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A Case of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) Positive Wegener's Granulomatosis

Journal of the Korean Pediatric Society 1994;37(8):1175-1181.
Published online August 15, 1994.
A Case of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) Positive Wegener's Granulomatosis
Won Tae Kim1, Woo Jeong Kim1, Joon Sik Kim1, Chin Moon Kang1, Kwan Kyu Park2
1Department of Pediatrics, Keimyung University, School of Medicine, Taegu, Korea
2Department of Pathology, Keimyung University, School of Medicine, Taegu, Korea
항호중구 항체(ANCA)양성의 소아 Wegener 육아종증 1례
김원태1, 김우정1, 김준식1, 강진무1, 박관규2
1계명대학교 의과대학 소아과학교실
2계명대학교 의과대학 병리학교실
Abstract
Wegener's granulomatosis is a disease of unknown etiology that is characterized by the clinicopathologic complex of necrotizing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and variable degrees of small vessel vasculitis. Recently Antineutrophil Cytoplasmic Antibody (ANCA) has been reported to be a highly specific test for the diagnosis of Wegener's granulomatosis. We have experienced a patient of Wegener's granulomatosis in a 11 year old girl who was admitted with complaints of arthralgia, hematuria, convulsion and associated with otitis media and sinusitis. Serologic test of C-ANCA was poitive and histologic findings of the kidney showed crescentic glomerulonephritis with sclerosis and surrounding infiltration of multinucleated giant cells. Patient was treated with pulse methylprednisolone without improvement. The clinical course progressed rapidly and expired due to the renal failure, gastrointestinal bleeding and status epilepticus. A brief review of literatures was made.
Key Words: Wegener's Granulomatosis, Antineutrophil Cytoplasmic Antibodies, Rapidly Progressive Glomerulonephritis


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