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Numerical modeling assisting in surgical treatment of total anomalous pulmonary venous connection in children. 辅助儿童全异常肺静脉连接手术治疗的数值模型。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-10-14 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2023-000741
Jie Jin, Kaiyun Gu, Jiawei Liang, Jing Yu, Xiangming Fan

Objective: To develop a model using patient-specific computational fluid dynamics (CFD) to predict the required anastomotic size for total anomalous pulmonary venous connection (TAPVC) surgery and to forecast surgical outcomes.

Methods: Based on clinical data from patients, a CFD model was used to simulate the anastomosis between pulmonary venous confluence and the left atrium. Blood flow velocity, wall shear stress, power loss, and pressure were calculated using numerical algorithms within the model. Various sizes of anastomosis were applied during the simulation. The energy dissipation at the anastomosis was computed from the results and compared with real-world data.

Results: As the simulated anastomotic size increased, blood flow velocity, pulmonary venous pressure, and energy loss decreased. However, when the anastomotic size exceeded 18 mm, the efficiency of energy conversion no longer improved. The realistic and simulated velocities matched well for anastomosis sizes ranging from 15 to 20 mm.

Conclusion: The model can assist surgeons in preoperative planning for determining the anastomotic size in TAPVC surgical treatment.

目的利用患者特异性计算流体动力学(CFD)建立模型,预测全异常肺静脉连接(TAPVC)手术所需的吻合口大小,并预测手术效果:方法:根据患者的临床数据,使用 CFD 模型模拟肺静脉汇合处与左心房之间的吻合口。血流速度、壁剪应力、功率损耗和压力均通过模型内的数值算法计算得出。模拟过程中应用了不同大小的吻合口。根据结果计算吻合口处的能量耗散,并与实际数据进行比较:结果:随着模拟吻合口尺寸的增加,血流速度、肺静脉压力和能量损耗都有所下降。然而,当吻合口尺寸超过 18 毫米时,能量转换效率不再提高。吻合口尺寸在 15 毫米到 20 毫米之间时,实际血流速度与模拟血流速度非常吻合:该模型可以帮助外科医生在 TAPVC 手术治疗中确定吻合口大小的术前规划。
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引用次数: 0
Long-term outcomes and quality of life in patients with Hirschsprung disease. 赫氏胃肠病患者的长期疗效和生活质量。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-10-09 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000859
Mikko P Pakarinen, Annika Mutanen

Although patients with Hirschsprung disease are mostly affected by imperfect bowel function and fecal control in the long term, they are also predisposed to lower urinary tract symptoms, impaired sexual functions, infertility, psychosocial issues, and decreased quality of life. Rare, but notable comorbidities, which may manifest after childhood, include inflammatory bowel disease and familial medullary thyroid cancer. Despite frequent occurrence of fecal incontinence and constipation, the overall long-term outlook is quite optimistic as social continence with a good quality of life can be achieved by efficient bowel management also in those affected patients, whose self-coping strategies and medical treatment remain insufficient. Bowel dysfunction and other potential long-term complications are best managed by an interdisciplinary specialized approach not overlooking psychosocial issues, which also helps to correctly identify areas requiring continuing input by adult healthcare for young adult patients after transition. Additional research is needed to unravel the pathophysiological mechanisms of the long-term bowel dysfunction to identify novel therapeutic targets for development of more efficient innovative management strategies and thereby improvement of quality of life.

