Purpose: To evaluate the efficacy and safety of perioperative sodium bicarbonate Ringer's solution (BRS) in biliary atresia (BA) infants undergoing Kasai portoenterostomy (KPE).
Methods: This study was conducted in 11 pediatric medical centers across China from 2020 to 2022. Infants were randomly assigned to either the BRS group (n = 64) or the control crystalloids group (n = 56) for perioperative fluid management. Anesthesia and fluid management were standardized. Primary outcomes were arterial blood pH, bicarbonate, and base excess; secondary outcomes included changes in other blood parameters and adverse events.
Results: The baseline characteristics of the two groups were similar. During KPE, BRS more effectively maintained arterial blood pH, bicarbonate, and base excess during KPE with no electrolyte abnormalities. Three days after KPE, the BRS group had lower potassium levels. Drug safety evaluation showed no significant differences, and the incidence of adverse events was comparable.
Conclusion: BRS effectively maintained the acid-base status of BA infants during KPE. With no significant safety concerns, it is suggested that BRS is a suitable option for perioperative fluid management.
{"title":"Efficacy of sodium bicarbonate Ringer's solution for perioperative fluid management in infants with biliary atresia: a prospective multicenter study.","authors":"Hongyi Zhang, Pei Wang, Ruohui Li, Chonggao Zhou, Kuai Chen, Zhe Wen, Yuanmei Liu, Xiang Liu, Qi Chen, Jinfa Tou, Hongxia Ren, Mingman Zhang, Feng Chen, Jiexiong Feng","doi":"10.1007/s00383-025-06021-x","DOIUrl":"https://doi.org/10.1007/s00383-025-06021-x","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate the efficacy and safety of perioperative sodium bicarbonate Ringer's solution (BRS) in biliary atresia (BA) infants undergoing Kasai portoenterostomy (KPE).</p><p><strong>Methods: </strong>This study was conducted in 11 pediatric medical centers across China from 2020 to 2022. Infants were randomly assigned to either the BRS group (n = 64) or the control crystalloids group (n = 56) for perioperative fluid management. Anesthesia and fluid management were standardized. Primary outcomes were arterial blood pH, bicarbonate, and base excess; secondary outcomes included changes in other blood parameters and adverse events.</p><p><strong>Results: </strong>The baseline characteristics of the two groups were similar. During KPE, BRS more effectively maintained arterial blood pH, bicarbonate, and base excess during KPE with no electrolyte abnormalities. Three days after KPE, the BRS group had lower potassium levels. Drug safety evaluation showed no significant differences, and the incidence of adverse events was comparable.</p><p><strong>Conclusion: </strong>BRS effectively maintained the acid-base status of BA infants during KPE. With no significant safety concerns, it is suggested that BRS is a suitable option for perioperative fluid management.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"107"},"PeriodicalIF":1.5,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143812060","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-08DOI: 10.1007/s00383-025-05998-9
Gaurav Ravi Kumar, Anand Chetan Shah, Anand Murali, Shalini Shree Krishnamurthy, Venkatraman Radhakrishnan, Anand Raja
Purpose: Wilms tumor (WT) is the most common renal neoplasm in children and the third most common pediatric cancer globally. Though survival rates exceed 90% in high-income countries, it is suboptimal in low- and middle-income countries (LMICs) due to late presentation, advanced disease stages, and limited healthcare access. We retrospectively analyzed WT patients undergoing definitive surgery between 2011 and 2023 from a single LMIC institution.
Methods: Patients were classified into Group I (upfront surgery as per COG guidelines) and Group II (neoadjuvant chemotherapy followed by surgery as per SIOP guidelines). Surgical outcomes, post-operative morbidity, and oncological outcomes, including overall survival (OS) and event-free survival (EFS), were analyzed. Thirty-six patients were included: ten in Group I and twenty-six in Group II.
Results: Significant post-operative complications occurred in 15.3% of Group II, while none were noted in Group I. Lymph node involvement rates were 10 and 3.8% in Groups I and II, respectively. The 5-year OS and EFS for the cohort were 90.9% (Group I 83%, Group II 92%) and 91.4% (Group I 80%, Group II 96%).
Conclusion: Risk-adapted strategy, multi-disciplinary decision-making, adherence to international protocols can emulate survival outcomes comparable to high-income countries, even in LMICs.
