移植物体重比对左外叶小儿活体肝移植术后短期肝功能的影响

目的 探讨移植物体重比(Graft recipient body weight ratio,GRBW)对小儿活体左外叶肝移植术后短期肝功能恢复的影响.方法 2006年6月至2009年3月共施行活体肝移植25例,其中17例左外叶活体肝移植患儿作为研究对象.分别于术后1～7 d测量患儿肝脏功能,采用SPSS15.0统计软件分析了解GRBW与术后短期肝功能恢复的相关性.结果 ①肝移植术后肝功能恢复良好(P<0.000 1);②GRBW与术后谷丙转氨酶(ALT)变化呈正相关(P<0.05)、与AST变化呈负相关(P<0.05).结论 左外叶小儿活体肝移植GRBW对于术后肝功能恢复意义重大,移植术中应充分保证足够的GRBW比值.     

Renal function following liver transplantation in children

The literature regarding the etiology and incidence of short and long-term renal functional impairment in pediatric liver allograft recipients was reviewed. Most of the reports include recipients receiving cyclosporine as the primary immunosuppressant. Using calculated glomerular filtration rate (cGFR), creatinine clearance or the serum creatinine level will lead to an overestimation of GFR. In contrast to data in adults, there are a limited number of pediatric recipients whose renal dysfunction has progressed to chronic kidney disease or end-state renal disease. Calcineurin inhibitors minimization has proven effective in reversing or preventing progressive deterioration of GFR; however, rejection episodes and complications have limited efficacy of this approach. Future multicenter studies using optimal GFR measurements are required to delineate the magnitude of renal dysfunction in pediatric recipients.     

婴幼儿亲体部分肝移植

目的回顾性分析我院2002年10月-2005年10月6例婴幼儿亲体部分肝移植临床疗效。方法婴幼儿亲体部分肝移植6例，年龄9个月～3岁，男3例，女3例；手术时体重为7～16kg。胆道闭锁Kasai术后肝硬化腹水4例，先天性肝内胆汁淤积症、肝硬化腹水1例，药物性肝炎1例。随访时间为6～36个月。结果供肝提供者分别为母2例，父4例，提供左外叶肝，平均住院7d，无并发症。5例生存，最长36个月。1例因凝血功能障碍，术后24h肾功能衰竭、肺出血死亡。术后免疫治疗药物包括甲基强的松龙、FK506和赛尼派。术后并发症为2例胆瘘，术后出血、再次进腹止血1例，肺部感染1例。结论婴儿期进行部分活体肝移植，扩大了供体来源，临床疗效较好，是婴儿终末期肝病有效的治疗方法。     

Renal function following liver transplantation for unresectable hepatoblastoma

Combination of cyclosporine (CsA) and tacrolimus immunosuppression post-liver transplantation (LT) and the chemotherapeutic drugs used to treat hepatoblastoma (HB), are nephrotoxic. We aimed to determine the severity and duration of nephrotoxicity in children following LT for unresectable HB. We reviewed all children undergoing LT for unresectable HB at the Liver Unit, Birmingham Children's Hospital, UK, from 1991 to July 2000. Thirty-six children undergoing LT for biliary atresia, matched for age and sex, were selected as controls to compare pre- and post-LT renal function. Renal function was determined by estimation of glomerular filtration rate (eGFR) derived from plasma creatinine using Schwartz's formula. Twelve children with HB (mean age of diagnosis 33 months) who underwent LT (mean age 47 months) and 36 controls (mean age of LT 34 months) were studied. CsA was the main immunosuppressive drug used in each group. The median eGFR before, and at 3, 6, 12, 24 and 36 months after LT in HB group was significantly lower than controls (93 vs. 152, 66 vs. 79, 62 vs. 86, 66 vs. 87, 64 vs. 94, 53 vs. 90 mL/min/1.73 m2, respectively; 0.01 < p < 0.03). The reductions in the median eGFR of both the HB group and controls before and at 36 months after LT were 49 and 41%, respectively. At 36 months after LT, there was a trend for partial recovery of the eGFR in the controls but not in the HB group. Children who underwent LT for unresectable HB had renal dysfunction before transplantation that persisted for 36 months after LT.     

