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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/29048471/a-new-staging-system-for-cardiac-transthyretin-amyloidosis
#1
Julian D Gillmore, Thibaud Damy, Marianna Fontana, Matthew Hutchinson, Helen J Lachmann, Ana Martinez-Naharro, Candida C Quarta, Tamer Rezk, Carol J Whelan, Esther Gonzalez-Lopez, Thirusha Lane, Janet A Gilbertson, Dorota Rowczenio, Aviva Petrie, Philip N Hawkins
Aims: Cardiac transthyretin (ATTR) amyloidosis is an increasingly recognized, progressive, and fatal cardiomyopathy, the natural history of which remains unclear. We sought to establish and validate a new prognostic staging system applicable to patients with both wild-type ATTR (ATTRwt) and hereditary variant ATTR (ATTRv) amyloid cardiomyopathy. Methods and results: Eight hundred and sixty-nine patients with cardiac ATTR amyloidosis (553 with ATTRwt and 316 with ATTRv) attending the UK National Amyloidosis Centre were stratified into three disease stages at baseline on the basis of cut points in two universally measured biomarkers, N-terminal pro-B-type natriuretic peptide (NT-proBNP) and estimated glomerular filtration rate (eGFR)...
October 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/29046749/an-unusual-case-of-chronic-lymphocytic-leukemia-multiple-myeloma-and-cardiac-amyloidosis
#2
Dongyan Liu, Hakim T Uqdah, Alisha D Gordy
Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted multiple myeloma with plasma cells causing AL amyloidosis involving the heart. While monoclonal immunoglobulins occasionallyproduced by CLL have previously been implicated in AL amyloidosis, there only a few cases reported of AL amyloidosis resulting from a distinct plasma cell dyscrasia that is not clonally related to the concurrent CLL...
October 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29043240/sequential-heart-and-autologous-stem-cell-transplantation-for-light-chain-cardiac-amyloidosis
#3
Jin-Young Huh, Seyoung Seo, Cheolwon Suh, Jooryung Huh, Dok Hyun Yoon, Jae-Joong Kim
No abstract text is available yet for this article.
September 2017: Blood Research
https://www.readbyqxmd.com/read/29037943/mesenchymal-stromal-cells-protect-human-cardiomyocytes-from-amyloid-fibril-damage
#4
Yi Lin, Marta Marin-Argany, Christopher J Dick, Keely R Redhage, Luis M Blancas-Mejia, Peggy Bulur, Greg W Butler, Michael C Deeds, Benjamin J Madden, Angela Williams, Jonathan S Wall, Allan Dietz, Marina Ramirez-Alvarado
BACKGROUND AIMS: Light chain (AL) amyloidosis is a protein misfolding disease characterized by extracellular deposition of immunoglobulin light chains (LC) as amyloid fibrils. Patients with LC amyloid involvement of the heart have the worst morbidity and mortality. Current treatments target the plasma cells to reduce further production of amyloid proteins. There is dire need to understand the mechanisms of cardiac tissue damage from amyloid to develop novel therapies. We recently reported that LC soluble and fibrillar species cause apoptosis and inhibit cell growth in human cardiomyocytes...
October 13, 2017: Cytotherapy
https://www.readbyqxmd.com/read/29024324/clinical-implications-of-gastrointestinal-symptoms-in-systemic-amyloidosis
#5
T Yen, F W Chen, R M Witteles, M Liedtke, L A Nguyen
BACKGROUND: Gastrointestinal (GI) symptoms in systemic amyloidosis patients are poorly characterized. This purpose of this study is to define the epidemiology and clinical implications of such symptoms. METHODS: This was a retrospective cohort study of 583 amyloid patients seen at a tertiary referral center. Of 96 symptomatic patients, 82 received endoscopic biopsies, subsequently grouped into those with histologic evidence of GI amyloid (biopsy proven) vs without (biopsy absent)...
