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Thin Glomerular Basement Membrane Disease를 동반한 베게너육아종증 1예

Other Titles
 A Case of Wegener`s Granulomatosis with Thin Glomerular Basement Membrane Disease 
 황학진  ;  손주익  ;  인병현  ;  박용범  ;  이수곤  ;  정현주  ;  송정식 
 Journal of the Korean Rheumatism Association (대한류마티스학회지), Vol.8(3) : 192-197, 2001 
Journal Title
 Journal of the Korean Rheumatism Association (대한류마티스학회지) 
Issue Date
Wegener's granulomatosis ; Thin glomerular basement membrane disease
Wegener`s granulomatosis is a necrotizing, granulomatous vasculitis that involves multiple organs including the upper and lower respiratory tract and the kidney. The kidney initially exhibits focal necrotizing glomerulonephritis, which progresses to crescentic glomerulonephritis in Wegener`s granulomatosis. We experienced a case of Wegener`s granulmatosis which was associated with a thin glomerular basement membrane disease. The patient suffered from nasal stuffiness, recurrent serous otitis media, and tinnitus. Despite antibiotic therapy and ventral tube insertion, symptoms did not improve and hearing difficulty was aggravated. Ulcerative, necrotizing granulomatous inflammations with multinucleated giant cells were seen on nasal biopsy. She had recurrent microscopic hematuria and the renal biopsy findings by light and immunofluorescent microscopy did not reveal any abnormalities but diffuse thinning of the glomerular basement membrane (226nm) was observed by electronmicroscopy. With the above clinical findings and biopsy results, we diagnosed Wegener`s granulmatosis with thin glomerular basement membrane disease. Thin glomerular basement membrane disease, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and hematuria. Weekly low-dose methotrexate together with prednisone was used as treatment regimen because nonglomerular microscopic hematuria may be the first sign of cyclophosphamide-induced renal toxicity. With the above combination therapy, she felt well-being sense and her hearing difficulty was also much improved. She has been treated as an outpatient with glucocorticoid.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Park, Yong Beom(박용범)
Lee, Soo Kon(이수곤)
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