Living-related liver transplantation for Wilson''s disease

Orthotopic liver transplantation has been applied to the treatment of Wilson's disease (WD), living-related liver transplantation (LRLT) has also been indicated for WD with increasing frequency. Between January 2001 and November 2003, 22 LRLTs were performed on patients (19 pediatric, three adults) with WD in liver transplantation center. Two patients were transplanted because of a presentation coexistent with fulminant hepatic failure. Twenty presented with chronic advanced liver disease with (n = 9) or without (n = 11) associated neurologic manifestations. All the recipients had low serum ceruloplasmin levels with a mean value of 12.8 +/- 3.2 mg/dl before transplantation and increased to an average of 26.0 +/- 3.6 mg/dl after LRLT at the latest evaluation. The survival patients with neurologic manifestations such as tremor, dysarthia, dysphagia, dystonia and sialorrhea had improved after LRLT. This suggests that LRLT not only resolves the hepatic but also ameliorates the neurologic consequences of WD.     

Living-related liver transplantation for Alagille syndrome

Alagille syndrome (AGS) is an autosomal dominant genetic disorder characterized by chronic cholestasis, congenital heart disease, peculiar facies, butterfly-like vertebrae, and posterior embryotoxon. Liver dysfunction is the common presentation of AGS, and liver transplantation may be indicated. This study examines the outcome of living-related liver transplantation (LRLT) for AGS. Twenty patients with AGS (median age 5.0 years, range 0.6-12.9) underwent LRLT at Kyoto University Hospital between June 1990 and February 2002. Five potential donors were excluded because of paucity of intrahepatic bile ducts diagnosed by preoperative liver biopsy and one because of a hepatic vascular anomaly. The overall 5-year patient survival was 80.4%. Three patients died as the result of the following: complications related to surgery, heart failure caused by progressive pulmonary artery stenosis, and a graft with unsuspected bile duct paucity. Liver dysfunction was improved in all successful cases, and catch-up growth occurred in 90% of patients. LRLT is an efficacious treatment modality for AGS if donors are selected by cautious evaluation to rule out unsuspected bile duct paucity.     

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.     

Living-related liver transplantation for primary biliary cirrhosis

BACKGROUND: The prognosis of patients with primary biliary cirrhosis has improved since the introduction of transplantation. However, there has been limited experience with living-related liver transplantation for primary biliary cirrhosis. PATIENTS AND METHODS: Between January 1996 and October 2000, 105 patients underwent living-related liver transplantation at the University of Tokyo Hospital. Eighteen of these patients with primary biliary cirrhosis were the subjects of this study. The risk scores in the conventional and updated Mayo models ranged from 5.9 to 11.6 and 6.9 to 13.7, respectively. RESULTS: The graft weight ranged from 330 to 533 g, corresponding to 33 to 55% of the recipient's standard liver volume. Two patients died of pneumonia, 1 died due to sepsis, and the remaining 15 patients survived. The risk scores for the conventional and updated Mayo models correlated well with the duration of hospitalization (P=0.01, R=0.67 for both). The scores of the dead patients by the two models were more than 10 and 12, respectively. The postoperative courses of all of the donors were uneventful. CONCLUSIONS: The risk score in the Mayo model adequately reflected the outcome of the patients. To achieve the best possible outcome, further therapeutic tactics are necessary for patients with conventional or updated Mayo risk scores of more than 10 or 12, respectively.     

Living-related liver transplantation for Crigler-Najjar syndrome in Saudi Arabia

OBJECTIVE: To analyse the outcome of six children with Crigler-Najjar syndrome type I (CNS-I) and report the first three living-related liver transplants for this syndrome in Saudi Arabia and the Middle East. SETTINGS: To review the medical records of six children suffering from CNS-I, three of whom underwent living-related liver transplantation (LRLT) between 22 November 1998 and January 2001. MAIN RESULTS: Living-related liver transplantation was performed in three children with a pre-transplant unconjugated bilirubin level of 362, 381 and 502 micromol/L, respectively, despite daily phototherapy of >or= 12 h. Two of the transplanted children developed acute hepatocellular rejection, which was successfully treated with methylprednisolone pulse therapy. One tested cytomegalovirus positive (using the PP65 method), but showed no signs of clinical infection and was treated with ganciclovir. One patient had a biliary leak at the cut surface of the graft which was surgically repaired. Post-operative bilirubin levels returned to normal in all three transplanted children and no further phototherapy was required. One patient, who was not transplanted but received phototherapy, developed severe neurological damage prior to the start of our living-related liver transplant programme with a bilirubin level of 450 micromol/L, her sister is still awaiting transplantation. A 14-yr-old child with a bilirubin level of 420 micromol/L is presently undergoing phototherapy whilst awaiting orthotopic liver transplantation because of the lack of a suitable living-related donor. Six siblings of the six children in our series were reported dead by the families. CONCLUSION: Crigler-Najjar syndrome type I is a relatively common disease in Saudi Arabia for which LRLT is a curative treatment when performed at an early age before the development of kernicterus and neurological deficiency. In countries where there is a severe shortage of cadaveric organs, as is the case in Saudi Arabia, LRLT is the optimum treatment modality for this syndrome.     

