Diaphragmatic hernia (DH) is one of the rare complications without the confirmed risk factors following pediatric liver transplantation (pLT). To analyze the clinical characteristics and risk factors of DH after pLT. A retrospective analysis of 15 DH cases after pLT among 1,863 cases of pLT from January 2014 to June 2024 was conducted. Using propensity score matching (PSM), DH patients (n = 15) were matched 1:3 with non-DH recipients (n = 45). DH cases were stratified into early-onset (≤ 3 months, n = 6) and late-onset (> 3 months, n = 9) groups according to the interval from LT surgery to DH diagnosis. Comparative analyses were performed to characterize DH and identify risk factors. DH incidence was approximately 0.8

BACKGROUND:The size mismatch between a liver graft and the recipient can result in complications and poor survival after pediatric whole liver transplantation (WLT). METHODS:A retrospective study was designed, which included 114 recipients. Multiple variable predictors of graft loss suggested the graft-to-native-liver weight ratio (GNLWR), and then the GNLWR was calculated and categorized into 2 groups. The demographic, operation, complications, and survival analysis data were collected and compared. RESULTS:Group 1 with a GNLWR < 0.41 had a higher incidence of hepatic artery thrombosis (HAT) and lower graft survival in the first 3 months (70.4% vs 96.6%, P < .001). Patient survival at 3 months was significantly different between groups (85.2% vs 97.7%, P = .010). As for the graft survival rate, it was 70.4% for group 1 at both 1 and 3 years, whereas it was 94.2% for group 2 at both 1 and 3 years. Additionally, the patient survival rate for group 1 was 85.2% at both 1 and 3 years, whereas for group 2, it was 95.4% at both 1 and 3 years. Further analysis showed the PELD score was the only independent risk factor for graft loss in the group with GNLWR < 0.41. It indicated a worse prognosis when the PELD score was more than 23.5. CONCLUSIONS:A GNLWR of less than 0.41 suggested a poor prognosis for grafts in pediatric WLT recipients with biliary atresia weighing less than 10 kg. Reducing blood transfusions may help improve graft survival.

OBJECTIVES:Biliary strictures (BS) remain a common and serious complication after pediatric liver transplantation (pLT), potentially leading to recurrent cholangitis and graft failure. Prompt diagnosis and appropriate management are essential to improve outcomes. METHODS:This retrospective, single-center study included 1454 pediatric liver transplants performed between January 2013 and December 2021. BS were classified as anastomotic strictures (AS), non-anastomotic strictures (NAS), or intraoperative technical misadventure (ITM). Cases involving both AS and NAS were classified under the BOTH group. A standardized percutaneous transhepatic cholangiography drainage (PTCD)-based protocol was applied: mild strictures were treated with PTCD alone, whereas severe strictures required balloon dilatation (BD) with external drainage. Surgical revision was performed when guidewire passage failed. Outcomes included stricture resolution, recurrence, and graft survival. RESULTS:BS occurred in 120 patients, with AS accounting for 63.3%, NAS & BOTH for 31.7%, and ITM for 5.0%. The overall incidence of BS declined significantly from 12.38% (2013-2016) to 7.07% (2017-2021). PTCD ± BD achieved an 85.7% resolution rate in AS, while surgical reanastomosis was successful in all refractory cases. NAS & BOTH were associated with significantly lower graft survival and showed limited response to both minimally invasive and surgical treatments. CONCLUSIONS:Minimally invasive approaches, particularly PTCD ± BD, combined with timely surgical intervention, is effective for AS after pLT. However, NAS & BOTH remain challenging and are associated with inferior graft outcomes, underscoring the need for improved early detection and the development of novel therapeutic strategies.

