Purpose: Perineal morphometric alterations and functional outcomes in patients with hypospadias remain a subject of ongoing debate. This study aimed to evaluate the changes in anogenital distance (AGD)/anal position index (API) values and their intestinal functional impacts in relation to the severity of hypospadias in children.
Method: Fifty-one children who underwent hypospadias surgery (2014-2024) and 19 controls presenting for circumcision (June-September 2024) were assessed. Patients were classified as distal hypospadias (n = 31) or proximal hypospadias (n = 20). Posterior scrotal raphe-anus center (AGD), posterior scrotal raphe-coccyx distance, and API were measured. Constipation was evaluated using Rome IV criteria. Percentile values were calculated.
Results: Patient ages (years): 7.42 ± 1.67/11.51 ± 2.79/8.8 ± 2.37 for control/distal/proximal hypospadias groups. Proximal hypospadias showed significantly lower AGD (33.39 ± 8.11 mm) and API (0.35 ± 0.07) compared to distal hypospadias (54.79 ± 15.65 mm; 0.53 ± 0.07) and controls (55.54 ± 15.57 mm; 0.59 ± 0.08) (p = 0.042), (p < 0.001). Constipation was strongly associated with proximal hypospadias (p < 0.001). AGD distribution varied significantly across percentiles (p < 0.001), whereas API distribution did not significantly (p = 0.587).
Conclusion: Changes in perineal morphometric parameters and their functional outcomes exhibit clinically meaningful distinctions in pediatric patients with proximal hypospadias. Furthermore, it is suggested that these perineal morphometric measurements should be taken into account in predicting and monitoring constipation status during postoperative follow-up in children with hypospadias.
{"title":"Investigation of anogenital distance/anal position index and intestinal functional outcomes in children with hypospadias.","authors":"Caner İsbir, Özlem Elvan, Şener Çolak, Hakan Taşkınlar","doi":"10.1007/s00383-025-06261-x","DOIUrl":"https://doi.org/10.1007/s00383-025-06261-x","url":null,"abstract":"<p><strong>Purpose: </strong>Perineal morphometric alterations and functional outcomes in patients with hypospadias remain a subject of ongoing debate. This study aimed to evaluate the changes in anogenital distance (AGD)/anal position index (API) values and their intestinal functional impacts in relation to the severity of hypospadias in children.</p><p><strong>Method: </strong>Fifty-one children who underwent hypospadias surgery (2014-2024) and 19 controls presenting for circumcision (June-September 2024) were assessed. Patients were classified as distal hypospadias (n = 31) or proximal hypospadias (n = 20). Posterior scrotal raphe-anus center (AGD), posterior scrotal raphe-coccyx distance, and API were measured. Constipation was evaluated using Rome IV criteria. Percentile values were calculated.</p><p><strong>Results: </strong>Patient ages (years): 7.42 ± 1.67/11.51 ± 2.79/8.8 ± 2.37 for control/distal/proximal hypospadias groups. Proximal hypospadias showed significantly lower AGD (33.39 ± 8.11 mm) and API (0.35 ± 0.07) compared to distal hypospadias (54.79 ± 15.65 mm; 0.53 ± 0.07) and controls (55.54 ± 15.57 mm; 0.59 ± 0.08) (p = 0.042), (p < 0.001). Constipation was strongly associated with proximal hypospadias (p < 0.001). AGD distribution varied significantly across percentiles (p < 0.001), whereas API distribution did not significantly (p = 0.587).</p><p><strong>Conclusion: </strong>Changes in perineal morphometric parameters and their functional outcomes exhibit clinically meaningful distinctions in pediatric patients with proximal hypospadias. Furthermore, it is suggested that these perineal morphometric measurements should be taken into account in predicting and monitoring constipation status during postoperative follow-up in children with hypospadias.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"32"},"PeriodicalIF":1.6,"publicationDate":"2025-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145687789","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}
Background: Mechanical bowel preparation (MBP) has traditionally been routine before pediatric intestinal surgery, based on the belief that it reduces the risk of infection. In adults, benefits are only observed when MBP is combined with oral antibiotics; however, evidence in pediatrics remains limited and inconsistent.
