关键词: 非肝硬化性门静脉高压, 病因, 诊断路径

Abstract: Objective The aim of this study was to investigate the etiology and diagnostic roadmap in patients with non-cirrhotic portal hypertension (NCPH). Methods 105 patients with NCPH were encountered in the First Affiliated Hospital, Guangxi Medical University between September 2020 and March 2022, and the etiologies and the main diagnostic methods were summarized retrospectively. Results The etiologies of NCPH in our series included prehepatic, hepatic and posthepatic entities; the common diseases were found with prehepatic portal hypertension (PH) in 69 cases (65.7%), the hepatic PH in 21 cases (20.0%) and the posthepatic PH in 4 cases (3.8%); the common diseases in patients with prehepatic ph were pancreaticogenic diseases in 22 cases (31.9%), portal vein obstruction in 15 cases (21.7%), and hematologic diseases in 15 cases (21.7%); the main diagnostic methods were imaging examination in 28 cases (40.6%), gastrointestinal endoscopy in 14 cases (20.3%) and bone marrow biopsies in 12 cases (17.4%); the top three methods for the etiological diagnosis in patients with NCPH were imaging examination (33.3%), comprehensive analysis (18.1%) and gastrointestinal endoscopy (13.9%). Conclusion The prehepatic PH should be considered firstly in patients with NCPH presentation, the laboratory and imaging examinations should be performed routinely, and the gastrointestinal endoscopy and bone marrow biopsy might be performed if necessary. For those without clear diagnosis after routine examination, the hepatic and posthepatic PH must be considered, and liver biopsy and inferior vena cava puncture angiography should be done for further validation of diagnosis.

Key words: Non-cirrhotic portal hypertension, Etiology, Diagnostic roadmap