活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

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.     

儿童DCD供肝肝移植术后胆道并发症高危因素研究

目的 分析儿童心脏死亡器官捐献(DCD)肝移植受者术后胆道并发症发生的高危因素.方法 收集天津市第一中心医院2013年3月至2015年3月施行的48例儿童DCD肝移植手术的临床资料,回顾性分析临床因素对受者胆道并发症的影响.结果 48例儿童DCD肝移植受者术后共11例发生胆道并发症,发生率为22.9％.单因素分析显示胆道并发症组与对照组间的热缺血时间(P=0.003)差异有统计学意义(P＜0.05),受体年龄(P--0.998)、受体性别(P=0.094)、MELD评分(P=0.159)、PELD评分(P=0.740)、Child-Pugh评分(P=0.159)、冷缺血时间(P=0.990)、受体ICU逗留时间(P=0.105)、是否发生感染(P=0.930)、有无其他并发症(门静脉狭窄/血栓形成、肝动脉栓塞、DGF)(P=0.268)以及A3O血型是否相容(P=1.106)差异无统计学意义(P＞0.05).多因素分析显示热缺血时间(P=0.020,OR=10.367,95％可信区间为1.451～74.089)是术后胆道并发症的独立危险因素.结论 胆道并发症仍然是儿童DCD肝移植术后的重要难题,热缺血时间是受者术后胆道并发症的独立危险因素.因此选择更短热缺血时间的CDC供肝可以降低儿童肝移植受者胆道并发症的发生率.     

胆道闭锁患儿亲体肝移植术后急性肺损伤风险因素分析

目的 分析胆道闭锁患儿亲体肝移植术后急性肺损伤的风险因素.方法 收集天津市第一中心医院2012年5月至2016年3月实施的112例胆道闭锁患儿亲体肝移植术临床资料,回顾性分析临床因素对患儿术后急性肺损伤的影响.结果 112例胆道闭锁患儿亲体肝移植患儿术后23例发生急性肺损伤,发生率为20.5％.单因素logistic回归分析显示肺部并发症组与对照组间年龄(P=0.010)、术前白蛋白(P=0.012)、术前总胆红素(P=0.001)、术前血清肌酐(P＜0.001)、术后1周内总胆红素峰值(P=0.035)差异有统计学意义(P＜0.05).多因素logistic回归分析显示,术前白蛋白(P=0.010,OR=0.830,可信区间为0.720～0.957)、术前总胆红素(P=0.001,OR=1.010,可信区间为1.004～1.016)及术前血清肌酐(P=0.001,OR=1.237,可信区间为1.104～1.387)是术后急性肺损伤发生的高危因素.结论 急性肺损伤是胆道闭锁患儿亲体肝移植术后的严重并发症,术前血清白蛋白、总胆红素及肌酐水平是患儿急性肺损伤发生的高危风险因素.     

肝移植术后完全停用免疫抑制剂三例儿童临床特点分析

目的 探讨肝移植术后完全停用免疫抑制剂儿童的临床特征,寻找可能与术后免疫耐受形成有关的因素.方法 回顾性分析我院2008年7月至2009年6月间完成的儿童肝移植中完全停用免疫抑制剂的3例患儿资料,包括:移植年龄、原发疾病、供肝来源、术后免疫抑制剂、随访检查结果等.结果 3例完全停药儿童的原发病均为胆道闭锁,平均移植年龄7个月17 d,均为母体活体供肝肝移植,肝移植术后免疫抑制剂方案均为强的松龙+环孢素A,服药至少2年以上,完全停药1年以上.结论 移植年龄＜1岁、原发病为胆道闭锁、母体供肝、服药2年以上、服用环孢素A是这些完全停药儿童的共同特征.     