Methods: We conducted a systematic review and meta-analysis (PROSPERO CRD420251133552) following PRISMA 2020 and Cochrane guidelines, searching databases up to August 2025. Eligible studies included Randomized controlled trials and comparative observational cohorts comparing MBP with no MBP in elective Pediatric intestinal surgery. The primary outcome was surgical site infection (SSI); secondary outcomes included anastomotic leak (AL), intra-abdominal abscess (IAA), ileus, bowel obstruction, and length of stay (LOS). Random-effects models produced pooled estimates, and certainty was assessed using the GRADE approach.
Results: Seventeen studies involving 3,036 children were included. MBP showed no reduction in SSI, AL, or IAA, with moderate certainty for SSI and low certainty for AL/IAA due to rare events. MBP was associated with longer LOS and possible non-significant increases in ileus and obstruction.
Conclusion: Current evidence suggests that MBP offers no significant benefits in pediatric elective intestinal surgery and may even delay recovery. Within the framework of ERAS protocols, routine use of MBP appears unnecessary. However, MBP might still be required for specific functional or reconstructive indications. Further high-quality multicentre trials are essential before any definitive conclusions can be drawn regarding its discontinuation.
{"title":"Preoperative mechanical bowel preparation versus no preparation for pediatric intestinal surgery: a systematic review and meta-analysis.","authors":"Rajpal Singh Sisodiya, Prince Raj, Akanksha Tomar, Himanshu Acharya, Balkrishna Tripathi, Vikesh Agrawal, Abhishek Tiwari","doi":"10.1007/s00383-025-06265-7","DOIUrl":"10.1007/s00383-025-06265-7","url":null,"abstract":"<p><strong>Background: </strong>Mechanical bowel preparation (MBP) has traditionally been routine before pediatric intestinal surgery, based on the belief that it reduces the risk of infection. In adults, benefits are only observed when MBP is combined with oral antibiotics; however, evidence in pediatrics remains limited and inconsistent.</p><p><strong>Methods: </strong>We conducted a systematic review and meta-analysis (PROSPERO CRD420251133552) following PRISMA 2020 and Cochrane guidelines, searching databases up to August 2025. Eligible studies included Randomized controlled trials and comparative observational cohorts comparing MBP with no MBP in elective Pediatric intestinal surgery. The primary outcome was surgical site infection (SSI); secondary outcomes included anastomotic leak (AL), intra-abdominal abscess (IAA), ileus, bowel obstruction, and length of stay (LOS). Random-effects models produced pooled estimates, and certainty was assessed using the GRADE approach.</p><p><strong>Results: </strong>Seventeen studies involving 3,036 children were included. MBP showed no reduction in SSI, AL, or IAA, with moderate certainty for SSI and low certainty for AL/IAA due to rare events. MBP was associated with longer LOS and possible non-significant increases in ileus and obstruction.</p><p><strong>Conclusion: </strong>Current evidence suggests that MBP offers no significant benefits in pediatric elective intestinal surgery and may even delay recovery. Within the framework of ERAS protocols, routine use of MBP appears unnecessary. However, MBP might still be required for specific functional or reconstructive indications. Further high-quality multicentre trials are essential before any definitive conclusions can be drawn regarding its discontinuation.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"33"},"PeriodicalIF":1.6,"publicationDate":"2025-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145687846","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: This study aimed to investigate the expression of cluster of differentiation 7 (CD7) in hepatoblastoma (HB) and its potential use as a novel biomarker of HB.
Methods: CD7 expression was investigated in human HB samples at the gene level by bulk, single-cell RNA sequencing, and spatial transcriptomic analyses, in addition to the protein level by immunohistochemical (IHC) staining. CD7 gene expression-based survival analysis was also conducted, along with gene set enrichment analysis (GSEA) of the CD7-SECTM1 receptor-ligand gene pair.
