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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/30193540/progressive-axonal-polyneuropathy-in-a-mitochondrial-disorder-an-uncommon-association-with-familial-amyloid-neuropathy
#1
Marco Luigetti, Guido Primiano, Giulia Bisogni, Cristina Cuccagna, Rosalba Carrozzo, Laura Obici, Daniela Bernardo, Cristina Sancricca, Serenella Servidei
No abstract text is available yet for this article.
September 7, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30193539/peak-v-o-2-is-an-independent-predictor-of-survival-in-patients-with-cardiac-amyloidosis
#2
Selina Hein, Fabian Aus Dem Siepen, Ralf Bauer, Hugo A Katus, Arnt V Kristen
INTRODUCTION: Cardiopulmonary exercise testing (CPET) has repeatedly been reported to reliably predict adverse outcomes in different forms of heart failure. However, it has not been elucidated in detail in cardiac amyloidosis (CA). Therefore, we evaluated the predictive value of CPET parameters in patients with CA regarding disease severity and prediction of mortality. METHODS: Twenty-seven consecutive patients with CA were assessed noninvasively, including electrocardiography, echocardiography, CPET, and laboratory tests...
September 7, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30193533/right-ventricular-involvement-in-transthyretin-amyloidosis
#3
Sandra Arvidsson, Michael Y Henein, Gerhard Wikström, Ole B Suhr, Per Lindqvist
BACKGROUND: The extent of right ventricular (RV) involvement in transthyretin amyloidosis (ATTR) is unknown. OBJECTIVES: This study sought to establish the degree of RV involvement in ATTR amyloidosis, and compare findings with RV involvement in hypertrophic cardiomyopathy (HCM). METHODS: Forty-two patients with ATTR amyloidosis and echocardiographic evidence of cardiac amyloidosis (cardiac ATTR), 19 ATTR patients with normal left ventricular (LV) wall thickness (non-cardiac ATTR), 25 patients with diagnosed HCM and 30 healthy controls were included in this study...
September 7, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30169971/seven-factors-predict-a-delayed-diagnosis-of-cardiac-amyloidosis
#4
Eve Bishop, Emily E Brown, Johana Fajardo, Lili A Barouch, Daniel P Judge, Marc K Halushka
INTRODUCTION: Diagnostic delay of cardiac amyloidosis (CAm) continues to challenge clinicians. We investigated features associated with delay and ascertained if a diagnostic delay had negative implications for the patient. METHODS: We performed a retrospective chart review identifying 82 subjects with biopsy-proven and mass-spectrometry-identified CAm with clinical and epidemiologic data including first potential symptom of amyloidosis. Pathology slides were scored for extent of amyloid...
August 31, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30169969/hereditary-transthyretin-amyloidosis-baseline-characteristics-of-patients-in-the-neuro-ttr-trial
#5
Marcia Waddington-Cruz, Elizabeth J Ackermann, Michael Polydefkis, Stephen B Heitner, Peter J Dyck, Fabio A Barroso, Annabel K Wang, John L Berk, P James B Dyck, Brett P Monia, Steven G Hughes, Li Tai, T Jesse Kwoh, Shiangtung W Jung, Teresa Coelho, Merrill D Benson, Morie A Gertz
BACKGROUND: Hereditary transthyretin (ATTRm) amyloidosis is a rare, progressive and fatal disease with a range of clinical manifestations. OBJECTIVE: This study comprehensively evaluates disease characteristics in a large, diverse cohort of patients with ATTRm amyloidosis. METHODS: Adult patients (N = 172) with Stage 1 or Stage 2 ATTRm amyloidosis who had polyneuropathy were screened and enrolled across 24 investigative sites and 10 countries in the NEURO-TTR trial ( www...
August 31, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30039724/a-case-of-transthyretin-amyloidosis-with-myopathy-neuropathy-and-cardiomyopathy-resulting-from-an-exceedingly-rare-mutation-transthyretin-ala120ser-c-418g-t-p-ala140ser
#6
Kavisha Patel, Clement Tagoe, Phyllis Bieri, Karen Weidenheim, James M Tauras
No abstract text is available yet for this article.
July 24, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30032658/a-library-of-attr-amyloidosis-patient-specific-induced-pluripotent-stem-cells-for-disease-modelling-and-in-vitro-testing-of-novel-therapeutics
#7
Richard M Giadone, Jessica D Rosarda, Prithvi Reddy Akepati, Arianne C Thomas, Batbold Boldbaatar, Marianne F James, Andrew A Wilson, Vaishali Sanchorawala, Lawreen H Connors, John L Berk, R Luke Wiseman, George J Murphy
Hereditary transthyretin amyloidosis (ATTR amyloidosis) is an autosomal dominant protein-folding disorder caused by over 100 distinct mutations in the transthyretin (TTR) gene. In ATTR amyloidosis, protein secreted from the liver aggregates and forms amyloid fibrils in downstream target organs, chiefly the heart and peripheral nervous system. Few animal models of ATTR amyloidosis exist and none recapitulate the multisystem complexity and clinical variability associated with disease pathogenesis in patients...
July 21, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30032653/patient-reported-distress-is-prevalent-in-systemic-light-chain-al-amyloidosis-but-not-determined-by-severity-of-disease
#8
Nicholas L Wright, Kathryn E Flynn, Ruta Brazauskas, Parameswaran Hari, Anita D'Souza
We conducted this retrospective study to assess patient-reported distress in light chain (AL) amyloidosis, using the Distress Thermometer (DT) survey at first evaluation at our center. Of 78 patients who completed the survey, 75 scored their distress (distress: <4 - low, 4-6 - moderate, >6 - high). Moderate and high distress were self-reported by 30% and 17% patients, respectively. More patients with distress lived alone and had lower haemoglobin than patients without. AL stage did not correlate with distress (Stage I/II median DT 4 compared to 3 in Stage III/IV, p = ...
July 21, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30032648/alan-s-cohen-1926-2018
#9
Martha Skinner, Per Westermark
No abstract text is available yet for this article.
July 21, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/30032640/outcomes-of-patients-with-al-amyloidosis-and-low-serum-free-light-chain-levels-at-diagnosis
#10
Vina P Nguyen, Allison Rosenberg, Lisa M Mendelson, Raymond L Comenzo, Cindy Varga, Vaishali Sanchorawala
Serum free light chains (sFLC) are independent prognostic markers of disease in light chain (AL) amyloidosis, and are used in the haematologic response criteria for treatment. However, up to 20% of patients have low sFLCs at diagnosis, with a difference between involved and uninvolved free light chains (dFLC) of less than 50 mg/L, making responses to treatment difficult to evaluate. In order to characterize this distinct subgroup of patients, we retrospectively analyzed 123 AL amyloidosis patients with dFLC <50 mg/L who were diagnosed between 2002 and 2013...
July 21, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29993288/cerebrospinal-fluid-and-vitreous-body-exposure-to-orally-administered-tafamidis-in-hereditary-attrv30m-p-ttrv50m-amyloidosis-patients
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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