Journal of pediatric surgery最新文献

{"title":"Report of the 57th Annual Meeting of the Pacific Association of Pediatric Surgeons","authors":"Patrick Ho Yu Chung, Mary Brindle, PAPS Publication Committee","doi":"10.1016/j.jpedsurg.2024.162071","DOIUrl":"10.1016/j.jpedsurg.2024.162071","url":null,"abstract":"","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 162071"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142829024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intradyadic Correlation Between Parent-reported and Child-reported Quality of Life in Patients With Anorectal Malformation and Hirschsprung's Disease in Comparison to Healthy Controls","authors":"Suganthi Rajasegaran ,&nbsp;Nur Aini Ahmad ,&nbsp;Shung Ken Tan ,&nbsp;Abhirrami Lechmiannandan ,&nbsp;Yew-Wei Tan ,&nbsp;Anand Sanmugam ,&nbsp;Srihari Singaravel ,&nbsp;Shireen Anne Nah","doi":"10.1016/j.jpedsurg.2024.161687","DOIUrl":"10.1016/j.jpedsurg.2024.161687","url":null,"abstract":"<div><h3>Background</h3><div>Quality of life (QOL) is an important clinical endpoint in paediatric chronic conditions. How parent-proxy reports differ from child self-reported QOL in patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) has not been well examined to date. This study evaluates agreement between parent-proxy and child-reported QOL scores in ARM and HD patients compared to healthy controls.</div></div><div><h3>Methods</h3><div>We recruited ARM and HD patients aged 5–17 years and their parents at four tertiary referral centres between December 2020 to February 2023 who had corrective surgery done &gt;12 months prior. Healthy controls were age-matched and gender-matched. They completed the Pediatric Quality of Life Inventory™ (PedsQL™) Generic Core Scales and General Well-Being (GWB) Scale. The questionnaires were administered in parallel parent-proxy-report and child self-report formats. Appropriate statistical analysis was performed with p &lt; 0.05 significance. Data are reported as median (interquartile range). Ethical approval was obtained.</div></div><div><h3>Results</h3><div>There were 65 ARM, 54 HD and 83 controls. There were no significant differences between parent-reported scores and child-reported scores overall in the Total, Psychosocial Health and Physical Health components of Core Scales for ARM, HD and controls. However, parent-reported scores were significantly higher than child-reported scores overall in ARM, HD and controls in the GWB Scale.</div></div><div><h3>Conclusion</h3><div>Our findings show that parent-rated and child-rated overall QOL was similar in Core Scales for ARM, HD, and controls. However, parents overestimated child's overall GWB for ARM, HD and controls. This highlights the importance of capturing the perspectives of both parents and children to inform strategies to improve patient care.</div></div><div><h3>Level of Evidence</h3><div>Level III.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161687"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Human Liver Organoids to Predict the Outcome of Kasai Portoenterostomy","authors":"Amy Wing Yi Wai ,&nbsp;Vincent Chi Hang Lui ,&nbsp;Clara Sze Man Tang ,&nbsp;Bin Wang ,&nbsp;Paul Kwong Hang Tam ,&nbsp;Kenneth Kak Yuen Wong ,&nbsp;Patrick Ho Yu Chung","doi":"10.1016/j.jpedsurg.2024.161686","DOIUrl":"10.1016/j.jpedsurg.2024.161686","url":null,"abstract":"<div><h3>Background</h3><div>Kasai portoenterostomy (KPE) remains the primary intervention for biliary atresia (BA), but its outcomes are highly variable. Reliable prognostic biomarkers remain elusive, complicating the management and prediction of postoperative progression.</div></div><div><h3>Method</h3><div>Liver biopsies from BA patients taken at and after KPE (post-KPE) were used to generate organoids for RNA-sequencing analysis. Control organoids were derived from non-BA livers. Differential gene expression and enrichment analyses were performed to assess post-KPE transcriptomic changes between native liver survivors (NLS) and patients who eventually became liver transplant recipients (LTR).