肝移植后并发桥脑中央髓鞘溶解症三例

目的总结肝移植后并发桥脑中央髓鞘溶解症(CPM)的诊治体会。方法回顾性分析3例肝移植后并发CPM的临床资料。结果例1于术后第18d出现定向障碍、四肢抽搐,随之昏迷,症状出现前后血电解质在正常范围;例2于术后第6d出现昏迷,有明显的低钠血症;例3于术后第7d出现意识模糊、被害妄想,随之昏迷,症状出现前后血电解质在正常范围。3例患者均经头颅磁共振检查诊断为CPM,虽经积极治疗,但最后均死亡。结论肝移植后并发CPM的原因是多方面的,其预后差,病死率高;磁共振是诊断CPM的首选方法。     

肝移植术后并发桥脑中央髓鞘溶解症三例

肝移植术后并发桥脑中央髓鞘溶解症(CPM)的发病率约为5%～10%,明显高于一般人群(0.16%～5.8%)[1,2].其发病原因复杂,治疗棘手,死亡率高[3].我院肝移植中心自2003年4月至2008年1月,共进行同种原位肝移植术115例次.其中3例患者术后并发CPM,发病率为2.6%(3/115).3例患者经治疗后,好转1例,死亡2例.现报道如下.     

肝移植术后桥脑中央髓鞘溶解症5例报告

探讨肝移植术后并发桥脑中央髓鞘溶解症(central pontine myelinolysis,CPM)的诊断和治疗.方法 回顾性分析5例肝移植后并发CPM的临床资料和治疗经过.结果 5例CPM患者均为男性,平均年龄49岁.原发病为慢性重症肝炎4例,原发性肝细胞癌1例,其中3例并发Ⅲ~Ⅳ度肝性脑病,3例并发严重肺部感染.CPM发生于术后4~13 d,平均发生时间为术后7 d,临床表现主要为闭锁综合征、肢体痉挛性瘫痪、缄默等.术前有明显低钠血症患者4例,发病时他克莫司血药浓度正常者4例,明显增高者1例.5例患者均经头颅磁共振成像检查诊断为CPM.采取病因治疗和对症处理相结合的治疗措施,重点防治并发症,其中两例由于早期合并水电解质、酸碱平衡紊乱,给予持续的肾脏替代治疗(continuous renal replacement therapy, CRRT).本组患者3例死亡,2例存活,但均存在神经系统损害.结论 肝移植后并发CPM的原因是多方面的,预后差,尚无有效的治疗方法,早期发现并积极防治并发症是降低病死率的主要措施.     

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。     

肝移植术后门静脉血栓形成的介入治疗(附1例报道)

患者,女,66岁,因原发性胆汁性肝硬变在我院行同种异体背驼式肝移植术.患者术前有上消化道出血、肝性脑病及2型糖尿病病史.术后给予普乐可复、阿司匹林治疗.术后第2个月复查血常规、血生化、凝血功能及腹部B超,未发现异常,后患者未按时至我院复查.肝移植术后4个月患者出现呕血伴乏力、纳差,至当地医院检查: HGB 56 g/L,红细胞压积(HCT)0.16,大便隐血阳性,肝功能正常,尿素氮47 mmol/L,肌酐184 μmol/L.入当地医院后再次呕血,出现意识模糊,呼气可闻及烂苹果味,血糖>29 mmol/L,考虑为上消化道出血合并糖尿病酮症酸中毒,次日转入我院.入院体检提示中度昏迷,心率114次/min,腹软,无压痛,神经系统检查无定位体征.     

