Present status and prospects of living-related liver transplantation

Living-related liver transplantation (LRLT) is a relatively new surgical modality that has developed, in part, to overcome the shortage of available cadaveric livers for transplantation and as a method to provide liver graft implants from living donors for patients end-stage with liver disease in areas where the use of cadaveric livers is not yet practiced or permitted. Since 1988 almost 500 LRLTs have been performed globally. The safety of donors who provide a portion of their liver for grafting is of utmost concern, and only one donor death from this procedure has been reported in the literature. Postoperative survival in recipients depends on their pretransplant physical status, but emergency patients in rapid need of a liver have a poorer survival than elective LRLT patients for whom survival is about 80%. Children and infants are the main recipients of LRLTs, but adult patients particularly in Japan, are increasing in number, and present indications for LRLT surgery include not only cholestatic end-stage liver diseases but also metabolic disorders affecting the liver and emergency LRLTs for fulminant hepatic failure. Many ethical problems relating to the concept of liver transplantation, donor liver source, recipient selection, and reimplantation have yet to be resolved. But we believe that LRLTs and cadaveric liver transplantations are saving lives and that the practice should be continued.     

Homozygosity for human leucocyte antigen-C ligands of KIR2DL1 is associated with increased risk of relapse after human leucocyte antigen-C-matched unrelated donor haematopoietic stem cell transplantation

Human leucocyte antigen (HLA)-C molecules regulate the function of natural killer cells and may be subdivided into two groups, C(1) and C(2), based on their specificity for inhibitory killer immunoglobulin-like receptors. We analysed the impact of the HLA-C genotype on outcome of HLA-C-matched unrelated donor haematopoietic stem cell transplantation (URD-HSCT) recipients. HLA-C(2) homozygous patients (n = 18) had lower probability of overall survival (P = 0.01) and disease-free survival (P = 0.02), resulting from increased relapse rate (P = 0.02) when compared with both HLA-C(1) homozygous (n = 43) and HLA-C(1),C(2) heterozygous (n = 50) subgroups. Patients lacking HLA-C(1) should, therefore, be considered at increased risk of relapse following HLA-C-matched URD-HSCT.     

Compressive stenosis of the inferior vena cava due to localized ascites after living-donor liver transplantation

A 54-year-old woman was admitted to our hospital following the diagnosis of decompensated liver cirrhosis with hepatitis C. She underwent living-donor liver transplantation, performed using the left hepatic lobe with the middle hepatic vein donated by her husband. After the transplantation, the patient suffered from massive ascites with liver dysfunction. Computed tomography demonstrated stenosis of the suprahepatic inferior vena cava (IVC) with focal collection of fluid. A second laparotomy was performed 19 days after the transplantation. When the encapsulated localized ascites on both sides of the IVC was opened, the ascites was flushed away. Subsequently, the grafted liver was easily mobilized and it was placed in the natural position without any tension, and the pressure gradient of the IVC was improved. Herein, we report a very rare case of compression stenosis of the IVC resulting in Budd-Chiari syndrome caused by localized encapsulated ascites.     

Wash‐out of the non‐heart‐beating donor liver: a matter of flush solution and temperature?

BACKGROUND AND AIMS: Ischemically damaged donor livers are prone to graft non-function. This can in part be explained by a suboptimal wash-out during procurement. An enriched machine perfusion (MP) preservation solution for livers, named Polysol, was developed. The aim of this study was to investigate the type of flush solution, temperature and anticoagulant content on the wash-out of the non-heart-beating donor (NHBD) rat liver. METHODS: Rat livers were flushed after 30 min warm ischemia. After excision, livers were reperfused at 37 degrees C, with analysis of damage and function, concerning (1) solutions (University of Wisconsin (UW), histidine-tryptophan-ketoglutarate (HTK) and Polysol); (2) temperature (4 degrees C, 18 degrees C and 37 degrees C); (3) addition of heparin and (4) wash-out followed by 24 h MP. RESULTS: (1) Reperfusion results were inferior in the UW group; (2) less damage and improved function were seen after wash-out using Polysol at 37 degrees C; (3) No effects were seen of the addition of heparin to Polysol; (4) MP after wash-out using HTK resulted in more liver damage and decreased liver function as compared with wash-out using Polysol. CONCLUSIONS: Polysol is applicable as a flush solution for the NHBD liver, resulting in equal to better wash-out as compared with UW and HTK. The best temperature for this NHBD wash-out is 37 degrees C.     

双胎输血综合征宫内治疗后供血胎和受血胎预后的比较

[目的]评价双胎输血综合征(TTTS)经不同治疗方式,包括羊水减量术(SA)、双极脐带电凝减胎术(BCC)和胎盘吻合血管激光凝固术(LCPV)后,供血胎和受血胎的临床结局.[方法]回顾性分析2008年1月至2013年12月在我院诊断治疗的双胎输血综合征93例,追踪妊娠结局和新生儿情况,分析比较供血胎和受血胎的临床结局.[结果]不同治疗方式组中严重的TTTS(TTTSⅢ期及以上)所占比例分别是:羊水减量术组50.0％(27/54),脐带电凝减胎术组93.9％(31/33),胎盘吻合血管激光凝固术组83.3％(5/6),P＜0.001,因此,3种治疗方式妊娠结局比较时仅纳入TTTSⅢ期及以上病例.SA组、BCC组和LCPV组双胎总体存活率分别是53.7％、24.2％和60.0％,P=0.002.SA组、BCC组和LCPV组分娩孕周中位数分别是31e(20+3～38+2)、28+5(20+2～38+6)和27+6((23+3～37+4)周,其差异无统计学意义(P=0.204).SA组胎膜早破的发生率是29.6％而BCC组和LCPV组胎膜早破的发生率分别是32.3％和60.0％,P=0.410.LCPV组28周前分娩的比例较SA组和BCC组高(分别是60.0％、18.5％和48.4％;P=0.033).TTTS双胎中受血胎存活率是44.1％(41/93)而供血胎存活率是46.2％(43/93),P=0.768;受血胎神经系统发育迟缓发生率是2.4％(1/41)而供血胎神经系统发育迟缓发生率是11.6％(5/43),其差异无统计学意义(P=0.202).[结论]胎盘吻合血管激光凝固术可提高TTTS的总体存活率;TTTS受血胎和供血胎的预后无明显差异.     

