背景:目前对特发性非肝硬化门脉高压症(INCPH)的发病机制了解甚少。豁免权改变了,血液病,感染,由于对HIV患者的疾病认识增加,一部分INCPH患者的先天性缺陷和药物暴露已得到证实,或各种血液疾病或自身免疫性疾病。我们旨在讨论INCPH的可能病因。
方法:我们报道了一例肠道感染马尔尼菲T.一组罕见的原发性免疫缺陷疾病,最终被诊断为INCPH胃食管静脉曲张破裂出血。诊断主要基于组织病理学特征。经颈静脉肝内门体分流术,随访6个月无黑便复发。
结论:在免疫缺陷的背景下,INCPH可能与肠道感染有关。因此,对不明原因的门静脉高压症患者进行肠源性感染和免疫紊乱的筛查是必要的.
BACKGROUND: The etiopathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) is so far poorly understood. Altered immunity, blood diseases, infections, congenital defects and drug exposure have been documented in a part of patients with INCPH owing to increased recognition of the disorder in patients with HIV, or various haematological disorders or autoimmune diseases. We aim to discuss the possible etiopathogenesis of INCPH.
METHODS: We reported that a patient with intestinal infection of T. Marneffei and hyper-IgE syndrome, a group of rare primary immunodeficiency disorders, was finally diagnosed with INCPH for gastroesophageal variceal bleeding. The diagnosis was mainly based on histopathological features. Transjugular intrahepatic portosystemic shunt was performed and there was no recurrence of melena during the six-month follow-up.
CONCLUSIONS: In the context of immunodeficiency, INCPH may associated with intestinal infections. Thus, screening for enterogenic infection and immunological disorders in patients with unexplained portal hypertension is necessary.