肝移植治疗肝豆状核变性的临床疗效

目的探讨肝移植治疗肝豆状核变性的临床疗效。方法回顾性分析中国人民解放军联勤保障部队第九〇〇医院肝胆外科1999年1月至2021年11月接受肝移植手术治疗的16例肝豆状核变性受者的临床资料。其中男性9例, 女性7例, 年龄29.5岁(14～54)岁。采用电话、门诊及住院复查方式进行随访, 随访起点为手术当日, 以受者死亡为终点, 观察受者术后生存情况、神经精神症状改善情况、角膜K-F环变化情况, 比较术后1个月及1、3、5年的肝功能和血清铜蓝蛋白水平, 随访截止时间为2021年11月24日。结果 16例肝移植受者中采用经典原位肝移植15例, 成人间活体部分肝移植1例, 无围手术期死亡。16例受者均获得随访, 随访时间为122个月(6～260)个月。16例受者1、5和10年的累积存活率分别为93.8%、85.2%和75.8%。10例角膜K-F环阳性受者术后均有不同程度的改善, 其中2例分别于术后7个月和11个月消失。5例伴有严重的神经精神症状, 除1例死亡外, 其余4例术后均有不同程度的改善。所有受者术后1个月及1、3、5年的肝功能较术前明显改善, 血清铜蓝蛋白水平升高, 差异有统计学意义...     

临床肝移植11例报道

目的 探讨肝移植治疗终末期肝病的临床疗效。方法 回顾分析原位肝移植7例及亲体部分肝移植（LRLT）4例的临床资料,其中肝炎肝硬变5例,肝豆状核变性6例。对乙肝肝硬变者采用拉米呋啶加小剂量抗HBV－Ig治疗。结果 10例康复出院,1例死于MODS。4例LRLT全部存活。术后并发症：腹腔出血2例,ARDS3例;4例乙型肝炎肝硬变者术后HBV－DNA（－）。6例肝豆状核变性,术后铜氧化酶均正常。结论 肝移植是终末期肝病的有效治疗手段,活体供肝是解决我国供肝来源问题又一方向,外科技术是减少围手术期并发症的关键。     

亲体部分肝移植治疗肝豆状核变性

目的　研究亲体部分肝移植术在肝豆状核变性 (Wilson’s病 )治疗中的应用价值。方法　 2 0 0 1年 1月至 2 0 0 1年 1 2月连续为 1 0例Wilson’s病患者施行肝移植术 ,供肝者均为患者的母亲。结果　手术全部获得成功 ,术后无严重并发症发生 ,均恢复顺利。术后铜氧化酶均显著升高 ,分别从术前 (9.9± 9.3)U/L升高至术后 (1 2 4 .0± 33 .8)U/L ;角膜缘K F环均明显变淡 ;神经系统症状改善 ;现已分别存活 1 2、1 1、1 0、8、7、7、3、3、2和 1个月。结论　肝移植术是治疗肝豆状核变性的有效手段。亲体部分肝移植术具有供肝来源可靠、临床疗效好、费用低的优点 ,应用前景广阔。     

肝移植多模式策略的初步探讨

目的：总结多种临床肝移植技术的经验。方法：对2000年9月至2002年3月完成的25例临床肝移植（27次移植手术）的资料进行回顾性分析。包括尸肝移植14例,活体肝移植11例,再次尸肝移植1次,减体积再次肝移植1次。结果：11例活体肝移植的供体中,10例为患者之母亲,1例急诊成人右叶活体肝移植供体为患者之妹。供肝方式：扩大左半肝6例,右半肝3例,右半肝2例;切取供肝重量270－620g。全组存活24例,1例成人活体肝移植受体于术后72d死于不可逆转的严重排斥反应。肝炎患者采用拉米呋啶加抗HVB－Ig治疗,10例乙肝、肝硬化患者术后随访时间4－21个月,复查HVB－DNA均为阴性。所有肝豆状核变性受体术后随访复查6－17个月,铜氧化酶、肝功能均正常。本组主要并发症包括：腹腔出血2例,需再次剖腹探查止血;ARDS 5例;急性肾功能衰竭2例;排斥反应4例,其中1例导致死亡。结论：综合开展包括尸体肝移植、活体肝移植、减体积肝移植等在内的各项技术,充分利用有限的供肝,是优化肝移植资源,提高移植疗效的重要途径。     

