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https://www.readbyqxmd.com/read/28943540/amyloid-polyneuropathy-and-myocardial-amyloidosis-10-years-after-domino-liver-transplantation-from-a-patient-with-a-transthyretin-ser50arg-mutation
#1
Masaaki Matsushima, Ichiro Yabe, Masaya Tsuda, Mamoru Sakakibara, Tsuyoshi Shimamura, Hidenao Sasaki
A 54-year-old man with polycystic liver disease received a domino liver transplantation (DLT) from a patient of hereditary ATTR amyloidosis with the transthyretin Ser50Arg mutation. Ten years after transplantation, he felt a slight numbness in his toes, and cardiac amyloidosis was simultaneously suspected upon a heart function evaluation. Biopsy specimens from the myocardium revealed transthyretin amyloidosis with the Ser50Arg mutation. Oral tafamidis therapy has inhibited the progression of neurological and cardiovascular symptoms this far...
September 25, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28906150/safety-and-efficacy-of-a-ttr-specific-antisense-oligonucleotide-in-patients-with-transthyretin-amyloid-cardiomyopathy
#2
Merrill D Benson, Noel R Dasgupta, Stacy M Rissing, Jessica Smith, Harvey Feigenbaum
OBJECTIVES: Cardiomyopathy is a major cause of death in both the hereditary form of transthyretin (TTR) amyloidosis and the sporadic late-age-onset transthyretin amyloidosis (ATTR wild-type (ATTRwt)). Clinically disease progression from time of diagnosis to death is usually quoted as 5- to 15-years. In prior studies, significant progression of cardiac parameters in patients with moderate to severe cardiomyopathy has been noted within a 12-month time span. METHODS: The present study was designed to prospectively monitor changes in cardiac parameters, both structural and functional, in patients with ATTR cardiomyopathy while treated with a TTR specific antisense oligonucleotide (ASO; IONIS-TTR℞) designed to lower blood levels of the amyloid fibril precursor protein...
September 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28906148/hip-and-knee-arthroplasty-are-common-among-patients-with-transthyretin-cardiac-amyloidosis-occurring-years-before-cardiac-amyloid-diagnosis-can-we-identify-affected-patients-earlier
#3
Jonah Rubin, Julissa Alvarez, Sergio Teruya, Adam Castano, Ronald A Lehman, Mark Weidenbaum, Jeffrey A Geller, Stephen Helmke, Mathew S Maurer
Transthyretin cardiac amyloidosis (ATTR-CA) causes a restrictive cardiomyopathy in older adults, often diagnosed at advanced stages when emerging therapies in late phase clinical trials may not have clinical benefit. This investigation aimed to detect clinical entities that may provide more advanced warning of ATTR-CA. Since ATTR preferentially deposits in ligaments, tendons, and articular cartilage, we hypothesized that ATTR-CA patients have a greater prevalence of total hip (THA) and knee (TKA) arthroplasties compared with the general population, and that arthroplasty occurs significantly before ATTR-CA diagnosis...
September 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28893208/trial-design-and-rationale-for-apollo-a-phase-3-placebo-controlled-study-of-patisiran-in-patients-with-hereditary-attr-amyloidosis-with-polyneuropathy
#4
David Adams, Ole B Suhr, Peter J Dyck, William J Litchy, Raina G Leahy, Jihong Chen, Jared Gollob, Teresa Coelho
BACKGROUND: Patisiran is an investigational RNA interference (RNAi) therapeutic in development for the treatment of hereditary ATTR (hATTR) amyloidosis, a progressive disease associated with significant disability, morbidity, and mortality. METHODS: Here we describe the rationale and design of the Phase 3 APOLLO study, a randomized, double-blind, placebo-controlled, global study to evaluate the efficacy and safety of patisiran in patients with hATTR amyloidosis with polyneuropathy...
September 11, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28887775/imaging-cardiac-innervation-in-amyloidosis
#5
Riemer H J A Slart, Andor W J M Glaudemans, Bouke P C Hazenberg, Walter Noordzij
Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. The use of iodine-123 labelled metaiodobenzylguanidine ([I-123]MIBG), a chemical modified analogue of norepinephrine, is well established in patients with heart failure and plays an important role in evaluation of sympathetic innervation in cardiac amyloidosis...
