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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/29993288/cerebrospinal-fluid-and-vitreous-body-exposure-to-orally-administered-tafamidis-in-hereditary-attrv30m-p-ttrv50m-amyloidosis-patients
#1
Cecilia Monteiro, Ana Martins da Silva, Natália Ferreira, Jaleh Mesgarzadeh, Marta Novais, Teresa Coelho, Jeffery W Kelly
Hereditary transthyretin (TTR) amyloidosis associated with the TTRV30M (p.TTRV50M) mutation presents predominantly as an axonal polyneuropathy, with variable involvement of other organs. Serious central nervous system (CNS) and eye manifestations, including stroke, dementia, vitreous opacities and glaucoma, have been reported in untreated V30M TTR amyloidosis patients, and in these patients after treatment with liver transplantation (LT). Distinct therapies for V30M TTR amyloidosis developed during the last decade exhibit promising results in slowing the peripheral and autonomic nervous system pathology...
July 11, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29855203/systemic-angiopathy-and-axonopathy-in-hereditary-transthyretin-amyloidosis-with-ala97gly-p-ala117gly-mutation-a-post-mortem-analysis
#2
Haruki Koike, Takeshi Yasuda, Ryoji Nishi, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Gen Sobue, Masahisa Katsuno
No abstract text is available yet for this article.
June 1, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29775082/profile-of-renal-aa-amyloidosis-in-older-and-younger-individuals-a-single-centre-experience
#3
Siyar Erdogmus, Zeynep Kendi Celebi, Serkan Akturk, Gizem Kumru, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Kenan Keven
OBJECTIVE: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis...
May 18, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29733684/mri-feature-tracking-strain-is-prognostic-for-all-cause-mortality-in-al-amyloidosis
#4
Jeffery E Illman, Shivaram P Arunachalam, Arvin Arani, Ian Cheng-Yi Chang, James F Glockner, Angela Dispenzieri, Martha Grogan, Philip A Araoz
OBJECTIVES: Cardiac involvement is a major determinate of mortality in light chain (AL) amyloidosis. Cardiac magnetic resonance imaging (MRI) feature tracking (FT) strain is a new method for measuring myocardial strain. This study retrospectively evaluated the association of MRI FT strain with all-cause mortality in AL amyloidosis. MATERIALS AND METHODS: Seventy-six patients with newly diagnosed AL amyloidosis underwent cardiac MRI. 75 had images suitable for MRI FT strain analysis...
May 7, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29706127/utility-of-the-18-f-florbetapir-positron-emission-tomography-in-systemic-amyloidosis
#5
Jaume Mestre-Torres, Carles Lorenzo-Bosquet, Gemma Cuberas-Borrós, Mercedes Gironella, Roser Solans-Laque, Andreu Fernández-Codina, Segundo Bujan-Rivas, Joan Castell-Conesa, Fernando Martínez-Valle
Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using 18 F-florbetapir. We hypothesize that combining whole body PET/CT with 18 F-Florbetapir can be useful to characterize the burden of the disease. We included 25 patients, 13 of them with different types of amyloidosis, and 12 with Alzheimer's disease as controls...
April 28, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29571269/a-functional-assay-to-identify-amyloidogenic-light-chains
#6
Emily B Martin, Angela D Williams, R Eric Heidel, James S Foster, Ronald H Lands, Stephen J Kennel, Jonathan S Wall
INTRODUCTION: Multiple myeloma (MM) and light chain monoclonal gammopathy of undetermined significance (LCMGUS) are plasma cell disorders associated with the secretion of monoclonal free light-chain (LC) proteins. Due to the high concentrations of LC in circulation, both of these populations are at risk for developing LC-associated amyloidosis (AL) - a protein misfolding disease characterized by the deposition of LC protein fibrils in organs and tissues, leading to dysfunction and significant morbidity...
March 23, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29529882/response-atrial-impairment-in-transthyretin-cardiac-amyloidosis-an-early-marker-of-cardiac-involvement-and-a-prognostic-factor
#7
Michal Y Henein, Per Lindqvist
No abstract text is available yet for this article.
March 12, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29529877/digoxin-use-in-systemic-light-chain-al-amyloidosis-contra-indicated-or-cautious-use
#8
Eli Muchtar, Morie A Gertz, Shaji K Kumar, Grace Lin, Barry Boilson, Alfredo Clavell, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, David Dingli, S Vincent Rajkumar, Angela Dispenzieri, Martha Grogan
AIM: Digoxin is considered contraindicated in light-chain (AL) amyloidosis, given reports of increased toxicity published 30-50 years ago. We sought to determine the frequency of digoxin toxicity in patients with AL. METHODS: We identified 107 patients with AL amyloidosis who received digoxin between 2000 and 2015. RESULTS: The median age was 65 and the median digoxin dose and estimated glomerular filtration rate were 0.125 mg/d and 55 ml/min/1...
March 12, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29523040/atrial-impairment-in-transthyretin-cardiac-amyloidosis-an-early-marker-of-cardiac-involvement-and-a-prognostic-factor
#9
Gianluca Di Bella, Fabio Minutoli
No abstract text is available yet for this article.