胃肠肿瘤根治联合肝移植术治疗晚期胃肠肿瘤并肝脏广泛转移

目的 总结胃肠肿瘤根治联合肝移植治疗晚期胃肠肿瘤并发肝脏多发转移的近期疗效。方法 1例胃癌和2例直肠癌并发肝脏多发转移患者，分别接受胃癌和直肠癌根治、联合原位同种异体肝移植手术，其中1例因肺结核同时行左上肺部分切除术。结果 3例患者无围手术期死亡。随访5-7个月，胃癌患者术后5个月死于肿瘤复发；1例直肠癌并肺结核患者术后7个月死于肝功能衰竭，无肿瘤复发；另1例直肠癌患者已完成3个月化疗，术后半年无复发，肝功能和血常规正常，精神食欲好，已恢复工作。结论 胃肠肿瘤根治联合肝移植为部分晚期肿瘤患者提供了生存的希望，远期效果有待进一步观察。     

肝移植术后肝动脉血栓形成的治疗方法探讨

目的探讨肝移植术后肝动脉血栓形成的治疗方法。方法回顾性分析我院自2001年11月至2005年1月收治肝移植术后肝动脉血栓形成患者14例,分别采用动脉溶栓、血管支架植入、高压氧治疗及再次肝移植治疗。结果动脉溶栓11例,成功率45.5%(5/11),2例随访12个月肝功能正常,另3例均于随访期死亡,其中2例因吻合口狭窄曾行血管支架植入术。高压氧治疗3例,随访7个月,临床效果较好。急诊再次肝移植2例,术后均死于多器官功能衰竭。结论肝移植术后肝动脉血栓形成,动脉溶栓及血管支架植入治疗存在局限性,高压氧治疗效果良好,可与其他保守治疗联合使用,应避免急诊再次肝移植。     

公民逝世后器官捐献供肝保护及功能评估临床分析

目的总结公民逝世后器官捐献供肝保护及功能评估的初步经验。方法回顾性分析35例公民逝世后器官捐献供者和33例受者的临床资料。总结供肝获取情况和受者预后情况;根据供者器官获取前的血清钠离子水平(血清钠),将相应受者分为血清钠155 mmol/L组、血清钠155~160 mmol/L组和血清钠161~180 mmol/L组,比较3组受者术后早期移植肝功能不全的发生率。结果 35例供者中,中国标准二类27例,中国标准三类8例,实施肝移植33例,余2例肝脏有淤血性肝硬化改变,弃用。33例肝移植受者中,30例恢复顺利,术后7~14 d肝功能逐渐恢复正常,长期门诊随访移植肝功能基本正常;3例受者死亡,其中2例死于门静脉血栓形成,1例死于肺部感染合并多器官功能衰竭。血清钠155 mmol/L组、血清钠155~160 mmol/L组和血清钠161~180 mmol/L组受者术后早期移植肝功能不全的发生率依次为18%、23%和4/5,其中,血清钠161~180 mmol/L组明显高于血清钠155 mmol/L组(P0.05)。结论对公民逝世后器官捐献供肝功能进行及时、准确的评估和维护是提高供肝利用率、保证供肝功能和取得较好移植效果的关键因素。     

PTCD长期引流治疗肝移植术后缺血型胆道病变的初步探讨

目的 探讨经皮肝穿刺胆道造影引流术(percutaneous transhepatic cholangiography and drainage,PTCD)长期引流治疗肝移植术后缺血型胆道病变的可行性,评价其疗效和安全性.方法 11例肝移植术后并发缺血型胆道病变的病人,男10例,女1例,平均年龄42.3岁,术前均经PTC或内窥镜逆行胆胰管造影术(endoscopic retrograde cholangopancreatography,ERCP)检查确诊.病人首先经内科治疗及内镜下引流、支架置入治疗无效,然后采用经皮肝穿刺胆道置管并长期带管引流,合并有胆泥者经双导丝抽吸技术予以清除.结果 11例缺血型胆道病变病人.肝内型7例,肝外型1例,肝内+肝外型3例.均成功置入PTCD内外引流管,技术成功率100%.术后1周内总胆红素(TBIL)、直接胆红素(DBIL)分别由(206.70±54.18)μmol/L、(170.65±53.97)μmol/L降至(90.63±13.00)μmol/L、(63.83±13.61)μmol/L.随访3～71个月,平均20个月.黄疸指数较正常值稍高,并呈波动性改变,TBIL在23.70～241.0 μmol/L之间,平均(55.3±15.6)μmol/L,DBIL在8.1～162.0μmol/L之间,平均(32.53±10.21)μmol/L.9例病人移植肝功能良好,其中5例带管引流6～12个月(平均8.2个月)后拔除,4例仍带管已引流3～6个月.另2例病人黄疸症状缓解,但因移植肝合成功能障碍分别于PTCD术后3个月、8个月行再次肝移植.结论 PTCD置管长期引流是一种安全、有效的治疗肝移植术后缺血型胆道并发症的方法.     

