Results 21 to 30 of about 5,095,980 (121)

Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome [PDF]

, 2013
Background Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (ATTRwt) is common and must be differentiated from other amyloid types.
Banypersad, SM, Whelan, CJ, Lachmann, HJ, Gillmore, JD, Gibbs, SD, Hawkins, PN, McCarthy, CA, Sattianayagam, P, Rowczenio, D, Wechalekar, A, Venner, CP, Petrie, A, Dungu, J, Wassef, N, Pinney, JH, Gilbertson, JA   +15 more
core   +1 more source

Amyloid light-chain deposition in a schwannoma

Interdisciplinary Neurosurgery, 2021
Systemic amyloid light-chain amyloidosis is a protein misfolding disorder characterized by extracellular deposition of amyloid fibrils derived from abnormal clonal immunoglobulin light chains in various organ systems.
Megan M. Jack, Brandon W. Smith, Christopher J. Klein, Taxiarchis Kourelis, Andrew L. Folpe, Robert J. Spinner, Ellen D. McPhail   +6 more
doaj   +1 more source

Diagnostic approach to light-chain cardiac amyloidosis and its differential diagnosis [PDF]

, 2018
Cardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease.
Joanna Rupa-Matysek, Lidia Gil, Tatiana Mularek, Monika Adamska, Anna Komosa   +4 more
core   +2 more sources

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

Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient

Nature Communications, 2019
Immunoglobulin Light Chain Amyloidosis (AL) is the most common systemic amyloidosis occurring in Western countries. Here the authors present the 4.0 Å cryo-EM structure of light chain AL55 fibrils that were isolated from the heart of an AL systemic ...
Paolo Swuec, Francesca Lavatelli, Masayoshi Tasaki, Cristina Paissoni, Paola Rognoni, Martina Maritan, Francesca Brambilla, Paolo Milani, Pierluigi Mauri, Carlo Camilloni, Giovanni Palladini, Giampaolo Merlini, Stefano Ricagno, Martino Bolognesi   +13 more
doaj   +1 more source

Genome-wide association study of immunoglobulin light chain amyloidosis in three patient cohorts: comparison with myeloma [PDF]

, 2017
Immunoglobulin light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibers derived from immunoglobulin light chain. AL amyloidosis and multiple myeloma (MM) originate from monoclonal gammopathy of undetermined significance.
Hegenbart, U., Merlini, G, Huhn, S., Milani, P., da Silva Filho, M. I, Jöckel, K. H., Palladini, G, Hemminki, K, da Silva Filho, MI, Goldschmidt, H., Jauch, A, Houlston, R., Landi, S, Johnson, D., Hemminki, K., Nickel, J., Schönland, S. O., Campo, C, Weinhold, N., MERLINI, GIAMPAOLO, Hegenbart, U, Meziane, I, Johnson, D, Nöthen, MM, Da Silva Filho, M. I., Nickel, J, Campo, C., Houlston, R, Mitchell, JS, Försti, A., Nöthen, M. M., Mitchell, J. S., Wechalekar, A., Landi, Stefano, Försti, A, Rowcieno, D, Wechalekar, A, Hoffmann, P., Merlini, G., Meziane, I., PALLADINI, GIOVANNI, Weinhold, N, Huhn, S, MILANI, PAOLO, Goldschmidt, H, Rowcieno, D., Milani, P, Jöckel, K-H, Morgan, GJ, Morgan, G. J., Hawkins, P., Palladini, G., Jauch, A., Hoffmann, P, Hawkins, P, CAMPO, MARIA CHIARA, Schönland, SO, Landi, S., Goldschmidt, H.,, Johnson, D.,, Försti, A.,, Mitchell, J. S.,, Nickel, J.,, Nöthen, M. M.,, Morgan, G. J.,, Hoffmann, P.,, Huhn, S.,, Weinhold, N.,, Meziane, I.,, Jöckel, K. H.,, Campo, C.,, Lund University., Wechalekar, A.,, Palladini, G.,, Jauch, A.,, Hegenbart, U.,, Hawkins, P.,, Landi, S.,, Schönland, S. O.,, Milani, P.,, Hemminki, K.,, Rowcieno, D.,, Houlston, R.,, Merlini, G.,, Da Silva Filho, M. I.,   +84 more
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Light Chain Stabilization: A Therapeutic Approach to Ameliorate AL Amyloidosis

Hemato, 2021
Non-native immunoglobulin light chain conformations, including aggregates, appear to cause light chain amyloidosis pathology. Despite significant progress in pharmacological eradication of the neoplastic plasma cells that secrete these light chains, in ...
Gareth J. Morgan, Joel N. Buxbaum, Jeffery W. Kelly   +2 more
doaj   +1 more source

Cardiac Amyloidosis – An Underdiagnosed Cause of Heart Failure with Preserved Ejection Fraction – Updated Diagnosis and Treatment Options [PDF]

Romanian Journal of Cardiology, 2021
Cardiac amyloidosis (CA) still represents a frequently missed cause of heart failure with preserved ejection fraction (HFpEF). In the light of many new and effective therapies for immunoglobulin light chain amyloidosis (AL) and for transthyretin ...
Roxana Cristina RIMBAS, Anca BALINISTEANU, Alexandra Maria CHITROCEANU, Dragos VINEREANU   +3 more
doaj   +1 more source

Duodenal biopsy: an unexpected confirmatory test for a patient with al-amyloidosis

Sri Lanka Journal of Medicine, 2019
SSystemic AL- amyloidosis is a disorder of protein folding in which there is extra-cellular accumulation as β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments. AL- amyloidosis is a rare clinical entity.
S. Pirasath, T. Kumanan, G. Selvaratnam, V. Sujanitha, D. D. Dikowita   +4 more
doaj   +1 more source

Skeletal Myopathy as the Initial Manifestation of Light Chain Multiple Myeloma

European Journal of Case Reports in Internal Medicine, 2020
Monoclonal gammopathies due to plasma cell dyscrasias can cause various rare neuromuscular disorders. The peripheral nervous system is most commonly affected, while muscle diseases associated with monoclonal gammopathies are rare. Skeletal myopathy, as a
Mohamed Reda Belkhribchia, Sara Moukhlis, Tarik Bentaoune, Najat Chourkani, Mohamed Zaidani, Mehdi Karkouri   +5 more
doaj   +1 more source