Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
권호 표지

A Case of Kimura's Disease Occurring During Remission of Steroid-responsive Nephrotic Syndrome

소아에서 스테로이드 반응성 신증후군의 관해기에 발병한 Kimura 병의 1례

  • Bae Keun-Wook (Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine) ;
  • Lee Min-Kyu (Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine) ;
  • Lee Joo-Hoon (Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine) ;
  • Huh Joo-Ryung (Department of Pathology, Asan Medical Center, University of Ulsan, College of Medicine) ;
  • Park Young-Seo (Department of Pediatrics, Asan Medical Center, University of Ulsan, College of Medicine)
  • 배근욱 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 이민규 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 이주훈 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 허주영 (울산대학교 의과대학 서울아산병원 진단병리과) ;
  • 박영서 (울산대학교 의과대학 서울아산병원 소아과)
  • Published : 2006.04.01
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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.

저자들은 스테로이드 반응성 재발성 신증후군의 과거력이 있는 환아에서 관해기에 발병한 Kimura 병을 종괴의 전절제 후 스테로이드 및 cyclosporin 투여로 치료한 증례를 경험하고 이를 보고하는 바이다.

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