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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/29308670/late-iodine-enhancement-and-myocardial-extracellular-volume-quantification-in-cardiac-amyloidosis-by-using-dual-energy-cardiac-computed-tomography-performed-on-a-dual-layer-spectral-detector-scanner
#1
Seitaro Oda, Takeshi Nakaura, Daisuke Utsunomiya, Kyoko Hirakawa, Seiji Takashio, Yasuhiro Izumiya, Kenichi Tsujita, Hiroyuki Hata, Yukio Ando, Yasuyuki Yamashita
No abstract text is available yet for this article.
January 8, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29303358/treatment-patterns-and-health-care-resource-utilization-among-patients-with-relapsed-refractory-systemic-light-chain-amyloidosis
#2
Parameswaran Hari, Huamao Mark Lin, Carl V Asche, Jinma Ren, Candice Yong, Katarina Luptakova, Douglas V Faller, Vaishali Sanchorawala
BACKGROUND: Treatment for patients with systemic light chain (AL) amyloidosis remains challenging. Our study aims to describe treatment patterns for both newly diagnosed and relapsed/refractory AL (RRAL) amyloidosis, and to assess clinical outcomes, healthcare costs, and resource utilization during the first year following a diagnosis of RRAL amyloidsis. METHODS: This was a retrospective observational study of adult patients with AL amyloidosis using the US Optum administrative claims data during 1/1/2008 to 6/30/2015...
January 5, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29260587/right-ventricular-longitudinal-strain-a-tool-for-diagnosis-and-prognosis-in-light-chain-amyloidosis
#3
Charles Uzan, Olivier Lairez, Pascale Raud-Raynier, Rodrigue Garcia, Bruno Degand, Luc P Christiaens, Michaela B Rehman
OBJECTIVES: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. METHODS: We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN...
December 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29241368/gastrointestinal-perforation-in-light-chain-amyloidosis-in-the-era-of-novel-agent-therapy-a-case-series-and-review-of-the-literature
#4
Adir Shaulov, Irit Avivi, Yael Cohen, Adrian Duek, Merav Leiba, Moshe E Gatt
Gastrointestinal (GI) perforation is remarkably rare in patients with light chain (AL) amyloidosis and has not yet been reported in patients with AL amyloidosis treated with novel agents. Only 24 cases of GI perforation have previously been reported in the setting of AL amyloidosis of which 15 had available information in English. All 15 did not receive novel agent therapy and six died early after experiencing GI perforation. This study reports the characteristics and outcome of AL patients that developed GI perforation in the era of novel agent treatment...
December 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29219632/budd-chiari-syndrome-associated-with-al-amyloidosis-a-coagulation-paradox
#5
Guilherme Grossi Lopes Cançado, Luciana Costa Faria, Fernanda Maria Farage Osório, Paula Vieira Teixeira Vidigal, Cláudia Alves Couto, Teresa Cristina de Abreu Ferrari
No abstract text is available yet for this article.
December 8, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29182024/first-nationwide-survey-on-systemic-wild-type-attr-amyloidosis-in-japan
#6
Yoshiki Sekijima, Masahide Yazaki, Mitsuharu Ueda, Haruki Koike, Masahito Yamada, Yukio Ando
OBJECTIVE: A nationwide survey on systemic wild-type ATTR (ATTRwt) amyloidosis was conducted to elucidate the frequency, clinical picture and possible diagnostic issues of ATTRwt amyloidosis in Japan. METHODS: A questionnaire was sent to 4629 clinical departments across Japan. A total of 2341 (50.6%) responses were returned completed for further analysis. RESULTS: Fifty-one patients with ATTRwt amyloidosis (82% male) were identified between January 2012 and December 2014...
November 28, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29166802/diagnostic-work-up-of-an-adult-female-patient-with-systemic-aa-amyloidosis-revealing-the-cause-of-infantile-mental-retardation
#7
Kaya Veelken, Stefan O Schönland, Georg Gdynia, Tim Weber, Norbert Blank, Ute Hegenbart
No abstract text is available yet for this article.
November 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29169262/correction-to-amyloid-iamy-vol-24-no-4-2017
#8
(no author information available yet)
No abstract text is available yet for this article.
