Long-term outcomes of pediatric liver transplantation in acute liver failure vs end-stage chronic liver disease:A retrospective observational study

BACKGROUND Children with acute liver failure(ALF)who meet the criteria are eligible for super-urgent transplantation,whereas children with end-stage chronic liver disease(ESCLD)are usually transplanted electively.Pediatric liver transplantation(PLT)in ALF and ESCLD settings has been well described in the literature,but there are no studies comparing the outcomes in these two groups.AIM To determine if there is a difference in post-operative complications and survival outcomes between ALF and ESCLD in PLT.METHODS This was a retrospective observational study of all primary PLTs performed at a single center between 2000 and 2019.ALF and ESCLD groups were compared for pretransplant recipient,donor and operative parameters,and post-operative outcomes including graft and patient survival.RESULTS Over a 20-year study period,232 primary PLTs were performed at our center;195 were transplanted for ESCLD and 37 were transplanted for ALF.The ALF recipients were significantly older(median 8 years vs 5.4 years;P=0.031)and heavier(31 kg vs 21 kg;P=0.011).Living donor grafts were used more in the ESCLD group(34 vs 0;P=0.006).There was no difference between the two groups concerning vascular complications and rejection,but there were more bile leaks in the ESCLD group.Post-transplant patient survival was significantly higher in the ESCLD group:1-,5-,and 10-year survival rates were 97.9%,93.9%,and 89.4%,respectively,compared to 78.3%,78.3%,and 78.3%in the ALF group(P=0.007).However,there was no difference in 1-,5-,and 10-year graft survival between the ESCLD and ALF groups(90.7%,82.9%,77.3%vs 75.6%,72.4%,and 66.9%;P=0.119).CONCLUSION Patient survival is inferior in ALF compared to ESCLD recipients;the main reason is death in the 1st year post-PLT in ALF group.Once the ALF children overcome the 1st year after transplant,their survival stabilizes,and they have good long-term outcomes.

Health-related quality of life of pediatric living donor liver transplantation donors who undergone donation surgery for 10 years

Objective:To investigate the quality of life(QOL)of living donor liver transplantation(LDLT)donors.Methods:The Euro Qol 5-dimensional questionnaire(EQ-5D)was used to measure the QOL of donors.Results:One donor repor ted“a little problem”of mobility(MO),and 2 donors(9.1%)repor ted“a little problem”of usual activities(UA).Moreover,there were 8 donors(36.4%)and 7 donors(31.8%)declaring“a little problem”of pain/discomfor t(PD)and anxiety/depression(AD),respectively.And both dimensions have a donor repor ting“moderate problem.”The mean visual analog scale(VAS)was 83.1±12.4.Conclusions:Donors can gain a stable and preferable QOL after donation in both the shor t and long terms.ED-5D application in the field of liver transplant could be an effective choice in QOL studies.

Risk factors for myocardial injury during living donor liver transplantation in pediatric patients with biliary atresia

BACKGROUND Cold ischemia-reperfusion of the liver is an inevitable occurrence in liver trans-plantation that may also cause damage to the heart.Perioperative myocardial injury during liver transplantation can increase the incidence of post-operative mortality,but there is little research on the incidence of myocardial injury in children who undergo living donor liver transplantation(LDLT).Therefore,this study mainly explores the independent risk factors for myocardial injury in children who undergo LDLT.factors for intraoperative myocardial injury.METHODS We retrospectively analyzed the inpatient records of pediatric patients who underwent LDLT in Tianjin First Central Hospital from January 1,2020,to January 31,2022.Recipient-related data and donor-related data were collected.The patients were divided into a myocardial injury group and a nonmyocardial injury group according to the value of the serum cardiac troponin I at the end of surgery for analysis.Univariate analysis and multivariate logistic regression were used to evaluate the risk factors for myocardial injury during LDLT in pediatric patients.RESULTS A total of 302 patients met the inclusion criteria.The myocardial injury group had 142 individuals(47%),and the nonmyocardial injury group included 160 patients(53%).Age,height,and weight were significantly lower in the myocardial injury group(P<0.001).The pediatric end-stage liver disease(PELD)score,total bilirubin,and interna-tional standardized ratio were significantly higher in the myocardial injury group(P<0.001).The mean arterial pressure,lactate,hemoglobin before reperfusion,duration of the anhepatic phase,cold ischemic time,incidence of postreperfusion syndrome(PRS),and fresh frozen plasma transfusion were significantly different between the two groups(P<0.05).The postoperative intensive care unit stay and peak total bilirubin values in the first 5 d after LDLT were significantly higher in the myocardial injury group(P<0.05).The pediatric patients with biliary atresia in the nonmyocardial injury group who underwent LDLT had a considerably higher one-year survival rate than those in the myocardial injury group(P=0.015).Multivariate logistic regression revealed the following independent risk factors for myocardial injury:a high PELD score[odds ratio(OR)=1.065,95%confidence interval(CI):1.013-1.121;P=0.014],a long duration of the anhepatic phase(OR=1.021,95%CI:1.003-1.040;P=0.025),and the occurrence of intraoperative PRS(OR=1.966,95%CI:1.111-3.480;P=0.020).CONCLUSION A high PELD score,a long anhepatic phase duration,and the occurrence of intraoperative PRS were independent risk factors for myocardial injury during LDLT in pediatric patients with biliary atresia.

