Results 61 to 70 of about 20,876 (147)

Emerging Therapeutics for the Treatment of Light Chain and Transthyretin Amyloidosis

JACC: Basic to Translational Science, 2019
Summary: Cardiac amyloidosis is a restrictive cardiomyopathy that results from the deposition of misfolded light chain or transthyretin proteins, most commonly, in cardiac tissue.
Kathleen W. Zhang, MD, Keith E. Stockerl-Goldstein, MD, Daniel J. Lenihan, MD   +2 more
doaj   +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

Negative for AL and ATTR: Could It Still Be Amyloid?

Annals of Internal Medicine: Clinical Cases
Amyloidosis is a systemic disease characterized by the extracellular deposition of misfolded proteins. We present a rare case of familial amyloidosis with multiorgan involvement.
Ahmed B. Elawad, Paul Wilkinson, Ryan Kimball, Ahmed F. Abdulrahim   +3 more
doaj   +1 more source

Apolipoprotein C-II Deposition Amyloidosis: A Potential Misdiagnosis as Light Chain Amyloidosis

Case Reports in Nephrology, 2016
Hereditary amyloidoses are rare and pose a diagnostic challenge. We report a case of hereditary amyloidosis associated with apolipoprotein C-II deposition in a 61-year-old female presenting with renal failure and nephrotic syndrome misdiagnosed as light ...
Sadichhya Lohani, Emily Schuiteman, Lohit Garg, Dhiraj Yadav, Sami Zarouk   +4 more
doaj   +1 more source

Neurodegenerative Diseases: Epidemiology, Risk Factors, Classification, Pathophysiology, Clinical Features, Diagnosis, and Therapeutic Strategies

MedComm, Volume 7, Issue 11, November 2026.
Neurodegenerative diseases share convergent risk architectures and pathological mechanisms, yet diverge in selective vulnerability, clinical presentation, and therapeutic responsiveness. This review integrates cross‐disease epidemiology, pathophysiology, diagnosis, and treatment, highlighting the transition from symptomatic care toward biomarker‐guided
Zimeng He, Jingjing Zeng, Guoqing Wang, Yuxin Li, Feng Zhang   +4 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

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

Light Chain Monoclonal Gammopathy of Undetermined Significance: Diagnosis, Biology, and Clinical Management

European Journal of Haematology, Volume 117, Issue 5, Page 1159-1167, November 2026.
ABSTRACT Light chain monoclonal gammopathy of undetermined significance (LC‐MGUS) is defined by an abnormal serum free light chain ratio and elevated involved light chain in the absence of a detectable immunoglobulin heavy chain on immunofixation and of end‐organ damage attributable to a plasma cell disorder.
Sigurður Yngvi Kristinsson, Thórir Einarsson Long, Sigrún Thorsteinsdóttir   +2 more
wiley   +1 more source

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

Advanced Science, Volume 13, Issue 58, 19 October 2026.
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

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

American Journal of Hematology, Volume 101, Issue 10, Page 2632-2651, October 2026.
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