Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Development of Crohn disease in patients with myelodysplastic syndrome : report of two children

골수이형성 증후군으로 진단받은 소아에서 발생한 크론병

  • Shim, Jeong Ok (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Seo, Jeong Kee (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Yang, Hye Ran (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Ko, Jae Sung (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Shin, Hee Young (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Ahn, Hyo Seop (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Kim, Woo Sun (Department of Radiology, Seoul National University College of Medicine) ;
  • Kang, Gyeong Hoon (Department of Pathology, Seoul National University College of Medicine)
  • 심정옥 (서울대학교 의과대학 소아과학교실) ;
  • 서정기 (서울대학교 의과대학 소아과학교실) ;
  • 양혜란 (서울대학교 의과대학 소아과학교실) ;
  • 고재성 (서울대학교 의과대학 소아과학교실) ;
  • 신희영 (서울대학교 의과대학 소아과학교실) ;
  • 안효섭 (서울대학교 의과대학 소아과학교실) ;
  • 김우선 (서울대학교 의과대학 진단방사선과학교실) ;
  • 강경훈 (서울대학교 의과대학 병리학교실)
  • Received : 2005.08.11
  • Accepted : 2005.10.06
  • Published : 2006.01.15
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Abstract

Crohn disease (CD) is rare, but the incidence of CD has been increasing over the past ten years. We found two cases of CD, associated with myelodysplastic syndrome (MDS), for the first time in children. In the first patient, MDS was diagnosed at three years of age and CD developed later at eight years of age. The patient presented with recurrent abdominal pain, diarrhea, bloody stools and failure to thrive. Colonoscopy revealed cobble stone like mucosa and mass like lesions with superficial ulceration and inflammatory exudates, observed from the cecum to ascending colon. Ileo-cecal biopsy samples showed ulcers with skipped areas and lymphoid infiltrations. The patient was started on treatment with mesalazine and deflazacort, and symptoms remitted. In the second patient, MDS was diagnosed at nine years of age and CD developed at 13 years of age. This patient has recurrent hematochezia, abdominal pain, vomiting and fever. Colonoscopy revealed a large, deep indurative ulceration on the cecal side of the ileo-cecal valve. Ileocecectomy was done, and histology revealed ulceration with transmural inflammation and lymphoid aggregates. Symptoms improved after ileocecectomy.

크론병은 매우 드문 질환이나 지난 10년간 발생률이 꾸준히 증가하고 있다. 저자들은 골수 이형성 증후군을 가진 환아에서 크론병이 발병한 2례를 소아에서는 최초로 보고하는 바이다. 첫번째 환아는 3세에 골수 이형성 증후군으로 진단받았고, 수 년간 지속된 반복적인 복통 및 설사, 혈변, 성장 부전이 있어 8세에 크론병으로 진단받았다. 대장 내시경 검사에서는 맹장에서 오름 결장에 걸쳐 조약돌상 점막과 표재성 궤양 및 염증성 삼출이 있었으며, 조직 소견은 궤양 사이에 정상 점막을 포함하고 있으면서 림프구 침윤을 보였다. Mesalazine과 deflazacort로 치료 후 증상은 호전을 보였다. 두 번째 환아는 9세에 골수 이형성증후군으로 진단받았으며, 13세에 반복되는 혈변과 복통, 구토, 발열로 크론병으로 진단받았다. 대장 내시경 검사에서 크고 깊은 경화성 궤양이 회맹판에서 맹장 쪽 주위에서 발견되었다. 이 외의 부위에는 병변이 없어 병변을 절제하였고, 조직은 경벽 염증과 림프구 집합을 동반한 궤양 소견을 보였다. 절제술 후 증상은 호전을 보였다.

Keywords

References

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