pediatrics

儿科学
  • 文章类型: Journal Article
    目的:由于现有的科学文献有限以及与治疗相关的高风险,婴儿和非常年幼的儿童(小于3至5岁)的高级别神经胶质瘤构成了重大挑战。本研究旨在调查它们的特点,治疗,和结果。
    方法:一项队列研究在儿童肿瘤医院进行,埃及。病例包括年龄<5岁的儿童,确诊为中枢神经系统高级别胶质瘤。基线临床和放射学特征,除了潜在的预后因素进行了评估。
    结果:总计,确定了76例,其中7人<1岁。在所有病例中,有32.9%实现了大致或几乎全部切除(GTR/NTR)。在测试的案例中,仅在5名受试者中存在H3K27M-改变。所有病例的3年OS和EFS分别为26.9%和15.4%,分别。切除程度是最重要的预后因素,与未达到GTR/NTR的人相比,达到GTR/NTR的人的生存率增加了一倍以上(p=0.05)。年龄对EFS有“双峰”效应,与1至3岁的人相比,年龄较小和较高的年龄组。与非中线肿瘤相比,中线肿瘤患者的生存率较差(1年EFS=18.5%vs35%,分别,p=0.02)。
    结论:这项在婴儿和非常年幼的儿童中进行的HGG大型队列研究提供了对特征和治疗挑战的见解。切除程度,年龄组,和肿瘤定位是重要的预后因素。有必要进行更大样本量的进一步研究,以完善治疗方法并改善结果。
    OBJECTIVE: High-grade gliomas in infants and very young children (less than 3 to 5 years old) pose significant challenges due to the limited scientific literature available and high risks associated with treatments. This study aims to investigate their characteristics, treatment, and outcomes.
    METHODS: A cohort study was conducted at Children\'s Cancer Hospital, Egypt. Cases included children aged < 5 years old with confirmed CNS high-grade glioma. Baseline clinical and radiological characteristics, besides potential prognostic factors were assessed.
    RESULTS: In total, 76 cases were identified, 7 of them were < 1 year old. Gross- or near-total resection (GTR/NTR) was achieved in 32.9% of all cases. Of the tested cases, H3K27M-alteration was present in 5 subjects only. The 3-year OS and EFS for all cases were 26.9% and 15.4%, respectively. Extent of resection was the most important prognostic factor, as those achieving GTR/NTR experienced more than double the survival compared to those who do not (p = 0.05). Age had a \"bimodal\" effect on EFS, with those aged 1 to 3 years old faring better than younger and older age groups. Subjects with midline tumors had worse survival compared to non-midline tumors (1-year EFS = 18.5% vs 35%, respectively, p = 0.02).
    CONCLUSIONS: This study in a large cohort of HGG in infants and very young children offers insights into the characteristics and treatment challenges. Extent of resection, age group, and tumor localization are important prognostic factors. Further research with larger sample size is warranted to refine treatment approaches and improve outcomes.
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  • 文章类型: Journal Article
    目的:为了稳定儿童和青少年1型糖尿病(T1D)患者的社会经济状况与血糖控制之间的关系,治疗依从性和糖尿病生活质量(DQoL)。
    横截面,我们进行了连续纳入的观察性研究.参与者年龄在8-18岁,T1D持续时间>1年。有关家庭结构的数据,家庭收入,对父母的教育水平和父母在初级糖尿病护理监督中的作用进行了登记。分析粘附性(DMQ-Sp)和DQoL(PedsQl)。针对人口统计进行调整的线性和逻辑回归模型,应用家庭结构和父母对初级糖尿病护理责任的作用。
    结果:共包括323例患者(T1D持续时间5,3±3,3年;HbA1c7,7±1,0%;年龄13,3±2,8岁;49,8%女性)。生活在核心家庭中的患者和父母双方共同接受主要糖尿病护理监督的患者显示HbA1c较低[根据人口统计学和家庭结构进行调整(7,06;CI95%6,52-7,59);根据人口统计学和对初级糖尿病护理监督的作用进行调整(7,43;CI95%6,57-8,28)]。父母共同接受糖尿病护理监督的患者的DMQ-Sp评分(根据人口统计学和主要监督角色进行调整)较高(84,56;CI95%73,93-95,19)。共享糖尿病护理监督的父母显示出明显更高的PedsQl得分(均为74,63±12,70,而母亲为68,53±14,59;p=0,001)。
    结论:患有T1D的儿童和青少年HbA1c较低,当生活在一个核心家庭中时,更好的治疗依从性和更好的DQoL,具有较高的社会经济地位和监督糖尿病护理的责任由父母双方共同承担.
