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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://read.qxmd.com/read/30931628/regional-amyloid-distribution-and-impact-on-mortality-in-light-chain-amyloidosis-a-t1-mapping-cardiac-magnetic-resonance-study
#1
Ke Wan, Weihao Li, Jiayu Sun, Yuanwei Xu, Jie Wang, Hong Liu, Yang Dong, Wei Cheng, Qing Zhang, Zhi Zeng, Xiaoyue Zhou, Yuchi Han, Yucheng Chen
BACKGROUND: T1 mapping allows quantitative assessment of "diffuse" deposition of amyloid protein in the myocardium. Early detection of cardiac involvement and potential prognostic improvement could benefit patients with AL amyloidosis. OBJECTIVES: This study aims to evaluate the regional variation of amyloid infiltration in the left ventricle and the prognostic value of T1 mapping in patients with AL amyloidosis. METHODS: We prospectively enrolled 77 patients with AL amyloidosis who underwent cardiac magnetic resonance on a 3...
April 1, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30929476/lipid-membranes-accelerate-amyloid-formation-in-the-mouse-model-of-aa-amyloidosis
#2
Aida Vahdat Shariat Panahi, Per Hultman, Karin Öllinger, Gunilla T Westermark, Katarzyna Lundmark
INTRODUCTION: AA amyloidosis develops as a result of prolonged inflammation and is characterized by deposits of N-terminal proteolytic fragments of the acute phase reactant serum amyloid A (SAA). Macrophages are usually found adjacent to amyloid, suggesting their involvement in the formation and/or degradation of the amyloid fibrils. Furthermore, accumulating evidence suggests that lipid membranes accelerate the fibrillation of different amyloid proteins. METHODS: Using an experimental mouse model of AA amyloidosis, we compared the amyloidogenic effect of liposomes and/or amyloid-enhancing factor (AEF)...
March 30, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30907141/treatment-of-hereditary-and-acquired-forms-of-transthyretin-amyloidosis-in-the-era-of-personalized-medicine-the-role-of-randomized-controlled-trials
#3
Joel N Buxbaum
There have now been randomized controlled trials of four different therapeutics for hereditary amyloid polyneuropathy related to transthyretin (TTR) deposition and one for amyloidotic cardiomyopathy of both genetic and sporadic origin. It is likely that in the next few months those not already approved by either the US Food and Drug Administration (FDA) and/or the European Medicines Authority (EMA) will receive similar approvals for treatment for all or particular groups of patients. This is a far cry from circumstances less than 10 years ago when the only available therapy was gene replacement by liver transplant...
March 24, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30806514/commentary-to-isabel-concei%C3%A3-%C3%A3-o-et-al-early-diagnosis-through-targeted-follow-up-of-identified-carriers-of-ttr-gene-mutations
#4
Ole B Suhr
No abstract text is available yet for this article.
February 26, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30793974/early-diagnosis-of-attr-amyloidosis-through-targeted-follow-up-of-identified-carriers-of-ttr-gene-mutations
#5
Isabel Conceição, Thibaud Damy, Manuel Romero, Lucía Galán, Shahram Attarian, Marco Luigetti, Menachem Sadeh, Stayko Sarafov, Ivailo Tournev, Mitsuharu Ueda
Diagnosis in the early stages of hereditary transthyretin (ATTR) amyloidosis is imperative to support timely treatment to prevent or delay disease progression. Genetic testing in the setting of genetic counselling enables identification of carriers of a TTR gene mutation who are therefore at risk of developing TTR-associated disease. Knowledge of different genotypes and how they manifest in symptomatic disease should facilitate development of a structured and targeted approach to enable diagnosis of symptomatic disease in ATTR amyloidosis mutation carriers on the first manifestation of the earliest detectable sign or symptom...
February 22, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30739503/cell-assay-for-the-identification-of-amyloid-inhibitors-in-systemic-aa-amyloidosis
#6
Ioana Puscalau-Girtu, Judith S Scheller, Stephanie Claus, Marcus Fändrich
Systemic AA amyloidosis is still, up to this day, a life-threatening complication of chronic inflammatory diseases. Despite the success of anti-inflammatory treatment, the prognosis of some AA patients is still poor, which is why therapies directed at the amyloidogenic pathway in AA amyloidosis are being sought after. The cell culture model of amyloid formation from serum amyloid A1 (SAA1) protein remodels crucial features of AA amyloid deposit formation in vivo. We here demonstrate how the cell model can be utilized for the identification of compounds with amyloid inhibitory activity...
February 9, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30688105/electrophysiological-demyelinating-features-in-hereditary-attr-amyloidosis
#7
Nobuhiko Ohashi, Minori Kodaira, Hiroshi Morita, Yoshiki Sekijima
OBJECTIVE: To elucidate the electrophysiological demyelinating features in patients with hereditary ATTR amyloidosis that may lead to a misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: In 102 patients with hereditary ATTR amyloidosis (85 Val30Met and 17 non-Val30Met; 37 and 65 from endemic and non-endemic areas, respectively), results of motor nerve conduction studies (MNCSs) with a 2-Hz low-cut filter in the unilateral ulnar and tibial nerves were retrospectively investigated to assess whether each MNCS parameter demonstrated demyelinating features that fulfil the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic (EFNS/PNS EDX) criteria for CIDP...