BackgroundIn pediatric liver transplantation (pLT), long-term immunosuppression (IS) contributes to infection risk and chronic toxicity, yet IS minimization or withdrawal requires balancing rejection risk. Practical biomarkers for baseline risk stratification at the start of planned withdrawal remain scarce. This study investigated whether baseline neutrophil-associated proteomic signatures and histone deacetylase 1 (HDAC1) levels are associated with IS withdrawal outcomes.MethodsWithin an institutional IS withdrawal program (n = 77), 59 recipients had evaluable outcomes by the follow-up cut-off (June 30, 2025). Among these, 31 pLT recipients underwent planned IS withdrawal (primary analytic cohort), and baseline plasma from 10 patients was analyzed via liquid chromatography-mass spectrometry (LC-MS) proteomics to identify tolerance-associated proteins and pathways. HDAC1 was subsequently quantified by ELISA in 39 recipients with baseline plasma available. The diagnostic performance of HDAC1 in distinguishing immune-tolerant (IT) from non-immune-tolerant (NIT) outcomes was evaluated using receiver operating characteristic (ROC) analysis. To corroborate tissue-level consistency, HDAC1 expression was assessed by immunohistochemistry (IHC) in baseline liver biopsy sections from 10 recipients (5 IT and 5 NIT) selected from the planned withdrawal cohort.ResultsProteomic profiling revealed distinct baseline differences enriched in neutrophil-related functions, including pathways linked to degranulation and neutrophil extracellular trap (NET) formation. HDAC1 was identified as a key candidate marker, with significantly lower baseline levels observed in the IT group. In the validation cohort, plasma HDAC1 demonstrated moderate discriminative performance for baseline risk stratification (AUC = 0.81). Furthermore, IHC analysis of baseline liver biopsies showed lower intrahepatic HDAC1 staining in IT recipients compared to the NIT group, consistent with the systemic plasma findings.ConclusionsBaseline neutrophil-linked proteomic signals and diminished HDAC1 expression are associated with successful IS withdrawal in pLT recipients. These findings support HDAC1 as a hypothesis-generating candidate biomarker for baseline risk stratification and provide a clinically oriented framework to refine patient selection and enhance early monitoring during IS minimization and withdrawal protocols.

Background:Full-size split liver transplantation (FSLT) offers an attractive way to expand the donor pool for adult patients. However, the efficacy and safety of FSLT remain under debate. Materials and Methods:One hundred and three recipients who underwent FSLT were enrolled, and 124 recipients who received whole-liver transplantation (WLT) were taken as a control group. Clinical data and short-term outcomes within 1 year posttransplantation from 2019 to 2023 were compared between the two groups. Survival rates were calculated using the Kaplan-Meier method and compared by the log-rank test. Meanwhile, propensity-score matching (PSM) using key prognostic factors was conducted and the results were compared. Univariate and multivariate logistic regression analyses were performed to identify risk factors for serious complications in FSLT. Results:Thirty-three (29.2%) and 25 (20.2%) recipients in FSLT and WLT experienced postoperative complications graded ≥ III, with no significant differences (P = 0.106). However, biliary and vascular complications were more frequent in FSLT than in WLT (11.5% vs. 4.0% and 12.4% vs. 1.6%). The 3- and 6-month mortality rates in FSLT did not differ significantly from those in WLT (6.2% and 8.8% vs. 3.2% and 4.8%). The 1-year recipient (OS) and graft survival (GS) rates were 87.6% and 85.8% for FSLT, and 92.7% and 91.9% for WLT, with no significant differences. After PSM, the 1-year OS and GS were 81.3% and 81.3% for FSLT, and 90.6% and 90.6% for WLT (P = 0.081). Multivariate analysis revealed that a graft with steatosis of 5%-10%, a MELD score ≥ 20, sarcopenia, a history of hepatectomy, and an operation time > 420 min were independent risk factors for the incidence of postoperative complications graded ≥ III in FSLT. Conclusions:FSLT remains a challenging procedure. However, the short-term outcomes of FSLT were acceptable compared with those of WLT, encouraging the use of this procedure to increase the donor pool whenever possible.