Results: CD7 was differentially expressed in human HB at both the gene level by various bioinformatics analyses, and the protein level by IHC, with remarkably higher expression levels in embryonal HB. Conversely, CD7 was not expressed in other primary adult liver tumors. CD7high HB cases showed poorer 5-year event-free survival (P = 0.016), and GSEA demonstrated that CD7 is linked to the embryonal MYCN transcription factor, as were protumor kinases such as JAK3, and marginally MAPK14 and MAPK3.
Conclusion: CD7 is expressed in human HB, especially the embryonal histological subtype, and appears to be linked to tumor progression and poor clinical outcomes. Nevertheless, CD7-targeted chimeric antigen receptor T cells could be proposed as a promising immunotherapy for embryonal HB.
{"title":"Identification of CD7 as a novel biomarker of embryonal hepatoblastoma.","authors":"Ahmad Adawy, Daiki Yoshii, Hiroki Hirao, Yoshihiro Komohara, Yukio Fujiwara, Masahiro Tomita, Masaki Honda, Jia Han, Yao Liu, Sohsuke Yamada, Masahiro Yamamoto, Eiso Hiyama, Taizo Hibi","doi":"10.1007/s00383-025-06255-9","DOIUrl":"https://doi.org/10.1007/s00383-025-06255-9","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to investigate the expression of cluster of differentiation 7 (CD7) in hepatoblastoma (HB) and its potential use as a novel biomarker of HB.</p><p><strong>Methods: </strong>CD7 expression was investigated in human HB samples at the gene level by bulk, single-cell RNA sequencing, and spatial transcriptomic analyses, in addition to the protein level by immunohistochemical (IHC) staining. CD7 gene expression-based survival analysis was also conducted, along with gene set enrichment analysis (GSEA) of the CD7-SECTM1 receptor-ligand gene pair.</p><p><strong>Results: </strong>CD7 was differentially expressed in human HB at both the gene level by various bioinformatics analyses, and the protein level by IHC, with remarkably higher expression levels in embryonal HB. Conversely, CD7 was not expressed in other primary adult liver tumors. CD7<sup>high</sup> HB cases showed poorer 5-year event-free survival (P = 0.016), and GSEA demonstrated that CD7 is linked to the embryonal MYCN transcription factor, as were protumor kinases such as JAK3, and marginally MAPK14 and MAPK3.</p><p><strong>Conclusion: </strong>CD7 is expressed in human HB, especially the embryonal histological subtype, and appears to be linked to tumor progression and poor clinical outcomes. Nevertheless, CD7-targeted chimeric antigen receptor T cells could be proposed as a promising immunotherapy for embryonal HB.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"30"},"PeriodicalIF":1.6,"publicationDate":"2025-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145669333","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 investigate vasoactive intestinal peptide (VIP) expression and distribution in fetal Sprague-Dawley (SD) rat lung with congenital diaphragmatic hernia (CDH). Assess the impact of VIP analog Aviptadil on CDH-associated lung hypoplasia.
Methods: CDH was induced in pregnant SD rats by nitrofen gavage on E9.5. The CDH + VIP group received Aviptadil via tail vein from E10.5. Lung development was assessed by hematoxylin and eosin (HE) staining. VIP, α-SMA, and CD31 were evaluated by immunofluorescence (IF). VIP mRNA and protein levels were quantified by RT-qPCR and Western blotting.
Results: The CDH group exhibited a significantly lower lung index compared to the control group (P < 0.001), with no significant difference observed between the CDH and CDH + VIP groups. Compared to controls, VIP expression in CDH lungs was significantly downregulated at both the mRNA (P = 0.049) and protein levels (P = 0.049). HE staining revealed mature alveolar structures in the control group, whereas the CDH group showed disrupted pulmonary architecture. Partial improvement was observed in the CDH + VIP group. IF analysis indicated that VIP was predominantly localized in the bronchi. VIP fluorescence intensity was significantly decreased in the CDH group compared to both the control group (P = 0.002) and the CDH + VIP group (P = 0.005), while no significant difference was found between the CDH + VIP and control groups. α-SMA fluorescence was primarily localized to pulmonary arterioles and bronchial smooth muscle. Compared to the control group, α-SMA expression was significantly upregulated in the CDH group (P < 0.001). The CDH + VIP group showed a significant reduction in α-SMA expression compared to the CDH group (P = 0.026), with no significant difference from the control group. CD31 was mainly localized to the vascular endothelium. CD31 fluorescence intensity was markedly increased in the CDH group compared to both the control (P < 0.001) and CDH + VIP groups (P < 0.001). The CDH + VIP group also had significantly higher CD31 levels than the control group (P = 0.005).
