World Journal of Pediatric Surgery最新文献

{"title":"Global Initiative for Children's Surgery (GICS): a decade in review.","authors":"Soham Bandyopadhyay, Godfrey Sama Philipo, Tahmina Banu, Zaitun Bokhary, Kokila Lakhoo","doi":"10.1136/wjps-2025-001084","DOIUrl":"10.1136/wjps-2025-001084","url":null,"abstract":"","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001084"},"PeriodicalIF":1.3,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12496102/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145233628","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Parental health at preconception and gestational age at birth: evidence from a population-based cohort using double machine learning.","authors":"Yuehong Ding, Lanqing Qu, Jinbiao Zhang, Tiezheng Li, Yunyan Wang, Weize Xu, Die Li","doi":"10.1136/wjps-2025-001078","DOIUrl":"10.1136/wjps-2025-001078","url":null,"abstract":"<p><strong>Background: </strong>Gestational age at birth is shaped by complex maternal and paternal factors prior to conception, yet few studies have integrated multidimensional clinical indicators from both parents to estimate their quantitative effects.</p><p><strong>Methods: </strong>Using a large retrospective cohort with routinely collected preconception health data from both mothers and fathers, we applied a double machine learning (DML) framework combining Lasso, Random Forest and XGBoost regressors to estimate associations between parental biomarkers and gestational age at birth. Model interpretability was enhanced through Shapley Additive Explanations (SHAP) analysis, stratified interaction testing and estimation of individualized treatment effects (ITE).</p><p><strong>Results: </strong>Elevated maternal fasting glucose, alanine aminotransferase, platelet count and anti-hepatitis B core seropositivity were consistently associated with shortened gestational age at birth across all DML models. For instance, a 10 mmol/L increase in fasting glucose corresponded to 2.8-4.9 days shorter gestation. Paternal Treponema pallidum seropositivity and increased monocyte proportion, defined as the fraction of monocytes within total circulating white blood cells, also demonstrated significant associations, with the former linked to gestational shortening and the latter to modest extension. Stratified and interaction analyses revealed that paternal immune markers modified the associations of maternal metabolic indicators with gestational age at birth. SHAP-based interpretation confirmed model consistency and ITE analysis indicated marked heterogeneity, particularly for fasting glucose.</p><p><strong>Conclusions: </strong>This study demonstrates the utility of interpretable DML methods for quantifying the effects of multidimensional preconception health indicators from both parents on gestational age at birth. Our findings support integrating maternal and paternal screening into preconception risk assessments to enable early, targeted prevention of preterm birth. Given the established links between preterm birth and neonatal surgical conditions, early risk identification via parental screening may help inform perinatal care strategies and optimize resource allocation in pediatric surgery.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001078"},"PeriodicalIF":1.3,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12481323/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Is sedation necessary for hydrostatic reduction in ileocolic intussusception? Evaluating procedural outcomes and parents' preferences.","authors":"Angelique Berthelot, Nora Larbi, Cornelius Sloots, Nanko de Graaf, Marieke Witvliet, Claudia Keyzer-Dekker","doi":"10.1136/wjps-2025-001072","DOIUrl":"10.1136/wjps-2025-001072","url":null,"abstract":"<p><strong>Introduction: </strong>The necessity for procedural sedation during hydrostatic reduction for ileocolic intussusception in children is being debated. This study aimed to compare outcomes of procedures with and without sedation, as well as parental preferences.</p><p><strong>Methods: </strong>This retrospective study (2019-2021) reviewed the medical records of children treated for ileocolic intussusception at two hospitals-one that did not provide sedation (non-sedation, NS) and one that did (with-sedation, WS) during hydrostatic reduction. The primary outcomes encompassed success rate, perforation, and recurrence. Parental satisfaction was assessed using a 5-point Likert scale questionnaire.