原位活体部分肝移植五例报告

目的：总结活体肝移植的临床经验与教训。方法：对5例原位活体部分肝移植的临床资料进行回顾性分析。结果：5名供者术后均顺利康复;5例患者手术顺利,4例原发病为肝豆状核变性者术后健康存活,现已分别存活21周,15周,12周及2周;1例肝癌患者移植术后12d死于心律紊乱,心跳骤停;术后并发症以血管并发症,胆道并发症,细菌及病毒感染和肺部并发症为主。结论：原位活体部分肝移植手术过程复杂,技术要求高,难度大;具有其它类型肝移植无法比似的优越性。     

改良背驮式肝移植治疗wilson's病

目的: 探讨改良背驮式肝移植术治疗肝豆状核变性的意义.方法: 2例患者采用改良背驮式原位肝移植术.结果: 手术成功,2例存活至今(分别为术后22个月和11个月),且生存质量明显改善.结论: 改良背驮式肝移植术是治疗肝豆状核变性的一种优选方法.     

背驮式肝移植一例

我院1999年12月24日成功地实施一例背驮式肝移植术,患者为肝豆状核变性女性,目前,已存活1年半。一般情况良好,现生活自理。本文将治疗过程报道如下。1.一般情况:病人,女,18岁,患有肝豆状核变性9年。曾在同济医院检查诊断为肝豆状核变性,以口服青霉胺、硫酸锌等药治疗,症状有所缓解。但近2个月来出现下肢不自主颤动,行走不稳。故来我院诊治,要求全肝移植     

婴幼儿活体肝移植胆道并发症的诊断及处理

<正>婴幼儿肝移植发展迅猛并逐步成为治疗婴幼儿终末期肝病的主要手段,主要适应证包括先天性胆道闭锁、肝豆状核变性(Wilson病)、糖原累积症、肝母细胞瘤等。由于供肝资源的缺乏,活体肝移植正逐步成为婴幼儿肝移植的主要类型。随着肝移植手术技巧的发展及围手术期护理水平的提高,婴幼儿活体肝移植受者的术后长期生存率明显增高,受者术后1年生存率可高达90%,多数婴幼儿受者可存活至成年[1]。然而,肝移植特别是活体肝移植术后     

活体肝部分移植术后的中长期管理

1997年 6月 30日我们为 1位先天性肝内胆管囊性扩张症患者成功实施了活体肝部分移植术[1] ,患者已健康生存 3年 6个月 ,2 0 0 0年 6月 2 5日我们又为 1位肝豆状核变性患者施行了原位辅助性活体肝部分移植术 ,患者目前已生存 6个月 ,我们 3年多来进行了密切随访 ,现就术后中长期管理的体会报告如下。临床资料例 1　 女 10岁 ,1997年 6月接受活体肝部分移植术[1] ,患儿于术后 4个月恢复正常儿童的生活 ,现己健康存活 3年6个月。例 2 　 女 2 0岁 ,体重 46ｋｇ ,身高 15 6ｃｍ ,1999年 3月在我院确诊为肝豆状核变性。体格检查发现发音障碍、流…     

亲属活体供肝移植治疗儿童终末期肝病六例

目的 探讨亲属活体供肝移植(LDLT)治疗儿童终末期肝病的效果,并总结治疗经验.方法 2005年9月至2007年1月对6例终末期肝病患儿进行了LDLT.6例患者中,原发病为肝内外胆管弥漫性囊性扩张症Ⅳ型伴肝硬化1例、肝豆状核变性2例、门静脉海绵样变性3例;供者为患儿的母亲3例、父亲2例及舅父1例,分别切取供者的右半肝1例和左半肝5例作为供肝;在切除受者全部病肝和保留下腔静脉后,对受者施行部分供肝的原位肝移植.术后依据供、受者的临床表现、血液学指标和影像学检查,对活体供肝移植的治疗效果进行评价.结果 术后对供、受者随访了6～21个月.6例供者均健康存活,未发生并发症.6例受者中,1例于术后第4天死于门静脉血栓形成,1例于术后5个月时死于肝内静脉血栓形成,其余4例均长期健康存活.结论 亲属活体供肝移植是治疗儿童终末期肝病的有效方法.术前进行仔细的供、受者选择和完善的影像学检查,术中应用精确的手术技术,术后给予严格的管理是儿童LDLT成功的关键.     