Conclusion: This study revealed that VIP is downregulated in CDH lungs. In this nitrofen-induced SD rat model, Aviptadil partially restored VIP levels and was associated with attenuation of vascular remodeling and alveolar dysplasia, suggesting a possible therapeutic role in CDH-related pulmonary hypoplasia that warrants further investigation.
{"title":"Investigation of vasoactive intestinal peptide expression and significance in a congenital diaphragmatic hernia animal model.","authors":"Jiajun Chen, Huijiao Xu, Li Yang, Feifan Chen, Kunpeng Li, Bing Xu, Wenying Liu, Fang Hou","doi":"10.1007/s00383-025-06257-7","DOIUrl":"https://doi.org/10.1007/s00383-025-06257-7","url":null,"abstract":"<p><strong>Purpose: </strong>To investigate vasoactive intestinal peptide (VIP) expression and distribution in fetal Sprague-Dawley (SD) rat lung with congenital diaphragmatic hernia (CDH). Assess the impact of VIP analog Aviptadil on CDH-associated lung hypoplasia.</p><p><strong>Methods: </strong>CDH was induced in pregnant SD rats by nitrofen gavage on E9.5. The CDH + VIP group received Aviptadil via tail vein from E10.5. Lung development was assessed by hematoxylin and eosin (HE) staining. VIP, α-SMA, and CD31 were evaluated by immunofluorescence (IF). VIP mRNA and protein levels were quantified by RT-qPCR and Western blotting.</p><p><strong>Results: </strong>The CDH group exhibited a significantly lower lung index compared to the control group (P < 0.001), with no significant difference observed between the CDH and CDH + VIP groups. Compared to controls, VIP expression in CDH lungs was significantly downregulated at both the mRNA (P = 0.049) and protein levels (P = 0.049). HE staining revealed mature alveolar structures in the control group, whereas the CDH group showed disrupted pulmonary architecture. Partial improvement was observed in the CDH + VIP group. IF analysis indicated that VIP was predominantly localized in the bronchi. VIP fluorescence intensity was significantly decreased in the CDH group compared to both the control group (P = 0.002) and the CDH + VIP group (P = 0.005), while no significant difference was found between the CDH + VIP and control groups. α-SMA fluorescence was primarily localized to pulmonary arterioles and bronchial smooth muscle. Compared to the control group, α-SMA expression was significantly upregulated in the CDH group (P < 0.001). The CDH + VIP group showed a significant reduction in α-SMA expression compared to the CDH group (P = 0.026), with no significant difference from the control group. CD31 was mainly localized to the vascular endothelium. CD31 fluorescence intensity was markedly increased in the CDH group compared to both the control (P < 0.001) and CDH + VIP groups (P < 0.001). The CDH + VIP group also had significantly higher CD31 levels than the control group (P = 0.005).</p><p><strong>Conclusion: </strong>This study revealed that VIP is downregulated in CDH lungs. In this nitrofen-induced SD rat model, Aviptadil partially restored VIP levels and was associated with attenuation of vascular remodeling and alveolar dysplasia, suggesting a possible therapeutic role in CDH-related pulmonary hypoplasia that warrants further investigation.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"31"},"PeriodicalIF":1.6,"publicationDate":"2025-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145669348","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}
{"title":"Outcomes of major liver resection for children with hepatoblastoma weighing less than 10 kg: avoiding liver transplantation with extended or extreme resection.","authors":"Pattamon Sutthatarn, Wipusit Taesombat, Matina Kruephate, Pongserath Sirichindakul, Bunthoon Nonthasoot, Pornchai Achatsachat, Piti Techavichit, Teerasak Phewplung, Paisarn Vejchapipat","doi":"10.1007/s00383-025-06249-7","DOIUrl":"10.1007/s00383-025-06249-7","url":null,"abstract":"","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"29"},"PeriodicalIF":1.6,"publicationDate":"2025-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145637452","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 : 2025-11-26DOI: 10.1007/s00383-025-06240-2
Chen-Yi Wang, Mehrsa Feizi, Bo Li, Carol Lee, Dorothy Lee, Jielin Yang, Ying Kang, Yu-Zuo Bai, Agostino Pierro
Purpose: Necrotizing enterocolitis (NEC) is a severe neonatal disease marked by intestinal injury, and epithelial damage has been linked to ferroptosis. This study aimed to determine the protective effect of Ferrostatin-1 (Fer-1), a ferroptosis inhibitor, on NEC-associated intestinal injury.
