肝窦阻塞综合征9例的临床分析

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。

Clinical features of 9 cases of sinusoidal obstruction syndrome

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.