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}
Pub Date : 2025-11-21DOI: 10.1007/s00383-025-06247-9
Rebeca Lopes Figueira, Fabian Doktor, Nazgol Moheimani, Giuseppe Lauriti, Jessie Lu, Giuseppe Speziale, Augusto Zani
Purpose: Pulmonary hypertension secondary to congenital diaphragmatic hernia (CDH-PH) is a major determinant for poor outcomes and high mortality patients with CDH. Identifying risk factors for PH is critical to improve clinical management and optimize treatment approaches. The aim of this study was to investigate factors associated with the development of CDH-PH.
Methods: A systematic review of the literature was registered on PROSPERO and performed according to the PRISMA guidelines. PubMed and Medline were searched for studies published until February 2025 reporting the incidence, mortality and/or risk factors of CDH-PH in human. Studies with overlapping patient populations not meeting inclusion criteria were excluded. CDH-PH incidence was estimated using a proportional meta-analysis. Risk ratios (RRs) for risk factors were calculated using the Mantel-Haenszel method with a randoms effect model in RevMan 5.4. Results are presented as proportions or RRs with 95% confidence intervals (CIs).
Results: Of 11,618 studies, 193 full-text articles were screened, and a total of 59 articles were included. The overall incidence of CDH-PH in infants was 45% (95%CI: 40-50%), with higher risk of death in comparison to CDH newborns without PH [RR 5.04 (3.43-7.39); p < 0.05]. Risk factors for CDH-PH were: pre-operative liver herniation [RR 1.75 (1.48-2.08; p < 0.05], non-left CDH (bilateral and Right-CDH) [RR 1.20 (1.08-1.33); p < 0.05], female sex [RR 1.07 (1.04-1.09); p < 0.05], and congenital heart disease [RR 2.15 (1.48-3.12); p < 0.05].
Conclusions: We found that almost half patients with CDH suffer from PH and that CDH-PH patients have a fivefold higher risk of death in comparison to CDH patients without PH. Although the etiology of CDH-PH remains elusive, the risk factors identified may indirectly indicate that a high degree of mechanical compression on the fetal lungs contributes to vascular remodeling and predisposes to CDH-PH.
{"title":"Incidence and risk factors of pulmonary hypertension in neonates with congenital diaphragmatic hernia: a systematic review and meta-analysis.","authors":"Rebeca Lopes Figueira, Fabian Doktor, Nazgol Moheimani, Giuseppe Lauriti, Jessie Lu, Giuseppe Speziale, Augusto Zani","doi":"10.1007/s00383-025-06247-9","DOIUrl":"10.1007/s00383-025-06247-9","url":null,"abstract":"<p><strong>Purpose: </strong>Pulmonary hypertension secondary to congenital diaphragmatic hernia (CDH-PH) is a major determinant for poor outcomes and high mortality patients with CDH. Identifying risk factors for PH is critical to improve clinical management and optimize treatment approaches. The aim of this study was to investigate factors associated with the development of CDH-PH.</p><p><strong>Methods: </strong>A systematic review of the literature was registered on PROSPERO and performed according to the PRISMA guidelines. PubMed and Medline were searched for studies published until February 2025 reporting the incidence, mortality and/or risk factors of CDH-PH in human. Studies with overlapping patient populations not meeting inclusion criteria were excluded. CDH-PH incidence was estimated using a proportional meta-analysis. Risk ratios (RRs) for risk factors were calculated using the Mantel-Haenszel method with a randoms effect model in RevMan 5.4. Results are presented as proportions or RRs with 95% confidence intervals (CIs).</p><p><strong>Results: </strong>Of 11,618 studies, 193 full-text articles were screened, and a total of 59 articles were included. The overall incidence of CDH-PH in infants was 45% (95%CI: 40-50%), with higher risk of death in comparison to CDH newborns without PH [RR 5.04 (3.43-7.39); p < 0.05]. Risk factors for CDH-PH were: pre-operative liver herniation [RR 1.75 (1.48-2.08; p < 0.05], non-left CDH (bilateral and Right-CDH) [RR 1.20 (1.08-1.33); p < 0.05], female sex [RR 1.07 (1.04-1.09); p < 0.05], and congenital heart disease [RR 2.15 (1.48-3.12); p < 0.05].</p><p><strong>Conclusions: </strong>We found that almost half patients with CDH suffer from PH and that CDH-PH patients have a fivefold higher risk of death in comparison to CDH patients without PH. Although the etiology of CDH-PH remains elusive, the risk factors identified may indirectly indicate that a high degree of mechanical compression on the fetal lungs contributes to vascular remodeling and predisposes to CDH-PH.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"23"},"PeriodicalIF":1.6,"publicationDate":"2025-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564923","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 microcolon cutoff value for diagnosing intestinal atresia using a neonatal contrast enema.
