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一组非肝硬化性门静脉高压症病因分析和诊断路径探讨

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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。
Etiology and diagnostic roadmap in patients with non-cirrhotic portal hypertension
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 in22 cases(31.9%),portal vein obstruction in15 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 in12 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.

Non-cirrhotic portal hypertensionEtiologyDiagnostic roadmap

冯彦菲、苏明华、殷倩冰、黎清梅、苏土梅、梁蘅恺、韦璐、黄建芳、江建宁

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530021 南宁市 广西医科大学第一附属医院感染性疾病科

非肝硬化性门静脉高压 病因 诊断路径

国家自然科学基金资助项目国家自然科学基金资助项目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

8196011582260124GXCDCKL202001GKE-ZZ202107

2024

实用肝脏病杂志
中华医学会安徽分会

实用肝脏病杂志

CSTPCD
影响因子:1.362
ISSN:1672-5069
年,卷(期):2024.27(2)
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