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Optimizing the anesthetic care of patients with aromatic l-amino acid decarboxylase deficiency. 优化芳香族 l-氨基酸脱羧酶缺乏症患者的麻醉护理。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-02-01 Epub Date: 2024-10-22 DOI: 10.1111/pan.15025
Megha K Kanjia, Edmund H Jooste, Melissa Illig, Jennifer Neifeld Capps, Christoph Eisner, Shou Zen Fan, Jerzy Lenarczyk, Rafał Wojdacz

Aromatic l-amino acid decarboxylase (AADC) deficiency is a rare autosomal recessive disorder that results in a lack of the monoamine neurotransmitters dopamine, serotonin, norepinephrine, and epinephrine. Patients present with a wide spectrum of symptoms, including motor and autonomic dysfunction, hypotonia, and developmental delay, often before the age of one. Until recently, treatment options were limited to symptom control, but the recent approval of the first gene therapy for AADC deficiency in Europe and the UK has provided an alternative to treating symptoms for this disease. Eladocagene exuparvovec is a one-time gene therapy, administered bilaterally to the putamen by magnetic resonance imaging-guided stereotactic neurosurgery. While administration of the gene therapy itself is minimally invasive, the anesthetic management of patients with AADC deficiency is challenging due to the absence of sympathetic regulation secondary to the lack of adrenergic neurotransmitters. Optimal anesthetic management requires an understanding of the complex and heterogeneous nature of the disease. Hemodynamic instability, temperature dysregulation, and hypoglycemia are of primary concern, but there are also challenges regarding intravenous access and airway management. A thorough preoperative assessment is essential and should be guided by the patient's history. Advanced planning is necessary regarding the timing of the procedure schedule and operative plan; meticulous preparation, simulation for the operating room, as well as communication with all perioperative staff members, are crucial. Intraoperatively, utmost care must be taken to protect the skin, maintain body temperature, and to prepare for inotropic and/or glycemic support as needed. Postoperative intensive care management is necessary for consideration of postoperative extubation and provision of supportive care. With careful planning, preparation, and vigilance, patients with AADC deficiency can safely undergo anesthesia.

芳香族 l-氨基酸脱羧酶(AADC)缺乏症是一种罕见的常染色体隐性遗传疾病,会导致缺乏单胺类神经递质多巴胺、血清素、去甲肾上腺素和肾上腺素。患者表现出多种症状,包括运动和自主神经功能障碍、肌张力低下和发育迟缓,通常在一岁前发病。直到最近,治疗方法还仅限于控制症状,但最近欧洲和英国批准了第一种针对 AADC 缺乏症的基因疗法,为这种疾病的症状治疗提供了另一种选择。Eladocagene exuparvovec是一种一次性基因疗法,通过磁共振成像引导下的立体定向神经外科手术在双侧大脑皮质实施。虽然基因治疗本身是微创手术,但由于缺乏肾上腺素能神经递质导致交感神经调节功能缺失,AADC 缺乏症患者的麻醉管理具有挑战性。要实现最佳麻醉管理,就必须了解这种疾病的复杂性和异质性。血流动力学不稳定、体温调节失调和低血糖是首要关注的问题,但在静脉通路和气道管理方面也存在挑战。全面的术前评估至关重要,并应以患者的病史为指导。有必要对手术时间安排和手术计划进行提前规划;精心准备、模拟手术室以及与所有围手术期工作人员的沟通都至关重要。术中必须格外注意保护皮肤、保持体温,并根据需要准备肌力和/或血糖支持。术后重症监护管理是考虑术后拔管和提供支持性护理所必需的。经过精心策划、准备和警惕,AADC 缺乏症患者可以安全地接受麻醉。
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引用次数: 0
Does cognitive aid app design influence the speed of actions during a critical event?: A simulation study. 认知辅助应用程序的设计是否会影响关键事件中的行动速度?
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-02-01 Epub Date: 2024-11-07 DOI: 10.1111/pan.15037
Brady Still, Anna Clebone
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引用次数: 0
Clinical Impact of Specific Extraocular Muscle Manipulation and the Oculocardiac Reflex on Postoperative Vomiting in Pediatric Strabismus Surgery: A Multicenter, Observational Study. 特殊眼外肌操作和眼心反射对儿童斜视术后呕吐的临床影响:一项多中心观察性研究。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-02-01 Epub Date: 2024-11-30 DOI: 10.1111/pan.15047
Taiki Kojima, Yusuke Yamauchi, Takashi Fujiwara, Soichiro Obara, Aya Sueda, Riku Takahashi, Sayuri Yasuda, Hiroshi Kitoh