Methods: A 20-year retrospective review of contrast enema images of neonates ≤ 4 days of age with abdominal distension was performed. The patients were categorized into five groups: intestinal atresia, meconium ileus, normal, Hirschsprung disease, and others. The maximum diameter of each part of the colon was measured when the splenic flexure, hepatic flexure, or terminal ileum was visualized. Receiver operating characteristic analysis was performed to determine the optimal cutoff value of the colonic diameter (CD) to L1 interpedicular distance (IPD) ratio for predicting intestinal atresia.
Results: The area under the curve (AUC) for the diagnostic performance of the CD/IPD ratio for intestinal atresia exceeded 0.8 in all colon parts at all time points. The AUC for the ascending CD/IPD at the time of terminal ileum visualization was the highest (0.907), and its optimal cutoff value was 0.955 (sensitivity, 90.9%; specificity, 84.4%).
Conclusion: An ascending CD less than the L1 IPD at the time of terminal ileum visualization on neonatal contrast enema should be considered indicative of a microcolon in small intestinal atresia.
{"title":"Diagnostic significance of microcolon findings in neonatal contrast enema for patients with intestinal atresia.","authors":"Masahiro Zenitani, Shohei Hiwatashi, Yuki Noguchi, Satoshi Umeda, Keigo Nara, Noriaki Usui","doi":"10.1007/s00383-025-06252-y","DOIUrl":"https://doi.org/10.1007/s00383-025-06252-y","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to investigate the microcolon cutoff value for diagnosing intestinal atresia using a neonatal contrast enema.</p><p><strong>Methods: </strong>A 20-year retrospective review of contrast enema images of neonates ≤ 4 days of age with abdominal distension was performed. The patients were categorized into five groups: intestinal atresia, meconium ileus, normal, Hirschsprung disease, and others. The maximum diameter of each part of the colon was measured when the splenic flexure, hepatic flexure, or terminal ileum was visualized. Receiver operating characteristic analysis was performed to determine the optimal cutoff value of the colonic diameter (CD) to L1 interpedicular distance (IPD) ratio for predicting intestinal atresia.</p><p><strong>Results: </strong>The area under the curve (AUC) for the diagnostic performance of the CD/IPD ratio for intestinal atresia exceeded 0.8 in all colon parts at all time points. The AUC for the ascending CD/IPD at the time of terminal ileum visualization was the highest (0.907), and its optimal cutoff value was 0.955 (sensitivity, 90.9%; specificity, 84.4%).</p><p><strong>Conclusion: </strong>An ascending CD less than the L1 IPD at the time of terminal ileum visualization on neonatal contrast enema should be considered indicative of a microcolon in small intestinal atresia.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"22"},"PeriodicalIF":1.6,"publicationDate":"2025-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564930","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 : 2025-11-20DOI: 10.1007/s00383-025-06248-8
Masahiro Shimizu, George Biouss, Jielin Yang, Naho Fujiwara, Bo Li, Carol Lee, Dorothy Lee, Felicia Balsamo, Fabiola Cassaro, Mina Yeganeh, Andrea Zito, Naoki Hashizume, Koshiro Sugita, Go Miyano, Atsuyuki Yamataka, Agostino Pierro
Purpose: To evaluate whether remote ischemic conditioning (RIC) improves intestinal anastomotic healing through modulation of different healing phases.
