Results 131 to 140 of about 720,196 (232)

Asymptomatic Carrier Neurologic Assessment: A Tool for Early Detection of Symptomatic Transition in Pathogenic TTR Gene Variant Carriers

European Journal of Neurology, Volume 33, Issue 9, September 2026.
There are no tools to support early detection of ATTRv signs/symptoms or to identify risk of transition to clinically detectable disease in carriers of pathogenic ATTRv variants. This retrospective cohort study describes the ACNA, a brief 23‐item assessment for use in routine clinical visits.
Isabel Conceição, Juan González‐Moreno, Andoni Echaniz‐Laguna, Kuangnan Xiong, Adam Castaño, Jonathan C. Fox, Julian D. Gillmore, Pablo Garcia‐Pavia, Mary M. Reilly   +8 more
wiley   +1 more source

Diagnostic and therapeutic challenges in rapidly progressing cardiac amyloidosis: a literature review based on case report

International Journal of Emergency Medicine
Introduction Cardiac amyloidosis is a rarely reported and potentially fatal variant of the systemic disease. Its early diagnosis could potentially lead to significantly improved clinical outcomes.
Nahid Senobari, Roozbeh Nazari, Pouya Ebrahimi, Hamidreza Soleimani, Maryam Taheri, Kaveh Hosseini, Homa Taheri, Robert J. Siegel   +7 more
doaj   +1 more source

Combating ageing beyond the cell: Emerging roles of extracellular proteostasis

The FEBS Journal, Volume 293, Issue 18, Page 5558-5584, September 2026.
Ageing challenges the body's ability to maintain a stable and functional proteome, leading to protein damage and aggregation both inside and outside cells. This review focuses on the less well understood mechanisms of extracellular protein quality control and how they become disrupted in ageing, particularly in neurodegenerative diseases.
Siddharth R. Venkatesh, Ivan Gallotta, Bolanle F. Olabiyi, Yoon Hee Choi, Sarah Inglesfield, Janet R. Kumita, Collin Y. Ewald, Della C. David   +7 more
wiley   +1 more source

Microvascular Obstruction in Cardiac Amyloidosis

European Journal of Heart Failure
Aims Cardiac amyloidosis (CA) is characterized by deposition of amyloid fibrils within the extracellular space, causing disarray of the myocardial structure and capillary architecture. This study aims to characterize the prevalence of microvascular obstruction (MVO) in patients with CA and to assess the association between MVO and prognosis.
Netti L., Ioannou A., Martinez-Naharro A., Razvi Y., Porcari A., Venneri L., Maestrini V., Knight D., Virsinskaite R., Rauf M. U., Kotecha T., Patel R. K., Wechelakar A., Lachmann H., Kellman P., Manisty C., Moon J., Hawkins P. N., Gillmore J. D., Fontana M.   +19 more
openaire   +3 more sources

Multimodality Imaging in Monoclonal Gammopathy of Undetermined Significance and ATTR Wild-Type Cardiac Amyloidosis

Life
Amyloidosis is characterized by the tissue deposition of insoluble fibrils derived from misfolded proteins. This case report describes a Hispanic man diagnosed with both monoclonal gammopathy of undetermined significance (MGUS) and wild-type ...
Amalia Peix, Aylen Perez, Yrving Figueredo, Leonel Torres, Lazaro O. Cabrera, Giselle Monzon, Hilda Roblejo, Alejandro Perera, Anita Brink, Diana Paez   +9 more
doaj   +1 more source

Neuropathy in Val122Ile Hereditary Transthyretin (ATTR) Amyloidosis: A Multicenter Retrospective Cohort Study

Journal of the Peripheral Nervous System, Volume 31, Issue 3, September 2026.
ABSTRACT Background and Aims The Val122Ile ATTR Amyloidosis has traditionally been linked to cardiac manifestations. Recent studies suggest that neuropathy may be relevant. In this study, we characterized its peripheral nerve manifestations in depth. Methods This was a national, multicenter, observational, retrospective study.
Anna Paula Paranhos, Marcela Câmara Machado Costa, Carolina Lavigne Moreira, Diogo Fernandes dos Santos, Marcondes Cavalcante França, Renan Flavio de França Nunes, Osvaldo José Moreira do Nascimento, Jemima Araujo da Silva Batista, Eduardo Boiteux Uchôa Cavalcanti, Cleonisio Leite Rodrigues, Ândrea Virgínia Ferreira Chaves, Marcelo Soares Kerstenetzky, Lara Albuquerque de Brito, Carlo Domênico Marrone, Marcus Vinicius Magno Gonçalves, Pedro José Tomaselli, Wilson Marques   +16 more
wiley   +1 more source

Impact of Baseline Polyneuropathy Severity on Eplontersen Efficacy in the NEURO‐TTRansform Clinical Trial

Journal of the Peripheral Nervous System, Volume 31, Issue 3, September 2026.
ABSTRACT Background and Aims In the NEURO‐TTRansform clinical trial (NCT04136184), eplontersen improved neuropathy impairment and quality of life (QoL) through Week 66 versus the NEURO‐TTR historical placebo in patients with hereditary transthyretin amyloidosis with polyneuropathy (ATTRv‐PN).
Thomas H. Brannagan III, Markus Weiler, Márcia Waddington Cruz, Sami Khella, John L. Berk, Morie Gertz, Ahmad Masri, Mathew S. Maurer, Jonas Wixner, Jonatan Nåtman, T. Jesse Kwoh, Maksym Pola, Laura Obici   +12 more
wiley   +1 more source

Left Atrioventricular Coupling for Predicting Incident Atrial Fibrillation in Cardiac Amyloidosis

openABSTRACT BACKGROUND: Amyloidosis is a heterogeneous group of infiltrative diseases characterized by protein misfolding and extracellular deposition of insoluble amyloid fibrils. When the heart is involved, cardiac amyloidosis (CA) occurs.
LASTEI, SOFIA
core  

Incidence, Diagnosis and Prognosis of Cardiac Amyloidosis [PDF]

, 2013
학위논문 (석사)-- 서울대학교 대학원 : 의학과 내과학 전공, 2013. 2. 손대원.Introduction: Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac
이민호
core  

Diagnostic imaging of cardiac amyloidosis [PDF]

, 2020
Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative
Fontana, M, Martinez-Naharro, A, Baksi, AJ, Hawkins, PN   +3 more
core