Objective:To evaluate the effect of parental liver donation on early acute cellular rejection(ACR)after liver transplantation(LT)in children aged under one year.Methods:From January 2018 to January 2021, retrospective review is conducted for clinical data of living donor LT recipients and donors aged under 1 year at Tianjin First Central Hospital.Donor livers are assigned into two groups of paternal donor liver(156 cases)and maternal donor liver(206 cases)according to the source of donor liver, Clinical characteristics and postoperative ACR occurrence of two groups are analyzed.Results:The rates of ACR during early postoperative period is 14.9%(54/362), 20.5%(32/156)in paternal liver donor group and 10.7%(22/206)in maternal liver donor group.There is statistically significant difference(λ 2=6.763, P=0.009).In analysis of gender matching of donor recipients, the rates of ACR is 22.6% in paternal donor group and 10.3% in maternal donor group.There is statistically significant difference(λ 2=5.411, P=0.020).Median time of initial postoperative ACR is 13.00(8.25~20.25)day in paternal liver donor group and 17.00(9.00~28.25)day in maternal donor group.The difference is not statistically significant( P>0.05). ACR is mostly mild-to-moderate in two groups . Conclusions:In living donor LT for children aged under 1 year, the rates of early ACR is lower for maternal donor than that for paternal donor, especially in female recipients.

Objective:To explore the risk factors for the occurrence of thrombocytopenia (TCP) within 2 weeks after pediatric liver transplantation (LT) and examine the relationship between the occurrence of TCP and prognosis.Methods:From January 2021 to November 2021, clinical data were retrospectively reviewed for 162 pediatric LT recipients aged under 4 years at Organ Transplantation Center of Tianjin First Central Hospital.Based upon the lowest value of platelet count at Week 2 post-operation, they were assigned into two groups of TCP (n=90) and non-TCP (n=72). General preoperative profiles, intraoperative findings, postoperative complications, types of commonly used antibiotics, anticoagulant dosing and prognosis of two groups were compared.Univariate and multivariate analyses were utilized for examining the independent risk factors for TCP.Receiver operating characteristic (ROC) curve was plotted for examining the cut-off value of independent risk factors for diagnosing TCP.Results:Among them, 90 (55.56%) developed TCP within 2 weeks post-operation and 25(15.43%) developed TCP at Day 1 post-operation.The median preoperative platelet count was 178×10 9/L and the lowest value was 65×10 9/L at Day 3(1-4) post-operation with a declining rate of 63.5% and platelet count of recipient normalized at Day 6(4-7.25) post-operation.The results of univariate analysis showed statistically significant inter-group differences in operative duration[(574.43±80.53)min vs.(526.75±72.42)min], intraoperative blood loss[400(300, 550)ml vs.320(300, 400)ml], red blood cell transfusion[2(2, 3)U vs.2(1.5, 2.0)U], preoperative platelet count[178.5(141.75, 242.5)×10 9/L vs.257 (209.75, 357)×10 9/L], postoperative infection rate[27.8%(25/90)vs.13.9%(10/72)] and dosing rates of piperacillin sodium and tazobactam sodium[8.9%(8/90)vs.25.0%(18/72)] ( P<0.05). Multivariate Logistic regression analysis revealed statistically significant inter-group differences in operative duration( P=0.008), red blood cell transfusion( P=0.01), preoperative platelet count( P<0.01) and postoperative infection rate ( P=0.02). The results of ROC curve analysis showed that the cut-off values of operative duration, red blood cell transfusion and preoperative platelet count were 535 min, 2.75 U and 183.5×10 9/L respectively.Length of ICU stay was higher in TCP group than that in non-TCP group, and the difference was statistically significant [4(3, 5) vs.3(3, 4) day, P=0.006]. Conclusions:LT children aged under 4 years with intraoperative red blood cell transfusion>2.75 U, operative duration>535 min and preoperative platelet count<183.5×10 9/L are more likely to develop post-transplantation TCP.And occurrence of TCP prolongs the length of ICU stay in pediatric recipients.