Methods: Juvenile mice underwent distal ileal end-to-end anastomosis and were assigned to three groups: sham (laparotomy only), anastomosis only, and anastomosis + RIC. RIC involved four cycles of 5-minute hindlimb ischemia and 5-minute reperfusion, applied immediately postoperatively and repeated on postoperative days (POD) 2 and 4. On POD 2 and POD5, tissues were analyzed for TNF-α and IL-6 expression. On POD 5, bursting pressure, adhesion scores, and anastomotic complication scores were assessed. On POD2 and POD5, bulk RNA sequencing was conducted on intestinal anastomosis segments comparing anastomosis vs. sham and anastomosis vs. anastomosis + RIC .
Results: Increased pro-inflammatory cytokine activity was observed on POD2 in the anastomotic site and the proximal area of the anastomosis, but not on POD5, indicating an initial phase of inflammation post anastomosis. No inflammation was found post-anastomosis in the distal area. Compared to anastomosis alone, RIC significantly reduced pro-inflammatory cytokine TNF-α and IL-6 gene and protein expression on in the anastomotic site and proximal area of anastomosis on POD 2 (qPCR), indicating decreased inflammation. Furthermore, functional analysis showed that on POD 5, the anastomosis + RIC group had significantly higher bursting pressure and lower adhesion scores compared to the anastomosis only group (p < 0.05). Bulk sequencing analysis showed an upregulation of inflammatory and apoptotic pathways in the anastomosis-only group, while RIC treated anastomosis group had upregulated wound healing pathways in the anastomotic site. These results suggested that RIC dampens early inflammation after intestinal anastomosis and improves the healing process in the anastomotic site.
Conclusion: RIC exerts phase-specific effects on anastomotic healing by attenuating early inflammation and enhancing mechanical strength. RIC may offer a simple and non-invasive strategy to improve surgical outcomes.
{"title":"Remote ischemic conditioning modulates the healing process after intestinal anastomosis.","authors":"Masahiro Shimizu, George Biouss, Jielin Yang, Naho Fujiwara, Bo Li, Carol Lee, Dorothy Lee, Felicia Balsamo, Fabiola Cassaro, Mina Yeganeh, Andrea Zito, Naoki Hashizume, Koshiro Sugita, Go Miyano, Atsuyuki Yamataka, Agostino Pierro","doi":"10.1007/s00383-025-06248-8","DOIUrl":"https://doi.org/10.1007/s00383-025-06248-8","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate whether remote ischemic conditioning (RIC) improves intestinal anastomotic healing through modulation of different healing phases.</p><p><strong>Methods: </strong>Juvenile mice underwent distal ileal end-to-end anastomosis and were assigned to three groups: sham (laparotomy only), anastomosis only, and anastomosis + RIC. RIC involved four cycles of 5-minute hindlimb ischemia and 5-minute reperfusion, applied immediately postoperatively and repeated on postoperative days (POD) 2 and 4. On POD 2 and POD5, tissues were analyzed for TNF-α and IL-6 expression. On POD 5, bursting pressure, adhesion scores, and anastomotic complication scores were assessed. On POD2 and POD5, bulk RNA sequencing was conducted on intestinal anastomosis segments comparing anastomosis vs. sham and anastomosis vs. anastomosis + RIC .</p><p><strong>Results: </strong>Increased pro-inflammatory cytokine activity was observed on POD2 in the anastomotic site and the proximal area of the anastomosis, but not on POD5, indicating an initial phase of inflammation post anastomosis. No inflammation was found post-anastomosis in the distal area. Compared to anastomosis alone, RIC significantly reduced pro-inflammatory cytokine TNF-α and IL-6 gene and protein expression on in the anastomotic site and proximal area of anastomosis on POD 2 (qPCR), indicating decreased inflammation. Furthermore, functional analysis showed that on POD 5, the anastomosis + RIC group had significantly higher bursting pressure and lower adhesion scores compared to the anastomosis only group (p < 0.05). Bulk sequencing analysis showed an upregulation of inflammatory and apoptotic pathways in the anastomosis-only group, while RIC treated anastomosis group had upregulated wound healing pathways in the anastomotic site. These results suggested that RIC dampens early inflammation after intestinal anastomosis and improves the healing process in the anastomotic site.</p><p><strong>Conclusion: </strong>RIC exerts phase-specific effects on anastomotic healing by attenuating early inflammation and enhancing mechanical strength. RIC may offer a simple and non-invasive strategy to improve surgical outcomes.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"16"},"PeriodicalIF":1.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564180","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}
Objective: To explore the feasibility of open pyeloplasty (OP) for treating pediatric pelviureteric junction obstruction in resource-limited settings in the era of robot assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP).
