Results 31 to 40 of about 79,953 (279)
Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain. [PDF]
Overproduction of immunoglobulin light chains leads to systemic amyloidosis, a lethal disease characterized by the formation of amyloid fibrils in patients' tissues. Excess light chains are in equilibrium between dimers and less stable monomers which can
Brumshtein, Boris +7 more
core +1 more source
Supportive Care in AL Amyloidosis [PDF]
Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response.
M Teresa, Cibeira +5 more
openaire +2 more sources
Incidence and clinical outcome of renal amyloidosis: A retrospective study
The kidneys are affected in almost all patients with amyloid A in secondary amyloidosis (AA) amyloidosis but less frequently in immunoglobulin light chains in primary systemic amyloidosis (AL) amyloidosis.
Emad Abdallah, Emam Waked
doaj +1 more source
Right Ventricle Outflow Obstruction in Biventricular Hypertrophic Cardiomyopathy in Amyloidosis [PDF]
Light-chain (AL) amyloidosis is the most common type of amyloidosis; cardiac involvement is rare but has a poor prognosis. Biventricular hypertrophic cardiomyopathy is an exceptional finding in amyloidosis and its association with obstructive right ...
Cherti, Mohammed +3 more
core +3 more sources
Duodenal biopsy: an unexpected confirmatory test for a patient with al-amyloidosis
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 +4 more
doaj +1 more source
Case Report: Diagnosis of Dual-Biopsy Negative Severe Cardiac Amyloidosis [PDF]
INTRODUCTION Amyloidosis is a rare disorder that involves the deposition of misfolded protein in extracellular tissue. Disease manifestations vary depending on the affected organs.
Kasprowicz, MD, Eric, Lohr, MD, Kristin
core +2 more sources
A bibliometric analysis of light chain amyloidosis from 2005 to 2024: research trends and hot spots
BackgroundLight chain (AL) amyloidosis stands as the most prevalent subtype of systemic amyloidosis, encompassing a group of rare diseases. Here, we evaluated the scientific landscape of AL amyloidosis to investigate research trends and identify hotspots
Xiangdong Liu +19 more
doaj +1 more source
Amyloid light-chain (AL) amyloidosis after kidney transplantation is a rare disease in the world, and only one case of systemic AL amyloidosis after kidney transplantation is reported in Korea.
Sung Hyun Park +8 more
doaj +1 more source
Dataset on the use of 3D speckle tracking echocardiography in light-chain amyloidosis [PDF]
The dataset presented in this article is related to the research article entitled “Biventricular assessment of light-chain amyloidosis using 3D speckle tracking echocardiography: Differentiation from other forms of myocardial hypertrophy” (Vitarelli et ...
Antonio Vitarelli +10 more
core +2 more sources
Junhui Xu,* Mangju Wang,* Ye Shen, Miao Yan, Weiwei Xie, Bingjie Wang, Huihui Liu, Xinan Cen Department of Hematology, Peking University First Hospital, Beijing, People’s Republic of China*These authors contributed equally to this ...
Xu J +7 more
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