虽然从长远来看,赫氏贲门失弛缓症患者主要受到肠道功能不完善和粪便控制不佳的影响,但他们也容易出现下尿路症状、性功能受损、不育、社会心理问题和生活质量下降。罕见但值得注意的合并症可能在儿童期后才出现,包括炎症性肠病和家族性甲状腺髓样癌。尽管经常出现大便失禁和便秘,但总体的长期前景还是相当乐观的,因为对于那些自我应对策略和药物治疗仍然不足的受影响患者来说,通过有效的肠道管理也可以实现社交自理和良好的生活质量。肠道功能障碍和其他潜在的长期并发症最好由跨学科的专业方法来处理,同时不要忽视社会心理问题,这也有助于正确识别年轻成年患者在转归后需要成人医疗保健持续投入的领域。还需要开展更多的研究,以揭示长期肠道功能障碍的病理生理机制,从而确定新的治疗目标,制定更有效的创新管理策略,从而提高生活质量。
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引用次数: 0
Controversies in Hirschsprung surgery. 赫氏脓肿手术的争议。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-09-25 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000887
Thomas O Xu, Marc A Levitt, Christina Feng

The treatment of Hirschsprung disease (HSCR) is surgical resection of aganglionic bowel and subsequent pull-through of ganglionated bowel. Despite many advances since the initial description of the disease and its surgical management more than half a century ago, there remain considerable controversies regarding the history of the surgical technique, the optimal timing of the primary and multistage pull-through, the best treatment for patients with a delayed diagnosis of HSCR, and the management of post pull-through complications such as soiling due to sphincter incompetence, the presence of a transition zone, and the prevention of enterocolitis. The following review will explore each of these controversies.

赫氏胃肠病(HSCR)的治疗方法是通过手术切除无神经节肠道,然后拉通神经节肠道。尽管自半个多世纪前首次描述该病及其手术治疗以来取得了许多进展,但在手术技术的历史、初次拉通和多级拉通的最佳时机、对 HSCR 诊断延迟患者的最佳治疗方法、拉通后并发症的处理(如括约肌功能不全引起的便秘、过渡区的存在以及肠套叠炎的预防)等方面仍存在相当大的争议。下面的综述将逐一探讨这些争议。
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引用次数: 0
Top 100 most-cited articles on pediatric anesthesia from 1990 to 2023. 1990 年至 2023 年小儿麻醉方面被引用次数最多的 100 篇文章。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-09-18 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000823
Minyue Qian, Jia Zhong, Zhongteng Lu, Wenyuan Zhang, Kai Zhang, Yue Jin

Pediatric anesthesia presents greater challenges than does adult anesthesia. This bibliometric analysis aimed to analyze the top 100 most cited articles to be better understand the hot spots and prospects in pediatric anesthesia. Articles and reviews related to pediatric anesthesia were retrieved from the Web of Science Core Collection from 1990 to 2023. A bibliometric analysis of the top 100 most cited articles was also performed using information such as topics, author names, countries, institutions, publication years, and journals. A total of 32 831 articles were identified, with a total of 32 230 citations for the top 100 articles. The peak period for pediatric anesthesia research was from 2005 to 2009. The USA has emerged as the most active country in pediatric anesthesia research. Major journals published included Anesthesia and Analgesia, Anesthesiology, and Pediatrics, underscoring their authority in the field. Clinical studies on the top 100 most cited articles have focused on different stages of the perioperative period, the use of different anesthetic agents, and adverse outcomes in pediatric patients. The current study conducted a bibliometric analysis of the top 100 most cited articles in the field of pediatric anesthesia. Such insights are valuable for identifying research hot spots, assessing academic impact and collaboration in pediatric anesthesia, and guiding future research directions.

