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Nomogram for predicting anastomotic stricture after choledochal cyst excision in children: retrospective cohort study of 1700 patients. 预测儿童胆总管囊肿切除术后吻合口狭窄的Nomogram: 1700例回顾性队列研究。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-21 DOI: 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.

目的:本研究旨在建立一种预测小儿胆总管囊肿切除术后吻合口狭窄(AS)风险的线图。方法:回顾性分析2014年3月至2023年12月行肝空肠Roux-en-Y吻合术的小儿胆总管囊肿切除术患者。符合条件的患者分为训练组和验证组(比例为8:2)。使用LASSO回归和多变量逻辑回归确定关键预测因子。使用一致性指数(C-index)、受试者工作特征曲线下面积(AUC)、校准曲线和决策曲线分析(DCA)来评估nomogram的性能。该模型在验证队列中得到验证。结果:共纳入1700例患者,其中32例为AS。LASSO回归确定了四个独立的预测因素:围手术期胆道感染、吻合口位置、吻合口直径和Roux-en-Y肢长(均为P)。结论:该nomogram为预测儿童胆总管囊肿手术后AS的风险提供了可靠的工具,从而有助于术中决策和优化术后监测,以减轻狭窄相关并发症。需要在前瞻性多中心队列中进一步验证。
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引用次数: 0
Human induced pluripotent stem cell-derived bioengineered skeletal muscle progenitor sheet. 人诱导多能干细胞衍生的生物工程骨骼肌祖细胞片。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-21 DOI: 10.1007/s00383-025-06245-x
Nozomi Aoki, Yoshikazu Matsuoka, Hiroshi Yamakawa, Rina Tanaka, Sakiko Yoshimoto, Tokiko Okunobo, Ryosuke Satake, Hiroki Nakamura, Hirofumi Hitomi, Takashi Doi
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引用次数: 0
Incidence and risk factors of pulmonary hypertension in neonates with congenital diaphragmatic hernia: a systematic review and meta-analysis. 先天性膈疝新生儿肺动脉高压的发病率和危险因素:一项系统回顾和荟萃分析。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-21 DOI: 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.

目的:先天性膈疝(CDH- ph)继发肺动脉高压是CDH患者预后不良和死亡率高的主要决定因素。确定PH的危险因素对改善临床管理和优化治疗方法至关重要。本研究的目的是探讨与CDH-PH发展相关的因素。方法:根据PRISMA指南对文献进行系统回顾,并在PROSPERO上注册。PubMed和Medline检索了截至2025年2月发表的关于人类冠心病- ph发病率、死亡率和/或危险因素的研究。不符合纳入标准的重叠患者群体的研究被排除在外。使用比例荟萃分析估计CDH-PH发病率。采用RevMan 5.4中随机效应模型的Mantel-Haenszel方法计算各危险因素的风险比(rr)。结果以95%置信区间(ci)的比例或rr表示。结果:在11618项研究中,筛选了193篇全文文章,共纳入59篇。婴儿中CDH-PH的总发病率为45% (95%CI: 40-50%),与没有PH的CDH新生儿相比,死亡风险更高[RR 5.04 (3.43-7.39);结论:我们发现几乎一半的CDH患者患有PH, CDH-PH患者的死亡风险比没有PH的CDH患者高5倍。虽然CDH-PH的病因尚不清楚,但所确定的危险因素可能间接表明胎儿肺部高度机械压迫有助于血管重塑并易患CDH-PH。
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引用次数: 0
Diagnostic significance of microcolon findings in neonatal contrast enema for patients with intestinal atresia. 新生儿对比灌肠对肠闭锁患者微结肠检查的诊断意义。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-21 DOI: 10.1007/s00383-025-06252-y
Masahiro Zenitani, Shohei Hiwatashi, Yuki Noguchi, Satoshi Umeda, Keigo Nara, Noriaki Usui

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.

