Transthyretin Amyloidosis: Update on the Clinical Spectrum, Pathogenesis, and Disease-Modifying Therapies
AbstractATTR amyloidosis is caused by systemic deposition of transthyretin (TTR) and comprises ATTRwt (wt for wild-type) amyloidosis, ATTRv (v for variant) amyloidosis, and acquired ATTR amyloidosis after domino liver transplantation. ATTRwt amyloidosis has classically been regarded as cardiomyopathy found in the elderly, whereas carpal tunnel syndrome has also become a major initial manifestation. The phenotypes of ATTRv amyloidosis are diverse and include neuropathy, cardiomyopathy, and oculoleptomeningeal involvement as the predominant features, depending on the mutation and age of onset. In addition to variant TTR, the deposition of wild-type TTR plays a significant role, even in patients with ATTRv amyloidosis. The formation of amyloid fibrils tends to occur in association with the basement membrane. The thickening or reduplication of the basement membrane surrounding endoneurial microvessels, which is similar to diabetic neuropathy, is observed in ATTRv amyloidosis, suggesting that common mechanisms, such as an accumulation of advanced glycation end products, may participate in the disease process. In addition to direct damage caused by amyloid fibrils, recent studies have suggested that the toxicity of nonfibrillar TTRs, such as TTR oligomers, participates in the process of tissue damage. Although liver transplantation has been performed for patients with ATTRv amyloidosis since 1990, late-onset patients were not eligible for this treatment. However, as the efficacy of...
Publication date: Available online 10 October 2020Source: American Journal of Kidney DiseasesAuthor(s): Shreeram Akilesh, Cynthia C. Nast, Michifumi Yamashita, Kammi Henriksen, Vivek Charu, Megan L. Troxell, Neeraja Kambham, Erika Bracamonte, Donald Houghton, Naila I. Ahmed, Chyi Chyi Chong, Bijin Thajudeen, Shehzad Rehman, Firas Khoury, Jonathan E. Zuckerman, Jeremy Gitomer, Parthassarathy C. Raguram, Shanza Mujeeb, Ulrike Schwarze, M. Brendan Shannon
Publication date: Available online 9 October 2020Source: Reumatología Clínica (English Edition)Author(s): Lina María Saldarriaga Rivera, Daniel Fernández Ávila, Wilson Bautista Molano, Daniel Jaramillo Arroyave, Alain Jasaf Bautista Ramírez, Adriana Díaz Maldonado, Jorge Hernán Izquierdo, Edwin Jáuregui, María Constanza Latorre Muñoz, Juan Pablo Restrepo, Juan Sebastián Segura Charry
CONCLUSION: The proposed PHARMAC criteria will give access to these important drugs to those people with T2DM who will likely benefit the most. PMID: 33032305 [PubMed - in process]
CONCLUSIONS: This single practice study showed total patient contact was similar over both sample periods, but most contact in 2020 was virtual. Further longitudinal multi-practice studies to confirm these findings and describe future consultation patterns are needed to inform general practice service delivery post-COVID-19. PMID: 33032304 [PubMed - in process]
Publication date: Available online 1 October 2020Source: Academic RadiologyAuthor(s): Neo Poyiadji, Chad Klochko, Jeff LaForce, Manuel L. Brown, Brent Griffith
Authors: Siamashvili M, Davis S Abstract INTRODUCTION: Bromocriptine mesylate quick release (QR) is a dopamine D2 receptor agonist and is the only oral, primarily centrally acting drug that can be used for the treatment of adults with type 2 diabetes. AREAS COVERED: The authors describe current recommendations on the use of bromocriptine mesylate QR. Major efficacy and safety parameters of the late phase trials, including The Cycloset Safety Trial, have been identified and presented. EXPERT OPINION: Efficacy of bromocriptine mesylate QR monotherapy appears to be low but is compensated by favorable safety pr...
Publication date: 15 February 2021Source: Personality and Individual Differences, Volume 170Author(s): Brian W. Haas, Fumiko Hoeft, Kazufumi Omura
Publication date: December 2020Source: Journal of Functional Foods, Volume 75Author(s): Tingting Wang, Lin Zheng, Tiantian Zhao, Qi Zhang, Zhitong Liu, Xiaoling Liu, Mouming Zhao
Authors: Hui KK PMID: 33034297 [PubMed - as supplied by publisher]
Authors: Lam PT PMID: 33034296 [PubMed - as supplied by publisher]