Living-related liver transplantation for Wilson's disease

Liver transplantation with liver grafts from deceased donors is the treatment of choice for patients suffering from Wilson's disease (WD) with end-stage liver disease. There are few reports, however, on the use of liver grafts from living-related donors for WD. Five (two pediatric and three adult recipients) underwent living-related liver transplantation (LRLT) for WD at the University of Tokyo. Two patients presented with fulminant hepatic failure with hemolysis, and the other three presented with decompensating cirrhosis, one with an overlapping neurologic WD. All recipients had a low serum ceruloplasmin level (median: 18 mg/dL), high urinary copper level (mean: 1119 microg/d), and presented with Kayser-Fleischer rings before transplantation. Although one patient died from early graft thrombosis unrelated to WD, the other four patients have shown an excellent long-term prognosis. Following successful transplantation, there was a significant reduction in urinary copper excretion (median: 64 microg/d) in all patients. The neurologic symptoms of WD in one patient, however, worsened after 2 months and gradually subsided, but not completely, over the 2-yr follow-up. For advanced liver failure in WD, we consider LRLT a valuable life-saving option. The improvement of neurologic symptoms, however, requires further evaluation.     

背驮式肝移植治疗Wilson′s病：附29例报告

目的:总结经典背驮式肝移植术(SP2BLT）和改良背驮式肝移植术（MPBLT）治疗Wilson′s病的临床经验。方法:对近12年来29例Wilson′s病患者施行背驮式肝移植的临床资料进行回顾性分析。男22例,女7例; 8～14岁者6例,14～37岁者23例。暴发性肝功能衰竭2例,慢性肝功能不全18例,肝功能基本正常9例; 伴有不同程度的神经精神障碍者24例。手术方式：SPBLT 13例,MPBLT 16例。结果:随访1个月至12年,中位随访时间为47个月。围手术期死亡4例,其中2例死于肝静脉回流受阻导致术后肝功能衰竭,均发生在SPBLT组; 另外2例分别死于肝动脉栓塞和胆瘘合并严重感染。受体1年存活率为86%,3年存活率为79%。血清铜和铜兰蛋白在术后4周基本恢复正常。神经精神障碍术后均有不同程度的缓解。最长存活期12年。结论:背驮式肝移植治疗Wilson′s病可获得满意的临床效果和生活质量,而MPBLT技术可减少术后并发症,提高手术存活率。     

单中心肝移植治疗多囊肝病的疗效分析

目的 探讨肝移植治疗多囊肝病的疗效.方法 回顾性分析2003年9月至2009年7月中山大学附属第三医院收治的8例接受肝移植术的多囊肝病患者的临床资料.手术方式均采用附加腔静脉整形的改良背驮式肝移植术,其中1例同时行肝肾联合移植.术后对急性排斥反应、并发症、移植物功能、患者生存时间等进行随访.结果 全组患者手术均获得成功.平均手术时间为(7.5±2.8)h(4～11 h);平均无肝期时间为(42±14)min(25～70 min);术中平均出血量为(2250±1850)ml(2000～6500ml).围手术期患者死亡2例,其中1例于肝移植术后第39天死于肺部感染和MODS,另1例于肝肾联合移植术后第59天死于MODS.1例患者于肝移植术后第45天因肝动脉狭窄行再次肝移植术.6例患者中位随访时间为60个月(37 ～93个月),生存3年以上6例、5年以上4例、7年以上2例.截至随访终点,未发现移植物急性排斥反应.结论 肝移植是治疗多囊肝病患者安全、有效的方法.     

