PDF(Pubmed)
Abstract：
Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. NCPH diagnosis is challenging, as there are no specific tests available to confirm the disease, and a high-quality liver biopsy, detailed clinical information, and an expert pathologist are necessary for diagnosis. Currently, the treatment of NCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied that aimed to modify the natural history of the disease; however, transjugular intrahepatic porto-systemic shunt (TIPS) placement, shunt and liver transplantation are considerable symptomatic options. In this review, we discuss the heterogeneity of NCPH as well as its etiopathogenesis, clinical presentation and management issues. Starting from the assumption that portal hypertension does not always mean cirrhosis, cooperative studies are probably needed to clarify the issues of etiology and the possible genetic background of this rare disease. This knowledge might lead to better treatment and perhaps better prevention.
摘要：
非肝硬化门静脉高压症（NCPH），也称为特发性非肝硬化门静脉高压症（INCPH）和门窦血管疾病（PSVD），是一种罕见的疾病，在没有肝硬化的情况下表现为肝内门脉高压（IPH）。IPH的确切病因是正在进行的研究领域。NCPH诊断具有挑战性，因为没有特定的测试来确认这种疾病，和高质量的肝活检，详细的临床信息，和专家病理学家是必要的诊断。目前,NCPH的治疗依赖于预防与门静脉高压相关的并发症，遵循当前的肝硬化门静脉高压症指南。尚未研究旨在改变疾病自然史的治疗方法；然而，经颈静脉肝内门体分流术（TIPS）放置，分流术和肝移植是相当多的症状选择.在这次审查中,我们讨论了NCPH的异质性及其病因，临床表现和管理问题。从门静脉高压并不总是意味着肝硬化的假设开始，可能需要合作研究来阐明这种罕见疾病的病因和可能的遗传背景。这些知识可能会导致更好的治疗和更好的预防。