March 9, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29516761/once-al-amyloidosis-not-always-al-amyloidosis
#10
Tulip Jhaveri, Shayna Sarosiek, Frederick L Ruberg, Omar Siddiqi, John L Berk, Vaishali Sanchorawala
No abstract text is available yet for this article.
March 8, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29424556/monoclonal-gammopathy-of-undetermined-significance-in-systemic-transthyretin-amyloidosis-attr
#11
Pooja Phull, Vaishali Sanchorawala, Lawreen H Connors, Gheorghe Doros, Frederick L Ruberg, John L Berk, Shayna Sarosiek
OBJECTIVE: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. PATIENTS AND METHODS: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29394116/normal-scores-of-deep-breathing-tests-beware-of-dysrhythmia-in-transthyretin-amyloidosis
#12
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...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
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
#13
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...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29364741/obesity-is-a-significant-susceptibility-factor-for-idiopathic-aa-amyloidosis
#14
Norbert Blank, Ute Hegenbart, Sascha Dietrich, Maik Brune, Jörg Beimler, Christoph Röcken, Carsten Müller-Tidow, Hanns-Martin Lorenz, Stefan O Schönland
BACKGROUND: To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis. METHODS: Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. FINDINGS: Patients with AA amyloidosis had either familial Mediterranean fever (FMF) or long-standing rheumatic diseases as underlying inflammatory disease (n = 111, median age 46 years)...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29357699/psychopathological-dimensions-in-subjects-with-hereditary-attr-v30m-amyloidosis-and-their-relation-with-life-events-due-to-the-disease
#15
Alice Lopes, Isabel Fonseca, Alexandra Sousa, Carla Rodrigues, Margarida Branco, Teresa Coelho, Jorge Sequeiros, Paula Freitas
BACKGROUND: Chronic physical illness has been associated with emotional distress. Chronic diseases may change usual family patterns with economic, social and family losses. Hereditary ATTR V30M amyloidosis is a rare, fatal inherited systemic amyloidosis, with chronic evolution and beginning in adulthood. AIMS AND METHODS: To evaluate psychopathological dimensions and how they correlated with disease-related life events, 209 symptomatic and asymptomatic carriers, participated in the study...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29303358/treatment-patterns-and-health-care-resource-utilization-among-patients-with-relapsed-refractory-systemic-light-chain-amyloidosis
#16
Parameswaran Hari, Huamao Mark Lin, Carl V Asche, Jinma Ren, Candice Yong, Katarina Luptakova, Douglas V Faller, Vaishali Sanchorawala
BACKGROUND: Treatment for patients with systemic light chain (AL) amyloidosis remains challenging. Our study aims to describe treatment patterns for both newly diagnosed and relapsed/refractory AL (RRAL) amyloidosis, and to assess clinical outcomes, healthcare costs, and resource utilization during the first year following a diagnosis of RRAL amyloidsis. METHODS: This was a retrospective observational study of adult patients with AL amyloidosis using the US Optum administrative claims data during 1/1/2008 to 6/30/2015...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29260587/right-ventricular-longitudinal-strain-a-tool-for-diagnosis-and-prognosis-in-light-chain-amyloidosis
#17
Charles Uzan, Olivier Lairez, Pascale Raud-Raynier, Rodrigue Garcia, Bruno Degand, Luc P Christiaens, Michaela B Rehman
OBJECTIVES: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. METHODS: We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29241368/gastrointestinal-perforation-in-light-chain-amyloidosis-in-the-era-of-novel-agent-therapy-a-case-series-and-review-of-the-literature
#18
Adir Shaulov, Irit Avivi, Yael Cohen, Adrian Duek, Merav Leiba, Moshe E Gatt
Gastrointestinal (GI) perforation is remarkably rare in patients with light chain (AL) amyloidosis and has not yet been reported in patients with AL amyloidosis treated with novel agents. Only 24 cases of GI perforation have previously been reported in the setting of AL amyloidosis of which 15 had available information in English. All 15 did not receive novel agent therapy and six died early after experiencing GI perforation. This study reports the characteristics and outcome of AL patients that developed GI perforation in the era of novel agent treatment...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29219632/budd-chiari-syndrome-associated-with-al-amyloidosis-a-coagulation-paradox
#19
Guilherme Grossi Lopes Cançado, Luciana Costa Faria, Fernanda Maria Farage Osório, Paula Vieira Teixeira Vidigal, Cláudia Alves Couto, Teresa Cristina de Abreu Ferrari
No abstract text is available yet for this article.
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29182024/first-nationwide-survey-on-systemic-wild-type-attr-amyloidosis-in-japan
#20
Yoshiki Sekijima, Masahide Yazaki, Mitsuharu Ueda, Haruki Koike, Masahito Yamada, Yukio Ando
OBJECTIVE: A nationwide survey on systemic wild-type ATTR (ATTRwt) amyloidosis was conducted to elucidate the frequency, clinical picture and possible diagnostic issues of ATTRwt amyloidosis in Japan. METHODS: A questionnaire was sent to 4629 clinical departments across Japan. A total of 2341 (50.6%) responses were returned completed for further analysis. RESULTS: Fifty-one patients with ATTRwt amyloidosis (82% male) were identified between January 2012 and December 2014...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
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