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Journal of the Korean Society of Pediatric Nephrology 2006;10(1): 52-57.
소아에서 스테로이드 반응성 신증후군의 관해기에 발병한 Kimura 병의 1례
배근욱, 이민규, 이주훈, 허주영, 박영서
1울산대학교 의과대학 서울아산병원 소아과
2울산대학교 의과대학 서울아산병원 소아과
3울산대학교 의과대학 서울아산병원 소아과
4울산대학교 의과대학 서울아산병원 진단병리과
5울산대학교 의과대학 서울아산병원 소아과
A Case of Kimura's Disease Occurring During Remission of Steroid-responsive Nephrotic Syndrome
Keun-Wook Bae, Min-Kyu Lee, Joo-Hoon Lee, Joo-Ryung Huh, Young-Seo Park
1Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
2Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
3Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
4Department of Pathology, Asan Medical Center, University of Ulsan, College of Medicine
5Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine
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ABSTRACT
Kimura's disease is a rare chronic inflammatory disease of unknown etiology which appears primarily in young Asian males as non-tender subcutaneous swellings in the head and neck region. Histologic characteristics are the presence of lymphoid follicles, vascular proliferation and infiltration of eosinophils. Peripheral eosinophilia and elevated serum IgE are frequently combined. Systemic steroid therapy with surgical excision is the mainstay of treatment, though recurrence after surgery or discontinued steroid treatment is common. It has been known that about 16% of the cases are associated with renal diseases, particularly nephrotic syndrome. We present an 8-year-old boy with a past history of steroid-responsive, infrequently relapsing nephrotic syndrome who developed right buccal swelling and peripheral eosinophilia during the remission state. He has been managed with surgical resection, steroid and cyclosporine due to multiple recurrences.
Key words: Kimura's disease | Steroid-responsive nephrotic syndrome
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