World Journal of Pediatric Surgery最新文献

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Exosome encapsulation of miR-205-5p suppresses neuroblastoma progression by targeting RUNX2. 外泌体包封miR-205-5p通过靶向RUNX2抑制神经母细胞瘤的进展。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-06-24 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2024-000993
Jiaxiang Tang, Qi Liu, Binyi Yang, Hongting Lu
{"title":"Exosome encapsulation of miR-205-5p suppresses neuroblastoma progression by targeting RUNX2.","authors":"Jiaxiang Tang, Qi Liu, Binyi Yang, Hongting Lu","doi":"10.1136/wjps-2024-000993","DOIUrl":"10.1136/wjps-2024-000993","url":null,"abstract":"<p><strong>Objective: </strong>This study investigates the tumor-suppressive role of microRNA (miR)-205-5p in neuroblastoma (NB) and evaluates exosome-mediated delivery of miR-205-5p as a therapeutic strategy.</p><p><strong>Methods: </strong>miR-205-5p expression in NB cells was quantified via quantitative reverse transcription PCR. Functional assays (CCK-8, colony formation, wound healing, Transwell) assessed proliferation, migration, and invasion. Bioinformatic tools and dual-luciferase assays identified miR-205-5p/Runt-related transcription factor 2 (RUNX2) binding. RUNX2 rescue experiments reversed miR-205-5p effects. Exosomes from SH-SY5Y cells transfected with miR-205-5p mimics/NC (negative control) lentiviruses were isolated, characterized, and co-cultured with recipient cells. In vivo, subcutaneous NB xenografts in nude mice were established using OE-miR-205-5p, sh-miR-205-5p, or NC lentiviral cells, followed by exosome injections to evaluate tumor growth.</p><p><strong>Results: </strong>miR-205-5p was downregulated in NB cells. Its overexpression suppressed proliferation, migration, invasion, and tumor growth in vitro and in vivo. RUNX2 was confirmed as a direct target; its restoration reversed miR-205-5p-mediated inhibition. Exosomes efficiently delivered miR-205-5p to recipient cells, downregulating RUNX2 and impairing malignant behaviors. In mice, miR-205-5p-enriched exosomes significantly inhibited tumor progression.</p><p><strong>Conclusions: </strong>Exosome-encapsulated miR-205-5p inhibits NB progression by targeting RUNX2, highlighting its potential as a novel therapeutic modality.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e000993"},"PeriodicalIF":0.8,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12198801/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508642","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}
引用次数: 0
Impact of preoperative chemotherapy cycles on tumor resectability and surgical timing in hepatoblastoma: a retrospective analysis. 肝母细胞瘤术前化疗周期对肿瘤可切除性和手术时机的影响:回顾性分析。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-06-19 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2024-000997
Chengdong Wang, Yeming Wu, Jia Shi, Zhixiang Wu, Fan Lv
{"title":"Impact of preoperative chemotherapy cycles on tumor resectability and surgical timing in hepatoblastoma: a retrospective analysis.","authors":"Chengdong Wang, Yeming Wu, Jia Shi, Zhixiang Wu, Fan Lv","doi":"10.1136/wjps-2024-000997","DOIUrl":"10.1136/wjps-2024-000997","url":null,"abstract":"<p><strong>Background: </strong>Hepatoblastoma (HB) is the most common malignant liver tumor in children. However, the optimal duration of preoperative chemotherapy remains unclear, particularly regarding its impact on tumor size and proximity to critical blood vessels.</p><p><strong>Methods: </strong>This retrospective study analyzed 24 patients with HB treated at Shanghai Xinhua Hospital from 2006 to 2022. All patients underwent neoadjuvant chemotherapy. Tumor size and distances to key vasculature, including the confluence of hepatic veins (COHV) and the portal vein bifurcation (PVB), were measured using CT scans after two and four chemotherapy cycles. Statistical analyses assessed changes in these parameters.