</p><p><strong>Results: </strong>Data from 65 children were included: 42 in the NS group and 23 in the WS group. The hydrostatic reduction success rates were 79% (NS) and 74% (WS) (<i>p</i>=0.670). One colon perforation occurred in each group (<i>p</i>=0.661). Recurrence rates were 14.3% (NS) and 17.4% (WS) (<i>p</i>=0.740). The questionnaire response rates were 48% (NS) and 57% (WS). Of the NS parents, 75% (15/20) were (very) satisfied with the procedure, compared with 85% (11/13) of WS parents (<i>p</i>=0.196). Ten (50%) NS parents would have preferred sedation, while 46% (6/13) of WS parents would have preferred to be present during the procedure.</p><p><strong>Conclusions: </strong>There were no significant differences in success rates, complications, or parental satisfaction between procedures performed with or without sedation during hydrostatic reduction. Effective pain management and a comforting approach are crucial for stress reduction. Shared decision-making with parents regarding the pros and cons of sedation is recommended.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001072"},"PeriodicalIF":1.3,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12443202/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145087478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of prenatal heparin-binding epidermal growth factor (HB-EGF) administration on necrotizing enterocolitis-induced lung injury in a murine model.","authors":"Beverly Giang, Andrei Radulescu, Georgi Dianov Mladenov, Christopher G Wilson","doi":"10.1136/wjps-2025-001034","DOIUrl":"10.1136/wjps-2025-001034","url":null,"abstract":"<p><strong>Background: </strong>Necrotizing enterocolitis (NEC) is a gastrointestinal emergency in premature neonates. NEC is mediated by toll-like receptor-4 (TLR-4) and associated with lung injury. Previously, we showed that prenatal heparin-binding epidermal growth factor (HB-EGF) administration decreases the incidence of intestinal injury in a rat model of NEC. We tested the hypothesis that prenatally administered HB-EGF would decrease TLR-4 activation and lung injury in a murine model.</p><p><strong>Methods: </strong>Pregnant mice were given HB-EGF (800 μg/kg/dose) via intraperitoneal injection prior to cesarean section. Pups were exposed to a NEC model and sacrificed on signs of NEC. We collected tissue, performed histological grading of NEC, evaluated alveolar morphometry, and counted TLR-4-expressing cells by immunohistochemistry, unbiased stereology, and quantified TLR-4 protein via ELISA.</p><p><strong>Results: </strong>Mean alveolar area was significantly different between HB-EGF and control groups compared with NEC only (HB-EGF>NEC; <i>p</i><0.0001; control>NEC; <i>p</i>=0.0008). Alveolar wall area was significantly decreased in HB-EGF and control groups versus NEC group (<i>p</i><0.0001). TLR-4-expressing cells were greater in the NEC group versus HB-EGF and control groups (<i>p</i>=0.002). TLR-4 protein was increased in pups exposed to the NEC protocol compared with control (<i>p</i>=0.005 for NEC only; <i>p</i>=0.0004 for HB-EGF treated). There was no difference in TLR-4 protein between HB-EGF and NEC groups.</p><p><strong>Conclusions: </strong>Our results suggest that prenatal HB-EGF administration preserves lung morphometry and decreases TLR-4 in a murine model of NEC. Possibly, the administration of HB-EGF prenatally to pregnant mothers at risk of delivering a premature infant susceptible to NEC may prevent lung injury.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001034"},"PeriodicalIF":1.3,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12414226/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145024232","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical treatment of proximal femur unicameral bone cysts in children: a retrospective single-center study.","authors":"Haibing Li, Xiao Fen Niu, Hang Su, Wensong Ye, Xin Tang","doi":"10.1136/wjps-2025-001014","DOIUrl":"10.1136/wjps-2025-001014","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to present clinical and radiological outcomes of surgical treatment for proximal femur unicameral bone cysts (UBCs) in children.</p><p><strong>Methods: </strong>Pediatric patients recruited from our institution between 2015 and 2024 with proximal femur UBC were analyzed retrospectively. Patients were divided into two groups according to whether internal fixation was used. Demographics, cyst activity, cyst area, healing time of pathological fractures and cysts, clinical and radiological outcomes, time to activity and complications were analyzed.