{"title":"Surgical practices and oncological outcomes of Wilms tumor in a resource limited setting: a risk-adapted approach.","authors":"Gaurav Ravi Kumar, Anand Chetan Shah, Anand Murali, Shalini Shree Krishnamurthy, Venkatraman Radhakrishnan, Anand Raja","doi":"10.1007/s00383-025-05998-9","DOIUrl":"https://doi.org/10.1007/s00383-025-05998-9","url":null,"abstract":"<p><strong>Purpose: </strong>Wilms tumor (WT) is the most common renal neoplasm in children and the third most common pediatric cancer globally. Though survival rates exceed 90% in high-income countries, it is suboptimal in low- and middle-income countries (LMICs) due to late presentation, advanced disease stages, and limited healthcare access. We retrospectively analyzed WT patients undergoing definitive surgery between 2011 and 2023 from a single LMIC institution.</p><p><strong>Methods: </strong>Patients were classified into Group I (upfront surgery as per COG guidelines) and Group II (neoadjuvant chemotherapy followed by surgery as per SIOP guidelines). Surgical outcomes, post-operative morbidity, and oncological outcomes, including overall survival (OS) and event-free survival (EFS), were analyzed. Thirty-six patients were included: ten in Group I and twenty-six in Group II.</p><p><strong>Results: </strong>Significant post-operative complications occurred in 15.3% of Group II, while none were noted in Group I. Lymph node involvement rates were 10 and 3.8% in Groups I and II, respectively. The 5-year OS and EFS for the cohort were 90.9% (Group I 83%, Group II 92%) and 91.4% (Group I 80%, Group II 96%).</p><p><strong>Conclusion: </strong>Risk-adapted strategy, multi-disciplinary decision-making, adherence to international protocols can emulate survival outcomes comparable to high-income countries, even in LMICs.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"105"},"PeriodicalIF":1.5,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143812066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-08DOI: 10.1007/s00383-025-06009-7
YiWei Fang, Dan Yang, Ning Sun, HongChen Song, WeiPing Zhang, YunMan Tang, LuGang Huang, Yi Yang, Min Chao, Hong Ma, JingTi Zhang, XuHui Zhang, ShouLin Li, Ning Li, Chao Chen, DaWei He, WenBo Wu, Hua Xie, Yong Guan, Yanfang Yang, Jianguo Zhang
Purpose: This multicenter study aimed to identify risk factors for postoperative complications following tubularized incised plate urethroplasty (TIP) in hypospadias patients and develop a clinical prediction model.
Methods: Retrospective data from 17 tertiary centers (2018-2021) were analyzed.
Inclusion criteria: primary TIP surgery with complete anatomical and follow-up data.
Exclusion criteria: prior urethral surgery, genetic disorders, or incomplete records. Multivariate logistic regression identified independent predictors. A nomogram model was constructed and internally validated using bootstrapping (1000 resamples). Discrimination (AUC) and calibration (Hosmer-Lemeshow test) were assessed.
Results: Among 493 patients (median follow-up: 42 months), the complication rate was 23.7% (117/493), including urethrocutaneous fistula (17.6%), stricture (6.5%), and diverticulum (2.8%). Independent predictors were: post-incision urethral plate width (OR: 0.01, 95% CI 0-0.05), glans width (OR: 0.81, 95% CI 0.66-0.98), and urethral defect length (OR: 1.47, 95% CI 1.00-2.16). The nomogram demonstrated moderate discrimination (AUC: 0.723, 95% CI 0.668-0.777) and good calibration (Hosmer-Lemeshow P = 0.382).
Conclusion: A clinical prediction model incorporating post-incision urethral plate width, glans width, and urethral defect length showed acceptable predictive accuracy for TIP complications.
