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[门静脉窦状隙血管疾病:消化性出血的一种罕见病因]

[Porto sinusoidal vascular disease: an unusual cause of digestive bleeding].

作者信息

Yance Stalin, Montes Pedro, Arevalo Fernando

机构信息

Servicio de Gastroenterología, Hospital Nacional Daniel Alcides Carrión, Lima, Perú; Universidad Científica del Sur, Lima, Perú.

Servicio de Anatomía Patológica, Hospital Nacional Daniel Alcides Carrión, Lima, Perú.

出版信息

Rev Gastroenterol Peru. 2024 Apr-Jun;44(2):145-149.

PMID:39019808
Abstract

Portal hypertension (PHT) is defined as an increase in pressure at the level of the portal vein above 5 mmHg, the most common cause being liver cirrhosis. Among the presinusoidal intrahepatic causes of PHT with portal venular involvement, what was traditionally known as idiopathic non-cirrhotic portal hypertension (NCIH) is described, with the requirements of excluding those patients who did not present PHT, as well as those with the presence of liver cirrhosis and thrombosis. portal venous vein (PVT). Currently, the diagnostic criteria for this entity have been reconsidered, and its name, being known as porto-sinusoidal vascular disease (PSVD), also does not exclude patients with PHT or the presence of underlying liver disease. Liver biopsy continues to be the gold standard for diagnosis. The clinical manifestations are derived from PHT and the management is similar to the complications that occur in patients with liver cirrhosis. The case of a male patient is presented who presents with symptoms of digestive bleeding, with findings of esophageal varices in upper endoscopy in addition to a study of viral, autoimmune liver disease and negative deposits, with a conclusive liver biopsy of porto-sinusoidal vascular disease.

摘要

门静脉高压(PHT)被定义为门静脉压力升高至5 mmHg以上,最常见的原因是肝硬化。在伴有门静脉小分支受累的肝内窦前性门静脉高压病因中,描述了传统上称为特发性非肝硬化性门静脉高压(NCIH)的情况,其要求排除那些未出现门静脉高压的患者,以及患有肝硬化和门静脉血栓形成(PVT)的患者。目前,对该实体的诊断标准已重新考虑,其名称为门静脉窦状血管疾病(PSVD),也不排除患有门静脉高压或存在潜在肝脏疾病的患者。肝活检仍然是诊断的金标准。临床表现源于门静脉高压,治疗方法与肝硬化患者出现的并发症相似。本文介绍了一名男性患者的病例,该患者出现消化性出血症状,上消化道内镜检查发现食管静脉曲张,此外还进行了病毒、自身免疫性肝病及阴性沉积物的检查,肝活检确诊为门静脉窦状血管疾病。

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