Methods: NEC was induced in mouse pups via formula feeding, hypoxia, and lipopolysaccharide exposure. Fer-1 (5 mg/kg) was administered intraperitoneally on postnatal days 6 and 8. Intestinal tissues were analyzed for morphological injury, epithelial proliferation (Ki67), ferroptosis markers (Gpx4 and Tfr1), and lipid peroxidation (4-HNE). Human NEC intestinal organoids derived from surgical samples were treated with Fer-1 (2 µM) for 48 h. Levels of ferrous ion (FerroOrange), lipid peroxide (BODIPY), and reactive oxygen species (DCFDA) were measured.
Results: Fer-1 significantly reduced NEC-induced epithelial injury in mice, leading to improved intestinal morphology and increased epithelial proliferation, as indicated by elevated Ki67 expression. The protective effect was associated with reduced ferroptosis, demonstrated by upregulated Gpx4 expression and decreased levels of Tfr1 and 4-HNE. Similarly, in human NEC organoids, Fer-1 significantly reduced the accumulation of ferrous ions, lipid peroxides, and ROS.
Conclusion: Fer-1 effectively protects against NEC-induced intestinal injury by inhibiting ferroptosis and reducing oxidative stress. These findings highlight its potential as a novel therapeutic strategy for managing intestinal damage in NEC.
{"title":"Ferrostatin-1 protects against necrotizing enterocolitis intestinal injury by inhibiting ferroptosis.","authors":"Chen-Yi Wang, Mehrsa Feizi, Bo Li, Carol Lee, Dorothy Lee, Jielin Yang, Ying Kang, Yu-Zuo Bai, Agostino Pierro","doi":"10.1007/s00383-025-06240-2","DOIUrl":"https://doi.org/10.1007/s00383-025-06240-2","url":null,"abstract":"<p><strong>Purpose: </strong>Necrotizing enterocolitis (NEC) is a severe neonatal disease marked by intestinal injury, and epithelial damage has been linked to ferroptosis. This study aimed to determine the protective effect of Ferrostatin-1 (Fer-1), a ferroptosis inhibitor, on NEC-associated intestinal injury.</p><p><strong>Methods: </strong>NEC was induced in mouse pups via formula feeding, hypoxia, and lipopolysaccharide exposure. Fer-1 (5 mg/kg) was administered intraperitoneally on postnatal days 6 and 8. Intestinal tissues were analyzed for morphological injury, epithelial proliferation (Ki67), ferroptosis markers (Gpx4 and Tfr1), and lipid peroxidation (4-HNE). Human NEC intestinal organoids derived from surgical samples were treated with Fer-1 (2 µM) for 48 h. Levels of ferrous ion (FerroOrange), lipid peroxide (BODIPY), and reactive oxygen species (DCFDA) were measured.</p><p><strong>Results: </strong>Fer-1 significantly reduced NEC-induced epithelial injury in mice, leading to improved intestinal morphology and increased epithelial proliferation, as indicated by elevated Ki67 expression. The protective effect was associated with reduced ferroptosis, demonstrated by upregulated Gpx4 expression and decreased levels of Tfr1 and 4-HNE. Similarly, in human NEC organoids, Fer-1 significantly reduced the accumulation of ferrous ions, lipid peroxides, and ROS.</p><p><strong>Conclusion: </strong>Fer-1 effectively protects against NEC-induced intestinal injury by inhibiting ferroptosis and reducing oxidative stress. These findings highlight its potential as a novel therapeutic strategy for managing intestinal damage in NEC.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"26"},"PeriodicalIF":1.6,"publicationDate":"2025-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145605455","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 : 2025-11-26DOI: 10.1007/s00383-025-06254-w
Shyam Sundar Sah, Abhishek Kumbhalwar