{"title":"Development of a clinical prediction model for postoperative complications following tubularized incised plate urethroplasty: a multicenter study.","authors":"YiWei Fang, Dan Yang, Ning Sun, HongChen Song, WeiPing Zhang, YunMan Tang, LuGang Huang, Yi Yang, Min Chao, Hong Ma, JingTi Zhang, XuHui Zhang, ShouLin Li, Ning Li, Chao Chen, DaWei He, WenBo Wu, Hua Xie, Yong Guan, Yanfang Yang, Jianguo Zhang","doi":"10.1007/s00383-025-06009-7","DOIUrl":"https://doi.org/10.1007/s00383-025-06009-7","url":null,"abstract":"<p><strong>Purpose: </strong>This multicenter study aimed to identify risk factors for postoperative complications following tubularized incised plate urethroplasty (TIP) in hypospadias patients and develop a clinical prediction model.</p><p><strong>Methods: </strong>Retrospective data from 17 tertiary centers (2018-2021) were analyzed.</p><p><strong>Inclusion criteria: </strong>primary TIP surgery with complete anatomical and follow-up data.</p><p><strong>Exclusion criteria: </strong>prior urethral surgery, genetic disorders, or incomplete records. Multivariate logistic regression identified independent predictors. A nomogram model was constructed and internally validated using bootstrapping (1000 resamples). Discrimination (AUC) and calibration (Hosmer-Lemeshow test) were assessed.</p><p><strong>Results: </strong>Among 493 patients (median follow-up: 42 months), the complication rate was 23.7% (117/493), including urethrocutaneous fistula (17.6%), stricture (6.5%), and diverticulum (2.8%). Independent predictors were: post-incision urethral plate width (OR: 0.01, 95% CI 0-0.05), glans width (OR: 0.81, 95% CI 0.66-0.98), and urethral defect length (OR: 1.47, 95% CI 1.00-2.16). The nomogram demonstrated moderate discrimination (AUC: 0.723, 95% CI 0.668-0.777) and good calibration (Hosmer-Lemeshow P = 0.382).</p><p><strong>Conclusion: </strong>A clinical prediction model incorporating post-incision urethral plate width, glans width, and urethral defect length showed acceptable predictive accuracy for TIP complications.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"106"},"PeriodicalIF":1.5,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143812042","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-04-05DOI: 10.1007/s00383-025-06011-z
Jia You, Gang Li
{"title":"Letter to \"How Many Lymph Nodes are Enough in Paratesticular Rhabdomyosarcoma?\"","authors":"Jia You, Gang Li","doi":"10.1007/s00383-025-06011-z","DOIUrl":"https://doi.org/10.1007/s00383-025-06011-z","url":null,"abstract":"","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"104"},"PeriodicalIF":1.5,"publicationDate":"2025-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143788681","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Purpose: To compare the outcomes of transvesicoscopic Cohen ureteral reimplantation (Cohen technique, Cohen group) and transvesicoscopic Politano-Leadbetter ureteral reimplantation (Politano-Leadbetter technique, Politano-Leadbetter group) in the treatment of ureterovesical junction obstruction (UVJO) in children.
Methods: From March 2008 to January 2024, 105 patients underwent Cohen technique and 87 underwent Politano-Leadbetter technique. The demographic data, perioperative parameters, surgical outcomes, and postoperative complications of the two groups were analyzed and compared.
Results: The operation time in the Cohen group was shorter than that in the Politano-Leadbetter group (128.2 ± 30.7 vs. 143.1 ± 31.5 min, p < 0.05), but the improvement in hydronephrosis 6 months after surgery in the Politano-Leadbetter group was better than that in the Cohen group (1.48 ± 0.73 vs. 1.63 ± 0.57 cm, p < 0.05). The success rates in the two groups were similar (97.1% vs. 96.6%, p = 0.855). There was no statistical difference in postoperative hematuria time, catheter retention time, hospital stay, and improvement in ureteral dilation between the two groups.
Conclusions: Two minimally invasive surgical methods via the bladder approach for UVJO have a similar results. Compared to Cohen technique, Politano-Leadbetter technique without change the direction of the ureter, but the surgical time is longer.
