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Journals Amyloid : the International Jo...

Amyloid : the International Journal of Experimental and Clinical Investigation

https://read.qxmd.com/read/37755317/intra-familial-variability-of-oculoleptomeningeal-amyloidosis-due-to-the-attr-i107m-c-381t%C3%A2-%C3%A2-g-mutation-diagnostic-challenges-of-a-rare-phenotype
#1
JOURNAL ARTICLE
Monica Alcantara, Vera Bril
No abstract text is available yet for this article.
September 27, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37668548/patient-reported-outcome-measures-for-transthyretin-cardiac-amyloidosis-the-italy-study
#2
JOURNAL ARTICLE
Alberto Aimo, Lucio Teresi, Vincenzo Castiglione, Anna Lisa Picerni, Martina Niccolai, Silvia Severino, Assunta Agazio, Anna Carnevale Baraglia, Laura Obici, Giovanni Palladini, Lucia Ponti, Alessia Argirò, Francesco Cappelli, Federico Perfetto, Matteo Serenelli, Giancarlo Trimarchi, Roberto Licordari, Gianluca Di Bella, Olena Chubuchna, Filippo Quattrone, Sabina Nuti, Sabina De Rosis, Claudio Passino, Claudio Rapezzi, Giampaolo Merlini, Michele Emdin, Giuseppe Vergaro
BACKGROUND: Transthyretin cardiac amyloidosis (ATTR-CA) has a deep impact on the quality of life (QoL), yet no specific patient-reported outcome measures (PROMs) for ATTR-CA exist. METHODS: The ITALY study involved 5 Italian referral centres (Pisa, Pavia, Ferrara, Florence, Messina) enrolling consecutive outpatients with ATTR-CA. RESULTS: Two 30-item questionnaires were created for wild-type (wt) and variant (v) ATTR-CA. Scores ranged from 100 (best condition) to 0 (worst condition)...
September 5, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37599395/reduction-in-99m-tc-dpd-myocardial-uptake-with-therapy-of-attr-cardiomyopathy
#3
JOURNAL ARTICLE
René Rettl, Raffaella Calabretta, Franz Duca, Christina Binder, Christina Kronberger, Robin Willixhofer, Michael Poledniczek, Carolina Donà, Christian Nitsche, Dietrich Beitzke, Christian Loewe, Michaela Auer-Grumbach, Diana Bonderman, Stefan Kastl, Christian Hengstenberg, Roza Badr Eslam, Johannes Kastner, Jutta Bergler-Klein, Marcus Hacker, Andreas Kammerlander
Aims: Novel ribonucleic acid interference (RNAi) therapeutics such as patisiran and inotersen have been shown to benefit neurologic disease course and quality of life in patients with hereditary transthyretin amyloidosis (ATTRv). We aimed to determine the impact of RNAi therapeutics on myocardial amyloid load using quantitative single photon emission computed tomography/computed tomography (SPECT/CT) imaging in patients with ATTRv-related cardiomyopathy (ATTRv-CM). We furthermore compared them with wild-type ATTR-CM (ATTRwt-CM) patients treated with tafamidis...
August 20, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37563759/correction
#4
(no author information available yet)
No abstract text is available yet for this article.
August 10, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37530216/correlation-of-99mtc-dpd-bone-scintigraphy-with-histological-amyloid-load-in-patients-with-attr-cardiac-amyloidosis
#5
JOURNAL ARTICLE
Maria Ungericht, Valeria Groaz, Moritz Messner, Thomas Schuetz, Luca Brunelli, Marc-Michael Zaruba, Daniela Lener, Eva Stocker, Axel Bauer, Alexander Stephan Kroiss, Agnes Mayr, Christoph Röcken, Gerhard Poelzl
BACKGROUND: The significance of measuring 99mTc-labelled-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) in transthyretin (ATTR) cardiac amyloidosis has not been adequately studied. This single-centre observational study evaluated the correlation between 99mTc-DPD scintigraphy and histological amyloid load in endomyocardial biopsy (EMB). METHODS: Twenty-eight patients with biopsy-proven ATTR amyloidosis and concomitantly available 99mTc-DPD scintigraphy were included...