{"title":"Comment on \"Portal hypertension in biliary atresia: a Japanese Biliary Atresia Registry study\".","authors":"Shyam Sundar Sah, Abhishek Kumbhalwar","doi":"10.1007/s00383-025-06254-w","DOIUrl":"https://doi.org/10.1007/s00383-025-06254-w","url":null,"abstract":"","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"27"},"PeriodicalIF":1.6,"publicationDate":"2025-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145605378","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: Intestinal neuronal dysplasia (IND) features abnormal enteric nervous system (ENS) development and symptom overlap with Hirschsprung's disease, yet fetal-stage mechanisms remain unclear. We established a Sox10-Venus⁺/Ncx⁻/⁻ mouse model of IND, enabling fluorescent labeling of enteric neural crest cells (ENCCs) to test the hypothesis whether neuronal/glial differentiation abnormalities arise during early fetal stages.
Methods: ENCCs were isolated from embryonic day 13.5 (E13.5) fetal gut of Sox10-Venus⁺/Ncx⁻/⁻ (n = 6) and Sox10-Venus⁺/Ncx⁺/⁺ (n = 6) embryos, dissociated, and cultured under non-adherent conditions for 14 days to generate neurospheres. Neurosphere diameter and the proportion of SOX10+ cells were measured using epifluorescence microscopy. Differentiation on day 14 was assessed by immunofluorescence for TUJ1 (neuronal) and S100β (S100B, glial) markers.
Results: Ncx⁻/⁻ neurospheres were significantly larger than controls on days 5 and 10, with no significant difference on day 14. The proportion of SOX10⁺ cells remained higher through day 10. On day 14, Ncx⁻/⁻ neurospheres exhibited a lower proportion of TUJ1⁺ cells, preserved S100β⁺ cell proportions, and disrupted spheroid organization with heterogeneous marker distribution.
Conclusion: These findings demonstrate that Ncx deficiency leads to abnormal ENS development beginning during the fetal period, providing a mechanistic basis for postnatal hyperganglionosis and validating this model for studying IND pathogenesis.
{"title":"Altered enteric neurodevelopment in the Ncx knockout mouse model of intestinal neuronal dysplasia.","authors":"Naho Fujiwara, Katsumi Miyahara, Nana Nakazawa-Tanaka, Masahiko Hatano, Atsuyuki Yamataka, Go Miyano","doi":"10.1007/s00383-025-06234-0","DOIUrl":"https://doi.org/10.1007/s00383-025-06234-0","url":null,"abstract":"<p><strong>Purpose: </strong>Intestinal neuronal dysplasia (IND) features abnormal enteric nervous system (ENS) development and symptom overlap with Hirschsprung's disease, yet fetal-stage mechanisms remain unclear. We established a Sox10-Venus⁺/Ncx<sup>⁻/⁻</sup> mouse model of IND, enabling fluorescent labeling of enteric neural crest cells (ENCCs) to test the hypothesis whether neuronal/glial differentiation abnormalities arise during early fetal stages.</p><p><strong>Methods: </strong>ENCCs were isolated from embryonic day 13.5 (E13.5) fetal gut of Sox10-Venus⁺/Ncx<sup>⁻/⁻</sup> (n = 6) and Sox10-Venus⁺/Ncx<sup>⁺/⁺</sup> (n = 6) embryos, dissociated, and cultured under non-adherent conditions for 14 days to generate neurospheres. Neurosphere diameter and the proportion of SOX10<sup>+</sup> cells were measured using epifluorescence microscopy. Differentiation on day 14 was assessed by immunofluorescence for TUJ1 (neuronal) and S100β (S100B, glial) markers.</p><p><strong>Results: </strong>Ncx<sup>⁻/⁻</sup> neurospheres were significantly larger than controls on days 5 and 10, with no significant difference on day 14. The proportion of SOX10⁺ cells remained higher through day 10. On day 14, Ncx<sup>⁻/⁻</sup> neurospheres exhibited a lower proportion of TUJ1⁺ cells, preserved S100β⁺ cell proportions, and disrupted spheroid organization with heterogeneous marker distribution.</p><p><strong>Conclusion: </strong>These findings demonstrate that Ncx deficiency leads to abnormal ENS development beginning during the fetal period, providing a mechanistic basis for postnatal hyperganglionosis and validating this model for studying IND pathogenesis.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"28"},"PeriodicalIF":1.6,"publicationDate":"2025-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145605449","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 : 2025-11-25DOI: 10.1007/s00383-025-06253-x
Huan Li, Mengyu Ke, Jun Yang
Objective: To investigate the efficacy and safety of ultrasound-guided hydrostatic enema reduction in treating recurrent intussusception in children.