January 26, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30686043/hereditary-systemic-amyloidosis-caused-by-k19t-apolipoprotein-c-ii-variant
#8
Konstantinos Liapis, Panagiota Panagopoulou, Evangelia Charitaki, Dorota Rowczenio, Janet Gilbertson, Alexandra Papathoma, Myrto Kostopoulou, Meletios A Dimopoulos, Julian D Gillmore, Efstathios Kastritis
No abstract text is available yet for this article.
January 26, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30675806/transthyretin-deposition-in-the-eye-in-the-era-of-effective-therapy-for-hereditary-attrv30m-amyloidosis
#9
Joel N Buxbaum, Thomas Brannagan, Juan Buades-Reinés, Eugenia Cisneros, Isabel Conceicao, Theodoros Kyriakides, Giampaolo Merlini, Laura Obici, Violaine Plante-Bordeneuve, Antoine Rousseau, Yoshiki Sekijima, Akira Imai, Márcia Waddington Cruz, Masahito Yamada
BACKGROUND: Ocular abnormalities have been known to occur in hereditary amyloidotic polyneuropathy since the 1950s. While vitreous opacities and scalloped pupils were described early it has become evident that every component of the eye from the conjunctiva to the retinal vasculature can be involved. Reports from the major centres in Japan, Portugal and Sweden, which primarily treat patients with ATTRV30M, have indicated that with the increased longevity seen in patients treated with liver transplantation the frequency of the more severe eye findings, notably vitreous opacities and subsequent glaucoma, are being detected more frequently...
January 24, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30661419/a-pilot-study-demonstrating-cardiac-uptake-with-18f-florbetapir-pet-in-al-amyloidosis-patients-with-cardiac-involvement
#10
Richa Manwani, Jo Page, Thirusha Lane, Maria Burniston, Annah Skillen, Helen J Lachmann, Julian D Gillmore, Marianna Fontana, Carol Whelan, Philip N Hawkins, Thomas Wagner, Ashutosh D Wechalekar
18 F-florbetapir is a promising tracer in amyloidosis. This study evaluates its use in patients with systemic AL amyloidosis (AL) before and after treatment as well as its serial utility in monitoring. Fifteen AL patients with cardiac involvement underwent 18 F-florbetapir PET imaging and three patients underwent repeat imaging after chemotherapy. All patients had demonstrable cardiac uptake with 18 F-florbetapir. Cardiac uptake appeared greater in chemotherapy-naïve vs. chemotherapy-established AL patients median (left ventricular retention index 0...
January 19, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30661416/dialysis-associated-%C3%AE-2-microglobulin-amyloidosis-occurs-even-in-the-era-of-high-flux-dialyzers
#11
Lucas L Falke, Sybren L N Maas, Stefano Rosati, Susan J J Logtenberg
No abstract text is available yet for this article.
January 19, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30638075/sudoscan-in-the-evaluation-and-follow-up-of-patients-and-carriers-with-ttr-mutations-experience-from-an-italian-centre
#12
Marco Luigetti, Giulia Bisogni, Angela Romano, Andrea Di Paolantonio, Francesco Barbato, Giulia Primicerio, Paolo Maria Rossini, Serenella Servidei, Mario Sabatelli
OBJECTIVE: To evaluate the utility of Sudoscan as possible marker of disease progression and disease onset in a cohort of hereditary ATTR amyloidosis (hATTR amyloidosis) polyneuropathy patients and carriers. PATIENTS AND METHODS: We regularly performed different clinical scales, nerve conductions studies (NCS), and Sudoscan on a cohort of hATTR amyloidosis patients and carriers from a single centre of central Italy, a non-endemic area, in the last 2 years. RESULTS: About 18 hATTR amyloidosis patients and 8 asymptomatic carriers were enrolled...
January 14, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30632809/cause-of-death-analysis-and-temporal-trends-in-survival-after-liver-transplantation-for-transthyretin-familial-amyloid-polyneuropathy
#13
Vincent Algalarrondo, Teresa Antonini, Marie Théaudin, Denis Chemla, Anouar Benmalek, Denis Castaing, Cécile Cauquil, François Rouzet, Delphine Mika, Eric Duong, Sylvie Dinanian, Ludivine Eliahou, Dominique Le Guludec, Didier Samuel, David Adams, Michel S Slama
BACKGROUND: Hereditary transthyretin amyloidosis (ATTR) is a multisystemic disease involving mainly the peripheral nervous system and the heart. Liver transplantation (LT) is the reference treatment for ATTR neuropathy and preoperative detection of high risk patients is crucial. We aimed to document the causes of death of ATTR patients after LT, their temporal trends, and to evaluate whether the available preoperative tools that predict the risk of death after LT for hereditary ATTR amyloidosis matched with these trends...
