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Cholestatic liver diseases (CLD) are characterized by impaired normal bile flow, culminating in excessive accumulation of toxic bile acids. The majority of patients with CLD ultimately progress to liver cirrhosis and hepatic failure, necessitating liver transplantation due to the lack of effective treatment. Recent investigations have underscored the pivotal role of the gut microbiota-bile acid axis in the progression of hepatic fibrosis via various pathways. The obstruction of bile drainage can induce gut microbiota dysbiosis and disrupt the intestinal mucosal barrier, leading to bacteria translocation. The microbial translocation activates the immune response and promotes liver fibrosis progression. The identification of therapeutic targets for modulating the gut microbiota-bile acid axis represents a promising strategy to ameliorate or perhaps reverse liver fibrosis in CLD. This review focuses on the mechanisms in the gut microbiota-bile acids axis in CLD and highlights potential therapeutic targets, aiming to lay a foundation for innovative treatment approaches.

Background Esophageal atresia (EA) with concomitant hypertrophic pyloric stenosis (HPS) is a rare condition, and its diagnosis can be challenging due to overlapping postoperative complications. Comprehensive clinical characterization remains limited. Methods We retrospectively reviewed five patients with EA with concomitant HPS treated at our center between 2018 and 2025. A systematic literature search of PubMed, Embase, Web of Science, and CNKI identified 48 additional cases. Clinical features, timing of diagnosis, treatment strategies, and outcomes were analyzed. Results Among 132 neonates with EA in our institution, five (3.8%) developed HPS. Four had Gross type C EA, and three were male. All underwent EA repair in the first week of life and later developed HPS, diagnosed at a median age of 45 days (range: 20–145). All patients underwent laparoscopic pyloromyotomy with uneventful recovery and were alive at a median follow-up of 50 months. Literature review revealed 48 reported cases, predominantly male (41/48) and Gross type C (42/48). Combined analysis of 53 patients showed HPS onset typically between 2 and 15 weeks, with a median age of 4 weeks (IQR: 2.5–6 weeks). Overall survival was 90.6%, with deaths mainly due to infection or severe associated anomalies. Conclusions HPS may coexist with EA, with an incidence of approximately 3.8%. It often presents later in the disease course. Its diagnosis can be delayed by postoperative complications or altered feeding. Long-term follow-up and multicenter studies are warranted.

Background/Purpose This study aimed to evaluate discrepancies in prognosis after the Kasai portoenterostomy (KPE) procedure between neonatal and non-neonatal periods among patients undergoing the KPE procedure less than 60 days after birth (≤ 60 days). Methods All type III BA patients who performed KPE less than 60 days from June 2020 to May 2024 in the Department of Neonatal Surgery of Beijing Children’s Hospital were retrospectively reviewed. They were divided into two groups according to the age at Kasai: the early KPE group (EK group, or neonatal group ≤ 30 days after birth) and the late KPE group (LK group, or non-neonatal group 31–60 days after birth), to evaluate the effect of surgical age on the postoperative efficacy following Kasai. Results 61 BA patients were included in our study. The median age at KPE in the EK group was 14.0 [10.0, 20.0] days and 47.0 [36.8, 51.1] days in the LK group. Regarding postoperative complications, Patients in the EK group tended to have a higher incidence of postoperative intestinal obstruction (10.5% vs. 0.0%, p  = 0.033) compared to those in the LK group. However, the postoperative long-term prognosis of KPE in the EK group seemed much better than in the LK group. Compared to the LK group, a significantly greater proportion of patients in the EK group achieved successful jaundice clearance (84.2% vs. 40.5%, p  = 0.002) and successful bile acids clearance (63.2% vs. 19.0%, p  = 0.001). Additionally, the one-year NLS in the EK group was also higher than the LK group despite without statistically significant difference (88.2% vs. 61.5%, p  = 0.046). Kaplan-Meier curve also showed a significant difference between the two groups ( p  = 0.049, HR = 3.91, 95%Cl 1.46–10.46). Further, we evaluate the changes in biochemical indexes and liver hardness indicators over time in BA patients who had achieved one-year NLS. We found that compared to BA patients in the LK group, those in the EK group exhibited faster jaundice clearance and bile acids clearance, and more normalized biochemical indicators and liver hardness one year after Kasai. Conclusion Performing KPE procedure in the neonatal period was associated with a better long-term prognosis despite of potential increased risk of postoperative adhesive ileus.

