Schouten Jeoffrey N L, Verheij Joanne, Janssen Harry L A
Afd. Maag-, darm- en leverziekten, Erasmus Medisch Centrum Rotterdam, The Netherlands.
Ned Tijdschr Geneeskd. 2010;154:A1276.
In three patients, two men aged 57 and 53 years, and a 43-year-old woman, idiopathic portal hypertension, also called non-cirrhotic portal hypertension (NCPH), was diagnosed. The first two patients presented with haematemesis. They were treated by endoscopic rubber band ligation of oesophageal varices. In the second patient, placement of a transjugular intrahepatic portosystemic shunt (TIPS) was necessary due to failure of the ligation treatment. The third patient was treated for HIV infection and had a gastroscopy because of nausea and vomiting, which revealed oesophageal varicosis. None of the patients had liver function impairment. Two of the patients had been treated with medication known to be associated with NCPH (azathioprine for Crohn's disease (second patient) and didanosine for HIV infection (third patient)). These medications were discontinued. The histological features of the patients were heterogeneous (nodular regenerative hyperplasia, periportal fibrosis and periportal dilated structures), but consistent with NCPH. Portal hypertension in the Western world is mostly associated with liver cirrhosis. When portal hypertension occurs in association with patent portal and hepatic veins, and in the absence of liver cirrhosis, NCPH must be considered. The prognosis of this disease is much better than that of cirrhosis.
三名患者被诊断为特发性门静脉高压症，也称为非肝硬化性门静脉高压症（NCPH），其中两名男性患者年龄分别为57岁和53岁，一名女性患者43岁。前两名患者表现为呕血。他们接受了内镜下食管静脉曲张橡皮圈套扎术治疗。第二名患者由于结扎治疗失败，需要进行经颈静脉肝内门体分流术（TIPS）。第三名患者因感染HIV接受治疗，因恶心和呕吐进行了胃镜检查，结果显示有食管静脉曲张。所有患者均无肝功能损害。其中两名患者曾使用过已知与NCPH相关的药物（第二名患者因克罗恩病使用硫唑嘌呤，第三名患者因HIV感染使用去羟肌苷）。这些药物已停用。患者的组织学特征各不相同（结节性再生性增生、门静脉周围纤维化和门静脉周围扩张结构），但与NCPH一致。在西方世界，门静脉高压大多与肝硬化有关。当门静脉高压与门静脉和肝静脉通畅同时出现，且无肝硬化时，必须考虑NCPH。这种疾病的预后比肝硬化好得多。