目的:探讨新生儿造影剂灌肠对小肠闭锁的诊断价值。方法:回顾性分析20年≤4日龄腹胀新生儿造影剂灌肠图像。患者分为5组:肠闭锁、胎粪肠梗阻、正常、巨结肠病和其他。当观察脾曲、肝曲或回肠末端时,测量结肠各部分的最大直径。通过受试者工作特征分析,确定结肠直径(CD)与L1椎弓根间距离(IPD)比值预测肠闭锁的最佳临界值。结果:所有结肠部位在各时间点CD/IPD比值诊断肠闭锁的曲线下面积(AUC)均大于0.8。回肠末端显像时CD/IPD上升曲线的AUC最高(0.907),其最佳截断值为0.955(敏感性90.9%,特异性84.4%)。结论:新生儿造影剂灌肠显示回肠末端时CD上升小于L1 IPD应考虑小肠闭锁微结肠的提示。
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引用次数: 0
Remote ischemic conditioning modulates the healing process after intestinal anastomosis. 远端缺血调节肠吻合术后愈合过程。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-20 DOI: 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.

目的:探讨远端缺血调节(RIC)是否通过调节肠吻合口不同愈合阶段来促进肠吻合口愈合。方法:幼鼠采用回肠远端端对端吻合术,分为假手术组、单纯吻合组和吻合组+ RIC组。RIC包括4个周期,5分钟后肢缺血和5分钟再灌注,术后立即应用,并在术后第2和第4天重复。在POD 2和POD5上分析组织中TNF-α和IL-6的表达。在POD 5上评估破裂压力、粘连评分和吻合口并发症评分。在POD2和POD5上,对肠吻合段进行大量RNA测序,比较吻合与假吻合、吻合与吻合+ RIC。结果:吻合部位及近端区POD2促炎细胞因子活性升高,而POD5无,表明吻合后炎症处于初始阶段。吻合后远端未见炎症反应。与单纯吻合相比,RIC显著降低吻合口及吻合口近端区促炎细胞因子TNF-α和IL-6基因及蛋白在POD 2上的表达(qPCR),表明炎症减轻。此外,功能分析显示,在POD 5上,吻合+ RIC组的破裂压力明显高于单纯吻合组,粘连评分明显低于吻合组(p)。结论:RIC通过减轻早期炎症和增强机械强度对吻合口愈合具有阶段特异性作用。RIC可以提供简单且无创的策略来改善手术结果。
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引用次数: 0
Is open pyeloplasty still a practical option for pediatric patients in resource-limited settings compared to laparoscopic and robotic approaches? 在资源有限的情况下,与腹腔镜和机器人方法相比,开放式肾盂成形术仍然是儿科患者的一个实用选择吗?
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-20 DOI: 10.1007/s00383-025-06226-0
Gaurav Sharma, Akshita Kundra, Surendra Sharma, Chitrangada Saikia, Bhavya Mehta, Himanshu Agrawal, Rahul Bhargava

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.

目的:探讨在机器人辅助腹腔镜肾盂成形术(RALP)和腹腔镜肾盂成形术(LP)时代,开放式肾盂成形术(OP)治疗资源有限的儿童肾盂输尿管结梗阻的可行性。方法:168例患者(各56例)随机分为RALP组、LP组和OP组。结果:RALP的手术时间明显高于(P)结论:由于成功率相当,并发症最少,OP是治疗小儿PUJO的可行替代微创RALP和LP,特别是在成本和设备可用性是限制因素的情况下。
{"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}
引用次数: 0
The essential role of cytoskeleton and ciliary abnormalities in the development of congenital pulmonary airway malformations. 细胞骨架和纤毛异常在先天性肺气道畸形发展中的重要作用。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-20 DOI: 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.

目的:先天性肺气道畸形(CPAM)是一种严重危害儿童健康的发育性肺畸形。本研究的目的是通过观察分子和细胞水平的变化来探讨CPAM的病因。方法:收集患者临床资料并进行分析。在CPAM手术期间,从CPAM病变和边缘正常肺组织中收集组织样本。采用苏木精和伊红(H&E)染色进行病理分析。分离组织RNA进行RNA测序,富集差异表达基因(DEGs)进行基因本体(GO)分析。细胞骨架和细胞亚型进行免疫荧光染色。结果:7例CPAM患者的RNA测序显示1252个deg,其中1041个上调,211个下调。氧化石墨烯分析显示,与纤毛组织和运动相关的生物过程非常丰富。蛋白-蛋白相互作用(PPI)网络分析显示,CCDC65、DNAH5、DNAH11、DNAH12、CFAP43、CFAP70、pihd3、RSPH4A和DNAH6等基因在CPAM中可能具有重要作用。免疫荧光染色显示细胞骨架和纤毛异常,与正常肺组织相比,CPAM病变中肺泡上皮II型(AEC II)细胞数量减少。结论:我们的研究显示细胞骨架和纤毛表达显著增加,同时AEC II显著减少。这些异常为CPAM的病因提供了潜在的见解,并可能指导改进诊断和治疗策略的发展。
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引用次数: 0
Quality of life and psychological impact in families with congenital lung malformations. 先天性肺畸形家庭的生活质量和心理影响。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-20 DOI: 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.