胆道闭锁患儿肝移植术后胆管炎的发生情况分析

目的分析胆道闭锁患儿肝移植术后胆管炎的发生情况。方法回顾性分析2013年1月至2016年7月于本院移植科接受肝移植手术的219例胆道闭锁患儿临床资料。根据肝移植术前是否接受过肝门-空肠吻合术(Kasai手术)、肝移植术前是否发生胆管炎以及肝移植术中肠袢的不同处理方式进行分组,并比较各组间肝移植术后胆管炎的发生率。结果 219例胆道闭锁患儿肝移植术后胆管炎的总体发生率为23.29%。Kasai手术组肝移植术后胆管炎的发生率高于非Kasai手术组(30.70%vs 15.24%),差异有统计学意义(χ~2=7.316,P=0.007)。而Kasai手术组患儿中,术前胆管炎组与术前非胆管炎组肝移植术后胆管炎的发生率组间比较(28.57%vs 32.76%),差异无统计学意义(χ~2=0.235,P=0.628);肠袢保留组与肠袢重建组肝移植术后胆管炎的发生率组间比较(31.43%vs30.38%),差异无统计学意义(χ~2=0.013,P=0.911)。在Kasai术后存在胆管炎的患儿中,原肠袢组与新肠袢组肝移植术后胆管炎发生率组间比较(33.33%vs 24.14%),差异无统计学意义(χ~2=0.579,P=0.447)。结论对于胆道闭锁患儿Kasai术后的顽固性胆管炎,肝移植手术可取得较为满意的结果。胆道闭锁患儿肝移植术后胆管炎较为常见,但基本可以治愈。保证胆肠吻合的输入端肠袢长度足够,可有效控制肝移植术后胆管炎的发生。     

儿童肝移植20例诊治分析

目的回顾性分析20例肝移植患儿的临床资料,旨在总结儿童肝移植的临床诊治经验。方法回顾性分析2017年5月至2019年6月湖南省儿童医院联合中南大学湘雅附二医院对20例儿童实施肝脏移植手术的临床资料,原发疾病主要包括胆道闭锁、Alagille综合征、先天性肝内门静脉海绵样变性。手术方式采用亲体部分肝移植手术(n=16)和儿童心脏死亡器官捐献(donation after cardiac death,DCD)肝脏移植手术(n=4)。20例均应用他克莫司联合激素免疫抑制方案,其中3例增加了吗替麦考酚酯联合抗排斥反应。术后患儿给予抗感染、抗排斥等对症支持治疗,密切监测肝血管吻合处血流情况及肝功能变化,观察术后并发症及预后。结果 20例患儿均移植成功,肝移植供体均康复出院,无并发症发生。受体术后早期主要并发症为感染,以细菌感染为主,感染部位为肺部和腹腔。术后1例出现肝动脉栓塞,3例出现乳糜漏,2例出现胆道狭窄,3例出现早期排斥反应,1例出现消化道大出血,经对症治疗均痊愈出院,检测肝功能及血药浓度均在正常范围。结论儿童终末期肝病可通过活体肝移植或DCD肝移植手术取得理想的效果,手术方式可根据患儿的年龄、体重进行选择。     

肝移植治疗胆道闭锁:附16例报告

目的 探讨肝移植治疗胆道闭锁的疗效.方法 对16例胆道闭锁的患儿实施肝移植手术,其中7例为劈离式肝移植,9例为活体部分肝移植.术后所有患儿均给予抗排斥、抗感染、抗凝及营养支持等治疗,密切监测移植肝血流及肝功能变化,观察术后并发症及预后.结果 16例胆道闭锁患儿全部手术存活,术后早期主要并发症为感染,以细菌感染为主,感染部位主要为腹腔及肺部.死亡2例,1例术后9 d死于感染性休克,1例术后1年死于严重EB病毒感染、溶血性黄疸;其余14例患儿随访1 ～ 4.5年(至2010年12月),检测肝功能良好.结论 肝移植是治疗胆道闭锁安全有效的方法.     

