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Sci Rep
January 2025
Department of Immunology, Genetics and Pathology, Uppsala University, Rudbeck Laboratory, C11, 75185, Uppsala, Sweden.
The existence of transmissible amyloid fibril strains has long intrigued the scientific community. The strain theory originates from prion disorders, but here, we provide evidence of strains in systemic amyloidosis. Human AA amyloidosis manifests as two distinct clinical phenotypes called common AA and vascular AA.
View Article and Find Full Text PDFMed Clin (Barc)
January 2025
China-Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100029, China; Department of Nephrology, China-Japan Friendship Hospital, Beijing 100029, China. Electronic address:
Objectives: Crescents play important roles in the pathophysiology of patients with biopsy-proven diabetic nephropathy (DN). However, their relationship to disease severity and progression has not been fully clarified.
Methods: We assessed 142 participants in a retrospective cohort study of biopsy-proven DN.
J Adv Res
January 2025
Guangdong Provincial Key Laboratory of Autophagy and Major Chronic Non-communicable Diseases, Clinical Research and Experimental Center, Department of Nephrology, Affiliated Hospital of Guangdong Medical University, Zhanjiang 524000, China; Department of Clinical Laboratory, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University. Guangzhou 510120, China. Electronic address:
Introduction: Developing strategies to improve the therapeutic efficacy of mesenchymal stem cell (MSC)-derived extracellular vesicles (EVs) in autoimmune diseases have garnered increased attention.
Objectives: To evaluate whether rapamycin-induced autophagy within the systemic lupus erythematosus (SLE) inflammatory microenvironment (Rapa-SLE) augments the therapeutic effects of MSC-derived EVs in SLE.
Methods: The therapeutic potential of the resulting EVs (Rapa-SLE-EV) was assessed in MRL/lpr mice.
Pathol Res Pract
January 2025
Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, China.
Anti-vascular endothelial growth factor-associated thrombotic microangiopathy (aVEGF-TMA) was recently discovered in patients with malignant tumors. Four aVEGF-TMA patients diagnosed by renal biopsy between 2018 and 2022 were identified, and all were females aged 30-62 years (mean age, 47 years). Two patients with malignant gastrointestinal stromal tumors who received sunitinib were analyzed.
View Article and Find Full Text PDFNephrology (Carlton)
February 2025
Phoenix Children's Hospital-Thomas Campus, Phoenix, Arizona, USA.
Complement 3 glomerulonephritis (C3GN) is a rare glomerular disease involving dysregulation of the complement system. We describe our experience using pegcetacoplan, an inhibitor of C3 and its activation fragment, C3b, for treatment-resistant C3GN in a 9-year-old boy referred for evaluation of refractory membranoproliferative glomerulonephritis. Despite treatment with intense immunosuppression (high-dose steroids, mycophenolate mofetil and calcineurin inhibitor), he continued to have high disease activity with low C3 levels (35 mg/dL), hypertension, symptomatic oedema, anaemia, and nephrotic-range proteinuria (e.
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