journal
MENU ▼
Read by QxMD icon Read
search

Amyloid: the International Journal of Experimental and Clinical Investigation

journal
https://www.readbyqxmd.com/read/27647161/identification-of-prognostic-markers-in-transthyretin-and-al-cardiac-amyloidosis
#1
Thibaud Damy, Arnaud Jaccard, Aziz Guellich, David Lavergne, Arnault Galat, Jean-François Deux, Luc Hittinger, Jehan Dupuis, Valérie Frenkel, Charlotte Rigaud, Violaine Plante-Bordeneuve, Diane Bodez, Dania Mohty
BACKGROUND: The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. AIMS: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers. METHODS AND RESULTS: Observational study...
September 20, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27646980/hereditary-amyloidosis-with-cardiomyopathy-caused-by-the-novel-variant-transthyretin-a36d
#2
Taro Yamashita, Mitsuharu Ueda, Nobuyuki Saga, Kiyonori Nanto, Masayoshi Tasaki, Teruaki Masuda, Yohei Misumi, Seitaro Oda, Akiko Fujimoto, Tomoko Amano, Kotaro Takamatsu, Satoshi Yamashita, Konen Obayashi, Hirotaka Matsui, Yukio Ando
No abstract text is available yet for this article.
September 20, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27884064/amyloid-fibril-proteins-and-amyloidosis-chemical-identification-and-clinical-classification-international-society-of-amyloidosis-2016-nomenclature-guidelines
#3
Jean D Sipe, Merrill D Benson, Joel N Buxbaum, Shu-Ichi Ikeda, Giampaolo Merlini, Maria J M Saraiva, Per Westermark
The Nomenclature Committee of the International Society of Amyloidosis (ISA) met during the XVth Symposium of the Society, 3 July-7 July 2016, Uppsala, Sweden, to assess and formulate recommendations for nomenclature for amyloid fibril proteins and the clinical classification of the amyloidoses. An amyloid fibril must exhibit affinity for Congo red and with green, yellow or orange birefringence when the Congo red-stained deposits are viewed with polarized light. While congophilia and birefringence remain the gold standard for demonstration of amyloid deposits, new staining and imaging techniques are proving useful...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27884058/efficiency-of-silencing-rna-for-removal-of-transthyretin-v30m-in-a-ttr-leptomeningeal-animal-model
#4
Paula Gonçalves, Helena Martins, Susete Costelha, Luis F Maia, Maria Joao Saraiva
Some TTR mutants target the central nervous system (CNS). Familial amyloid polyneuropathy (FAP) with leptomeningeal involvement has been described in 9% of transthyretin (TTR) mutations and in valine for methionine at position 30 (V30M) patients. These individuals present dementia, ataxia, brain hemorrhages and focal neurological episodes (FNEs). FNEs occurred also in V30M FAP patients with longer disease duration, who have undergone liver transplant to remove the source of plasma mutant TTR as a form of treatment...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27879149/increasing-amount-of-amyloid-are-associated-with-the-severity-of-clinical-features-in-hereditary-gelsolin-agel-amyloidosis
#5
Tiia Pihlamaa, Sinikka Suominen, Sari Kiuru-Enari, Maarit Tanskanen
BACKGROUND: Patients with hereditary gelsolin (AGel) amyloidosis (HGA) present with hanging skin (cutis laxa) and bilateral cranial neuropathy, and require symptomatic plastic surgery. Our clinical observation of tissue fragility prompted us to design a prospective study. METHODS: Twenty-nine patients with HGA undergoing surgery were interviewed and clinically examined. The height and thickness of skin folds in standard anatomical localizations were measured. The presence and distribution of amyloid in skin samples were analyzed using Congo red staining and immunohistochemistry using antibodies against gelsolin amyloid (AGel) subunit...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27879147/pre-transplantation-novel-agent-induction-predicts-progression-free-survival-for-patients-with-immunoglobulin-light-chain-amyloidosis-undergoing-high-dose-melphalan-and-autologous-stem-cell-transplantation