</div></div><div><h3>Results</h3><div>Organoid datasets: 70 from liver biopsies at KPE (10 patients), 112 from post-KPE livers (13 livers; 12 patients), and 47 from control livers (9 patients). At KPE, BA organoids displayed mainly hepatocyte expression, a trait notably reduced in control organoids. Similarly, post-KPE organoids from NLS revealed a significant decrease in hepatocyte expression features and an overall increase in cholangiocyte expression features. A similar hepatocyte-to-cholangiocyte expression transition was evidenced in paired liver organoids (at- and post-KPE) generated from an NLS. In contrast, post-KPE organoids from LTR maintained a high level of hepatocyte expression features.</div></div><div><h3>Conclusion</h3><div>Our study demonstrated that an elevated expression of hepatocyte features in KPE organoids may indicate aberrant cholangiocyte development in BA livers. In contrast, a post-KPE hepatocyte-to-cholangiocyte expression transition in NLS may imply effective biliary recovery. The lack of this transition in LTR organoids indicates ongoing disease progression, highlighting the potential for organoid-based transcriptomic profiling to inform KPE success and guide BA management.</div></div><div><h3>Level of Evidence</h3><div>Level III.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161686"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142251294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Six Years of Quality Improvement in Pectus Excavatum Repair: Implementation of Intercostal Nerve Cryoablation and ERAS Protocols for Patients Undergoing Nuss Procedure","authors":"Jordan M. Rook ,&nbsp;Lisa K. Lee ,&nbsp;Justin P. Wagner ,&nbsp;Veronica F. Sullins ,&nbsp;Steven L. Lee ,&nbsp;Shant Shekherdimian ,&nbsp;Daniel A. DeUgarte ,&nbsp;Christine E. Dichter ,&nbsp;Howard C. Jen","doi":"10.1016/j.jpedsurg.2024.07.019","DOIUrl":"10.1016/j.jpedsurg.2024.07.019","url":null,"abstract":"<div><h3>Background</h3><div>The Nuss procedure for pectus excavatum is associated with prolonged hospitalizations due to pain. We evaluated implementation of intercostal nerve cryoablation and enhanced recovery after surgery (ERAS) protocols on outcomes of Nuss procedures performed over six years at a single institution.</div></div><div><h3>Methods</h3><div>This retrospective cohort study included patients who underwent Nuss procedure from 10/2017 to 09/2023. Patients received epidurals prior to 06/2019, cryoablation from 06/2019 to 07/2021, and ERAS with cryoablation and intraoperative methadone administration after 07/2021. We used multivariable linear regression to evaluate length of stay (LOS), inpatient morphine milligram equivalents (MMEs), and discharge opioids. We assessed the balancing measures of operative time, postoperative pain scores, and complications.</div></div><div><h3>Results</h3><div>We identified 62 patients; 15 who received epidurals, 18 cryoablation, and 29 cryoablation with ERAS. Cryoablation was associated with a 62.3% (p &lt; 0.001) decrease in length of stay, an 86.6% (p &lt; 0.001) decrease in inpatient MMEs, and a 72.9% (p &lt; 0.001) decrease in discharge opioids. Cryoablation was additionally associated with 24.5% (p = 0.02) longer operative times and 46.4% (p = 0.04) higher postoperative day one pain scores. Subsequent implementation of an ERAS protocol was associated with a further 82.8% (p = 0.04) decrease in discharge opioids and a 25.0% (p = 0.04) decrease in postoperative day one pain scores.</div></div><div><h3>Conclusions</h3><div>Over six years of quality improvement efforts, we found the implementation of cryoablation and ERAS protocols to be associated with a significant decrease in length of stay and opioid exposures. Protocolized pain management and cryoablation may work synergistically to improve outcomes without compromising patient experience.</div></div><div><h3>Level of Evidence</h3><div>Level III – Retrospective comparative study.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161634"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141841142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Repair of the Bulbospongiosus Muscle to Suspend the Penis to the Pubic Bones in Proximal Hypospadias","authors":"S. Hennayake ,&nbsp;M. Gopal ,&nbsp;H.M. Seleim ,&nbsp;T. Cserni ,&nbsp;P. Hajduk ,&nbsp;A. Ajao ,&nbsp;A. Bianchi","doi":"10.1016/j.jpedsurg.2024.162074","DOIUrl":"10.1016/j.jpedsurg.2024.162074","url":null,"abstract":"<div><h3>Background</h3><div>In proximal hypospadias, ventral curvature is invariable and most have penoscrotal transposition, and we observed that the base of the penis (BOP) was located on the inferior aspect of the pubic bones in those, in contrast to the location of the BOP at the anterior end in normal penises. We also observed an unfused bulbospongiosus muscle (BSM) at surgery in those. The aim was to assess the impact of repairing the unfused BSM or transection and straightening of the urethral plate at the first operation on the low BOP.