Background: Strabismus surgery, which is commonly performed in children, poses a high risk of postoperative vomiting. The current anesthesia guidelines for the prevention of postoperative vomiting in children are based on heterogeneous populations involving different types of surgery, and risk factors for postoperative vomiting in, specifically, the pediatric strabismus surgery population are unclear. Moreover, the effects of manipulating the deeply attached extraocular muscles and the oculocardiac reflex on this risk remain inconclusive.

Aim: To evaluate the associations among inferior oblique muscle manipulation, the oculocardiac reflex, and postoperative vomiting in children with retrospectively collected data.

Methods: The study had a multicenter retrospective cross-sectional design and was conducted at three institutions (two tertiary-care children's hospitals and one pediatric-adult mixed community hospital). It included children aged < 18 years and without major comorbidities undergoing strabismus surgery. The primary exposure was inferior oblique muscle manipulation during surgery. The outcome of interest was postoperative vomiting or antiemetic medication usage within 24 h postsurgery or by discharge.

Results: Among 3152 children postoperative vomiting occurred in 108/795 (13.6%) children with and 227/2357 (9.6%) without inferior oblique muscle manipulation (unadjusted odds ratio, 1.57; 95% confidence interval, 1.21-2.05; p = 0.001). Multilevel logistic regression analysis, adjusting for potential confounders and surgeon-related variance, revealed that inferior oblique muscle manipulation (adjusted odds ratio, 1.58; 95% confidence interval, 1.15-2.18; p = 0.005), but not the oculocardiac reflex (adjusted odds ratio, 1.06; 95% confidence interval, 0.76-1.48; p = 0.73), was associated with postoperative vomiting after adjusting for confounders.

Conclusions: Stronger preventive measures against postoperative vomiting are recommended in healthy children undergoing strabismus surgery with inferior oblique muscle manipulation. Additionally, inferior oblique muscle manipulation should be considered a potential confounder in future related studies. However, the oculocardiac reflex was not associated with postoperative vomiting in pediatric strabismus surgery.

背景:斜视手术通常在儿童中进行,术后呕吐的风险很高。目前预防儿童术后呕吐的麻醉指南是基于涉及不同手术类型的异质人群,特别是儿童斜视手术人群术后呕吐的危险因素尚不清楚。此外,操纵深附着眼外肌和心房反射对这种风险的影响仍不确定。目的:回顾性分析小儿下斜肌操作、心房反射和术后呕吐之间的关系。方法:本研究采用多中心回顾性横断面设计,在三家机构(两家三级保健儿童医院和一家儿科-成人混合社区医院)进行。结果:在3152例患儿中,有下斜肌操作的患儿术后呕吐发生率为108/795(13.6%),没有下斜肌操作的患儿术后呕吐发生率为227/2357(9.6%)(未校正优势比为1.57;95%置信区间为1.21-2.05;p = 0.001)。多水平logistic回归分析,调整潜在混杂因素和手术相关方差,显示下斜肌操作(调整优势比,1.58;95%置信区间为1.15-2.18;P = 0.005),但心房反射无统计学意义(校正优势比1.06;95%置信区间为0.76-1.48;P = 0.73),校正混杂因素后与术后呕吐相关。结论:健康儿童斜视手术下斜肌操作应加强对术后呕吐的预防。此外,在未来的相关研究中,下斜肌操作应被视为潜在的混杂因素。然而,心眼反射与儿童斜视手术后呕吐无关。
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引用次数: 0
Design considerations for development of cuffed endotracheal tube for small airways. 开发用于小气道的袖带式气管导管的设计考虑因素。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-02-01 Epub Date: 2024-11-19 DOI: 10.1111/pan.15033
Seamus Maguire, Daniel Wade, James Curley, Sean Morris