Comment on pediatric living donor liver transplantation decade progress in Shanghai: Characteristics and risks factors of mortality

Since the first successful liver transplantation was performed five decades ago,pediatric liver transplantation has become the gold standard treatment choice for pediatric liver disease,including metabolic diseases,liver tumors,and some acute liver failure.With improvements in immunosuppression,surgical techniques,and postoperative medical care,long-term outcomes of patients after liver transplantation have markedly improved,especially in pediatric patients.

Differences in parents of pediatric liver transplantation and chronic liver disease patients

BACKGROUND With advancements in the treatment of chronic liver disease(CLD),including liver transplantation(LT),quality of life and satisfaction after LT have become an important issue for pediatric patients and their parents.More evidence-based information is needed to describe and assess the impact of pediatric CLD on parents and the satisfaction of parents with treatment to better understand their needs.AIM To assess the satisfaction of parents of pediatric LT patients and that of parents of pediatric CLD patients METHODS During this survey,data were collected from parents of pediatric patients who underwent LT between January 2010 and April 2017(LT group;n=91)and parents of pediatric patients with chronic liver disease(CLD group;n=94).Group comparisons were made based on the pediatric health-related quality of life(PedsQL)health care parent satisfaction scale,impact on family scale(IFS)and demographic characteristics.The PedsQL was administered to parents during a phone interview and the results were used to assess the health carerelated satisfaction of parents.The IFS was used to assess the impact of the child’s CLD status on the family.Demographic variables such as education level(elementary vs middle vs high vs university),monthly income(low vs middle vs high),and place of residence(village vs town vs city)were compared between CLD and LT parent groups.Finally,PedsQL and IFS results were also analyzed according to demographic variables.RESULTS A total of 185 parents aged 19 to 65 years were included.There were statistically significant differences between the LT and CLD groups in terms of career(P<0.001),monthly income(P=0.016),and education level(P=0.041).According to the PedsQL results,family inclusion,communication,technical skills,emotional needs,and overall satisfaction were significantly different between the groups;the LT group had consistently higher scores(P<0.001).Additionally,scores for the IFS parameters of financial impact,familial-social impact,personal strain,and total impact were consistently higher for the LT group(P<0.001).There were statistically significant relationships between education level,monthly income,and place of residence according to the IFS results but not the PedsQL results.There were inverse relationships between the difficulties that parents experience because of their child’s health and education levels,monthly income,and place of residence.However,no relationship was found between education level,monthly income,or place of residence and satisfaction with health care services provided in the hospital according to the PedsQL results.CONCLUSION Parents of children who underwent LT were very satisfied with the health care services provided to their children.However,they had more difficulties than parents of children with CLD.