</div></div><div><h3>Method</h3><div>All consecutive proximal hypospadias operations from January 2021 to August 2023 that had a low BOP were retrospectively studied. At the first operation, some had urethral plate transection only, with no BSM repair. Some had BSM repair with or without plate transection. The BOP position was reassessed post-intervention.</div></div><div><h3>Results</h3><div>Thirty-three cases of proximal hypospadias with low BOP were studied. At the first operation, as the key distinguishing step, 18 had BSM repair and 15 urethral plate transections. BOP shifted to the anterior end of the pubic bones in all 18 patients following BSM repair but showed no change in the 15 without BSM repair (p &lt; 0.01). Subsequent BSM repair, during the second stage, normalized BOP in those 15. Normalising the BOP corrected penoscrotal transposition because the anterior end of the scrotum was at the anterior end of the pubic bones.</div></div><div><h3>Conclusion</h3><div>Repairing BSM is essential for normalising the BOP, which results in a normal penoscrotal relationship and normal anterior penile projection.</div></div><div><h3>Level of Evidence</h3><div>IV.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 162074"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Worth the Wait? The Impact of Timing of Repair of Esophageal Atresia With Tracheoesophageal Fistula on Outcomes","authors":"Utsav Patwardhan ,&nbsp;Erin West ,&nbsp;Romeo C. Ignacio ,&nbsp;Gerald Gollin","doi":"10.1016/j.jpedsurg.2024.08.020","DOIUrl":"10.1016/j.jpedsurg.2024.08.020","url":null,"abstract":"<div><h3>Introduction</h3><div>Infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) are at increased risk for respiratory compromise and gastric perforation until fistula ligation. We sought to describe current practice regarding the timing of EA/TEF repair and hypothesized that age at repair is a predictor of adverse outcomes.</div></div><div><h3>Methods</h3><div>The Pediatric Health Information System (PHIS) database was used to identify patients with EA/TEF who underwent fistula ligation and esophago-esophagostomy at US children's hospitals from July 2016–June 2021. Patients with a repair &gt;10 days of age, a long-gap atresia, or H-type fistula were excluded. Comorbidities including prematurity and operative congenital heart disease were noted. Outcomes including anastomotic leak, gastric perforation, and post-operative respiratory failure were assessed for association with age and day of the week of operation.</div></div><div><h3>Results</h3><div>Among 863 patients that were evaluated, the plurality of operations was on DOL 2 (36%) and 83% were on a weekday (random rate = 71%). Later operations had shorter LOS (p = 0.04) and more recurrent nerve injuries (p = 0.01). Weekend repairs were associated with equivalent outcomes. Gastric perforations occurred in 18 (2.0%) patients; 11 (61%) of these occurred after DOL 2.</div></div><div><h3>Conclusions</h3><div>We found no significant differences in outcomes other than more recurrent nerve injury and decreased LOS with EA/TEF repair at older ages. Although repair beyond DOL 2 was safe from a respiratory standpoint, most gastric perforations occurred after this point. In the absence of contraindications or significantly reduced weekend capabilities, we recommend repair of EA/TEF by DOL 2.</div></div><div><h3>Level of Evidence</h3><div>III.