September 8, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28883786/arterial-pulsations-cannot-drive-intramural-periarterial-drainage-significance-for-a%C3%AE-drainage
#6
Alexandra K Diem, Matthew MacGregor Sharp, Maureen Gatherer, Neil W Bressloff, Roxana O Carare, Giles Richardson
Alzheimer's Disease (AD) is the most common form of dementia and to date there is no cure or efficient prophylaxis. The cognitive decline correlates with the accumulation of amyloid-β (Aβ) in the walls of capillaries and arteries. Our group has demonstrated that interstitial fluid and Aβ are eliminated from the brain along the basement membranes of capillaries and arteries, the intramural periarterial drainage (IPAD) pathway. With advancing age and arteriosclerosis, the stiffness of arterial walls, this pathway fails in its function and Aβ accumulates in the walls of arteries...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28867709/neuroprotective-effect-of-corydalis-ternata-extract-and-its-phytochemical-quantitative-analysis
#7
Yu Jin Kim, Hye-Sun Lim, Yoonju Kim, Jun Lee, Bu-Yeo Kim, Soo-Jin Jeong
The tubers of Corydalis ternata have been used to treat cardiovascular diseases such as hypertension and cardiac arrhythmia. Its active components have anticholinesterase, antiamnesic, and anti-inflammatory activities, and analgesic effects. In the present study, we performed quantitative analyses of the two components of C. ternata, coptisine and berberine, using HPLC. A 70% ethanol extract of C. ternata was prepared and the two components were separated using a C-18 analytical column on a gradient solvent system of acetonitrile and 0...
2017: Chemical & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/28862927/inhibition-of-mutant-%C3%AE-b-crystallin-induced-protein-aggregation-by-a-molecular-tweezer
#8
Na Xu, Gal Bitan, Thomas Schrader, Frank-Gerrit Klärner, Hanna Osinska, Jeffrey Robbins
BACKGROUND: Compromised protein quality control causes the accumulation of misfolded proteins and intracellular aggregates, contributing to cardiac disease and heart failure. The development of therapeutics directed at proteotoxicity-based pathology in heart disease is just beginning. The molecular tweezer CLR01 is a broad-spectrum inhibitor of abnormal self-assembly of amyloidogenic proteins, including amyloid β-protein, tau, and α-synuclein. This small molecule interferes with aggregation by binding selectively to lysine side chains, changing the charge distribution of aggregation-prone proteins and thereby disrupting aggregate formation...
August 8, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28861330/a-plasma-cell-dyscrasia-presenting-as-amyloid-cardiomyopathy-and-autonomic-dysfunction-in-a-healthy-patient
#9
Rabih Tabet, Julie Zaidan, Boutros Karam, Samer Saouma, Foad Ghavami
Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient...
June 29, 2017: Curēus
https://www.readbyqxmd.com/read/28857614/attitudes-about-when-and-how-to-treat-patients-with-al-amyloidosis-an-international-survey
#10
Paolo Milani, Morie A Gertz, Giampaolo Merlini, Angela Dispenzieri
The aim of this survey was to describe the treatment decision making of expert physicians in when and how to treat patients with AL amyloidosis. Fifty amyloid expert physicians completed the survey. Autologous stem cell transplant (ASCT) was considered the first line therapy, if medically feasible, by 73% of the physicians. Excluding ASCT, cyclophosphamide-bortezomib-dexamethasone regimen was the preferred strategy by 72%. Depending on organ involvement, the goal for treatment was CR for 27-35% and very good partial response (VGPR) for 65-72%...
August 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28844519/functional-cardiac-recovery-and-hematologic-response-to-chemotherapy-in-patients-with-light-chain-amyloidosis-from-the-stanford-university-amyloidosis-registry
#11
Mirela Tuzovic, Yukari Kobayashi, Matthew Wheeler, Christopher Barrett, Michaela Liedtke, Richard Lafayette, Stanley Schrier, Francois Haddad, Ronald Witteles
Cardiac involvement is common in patients with light-chain (AL) amyloidosis and portends a poor prognosis, although little is known about the changes in cardiac mechanics after chemotherapy. We sought to explore the relation between amyloidosis staging and baseline cardiac mechanics and to investigate short-term changes in cardiac mechanics after chemotherapy. We identified 41 consecutive patients from the Stanford Amyloid Center who had echocardiograms and free light-chain values before and after chemotherapy, along with 40 age- and gender-matched controls...
