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.     

背驮式原位肝移植术四例报告

目的 为了提高终末期肝病的存活率。方法 于１９９６年５月、８月、１９９７的上月和１９９８年２月先后成功地施行了４例背驮式原位肝移植术。结果 除例１忆 炎后肝硬变,受体术后２１４天突变死于乙肝复发并急生肝坏死和急性胰腺炎外。例２、例３和例４肝豆状核变性受体术后恢复顺利,效果良好,已分别健康生存５７５天、３９０天和１６２天。结论 肝移植是目前治疗大部分终末期肝移植的最有效手段。     

亲体部分肝移植术后肝动脉栓塞的防治

目的 探讨亲体部分肝移植（LRLT）术后肝动脉栓塞（HAT）的预防和治疗。方法 于2001年1月至6月成功的对6例肝豆状核变性患者进行了亲体部分肝移植。术中重视肝动脉吻合技术；根据红细胞压积（HCT）控制输血量；术后根据B超检查肝动脉血流及凝血相结果，选择不同的血浆输注,并采用低分子肝素、华法令和低分子右旋糖酐等联合抗凝治疗。结果 6例肝移植后均健康存活，1例发生HAT，经急诊取栓并行肝动脉重建术后无效，再次肝移植后存活。结论 理想的肝动脉吻合、适当控制血制品和正确使用抗凝治疗，是预防LRLT后HAT的重要手段，一旦发生HAT，应急诊取栓，进行肝动脉重建术，甚至进行再次肝移植。     

原位肝移植70例报告

目的　探讨肝移植治疗终末期肝病的临床效果。方法　在过去2年内,70例终末期肝病患者接受了72例次原位肝移植手术,其中包括1例小儿减体肝移植。结果　手术近期死亡18例（25.7%）,随访期间死亡14例(26.9%),术后胆道并发症发生率23%,肝动脉并发症20%。术后生存时间超过6个月者30例,超过1年者17例,其中肝癌组（31例）,有9例术后生存已超过6个月,3例超过1年。结论　减少外科手术并发症是提高肝移植手术成功率以及长期存活的关键。对于肝硬化合并原发性肝癌的患者,肝移植应作为首选的治疗方法。     

原位肝移植70报告

目的：探讨肝移植治疗终末期肝病的临床效果。方法：在过去2年内,70例终末期肝病患者接受了72例次原位肝移植手术,其中包括1例小儿减体肝移植。结果：手术近期死亡18例（25．7％）,随访期间死亡14例（26．9％）,术手胆道并发症发生率23％,肝动脉并发症20％。术后生存时间超过6个月者30例,超过1年者17例,其中肝癌组（31例）,有9例术后生存已超过6个月,3例超过1年。结论减少外科手术并发症是提高肝移植手术成功率及长期存活的关键。对于肝硬化合并原发性肝癌的患者,肝移植应作为首选的治疗方法。     

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。     

背驮式肝移植术及其并发症的预防

目的 探讨同种原位背驮式肝移植术（PBOLT）常见手术并发症的预防。方法 4例终末期良性肝病行5次PBOLT,其中2例为肝肾联合移植,1例为肝移植术后慢性排斥行再次肝移植。结果 5次肝移植术后肝功能恢复良好,未发生与背驮式手术相关的并发症,最长存活1年,生活质量良好。结论 PBOLT可适用于任何终末期良性肝病患者,包括再次肝移植,正确处理第三肝门,以三根肝静脉成形后与供肝肝上下腔静脉吻合,术中维持血液动力学稳定是减少或避免该术式有关手术并发症的重要措施。     

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     

儿童心脏死亡捐肝移植治疗儿童肝糖原累积症一例

儿童肝移植是治疗儿童终末期肝病惟一有效的方法,适用于胆管闭锁、肝硬化、暴发性肝衰、肝糖原累积症、肝豆状核变性等小儿先天性疾病.以往儿童肝移植供体的来源,大多为亲属供肝或成人供肝行劈离式肝移植.这种供肝来源手术技术难度大、风险高、术后并发症发生率高,严重影响儿童肝移植的成功率和术后生存质量.儿童心脏死亡器官捐赠(DCD)供肝避免了肝断面带来的技术难度和术后相关并发症,而且儿童间供受体肝移植更符合儿童生理生长发育需要,是儿童肝移植的理想供体来源.2011年7月我院完成1例儿童DCD供肝移植,现报告如下.     

