Results 61 to 70 of about 11,291,129 (270)

Lipid Metabolic Dysregulation Driven by OSMR Mutations Underlies Amyloidogenesis in Primary Localized Cutaneous Amyloidosis

Advanced Science, EarlyView.
OSMR mutations disrupt STAT5 signaling and AKR1B10‐mediated lipid metabolism, leading to lipid accumulation and protein aggregation in keratinocytes. This metabolic reprogramming drives amyloid deposition in primary localized cutaneous amyloidosis, revealing a mechanistic link between lipid dysregulation and amyloidogenesis.
Huiting Liu, Wen Zheng, Chao Yang, Tao Yang, Qiuyu Chen, Ping Lu, Huan Yang, Jun Liu, Bin Yang   +8 more
wiley   +1 more source

Epidemiology of AL amyloidosis: a real-world study using US claims data

Blood Advances, 2018
: Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States ...
Tiffany P. Quock, Tingjian Yan, Eunice Chang, Spencer Guthrie, Michael S. Broder   +4 more
doaj   +1 more source

Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina.

PLoS ONE, 2022
Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management.
Maria Lourdes Posadas-Martinez, María Adela Aguirre, Erika Brulc, Maria Soledad Saez, Patricia Sorroche, Gerardo Machnicki, Mariana Fernandez, Elsa Mercedes Nucifora   +7 more
doaj   +1 more source

Injectable Peptide‐Based Functional Amyloid Hydrogels as Tunable Depots for Sustained Drug Delivery

Advanced Science, EarlyView.
An injectable and tunable peptide‐based functional amyloid hydrogel provides sustained insulin release while preserving insulin stability and bioactivity. The hydrogel enables prolonged glycemic regulation in vivo, exhibits excellent biocompatibility, and shows no detectable IAPP and α‐Syn heterologous cross‐seeding, highlighting its potential as a ...
Ranjit Shaw, Niyamat M. A. Chimthanawala, Shouvik Manna, Sadhana Sathaye, Samir K. Maji   +4 more
wiley   +1 more source

Upfront CyBorD in AL amyloidosis [PDF]

Blood, 2015
In this issue of Blood , [Palladini et al][1] report on the outcome of a large series of 230 patients with systemic immunoglobulin (Ig) amyloid light chain (AL) amyloidosis treated frontline with cyclophosphamide, bortezomib, and dexamethasone (CyBorD) at 2 referral centers.[1][2] ![Figure][3]
M Teresa, Cibeira, Joan, Bladé
openaire   +2 more sources

Improving management of AL amyloidosis

Annals of the Academy of Medicine, Singapore, 2023
In this issue of the Annals, Tan et al. on behalf of Singapore Myeloma Study Group presented the consensus guidelines on light chain (AL) amyloidosis.1 This is an encouraging effort as AL amyloidosis is a rare disease, with diagnostic and therapeutic challenges.
Yuh Shan, Lee, Jeffrey, Huang
openaire   +2 more sources

Chromosomal Abnormalities, Drug Sensitivity and Resistance in Hematological Malignancies: A Comprehensive Overview by the Francophone Group of Hematological Cytogenetics (GFCH)

American Journal of Hematology, EarlyView.
ABSTRACT Chromosomal abnormalities have a major clinical impact on hematological malignancies, particularly with regard to treatment strategies. To preserve and consolidate fundamental knowledge in this rapidly evolving field, the Francophone Group of Hematological Cytogenetics (Groupe Francophone de Cytogénétique Hématologique, GFCH) conducted a ...
Florence Nguyen‐Khac, Audrey Bidet, Elise Chapiro, Nathalie Douet‐Guilbert, Jean‐Baptiste Gaillard, Nada Assaf, Nathalie Auger, Hend Chaker, Jasmine Chauzeix, Inge Govaerts, Hippolyte Guérineau, Christine Lefebvre, Pauline Roynard, Hélène Guermouche, Lucienne Michaux, Marie‐Bérengère Troadec, Lauren Véronèse   +16 more
wiley   +1 more source

Recurrent syncope and cardiac arrest in a patient with systemic light chain amyloidosis treated with bortezomib

Hematology Reports, 2016
About 10-15% of patients with multiple myeloma develop light chain (AL) amyloidosis. AL amyloidosis is a systemic disease that may involve multiple organs, often including the heart. It may present clinically with bradyarrhythmia and syncope.
Navin Jaipaul, Alexander Pi, Zhiwei Zhang   +2 more
doaj   +1 more source

Eprodisate for the treatment of renal disease in AA amyloidosis [PDF]

, 2007
Background: Amyloid A (AA) amyloidosis is a complication of chronic inflammatory conditions that develops when proteolytic fragments of serum amyloid A protein (SAA) are deposited in tissues as amyloid fibrils.
Dember, L.M., Hazenberg, B.P.C., Garceau, D., Puéchal, X., Livneh, A., Lesnyak, O., Skinner, Martha, Lesnyak, Olga, Livneh, Avi, Butrimiene, I., Merlini, G., Hauck, Wendy, Obici, L., Dember, Laura M., Gorevic, Peter D., Skinner, M., Butrimiene, Irena, Hawkins, Philip N., Balshaw, Robert, Hazenberg, Bouke P. C.; id_orcid, Lachmann, Helen J., Obici, Laura, Gorevic, P.D., Puechal, Xavier, Hazenberg, Bouke P. C., Hawkins, P.N., Garceau, Denis, Balshaw, R., Lachmann, H.J., Merlini, Giampaolo, Hauck, W.   +30 more
core  

Outcomes of Patients With AL Amyloidosis and End‐Stage Renal Disease After Initiation of Therapy

American Journal of Hematology, EarlyView.
ABSTRACT In AL amyloidosis, advanced renal disease can impact treatment options and cause significant morbidity. Patients with ESRD requiring dialysis are a group with currently unmet needs. In this collaborative study, we identified 362 patients from three European centers who initiated dialysis during the course of the disease.
Foteini Theodorakakou, Lilli Sophie Sester, Ute Hegenbart, Paolo Milani, Claudia Bellofiore, Erasmia Psimenou, Charikleia Gakiopoulou, Meletios A. Dimopoulos, Giovanni Palladini, Stefan O. Schönland, Efstathios Kastritis   +10 more
wiley   +1 more source