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Fetal therapy for congenital diaphragmatic hernia: past, present and future. 先天性膈疝的胎儿治疗:过去、现在和未来。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-09-19 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2024-000835
Nimrah Abbasi, Tim Van Mieghem, Greg Ryan

Congenital diaphragmatic hernia (CDH) affects 1/2500-5000 infants and is associated with significant neonatal morbidity and mortality related to pulmonary hypoplasia and pulmonary hypertension. Current estimates of perinatal mortality are between 30-40%. With advances in neonatal and surgical management and now improvements in prenatal diagnosis and intervention, further reduction in mortality is anticipated. Data from the international Tracheal Occlusion to Accelerate Lung Growth (TOTAL) trials, have demonstrated the efficacy of fetal endoscopic tracheal occlusion (FETO) in severe left CDH (LCDH). Although promising, this intervention also has potential for significant morbidity related to prematurity and iatrogenic mortality if reversal of tracheal occlusion is unsuccessful. The implementation of FETO must proceed cautiously within Level III fetal therapy centers and with rigorous outcomes monitoring of centers offering this therapy, ensuring that they are experienced in antenatal severity assessment of CDH, FETO insertion and removal and are integrated with expert, standardized neonatal CDH centers with availability of Extracorporeal life support (ECLS). Further research is needed to better understand the impact of prematurity on FETO survivors, the role of FETO in moderate LCDH, Right CDH (RCDH) and non-isolated CDH in carefully selected circumstances as well as the development of alternative, less invasive, fetal therapies that can specifically target both pulmonary hypoplasia and pulmonary hypertension.

先天性膈疝(CDH)影响1/2500-5000名婴儿,与肺发育不全和肺动脉高压相关的新生儿发病率和死亡率显著相关。目前估计围产期死亡率在30-40%之间。随着新生儿和外科治疗的进步以及产前诊断和干预的改进,预计死亡率将进一步降低。来自国际气管闭塞加速肺生长(TOTAL)试验的数据已经证明了胎儿内镜气管闭塞(FETO)治疗严重左CDH (LCDH)的有效性。虽然很有希望,但如果气管闭塞的逆转不成功,这种干预也有可能导致与早产和医源性死亡相关的显著发病率。FETO的实施必须在三级胎儿治疗中心谨慎进行,并对提供这种治疗的中心进行严格的结果监测,确保他们在CDH的产前严重程度评估、FETO插入和取出方面经验丰富,并与专家、标准化的新生儿CDH中心整合,并提供体外生命支持(ECLS)。需要进一步的研究来更好地了解早产对FETO幸存者的影响,FETO在中度LCDH、右CDH (RCDH)和非孤立性CDH中的作用,以及开发可特异性针对肺发育不全和肺动脉高压的替代、侵入性更小的胎儿治疗。
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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
Association of initial assessment variables and mortality in severe pediatric traumatic brain injury. 严重小儿脑外伤的初始评估变量与死亡率之间的关系。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-05-28 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2023-000718
Katherine C Bergus, Kelli N Patterson, Lindsey Asti, Josh Bricker, Tariku J Beyene, Lauren N Schulz, Dana M Schwartz, Rajan K Thakkar, Eric A Sribnick

Background: Predictive scales have been used to prognosticate long-term outcomes of traumatic brain injury (TBI), but gaps remain in predicting mortality using initial trauma resuscitation data. We sought to evaluate the association of clinical variables collected during the initial resuscitation of intubated pediatric severe patients with TBI with in-hospital mortality.

Methods: Intubated pediatric trauma patients <18 years with severe TBI (Glasgow coma scale (GCS) score ≤8) from January 2011 to December 2020 were included. Associations between initial trauma resuscitation variables (temperature, pulse, mean arterial blood pressure, GCS score, hemoglobin, international normalized ratio (INR), platelet count, oxygen saturation, end tidal carbon dioxide, blood glucose and pupillary response) and mortality were evaluated with multivariable logistic regression.

Results: Among 314 patients, median age was 5.5 years (interquartile range (IQR): 2.2-12.8), GCS score was 3 (IQR: 3-6), Head Abbreviated Injury Score (hAIS) was 4 (IQR: 3-5), and most had a severe (25-49) Injury Severity Score (ISS) (48.7%, 153/314). Overall mortality was 26.8%. GCS score, hAIS, ISS, INR, platelet count, and blood glucose were associated with in-hospital mortality (all p<0.05). As age and GCS score increased, the odds of mortality decreased. Each 1-point increase in GCS score was associated with a 35% decrease in odds of mortality. As hAIS, INR, and blood glucose increased, the odds of mortality increased. With each 1.0 unit increase in INR, the odds of mortality increased by 1427%.

Conclusions: Pediatric patients with severe TBI are at substantial risk for in-hospital mortality. Studies are needed to examine whether earlier interventions targeting specific parameters of INR and blood glucose impact mortality.