BACKGROUND:Post-liver transplantation-Burkitt lymphoma (PLT-BL) is an aggressive and life-threatening complication after pediatric liver transplantation. The research on the disease characteristics, treatment, and prognosis of pediatric PLT-BL is limited. METHODS:A retrospective, observational study was performed to analyze the disease characteristics, treatment, and outcomes in 12 pediatric liver transplant (LT) recipients diagnosed with PLT-BL. RESULTS:The medium time from liver transplantation to diagnosis was 32.71 months (range, 25.78-37.85 months). All patients (100%) tested positive for EBV viremia at diagnosis. Abdomen and peripheral lymph nodes were the most frequently involved sites (11 [91.66%] and 10 [83.33%], respectively). Three pediatric patients were diagnosed as relapsed or refractory PLT-BL and treated with chimeric antigen receptor (CAR) T-cell therapy, and two of them achieved complete remission (CR). Nine patients were alive with CR at the last follow-up. Three deaths were attributed to progression and tumor lysis syndrome, characterized by significantly elevated lactate dehydrogenase (LDH) levels (p = 0.027), more advanced tumor stages (p = 0.045), and an increased number of involved sites (p = 0.009). With a median follow-up of 2.7 years after diagnosis, the event-free survival (EFS) and overall survival (OS) rates for PLT-BL patients were 66.7% and 74.1%, respectively. In four patients, immunosuppression was successfully withdrawn and they maintained tolerance status. CONCLUSION:Elevated LDH levels, more advanced tumor stages, and increased number of involved sites are potentially associated with poor outcome. Immunosuppression withdrawal is a safe and feasible approach for PLT-BL.

Background The definition of early allograft dysfunction (EAD) varies, and has hardly been studied in pediatric transplantation (pLT) since the adult EAD definition is not easily applicable to pLT. Methods A retrospective analysis was conducted on consecutive pLT patients aged <18 at the Department of Pediatric Transplantation of Tianjin First Central Hospital from April 2013 to December 2022. The definition of EAD explored in this study is (1) international normalized ratio ≥2.8 on day 1 and aspartate aminotransferase >1500 IU/mL within the first 7 days or (2) total bilirubin ≥5 mg/dL on day 7. The overall survival of patients and graft survival at 90 days after surgery were compared between this new definition and the adult EAD definition. Results A total of 1620 pLT recipients were included in the study, of which 179 (11.0%) recipients met the new definition of EAD for pLT. Twenty-five (13.97%) died and 37 (20.67%) graft lost within 90 days. The RR_death under Olthoff's EAD definition and our EAD definition are 3.45 and 9.57, respectively; The RR_graft_loss under Olthoff's EAD definition and our EAD definition are 4.18 and 11.48, respectively. A total of 97 (18.98%) of 511 recipients who received deceased donor liver transplantation (DDLT) met the new definition of EAD, 18 (18.56%) died and 29 (29.90%) graft lost within 90 days. In DDLT group, the RR_death under Olthoff's EAD definition and our EAD definition are 2.29 and 10.98, respectively; The RR_graft_loss under Olthoff's EAD definition and our EAD definition are 2.34 and 11.24, respectively. Conclusion The broadly used Olthoff’s EAD definition in adult liver transplantation is unsuitable for pLT use. The EAD definition established in this study is more suitable for patients <18 years old who received pLT, especially those <18 years old who received DDLT.

BACKGROUND & AIMS: The incidence of graft fibrosis elevated after pediatric liver transplantation (pLT) and influenced by cold ischemic time (CIT). Myosin light chain (MYL9), a member of the myosin family, could act on hepatic stellate cells (HSCs) and induce a transition to active phase. hypothesized that cold ischemic injury could stimulate expression and lead to graft fibrosis. METHODS: We tested the hypothesis by analyzing multi-omics data from human protocol liver biopsy samples 2 years LT, performing rat LT with different CIT and conducting in studies in HSC cell lines with MYL9 knockdown and overexpression. RESULTS: Clinically, CIT is an independent risk factor for graft fibrosis after pLT. Omics analysis identified MYL9 as a prominent contributor in graft fibrosis. MYL9 is strongly correlated with liver fibrosis grade and the progression of fibrosis. The study of rat LT model demonstrated MYL9 expression increases with the prolongation of CIT, and its role is specific to transplant setting. Mechanistically, in vitro experiments with HSCs exposed to hypoxia/reoxygenation revealed a substantial decrease in HSCs activation after MYL9 knockdown. Conversely, overexpression of MYL9 significantly enhanced the activation of HSCs. Subsequent transcriptome sequencing of HSCs with MYL9 knockdown unveiled that MYL9 primarily functions through the TLR4/MYD88/NF-KB signaling pathway. Liver graft fibrosis was ameliorated when toll like receptor 4 signaling was inhibited in rats. CONCLUSIONS: Our findings demonstrate that prolonged CIT up- regulates the expression of MYL9 in liver graft after LT. MYL9 activates HSCs and promotes fibrosis through a TLR4/MYD88/NF-KB signaling dependent manner. (Cell Mol Gastroenterol Hepatol 2025;19:101453; https://doi.org/10.1016/j.jcmgh.2024.101453)

