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Human Liver Organoids to Predict the Outcome of Kasai Portoenterostomy 预测葛西肠管造口术结果的人体肝脏器官组织
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.161686
Amy Wing Yi Wai , Vincent Chi Hang Lui , Clara Sze Man Tang , Bin Wang , Paul Kwong Hang Tam , Kenneth Kak Yuen Wong , Patrick Ho Yu Chung

Background

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.

Method

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).

Results

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.

Conclusion

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.

Level of Evidence

Level III.
葛西肠管造口术(KPE)仍是治疗胆道闭锁(BA)的主要方法,但其结果却千差万别。可靠的预后生物标志物仍未出现,使术后管理和病情发展预测变得更加复杂。KPE术前和术后(KPE术后)BA患者的肝活检组织被用来生成RNA序列分析所需的器官组织。对照组器官组织来自非 BA 患者的肝脏。进行了差异基因表达和富集分析,以评估原肝存活者(NLS)和最终成为肝移植受者(LTR)的患者在 KPE 后转录组的变化。类器官数据集:70 个来自 KPE 时的肝活检组织(10 名患者),112 个来自 KPE 后的肝脏(13 个肝脏;12 名患者),47 个来自对照组肝脏(9 名患者)。在 KPE 时,BA 器官主要表现为肝细胞表达,而对照组器官的肝细胞表达明显减少。同样,来自 NLS 的 KPE 后器官组织显示肝细胞表达特征显著减少,而胆管细胞表达特征总体增加。由 NLS 生成的成对肝脏器官组织(KPE 前和 KPE 后)也出现了类似的肝细胞向胆管细胞表达的转变。相比之下,来自 LTR 的后 KPE 有机体保持了高水平的肝细胞表达特征。我们的研究表明,KPE 有机体中肝细胞特征表达的升高可能表明 BA 肝中胆管细胞发育异常。相反,NLS 中 KPE 后肝细胞向胆管细胞表达的转变可能意味着胆道的有效恢复。LTR 器官组织中缺乏这种转变表明疾病仍在进展,这凸显了基于器官组织的转录组学分析为 KPE 成功与否提供信息并指导 BA 管理的潜力。三级
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引用次数: 0
Six Years of Quality Improvement in Pectus Excavatum Repair: Implementation of Intercostal Nerve Cryoablation and ERAS Protocols for Patients Undergoing Nuss Procedure 胸大肌修复术六年来的质量改进:对接受 Nuss 手术的患者实施肋间神经冷冻消融术和 ERAS 方案
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.07.019
Jordan M. Rook , Lisa K. Lee , Justin P. Wagner , Veronica F. Sullins , Steven L. Lee , Shant Shekherdimian , Daniel A. DeUgarte , Christine E. Dichter , Howard C. Jen

Background

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.

Methods

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.

Results

We identified 62 patients; 15 who received epidurals, 18 cryoablation, and 29 cryoablation with ERAS. Cryoablation was associated with a 62.3% (p < 0.001) decrease in length of stay, an 86.6% (p < 0.001) decrease in inpatient MMEs, and a 72.9% (p < 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.

Conclusions

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.

Level of Evidence

Level III – Retrospective comparative study.
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引用次数: 0
Repair of the Bulbospongiosus Muscle to Suspend the Penis to the Pubic Bones in Proximal Hypospadias 尿道下裂近端阴茎悬吊至耻骨的球海绵肌修复。
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.162074
S. Hennayake , M. Gopal , H.M. Seleim , T. Cserni , P. Hajduk , A. Ajao , A. Bianchi

Background

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.

Method

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.

Results

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 < 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.

Conclusion

Repairing BSM is essential for normalising the BOP, which results in a normal penoscrotal relationship and normal anterior penile projection.

