Objective:To investigate the etiology,clinical features and prognosis of pediatric liver retransplantation.Methods:The data of 1 024 cases of pediatric liver transplantation (<18 years old) from January 2014 to December 2019 operated at Tianjin First Central Hospital were collected,retrospectively. Retransplantation was performed in 26 cases,among which 25 cases received secondary liver transplantation and 1 case received a third liver transplantation. There were 13 male and 12 female patients among the 25 patients. The median age was 12.9(20.5) months(range: 5.8 to 134.8 months), the body weight was 8.0(5.6) kg(range: 5.0 to 30.0 kg) at the time of retransplantation. The pediatric end-stage liver disease(PELD) score was 17.0(21.3) (range: 0 to 45) before retransplantation. The etiology of retransplantation was biliary complications in 7 cases,primary nonfunction of liver graft in 5 cases,antibody-mediated rejection in 4 cases,hepatic artery thrombosis in 3 cases,portal vein thrombosis in 3 cases,concomitant hepatic artery and portal vein thrombosis in 2 cases,thrombogenesis of inferior Vena Cava in 1 case and sinusoidal obstruction syndrome in 1 case. The patients were divided into two groups according to the time interval(30 days) between two liver transplantations,8 patients were classified into early-retransplantation(≤30 days) group and 18 patients were classified into late-retransplantation (>30 days) group. The etiology of liver retransplantation,pre-transplant score,time interval between two transplantations,surgical aspects,major complications and survival rates were compared between the two groups. Continuous variables with normal distribution were compared with t test,while Mann-Whitney U test was applied to compare variables without normal distribution. Categorical variables were compared with chi-square test. The survival curves were created by Kaplan-Meier method and compared by Log Rank test. Results:The median follow-up time was 26.8(30.2) months(range: 1 day to 85.7 months), and the incidence of retransplantation was 1.9%. In the early-retransplantation group,the duration of surgery was (439.8±151.0)minutes,the graft-to-recipient weight ratio was 5.0(1.8)%(range:3.6% to 6.1%),the main cause for retransplantation were primary nonfunction and vascular complications. In the late-retransplantation group,the duration of surgery was (604.4±158.0)minutes,the graft-to-recipient weight ratio was 3.4(2.1)%(range:1.4% to 5.3%),the main cause for retransplantation were biliary complications,antibody mediated rejection and vascular complications.The 3-month,1-year and 2-year recipient survival rates in the early-retransplantation group were all 62.3%,while the recipient survival rates in the late-retransplantation group were 100%,93.8% and 93.8%,respectively. The difference of recipient survival rates was significant between the early-retransplantation group and the late-retransplantation group( P=0.019). The overall 3-month,1-year and 3-year recipient survival rates after the primary liver transplantation were 97.1%,95.4%,94.1%,respectively. Conclusions:The vascular complications,biliary complications,primary nonfunction and antibody-mediated rejection are the main causes of liver retransplantation.The PELD score is higher in patients receiving early retransplantation,while the surgery is relatively more complex in patients receiving late retransplantation,which is reflected by longer duration of surgeries. Patients in the late-retransplantation group showed similar recipient survival rates with primary liver transplantation recipients,and the survival rates are superior to those of patients in the early-retransplantation group. Infection and multiple organ failure are the most common fatal causes after retransplantation.

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 85 th 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.