August 2, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37493395/diagnostic-and-prognostic-contribution-of-dpd-scintigraphy-in-transthyretin-v30m-cardiac-amyloidosis
#6
JOURNAL ARTICLE
Maria C Azevedo Coutinho, Nuno Cortez-Dias, Guilhermina Cantinho, Susana Gonçalves, Nelson Cunha, Tiago Rodrigues, Laura Santos, Isabel Conceição, João Agostinho, Fausto J Pinto
BACKGROUND: Early diagnosis and prognostic stratification of cardiac transthyretin amyloidosis are crucial. Although 99m Tc 3,3-diphosphono-1,2-propanedicarboxylic acid (DPD) scintigraphy is the preferred method for the non-invasive diagnosis, its accuracy appears to be limited in transthyretin amyloidosis protein (ATTR) V30M mutation. Furthermore, its prognostic value in this mutation is unknown. This study investigated the diagnostic value of DPD scintigraphy to detect ATTR cardiomyopathy in V30M mutation and explored its prognostic value regarding mortality...
July 26, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37486102/technetium-99m-pyrophosphate-imaging-based-computed-tomography-guided-core-needle-biopsy-of-internal-oblique-muscle-in-wild-type-transthyretin-cardiac-amyloidosis
#7
JOURNAL ARTICLE
Koji Takahashi, Yoshiyasu Hiratsuka, Takaaki Iwamura, Daisuke Sasaki, Nobuhisa Yamamura, Sohei Kitazawa, Mitsuharu Ueda, Hiroe Morioka, Takafumi Okura, Daijiro Enomoto, Shigeki Uemura, Taizo Kono, Tomoki Sakaue, Shuntaro Ikeda
BACKGROUND: Technetium-99m-pyrophosphate (99m Tc-PYP) uptake in the internal oblique muscle (IOM), which is often observed in patients with wild-type transthyretin cardiac amyloidosis (ATTR-CA), indicates amyloid transthyretin (ATTR) deposition. OBJECTIVE: This study aimed to assess the safety and efficacy of 99m Tc-PYP imaging-based computed tomography (CT)-guided core-needle biopsy of the IOM as a new extracardiac screening biopsy for confirming the presence of ATTR deposits...
July 24, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37469249/treatment-response-and-neurofilament-light-chain-levels-with-long-term-patisiran-in-hereditary-transthyretin-mediated-amyloidosis-with-polyneuropathy-24-month-results-of-an-open-label-extension-study
#8
JOURNAL ARTICLE
Simina Ticau, Emre Aldinc, Michael Polydefkis, David Adams, Teresa Coelho, Mitsuharu Ueda, Cecilia Hale, John Vest, Paul Nioi
BACKGROUND: Longitudinal changes in neurofilament light chain (NfL) levels were evaluated alongside prespecified clinical assessments 24 months into the patisiran Global open-label extension (OLE) study in patients with ATTRv amyloidosis with polyneuropathy. METHODS: All patients enrolled in the Global OLE, from phase III APOLLO and phase II OLE parent studies, received patisiran. Assessments included measures of polyneuropathy (modified Neuropathy Impairment Score+7 (mNIS+7)), quality of life (QOL; Norfolk QOL-Diabetic Neuropathy questionnaire (Norfolk QOL-DN)), and plasma NfL...
July 20, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37459334/patients-with-transthyretin-amyloidosis-enrolled-in-thaos-between-2018-and-2021-continue-to-experience-substantial-diagnostic-delay
#9
JOURNAL ARTICLE
Teresa Coelho, Angela Dispenzieri, Martha Grogan, Isabel Conceição, Márcia Waddington-Cruz, Arnt V Kristen, Jonas Wixner, Igor Diemberger, Juan Gonzalez-Moreno, Mathew S Maurer, Violaine Planté-Bordeneuve, Pablo Garcia-Pavia, Ivailo Tournev, Jose Gonzalez-Costello, Eve Cariou, Alejandra González-Duarte, Oliver Glass, Doug Chapman, Leslie Amass
No abstract text is available yet for this article.