    OBJECTIVE: To stablish the relationship between socioeconomic status of a cohort of children and adolescents with type 1 diabetes (T1D) with glycemic control, therapeutic adherence and diabetes quality of life (DQoL).
    UNASSIGNED: A cross-sectional, observational study with consecutive inclusion was carried out. Participants aged 8-18 years with T1D duration >1 year. Data on family structure, family income, parents\' educational level and parental role on primary diabetes care supervision were registered. Adherence (DMQ-Sp) and DQoL (PedsQl) were analyzed. Linear and logistic regression models adjusted for demographics, family structure and parental role on primary diabetes care responsibility were applied.
    RESULTS: A total of 323 patients (T1D duration 5,3 ± 3,3 years; HbA1c 7,7 ± 1,0%; age 13,3 ± 2,8 years; 49,8% females) were included. Patients living in a nuclear family and those whose main diabetes care supervision was shared by both parents showed lower HbA1c [adjusted for demographics and family structure (7,06; CI 95% 6,52-7,59); adjusted for demographics and role on primary diabetes care supervision (7,43; CI 95% 6,57-8,28)]. DMQ-Sp score (adjusted for demographics and role on main supervision) was higher in patients whose parents shared the diabetes care supervision (84,56; CI 95% 73,93-95,19). Parents sharing diabetes care supervision showed a significantly higher PedsQl score (both 74,63 ± 12,70 vs mother 68,53 ± 14,59; p = 0,001).
    CONCLUSIONS: Children and adolescents with T1D had lower HbA1c, better therapeutic adherence and better DQoL when lived in a nuclear family, with higher socioeconomic status and the responsibility for supervising diabetes care was shared by both parents.
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  • 文章类型: Journal Article
    背景:对筛查和干预患者社会风险因素的临床计划的兴趣正在增长,包括住房,食物,和交通。尽管一些研究表明这些项目可以对健康产生积极影响,很少有人研究这些影响的潜在机制。本研究探讨了识别和干预社会风险可以影响家庭健康的途径。
    方法:这项定性研究被嵌入一项随机临床试验中,该试验检查了参与社会服务导航计划对健康的影响。我们对27名参与导航计划的儿科患者的英语或西班牙语护理人员进行了半结构化访谈。访谈采用主题分析法进行分析。
    结果:看护者描述了导航计划影响整体儿童和/或看护者健康的3条途径:1)增加家庭对社会服务的了解和获得;2)帮助家庭与医疗保健服务联系;3)提供情感支持,以减少看护者的孤立和焦虑。参与者建议,即使导航程序不直接影响资源访问,也可以影响健康。
    结论:社会护理计划可能通过多种潜在途径影响健康。计划的影响似乎取决于计划在多大程度上增加了对社会和医疗保健服务的了解和获得,并支持家庭与计划人员之间的积极关系。
    BACKGROUND: Interest is growing in clinic-based programs that screen for and intervene on patients\' social risk factors, including housing, food, and transportation. Though several studies suggest these programs can positively impact health, few examine the mechanisms underlying these effects. This study explores pathways through which identifying and intervening on social risks can impact families\' health.
    METHODS: This qualitative study was embedded in a randomized clinical trial that examined the health impacts of participation in a social services navigation program. We conducted semi-structured interviews with 27 English or Spanish-speaking caregivers of pediatric patients who had participated in the navigation program. Interviews were analyzed using thematic analysis.