December 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28906148/hip-and-knee-arthroplasty-are-common-among-patients-with-transthyretin-cardiac-amyloidosis-occurring-years-before-cardiac-amyloid-diagnosis-can-we-identify-affected-patients-earlier
#9
Jonah Rubin, Julissa Alvarez, Sergio Teruya, Adam Castano, Ronald A Lehman, Mark Weidenbaum, Jeffrey A Geller, Stephen Helmke, Mathew S Maurer
Transthyretin cardiac amyloidosis (ATTR-CA) causes a restrictive cardiomyopathy in older adults, often diagnosed at advanced stages when emerging therapies in late phase clinical trials may not have clinical benefit. This investigation aimed to detect clinical entities that may provide more advanced warning of ATTR-CA. Since ATTR preferentially deposits in ligaments, tendons, and articular cartilage, we hypothesized that ATTR-CA patients have a greater prevalence of total hip (THA) and knee (TKA) arthroplasties compared with the general population, and that arthroplasty occurs significantly before ATTR-CA diagnosis...
December 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28857614/attitudes-about-when-and-how-to-treat-patients-with-al-amyloidosis-an-international-survey
#10
Paolo Milani, Morie A Gertz, Giampaolo Merlini, Angela Dispenzieri
The aim of this survey was to describe the treatment decision making of expert physicians in when and how to treat patients with AL amyloidosis. Fifty amyloid expert physicians completed the survey. Autologous stem cell transplant (ASCT) was considered the first line therapy, if medically feasible, by 73% of the physicians. Excluding ASCT, cyclophosphamide-bortezomib-dexamethasone regimen was the preferred strategy by 72%. Depending on organ involvement, the goal for treatment was CR for 27-35% and very good partial response (VGPR) for 65-72%...
December 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29052438/prevalence-of-mutant-attr-cardiac-amyloidosis-in-elderly-african-americans-with-heart-failure
#11
Marios Arvanitis, Gloria G Chan, Daniel R Jacobson, John L Berk, Lawreen H Connors, Frederick L Ruberg
No abstract text is available yet for this article.
October 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29052436/prognostic-impact-of-immunoparesis-at-diagnosis-and-after-treatment-onset-in-patients-with-light-chain-amyloidosis
#12
Luis Gerardo Rodríguez-Lobato, Carlos Fernández de Larrea, Maria Teresa Cibeira, Natalia Tovar, Ignacio Isola, Juan I Aróstegui, Laura Rosiñol, Tania Díaz, Ester Lozano, Jordi Yagüe, Joan Bladé
OBJECTIVES: Immunoparesis (IP) is a risk factor associated with an unfavourable outcome in several plasma cell disorders. It has been suggested that its presence in light-chain (AL) amyloidosis could be associated with worse prognosis. However, the relevance of IP after treatment has not been evaluated to date. The aim of this study was to determine the prognostic impact of IP at diagnosis and one year after treatment onset in patients with AL amyloidosis. METHODS: The clinical records of 69 patients with AL amyloidosis treated at a single institution from January 2006 to January 2016 were included in the study...
October 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29016222/misidentification-of-transthyretin-and-immunoglobulin-variants-by-proteomics-due-to-methyl-lysine-formation-in-formalin-fixed-paraffin-embedded-amyloid-tissue
#13
Diana Canetti, Nigel Brian Rendell, Lucia Di Vagno, Janet A Gilbertson, Dorota Rowczenio, Tamar Rezk, Julian D Gillmore, Phillip N Hawkins, Guglielmo Verona, Palma Patrizia Mangione, Sofia Giorgetti, Pierluigi Mauri, Sara Motta, Antonella De Palma, Vittorio Bellotti, Graham W Taylor
Proteomics is becoming the de facto gold standard for identifying amyloid proteins and is now used routinely in a number of centres. The technique is compound class independent and offers the added ability to identify variant and modified proteins. We re-examined proteomics results from a number of formalin-fixed paraffin-embedded amyloid samples, which were positive for transthyretin (TTR) by immunohistochemistry and proteomics, using the UniProt human protein database modified to include TTR variants. The amyloidogenic variant, V122I TTR, was incorrectly identified in 26/27 wild-type and non-V122I variant samples due to its close mass spectral similarity with the methyl lysine-modified WT peptide [126KMe]105-127 (p...
October 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28984490/pitfalls-in-conducting-prospective-trials-in-stage-iii-cardiac-amyloidosis-experience-from-the-reveal-study
#14
Elizabeth H Phillips, Stephen Nash, Toyin Adedayo, Carol J Whelan, Marianna Fontana, Shameem Mahmood, Helen J Lachmann, Julian D Gillmore, Paul Smith, Laura Clifton-Hadley, Philip N Hawkins, Ashutosh D Wechalekar
No abstract text is available yet for this article.