First two decades of paediatric heart transplantation in Sweden - outcome of listing and post-transplant results

Aims: To evaluate outcome in the first generation of children with end‐stage heart disease to whom heart transplantation was available. Methods: Retrospective review of all 135 Swedish children <18 years old listed for heart transplantation 1989–2009, followed to December 31, 2009, including 74 (55%) with cardiomyopathy and 61 (45%) with congenital heart disease; 34 (25%) were infants (<1 year). Cumulative risk of requiring heart transplantation was 1:17 300 (11 patients who improved were omitted from outcome analysis). Results: Waiting‐list mortality was 31% (44% in infants). Median waiting time in 82 transplanted patients was 57 days (0–585 days). Post‐transplant follow‐up time was median 5.9 years (0.03–20.1 years), and actuarial survival was 92% at 1 year, 82% at 5 years, 76% at 10 years and 58% at 15 years. Survival after listing was 64% at 1 year, 58% at 5 years, 52% at 10 years and 40% at 15 years. Post‐transplant complications included rejections (34%), malignancies (12%), renal failure (8%), coronary artery vasculopathy (6%) and re‐transplantation (5%). Among 64 survivors, 84% were free of complications affecting prognosis. Conclusion: High waiting‐list mortality and post‐transplant attrition precluded 60% of this pioneer population from reaching adulthood. Functional status in survivors is generally good.     

儿童多米诺肝移植初步报告

目的探讨儿童多米诺肝移植的可行性和安全性。方法将一成人左外叶移植给家族性高胆固醇血症3岁男性患儿，同时采用多米诺肝移植技术将该患儿的肝脏移植给先天性胆道闭锁4个月女性患儿。结果家族性高胆固醇血症患儿的血清总胆固醇及低密度脂蛋白于术后第三天降至正常范围，手术后第8天死于心力衰竭。先天性胆道闭锁患儿随访16个月，肝肾功能化验正常，血清总胆固醇及低密度脂蛋白分别为8．87mmol／L和6．21mmol／L，明显低于多米诺供体患儿的术前水平。结论尽管术后多米诺受者出现获得性高胆固醇血症，但FHC可以作为多米诺供肝，近期效果满意，远期效果需进一步观察。     

小儿肝移植近期并发症防治

目的探讨小儿肝移植术后管理经验以及近期并发症的防治。方法2001年11月至2003年12月行小儿肝移植7人8例次，其中亲体肝移植2例，减体积肝移植3例，劈离式肝移植2例。术后即送至ICU监护并监测重要脏器功能、凝血功能及生化指标，早期用免疫抑制剂和预防性应用抗生素，每日Doppler检查肝脏血流速度和频谱。结果1例术后第5d死于急性肾功能衰竭；其他近期并发症还包括：腹腔内大出血2例、门静脉栓塞1例、肝静脉狭窄1例、右上肺不张5例、成人呼吸窘迫综合征(ARDS)及肺炎2例、消化道出血3例、腹腔感染1例、伤口感染2例、病毒感染3例、肾功能损伤2例、胆道并发症2例、急性排斥反应2例。结论小儿可成功施行肝移植手术，然而，术后并发症的风险却不容忽视。     

儿童肝脏移植术后感染现状分析

自1989年首例儿童活体肝脏移植手术成功以来,肝脏移植术逐渐成为治疗儿童终末期和代谢性肝脏疾病的有效手段.近20年来,随着外科技术的日益完善及免疫抑制剂的合理应用,儿童肝脏移植术后的短期、长期存活率明显提高,但感染仍是肝脏移植术后最常见的并发症,严重影响预后.该文对儿章肝脏移植术后感染现状及合理用药作一概述.     

Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation

A 16-year-old girl is described with abetalipoproteinaemia who underwent liver transplantation for hepatic cirrhosis. After this procedure her serum lipoprotein profile was corrected; however, fat malabsorption and steatorrhea persisted because the primary defect, a mutant microsomal triglyceride-transfer protein, remains expressed in the intestine. Received: 1 September 1997 / Accepted in revised form: 8 December 1997     

胆道闭锁婴幼儿肝移植围术期液体管理

目的 分析胆道闭锁( biliary atresia,BA)婴幼儿肝移植围术期各阶段内环境的变化特点,并探讨相应的液体管理措施.方法 2006年9月至2011年8月我院行胆道闭锁婴幼儿肝脏移植手术24例,围术期连续监测患儿血流动力学,定时采血检测酸碱平衡、乳酸、电解质、血糖等指标,记录液体治疗措施.结果 患儿术中血pH值及碱剩余(BE值)于无肝期即刻下降至术毕接近正常,分别为(7.38±0.09)、(-1.44±0.64),术后开始升高、至术后12h达峰,此后逐渐下降至正常;乳酸于无肝期开始升高(3.31 -±0.8) mmol/L,新肝期30 min达峰,术后6h明显下降,术后12h完全降至正常;血清钾浓度(K+)于新肝期后出现显著下降(3.21±0.52) re mol/L;血清钙浓度(Ca2+)自无肝期出现明显下降(1.03±0.12) m mol/L;血清钠浓度(Na+)于新肝期后出现逐渐升高趋势(141.2±2.9)mmol/l;血糖(BG)于无肝期后明显高于术前水平(7.4±2.8) mmol/L.术后患儿电解质及血糖水平有所波动,但与术后即刻比较无明显变化.24例患儿均顺利完成手术,围术期未发生脱水及心肺事件.术后ICU死亡4例,其余患儿均顺利回到病房.结论 胆道闭锁婴幼儿肝移植围术期内环境呈现规律性变化.术中液体治疗原则与一般腹部大手术有所不同;补液速度相似,但是液体比例以胶体液为主,占液体总量的50 ％以上.术后即期液体治疗与普通外科手术相近.     

Surgical complications and outcome of paediatric liver transplantation: the Singapore experience

The purpose of this study was to analyse the early and late results of paediatric liver transplantation (LT), with particular reference to complications that required surgical intervention. The charts of all children who underwent LT between 1990 and 2002 were reviewed retrospectively. Results were analysed with a minimum follow up of 9 months. Thirty-five children have undergone 38 LTs; 22 received grafts from their parents, 16 received cadaveric organs and three children had retransplantation. The ages of the children ranged from 12 to 168 months. Biliary atresia was the most frequent indication for transplant (n=27). Twenty-seven children had complications that required surgical or radiological interventional procedures. Vascular complications included hepatic artery thrombosis (n=2), hepatic vein (HV) thrombosis (n=1), and the majority being portal vein thrombosis (n=6). Bile leaks were observed in eight children. Other complications included intestinal perforation (n=2), intra-abdominal abscesses (n=1), wound dehiscence (n=2), post-operative bleed (n=2), intestinal obstruction (n=2), ventral hernia (n=1), and multiple abdominal wound sinuses (n=1). Three children underwent retransplantation, two for hepatic artery thrombosis with multiple episodes of cholangitis and intrahepatic biliomas and the third was done for hepatic vein thrombosis. Patient and graft survival at 1 year is 81.5 and 74.2%, respectively. Paediatric LT is associated with significant morbidity, the main complications being vascular and biliary. The article was presented at the 8th Congress of the Asian Society of Transplanatation (8th CAST) in September 2003 held in Kuala Lumpur, Malaysia.     