    RESULTS: Caregivers described 3 pathways through which the navigation program affected overall child and/or caregiver health: 1) increasing families\' knowledge of and access to social services; 2) helping families connect with health care services; and 3) providing emotional support that reduced caregiver isolation and anxiety. Participants suggested that navigation programs can influence health even when they do not directly impact resource access.
    CONCLUSIONS: Social care programs may impact health through multiple potential pathways. Program impacts seem to be mediated by the extent to which programs increase knowledge of and access to social and health care services and support positive relationships between families and program personnel.
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  • 文章类型: Journal Article
    背景:哮喘是伊利诺伊州儿童中普遍存在的健康问题,管理受社会决定因素的影响很大。17个州通过了库存吸入器法律,但实施差异很大。
    目的:评估在IL学校哮喘护理中实施和解决库存吸入器方案可持续性的关键障碍。
    方法:对伊利诺伊州高哮喘负担学区进行了半结构化访谈,以评估实施库存吸入器政策和由此产生的计划的障碍。进行了主题分析,使用Atlas。ti识别和编码“威胁”对未来的可持续性。综合了数据,并提交给利益攸关方,以缓解障碍。创建了概述支持可持续性的步骤的示意性流程图。
    结果:与伊利诺伊州八个学区的主要社区合作伙伴进行了18次访谈,代表农村,城市,和郊区。分析揭示了25个障碍,有几个被确定为对未来可持续性的“威胁”,包括责任问题,后续护理保证,资金/资源,药房配药实践,地区层面的变革准备,和护士人员配备。利益相关者组成了一个全州联盟来解决这些障碍,提高认识,计划评估,并就国家资金分配提供建议。开发了适合学校行政需求的国家库存吸入器工具包,以支持可持续性努力。
    结论:战略利益相关者和社区参与对于建立和维持符合政策要求的库存吸入器计划至关重要。许多地区面临着在没有关键障碍缓解和支持的情况下启动和维护此类计划的挑战。协作解决方案是必要的,以确保有效的基于学校的哮喘管理和减轻持续的小儿哮喘健康差异。
    BACKGROUND: Asthma is a prevalent health concern among Illinois children and management is significantly influenced by social determinants. Seventeen states have adopted stock inhaler laws, but implementation varies widely.
    OBJECTIVE: To assess critical barriers to implementation and address sustainability of stock inhaler programming in school-based asthma care in IL.
    METHODS: Semi-structured interviews were conducted with high asthma burden school districts in IL to assess barriers in implementing stock inhaler policies and resultant programming. Thematic analysis was performed, using Atlas.ti to identify and code \"threats\" to future sustainability. Data was synthesized and presented to stakeholders for barrier mitigation. A schematic flow chart outlining steps to support sustainability was created.
    RESULTS: Eighteen interviews were conducted with key community partners across eight Illinois school districts, representing rural, urban, and suburban areas. Analysis revealed 25 barriers, with several identified as \"threats\" to future sustainability, including liability concerns, follow-up care assurance, funding/resources, pharmacy dispensing practices, district-level readiness to change, and nurse staffing. Stakeholders formed a statewide coalition to address these barriers, increase awareness, plan evaluations, and advise on state funding allocation. A national stock inhaler toolkit tailored to school administrative needs was developed to support sustainability efforts.
    CONCLUSIONS: Strategic stakeholder and community engagement are vital for establishing and sustaining stock inhaler programs that adhere to policy mandates. Many districts face challenges initiating and maintaining such programs without critical barrier mitigation and support. Collaborative solutions are necessary to ensure effective school-based asthma management and mitigate persistent pediatric asthma health disparities.