Methods: A total of 168 patients (56 each) were randomized to receive RALP, LP, or OP, respectively.
Results: The operative time for the RALP was significantly higher (P < 0.001) compared to LP and open OP. The length of stay (LOS) for RALP and LP was substantially lower (P < 0.001) compared to the OP, with average stays of 2.8 ± 1.5 days, 3.1 ± 1.2 days, and 6.4 ± 8.1 days, respectively. In the RALP group, 54 (96.4%) had a non-obstructed drainage pattern post-operatively compared to 52 (92.8%) in the LP group, and 53 (94.6%) patients in the OP group (P = 0.363). Only 2 (3.6%) patients in the RALP group and 4 (7.1%) patients in the LP group, and 3 (5.3%) patients in OP group, exhibited obstructed drainage on dynamic nuclear scan. Grade II complications (urine leakage) occurred in 2 patients in the RALP cohort, and in 3 patients in the LP cohort. The leakage typically resolved within 10 days and did not require further intervention. Higher-grade complications (Clavien Grade 3 and 4a), such as hydronephrosis, were identified in both the RALP and OP groups (one case each).
Conclusion: Due to comparable success rates and minimal complications, OP is a viable alternative to minimally invasive RALP and LP in treating pediatric patients with PUJO, particularly where cost and equipment availability are limiting factors.
{"title":"Is open pyeloplasty still a practical option for pediatric patients in resource-limited settings compared to laparoscopic and robotic approaches?","authors":"Gaurav Sharma, Akshita Kundra, Surendra Sharma, Chitrangada Saikia, Bhavya Mehta, Himanshu Agrawal, Rahul Bhargava","doi":"10.1007/s00383-025-06226-0","DOIUrl":"10.1007/s00383-025-06226-0","url":null,"abstract":"<p><strong>Objective: </strong>To explore the feasibility of open pyeloplasty (OP) for treating pediatric pelviureteric junction obstruction in resource-limited settings in the era of robot assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP).</p><p><strong>Methods: </strong>A total of 168 patients (56 each) were randomized to receive RALP, LP, or OP, respectively.</p><p><strong>Results: </strong>The operative time for the RALP was significantly higher (P < 0.001) compared to LP and open OP. The length of stay (LOS) for RALP and LP was substantially lower (P < 0.001) compared to the OP, with average stays of 2.8 ± 1.5 days, 3.1 ± 1.2 days, and 6.4 ± 8.1 days, respectively. In the RALP group, 54 (96.4%) had a non-obstructed drainage pattern post-operatively compared to 52 (92.8%) in the LP group, and 53 (94.6%) patients in the OP group (P = 0.363). Only 2 (3.6%) patients in the RALP group and 4 (7.1%) patients in the LP group, and 3 (5.3%) patients in OP group, exhibited obstructed drainage on dynamic nuclear scan. Grade II complications (urine leakage) occurred in 2 patients in the RALP cohort, and in 3 patients in the LP cohort. The leakage typically resolved within 10 days and did not require further intervention. Higher-grade complications (Clavien Grade 3 and 4a), such as hydronephrosis, were identified in both the RALP and OP groups (one case each).</p><p><strong>Conclusion: </strong>Due to comparable success rates and minimal complications, OP is a viable alternative to minimally invasive RALP and LP in treating pediatric patients with PUJO, particularly where cost and equipment availability are limiting factors.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"19"},"PeriodicalIF":1.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564954","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 : 2025-11-20DOI: 10.1007/s00383-025-06250-0
Tianqi Zhu, Xinyao Meng, Qingxuan Hu, Ke Chen, Xiaofeng Xiong, Ye Yin, Didi Zhuansun, Ying He, Jun Wu, Xuan Zhang, Jiexiong Feng, Xuyong Chen
Purpose: Congenital pulmonary airway malformation (CPAM) is a developmental lung malformation that seriously endangers children's health. The objective of this study was to investigate the etiology of CPAM by observing changes at the molecular and cellular levels.
