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成人多囊肝病临床分期对治疗方式选择的价值
张海斌1△,李团结2△,杨宁1,卢军华1,赵军1,司马辉1,杨广顺1*
0
(1.第二军医大学东方肝胆外科医院胆道二科,上海 200438;2.中山大学附属第三医院肝移植中心,广州 510630)
摘要:
目的:探讨成人多囊肝病(APLD)的临床分期对治疗方式选择的指导意义。方法:回顾分析我院自1995年5月~2007年10月间共收治的43例APLD患者的临床资料,根据临床表现及影像学检查将其分为3期:无症状或轻微症状期、明显症状期、致死症状期。26例接受肝部分切除联合广泛囊肿开窗术治疗,17例门诊随访观察。结果:15例门诊随访无症状或轻微症状期患者,其中1例合并原发性肝癌,发病后4个月死亡;其余14例平均随访(42.8±37.9)个月,12例病程进展缓慢,2例病情发展较快,后接受手术治疗。24例明显症状期患者接受肝部分切除联合囊肿广泛开窗术治疗,无围手术期死亡,平均随访(61.4±43.0)个月,2例死于肾功能衰竭,3例术后症状复发。4例致死症状期患者,2例接受肝部分切除联合广泛囊肿开窗术治疗,其中1例术后症状无缓解,1例术后发生肝功能不全,于术后8个月接受肝移植治疗;2例门诊接受经皮穿刺抽液治疗,1例随访5个月后死于上消化道出血,1例接受经皮穿刺抽液治疗后症状稍缓解。结论:成人多囊肝病的临床分期有助于患者治疗方式的选择:无症状或轻微症状期患者一般可临床随访;明显症状期患者可采用手术治疗;致死症状期患者不宜接受常规手术治疗,可能需行肝移植。
关键词:  成人多囊肝病  分期  肝切除术  开窗术
DOI:10.3724/SP.J.1008.2009.0676
投稿时间:2009-01-16修订日期:2009-03-27
基金项目:
Value of clinical classification of adult polycystic liver disease for selection of treatments
ZHANG Hai-bin1△,LI Tuan-jie2△,YANG Ning1,LU Jun-hua1,ZHAO Jun1,SIMA Hui1,YANG Guang-shun1*
(1.Department of Biliary Tract Surgery Ⅱ,Eastern Hepatobiliary Hospital,Second Military Medical University,Shanghai 200438,China;2.Center of Liver Transplantation,The Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510630)
Abstract:
Objective:To study the role of clinical classification of adult polycystic liver disease (APLD) in guiding the selection of treatments for the patients.Methods:The clinical data of 43 APLD patients who were treated in our hospital from May 1995 to Oct. 2007 were retrospectively analyzed.The patients were divided into 3 stages according to the clinical manifestations and imaging examination:non-symptom or slight symptom stage,obvious symptom stage,and lethal symptom stage.Seventeen patients in our group received outpatient clinical follow-up;26 were treated by partial hepatectomy combined with fenestration.Results:Among the 15 cases at non-symptom or slight symptom stage,one had primary hepatic carcinoma simultaneously and died 4 mouths after diagnosis; the rest 14 case were followed up for a mean of (42.8±37.9) months; 12 of them had slow progression and 2 had rapid progression of APLD; the latter 2 cases received operation.The 24 cases at obvious symptom stage were treated by partial hepatectomy combined with fenestration.There were no perioperation deathes and the mean follow-up period was (61.4±43.0) months; 2 cases died of renal failure and 3 had post-operation recurrence.Two of the 4 cases at the lethal symptom stage were treated by partial hepatectomy combined with fenestration:one had no symptom relieve after operation and one developed hepatic dysfunction and received liver transplantation 8 months after operation; the other 2 patients received percutaneous cyst aspiration at the outpatient department,one patient died of upper gastrointestinal hemorrhage 5 months later,and one had the symptom slightly relieved.Conclusion:Our classification of APLD can help to select treatments for patients:patients at non-symptom or slight symptom stage should be followed up,those at the obvious symptom stage can be treated surgically,and those at the lethal symptom stage are not suitable for routine operation and liver transplantation may be the best choice for them.
Key words:  adult polycystic liver disease  classification  hepatectomy  fenestration