#6
Andrew J Cowan, Zandra K Klippel, Philip A Stevenson, Teresa S Hyun, Sherilyn Tuazon, Pamela S Becker, Damian J Green, Leona A Holmberg, David G Coffey, Ajay K Gopal, Edward N Libby
INTRODUCTION: High-dose melphalan and autologous stem cell transplantation (HDM/SCT) is an effective treatment modality for immunoglobulin light-chain (AL) amyloidosis; however, its application remains restricted to patients with good performance status and limited organ involvement. In recent years, the paradigm for AL amyloidosis has changed with the introduction of novel agents such as immunomodulatory drugs (IMiDs) and proteasome inhibitors (PIs). We hypothesized that use of novel agent induction regimens has improved outcomes for patients with AL amyloidosis undergoing HDM/SCT at our center...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27809600/plasma-hepatocyte-growth-factor-is-a-novel-marker-of-al-cardiac-amyloidosis
#7
Kristopher J Swiger, Eitan A Friedman, Evan L Brittain, Kelsey A Tomasek, Shi Huang, Yan R Su, Douglas B Sawyer, Daniel J Lenihan
BACKGROUND: Cardiac amyloidosis is an infiltrative cardiomyopathy that is challenging to diagnose. We hypothesized that the novel biomarkers hepatocyte growth factor (HGF), galectin-3 (GAL-3), interleukin-6 (IL-6), and vascular endothelial growth factor (VEGF) would be elevated in cardiac amyloidosis and may be able to discriminate from non-cardiac systemic amyloidosis or other cardiomyopathies with similar clinical or morphologic characteristics. METHODS: Patients were selected from the Vanderbilt Main Heart Registry according to the following groups: (1) amyloid light-chain (AL) cardiac amyloidosis (n = 26); (2) transthyretin (ATTR) cardiac amyloidosis (n = 7); (3) left ventricular hypertrophy (LVH) (n = 45); (4) systolic heart failure (n = 42); and (5) non-cardiac systemic amyloidosis (n = 7)...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27791396/citrullination-and-deamidation-affect-aggregation-properties-of-amyloid-%C3%AE-proteins
#8
Dai Osaki, Hirotsugu Hiramatsu
Citrullination and deamidation, which are aging-related posttranslational modifications, increase the number of negative charges on amyloid β-protein (Aβ) at neutral pH. We investigated the effects of these modifications on the fibrillation properties of Aβ. The Arg5→Cit modification of Aβ1-40 did not affect the fibrillation rate, and brought β-sheet structures unlike that in the Aβ1-40 fibril. The Asn27→Asp modification of Aβ1-40 stopped the fibrillation and induced the formation of aggregates that involved an anti-parallel β-sheet...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27791393/long-term-follow-up-on-tocilizumab-treatment-of-aa-amyloidosis-secondary-to-polyarteritis-nodosa
#9
Jaka Ostrovršnik, Alojzija Hočevar, Boris Lestan, Snežna Sodin Šemrl, Katja Lakota, Matija Tomšič
No abstract text is available yet for this article.
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27748624/tafamidis-in-hereditary-attr-amyloidosis-our-experience-on-monitoring-the-ocular-manifestations
#10
Inês Casal, Sílvia Monteiro, João Melo Beirão
No abstract text is available yet for this article.