与成人麻醉相比,小儿麻醉面临着更大的挑战。这项文献计量学分析旨在分析被引用次数最多的前100篇文章,以便更好地了解儿科麻醉的热点和前景。我们从 Web of Science 核心数据库中检索了 1990 年至 2023 年与儿科麻醉相关的文章和综述。此外,还利用主题、作者姓名、国家、机构、出版年份和期刊等信息对引用率最高的前 100 篇文章进行了文献计量分析。共鉴定出 32 831 篇文章,前 100 篇文章的总引用次数为 32 230 次。2005 年至 2009 年是儿科麻醉研究的高峰期。美国成为儿科麻醉研究最活跃的国家。发表的主要期刊包括《麻醉与镇痛》(Anesthesia and Analgesia)、《麻醉学》(Anesthesiology)和《儿科学》(Pediatrics),彰显了其在该领域的权威性。被引用次数最多的前 100 篇文章的临床研究主要集中在围术期的不同阶段、不同麻醉剂的使用以及儿科病人的不良后果等方面。本研究对儿科麻醉领域被引用次数最多的前 100 篇文章进行了文献计量分析。这些见解对于确定研究热点、评估儿科麻醉的学术影响和合作以及指导未来的研究方向非常有价值。
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引用次数: 0
Pullthrough pitfalls in treating Hirschsprung disease. 治疗赫氏胃肠病的牵引陷阱
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-09-13 DOI: 10.1136/wjps-2024-000862
Kristy L Rialon,Jacob C Langer
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引用次数: 0
Hirschsprung disease: common and uncommon variants. 赫氏prung 病:常见和不常见变异。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-08-28 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000864
Lily S Cheng, Richard J Wood
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引用次数: 0
Surgical management of the diaphragmatic defect in congenital diaphragmatic hernia: a contemporary review. 先天性膈疝膈肌缺损的手术治疗:当代综述。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-08-21 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2023-000747
Marietta Jank, Michael Boettcher, Richard Keijzer

Worldwide, 150 children are born each day with congenital diaphragmatic hernia (CDH), a diaphragmatic defect with concomitant abnormal lung development. Patients with CDH with large defects are particularly challenging to treat, have the highest mortality, and are at significant risk of long-term complications. Advances in prenatal and neonatal treatments have improved survival in high-risk patients with CDH, but surgical treatment of large defects lacks standardization. Open repair by an abdominal approach has long been considered the traditional procedure, but the type of defect repair (patch or muscle flap) and patch material (non-absorbable, synthetic or absorbable, biological) remain subjects of debate. Increased experience and improved techniques in minimally invasive surgery (MIS) have expanded selection criteria for thoracoscopic defect repair in cardiopulmonary stable patients with small defects. However, the application of MIS to repair large defects remains controversial due to increased recurrence rates and unknown long-term effects of perioperative hypercapnia and acidosis resulting from capnothorax and reduced ventilation. Current recommendations on the surgical management rely on cohort studies of varying patient numbers and data on the long-term outcomes are sparse. Here, we discuss surgical approaches for diaphragmatic defect repair highlighting advancements, and knowledge gaps in surgical techniques (open surgery and MIS), patch materials and muscle flaps for large defects, as well as procedural adjuncts and management of CDH variants.

全世界每天有 150 名先天性膈疝(CDH)患儿出生,这种膈肌缺陷同时伴有肺部发育异常。缺陷较大的 CDH 患者尤其难以治疗,死亡率最高,并面临长期并发症的巨大风险。产前和新生儿治疗的进步提高了高风险 CDH 患者的存活率,但大面积缺损的手术治疗缺乏标准化。腹部开腹修复一直被认为是传统的手术方法,但缺损修复的类型(补片或肌瓣)和补片材料(非吸收、合成或可吸收、生物)仍存在争议。随着微创手术(MIS)经验的增加和技术的改进,胸腔镜缺损修补术的选择标准也在不断扩大,适用于心肺功能稳定且缺损较小的患者。然而,由于复发率增加,以及帽状气胸和通气量减少导致的围手术期高碳酸血症和酸中毒的长期影响未知,MIS 在修复大缺损方面的应用仍存在争议。目前关于手术治疗的建议主要依赖于不同患者人数的队列研究,而关于长期疗效的数据却很少。在此,我们将讨论膈肌缺损修复的手术方法,重点介绍手术技术(开放手术和 MIS)、修补材料、大缺损的肌肉瓣、手术辅助工具和 CDH 变异的处理等方面的进展和知识差距。
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引用次数: 0
Role of genetics and the environment in the etiology of congenital diaphragmatic hernia. 遗传和环境在先天性膈疝病因中的作用。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-08-21 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000884
Siyuan Liu, Lan Yu