October 10, 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/29021100/the-value-of-endomyocardial-biopsy-in-diagnosis-and-guiding-therapy
#6
Tayyaba Khan, Dinesh Selvakumar, Siddharth Trivedi, Karan Rao, Mehmet Harapoz, Aravinda Thiagalingam, A Robert Denniss, Winny Varikatt
Endomyocardial biopsy (EMB) is a highly-specialised procedure that is associated with some controversy as to its diagnostic role due to its inconsistency in diagnosing a wide variety of cardiac diseases. Given the advances and sophistication in echocardiography and cardiac magnetic resonance imaging (MRI), the vast majority of cardiac diseases can be diagnosed by these non-invasive procedures. Under-sampling and the fact that biopsy site is limited to the right side of the interventricular septum further limits its value...
October 8, 2017: Pathology
https://www.readbyqxmd.com/read/29019809/atrial-fibrillation-and-central-nervous-complications-in-liver-transplanted-hereditary-transthyretin-amyloidosis-patients
#7
Niklas Wange, Intissar Anan, Bo-Göran Ericzon, Johanna Pennlert, Björn Pilebro, Ole B Suhr, Jonas Wixner
BACKGROUND: Central nervous system (CNS) complications are increasingly noted in liver transplanted (LTx) hereditary transthyretin amyloid (ATTRm) amyloidosis patients; this suggests that the increased survival allows for intracranial ATTRm formation from brain synthesised mutant TTR. However, atrial fibrillation (AF), a recognised risk factor for ischemic CNS complications, is also observed after LTx. The aim of the study was to investigate the occurrence of CNS complications and AF in LTx ATTRm amyloidosis patients...
October 11, 2017: Transplantation
https://www.readbyqxmd.com/read/29019616/dangerous-relationships-aortic-stenosis-and-transthyretin-cardiac-amyloidosis
#8
Paola Gargiulo, Pasquale Perrone-Filardi
No abstract text is available yet for this article.
October 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/29019612/unveiling-transthyretin-cardiac-amyloidosis-and-its-predictors-among-elderly-patients-with-severe-aortic-stenosis-undergoing-transcatheter-aortic-valve-replacement
#9
Adam Castaño, David L Narotsky, Nadira Hamid, Omar K Khalique, Rachelle Morgenstern, Albert DeLuca, Jonah Rubin, Codruta Chiuzan, Tamim Nazif, Torsten Vahl, Isaac George, Susheel Kodali, Martin B Leon, Rebecca Hahn, Sabahat Bokhari, Mathew S Maurer
Aims: Transthyretin cardiac amyloidosis (ATTR-CA) has been reported in patients with aortic stenosis (AS) but its prevalence and phenotype are not known. We examine elderly patients with severe symptomatic AS undergoing transcatheter aortic valve replacement (TAVR) and determine the prevalence and phenotype of ATTR-CA non-invasively. Methods and results: We performed technetium-99m pyrophosphate (99mTc-PYP) cardiac scintigraphy prospectively on patients who underwent TAVR, to screen for ATTR-CA...
October 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/29016528/transthyretin-v30m-familial-amyloidosis-presenting-as-isolated-retinal-angiopathy
#10
Judy J Chen, Ananda Kalevar, Robin A Vora, Ying Qian, Peter H Gorenberg, Richard Garcia-Kennedy, Emmett T Cunningham
PURPOSE: To describe a patient with confirmed transthyretin V30M form of familial amyloidosis who presented initially with isolated retinal angiopathy. METHODS: Retrospective chart review. RESULTS: A 66-year-old woman presented with bilateral retinal angiopathy. Extensive workup for an infectious, inflammatory, or hypercoagulable cause was unrevealing. The patient subsequently developed bilateral neovascularization of the optic nerve and iris complicated by recurrent vitreous hemorrhages, which were treated with intravitreal bevacizumab and panretinal photocoagulation...
October 9, 2017: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/29016222/misidentification-of-transthyretin-and-immunoglobulin-variants-by-proteomics-due-to-methyl-lysine-formation-in-formalin-fixed-paraffin-embedded-amyloid-tissue
#11
Diana Canetti, Nigel Brian Rendell, Lucia Di Vagno, Janet A Gilbertson, Dorota Rowczenio, Tamar Rezk, Julian D Gillmore, Phillip N Hawkins, Guglielmo Verona, Palma Patrizia Mangione, Sofia Giorgetti, Pierluigi Mauri, Sara Motta, Antonella De Palma, Vittorio Bellotti, Graham W Taylor
Proteomics is becoming the de facto gold standard for identifying amyloid proteins and is now used routinely in a number of centres. The technique is compound class independent and offers the added ability to identify variant and modified proteins. We re-examined proteomics results from a number of formalin-fixed paraffin-embedded amyloid samples, which were positive for transthyretin (TTR) by immunohistochemistry and proteomics, using the UniProt human protein database modified to include TTR variants. The amyloidogenic variant, V122I TTR, was incorrectly identified in 26/27 wild-type and non-V122I variant samples due to its close mass spectral similarity with the methyl lysine-modified WT peptide [126KMe]105-127 (p...
