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Journals Amyloid : the International Jo...

Amyloid : the International Journal of Experimental and Clinical Investigation

https://read.qxmd.com/read/36988111/echocardiographic-findings-in-subjects-with-an-amyloidogenic-apolipoprotein-a1-pathogenic-variant
#1
JOURNAL ARTICLE
Daniela Tomasoni, Alberto Aimo, Marianna Adamo, Matilde Nardi, Carlo Mario Lombardi, Valentina Regazzoni, Maria Grazia De Angelis, Iacopo Fabiani, Giampaolo Merlini, Roberta Mussinelli, Laura Obici, Giorgia Panichella, Giuseppe Vergaro, Claudio Passino, Francesco Scolari, Stefano Perlini, Michele Emdin, Marco Metra
BACKGROUND: Very small case series of patients with apolipoprotein A1 (ApoA1) amyloidosis are available. METHODS: We described the clinical and echocardiographic characteristics of individuals with the pathogenic APOA1 variant Leu75Pro (p. Leu99Pro), referred for cardiac screening. RESULTS: We enrolled 189 subjects, 54% men, median age 55 years (interquartile range 42-67), 39% with concomitant renal disease and 31% with liver disease...
March 29, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36947059/decreased-expression-of-s100a8-a9-in-v30m-related-attrv-amyloidosis
#2
JOURNAL ARTICLE
João Moreira, Sofia Martins, Margarida Saraiva, Maria João Saraiva
INTRODUCTION: Hereditary Transthyretin Amyloidosis is a rare, progressive and life-threatening systemic disease with predominant peripheral and autonomic nervous system involvement caused by mutation of the transthyretin protein. The most common TTR mutation regarding to ATTRv is a substitution of a Methionine for a Valine at position 30 that predisposes TTR to form aggregates and fibrils. METHODS: S100A8 protein levels were measured in plasma samples from ATTRV30M patients and healthy donors...
March 22, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36919584/a-novel-apoa1-frameshift-mutation-glu120glyfs-60-with-upper-gastrointestinal-involvement-and-an-indolent-course
#3
JOURNAL ARTICLE
Eli Muchtar, Surendra Dasari, Jason D Theis, Laura Ongie, Huong T Cabral, Ellen D McPhail, Angela Dispenzieri, Morie A Gertz, Karen L Rech
No abstract text is available yet for this article.
March 15, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36908191/neurofilament-light-chain-as-a-biomarker-for-monitoring-response-to-change-in-treatment-in-hereditary-attr-amyloidosis
#4
JOURNAL ARTICLE
Mitsuto Sato, Yusuke Mochizuki, Yusuke Takahashi, Ken Takasone, Emre Aldinc, Simina Ticau, Gang Jia, Yoshiki Sekijima
No abstract text is available yet for this article.
March 12, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36994840/a-multicentric-study-of-the-disease-risks-and-first-manifestations-in-hereditary-transthyretin-amyloidosis-attrv-insights-for-an-earlier-diagnosis
#5
JOURNAL ARTICLE
Violaine Planté-Bordeneuve, Farida Gorram, Malin Olsson, Intissar Anan, Anna Mazzeo, Luca Gentile, Eugenia Cisneros-Barroso, Juan Gonzalez-Moreno, Ines Losada, Marcia Waddington-Cruz, Luiz Felipe Pinto, Yeşim Parman, Pascale Fanen, Flora Alarcon, Gregory Nuel
BACKGROUND: In hereditary transthyretin amyloidosis (ATTRv), early manifestation and age at onset (AO) may vary strikingly. We assessed the disease'risk (penetrance), AO and initial features in ATTRv families to gain insights on the early disease presentation. METHODS: Genealogical information, AO and first disease manifestations were collected in ATTRv families, from Sweden, Italy (Sicily), Spain (Mallorca), France, Turkey, Brazil. Penetrance was computed using a non-parametric survival method...
February 21, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36795075/steep-increase-in-the-number-of-transthyretin-positive-cardiac-biopsy-cases-in-japan-evidence-obtained-by-the-nation-wide-pathology-consultation-for-the-typing-diagnosis-of-amyloidosis
#6
JOURNAL ARTICLE
Hironobu Naiki, Aina Yamaguchi, Yoshiki Sekijima, Mitsuharu Ueda, Kenichi Ohashi, Kinta Hatakeyama, Yoshihiko Ikeda, Yoshinobu Hoshii, Yukako Shintani-Domoto, Aya Miyagawa-Hayashino, Hanako Tsujikawa, Jin Endo, Tomio Arai, Yukio Ando
BACKGROUND: In 2019, 2020 and 2022, the Japanese Government approved the use of tafamidis and two technetium-scintigraphies for transthyretin amyloid (ATTR) cardiomyopathy, and announced the patient criteria for tafamidis therapy. In 2018, we had started a nation-wide pathology consultation of amyloidosis. OBJECTIVE: To reveal the impact of approval of tafamidis and technetium-scintigraphy on the diagnosis of ATTR cardiomyopathy. METHODS: Ten institutes participated in this study on the pathology consultation of amyloidosis and shared rabbit polyclonal anti-κ116-133 , anti-λ118-134 , and anti-transthyretin115-124 antibodies...