Methods: Medical records of patients with recurrent intussusception treated by ultrasound-guided hydrostatic enema reduction at Wuhan Children's Hospital from July 2019 to May 2024 were retrospectively analyzed. Patient gender, age, recurrence frequency, and reduction success rates were observed.
Results: From July 2019 to May 2024, a total of 3,084 children underwent ultrasound-guided hydrostatic reduction at Wuhan Children's Hospital. Of these, 2,961 cases achieved successful reduction (success rate: 96.0%) and 123 cases experienced reduction failure (failure rate: 4.0%). During hospitalization, 215 patients developed recurrence (recurrence rate: 7.3%). These 215 patients with recurrence were enrolled in the present study, including 156 males and 59 females, aged 0.59-10.42 years (mean 3.04 years). The number of recurrences during hospitalization ranged from 1 to 9 times (average 1.73 times). The distribution of recurrence frequency was as follows: 143 patients (66.5%) experienced 1 recurrences, 32 patients (14.9%) had 2 recurrences, 19 patients (8.8%) had 3 recurrences, and 21 patients (9.8%) had 4 or more recurrences. All patients underwent successful ultrasound-guided hydrostatic enema reduction without significant secondary factors identified, and none required surgical exploration.
Conclusion: Ultrasound-guided hydrostatic enema reduction is safe and effective for recurrent intussusception in children. In the absence of obvious secondary factors, enema reduction may be considered as a preferred first-line treatment for recurrent intussusception to avoid unnecessary surgical exploration.
{"title":"Ultrasound-guided hydrostatic enema reduction for recurrent intussusception in children: a retrospective analysis of 215 cases.","authors":"Huan Li, Mengyu Ke, Jun Yang","doi":"10.1007/s00383-025-06253-x","DOIUrl":"10.1007/s00383-025-06253-x","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the efficacy and safety of ultrasound-guided hydrostatic enema reduction in treating recurrent intussusception in children.</p><p><strong>Methods: </strong>Medical records of patients with recurrent intussusception treated by ultrasound-guided hydrostatic enema reduction at Wuhan Children's Hospital from July 2019 to May 2024 were retrospectively analyzed. Patient gender, age, recurrence frequency, and reduction success rates were observed.</p><p><strong>Results: </strong>From July 2019 to May 2024, a total of 3,084 children underwent ultrasound-guided hydrostatic reduction at Wuhan Children's Hospital. Of these, 2,961 cases achieved successful reduction (success rate: 96.0%) and 123 cases experienced reduction failure (failure rate: 4.0%). During hospitalization, 215 patients developed recurrence (recurrence rate: 7.3%). These 215 patients with recurrence were enrolled in the present study, including 156 males and 59 females, aged 0.59-10.42 years (mean 3.04 years). The number of recurrences during hospitalization ranged from 1 to 9 times (average 1.73 times). The distribution of recurrence frequency was as follows: 143 patients (66.5%) experienced 1 recurrences, 32 patients (14.9%) had 2 recurrences, 19 patients (8.8%) had 3 recurrences, and 21 patients (9.8%) had 4 or more recurrences. All patients underwent successful ultrasound-guided hydrostatic enema reduction without significant secondary factors identified, and none required surgical exploration.</p><p><strong>Conclusion: </strong>Ultrasound-guided hydrostatic enema reduction is safe and effective for recurrent intussusception in children. In the absence of obvious secondary factors, enema reduction may be considered as a preferred first-line treatment for recurrent intussusception to avoid unnecessary surgical exploration.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"25"},"PeriodicalIF":1.6,"publicationDate":"2025-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145605425","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 : 2025-11-21DOI: 10.1007/s00383-025-06243-z
Xin Ding, Xinyuan Chen, Yifeng Shao, Mei Diao, Long Li
Purpose: This study aimed to develop a nomogram to predict the risk of postoperative anastomotic stricture (AS) after choledochal cyst excision in pediatric patients.