The effectiveness of bone marrow mesenchymal stem cells (BMSCs) in post-transplantation liver fibrosis has not been studied. The aim of this study was to investigate the effect of BMSCs on liver fibrosis and their role in the Janus-activated kinase (JAK) 1/ signal transducer and activator of transcription (STAT) 5 pathway after liver transplantation (LT). A rat model of post-LT liver fibrosis induced by cold ischemia injury was successfully established. BMSCs were injected into the rats through the portal vein. Hepatic stellate cell (HSC)-T6 were co-cultured with BMSCs in vitro after hypoxia–reoxygenation. JAK1 inhibitor Abrocitinib and JAK1 agonist RO8191 were used to study the JAK1/STAT5 signaling pathway. BMSCs significantly alleviated liver fibrosis caused by cold ischemia–reperfusion injury after rat LT in vivo. After BMSCs transplantation, the levels of JAK1 and p-STAT5 in rat liver were significantly reduced. After using Abrocitinib, the stage of liver fibrosis and the levels of collagen type I alpha 1 chain (COL1A1) and actin alpha 2 (ACTA2) decreased. After using RO8191, the stage of liver fibrosis and the levels of COL1A1 and ACTA2 increased. BMSCs significantly reduced the activation of HSC-T6 after hypoxia–reoxygenation in vitro. After co-culturing with BMSCs after HSC-T6 hypoxia–reoxygenation, the levels of JAK1 and p-STAT5 were significantly reduced. After the addition of Abrocitinib, the levels of COL1A1 and ACTA2 decreased in HSC-T6; in contrast, after adding RO8191, the levels of COL1A1 and ACTA2 increased in HSC-T6 after hypoxia–reoxygenation. After using anti-IL7 antibody or anti-IL7Rα in vivo and in vitro, the stage of liver fibrosis and the levels of COL1A1 and ACTA2 decreased as well as the phosphorylation level of STAT5. BMSCs alleviate hepatic cell damage, reduce hepatic cell-derived IL7, downregulate IL7R/JAK1/STAT5 in HSCs, thereby reducing HSCs’ activation and ultimately alleviating liver fibrosis after liver transplantation. BMSCs can alleviate liver fibrosis after rat liver transplantation in vivo. BMSCs can reduce activation of HSCs after hepoxia-reoxygenation in vitro. BMSCs inhibit activation of HSCs after injury through the JAK1/STAT5 pathway, thereby reducing liver fibrosis after liver transplantation.

BACKGROUND:A growing number of studies indicate that acute kidney injury (AKI) and the pattern of recovery after kidney injury are associated with poor outcomes for grafts and long-term renal function after liver transplantation (LT). However, few systematic studies have been conducted on the pediatric liver transplantation (PLT) population. MATERIALS AND METHODS:In this single-centered retrospective observational study, according to AKI degree and whether AKI was persistent, patients were divided into three groups: non-AKI/stage 1 AKI, stage 2/3 transient AKI, and stage 2/3 persistent AKI. We compared the survival of patients, graft, and chronic kidney disease (CKD) among the three groups, analyzing the risk factors for the stage 2/3 persistent AKI. RESULTS:Among 700 patients, the total incidence of AKI was 39.57%; of children with stage 2/3 AKI, the condition was persistent in 38.06%. In the stage 2/3 persistent-AKI group, the risk of graft loss was 3.264× greater than in the no-AKI/stage 1 AKI group and 4.329× greater than in the stage 2/3 transient AKI group. Pediatric End-stage Liver Disease (PELD) score, preoperative estimated glomerular filtration rate (eGFR), duration of vena cava occlusion, postoperative respiratory support time, and postoperative early allograft dysfunction (EAD) were significantly correlated with the occurrence of stage 2/3 persistent AKI. CONCLUSIONS:By combining AKI recovery mode with AKI grading, clinicians can screen out stage 2/3 persistent AKI with a worse prognosis. This method was more accurate in predicting prognosis than applying AKI grading alone.