Background In select patients with type C esophageal atresia, primary anastomosis is not appropriate and a staged approach is required. We aim to summarize our experience in the management of type C EA using a staged approach. Methods A retrospective chart-review of patients with type C EA admitted to Beijing Children’s Hospital between July 2020 to October 2023 were conducted. Those diagnosed with type C EA who were not amendable to primary anastomosis were included for analysis. Clinical information was recorded, and follow- up was performed. Results Seven (five boys) patients with type C EA who received staged repair were included in the study. Initial surgeries included thoracotomy and thoracoscopy. 71% (5/7) patient had complications after the initial surgery, including pyopneumothorax, pneumonia, recurrent tracheoesophageal fistula (rTEF), and anastomotic leak. Esophageal elongation techniques were applied in 3 patients. All delayed anastomosis were performed thoracoscopically, except for in one case where spontaneous fistulization occurred and no anastomosis were necessary. Complications after delayed anastomosis included recurrent esophageal pulmonary fistula (rEPF) in 50% (3/6), anastomotic leak in 33% (2/6), and esophageal stricture in all (6/6) patients. After a median follow-up of 14 months (range: 2–24), all patients were in generally good condition. Conclusion Primary operations should be kept simple and minimal in patients diagnosed with type C EA who are not appropriate for primary anastomosis. Internal traction is an effective method that allows for subsequent anastomosis. Intraoperative indocyanine green fluorescence can aid in fistula determination and anastomosis.

Background Recurrent tracheoesophageal fistula (rTEF) is a rare complication following initial esophageal atresia (EA) surgical repair, posing challenges in localization the fistula during surgery due to severe thoracic adhesions and structural ambiguity from previous operations. Objective We introduced two new localization methods for rTEF patients during surgery and aimed to compare the impact of using these localization techniques versus not using them on the surgical outcomes for rTEF patients. Methods We retrospectively analyzed the clinical data of rTEF cases that underwent thoracoscopic repair at our hospital from September 2017 to December 2024. Patients were divided into localization group and non-localization group based on whether using intraoperative localization techniques, and comparative analysis of clinical variables was conducted between groups. Results A total of 106 patients were included in this study, undergoing a total of 113 thoracoscopic rTEF repair surgeries at our center. Their fistula type included 89 cases of tracheoesophageal fistula (TEF), 19 cases of esophageal-pulmonary fistula (EPF), 3 cases of esophageal bronchial fistula (EBF), and 2 cases of combined EPF and TEF. All cases were categorized based on whether using localization techniques, resulting in the localization group ( n  = 52) and the non-localization group ( n  = 61). The median operation time in the localization group (2.5 h) was significantly lower than in the latter (3.0 h) ( P  = 0.001), and regardless of the fistula type being TEF or EPF. Additionally, the average postoperative hospital stay was significantly shorter in the localization group (17.7 ± 7.5 days) than in the non-localization group (23.6 ± 20.0 days) regarding the fistula type of TEF ( P  = 0.03). Conclusions The use of localization techniques in thoracoscopic surgery for rTEF leads to better outcomes, evidenced by reduced operation time and hospital stay, suggesting enhanced surgical accuracy and improved patient postoperative recovery. Level of evidence LEVEL III.

Revision surgery for the complications after repair of esophageal atresia is often complex because of previous surgeries and chest infections and thus requires surgical expertise. This study describes surgical experiences with the use of indocyanine green (ICG) fluorescence imaging localization-assisted thoracoscopy during revision surgery, including recurrent tracheoesophageal fistula (rTEF) (8 cases, one of which was esophageal-pulmonary fistula) and delayed esophageal closure (1 case). We performed fistula repair and esophageal reconstruction according to the indications of ICG. The application of this method avoids the excessive trauma caused by freeing the trachea and esophagus. Contrast imaging taken one week and one month after surgery indicated no spillover of the contrast agent from the esophagus, except in 1 case. Indocyanine green fluorescence imaging localization-assisted thoracoscopy is worth promoting for revision surgery after esophageal atresia repair.

Background: Biliary atresia (BA) is the most common cholestatic liver disease in neonates. Herein, we aimed at characterizing the gut microbiota and fecal bile acid profiles of BA patients, defining the correlations between them, and evaluating the relationship between the clinical pathogenesis and changes in the gut microbiota and bile acid profiles.Methods: A total of 84 fecal samples from BA patients (n = 46) and matched healthy controls (HCs, n = 38) were subjected to sequencing by 16S rRNA gene amplification, and fecal bile acid were analyzed by targeted metabolomics.Findings: Compared with the controls, a structural separation of the intestinal flora of BA patients was uncovered, which was accompanied by changes in the composition of fecal bile acids. In the BA group, Actinobacillus, Monoglobus, and Agathobacter were enriched in patients without cholangitis (p < 0.05). Selenomonadaceae and Megamonas were more abundant in patients without recurrent cholangitis episodes (p < 0.05), while Lachnospiraceae and Ruminococcaceae were enriched in patients with multiple recurrences of cholangitis (p < 0.05). Postoperative jaundice clearance was associated with Campylobacter and Rikenellaceae (p < 0.05), and tauroursodeoxycholic acid was associated with jaundice clearance (p < 0.001).Conclusion: BA patients are characterized by different compositions of gut microbiota and bile acids, and their interaction is involved in the process of liver damage in BA, which may be closely related to the occurrence of postoperative cholangitis and jaundice clearance.

