小儿肝移植的探讨

目的本研究探讨肝移植治疗小儿终末期肝病的方法和疗效。方法我院分别于2001年11月至2002年12月对7例患儿(先天性胆道闭锁5例，先天性肝纤维化1例和肝脏血管内皮肉瘤1例)进行肝移植术，患儿年龄6个月至12岁。其中亲体部分肝移植术两例，供体分别为患儿的母亲和父亲。手术分别切取供体的右半肝和左外侧叶。受体行保留下腔静脉的全肝切除术，然后将供肝进行原位移植。减体积肝移植3例，取左半肝和左外侧叶为移植物。割离式肝移植2例，分别取左外后叶和右后叶为移植物。结果7例患儿分别移植肝重260～565g，亲体肝移植的两例供体随访14～15个月，均无并发症健康生活。接受移植的7例患儿中6例术后已经分别健康存活15～1个月，1例于术后第5天死于心功能衰竭。结论肝移植是治疗小儿终末期肝病的有效方法，术前准确的血管影像学检查，严格的围手术期管理特别是精确的手术技术是小儿肝移植成功的关键。     

Isolated liver transplantation for decompensated end-stage liver disease in children with intestinal failure

Cholestasis progressing to end-stage liver disease (ESLD) is common in intestinal failure (IF) in infants. Isolated liver transplantation (OLT) is performed when eventual enteral sufficiency is expected. We reviewed our experience with OLT for ESLD in patients with residual IF. From 1998 to 2004, four IF patients underwent OLT for ESLD at our institution. Three were performed as UNOS status I for acute decompensation of chronic liver failure; one other with severe cholestasis with a living donor. The living donor recipient died within months after OLT of chronic respiratory failure despite normal liver function. One recipient remains on parenteral nutrition (PN) and continues to receive partial enteral feeds with normal liver function. Two other recipients became enterally sufficient after the OLT. Biliary complications occurred in two patients, one with late hepatic artery thrombosis. Resolution was achieved with serial balloon dilatations and the other by conversion from duct-to-duct anastomosis to a choledochoduodenostomy. The morbidity of OLT for this indication is higher than for others likely because of comorbidities like sepsis and pulmonary insufficiency. OLT is complicated further by the attempt to maximally preserve residual bowel length for the biliary reconstruction. OLT can be an emergent life-saving procedure in IF patients despite higher morbidity. Improved liver function and diminished portal pressures may shorten the time to enteral sufficiency. Management of parenteral nutrition after OLT can avoid ESLD and eliminate small bowel transplantation in selected patients.     

Emergent right lobe adult-to-adult living-donor liver transplantation for high model for end-stage liver disease score severe hepatitis

The aim of this study was to explore the feasibility of emergency right lobe adult-to-adult living-donor liver transplantation (LDLT) for high model for end-stage liver disease (MELD) score severe hepatitis. Consecutive 10 high MELD score severe hepatitis patients underwent emergency right lobe adult-to-adult LDLT in our hospital from April to December 2007. The MELD score was 34.50 ± 2.088. The outcomes of these recipients were retrospectively analyzed. Among them, eight cases of ABO blood group were identical and two cases compatible, one case was Rh negative. Two recipients died and the rest of the recipients and all donors are safe; perioperative and 2-year survival rate was 80%. The mean graft-recipient weight ratio (GRWR) was 1.27% ± 0.25%, and graft volume to recipient standard liver volume ratio (GV/ESLVR) was 56.7% ± 6.75%. Of the 10 patients, three received right lobe grafts with middle hepatic vein (MHV), four without MHV, three without MHV but followed by V and VIII hepatic vein outflow reconstruction. An encouraging outcome was achieved in this group: elevated serum creatinine, serum endotoxin, decreased serum prothrombin activity, and Tbil returned to normal on postoperative days 3, 7, 14, and 28, respectively. One-year survival rate was 80%. Outcomes of emergency right lobe adult-to-adult LDLT for high MELD score severe hepatitis were fairly encouraging and acceptable. Emergency right lobe adult-to-adult LDLT is an effective and life-saving modality for high MELD score acute liver failure patients following severe hepatitis.     

Living donor liver transplantation in children with cholestatic liver disease: a single-center experience

Objectives

Cholestatic liver disease (CLD) is the main indication for liver transplantation in children. This retrospective study evaluated the outcomes of living donor liver transplantation (LDLT) in children with CLD.

Methods

One hundred fifty-nine children with CLD who underwent 164 LDLT between May 2001 and May 2011 were evaluated. Their original diseases were biliary atresia (n = 145, 91%), Alagille syndrome (n = 8, 5%), primary sclerosing cholangitis (n = 2), and the others (n = 4). The mean age and body weight of the recipients at LDLT was 42 ± 53 months and 14.0 ± 11.0 kg, respectively.

