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https://www.readbyqxmd.com/read/28813699/t1-and-t2-mapping-in-cardiology-mapping-the-obscure-object-of-desire
#1
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
#2
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
#3
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
#4
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
#5
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, 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 28, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28743670/xuefu-zhuyu-decoction-ameliorates-obesity-hepatic-steatosis-neuroinflammation-amyloid-deposition-and-cognition-impairment-in-metabolically-stressed-appswe-ps1de9-mice
#6
Chih-Wen Yeh, Hui-Kang Liu, Lie-Chwen Lin, Kou-Tong Liou, Yung-Cheng Huang, Chien-Hung Lin, Tsai-Teng Tzeng, Feng-Shiun Shie, Huey-Jen Tsay, Young-Ji Shiao
ETHNOPHARMACOLOGICAL RELEVANCE: Metabolic syndrome and vascular dysfunction was suggested to be the risk factors for Alzheimer's disease (AD). Xuefu Zhuyu decoction (XZD) is a traditional Chinese medicine used to treat metabolic syndrome and cardiac-cerebral vascular disease. The effects of XZD on ameliorating metabolic syndrome, amyloid-related pathologies and cognitive impairment in an animal model of AD with metabolic stress was investigated. MATERIALS AND METHOD: The animal model of AD with metabolic stress was created by administrating high-fat diet and a low-dose injection of streptozotocin prior to the appearance of senile plaques in APP/PS1 transgenic mice...
July 22, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#7
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28728692/magnetic-resonance-in-transthyretin%C3%A2-cardiac-amyloidosis
#8
Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Giulia Zumbo, Daniel S Knight, Tushar Kotecha, Rohin Francis, David F Hutt, Tamer Rezk, Stefania Rosmini, Candida C Quarta, Carol J Whelan, Peter Kellman, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#9
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#10
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
August 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28697563/autonomic-cardiac-function-in-preclinical-alzheimer-s-disease
#11
Cláudia Y Santos, Jason T Machan, Wen-Chih Wu, Peter J Snyder
To explore early autonomic cardiac changes in pre-clinical Alzheimer's disease (AD), we have evaluated electrocardiologic measures of vagal tone for 63 adults (ages 55-75) at rest, during cognitive testing, and then again at rest. All subjects had multiple risk factors for AD, and all completed amyloid PET scans (18F-Florbetapir) to determine amyloid positivity (Aβ+). No change in electrocardiographic measures were observed for Aβ+ participants under each testing condition, whereas Aβ-subjects showed an expected increase in vagal tone during the cognitive stress condition...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28694632/late-onset-familial-amyloidotic-polyneuropathy-with-bence-jones-proteinuria-and-cardiomyopathy
#12
Sira Carrasco García de León, Amalia Hernández González, Carmen Orellana Alonso, Laura Burriel Lobo
Familial amyloidotic polyneuropathy is a genetically determined disease characterized by deposition of an anomalous transthyretin. A high index of suspicion is needed for this multisymptomatic and lethal disease to be diagnosed. The patient was a 70-year-old male examined due to hypesthesia in the hands and feet, plus difficulty walking. A neurophysiological study delivered the diagnosis of axonal sensorimotor polyneuropathy. He later developed cardiac symptoms and diarrhea. Urine laboratory analyses revealed a monoclonal spike of light chains (kappa)...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28689003/primary-amyloidosis-with-renal-involvement-outcomes-in-77-consecutive-patients-at-a-single-center
#13
Sandy W Wong, Denis Toskic, Melissa Warner, Cindy Varga, Alejandro Moreno-Koehler, Daniel Fein, Teresa Fogaren, Lisa Lee, Colin M Oliver, Spencer D Guthrie, Raymond L Comenzo
BACKGROUND: Outcomes in primary amyloid renal patients are of interest as the era of monoclonal antibody therapies begins. PATIENTS AND METHODS: We studied 77 consecutive primary amyloid renal patients (58% men) for renal progression (end stage renal disease [ESRD]), renal response (RR), and overall survival (OS). RESULTS: At diagnosis median age was 63 (range, 35-81) years, estimated glomerular filtration rate 70 mL/min (range, 5-114), difference between involved and uninvolved free light chains 127 mg/L (range, 1-9957), ESRD 4%, renal stage 2 and 3 78%, and cardiac stage 2 and 3 56%...
June 17, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28678923/cardiac-amyloidosis-and-its-new-clinical-phenotype-heart-failure-with-preserved-ejection-fraction
#14
Evandro Tinoco Mesquita, Antonio José Lagoeiro Jorge, Celso Vale Souza, Thais Ribeiro de Andrade
Heart failure with preserved ejection fraction (HFpEF) is now an emerging cardiovascular epidemic, being identified as the main phenotype observed in clinical practice. It is more associated with female gender, advanced age and comorbidities such as hypertension, diabetes, obesity and chronic kidney disease. Amyloidosis is a clinical disorder characterized by the deposition of aggregates of insoluble fibrils originating from proteins that exhibit anomalous folding. Recently, pictures of senile amyloidosis have been described in patients with HFpEF, demonstrating the need for clinical cardiologists to investigate this etiology in suspect cases...
