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Pediatric double-seropositive anti-glomerular basement membrane antibody disease: A case report and literature review.
Clin Nephrol Case Stud
November 2024
Department of Pediatrics, Cheikh Khalifa International University Hospital, Faculty of Medicine, Mohammed VI University of Health Sciences (UM6SS), Casablanca, Morocco.
Introduction: Glomerular basement membrane (GBM) disease is a severe and exceedingly rare disorder characterized by the presence of circulating antibodies targeting the non-collagen NC1 domain of the α3 chain of collagen type IV in glomerular and alveolar basement membranes. It typically presents as rapidly progressive glomerulonephritis (RPGN), often accompanied by pulmonary hemorrhage. The occurrence of double-seropositivity for anti-GBM antibody and anti-neutrophil cytoplasmic antibody (ANCA), primarily with myeloperoxidase specificity (MPO-ANCA), is particularly uncommon in pediatric cases.
View Article and Find Full Text PDFNon-accidental trauma in the setting of hemophilia A - A case report.
Trauma Case Rep
December 2024
Wake Forest University School of Medicine, United States of America.
Hundreds of thousands of children are victims of non-accidental trauma each year. Its presentation can vary, and it may be difficult to recognize. However, due to its increased risk for fatality, it is imperative that physicians are aware and receptive to the signs of non-accidental trauma to assist with early detection.
View Article and Find Full Text PDFOutcome of rituximab treatment in children with non-dialysis-dependent anti-GBM disease.
Pediatr Nephrol
February 2025
Department of Pediatrics, Dr. v. Hauner Children's Hospital, LMU University Hospital, LMU Munich, Munich, Germany.
Article Synopsis
- Anti-GBM disease is a rare and serious condition characterized by the body producing antibodies that attack a specific type of collagen in the kidneys, leading to quick kidney failure if not treated.
- Current treatment guidelines recommend using plasma exchanges and drugs like steroids and cyclophosphamide, but cyclophosphamide can have severe side effects, prompting the search for alternatives like rituximab and mycophenolate mofetil.
- A study analyzing five adolescent patients treated with rituximab and mycophenolate mofetil found promising results, with 80% showing preserved or improved kidney function after treatment, suggesting these alternatives may be less toxic and effective.
Anti-GBM Nephritis in an 11-Year-Old Female Child: A Rare Case Report.
Cureus
August 2024
Paediatrics, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, IND.
Anti-glomerular basement membrane (GBM) nephritis is a rare autoimmune condition involving the glomerular basement membrane of the kidneys. This case report describes an 11-year-old female who presented with edema, decreased urine output, and altered sensorium, progressing to hypertension and requiring emergent hemodialysis. A renal biopsy showing Immunoglobulin G (IgG) linear deposits confirmed the diagnosis.
View Article and Find Full Text PDFPathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome.
Int J Mol Sci
May 2024
Department of Respiratory Medicine, Medical School, Democritus University of Thrace, 68100 Alexandroupolis, Greece.
Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease.
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