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KMID : 0367419940370081175
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Journal of Korean Pediatric Society
1994 Volume.37 No. 8 p.1175 ~ p.1181
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A Case of Anti-Neutrophil Cytoplasmic Antibodies (ABCA) Positive Wegener's Granulomatosis
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김원태
김우정/김준식/강진무/박관규
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Abstract
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Wegener's granulomatosis is a disease of unknown etiology that is characterized by the clinicopathologic complex of necrotixing 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 f arthralgia, hematuria, convulsion and associated with otitis media and sinusitis. Serologic test of C-ANCA was positive 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.
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