</p><p><strong>Results: </strong>The maximum tumor diameter decreased significantly after two cycles of chemotherapy (11.9±2.5 cm to 8.3±2.0 cm, <i>p</i><0.0001) and further to 7.1±1.9 cm after four cycles. The distance to COHV (DSTCOHV) increased from 0.5±0.9 cm to 1.4±1.2 cm after two cycles (<i>p</i><0.001) and to 1.6±1.4 cm after four cycles (<i>p</i><0.0001). However, for distance to PVB (DSTPVB), the increase was more modest, from 0.1±0.3 cm to 0.6±0.5 cm after two cycles (<i>p</i><0.001) and to 0.8±0.6 cm after four cycles (<i>p</i><0.0001). Patients with limited response after two cycles gained minimal benefit from additional chemotherapy.</p><p><strong>Conclusions: </strong>Preoperative chemotherapy significantly reduces tumor size and improves surgical margins, particularly during the first two cycles. For tumors near PVB, prolonged chemotherapy may offer limited benefit, emphasizing the need for individualized treatment planning.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e000997"},"PeriodicalIF":0.8,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12182197/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369305","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}
引用次数: 0
Syndromic variants of biliary atresia. 胆道闭锁的综合征变异。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-06-08 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-001040
Mark Davenport
{"title":"Syndromic variants of biliary atresia.","authors":"Mark Davenport","doi":"10.1136/wjps-2025-001040","DOIUrl":"10.1136/wjps-2025-001040","url":null,"abstract":"<p><p>Biliary atresia (BA) may be characterized as an obliterative cholangiopathy presenting in the newborn period with conjugated jaundice, pale stools, and dark urine. It is usually thought of as an isolated anomaly in otherwise normal infants. However, in a minority, other anomalies may be present, some as defined syndromes, others as a non-random association. The most fully characterized is that of the biliary atresia splenic malformation syndrome seen in about 10% of European and North American series with a typical array of unusual extrahepatic anomalies (<i>e.g.</i>, situs inversus, polysplenia, absence of the inferior vena cava, and a preduodenal portal vein). Its underlying genetic background is obscure in most cases. There are other syndromes with a definite link to BA, such as Cat-Eye syndrome and Kabuki syndrome, and still others that may have a link, such as Zimmerman-Laband syndrome.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e001040"},"PeriodicalIF":0.8,"publicationDate":"2025-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161394/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144302923","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}
引用次数: 0
Wound dehiscence rates decreased in reconstructive surgery of anorectal malformations after introduction of a standardized postoperative treatment program. 引入标准化的术后治疗方案后,肛肠畸形重建手术的伤口裂开率下降。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-06-08 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-001003
Louise Tofft, Christina Granéli, Kristine Hagelsteen, Matilda Wester Fleur, Pernilla Stenström
{"title":"Wound dehiscence rates decreased in reconstructive surgery of anorectal malformations after introduction of a standardized postoperative treatment program.","authors":"Louise Tofft, Christina Granéli, Kristine Hagelsteen, Matilda Wester Fleur, Pernilla Stenström","doi":"10.1136/wjps-2025-001003","DOIUrl":"10.1136/wjps-2025-001003","url":null,"abstract":"<p><strong>Background: </strong>Wound dehiscence is a known complication after anorectal malformations (ARMs) surgery. The aim was to evaluate if a standardized post-posterior sagittal anorectoplasty (PSARP) treatment program decreased wound dehiscence rates.</p><p><strong>Methods: </strong>Wound dehiscence rates within 30 days post-PSARP were compared in a case-control single-center study between patients with a standardized post-PSARP treatment 2017-2023, and a cohort with a non-standardized management 2001-2016. The standardized post-PSARP program comprised a minimum of 3 days of: intravenous antibiotics imipenem+cilastatin (with optional subsequent oral amoxicillin+clavulanic acid+metronidazole), fasting after primary PSARP (no stoma), urinary catheter and regular wound cleansing.