肝移植治疗肝豆状核变性的单中心疗效分析

目的探讨肝移植治疗肝豆状核变性的疗效。方法 回顾性分析2003年9月至2009年7月接受肝移植手术治疗的5例肝豆状核变性患者的临床资料。结果 采用附加腔静脉整形的改良背驮式肝移植3例,成人间活体部分肝移植2例。围手术期死亡1例,死于肺部真菌感染和多器官功能衰竭,另4例患者恢复良好且存活,其中生存时间≥1年4例,≥3年3例。3例伴有神经精神功能障碍的患者中,除1例死亡外,另2例症状得到不同程度的改善。存活的4例术后1个月肝功能较术前明显改善,血清铜蓝蛋白水平明显升高。结论 全肝移植或活体部分肝移植术能改善肝豆状核变性患者的铜代谢和神经精神症状,提高患者的生活质量和存活率。     

辅助性肝移植研究进展

辅助性肝移植足指住保留受体自身全部或部分肝脏的情况下,将供肝植入受体体内.根据供肝植入部位不同,可分为异位辅助性肝移植和原位辅助性肝移植;根据植入肝体积的多少,可分为全肝辅助性肝移植和部分肝辅助性肝移植;供肝可来源于脑死亡供体也可以来源于活体供体.山于原位辅助性肝移植只能移植部分肝脏,故通常称为原位辅助性部分肝移植.辅助性肝移植伴随着肝移植的发展而不断发展,虽然还存在许多尚待解决的问题,但辅助性肝移植独特的优势再次引起肝移植界的广泛关注.     

Auxiliary Partial Orthotopic Living Donor Liver Transplantation: Kyoto University Experience

Auxiliary partial orthotopic liver transplantation (APOLT) was initially indicated as a potentially reversible fulminant hepatic failure and non-cirrhotic metabolic liver disease to compensate for enzyme deficiency without complete removal of the native liver. We expand our indication of APOLT for small-for-size grafts to support the function of implanted grafts during the early post-operative period, and for ABO-incompatibility to sustain a patient's life if the patient has a graft failure. We retrospectively reviewed 31 patients undergoing APOLT from living donor. The indication of APOLT was fulminant hepatic failure in 6, non-cirrhotic metabolic liver disease in 6, small-for-size grafts in 13 and ABO-incompatible cases in 6. The cumulative survival rate for APOLT at 1 and 5 years was 57.9% and 50.6%, and 78.8% and 73.8% for standard LDLT. None of the patients who underwent transplantation with APOLT for fulminant hepatic failure had long-term patient survival. The incidence of acute cellular rejection was higher in APOLT (58.1%) than standard LDLT (35.0%). Biliary complication was higher and the need for retransplantation was greater in APOLT than standard LDLT (p < 0.01). The results suggest that the indications of APOLT should be reconsidered in view of the risk for complications and retransplantation.     

Functional, life-threatening disorders and splenectomy following liver transplantation

Splenectomy (SPL) in cirrhotic patients undergoing liver transplantation (LTx) may resolve specific problems related to the procedure itself, in case of functional and life-threatening clinical situations often occurring as a result of liver cirrhosis and portal hypertension. METHOD: A single-center experience of ten splenectomies in a series of 180 consecutive adult liver transplant patients over a period of 6 yr is reported. The mean patient age was 46.8 +/- 9.5 yr (range 25 57 yr). Indications for SPL were post-operative massive ascitic fluid loss (n = 3), severe thrombocytopenia (n = 3), acute intra-abdominal hemorrhage (n = 2), infarction of the spleen (n = 1), and multiple splenic artery aneurysms (n = 1). RESULTS: Extreme ascites production due to functional graft congestion disappeared post-SPL, with an improvement of the hepatic and renal functions. SPL was also effective in cases of thrombocytopenia persistence post-LTx, leading to an increase in the platelet count after about 1 wk. Bleeding episodes related to left-sided portal hypertension or trauma were also resolved. The rejection rate during hospitalization was 0%, and no other episodes were recorded in the course of the long-term follow-up. However, sepsis with a fatal outcome occurred in 4 patients, i.e. between 2 and 3 wk post-SPL in three cases and 1 yr after the procedure as a result of pneumococcal infection in the last case. Fatal traumatic cranial injury occurred 3 yr post-LTx in another case. Five patients (50%) are still alive and asymptomatic after a median follow-up period of 36 months. CONCLUSION: The lowering of the portal flow appears to resolve unexplained post-operative ascitic fluid loss as a result of functional graft congestion following LTx. However, because of the enhanced risk of SPL-related sepsis, a partial splenic embolization (PSE) or a spleno-renal shunt could be used as an alternative procedure because it allows us to preserve the immunological function of the spleen. SPL is indicated in case of post-transplant bleeding due to left-sided portal hypertension and trauma, spleen infarction, and to enable prevention of hemorrhage in liver transplant patients with multiple splenic artery aneurysms. Severe and persistent thrombocytopenia could be treated with PSE. Because the occurrence of fatal sepsis post-SPL is a major complication in LTx, functional disorders, such as ascites and thrombocytopenia, should be treated with a more conservative approach.     