Endotracheal tubes (ETTs) are life-supporting devices that are designed to maintain a patent airway in patients who are unable to sustain an airway due to illness or injury. Patients with small airways, such as neonates and pediatrics, have unique structural and functional features, making it essential that ETT design considers and executes on these particular needs. Though uncuffed ETTs have historically been preferred for patients younger than eight years of age, advances in cuffed ETT design and construction can be utilized to manufacture ETTs that are optimized for the smallest, most fragile airways. The purpose of this article is to discuss certain design features of cuffed ETTs in respect to small airways.

气管内导管(ETT)是一种生命支持设备,旨在为因疾病或受伤而无法维持气道通畅的患者维持通畅的气道。新生儿和儿科等气道狭小的患者具有独特的结构和功能特征,因此 ETT 的设计必须考虑并满足这些特殊需求。尽管无袖带 ETT 一直以来都是 8 岁以下患者的首选,但可以利用袖带 ETT 设计和结构方面的进步,制造出最适合最小、最脆弱气道的 ETT。本文旨在讨论针对小气道的带袖带式 ETT 的某些设计特点。
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引用次数: 0
Factors associated with the use of regional anesthesia for calcaneal osteotomy in pediatric patients: A single-center, retrospective cohort study. 儿科患者小腿截骨术中使用区域麻醉的相关因素:单中心回顾性队列研究。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-02-01 Epub Date: 2024-11-09 DOI: 10.1111/pan.15030
Benjamin Y Andrew, Kayla E Pfaff, Sarah Jooste, Lisa M Einhorn

Background: Despite known disparities in pediatric perioperative outcomes, few studies have examined factors associated with the use of regional anesthesia for pediatric orthopedic surgery.

Aims: This investigation aimed to determine if minority and developmental disability status were associated with the allocation of peripheral nerve blocks in calcaneal osteotomy.

Methods: We conducted a single-center, retrospective study of records of patients <18 years who underwent calcaneal osteotomy from 2013 to 2023. Regional technique was classified into three groups: popliteal-sciatic single-shot block, popliteal-sciatic catheter, and no block. Patients were classified as either nonminority (white, non-Hispanic) or minority. Developmental disability status was defined based on medical history and classified as binary. Anesthesiologists were classified as "regional" or "nonregional" based on clinical expertise. A Bayesian hierarchical multinomial model with random intercepts for patients and surgeons was used to investigate the association of minority status, developmental disability, and anesthesiologist expertise with block selection.

Results: We analyzed 287 cases in 225 patients; of these, 55% occurred in minority patients and 28% occurred in patients with developmental disability. Catheters were placed in 45% of cases, single shot blocks in 41%, and no block in 14%. Minority and nonminority patients had a similar likelihood of receiving of any block. Patients with developmental disability had a -22% absolute difference of receiving any block (95% credible interval [-38%, -7%]) compared to those without developmental disability (55% vs. 77%), an effect primarily driven by a lower rate of catheter placement in these children. Regional anesthesiologists were more likely to place catheters (23% absolute increase; 36% vs. 13%) and more likely to perform any block in children with developmental disability (30% absolute increase; 67% vs. 37%) than nonregional anesthesiologists.

Conclusions: Decision-making surrounding the placement of regional anesthesia techniques is complex. In this study, developmental disability status and anesthesiologist experience were associated with a difference in the use of regional anesthesia in patients undergoing calcaneal osteotomy.