Methods: Patient clinical data were collected and analyzed. Tissue samples were collected from CPAM lesions and marginal normal lung tissue during CPAM surgery. The samples were subjected to hematoxylin and eosin (H&E) staining for pathological analysis. Tissue RNA was isolated for RNA sequencing, and the differentially expressed genes (DEGs) were enriched for Gene Ontology (GO) analysis. The cytoskeletal and cell subtypes were subjected to immunofluorescence staining.
Results: RNA sequencing of 7 CPAM patients revealed 1252 DEGs, with 1041 upregulated and 211 downregulated. GO analysis revealed that biological processes related to cilium organization and movement were strongly enriched. Protein-protein interaction (PPI) network analysis highlighted genes such as CCDC65, DNAH5, DNAH11, DNAH12, CFAP43, CFAP70, PIH1D3, RSPH4A and DNAH6 as potentially important in CPAM. Immunofluorescence staining revealed abnormalities in cytoskeleton and cilia, and a reduction in the number of alveolar epithelial type II (AEC II) cells in CPAM lesions compared with normal lung tissue.
Conclusion: Our study revealed a significant increase in cytoskeletal and cilia expression, along with a marked reduction in AEC II. These abnormalities provide potential insights into the etiology of CPAM and may guide the development of improved diagnostic and therapeutic strategies.
{"title":"The essential role of cytoskeleton and ciliary abnormalities in the development of congenital pulmonary airway malformations.","authors":"Tianqi Zhu, Xinyao Meng, Qingxuan Hu, Ke Chen, Xiaofeng Xiong, Ye Yin, Didi Zhuansun, Ying He, Jun Wu, Xuan Zhang, Jiexiong Feng, Xuyong Chen","doi":"10.1007/s00383-025-06250-0","DOIUrl":"https://doi.org/10.1007/s00383-025-06250-0","url":null,"abstract":"<p><strong>Purpose: </strong>Congenital pulmonary airway malformation (CPAM) is a developmental lung malformation that seriously endangers children's health. The objective of this study was to investigate the etiology of CPAM by observing changes at the molecular and cellular levels.</p><p><strong>Methods: </strong>Patient clinical data were collected and analyzed. Tissue samples were collected from CPAM lesions and marginal normal lung tissue during CPAM surgery. The samples were subjected to hematoxylin and eosin (H&E) staining for pathological analysis. Tissue RNA was isolated for RNA sequencing, and the differentially expressed genes (DEGs) were enriched for Gene Ontology (GO) analysis. The cytoskeletal and cell subtypes were subjected to immunofluorescence staining.</p><p><strong>Results: </strong>RNA sequencing of 7 CPAM patients revealed 1252 DEGs, with 1041 upregulated and 211 downregulated. GO analysis revealed that biological processes related to cilium organization and movement were strongly enriched. Protein-protein interaction (PPI) network analysis highlighted genes such as CCDC65, DNAH5, DNAH11, DNAH12, CFAP43, CFAP70, PIH1D3, RSPH4A and DNAH6 as potentially important in CPAM. Immunofluorescence staining revealed abnormalities in cytoskeleton and cilia, and a reduction in the number of alveolar epithelial type II (AEC II) cells in CPAM lesions compared with normal lung tissue.</p><p><strong>Conclusion: </strong>Our study revealed a significant increase in cytoskeletal and cilia expression, along with a marked reduction in AEC II. These abnormalities provide potential insights into the etiology of CPAM and may guide the development of improved diagnostic and therapeutic strategies.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"20"},"PeriodicalIF":1.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564453","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 : 2025-11-20DOI: 10.1007/s00383-025-06251-z
Federica Pederiva, Maria Elena Bolis, Marilù Nassano, Marta Quadrelli, Barbara Sgobbi, Paolo Dalena, Massimo Agosti, Valerio Gentilino
Background and objectives: The diagnosis and surgery of congenital lung malformations (CLMs) affect not only children's physical health but also emotional, psychological, and social well-being of their families. This study assessed the long-term quality of life (QoL) and psychological impact in families of children who underwent surgery for CLMs during infancy.