小儿尸体肝移植探讨

目的　本研究探讨尸体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2 0 0 2年 4月～ 2 0 0 2年 12月对 5例患儿 (先天性胆道闭锁 4例 ,肝脏血管内皮肉瘤 1例 )进行肝移植术 ,患儿年龄 6个月～ 5岁。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。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。结果　 5例患儿移植肝重 2 6 0～ 5 6 5 g ,占受体体重1.94 %～ 5 .4 6 % ,4例术后已经健康存活 7～ 15个月 ,1例于术后第 5d死于心功能衰竭。结论　尸体肝移植是治疗小儿终末期肝病的有效方法 ,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。     

Health-related quality of life in long-term survivors of paediatric liver transplantation

BACKGROUND:Long-term survival after paediatric liver transplantation is now the rule rather than the exception. Improving long-term outcomes after transplantation must consider not only the quantity but also the quality of life years restored.OBJECTIVES:To characterize health-related quality of life (HRQOL) of LT recipients ≥15 years after paediatric LT.METHODS:Recipients of a paediatric LT performed before December 1996 in a single institution with continuous follow-up at either the paediatric or adult partner centre were identified. Patients with severe developmental or neurological impairment were excluded. HRQOL was assessed using the Pediatric Quality of Life Inventory 4.0, the Medical Outcomes Study Short Form-36 version 2 and the Pediatric Liver Transplant Quality of Life Tool.RESULTS:A total of 27 (67% male) subjects (mean age 24.3±6.7 years [median 23.2 years; range 16.6 to 40.3 years]) participated. The median age at transplant was 1.7 years (range 0.5 to 17.0 years). Seven (26%) participants underwent retransplantation. Seventeen (63%) participants were engaged in full-time work/study. Mean Short Form-36 version 2 scores included physical (49.6±11.1) and mental (45.3±12.5) subscale scores. The mean score for the disease-specific quality of life tool for paediatric liver transplant recipients (the Pediatric Liver Transplant Quality of Life Tool) was 64.70±15.2. The physical health of the young adults strongly correlated with level of involvement in work/study (r=0.803; P<0.05).CONCLUSIONS:The self-reported HRQOL of participants <18 years of age was comparable with a standardized healthy population. In contrast, participants between 18 and 25 years of age had HRQOL scores that were more similar to a group with chronic illness. Participants engaged in full-time work/study experienced enhanced physical health.     

亲体肝移植术后并发噬血细胞综合征的治疗

目的 总结儿童重型肝炎行儿童亲体肝移植术后并发噬血细胞综合征的治疗经验.方法 1例6岁女孩,因不明原因急性进行性黄疸、腹水及全身出血等住院,经化验、彩色多普勒、螺旋CT等确诊为亚急性重型肝炎,于2008年2月26日施行亲体肝移植术,供肝为患儿父亲的左外叶.术后3 d开始出现全血象进行性下降,13 d降至最低,血清铁蛋白升高,EB病毒DNA阳性,骨髓细胞学检查发现噬血细胞,确诊为儿童亲体肝移植术后EB病毒相关性噬血细胞综合征,用环孢素A、地塞米松和静脉注射丙种球蛋白等治疗,并进入层流病房,加强抗感染治疗.结果 术后患儿肝功能恢复顺利,各种酶学指标术后3d各种开始下降,7d接近正常,14d完全正常.术后20 d全血象开始逐渐上升,40 d升至正常水平,准予出院随访,至今无复发,情况良好.结论 儿童亲体肝移植手术后出现不明原因的全血象下降时应想到并发噬血细胞综合征的可能,早期诊断,及时正确的治疗能使患儿顺利康复.     

Current status of liver transplantation

Liver transplantation is accepted therapy for acute or chronic liver failure. Survival after LT has improved significantly in developed countries and this has increased the awareness of this treatment modality in the developing world. Successful LT in both children and adults have now been reported from India. Chronic liver failure secondary to cholestatic liver disease in the most frequent indication for LT, with biliary with atresia as the single commonest cause. Innovative techniques such as reduced size, splint, and living donor liver transplantation are being applied more often to decrease long waiting times and reduce associated morbidity and mortality. Early postoperative complications include primary graft failure, venous thrombosis, rejection, biliary complications and infections. Late complication includes CMV or EBV infections, side effects of immunosuppression, post transplantation lymphoproliferative disease and late biliary strictures. Most children achieve good quality of life. There are still many lessons to learn and there are future challenges such as the ever increasing problems of donor scarcity and the search for potent but less toxic immunosuppressive agents.     