Level of Evidence

IV.
背景:在尿道下裂近端,腹侧弯曲是不变的,大多数有阴茎阴囊转位,我们观察到阴茎基部(BOP)位于耻骨的下侧面,而正常阴茎的BOP位于前端。我们也在手术中观察到未融合的球海绵肌(BSM)。目的是评估在低BOP的首次手术中修复未融合的BSM或横断并拉直尿道板的影响。方法:回顾性研究2021年1月至2023年8月连续进行的低BOP的近端尿道下裂手术。在第一次手术中,一些患者只有尿道板横断,没有BSM修复。部分患者行椎板横断或不横断修复。干预后重新评估防喷器位置。结果:对33例低BOP的近端尿道下裂进行了分析。在第一次手术中,作为关键的区分步骤,18例行BSM修复,15例行尿道板横断。修复BSM后的18例患者BOP均移至耻骨前端,而未修复的15例患者BOP无变化(p)结论:修复BSM对BOP的正常化至关重要,这将导致正常的阴茎阴囊关系和正常的阴茎前投射。证据等级:四级。
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引用次数: 0
Worth the Wait? The Impact of Timing of Repair of Esophageal Atresia With Tracheoesophageal Fistula on Outcomes 值得等待吗? 食管闭锁伴气管食管瘘修复时机对疗效的影响。
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.08.020
Utsav Patwardhan , Erin West , Romeo C. Ignacio , Gerald Gollin

Introduction

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.

Methods

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 >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.

Results

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.

Conclusions

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.

Level of Evidence

III.
导言患有食管闭锁和气管食管瘘(EA/TEF)的婴儿在瘘管结扎前呼吸困难和胃穿孔的风险较高。我们试图描述目前有关 EA/TEF 修复时机的做法,并假设修复时的年龄是不良后果的预测因素。方法使用儿科健康信息系统(PHIS)数据库来识别 2016 年 7 月至 2021 年 6 月期间在美国儿童医院接受瘘管结扎和食管-食管造口术的 EA/TEF 患者。排除了修复时间大于 10 天、长间隙闭锁或 H 型瘘管的患者。注意到包括早产儿和先天性心脏病在内的合并症。对吻合口漏、胃穿孔和术后呼吸衰竭等结果进行了评估,以确定其与年龄和手术周日的关系。结果在接受评估的863名患者中,大多数手术是在DOL 2进行的(36%),83%的手术是在工作日进行的(随机率=71%)。较晚进行手术的患者住院时间较短(p = 0.04),复发性神经损伤较多(p = 0.01)。周末修复手术的结果相当。18例(2.0%)患者发生胃穿孔,其中11例(61%)发生在DOL 2之后。结论 我们发现,除了年龄较大时进行EA/TEF修复会导致更多的复发性神经损伤和更短的LOS外,其他结果没有显著差异。尽管从呼吸角度来看,超过 DOL 2 的修复是安全的,但大多数胃穿孔发生在这一时间点之后。如果没有禁忌症或周末能力明显下降,我们建议在 DOL 2 之前进行 EA/TEF 修复。
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引用次数: 0
Can We Predict 30-day Mortality After Neonatal Surgery for Major Gastrointestinal Conditions? A Prospective Cohort Study 我们能否预测新生儿重大胃肠道疾病手术后 30 天的死亡率?一项前瞻性队列研究。
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.161907
Mohamed Zouari, Manel Belhajmansour, Amel Ben Hamad, Najoua Ben Kraiem, Mahdi Ben Dhaou
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引用次数: 0
Analysis of Risk Factors for Kasabach Merritt Phenomenom in Children With Kaposiform Hemangioendothelioma 卡巴赫-梅里特现象在罹患卡波斯状血管内皮瘤的儿童中的风险因素分析
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.161932
Chen Chen , Hanlei Yan , Wei Yao , Zuopeng Wang , Kai Li

Background

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).

Methods

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.

Results

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 >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 >60 mm carried a 17-fold higher risk of KMP compared to lesions ≤60 mm (95% CI 2.999–96.157).
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.

Conclusion

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.

Category of the manuscript

Clinical Research article.