BACKGROUND:Right ventricular outflow tract (RVOT) dysfunction is a long-term postsurgical complication of tetralogy of Fallot (TOF) that needs long-term multidisciplinary treatment. CASE SUMMARY:We report a case of TOF patient who underwent radical surgical repair in infancy and who presented with pulmonary artery stenosis and pulmonary regurgitation during follow-up. Pulmonary stent placement and percutaneous pulmonary valve implantation (PPVI) were pursued successfully when he was aged 5 and 13 years, respectively. DISCUSSION:PPVI has been widely used as a minimally invasive treatment alternative to surgical pulmonary valve replacement. This technology has shown significant advantages in pediatric patients to improve RVOT dysfunction, relieve symptoms, optimize hemodynamic parameters, and avoid reintervention. TAKE-HOME MESSAGE:This case provides a long-term and multidisciplinary strategy for TOF in pediatrics, with a focus on the possibility and effect of PPVI in adolescent patients.

Organ transplantation is the most effective treatment for end-stage organ failure. However, donor shortage leads to many patients dying while waiting for transplants. With breakthroughs in basic research,advancements in gene editing technologies,and developments of new immunosuppressive drugs,xenotransplantation is regarded as the most promising solution to address the organ shortage crisis. In recent years, researchers both domestically and internationally have conducted multiple studies on non-human primates (NHPs), brain-dead donors,and patients with end-stage heart failure, end-stage renal disease, and advanced liver cancer. Nevertheless, challenges such as immune rejection and coagulation dysfunction continue to limit recipient survival, hindering significant breakthroughs in the clinical application of xenotransplantation. This review summarizes the latest developments in xenotransplantation research globally, with a focus on immune rejection and coagulation dysfunction following liver xenotransplantation.

Objective:HAEMOMASTER system developed by NIKKISO is a feedback control technology that combines blood volume monitoring, which is now increasingly used in many dialysis centers. We investigated the effectiveness and safety of five slopes provided by HAEMOMASTER system. Methods:Patients undergoing hemodialysis with the support of a blood volume monitor (BVM) were enrolled. The NIKKISO DBB-05 Hemodialysis machine had automatically recorded real-time data such as BV and BP. Data from the patients' previous 10 dialysis sessions were collected into the HAEMOMASTER system for data fitting and the calculated target ΔBV. Patients received dialysis treatment with five slopes of the HEAMOMASTER system. We record the actual ΔBV and reverse events of every slope. Relative index to ΔBV of different slopes and sub-analysis was conducted by two-variable Spearman correlation analysis. Results:One hundred participants entered, and 78 completed the study. Slope1 and Slope2 had a lower incidence of adverse reactions (5.3% and 3.8%) and higher correlation coefficients (0.827 and 0.831, P < .001), which means they can reflect dialysis physiology better. HEAMOMASTER system helps the hemodialysis physician develop an optimal individual hemodialysis plan for the patient, reduce adverse effects such as hypotension ,obvious sweating, palpitation, fatigue, and the hemodialysis process is interrupted or the ultrafiltration volume being adjusted. Conclusion:We evaluated the safety and effectiveness of the HAEMOMASTER System in hemodialysis patients. This study serves as a roadmap for the development and widespread use of the HAEMOMASTER system and a resource for the creation of novel biofeedback control strategies. The HAEMOMASTER system has good clinical application prospects in hemodialysis patients and can be used to develop individualized ultrafiltration schemes for patients and improve the comfort and safety of hemodialysis. Slope1 and Slope2 of HAEMOMASTER are more suitable for the majority of patients with a better fit to the actual physiological conditions and lower incidence of adverse events.