Objective:To explore the reconstruction strategy and technical selection of S3 hepatic vein with middle hepatic vein confluence in pediatric liver transplantation(LT)using living donor left lateral segment to lower the risk of vascular complications caused by variant grafts.Methods:From January 2015 to June 2021, retrospective analysis is performed for 840 consecutive cases of pediatric living donor LT using left lateral segment(LLS).There are 32 cases of S3 hepatic vein with middle hepatic vein confluence with an overall incidence of 3.81%.Individualized reconstruction strategies are implemented according to the specific conditions of variation and different interposition vessels available: group I unification venoplasty technique with interposition vein graft is employed for reconstructing HV from grafts, prolonged S3 is formed into a single opening with S2 and then anastomosed with recipient(21 cases); group Ⅱ dual HV reconstructions were performed(11 cases); venoplasty of recipients'LHV, MHV and inferior vena cava(IVC)is performed for creating a large orifice for anastomosis with S2 HV from graft and S3 is anastomosed with stump of recipient right HV directly or interposed blood vessels.Clinical features and prognosis of two groups, the incidence, treatment and prognosis of HVOO and the incidence of HVOO between variant and non-variant groups were compared.Results:The median follow-up time of variant group(32 cases)is 23.8 month with an incidence of HVOO at 15.6%.During the same period, the non-variant group incidence of HVOO is 4.5%.There is inter-group statistical difference( P=0.014).The only statistical difference between groups Ⅰ and Ⅱ is ultrasonic blood flow velocity of S3 HV at 14 POD [(39.15±16.37)vs(20.05±8.52)cm/s, P=0.001].HVOO occurred in 7 cases and 6 cases respectively in groupⅠ and group Ⅱ.There is no statistical difference( P=0.310).There are no intractable vascular complications.Long-term vascular patency of allogeneic and autologous interposition vein is satisfactory and there is no graft failure or mortality related to HVOO. Conclusions:Selecting strategies and techniques for reconstructing S3 hepatic vein with middle hepatic vein confluence at our center are reasonable, safe and effective.And the overall treatment efficacy is satisfactory.Reasonable selection of multidimensional reconstruction methods and accurate application of various technologies are conducive to improving patient prognosis.

Objective:To explore the correlation between the percentage of eosinophils in peripheral blood and the occurrence of acute rejection and evaluate the predictive value of the percentage of eosinophils in blood for early rejection in children after liver transplantation (LT).Methods:From January 2019 to December 2020, clinical data were retrospectively reviewed for children undergoing LT.They were divided into two groups of acute rejection and non-rejection based upon the pathological findings of liver biopsy within 6 months.The mean percentage of early postoperative eosinophil was compared between two groups.With a contour graph, repeated measurement ANOVA was performed for determining the correlation with the occurrence of acute rejection.And receiver operating characteristic (ROC) curve was plotted for determining the critical mean percentage of eosinophil during an early stage of rejection.Results:A total of 396 children undergoing LT were recruited.The postoperative incidence of early acute rejection was 17.93%(71/396). After stratification by age (0~1y/1-6y/>6y), the mean percentage of eosinophil during an early postoperative period was significantly correlated with the occurrence of rejection within 6 months in children aged 0~1 years, but not in children of other ages.The results of repeated measurement indicated that the mean percentage of eosinophil in acute rejection group was significantly higher than that in non-rejection group among children aged 0~1 ( F=25.38, P<0.001). ROC curve of the mean percentage of eosinophil predicting the rejection reaction at 1~7 days showed that its area under curve (AUC) was 0.743 (95% CI: 0.668-0.817) with a sensitivity of 65%, a specificity of 74%, a critical value of 3.23%, a positive predictive value of 31.18% and a negative predictive value of 91.05%. Conclusion:Among pediatric LT recipients, the mean percentage of eosinophil at Day 1-7 has certain predictive value for the occurrence of acute rejection after LT in children aged 0~1 years.With high specificity and negative predictive value, it may be utilized as an indicator of early risk screening.

患儿 女，8月龄。因“诊断为原发性高草酸尿症Ⅰ型5个月，发展至肾功能衰竭尿毒症期 2个月”，为求进一步治疗于 2021年11月3日就诊于我院。患儿3月龄时突发休克、呼吸心跳骤停，于当地医院经心肺复苏、呼吸机辅助通气、体外膜肺氧合（extracorporeal membrane oxygenation，ECMO）联合连续性肾脏替代治疗 2周，心肺功能逐渐恢复；撤除ECMO治疗后仍无尿；肾脏穿刺活检病理学检查结果显示，肾小管内有大量钙盐沉积，结合检测AGXT基因的2个杂合突变，确诊为原发性高草酸尿症Ⅰ型。患儿每周接受3次透析治疗（每次透析12~24 h）。患儿父母各有1个AGXT基因杂合突变。患儿身长68 cm，体重6.7 kg。入院后CT检查结果提示，双肾密度增高（图1）。经评估患儿为高草酸尿症Ⅰ型、慢性肾功能衰竭、尿毒症，无器官移植手术禁忌证，将其加入肝、肾移植等待名单。