{"title":"Comparison of transvesicoscopic Cohen and transvesicoscopic Politano-Leadbetter ureteral reimplantation in the treatment of ureterovesical junction obstruction (UVJO) in children: a single-center long-term follow-up study.","authors":"Guanglun Zhou, Yefeng Zeng, Shunqi Liang, Xiaodong Liu, Xuerui Sun, Shoulin Li","doi":"10.1007/s00383-025-06005-x","DOIUrl":"10.1007/s00383-025-06005-x","url":null,"abstract":"<p><strong>Purpose: </strong>To compare the outcomes of transvesicoscopic Cohen ureteral reimplantation (Cohen technique, Cohen group) and transvesicoscopic Politano-Leadbetter ureteral reimplantation (Politano-Leadbetter technique, Politano-Leadbetter group) in the treatment of ureterovesical junction obstruction (UVJO) in children.</p><p><strong>Methods: </strong>From March 2008 to January 2024, 105 patients underwent Cohen technique and 87 underwent Politano-Leadbetter technique. The demographic data, perioperative parameters, surgical outcomes, and postoperative complications of the two groups were analyzed and compared.</p><p><strong>Results: </strong>The operation time in the Cohen group was shorter than that in the Politano-Leadbetter group (128.2 ± 30.7 vs. 143.1 ± 31.5 min, p < 0.05), but the improvement in hydronephrosis 6 months after surgery in the Politano-Leadbetter group was better than that in the Cohen group (1.48 ± 0.73 vs. 1.63 ± 0.57 cm, p < 0.05). The success rates in the two groups were similar (97.1% vs. 96.6%, p = 0.855). There was no statistical difference in postoperative hematuria time, catheter retention time, hospital stay, and improvement in ureteral dilation between the two groups.</p><p><strong>Conclusions: </strong>Two minimally invasive surgical methods via the bladder approach for UVJO have a similar results. Compared to Cohen technique, Politano-Leadbetter technique without change the direction of the ureter, but the surgical time is longer.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"103"},"PeriodicalIF":1.5,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Bowel perforation is a severe complication in neonates with Hirschsprung disease (HD), particularly during the neonatal period. Identifying risk factors may aid in early detection and intervention.
Methods: We retrospectively analyzed the clinical data of 300 neonates diagnosed with HD at our hospital from 2007 to 2024. The patients were divided into bowel perforation and non-perforation groups based on whether they developed bowel perforation during the neonatal period. The clinical characteristics of both groups were compared. Univariate and multivariate logistic regression analyses were performed to identify the associated risk factors, including general information, clinical symptoms, examination results, and birth history.
Results: A total of 300 neonates with HD were included in this study, with a bowel perforation rate of 6.0% (18/300). The sites of perforation were the sigmoid colon (44.4%, 8/18), descending colon (27.8%, 5/18), ascending colon (11.1%, 2/18), terminal ileum (11.1%, 2/18), and transverse colon (5.6%, 1/18). The results of univariate and multivariate logistic regression analyses revealed that age at admission < 5 days, CRP > 20.5 mg/L, and radiographic findings of intestinal wall pneumatosis were independent risk factors for bowel perforation in neonates with HD, with OR (95% CI) of 4.709 (1.187, 18.684), 35.185 (9.394, 131.782), and 9.667 (2.264, 41.278), respectively.
Conclusion: The incidence of bowel perforation in neonates with Hirschsprung disease is 6.0%. Bowel perforation in neonates with HD is associated with early age at admission, elevated CRP, and intestinal wall pneumatosis.
{"title":"Risk factors of bowel perforation in neonates with Hirschsprung disease.","authors":"Zhaozhou Liu, Yanan Zhang, Dayan Sun, Yongwei Chen, Weihong Guo, Jingbin Du, Jinshi Huang","doi":"10.1007/s00383-025-05999-8","DOIUrl":"https://doi.org/10.1007/s00383-025-05999-8","url":null,"abstract":"<p><strong>Background: </strong>Bowel perforation is a severe complication in neonates with Hirschsprung disease (HD), particularly during the neonatal period. Identifying risk factors may aid in early detection and intervention.</p><p><strong>Methods: </strong>We retrospectively analyzed the clinical data of 300 neonates diagnosed with HD at our hospital from 2007 to 2024. The patients were divided into bowel perforation and non-perforation groups based on whether they developed bowel perforation during the neonatal period. The clinical characteristics of both groups were compared. Univariate and multivariate logistic regression analyses were performed to identify the associated risk factors, including general information, clinical symptoms, examination results, and birth history.</p><p><strong>Results: </strong>A total of 300 neonates with HD were included in this study, with a bowel perforation rate of 6.0% (18/300). The sites of perforation were the sigmoid colon (44.4%, 8/18), descending colon (27.8%, 5/18), ascending colon (11.1%, 2/18), terminal ileum (11.1%, 2/18), and transverse colon (5.6%, 1/18). The results of univariate and multivariate logistic regression analyses revealed that age at admission < 5 days, CRP > 20.5 mg/L, and radiographic findings of intestinal wall pneumatosis were independent risk factors for bowel perforation in neonates with HD, with OR (95% CI) of 4.709 (1.187, 18.684), 35.185 (9.394, 131.782), and 9.667 (2.264, 41.278), respectively.</p><p><strong>Conclusion: </strong>The incidence of bowel perforation in neonates with Hirschsprung disease is 6.0%. Bowel perforation in neonates with HD is associated with early age at admission, elevated CRP, and intestinal wall pneumatosis.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"102"},"PeriodicalIF":1.5,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753996","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-25DOI: 10.1007/s00383-025-06000-2
Yiping Xu, Jiankun Liang, Yurong Cai, Zhe Wen, Jianguo Wang
Background: The primary pathogenetic factor for biliary atresia is an atypical immune response. Previous studies have suggested that the expression of B cell-activating factor is upregulated in BA and BAFF is related to T-cell function. However, the way in which BAFF influences T cell function in BA is still not fully clarified. We therefore investigated the effects of BAFF on T cells in experimental biliary atresia.