Auxiliary heterotopic partial liver transplantation in pigs with acute liver failure

Fulminant hepatic failure is usually fatal without liver transplantation; however, orthotopic liver transplantation is often difficult to perform due to the high risk of coagulopathy and the development of multiple organ failure. Auxiliary heterotopic partial liver transplantation (APLT), however, has the potential to provide an effective hepatic support system considering that the host liver is left in situ and the surgical procedure is less invasive. In this report, we describe the beneficial effects of performing 60% APLT on the hepatic function and survival of pigs with acute hepatic failure induced by hepatic artery ligation. The pigs were divided into a control group of nine animals (group 1) that had portal vein and hepatic artery ligation with a side-to-side portacaval shunt, and an APLT group of seven animals (group 2) that had portal vein and hepatic artery ligation with APLT. The two left lateral lobes of the donor liver were resected, reducing the liver weight to about 60%, and the graft was placed in the right subhepatic space. No deaths occurred intraoperatively. In group 1, eight pigs died of massive liver necrosis within 48 h and one died between 48 and 72 h (median surivival 23 h). In group 2, two pigs died within 72 h due to preservation or anesthetic problems, but five survived for more than 3 days (median survival 13.4 days), with a significant difference between the two groups (P<0.05). One animal was killed 30 days after APLT and excellent graft function was demonstrated by the synthesis of clotting factors, ammonia detoxification, and glucohomeostasis. Moreover, evidence of hepatic regeneration was found in the transplanted livers. These results indicate that APLT provides metabolic support and improves survival in animals with induced acute liver failure.     

Survival following right lobe split graft,living‐ and deceased‐donor liver transplantation in adult patients: a systematic review and network meta‐analysis

Graft and patient survival outcomes following split liver transplantation (SLT), living‐donor liver transplantation (LDLT) and deceased‐donor liver transplantation (DDLT) were estimated using Bayesian network meta‐analysis. Databases were searched for relevant articles over the previous 20 years (MEDLINE, Embase, Cochrane Library and Google Scholar). Systematic review, pairwise meta‐analysis and Bayesian network meta‐analysis were performed. Pairwise meta‐analysis demonstrated that there were no significant differences in graft and patient survival outcomes. Consequently, Bayesian network meta‐analysis demonstrated no significant differences in 1‐, 3‐ and 5‐year graft and patient survival between the three alternative liver transplantations. No discrepancies were demonstrated after comparisons of direct and indirect evidence of 1‐, 3‐ and 5‐year patient and graft survival of the three node‐split models namely SLT, LDLT and DDLT. The 1‐, 3‐ and 5‐year graft and patient survival of the SLT and LDLT cohorts compared to the DDLT cohort demonstrated no significant differences. The direct and indirect evidence of this study can serve as comparator for future studies.     

活体部分肝移植、全肝移植的临床研究(附22例报告)

目的：总结临床肝移植的经验。方法：对１０例活体部分肝移植（包括一例活体肝移植肝动脉栓塞后施行减体积肝移植）和１２例全肝移植的临床资料进行回顾性分析。结果：全组存活２１例,一例原位肝移植死于多脏器功能衰竭。乙型肝炎病人采用拉米夫定加小剂量ＨＢＩｇ治疗,１０例病人术后随访１～１３月,复查发现Ｋ－Ｆ环有不同程度变浅。术后并发症：腹腔出血２例,需再次剖腹探查止血;ＡＲＤＳ５例;急性排斥的反应一例;急性肾功能衰竭一例。结论：肝移植是治疗终末期肝病的有效方法。完善的手术技术和正确的围手术期处理是肝移植成功的关键,活体部分肝移植是缓解供肝短缺问题又一方向。     

Side-to-side cavo-cavostomy: a useful aid in “complicated” piggy-back liver transplantation

Piggy-back liver transplantation is a useful technical variant of orthotopic liver transplantation. Its success can, however, be compromised by severe stenosis or obstruction of the recipient's inferior vena cava at the level of the anastomosis. A technique is described — side-to-side cavocavostomy — to resolve this difficult intraoperative situation.     

Pediatric liver transplantation from neonatal donors

Sixteen recipients of neonatal liver grafts were compared with 114 contemporaneous pediatric recipients of grafts from older donors. Graft and patient survival were worse in the neonatal group although the differences were not statistically significant. Patients with neonatal livers who had no technical complications required a longer time postoperatively to correct jaundice and a prolonged prothrombin time. These functional differences were limited to the 1st postoperative month and the end result was the same as with liver transplantation from older donors.     

Graft size-matching in living related partial liver transplantation in relation to tissue oxygenation and metabolic capacity

The influence of graft size-matching on tissue oxygenation and metabolic capability was studied in living related partial liver transplantations for 47 pediatric patients. Their age ranged from 4 months to 17 years 3 months, their body weight from 4.0 to 58.0 kg, graft weight from 191 to 440 g, and graft weight/recipient body weight ratio from 0.61% to 6.0%. Tissue oxygenation and its heterogeneity were investigated by measuring oxygen saturation of hemoglobin in the liver sinusoid (SO₂), coefficient of variation of SO₂, and arterial ketone body ratio. The metabolic capacity of the graft was investigated by measuring bilirubin clearance, recovery of cholesterol esterification, and ketone body production. In infants with a relatively large liver graft, both intra- and extracellular oxygenation remained low soon after reperfusion but recovered to the control value by the end of the operation. In adolescent recipients of a relatively small graft, by contrast, synthetic and detoxification capacities were relatively deficient; however, these improved with time. These results indicate that sufficient tissue oxygenation and liver regeneration are essential for successful liver transplantation with relatively large and small grafts, respectively.