肝窦阻塞综合征9例的临床分析

doi:10.3969/j.issn.1006-7795.2014.05.017

首都医科大学学报 ›› 2014, Vol. 35 ›› Issue (5): 604-607.doi: 10.3969/j.issn.1006-7795.2014.05.017

肝窦阻塞综合征9例的临床分析

王峻瑶, 张国艳, 朱元民, 刘玉兰, 陈宁

北京大学人民医院消化内科, 北京 100044

收稿日期:2014-07-30 出版日期:2014-10-21 发布日期:2014-10-20
通讯作者: 陈宁 E-mail:chenning79@gmail.com
基金资助:
北京市科技新星计划基金资助项目（ZXKTYANLI001）。

Clinical features of 9 cases of sinusoidal obstruction syndrome

Wang Junyao, Zhang Guoyan, Zhu Yuanmin, Liu Yulan, Chen Ning

Department of Gastroenterology, Peking University People's Hospital, Beijing 100044, China

Received:2014-07-30 Online:2014-10-21 Published:2014-10-20
Supported by:
This study was supported by Beijing New Star Plan of Science Technoogy Program(ZXKTYANLI001).

摘要/Abstract

摘要：

目的收集肝窦阻塞综合征（sinusoidal obstruction syndrome， SOS）的资料，总结其临床特点。方法回顾性分析北京大学人民医院自2009年10月至2014年8月收治的9例肝SOS患者病史、实验室检查、影像学检查、转归等资料。结果 9例患者中大部分发病前有中药服用史，临床表现为腹胀、肝区疼痛、体质量增加，实验室指标示转氨酶升高，天门冬氨酸氨基转移酶（aspartate aminotransferase，AST）>丙氨酸氨基转移酶（alanine aminotransferase，ALT），胆红素升高，腹水为漏出液，影像学检查可见肝脏增大、实质不均质改变、血管不清，肝活检结果提示肝窦扩张及肝细胞坏死，治疗以对症支持为主，2例重症SOS死亡，其余7例好转。结论所总结9例SOS多数与自行服用中药相关，根据临床、理化、影像学检查综合诊断，治疗手段有限，重症病例预后不良。

关键词: 肝窦阻塞综合征, 肝静脉闭塞性疾病, 中草药

Abstract:

Objective Sinusoidal obstruction syndrome (SOS) is a clinical syndrome characterized by tender hepatomegaly, jaundice, and ascites, but not always easy to diagnose. This study aimed at summarizing several informative clinical presentations of SOS.Methods A total of 9 SOS cases seen from October 2009 to August 2014 were collected. Clinical materials including medical history, laboratory data, imaging features and outcomes were retrospectively analyzed.Results Majority of the 9 cases had a history of applying Chinese herbs before the onset of SOS. Most common clinical manifestations were bloating, right upper quadrant pain and weight gain. Biochemical tests revealed elevation of serum transaminase (especially AST>ALT) and hyperbilirubinemia. Serum-ascites albumin concentration gradient ≥11 g/L. Imaging examinations demonstrated hepatomegaly, inhomogeneous liver parenchyma and attenuated hepatic veins. Sinusoidal dilatation and hepatic cell necrosis were obvious in liver biopsy. Treatments were generally symptomatic and supportive. 2 cases of severe SOS died while the other 7 finally improved.Conclusion These 9 cases of SOS were mostly associated with Chinese herbs. Diagnosis was comprehensively based on clinical, laboratory, imaging and histological features. There were limited therapeutic options for SOS and severe cases had poor prognosis.

Key words: sinusoidal obstruction syndrome, hepatic veno-occlusive disease, Chinese herbs

中图分类号:

R575

王峻瑶, 张国艳, 朱元民, 刘玉兰, 陈宁. 肝窦阻塞综合征9例的临床分析[J]. 首都医科大学学报, 2014, 35(5): 604-607.

Wang Junyao, Zhang Guoyan, Zhu Yuanmin, Liu Yulan, Chen Ning. Clinical features of 9 cases of sinusoidal obstruction syndrome[J]. Journal of Capital Medical University, 2014, 35(5): 604-607.

参考文献

[1] McDonald G B, Hinds M S, Fisher L D, et al. Veno-occlusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients[J]. Ann Intern Med, 1993,118(4):255-267.
[2] Jones R J, Lee K S, Beschorner W E, et al. Venoocclusive disease of the liver following bone marrow transplantation[J]. Transplantation, 1987,44(6):778-783.
[3] DeLeve L D, Valla D C, Garcia-Tsao G. Vascular disorders of the liver[J]. Hepatology, 2009,49(5):1729-1764.
[4] 彭涛, 张国艳, 刘玉兰. 布-加综合征、肝血窦阻塞综合征与肝硬化的鉴别[J]. 临床肝胆病杂志, 2011,27(10):1022-1026,1031.
[5] Senzolo M, Germani G, Cholongitas E, et al. Veno occlusive disease: update on clinical management[J]. World J Gastroenterol, 2007,13(29):3918-3924.
[6] Jelliffe D B, Bras G, Stuart K L. Veno-occlusive disease of the liver[J]. Pediatrics, 1954,14(4):334-339.
[7] 宋宇, 樊艳华. 土三七所致肝小静脉闭塞病102例临床分析[J]. 临床肝胆病杂志, 2011,27(5):496-499.
[8] Ramachandran R, Kakar S. Histological patterns in drug-induced liver disease[J]. J Clin Pathol, 2009,62(6):481-492.
[9] Carreras E, Granena A, Navasa M, et al. On the reliability of clinical criteria for the diagnosis of hepatic veno-occlusive disease[J]. Ann Hematol, 1993,66(2):77-80.
[10] Blostein M D, Paltiel O B, Thibault A, et al. A comparison of clinical criteria for the diagnosis of veno-occlusive disease of the liver after bone marrow transplantation[J]. Bone Marrow Transplant, 1992,10(5):439-443.
[11] 徐小俊, 陈洪潭, 单国栋. 土三七致肝窦阻塞综合征42例诊治分析[J]. 中华危重症医学杂志:电子版, 2010,3(3):178-180.
[12] 朱晚林, 陈韶华, 陈卫星, 等. 肝小静脉闭塞病50例临床分析[J]. 中华消化杂志, 2012,32(9):620-624.
[13] Gao H, Li N, Wang JY, et al. Definitive diagnosis of hepatic sinusoidal obstruction syndrome induced by pyrrolizidine alkaloids[J]. J Dig Dis, 2012,13(1):33-39.
[14] 彭俏, 贺德志, 李建生. 肝小静脉闭塞病的临床现状及研究进展[J]. 世界华人消化杂志, 2012,20(12):1030-1035.
[15] Coppell J A, Richardson P G, Soiffer R, et al. Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome[J]. Biol Blood Marrow Transplant, 2010,16(2):157-168.
[16] 陈哲, 霍继荣,朱洪怡,等. 川芎嗪对土三七诱导的肝小静脉闭塞病小鼠肝组织PAI-1表达的影响 [J]. 中国医科大学学报,2012,41(8):709-712.
[17] 窦淼. 布-加综合征1例误诊分析 [J].中国煤炭工业医学杂志,2011,14(11):1697-1698.

肝窦阻塞综合征9例的临床分析

Clinical features of 9 cases of sinusoidal obstruction syndrome

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