Jianghua Zhan

To develop an interpretable model using routine outpatient data to assist risk stratification and referral assessment in children with biliary atresia (BA) after Kasai portoenterostomy (KPE). This multicenter retrospective study included 512 children with type III BA who underwent KPE across eight pediatric centers. Clinical, laboratory, and ultrasonographic variables collected during outpatient follow-up were analyzed. Feature selection was performed using least absolute shrinkage and selection operator, and eight machine learning algorithms were evaluated. Model performance was assessed using discrimination, calibration, and decision curve analysis. The optimal model was further interpreted using Shapley Additive Explanations analysis and implemented as an online calculator. Ten variables were selected for model development. Among the evaluated algorithms, the K-nearest neighbors model showed the best overall performance in the test set (area under the receiver operating characteristic curve 0.954) with good calibration and clinical utility. Key contributors included albumin, cholestasis index, the variceal prediction rule, and total bile acids. This model provides an accessible tool for outpatient assessment after KPE and may assist clinicians in identifying high-risk patients and improving risk stratification during follow-up.

Background & aims: Biliary atresia (BA) is the leading cause of cholestatic liver disease and cirrhosis in infancy. Currently, there is a lack of efficient biomarkers for early diagnosis and prognostic evaluation of this disease, which urgently needs to be addressed. This study aimed to screen and validate key biomarkers closely associated with BA liver fibrosis progression and prognosis through bioinformatics analysis combined with clinical sample verification. Methods: First, the public transcriptomic dataset GSE122340 was thoroughly analyzed to screen differentially expressed genes, followed by construction of a protein–protein interaction (PPI) network. Epithelial cell transforming sequence 2 (ECT2) was ultimately identified as the core hub gene. Subsequently, clinical samples were collected, including 20 cases in the BA group (stratified by liver fibrosis severity) and 10 cases in the choledochal cyst control group. The expression level of ECT2 was clinically verified using immunohistochemistry, real-time quantitative polymerase chain reaction (qPCR), and enzyme-linked immunosorbent assay (ELISA). Receiver operating characteristic (ROC) curve analysis was performed to validate the predictive efficacy of serum ECT2 level for BA diagnosis, fibrosis stratification, and postoperative native liver survival. Kaplan–Meier (KM) curve analysis was used to verify the relationship between serum ECT2 level and postoperative native liver survival. Results: ECT2 was significantly highly expressed in liver tissue (both mRNA and protein levels) and serum of BA patients, and its expression intensity was positively correlated with the severity of liver fibrosis. ROC curve analysis showed that serum ECT2 exhibited favorable efficacy in BA diagnosis, liver fibrosis stratification, and prediction of 2-year postoperative native liver survival, with area under the curve (AUC) values of 0.906, 0.807, and 0.844.Compared with MMP7, it demonstrates comparable predictive performance in the diagnosis of BA (AUC = 0.924) and the stratification of liver fibrosis (AUC = 0.829), and outperforms MMP7 in predicting autologous liver survival (AUC = 0.767). Its overall predictive performance is superior to that of traditional liver function markers (GGT, TBIL, TBA). Kaplan–Meier survival analysis further confirmed that BA patients with high serum ECT2 expression had significantly reduced native liver survival rates postoperatively. Conclusion: This study systematically demonstrates that ECT2 is characteristically highly expressed in BA and its association with disease fibrosis severity and poor prognosis. Preliminary findings suggest that serum ECT2 as a promising non-invasive biomarker, providing new technical approaches and theoretical basis for clinical diagnosis, disease assessment, and prognostic judgment of BA.

To investigate the association between cholangitis and portal hypertension (PH) risk, and the efficacy of UDCA in native liver survivors after successful Kasai portoenterostomy (KPE). A multicenter, retrospective cross-sectional study was conducted at nine Chinese pediatric centers. Patient grouping was based on cumulative cholangitis episodes and UDCA regimens. After a median follow-up of 56 months, the overall incidence of PH was 23.63% (198/838). Recurrent cholangitis (≥ 3 episodes) was an independent risk factor for PH (OR = 4.25, 95% CI 2.61-6.92, P < 0.001), associated with poorer liver function recovery, greater spleen thickness, and lower peak velocity of the main portal vein. Continuous UDCA therapy significantly attenuated the PH risk induced by recurrent cholangitis (RERI: - 7.66). Among this high-risk subgroup, low-dose UDCA (≤ 10 mg/kg/day) constituted an independent protective factor (OR = 0.17, 95%CI 0.04-0.67, P = 0.015) and showed superior efficacy in reducing total bilirubin. These benefits were evident in children under 48 months, but no clear advantage observed beyond this age. Among native liver survivors with jaundice clearance, recurrent cholangitis (≥ 3 episodes) warrants long-term monitoring for PH. Continuous low-dose UDCA until 48 months is recommended for high-risk patients to improve outcomes.

