Results 101 to 110 of about 34,251 (240)

Light chain type predicts organ involvement and survival in AL amyloidosis patients receiving stem cell transplantation

Blood Advances, 2018
: We evaluated the impact of light chain type, lambda (λ) or kappa (κ), on disease features and outcomes in patients with immunoglobulin light chain (AL) amyloidosis receiving stem cell transplant at the Mayo Clinic between October 2002 and August 2016 ...
M Hasib Sidiqi, Mohammed A. Aljama, Eli Muchtar, Francis K. Buadi, Rahma Warsame, Martha Q. Lacy, Angela Dispenzieri, David Dingli, Nelson Leung, Wilson I. Gonsalves, Shaji K. Kumar, Prashant Kapoor, Taxiarchis V. Kourelis, William J. Hogan, Morie A. Gertz   +14 more
doaj   +1 more source

Classic features of primary systemic amyloidosis (AL amyloidosis) leading to diagnosis of plasma cell myeloma [PDF]

, 2019
The diagnosis of primary systemic amyloidosis, also known as AL (amyloid light-chain) amyloidosis, is often delayed owing to its nonspecific manifestations as well as its rarity.
Behrens, Emily, Tan, Kendra W, Tarbox, Michelle B, Zhu, Brian   +3 more
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Investigating transthyretin variants H88R and I107V in amyloid priming: From destabilization to complete dissociation

The FEBS Journal, EarlyView.
Investigated mutations in transthyretin (TTR) disrupt the F87‐centered hydrophobic core that stabilizes its tetrameric structure. The mild I107V mutation weakens inter‐chain packing, while H88R fully abolishes tetramer formation, yielding a monomeric, aggregation‐prone form. Structural, biophysical, and computational analyses reveal that both mutations
István L. Bódy, Zsolt Fazekas, Nóra Wágner, Anna J. Kiss‐Szemán, Veronika Harmat, Zoltán Pozsonyi, Dóra K. Menyhárd, András Perczel   +7 more
wiley   +1 more source

Case Report: Left bundle branch pacing in an amyloid light-chain cardiac amyloidosis patient with atrioventricular block

Frontiers in Cardiovascular Medicine
IntroductionAmyloid light-chain cardiac amyloidosis is a progressive infiltrative disease characterized by the deposition of amyloid fibrils in the cardiac tissue, which can cause serious atrioventricular block requiring pacemaker implantation.
Jiaqi Yu, Fanyi Kong, Peng Gao, Taibo Chen, Yongtai Liu, Zhongwei Cheng, Hua Deng, Jinzhi Lai, Lihua Zhang, Jingbo Fan, Jiaqi Wang, Xiaohan Qin, Keyue Sun, Jian Li, Quan Fang, Deyan Yang, Kang’an Cheng   +16 more
doaj   +1 more source

Púrpura. Manifestação de Amiloidose Sistémica Primária [PDF]

, 2009
Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are
Apetato, M, Ferreira, A, Gonçalves, A, João, A, Lestre, S   +4 more
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The Underscreening of Cardiac Amyloidosis in Patients With Pacemakers—A Single Centre Retrospective Audit

Journal of Cardiovascular Electrophysiology, EarlyView.
ABSTRACT Background Cardiac amyloidosis is commonly associated with cardiac conduction disease. We sought to determine the prevalence of advanced conduction disease requiring a pacemaker in patients with known cardiac amyloidosis to evaluate current screening practices among patients receiving pacemakers.
Peishan Cai, Timothy Scully, Jason Nogic, Kah Peck, Jithin Sajeev   +4 more
wiley   +1 more source

Utilization and Prognosis of Cardiac Device Implantation in AL Versus ATTR Amyloidosis

Pacing and Clinical Electrophysiology, EarlyView.
ABSTRACT Introduction Cardiac amyloidosis can cause congestive heart failure, arrhythmias, and heart blocks, which frequently require cardiac device implantation (CDI). However, the differences between light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis CDI requirements are unknown. Methods A retrospective analysis was conducted using the
Bilal Hussain, Sanchit Duhan, Khawaja Hassan Akhtar, Amith Reddy Seri, Krittapoom Akrawinthawong, Janardhana Gorthi, Bijeta Keisham, Tarun Dasari   +7 more
wiley   +1 more source

Localized Gastrointestinal Light Chain (AL) Amyloidosis Under Surveillance for Five Years: A Case Report

DEN Open, Volume 7, Issue 1, April 2027.
ABSTRACT Amyloidosis, characterized by the deposition of abnormal protein fibrils in organs, is classified as systemic or localized. Amyloid light chain (AL)‐type localized amyloidosis is uncommon, particularly when confined to the gastrointestinal tract.
Shunsuke Kojimahara, Keiichi Tominaga, Akira Yamamiya, Mimari Kanazawa, Takanao Tanaka, Takeshi Sugaya, Nagaaki Katoh, Atsushi Irisawa   +7 more
wiley   +1 more source

Clinical diagnosis and typing of systemic amyloidosis in subcutaneous fat aspirates by mass spectrometry-based proteomics

Haematologica, 2014
Examination of abdominal subcutaneous fat aspirates is a practical, sensitive and specific method for the diagnosis of systemic amyloidosis. Here we describe the development and implementation of a clinical assay using mass spectrometry-based proteomics ...
Julie A. Vrana, Jason D. Theis, Surendra Dasari, Oana M. Mereuta, Angela Dispenzieri, Steven R. Zeldenrust, Morie A. Gertz, Paul J. Kurtin, Karen L. Grogg, Ahmet Dogan   +9 more
doaj   +1 more source

Multiple Myeloma : an update on disease biology and therapy [PDF]

, 2004
Multiple myeloma is a malignancy of immunoglobulin producing plasma cells. Clinical features include bone pain due to lytic bone lesions or pathological fractures, anemia, symptomatic hypercalcemia, renal insufficiency, recurrent infections and ...
Cook, Rachel J., Dingli, David
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