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中国吡咯烷生物碱致肝窦阻塞综合征的临床现状分析
Clinical features of sinusoidal obstruction syndrome induced by pyrrolidine alkaloids in China
文章发布日期:2018年05月07日  来源:  作者:郭严,张世荣,文良志,等  点击次数:282次  下载次数:28次

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【摘要】:目的探讨我国吡咯烷生物碱致肝窦阻塞综合征的临床表现、治疗方法及预后。方法检索1980年1月-2017年4月我国107篇肝窦阻塞综合征临床报道文献,共计407例患者,总结该病的性别、年龄及分布地区,该病的临床表现、影像学、病理结果及预后情况。结果407例患者中男女比为1∶1.22;发病年龄1岁1个月~81岁。临床表现多为腹胀、腹痛、腹水和肝肿大。典型的腹部CT表现为肝肿大,增强后呈“地图状”或斑片状改变。病理可见肝窦扩张淤血,肝小静脉不同程度狭窄。407例患者中治疗有效302例,死亡90例,病死率22.11%。结论我国吡咯碱致肝窦阻塞综合征发病女性高于男性,任何年龄均可发生,以腹胀、腹痛、腹水和肝肿大为突出临床表现,结合典型的肝脏CT表现可明确诊断,肝穿刺病理表现为诊断金标准。治疗上以对症治疗为主,部分患者预后较差。
【Abstract】:ObjectiveTo investigate the clinical manifestations, treatment, and prognosis of sinusoidal obstruction syndrome induced by pyrrolidine alkaloids in China. MethodsRelated databases were searched, and 107 articles on sinusoidal obstruction syndrome published from January 1980 to April 2017 in China were included, with 407 patients in total. Related data of this disease were analyzed, including sex, age, regional distribution, clinical manifestations, imaging and pathological findings, and prognosis. ResultsThe male/female ratio of 407 patients was 1:1.22, and the age of onset ranged from 13 months to 81 years. Major clinical manifestations included abdominal distension, abdominal pain, ascites, and hepatomegaly. The most typical abdominal CT findings were hepatomegaly and a map-like or patchy change after enhancement. Pathological examination showed sinusoidal expansion and congestion and varying degrees of stenosis of small hepatic veins. Of all 407 patients, 302 had response and 90 died, resulting in a mortality rate of 22.1%. ConclusionIn China, the male population has a higher incidence rate of sinusoidal obstruction syndrome than the female population, and sinusoidal obstruction syndrome can occur at any age. Major clinical manifestations include abdominal distension, abdominal pain, ascites, and hepatomegaly, which can be used to make a definite diagnosis in combination with typical liver CT manifestations. Pathological findings by liver biopsy are the gold standard for diagnosis. Symptomatic treatment is the major treatment modality, and some patients may have poor prognosis.
【关键字】:肝静脉闭塞性疾病; 吡咯烷类; 中国
【Key words】:hepatic veno-occlusive disease; pyrrolidines; China
【引证本文】:
GUO Y, ZHANG SR, WEN LZ, et al. Clinical features of sinusoidal obstruction syndrome induced by pyrrolidine alkaloids in China[J]. J Clin Hepatol, 2018, 34(6): 1277-1281. (in Chinese) 郭严, 张世荣, 文良志, 等. 我国吡咯烷生物碱致肝窦阻塞综合征的临床特征分析[J]. 临床肝胆病杂志, 2018, 34(6): 1277-1281.

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