July 17, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37449354/rare-variant-collapsing-and-bioinformatic-analyses-for-amyloidosis-dementia-and-parkinson-s-disease-in-the-uk-biobank-reveal-novel-susceptibility-loci
#10
JOURNAL ARTICLE
Bengt Zöller, Eric Manderstedt, Christina Lind-Halldén, Christer Halldén
No abstract text is available yet for this article.
July 14, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37439769/mapping-cellular-response-to-destabilized-transthyretin-reveals-cell-and-amyloidogenic-protein-specific-signatures
#11
JOURNAL ARTICLE
Sabrina Ghosh, Carlos Villacorta-Martin, Jonathan Lindstrom-Vautrin, Devin Kenney, Carly S Golden, Camille V Edwards, Vaishali Sanchorawala, Lawreen H Connors, Richard M Giadone, George J Murphy
BACKGROUND: In ATTR amyloidosis, transthyretin (TTR) protein is secreted from the liver and deposited as toxic aggregates at downstream target tissues. Despite recent advancements in treatments for ATTR amyloidosis, the mechanisms underlying misfolded TTR-mediated cellular damage remain elusive. METHODS: In an effort to define early events of TTR-associated stress, we exposed neuronal (SH-SY5Y) and cardiac (AC16) cells to wild-type and destabilized TTR variants (TTRV122I (p...
July 13, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37431733/a-rare-occurrence-and-near-miss-should-a-ttr-gene-test-be-routinely-performed-for-suspected-attr-cardiomyopathy
#12
JOURNAL ARTICLE
Bharadwaj Satyavolu, Genise Green, Anthony Jake Demetris, Prem Soman
No abstract text is available yet for this article.
July 11, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37431668/human-lysozyme-inhibits-the-fibrillation-of-serum-amyloid-a-protein-from-systemic-aa-amyloidosis
#13
JOURNAL ARTICLE
Tim Moderer, Ioana Puşcalău-Gîrţu, Christian Haupt, Julian Baur, Armando Rodríguez-Alfonso, Sebastian Wiese, Christoph Q Schmidt, Miroslav Malešević, Wolf-Georg Forssmann, Ludger Ständker, Marcus Fändrich
BACKGROUND: Systemic AA amyloidosis is a world-wide occurring protein misfolding disease in humans and animals that arises from the formation of amyloid fibrils from serum amyloid A (SAA) protein and their deposition in multiple organs. OBJECTIVE: To identify new agents that prevent fibril formation from SAA protein and to determine their mode of action. MATERIALS AND METHODS: We used a cell model for the formation of amyloid deposits from SAA protein to screen a library of peptides and small proteins, which were purified from human hemofiltrate...
July 11, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37431662/prevalence-of-amyloid-in-ligamentum-flavum-of-patients-with-lumbar-spinal-stenosis
#14
JOURNAL ARTICLE
Francesco Marchi, Chiara Kessler, Daniela Distefano, Lodovico Terzi di Bergamo, Luca Fumagalli, Manuela Averaimo, Emanuele Crupi, Fabio Bergamini, Giorgia Melli, Georg Stussi, Davide Rossi, Claudio Gobbi, Paolo Ripellino, Emanuele Pravatà, Dominique E Kuhlen, Christoph Röcken, Pietro Scarone, Bernhard Gerber, Adalgisa Condoluci
BACKGROUND: Transthyretin (ATTR) amyloidosis is often diagnosed in an advanced stage, when irreversible cardiac damage has occurred. Lumbar spinal stenosis (LSS) may precede cardiac ATTR amyloidosis by many years, offering the opportunity to detect ATTR already at the time of LSS surgery. We prospectively assessed the prevalence of ATTR in the ligamentum flavum by tissue biopsy in patients aged >50 years undergoing surgery for LSS. METHODS: Ligamentum flavum thickness was assessed pre-operatively on axial T2 magnetic resonance imaging (MRI) slices...
