A Case of Idiopathic Portal Hypertension in a 3-year-old Girl

K. Son, S. Baek, K. Chung
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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. (Korean J Pediatr Gastroenterol Nutr 2007; 10: 221∼ 225)
特发性门静脉高压症1例3岁女童
一名先前健康的3岁女孩因突然出现黑痘症状而被塞伯伦斯医院儿科收治。生命体征稳定,体格检查发现脾肿大。患者有中度血小板减少症。没有自身免疫性疾病的证据上消化道内镜及食道造影显示下食道静脉曲张。多普勒超声显示肝实质回声粗大,门静脉周围回声增强。门静脉流速轻度增高。磁共振胆管造影显示门静脉结构正常。超声引导下行肝活检,病理结果无明显变化。基于这些发现,我们诊断患者为特发性门静脉高压症。患者出院并接受口服受体阻滞剂治疗。我们报告一例特发性门静脉高压症,并简要回顾文献。韩国儿科胃肠病学杂志2007;10: 221 ~ 225)
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