Methods: A retrospective analysis was conducted on pediatric patients who underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy between March 2014 and December 2023. Eligible patients were divided into training and validation cohorts (8:2 ratio). Key predictors were identified using LASSO regression and multivariable logistic regression. The nomogram's performance was evaluated using the concordance index (C-index), the area under the receiver operating characteristic curve (AUC), calibration curves, and decision curve analysis (DCA). The model was validated in the validation cohort.
Results: A total of 1700 patients were included, of whom 32 had AS. The LASSO regression identified four independent predictors: perioperative biliary infection, anastomotic location, anastomotic diameter, and Roux-en-Y limb length (all P < 0.05). The nomogram demonstrated excellent discrimination in both the training (C-index = 0.826, 95% CI: 0.724-0.912) and the validation cohorts (C-index = 0.884, 95% CI: 0.791-0.977). Calibration curves (Hosmer-Lemeshow test, P = 0.221) and DCA confirmed its calibration and clinical utility.
Conclusion: This nomogram provides a reliable tool for predicting the risk of AS in children after choledochal cyst surgery, thereby facilitating intraoperative decision-making and optimizing postoperative surveillance to mitigate stricture-related complications. Further validation in prospective multicenter cohorts is warranted.
{"title":"Nomogram for predicting anastomotic stricture after choledochal cyst excision in children: retrospective cohort study of 1700 patients.","authors":"Xin Ding, Xinyuan Chen, Yifeng Shao, Mei Diao, Long Li","doi":"10.1007/s00383-025-06243-z","DOIUrl":"https://doi.org/10.1007/s00383-025-06243-z","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to develop a nomogram to predict the risk of postoperative anastomotic stricture (AS) after choledochal cyst excision in pediatric patients.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on pediatric patients who underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy between March 2014 and December 2023. Eligible patients were divided into training and validation cohorts (8:2 ratio). Key predictors were identified using LASSO regression and multivariable logistic regression. The nomogram's performance was evaluated using the concordance index (C-index), the area under the receiver operating characteristic curve (AUC), calibration curves, and decision curve analysis (DCA). The model was validated in the validation cohort.</p><p><strong>Results: </strong>A total of 1700 patients were included, of whom 32 had AS. The LASSO regression identified four independent predictors: perioperative biliary infection, anastomotic location, anastomotic diameter, and Roux-en-Y limb length (all P < 0.05). The nomogram demonstrated excellent discrimination in both the training (C-index = 0.826, 95% CI: 0.724-0.912) and the validation cohorts (C-index = 0.884, 95% CI: 0.791-0.977). Calibration curves (Hosmer-Lemeshow test, P = 0.221) and DCA confirmed its calibration and clinical utility.</p><p><strong>Conclusion: </strong>This nomogram provides a reliable tool for predicting the risk of AS in children after choledochal cyst surgery, thereby facilitating intraoperative decision-making and optimizing postoperative surveillance to mitigate stricture-related complications. Further validation in prospective multicenter cohorts is warranted.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"24"},"PeriodicalIF":1.6,"publicationDate":"2025-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564956","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}