Results

Parents were living donors in 98%. The left lateral segment was the most common type of graft (77%). There were no reoperations and no mortality in any living donor. Recipients' postoperative surgical complications consisted mainly of hepatic arterial problems (7%), hepatic vein stenosis (5%), portal vein stenosis (13%), biliary stricture (18%), intestinal perforation (3%). The overall rejection rate was 31%. Cytomegalovirus infection and Epstein-Barr virus disease were observed in 26% and 5%, respectively. Retransplantation was performed five times in four patients; the main cause was hepatic vein stenosis (n = 3). Four patients died; the main cause was gastrointestinal perforation (n = 2). The body height of Alagille syndrome patients less than 2 years old significantly improved compared with older patients after LDLT. The 1-, 5-, and 10-year patient survival rates were 98%, 97%, and 97%, respectively.

Conclusions

LDLT for CLD is an effective treatment with excellent long-term outcomes.     

Anesthesia for living-donor liver transplantation in a patient with adult polycystic liver disease

Anesthesia for living-donor liver transplantation (LDLT) was performed for two patients with adult polycystic liver disease (APLD). APLD is characterized by gradual cystic transformation of both lobes of the liver. Abdominal enlargement, poor appetite, abdominal pain, infection of liver cysts and portal hypertension are symptoms of this disease. Liver transplantation is indicated as the final therapy. Our two patients had very large livers (7400 g and 9500 g). The second patient had suffered renal failure due to a polycystic kidney so that continuous hemodiafiltration had to be performed after surgery. In both cases, sudden hypotension frequently occurred during manipulation of the enlarged liver. In the first case, sudden massive bleeding occurred as a result of laceration of the middle and left hepatic vein when the liver was dropped from the surgeon's hand. In both cases, the position of endotracheal tube became 2 cm shallower after surgery probably because of the shift in the position of the mediastinum after elimination of abdominal compression caused by the enlarged liver. One patient was discharged 39 days and the other 115 days after surgery. Anesthesiologists should pay special attention to the features reported here during LDLT for patients with APLD.     

Split-liver transplantation: an underused resource in liver transplantation

Split-liver transplantation is an efficient tool to increase the number of liver grafts available for transplantation. More than 15 years after its introduction only the classical splitting technique has reached broad application. Consequently children are benefiting most from this possibility. Full-right full-left splitting for two adult recipients has been shown to work but is hampered mainly by the dangers of small-for-size transplantation. A solution to this last problem would completely change the scope of split-liver transplantation. Organ allocation systems and collaboration between centers play a crucial role in the chances to let suitable patients profit from this valuable source of extra grafts.     

Renal ultrasound abnormalities in children with end-stage liver disease — reversal by liver transplantation

Ultrasound (US) imaging of the kidneys was performed in 43 pediatric patients with end-stage liver disease evaluated for orthotopic liver transplantation. Renal size was increased in 8 patients (19%) and echogenicity of the kidneys was increased in 12 patients (28%). In 12 patients studied after liver transplantation, US revealed normal renal size in all and increased echogenicity in only 2 patients shortly after transplantation. Normalization of renal US findings was also found in 2 patients studied before and after liver transplantation. The glomerular filtration rate varied from 65 to 225 ml/min per 1.73 m² in 5 patients with abnormal US, and from 74 to 116 ml/min per 1.73 m² in 3 patients with normal US. Nephromegaly and increased echogenicity on renal US are frequent in children with end-stage liver disease and appear to be mostly reversible by liver transplantation.     

活体肝移植治疗终末期肝病

目的 探讨活体肝移植(1iving donor liver transplantation,LDLT)供、受者术前评估和手术方式的选择.方法 回顾性分析1995年1月至2007年10月我中心95例LDLT患者的临床资料.良性终末期肝病92例,其中Wilson病45例;肝脏恶性肿瘤3例.结果 供肝切取不带肝中静脉右半肝31例,带肝中静脉右半肝3例,带肝中静脉左半肝51例,不带肝中静脉左半肝或左外叶10例.所有供者术后顺利恢复,均未出现严重并发症.受者随访1～86个月,良性终末期肝病受者1、3、5年累积生存率分别为89%(82例)、78%(71例)和73%(67例),其中Wilson病受者1、3、5年累积生存率分别为92%(42例)、89%(40例)和76%(34例).3例肝脏恶性肿瘤患者死亡2例,1例长期生存.供、受者铜代谢均恢复正常.结论 建立供者安全保障体系是LDLT开展的先决条件,选择合理的手术方式是提高受者生存率的关键.亲体肝移植是治疗Wilson病的有效手段.     