原位肝移植治疗终末期自身免疫性肝病

目的 评价肝移植治疗终末期自身免疫性肝病的疗效并总结临床经验.方法 回顾性分析2003年9月至2009年7月间因终末期自身免疫性肝病接受肝移植手术的11例患者的临床资料,其中8例为原发性胆汁性肝硬化,2例为自身免疫性肝炎,1例为原发性硬化性胆管炎.平均年龄为(44.2±8.7)岁.手术方式均采用附加腔静脉整形的改良背驮式肝移植术.术后免疫抑制治疗采用他克莫司或环孢素A联合激素的二联免疫抑制方案,部分患者使用骁悉和熊去氧胆酸.结果 本组11例患者中2例原发性胆汁性肝硬化患者死亡,其中1例于术后第5天死于肺部感染和多器官功能衰竭,另1例于术后964 d死于脓毒症和移植肝失功.5例患者术后1个月内出现急性排斥反应,加强免疫抑制治疗后痊愈.9例患者生存良好并存活至今,随访期7～62个月,中位随访时间为38个月.受体1年存活率为91%,3年存活率为82%,最长存活期5年.随访期间未发现复发病例.结论 肝移植是治疗终末期自身免疫性肝病的惟一有效手段,手术时机的正确把握和有效的免疫抑制治疗是减少肝移植术后并发症的关键.

Abstract：

Objective To evaluate the effect of liver transplantation for end-stage autoimmune liver disease (ESALD) and summarize the clinical experience of liver transplantation in the treatment of ESALD.Methods The clinical data of 11 ESALD cases who underwent liver transplantation from September 2003 to July 2009 were analyzed retrospectively. There were 2 males and 9 females ( median age, 44. 2 ± 8. 7years). The indication of liver transplantation was end stage of primary biliary cirrhrosis (8 cases),autoimmune hepatitis (2 cases), and primary sclerosing cholangitis ( 1 case). In all cases, modified piggyback liver transplantation with venacavaplasty was carried out. Postoperatively all patients were treated with immunosuppressive agents including tacrolimus (or cyclosporine A) and prednisone, some patients were treated additionally with mycophenolate mofetil and ursodeoxycholic acid. Results Postoperatively 2patients of primary biliary cirrhosis died, one of lung infection and multiple organ failure on the 5th postoperative day, the other dying of sepsis and graft dysfunction on the 964th postoperative day. Five cases suffered from episodes of acute cellular rejection within 1 month after transplantation and was successfully reversed by strengthened immunosuppressive therapy. Nine patients recovered satisfactorily and with excellent life quality until now. Patients were followed up from 7 months to 62 months with the median follow-up time of 38 months. The recipient survival rate at 1 year and 3 years was 91% and 82% ,respectively. One patient has now survived for 5 years. No recurrent ALD case was found during follow up.Conclusions Orthotopic liver transplantation is an exclusive treatment for ESALD. Optimum operation timing and effective immunosuppressive treatment are very important for decreasing occurrence of complications.     

在两个阶段开展临床肝移植的体会(附8例报告)

目的总结两个阶段开展临床肝移植的经验和体会。方法分析8例人体原位肝移植,包括1例肝肾联合移植的治疗结果。供肝保存第一阶段采用门静脉及肝动脉离体灌注,第二阶段采用腹主动脉及肠系膜上静脉UW液快速原位灌注。受体手术采用原位肝脏植入,吻合次序为肝上下腔静脉、门静脉、肝下下腔静脉、肝动脉和胆管,完成门静脉吻合后即开放门静脉结束无肝期。两个阶段均使用了免疫抑制剂但方法不同。结果第一阶段移植生存期最短39天,最长203天。除1例死于肿瘤复发外,全部死于败血症及严重感染。第二阶段移植病人1例半年后死于肿瘤转移,另3例仍健存,其中肝肾联合移植者生存已超过3年,在社会上正常生活工作。结论第二阶段取得了较好的效果。其中合理选择适应症,良好的供肝保存以及免疫抑制剂的使用等对肝移植的成功有重要影响。     

Liver transplantation in Jehovah''s witnesses

For religious reasons, Jehovah's witnesses refuse transfusion of blood products (red cells, platelets, plasma), but may accept organ transplantation. The authors developed a multidisciplinary protocol for liver transplantation in Jehovah's witnesses. In a 6-year period, nine Jehovah's witness patients were listed for liver transplantation. They received preoperative erythropoietin therapy, with iron and folic acid that allowed significant haematocrit increase. Two patients underwent partial spleen embolization to increase platelet count. Seven patients underwent cadaveric whole liver transplantation, and two right lobe living-related liver transplantation, using continuous circuit cell saving system and high dose aprotinin. No patient received any blood product during the surgical procedure. One patient suffering from deep anaemia after living-related liver transplantation was transfused as required by his family, but died from aspergillus infection. One 6-year-old child was transfused against her parent's will. The authors demonstrated that it is possible to increase haematocrit and platelet levels in cirrhotic patients awaiting liver transplantation. They were able to reduce intraoperative need for blood products, allowing liver transplantation in prepared Jehovah's witness patients. This experience may be beneficial for non-Jehovah's witness liver transplant recipients.     

Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome)

Thirteen out of 268 children (<18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD.     

亲属活体肾移植101例分析

目的 总结16年亲属活体肾移植的经验。方法 101例亲属活体肾移植，除3例为夫妻间供肾外，其余为血缘亲属供肾。供、受者为同卵孪生2例，HLA全配24例，HLA单倍体相同69例，HLA有5个抗原错配者4例，HLA完全错配者2例。73例取供者右肾，28例取左肾；100例经开放手术取肾，1例经腹腔镜取肾。术后采用环孢素A（或他克莫司）、硫唑嘌呤（或霉酚酸酯）及泼尼松预防排斥反应。结果 所有供者术后1周内出院，随访6个月，血肌酐正常。术后96例受者存活，存活时间最长者达15年，其中4例移植肾功能丧失，其原因分别为超急性排斥反应（1例，术中切除肾脏）、慢性移植肾肾病与肾病复发（3例）；5例死亡，除1例术后发生移植肾功能恢复延迟，透析期间因肺出血死亡外，另4例死亡与移植肾无关。术后5例发生急性排斥反应，4例Banff分级为Ⅰ级，经甲泼尼龙冲击治疗，4例逆转，1例无效，恢复透析治疗。术后2例发生尿瘘，5例发生移植肾输尿管慢性梗阻，经手术治疗痊愈。结论 术前对供、受者进行全面综合评估是亲属活体肾移植成功的保证；亲属活体肾移植的组织配型好，供肾缺血时间短，排斥反应发生少，免疫抑制剂用量小，移植肾长期存活率高。     

肝移植术后早期急性肾功能衰竭的诊断和治疗(附41例回顾性分析)

目的总结肝移植术后早期急性肾功能衰竭的防治经验。方法回顾性分析41例肝移植术后早期肾功能不全的发病机制、治疗方法及效果。结果41例肝移植受者中,死亡14例(34.15%)。术后并发症:肺部感染24例,多器官功能障碍综合征(MODS)13例,腹腔出血6例,急性排斥反应6例,胆道并发症3例,门静脉血栓1例,原发性移植肝无功能1例,全身播散性真菌感染1例。术后2例生存超过5年,4例生存超过4年,7例生存超过3年,11例生存超过2年。结论在背驮式肝移植中行腔-腔静脉侧侧吻合(SSCCA)、采用抗CD25单克隆抗体诱导治疗、降低钙神经蛋白抑制剂用量、实施环孢素C2监测对降低肝移植术后早期急性肾功能衰竭的发生率可能有一定意义,对于出现难以逆转的肾损害同时无法耐受血液透析的受者,肾移植可能是惟一的选择。     

Living-related liver transplantation: Report of experiences at Shinshu University Hospital

In view of the relative scarcity of pediatric cadaveric donors, living-related liver transplantation has recently been accepted as an alternative approach. It is also the only method of liver transplantation available in countries where cadaveric organ procurement is prohibited. Here we describe our experience of living-related liver transplantation in 17 patients at Shinshu University Hospital. The safety of the donor operation is of paramount importance in this type of liver transplantation. In Japan, retransplantation is very difficult in the event of the liver graft becoming nonfunctional. We have therefore placed emphasis on the donor hepatectomy technique as well as on surgical procedures and postoperative care to prevent graft loss in the recipient. Fifteen of the 17 patients who received liver transplants are currently alive; and 1 died of cytomegalovirus infection, and 1 of pulmonary complications. The actuarial 1-year survival rate for our series, determined by Kaplan-Meier analysis, was 89.5%. Although living-related liver transplantation requires a complicated surgical procedure, it has achieved reasonable results for both donors and recipients. We consider that living-related liver transplantation is a useful and reasonable option for patients requiring liver transplantation. This work was supported in part by a grant-in-aid for scientific research from the Ministry of Education, Science and Culture of Japan (03404037, 04557056).