Burkitt淋巴瘤(Burkitt's lymphoma,BL)是一种高度侵袭性B细胞非霍奇金淋巴瘤,约占儿童淋巴瘤病例的25％～40％ [1].BL可分为地方性、散发性和免疫缺陷相关性3种不同的临床形式.实体器官移植受者长期接受免疫抑制剂治疗,其BL 发病率约为每年10.8/10万人,比普通人群高出 23倍,其中儿童器官移植受者BL发病率约为成人受者的3.5倍[2-3].BL临床大多起病较急、进展快,除淋巴结肿大以外,颌面肿物及腹部占位引起的急腹症是最常见表现;患者可以迅速出现骨髓转移,晚期可呈现恶液质;肿瘤增殖速度快,病死率高、预后不佳.目前针对BL一般采用短疗程、高强度、多药联合和中枢神经系统预防的治疗策略,同时给予利妥昔单抗等靶向治疗药物,治疗效果明显提高, 5年生存率甚至可达80％.结合相关文献,现将我科诊治的1例儿童肝移植术后BL病例报告如下.

目的 探讨肝移植治疗儿童胆道闭锁(biliary atresia,BA)合并肝脏恶性肿瘤的临床疗效及预后.方法 2015年1月1日—2019年12月31日期间天津市第一中心医院儿童器官移植科的儿童肝移植患者中,有4例原发疾病为胆道闭锁合并肝恶性肿瘤,回顾性分析这4例患者的临床资料.结果 4例患者中BA合并肝母细胞瘤(hepatoblastoma,HB)3例,BA合并肝细胞癌(hepatocellular carcinoma,HCC)1例,所纳入的患者术前均诊断BA,其中1例术前诊断为BA合并HB,其余3例为肝移植术后病理诊断为BA合并HB或HCC.4例患者肝移植术后均顺利恢复,术后中位随访时间为25.5个月,4例患者全部存活,随访期内均未发现肿瘤复发.结论 肝移植是BA合并肝脏恶性肿瘤的有效治疗方式,对于低风险患者,移植术后未行辅助化疗的近期疗效确切,远期疗效尚需进一步研究.

Objectives: To study the risk factors for massive intraoperative blood loss in children with biliary atresia who underwent liver transplantation for the first time,and to analyze their impacts on graft survival,hospital stay and postoperative complications. Methods: The data of 613 children with biliary atresia who underwent liver transplantation at Department of Pediatric Organ Transplantation,Tianjin First Central Hospital from January 2015 to December 2018 were collected and analyzed. There were 270 males and 343 females, aged 7.4 (3.9) months (range: 3.2 to 148.4 months), the body weight of the recipients were (7.8±3.5) kg (range: 4.0 to 43.3 kg).According to the 85th quad of estimated blood loss(EBL),they were divided into two groups:massive EBL group(96 cases) and non massive EBL group(517 cases). The age,height,weight and other factors between the two groups were analyzed and compared. Univariate Logistic regression and multiple stepwise regression were used to determine the risk factors of massive EBL. Then,the postoperative complications of the two groups,including portal vein thrombosis and portal vein anastomotic stenosis etc.,were analyzed and compared by chi square test. Kaplan Meier curve and log rank test were used to analyze the recipient and graft survival rate of the two groups. Results: During the study period,713 transplants were performed and 613 patients were enrolled in the study. Ninety-six patients(15.7%) had massive EBL,and the postoperative hospital stay was 21(16) days(range:2 to 116 days),the hospital stay of non-massive EBL group was 22(12)days(range:3 to 138 days)(U=24 224.0,P=0.32). Univariate Logistic regression analysis showed that the recipient's weight,Kasai portoenterostomy,platelet count,operation time and cold ischemia time were the risk factors of massive EBL during biliary atresia transplantation. Multiple regression analysis showed that cold ischemia time ≥10 hours,prolonged operation time(≥8 hours) and body weight<5.5 kg were important independent risk factors for massive EBL.The incidence of portal vein thrombosis,hepatic vein stenosis,intestinal leakage and pulmonary infection in patients with massive EBL were significantly higher than those without massive EBL(3.1% vs. 0.8%,9.4% vs. 2.1%,6.3% vs. 0.8%,30.2% vs. 20.1%,all P<0.05). The 3-year overall graft and recipient survival rate were significantly lower in patients with massive EBL than those without massive EBL(87.5% vs. 95.7%,P=0.001;84.4% vs. 95.4%,P<0.01,respectively). Conclusions: In children with biliary atresia who underwent liver transplantation for the first time,the effective control of intraoperative bleeding should shorten the operation time and reduce the cold ischemia time as far as possible,on the premise of ensuring the safety of operation. For children without growth disorder,the weight of children should be increased to more than 5.5 kg as far as possible to receive the operation. Reducing intraoperative bleeding is of great significance to the prognosis of children.