Living-related liver transplantation in a patient with hepatopulmonary syndrome

We experienced the perioperative management of the living related liver transplantation (LRLT) in a patient with hepatopulmonary syndrome (HPS). HPS is seen in 15% of patients of the endstage liver failure, and it accompanies the various types of hypoxia. The diagnostic standards of HPS are chronic liver disease usually complicated by portal hypertension with or without cirrhosis, arterial hypoxemia (PaO2 < 70 mmHg or A-aDO2 gradient > 20 mmHg), and intrapulmonary vascular dilation. The present case conformed to the diagnostic standard. But this case was of a mild type of HPS, because PaO2 was elevated after O2 inhalation and extrapulmonary uptake of 99mTcMAA after lung perfusion was lower than 40%. During perioperative period of LRLT, there were no complications such as hypoxia, acute rejection, bleeding and infection. Therefore HPS would be improved after LRLT. In the management of perioperative period it is important to be aware of hypoxia and to evaluate preoperatively the condition of the patient properly.     

亲体部分肝移植治疗Wilson病20例报告

目的探讨亲体部分肝移植治疗肝豆状核变性病 (Wilson病 )的价值。方法 2 0 0 1年 1月至 2 0 0 3年 10月 ,我院连续为 2 0例Wilson病患者成功施行亲体部分肝移植术 ,男性 8例 ,女性 12例 ,年龄 7～ 2 0岁 ,平均 11 1岁。 3例是暴发性肝功能衰竭 ,17例慢性进行性肝损害。供肝者为患者父亲或母亲。结果供受体手术顺利 ,术后 1个月肝功能和铜蓝蛋白恢复正常水平。 19例病人健康存活 ,1例术后 72d死于排斥反应。术后并发症包括 :醒状昏迷 1例 ,肝动脉血栓形成 1例 ,创面胆漏 1例 ,平均随访 18 9个月。结论亲体部分肝移植是治疗Wilson病并发肝功能衰竭的有效疗法。     

Vascular complications after liver transplantation in pediatric patients

Vascular complications are the major cause of morbidity and mortality after liver transplantation, particularly in pediatric patients, owing to their smaller vascular diameters. Between September 2001 and June 2004, among 21 (16 boys and 5 girls) pediatric liver transplantations of mean age 8.3 +/- 5.1 years, hepatic arterial thrombosis (HAT) was diagnosed in 2 (9.5%) patients, and hepatic arterial stenosis (HAS) in 4 (19.4%). Vascular patency was evaluated with Doppler ultrasonography every 12 hours in the first postoperative week and daily in the second postoperative week. When occlusion was suspected, conventional angiography was performed. Thrombectomy was performed in one patient, and thrombectomy and reanastomosis were performed in another patient with HAT. Two patients with HAS were treated with balloon angioplasty. A third patient was treated with balloon angioplasty and endoluminal stent placement at the same time. The last patient with HAS had an intimate dissection, which occurred 24 hours after balloon angioplasty, that was treated with subsequent endoluminal stent placement. Mean follow-up for the patients with vascular complications was 9.5 +/- 5.7 months (range, 4 to 18 months). The overall mortality rate was 14.1% (3/21); however, no deaths were caused by vascular complication. Routine Doppler ultrasonographic evaluation is an effective choice for diagnosing vascular complications seen after liver transplantation. Immediate surgical intervention is required for acute vascular complications, whereas late complications may be treated with balloon angioplasty and/or endoluminal stent placement.     

Living-related liver transplantation: a Thai family's stressors and concerns

This case study reviews the experience of a Thai family who traveled to Japan for living-related liver transplantation. The recipient experienced multiple problems after transplantation, which placed additional stress on the family. The cultural gap and language barrier resulted in a lack of understanding and often made it difficult for the medical team and family to communicate effectively. However, the use of interpreters helped to resolve these problems. This study shows that it is especially important for transplant coordinators and social workers to facilitate the collaboration and understanding between families and the medical team when cultural and language differences exist.     

亲缘性活体部分小肠移植术

目的 介绍我国首例亲缘性活体部分小肠移植术的临床处理体会。方法 受体为男性,18岁,因短肠综合征而接受小肠移植。供体,男性,44岁,为受体之父。取供体回肠末段150cm,移植给患者,术后给予抗免疫排斥、抗感染、抗凝及营养支持等治疗。结果 目前,患者已健康生存19个月,移植肠功能恢复良好。结论 亲缘性活体部分小肠移植术是治疗短肠综合征的有效手段,良好的术后管理是确保活体小肠移植手术成功的关键。     

辅助性原位活体肝部分移植一例报告

目的：探讨应用辅助性原位活体肝部分移植治疗Willson's病的可行性。方法：受体女性，20岁，O型血，因肝豆状核变性而接受辅助性原位活体肝部分移植。供体男性，21岁，A型血。手术切除受体病肝左外叶260g，取供体左外叶肝脏295g原位移植于受体。因供受体血型不同，术前行血浆置换，术后以FK506，激素，环磷酰胺联合免疫抑制治疗。结果；受体术后15d出现肝动脉血塞形成，予以溶栓治疗后出现腹腔内出血，术后17d开腹止血，清除血肿，术后发生腹水，肺不张，胆瘘等，均治愈。至今患者已生存1年3个月，并恢复正常生活，铜蓝蛋白水平正常。移植肝脏体系明显增大，手足震颤明显减轻。结论：辅助性原位活体肝部分移植是治疗Willson's病可行的方法。