De-novo hepatocellular carcinoma after pediatric living donor liver transplantation

De-novo malignancies carry an incidence ranging between 3%-26% after transplant and account for the second highest cause of post-transplant mortality behind cardiovascular disease. While the majority of de-novo malignancies after transplant usually consist of skin cancers, there has been an increasing rate of solid tumor cancers over the last 15 years. Although, recurrence of hepatocellular carcinoma(HCC) is well understood among patients transplanted for HCC, there are increasing reports of de-novo HCC in those transplanted for a non-HCC indication. The proposed pathophysiology for these cases has been mainly connected to the presence of advanced graft fibrosis or cirrhosis and always associated with the presence of hepatitis B or C virus. We report the first known case of de-novo HCC in a recipient, 14 years after a pediatric living related donor liver transplantation for end-stage liver disease due to biliary atresia without the presence of hepatitis B or C virus before and after transplant. We present this case report to increase the awareness of this phenomenon and address on the utility for screening and surveillance of hepatocellular carcinoma among these individuals. One recommendation is to use similar guidelines for screening, diagnosis, and treatment for HCC as those used for primary HCC in the pre-transplant patient, focusing on those recipients who have advanced fibrosis in the allograft, regardless of etiology.

Pediatric and adult liver transplantation in Bahrain: The experiences in a country with no available liver transplant facilities

BACKGROUND Liver transplantation(LT)is a life-saving procedure for patients with end-stage liver disease and has become the standard and most effective treatment method for these patients.There are many indications for LT that vary between countries and settings.The outcome of LT depends on the available facilities and surgical expertise,as well as the types of liver graft donors available.AIM To assess the clinical characteristics of patients from Bahrain who underwent LT overseas,and analyze factors affecting their survival.METHODS In this retrospective cohort study,we reviewed the medical records and overseas committee registry information of all pediatric and adult patients who were sent overseas to undergo LT by the Pediatric and Medical Departments of Salmaniya Medical Complex and Bahrain Defence Force Hospital via the Overseas Treatment Office,Ministry of Health,Kingdom of Bahrain,between 1997 and 2023.Demographic data,LT indication,donor-recipient relationship,overseas LT center,graft type,post-LT medications,and LT complications,were collected.Outcomes measured included the overall and 5-year LT survival rate.Fisher’s exact,Pearsonχ2,and Mann-Whitney U tests were used to compare the pediatric and the adults’group in terms of clinical characteristics,donor-recipient relationship,medication,complications,and outcome.Survival analysis was estimated via the Kaplan-Meier’s method.Univariate and multivariate analyses were used to detect predictors of survival.RESULTS Of the 208 eligible patients,170(81.7%)were sent overseas to undergo LT while 38(18.3%)remained on the waiting list.Of the 170 patients,167(80.3%)underwent LT and were included in the study.The majority of the patients were Bahraini(91.0%),and most were males(57.5%).One-hundred-and-twenty(71.8%)were adults and 47(28.3%)were children.The median age at transplant was 50.0[interquartile range(IQR):14.9–58.4]years.The main indication for pediatric LT was biliary atresia(31.9%),while that of adult LT was hepatitis C-related cirrhosis(35.0%).Six(3.6%)patients required re-transplantation.Most patients received a living-related liver graft(82%).Pediatric patients received more living and related grafts than adults(P=0.038 and P=0.041,respectively),while adult patients received more cadaveric and unrelated grafts.Most patients required long-term immunosuppressive therapy after LT(94.7%),of which tacrolimus was the most prescribed(84.0%),followed by prednisolone(50.7%),which was prescribed more frequently for pediatric patients(P=0.001).Most patients developed complications(62.4%)with infectious episodes being the most common(38.9%),followed by biliary stricture(19.5%).Tonsilitis and sepsis(n=12,8.1%for each)were the most frequent infections.Pediatric patients experienced higher rates of infection,rejection,and early poor graft function than adult patients(P<0.001,P=0.003,and P=0.025,respectively).The median follow-up time was 6.5(IQR:2.6–10.6)years.The overall survival rate was 84.4%,the 5-year survival rate,86.2%,and the mortality rate,15.6%.Younger patients had significantly better odds of survival(P=0.019)and patients who survived had significantly longer follow-up periods(P<0.001).CONCLUSION Patients with end-stage liver disease in Bahrain shared characteristics with those from other countries.Since LT facilities are not available,an overseas LT has offered them great hope.

