UNASSIGNED:闭合性门脉静脉病(OPV)是非肝硬化门脉高压的原因之一。然而,OPV的许多方面仍不清楚,包括病因,发病机制,和自然史。这项研究的目的是描述OPV在巴西的一系列患者中的临床特征,这些患者通过肝活检诊断为OPV。
UNASSIGNED:根据组织学标准,回顾性选择了43例连续的OPV成年患者作为病例系列。定义为至少存在门静脉纤维化,静脉硬化,门静脉分支的消失和/或口径减少,并排除肝硬化。分析临床和实验室数据。在存在食管静脉曲张和/或腹水的情况下,考虑了临床上重要的门脉高压。
未经证实:诊断时患者的平均年龄为44.5±11岁,主要是女性(81%)。在28%的病例中发现了临床上显着的门静脉高压症。肝活检最常见的指征是肝酶升高,76%的患者主要是γ-谷氨酰转移酶(GGT),平均222IU/L(正常上限高达40IU/L)和丙氨酸氨基转移酶(ALT)在64%,平均84IU/L(38IU/L)。我们三分之一的病人接触过药物,尤其是草药,在酶变化的时候。突出的其他危险因素是25%的患者的自身免疫特征或20%的血栓形成倾向。
未经证实:OPV可在门脉高压发作之前确诊,ALT标高,在大多数情况下,尤其是GGT升高。其病因尚未明确,但是自身免疫性疾病,血栓形成倾向,使用药物或草药可能会发挥作用。
UNASSIGNED: Obliterative portal venopathy (OPV) is one of the causes of non-cirrhotic portal hypertension. However, many aspects of OPV remain unclear, including the etiology, pathogenesis, and natural history. The aim of this study was to describe the clinical features of OPV in a series of patients in Brazil in whom OPV was diagnosed through liver biopsy.
UNASSIGNED: Forty-three consecutive adult patients with OPV were retrospectively selected as a case series based on histologic criteria, defined by the presence of at least portal fibrosis, phlebosclerosis, disappearance and/or reduction of the caliber of portal vein branches, and exclusion of cirrhosis. Clinical and laboratory data were analyzed. Clinically significant portal hypertension was considered in the presence of esophageal varices and/or ascites.
UNASSIGNED: The mean age of patients at diagnosis was 44.5 ± 11 years, who were predominantly female (81%). Clinically significant portal hypertension was found in 28% of cases. The most frequent indication for liver biopsy was the elevation of liver enzymes, mostly γ-glutamyl transferase (GGT) in 76% of patients, averaging 222 IU/L (upper limit of normality up to 40 IU/L) and alanine aminotransferase (ALT) in 64%, mean 84 IU/L (38 IU/L). One-third of our patients had exposure to medications, especially herbal medicines, at the time of enzymatic changes. Other risk factors highlighted were features of autoimmunity in 25% of patients or thrombophilia in 20%.
UNASSIGNED: OPV can be diagnosed even before the onset of portal hypertension, ALT elevation, and especially GGT elevation in most cases. Its etiology is not defined, but autoimmune diseases, thrombophilia, and the use of medications or herbal medicines may play a role.