Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Intestinal pseudo-obstruction as the initial presentation of systemic lupus erythematosus in a 13-year-old girl

장 가성 폐쇄로 진단된 전신 홍반 루푸스 1예

  • Cho, Ky Young (Department of Pediatrics, School of Medicine, Ewha Womans University) ;
  • Khil, Tae Young (Department of Pediatrics, School of Medicine, Ewha Womans University) ;
  • Ahn, Hye Mi (Department of Pediatrics, School of Medicine, Ewha Womans University) ;
  • Lee, Sun Wha (Department of Radiology, School of Medicine, Ewha Womans University) ;
  • Seo, Jeong Wan (Department of Pediatrics, School of Medicine, Ewha Womans University)
  • 조기영 (이화여자대학교 의과대학 소아과학교실) ;
  • 길태영 (이화여자대학교 의과대학 소아과학교실) ;
  • 안혜미 (이화여자대학교 의과대학 소아과학교실) ;
  • 이선화 (이화여자대학교 의과대학 영상의학과학교실) ;
  • 서정완 (이화여자대학교 의과대학 소아과학교실)
  • Received : 2008.01.17
  • Accepted : 2008.03.18
  • Published : 2008.06.15
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Abstract

Intestinal pseudo-obstruction (IPO) is a rare and poorly understood manifestation of systemic lupus erythematosus (SLE), especially in children. The characteristic clinical feature of IPO is obstruction without an identifiable obstructive lesion. The authors a 13-year-old girl whose first symptom of SLE was IPO. The patient presented with a 3-day history of nausea, bilious vomiting, abdominal distention, and no bowel movement. Simple abdominal radiographs revealed mild dilatation with partial air-fluid levels in the small intestine. Abdominal CT and methylcellulose small bowel studies showed massive ascites, engorgement of the small mesenteric vessels, pleural effusion, and diffuse bowel wall thickening of the gastric antrum, duodenum. and jejunum. The delayed passage of contrast for 15 days after the methylcellulose small bowel studies was suggestive of decreased bowel motility. Laboratory findings were positive for ANA, anti-double-stranded DNA, anti-Smith and lymphopenia. After 10-day treatment with high-dose corticosteroids, the symptoms improved. IPO associated with SLE should be considered in the differential diagnosis for patients presenting with symptoms of intestinal obstruction. Early recognition of IPO in SLE and appropriate therapy are important for prevention of complications and unnecessary surgery. This case raises awareness among pediatricians that although rare, IPO can be the presenting symptom of SLE in children.

전신 홍반 루푸스는 다양한 증상으로 발현되는 자가면역질환이다. 위장관 증상도 질환의 경과 중에 나타날 수 있으나, 장 가성 폐쇄로 처음 진단되는 경우는 소아에서 매우 드물다. 장 가성 폐쇄는 원발성 또는 속발성으로 장의 평활근이나 신경계에 이상이 있어 해부학적 원인 없이 장폐쇄의 증상과 징후가 나타나는 것이며, 장폐쇄로 인하여 수술을 하였다는 보고도 있다. 그러나 장 가성 폐쇄가 전신 홍반 루푸스에 속발한 경우 장간막 혈관의 폐쇄와 장괴사로 진행하기 전에 조기에 진단하고 치료하면 합병증을 예방하고 수술을 피할 수 있다. 저자들은 장 가성 폐쇄의 증상으로 내원한 13세 여아에서 전신 홍반 루푸스를 진단하여, 불필요한 수술을 피하고 조기에 치료한 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Keywords

References

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  1. Intestinal Pseudo-Obstruction as an Initial Manifestation of Systemic Lupus Erythematosus vol.13, pp.3, 2008, https://doi.org/10.5217/ir.2015.13.3.282