Protocol liver biopsy is the only examination that can detect mid-term graft fibrosis after pediatric liver transplantation

AIM: To assessed the clinical significance of protocol liver biopsy (PLB) in pediatric liver transplantation (LT).

Risk factors for de novo hepatitis B infection in pediatric living donor liver transplantation

AIM: To investigate the incidence of de novo hepatitis B virus (HBV) infection after pediatric living donor liver transplantation (LDLT) and to analyze the risk factors associated with this de novo HBV infection.

Hepatitis C in the pediatric population: Transmission, natural history, treatment and liver transplantation

The number of children affected by the hepatitis C virus (HCV) in the United States is estimated to be between 23000 to 46000. The projected medical cost for children with HCV in the United States is upwards of 200 million over the next decade. The implementation of routine screening of blood supply has virtually eliminated transmission via transfusion and vertical transmission is now the most common mode of infection in children. Infections acquired during infancy are more likely to spontaneously resolve and fibrosis of the liver tends to increase with age suggesting slow progressive histologic injury. Anti-viral treatment may be warranted in children with persistently elevated liver enzymes or with significant fibrosis on liver biopsy. Current standard of care includes weekly pegylated interferon and ribavirin twice daily. Predictors of high sustained viral response include genotype 2 and 3 and low viral load in children with genotype 1 (&#x0003c; 600000 IU/mL). Triple therapy is associated with a significantly higher rate of sustained virologic response (&#x0003e; 90%). Only 34 pediatric patients were transplanted with hepatitis C between January 2008 and April 2013. The majority of pediatric patients were born prior to universal screening of blood products and, as of June 2013, there are only two pediatric patients awaiting liver transplantation for end-stage liver disease secondary to hepatitis C. Pediatric survival rates post-transplant are excellent but graft survival is noticeably reduced compared to adults (73.73% for pediatric patients at one year compared to 87.69% in adult patients). New safe potent, and all-oral effective antiviral therapies for recurrent HCV should help increase graft survival.

Balloon dilatation for treatment of hepatic venous outflow obstruction following pediatric liver transplantation

AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures(two patients underwent two balloon dilatation procedures) were included in this analysis. The demographic data,types of donor and liver transplant, interventional examination and therapeutic outcomes of these five children were analyzed. The median interval time between pediatric liver transplantation and balloon dilatation procedures was 9.8 mo(range: 1-32).RESULTS Five children with HVOO were successfully treated by balloon angioplasty without stent placement, with seven procedures performed for six stenotic lesions. All children underwent successful percutaneous intervention. Among these five patients, four were treated by single balloon angioplasty, and these patients did not develop recurrent stenosis. In seven episodes of balloon angioplasty across the stenosis, the pressure gradient was 12.0 ± 8.8 mm Hg before balloon dilatation and 1.1 ± 1.5 mm Hg after the procedures, which revealed a statistically significant reduction(P < 0.05). The overall technical success rate among these seven procedures was 100%(7/7), and clinical success was achieved in all five patients(100%). The patients were followed for 4-33 mo(median: 15 mo). No significant procedural complications or procedurerelated deaths occurred.CONCLUSION Balloon dilatation is an effective and safe therapeutic option for HVOO in children undergoing pediatric liver transplantation. Venous angioplasty is also recommended in cases with recurrent HVOO.

Hepatic artery reconstruction in pediatric liver transplantation:Experience from a single group

Background:The reconstruction of hepatic artery is a challenging part of the pediatric liver transplantation procedure.Hepatic artery thrombosis(HAT)and stenosis are complications which may result in ischemic biliary injury,causing early graft lost and even death.Methods:Two hundred and fifty-nine patients underwent liver transplantation in 2017 in a single liver transplantation group.Among them,225 patients were living donor liver transplantation(LDLT)and 34 deceased donor liver transplantation(DDLT).Results:In LDLT all reconstructions of hepatic artery were microsurgical,while in DDLT either microsurgical reconstruction or traditional continuous suture technique was done depending on different conditions.There were five(1.9%)HATs:four(4/34,11.8%)in DDLT(all whole liver grafts)and one(1/225,0.4%)in LDLT(P=0.001).Four HATs were managed conservatively using anticoagulation,and 1 accepted salvage surgery with re-anastomosis.Until now,3 HAT patients remain in good condition,whereas two developed biliary complications.One of them needed to be re-transplanted,and the other patient died due to biliary complications.Conclusions:Microsurgical technique significantly improves the reconstruction of hepatic artery in pediatric liver transplantation.The risk for arterial complications is higher in DDLT.Conservative therapy can achieve good outcome in selected HAT cases.

