原位肝移植治疗终末期肝病9例初步报告

１９９３年９月至１９９６年７月分别为９例终末期肝病病人施行了原位肝移植术。其中４例为原发性肝脏恶性肿瘤，５例为良性终末期肝病。移植术式除１例背肽式肝移植和１例减体积肝移植外，其余７例均为原位全肝移植，并为１例多囊肝，多囊肾，合并肝，肾功能损害闰人施行了肝，肾联合移植术。     

Pediatric liver transplantation. Introduction of a program for southern Germany in Heidelberg

Abstract:  Pediatric liver transplantation in Germany is exclusively performed in four centers in the north of Germany. We report our experience with the implementation of a new pediatric liver transplantation program in Heidelberg for children living in the south of Germany. In a pilot phase, we have transplanted eight children with various transplantation techniques including reduced size, left lateral split (segments 2 and 3), and full left split (segments 1–4). All transplantations were successful. No vascular complications occurred. The immunosuppressive regimen we used was cyclosporine A and methylprednisolone. The rate of acute rejection was three of eight patients. No patient required a retransplantation. One patient died due to a severe fungal sepsis he had acquired prior to transplantation. We conclude that in a multidisciplinary approach with careful patient selection a new program for pediatric liver transplantation can be successfully established.     

De novo hepatocellular carcinoma in a non-cirrhotic allograft 27 years after liver transplantation: A case report

Hepatocellular carcinoma recurrence after liver transplantation is a well-known complication but the development of de novo hepatocellular carcinoma in non-cirrhotic allograft with no previous history of hepatic malignancy either in the donor or the recipient is extremely rare. A 33-year-old man underwent deceased donor liver transplantation due to HBV-HDV cirrhosis in 1991. The donor was healthy, with negative viral serology. Pretransplant assessment and explant liver pathology revealed no tumor. He developed an 8 cm mediastinal thymus cancer in 2014, a chronic myeloid leukemia in 2015 and a 16 mm renal cell carcinoma in 2017. After 27 years, in 2018, his routine follow-up sonography showed incidentally a 37 mm hepatic nodule in segment VII which revealed after percutaneous liver guided biopsy a hepatocellular carcinoma. As no extra hepatic metastasis was noted, segmentectomy was done. The pathological report confirmed a moderately differentiated hepatocellular carcinoma nodule of 50 mm diameter with absence of microvascular invasion and the non-tumoral liver showed histological features of NASH (SAF score: S1A2F3, NAS score: A3F3 and LAFSc:5) with absence of HBsAg and HBcAg. This case emphasizes the importance of long-term close surveillance by imaging of the graft even in the absence of viral recurrence and graft cirrhosis.     

肝肾联合移植术中肝脏对肾脏的保护作用

目的：探讨肝肾联合移植术中肝脏对肾脏的保护作用。方法：给1例肾移植术后移植肾失功并肝炎后肝硬化患者先行丧失功能移植肾切除术，术后第5天始每天口服环磷酰胺50mg，连服3个半月。经两次血浆置换，群体反应抗体(PRA)由66％降至22．5％，施行一期肝肾联合移植。供肝血流开放前及开放后每间隔半小时动态检测PRA，共7次，术后1个月、3个月、9个月复查PRA。术后免疫抑制治疗采用FK506、霉酚酸酯(MMF)和激素联合应用。结果：术后供肝、肾立即发挥功能，肝动静脉血流开放后，PRA由22．5％降至5％，并维持在7．5％(见图1)，术后未发生排斥反应现象。随访9个月，移植肝、肾功能正常，术后1个月、3个月、9个月复查PRA分别为7．5％、8．33％和7．5％。结论：肝肾联合移植术中肝脏对肾脏有一定的保护作用。     

原位肝移植治疗终末期肝病36例报告

目的 探讨肝移植治疗晚期肝病的疗效。方法 回顾过去2000年8月至2002年1月1日内实施的36例原位肝移植病例,对手术适应证、并发症、死亡病例等进行分析。结果36例病人术后存活25例,近期死于各种并发症9例,19例肝癌病人中有6例术后已经生存14-20个月。乙肝病人有2例1年后乙肝再感染。术后近期主要并发症有:胸、腹腔出血7例,动脉血栓形成3例,肾功能衰竭8例,腹腔或肺部感染5例,急性排斥发应31例次。术前肝功能太差者易发生术中、术后严重出血,从而导致休克、感染、肾衰等并发症,是术后近期死亡的主要原因。肝癌病人凝血状态较好,术后易发生动脉血栓形成。结论 乙肝所致肝硬化和肝癌是肝移植较好的适应证;在病人出现ICU依赖后实施肝移植将增加手术死亡率;恰当地处理病人的凝血状态,可减少出血和血栓形成等严重并发症的发生;及时检查并调整环孢素A和FK506的血药浓度,提高对急性排斥的临床和病理诊断的认识,将有助于减少和正确处理急性排斥。     

