A 66-year-old Asian woman presented with severe kidney injury, microscopic haematuria and subnephrotic range proteinuria with elevated serum anti-glomerular basement membrane (anti-GBM) titre. She had a history of renal cell carcinoma. Renal biopsy revealed dual pathology with immunofluorescence showing 3+ linear glomerular IgG staining and 3+ IgA mesangial staining. Cellular crescents were present on light microscopy and electron microscopy revealed increased mesangial matrix. She was treated with plasma exchange and immunosuppression and remained in stage 4 chronic kidney disease. This case describes the coexistence of anti-GBM disease and IgA nephropathy, a phenomenon not well described in the literature. The report also explores the association of malignancy and glomerulonephritis as well as the role of genetics and the utility of human leukocyte antigen (HLA) typing in risk stratification.
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http://dx.doi.org/10.1136/bcr-2020-236555 | DOI Listing |
World J Emerg Med
January 2024
Department of Emergency, General Hospital of Tianjin Medical University, Tianjin 300052, China.
Adv Kidney Dis Health
November 2024
Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:
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.
View Article and Find Full Text PDFBMJ Case Rep
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.
View Article and Find Full Text PDFClin 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 PDFKidney Res Clin Pract
November 2024
Division of Nephrology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Republic of Korea.
Kidney transplantation is recognized as an effective treatment for end-stage renal disease in Alport syndrome, demonstrating outcomes comparable to or even superior to those in other causes of renal failure. When considering living related donor kidney transplantation for Alport syndrome patients, it is crucial to consider genetic factors during the donor selection process. In addition to a comprehensive health check, genetic testing is strongly recommended for potential donors at risk of carrying mutations in COL4A3-COL4A5 before undergoing kidney transplantation.
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