February 16, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36795029/comparison-between-tafamidis-and-liver-transplantation-as-first-line-therapy-for-hereditary-transthyretin-amyloidosis
#7
JOURNAL ARTICLE
Pierre Socie, Anouar Benmalek, Cécile Cauquil, Eve Piekarski, Ilias Kounis, Ludivine Eliahou, Antoine Rousseau, François Rouzet, Andoni Echaniz-Laguna, Didier Samuel, David Adams, Michel S Slama, Vincent Algalarrondo
BACKGROUND: By stabilizing transthyretin, tafamidis delays progression of amyloidosis due to transthyretin variant (ATTRv) and replaced liver transplantation (LT) as the first-line therapy. No study compared these two therapeutic strategies. METHODS: In a monocentric retrospective cohort analysis, patients with ATTRv amyloidosis treated with either tafamidis or LT were compared using a propensity score and a competing risk analysis for three endpoints: all-cause mortality, cardiac worsening (heart failure or cardiovascular death) and neurological worsening (worsening in PolyNeuropathy Disability score)...
February 16, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36779691/impact-of-cytogenetic-abnormalities-on-treatment-outcomes-in-patients-with-amyloid-light-chain-amyloidosis-subanalyses-from-the-andromeda-study
#8
JOURNAL ARTICLE
Shaji Kumar, Angela Dispenzieri, Divaya Bhutani, Morie Gertz, Ashutosh Wechalekar, Giovanni Palladini, Raymond Comenzo, Rafael Fonseca, Arnaud Jaccard, Efstathios Kastritis, Stefan Schönland, Charles la Porte, Huiling Pei, NamPhuong Tran, Giampaolo Merlini
BACKGROUND: Cytogenetic abnormalities are common in patients with amyloid light-chain (AL) amyloidosis; some are associated with poorer outcomes. This post hoc analysis of ANDROMEDA evaluated the impact of certain cytogenetic abnormalities on outcomes in this patient population. METHODS: Patients with newly diagnosed AL amyloidosis were randomised 1:1 to daratumumab, bortezomib, cyclophosphamide, and dexamethasone (D-VCd) or VCd. Outcomes were evaluated in the intent-to-treat (ITT) population and in patients with t(11;14), amp1q21, del13q14, and del17p13...
February 13, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36718932/factors-affecting-the-accuracy-of-amyloidosis-identification-and-referral-to-a-specialty-centre
#9
JOURNAL ARTICLE
Andrew Staron, Lisa M Mendelson, Tracy Joshi, Frederick L Ruberg, Vaishali Sanchorawala
OBJECTIVE: Diagnostic algorithms for amyloidosis have evolved over the past decade, particularly with the incorporation of imaging-based techniques to detect amyloid cardiomyopathy. We sought to identify the key sources of amyloidosis misidentification in the community, which lead to false positive referrals to a tertiary centre. METHODS: We conducted a retrospective review of all referrals to the Amyloidosis Centre from 2010 to 2021 and identified cases lacking amyloid pathology upon final adjudication after extensive assessment at the centre...
January 31, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36691999/tafamidis-concentration-required-for-transthyretin-stabilisation-in-cerebrospinal-fluid
#10
JOURNAL ARTICLE
Felix J Tsai, Marcus Jaeger, Teresa Coelho, Evan T Powers, Jeffery W Kelly
BACKGROUND: Hereditary transthyretin (TTR) amyloidosis (ATTRv) initially presents as a polyneuropathy and/or a cardiomyopathy. Central nervous system (CNS) pathology in ATTRv amyloidosis, including focal neurological episodes, dementia, cerebrovascular bleeding, and seizures, appears around a decade later. Wild-type (WT) TTR amyloidosis (ATTRwt) causes a cardiomyopathy. CNS pathology risk likely also increases in these patients as cardiomyopathy progresses. Herein, we study tafamidis-mediated TTR kinetic stabilisation in cerebrospinal fluid (CSF)...
January 24, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36661201/apolipoprotein-a-iv-amyloidosis-in-a-cotton-top-tamarin-saguinus-oedipus
#11
JOURNAL ARTICLE
Niki Sedghi Masoud, Susumu Iwaide, Yoshiyuki Itoh, Miki Hisada, Yumi Une, Tomoaki Murakami
No abstract text is available yet for this article.
January 20, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36655505/obituary
#12
JOURNAL ARTICLE
Stefano Perlini
No abstract text is available yet for this article.
January 19, 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36989412/obituary
#13
JOURNAL ARTICLE
Stefano Perlini
No abstract text is available yet for this article.
