Anti glomerular basement membrane disease (AGBM) is an autoinmune disorder characterised by the presence of anti-glomerular basement membrane (Anti-GBM) antibodies, alveolar hemorrhage, necrotizing glomerulonephritis, and linear deposition of immunoglobulins through direct inmunofluorescence. Genetic predisposition, among other factors, plays an important role in the development of the disease. Previous studies have shown that HLA-DR15 and HLA-DR4 increase the risk of presenting it, while HLA-DR1 and HLA-DR7 protect against its development. We describe the first case of two non-twin siblings with AGBM and identical HLA, with HLA-DR4 as risk factor and HLA-DR7 as protection factor. We propose the importance of analysing HLA in siblings of patients with AGBM, to determine the degree of genetic susceptibility and to carry out a close follow-up on them, with the aim of achieving an early diagnosis and treatment in case of presenting the disease.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.nefroe.2021.04.010 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Biomarkers of Extracellular Matrix Fragments in Patients with Psoriasis.
Int J Mol Sci
December 2024
Department of Dermatology and Allergy, Copenhagen University Hospital-Herlev and Gentofte, 2900 Hellerup, Denmark.
Blood-based extracellular matrix (ECM) fragments have been identified as potential pharmacologic biomarkers in spondyloarthritis and diagnostic biomarkers in psoriatic arthritis and psoriasis vulgaris. This study aimed to explore whether ECM fragments can differentiate patients with psoriasis from healthy controls (HC) and determine their potential as biomarkers for response to treatment in psoriasis. The study population included 59 patients with moderate to severe psoriasis, not receiving systemic anti-psoriatic treatment at inclusion, and 52 HC matched by age, sex, and BMI.
View Article and Find Full Text PDFIntegrin-activating protein Invasin sustains long-term expansion of primary epithelial cells as 2D organoid sheets.
Proc Natl Acad Sci U S A
January 2025
Oncode Institute, Hubrecht Institute-Royal Netherlands Academy of Arts and Science, Utrecht 3584 CT, The Netherlands.
Matrigel/BME, a basement membrane-like preparation, supports long-term growth of epithelial 3D organoids from adult stem cells [T. Sato , , 262-265 (2009); T. Sato , , 1762-1772 (2011)].
View Article and Find Full Text PDFSarcolemma resilience and skeletal muscle health require O-mannosylation of dystroglycan.
Skelet Muscle
January 2025
Department of Molecular Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, Senator Paul D. Wellstone Muscular Dystrophy Specialized Research Center, Roy J. and Lucille A. Carver College of Medicine, The University of Iowa, Iowa City, Iowa, USA.
Background: Maintaining the connection between skeletal muscle fibers and the surrounding basement membrane is essential for muscle function. Dystroglycan (DG) serves as a basement membrane extracellular matrix (ECM) receptor in many cells, and is also expressed in the outward-facing membrane, or sarcolemma, of skeletal muscle fibers. DG is a transmembrane protein comprised of two subunits: alpha-DG (α-DG), which resides in the peripheral membrane, and beta-DG (β-DG), which spans the membrane to intracellular regions.
View Article and Find Full Text PDFRepair of Descemet's membrane detachment following inadvertent intrastromal viscoelastic injection for hypotony: anterior and posterior approaches.
BMJ Case Rep
January 2025
Department of Ophthalmology, Royal Free London NHS Foundation Trust, London, UK
Descemet's membrane (DM) detachment is a significant, if rare, risk of intracameral injection of viscoelastic to treat hypotony after glaucoma filtration surgery. We describe two cases of DM detachment following inadvertent injection of viscoelastic into the posterior stroma and the techniques used for their repair. In both cases, conventional air tamponade failed to resolve the detachment, and further surgical intervention was required.
View Article and Find Full Text PDFObjective: In severe equine asthma, structural remodeling of the airways ultimately leads to bronchial wall thickening and airflow obstruction. Increased bronchial vascularization has been described in horses affected by the severe form of the disease, but whether it contributes to bronchial remodeling in milder forms of asthma remains to be determined. In a blinded, retrospective case-control study, we evaluated the presence of bronchial angiogenesis in horses with mild and moderate equine asthma (MEA) and its correlation to airway smooth muscle remodeling.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!