Long-term follow-up in adult living donors for combined liver/bowel transplant in pediatric recipients: a single center experience

Pediatric candidates for combined liver/bowel transplant (LBTx) experience a very high mortality on the cadaver waiting list. Our transplant center has successfully used adult living donors to treat pediatric candidates for LBTx. We report the long-term follow-up of this unique cohort of organ donors. The charts of six adult donors for LBTx performed between 2004 and 2007 were reviewed. All the pertinent clinical data were carefully reviewed and integrated with phone interviews of all donors. A total of six children (average age 13.5 months) received living donor LBTx. Average follow-up for the donors was 42 months (range 29-51). The donors' median age was 25 yr (19-32); five women and one man. The average median hospital stay was nine days. There were no peri-operative complications. At present all donors remain in good health. Three of the five mothers became pregnant after donation. Five of the six children are currently alive and well whereas one died with functioning grafts six months post-transplant due to plasmoblastic lymphoma. Living donor LBTx is an effective therapy for combined hepatic and intestinal failure in children less than five yr. The donor operation can be performed with minimal morbidity.     

Preoperative imaging evaluation of the hepatic vasculature in biliary atresia patients undergoing LDLT: Comparison of MDCT and MRI

Abstract: LT is the definitive treatment option in the management of end‐stage liver disease. Preoperative vascular evaluation plays an important role for a safe and successful operation in LDLT. The purpose of this study is to assess the usefulness and accuracy of CTA and MRA in evaluating vascular anomalies in BA patients undergoing LDLT. Images of CTA and MRA for preoperative vascular evaluation in 57 BA patients undergoing LDLT were reviewed with the operative and pathologic findings. All underwent preoperative CTA and MRA. Pathologic PV (n = 20), interruption of the retro‐hepatic vena cava (n = 1), aberrant right HA from the SMA (n = 2) were confirmed during the transplant operation. The success rate of CTA and MRA in identifying vascular anomalies was 96% and 82%, respectively (p = 0.01). The IQR scores were 3.25 ± 0.53 for CTA and 2.91 ± 0.70 for MRA (p = 0.001). The sensitivity, specificity and accuracy of CTA were 85%, 97% and 93%, respectively; and for MRA, were 65%, 95% and 84%, respectively. CTA is superior than MRA in the preoperative evaluation of the vascular anatomy in pediatric BA LDLT candidates.     

Positive association of macrophage migration inhibitory factor gene-173G/C polymorphism with biliary atresia

BACKGROUND: Macrophage migration inhibitory factor (MIF) is a pleiotrophic lymphocyte and macrophage cytokine; it is likely to play an important role in innate immunity. Its expression was increased in several inflammatory diseases, and MIF gene polymorphisms have an effect on disease outcome and response to glucocorticoid treatment. AIM: To investigate the role of the 173G/C polymorphism of the MIF gene for susceptibility to biliary atresia (BA). METHOD: Between February 2002 and November 2004, 18 patients (mean age 1 +/- 0.4 years) diagnosed as having BA were studied. After informed consent, blood was collected, and DNA was obtained. MIF 173C/G polymorphism was detected using the polymerase chain reaction-restriction fragment length polymorphism based method. BA patients were compared with a group of chronic liver disease patients (CLD) (n = 36) and a group of unrelated healthy controls (n = 103). RESULTS: MIF-173C allele frequency was significantly higher than both the CLD and healthy control groups (P = 0.03, odds ratio [OR] 4.4, 95% confidence interval [CI] 1.3-15.1; P = 0.000, OR 4.1, 95% CI 2.3-7.6, respectively). Univariate analysis showed that MIF-173G/C polymorphism was significantly associated with BA (for GC genotype, OR = 6, 95 % CI 2.8-11.5, P = 0.000). There was no significant correlation between pediatric end stage liver disease score and MIF genotypes both in BA and CLD groups. CONCLUSION: Our results suggest that the -173C allele of the MIF gene might be associated with the susceptibility to BA.     

