Objective To evaluate the clinical efficacy and prognosis of liver transplantation for langerhans cell histiocytosis（LCH）in children with liver cirrhosis. Methods The clinical data and treatment experience of 10 cases of liver transplantation for LCH in children with liver cirrhosis in the Tianjin First Central Hospital were descriptively analyzed. Results All children were in decompensated stage of liver cirrhosis caused by LCH before liver transplantation. Among the 10 LCH patients，7 patients underwent cadaveric liver transplantation，3 patients underwent living donor liver transplantation. The type of graft was left lateral lobe in 7 cases，whole liver in 2 cases，and right hemi-liver in 1 case. The graft to recipient weight ratio ranged from 1.41% to 4.53%. The valuesof alanine transaminase（ALT），aspartate aminotransferase（AST），and total bilirubin（TBil）started to decreasefrom the first postoperative day and continued with a downward daily trend in most recipients. The median followup time was 12.11 months. Two patients presented with extrahepatic recurrence and were well-controlled after theadministration of chemotherapy and targeted drugs. The survival rate of the patients after liver transplantation was90%. Conclusion Liver transplantation is an effective treatment for the decompensated stage of liver cirrhosis caused by LCH in children，a better survival outcome may be achieved. 

Objective To evaluate the clinical efficacy of liver transplantation in the treatment of ornithinetranscarbamylase deficiency（OTCD）. Methods This study retrospectively analyzed 5 children with OTCD induced hyperammonemia who underwent liver transplantation in our center，surgical related complications，postoperative blood ammonia levels，and liver function recovery were analyzed. Results All 5 patients received liver transplantation treatment，with 1 patient underwent secondary cholangiojejunostomy due to biliary fistula and 1 patient underwent secondary liver transplantation due to primary graft failure. All 5 patients had a rapid decrease in postoperative blood ammonia levels，which returned to normal levels within 14 days after surgery. Liver functionreturned to normal levels within 9 days，and partial relief of neurological damage was observed during long-termfollow-up. Conclusion Liver transplantation is a safe and effective treatment for OTCD patients，complete cure and improved their long-term quality of life could be achieved. 

Objective To summarize the genotype and phenotype characteristics of children with coenzyme Q8B（COQ8B）-associated nephropathy and the prognosis related to kidney transplantation，in order to provide reference for the diagnosis and treatment of patients with COQ8B-associatednephropathy. Methods The genotypes，phenotypes and prognosis of children with COQ8B-relatednephropathy who underwent kidney transplantation in the First Affiliated Hospital of Sun Yat-sen University from 2017 to 2022 were retrospectively analyzed. The inclusion criteria of the patients were carriers of pathogenic mutation of COQ8B detected by next-generation sequencing（NGS）. Results A total number of18 children with COQ8B-associated nephropathy were included in the study，including 13 children with compound heterozygous mutation of COQ8B and 5 with homozygous mutation of COQ8B，c.748G ＞ C was the most commonmutation in this cohort. The average age of disease onset was（9.09±2.57）years，and the average age of reaching end-stage renal disease was（9.72±2.84）years. Among the 18 cases，15 cases showed isolated nephropathy and3 cases had extrarenal organ involvement. The average follow-up time was 31.7 months，during the follow-up period，only one patient had rejection，and there was no recurrence of primary nephropathy and renal allograft failure. Thegrowth of the children was improved after operation. The median height-for-age z-score（HAZ）before operation was -1.47（-1.95，-0.9），the median HAZ one year after operation was -1.25（-1.77，-0.7），and the median HAZ of the second year after operation was -0.91（-1.24，-0.34）. Conclusion COQ8B-associated nephropathy is characterized by isolated nephrotic syndrome and less involvement of extrarenal organs. Kidney transplantation is a safe and effective treatment for children who are already in the stage of advanced nephropathy.

