Man Shrestha Santosh
Liver Foundation Nepal, Sitapaela Height Nagarjun Municipality, Kathmandu, Nepal.
Hepatol Forum. 2021 May 24;2(2):69-75. doi: 10.14744/hf.2021.2021.0008. eCollection 2021 May.
Hepatic vena cava syndrome (HVCS) is a bacterial infection-induced obliterative disease of the inferior vena cava at the site of the hepatic vein openings that causes chronic liver disease with a high incidence of liver cirrhosis and a moderate incidence of hepatocellular carcinoma (HCC). HVCS typically manifests clinically as recurrent mild jaundice and/or a mild elevation of transaminases or ascites as a result of hepatic venous outflow obstruction. This study is an investigation of the complications and treatment results of a large cohort of HVCS patients with an emphasis on splenomegaly and hypersplenism.
A total of 1935 (1335 male and 600 female) patients with HVCS seen between 2004 and 2019 for splenomegaly and hypersplenism were enrolled in this retrospective study. The incidence of splenomegaly, ascites, cirrhosis, HCC, and other complications was measured and analyzed.
Long-term follow-up of a large study group seen over several years indicated that 16% had mild splenomegaly, and 50% of these developed cytopenia commonly thrombocytopenia and/or leucopenia.
The development of hypersplenism was related to recurrent or prolonged acute exacerbation of HVCS. This resulted in an increased incidence of ascites and cirrhosis as well as complications, such as hepatic encephalopathy, gastrointestinal or mucosal bleeding, or mortality.
肝静脉腔静脉综合征（HVCS）是一种由细菌感染引起的肝静脉开口处下腔静脉闭塞性疾病，可导致慢性肝病，肝硬化发生率高，肝细胞癌（HCC）发生率中等。HVCS的临床典型表现为因肝静脉流出道梗阻导致的反复轻度黄疸和/或转氨酶轻度升高或腹水。本研究旨在调查一大群HVCS患者的并发症及治疗结果，重点关注脾肿大和脾功能亢进。
本回顾性研究纳入了2004年至2019年间因脾肿大和脾功能亢进就诊的1935例（男性1335例，女性600例）HVCS患者。测量并分析脾肿大、腹水、肝硬化、HCC及其他并发症的发生率。
对多年来观察的一个大型研究组进行长期随访表明，16%的患者有轻度脾肿大，其中50%出现血细胞减少，常见血小板减少和/或白细胞减少。
脾功能亢进的发生与HVCS反复或长期急性加重有关。这导致腹水和肝硬化的发生率增加以及肝性脑病、胃肠道或黏膜出血等并发症或死亡率增加。