Orthotopic liver transplantation in hemophilia B: a case report

Liver transplantation is a treatment modality that is being used with increasing frequency in cases of liver-based metabolic defects. This is a case report of a patient with hemophilia B who was treated since childhood with factor IX replacement for recurrent hemarthroses. Subsequent hepatitis B (HBV) and C (HCV) infection had resulted in the development of chronic active hepatitis, ultimately leading to cirrhosis. Orthotopic liver transplantation performed for endstage liver disease resulted in a rise in factor IX levels from 2% to 83% of normal values within 24 h post-operatively, and levels remained above 90% of normal values after postoperative day 3 without factor IX replacement. To our knowledge, only two cases of hemophilia B treated by orthotopic liver transplantation have been reported. This procedure has, however, only been implemented in cases of terminal liver insufficiency in hemophiliacs.     

Acute liver necrosis in the HELLP syndrome: successful outcome after orthotopic liver transplantation. A case report

We discuss the case of a 30-year-old primipara woman who developed a liver rupture as a complication of the HELLP syndrome. A liver necrosis and bleeding made a hepatectomy necessary. A portocaval shunt was able to maintain the patient until she underwent urgent liver transplantation. In an excellent state of recovery, the woman and her baby were discharged from the hospital 66 days after having been admitted.     

Post-regraft supra-hepatic caval obstruction in liver transplantation: a successful outcome with expandable stents. A case report

Supra-hepatic inferior vena cava (IVC) obstruction is an unusual complication following an orthotopic liver transplantation (OLT) and is seen more often in hepatic regrafts. Dilatation of these stenotic lesions and the use of endovascular stents has been described in the past. Although the results of this technique are unquestionably superior to surgical correction, their use in the very early post-operative period is not without danger. Herein we describe a case where this modality was used successfully within 24 h of an OLT. Received: 17 June 1997 Received after revision: 30 September 1997 Accepted: 8 October 1997     

Piggy-back versus conventional technique in liver transplantation: report of a randomized trial

Liver transplantation with preservation of the recipient vena cava (the “piggy-back” technique) has been proposed as an alternative to the traditional method. We performed a randomized study on 39 cirrhotic patients, 20 who underwent the piggy-back technique (group 1) and 19 the traditional method using venovenous bypass (group 2) to evaluate the feasibility and true advantages of the piggy-back technique compared to the traditional method. Two patients were switched to the conventional technique due to the presence of a caudate lobe embracing the vena cava in one patient and a caval lesion in the other. Statistically significant differences between the two groups were only found for the warm ischemia time (48.5 ± 13 min for piggy-back vs 60 ± 12 min for the conventional method) and for renal failure (zero cases in group 1 vs four cases in group 2). We therefore believe that liver transplantation with the piggy-back technique can easily be performed in almost all cases, and that only a few, specific situations, such as a very enlarged caudate lobe, do not justify its routine use. Received: 30 May 1996 Received after revision: 7 October 1996 Accepted: 28 October 1996     

婴幼儿活体肝移植33例

目的 探讨活体肝移植治疗婴幼儿终末期肝病的疗效.方法 回顾性分析2006年10月至2009年9月上海交通大学医学院附属仁济医院33例实施活体肝移植的婴幼儿的临床资料.本组患儿中位年龄10.9个月,平均体质量8.2 kg,供肝均采用肝左外叶.术后采用他克莫司或环孢素A+激素二联方案或在此基础上再加用吗替麦考酚酯的三联方案行免疫抑制治疗.分析评价手术方法、围手术期处理和随访结果.结果 供者和受者手术时间、术中出血量、术中输血量分别为(384±108)min、(183±35)ml、0和(500±103)min、(296±163)ml、(292±159)ml,供肝冷缺血时间为(64±23)min,移植物质量为(249±52)g,移植物质量与受者体质量比为2.1%±0.4%.全部供者均顺利康复,无手术并发症.受者出现肝动脉栓塞3例,门静脉栓塞2例,各类胆道并发症9例,感染11例,急性排斥反应2例,围手术期死亡5例.本组患儿1年累积生存率为85%(28/33).结论 婴幼儿终末期肝病可通过活体肝移植取得理想的效果.外科技术的提高、围手术期管理经验的积累和规范的随访可提高手术成功率和长期生存率.

