一组非肝硬化性门静脉高压症病因分析和诊断路径探讨  

Etiology and diagnostic roadmap in patients with non-cirrhotic portal hypertension

作　　者：冯彦菲 苏明华[1] 殷倩冰 黎清梅 苏土梅 梁蘅恺 韦璐 黄建芳 江建宁[1] Feng Yanfei;Su Minghua;Yin Qianbin(Department of Infectious Diseases,First Affiliated Hospital,Guangxi Medical University,Nanning 530021,Guangxi Zhuang Autonomous Region,China)

机构地区：[1]广西医科大学第一附属医院感染性疾病科,南宁市530021

出　　处：《实用肝脏病杂志》2024年第2期246-250,共5页Journal of Practical Hepatology

基　　金：国家自然科学基金资助项目(编号:81960115,82260124);广西壮族自治区病毒性肝炎防治研究重点实验室开放课题基金项目(编号:GXCDCKL202001);教育部区域性高发肿瘤早期防治研究重点实验室自主课题(编号:GKE-ZZ202107)。

摘　　要：目的分析一组非肝硬化性门静脉高压(NCPH)患者的病因和诊断方法,归纳总结诊断路径。方法2020年9月~2022年3月广西医科大学第一附属医院收治的NCPH患者105例,常规临床检查和基因分析诊断。结果本组经综合检查,诊断肝前型69例(65.7%),肝内型21例(20.0%)、肝后型4例(3.8%)和未诊断11例(10.5);常见疾病为胰源性疾病22例(31.9%),门静脉阻塞15例(21.7%),血液性疾病15例(21.7%);主要诊断方法为影像学检查28例(40.6%),消化内镜检查14例(20.3%),骨髓穿刺活检术12例(17.4%);NCPH病因诊断前三位的方法依次为影像学检查(33.3%)、综合分析(18.1%)和消化内镜(13.9%)检查。结论NCPH以肝前型门静脉高压为常见,常规行实验室和影像学检查,必要时行消化内镜和骨髓穿刺活检术可明确诊断。对于常规检查仍不能诊断的,再考虑肝内型和肝后型门静脉高压,行肝穿刺活检或下腔静脉穿刺造影等检查往往能进一步明确诊断。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.

关 键 词：非肝硬化性门静脉高压 病因 诊断路径 

分 类 号：R575.2[医药卫生—消化系统]