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  • 文章类型: Journal Article
    Chronic kidney disease (CKD) has severe consequences on the quality and expectancy of life and is considered a major health problem worldwide. This is, especially relevant in pediatric patients, as they have unique characteristics and a mortality rate 30 times higher (in advanced stages) than healthy people. This review aims to define the minimum components for the diagnostic approach and monitoring of CKD in the pediatric population from primary health care to promote comprehensive care and adequate risk management. For this purpose, we performed a systematic review of the literature with a panel of experts. Based on the evidence, to optimize the definition, diagnosis, and timely treatment of CKD in the pediatric population, we formulated 21 recommendations. These were approved by the research team and peer-reviewed by clinical experts. They will facilitate the definition of the diagnostic approach for CKD in the pediatric population in primary health-care settings, allowing for timely treatment intervention, comprehensive care, and monitoring of this disease.
    La enfermedad renal crónica (ERC) tiene graves consecuencias en la calidad y la esperanza de vida, y se considera un importante problema de salud a nivel mundial. Esto es especialmente relevante en pacientes pediátricos, ya que presenta características únicas y una tasa de mortalidad en etapas avanzadas que es 30 veces mayor que en personas sanas. El objetivo de esta revisión fue definir los componentes mínimos para el abordaje diagnóstico y para el seguimiento de la ERC en la población pediátrica desde la atención primaria en salud, con el fin de promover la atención integral y una adecuada gestión del riesgo. Para esto, se realizó una revisión sistemática de la literatura con panel de discusión de expertos. Basándonos en la evidencia, y con el objetivo de optimizar la definición, diagnóstico y tratamiento oportuno de la ERC en la población pediátrica, se formularon 21 recomendaciones. Estas fueron aprobadas por el equipo desarrollador y los pares expertos clínicos evaluadores, y permitirán definir de manera oportuna el abordaje diagnóstico de la ERC en la población pediátrica desde la atención primaria en salud, facilitando la intervención temprana, una atención integral y el seguimiento de esta patología.
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  • 文章类型: Journal Article
    BACKGROUND: Central line-associated bloodstream infections (CLABSIs) are among the most epidemiologically relevant health care-associated infections. The aseptic non-touch technique (ANTT) is a standardized practice used to prevent CLABSIs. In a pediatric hospital, the overall CLABSI rate was 1.92/1000 catheter days (CD). However, in one unit, the rate was 5.7/1000 CD.
    METHODS: Nurses were trained in ANTT. For the implementation, plan-do-study-act (PDSA) cycles were completed. Adherence monitoring of the ANTT and epidemiological surveillance were performed.
    RESULTS: ANTT adherence of 95% was achieved after 6 PDSA cycles. Hand hygiene and general cleaning reached 100% adherence. Port disinfection and material collection had the lowest adherence rates, with 76.2% and 84.7%, respectively. The CLABSI rate decreased from 5.7 to 1.26/1000 CD.
    CONCLUSIONS: The implementation of ANTT helped reduce the CLABSI rate. Training and continuous monitoring are key to maintaining ANTT adherence.
    UNASSIGNED: Las infecciones relacionadas con catéteres venosos centrales son unas de las infecciones asociadas a la atención de salud con mayor relevancia epidemiológica. La técnica aséptica «no tocar» es una práctica estandarizada que se utiliza para prevenir estas infecciones. En un hospital pediátrico, la tasa de infecciones relacionadas con catéteres venosos centrales fue de 1.92/1000 días de catéter. Sin embargo, en una de las unidades la tasa fue de 5.7/1000 días de catéter.
    UNASSIGNED: Se capacitaron enfermeras en la técnica aséptica «no tocar». Para la implementación se cumplieron ciclos de planificar-hacer-estudiar-actuar (PHEA). Se realizaron seguimiento de la adherencia a la técnica y vigilancia epidemiológica.
    RESULTS: Se logró una adherencia a la técnica aséptica «no tocar» del 95% después de seis ciclos. La higiene de manos y la limpieza general alcanzaron un 100% de cumplimiento. La desinfección de los puertos y la recolección de material alcanzaron la menor adherencia, con un 76.2% y un 84.7%, respectivamente. La tasa de infecciones relacionadas con catéteres venosos centrales disminuyó de 5.7 a 1.26 por 1000 días de catéter.