背景:目的:本调查旨在确定少数民族和发育障碍状况是否与小腿截骨术中外周神经阻滞的分配有关:结果:我们分析了 22 个病例中的 287 个病例:我们对 225 名患者的 287 个病例进行了分析;其中 55% 的病例发生在少数族裔患者身上,28% 的病例发生在发育障碍患者身上。45%的病例放置了导管,41%的病例进行了单次阻断,14%的病例未进行阻断。少数族裔和非少数族裔患者接受任何阻滞的可能性相似。与无发育障碍的患者(55% 对 77%)相比,有发育障碍的患者接受任何阻滞的绝对差异为-22%(95% 可信区间 [-38%, -7%]),这种影响主要是由于这些儿童的导管置入率较低。与非区域麻醉医生相比,区域麻醉医生更有可能为发育障碍儿童放置导管(绝对增加 23%;36% 对 13%),更有可能为他们进行任何阻滞(绝对增加 30%;67% 对 37%):结论:围绕区域麻醉技术的决策非常复杂。在这项研究中,发育障碍状况和麻醉师经验与小腿截骨术患者使用区域麻醉的差异有关。
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引用次数: 0
In This Issue March 2025.
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-01-30 DOI: 10.1111/pan.15073
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引用次数: 0
Evaluation of the Pediatric Regional Anesthesia Time-Out Checklist: A Simulation Study.
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-01-24 DOI: 10.1111/pan.15069
Anna Clebone, Brian Duggar, Tessa N Mandler, Barbara K Burian, Melissa M Masaracchia, David Polaner

Introduction: The Society for Pediatric Anesthesia Quality and Safety Committee developed the Pediatric Regional Anesthesia Time-Out Checklist, consisting of 14 safety items intended to be reviewed by an anesthesia team prior to a regional anesthetic. Primarily, we hypothesized that use of this Checklist would increase the number of safety items performed compared with no checklist, evaluating the usefulness of this tool. Secondarily, we hypothesized that, after checklist training, subjects would show better clinical judgment by electing to perform a regional anesthetic in scenarios in which no programmed error existed and electing to not perform a regional anesthetic in scenarios in which a programmed error did exist.

Methods: Each anesthesia attending/trainee pair participated in 12 different randomized video-recorded medium-fidelity regional anesthesia simulation scenarios, receiving checklist training after half of the scenarios had been completed by each pair. In four of the scenarios, subjects were expected to decline to perform the regional anesthetic because of an error programmed into the scenario. Two errors consisted of a maximum dose of local anesthetic given by the surgeon immediately prior to the planned regional anesthetic and two errors consisted of coagulation issues prior to neuraxial block (1 with a low platelet count and 1 receiving low molecular weight heparin). Scenarios were scored for the number of safety items identified and performed by the subjects. Additionally, the team's choice to perform the regional anesthetic or abort was recorded.

Results: One-hundred and thirty-two scenarios were performed by 22 physicians. A greater number of safety items were completed after training on the Pediatric Regional Anesthesia Time-Out Checklist, for each of 11 individual groups and when data from all groups was pooled, p < 0.001, 95% CI (0.33, 0.41). Overall, 78% of safety items studied were performed after checklist training compared to 41% of safety items performed prior to training. The team's choice to perform or abort the regional anesthetic occurred as expected more often (92% of scenarios) after Checklist training, compared to before checklist training (77% of scenarios), t = 3.41; p = 0.001, 95% CI (0.03, 0.27). Teams chose to perform the regional anesthetic despite a programmed error in three scenarios (0.05%) prior to Checklist training and no scenarios (0%) after Checklist training.

Conclusion: Pediatric Regional Anesthesia Time-Out Checklist training led to an increased number of safety items performed prior to a simulated anesthetic.

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引用次数: 0
Supervision of Pediatric Anesthesia After-Hours: A Survey of Pediatric Anesthetists in Australia and New Zealand. 下班后儿科麻醉的监督:对澳大利亚和新西兰儿科麻醉师的调查。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-01-22 DOI: 10.1111/pan.15068
Steven Cai, Fiona Taverner

Background: After-hours pediatric anesthesia may pose increased risks, with a heightened potential for sudden cardio-respiratory decline. While mortality rates are low in Australia and New Zealand, critical events and morbidity occur more frequently and present ongoing challenges. However, little is known about how trainees are supervised during these high-risk periods.