背景:预测量表已被用于预测创伤性脑损伤(TBI)的长期预后,但在使用初始创伤复苏数据预测死亡率方面仍存在差距。我们试图评估在插管儿科严重创伤性脑损伤患者初始复苏期间收集的临床变量与院内死亡率之间的关联:方法:插管的儿科创伤患者:在314名患者中,中位年龄为5.5岁(四分位间距(IQR):2.2-12.8),GCS评分为3分(IQR:3-6),头部简略损伤评分(hAIS)为4分(IQR:3-5),大多数患者的损伤严重程度评分(ISS)为重度(25-49)(48.7%,153/314)。总死亡率为 26.8%。GCS 评分、hAIS、ISS、INR、血小板计数和血糖与院内死亡率有关(所有 p 结论:严重创伤性脑损伤的儿科患者有很大的院内死亡风险。需要进行研究,探讨针对 INR 和血糖特定参数的早期干预是否会影响死亡率。
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引用次数: 0
Social determinants of gastrointestinal malformation mortality in Brazil: a national study. 巴西胃肠道畸形死亡率的社会决定因素:一项全国性研究。
IF 0.8 4区 医学 Q4 PEDIATRICS Pub Date : 2024-05-21 eCollection Date: 2024-01-01 DOI: 10.1136/wjps-2023-000759
Ayla Gerk, Amanda Rosendo, Luiza Telles, Arícia Gomes Miranda, Madeleine Carroll, Bruna Oliveira Trindade, Sarah Bueno Motter, Esther Freire, Gabriella Hyman, Julia Ferreira, Fabio Botelho, Roseanne Ferreira, David P Mooney, Joaquim Bustorff-Silva

Introduction: In Brazil, approximately 5% are born with a congenital disorder, potentially fatal without surgery. This study aims to evaluate the relationship between gastrointestinal congenital malformation (GICM) mortality, health indicators, and socioeconomic factors in Brazil.

Methods: GICM admissions (Q39-Q45) between 2012 and 2019 were collected using national databases. Patient demographics, socioeconomic factors, clinical management, outcomes, and the healthcare workforce density were also accounted for. Pediatric Surgical Workforce density and the number of neonatal intensive care units in a region were extracted from national datasets and combined to create a clinical index termed 'NeoSurg'. Socioeconomic variables were combined to create a socioeconomic index termed 'SocEcon'. Simple linear regression was used to investigate if the temporal changes of both indexes were significant. The correlation between mortality and the different indicators in Brazil was evaluated using Pearson's correlation coefficient.

Results: Over 8 years, Brazil recorded 12804 GICM admissions. The Southeast led with 6147 cases, followed by the Northeast (2660), South (1727), North (1427), and Midwest (843). The North and Northeast reported the highest mortality, lowest NeoSurg, and SocEcon Index rates. Nevertheless, mortality rates declined across regions from 7.7% (2012) to 3.9% (2019), a 51.7% drop. The North and Midwest experienced the most substantial reductions, at 63% and 75%, respectively. Mortality significantly correlated with the indexes in nearly all regions (p<0.05).

Conclusion: Our study highlights the correlation between social determinants of health and GICM mortality in Brazil, using two novel indexes in the pediatric population. These findings provide an opportunity to rethink and discuss new indicators that could enhance our understanding of our country and could lead to the development of necessary solutions to tackle existing challenges in Brazil and globally.

简介在巴西,约有5%的人在出生时患有先天性疾病,如果不进行手术可能会致命。本研究旨在评估巴西胃肠道先天性畸形(GICM)死亡率、健康指标和社会经济因素之间的关系:方法:利用国家数据库收集了2012年至2019年期间的胃肠道先天性畸形(GICM)入院病例(Q39-Q45)。此外,还考虑了患者人口统计学、社会经济因素、临床管理、结果和医护人员密度。我们从国家数据集中提取了儿科外科劳动力密度和地区新生儿重症监护室数量,并将其结合起来,创建了一个名为 "NeoSurg "的临床指数。社会经济变量被合并为社会经济指数,称为 "SocEcon"。使用简单线性回归来研究这两个指数的时间变化是否显著。使用皮尔逊相关系数评估了巴西死亡率与不同指标之间的相关性:8 年间,巴西共记录了 12804 例 GICM 住院病例。其中,东南部有 6147 例,其次是东北部(2660 例)、南部(1727 例)、北部(1427 例)和中西部(843 例)。北部和东北部的死亡率最高,新手术率和社会经济指数最低。然而,各地区的死亡率从 7.7%(2012 年)下降到 3.9%(2019 年),降幅达 51.7%。北部和中西部的降幅最大,分别为 63% 和 75%。几乎所有地区的死亡率都与这些指数密切相关(p 结论:我们的研究强调了健康的社会决定因素与巴西 GICM 死亡率之间的相关性,在儿科人群中使用了两个新的指数。这些发现为我们提供了一个重新思考和讨论新指标的机会,这些新指标可以加深我们对巴西的了解,并能帮助我们制定必要的解决方案,以应对巴西和全球现有的挑战。
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引用次数: 0
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World Journal of Pediatric Surgery
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