Background. Optimizing the immunosuppressive regimen is essential to improve the long-term outcomes of pediatric liver transplant recipients. Methods. We conducted a prospective, randomized, open-label study to compare the safety and efficacy of 2 treatment approaches during pediatric liver transplantation: tacrolimus monotherapy following basiliximab induction (the study group) and a dual regimen of tacrolimus plus steroids (the control group). A total of 150 patients were enrolled, with 75 patients allocated to each group. Results. In both groups, recipients achieved graft and recipient overall survival rates exceeding 93%, with no statistically significant differences between them. However, the study group exhibited a significantly lower incidence of acute cellular rejection (ACR), delayed occurrence of ACR, and an improved ACR-free survival rate at 2 y compared with the control group. Notably, the study group also showed a significant reduction in the incidence of de novo donor-specific antibodies at 3-mo and 2-y posttransplant. Furthermore, 6 mo after the transplant, the study group demonstrated significant improvements in weight-for-age Z score and height-for-age Z score. No notable differences were observed in postoperative complications or the incidence of liver fibrosis between the 2 groups. Conclusions. Basiliximab induction combine with tacrolimus (TAC) monotherapy is a safe and effective immunosuppressive regimen to reduce the episodes of ACR without influencing the development of liver fibrosis and graft and recipient survival rate after pediatric liver transplantation.

BackgroundPosttransplant lymphoproliferative disease (PTLD) is a serious complication after pediatric liver transplantation (pLT), which may lead to death. F-18-FDG PET/CT is rarely considered in PTLD after pLT and lacks clear diagnostic guidelines, especially in the differential diagnosis of nondestructive PTLD. The aim of this study was to find a quantifiable F-18-FDG PET/CT index to identify nondestructive PTLD after pLT. MethodsThis retrospective study collected the data of patients who underwent pLT, postoperative lymph node biopsy, and F-18-FDG PET/CT at Tianjin First Central Hospital from January 2014 to December 2021. Quantitative indexes were established using lymph node morphology and the maximum standardized uptake value (SUVmax). ResultsA total of 83 patients met the inclusion criteria and were included in this retrospective study. To distinguish between PTLD-negative cases and nondestructive PTLD cases, according to the receiver operating characteristic curve, (the shortest diameter of the lymph node at the biopsy site [SDL]/the longest diameter of the lymph node at the biopsy site [LDL])*(SUVmax at the biopsy site [SUVmaxBio]/SUVmax of the tonsils [SUVmaxTon]) had the maximum area under the curve (0.923; 95% confidence interval: 0.834-1.000), and the cutoff value was 0.264 according to the maximum value of Youden's index. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 93.6%, 94.7%, 97.8%, 85.7%, and 93.9%, respectively. Conclusions(SDL/LDL)*(SUVmaxBio/SUVmaxTon) has good sensitivity, specificity, positive predictive and negative predictive values, and accuracy, and can be used as a good quantitative index for the diagnosis of nondestructive PTLD.

BackgroundDonor shortage is an important limitation of liver transplantation (LT). Split liver transplantation (SLT) may increase the sources of donors and reduce the problem of organ shortage. However, there are no standard criteria of the selection of SLT donor, especially regarding the donor age.MethodsWe retrospectively analyzed the clinical data of children who received initial SLT between January 2015 and December 2021. Based on the age of donors, the patients were divided into groups A (1–10 years old; n = 26), B (10–45 years old; n = 87), and C (45–55 years old; n = 27). The short-term (<1 year after SLT) outcomes of the recipients were analyzed.ResultsA total of 140 patients received SLT from 122 donors. The 1-, 3- and 12-month patient survival rates in group A were 100.0%, and the graft survival rates were 92.3%. The 1-, 3- and 12-month survival rates of patient and graft in group B were 97.7%, 96.6%, and 95.0%, respectively, and in group C were 85.2%, 85.2%, and 81.1%, respectively. The patient survival rate was significantly lower in group C than in groups A and B (p = 0.0082). There was no significant difference in graft survival between the three groups (p = 0.0545).ConclusionsSimilar results were obtained for pediatric SLT with donors <10 years old and 10–45 years old. Pediatric SLT can be performed with older donors (45–55 years) after strict donor selection and selection of appropriate recipients.