Level of evidence

LEVEL Ⅲ.
研究背景本研究归纳并分析了卡帕斯状血管内皮细胞瘤(KHE)患者的临床特征,旨在阐明导致卡萨巴赫-梅里特现象(KMP)表现的风险因素:我们回顾性分析了2013年1月至2021年12月在复旦大学附属儿童医院确诊为KHE的96例儿科病例。其中,62 名患者(65%)出现 KMP(KHE + KMP 组),34 名患者(35%)未出现 KMP(KHE-KMP 组)。通过单变量分析和二元逻辑回归分析,比较了KHE + KMP组与KHE-KMP组之间的差异,分析了与KHE相关的KMP风险因素:单变量分析表明,两组患者在性别、早产、家族史或受累皮肤颜色方面的差异无统计学意义。然而,在发病年龄、皮损部位和皮损深度方面,两组之间存在明显的统计学差异。多变量分析表明:与发病年龄大于 1 个月的儿童相比,发病年龄小于 1 个月的儿童患 KMP 的风险增加了 51 倍(95% CI 5.238-501.663);与四肢部位相比,非四肢部位患 KMP 的风险增加了 21 倍(95% CI 3.970-105.958)。970-105.958);与浅表病变相比,深层病变发生 KMP 的风险高 5 倍(95% CI 1.073-21.005);与≤60 mm 的病变相比,>60 mm 的病变发生 KMP 的风险高 17 倍(95% CI 2.999-96.157)。利用拟合公式建立了一个综合预测模型:Logit (P) = 3.937∗(发病年龄)+1.558∗(病变深度)+3.021∗(病变部位)+2.832∗(病变大小),准确率为 82.9%。此外,还建立了一个评分系统来评估发生 KMP 的可能性。根据接收者工作特征曲线(ROC)分析,被诊断为KHE的儿童如果得分超过72.5分,就有可能患有KMP:结论:发病年龄≤1个月、病灶较深、非四肢部位、病灶>60毫米是KHE患儿发生KMP的独立危险因素。这些因素的累积会增加 KMP 发生的可能性。此外,识别这些因素可以及早发现KHE患儿中潜在的KMP病例,便于及时进行治疗干预:稿件类别:临床研究文章:证据等级:Ⅲ级。
{"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}
引用次数: 0
Predictive Value of Esophageal Deviation Index for Clinical Outcomes of Patients With Left-Sided Congenital Diaphragmatic Hernia 食管偏离指数对左侧先天性膈疝患者临床结局的预测价值
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.161929
Bo Xia , Qiu-Ming He, Wei Zhong, Jun-Jian Lv, Wen-Hai Fang, Wen-Jie Luo

Purpose

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).

Method

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> 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.

Results

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>16.1% (P<0.001). Among the 133 neonates with L-CDH, 24.8% had an EDI>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>16.1% was associated with higher mortality and a greater need for ECMO support compared to an EDI≤16.1% (P<0.001).

Conclusion

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%.

Level of Evidence

III.
目的:本研究旨在评估出生后24小时内测量的食管偏离指数(EDI)预测左侧先天性膈疝(L-CDH)新生儿死亡率和发病率的预后价值:这项回顾性研究分析了广州市妇女儿童医疗中心在2016年1月至2024年1月期间收治的133名左侧先天性膈疝新生儿的临床数据。根据结果将患者分为两组:存活者(108人)和非存活者(27人)。通过单变量和多变量分析确定了死亡率的风险因素。利用接收器操作特征(ROC)曲线评估了 EDI 对 L-CDH 患者死亡率的预测价值。随后,患者被分为两组:EDI>16.1%和EDI≤16.1%。采用卡普兰-梅耶分析、卡方检验、费雪精确检验和多变量分析法分析了EDI与死亡率和发病率之间的关系:通过单变量和多变量逻辑回归分析,EDI(调整后 OR:0.822,95% CI 0.723-0.935;P = 0.003)被确定为死亡率的独立预测因子。ROC 曲线显示,EDI 预测死亡率的曲线下面积(AUC)为 0.854(95%CI:0.782-0.930),最佳临界值为 16.125%。EDI>16.1%的患者在第200天的累积死亡率更高(P<0.001)。在133名患有L-CDH的新生儿中,24.8%的EDI>16.1%。这与CDH特征明显恶化有关,包括胸内胃发生率高、高风险缺损大小(C/D型)发生率高(P<0.001)以及更严重的肺动脉高压(P<0.001)。EDI>16.1%与EDI≤16.1%相比,死亡率更高,更需要ECMO支持(P<0.001):结论:L-CDH 患者生命最初 24 小时内的 EDI 与死亡率和 ECMO 需求增加有关,尤其是当 EDI 超过 16.1% 时:证据等级:III。
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引用次数: 0
CAMK2G Promotes Neuronal Differentiation and Inhibits Migration in Neuroblastoma CAMK2G 促进神经细胞分化并抑制神经母细胞瘤的迁移
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.08.019
Yujie Ma , Cong He , Weihong Lin , Jing Wang , Chaoliang Xu , Deshen Pan , Zuopeng Wang , Wei Yao , Rui Dong , Deshui Jia , Kai Li

Purpose

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.