October 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28992906/renal-amyloidosis-associated-with-kartagener-syndrome-in-a-dog
#12
Bartolo Celona, Chiara Crinò, Carmelo Bruno, Simona Di Pietro, Elisabetta Giudice
A 4-year-old cocker spaniel, male, of 12kg body weight was presented because of the onset of polyuria or polydipsia. From the first months of its life, the dog had exhibited constant serous to mucopurulent nasal discharge, productive cough, sneezing, reverse sneezing, otitis, and recurrent episodes of fever. The respiratory signs had been treated several times with antibiotics, without ever achieving a complete resolution. Clinical examination revealed normal rectal temperature (38.3°C), increased respiratory rate (40breaths/min), a copious mucous nasal discharge and right deviation of the heart apex beat (ictus cordis)...
June 2017: Topics in Companion Animal Medicine
https://www.readbyqxmd.com/read/28992817/clinical-recommendations-for-cardiovascular-magnetic-resonance-mapping-of-t1-t2-t2-and-extracellular-volume-a-consensus-statement-by-the-society-for-cardiovascular-magnetic-resonance-scmr-endorsed-by-the-european-association-for-cardiovascular-imaging-eacvi
#13
REVIEW
Daniel R Messroghli, James C Moon, Vanessa M Ferreira, Lars Grosse-Wortmann, Taigang He, Peter Kellman, Julia Mascherbauer, Reza Nezafat, Michael Salerno, Erik B Schelbert, Andrew J Taylor, Richard Thompson, Martin Ugander, Ruud B van Heeswijk, Matthias G Friedrich
Parametric mapping techniques provide a non-invasive tool for quantifying tissue alterations in myocardial disease in those eligible for cardiovascular magnetic resonance (CMR). Parametric mapping with CMR now permits the routine spatial visualization and quantification of changes in myocardial composition based on changes in T1, T2, and T2*(star) relaxation times and extracellular volume (ECV). These changes include specific disease pathways related to mainly intracellular disturbances of the cardiomyocyte (e...
October 9, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28990174/immunoparesis-defined-by-heavy-light-chain-suppression-is-a-novel-marker-of-long-term-outcomes-in-cardiac-al-amyloidosis
#14
Sajitha Sachchithanantham, Oscar Berlanga, Azra Alvi, Shameem A Mahmood, Helen J Lachmann, Julian D Gillmore, Philip N Hawkins, Stephen Harding, Ashutosh D Wechalekar
Cardiac involvement and presenting dFLC (difference between involved and uninvolved free light chains) are independent predictors of outcome in systemic AL amyloidosis. These markers have limited prognostic utility in patients surviving the initial months following diagnosis. Here we assessed immunoparesis, as determined by novel heavy+light chain (HLC) immunoassays, as a prognostic marker for survival in AL amyloidosis. HLC measurements identified immunoparesis of at least one immunoglobulin (Ig) isotype in 145 (85%) patients; and severe immunoparesis (≥2 Ig isotypes suppressed by >50% below normal levels) in 29 (17%) patients...
October 8, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28984490/pitfalls-in-conducting-prospective-trials-in-stage-iii-cardiac-amyloidosis-experience-from-the-reveal-study
#15
Elizabeth H Phillips, Stephen Nash, Toyin Adedayo, Carol J Whelan, Marianna Fontana, Shameem Mahmood, Helen J Lachmann, Julian D Gillmore, Paul Smith, Laura Clifton-Hadley, Philip N Hawkins, Ashutosh D Wechalekar
No abstract text is available yet for this article.