Methods: In May 2024, the national CLM patient association invited parents of children with surgically treated CLMs to complete Parenting Stress Index, Pediatric Quality of Life Inventory (PedsQL), and PedsQL Healthcare Satisfaction Module. Most children had undergone surgery in infancy and were grouped by current age (13-24 months, 2-4 years, 5-7 years, and 8-12 years). The survey assessed parents' current perceptions of their child's QoL, their own stress, and healthcare satisfaction. Maternal and paternal responses were compared, and linear regression explored associations with clinical variables.
Results: Seventy-eight mothers and 47 fathers responded. Overall QoL was high. Lower scores were associated with lack of psychological support and longer hospital stays. Compared to norms, children reported reduced emotional and school functioning. Fathers expressed higher satisfaction, while mothers reported greater distress, especially in the 8-12 year group.
Conclusions: Children generally experience good long-term QoL, but families, particularly mothers, report persistent psychological distress. Integrating psychological care into long-term follow-up is essential.
{"title":"Quality of life and psychological impact in families with congenital lung malformations.","authors":"Federica Pederiva, Maria Elena Bolis, Marilù Nassano, Marta Quadrelli, Barbara Sgobbi, Paolo Dalena, Massimo Agosti, Valerio Gentilino","doi":"10.1007/s00383-025-06251-z","DOIUrl":"https://doi.org/10.1007/s00383-025-06251-z","url":null,"abstract":"<p><strong>Background and objectives: </strong>The diagnosis and surgery of congenital lung malformations (CLMs) affect not only children's physical health but also emotional, psychological, and social well-being of their families. This study assessed the long-term quality of life (QoL) and psychological impact in families of children who underwent surgery for CLMs during infancy.</p><p><strong>Methods: </strong>In May 2024, the national CLM patient association invited parents of children with surgically treated CLMs to complete Parenting Stress Index, Pediatric Quality of Life Inventory (PedsQL), and PedsQL Healthcare Satisfaction Module. Most children had undergone surgery in infancy and were grouped by current age (13-24 months, 2-4 years, 5-7 years, and 8-12 years). The survey assessed parents' current perceptions of their child's QoL, their own stress, and healthcare satisfaction. Maternal and paternal responses were compared, and linear regression explored associations with clinical variables.</p><p><strong>Results: </strong>Seventy-eight mothers and 47 fathers responded. Overall QoL was high. Lower scores were associated with lack of psychological support and longer hospital stays. Compared to norms, children reported reduced emotional and school functioning. Fathers expressed higher satisfaction, while mothers reported greater distress, especially in the 8-12 year group.</p><p><strong>Conclusions: </strong>Children generally experience good long-term QoL, but families, particularly mothers, report persistent psychological distress. Integrating psychological care into long-term follow-up is essential.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"17"},"PeriodicalIF":1.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564984","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 : 2025-11-20DOI: 10.1007/s00383-025-06246-w
Lihua Yuan, Xiaobo Wang, Kanglin Dai, Kenneth Kak Yuen Wong
Purpose: PTTG1 is an oncogene that is highly expressed in various cancers and is involved in regulating the cell cycle in neuroblastoma (NB) cells. However, the specific role of PTTG1 in NB has not been extensively reported. We undertook this study to investigate the expression of PTTG1 in various NB cell lines to identify the gene expression patterns.