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161680"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142181065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Can We Predict 30-day Mortality After Neonatal Surgery for Major Gastrointestinal Conditions? A Prospective Cohort Study","authors":"Mohamed Zouari,&nbsp;Manel Belhajmansour,&nbsp;Amel Ben Hamad,&nbsp;Najoua Ben Kraiem,&nbsp;Mahdi Ben Dhaou","doi":"10.1016/j.jpedsurg.2024.161907","DOIUrl":"10.1016/j.jpedsurg.2024.161907","url":null,"abstract":"","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161907"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142348784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Analysis of Risk Factors for Kasabach Merritt Phenomenom in Children With Kaposiform Hemangioendothelioma","authors":"Chen Chen ,&nbsp;Hanlei Yan ,&nbsp;Wei Yao ,&nbsp;Zuopeng Wang ,&nbsp;Kai Li","doi":"10.1016/j.jpedsurg.2024.161932","DOIUrl":"10.1016/j.jpedsurg.2024.161932","url":null,"abstract":"<div><h3>Background</h3><div>This study generalized and analyzed the clinical attributes observed in patients afflicted with Kaposiform hemangioendothelioma (KHE) with the aim of elucidating the risk factors contributing to the manifestation of Kasabach-Merritt phenomenon (KMP).</div></div><div><h3>Methods</h3><div>We retrospectively analyzed 96 pediatric cases diagnosed with KHE at the Children's Hospital of Fudan University from January 2013 to December 2021. Among them, 62 patients (65%) showed KMP (KHE + KMP group), while 34 patients (35%) did not (KHE-KMP group). The risk factors for KMP associated with KHE were analyzed using univariate analysis and binary logistic regression analysis, comparing the differences between KHE + KMP group and KHE-KMP group.</div></div><div><h3>Results</h3><div>Univariate analysis indicated no statistically significant differences between the two groups in gender, prematurity, family history, or color of involved skin. However, statistically significant differences were observed in age of onset, lesion site, and lesion depth. Multivariate analysis revealed significant associations: children with onset age ≤1 month had a 51-fold increased risk of KMP compared to those with onset age &gt;1 month (95% CI 5.238–501.663); non-extremity lesion sites exhibited a 21-fold higher risk of KMP compared to extremity sites (95% CI 3.970–105.958); deeper lesions conferred a 5-fold higher risk of KMP compared to superficial lesions (95% CI 1.073–21.005); lesions &gt;60 mm carried a 17-fold higher risk of KMP compared to lesions ≤60 mm (95% CI 2.999–96.157).</div><div>A comprehensive predictive model was developed using the fitting formula: Logit (P) = 3.937∗(age at onset) + 1.558∗(lesion depth) + 3.021∗(lesion site) + 2.832∗(lesion size), demonstrating an accuracy of 82.9%. Furthermore, a scoring system was established to assess the likelihood of KMP occurrence. Children diagnosed with KHE were likely to have KMP if their score exceeded 72.5, as determined by Receiver Operating Characteristic (ROC) curve analysis.</div></div><div><h3>Conclusion</h3><div>Age of onset ≤1 month, deeper lesions, non-extremity sites, and lesions ＞60 mm are independent risk factors for KMP in children with KHE. The cumulative presence of these factors escalates the likelihood of KMP development. Additionally, the identification of these factors allows for the early recognition of potential KMP cases among children with KHE, facilitating prompt therapeutic intervention.</div></div><div><h3>Category of the manuscript</h3><div>Clinical Research article.</div></div><div><h3>Level of evidence</h3><div>LEVEL Ⅲ.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161932"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictive Value of Esophageal Deviation Index for Clinical Outcomes of Patients With Left-Sided Congenital Diaphragmatic Hernia","authors":"Bo Xia ,&nbsp;Qiu-Ming He,&nbsp;Wei Zhong,&nbsp;Jun-Jian Lv,&nbsp;Wen-Hai Fang,&nbsp;Wen-Jie Luo","doi":"10.1016/j.jpedsurg.2024.161929","DOIUrl":"10.1016/j.jpedsurg.2024.161929","url":null,"abstract":"<div><h3>Purpose</h3><div>This study aimed to evaluate the prognostic value of postnatal esophageal deviation index (EDI) measured within the first 24 h of life for predicting mortality and morbidity in neonates with left-sided congenital diaphragmatic hernia (L-CDH).