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27682970/capillary-electrophoresis-immunosubtraction-as-a-better-alternative-to-immunofixation-for-detecting-and-immunotyping-serum-monoclonal-proteins-in-patients-with-immunoglobulin-light-chain-al-amyloidosis
#11
Kanji Miyazaki, Kenshi Suzuki
Capillary electrophoresis/immunosubtraction (CE/IS) is a simple method for detecting and immunotyping serum or urine monoclonal proteins. To our knowledge, there are no previous reports of the use of CE/IS for characterizing patients with Immunoglobulin light chain (AL) amyloidosis, and there are no convincing data available to compare CE/IS with serum immunofixation electrophoresis (IFE) and free light chain (FLC) assay. The aim of this study was to evaluate the clinical utility of CE/IS in patients with AL amyloidosis as a diagnostic accuracy study...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27677679/immunoglobulin-heavy-light-chain-test-quantifies-clonal-disease-in-patients-with-al-amyloidosis-and-normal-serum-free-light-chain-ratio
#12
Tatiana Prokaeva, Brian Spencer, Fangui Sun, Richard M O'Hara, David C Seldin, Lawreen H Connors, Vaishali Sanchorawala
BACKGROUND: Serum and urine immunofixation electrophoreses (SIFE/UIFE) are used for clonal detection in plasma cell dyscrasias, while serum free light chain (sFLC) testing provides quantitation of clonal disease. Up to 20% of patients with light chain (AL) amyloidosis may present with normal FLC ratio (FLCr). METHODS: We assessed the diagnostic, quantitative and prognostic potential of serum heavy light chain ratio (HLCr) in 199 untreated patients at initial evaluation...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27494299/early-intervention-with-tafamidis-provides-long-term-5-5-year-delay-of-neurologic-progression-in-transthyretin-hereditary-amyloid-polyneuropathy
#13
Márcia Waddington Cruz, Leslie Amass, Denis Keohane, Jeffrey Schwartz, Huihua Li, Balarama Gundapaneni
: Transthyretin hereditary amyloid polyneuropathy, also traditionally known as transthyretin familial amyloid polyneuropathy (ATTR-FAP), is a rare, relentless, fatal hereditary disorder. Tafamidis, an oral, non-NSAID, highly specific transthyretin stabilizer, demonstrated safety and efficacy in slowing neuropathy progression in early-stage ATTRV30M-FAP in a 1.5-year, randomized, double-blind, placebo-controlled trial, and 1-year open-label extension study, with a second long-term open-label extension study ongoing...
August 5, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27494229/2a4-binds-soluble-and-insoluble-light-chain-aggregates-from-al-amyloidosis-patients-and-promotes-clearance-of-amyloid-deposits-by-phagocytosis-%C3%A2
#14
Mark Renz, Ronald Torres, Philip J Dolan, Stephen J Tam, Jose R Tapia, Lauri Li, Joshua R Salmans, Robin M Barbour, Paul J Shughrue, Tarlochan Nijjar, Dale Schenk, Gene G Kinney, Wagner Zago
Amyloid light chain (AL) amyloidosis is characterized by misfolded light chain (LC) (amyloid) deposition in various peripheral organs, leading to progressive dysfunction and death. There are no regulatory agency-approved treatments for AL amyloidosis, and none of the available standard of care approaches directly targets the LC protein that constitutes the amyloid. NEOD001, currently in late-stage clinical trials, is a conformation-specific, anti-LC antibody designed to specifically target misfolded LC aggregates and promote phagocytic clearance of AL amyloid deposits...
August 5, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27470486/incidence-rate-of-amyloidosis-in-patients-from-a-medical-care-program-in-buenos-aires-argentina-a-prospective-cohort
#15
María Adela Aguirre, Bruno Rafael Boietti, Elsa Nucifora, Patricia Beatriz Sorroche, Fernán González Bernaldo de Quirós, Diego Hernan Giunta, María Lourdes Posadas-Martínez
BACKGROUND: There are limited data concerning the incidence density (ID) of ATTRwt, AL and AA amyloidosis in the Argentinean population. Our aim was to estimate the ID of ATTRwt, AL and AA amyloidosis at the Hospital Italiano Medical Care Program in Buenos Aires, Argentina. METHODS: Population was all members of a hospital-based health maintenance organization who were affiliated since January 2006 to December 2014. Each person was followed contributing time at risk since January 2006 or enrollment date to the final date...
July 28, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27460276/depression-and-anxiety-in-patients-with-al-amyloidosis-as-assessed-by-the-sf-36-questionnaire-experience-in-1226-patients
#16
Janet Shu, Stephen Lo, Margot Phillips, Fangui Sun, David C Seldin, Isidore Berenbaum, John L Berk, Vaishali Sanchorawala
BACKGROUND: Our study examines depression and anxiety in patients with immunoglobulin light chain (AL) amyloidosis, and determines the associations between the mental health problems and patient characteristics (age, gender, race, marital status, alcohol consumption, smoking status and cardiac involvement). METHODS: Patients with AL amyloidosis who completed the 36-item Short Form General Health Survey (SF-36) during initial evaluation at a single center were studied...
