Results 31 to 40 of about 71,732 (247)

Dissecting the Molecular Features of Systemic Light Chain (AL) Amyloidosis: Contributions from Proteomics

Medicina, 2021
Amyloidoses are characterized by aggregation of proteins into highly ordered amyloid fibrils, which deposit in the extracellular space of tissues, leading to organ dysfunction.
Paola Rognoni, Giulia Mazzini, Serena Caminito, Giovanni Palladini, Francesca Lavatelli   +4 more
doaj   +1 more source

Aberrant GRP78 Phase Transition Sustains Endothelial IRE1α Signaling and Drives Blood–Brain Barrier Failure in Cerebral Amyloid Angiopathy

Advanced Science, EarlyView.
Vascular Aβ40 corrupts GRP78 phase behavior in brain endothelial cells, sustaining IRE1α–TRAF2–JNK signaling and driving apoptosis, tight junction loss, and blood–brain barrier failure in cerebral amyloid angiopathy. Pharmacological IRE1α inhibition restores vascular integrity, reduces leakage, and improves functional outcomes, revealing a targetable ...
Honglin Zheng, Qiang Li, Haiyang Luo, Yapei Yuan, Na Zhang, Yongting Lu, Suying Duan, Jieshi Zhong, Hang Zhang, Chenyang Liu, Yaochong Zhang, Wenzhuo Zhao, Yaxuan Song, Tuo Wang, Si Yan, Yapeng Li, Han Liu, Yuan Gao, Zongping Xia, Yuming Xu   +19 more
wiley   +1 more source

Real-world treatment management in hereditary transthyretin amyloidosis – an experience report and proposal for therapy switch decision criteria

Neurological Research and Practice
Background Hereditary transthyretin amyloidosis is a rapidly progressive and lethal disease. Thanks to the increasing number of disease-modifying treatments, prognosis has improved significantly.
Duc Chu Dieu, Helena F. Pernice, Harisa Muratovic, Paul J. Wetzel, Gina Barzen, Nicolas W. Wieder, Stefanie M. Werhahn, Bettina Heidecker, Sebastian Spethmann, Katrin Hahn   +9 more
doaj   +1 more source

Radical Resection of the Third Portion of the Duodenum for Secondary Aortoduodenal Fistula

Annals of Gastroenterological Surgery, EarlyView.
Secondary aortoduodenal fistula most commonly involves the third portion of the duodenum and requires definitive management of both vascular and gastrointestinal components. We demonstrate a step‐by‐step technique for radical duodenal resection and reconstruction performed in structured collaboration with cardiovascular surgeons.
Koji Kubota, Akira Shimizu, Tsuyoshi Notake, Tatsuichiro Seto, Yuji Soejima   +4 more
wiley   +1 more source

Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience

Frontiers in Cardiovascular Medicine, 2023
AimsTransthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to cardiac transplantation (CT).
Yousuf Razvi, Aldostefano Porcari, Aldostefano Porcari, Concetta Di Nora, Rishi K. Patel, Adam Ioannou, Muhammad U. Rauf, Ambra Masi, Steven Law, Liza Chacko, Tamer Rezk, Sriram Ravichandran, Janet Gilbertson, Dorota Rowczenio, Iona J. Blakeney, Nandita Kaza, David F. Hutt, Helen Lachmann, Ashutosh Wechalekar, William Moody, Sern Lim, Colin Chue, Carol Whelan, Lucia Venneri, Ana Martinez-Naharro, Marco Merlo, Gianfranco Sinagra, Ugolino Livi, Philip Hawkins, Marianna Fontana, Julian D. Gillmore   +30 more
doaj   +1 more source

Misdiagnosis of Hereditary Amyloidosis as AL (Primary) Amyloidosis [PDF]

New England Journal of Medicine, 2002
Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A alpha-chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential diagnosis of systemic amyloidosis unless there is a family history.We studied 350 patients with systemic ...
Lachmann, H.J., Booth, D.R., Booth, S.E., Bybee, A., Gilbertson, J.A., Gillmore, J.D., Pepys, M.B., Hawkins, P.N.   +7 more
openaire   +3 more sources

POEMS Syndrome: 2026 Update on Diagnosis, Risk‐Stratification, and Management

American Journal of Hematology, EarlyView.
ABSTRACT Disease Overview POEMS syndrome is a life‐threatening syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease.
Angela Dispenzieri
wiley   +1 more source

Evaluation of a new ELISA assay for monoclonal free‐light chain detection in patients with cardiac amyloidosis

eJHaem, 2022
The causal protein of amyloid light‐chain (AL) amyloidosis is a monoclonal immunoglobulin free light chain (mFLC), which must be quantified in the serum for patient diagnosis and monitoring.
Hajer Abroud, Asma Beldi‐Ferchiou, Vincent Audard, François Lemonnier, Fabien Le Bras, Karim Belhadj, Anissa Moktefi, Elsa Poullot, Khalil El Karoui, Jehan Dupuis, Alizée Maarek, Louise Roulin, Marie‐Hélène Delfau‐Larue, Silvia Oghina, Mounira Kharoubi, Mélanie Bézard, Amira Zaroui, Thibaud Damy, Valérie Molinier‐Frenkel   +18 more
doaj   +1 more source

Distribution of Big Tau Isoforms in the Human Central and Peripheral Nervous System

Annals of Neurology, EarlyView.
Objective Tau is widely studied in neurodegeneration, yet most work has focused on canonical brain tau isoforms. A longer isoform, “big tau,” produced by inclusion of exon 4a, is expressed in the peripheral nervous system (PNS) and central nervous system (CNS) regions.
Rama Krishna Koppisetti, Nicolas R. Barthélemy, Kanta Horie, Cindy V. Ly, Kaleigh F. Roberts, Srinivas Koutarapu, Richard J. Perrin, Erin E. Franklin, Chiara Pedicone, Joshua Orrick, Justin Melendez, Timothy M. Miller, Chihiro Sato, Nupur Ghoshal, Alison M. Goate, Celeste M. Karch, Randall J. Bateman, Soumya Mukherjee   +17 more
wiley   +1 more source

Amyloidosis and anesthesia

Anesthesia: Essays and Researches, 2017
The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition.Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered
Wani, Zara, Harkawat, Dev Kumar, Sharma, Meenaxi   +2 more
openaire   +3 more sources