</div></div><div><h3>Method</h3><div>This retrospective study analyzed clinical data from 133 neonates with L-CDH admitted to Guangzhou Women and Children's Medical Center between January 2016 and January 2024. Patients were categorized into two groups based on outcomes: survivors (n = 108) and non-survivors (n = 27). Risk factors for mortality were identified using both univariate and multivariate analyses. A receiver operating characteristic (ROC) curve was utilized to evaluate the predictive value of EDI for mortality in L-CDH patients. Subsequently, patients were divided into two groups: those with an EDI&gt; 16.1% and those with an EDI≤16.1%. The relationship between EDI and both mortality and morbidity was analyzed using Kaplan–Meier analysis, chi-square test, Fisher's exact test, and multivariate analysis.</div></div><div><h3>Results</h3><div>EDI (adjusted OR: 0.822, 95% CI 0.723–0.935; P = 0.003) was identified as the independent predictor of mortality through both univariate and multivariate logistic regression analysis. The ROC curve demonstrated that the area under the curve (AUC) for predicting the mortality was 0.854 (95%CI: 0.782–0.930) for EDI, with an optimal cut-off value of 16.125%. The cumulative mortality rate through Day 200 was higher in patients with an EDI&gt;16.1% (P＜0.001). Among the 133 neonates with L-CDH, 24.8% had an EDI&gt;16.1%. This was associated with significantly worse CDH characteristics, including a high incidence of intrathoracic stomach and a high occurrence of high-risk defect sizes (type C/D), (P＜0.001), as well as more severe pulmonary hypertension (P＜0.001). An EDI&gt;16.1% was associated with higher mortality and a greater need for ECMO support compared to an EDI≤16.1% (P＜0.001).</div></div><div><h3>Conclusion</h3><div>EDI within the first 24 h of life in patients with L-CDH is associated with increased mortality and the need for ECMO, particularly when EDI exceeds 16.1%.</div></div><div><h3>Level of Evidence</h3><div>III.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161929"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400549","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CAMK2G Promotes Neuronal Differentiation and Inhibits Migration in Neuroblastoma","authors":"Yujie Ma ,&nbsp;Cong He ,&nbsp;Weihong Lin ,&nbsp;Jing Wang ,&nbsp;Chaoliang Xu ,&nbsp;Deshen Pan ,&nbsp;Zuopeng Wang ,&nbsp;Wei Yao ,&nbsp;Rui Dong ,&nbsp;Deshui Jia ,&nbsp;Kai Li","doi":"10.1016/j.jpedsurg.2024.08.019","DOIUrl":"10.1016/j.jpedsurg.2024.08.019","url":null,"abstract":"<div><h3>Purpose</h3><div>Neuroblastoma (NB) originates from differentiation arrest of sympathoadrenal progenitors in the neural crest. It is necessary to reveal the differentiation mechanism of NB. Previously, we reported that Purkinje cell protein 4 (PCP4) is a well-differentiated marker of NB tissues. Herein, we explored the underlying mechanism of PCP4 induced differentiation in order to find better treatment options for patients.</div></div><div><h3>Methods</h3><div>We screened the interacting proteins of PCP4 by co-immunoprecipitation (Co-IP) and liquid chromatography-mass spectrometry (LC-MS/MS). Then we investigated the relevance between expression of calmodulin-dependent protein kinase II gamma (CAMK2G) and clinical features using R2 platform. We also explored the function of CAMK2G in NB cells by knockdown and RNA sequencing.</div></div><div><h3>Results</h3><div>Here, we verified the binding of PCP4 and calmodulin (CaM) by Co-IP and identified a target kinase of CaM, CAMK2G, by LC-MS/MS. PCP4 overexpression activates the autophosphorylation of CAMK2G. Patients with high CAMK2G expression had better survival while low CAMK2G was associated with unfavorable clinical features including <em>MYCN</em>-amplification, unfavorable histology, progression and high INSS stage. CAMK2G knockdown inhibited neurite outgrowth and down-regulated neuronal differentiation markers (NF–H, MAP2), yet promoted migration, invasion and proliferation. Gene Ontology (GO) analysis showed that knockdown of CAMK2G downregulated the expression of neuronal differentiation-related genes. Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis showed that knockdown of CAMK2G upregulated the expression of migration-related genes.</div></div><div><h3>Conclusion</h3><div>These findings indicate that CAMK2G activated by PCP4/CaM complex promotes differentiation and inhibits migration in NB cells.</div></div><div><h3>Level of Evidence</h3><div>Not applicable.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161679"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142251295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}