</p><p><strong>Results: </strong>A total of 149 patients (61% males) with various ARM subtypes were included of which 51% were reconstructed with stomas. Overall, wound dehiscence developed in 8 of 59 patients (14%) in the standardized post-PSARP program group versus 28 of 90 patients (31%) in the control group (<i>p</i>=0.014). In primary PSARPs (no stoma, 59% males), wound dehiscence developed in 6 of 33 patients (18%) in the standardized post-PSARP program group versus 17 of 40 patients (43%) in the control group (<i>p</i>=0.026).</p><p><strong>Conclusion: </strong>Wound dehiscence rates may be reduced using a standardized post-PSARP treatment program.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e001003"},"PeriodicalIF":0.8,"publicationDate":"2025-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161295/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144302924","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}
引用次数: 0
Genetic background and biliary atresia. 遗传背景和胆道闭锁。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-06-06 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-001023
Yu Meng, Qianhui Yang, Shaowen Liu, Xingyuan Ke, Jianghua Zhan
{"title":"Genetic background and biliary atresia.","authors":"Yu Meng, Qianhui Yang, Shaowen Liu, Xingyuan Ke, Jianghua Zhan","doi":"10.1136/wjps-2025-001023","DOIUrl":"10.1136/wjps-2025-001023","url":null,"abstract":"<p><p>Biliary atresia (BA) is a lethal hepatobiliary disorder in infants characterized by progressive destruction of intrahepatic and extrahepatic bile ducts and obstructive biliary fibrosis. Although hepatic portoenterostomy (Kasai procedure) can temporarily reconstruct bile drainage, persistent postoperative inflammation and hepatic fibrosis still lead to over half of the patients requiring liver transplantation for survival. Epidemiological studies reveal significant geographical and ethnic disparities in BA incidence, suggesting that genetic susceptibility plays an indispensable role in its pathogenesis. This article is based on the multidimensional interactive pathogenic hypothesis of BA of 'embryonic developmental abnormalities, perinatal injury, and dysregulated immune microenvironment' in addition to progressive hepatobiliary fibrosis. We review advances in the genetic and epigenetic regulatory networks of BA with the aim of providing ideas for future genetic research on this disease.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 3","pages":"e001023"},"PeriodicalIF":0.8,"publicationDate":"2025-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161372/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144302922","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}
引用次数: 0
Determination of optimal combined doses of oral midazolam and intranasal dexmedetomidine for use in pediatric magnetic resonance imaging. 小儿磁共振成像中使用口服咪达唑仑和鼻内右美托咪定的最佳联合剂量的确定。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-05-28 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-001000
Haiya Tu, Jing Gao, Chunying Bao, Jialian Zhao, Jian Tang, Yaoqin Hu
{"title":"Determination of optimal combined doses of oral midazolam and intranasal dexmedetomidine for use in pediatric magnetic resonance imaging.","authors":"Haiya Tu, Jing Gao, Chunying Bao, Jialian Zhao, Jian Tang, Yaoqin Hu","doi":"10.1136/wjps-2025-001000","DOIUrl":"10.1136/wjps-2025-001000","url":null,"abstract":"<p><strong>Background: </strong>Sedation is often required for infant and preschool children to obtain clear magnetic resonance imaging (MRI). This study was designed to determine the 95% effective dose (ED<sub>95</sub>) of oral midazolam (MID) and intranasal dexmedetomidine (DEX) in combination for sedation in pediatric MRI.</p><p><strong>Methods: </strong>We have used a biased coin design up-and-down sequential methodology. Initially, 144 patients were split into two groups. A total of 72 patients were randomly assigned to determine the ED<sub>95</sub> of DEX in combination with a fixed dose of 0.5 mg/kg MID, and 72 were given various doses of MID combined with a fixed dose of DEX at 1 µg/kg to determine the ED<sub>95</sub> of MID. ED<sub>95</sub> was calculated using isotonic regression. At last, the plan was to include 225 cases to test the sedation success rate of DEX combined with MID ED<sub>95</sub> dose. Adverse events were recorded.