Objective:To investigate the etiology,clinical features and prognosis of pediatric liver retransplantation.Methods:The data of 1 024 cases of pediatric liver transplantation (<18 years old) from January 2014 to December 2019 operated at Tianjin First Central Hospital were collected,retrospectively. Retransplantation was performed in 26 cases,among which 25 cases received secondary liver transplantation and 1 case received a third liver transplantation. There were 13 male and 12 female patients among the 25 patients. The median age was 12.9(20.5) months(range: 5.8 to 134.8 months), the body weight was 8.0(5.6) kg(range: 5.0 to 30.0 kg) at the time of retransplantation. The pediatric end-stage liver disease(PELD) score was 17.0(21.3) (range: 0 to 45) before retransplantation. The etiology of retransplantation was biliary complications in 7 cases,primary nonfunction of liver graft in 5 cases,antibody-mediated rejection in 4 cases,hepatic artery thrombosis in 3 cases,portal vein thrombosis in 3 cases,concomitant hepatic artery and portal vein thrombosis in 2 cases,thrombogenesis of inferior Vena Cava in 1 case and sinusoidal obstruction syndrome in 1 case. The patients were divided into two groups according to the time interval(30 days) between two liver transplantations,8 patients were classified into early-retransplantation(≤30 days) group and 18 patients were classified into late-retransplantation (>30 days) group. The etiology of liver retransplantation,pre-transplant score,time interval between two transplantations,surgical aspects,major complications and survival rates were compared between the two groups. Continuous variables with normal distribution were compared with t test,while Mann-Whitney U test was applied to compare variables without normal distribution. Categorical variables were compared with chi-square test. The survival curves were created by Kaplan-Meier method and compared by Log Rank test. Results:The median follow-up time was 26.8(30.2) months(range: 1 day to 85.7 months), and the incidence of retransplantation was 1.9%. In the early-retransplantation group,the duration of surgery was (439.8±151.0)minutes,the graft-to-recipient weight ratio was 5.0(1.8)%(range:3.6% to 6.1%),the main cause for retransplantation were primary nonfunction and vascular complications. In the late-retransplantation group,the duration of surgery was (604.4±158.0)minutes,the graft-to-recipient weight ratio was 3.4(2.1)%(range:1.4% to 5.3%),the main cause for retransplantation were biliary complications,antibody mediated rejection and vascular complications.The 3-month,1-year and 2-year recipient survival rates in the early-retransplantation group were all 62.3%,while the recipient survival rates in the late-retransplantation group were 100%,93.8% and 93.8%,respectively. The difference of recipient survival rates was significant between the early-retransplantation group and the late-retransplantation group( P=0.019). The overall 3-month,1-year and 3-year recipient survival rates after the primary liver transplantation were 97.1%,95.4%,94.1%,respectively. Conclusions:The vascular complications,biliary complications,primary nonfunction and antibody-mediated rejection are the main causes of liver retransplantation.The PELD score is higher in patients receiving early retransplantation,while the surgery is relatively more complex in patients receiving late retransplantation,which is reflected by longer duration of surgeries. Patients in the late-retransplantation group showed similar recipient survival rates with primary liver transplantation recipients,and the survival rates are superior to those of patients in the early-retransplantation group. Infection and multiple organ failure are the most common fatal causes after retransplantation.

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 85 th 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.

Objective:To explore the application of extracorporeal membrane oxygenation (ECMO)for severe acute respiratory distress syndrome(ARDS)in children after liver transplantation.Methods:The clinical data were retrospectively analyzed for two ECMO-supported children with severe ARDS after liver transplantation. There were suspected pneumocystis carinii pneumonia(n=1)and identified pneumocystis carinii pneumonia(n=1).Results:Veno-arterial ECMO(VA-ECMO)was performed and oxygen saturation index(OSI)before an initiation of ECMO was 31.8 and 23.9 respectively. Both were successfully separated from ECMO after 219 h and 168 h support respectively, and both were weaned from ventilator after 342 h and 232 h invasive mechanical ventilation respectively. The length of ICU stay was 31 and 18 days and the length of hospital stay 57 and 33 days respectively. During ECMO support, liver function remained stable and there was no new onset of organ dysfunction or life-threatening complications.Conclusions:ECMO is a potential therapy for children with severe ADRS after liver transplantation and the assessment and management of complications with ECMO support should be further studied.

Objective To explore the clinical efficacy of pediatric blood type incompatible living donor liver transplantation. Methods The clinical data from 242 cases of pediatric living donor liver transplantation recipients were retrospectively analyzed. Recipients were assigned to group A (ABO-identical group, n=165), group B (ABO-compatible group, n=42) and group C (ABO-incompatible group, n=35) according to the blood type compatibility between the recipients and the donors. The occurrence of postoperative complications and development of postoperative donor specific antibody (DSA) among the 3 groups were observed and compared. And the blood type distribution of donors and recipients and development of erythrocyte antibodies in group C were analyzed. The survival situation of recipients after liver transplantation was compared among the 3 groups. Results There was no significant difference in the incidence of complications among the 3 groups(all P > 0.05). DSA was dominated by human leukocyte antigen (HLA) Ⅱ antibodies after liver transplantation, mostly anti-HLA-DR and anti-HLA-DQ. The postoperative erythrocyte antibodies for liver transplant recipients in group C were dominated by IgM, with titers ≤1:2 for all. The differences in postoperative survival rates were not statistically significant among 3 groups(all P > 0.05). Conclusions Pediatric blood type incompatible living donor liver transplantation is a safe and effective treatment, which can effectively expand the source of liver transplant donors and save the children's lives.

Objective To evaluate the feasibility and safety of using pediatric donation after brain death donors during split liver transplantation .Methods The clinical data were retrospectively reviewed for 8 pediatric recipients undergoing split liver transplantation with a donor age of 2 .7-7 years .The clinical characteristics of donors/recipients ,perioperative course ,postoperative recovery and complications along with graft and recipient survival rate were analyzed .Results The split procedure was performed ex situ (n=3) and in situ (n=1) ,all liver grafts were split into left lateral lobes and extended right lobes . The recipients were children aged 4 .7-105 .5 months . The mean follow-up period was (8 .1 ± 0 .6) months and the graft/recipient survival rates approached 100％ . Graft functions remained normal in all recipients at the end of follow-ups .Two recipients undergoing liver grafting with long cold ischemia time exhibited slower recovery of graft function .Pathological examination of graft biopsy indicated ischemic and hypoxic changes .Portal vein stenosis occurred in one recipient .Percutaneous transhepatic portal vein balloon dilatation was performed and the recipient recovered well .Cytomegalovirus infection occurred in 5/8 recipients and serum virological marker returned to normal after ganciclovir therapy . The youngest donor age was 2 .7 years and both recipients of donor liver recovered well .Conclusions Split liver transplantation with a donor age of 2 .7-7 .0 years may achieve ideal clinical outcomes in well-matched donors and recipients .

