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Abstract Objectives To validate an appropriate evaluation method of liver fibrosis assessment based on the unique pathological features of biliary atresia (BA) that could well predict its prognosis. Methods A total of 68 patients with BA who underwent Kasai procedure (KP) and an intraoperative liver biopsy, followed up from January 2019 to December 2021, were recruited in a retrospective analysis. Ishak, Metavir, and BA-specific staging systems in relation to outcomes were analyzed using logistic regression, COX proportional hazard regression, Kaplan-Meier analysis, etc. Results Kaplan-Meier analysis determined a significant difference in native liver survival according to the BA-specific stage (p = 0.002). The ROC curve analysis for predicting prognosis showed that the AUC of BA-specific staging combined with iBALF and severe bile duct proliferation (BDP) (0.811, 95% CI: 0.710–0.913, p

Introduction: To investigate the diagnostic value of serum MMP-7 and SOX9 levels in patients with biliary atresia (BA) and to further analyze their relationship with the liver fibrosis and prognosis. Methods: Sixty-eight patients hospitalized for jaundice and underwent surgical treatment from March 2019 to February 2022 were retrospectively analyzed, including 44 patients with BA and 24 patients with other cholestatic liver diseases as disease controls (DC). Serum levels of MMP-7 and SOX9 were detected using enzyme-linked immunosorbent assay (ELISA). The diagnostic value of MMP-7 and SOX9 for BA was evaluated using the receiver operating characteristic (ROC) curve. Results: Serum levels of MMP-7 and SOX9 were higher in the BA group compared to the DC group (P = 0.0002, P = 0.0006). The AUC for the combined diagnosis of serum MMP-7, SOX9 and GGT was 0.888 (95 % CI:0.788–0.952, P < 0.0001), which was significantly different from the diagnosis of MMP-7 or SOX9 alone (P = 0.034, P = 0.030). Serum levels of MMP-7 and SOX9 were significantly positively correlated with the liver fibrosis score (rs = 0.41, P = 0.006; rs = 0.58, P < 0.0001). BA patients who maintained native liver survival had lower preoperative serum levels of MMP-7 and SOX9 (P = 0.008, P = 0.0008). Conclusions: The combined diagnosis of BA by MMP-7, SOX9 and GGT has better accuracy than MMP-7 or SOX9 alone. Serum SOX9 levels can assess the severity of BA liver fibrosis and correlate with BA native liver survival, but more sample data are needed to further investigate its predictive value.

BackgroundHepato-pancreato-biliary (HPB) disease has different causes and types between children and adults, which has been increasingly diagnosed in the pediatric group. Endoscopic retrograde cholangiopancreatography (ERCP) has been gradually considered as a therapeutic method in adults, while in pediatric patients, there are not many reports of its usage. This systematic review and meta-analysis aims to assess the use condition of therapeutic ERCP in the management of pediatric HPB diseases.MethodsThis systematic literature search was conducted in the PubMed, Embase, Web of Science, and Cochrane library databases to identify all relevant articles published from inception to February 2022 that evaluated therapeutic ERCP in pediatric patients with HPB diseases. The researchers included studies in which patients were less than 18 years old and underwent therapeutic ERCP procedures. A random-effects model was used to analyze the usage rate of therapeutic ERCP procedures, procedural success rates, adverse event rates, and the rate of different therapeutic procedures. Subgroup analysis, sensitivity analysis, and meta-regression were conducted to analyze the source of heterogeneity.ResultsA total of 33 articles were included. After homogenization, the overall use of therapeutic interventions accounts for 77% [95% confidence interval (CI) 74–81%] of all ERCP procedures. After excluding outlier studies, the estimation success rate of the therapeutic procedure is 74% (95% CI 69–79%), and adverse event rate is 8% (95% CI 6–10%). In our study, stent placement is the most common procedure, which makes up 75% (95% CI 65–86%) of all therapeutic procedures. In addition, the usage proportion of sphincterotomy (ST), stone extraction/removal, bougienage/balloon dilation is, respectively, 46% (95% CI 39–53%), 34% (95% CI 31–38%), and 26% (95% CI 22–29%).ConclusionThe ERCP procedure is gradually considered a therapeutic technique in pediatric patients, the proportion of therapeutic ERCP is 77% of total usage, which is increasing every year. Meanwhile, its success rate is relatively high. It reflects that this operation modality is promising in the treatment of HPB disorders and is gradually expanded as more branch technologies are being used. A variety of operations can be achieved through ERCP procedures, and more functions should be developed in the future.Systematic Review Registration[https://www.crd.york.ac.uk/prospero/], identifier [CRD42022302911].