October 6, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28949257/a-novel-transthyretin-variant-v28s-p-v48s-with-a-double-nucleotide-substitution-in-the-same-codon
#15
Masamitsu Okada, Yohei Misumi, Mitsuharu Ueda, Taro Yamashita, Teruaki Masuda, Masayoshi Tasaki, Yukio Ando
No abstract text is available yet for this article.
September 26, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28906150/safety-and-efficacy-of-a-ttr-specific-antisense-oligonucleotide-in-patients-with-transthyretin-amyloid-cardiomyopathy
#16
Merrill D Benson, Noel R Dasgupta, Stacy M Rissing, Jessica Smith, Harvey Feigenbaum
OBJECTIVES: Cardiomyopathy is a major cause of death in both the hereditary form of transthyretin (TTR) amyloidosis and the sporadic late-age-onset transthyretin amyloidosis (ATTR wild-type (ATTRwt)). Clinically disease progression from time of diagnosis to death is usually quoted as 5- to 15-years. In prior studies, significant progression of cardiac parameters in patients with moderate to severe cardiomyopathy has been noted within a 12-month time span. METHODS: The present study was designed to prospectively monitor changes in cardiac parameters, both structural and functional, in patients with ATTR cardiomyopathy while treated with a TTR specific antisense oligonucleotide (ASO; IONIS-TTR℞) designed to lower blood levels of the amyloid fibril precursor protein...
September 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28868918/successful-use-of-palliative-inotrope-therapy-in-end-stage-cardiac-attr-amyloidosis
#17
Muhammad S Panhwar, Sadeer Al-Kindi, Guilherme H Oliveira, Mahazarin Ginwalla
No abstract text is available yet for this article.
September 3, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28758793/long-term-safety-and-efficacy-of-tafamidis-for-the-treatment-of-hereditary-transthyretin-amyloid-polyneuropathy-results-up-to-6-years
#18
Fabio A Barroso, Daniel P Judge, Ben Ebede, Huihua Li, Michelle Stewart, Leslie Amass, Marla B Sultan
BACKGROUND: The objective of the present study was to evaluate the long-term safety and efficacy of tafamidis in treating hereditary transthyretin amyloid polyneuropathy. METHODS: A prospectively planned interim analysis was conducted on an on-going, phase III, open-label extension study following an 18-month, randomized, controlled study and 12-month, open-label extension study in ATTRV30M patients and a single-arm, open-label study in non-ATTRV30M patients. Thirty-seven ATTRV30M patients received placebo for 18 months, then switched to tafamidis and 38 ATTRV30M patients and 18 non-ATTRV30M patients continuously received tafamidis from day 1, up to 6 years...
September 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28758811/novel-pathologic-scoring-tools-predict-end-stage-kidney-disease-in-light-chain-al-amyloidosis
#19
Samuel Rubinstein, Robert F Cornell, Liping Du, Beatrice Concepcion, Stacey Goodman, Shelton Harrell, Sara Horst, Daniel Lenihan, David Slosky, Agnes Fogo, Anthony Langone
BACKGROUND AND OBJECTIVES: Light chain (AL) amyloidosis frequently involves the kidney, causing significant morbidity and mortality. A pathologic scoring system with prognostic utility has not been developed. We hypothesized that the extent of amyloid deposition and degree of scarring injury on kidney biopsy, could provide prognostic value, and aimed to develop pathologic scoring tools based on these features. METHODS: This is a case-control study of 39 patients treated for AL amyloidosis with biopsy-proven kidney involvement at a large academic medical center...
July 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28745926/safety-and-efficacy-of-empirical-interleukin-1-inhibition-using-anakinra-in-aa-amyloidosis-of-uncertain-aetiology
#20
Thirusha Lane, Ashutosh D Wechalekar, Julian D Gillmore, Philip N Hawkins, Helen J Lachmann
OBJECTIVE: AA amyloidosis is a serious complication of persistent inflammation, which, untreated will progress to renal failure and death. Effective suppression of the underlying inflammatory disease is the focus of treatment. However, in approximately 20% of cases the underlying condition remains uncertain, presenting a dilemma as to choice of treatment. METHODS: We conducted a retrospective study of a cohort of 11 patients diagnosed with AA amyloidosis of unknown aetiology, who had been empirically treated with anakinra...
July 26, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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