Gioia Stefania, Nardelli Silvia, Riggio Oliviero, Faccioli Jessica, Ridola Lorenzo
Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy.
J Clin Med. 2021 Dec 25;11(1):101. doi: 10.3390/jcm11010101.
Hepatic encephalopathy (HE) is one of the most frequent complications of cirrhosis. Several studies and case reports have shown that cognitive impairment may also be a tangible complication of portal hypertension secondary to chronic portal vein thrombosis and to porto-sinusoidal vascular disease (PSVD). In these conditions, representing the main causes of non-cirrhotic portal hypertension (NCPH) in the Western world, both overt and minimal/covert HE occurs in a non-neglectable proportion of patients, even lower than in cirrhosis, and it is mainly sustained by the presence of large porto-systemic shunt. In these patients, the liver function is usually preserved or only mildly altered, and the development of porto-systemic shunt is either spontaneous or iatrogenically frequent; HE is an example of type-B HE. To date, in the absence of strong evidence and large cooperative studies, for the diagnosis and the management of HE in NCPH, the same approach used for HE occurring in cirrhosis is applied. The aim of this paper is to provide an overview of type B hepatic encephalopathy, focusing on its pathophysiology, diagnostic tools and management in patients affected by porto-sinusoidal vascular disease and chronic portal vein thrombosis.
肝性脑病（HE）是肝硬化最常见的并发症之一。多项研究和病例报告表明，认知障碍也可能是慢性门静脉血栓形成和肝内门体静脉分流性疾病（PSVD）继发门静脉高压的一种明显并发症。在这些构成西方世界非肝硬化门静脉高压（NCPH）主要病因的情况下，显性和轻微/隐匿性HE在相当比例的患者中出现，甚至低于肝硬化患者，其主要原因是存在大量门体分流。在这些患者中，肝功能通常保持正常或仅有轻微改变，门体分流的形成多为自发性或医源性；HE是B型HE的一个例子。迄今为止，由于缺乏有力证据和大型合作研究，对于NCPH中HE的诊断和管理，采用的是与肝硬化中HE相同的方法。本文旨在概述B型肝性脑病，重点关注其病理生理学、诊断工具以及在肝内门体静脉分流性疾病和慢性门静脉血栓形成患者中的管理。