Congenital diaphragmatic hernia (CDH) is a congenital malformation characterized by failure of diaphragm closure during embryonic development, leading to pulmonary hypoplasia and pulmonary hypertension, which contribute significantly to morbidity and mortality. The occurrence of CDH and pulmonary hypoplasia is theorized to result from both abnormalities in signaling pathways of smooth muscle cells in pleuroperitoneal folds and mechanical compression by abdominal organs within the chest cavity on the developing lungs. Although, the precise etiology of diaphragm maldevelopment in CDH is not fully understood, it is believed that interplay between genes and the environment contributes to its onset. Approximately 30% of patients with CDH possess chromosomal or single gene defects and these patients tend to have inferior outcomes compared with those without genetic associations. At present, approximately 150 gene variants have been linked to the occurrence of CDH. The variable expression of the CDH phenotype in the presence of a recognized genetic predisposition can be explained by an environmental effect on gene penetrance and expression. The retinoic acid pathway is thought to play an essential role in the interactions of genes and environment in CDH. However, apart from the gradually maturing retinol hypothesis, there is limited evidence implicating other environmental factors in CDH occurrence. This review aims to describe the pathogenesis of CDH by summarizing the genetic defects and potential environmental influences on CDH development.

先天性膈疝(CDH)是一种先天性畸形,其特点是膈肌在胚胎发育过程中闭合失败,导致肺发育不全和肺动脉高压,从而大大增加了发病率和死亡率。据推测,CDH 和肺发育不全的发生是由于胸膜腹膜皱襞平滑肌细胞信号传导途径异常以及胸腔内腹腔器官对发育中肺部的机械压迫所致。虽然 CDH 中横膈膜发育不良的确切病因尚未完全明了,但人们认为基因和环境之间的相互作用是导致其发病的原因。大约 30% 的 CDH 患者存在染色体或单基因缺陷,与无遗传关联的患者相比,这些患者的预后往往较差。目前,约有 150 种基因变异与 CDH 的发生有关。在存在公认的遗传易感性的情况下,CDH 表型的不同表现可通过环境对基因渗透性和表达的影响来解释。视黄酸途径被认为在 CDH 的基因和环境相互作用中扮演了重要角色。然而,除了逐渐成熟的视黄醇假说外,与 CDH 发生有关的其他环境因素证据有限。本综述旨在通过总结遗传缺陷和环境对 CDH 发病的潜在影响,描述 CDH 的发病机制。
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引用次数: 0
Lung-protective ventilation in the management of congenital diaphragmatic hernia. 先天性膈疝治疗中的肺保护性通气。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-08-05 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000789
Mike Traynor

Prioritizing lung-protective ventilation has produced a clear mortality benefit in neonates with congenital diaphragmatic hernia (CDH). While there is a paucity of CDH-specific evidence to support any particular approach to lung-protective ventilation, a growing body of data in adults is beginning to clarify the mechanisms behind ventilator-induced lung injury and inform safer management of mechanical ventilation in general. This review summarizes the adult data and attempts to relate the findings, conceptually, to the CDH population. Critical lessons from the adult studies are that much of the damage done during conventional mechanical ventilation affects normal lung tissue and that most of this damage occurs at the low-volume and high-volume extremes of the respiratory cycle. Consequently, it is important to prevent atelectasis by using sufficient positive end-expiratory pressure while also avoiding overdistention by scaling tidal volume to the amount of functional lung tissue rather than body weight. Paralysis early in acute respiratory distress syndrome improves outcomes, possibly because consistent respiratory mechanics facilitate avoidance of both atelectasis and overdistention-a mechanism that may also apply to the CDH population. Volume-targeted conventional modes may be advantageous in CDH, but determining optimal tidal volume is challenging. Both high-frequency oscillatory ventilation and high-frequency jet ventilation have been used successfully as 'rescue modes' to avoid extracorporeal membrane oxygenation, and a prospective trial comparing the two high-frequency modalities as the primary ventilation strategy for CDH is underway.