Methods: An anonymized online survey of members of the Society for Pediatric Anesthesia in New Zealand and Australia (SPANZA) was conducted to explore supervising anesthetists attitudes towards after-hours supervision. The survey examined the influence of patient age, medical history, trainee experience, and surgery type on supervision practices.

Results: Respondents identified age and physical status as key risk factors but reported providing less direct supervision than recommended by international studies, especially for non-complex surgeries in healthy children. Trainee experience was a significant factor in supervision decisions.

Conclusion: Pediatric anesthetists in Australia and New Zealand recognize major risk factors but tend to supervise more remotely after-hours. Further research is needed to evaluate the effects of these supervision practices on outcomes.

背景:小儿小时后麻醉可能会增加风险,增加突发心肺功能衰退的可能性。虽然澳大利亚和新西兰的死亡率很低,但重大事件和发病率发生得更频繁,并构成持续的挑战。然而,在这些高风险时期,学员是如何受到监督的,人们知之甚少。方法:对新西兰和澳大利亚儿科麻醉学会(SPANZA)成员进行匿名在线调查,探讨监督麻醉师对下班后监督的态度。调查考察了患者年龄、病史、实习经验和手术类型对监督实践的影响。结果:受访者认为年龄和身体状况是关键的危险因素,但报告提供的直接监督少于国际研究的建议,特别是对健康儿童的非复杂手术。实习经验是监督决策的重要因素。结论:澳大利亚和新西兰的儿科麻醉师认识到主要的危险因素,但倾向于在下班后进行远程监督。需要进一步的研究来评估这些监督实践对结果的影响。
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引用次数: 0
Use of real-time respiratory function monitor improves neonatal face mask ventilation: Cross-over simulation study. 使用实时呼吸功能监测仪改善新生儿面罩通气:交叉模拟研究。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-01-01 Epub Date: 2024-10-07 DOI: 10.1111/pan.15020
Prakash Kannan Loganathan, Charlotte Ashton, Emily Harrold, Sarah Wigston, Le Minh Thao Doan, Annalisa Occhipinti

Background: Neonatal resuscitation airway training can be difficult as there is no feedback on the face mask technique. "JUNO" is a training respiratory function monitor that provides feedback on mask leak, ventilatory rate, and tidal volume.

Objective: To evaluate whether the use of the JUNO improves face mask ventilation techniques in manikin models.

Methods: We conducted an observational cross-over study with our unit staff. Following instructions, each participant performed a single-person technique, followed by a two-person technique with no JUNO feedback. This was repeated with JUNO feedback visible. A similar sequence was performed both in term and preterm manikins, giving a total of 8 sequences. Each participant was instructed to perform 1 min of positive pressure ventilation providing 30 inflations/minute for all of the simulations. Each of the simulation data underwent a data cleaning process.

Results: Thirty-eight subjects provided a total of 304 sequences of positive pressure ventilation. A total of 13 354 inflations were analyzed. The feedback group had significantly lower rates of inflations with leak >60%, lower rates of excessive tidal volumes, lower mean leak percentage, and a lower mean inspiratory tidal volumes. When analyzed based on the technique (single person and two-person), similar positive results were noted in the "feedback group", across all the strata of staff. All of the staff reported that JUNO improved their ventilation technique and would recommend it for staff training.

Conclusions: The use of JUNO significantly improved mask ventilation consistently across manikin types, staff roles, and techniques (either single- or two-person).