January 11, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30614283/amyloid-nomenclature-2018-recommendations-by-the-international-society-of-amyloidosis-isa-nomenclature-committee
#14
Merrill D Benson, Joel N Buxbaum, David S Eisenberg, Giampaolo Merlini, Maria J M Saraiva, Yoshiki Sekijima, Jean D Sipe, Per Westermark
The nomenclature committee of the International Society of Amyloidosis (ISA) meets every second year to discuss and formulate recommendations. The conclusions from the discussion at the XVI International Symposium on Amyloidosis in Kumamoto, Japan, 25-29 March 2018 and afterwards are summarized in this Nomenclature Article. From having recommended the use of the designation "amyloid fibril" for in vivo material only, ISA's nomenclature committee now accepts its use more broadly following the international scientific literature...
January 7, 2019: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30572722/epidemiology-of-attrv30m-neuropathy-in-cyprus-and-the-modifier-effect-of-complement-c1q-on-the-age-of-disease-onset
#15
Savanna Andreou, Elena Panayiotou, Kyriaki Michailidou, Panayiota Pirpa, Andreas Hadjisavvas, Adonis El Salloukh, Daniel Barnes, Antonis Antoniou, Petros Agathangelou, Katia Papastavrou, Kyproula Christodoulou, George A Tanteles, Theodoros Kyriakides
BACKGROUND: ATTRV30M amyloidosis is a lethal autosomal dominant sensorimotor and autonomic neuropathy caused by amyloid deposition composed of aggregated misfolded TTR monomers with the V30M mutation. The age of onset in patients with ATTRV30M varies in different foci and the mechanism behind it is still unknown. METHODS: The tertiary neurology center following all ATTRV30M patients in Cyprus was used to collect demographic data to estimate; prevalence, incidence, penetrance, anticipation, time from disease onset to diagnosis and transplantation...
December 20, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30513220/haematological-response-and-overall-survival-in-two-consecutive-dutch-patient-cohorts-with-al-amyloidosis-diagnosed-between-2008-and-2016
#16
Karlijn H G Rutten, Reinier A P Raymakers, Bouke P C Hazenberg, Hans L A Nienhuis, Edo Vellenga, Monique C Minnema
BACKGROUND: Although survival has improved in recent decades, the short-term prognosis of patients with immunoglobulin light chain (AL) amyloidosis remains grim. We aimed to assess overall survival (OS) of AL amyloidosis patients by comparing cohorts in two consecutive time periods. METHODS: Data were collected and compared on 126 patients from two tertiary referral centres in The Netherlands during the time periods 2008-2012 and 2013-2016. RESULTS: There was a non-significant trend to improved 6-month OS in the last cohort (78% vs...
December 4, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30773060/electrochemical-skin-conductance-in-hereditary-amyloidosis-related-to-transthyretin-v30m-a-promising-tool-to-assess-treatment-efficacy
#17
José Castro, João Costa, Isabel de Castro, Isabel Conceição
No abstract text is available yet for this article.
December 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30773059/alan-s-cohen-1926-2018
#18
Martha Skinner, Per Westermark
No abstract text is available yet for this article.
December 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30663408/efficacy-of-lenalidomide-as-salvage-therapy-for-patients-with-al-amyloidosis
#19
Efstathios Kastritis, Maria Gavriatopoulou, Maria Roussou, Tina Bagratuni, Magdalini Migkou, Despina Fotiou, Dimitrios C Ziogas, Nikolaos Kanellias, Evangelos Eleutherakis-Papaiakovou, Ioanna Dialoupi, Ioannis Ntanasis-Stathopoulos, Mairylin Spyropoulou-Vlachou, Erasmia Psimenou, Harikleia Gakiopoulou, Smaragdi Marinaki, Elektra Papadopoulou, Argyrios Ntalianis, Evangelos Terpos, Meletios A Dimopoulos
We retrospectively evaluated 55 consecutive patients who received at least one dose of lenalidomide for relapsed/refractory AL amyloidosis. Their median age was 63 years; 72% had heart and 75% kidney involvement and 13% were on dialysis; while 20%, 46% and 34% had Mayo stage -1, -2 and -3 disease, respectively. Median time from start of primary therapy to lenalidomide was 15 months (range 2-100) and median number of prior therapies was 1 (range 1-4); 73% of the patients had prior bortezomib and 42% were bortezomib-refractory...
December 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://read.qxmd.com/read/30388377/diflunisal-tolerability-in-transthyretin-cardiac-amyloidosis-a-single-center-s-experience
#20
Asad Ikram, Joseph P Donnelly, Brett W Sperry, Christy Samaras, Jason Valent, Mazen Hanna
OBJECTIVE: Transthyretin (ATTR) amyloidosis is an under-recognized, progressive disease manifesting as cardiomyopathy and/or polyneuropathy. Diflunisal, a nonsteroidal anti-inflammatory drug (NSAID), has demonstrated transthyretin stabilization in vitro and slowing of polyneuropathy progression in the hereditary ATTR subtype (ATTRm). However, the use of diflunisal has only been described in a small cohort of patients with ATTR cardiac amyloidosis (CA). We hypothesized that selected patients with ATTR-CA, both hereditary and wild-type (ATTRwt), would tolerate diflunisal with limited adverse events...
November 2, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
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