March 2023: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36541892/collagen-inhibits-phagocytosis-of-amyloid-in%C3%A2-vitro-and-in%C3%A2-vivo-and-may-act-as-a-don-t-eat-me-signal
#14
JOURNAL ARTICLE
Joseph W Jackson, James S Foster, Emily B Martin, Sallie Macy, Craig Wooliver, Manasi Balachandran, Tina Richey, R Eric Heidel, Angela D Williams, Stephen J Kennel, Jonathan S Wall
BACKGROUND: Systemic amyloidosis refers to a group of protein misfolding disorders characterized by the extracellular deposition of amyloid fibrils in organs and tissues. For reasons heretofore unknown, amyloid deposits are not recognized by the immune system, and progressive deposition leads to organ dysfunction. METHODS: In vitro and in vivo phagocytosis assays were performed to elucidate the impact of collagen and other amyloid associated proteins (eg serum amyloid p component and apolipoprotein E) had on amyloid phagocytosis...
December 21, 2022: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36541785/cardiac-amyloidosis-remains-significantly-underdiagnosed-in-patients-undergoing-tavr-analysis-of-national-inpatient-sample
#15
JOURNAL ARTICLE
Dimitrios Terentes-Printzios, Alexios S Antonopoulos, Mohamed Omer, Ioannis Panagiotopoulos, Konstantinos Toutouzas, Konstantinos Tsioufis, Islam Elgendy, Charalambos Vlachopoulos
No abstract text is available yet for this article.
December 21, 2022: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36533960/the-impact-of-post-transplant-doxycycline-in-al-amyloidosis-updated-results-after-long-term-follow-up
#16
JOURNAL ARTICLE
Nadine Abdallah, Angela Dispenzieri, Eli Muchtar, Francis K Buadi, Prashant Kapoor, Martha Q Lacy, Yi L Hwa, Amie Fonder, Miriam A Hobbs, Suzanne R Hayman, Nelson Leung, David Dingli, Ronald S Go, Yi Lin, Wilson I Gonsalves, Moritz Binder, Taxiarchis Kourelis, Rahma Warsame, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz, Shaji K Kumar
INTRODUCTION: The current treatment paradigm of AL amyloidosis lacks effective fibril-directed therapies. Doxycycline has been shown to have anti-fibril properties in preclinical models. In 2012, we reported that posttransplant prophylaxis with doxycycline was associated with improved survival compared to penicillin in patients with haematologic response. We provide here updated results after long-term follow up. METHODS: We included 553 patients who underwent transplant between July 24th , 1996, and June 24th , 2014...
December 19, 2022: 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
#17
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)...
December 15, 2022: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36495239/macrophages-in-the-reticuloendothelial-system-inhibit-early-induction-stages-of-mouse-apolipoprotein-a-ii-amyloidosis
#18
JOURNAL ARTICLE
Hiroki Miyahara, Jian Dai, Ying Li, Xiaoran Cui, Hibiki Takeuchi, Naomi Hachiya, Fuyuki Kametani, Masahide Yazaki, Masayuki Mori, Keiichi Higuchi
Amyloidosis refers to a group of degenerative diseases that are characterized by the deposition of misfolded protein fibrils in various organs. Deposited amyloid may be removed by a phagocyte-dependent innate immune system; however, the precise mechanisms during disease progression remain unclear. We herein investigated the properties of macrophages that contribute to amyloid degradation and disease progression using inducible apolipoprotein A-II amyloidosis model mice. Intravenously injected AApoAII amyloid was efficiently engulfed by reticuloendothelial macrophages in the liver and spleen and disappeared by 24 h...
December 10, 2022: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36476295/a-second-case-of-liraglutide-type-localised-amyloidosis
#19
JOURNAL ARTICLE
Sara Muhammad, Ellen D McPhail, W Oliver Tobin, Surendra Dasari, Jason Theis, Julie A Vrana, Elie Naddaf
No abstract text is available yet for this article.
December 8, 2022: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/36420821/amyloid-nomenclature-2022-update-novel-proteins-and-recommendations-by-the-international-society-of-amyloidosis-isa-nomenclature-committee
#20
JOURNAL ARTICLE
Joel N Buxbaum, Angela Dispenzieri, David S Eisenberg, Marcus Fändrich, Giampaolo Merlini, Maria J M Saraiva, Yoshiki Sekijima, Per Westermark
The Nomenclature Committee of the International Society of Amyloidosis met at the XVIII International Symposium on Amyloidosis in September and virtually in October 2022 with discussions resulting in this upgraded nomenclature recommendation. The nomenclature principles remain unchanged but there is an ongoing discussion regarding the importance and varying nature of intracellular protein aggregates, particularly those associated with neurodegenerative diseases. Six novel proteins were added to the list of human amyloid fibril proteins...
December 2022: Amyloid: the International Journal of Experimental and Clinical Investigation
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