July 11, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37377439/comparison-of-cardiac-involvement-extracardiac-manifestations-and-outcomes-between-homozygote-and-heterozygote-transthyretin%C3%A2-p-val142ile-v122i-variant-in-patients-with-hereditary-transthyretin%C3%A2-amyloidosis-a-cohort-study
#15
JOURNAL ARTICLE
Grégoire Albenque, Mélanie Bézard, Mounira Kharoubi, Shirley Odouard, Ariane Lunati, Elsa Poullot, Amira Zaroui, Emmanuel Teiger, Luc Hittinger, Vincent Audard, Khalil El Karoui, Benoît Funalot, Pascale Fanen, Thibaud Damy, Silvia Oghina
BACKGROUND: Hereditary transthyretin (ATTRv) p.Val142Ile (V122I) mutation is the most common inherited cause of cardiac amyloidosis and little is known about the phenotype and outcome of the rare homozygotic genotype. This study aimed to compare phenotypic characteristics and outcomes between heterozygous and homozygous patients with ATTRv V122I amyloidosis. MATERIAL AND METHODS: This monocentric, observational, retrospective study conducted at the French National Referral Centre for Cardiac Amyloidosis (Henri Mondor Hospital, Créteil), described clinical, electrocardiographic, cardiac imaging features and prognostic data for patients with ATTRv V122I amyloidosis...
June 28, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37353960/attr-and-afib-amyloid-two-different-types-of-amyloid-in-the-annular-ligament-of-trigger-finger
#16
JOURNAL ARTICLE
Christian Treitz, Neelis Müller-Marienburg, Rolf Rüdiger Meliß, Peter Urban, Hans-Detlef Axmann, Frank Siebert, Karsten Becker, Klaus Martens, Hans-Michael Behrens, Eva Gericke, Andreas Tholey, Christoph Röcken
INTRODUCTION: Histological examination of tissue specimens obtained during surgical treatment of trigger finger frequently encountered unclassifiable amyloid deposits in the annular ligament. We systematically explored this unknown type by a comprehensive analysis using histology, immunohistochemistry, and quantitative mass spectrometry-based proteomics. METHODS: 205 tissue specimens of annular ligaments were obtained from 172 patients. Each specimen was studied by histology and immunohistochemistry...
June 23, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37353941/neuropathy-progression-in-hereditary-transthyretin-amyloidosis-attrv-patients-after-liver-transplantation
#17
JOURNAL ARTICLE
Catarina Falcão de Campos, Isabel Conceição
No abstract text is available yet for this article.
June 23, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37318263/how-well-does-liver-span-as-part-of-the-consensus-criteria-for-liver-involvement-in-al-amyloidosis-perform
#18
JOURNAL ARTICLE
Anne F Brunger, Ronald van Rheenen, Reinold O B Gans, Bouke P C Hazenberg, Hans L A Nienhuis
No abstract text is available yet for this article.
June 15, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/37294189/immunoglobulin-light-chain-amyloidosis-mimicking-bulbar-amyotrophic-lateral-sclerosis
#19
JOURNAL ARTICLE
Antoine Pegat, Emilien Bernard
No abstract text is available yet for this article.
June 9, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36519495/quantitative-sensory-testing-a-good-tool-to-identify-subclinical-neuropathy-in-attrv30m-amyloidosis-patients
#20
JOURNAL ARTICLE
Isabel Conceição, Isabel de Castro, Andrés Diaz, José Castro
BACKGROUND: Quantitative sensory testing (QST) has been one of the neurophysiological tools used for follow-up and disease progression assessment in ATTRv amyloidosis. We aimed to detect the utility of QST in identifying subclinical neuropathic involvement in ATTRV30M amyloidosis carriers. METHODS: A cohort of ATTRV30M amyloidosis carriers were assessed with vibratory (VDT) and cooling (CDT) detection thresholds and heat pain responses. Subjects were divided into asymptomatic carriers (Group 1), paucisymptomatic carriers (Group 2) and stage 1 ATTRv-PN patients (Group 3)...
June 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
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