門脈圧亢進症の原因への手掛かり。雨粒を見てください

Clue to the cause of portal hypertension: Look at the raindrops.

• Shubhra Mishra
• Rahul Mahajan
• Uma Saikia
• Pankaj Gupta
• Usha Dutta
• Vishal Sharma
• Harshal S Mandavdhare

抄録

門脈圧亢進症は、脾腫を伴うか否かにかかわらず、脾腫大と食道胃静脈瘤を特徴とする臨床症候群である。非硬直性門脈圧亢進症（NCPH）は門脈圧亢進症の前症候群に属している．症例は35歳の若年男性で，2回の上部消化管出血を呈した．内視鏡検査で大きな食道静脈瘤を認めた。全身検査では全身にびまん性の色素沈着性丘疹・結節を認め，掌蹠肥厚を認めた．肝機能検査は正常であった．腹部CT検査で門脈と脾臓静脈が拡張して脾腫大となり，肝臓サイズは正常，病理組織検査では非寛解性門脈線維症（NCPF）と診断された．皮膚病変に対する内服薬の既往歴を有していた。調査の結果、慢性ヒ素症が発見され、これがNCPFの基礎病因である可能性が高いと考えられた。したがって、NCPFの患者を評価する際には、特にインドでは内服薬の使用を除外することが重要である。

Portal hypertension is a clinical syndrome characterized by splenomegaly, with or without hypersplenism, and esphagogastric varices. Non-cirrhotic portal hypertension (NCPH) belongs to the presinusoidal sub category of portal hypertension. We present the case of a young 35-year-old male who had presented with two episodes of upper gastrointestinal bleed. On endoscopy, he was found to have large esophageal varices. On general physical examination, there were diffuse hyperpigmented papules and nodules all over the body, with palmoplantar thickening. His liver function tests were normal. Computed tomography of the abdomen showed a dilated portal and splenic vein with splenomegaly and normal liver size and histopathology showed non-cirrhotic portal fibrosis (NCPF). He had history of intake of oral indigenous medications for skin lesions. On investigating, patient was found to have chronic arsenicosis, which was likely the underlying etiology for NCPF as well. Hence, while evaluating patients of NCPF, it is imperative to rule out the use of indigenous medications, especially in the Indian scenario.

© 2019 The Authors. JGH Open: An open access journal of gastroenterology and hepatology published by Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.