Perioperative blood transfusion decreases long-term survival in pediatric living donor liver transplantation

BACKGROUND The impact of perioperative blood transfusion on short-and long-term outcomes in pediatric living donor liver transplantation(PLDLT)must still be ascertained,mainly among young children.Clinical and surgical postoperative complications related to perioperative blood transfusion are well described up to three months after adult liver transplantation.AIM To determine whether transfusion is associated with early and late postoperative complications and mortality in small patients undergoing PLDLT.METHODS We evaluated the effects of perioperative transfusion on postoperative complications in recipients up to 20 kg of body weight,submitted to PLDLT.A total of 240 patients were retrospectively allocated into two groups according to postoperative complications:Minor complications(n=109)and major complications(n=131).Multiple logistic regression analysis identified the volume of perioperative packed red blood cells(RBC)transfusion as the only independent risk factor for major postoperative complications.The receiver operating characteristic curve was drawn to identify the optimal volume of the perioperative RBC transfusion related to the presence of major postoperative complications,defining a cutoff point of 27.5 mL/kg.Subsequently,patients were reallocated to a low-volume transfusion group(LTr;n=103,RBC≤27.5 mL/kg)and a high-volume transfusion group(HTr;n=137,RBC>27.5 mL/kg)so that the outcome could be analyzed.RESULTS High-volume transfusion was associated with an increased number of major complications and mortality during hospitalization up to a 10-year follow-up period.During a short-term period,the HTr showed an increase in major infectious,cardiovascular,respiratory,and bleeding complications,with a decrease in rejection complications compared to the LTr.Over a long-term period,the HTr showed an increase in major infectious,cardiovascular,respiratory,and minor neoplastic complications,with a decrease in rejection complications.Additionally,Cox hazard regression found that high-volume RBC transfusion increased the mortality risk by 3.031-fold compared to low-volume transfusion.The Kaplan-Meier survival curves of the studied groups were compared using log-rank tests and the analysis showed significantly decreased graft survival,but with no impact in patient survival related to major complications.On the other hand,there was a significant decrease in both graft and patient survival,with high-volume RBC transfusion.CONCLUSION Transfusion of RBC volume higher than 27.5 mL/kg during the perioperative period is associated with a significant increase in short-and long-term postoperative morbidity and mortality after PLDLT.

Effect of remote ischemic preconditioning among donors and recipients following pediatric liver transplantation:A randomized clinical trial

BACKGROUND Studies suggested that remote ischemic preconditioning(RIPC)may effectively lessen the harmful effects of ischemia reperfusion injury during organ transplantation surgery.AIM To investigate the protective effects of RIPC on living liver donors and recipients following pediatric liver transplantation.METHODS From January 2016 to January 2019 at Renji Hospital Affiliated with Shanghai Jiao Tong University School of Medicine,208 donors were recruited and randomly assigned to four groups:S-RIPC group(no intervention;n=55),D-RIPC group(donors received RIPC;n=51),R-RIPC group(recipients received RIPC,n=51)and DR-RIPC group(both donors and recipients received RIPC;n=51).We primarily evaluated postoperative liver function among donors and recipients and incidences of early allograft dysfunction,primary nonfunction and postoperative complications among recipients.RESULTS RIPC did not significantly improve alanine transaminase and aspartate aminotransferase levels among donors and recipients or decrease the incidences of early allograft dysfunction,primary nonfunction,and postoperative complications among recipients.Limited protective effects were observed,including a lower creatinine level in the D-RIPC group than in the S-RIPC group on postoperative day 0(P<0.05).However,no significant improvements were found in donors who received RIPC.Furthermore,RIPC had no effects on the overall survival of recipients.CONCLUSION The protective effects of RIPC were limited for recipients who received living liver transplantation,and no significant improvement of the prognosis was observed in recipients.