October 6, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28979560/a-case-study-of-likely-wild-type-cardiac-transthyretin-amyloidosis-causing-rapid-deterioration
#16
Thomas Davies, Aarash Saleh, Gerry Coghlan, Carol Whelan, Banwari Agarwal
We present the case of an 88-year-old gentleman who presented to hospital septic with bilateral leg cellulitis, pulmonary oedema and hypotension. He had no history of heart disease but had had bilateral carpal tunnel releases. His condition deteriorated with refractory hypotension in spite of fluid filling, inotropic and vasopressor support. His echocardiogram showed an infiltrative cardiomyopathy with a speckled myocardium, severe concentric left and right ventricular increased wall thickness, diastolic dysfunction, biatrial dilatation and restrictive physiology in keeping with cardiac amyloidosis...
May 2017: J Intensive Care Soc
https://www.readbyqxmd.com/read/28963698/hereditary-lysozyme-amyloidosis-with-sicca-syndrome-digestive-arterial-and-tracheobronchial-involvement-case-based-review
#17
REVIEW
Audrey Benyamine, Fanny Bernard-Guervilly, Céline Tummino, Nicolas Macagno, Laurent Daniel, Sophie Valleix, Brigitte Granel
Lysozyme amyloidosis (ALys) is a rare autosomal dominant hereditary systemic amyloidosis associated with a large spectrum of clinical manifestations. ALys phenotype mainly involves the digestive tract, liver and spleen, kidneys, lymph nodes, skin, and lachrymal and salivary glands. Very recently, cardiac involvement and peripheral neuropathy associated with a new p.Leu102Ser variant of lysozyme have been documented. In the present observation, we extend the phenotypic heterogeneity of ALys to the tracheobronchial tree with histologically proven bronchial ALys-amyloid deposits...
November 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28956153/predictors-of-survival-stratification-in-patients-with-wild-type-cardiac-amyloidosis
#18
F Aus dem Siepen, R Bauer, A Voss, S Hein, M Aurich, J Riffel, D Mereles, C Röcken, S J Buss, H A Katus, Arnt V Kristen
OBJECTIVES: To analyze clinical predictors of mortality in wild-type transthyretin amyloidosis (wt-ATTR). METHODS: In total, 191 patients (73.8 ± 0.5 years; 176 males, 15 females) with histologically proven wt-ATTR amyloidosis and genetic exclusion of a transthyretin gene variant were included. Comprehensive clinical characteristics, ECG, biomarkers, and echocardiography were analyzed retrospectively. Strain analyses were performed offline using TomTec Imaging Systems, Germany...
September 27, 2017: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/28956112/at-least-partial-hematological-response-after-first-cycle-of-treatment-predicts-organ-response-and-long-term-survival-for-patients-with-al-amyloidosis-receiving-bortezomib-based-treatment
#19
Kai-Ni Shen, Jun Feng, Xu-Fei Huang, Chun-Lan Zhang, Cong-Li Zhang, Xin-Xin Cao, Lu Zhang, Dao-Bin Zhou, Jian Li
AL amyloidosis is a rare plasma cell dyscrasia characterized by multi-organ involvement and poor prognosis. We retrospectively evaluated the organ response (OR) and long-term survival of newly diagnosed AL amyloidosis patients who received first-line bortezomib-containing induction therapy, aiming to identify the clinical indication of a 50% reduction in the difference between involved and uninvolved free light chains (dFLC) after first cycle of treatment. Among the 89 patients included, 78.7% had cardiac involvement and 42...
September 27, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28954631/measurement-of-myocardial-native-t1-in-cardiovascular-diseases-and-norm-in-1291-subjects
#20
Joanna M Liu, Alexander Liu, Joana Leal, Fiona McMillan, Jane Francis, Andreas Greiser, Oliver J Rider, Saul Myerson, Stefan Neubauer, Vanessa M Ferreira, Stefan K Piechnik
BACKGROUND: Native T1-mapping provides quantitative myocardial tissue characterization for cardiovascular diseases (CVD), without the need for gadolinium. However, its translation into clinical practice is hindered by differences between techniques and the lack of established reference values. We provide typical myocardial T1-ranges for 18 commonly encountered CVDs using a single T1-mapping technique - Shortened Look-Locker Inversion Recovery (ShMOLLI), also used in the large UK Biobank and Hypertrophic Cardiomyopathy Registry study...
September 28, 2017: Journal of Cardiovascular Magnetic Resonance
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