在患有先天性膈疝(CDH)的新生儿中,优先考虑肺保护性通气可明显降低死亡率。虽然支持任何特定肺保护性通气方法的 CDH 特异性证据很少,但越来越多的成人数据开始阐明呼吸机诱发肺损伤背后的机制,并为更安全的机械通气管理提供依据。本综述总结了成人数据,并试图从概念上将研究结果与 CDH 患者联系起来。从成人研究中得到的重要启示是,常规机械通气过程中造成的大部分损伤都会影响正常肺组织,而且大部分损伤都发生在呼吸周期的低通气量和高通气量两个极端。因此,必须通过使用足够的呼气末正压来防止肺不张,同时还要根据肺功能组织的数量而不是体重来调整潮气量,以避免过度阻滞。急性呼吸窘迫综合征早期瘫痪可改善预后,这可能是因为一致的呼吸力学有助于避免出现肺不张和过度滞留--这一机制可能也适用于 CDH 患者。以容量为目标的传统模式可能对 CDH 有利,但确定最佳潮气量具有挑战性。高频振荡通气和高频喷射通气已被成功用作避免体外膜氧合的 "救援模式",目前正在进行一项前瞻性试验,比较这两种高频模式作为 CDH 的主要通气策略。
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引用次数: 0
Efficacy of robot-assisted thoracoscopic surgery in the treatment of pulmonary sequestration in children. 机器人辅助胸腔镜手术治疗儿童肺动脉栓塞的疗效。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-08-01 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2023-000748
Liang Liang, Zheng Tan, Ting Huang, Yue Gao, Jian Zhang, Jiangen Yu, Jie Xia, Qiang Shu

Objective: This study was performed to evaluate the efficacy of robot-assisted thoracoscopic surgery (RATS) in the treatment of pulmonary sequestration (PS) in children.

Methods: All video-assisted thoracoscopic surgery (VATS) and RAST performed on patients with PS at a single center from May 2019 to July 2023 were identified. The χ 2 and Wilcoxon tests were used to compare the perioperative outcomes between VATS and RATS groups.

Results: Ninety-three patients underwent RATS while 77 patients underwent VATS. In both two groups, one patient converted to thoracotomy and no surgical mortality case. The median operation time was longer for the RATS group compared with the VATS group (75 min vs. 60 min, p <0.001). A lower ratio of chest tube indwelling (61.3% vs. 90.9%, p <0.001), fewer drainage days (1.0 day vs. 2.0 days, p <0.001), and a shorter postoperative length of stay (5.0 days vs. 6.0 days, p <0.001) were found in the RATS group than that in the VATS group. No significant difference was found in the incidence of short-term postoperative complications (hydrothorax and pneumothorax) between two groups.

Conclusions: RATS was safe and effective in children with PS over 6 months old and more than 7 kg. Furthermore, RATS led to better short-time postoperative outcome than VATS. Multi-institutional studies are warranted to compare differences in long-term outcomes between RATS and VATS.

研究目的本研究旨在评估机器人辅助胸腔镜手术(RATS)治疗儿童肺动脉栓塞(PS)的疗效:方法:对2019年5月至2023年7月在一个中心对PS患者实施的所有视频辅助胸腔镜手术(VATS)和RAST进行鉴定。采用χ 2检验和Wilcoxon检验比较VATS组和RATS组的围手术期结果:93名患者接受了RATS手术,77名患者接受了VATS手术。两组中均有一名患者转为开胸手术,无手术死亡病例。与 VATS 组相比,RATS 组的中位手术时间更长(75 分钟 vs. 60 分钟,p vs. 90.9%,p vs. 2.0 天,p vs. 6.0 天,p 结论:对于 6 个月以上、体重超过 7 公斤的 PS 患儿,RATS 是安全有效的。此外,与 VATS 相比,RATS 术后短期疗效更好。有必要进行多机构研究,以比较 RATS 和 VATS 在长期疗效上的差异。
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引用次数: 0
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World Journal of Pediatric Surgery
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