Preoperative hepatic hemodynamics in the prediction of early portal vein thrombosis after liver transplantation in pediatric patients with biliary atresia

BACKGROUND： Portal vein thrombosis （PVT） is one of the main vascular complications after liver transplantation （LT）, especially in pediatric patients with biliary atresia （BA）. This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT.METHODS： One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultra- sound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT （within 1 month following LT） and those without PVT. Receiver oper- ating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. RESULTS： Of the 128 transplant recipients, 41 （32.03%） had a hypoplastic portal vein （PV）, 52 （40.63%） had hepatofugal PV flow and 40 （31.25%） had a high hepatic artery resistance index （HARI） of 〉1. Nine cases （7.03%） experienced early PVT. A PV diameter 〈4 mm （sensitivity 88.89%, specificity 72.27%）, and a hepatofugal PV flow （sensitivity 77.78%, specificity 62.18%） with a high HARI ≥1 （sensitivity 77.78%, specificity 72.27%） were hepatic hemodynamic risk factors for early PVT.（ CONCLUSIONS： Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter （≤4 mm） and hepatofugal PV flow combined with high HARI （≥1） are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.

Novel approach for the diagnosis of occult cytomegalovirus cholangitis after pediatric liver transplantation:A case report

BACKGROUND Cytomegalovirus(CMV)infection is a common infection in liver transplant recipients,which is related to chronic rejection and biliary complications.It is often diagnosed based on serum CMV-DNA or CMV pp65.To our knowledge,this is the first report of the successful treatment of occult CMV cholangitis in a pediatric liver transplantation(LT)recipient.CASE SUMMARY A 7-mo-old baby girl received LT due to biliary atresia and cholestasis cirrhosis.At 1 mo following LT,the patient suffered from aggravated jaundice with no apparent cause.As imaging results showed intrahepatic and extrahepatic bile duct dilatation,the patient was diagnosed with biliary complications and percutaneous cholangiography and biliary drainage was performed.However,there was little biliary drainage and her liver function deteriorated.CMV-DNA was isolated from the bile with the surprising outcome that 3×106 copies/mL were present,whereas the CMV-DNA in serum was negative.Following antiviral therapy with ganciclovir,she gradually recovered and bilirubin decreased to normal levels.During the 4-year follow-up period,her liver function remained normal.CONCLUSION Bile CMV sampling can be used for the diagnosis of occult CMV infection,especially in patients with negative serum CMV-DNA and CMV pp65.Testing for CMV in the biliary tract may serve as a novel approach for the diagnosis of cholestasis post-LT.Timely diagnosis and treatment will decrease the risk of graft loss.

Preoperative nutrition and postoperative liver function:a correlation study of pediatric living donor liver transplantation

Objective： There is little information focusing on the nutritional issue of pediatric recipients before they receive living donor liver transplantation. This study illustrates the relationship between nutritional status and graft liver function and provides a reference regarding nutritional interventions in future studies.Methods： We prospectively collected data from 30 pediatric living donor liver transplant recipients from January 1, 2016, to June 30, 2016. The information included demographic data, preoperative nutritional assessment, and postoperative laboratory examinations. The nutritional assessment included the serum concentration of vitamin D, bone density, trace element, and weight Z value. The laboratory examinations included white blood cell count, neutrophil percentage, hemoglobin, blood platelet, total protein, albumin, total bilirubin, direct bilirubin, alanine transaminase, aspartate aminotransferase （AST）, alkaline phosphatase, gamma-glutamyl transpeptidase, creatinine, bile acid, blood glucose （Glu）, prothrombin time, international normalized ratio, tacrolimus concentration, and graft-to-recipient weight ratio （GRWR）. The data were collected on Days 1 ,2 ,3 ,4 ,5 ,6 ,7 ,1 4 ,3 0 , and 60 after liver transplantation. Results： The recipients consisted of 15 （50%） males and 15 （50%） females. K median age was 7 months （4-48 months）. The mean height and weight were 69.07±9.98 cm and 8.09±2.63 kg, respectively. According to the univariate analysis, the gender, diagnosis, blood type, and GRWR did not significantly impact the liver function after the operation. The posttransplantation AST levels and Glu showed significant differences in terms of the nutritional status, with P〈0.05. The multivariate correlation analysis showed that the serum concentrations of vitamin D and AST were midrange positively correlated, with P〈0.05.Conclusions： The nutritional status of patients with biliary atresia is relatively poor. There is a definite midrange positive correlation between nutrition and graft liver function that might play a relatively important role in the recovery of the graft.