Objective To summarize the experiences of diagnosing and treating portal vein stenosis (PVS) after pediatric liver transplantation from China donation after citizen 's death (CDCD) grafts .Methods Retrospective analysis was performed for 30 cases of pediatric CDCD liver transplantation recipients with PVS .The screening ,diagnosis ,treatment and prognosis of PVS were analyzed .Results Among 218 pediatric liver transplantation recipients with CDCD grafts ,PVS was diagnosed in 30 cases with an incidence rate of 13 .8％ (30/218) .The initial diagnosis of PVS ranged from 5 days to 27 months post-operation with a median age of 2 .9 months .Ultrasonography indicated that stenotic rate of anastomotic site diameter was (41 .28 ± 12 .93)％ and blood flow velocity ratio (358 .77 ± 117 .82)％ .Intervention examination showed average pressure gradient was (9 .06 ± 5 .34) mmHg between both sides of stenosis . All cases underwent percutaneous intrahepatic balloon dilatation .The recipients were followed up for a median follow-up time of 23(3-63) months .For three cases of restenosis ,percutaneous intrahepatic balloon dilatation was repeated .Two cases underwent stent implantation due to ineffective balloon dilation .After treatment ,the stenotic rate of anastomotic site diameter was (34 .69 ± 8 .82) and blood flow velocity ratio (61 .18 ± 63 .11)％ on ultrasound while the average pressure gradient was (1 .03 ± 0 .85) mmHg .Conclusions PVS is a common vascular complication after pediatric CDCD liver transplantation .Portal vein balloon dilation is both safe and efficacious .However ,some cases require repeated balloon dilation and stent implantation serves as the last option for intractable PVS .Color ultrasound is both convenient and effective for making a primary diagnosis and evaluating outcomes .

Objective To analyze the clinical efficacy and prognosis of living donor liver transplantation (LDLT) in children with biliary atresia (BA).Methods The clinical data of 306 cases of BA patients who received LDLT from June 2013 to December 2017 in the Department of Pediatric Liver Transplantation of Tianjin First Center Hospital were retrospectively analyzed.The incidence of post-LDLT complications was summarized and different factors influencing long-term survival of the recipients were analyzed.Results The median age of recipients at transplantation was 7 (6,9) months,and 88.9％ of the recipients received left lateral lobes.The surgical-related complications mainly included lymphatic leakage (30.7％),bile duct stricture (7.8％) and portal vein stenosis (6.9％).The non-surgical-related complications were mainly EBV infection (57.8％) and CMV infection (36.6％).The incidence of pulmonary infection and acute rejection was 18.6％ and 13.7％,respectively.The 1-,3-,and 5-year survival rates of recipients and grafts were 97.2％,97.2％,97.2％ and 97.2％,96.4％,and 94.6％,respectively.A total number of 8 patients died after LDLT,mainly due to the complications of cardio-pulmonary system.Two patients underwent retransplantation due to graft dysfunction caused by antibody-mediated rejection.Recipient age,PELD scores,GRWR,previous surgical history and matching of ABO blood group between donors and recipients did not affect the long-term survival rates of recipients (P＞0.05).Conclusions Children with biliary atresia who received LDLT can obtain satisfactory clinical results.

Objective To explore the diagnosis and treatment of diaphragmatic hernia (DH)secondary to living donor liver transplantation (LDLT) in pediatrics.Methods The primary disease was biliary atresia and all of the 4 patients underwent LDLT using a donor's left lateral graft.The ages of recipients were 5-7 months at LDLT and the onset of DH were 1.5-16 months after LDLT.There were 3 right DH and 1 left DH,and 3 were emergency cases.Results The graft weight and graft to recipient body weight ratio (GRWR) were respectively between 170-290 g and between 2.7％-5.0％.Clinical symptoms included urgent respiratory distress,dyspepsia or gastrointestinal obstruction.DH was diagnosed by computed tomography scan or X-ray of the chest.Laparotomy were performed successfully to repair the DH including emergency laparotomy in three patients.Herniated organs were partial intestines or colon and partial stomach.All cases recovered without major complications.Conclusions DH post-LDLT is an unusual complication,often calls for emergent management.