</p><p><strong>Results: </strong>Thirty-seven pediatric patients were included in this study. There were 18 patients in the non-internal fixation group and 19 patients in the internal fixation. There was no significant difference between these two groups in terms of age, duration of follow-up, fracture at diagnosis, cyst activity or healing time of pathological fractures and cysts. Cyst area in the internal fixation group was 1609±1131 mm², which was significantly greater than that in the group without internal fixation (936±597 mm², <i>p</i><0.05). There was no significant difference in clinical or radiological outcomes between the two groups. Patients who were treated with internal fixation were able to return to activity significantly sooner than those who were not (5.9 months <i>vs.</i> 7.7 months; <i>p</i><0.05). There was no significant difference in complications between the two groups (<i>p</i>=1.000).</p><p><strong>Conclusions: </strong>Surgical treatment of proximal femoral UBCs in children remains a challenge. The use of internal fixation is beneficial for accelerating the healing process and reducing the time to activity.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001014"},"PeriodicalIF":1.3,"publicationDate":"2025-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12374617/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144971421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Robotic liver resection for hepatic focal nodular hyperplasia in children: comparison with open surgery.","authors":"Xuan Wu, Min He, Yinbing Tang, Ming Chen, Jiabin Cai, Lifeng Zhang, Yuwei Wang, Ting Tao, Jinhu Wang","doi":"10.1136/wjps-2025-001028","DOIUrl":"10.1136/wjps-2025-001028","url":null,"abstract":"<p><strong>Background: </strong>Robot-assisted surgery is becoming increasingly used in pediatric oncology. The present study aimed to evaluate the feasibility and safety of robotic liver resection (RLR) for hepatic focal nodular hyperplasia (FNH) in children and compare surgical outcomes between RLR and open liver resection (OLR).</p><p><strong>Methods: </strong>Pediatric patients with liver FNH undergoing lesion resection between January 2020 and June 2024 were included in the study. Patient demographics, operative details, postoperative outcomes, and follow-up were recorded and analyzed.</p><p><strong>Results: </strong>A total of 20 patients were included in this study. Twelve patients underwent RLR and eight underwent OLR. In the RLR group, the median age was 93.1 months (range, 28-134 months) with a median weight of 32.4 kg (range, 9.7-80 kg). The median maximum tumor diameter at operation was 62.6 mm (range, 49-80 mm) and the median tumor volume was 94.3 mL (range, 35-254.1 mL). Operative time was 168.5 min (range, 116-245 min), intraoperative blood loss was 23.3 mL (range, 5-50 mL) and the length of postoperative hospital stay was 5.7 days (range, 4-11 days). There was a significant difference (<i>p</i><0.05) between the RLR and OLR groups for: age (93.1 months <i>vs.</i> 137.6 months), maximum tumor diameter (62.6 mm <i>vs.</i> 98 mm), tumor volume (94.3 mL <i>vs.</i> 496.2 mL), operative time (168.5 min <i>vs.</i> 281.4 min), blood loss (23.3 mL <i>vs.</i> 288.7 mL), and length of postoperative hospital stay (5.7 days <i>vs.</i> 9.5 days). There was a borderline significant association between surgical approach (RLR/OLR) and fluctuation in the magnitude of Alanine aminotransferase (ALT) (odds ratio=0.004, 95% confidence interval: 0.000 to 1.096, <i>p</i>=0.05).</p><p><strong>Conclusions: </strong>Our initial experience suggested that RLR for hepatic FNH in children was both feasible and safe. Tumors in the RLR group were significantly smaller than the OLR group: it proved possible to excise tumors larger than 250 mL in volume.