June 29, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28678039/familial-amyloid-polyneuropathy
#15
David Adams, Cécile Cauquil, Céline Labeyrie
PURPOSE OF REVIEW: Transthyretin familial amyloid polyneuropathy is the most disabling hereditary polyneuropathy of adult onset because of a point mutation of transthyretin gene. This review updates our knowledge about natural history of the disease, phenotypes, diagnosis tools for small and large fibers involvement, expert's consensus for both symptomatic and asymptomatic follow-up, and treatment's research. RECENT FINDINGS: Access to TTR gene sequencing permit diagnosis and first reports of the disease in nonendemic countries (EU countries, United States, China, India)...
July 3, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#16
P Patrizia Mangione, Giuseppe Mazza, Janet A Gilbertson, Nigel B Rendell, Diana Canetti, Sofia Giorgetti, Luca Frenguelli, Marco Curti, Tamer Rezk, Sara Raimondi, Mark B Pepys, Philip N Hawkins, Julian D Gillmore, Graham W Taylor, Massimo Pinzani, Vittorio Bellotti
Diagnosis and treatment of systemic amyloidosis depend on accurate identification of the specific amyloid fibril protein forming the tissue deposits. Confirmation of monoclonal immunoglobulin light chain amyloidosis (AL), requiring cytotoxic chemotherapy, and avoidance of such treatment in non-AL amyloidosis, are particularly important. Proteomic analysis characterises amyloid proteins directly. It complements immunohistochemical staining of amyloid to identify fibril proteins and gene sequencing to identify mutations in the fibril precursors...
June 21, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28646538/a-novel-attr-l32v-mutation-causes-familial-amyloid-polyneuropathy-in-a-bolivian-family
#17
P L Martínez-Ulloa, M Vallejo, I Corral, N García-Barragán, A Alcazar, Emma Martínez-Alonso, J Martínez-Poles, H Pian, A Jiménez-Escrig
We report a new transthyretin (ATTR) gene c.272C>G mutation and variant protein, p.Leu32Val, in a kindred of Bolivian origin with a rapid progressive peripheral neuropathy and cardiomyopathy. Three individuals from a kindred with peripheral nerve and cardiac amyloidosis were examined. Analysis of the TTR gene was performed by Sanger direct sequencing. Neuropathologic examination was obtained on the index patient with mass spectrometry study of the ATTR deposition. Direct DNA sequence analysis of exons 2, 3 and 4 of the TTR gene demonstrated a c...
June 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28637305/left-atrial-structure-and-function-in-cardiac-amyloidosis
#18
Kotaro Nochioka, Candida Cristina Quarta, Brian Claggett, Gabriela Querejeta Roca, Claudio Rapezzi, Rodney H Falk, Scott D Solomon
Aims: Although cardiac amyloidosis (CA) is characterized by significant left atrial (LA) dilatation, the characteristics of LA function remain to be fully investigated. Methods and results: We assessed LA function by speckle-tracking echocardiography in 124 patients with CA and sinus rhythm: 68 with light chain (AL), 29 with mutant (ATTRm), 27 with wild-type (ATTRwt) transthyretin amyloidosis. Conventional and strain-derived parameters, including LA peak longitudinal strain (LS) and strain rate (peak LSR: reservoir function; early LSR: conduit function; late LSR: active function), were assessed compared between CA patients and 20 healthy controls of similar age and gender...
June 16, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#19
Johanna Denman, Kaveh Manavi, Mark Cook
We present a case of an HIV-positive man with systemic immunoglobulin light chain (AL) amyloid with cardiac involvement. At relapse, he was treated with lenalidomide and dexamethasone having previously developed autonomic neuropathy with bortezomib-based chemotherapy. The patient achieved a serological complete response with symptomatic improvement. After 11 cycles, lenalidomide was discontinued due to extensive ischaemia of the gastrointestinal tract. The patient remains symptomatically stable with normal levels of serum-free light chains 11 months after the treatment was discontinued...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28630445/myocardial-deformation-in-cardiac-amyloid-light-chain-amyloidosis-assessed-with-3t-cardiovascular-magnetic-resonance-feature-tracking
#20
Rui Li, Zhi-Gang Yang, Hua-Yan Xu, Ke Shi, Xi Liu, Kai-Yue Diao, Ying-Kun Guo
Clinically, assessment of myocardial function is essential in patients with amyloid light-chain cardiac amyloidosis (AL-CA) to predict outcome and determine therapeutic approach. The aim of this study was to investigate the feasibility of cardiovascular magnetic resonance (CMR)-derived feature tracking algorithm for assessing left ventricular (LV) myocardial deformation in AL-CA, and to determine if these abnormal myocardial deformation parameters are correlated to impaired LV myocardial microvascular dysfunction...
June 19, 2017: Scientific Reports
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