Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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A Case of Idiopathic Portal Hypertension in a 3-year-old Girl

3세 여아에서 진단된 특발성 문맥 고혈압 1예

  • Son, Ki-Young (Department of Pediatrics, Yonsei University College of Medicine) ;
  • Baek, Seoung-Yon (Department of Pediatrics, Yonsei University College of Medicine) ;
  • Chung, Ki-Sup (Department of Pediatrics, Yonsei University College of Medicine)
  • 손기영 (연세대학교 의과대학 소아과학교실) ;
  • 백승연 (연세대학교 의과대학 소아과학교실) ;
  • 정기섭 (연세대학교 의과대학 소아과학교실)
  • Received : 2007.07.18
  • Accepted : 2007.08.30
  • Published : 2007.09.30
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Abstract

A previously healthy 3-year-old girl was admitted to the Department of Pediatrics in Severance Hospital with sudden symptoms of melena. The vital signs were stable, and splenomegaly was found in a physical examination. The patient had moderate thrombocytopenia. There was no evidence of autoimmune disease. A upper gastrointestinal endoscopy and esophagogram showed a varix on the lower esophagus. Coarse liver parenchymal echoes and increased periportal echogenicity were seen on a Doppler sonogram. The velocity of the portal vein mildly increased. Magnetic-resonance-cholangiopancreatogram (MRCP) demonstrated normal portal structures. A sono-guided liver biopsy was performed, but the pathological findings were unremarkable. Based on these findings, we diagnosed the patient with idiopathic portal hypertension. The patient was discharged and was treated with oral beta blocker. We report a case of idiopathic portal hypertension with a brief review of the literature.

저자들은 혈변을 호소하였던 3세된 여아에서 소아에서 비교적 드문 식도 정맥류와 비종대가 합병된 특발성 문맥 고혈압 1예를 경험하였기에 문헌 고찰과 더불어 보고하는 바이다.

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