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001028"},"PeriodicalIF":1.3,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12366609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144971409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Brainstem microglial cell morphology in neonatal rats with necrotizing enterocolitis and the effects of prenatal heparin-binding epidermal growth factor-like growth factor.","authors":"Vonita Chawla, Yomara S Mendez, Lorraine C Siebold, Julia C Vickery, Marla A Sacks, Georgi D Mladenov, Andrei Radulescu, Christopher G Wilson","doi":"10.1136/wjps-2025-001049","DOIUrl":"10.1136/wjps-2025-001049","url":null,"abstract":"<p><strong>Abstract: </strong></p><p><strong>Background: </strong>Necrotizing enterocolitis (NEC) is associated with increased neurodevelopmental impairment. Gut-brain interactions through the brainstem may be central to NEC-related microglia-driven neuroinflammation. Heparin-binding epidermal growth factor-like growth factor (HB-EGF) has intestinal protective properties and is a potential therapy for NEC. The aim of this study is to test the hypothesis that HB-EGF in pregnant rats reduces both NEC incidence and proinflammatory changes in the brainstem microglia of newborn rats.</p><p><strong>Methods: </strong>We compared four experimental groups, HB-EGF⁺/NEC^-, HB-EGF^-/NEC^-, HB-EGF⁺/NEC⁺ and HB-EGF^-/NEC⁺, depending on whether HB-EGF was given prenatally, and whether the newborn rats underwent the NEC induction protocol. We stained brainstem microglia and performed fractal analyses to provide objective measures of morphological changes.</p><p><strong>Results: </strong>NEC incidence was lower in the HB-EGF⁺/NEC⁺ group (<i>n</i>=64, <i>p</i><0.005) than in the HB-EGF^-/NEC⁺ group. Brainstem microglia of breastfed rats had a larger cell area, perimeter, roughness, and less circularity compared with smaller, denser, compact cells in the NEC⁺ pups (<i>p</i><0.0001, <i>n</i>=320 cells). HB-EGF⁺/NEC⁺ and HB-EGF^-/NEC⁺ pups had similar-appearing microglia.</p><p><strong>Conclusions: </strong>Prenatal HB-EGF treatment reduces NEC incidence in neonatal rats. NEC-related proinflammatory changes are seen in microglial cells present in crucial centers controlling the gut-brain pathway. HB-EGF has a growth-promoting effect on healthy microglia in the offspring but does not avert microglial activation in the brainstem of newborn rats with NEC.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 4","pages":"e001049"},"PeriodicalIF":1.3,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12336531/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144837939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical pathology of Hirschsprung disease (HSCR).","authors":"Hector L Monforte, Michael Wilsey, Raquel Gonzalez","doi":"10.1136/wjps-2024-000882","DOIUrl":"10.1136/wjps-2024-000882","url":null,"abstract":"<p><p>The practice, art and science of surgical pathology for disease entities evolves continuously. Standards for diagnosis and management of Hirschsprung disease (HSCR), variants and related dysmotility disorders are no exception. Morphologic parameters that withstand the test of time still fulfill aims for 'personalized' and precision medicine. The expert management of these patients relies on critical points of interaction between pathologists, gastroenterologists and pediatric surgeons for: (1) diagnosis or exclusion within the HSCR spectrum, (2) intraoperative determination of the extent of aganglionosis and HSCR transition zone features, (3) validation of optimal proximal margin for primary pull-through or ostomy site and (4) confirmation of specimen adequacy and diagnoses in the event of postoperative dysfunction or need of outside case material review for referred patients. Additional roles in pathologists' scope include specimen triage for HSCR specialized ancillary procedures and other intestinal motility disorders, and in contributing surgical specimens for research collaboratives. This review highlights the cumulative experience of the authors' integrated practice model for patients with HSCR and explores the approach for patients with prototypical or challenging presentations and management. It is intended primarily for pediatric surgeons and gastroenterologists who seek to assimilate surgical pathology practice based on available relevant HSCR literature, recognizing that each patient poses a unique constellation of functional manifestations and histopathologic expression of Hirschsprung Disease.