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Comment on "Portal hypertension in biliary atresia: a Japanese Biliary Atresia Registry study". 对“胆道闭锁的门脉高压:一项日本胆道闭锁登记研究”的评论。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-26 DOI: 10.1007/s00383-025-06254-w
Shyam Sundar Sah, Abhishek Kumbhalwar
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引用次数: 0
Altered enteric neurodevelopment in the Ncx knockout mouse model of intestinal neuronal dysplasia. Ncx基因敲除小鼠肠神经元发育不良模型中肠神经发育的改变。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-26 DOI: 10.1007/s00383-025-06234-0
Naho Fujiwara, Katsumi Miyahara, Nana Nakazawa-Tanaka, Masahiko Hatano, Atsuyuki Yamataka, Go Miyano

Purpose: Intestinal neuronal dysplasia (IND) features abnormal enteric nervous system (ENS) development and symptom overlap with Hirschsprung's disease, yet fetal-stage mechanisms remain unclear. We established a Sox10-Venus⁺/Ncx⁻/⁻ mouse model of IND, enabling fluorescent labeling of enteric neural crest cells (ENCCs) to test the hypothesis whether neuronal/glial differentiation abnormalities arise during early fetal stages.

Methods: ENCCs were isolated from embryonic day 13.5 (E13.5) fetal gut of Sox10-Venus⁺/Ncx⁻/⁻ (n = 6) and Sox10-Venus⁺/Ncx⁺/⁺ (n = 6) embryos, dissociated, and cultured under non-adherent conditions for 14 days to generate neurospheres. Neurosphere diameter and the proportion of SOX10+ cells were measured using epifluorescence microscopy. Differentiation on day 14 was assessed by immunofluorescence for TUJ1 (neuronal) and S100β (S100B, glial) markers.

Results: Ncx⁻/⁻ neurospheres were significantly larger than controls on days 5 and 10, with no significant difference on day 14. The proportion of SOX10⁺ cells remained higher through day 10. On day 14, Ncx⁻/⁻ neurospheres exhibited a lower proportion of TUJ1⁺ cells, preserved S100β⁺ cell proportions, and disrupted spheroid organization with heterogeneous marker distribution.

Conclusion: These findings demonstrate that Ncx deficiency leads to abnormal ENS development beginning during the fetal period, providing a mechanistic basis for postnatal hyperganglionosis and validating this model for studying IND pathogenesis.

目的:肠神经发育不良(IND)表现为肠神经系统(ENS)发育异常,症状与巨结肠病重叠,但胎儿期机制尚不清楚。我们建立了Sox10-Venus⁺/Ncx⁻/⁻mouse模型,对肠神经嵴细胞(enteric neural crest cells, ENCCs)进行荧光标记,以验证胎儿早期是否会出现神经元/胶质分化异常的假设。方法:从Sox10-Venus⁺/Ncx⁻(n = 6)和Sox10-Venus⁺/Ncx⁺/⁺(n = 6)胎龄13.5天(E13.5)的胎儿肠道中分离出ENCCs,解离,在非贴壁条件下培养14天生成神经球。荧光显微镜下检测神经球直径和SOX10+细胞比例。第14天用免疫荧光法检测TUJ1(神经元)和S100β(胶质)标记物的分化情况。结果:在第5天和第10天,Ncx -毒血症/毒血症球明显大于对照组,在第14天没有显著差异。SOX10 +细胞的比例在第10天保持较高。第14天,Ncx⁻/⁻神经球的TUJ1 +细胞比例较低,S100β +细胞比例保存完好,球体组织被破坏,标记物分布不均。结论:Ncx缺乏导致胎儿期开始的ENS发育异常,为出生后神经节过多症提供了机制基础,为研究IND发病机制提供了验证。
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引用次数: 0
Ultrasound-guided hydrostatic enema reduction for recurrent intussusception in children: a retrospective analysis of 215 cases. 超声引导下液体静压灌肠复位治疗儿童复发性肠套叠215例回顾性分析。
IF 1.6 3区 医学 Q2 PEDIATRICS Pub Date : 2025-11-25 DOI: 10.1007/s00383-025-06253-x
Huan Li, Mengyu Ke, Jun Yang

Objective: To investigate the efficacy and safety of ultrasound-guided hydrostatic enema reduction in treating recurrent intussusception in children.

Methods: Medical records of patients with recurrent intussusception treated by ultrasound-guided hydrostatic enema reduction at Wuhan Children's Hospital from July 2019 to May 2024 were retrospectively analyzed. Patient gender, age, recurrence frequency, and reduction success rates were observed.

Results: From July 2019 to May 2024, a total of 3,084 children underwent ultrasound-guided hydrostatic reduction at Wuhan Children's Hospital. Of these, 2,961 cases achieved successful reduction (success rate: 96.0%) and 123 cases experienced reduction failure (failure rate: 4.0%). During hospitalization, 215 patients developed recurrence (recurrence rate: 7.3%). These 215 patients with recurrence were enrolled in the present study, including 156 males and 59 females, aged 0.59-10.42 years (mean 3.04 years). The number of recurrences during hospitalization ranged from 1 to 9 times (average 1.73 times). The distribution of recurrence frequency was as follows: 143 patients (66.5%) experienced 1 recurrences, 32 patients (14.9%) had 2 recurrences, 19 patients (8.8%) had 3 recurrences, and 21 patients (9.8%) had 4 or more recurrences. All patients underwent successful ultrasound-guided hydrostatic enema reduction without significant secondary factors identified, and none required surgical exploration.

Conclusion: Ultrasound-guided hydrostatic enema reduction is safe and effective for recurrent intussusception in children. In the absence of obvious secondary factors, enema reduction may be considered as a preferred first-line treatment for recurrent intussusception to avoid unnecessary surgical exploration.

目的:探讨超声引导下液体静压灌肠术治疗儿童复发性肠套叠的疗效和安全性。方法:回顾性分析武汉市儿童医院2019年7月至2024年5月超声引导下液体静压灌肠术治疗复发性肠套叠患者的病历。观察患者性别、年龄、复发率和复位成功率。结果:2019年7月至2024年5月,武汉市儿童医院超声引导下静压复位患儿共3084例。其中复位成功2961例(成功率96.0%),复位失败123例(失败率4.0%)。住院期间复发215例,复发率7.3%。215例复发患者纳入本研究,其中男性156例,女性59例,年龄0.59 ~ 10.42岁(平均3.04岁)。住院期间复发次数1 ~ 9次,平均1.73次。复发频率分布:1次复发143例(66.5%),2次复发32例(14.9%),3次复发19例(8.8%),4次及以上21例(9.8%)。所有患者均成功行超声引导的静液灌肠复位,无明显继发性因素,无手术探查。结论:超声引导下液体静压灌肠术治疗儿童复发性肠套叠安全有效。在无明显继发性因素的情况下,为避免不必要的手术探查,可考虑将灌肠复位作为复发性肠套叠的首选一线治疗。
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引用次数: 0
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。
{"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}
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。
{"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}
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Pediatric Surgery International
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