肝移植治疗终末期酒精性肝病

目的评价肝移植治疗终末期酒精性肝病(alcoholic liver disease,ALD)的可行性及疗效。方法回顾性地分析中山大学附属第三医院2003年12月至2007年4月进行的18例终末期ALD病人接受肝移植治疗后的生存情况及主要并发症发生率。结果18例ALD和229例非ALD良性终末期肝病病人肝移植术后1、2、3年存活率分别为88.9%、77.8%、77.8%和90.4%、84.0%和78.2%,两组间存活率的差异无统计学意义(P=0.778)。两组术后并发症的发生率分别为:肺部感染44.4%(8/18)和33.2%(76/229)(P=0.538),胆道并发症16.7%(3/18)和24.9%(57/229)(P=0.574),动脉并发症11.1%(2/18)和7.0%(16/229)(P=0.628),排斥反应11.1%(2/18)和6.6%(15/229)(P=0.357)。ALD组术后5.6%(1/18)恢复少量饮酒,没有病人重新出现酒精依赖。结论肝移植是治疗终末期酒精性肝病的有效手段,术后生存情况和非ALD良性终末期肝病接近。感染性并发症是ALD肝移植术后最主要的死亡原因,移植后应加强感染性并发症的监测和治疗。     

Anaesthesia for living-donor liver transplantation

In Japan, living-donor liver transplantation has long been the only solution for end-stage liver disease. During the past 10 years, 526 cases of living-donor liver transplantation have been performed at Kyoto University Hospital. This report reviews the authors experience of anaesthesia. The most important issue is the safety of the donor. As the percentage of adult recipients has increased, so has the age of donors and because risk for the donor increases with age, careful preoperative evaluation of the donor by the anaesthetist is needed. The principal differences between living-donor liver transplantation and cadaveric donor liver transplantation are surgical technique (the former is always a partial liver transplantation) and viability of the graft (should always be better with a living donor). The major problems concerning the intraoperative management of recipients in living-donor liver transplantation are how to deal with massive blood loss and postreperfusion syndrome.     

Bone mineral density and height gain in children with chronic cholestatic liver disease undergoing transplantation

BACKGROUND: Osteodystrophy is a well-described complication of chronic liver disease. Previous reports in adults and children undergoing liver transplantation (LT) were discordant, with the former showing no improvement of bone disease in the first year after transplantation and the latter demonstrating remarkable benefit from it. Our aim was to perform a pilot study on osteodystrophy in children undergoing LT and evaluate the contribution of growth on bone mineral density (BMD) changes. METHODS: We studied six patients (two male), with a median age at transplantation of 8.8 (range 3.8-16.6) years. Indications for transplantation were biliary atresia and progressive familial intrahepatic cholestasis (three patients each). BMD was studied with dual-energy x-ray absorptiometry and biochemical markers of liver and bone function in patients before and at 3, 6, and 12 months after LT. RESULTS: Median L2-L4 spinal BMD was 0.54 g/cm2 (range 0.29-0.87) before LT, and 0.58 g/cm2 (0.27-0.86) at 3 months, 0.66 g/cm2 (0.36-1.00) at 6 months, and 0.76 g/cm2 (0.44-1.02) at 12 months after LT (P=0.005). Median height was 133 (range 93-167) cm before LT, and 134 (93-167) at 3 months, 136 (97-167) at 6 months, and 139 (102-167) at 12 months after LT. There was direct correlation between height gain and total body BMD improvement (r=0.929, P=0.007). CONCLUSION: BMD in children with chronic cholestatic liver disease improves remarkably by 12 months after LT. Catch-up growth in children can account for the different effect of LT on bone density between adult and pediatric populations in the first year after surgery.     

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.     

The optimal timing of liver transplantation in patients with chronic cholestatic liver disease

The current opinion is that liver transplantation for chronic cholestatic liver disease should be done before the terminal, high-risk stage. However, most studies do not take waiting list mortality into account. We analysed 113 consecutive patients with chronic cholestatic liver disease, stratified according to estimated survival. Overall and post-transplantation survival was calculated using the Kaplan-Meier method. Including patients who died awaiting transplantation lowered the 1-year survival by 19% in the high-risk category. In this group survival at 4 years was 45%, with an estimated survival benefit of 45%. For the intermediate- and low-risk groups these numbers were 56% and 36% vs. 81% and 7%. Including the waiting list period in the analysis of the benefits of liver transplantation strengthens the case for early transplantation. Our study confirms that liver transplantation should be considered before the high-risk stage of chronic cholestatic liver disease is reached.