Objective To summarize the perioperative characteristics and management of isolatedmethylmalonic acidemia after liver transplantation. Methods A total of 13 MMA patients undergoing LT wereenrolled in our study from December 2014 to December 2019，the clinical characteristics，laboratory datas，chronological changes in urine MMA levels and treatment were analyzed retrospectively. Results All the 13 caseswere diagnosed with isolated MMA. Five cases were received deceased donor liver transplantation（DDLT），and eight cases received living donor liver transplantation（LDLT）. Within the early days after LT surgery，metabolic condition fluctuated. The mean base excess of blood value and plasma bicarbonate（HCO3 - ）restored to normal graduallywithin two weeks after LT. Our study also indicated that propionyl carnitine（C3）and C3/C2 level showed markedreduction after LT. The mean urine MMA was reduced by 82.41% compared with the urine level of MMA before liver transplantation（P ＜ 0.01），but remained ＞ 63 folds higher than upper limit of normal. The two patients with renal insufficiency before LT maintained stable by adjusting the immunosuppressive regimen during the observation period. Imaging changes of the nervous system were observed in 2 cases. All patients survive to date. Conclusion LT is regarded as an effective treatment to prevent metabolic crisis of MMA，we should give closely monitor the blood gas analysis to avoid the acidosis episodes by implementing sodium bicarbonate. Personalized immunosuppressive regimen should be developed for those MMA patients with kidney dysfunction. 

Objective To explore the therapeutic benefit of liver transplantation（LT）on congenital bile acid synthesis disorder（CBAS）. Methods The clinical data of 5 cases with CBAS who underwent liver transplantation at Huashan Hospital affiliated to Fudan University from October 2015 to December 2022，were analyzed retrospectively. Among them，there was 1 patient with CBAS type 1，2 patients with CBAS type 2 and 2 patients with cerebrotendinous xanthomatosis（CTX）. There were 3 males and 2 females. The median age was 9（7 ~ 12）months. There were 4 cases of living donor liver transplantation and 1 case of donation after citizen's death（DCD）liver transplantation. The median follow-up time after surgery was 74（60 ~ 84）months. The preoperative clinical characteristics，intraoperative conditions，postoperative follow-up data of the children were analyzed to evaluate the efficacy of liver transplantation. Results All the surgeries were completed successfully，and one child had rejection，one child had chyllesomerhea，and two children were infected with hepatitis B virus（HBV）. After surgery，one child died due to lung infection，and the remaining 4 children recovered their liver function welland survived normally. Symptoms suchasjaundice and itching subsided，and their growth and development improved. Conclusion Liver transplantation is aneffective treatment for children withCBAS whodo notrespond well to medicaltreatment. 

Objective To explore the risk factors of cytomegalovirus（CMV）infection after liver transplantation by analyzing the difference of clinical characteristics and the intrapatient variability（IPV）intacrolimus of the recipients after liver transplantation. Methods A retrospective cohort study was conducted inpatients who underwent liver transplantation in the Liver transplantation center of the First Affiliated Hospital of Xi'an Jiaotong University from January 2015 to December 2019. Among the patients of tacrolimus-based immunosuppressionregiments，290 cases were included according to inclusion and exclusion criteria，and were divided into infection group and control group according to the occurrence for CMV infection. Tacrolimus IPV expressed as the coefficientof variation（CV）was calculated from at least 3 tacrolimus trough levels obtained between months 2 and 6 after liver transplantation，P ＜ 0.05 was considered statistically significant. Results As of December 31 2021，290 liver transplant recipients included in the study，the follow-up ranged from 6 to 83 months. The survival time of recipients in CMV infection group was shorter than that in control group〔（1 707.08±225.96）d vs.（2 214.16±45.03）d，P＜0.05〕.The clinical data of recipients in the CMV infection group and the control group included basic characteristics of donor and recipient，operation-related data，postoperative complications，and the tacrolimus C0 of 2 to 6 months after surgery，there were no significant differences（P ＞ 0.05）. However，the incidence of severe infection and acute rejection in CMV infection group were higher than that in control group（31.58% vs. 4.43%，15.79% vs. 0.74%， P ＜ 0.05）. The influence of some variables on the occurrence of CMV infection after liver transplantation was analyzed by the Cox proportional hazards model analyzed. The results showed that the recipients with high tacrolimus CV had a significantly increased risk of CMV infection after liver transplantation（P ＜ 0.05）. Furthermore，the results showed that CMV-IgM positivity before of liver transplantation，postoperative ICU stay time and tacrolimus CV within 2 to 6 months were an independent risk factors for CMV infection after liver transplantation（all P ＜ 0.05）. Conclusion The preoperative CMV-IgM in liver transplant recipients，the time of ICU stay and the high tacrolimus IPV（CV ≥ 34.36%）within 2 to 6 months after surgery were significantly associated with CMV infection after liver transplantation. CMV infection may lead to an increased incidence of severe infection and acute rejection after livertransplantation. 