肝脏移植术后桥脑中央髓鞘溶解症的病因探讨

目的　进一步总结肝脏移植术后桥脑中央髓鞘溶解症 (centralpontinemyelinolysis,CPM )的临床特征及发病原因。方法　回顾性研究 1999年 1月至 2 0 0 3年 5月 14 2例原位肝脏移植患者的临床资料 ,比较分析移植后有无中枢神经系统并发症及有无CPM者 ,围手术期血钠、血镁、血浆渗透压及环孢素A(CsA)浓度的变化和总手术时间。结果　 14 2例肝脏移植患者中 13例 (9 2 % )出现中枢并发症 ,其中CPM 5例 (3 5 % ) ,脑出血 /脑栓塞 8例 (5 6 % )。CPM患者中 ,移植前 2例血钠 <130mmol/L ,平均 (130 6± 5 5 4 )mmol/L ,与无中枢并发症和非CPM中枢并发症者比较显著降低 (P <0 0 5 )。CPM与非CPM中枢并发症及无中枢并发症患者移植前后 4 8h血钠的变化分别为(19 5± 6 5 )mmol/L ,(10 1± 6 4 )mmol/L ,(4 5± 4 3)mmol/L(P <0 0 1)。CPM患者术后血浆渗透压显著升高。所有患者肝脏移植前后均存在低镁血症 ,但无组间差异。并发CPM患者的总手术时间为 (492± 190 )min ,显著长于无中枢并发症患者 (P <0 0 5 ) ;术后所有移植患者的血CsA浓度在正常范围 ,但有中枢并发症高于无中枢并发症者 (P <0 0 5 )。结论　肝脏移植者是CPM的易患人群。CPM的发生可能与慢性低钠血症 ,围手术期血钠大幅波动 ,术后血浆渗透     

Pontine and extrapontine myelinolysis following liver transplantation. Relationship to serum sodium

The relationship between central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) and serum sodium changes in the setting of orthotopic liver transplantation (OLT) is examined. Postmortem examination of 14 patients with end-stage liver disease who underwent liver transplantation revealed CPM in four, of which three also had EPM. A retrospective review of clinical and laboratory data was performed on all patients. There were marked perioperative rises (21-32 mEq/L) in the serum sodium concentration in all four patients who developed myelinolysis. In contrast, the largest increase in sodium in patients without demyelination was 16 mEq/L. We conclude that perioperative rises in the serum sodium concentration increase the risk of myelinolysis. CPM and EPM should be considered if the patient develops mental status changes or focal neurological deficits several days after OLT.     

Central pontine and extrapontine myelinolysis: a rare and fatal complication after liver transplantation

Central pontine myelinolysis (CPM) may be more prevalent after liver transplantation (OLT). Central pontine and extrapontine myelinolysis (CPEM) is rare. The occurence of CPM may be associated with hyponatremia, a rapid rise in serum sodium concentrations, postoperatively increased plasma osmolality, and the duration of the operation. Only 1 patient had abnormal sodium levels before LT. No abnormalities were detected in immunosuppressive drug blood levels. The aim of this paper was to report our experience with CPEM among LT patients.     

Blood group Lewis alloantibodies cause antibody-mediated rejection in renal transplant recipients

