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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/29052438/prevalence-of-mutant-attr-cardiac-amyloidosis-in-elderly-african-americans-with-heart-failure
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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/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
#7
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/28868918/successful-use-of-palliative-inotrope-therapy-in-end-stage-cardiac-attr-amyloidosis
#8
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/28857614/attitudes-about-when-and-how-to-treat-patients-with-al-amyloidosis-an-international-survey
#9
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%...
August 31, 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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28719238/mining-databases-for-protein-aggregation-a-review
#13
Paraskevi L Tsiolaki, Katerina C Nastou, Stavros J Hamodrakas, Vassiliki A Iconomidou
Protein aggregation is an active area of research in recent decades, since it is the most common and troubling indication of protein instability. Understanding the mechanisms governing protein aggregation and amyloidogenesis is a key component to the aetiology and pathogenesis of many devastating disorders, including Alzheimer's disease or type 2 diabetes. Protein aggregation data are currently found "scattered" in an increasing number of repositories, since advances in computational biology greatly influence this field of research...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#14
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28719235/artificial-neural-network-modelling-of-attr-amyloidosis-is-now-the-time
#15
John L Berk
No abstract text is available yet for this article.
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28699800/towards-the-improvement-in-stability-of-an-anti-a%C3%AE-single-chain-variable-fragment-scfv-h3d6-as-a-way-to-enhance-its-therapeutic-potential
#16
Laia Montoliu-Gaya, Javier Murciano-Calles, Jose C Martinez, Sandra Villegas
ScFv-h3D6 is a single-chain variable fragment derived from the monoclonal antibody bapineuzumab that prevents Aβ-induced cytotoxicity by capturing Aβ oligomers. The benefits of scFv-h3D6 treatment in Alzheimer's disease are known at the behavioural, cellular and molecular levels in the 3xTg-AD mouse model. Antibody-based therapeutics are only stable in a limited temperature range, so their benefit in vivo depends on their capability for maintaining the proper fold. Here, we have stabilized the scFv-h3D6 folding by introducing the mutation VH-K64R and combining it with the previously described elongation of the VL domain (C3)...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28699793/predictors-of-early-treatment-failure-following-initial-therapy-for-systemic-immunoglobulin-light-chain-amyloidosis
#17
Nidhi Tandon, Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Morie A Gertz, Martha Q Lacy, Robert A Kyle, Francis K Buadi, David Dingli, Suzanne R Hayman, Amie L Fonder, Miriam A Hobbs, Wilson I Gonsalves, Prashant Kapoor, Yi Lisa Hwa, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, Shaji K Kumar
We analysed factors predicting early treatment failure (ETF), after first-line therapy for light-chain amyloidosis (AL). AL amyloidosis patients seen at Mayo Clinic within 90 days of diagnosis, from 2006 to 2015, excluding those who died within 3 months of initial therapy, were analysed retrospectively. ETF was defined as progression requiring treatment change or death within 12 (ETF12) or 24 (ETF24) months of first-line treatment. Non-ETF included those with a follow-up of more than 12 or 24 months who had progression beyond 12 or 24 months...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28686525/kidney-biopsy-in-aa-amyloidosis-impact-of-histopathology-on-prognosis
#18
Zeynep Kendi Celebi, Saba Kiremitci, Bengi Ozturk, Serkan Akturk, Siyar Erdogmus, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Arzu Ensari, Kenan Keven
In AA amyloidosis, while kidney biopsy is widely considered for diagnosis by clinicians, there is no evidence that the detailed investigation of renal histopathology can be utilized for the prognosis and clinical outcomes. In this study, we aimed to obtain whether histopathologic findings in kidney biopsy of AA amyloidosis might have prognostic and clinical value. This is a retrospective cohort study that included 38 patients who were diagnosed with AA amyloidosis by kidney biopsy between 2005 and 2013.The kidney biopsy specimens of patients were evaluated and graded for several characteristics of histopathological lesions and their relationship with renal outcomes...
July 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28686088/changing-epidemiology-of-aa-amyloidosis-clinical-observations-over-25-years-at-a-single-national-referral-centre
#19
Thirusha Lane, Jennifer H Pinney, Janet A Gilbertson, David F Hutt, Dorota M Rowczenio, Shameem Mahmood, Sajitha Sachchithanantham, Marianna Fontana, Taryn Youngstein, Candida C Quarta, Ashutosh D Wechalekar, Julian D Gillmore, Philip N Hawkins, Helen J Lachmann
OBJECTIVE: Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology of AA amyloidosis over a 25-year period at a single national referral centre. METHODS: We conducted a retrospective study of all patients diagnosed with AA amyloidosis who had attended the centre between 1990 and 2014 inclusive. Six hundred and twenty-five patients were studied in three cohorts: C1: 1990-1997; C2: 1998-2006; C3: 2007-2014...
July 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28632454/localized-amyloidogenic-immunoglobulin-light-chain-derived-amyloidosis-in-a-young-boy-and-an-adolescent-girl
#20
Shu-Ichi Ikeda
No abstract text is available yet for this article.
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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