Methods: BAFF levels were measured in serum and liver of patients with BA and controls. Immunofluorescence analysis was performed to assess the co-localization of BAFF with myeloid cells (CD14 or CD68) in liver biopsies. A multiplex assay (MAGPIX) was used to measure the levels of Th1 [interferon (IFN)-γ], Th2 [interleukin (IL)-4], and Th17 (IL-17). IFN-γ expression was measured in CD4+ and CD8+ T cells in vitro. An anti-BAFF antibody was administered in a rhesus rotavirus (RRV)-induced BA model to assess the effect of BAFF. Finally, survival, histology, liver function index, liver immune cell subsets, and cytokine production were analyzed.
Results: BAFF expression was significantly higher in patients with BA than controls. Furthermore, BAFF levels in liver supernatants significantly correlated with liver inflammation severity in patients with BA. In addition, BAFF levels positively correlated with IFN-γ levels. Serum BAFF combined with GGT may offer a more reliable approach for confirming the diagnosis of BA. In vitro experiments revealed that BAFF increased IFN-γ expression in CD4+ and CD8+ T cells of infants with BA. BAFF inhibition was associated with a decrease in portal tract inflammation and IFN-γ expression in CD4+ and CD8+ T cells, with the simultaneous expansion of regulatory T cells in an RRV-induced BA model.
Conclusion: BAFF may participate in inflammatory responses in BA by affecting T cells, suggesting the potential role of BAFF in BA pathogenesis.
{"title":"Blockade of B-cell-activating factor may alleviate liver injury by restraining interferon-γ expression in T cells in experimental biliary atresia.","authors":"Yiping Xu, Jiankun Liang, Yurong Cai, Zhe Wen, Jianguo Wang","doi":"10.1007/s00383-025-06000-2","DOIUrl":"https://doi.org/10.1007/s00383-025-06000-2","url":null,"abstract":"<p><strong>Background: </strong>The primary pathogenetic factor for biliary atresia is an atypical immune response. Previous studies have suggested that the expression of B cell-activating factor is upregulated in BA and BAFF is related to T-cell function. However, the way in which BAFF influences T cell function in BA is still not fully clarified. We therefore investigated the effects of BAFF on T cells in experimental biliary atresia.</p><p><strong>Methods: </strong>BAFF levels were measured in serum and liver of patients with BA and controls. Immunofluorescence analysis was performed to assess the co-localization of BAFF with myeloid cells (CD14 or CD68) in liver biopsies. A multiplex assay (MAGPIX) was used to measure the levels of Th1 [interferon (IFN)-γ], Th2 [interleukin (IL)-4], and Th17 (IL-17). IFN-γ expression was measured in CD4<sup>+</sup> and CD8<sup>+</sup> T cells in vitro. An anti-BAFF antibody was administered in a rhesus rotavirus (RRV)-induced BA model to assess the effect of BAFF. Finally, survival, histology, liver function index, liver immune cell subsets, and cytokine production were analyzed.</p><p><strong>Results: </strong>BAFF expression was significantly higher in patients with BA than controls. Furthermore, BAFF levels in liver supernatants significantly correlated with liver inflammation severity in patients with BA. In addition, BAFF levels positively correlated with IFN-γ levels. Serum BAFF combined with GGT may offer a more reliable approach for confirming the diagnosis of BA. In vitro experiments revealed that BAFF increased IFN-γ expression in CD4<sup>+</sup> and CD8<sup>+</sup> T cells of infants with BA. BAFF inhibition was associated with a decrease in portal tract inflammation and IFN-γ expression in CD4<sup>+</sup> and CD8<sup>+</sup> T cells, with the simultaneous expansion of regulatory T cells in an RRV-induced BA model.</p><p><strong>Conclusion: </strong>BAFF may participate in inflammatory responses in BA by affecting T cells, suggesting the potential role of BAFF in BA pathogenesis.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"101"},"PeriodicalIF":1.5,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-24DOI: 10.1007/s00383-025-05996-x
Hao Chen, Zhu-Xin Chen, Guo-Qing Shi