Methods

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.

Results

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 MYCN-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.

Conclusion

These findings indicate that CAMK2G activated by PCP4/CaM complex promotes differentiation and inhibits migration in NB cells.

Level of Evidence

Not applicable.
神经母细胞瘤(NB)起源于神经嵴中交感肾上腺祖细胞的分化停滞。有必要揭示 NB 的分化机制。此前,我们报道了浦肯野细胞蛋白4(PCP4)是NB组织分化良好的标志物。在此,我们探讨了 PCP4 诱导分化的潜在机制,以便为患者找到更好的治疗方案。我们通过共免疫沉淀(Co-IP)和液相色谱-质谱(LC-MS/MS)技术筛选了与PCP4相互作用的蛋白。然后,我们利用 R2 平台研究了钙调素依赖性蛋白激酶 II gamma(CAMK2G)的表达与临床特征之间的相关性。我们还通过基因敲除和 RNA 测序探讨了 CAMK2G 在 NB 细胞中的功能。在此,我们通过 Co-IP 验证了 PCP4 与钙调蛋白(CaM)的结合,并通过 LC-MS/MS 鉴定了 CaM 的靶激酶 CAMK2G。PCP4的过表达激活了CAMK2G的自身磷酸化。CAMK2G表达量高的患者生存率更高,而CAMK2G表达量低的患者临床特征不佳,包括扩增、组织学不佳、病情进展和INSS分期高。CAMK2G基因敲除抑制了神经元的生长,下调了神经元分化标志物(NF-H、MAP2),但促进了迁移、侵袭和增殖。基因本体(GO)分析表明,CAMK2G的敲除下调了神经元分化相关基因的表达。京都基因组百科全书》(KEGG)分析显示,敲除 CAMK2G 会上调迁移相关基因的表达。这些发现表明,PCP4/CaM 复合物激活的 CAMK2G 可促进 NB 细胞的分化并抑制其迁移。不适用。
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引用次数: 0
Geographic Differences in Pediatric Surgical Mortality in Canada: A Retrospective Cohort Study 加拿大小儿外科死亡率的地域差异:回顾性队列研究
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.07.030
Oluwatomilayo Daodu , Saffa Aziz , Melanie Morris , Mary E. Brindle

Objective

This study describes differences in postoperative mortality for pediatric patients in rural communities compared to urban communities.

Background

Canada has the second largest land mass in the world, with a population density of 4 people per km2. There are 18 children’s hospitals in Canada offering pediatric surgical services, all in urban centres, yet nearly one-fifth of the population lives in rural or remote communities. Children who live in rural settings may have worse surgical outcomes, including mortality rates, compared with urban populations.

Methods

Pediatric patients, from birth to 18 years old, who had surgery from January 1, 2011, to December 31, 2021, at a single Children's Hospital were included in the study. Data was obtained from the provincial Operating Room Information System (ORIS) database. Postal code, rural and urban status, distance to children's hospital (0-50 km, 51-100 km, 101-150 km, 151-200 km, and >200 km), and procedure urgency were collected. 30-day mortality for all procedures was collected.

Results

85,998 surgical procedures were performed at ACH between 2011 and 2021. 17,773 (20.7%) of patients lived >50 km or more from the hospital – 5,329 (6.2%) 51- 100 km, 4,053 (4.7%) 101-150 km, n=2,323 (2.7%) 151-200 km, and 6,070 (7.1%) >200 km. Rural patients had higher 30-day mortality rates than urban patients, with an odds ratio of mortality (rural vs urban) of 2.30 (95% CI, 0.95 to 5.60). When stratified by distance, patients living closer to the hospital (0-50 km) had lower odds of mortality.