July 27, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27355239/suppressing-transthyretin-production-in-mice-monkeys-and-humans-using-2nd-generation-antisense-oligonucleotides
#17
Elizabeth J Ackermann, Shuling Guo, Merrill D Benson, Sheri Booten, Sue Freier, Steven G Hughes, Tae-Won Kim, T Jesse Kwoh, John Matson, Dan Norris, Rosie Yu, Andy Watt, Brett P Monia
Transthyretin amyloidosis (ATTR amyloidosis) is a rare disease that results from the deposition of misfolded transthyretin (TTR) protein from the plasma into tissues as amyloid fibrils, leading to polyneuropathy and cardiomyopathy. IONIS-TTRRx (ISIS 420915) is a 2nd-Generation 2'-O-(2-methoxyethyl) modified "2'-MOE" antisense oligonucleotide (ASO) that targets the TTR RNA transcript and reduces the levels of the TTR transcript through an RNaseH1 mechanism of action, leading to reductions in both mutant and wild-type TTR protein...
June 29, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27348696/prognostic-value-of-right-ventricular-systolic-function-in-cardiac-amyloidosis
#18
Diane Bodez, Julien Ternacle, Aziz Guellich, Arnault Galat, Pascal Lim, Costin Radu, Soulef Guendouz, Eric Bergoend, Jean-Paul Couetil, Luc Hittinger, Jean-Luc Dubois-Randé, Violaine Plante-Bordeneuve, Jean-François Deux, Dania Mohty, Thibaud Damy
BACKGROUND: Right ventricular (RV) dysfunction is a strong predictor of poor outcomes in heart failure. Its prognostic meaning in cardiac amyloidosis (CA) is under-investigated. METHODS: Hundred and twenty nine patients with suspected CA and an interventricular septum thickness (IVST) ≥ 12 mm underwent echocardiography with measurement of left ventricular (LV) and RV longitudinal strain (LS), late gadolinium-enhancement (LGE) cardiac MRI, and standard evaluation...
June 27, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27132767/abnormal-stress-echocardiography-findings-in-cardiac-amyloidosis
#19
Kevin C Ong, J Wells Askew, Angela Dispenzieri, Joseph J Maleszewski, Kyle W Klarich, Nandan S Anavekar, Sharon L Mulvagh, Martha Grogan
BACKGROUND: Cardiac involvement in immunoglobulin light chain (amyloid light chain, AL) amyloidosis is characterized by myocardial interstitial deposition but can also cause obstructive deposits in the coronary microvasculature. METHODS: We retrospectively identified 20 patients who underwent stress echocardiography within 1 year prior to the histologic diagnosis of AL amyloidosis. Only patients with cardiac amyloidosis and no known obstructive coronary disease were included...
June 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27097047/structural-requirements-of-glycosaminoglycans-for-facilitating-amyloid-fibril-formation-of-human-serum-amyloid-a
#20
Hiroka Takase, Masafumi Tanaka, Aki Yamamoto, Shiori Watanabe, Sanae Takahashi, Satomi Nadanaka, Hiroshi Kitagawa, Toshiyuki Yamada, Takahiro Mukai
Serum amyloid A (SAA) is a precursor protein of amyloid fibrils. Given that heparan sulfate (HS), a glycosaminoglycan (GAG), is detected in amyloid deposits, it has been suggested that GAG is a key component of amyloid fibril formation. We previously reported that heparin (an analog of HS) facilitates the fibril formation of SAA, but the structural requirements remain unknown. In the present study, we investigated the structural requirements of GAGs for facilitating the amyloid fibril formation of SAA. Spectroscopic analyses using structurally diverse GAG analogs suggested that the fibril formation of SAA was facilitated irrespective of the backbone structure of GAGs; however, the facilitating effect was strongly correlated with the degree of sulfation...
June 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
journal
journal
31990
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"