Methods: Small interfering RNA (siRNA) targeting PTTG1 was designed and used to transfect NB cells. Cell proliferation levels, wound healing and transwell experiments were undertaken to assess the invasion and migration abilities of transfected and control NB cells. Western blot, PCR, and immunofluorescence experiments were utilized to detect the expression of migration-related proteins, differentiation-related proteins, and autophagy-related proteins in NB cells. Different doses of the autophagy inhibitor 3-methyladenine (3-MA) were used for validating the mechanism.
Results: High expression of PTTG1 was seen in three types of NB cell lines, with the most significant levels observed in SK-N-SH cells. Interference of PTTG1 significantly inhibited the activity of SK-N-SH cells, reducing their proliferation, invasion, and migration abilities, and was accompanied by a decrease in MMP2 and MMP9 protein expression. In addition, there was enhancement of fluorescence intensity of the differentiation marker TUBB3 and the autophagy marker LC3II, and upregulated the protein expression and mRNA levels of GAP43, TH, MEG, TUBB3, LC3II/LC3I, and beclin1, while downregulated the expression levels of P62 and mTOR. After applying the autophagy inhibitor 3-MA, the regulation of SK-N-SH cell proliferation and differentiation by PTTG1 interference was significantly reduced.
Conclusions: PTTG1 is highly expressed in various NB cells. Interfering with PTTG1 induces autophagy, thereby inhibiting SK-N-SH cell proliferation and promoting differentiation.
{"title":"Inhibition of PTTG1 suppresses proliferation and promotes differentiation of neuroblastoma cells by inducing autophagy.","authors":"Lihua Yuan, Xiaobo Wang, Kanglin Dai, Kenneth Kak Yuen Wong","doi":"10.1007/s00383-025-06246-w","DOIUrl":"10.1007/s00383-025-06246-w","url":null,"abstract":"<p><strong>Purpose: </strong>PTTG1 is an oncogene that is highly expressed in various cancers and is involved in regulating the cell cycle in neuroblastoma (NB) cells. However, the specific role of PTTG1 in NB has not been extensively reported. We undertook this study to investigate the expression of PTTG1 in various NB cell lines to identify the gene expression patterns.</p><p><strong>Methods: </strong>Small interfering RNA (siRNA) targeting PTTG1 was designed and used to transfect NB cells. Cell proliferation levels, wound healing and transwell experiments were undertaken to assess the invasion and migration abilities of transfected and control NB cells. Western blot, PCR, and immunofluorescence experiments were utilized to detect the expression of migration-related proteins, differentiation-related proteins, and autophagy-related proteins in NB cells. Different doses of the autophagy inhibitor 3-methyladenine (3-MA) were used for validating the mechanism.</p><p><strong>Results: </strong>High expression of PTTG1 was seen in three types of NB cell lines, with the most significant levels observed in SK-N-SH cells. Interference of PTTG1 significantly inhibited the activity of SK-N-SH cells, reducing their proliferation, invasion, and migration abilities, and was accompanied by a decrease in MMP2 and MMP9 protein expression. In addition, there was enhancement of fluorescence intensity of the differentiation marker TUBB3 and the autophagy marker LC3II, and upregulated the protein expression and mRNA levels of GAP43, TH, MEG, TUBB3, LC3II/LC3I, and beclin1, while downregulated the expression levels of P62 and mTOR. After applying the autophagy inhibitor 3-MA, the regulation of SK-N-SH cell proliferation and differentiation by PTTG1 interference was significantly reduced.</p><p><strong>Conclusions: </strong>PTTG1 is highly expressed in various NB cells. Interfering with PTTG1 induces autophagy, thereby inhibiting SK-N-SH cell proliferation and promoting differentiation.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"18"},"PeriodicalIF":1.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12634700/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564971","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}
The historical legacy of using fixed distances from the dentate line (DL) for rectal resection during pull-through for Hirschsprung's disease (HD) has never really been investigated despite widespread continuous use. Swenson initially used 1.5-2.0 cm from the anal verge for resection. In 1962, Bill and Chapman reported an association between residual aganglionic segments, obstructive symptoms, and postoperative enterocolitis. In 1964, Swenson modified his resection to 1.5-2.0 cm from the DL anteriorly and 1.0 cm or less posteriorly. Other master surgeons also explored lines for rectal resection; however almost all were based on anecdotal experience. More recently, possible associations between resection lines and postoperative complications were reported by De la Torre (2017) and a nationwide survey in Japan (2019). In 2007, co-author AY adopted the anorectal line (ARL) exclusively as an alternative to the DL specifically because it was readily identifiable, independent of age/build, functionally relevant as the distal margin of the enteric nervous system, and indispensable for normal anal sensation as the upper border of the anal transitional zone histopathologically. While the anal verge and the DL have been important historically, the ARL objectively enhances the reliability of rectal resection of the aganglionic segment without causing injury to the anal transitional zone.
