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서영진 ( Young Jin Seo ),김윤정 ( Yoon Jung Kim ),문지미 ( Ji Mi Moon ),최혜윤 ( Hae Yoon Choi ),우진현 ( Jin Hyun Woo ),최성재 ( Seong Jae Choi ),이영호 ( Young Ho Lee ),지종대 ( Jong Dae Ji ),정운용 ( Woon Yong Jung ),송관규 ( G 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.6
Rapidly progressive glomerulonephritis (RPGN) in Wegener`s granulomatosis patients typically has been characterized by pauci-immune glomerulonephritis (PIGN). In some patients, however, significant amount of glomerular immune deposits was detected and reported that they may have poor prognosis. A 30 year-old-female visited due to the skin rash of both lower extremities, arthralgia and nasal stiffness. She had sinusitis, lung opacity, and proteinuria. Serologic PR-3 ANCA was positive and histologic findings of nasal cavity and lung also showed necrotizing vasculitis and granuloma. Thus we could diagnose Wegener`s granulomatosis. However, gross hematuria developed and renal function worsened in spite of treatment with high dose prednisolone and oral cyclophosphamide. Therefore we performed a kidney biopsy. The kidney biopsy showed crescentic glomerulonephritis with Ig A deposition in the mesangium. We experienced a case of Wegener`s granulomatosis patient with significant IgA deposition in glomeruli. We report this case with brief review of the literature.