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Background: Both intrinsic renal cells and immune cells contribute to driving renal inflammation and damage. However, the respective roles of intrinsic renal cells and immune cells in crescentic glomerulonephritis, and the key molecular factors driving pathogenesis are still unclear.

Methods: The roles of intrinsic renal cells and renal infiltrating immune cells in crescent formation were explored using renal transplantation after experimental anti-GBM disease induction in 129x1/svJ and C57BL/6J mice.

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Rapidly progressive glomerulonephritis (RPGN) is a syndrome characterized by a swift decline in kidney function, often over a few months, accompanied by features of nephritic syndrome. It can result in decreased urine output and commonly involves the presence of extensive crescents in kidney biopsies. RPGN is classified into 3 main types based on immune deposit distribution and visualization through immunofluorescence and electron microscopy: antiglomerular basement membrane disease, immune complex glomerulonephritis, and pauci-immune glomerulonephritis.

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Atypical anti-GBM disease in pregnancy.

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November 2024

Kidney Health Service Metronorth, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia.

A woman in her 20s presented with nephrotic syndrome and hyperemesis in early pregnancy. Pertinent initial investigations revealed a severe acute kidney injury, a serum albumin of 19 g/L, a random protein creatinine ratio of 800 g/mol and microscopic haematuria. All immunological and infection serology testing including anti-glomerular basement membrane (anti-GBM; ELISA) were negative.

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Pediatric double-seropositive anti-glomerular basement membrane antibody disease: A case report and literature review.

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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.

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