Results 11 to 20 of about 19,748 (182)

Restrictive Atrial Dysfunction in Cardiac Amyloidosis: Differences between Immunoglobulin Light Chain and Transthyretin Cardiac Amyloidosis Patients

Biomedicines, 2022
Background: In cardiac amyloidosis, the prevalence of thromboembolic events and atrial fibrillation is higher in transthyretin amyloidosis compared to immunoglobulin light chain amyloidosis.
Mathijs O. Versteylen, Maaike Brons, Arco J. Teske, Marish I. F. J. Oerlemans   +3 more
doaj   +1 more source

Systemic immunoglobulin light chain amyloidosis [PDF]

Nature Reviews Disease Primers, 2018
Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction.
Merlini G., Dispenzieri A., Sanchorawala V., Schonland S. O., Palladini G., Hawkins P. N., Gertz M. A.   +6 more
openaire   +6 more sources

Diverse patterns of antibody variable gene repertoire disruption in patients with amyloid light chain (AL) amyloidosis.

PLoS ONE, 2020
Immunoglobulin light chain amyloidosis is the most common form of systemic amyloidosis. AL amyloidosis is caused by a misfolded light chain produced by a clonal population of plasma cells.
Elaine C Chen, Samuel Rubinstein, Cinque Soto, Robin G Bombardi, Samuel B Day, Luke Myers, Alexey Zaytsev, Mahsa Majedi, R Frank Cornell, James E Crowe   +9 more
doaj   +1 more source

Pseudoxanthoma Elasticum and Light-Chain Amyloidosis [PDF]

European Journal of Inflammation, 2012
Pseudoxanthoma elasticum is a heritable disorder of connective tissue characterized by cutaneous, vascular and ocular changes that result from the accumulation of fragmented elastic fibres. Even though the etiopathogenesis is not still completely understood, in recent years in literature some Authors have considered pseudoxanthoma elasticum as a ...
M. Carlesimo, C. Abruzzese, G. Cortesi, A.M. Ciccone, C. Poggi, M. Lombardi, A. Moscetti, G. La Verde, E. Mari   +8 more
openaire   +2 more sources

Light-Chain Amyloidosis: The Great Impostor

Life, 2023
Light-chain amyloidosis (AL) is a disease of protean manifestations due to a wide spectrum of organs that can be affected. The disorder is caused by the deposition of an extracellular amorphous material, the amyloid, which is produced by malignant plasma cells.
Georgia Stefani, Evangelia Kouvata, George Vassilopoulos   +2 more
openaire   +3 more sources

AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition; a case mimicking AHL amyloidosis

BMC Nephrology, 2018
Background Immunoglobulin heavy-and-light-chain amyloidosis (AHL amyloidosis) is a newly established disease entity where both the immunoglobulin heavy-chain and light-chain compose amyloid fibrils. The immunoglobulins responsible for the amyloid fibrils
Shun Manabe, Chihiro Iwasaki, Michiyasu Hatano, Fuyuki Kametani, Masahide Yazaki, Kosaku Nitta, Michio Nagata   +6 more
doaj   +1 more source

Biopsy Evidence of Sequential Transthyretin and Immunoglobulin Light-Chain Cardiac Amyloidosis in the Same Patient

JACC: Case Reports, 2021
Currently adopted diagnostic flow charts consider transthyretin and light-chain cardiac amyloidosis as mutually exclusive. Here, we report for the first time, to our knowledge, the demonstration of a biopsy-proven dual pathology in an 80-year-old man ...
Giuseppe Vergaro, MD, PhD, Vincenzo Castiglione, MD, Roberta Poletti, MD, Gabriele Buda, MD, Angela Pucci, MD, PhD, Veronica Musetti, BSc, Dario Genovesi, MD, Alberto Aimo, MD, Claudio Passino, MD, Michele Emdin, MD, PhD   +9 more
doaj   +1 more source

Novel Therapies in Light Chain Amyloidosis [PDF]

Kidney International Reports, 2018
Light chain (AL) amyloidosis is the most common form of amyloidosis involving the kidney. It is characterized by albuminuria, progressing to overt nephrotic syndrome and eventually end-stage renal failure if diagnosed late or ineffectively treated, and in most cases by concomitant heart involvement.
Milani P., Merlini G., Palladini G.
openaire   +4 more sources

Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021

Blood Cancer Journal, 2021
Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients ...
M. Hasib Sidiqi, Morie A. Gertz
doaj   +1 more source

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

Haematologica, 2013
Amyloidosis of the gastrointestinal tract, with biopsy-proven disease, is rare. We reviewed a series of patients who presented with biopsy-proven gastrointestinal amyloidosis and report their clinical characteristics, treatments, and survival.
Andrew J. Cowan, Martha Skinner, David C. Seldin, John L. Berk, David R. Lichtenstein, Carl J. O'Hara, Gheorghe Doros, Vaishali Sanchorawala   +7 more
doaj   +1 more source