Duct-to-duct biliary reconstruction in pediatric living donor liver transplantation

The results of duct-to-duct biliary reconstruction in six pediatric patients who received a living donor liver transplant aged from 2 months to 11 yr old are reported. The graft was either entire or a part of the left lateral segments. The orifice of the bile duct of the graft was anastomosed to the recipients' hepatic duct in an end-to-end fashion by interrupted suture using 6-0 absorbable material. A transanastomotic external stent tube (4 Fr) was passed through the stump of the recipients' cystic duct. Mean time for reconstruction was 24 min. All the recipients survived the operation and reinitiated oral intake on postoperative day 3. There were no early biliary complications. One 5-yr-old boy suffered from an anastomotic stenosis 9 months after transplantation. He underwent re-anastomosis by Roux-en Y (R-Y) procedure and recovered uneventfully. Duct-to-duct anastomosis in pediatric living donor liver transplantation has benefits while the complication rate is comparable to R-Y reconstruction.     

Studies of Pediatric Liver Transplantation 2002: patient and graft survival and rejection in pediatric recipients of a first liver transplant in the United States and Canada

Studies of Pediatric Liver Transplantation (SPLIT) is a cooperative research network comprising 38 pediatric liver transplant centers in North America. Data from the 1092 patients who have received a first liver transplant since 1995 were analyzed for factors influencing patient survival, graft survival and acute rejection. The 3, 12, 24 and 36 month Kaplan-Meier estimates of patient/graft survival were 90.9/85.5, 86.3/80.2, 84.3/76.0, and 83.8/75.3% respectively. Univariate analysis identified initial diagnosis, type of graft (whole vs. living and cadaveric technical variant), growth failure and continuous hospitalization or ICU admission prior to transplantation as significantly influencing patient and graft survival. Subsequent multivariate analysis identified as risk factors for death: fulminant liver failure (RR = 3.05, p < 0.05), cadaveric technical variant grafts (RR = 1.95, p < 0.05), continuous hospitalization pre-transplant (RR = 1.79, p < 0.05), height deficit >2 s.d. from mean (RR = 3.22, p < 0.05). Risk factors for graft loss included: fulminant liver failure (RR = 2.27, p < 0.05), cadaveric technical variant grafts, (RR = 1.97, p < 0.05). Eleven percent of the 1092 patients were re-transplanted; vascular complications, particularly hepatic artery thrombosis (8.3% overall; 36.3% of graft failures), were responsible for the majority of re-transplants. Infection was the single most important cause of death (40 of 141, 28.4%) and was a contributing cause in 55 (39%), particularly with bacterial or fungal organisms. The cumulative Kaplan-Meier estimates of first rejection at 3, 12, 24 and 36 months were 44.8, 52.9, 59.1, and 60.3%. Initial immunosuppression with tacrolimus reduced the probability of rejection (RR = 0.62, p < 0.05). Eleven percent of rejections were steroid-resistant; chronic rejection led to 7 of 121 (5.8%) re-transplants. The SPLIT registry, in compiling data from a large number of centers, reflects the current outcomes for pediatric liver transplants in North America.     

Liver transplantation for biliary atresia: A nationwide investigation from 1996 to 2013 in mainland China

We investigated the overall situation of LT for BA in mainland China and analyzed their survival outcomes based on data from CLTR. Between January 1996 and December 2013, 509 liver transplants for BA were performed in mainland China and were included in this study. Patients' median age was 9.6 months (range: 4.8–175.2 months). KP was previously performed in 194 cases (38.1%). Grafts from living donors were used in 380 cases (74.7%). Era I (1996.1–2010.12) and era II (2011.1–2013.12) comprised 151 cases (29.7%) and 358 cases (70.3%), respectively. Twenty‐five centers had performed at least one liver transplant for children with BA. Centers from Shanghai (197 cases), Tianjin (143 cases) and Beijing (81 cases) involved 82.7% of the 509 cases. One‐ and five‐yr graft survival rates were 84.7% and 72.6%, respectively. Split grafts, center volume <20, GRWR ≥4.0%, and steroid‐free immunosuppression regimen were independent risk factors for graft survival. In conclusion, the dramatic expansion of pediatric liver transplant programs in mainland China has enabled improved survival for those children affected by this devastating disease. However, screening of BA in neonates should be emphasized throughout the country to enhance early referrals for KP.     