背景:新生儿复苏气道训练很困难,因为面罩技术没有反馈。"JUNO "是一种训练用呼吸功能监测仪,可提供面罩泄漏、通气率和潮气量的反馈信息:评估 JUNO 的使用是否能改善人体模型的面罩通气技术:我们对本单位的员工进行了一项观察性交叉研究。根据指导,每位参与者先进行单人操作,然后在没有 JUNO 反馈的情况下进行双人操作。然后,在可以看到 JUNO 反馈的情况下重复这一过程。在足月儿和早产儿人体模型上都进行了类似的操作,总共进行了 8 次操作。在所有模拟过程中,每位参与者都被要求进行 1 分钟的正压通气,每分钟充气 30 次。每个模拟数据都经过了数据清理过程:结果:38 名受试者共进行了 304 次正压通气。共分析了 13 354 次充气。反馈组泄漏率大于 60% 的充气次数明显较少,潮气量过多的比率较低,平均泄漏率较低,平均吸气潮气量较低。在根据技术(单人和双人)进行分析时,"反馈组 "在所有员工中都取得了类似的积极结果。所有工作人员都表示,JUNO 改善了他们的通气技术,并建议将其用于人员培训:结论:无论人体模型类型、工作人员角色和技术(单人或双人)如何,使用 JUNO 都能明显改善面罩通气效果。
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引用次数: 0
Trauma-induced coagulopathy across age pediatric groups: A retrospective cohort study evaluating testing and frequency. 不同年龄儿科群体的创伤诱发凝血病:一项评估测试和频率的回顾性队列研究。
IF 1.7 4区 医学 Q2 ANESTHESIOLOGY Pub Date : 2025-01-01 Epub Date: 2024-10-22 DOI: 10.1111/pan.15024
Shyam J Deshpande, Hamilton C Tsang, Jim Phuong, Rida Hasan, Zhinan Liu, Lynn G Stansbury, John R Hess, Monica S Vavilala

Background: Trauma-induced coagulopathy (TIC) is associated with negative outcomes. Pediatric TIC has been described most often in older children. Children undergo normal developmental hemostasis, but it is unknown how this process impacts the risk of TIC across childhood.

Aims: To understand variations in coagulation testing and TIC across pediatric age groups.

Methods: We evaluated testing patterns of coagulation studies at presentation and over the first 72 h of hospitalization by pediatric age group at a large, Level I trauma center, 2015-2020. The frequency of TIC was determined using published, age-specific reference ranges and controlling for injury severity. We performed subgroup analyses of those with isolated severe traumatic brain injury (TBI) and those who presented directly from the scene of injury.

Results: Data from 2409 pediatric patients were available; 333 patients had isolated severe TBI. Children <1 year were least likely to be tested for TIC at presentation and over the first 72 h, even among the most injured. Fibrinogen testing was uncommon, regardless of injury severity. TIC was common: 22% of patients had TIC at presentation and 35% by 72 h. Greater injury severity was associated with TIC. Children 1-4 and 5-9 years had a higher frequency of TIC at presentation and over 72 h compared to older children in the least injured cohort. We saw no difference in frequency of TIC between age groups in the subset with isolated severe TBI. Using age-specific criteria, patients most often met TIC criteria by INR/PT, followed by platelet count, and least commonly by aPTT. The presence of TIC was associated with in-hospital mortality (OR 4.10, 95% CI 2.06-8.17).

Conclusions: Significant sampling bias exists in clinical data collection among injured children and adolescents. Contrary to previous reports and using age-specific TIC criteria, younger children are not at lower risk of TIC than older children when controlling for injury severity.

背景:创伤诱发凝血病(TIC)与不良后果相关。小儿 TIC 多见于年龄较大的儿童。儿童在发育过程中会经历正常的止血过程,但这一过程如何影响整个儿童期的 TIC 风险尚不清楚:我们评估了一家大型一级创伤中心在 2015-2020 年期间按儿科年龄组别分列的发病时和住院后 72 小时内的凝血检查模式。我们使用已公布的特定年龄参考范围确定了TIC的频率,并对损伤严重程度进行了控制。我们对孤立性严重创伤性脑损伤(TBI)患者和从受伤现场直接送来的患者进行了分组分析:结果:我们获得了 2409 名儿童患者的数据,其中 333 名患者患有孤立性严重创伤性脑损伤。儿童 结论:受伤儿童和青少年的临床数据收集存在严重的抽样偏差。与之前的报告相反,使用特定年龄的 TIC 标准,在控制受伤严重程度的情况下,年龄较小的儿童发生 TIC 的风险并不比年龄较大的儿童低。
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引用次数: 0
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Pediatric Anesthesia
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