This review examines the common risk factors associated with anastomotic stricture (AS) following esophageal atresia (EA) repair and evaluates the current understanding and clinical practices concerning esophageal dilation, which remains the predominant therapeutic approach. We present emerging evidence on adjunctive therapies for recurrent and refractory strictures, including esophageal stenting, endoscopic incision therapy, mitomycin C application, local steroid injections, cell sheet transplantation, and surgical interventions. A comprehensive literature review was conducted using PubMed, with a cutoff date of December 31, 2024, encompassing all relevant studies and reviews pertinent to this topic, with a particular emphasis on refractory and recurrent strictures. Given the scarcity of evidence-based data on AS in children with EA, we also critically analyze findings from adult literature and studies addressing esophageal strictures caused by various etiologies to provide a more comprehensive understanding. The pathogenesis of AS is multifactorial, with a key contributing factor being the significant gap between the proximal and distal esophageal segments. This anatomical disparity increases anastomotic tension, thereby elevating the likelihood of stricture formation. A thorough identification and detailed understanding of the risk factors associated with AS are crucial for enhancing patient outcomes and developing effective preventive strategies. Currently, the evidence regarding esophageal dilation is insufficient to establish the superiority of any single technique, particularly between balloon and bougie dilators. Recurrent and refractory strictures remain a challenge, as no pharmacological and mechanical adjunctive interventions have demonstrated sufficient feasibility, efficacy, and safety to fundamentally change clinical practice. While some results show promise, transformative outcomes have not yet been achieved, and further research is needed to establish evidence-based best treatment strategies.
{"title":"Risk factors and prevention and treatment methods of anastomotic stricture after esophageal atresia repair: a literature review.","authors":"Hao Chen, Zhu-Xin Chen, Guo-Qing Shi","doi":"10.1007/s00383-025-05996-x","DOIUrl":"https://doi.org/10.1007/s00383-025-05996-x","url":null,"abstract":"<p><p>This review examines the common risk factors associated with anastomotic stricture (AS) following esophageal atresia (EA) repair and evaluates the current understanding and clinical practices concerning esophageal dilation, which remains the predominant therapeutic approach. We present emerging evidence on adjunctive therapies for recurrent and refractory strictures, including esophageal stenting, endoscopic incision therapy, mitomycin C application, local steroid injections, cell sheet transplantation, and surgical interventions. A comprehensive literature review was conducted using PubMed, with a cutoff date of December 31, 2024, encompassing all relevant studies and reviews pertinent to this topic, with a particular emphasis on refractory and recurrent strictures. Given the scarcity of evidence-based data on AS in children with EA, we also critically analyze findings from adult literature and studies addressing esophageal strictures caused by various etiologies to provide a more comprehensive understanding. The pathogenesis of AS is multifactorial, with a key contributing factor being the significant gap between the proximal and distal esophageal segments. This anatomical disparity increases anastomotic tension, thereby elevating the likelihood of stricture formation. A thorough identification and detailed understanding of the risk factors associated with AS are crucial for enhancing patient outcomes and developing effective preventive strategies. Currently, the evidence regarding esophageal dilation is insufficient to establish the superiority of any single technique, particularly between balloon and bougie dilators. Recurrent and refractory strictures remain a challenge, as no pharmacological and mechanical adjunctive interventions have demonstrated sufficient feasibility, efficacy, and safety to fundamentally change clinical practice. While some results show promise, transformative outcomes have not yet been achieved, and further research is needed to establish evidence-based best treatment strategies.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"99"},"PeriodicalIF":1.5,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700852","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-24DOI: 10.1007/s00383-025-05994-z
Yujia Wu, Jie Yu, Yaguang Peng, Chenghao Chen, Na Zhang, Qi Zeng
Objective: The objective of this study was to summarize the clinical characteristics and surgical outcomes of pediatric patients with primary spontaneous pneumothorax (PSP) treated by thoracoscopic surgery at a single center. Additionally, we aimed to identify risk factors for postoperative recurrence.