目的 比较拉米呋定与乙肝疫苗方案预防乙型肝炎核心抗体(hepatitis B core antibody,HBcAb)阳性供肝儿童肝移植术后新发乙型肝炎病毒(hepatitis B virus,HBV)感染效果.方法 对天津市第一中心医院自2013年5月—2019年6月251例接受HBcAb阳性供肝儿童肝移植的资料进行回顾性分析,依据采用预防方案的不同分为拉米呋定组和乙肝疫苗组,对两组患儿的新发乙肝病毒感染情况以及临床资料进行比较分析.结果 拉米呋定组45例和乙肝疫苗组206例,两组在供受者的临床特征方面无显著差异,两组的新发乙肝病毒感染例数分别为5例(11.1％)和10例(4.9％),发生率无显著统计意义(P=0.075),停用拉米呋定与新发乙肝存在关系.结论 单用拉米呋定和乙肝疫苗均是有效预防HBcAb阳性供肝术后新发乙肝的方案,停用拉米呋定会增加新发乙肝的风险.

目的:研究儿童肝移植术后5年发生纤维化的临床相关危险因素。方法:2013年1月至2016年12月天津市第一中心医院共完成儿童肝移植498例，其中94例于术后4～6年行肝穿刺活检。根据术后肝活检的结果将受者分为纤维化组和非纤维化组，采用单因素分析及多因素Logistic回归分析儿童肝移植术后肝纤维化临床危险因素。根据筛选出的独立危险因素建立Logistic回归模型，得到联合预测因子的预测值。绘制联合预测因子的受试者工作特征曲线(ROC)，评价其预测纤维化发生的准确性。结果:94例儿童受者中有54例(57.5%)出现肝纤维化。单因素分析显示，两组受者在冷缺血时间( Z＝-2.094)、热缺血时间( Z＝-2.421)、药物性肝损伤( χ2＝7.389)、胆道狭窄( χ2＝4.560)、肝动脉血栓、排斥反应( χ2＝6.955)上差异有统计学意义(均 P<0.05)。多因素Logistic回归分析显示冷缺血时间(OR＝1.003, 95%CI：1.000～1.006)、药物性肝损伤(OR＝6.493, 95%CI：1.615～26.101)、胆道狭窄(OR＝6.451，95%CI：1.205～33.295)和排斥反应(OR＝2.735，95%CI：1.057～7.077)是肝移植术后发生肝纤维化的独立危险因素。联合预测因子的ROC曲线下面积(AUC)为0.786(95%CI：0.691～0.881)，临界值为0.311，灵敏度为90.70%，特异度为60.00%，且联合预测因子的诊断价值与其他独立危险因素单独诊断的价值差异有统计学意义( P<0.05)。 结论:儿童肝移植术后5年肝纤维化的发生率为57.5%，冷缺血时间、胆道狭窄、排斥反应和药物性肝损伤是儿童肝移植术后5年发生纤维化的独立危险因素。联合预测因子对儿童肝移植术后肝纤维化的发生具有较高的预测价值。

目的 探讨小儿血型不合活体肝移植的临床疗效.方法 回顾性分析242例小儿活体肝移植受者的临床资料.根据供、受者血型相合情况分为A组(供、受者血型相同,165例)、B组(供、受者血型相合,42例)、C组(供、受者血型不合,35例).观察并比较3组受者的术后并发症发生情况、术后供体特异性抗体(DSA)产生情况;分析C组供、受者血型分布及红细胞抗体产生情况;比较3组受者肝移植术后的生存情况.结果 3组受者并发症发生率比较,差异均无统计学意义(均为P>0.05).肝移植术后DSA以人类白细胞抗原(HLA)Ⅱ类抗体为主,多为抗HLA-DR和抗HLA-DQ.C组肝移植受者术后红细胞抗体多为IgM,所有抗体滴度均为≤12.3组受者术后生存率差异均无统计学意义(均为P>0.05).结论 小儿血型不合活体肝移植是一种安全有效的治疗方式,可有效扩大肝移植供者来源,挽救患儿生命.