July 25, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28840452/transthyretin-cardiac-amyloidosis
#12
REVIEW
Anit K Mankad, Keyur B Shah
PURPOSE OF REVIEW: Transthyretin (TTR)-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive, hypertrophic heart disease and may go undiagnosed. Transthyretin-derived amyloidosis accounts for 18% of all cases of cardiac amyloidosis. Thus, the study's purpose is to provide a comprehensive review of transthyretin cardiac amyloidosis. RECENT FINDINGS: Wild-type transthyretin (ATTRwt) protein causes cardiac amyloidosis sporadically, with 25 to 36% of the population older than 80 years of age are at risk to develop a slowly progressive, infiltrative amyloid cardiomyopathy secondary to ATTRwt...
August 24, 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28840397/right-ventricular-involvement-evaluated-by-cardiac-magnetic-resonance-imaging-predicts-mortality-in-patients-with-light-chain-amyloidosis
#13
Ke Wan, Jiayu Sun, Yuchi Han, Yong Luo, Hong Liu, Dan Yang, Wei Cheng, Qing Zhang, Zhi Zeng, Yucheng Chen
Few studies have focused on right ventricular (RV) involvement in cardiac amyloidosis (CA). We investigated the prognostic value of RV assessment by cardiovascular magnetic resonance (CMR) in CA. In 2011-2014, consecutive patients with suspected CA referred for CMR were retrospectively evaluated. Demographic and baseline clinical characteristics were collected. Healthy volunteers were matched for sex and age and served as controls. All subjects underwent a contrast-enhanced CMR examination. RV size, function, and late gadolinium enhancement (LGE) were analyzed...
August 24, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28836315/inotropic-myocardial-reserve-deficiency-is-the-predominant-feature-of-exercise-haemodynamics-in-cardiac-amyloidosis
#14
Tor Skibsted Clemmensen, Henning Mølgaard, Jens Sörensen, Hans Eiskjaer, Niels Frost Andersen, Søren Mellemkjaer, Mads Jønsson Andersen, Lars Poulsen Tolbod, Hendrik J Harms, Steen Hvitfeldt Poulsen
AIMS: This study aimed to characterize invasive haemodynamics during exercise in subjects with cardiac amyloidosis (CA). METHODS AND RESULTS: The study population numbered 44 subjects. Group A (CA-positive, n = 24) comprised wild-type transthyretin patients (n = 10), familial transthyretin amyloidosis mutation carriers (ATTRm) with cardiac involvement (n = 5), and light-chain amyloidosis patients with cardiac involvement (n = 9). Group B (CA-negative, n = 20) comprised four healthy ATTRm subjects without cardiac involvement documented by (11) C-PIB positron emission tomography and 16 healthy controls...
August 24, 2017: European Journal of Heart Failure
https://www.readbyqxmd.com/read/28830801/presence-of-fluorescent-in-situ-hybridization-abnormalities-is-associated-with-plasma-cell-burden-in-light-chain-amyloidosis
#15
Lindsay Hammons, Ruta Brazauskas, Marcelo Pasquini, Mehdi Hamadani, Parameswaran Hari, Anita D'Souza
OBJECTIVE/BACKGROUND: To assess abnormalities found on CD138-enriched fluorescent in situ hybridization (FISH) studies on pretreated bone marrow in systemic amyloid light-chain (AL) amyloidosis and correlate findings between these abnormalities with organ involvement and 1-year survival. METHODS: We reviewed 107 patients with systemic AL to identify the impact of a diagnostic FISH study done on plasma cell clones in our institution between January 2010 and January 2015; 77 had pretreatment testing performed...
August 16, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28813699/t1-and-t2-mapping-in-cardiology-mapping-the-obscure-object-of-desire
#16
Sophie Mavrogeni, Dimitris Apostolou, Panayiotis Argyriou, Stella Velitsista, Lilika Papa, Stelios Efentakis, Evangelos Vernardos, Mikela Kanoupaki, George Kanoupakis, Athanassios Manginas
The increasing use of cardiovascular magnetic resonance (CMR) is based on its capability to perform biventricular function assessment and tissue characterization without radiation and with high reproducibility. The use of late gadolinium enhancement (LGE) gave the potential of non-invasive biopsy for fibrosis quantification. However, LGE is unable to detect diffuse myocardial disease. Native T1 mapping and extracellular volume fraction (ECV) provide knowledge about pathologies affecting both the myocardium and interstitium that is otherwise difficult to identify...
