Pediatric Infection and Vaccine

The Korean Society of Pediatric Infectious Diseases (대한소아감염학회)
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A Case of Esophageal Obstruction Complicated in a Patient with Chronic Granulomatous Disease - Esophageal obstruction in Chronic Granulomatous Disease

만성육아종질환 환자에서 합병된 식도 폐쇄 사례 -만성육아종질환의 육아종성 식도 폐쇄

  • Kim, Ji Soo (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Hwang, Jinsol (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Choi, Young Hun (Department of Radiology, Seoul National University College of Medicine) ;
  • Kim, Woo Sun (Department of Radiology, Seoul National University College of Medicine) ;
  • Kim, Joong Gon (Department of Pediatrics, Seoul National University College of Medicine)
  • 김지수 (서울대학교 의과대학 소아과학교실) ;
  • 황진솔 (서울대학교 의과대학 소아과학교실) ;
  • 최영훈 (서울대학교 의과대학 영상의학교실) ;
  • 김우선 (서울대학교 의과대학 영상의학교실) ;
  • 김중곤 (서울대학교 의과대학 소아과학교실)
  • Received : 2013.05.24
  • Accepted : 2013.09.09
  • Published : 2014.04.25
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Abstract

Chronic granulomatous disease (CGD) is an inherited immunodeficient disease characterized by recurrent infections and granuloma formation. Granulomatous obstruction of esophagus is one of the rare complications of CGD. The use of steroids and antimicrobials for esophageal obstruction by granuloma in CGD patients has been controversial due to the possibility of concomitant inapparent infection. We report a case of esophageal obstruction in an 8-year-old CGD patient showing the poor response to antibiotics therapy. However, dramatic improvement of symptoms and radiologic findings of esophageal obstruction were achieved after steroid therapy. One month after discontinuation of steroid, esophageal obstruction recurred and the patient was re-treated with steroid. After that time, he experienced one more recurrence of esophageal obstruction. This symptom subsided after antibiotics therapy without steroid and he has been followed up to the present without further relapse.

CGD 환자에서는 면역 체계의 결함으로 인한 반복적인 감염과 육아종 형성이 특징적이다. CGD 환자에서 육아종의 형성에 의한 식도 폐쇄는 매우 드문 합병증 중 하나로 CGD 환자의 육아종 형성에 의한 식도 폐쇄의 치료에 대해서는 불현성 감염이 동반될 가능성이 있어 스테로이드 치료와 항생제 치료에 대한 논란이 있어왔다. 저자들은 항생제 치료에 반응하지 않는 육아종 형성에 의한 식도 폐쇄 증상을 보인 8세 만성육아종질환 환자를 보고하는 바이다. 환자는 스테로이드 치료 이후에 임상적 증상과 영상 검사 상의 급격한 호전을 보였으나 스테로이드 치료를 중단한 지 한 달 뒤 환자의 식도 폐쇄 증상은 재발하였고, 이는 스테로이드를 재 투약 후 호전되었다. 스테로이드 중단 이후 한 차례 다시 재발하였으나, 항생제 치료 후 호전되었고 이후 추가적 재발 없이 경과 관찰 중이다.

Keywords

References

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