目的 探讨多米诺肝移植(domino liver transplantation,DLT)治疗儿童肝脏疾病的临床疗效.方法 本研究纳入3组6例DLT患者,包括2例活体捐献者(living donor,LD)及1例脑死亡捐献者、3例枫糖尿病(maple syrup urine disease,MSUD)患者及3例胆管闭锁(biliary atresia,BA)DLT受者.每组DLT供受者手术同期进行.活体供者获取左外叶,脑死亡器官捐献者(donation after brain death,DBD)获取全肝,根据供肝血管条件决定是否行血管成型.MSUD患者均切除全部病肝,其中2例分别接受肝左外叶活体肝移植(living donor liver transplantation,LDLT),1例接受DBD供肝移植.DLT受者切除全部病肝,分别接受MSUD患者病肝全肝供肝肝移植.结果 3例MSUD患者术后2周内肝功能均降至正常范围,移植肝血流无异常.3例DLT受者术后2周内胆红素基本降至正常范围,移植肝血流均正常.其中1例MSUD患者术后1个月后出现门静脉吻合口狭窄,介入下行球囊扩张后好转.1例MSUD患者术后2周余因出现急性细胞性排斥反应,激素冲击治疗后好转.1例DLT受者术后1周余即出现急性排斥反应,应用激素后好转.1例DLT受者术后1周出现肺感染,在重症监护病房(intensive care unit,ICU)停留时间较长,经抗感染治疗后好转.随访期间(8～16个月)所有肝移植患者及移植物均存活.结论 应用MSUD患者的供肝行DLT在技术上是可行的,预后良好,在一定程度上扩大了肝移植供体的来源.

Objective:This article discussed the risk factors of portal vein stenosis (PVS) after living liver transplantation (LDLT) so as to provide rationales for clinical prevention and treatment of PVS in children.Methods:The clinical data of 429 pediatric LDLT were retrospectively analyzed and the risk factors of PVS post-LDLT screened by univariate and multivariate analyses.Results:Among them, the overall incidence of PVS post-LDLT was 6.8% (29/429) and the median time of its initial diagnosis3 months (7 days to 14 months). Univariate analysis revealed that recipient's gender ( P=0.039), intraoperative portal vein anastomosis ( P=0.000), portal vein reconstruction with vein graft ( P=0.003) and intraoperative portal vein thrombosis ( P=0.000) were correlated with the occurrence of PVS post-LDLT. Multivariate analysis indicated that recipient's gender ( P=0.019) and intraoperative portal vein thrombosis ( P=0.000) were the risk factors for portal stenosis post-LDLT. Conclusions:Male and intraoperative portal vein thrombosis are risk factors for PVS after pediatric LDLT. For recipients with the above-mentioned risk factors, during postoperative follow-ups, special attention must be paid to the screening of PVS. Thus timely detection and prompt treatment are implemented for lowering the risk of LDLT failure due to PVS.

Objective To explore the use of muhifocal electroretinograms (mERG) in detecting changes in chronic arsenic poisoning. Methods 18 patients who were diagnosed chronic arsenic poisoning underwent mERG testing in both eyes using RETI Scan multifocal ERG (Version 5.2) , 18 ages matched normal peoples were included as controls. In order to observe changes in condition, they were tested in 21,60, 90 days. The first order kernel responses of mERG were recorded and to analyze in six concentric rings centred on the fovea. Results There were no significant differences in the amplitudes and the latencies between the 1 ring regions when compared with the normal control group. There was a significant difference in N1 wave amplitude and a significant difference in the P1 wave amplitude between the two groups at ring 2 - 6 (P0.01). And with the changes condition, the ring appears significant difference compared with normal controls. We also found that mERG changes even earlier than clinical manifestations. Conclusions Chronic arsenic poisoning will appear irreversible damage to the retina in the earlier mobility. The mERG is a useful test for clinical diagnosis and monitoring of disease change.