August 17, 2017: Cardiology
https://www.readbyqxmd.com/read/28812126/myocardial-iodine-concentration-measurement-using-dual-energy-computed-tomography-for-the-diagnosis-of-cardiac-amyloidosis-a-pilot-study
#17
Virgile Chevance, Thibaud Damy, Vania Tacher, François Legou, Fourat Ridouani, Alain Luciani, Hicham Kobeiter, Alain Rahmouni, Jean-François Deux
OBJECTIVE: To measure myocardium iodine concentration (MIC) in patients with cardiac amyloidosis (CA) using dual-energy computed tomography (DECT). METHODS: Twenty-two patients with CA, 13 with non-amyloid hypertrophic cardiomyopathies (CH) and 10 control patients were explored with pre-contrast, arterial and 5-minute DECT acquisition (Iomeprol; 1.5 mL/kg). Inter-ventricular septum (IVS) thickness, blood pool iodine concentration (BPIC), MIC (mg/mL), iodine ratio and extra-cellular volume (ECV) were calculated...
August 10, 2017: European Radiology
https://www.readbyqxmd.com/read/28801077/myocardial-amyloid-quantification-with-look-locker-magnetic-resonance-sequence-in-cardiac-amyloidosis-diagnostic-accuracy-in-clinical-practice-and-histological-validation
#18
Eduardo Pozo, Jose M Castellano, Anubhav Kanwar, Rajiv Deochand, Mireia Castillo-Martin, Pablo Pazos-López, Carlos González-Lengua, Keren Osman, Matthew Cham, Carlos Cordon-Cardo, Jagat Narula, Valentin Fuster, Javier Sanz
BACKGROUND: Cardiac magnetic resonance (CMR) has demonstrated its utility in the non-invasive diagnosis of cardiac amyloidosis (CA). Our aim was to evaluate the ability of standard Look-Locker sequences to quantify amyloid deposition in CA. METHODS AND RESULTS: Consecutive patients referred for CMR for possible CA were retrospectively evaluated. Positive cardiac biopsy and/or typical pattern of late gadolinium enhancement were required for the diagnosis of CA. Post-contrast T1 values were obtained from Look-Locker sequences, and correlated with markers of severity of disease and major events...
August 8, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28782443/cerebral-a%C3%AE-40-and-systemic-hypertension
#19
Hannah M Tayler, Jennifer C Palmer, Taya L Thomas, Patrick G Kehoe, Julian Fr Paton, Seth Love
Mid-life hypertension and cerebral hypoperfusion may be preclinical abnormalities in people who later develop Alzheimer's disease. Although accumulation of amyloid-beta (Aβ) is characteristic of Alzheimer's disease and is associated with upregulation of the vasoconstrictor peptide endothelin-1 within the brain, it is unclear how this affects systemic arterial pressure. We have investigated whether infusion of Aβ40 into ventricular cerebrospinal fluid modulates blood pressure in the Dahl salt-sensitive rat...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28760409/development-and-reliability-of-a-user-friendly-multicenter-phenotyping-application-for-hemorrhagic-and-ischemic-stroke
#20
Mayowa Owolabi, Godwin Ogbole, Rufus Akinyemi, Kehinde Salaam, Onoja Akpa, Pattanasak Mongkolwat, Adeleye Omisore, Atinuke Agunloye, Richard Efidi, Joseph Odo, Akintomiwa Makanjuola, Albert Akpalu, Fred Sarfo, Lukman Owolabi, Reginald Obiako, Kolawole Wahab, Emmanuel Sanya, Philip Adebayo, Morenikeji Komolafe, Abiodun Moshood Adeoye, Michael B Fawale, Joshua Akinyemi, Godwin Osaigbovo, Taofiki Sunmonu, Paul Olowoyo, Innocent Chukwuonye, Yahaya Obiabo, Philip Ibinaiye, Abdul Dambatta, Yaw Mensah, Salaam Abdul, Eunice Olabinri, Joyce Ikubor, Olalekan Oyinloye, Femi Odunlami, Ezinne Melikam, Raelle Saulson, Philip Kolo, Adesola Ogunniyi, Bruce Ovbiagele
BACKGROUND: Annotation and Image Markup on ClearCanvas Enriched Stroke-phenotyping Software (ACCESS) is a novel stand-alone computer software application that allows the creation of simple standardized annotations for reporting brain images of all stroke types. We developed the ACCESS application and determined its inter-rater and intra-rater reliability in the Stroke Investigative Research and Educational Network (SIREN) study to assess its suitability for multicenter studies. METHODS: One hundred randomly selected stroke imaging reports from 5 SIREN sites were re-evaluated by 4 trained independent raters to determine the inter-rater reliability of the ACCESS (version 12...
July 29, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
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