Three patients with negative Lewis phenotypes who displayed anti-Lewis antibodies suffered severe kidney allograft dysfunction. One woman and two men (22-44 years) received ABO compatible kidney transplants with negative donor-recipient cross-match tests. Two patients had the phenotype Le(a-b-) with anti-Le(a) and anti-Le(b) complement binding antibodies. The third patient of phenotype Le(a+b-) developed anti-Lewis(b) antibody a few months after transplantation. One patient presented recurrence of worsened graft function from the day 6 to 4 months after transplantation; despite treatment there was not full recovery. The second patient had recurrences of acute graft dysfunction at 4 and 6 months after transplantation with nephrotic range proteinuria. The third patient showed progressive graft dysfunction at 7 months after transplantation. Biopsy specimens showed histological changes of antibody-mediated rejection. In the third patient, we observed fibrinoid necrosis and thrombosis of arterioles and glomerular capillaries. Immunofluorescence studies showed immunoglobulin IgG and IgM in glomerular capillaries and C4d and C3 on endothelial cells of peritubular capillaries. Posttransplantation cross-match tests with donor lymphocytes were negative. Anti-Lewis antibodies were observed during follow-up. All patients were treated with methylprednisolone boluses. In addition, one subject received antithymocyte globulin (ATG) and 1 received plasmapheresis. Two patients had moderate renal dysfunction (creatinine levels 1.8 and 1.9 mg/dL) after 8-17 months follow-up. The third patient lost her graft at 11 months after transplantation. Lewis antibodies may injure a renal allograft. C4d deposition and failure to show donor-specific anti-HLA antibodies suggested the participation of other antibodies.     

Clinicopathologic findings of recurrent primary sclerosing cholangitis after orthotopic liver transplantation

Whether primary sclerosing cholangitis (PSC) occurs after orthotopic liver transplantation is controversial, largely because the pre-transplant diagnosis of PSC is based on nonspecific radiological and histological findings. We reviewed clinical, radiological, and histological records of 53 patients who underwent liver transplantation for PSC between 1985 and 1998. Three patients with patent hepatic arteries and no evidence of chronic rejection had radiological and histological findings that may have been due to recurrent PSC. Bile duct stricturing in these patients proved permanent and progressive and affected both the quality of life and graft survival. The first patient, who is 110 months after transplantation, has had repeated episodes of cholangitis for the last year. The second patient underwent excision of a strictured hepatic duct 45 months after transplantation and was ultimately retransplanted 95 months after initial transplantation. The third patient underwent left hemihepatectomy of an atrophied lobe 50 months after transplantation. Although the patient population assessed in this study is limited, putative recurrent PSC in the allografts has led either to graft loss or to clinically significant hepatobiliary complications of the graft. Received for publication on March 8, 1999; accepted on April 30, 1999     

Experience With the Use of Sirolimus in Liver Transplantation—Use in Patients for Whom Calcineurin Inhibitors Are Contraindicated

Sirolimus (SRL) provides effective immunosuppression for kidney transplantation and may be useful in patients with delayed allograft function after kidney transplantation. We review our experience with SRL in liver transplant recipients for whom calcineurin inhibitors are undesirable. Fourteen patients with renal insufficiency or acute mental status impairment were administered SRL after liver transplantation (5- to 10-mg load, 1 to 4 mg/d). Immunosuppression also consisted of mycophenolate mofetil and corticosteroids. On resolution of neurological or renal dysfunction (return to baseline mental status or serum creatinine level), tacrolimus (TAC) therapy was initiated. Twelve patients received primary transplants, 1 patient received a combined liver-kidney transplant, and 1 patient received a third transplant. Follow-up was 2 to 7 months. Calcineurin inhibitors were initially withheld in 9 patients, and therapy was aborted because of toxicity in the remaining 5 patients. Mean times to the initiation of SRL and TAC therapy were 5.4 ± 4.6 and 26.8 ± 24.4 days, respectively. Serum trough levels of SRL did not correlate with dose or other patient variables. Two patients died after prolonged pretransplantation hospital courses in the intensive care unit. Six patients experienced acute rejection, but only 1 patient required antilymphocyte therapy. Serum creatinine levels at the start of SRL therapy were 2.2 ± 1.1 and 1.2 ± 0.6 mg/dL at 3 months. All 3 patients with neurological indications for SRL had a return to their baseline mental status. All patients had improved liver function chemistry test results and prothrombin times. No patients developed leukopenia or thrombocytopenia. SRL is safe after liver transplantation in patients with acute neurological or renal impairment. SRL is an attractive alternative when calcineurin inhibitors are undesirable, but serum trough levels of SRL should be monitored. A prospective randomized study of an SRL-based calcineurin inhibitor–avoiding regimen compared with standard therapy in patients with renal insufficiency will further evaluate the role for SRL in liver transplantation. (Liver Transpl 2000;6:734-740.)     