Abstract：

Objective To evaluate the efficacy of living donor liver transplantation in the treatment of infants with end-stage liver diseases. Methods The clinical data of 33 infants who received living donor liver transplantation at the Renji Hospital of Shanghai Jiaotong University from October 2006 to September 2009 were retrospectively analyzed. The median age of the infants was 10.9 months, and the mean body weight was 8.2 kg.All of the grafts were left lateral lobes. Tacrolimus (or cyclosporine A) + steroid or tacrolimus (or cyclosporine A)+ steroid + mycophenolate mofeti] were applied to the infants to suppress the immune reaction. Operative techniques, perioperative management and results of follow-up were analyzed. Results The mean operation time,blood loss and blood transfusion of the donors were (384±108)minutes, (183±35) ml and O, and the three indexes of the recipients were (500± 103) minutes, (296±163) ml and (292 ± 159) ml , respectively. The cold preservation time of the grafts was (64 ±23)minutes, the mean weight of the grafts was (249 ±52)g, and the mean graft to recipient weight ratio was 2.1% ± 0.4%. All donors recovered smoothly and no complication occurred. Of the recipients, three were complicated with hepatic artery thrombosis, two with portal vein thrombosis,nine with biliary complications, 11 with infection, two with acute rejection and five infants died perioperatively.The one-year cumulative survival rate of the infants was 85% (28/33). Conclusions Infants with end-stage liver diseases could be treated by living donor liver transplantation. The development of surgical techniques and perioperative managements improves the success rate of operation and the long-term survival rate.     

Predictive factors for early mortality following liver transplantation

AIMS: To retrospectively review our liver transplant performance to identify factors that influenced early outcomes and to prospectively test their validity in predicting outcomes. METHODS: Clinical records from 190 patients with liver transplants (LT; n = 200) performed between 1991 and 1997 were reviewed and the data evaluated by univariate and multivariate analyses regarding clinical outcome. The prognostic model thus obtained was prospectively evaluated in 55 patients undergoing transplant between 1999 and 2000. RESULTS: Main indication for transplant was post-necrotic cirrhosis (61%), mostly HCV(+). The majority of patients were Child-Pugh C status (46%). Post-operative mortality at 3 months was 15.3%. Risk factors predicting death were: Child-Pugh C status (OR 1.3), pre-LT renal insufficiency (OR 5.8), malnutrition (OR 2.9) and technically complex surgery requiring cross-clamping with or without bypass (OR 4.9). None of the donor factors was significant. Prospectively applied to predict outcome in the 55 patients, the model had a sensitivity of 80% and a specificity of 88.8% with a higher-than-anticipated accuracy with a positive predictive value of 61.5% and a negative predictive value of 95.3%. CONCLUSIONS: Pre-LT renal insufficiency is the most significant risk factor for early mortality and suggests that LT should be performed before evidence of irreversible renal insufficiency becomes manifest.     

Central pontine myelinolysis after liver transplantation: a case report

A patient development deteriorating mental status, quadriparesis, and severe pseudobulbar palsy with the inability to speak or swallow following orthotopic liver transplantation (OLT). Subsequently, abnormalities were found in the pons on MRI that were consistent with central pontine myelinolysis (CPM). Marked recovery occurred following transfer to the rehabilitation medicine service. Seven months following development of CPM, a mild dysarthria has persisted, but full ambulation has returned. Although no significant fluctuations in serum sodium were seen perioperatively, multiple risk factors associated with the development of CPM were present, including end-stage liver disease, a history of alcohol abuse, malnutrition, hypoxia, and use of cyclosporin medication postoperatively. This case demonstrates that the development of CPM may occur following OLT despite meticulous attention to serum sodium concentrations. We conclude that CPM is multifactorial in nature. There can be a great variation in its clinical course.     

Pontine myelinolysis following liver transplantation: a report of two cases

Two cases of central pontine myelinolysis (CPM) following orthotopic liver transplantation (OLT) are reported. Several months after the onset of this neurological syndrome, the two patients are still alive but with severe neurological sequelae. Some patients undergoing emergency OLT present a high risk of CPM because of pre-existing malnutrition, encephalopathy, and hepatic insufficiency. All of these are associated with an inevitable abrupt rise in sodium serum concentration due to intraoperative compensation of blood losses with high-sodium content blood products. Whenever the renal capacity to excrete sodium is impaired by the surgical procedure, continuous intraoperative venouvenous hemofiltration is recommended.     