Methods: We retrospectively collected data from patients who underwent thoracoscopic surgery for PSP at the Department of Thoracic Surgery at Beijing Children's Hospital from March 2018 to March 2024. All patients underwent thoracoscopic wedge resection and pleurodesis. Based on Vanderschueren classification, we recorded intraoperative findings in the lung apices. Kaplan-Meier curves were used to assess postoperative recurrence trends over time. The log-rank test was used for univariate analysis, and the statistically significant factors in the univariate analysis were selected for further analysis in the multifactorial Cox regression model to explore the risk factors affecting postoperative recurrence in patients with PSP.
Results: A total of 90 cases of PSP were recorded during the study period and 63 cases of PSP were included in the study. The median age was 15.0 years, and male to female ratio was about 3:1. A total of 30 (30/63) patients had history of contralateral pneumothorax, 15 (15/63) had history of minimally invasive repair of pectus excavatum, and 6 (6/63) had Marfan's syndrome. All patients were treated with thoracoscopic pulmonary wedge resection and pleurodesis and were followed regularly after surgery. The K-Meier curve showed a declining trend in the first 2 years after surgery. Univariate analysis showed that gender, age, history of minimally invasive repair of pectus excavatum, history of contralateral pneumothorax, Marfan syndrome and Vanderschueren classification were significant risk factors for postoperative recurrence. Cox analysis demonstrated that male sex (HR = 5.25, p = 0.02, 95% CI (1.37-20.09)) and history of minimally invasive repair of pectus excavatum (HR = 3.767, p = 0.04, 95% CI (1.04-13.69)) were risk factors for recurrence.
Conclusion: Thoracoscopic treatment for PSP in children has a high rate of recurrence after surgery, particularly within the first 2 years after VATS. Female patients and patients with a history of MIPRE are possible high-risk groups for PSP after VATS. Recurrence needs to be monitored for 2 years after surgery, and reoperation may be required in cases of recurrence.
{"title":"Survival analysis of primary spontaneous pneumothorax in children treated with thoracoscopy: a single-center experience.","authors":"Yujia Wu, Jie Yu, Yaguang Peng, Chenghao Chen, Na Zhang, Qi Zeng","doi":"10.1007/s00383-025-05994-z","DOIUrl":"https://doi.org/10.1007/s00383-025-05994-z","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study was to summarize the clinical characteristics and surgical outcomes of pediatric patients with primary spontaneous pneumothorax (PSP) treated by thoracoscopic surgery at a single center. Additionally, we aimed to identify risk factors for postoperative recurrence.</p><p><strong>Methods: </strong>We retrospectively collected data from patients who underwent thoracoscopic surgery for PSP at the Department of Thoracic Surgery at Beijing Children's Hospital from March 2018 to March 2024. All patients underwent thoracoscopic wedge resection and pleurodesis. Based on Vanderschueren classification, we recorded intraoperative findings in the lung apices. Kaplan-Meier curves were used to assess postoperative recurrence trends over time. The log-rank test was used for univariate analysis, and the statistically significant factors in the univariate analysis were selected for further analysis in the multifactorial Cox regression model to explore the risk factors affecting postoperative recurrence in patients with PSP.</p><p><strong>Results: </strong>A total of 90 cases of PSP were recorded during the study period and 63 cases of PSP were included in the study. The median age was 15.0 years, and male to female ratio was about 3:1. A total of 30 (30/63) patients had history of contralateral pneumothorax, 15 (15/63) had history of minimally invasive repair of pectus excavatum, and 6 (6/63) had Marfan's syndrome. All patients were treated with thoracoscopic pulmonary wedge resection and pleurodesis and were followed regularly after surgery. The K-Meier curve showed a declining trend in the first 2 years after surgery. Univariate analysis showed that gender, age, history of minimally invasive repair of pectus excavatum, history of contralateral pneumothorax, Marfan syndrome and Vanderschueren classification were significant risk factors for postoperative recurrence. Cox analysis demonstrated that male sex (HR = 5.25, p = 0.02, 95% CI (1.37-20.09)) and history of minimally invasive repair of pectus excavatum (HR = 3.767, p = 0.04, 95% CI (1.04-13.69)) were risk factors for recurrence.