ according to the amyloidosis type. The notion that protein misfolding and aggregation play a more general role in human cardiomyopathies has further raised attention towards the definition of the proteotoxicity mechanisms...
September 28, 2016: Expert Review of Proteomics
Stefania Rosmini, Thomas A Treibel, Steve Bandula, Tyler Stroud, Marianna Fontana, Philip N Hawkins, James C Moon
No abstract text is available yet for this article.
September 15, 2016: Journal of Cardiovascular Computed Tomography
Daniel R Jacobson, Alice A Alexander, Clement Tagoe, W T Garvey, Scott M Williams, Sara Tishkoff, David Modiano, Sodiomon B Sirima, Issa Kalidi, Amadou Toure, Joel N Buxbaum
BACKGROUND: Transthyretin (TTR) pV142I (rs76992529-A) is one of the 113 variants in the human TTR gene associated with systemic amyloidosis. It results from a G to A transition at a CG dinucleotide in the codon for amino acid 122 of the mature protein (TTR V122I). The allele frequency is 0.0173 in African Americans. METHODS: PCR-based assays to genotype 2767 DNA samples obtained from participants in genetic studies from various African populations supplemented with sequencing data from 529 samples within the 1000 Genomes Project...
September 2016: Molecular Genetics & Genomic Medicine
Sabahat Bokhari, Rachelle Morgenstern, Richard Weinberg, Mona Kinkhabwala, Demetrios Panagiotou, Adam Castano, Albert DeLuca, Andrew Kontak, Zhezhen Jin, Mathew S Maurer
No abstract text is available yet for this article.
September 20, 2016: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Thibaud Damy, Arnaud Jaccard, Aziz Guellich, David Lavergne, Arnault Galat, Jean-François Deux, Luc Hittinger, Jehan Dupuis, Valérie Frenkel, Charlotte Rigaud, Violaine Plante-Bordeneuve, Diane Bodez, Dania Mohty
BACKGROUND: The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. AIMS: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers. METHODS AND RESULTS: Observational study...
September 20, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
T Jelinek, E Kryukova, Z Kufova, F Kryukov, R Hajek
Proteasome inhibitors are the backbone in the treatment of multiple myeloma with 3 of its representatives (bortezomib, carfilzomib, and ixazomib) having already been approved. There is a different situation altogether in the treatment of amyloid light chain (AL) amyloidosis where owing to the rarity of this entity neither of these drugs has currently gained approval. Amyloid light chain plasma cells are possibly more vulnerable to bortezomib than myeloma plasmocytes because of a slightly distinct mechanism of action, which is described in depth in this manuscript...
September 20, 2016: Hematological Oncology
Björn Pilebro, Sandra Arvidsson, Per Lindqvist, Torbjörn Sundström, Per Westermark, Gunnar Antoni, Ole Suhr, Jens Sörensen
BACKGROUND: DPD scintigraphy has been advocated for imaging cardiac amyloid in ATTR amyloidosis. PET utilizing (11)C-Pittsburgh compound B (PIB) is the gold standard for imaging brain amyloid in Alzheimer's disease. PIB was recently shown to identify cardiac amyloidosis in both AL and ATTR amyloidosis. In the ATTR population, two types of amyloid fibrils exist, one containing fragmented and full-length TTR (type A) and the other only full-length TTR (type B). The aim of this study was to further evaluate PIB-PET in patients with hereditary ATTR amyloidosis...
September 19, 2016: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Sergio Barros-Gomes, Brittney Williams, Lara F Nhola, Martha Grogan, Joseph F Maalouf, Angela Dispenzieri, Patricia A Pellikka, Hector R Villarraga
OBJECTIVES: This study evaluated whether 2-dimensional speckle-tracking echocardiography (2D-STE) has incremental value for prognosis over traditional clinical, echocardiographic, and serological markers-with main focus on the current prognostic staging system-in light-chain (AL) amyloidosis patients with preserved left ventricular ejection fraction. BACKGROUND: Cardiac amyloidosis (CA) is the major determinant of outcome in AL amyloidosis. The current prognostic staging system is based primarily on serum levels of cardiac troponin T (cTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain differential (FLC-diff)...
September 8, 2016: JACC. Cardiovascular Imaging
Pradeep Bhambhvani, Fadi G Hage
No abstract text is available yet for this article.
September 8, 2016: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Rodney H Falk, Kevin M Alexander, Ronglih Liao, Sharmila Dorbala
The amyloidoses are a group of protein-folding disorders in which ≥1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease. The last decade has seen considerable progress in understanding the amyloidoses...
September 20, 2016: Journal of the American College of Cardiology
Wakako Tsuji, Eiji Takeuchi, Satoshi Oka, Taro Yamashita, Fumiaki Yotsumoto
BACKGROUND: Primary amyloidosis of the breast is an unusual benign disease that mostly occurs in postmenopausal elderly women. Amyloidosis is the deposition of amorphous protein within tissues. Breast biopsy is necessary to make a definite diagnosis in order to avoid unnecessary surgical methods. Localized primary amyloidosis of the breast has a good prognosis. However, secondary amyloidosis is a systemic disease and has a poor prognosis. CASE PRESENTATION: We report the case of a 77-year-old female with primary amyloidosis of the breast...
2016: BMC Surgery
Kevin M Alexander, Rodney H Falk
No abstract text is available yet for this article.
September 2016: Circulation. Heart Failure
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