门窦血管疾病:何时怀疑及如何治疗?

IF 3.7 3区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY
Sarah Shalaby, Wagner Enrique Ramírez-Quesada, Asunción Ojeda, Valeria Perez-Campuzano, Anna Baiges, Fanny Turon, Carla Fuster, Alba Díaz, Virginia Hernández-Gea, Juan Carlos García-Pagán
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引用次数: 0

摘要

门窦血管病变(PSVD)是一组罕见的以肝脏血管结构异常为特征的疾病,通常在没有肝硬化的情况下表现为门静脉高压(PH)的临床特征。PSVD的病理生理机制尚不清楚,但它通常与潜在的免疫疾病、药物、血液系统疾病和血栓病有关。实验室检查通常显示肝功能保留,转氨酶谱有或无轻微改变。一个关键的诊断特征是存在明显的PH征象,同时肝僵硬度和肝静脉压梯度正常或仅轻微升高。肝活检对于确认诊断和排除PH和肝硬化的其他原因仍然是必不可少的。然而,组织学检查可能会显示细微或轻微的变化,因此专家病理分析和高质量标本对于准确诊断至关重要。在某些情况下,可以在没有明显PH的患者中发现特征性组织学发现,这可能代表疾病的早期阶段。PSVD患者的长期预后主要受基础疾病的严重程度和ph的发展影响。然而,改变疾病自然史的治疗方法仍然缺乏,治疗主要集中在控制ph相关的并发症。需要进一步研究PSVD的发病机制和潜在的治疗策略,以改善患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Portosinusoidal Vascular Disorder: When to Suspect and How to Manage?

Portosinusoidal vascular disorders (PSVD) represent a group of rare conditions characterized by abnormalities in the liver's vascular architecture, often manifesting with clinical features of portal hypertension (PH), in the absence of cirrhosis. The pathophysiology of PSVD remains unclear, but it is frequently linked to underlying immunological disorders, medications, hematological disorders, and thrombophilia. Laboratory tests typically show preserved liver function with or without slight alteration on the transaminase profile. A key diagnostic feature is the presence of clear signs of PH alongside normal or only slightly elevated liver stiffness and hepatic venous pressure gradient. Liver biopsy remains essential for confirming the diagnosis and excluding other causes of PH and cirrhosis. However, histological examination may reveal subtle or mild changes, making expert pathological analysis and high-quality specimens crucial for an accurate diagnosis. In some cases, characteristic histological findings may be identified in patients without overt PH, which could represent an early stage of the disease. The long-term prognosis for patients with PSVD is mainly influenced by severity of the underlying condition and development of PH. However, treatments that modify the disease's natural history are still lacking, and management primarily focuses on controlling complications related to PH. Further research into the pathogenesis and potential therapeutic strategies for PSVD is needed to improve patient outcomes.

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来源期刊
Seminars in liver disease
Seminars in liver disease 医学-胃肠肝病学
CiteScore
8.20
自引率
2.40%
发文量
38
期刊介绍: Seminars in Liver Disease is a quarterly review journal that publishes issues related to the specialties of hepatology and gastroenterology. As the premiere review journal in the field, Seminars in Liver Disease provides in-depth coverage with articles and issues focusing on topics such as cirrhosis, transplantation, vascular and coagulation disorders, cytokines, hepatitis B & C, Nonalcoholic Steatosis Syndromes (NASH), pediatric liver diseases, hepatic stem cells, porphyrias as well as a myriad of other diseases related to the liver. Attention is also given to the latest developments in drug therapy along with treatment and current management techniques. Seminars in Liver Disease publishes commissioned reviews. Unsolicited reviews of an exceptional nature or original articles presenting remarkable results will be considered, but case reports will not be published.
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