Biliary atresia (BA) is the most common form of severe neonatal obstructive jaundice. The etiology and pathogenesis of BA are multifactorial, and different factors may interact to produce heterogeneous pathological features and clinical outcomes. Despite different pathological features, all patients received the same treatment strategy. This study performed integrative clustering analysis based on multiple high-throughput datasets to identify the molecular subtypes of BA and provide a new treatment strategy for personalized treatment of the different subtypes of BA. The RNA sequence dataset GSE122340 in the Gene Expression Omnibus (GEO) database was downloaded; 31 BA and 20 control normal liver tissues were collected at our center for transcriptome sequencing, and clinical and follow-up data of BA patients were available. Molecular subtypes were identified using integrated unsupervised cluster analysis involving gene expression, biliary fibrosis, and immune enrichment scores based on the transcriptome dataset, and the results were validated using independent datasets. Based on the results of the integrated unsupervised clustering analysis, four molecular subtypes were identified: autoimmune, inflammatory, virus infection-related, and oxidative stress. The autoimmune subtype with a moderate prognosis was dominated by autoimmune responses and morphogenesis, such as the Fc-gamma receptor and Wnt signaling pathway. The biological process of the inflammatory subtype was mainly the inflammatory response, with the best prognosis, youngest age at surgery, and lowest liver stiffness. The virus infection-related subtype had the worst prognosis and was enriched for a variety of biological processes such as viral infection, immunity, anatomical morphogenesis, and epithelial mesenchymal transition. The oxidative stress subtype was characterized by the activation of oxidative stress and various metabolic pathways and had a poor prognosis. The above results were verified independently in the validation sets. This study identified four molecular subtypes of BA with distinct prognosis and biological processes. According to the pathological characteristics of the different subtypes, individualized perioperative and preoperative treatment may be a new strategy to improve the prognosis of BA.

Importance Neonatal appendicitis (NA) is a rare and potentially fatal neonatal disease. However, misdiagnosis is common owing to atypical clinical manifestations and non‐specific laboratory tests. Objective The aim of this study was to summarize the clinical characteristics, treatments, and prognoses of infants with NA. Methods This retrospective analysis included 69 patients diagnosed with NA admitted to Beijing Children's Hospital between 1980 and 2019. The patients were divided into surgical and non‐surgical groups based on whether surgery was performed. Their clinical characteristics were analyzed using the chi‐square test, t‐test, or the Mann‐Whitney U test. Results The study included 47 males and 22 females with NA. The primary symptoms were abdominal distension (n = 36, 52.2%), fever (n = 19, 27.5%), refusal to feed or decreased feeding (n = 16, 23.2%), and vomiting (n = 15, 21.7%). Sixty‐five patients underwent abdominal ultrasound examinations; 43 had definite appendiceal abnormalities, 10 had right lower abdominal adhesive masses, and 14 had neonatal enterocolitis manifestations. Twenty‐nine and 40 patients were in the surgical and non‐surgical groups, respectively. No statistically significant differences were observed between the groups regarding sex, age at onset, birth weight, admission weight, or hospitalization time. However, parenteral nutrition was prolonged in the surgical group (P = 0.001). Additionally, two patients (2.9%) died. Interpretation NA is a rare neonatal disease with atypical clinical manifestations. Abdominal ultrasonography may aid in the diagnosis. Similarly, appropriate treatment can improve the prognosis. Neonatal appendicitis (NA) is a rare disease with atypical signs and symptoms. We summarized the clinical characteristics, treatments, and prognoses of patients with NA. Early identification and reasonable treatment can improve the prognosis of NA.

Background To analyze the possible causes, treatment and outcomes of postoperative pneumothorax in patients with Gross type C esophageal atresia/tracheoesophageal fistula (EA/TEF). Methods Medical records of patients with Gross type C EA/TEF who were diagnosed and treated in Beijing Children’s Hospital from January 2007 to January 2020 were retrospectively collected. They were divided into 2 groups according to whether postoperative pneumothorax occurred. Univariate and multivariate logistic regression analysis were performed to identify risk factors for pneumothorax. Results A total of 188 patients were included, including 85 (45 %) in the pneumothorax group and 103 (55 %) in the non-pneumothorax group. Multivariate logistic regression analysis showed that postoperative anastomotic leakage [ P  < 0.001, OR 3.516 (1.859, 6.648)] and mechanical ventilation [ P  = 0.012, OR 2.399 (1.210, 4.758)] were independent risk factors for pneumothorax after EA/TEF repair. Further analysis of main parameters of mechanical ventilation after surgery showed that none of them were clearly related to the occurrence of pneumothorax. Among the 85 patients with pneumothorax, 33 gave up after surgery and 52 received further treatment [conservative observation (n = 20), pleural puncture (n = 11), pleural closed drainage (n = 9), both pleural puncture and closed drainage (n = 12)]. All of the 52 patients were cured of pneumothorax at discharge. Conclusions Anastomotic leakage and postoperative mechanical ventilation were risk factors for pneumothorax after repair of Gross type C EA/TEF, but the main parameters of mechanical ventilation had no clear correlation with pneumothorax. After symptomatic treatment, the prognosis of pneumothorax was good.