Purpose We compared the long-term outcomes of open (OKPE) versus laparoscopic Kasai portoenterostomy (LKPE) for children with biliary atresia (BA) who ultimately progressed to liver transplantation (LT). Methods The subjects of this retrospective multicenter analysis were 276 BA patients (OKPE:225, LKPE:51) undergoing living donor liver transplantation (LDLT) between 2021 and 2023. Propensity score matching (PSM, 51 pairs) and inverse probability treatment weighting (IPTW) balanced the confounders. The time from KPE to LDLT was assessed using Kaplan-Meier curves and Cox regression analysis. Results There were no significant differences in the median time from KPE to LDLT between the OKPE and LKPE groups (228 days vs. 203 days, p = 0.756). Multivariable Cox regression identified patient age at KPE (HR = 1.002, p < 0.01), but not the surgical approach, as a factor influencing the timing of transplantation. No significant differences were observed in postoperative complications, LDLT-free survival rates, or intraoperative transplant metrics between the groups. Conclusions For BA infants who progress to LT after KPE, OKPE and LKPE have comparable impact on post-KPE complications, survival time, and outcomes following LT. Thus, the emphasis should be on optimal surgical timing and standardized postoperative management.

Background: Pediatric cholelithiasis is a biliary system calculus resulting from complex pathogenesis, which is a significant public health issue affecting children’s health. This study aimed to analyze the clinical characteristics of pediatric cholelithiasis, including demographic characteristics, primary diseases, symptoms, and imaging features, then investigated the treatment strategies and effects of different types of cholelithiasis. Methods: The study retrospectively included and analyzed clinical data of children with cholelithiasis, who were admitted to the Surgery Department of Tianjin Children’s Hospital between June 2017 and January 2024. These patients underwent either conservative or surgical management during their hospitalization. The following data were systematically collected: general clinical characteristics, primary comorbidities, imaging features, and perioperative laboratory indicators. Based on the World Health Organization (WHO) Child Growth Standards, the growth condition of patients was categorized into four levels: normal, overweight, obesity, and thinness. The treatment modalities for gallbladder stones and cholangiolithiasis were summarized and analyzed, and patients who received surgical treatment underwent follow-up evaluation. Results: A total of 185 patients were retrospectively reviewed: 57.8% patients had gallbladder stones, 25.4% patients had cholangiolithiasis, and 16.8% of them suffered from both conditions. The number of female patients exceeded that of male patients, but no statistically significant differences were observed between genders. The median age of onset for cholelithiasis was 7.5 (3.7–11.0) years, only 22.4% were classified as overweight or obesity. About 60% of patients had normal body mass index (BMI) values. In patients diagnosed with gallbladder stones, laparoscopic cholecystectomy (LC) and laparoscopic choledochoscopic cholecystolithotomy were predominantly employed in clinical practice. Postoperative follow-up of the cholecystectomy group was 43.8±33.0 months, with no recurrence observed. The recurrence rate of Gallbladder-preserving lithotomy group was 14.3%, following a mean follow-up of 23.1±12.3 months. Among patients with bile duct stones, endoscopic therapy accounted for 20.5% of interventions. The average postoperative hospital stay was 5.6±2.2 days. Two cases of recurrence were observed at 48 and 3 months post-endoscopic retrograde cholangiopancreatography (ERCP), respectively. However, follow-up periods should be extended to ensure long-term outcomes. Conclusions: Our study indicated that attention should be paid to pediatric patients with normal body weight, and individualized therapeutic approaches should be promoted in the management of pediatric cholelithiasis. Conservative observation should be the first-line treatment, cholecystectomy is effective for severe conditions such as severe purulent exudation, atrophy, necrosis, and perforation of the gallbladder wall. Gallbladder-preserving lithotomy could be considered when gallbladder morphology and function are normal. Endoscopic therapy effectively resolves biliary obstruction and secondary bile duct dilatation, improving liver function simultaneously.

Purpose Evaluate clinical efficacy of ursodeoxycholic acid (UDCA) in biliary atresia (BA) infants post-Kasai portoenterostomy (KPE) using real-world data. Methods Retrospective analysis of 698 BA patients from eight Chinese pediatric centers (2020–2025). Age-stratified analyses used propensity score matching (PSM) and inverse probability treatment weighting (IPTW) to adjust baseline differences. Primary outcomes were UDCA utilization and effects on bilirubin (TBil, DBil) and total bile acid (TBA) levels. Results The median follow-up duration for the cohort was 49 months (IQR: 25–74), with male patients accounting for 54.2% (379/698). The median age at surgery was 59 days (IQR: 46–75). A history of cholangitis was present in 53.1% of patients (371 cases), and 63.5% (443 cases) underwent open Kasai portoenterostomy. After PSM/IPTW adjustment, UDCA within 12 months significantly reduced TBil, DBil, and TBA. Initiation before 12 months reduced TBil-elevated (TBil > 20 μmol/L) odds by 80% (OR = 0.20, 95% CI 0.05–0.72) and hyperbilirubinemia (TBil > 34 μmol/L) by 86% (OR = 0.14, 95% CI 0.03–0.50). Low-dose UDCA (≤ 10 mg/kg/day) within 12 months provided optimal bilirubin/hepatic improvement without dose-dependence. UDCA beyond 36 months nearly doubled TBA levels, potentially reflecting dose-dependent TBA elevation. Conclusion UDCA efficacy post-KPE is age-dependent. Low-dose therapy (≤ 10 mg/kg/day) within 12 months significantly improves jaundice and liver function, with limited benefits at 12–36 months. Use beyond 36 months may be harmful. Optimal timing/dosing requires prospective validation.