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e000882"},"PeriodicalIF":1.3,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12314976/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144776318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictive value of plasma zonulin for postoperative Hirschsprung-associated enterocolitis.","authors":"Lihua Wu, Ya Gao, Ruijie Zhou, Ping Xiao, Zhen Zhang, Bo Li, Agostino Pierro, Long Li, Qian Jiang, Qi Li","doi":"10.1136/wjps-2025-001057","DOIUrl":"10.1136/wjps-2025-001057","url":null,"abstract":"<p><strong>Background: </strong>Hirschsprung's disease (HSCR) is a functional obstruction of the gastrointestinal tract characterized by abdominal distension, constipation, and vomiting. The protein zonulin serves as a biomarker for intestinal permeability. We sought to explore the changes in plasma zonulin levels in patients with HSCR and to assess its predictive role in the development of postoperative Hirschsprung-associated enterocolitis (HAEC).</p><p><strong>Methods: </strong>There are 60 patients with HSCR were recruited for this study, categorized into short-segment disease (S-HSCR) (<i>n</i>=33), long-segment disease (L-HSCR) (<i>n</i>=15), and total colonic aganglionosis (TCA) (<i>n</i>=12). Venous blood samples were taken from all participants before and after pull-through surgery. Plasma concentrations of zonulin were determined using an ELISA. Immunohistochemistry (IHC) analysis was conducted to evaluate the expression of zonula occludens-1 (ZO-1) and occludin in colonic tissues. Hematoxylin and Eosin stained (H&E-stained) sections were used to evaluate the degree of inflammation in the dilated (ganglionic) segment. Postoperative outcomes were assessed through a combination of online questionnaires and telephone interviews. Diagnostic threshold for HAEC was based on clinical symptom sets and the HAEC scoring system developed previously.</p><p><strong>Results: </strong>Preoperative zonulin levels in patients with TCA were statistically lower than those in patients with S-HSCR (<i>p</i>=0.008) and L-HSCR (<i>p</i>=0.028). The incidence of postoperative HAEC was 16.7%, 57.1%, and 14.3% in TCA, L-HSCR, and S-HSCR groups, respectively. Patients who experienced an increase in plasma zonulin levels of more than 1.5 times those on the first day after surgery had a higher risk of developing HAEC (<i>p</i>=0.005). IHC staining further confirmed decreased expression of ZO-1 and occludin in colonic tissues of patients with HSCR who experienced postoperative HAEC.</p><p><strong>Conclusion: </strong>Preoperative zonulin levels were lowest in the TCA group. The change in zonulin levels on the first day after surgery can serve as a useful indicator for predicting the risk of postoperative HAEC occurrence.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e001057"},"PeriodicalIF":0.8,"publicationDate":"2025-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12228470/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144576451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Strengthening collaboration within Europe: biliary atresia and the rise of the European Reference Networks.","authors":"Omid Madadi-Sanjani, Christoph Slavetinsky, Marie Uecker","doi":"10.1136/wjps-2025-001025","DOIUrl":"10.1136/wjps-2025-001025","url":null,"abstract":"<p><p>Biliary atresia (BA) is a rare and devastating cholangiopathy with an incidence of 1:15 000-20 000 in Europe. There is a consensus that BA and related rare diseases should be managed at centers of expertise. However, current BA care in Europe is heterogeneous, with decentralized treatment in the majority of European countries. The varying outcomes in Europe, referring to overall and native liver survival following Kasai procedure, have led to an ongoing discussion on international clinical and scientific collaborations. In 2017, the European Reference Networks (ERN) have been launched by the European Commission to improve the treatment of rare diseases. The ERNs bring together centers of expertise, which undergo qualification and monitoring processes. The European Reference Network on Rare Hepatological Diseases (ERN RARE-LIVER) represents a network of medical practitioners and patient representatives with the goal of improving care for rare liver diseases with BA as one key disorder. Exchange programs, digital case discussions and the endorsement of clinical studies are part of the RARE-LIVER agenda, including the prospective European Biliary Atresia Registry (EBAR), which has recently been launched. Such sustainable joint European efforts and strategies are crucial to improve BA outcomes in the short term and long term.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e001025"},"PeriodicalIF":0.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12211850/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144545102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}