Bile duct carcinoma following abdominal radiation for urologic neoplasms

This report concerns three cases of bile duct carcinoma which occurred 15–40 years after patients received treatment for urogenital neoplasia. All three patients had initially received radiation therapy in addition to urological surgery. Jaundice was the major clinical symptom of the second tumor. Two of the three patients were able to be surgically treated when presenting with their second carcinoma (partial pancreaticoduodenectomy, bile duct resection; central liver resection), whereas the third patient was in such a poor state of health that he was biopsied only and treated with a pigtail drain. All three patients died within 3 months to 3 years following the diagnosis of the second tumor. The more than coincidential occurrence of the rare bile duct carcinoma in our three patients with previous urogenital neoplasia warrants a new discussion concerning the connection between primary carcinoma, radiation therapy, and the occurrence of a second tumor.     

Sodium-reduced continuous venovenous hemodiafiltration (CVVHDF) for the prevention of central pontine myelinolysis (CPM) in hyponatremic patients scheduled for orthotopic liver transplantation

Two patients in end-stage hepatic failure presented for orthotopic liver transplantation with longstanding severe hyponatremia (121 and 122 mmol/L). Both patients underwent liver transplantation with the concomitant use of continuous venovenous hemodiafiltration. Replacement and dialysate solutions were prepared individually to contain a sodium level that was individually considered safe with regard to the development of central pontine myelinolysis. The sodium increase in both patients was within the expected and planned limits despite a situation of mass transfusion. Both patients did well postoperatively and neither patient suffered neurological deficits.     

Outcome of lamivudine resistant hepatitis B virus mutant post-liver transplantation on lamivudine monoprophylaxis

BACKGROUND: We aimed to investigate the clinical outcome of patients who develop lamivudine resistant hepatitis B virus mutants (YMDD mutants) after liver transplantation. METHODS: Patients who received liver transplantation for hepatitis B-related liver diseases from 1999 to 2002 were studied. All patients received lamivudine monotherapy before and after liver transplantation. HBsAg and HBV DNA were regularly monitored, and YMDD mutation was detected by direct sequencing. RESULTS: Twenty patients were followed up for median 94 wk (range: 15-177 wk) post-liver transplantation. Six patients developed YMDD mutants, and the cumulative probability of developing YMDD mutations post-liver transplantation was 21% in 1 yr and 34% in 2 yr. One patient developed YMDD mutants before liver transplantation and died of hepatitis reactivation and liver failure 15 wk post-transplantation. The other five patients developed YMDD mutants 32-72 wk after liver transplantation. Two of them developed severe hepatitis which responded promptly to adefovir dipivoxil. The remaining three patients with YMDD mutants had minimal to mild hepatitis. The cumulative survival for patients with YMDD mutants was 83% and 28% at 1 and 2 yr, respectively. Only one patient who did not develop YMDD mutants died at week 119 due to chronic rejection. The post-transplant survival for patients with YMDD mutants was significantly poorer than those without YMDD mutants (log rank test p = 0.083). CONCLUSIONS: The emergence of YMDD mutants after liver transplantation on lamivudine monoprophylaxis had wide range of clinical presentations and was associated with increased mortality.     

肝移植术后精神并发症的早期诊断与治疗

目的探讨肝移植术后精神并发症的早期诊断和治疗。方法回顾性分析24例原位肝移植患者的临床资料,分析肝移植术后精神并发症的发生原因和诊断,总结治疗经验。结果15例患者于术后1周内出现不同程度的精神并发症,其中5例症状较为明显。临床表现为亢奋、蹂狂、抑郁、焦虑、睡眠障碍、震颤及认知改变等。术前有无肝性脑病、术后感染及使用免疫抑制剂与术后精神并发症的发生有关。结论肝移植术后精神并发症的发生率较高。病因与多种因素有关,临床表现形式多样。需排除脑桥中央髓鞘溶解症。针对不同病因和及时处理,可有效改善与控制症状。