</p><p><strong>Results: </strong>The ED<sub>95</sub> of DEX was 0.89 µg/kg (95% confidence interval (CI) 0.68 to 0.95) combined with a fixed dose of MID at 0.5 mg/kg. The ED<sub>95</sub> of MID was 0.47 mg/kg (95% CI 0.30 to 0.50) combined with a fixed intranasal dose of 1 µg/kg DEX. Using 1 µg/kg DEX combined with 0.5 mg/kg MID, the sedation success rate was 95.1% in a verification group of 225 children.</p><p><strong>Conclusions: </strong>This study reports relatively low ED<sub>95</sub> doses of intranasal DEX and oral MID when DEX is determined with a fixed dose of MID and MID determined with a fixed dose of DEX. The combination provides efficient and safe sedation for young children for MRI scanning. Further validation is required.</p><p><strong>Trial registration number: </strong>ChiCTR2300068611.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 2","pages":"e001000"},"PeriodicalIF":0.8,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12121563/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144182092","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}
引用次数: 0
History of surgery for Hirschsprung disease: a view from Melbourne. 巨结肠病的手术史:来自墨尔本的观点。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-05-26 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2024-000935
Sebastian King, John Hutson
{"title":"History of surgery for Hirschsprung disease: a view from Melbourne.","authors":"Sebastian King, John Hutson","doi":"10.1136/wjps-2024-000935","DOIUrl":"10.1136/wjps-2024-000935","url":null,"abstract":"","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 2","pages":"e000935"},"PeriodicalIF":0.8,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107574/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144162546","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}
引用次数: 0
Japanese biliary atresia registry. 日本胆道闭锁登记。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-05-26 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-001024
Ryuji Okubo, Masaki Nio, Hideyuki Sasaki, Motoshi Wada
{"title":"Japanese biliary atresia registry.","authors":"Ryuji Okubo, Masaki Nio, Hideyuki Sasaki, Motoshi Wada","doi":"10.1136/wjps-2025-001024","DOIUrl":"10.1136/wjps-2025-001024","url":null,"abstract":"<p><p>The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS) to investigate the epidemiology and etiology of biliary atresia and to improve surgical outcomes. The JBAR collects data through initial, liver transplantation, and follow-up questionnaires. Pediatric surgeons from JBAS member institutions and hospitals affiliated with the Japanese Society of Pediatric Surgeons are responsible for registering patients and submitting data through an online system. Each patient is to be followed up for 40 years. As of 2023, 3951 patients had been registered, with 1688 undergoing liver transplantation. The native liver survival rates in the 10th, 20th, and 30th year surveys were 50.5%, 44.4%, and 40.9%, respectively. The overall survival rates in the 10th, 20th, and 30th year surveys were 88.9%, 87.6%, and 85.7%, respectively. The surgical outcome of biliary atresia has markedly improved owing to the cooperation between Kasai portoenterostomy and liver transplantation. A comprehensive Japanese database of patients with biliary atresia (https://jbas.net/en/national-registration/) is now available.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 2","pages":"e001024"},"PeriodicalIF":0.8,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107581/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144162559","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}
引用次数: 0
Bananagram: an alternative to distal loopogram prior to colostomy closure for Hirschsprung disease. 香蕉图:巨结肠疾病结肠造口闭合前远端环图的替代方法。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-05-22 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-000998
Md Sharif Imam, Sanchita Roy, Moumita Kar, Tasmiah Tahera Aziz, Afruzul Alam, Rupam Talukder, Md Mozammel Hoque, Tanvir Kabir Chowdhury, Tahmina Banu
