Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A Case of Alport Syndrome Associated with Diffuse Esophageal Leiomyomatosis

식도 평활근종증이 동반된 Alport 증후군 1례

  • Jung, Jin Young (Department of Pediatrics, College of Medicine, University of Ulsan) ;
  • Kim, Cheol-Min (Department of Pediatrics, College of Medicine, University of Ulsan) ;
  • Lim, Yean Jung (Department of Pediatrics, College of Medicine, University of Ulsan) ;
  • Kim, Ja Hyung (Department of Pediatrics, College of Medicine, University of Ulsan) ;
  • You, Chong Woo (Department of Diagnostic Pathology, College of Medicine, University of Ulsan) ;
  • Choi, Bo-Hwa (Department of Pediatrics, College of Medicine, University of Ulsan) ;
  • Hong, Soo-Joung (Department of Pediatrics, College of Medicine, University of Ulsan) ;
  • Park, Young Seo (Department of Pediatrics, College of Medicine, University of Ulsan)
  • 정진영 (울산대학교 의과대학 소아과학교실) ;
  • 김철민 (울산대학교 의과대학 소아과학교실) ;
  • 임연정 (울산대학교 의과대학 소아과학교실) ;
  • 김자형 (울산대학교 의과대학 소아과학교실) ;
  • 유종우 (울산대학교 의과대학 진단병리과학교실) ;
  • 최보화 (울산대학교 의과대학 소아과학교실) ;
  • 홍수종 (울산의대 서울아산병원 소아과) ;
  • 박영서 (울산대학교 의과대학 소아과학교실)
  • Received : 2002.04.24
  • Accepted : 2002.07.05
  • Published : 2002.09.15
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Abstract

We report a case of Alport syndrome associated with esophageal leiomyomatosis, presenting as recurrent pneumonia. A 5-year old girl who had a history of cataract visited the out patient clinic with a complaint of recurrent wheezing and respiratory difficulty which had started five months previously. Chest magnetic resonance image(MRI) and esophagography, checked on the suspicion of achalasia, revealed esophageal leiomyomatosis and renal biopsy revealed Alport syndrome. In the pediatric population, this tumor is a rare cause of dysphagia and is often misdiagnosed as an esophageal motility disorder. Although a number of Alport syndrome associated with leiomyomatosis were reported in the literature, this is a second case report presented with recurrent pneumonia in Korea.

저자들은 반복적인 천명과 폐렴을 주소로 내원한 환아에서 식도 평활근종이 동반된 Alport 증후군 1례를 경험하였기에 보고하는 바이다.

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