Conclusions

Canadian Rural patients have higher operative mortality risks than urban patients. This study identifies a vulnerable group of patients who do not have equal access to care and may experience worse outcomes.
目的: 本研究描述了农村社区与城市社区儿科患者术后死亡率的差异:本研究描述了农村社区与城市社区儿科患者术后死亡率的差异:背景:加拿大拥有世界第二大国土面积,人口密度为每平方公里 4 人。加拿大有 18 家儿童医院提供儿科手术服务,全部位于城市中心,但近五分之一的人口居住在农村或偏远社区。与城市人口相比,生活在农村地区的儿童的手术效果(包括死亡率)可能更差:研究对象包括 2011 年 1 月 1 日至 2021 年 12 月 31 日期间在一家儿童医院接受手术的出生至 18 岁的儿童患者。数据来自省级手术室信息系统(ORIS)数据库。收集了邮政编码、农村和城市状况、与儿童医院的距离(0-50 公里、51-100 公里、101-150 公里、151-200 公里和大于 200 公里)以及手术的紧急程度。收集了所有手术的 30 天死亡率:结果:2011 年至 2021 年间,共有 85,998 例手术在亚利桑那州立医院进行。17773名(20.7%)患者的居住地距离医院>50公里或更远,其中5329人(6.2%)居住地距离医院51-100公里,4053人(4.7%)居住地距离医院101-150公里,2323人(2.7%)居住地距离医院151-200公里,6070人(7.1%)居住地距离医院>200公里。农村患者的 30 天死亡率高于城市患者,死亡率的几率比(农村与城市)为 2.30(95% CI,0.95 至 5.60)。如果按距离分层,距离医院较近(0-50 公里)的患者死亡率较低:加拿大农村患者的手术死亡率高于城市患者。这项研究确定了一个脆弱的患者群体,他们无法平等地获得医疗服务,可能会经历更糟糕的结果。
{"title":"Geographic Differences in Pediatric Surgical Mortality in Canada: A Retrospective Cohort Study","authors":"Oluwatomilayo Daodu ,&nbsp;Saffa Aziz ,&nbsp;Melanie Morris ,&nbsp;Mary E. Brindle","doi":"10.1016/j.jpedsurg.2024.07.030","DOIUrl":"10.1016/j.jpedsurg.2024.07.030","url":null,"abstract":"<div><h3>Objective</h3><div>This study describes differences in postoperative mortality for pediatric patients in rural communities compared to urban communities.</div></div><div><h3>Background</h3><div>Canada has the second largest land mass in the world, with a population density of 4 people per km<sup>2</sup>. There are 18 children’s hospitals in Canada offering pediatric surgical services, all in urban centres, yet nearly one-fifth of the population lives in rural or remote communities. Children who live in rural settings may have worse surgical outcomes, including mortality rates, compared with urban populations.</div></div><div><h3>Methods</h3><div>Pediatric patients, from birth to 18 years old, who had surgery from January 1, 2011, to December 31, 2021, at a single Children's Hospital were included in the study. Data was obtained from the provincial Operating Room Information System (ORIS) database. Postal code, rural and urban status, distance to children's hospital (0-50 km, 51-100 km, 101-150 km, 151-200 km, and &gt;200 km), and procedure urgency were collected. 30-day mortality for all procedures was collected.</div></div><div><h3>Results</h3><div>85,998 surgical procedures were performed at ACH between 2011 and 2021. 17,773 (20.7%) of patients lived &gt;50 km or more from the hospital – 5,329 (6.2%) 51- 100 km, 4,053 (4.7%) 101-150 km, n=2,323 (2.7%) 151-200 km, and 6,070 (7.1%) &gt;200 km. Rural patients had higher 30-day mortality rates than urban patients, with an odds ratio of mortality (rural vs urban) of 2.30 (95% CI, 0.95 to 5.60). When stratified by distance, patients living closer to the hospital (0-50 km) had lower odds of mortality.</div></div><div><h3>Conclusions</h3><div>Canadian Rural patients have higher operative mortality risks than urban patients. This study identifies a vulnerable group of patients who do not have equal access to care and may experience worse outcomes.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 2","pages":"Article 161645"},"PeriodicalIF":2.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142004410","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}
引用次数: 0
Functional Outcome in Patients With Anorectal Malformation With Recto-prostatic or Recto-bulbar Urethral Fistula and Comparison Between Different Surgical Approaches: A Multi-center Study 肛门直肠畸形伴直肠-肛门或直肠-球部尿道瘘患者的功能预后及不同手术方法的比较:一项多中心研究。
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-01 DOI: 10.1016/j.jpedsurg.2024.07.037
Carol Wing Yan Wong , Hiroyuki Koga , Koshiro Sugita , Daiki Kato , Annika Mutanen , Patrick Ho Yu Chung , Go Miyano , Toshio Harumatsu , Satoshi Ieiri , Yoichi Nakagawa , Hiroo Uchida , Mikko P. Pakarinen , Kenneth Kak Yuen Wong