Pediatric liver transplantation : The Indian perspective

Liver transplantation is now the accepted treatment for end stage liver disease, hepaticbased inborn errors of metabolism and localised primary hepatic malignancies. In children the commonest indication is biliary atresia. Innovations in surgical techniques, particularly liver reductions/splits and living related transplants have not only shortened the waiting period but have improved the results of surgery as well. The long term survival for pediatric liver transplant recipients in most large series from the Western world is more than 85%. Treatment protocols for ITU care of donors and recipients, anaesthetic care during surgery and post-operative immunosuppression are available. A country like India has a crying need for the development of facilities for liver transplantation in children but a number of factors have hampered the progress. The cost of surgery and post-operative immunosuppression and attitudes on life and death issues have been the greatest impediment. A few organisational details have also to be addressed to in order to minimise interdepartmental clashes of interest. It is hoped that a viable programme would soon be available for the children in India.     

儿童肝移植术后监护

经过半世纪发展,肝移植成为解决终末期儿童肝病的重要手段,明显改善患者预后.术后监护和治疗是肝移植的重要部分,并且具有一定的特殊性,如需要保持血管内等容量状态和正常血压以维持移植肝脏的供血,需要适当抗凝抗血栓保持肝脏血管通畅,早期抗排斥药物的应用和监测等,本文就儿童肝移植术后各脏器监护和各种并发症诊治进行综述和总结.     

亲属活体供肝儿童肝移植疗效分析

目的 总结我院10例亲属活体供肝儿童肝移植手术经验,评估其临床疗效.方法 回顾性分析2006年6月至2008年5月期间完成的10例亲属活体供肝儿童肝移植供受体手术经验及临床疗效.本组男5例,女5例.肝移植年龄6个月至14岁,体重7至42 kg.其中,Wilson'S病2例,胆道闭锁3例,严重门静脉高压症3例,重型肝炎1例和肝糖原蓄积症1例.术后8例用他克莫司(FK506)及强的松,2例用环孢素A(CsA)及强的松等抗免疫排斥.结果 10例供体均顺利出院,健康生活,无并发症发生;10例受体中除1例因门静脉血栓患儿围手术期死亡外,其余9例均痊愈出院.随访中除1例于术后5个月死于排异反应、1例术后7个月死于当地意外食物中毒外,其他7例存活至今,最长生存期24个月.术后并发症5例:门静脉血栓1例,胆道狭窄1例,腹腔淋巴漏1例,ICU监护期间严重感染2例等.结论 亲属活体供肝儿童肝移植手术技术要求高,术前仔细的供、受体选择,准确的血管影像学检查、精确的手术技术和严格的围手术期管理是亲属活体供肝儿童肝移植成功的关键.     

Psychological effects of liver disease and transplantation

Psychological adjustment in children with liver disease was investigated. Three groups of children 6–15 years old participated: ten had undergone a liver transplant (Gp1), 15 had ongoing chronic liver disease (Gp2) and 15 were healthy controls (Gp3). Children who had had a transplant appeared well adjusted and thought of themselves as healthy rather than ill, although areas of vulnerability were present, for example increased anxiety. No differences emerged in terms of coping with common or illness-related problems or understanding of the causes of illness and use of medication. Gps 1 and 2 showed higher levels of understanding of the functions of the liver but less understanding of illness prevention when compared to their healthy peers. Gp2 experienced less control over their health when compared to the other two groups. Gp1 rated themselves as more “healthy” than Gp2 but less so than Gp3. Conclusion Children with chronic liver disease are able to communicate how they deal with the stresses of the condition. Though well adjusted in many ways, those who have had a transplant still show areas of psychological vulnerability which need to be addressed in clinical practice. Received: 6 January 1997 / Accepted in revised form: 5 January 1998