Plasma endothelin-1 levels in patients with biliary atresia: possible role in development of portal hypertension

BACKGROUND: Biliary atresia (BA) is a severe neonatal liver disease characterized by progressive extrahepatic biliary tract and intrahepatic inflammatory process. Hepatic fibrosis and portal hypertension (PH) still occur despite the disappearance of jaundice following successful hepatic portoenterostomy. Endothelin-1 (ET-1) is a potent vasoconstrictor and has been reported to stimulate hepatic collagen synthesis. The aim of this study was to demonstrate the potential role of ET-1 in the pathogenesis of the progressive inflammation, fibrosis and PH in BA. METHODS: Thirty pediatric patients with biliary atresia post-hepatic portoenterostomy and 12 healthy children were examined. The ET-1 level was determined by commercially available enzyme-linked immunosorbent assay kits. RESULTS: Endothelin-1 levels were elevated in the patients compared with those of the controls (5.45+/-3.34 vs. 2.74+/-2.17 pg/ml, P = 0.01). Moreover, patients with PH also had greater levels of ET-1 than those without PH (6.73+/-3.27 vs. 3.26+/-2.2 pg/ml, P = 0.004). Patients with abnormal transaminase enzymes had significantly higher ET-1 levels than those with normal enzymes (6.43+/-3.33 vs. 3.17+/-2.1 pg/ml, P = 0.01). In the jaundice-free group, endothelin-1 levels were elevated in the patients with PH compared with those without PH (5.93+/-2.15 vs. 2.88+/-2.1 pg/ml, P = 0.02). CONCLUSIONS: Our findings showed elevation of plasma ET-1 levels in patients with BA, especially in those with PH. ET-1 levels were also higher in patients with elevated transminase enzymes as well as in the jaundice-free group with PH. ET-1 might play a role in the pathogenesis of the progressive inflammation, fibrosis and PH in BA.     

Management of hepatic venous obstruction after split-liver transplantation

Stenosis of the hepatic vein anastomosis is an unusual but critical complication after liver transplantation. In pediatric liver transplantation, the scarcity of size-matched donors has required the use of segmental liver allografts, either as reduced-size or split-liver grafts. This report illustrates the primary use of a hepatic vein stent to manage hepatic venous outflow obstruction in a pediatric split-liver recipient, and reviews experience in the management of hepatic venous outflow obstruction after liver transplant using stent methods.     

Does intrauterine growth restriction affect quality of life in adulthood?

OBJECTIVE: To compare health-related quality of life in 50-year-old adults who were born at term (> or =37 to 42 weeks' gestation) with intrauterine growth restriction (IUGR; birth weight <10th centile) and a group born at term without IUGR (> or =10th centile). DESIGN: Case control study. SETTING: A large regional maternity hospital in Northern Ireland. SUBJECTS: 235 adults who were born between 1954 and 1956 in the Royal Maternity Hospital, Belfast. 111 subjects born with IUGR and 124 controls with normal birth weight for gestation were compared. MAIN OUTCOME MEASURE: Health-related quality of life in adulthood was assessed using the Short Form-36 Health Survey (SF-36). RESULTS: The two groups reported similar health-related quality of life on each of the eight dimensions of the SF-36 and there were no significant differences between them. Adjusting for potential confounding variables did not alter this conclusion. CONCLUSIONS: The similarity of SF-36 scores indicated that those born with IUGR did not perceive this to adversely influence health-related quality of life at 50 years of age compared with those with normal birth weight.