Laparoscopic left lateral sectionectomy in pediatric living donor liver transplantation by single-port approach:A case report

BACKGROUND Single-port laparoscopy has been used in a variety of abdominal operations.We report the first case of single-port laparoscopic left lateral sectionectomy in pediatric laparoscopic living donor liver transplantation.CASE SUMMARY A 28-year-old man volunteered for living liver donation to his daughter who was diagnosed with liver cirrhosis and portal hypertension after the Kasai procedure for biliary atresia.His body mass index was 20.5 kg/m2.Liver dynamic computed tomography showed:(1)Left lateral graft volume of 232.76 cm3 with a graft-torecipient weight ratio of 2.59%;and(2)Right hepatic artery derived from the superior mesenteric artery.A single-port access system was placed through a transumbilical incision,including four trocars:two 12-mm ports for a camera and endoscopic stapler and two 5-mm working ports.Liver parenchyma was dissected by a Harmonic and Cavitron Ultrasonic Surgical Aspirator,while bipolar was used for coagulation.The bile duct was transected above the bifurcation by indocyanine green fluorescence cholangiography.The specimen was retrieved from the umbilical incision.The total operation time was 4 h without blood transfusion.The final graft weight was 233.6 g with graft-torecipient weight ratio of 2.60%.The donor was discharged uneventfully on postoperative day 4.CONCLUSION Single-port laparoscopic left lateral sectionectomy is feasible in pediatric laparoscopic living donor liver transplantation in an experienced transplant center.

Compromised therapeutic value of pediatric liver transplantation in ethylmalonic encephalopathy: A case report

BACKGROUND Ethylmalonic encephalopathy(EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progressive psychomotor regression and neurological impairment usually contribute to early death in the first decade of life. Liver transplantation(LT) is emerging as a novel therapeutic option for EE;however, worldwide experience is still limited.CASE SUMMARY An 18-mo-old patient with the diagnosis of EE received a living donor liver transplant in our institution, which, to our knowledge, is the first case in AsianPacific countries. During 20 mo of follow-up, the longitudinal metabolic evaluations revealed a wild fluctuation in urinary EMA levels, still far beyond the normal range. Urinary 2-methylsuccinic acid levels gradually restored to normal, whereas the concentrations of urinary isobutyrylglycine and plasma C4-and C5-acylcarnitines fluctuated markedly and still remained above the reference limits. Only mild amelioration of petechiae and ecchymosis was observed, and no dramatic reversion of chronic mucoid diarrhea and orthostatic acrocyanosis occurred. Although brain magnetic resonance imaging suggested a certain improvement in basal ganglia lesions, the patient still presented developmental delay and neurologic disability.CONCLUSION LT may bring about a partial but not complete cure to EE. Given its definite role in defending against the devastating natural progression of EE, LT should still be considered for patients with EE in the absence of other superior therapeutic options. Early establishment of diagnosis and initiation of conventional treatment pre-transplant, timely LT, and continuous administration of metabolismcorrecting medications post-transplant may be helpful in minimizing the neurologic impairment and maximizing the therapeutic value of LT in EE.

Liver transplantation for pediatric inherited metabolic liver diseases

Liver transplantation(LT)remains the gold standard treatment for end stage liver disease in the pediatric population.For liver based metabolic disorders(LBMDs),the decision for LT is predicated on a different set of paradigms.With improved outcomes post-transplantation,LT is no longer merely life saving,but has the potential to also significantly improve quality of life.This review summarizes the clinical presentation,medical treatment and indications for LT for some of the common LBMDs.We also provide a practical update on the dilemmas and controversies surrounding the indications for transplantation,surgical considerations and prognosis and long terms outcomes for pediatric LT in LBMDs.Important progress has been made in understanding these diseases in recent years and with that we outline some of the new therapies that have emerged.