Health‐related quality of life and sleep among Chinese children after living donor liver transplantation

LDLT is a well‐established treatment for most terminal liver diseases in children. Survival rates have improved, yet few studies have considered HRQoL or sleep problems in LDLT recipients. In this cross‐sectional study, we enrolled 51 children who had undergone LDLT in Renji Hospital. PedsQL^? 4.0 Generic Core Scales, PedsQL^? 3.0 Transplant Module, and Pediatric Sleep Questionnaire were used to assess outcomes. Of all participants, 11.8% (6/51) reported low total HRQoL scores. Participants’ scores on most HRQoL subscales were comparable to the scores of healthy children. However, compared with solid organ transplant recipients, LDLT recipients scored significantly lower in About My Medicines II (t = 3.092, p = 0.002) and Worry (t = 2.760, p = 0.006). Sleep problems (41.2%) were common among participants. Hierarchical regression analyses showed that SRBD accounted for significant variance in HRQoL on total generic HRQoL (R² = 0.446, p < 0.001), psychosocial health (R² = 0.372, p = 0.001), physical health (R² = 0.345, p = 0.003), total transplant‐specific HRQoL (R² = 0.514, p < 0.001), About My Medicines I (R² = 0.365, p = 0.013), My Transplant and Others (R² = 0.334, p = 0.005), Pain and Hurt (R² = 0.544, p < 0.001), Worry (R² = 0.401, p = 0.001), Treatment Anxiety (R² = 0.526, p < 0.001), How I Look (R² = 0.221, p = 0.040), and Communication (R² = 0.343, p = 0.012). In conclusion, sleep problems are non‐negligible in children after LDLT and predicted significant variance on HRQoL.     

Technique advance to avoid hepatic venous outflow obstruction in pediatric living‐donor liver transplantation

HVOO represents a serious critical complication of pediatric living‐donor liver transplantation because open surgical repair is virtually impossible. Currently, despite several technical innovations and the introduction of triangulated anastomosis for hepatic vein reconstruction, the reported incidence of HVOO is still considerable. The aim of this study was to propose a new technique for hepatic venous reconstruction that avoids the original orifice of the recipient hepatic veins. Instead, anastomosis is performed in a newly created wide longitudinal orifice in the anterior wall of the recipient inferior vena cava. A total of 210 living related‐donor liver transplantations were performed using two methods for reconstruction of the hepatic vein. Group 1 included 69 patients subjected to direct anastomosis of the orifice of the graft hepatic vein and a wide orifice created in the recipient inferior vena cava by the confluence of the orifices of the right, left, and middle hepatic veins. Group 2 included 141 patients in whom the original orifices of the recipient hepatic veins were closed, the inferior vena cava was widely opened, and a long longitudinal anastomosis was performed using two lines of continuous sutures. Diagnosis of HVOO was suspected based on clinical findings and ultrasound studies and then confirmed by liver biopsy and interventional radiology examinations. Among the 69 recipients in group 1, 16 patients died due to graft problems during the postoperative period and eight of the survivors (15.1%) presented with HVOO. In group 2 (141 patients), 21 patients died, and there were no cases of HVOO. A comparison of the incidence of HVOO between groups revealed a significant difference (p = 0.01). Hepatic venous reconstruction during pediatric living‐donor liver transplantation should be performed using a wide longitudinal incision in the anterior wall of the recipient inferior vena cava because this technique eliminated anastomosis complications.     

First living‐related liver transplant to cure factor VII deficiency

Congenital factor VII deficiency is an autosomal recessive serious disorder of blood coagulation with wide genotypic and phenotypic variations. The clinical presentation can vary from asymptomatic patients to patients with major bleedings in severe deficiency (factor VII <1%). Investigations show prolonged PT and low factor VII. Treatment modalities include FFP and repeated recombinant factor VII infusions. We hereby report the first successful LRLT for factor VII deficiency in an infant, the first‐ever youngest baby reported worldwide. A six‐month‐old male child presented with easy bruisability, ecchymotic patches, hematuria, and convulsions. CT of the head showed subdural hemorrhage, which was treated conservatively. He had markedly increased PT (120 s) with normal platelets, and aPTT with factor VII level <1%. Despite the treatment by rFVIIa administration weekly, which was very expensive, he still had repeated life‐threatening bleeding episodes. LRLT was performed with mother as the donor, whose factor VII level was 57%. A factor VII infusion plan for pre‐, intra‐ and postoperative periods was formulated and TEG followed. Postoperatively, his factor VII started increasing from third day and was 38% on 24th day with PT <14 s. He had uneventful intraoperative and postoperative courses. LT is a safe and definite cure for factor VII deficiency.