在先天性巨结肠病(HD)的拔管过程中,使用离齿状线(DL)固定距离的直肠切除术的历史遗产从未真正研究过,尽管它被广泛使用。Swenson最初在距肛门边缘1.5-2.0 cm处切除。1962年,Bill和Chapman报道了残留的神经节节段、梗阻性症状和术后小肠结肠炎之间的关系。1964年,Swenson修改了他的手术,切除前DL 1.5-2.0 cm,后DL 1.0 cm或更少。其他大师级外科医生也探索了直肠切除术的路线;然而,几乎所有这些都是基于轶事经验。最近,De la Torre(2017年)和日本的一项全国性调查(2019年)报道了切除线与术后并发症之间可能存在的关联。2007年,合著者AY采用了肛门直肠线(ARL)作为DL的替代方法,因为它易于识别,与年龄/构造无关,功能上与肠神经系统远端边缘相关,并且在组织病理学上作为肛门过渡带的上边界对于正常的肛门感觉是必不可少的。虽然肛门边缘和DL在历史上一直很重要,但ARL客观上提高了肛门节段直肠切除术的可靠性,而不会对肛门过渡区造成损伤。
{"title":"Historical aspects of anatomic landmarks during pull-through for hirschsprung disease: focusing on resection levels of the aganglionic rectum and rectal cuff issues.","authors":"Masahiro Takeda, Hiroyuki Koga, Geoffrey J Lane, Nana Tanaka, Yuichi Nagakawa, Tadaharu Okazaki, Masahiko Urao, Atsuyuki Yamataka","doi":"10.1007/s00383-025-06236-y","DOIUrl":"10.1007/s00383-025-06236-y","url":null,"abstract":"<p><p>The historical legacy of using fixed distances from the dentate line (DL) for rectal resection during pull-through for Hirschsprung's disease (HD) has never really been investigated despite widespread continuous use. Swenson initially used 1.5-2.0 cm from the anal verge for resection. In 1962, Bill and Chapman reported an association between residual aganglionic segments, obstructive symptoms, and postoperative enterocolitis. In 1964, Swenson modified his resection to 1.5-2.0 cm from the DL anteriorly and 1.0 cm or less posteriorly. Other master surgeons also explored lines for rectal resection; however almost all were based on anecdotal experience. More recently, possible associations between resection lines and postoperative complications were reported by De la Torre (2017) and a nationwide survey in Japan (2019). In 2007, co-author AY adopted the anorectal line (ARL) exclusively as an alternative to the DL specifically because it was readily identifiable, independent of age/build, functionally relevant as the distal margin of the enteric nervous system, and indispensable for normal anal sensation as the upper border of the anal transitional zone histopathologically. While the anal verge and the DL have been important historically, the ARL objectively enhances the reliability of rectal resection of the aganglionic segment without causing injury to the anal transitional zone.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"42 1","pages":"14"},"PeriodicalIF":1.6,"publicationDate":"2025-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145541682","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}