Successful management of hemolysis in ABO-nonidentical orthotopic liver transplantation by steroid therapy: a case report

Hemolysis due to donorderived B lymphocytes has been reported in patients who have undergone ABO-nonidentical orthotopic liver transplantation (OLT). Yet, until now, little was known about the management of this transplantation-induced hemolysis. In this report we describe our experience with hemolysis in a patient after OLT. In addition, based on theoretical assumption, we hypothesize that corticosteroids can be helpful in the management of ABO-nonidentical OLT-induced hemolysis.     

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目的 报告国内首例同种异体肝小肠联合移植，并就肝肠联合移植适应证、排斥诊断与治疗、感染防治、肠功能恢复与营养支持进行讨论。方法 供肠长380cm，供肠肠系膜上动脉、门静脉分别与受体腹主动脉、下腔静脉端侧吻合；供肝行改良背驮式肝移植。术后免疫抑制剂采用FK506 激素 骁悉 赛尼哌方案。排斥监测采用内镜指导下肠粘膜活检病理学检查及临床观察。并采取措施防治感染和促进移植肠功能的恢复。结果病人恢复顺利，移植肠和移植肝功能良好，未发生排斥反应，术后3个月时已摆脱全肠外营养，依靠肠内营养维持营养状态。结论 肝小肠联合移植对肠衰竭合并全肠外营养所致肝功能损害病人，是可行、有效的治疗方法。     

Relevance of a positive crossmatch in liver transplantation

We studied 27 liver transplants in 24 patients performed between November 1984 and January 1988. We investigated retrospectively the importance of donor reactive HLA class I and class II and of non-HLA antibodies for graft survival in these patients. In order to determine the specificity and class of the antibodies, we used monoclonal antibodies to HLA-A,-B,-C and DR and DQ antigens to block cytotoxicity of sera and the reagent dithiothreitol to characterize the immunoglobulin class. We found that humoral immunity to HLA antigens in liver-grafted patients, demonstrable as the presence of cytotoxic antibodies reactive with donor splenic T and/or B cells in the pretransplantation period, is associated with significantly lower graft survival as compared with patients without demonstrable preformed HLA antibodies (P=0.01). In addition we found that a substantial proportion of patients had donor-reactive cytotoxic antibodies which were not HLA specific. Thus, our study shows that HLA immunity can influence liver allograft survival, and that it is useful to have patient cytotoxic antibodies characterized with regard to HLA reactivity prior to transplantation.     

Vascular events after liver transplantation: a long-term follow-up study

Long‐term follow‐up studies on the impact of vascular events (VE) and risk factors of liver transplant recipients are scarce. In this study, 311 recipients of a first isolated liver transplant who survived at least 1 year were followed up from 1979 to 2002. The median follow‐up duration was 6.2 (range1–22.7) years. Overall median survival was 18.7 [95% confidence interval (CI): 15.5–20.1] years and this was significantly lower compared with age‐ and sex‐matched controls. Eleven (21%) of the patients had a vascular cause of death and VE were the third cause of death. VE occurred later compared with other causes of death (mean 10.3 years vs. 4.5 years, P < 0.0001, 95% CI: 2.7–8.9). Systolic hypertension, systolic blood pressure, smoking, renal failure, age, hypertriglyceridemia, serum total cholesterol levels and hypercholesterolemia at the 1‐year follow‐up visit were associated with the occurrence of VE, but renal failure and age at 1 year after transplantation were the only independent risk factors for vascular death (hazard ratio 0.06, 95% CI: 0.01–0.41 and hazard ratio 1.17, 95% CI: 1.02–1.34, respectively). Finally, it was shown that the adequate treatment of hypertension was associated with a significant reduced risk of vascular death. Therefore, vascular risk factors should be treated aggressively to prevent VE in the long term.     

Tuberculosis following liver transplantation: report of a case and review of the literature

We report on a 44-year-old man who developed tuberculosis 4 months after liver transplantation. The diagnosis was confirmed using a polymerase chain reaction (PCR) technique in bronchial alveolar lavage (BAL) fluid, and the patient was successfully treated by reducing his immunosuppression and administering antituberculous drugs. The patient became afebrile 20 days after starting antituberculous therapy and remains well at home. A review of the literature revealed that tuberculosis after liver transplantation is a rare complication with a reported mortality rate of as high as 40%. The mortality is highest for patients who become symptomatic within 3 months after transplantation (83% vs 0%, P<0.01; Fisher's exact test) and for those with an interval between the initial symptom and diagnosis of more than 2 weeks (71% vs 0%, P<0.05). Early diagnosis is, therefore, essential for successful resolution of tuberculosis after liver transplantation.