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Malays J Med Sci
December 2024
Nephrology Division, Department of Paediatric, Faculty of Medicine, Universitas Brawijaya, Dr. Saiful Anwar General Hospital, Malang, Indonesia.
Nephrotic syndrome is the most common glomerular disease in children. While the exact pathogenesis of nephrotic syndrome is not fully understood, recent research has shed light on some of the underlying mechanisms involved in it. Improvement by B cell depletion therapy using antiCD20 in nephrotic syndrome has led to a paradigm shift from immunoinflammatory disease influenced by T cell dysregulation to B cell involvement in the pathogenesis of nephrotic syndrome.
View Article and Find Full Text PDFJ Clin Lipidol
December 2024
Department of Internal Medicine, Erasmus MC Cardiovascular Institute, University Medical Center Rotterdam, Rotterdam, the Netherlands. Electronic address:
This report describes a rare case of lipoprotein glomerulopathy. A 63 year-old man presented with nephrotic syndrome unresponsive to rituximab and tacrolimus. Blood tests showed a mild- to moderate hypertriglyceridemia suggesting familial dysbetalipoproteinemia (FD).
View Article and Find Full Text PDFClin Nephrol Case Stud
January 2025
Department of Medicine.
Minimal change disease (MCD) accounts for 10 - 15% of idiopathic nephrotic syndromes in adults. Chronic hepatitis C virus (HCV) infection is rarely ascribed as a cause of MCD and was previously associated with interferon-based therapy. MCD in treatment-naïve chronic HCV infection is extremely rare, with only 3 cases reported in the literature.
View Article and Find Full Text PDFNarra J
December 2024
Eijkman Research Center for Molecular Biology, National Research and Innovation Agency (BRIN), Bogor, Indonesia.
Nephrotic syndrome, a multifaceted medical condition characterized by significant proteinuria, has recently prompted a reorientation of research efforts toward B-cell-mediated mechanisms. This shift underscores the pivotal role played by B-cells in its pathogenesis. The aim of this study was to explore potential therapeutic pathways, with specific attention given to compounds found in , including withanolides, such as physalins, which constitute one of the five distinct withanolide subgroups identified in .
View Article and Find Full Text PDFPediatr Nephrol
January 2025
Department of Nephrology, Wuhan Children's Hospital (Wuhan Maternal and Child Healthcare Center), Tongji Medical College, Huazhong University of Science & Technology, Wuhan, China.
Background: Steroid-resistant nephrotic syndrome (SRNS) is insensitive to steroid therapy and overwhelmingly progresses to kidney failure (KF), the known pathogenic genes of which include key subunits of the nuclear pore complex (NPC), a less-recognized contributor to glomerular podocyte injury.
Methods: After analyzing their clinical characterizations and obtaining parental consent, whole-exome sequencing (WES) was performed on patients with SRNS. Several nucleoporin (NUP) biallelic pathogenic variants were identified and further analyzed by cDNA-PCR sequencing from white cells of peripheral blood, minigene assay, immunohistochemical (IHC) staining, and electron microscopy (EM) ultrastructure observation of kidney biopsy, as well as multiple in silico prediction tools, including 3D protein modeling.
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