{"title":"Bananagram: an alternative to distal loopogram prior to colostomy closure for Hirschsprung disease.","authors":"Md Sharif Imam, Sanchita Roy, Moumita Kar, Tasmiah Tahera Aziz, Afruzul Alam, Rupam Talukder, Md Mozammel Hoque, Tanvir Kabir Chowdhury, Tahmina Banu","doi":"10.1136/wjps-2025-000998","DOIUrl":"10.1136/wjps-2025-000998","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to investigate if mashed ripe bananas (bananagram) could be used to assess the integrity of the gut before transverse colostomy closure in patients with Hirschsprung disease (HSCR) in a low- and middle-income country tertiary-level hospital.</p><p><strong>Methods: </strong>This is a prospective, single-center, hospital-based cohort study. A feeding tube tip was inserted about 5-8 cm through the distal loop, and 15 mL of mashed banana was introduced. The amount introduced and the spontaneous expulsion of mashed banana were measured. We also measured colonic transit time (CTT), input and expulsion ratio, color of the expulsed banana stream, size of the banana stream passed through the anus and clinical signs of bowel obstruction or perforation after the procedure.</p><p><strong>Results: </strong>From January 2018 to June 2023, 266 HSCR patients with a transverse colostomy were included. The mean±standard deviation (SD) age was 26.45±6.41 months. The median CTT of mashed banana was 8 min (interquartile range (IQR): 4-13 min). The mean amount of mashed banana spontaneously expelled through the anus was 13.25±0.95 mL. More than 90% of patients expelled unchanged mashed bananas, and 9.4% expelled stool-banana mixtures. Banana stream was 0.3-1.5 cm in 257 patients and <0.3 cm in nine patients. These nine patients needed revision pull-through.</p><p><strong>Conclusion: </strong>Bananagram can be performed at the bedside. It is easy to perform, cost-effective, available throughout the year and does not pose radiation hazards.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 2","pages":"e000998"},"PeriodicalIF":0.8,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12104934/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144152039","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}
引用次数: 0
Organoids in biliary atresia. 胆道闭锁的类器官。
IF 0.8 4区 医学
World Journal of Pediatric Surgery Pub Date : 2025-05-15 eCollection Date: 2025-01-01 DOI: 10.1136/wjps-2025-001010
Vincent Chi Hang Lui
{"title":"Organoids in biliary atresia.","authors":"Vincent Chi Hang Lui","doi":"10.1136/wjps-2025-001010","DOIUrl":"10.1136/wjps-2025-001010","url":null,"abstract":"<p><p>Organoids are three-dimensional and self-organizing cell cultures of various lineages that resemble structures and functions of an organ in many ways, and they are versatile tools in disease modeling and patho-mechanistic study of human diseases affecting their tissues of origin. Biliary atresia (BA), a cholangiopathy affecting the bile ducts of the liver, is a heterogeneous and multifaceted liver disease of complex pathogenesis. Cholangiopathies refer to a category of liver diseases that affect the cholangiocytes, the epithelial cells lining the lumen of the biliary trees. Biliary organoids consist of cholangiocytes in a spherical monolayer epithelium, which favorably resembles the structures and functional properties of the bile duct cholangiocytes. Biliary tissue-derived cells, pluripotent stem cells or embryonic stem cells, and hepatic progenitor cells are capable of generating biliary organoids. In the last decade, a considerable advancement has been made in the generation of biliary organoids for modeling liver physiology and pathophysiology. Using biliary organoids, scientists have advanced our knowledge underlying the pathogenic roles of genetic susceptibility, dysregulated hepatobiliary development/structure, environmental factors, and dysregulated immune-inflammatory responses to an injury in BA. This review will summarize and discuss the derivation and the use of biliary organoids in the disease modeling and patho-mechanistic study of BA.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 2","pages":"e001010"},"PeriodicalIF":0.8,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12083310/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144095270","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}
引用次数: 0
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