Objective To investigate the effect of tacrolimus on intestinal flora in donation after circulatorydeath（DCD）rats with acute rejection after liver transplantation. Methods To establish a stable normothermic machine perfusion（NMP）system. The thoracic aorta of Lewis rats was clamped for 30 minutes to obtain the DCD livers. The acute rejection model of orthotopic liver transplantation was established with brown norway（BN）rats as recipients. According to different treatment methods of liver，BN rats were divided into the following three groups ：sham operation（Sham）group，simple NMP（NMP）group and NMP combined with postoperative tacrolimus（FMP）group，with 6 rats in each group. Blood，liver and intestinal content samples were collected 14 days after operation. The liver function of blood samples was detected，the histological changes of liver were observed by HE staining，and the rejection activity index （RAI）was calculated. The intestinal contents were detected by 16SrDNA，and the changes of intestinal florastructure，diversity and function in each group were observed. Results The survival timeof ratsin FMP group（median survival time ＞ 60 d）was longer than that in NMP group（median survival time ＝ 16.5 d），and the liver function and liverhistopathology were significantly improved than those in NMP group（P ＜ 0.05）. The results of RAI showed that FMP group（2.00±1.23）was significantly lower than NMP group（7.60 ± 1.14）（P ＜ 0.05）. Compared with the NMP group，the abundance of intestinal flora in the FMP group was significantly decreased（P ＜ 0.05），and there were significant differences between the two groups. FMP group was rich in Firmicutes，Akkermansiaceae andLachnospiraceae，as well as the other glycan degradation and sphingolipid metabolism pathways.Conclusion Tacrolimus can improve liver injuryin rats withacute rejection after transplantation，andregulate the structure of intestinal flora and metabolic pathway. 

Objective Through a retrospective analysis of clinical data of patients after lung transplantation，the correlation between tacrolimus trough concentration and early prognosis after lung transplantationwas analyzed. Methods From January 1，2017 to December 1，2020，the clinical data of 64 patients with good earlyprognosis of lung transplantation surgery（survival at 30 d）and 36 patients with poor prognosis in the early stage of lung transplantation surgery（30 d of non-survival）were admitted to the Department of Critical Care Medicine of Wuxi People'sHospital. According to the tacrolimus trough concentration on the first day after surgery，the patients were divided into low tacrolimus group（＜ 10 ng/ml）and high tacrolimus group（≥ 10 ng/ml）for prognostic analysis. The efficacy of tacrolimus and other clinical factors in predicting patients after lung transplantation outcomes were determined using univariate and multivariate Cox proportional hazard analyses. Results There was a significant difference in survival at30 days after lung transplantation between the high and low tacrolimus group（P ＜ 0.05）. According to the 30 d survival group comparison，there were significant statistical differences（P ＜ 0.05）in preoperative alanine aminotransferase，aspartate aminotransferase，tacrolimustrough concentration on the first postoperative day，acute renal insufficiency，postoperative time of mechanical ventilation，number of days in intensive care uint and primary graft dysfunction（PGD），and univariate Cox regression analysis showed that preoperative alanine aminotransferase，glutamate aminotransferase. The effect of tacrolimus trough concentration on survival time on the first postoperative day was statistically significant. Multivariate Cox regressionanalysis showed that tacrolimus trough concentration on the first postoperative day was not an independent risk factor for survival time. Conclusion Tacrolimus concentration hassome value in the early prognosis of patients after lung transplantation. 

Objective To explore the correlations of belief ，outcome and adherence to immunosuppressantsin heart transplant recipients. Methods A survey was conducted on 116 heart transplant recipients. The instruments included a self-designed general information questionnaire，the Basel Assessment of Adherence with Immunosuppressive Medication Scale（BAASIS）and Beliefs about Medication Questionnaire（BMQ）. Results 58.62% heart transplant recipients were non-adherent. Therecipients score was（11.12±2.83）on harm of medication，（10.07±2.62）on overuse of medication，（18.19±2.43）on necessity of prescribed medication and（16.21±3.89）on concerns about potentialadverse consequences. The mean necessity-concerns differential was（1.98±5.03）. The scores of overuse，concerns andnecessity-concerns differential between the adherent and non-adherent recipients had significant differences（P ＜ 0.05）. Medication adherence was positively correlated with rejection andunplanned admission，and negatively correlatedwithpostoperative complications（P ＜0.05）. Conclusion Medication adherence is closely related to medication belief and patient outcomes. It is necessary to improve the medication belief of patients and promote their medication adherence，so as to improve patientoutcomes.