Tan Youwen, Zheng Sainan
Department of Hepatology, Third Hospital of the Zhenjiang Affiliated Jiangsu University, No. 300, Daijiamen, Runzhou Distinct, Zhenjiang 212003, China.
Open Med (Wars). 2023 Jun 12;18(1):20230737. doi: 10.1515/med-2023-0737. eCollection 2023.
Tusanqi-induced hepatic sinusoidal obstruction syndrome (HSOS) is caused by exposure to pyrrolizidine alkaloids (PAs) and manifests as abdominal distension, liver pain, ascites, jaundice, and hepatomegaly. Pathologically, hepatic congestion and sinusoidal occlusion are observed in HSOS. We summarized the clinical characteristics of 124 patients with HSOS caused by Tusanqi in China between 1980 and 2019, along with those of 831 patients from seven English case series. The main clinical manifestations of PA-HSOS included abdominal pain, ascites, and jaundice. Common imaging features included characteristic heterogeneous density, slender hepatic veins, and other nonspecific changes. The acute stage is primarily manifested as hepatic sinus congestion and necrosis. Meanwhile, the persistence of hepatic sinus congestion and the onset of perisinusoidal fibrosis were observed during the repair stage. Finally, the persistence of hepatic sinusoidal fibrosis and resultant central hepatic vein occlusion were observed in the chronic stage. The new Nanjing standard for PA-HSOS incorporates the history of PA consumption and imaging features and eliminates weight gain and the serum total bilirubin value. Preliminary clinical validation of the Nanjing standard for PA-HSOS diagnosis revealed a sensitivity and specificity of 95.35 and 100%, respectively.
土三七所致肝窦阻塞综合征（HSOS）由接触吡咯烷生物碱（PAs）引起，表现为腹胀、肝区疼痛、腹水、黄疸和肝肿大。病理上，HSOS可见肝淤血和肝窦阻塞。我们总结了1980年至2019年间中国124例土三七所致HSOS患者的临床特征，以及来自7个英文病例系列的831例患者的临床特征。PA-HSOS的主要临床表现包括腹痛、腹水和黄疸。常见的影像学特征包括特征性的密度不均、肝静脉纤细及其他非特异性改变。急性期主要表现为肝窦淤血和坏死。同时，修复期可见肝窦淤血持续存在及肝窦周围纤维化的发生。最后，慢性期可见肝窦纤维化持续存在及由此导致的肝中央静脉阻塞。PA-HSOS的新南京标准纳入了PA摄入史和影像学特征，摒弃了体重增加和血清总胆红素值。PA-HSOS诊断南京标准的初步临床验证显示，其敏感性和特异性分别为95.35%和100%。
