非肝硬化门静脉高压:当前趋势和未来方向。

IF 2.6 3区 医学 Q2 GASTROENTEROLOGY & HEPATOLOGY
Babu Lal Meena, Omkar S Rudra, Deepti Sharma, Shiv Kumar Sarin
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引用次数: 0

摘要

回顾目的:非肝硬化门静脉高压症(NCPH)包括以门静脉压力升高为特征的多种血管性肝脏疾病,但无肝硬化。由于重叠的临床特征,区分NCPH与肝硬化和门窦血管病变(PSVD)仍然具有挑战性。这篇综述探讨了诊断、鉴别和治疗策略的最新进展。最近发现:NCPH的特点是肝功能保持不变,肝静脉压梯度(HVPG)接近正常。它与PSVD有共同的危险因素,包括感染、药物、毒素和血栓形成前疾病。诊断方面的进步,如肝硬度测量(LSM)和脾硬度测量(SSM),提供了与肝硬化的无创区分,而肝活检对于确认PSVD和非肝硬化门脉纤维化(NCPF)仍然至关重要。影像学诊断肝外门静脉阻塞(EHPVO)是可靠的。经颈静脉肝内门体分流术(TIPS)治疗难治性静脉曲张出血或腹水,72-80%的病例实现再出血控制。手术分流术和脾切除术对于不受控制的出血和门脉胆道病仍然是必不可少的,显示出良好的静脉曲张控制(93-95%)。总结:NCPH的诊断需要高度的怀疑指数。肝硬化和PSVD的鉴别依赖于临床、组织学和血流动力学评估。管理侧重于内窥镜、介入和手术策略,以适应疾病的严重程度。未来的研究应该标准化诊断标准,探索靶向治疗,完善预后工具以改善预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Noncirrhotic portal hypertension: current trends and future directions.

Purpose of review: Noncirrhotic portal hypertension (NCPH) comprises a diverse group of vascular liver disorders characterized by elevated portal pressure without cirrhosis. Due to overlapping clinical features, distinguishing NCPH from cirrhosis and porto-sinusoidal vascular disorder (PSVD) remains challenging. This review explores recent advancements in diagnosis, differentiation, and evolving treatment strategies.

Recent findings: NCPH is characterized by preserved liver function and near-normal hepatic venous pressure gradients (HVPG). It shares risk factors with PSVD, including infections, drugs, toxins, and prothrombotic conditions. Diagnostic advancements, such as liver stiffness measurement (LSM) and splenic stiffness measurement (SSM), offer noninvasive differentiation from cirrhosis, while liver biopsy remains crucial for confirming PSVD and noncirrhotic portal fibrosis (NCPF). Imaging is reliable for diagnosing extrahepatic portal vein obstruction (EHPVO). Transjugular intrahepatic portosystemic shunts (TIPS) for refractory variceal bleeding or ascites, achieving rebleeding control in 72-80% of cases. Surgical shunts and splenectomy remain essential for uncontrolled bleeding and portal biliopathy, demonstrating excellent variceal control (93-95%).

Summary: NCPH requires a high index of suspicion for diagnosis. Differentiation from cirrhosis and PSVD relies on clinical, histological, and hemodynamic assessments. Management focuses on endoscopic, interventional, and surgical strategies tailored to disease severity. Future research should standardize diagnostic criteria, explore targeted therapies, and refine prognostic tools to improve outcomes.

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来源期刊
Current Opinion in Gastroenterology
Current Opinion in Gastroenterology 医学-胃肠肝病学
CiteScore
5.30
自引率
0.00%
发文量
137
审稿时长
6-12 weeks
期刊介绍: ​​​​​​​Published bimonthly and offering a unique and wide ranging perspective on the key developments in the field, each issue of Current Opinion in Gastroenterology features hand-picked review articles from our team of expert editors. With twelve disciplines published across the year – including gastrointestinal infections, nutrition and inflammatory bowel disease – every issue also contains annotated references detailing the merits of the most important papers.
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