Purpose

To analyze and compare the outcomes in patients with anorectal malformation with rectoprostatic and rectourethral fistula between laparoscopic-assisted anorectoplasty (LAARP) versus posterior sagittal anorectoplasty (PSARP).

Method

We performed a retrospective review on all males with anorectal malformation (ARM) with recto-prostatic (ARM-RP) or recto-bulbar urethral fistula (ARM-RB) treated in five tertiary paediatric surgical centres in the past 25 years. Defecative function was assessed using the Krickenbeck classification and Kelly's score. Functional outcomes between patients with LAARP and PSARP were compared.

Results

There were a total of 136 males with ARM-RP and ARM-RB for analysis, among which 73 (53.7%) had ARM-RP and 63 (46.3%) had ARM-RB. The median age of the patients was 9.4 years (range 0.8–24.7 years) and the median age at operation was 0.4 years (0 day–3.1 years). 57 (41.9%) and 79 patients (58.1%) underwent PSARP and LAARP respectively. 34 patients (25%) had VACTERL association. 111 (81.6%) and 103 patients (75.7%) had sacral and spinal cord anomalies respectively. 19 patients (13.9%) eventually required Malone's Antegrade Continence Enema (MACE).
For the comparison between PSARP and LAARP, no difference in Kelly scores (4.58 ± 1.63 versus 4.67 ± 1.36) was identified (p = 0.79). Logistic regression for voluntary bowel movement showed that VACTER association (p = 0.02) and fistula location (p = 0.01) were significant prognostic factors, whereas the operation approach (PSARP or LAARP) was not (p = 0.65).

Conclusion

VACTERL association and fistula location were significant prognostic factors for voluntary bowel movement, and there appeared to be no significant difference in functional outcome between PSARP and LAARP.

Level of Evidence

IV.
目的:分析并比较腹腔镜辅助肛门直肠成形术(LAARP)与后矢状肛门直肠成形术(PSARP)对肛门直肠畸形伴直肠前列腺和直肠尿道瘘患者的治疗效果:我们对过去25年中在5家三级儿科外科中心接受治疗的所有男性肛门直肠畸形(ARM)伴直肠-肛门瘘(ARM-RP)或直肠-球部尿道瘘(ARM-RB)患者进行了回顾性研究。排便功能采用克里肯贝克分类法和凯利评分法进行评估。对LAARP和PSARP患者的功能结果进行了比较:共有136名男性患者接受了ARM-RP和ARM-RB分析,其中73人(53.7%)患有ARM-RP,63人(46.3%)患有ARM-RB。患者年龄中位数为 9.4 岁(0.8-24.7 岁),手术年龄中位数为 0.4 岁(0 天-3.1 岁)。57名患者(41.9%)和79名患者(58.1%)分别接受了PSARP和LAARP手术。34名患者(25%)与 VACTERL 有关。111名患者(81.6%)和103名患者(75.7%)分别患有骶骨和脊髓异常。19名患者(13.9%)最终需要进行马隆氏前向通便灌肠(MACE)。在 PSARP 和 LAARP 的比较中,Kelly 评分(4.58 ± 1.63 与 4.67 ± 1.36)没有差异(P = 0.79)。自主排便的逻辑回归显示,VACTER关联(p = 0.02)和瘘管位置(p = 0.01)是重要的预后因素,而手术方式(PSARP或LAARP)则不是(p = 0.65):结论:VACTERL关联和瘘管位置是自主排便的重要预后因素,PSARP和LAARP在功能预后方面似乎没有显著差异:证据等级:IV。
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引用次数: 0
期刊
Journal of pediatric surgery
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