背景和目的:先天性肺畸形(CLMs)的诊断和手术不仅影响儿童的身体健康,而且影响其家庭的情感、心理和社会福祉。本研究评估了在婴儿期接受clm手术的儿童家庭的长期生活质量(QoL)和心理影响。方法:2024年5月,全国CLM患者协会邀请手术治疗CLM患儿的家长填写养育压力指数、儿童生活质量量表(PedsQL)和PedsQL医疗保健满意度模块。大多数儿童在婴儿期接受手术,并按当前年龄(13-24个月,2-4岁,5-7岁和8-12岁)分组。该调查评估了父母目前对孩子生活质量、自身压力和医疗满意度的看法。母亲和父亲的反应进行比较,线性回归探讨与临床变量的关系。结果:78名母亲和47名父亲做出了回应。总体生活质量高。较低的得分与缺乏心理支持和较长的住院时间有关。与正常情况相比,儿童的情绪和学校功能有所下降。父亲表现出更高的满意度,而母亲则表现出更大的痛苦,尤其是在8-12岁的年龄组中。结论:儿童总体上具有良好的长期生活质量,但家庭,特别是母亲,报告持续的心理困扰。将心理护理纳入长期随访至关重要。
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引用次数: 0
Inhibition of PTTG1 suppresses proliferation and promotes differentiation of neuroblastoma cells by inducing autophagy. 抑制PTTG1可通过诱导自噬来抑制神经母细胞瘤细胞的增殖并促进其分化。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-20 DOI: 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.

目的:PTTG1是一种在多种癌症中高表达的致癌基因,参与调节神经母细胞瘤(NB)细胞的细胞周期。然而,PTTG1在NB中的具体作用尚未被广泛报道。我们开展了这项研究,研究PTTG1在不同NB细胞系中的表达,以确定基因的表达模式。方法:设计靶向PTTG1的小干扰RNA (siRNA)转染NB细胞。通过细胞增殖水平、伤口愈合和transwell实验来评估转染和对照NB细胞的侵袭和迁移能力。利用Western blot、PCR和免疫荧光实验检测NB细胞中迁移相关蛋白、分化相关蛋白和自噬相关蛋白的表达。采用不同剂量的自噬抑制剂3-甲基腺嘌呤(3-MA)验证其作用机制。结果:PTTG1在3种NB细胞系中均有高表达,其中SK-N-SH细胞表达水平最高。干扰PTTG1可显著抑制SK-N-SH细胞活性,降低其增殖、侵袭和迁移能力,并伴有MMP2和MMP9蛋白表达降低。分化标志物TUBB3和自噬标志物LC3II的荧光强度增强,上调GAP43、TH、MEG、TUBB3、LC3II/LC3I、beclin1的蛋白表达和mRNA水平,下调P62、mTOR的表达水平。应用自噬抑制剂3-MA后,PTTG1干扰对SK-N-SH细胞增殖分化的调节作用明显减弱。结论:PTTG1在多种NB细胞中高表达。干扰PTTG1诱导自噬,从而抑制SK-N-SH细胞增殖,促进分化。
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引用次数: 0
Historical aspects of anatomic landmarks during pull-through for hirschsprung disease: focusing on resection levels of the aganglionic rectum and rectal cuff issues. 先天性巨结肠疾病拔管过程中解剖标志的历史方面:聚焦于节结直肠的切除水平和直肠袖问题。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-17 DOI: 10.1007/s00383-025-06236-y
Masahiro Takeda, Hiroyuki Koga, Geoffrey J Lane, Nana Tanaka, Yuichi Nagakawa, Tadaharu Okazaki, Masahiko Urao, Atsuyuki Yamataka

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。
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Pediatric Surgery International
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