</p><p><strong>Conclusion: </strong>Thoracoscopic treatment for PSP in children has a high rate of recurrence after surgery, particularly within the first 2 years after VATS. Female patients and patients with a history of MIPRE are possible high-risk groups for PSP after VATS. Recurrence needs to be monitored for 2 years after surgery, and reoperation may be required in cases of recurrence.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"100"},"PeriodicalIF":1.5,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-21DOI: 10.1007/s00383-025-05997-w
Hui-Min Mao, Wan-Liang Guo, San-Li Shi
Purpose: The etiology and pathogenesis of pancreaticobiliary maljunction (PBM) remain unclear, thus a comprehensive investigation of cellular diversity and microenvironmental differences is pivotal to elucidate the mechanisms driving PBM.
Methods: We performed single-cell RNA sequencing on bile duct tissues from six patients, including three with PBM and three without (non-PBM). Pathway enrichment, transcription factor analysis, and cell-cell communication were analyzed to explore cellular interactions and functional states.
Results: A total of 90,996 single cells and 11 distinct cell lineages were identified, revealing significant differences in cellular composition between the two groups. PBM group was characterized by a higher proportion of endothelial cells and fibroblasts, while B and T cells were less abundant. Three subtypes of fibroblasts, antigen-presenting, inflammatory, and myofibroblastic cancer-associated fibroblasts, with the myofibroblast subtype being predominant in PBM. We found heightened activity of the WNT and TWEAK signaling pathways in PBM, as well as increased ligand-receptor interactions between fibroblasts and other cell types, including epithelial and endothelial cells.
Conclusion: Fibroblasts play a central role in driving fibrosis and tissue remodeling in PBM through specific signaling pathways. These insights provide a foundation for future therapeutic strategies aimed at modulating fibroblast activity to prevent or mitigate fibrosis in PBM.
{"title":"Diversity and heterogeneity in human pancreaticobiliary maljunction revealed by single-cell RNA sequencing.","authors":"Hui-Min Mao, Wan-Liang Guo, San-Li Shi","doi":"10.1007/s00383-025-05997-w","DOIUrl":"https://doi.org/10.1007/s00383-025-05997-w","url":null,"abstract":"<p><strong>Purpose: </strong>The etiology and pathogenesis of pancreaticobiliary maljunction (PBM) remain unclear, thus a comprehensive investigation of cellular diversity and microenvironmental differences is pivotal to elucidate the mechanisms driving PBM.</p><p><strong>Methods: </strong>We performed single-cell RNA sequencing on bile duct tissues from six patients, including three with PBM and three without (non-PBM). Pathway enrichment, transcription factor analysis, and cell-cell communication were analyzed to explore cellular interactions and functional states.</p><p><strong>Results: </strong>A total of 90,996 single cells and 11 distinct cell lineages were identified, revealing significant differences in cellular composition between the two groups. PBM group was characterized by a higher proportion of endothelial cells and fibroblasts, while B and T cells were less abundant. Three subtypes of fibroblasts, antigen-presenting, inflammatory, and myofibroblastic cancer-associated fibroblasts, with the myofibroblast subtype being predominant in PBM. We found heightened activity of the WNT and TWEAK signaling pathways in PBM, as well as increased ligand-receptor interactions between fibroblasts and other cell types, including epithelial and endothelial cells.</p><p><strong>Conclusion: </strong>Fibroblasts play a central role in driving fibrosis and tissue remodeling in PBM through specific signaling pathways. These insights provide a foundation for future therapeutic strategies aimed at modulating fibroblast activity to prevent or mitigate fibrosis in PBM.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"41 1","pages":"98"},"PeriodicalIF":1.5,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143674474","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}