    CONCLUSIONS: La implementación de la técnica aséptica «no tocar» ayudó en la reducción de infecciones relacionadas con catéteres venosos centrales. La capacitación y el seguimiento continuo son clave para mantener el cumplimiento de la técnica.
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  • 文章类型: Journal Article
    BACKGROUND: The worldwide prevalence of arterial hypertension in pediatric patients is 3.5%, and it has repercussions at renal, cardiovascular, neurological, and lifestyle levels. This study aimed to estimate the prevalence of arterial hypertension, mortality, and follow-up in patients with acute renal failure in the nephrology outpatient clinic at a second-level hospital in Northwestern Mexico.
    METHODS: We conducted a descriptive, retrospective, and observational study. Men and women aged 1-18 years diagnosed with acute kidney injury were analyzed from January 1, 2012, to December 31, 2021. The medical and electronic records of the candidate patients were analyzed, and nutritional data, laboratory analysis, most frequent etiology, and follow-up in the pediatric nephrology clinic were collected. Those with exacerbated chronic kidney disease and previous diagnosis of high blood pressure were excluded.
    RESULTS: One hundred and seventy-four patients were evaluated, and only 40 were eligible for the study (22.98%), predominantly males with a mean age of 9.9 years. The degree of arterial hypertension was 50% for grade I and 50% for grade II (p = 0.007); the mortality rate was 32%. One hundred percent of hypertension cases were controlled at 6 months after discharge (p = 0.000080).
    CONCLUSIONS: Our results were similar to those reported in other studies. Follow-up and early detection of arterial hypertension in children need to be strengthened.
    UNASSIGNED: La prevalencia de hipertensión arterial a nivel mundial es 3.5% en los pacientes pediátricos y tiene repercusiones tanto a nivel renal, cardiovascular, neurológico y estilo de vida. El objetivo de este estudio fue estimar la prevalencia de hipertensión arterial en pacientes con insuficiencia renal aguda, estimar la mortalidad y el seguimiento de los pacientes en la consulta externa de nefrología en un hospital de segundo nivel en el Noroeste de México.
    UNASSIGNED: Estudio observacional descriptivo, retrospectivo. Se analizaron hombres y mujeres entre 1 a 18 años de edad con el diagnóstico de lesión renal aguda, entre 1 de enero del 2012 hasta 31 de diciembre del 2021. Se analizaron las historias clínicas y el expediente electrónico de los pacientes candidatos, se recolectaron datos nutricionales, análisis de laboratorio, etiología más frecuente y el seguimiento en la consulta de nefrología pediátrica. Se excluyeron aquellos con enfermedad renal crónica agudizada y diagnóstico previo de hipertensión arterial.
    RESULTS: 174 pacientes fueron evaluados y solamente 40 fueron candidatos al estudio (22.98%), de los cuales predominaron masculinos con una edad media de 9.9 años. El grado de hipertensión arterial fue 50% para grado I y 50% para grado II (p = 0.007); tasa de mortalidad 32%. El 100% del control de la hipertensión se logró en el seguimiento del egreso de los pacientes en 6 meses (p = 0.000080).
    CONCLUSIONS: Nuestros resultados fueron similares a los reportados en otros estudios. Se debe reforzar el seguimiento y detección oportuna de hipertensión arterial en los niños.
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  • 文章类型: Journal Article
    在过去的几年中,具有种系易感性的血液肿瘤越来越被认为是一种独特的肿瘤。因此,该类别已添加到世界卫生组织(WHO)第4版,并保留在WHO第5版和国际共识分类(ICC)2022分类系统中。在实践中,这些肿瘤需要高度怀疑和通过分子检测确认。流式细胞术是一种经济有效的诊断工具,通常对外周血和骨髓样本进行。在这次审查中,我们试图总结目前与流式细胞术免疫表型相关的研究,以评估其在生殖系血液肿瘤诊断和临床决策中的效用。我们还使用主要来自我们自己机构的案例来说明这些发现。我们回顾了一些更常见的突变基因,包括CEBPA,DDX41,RUNX1,ANKRD26,GATA2,范可尼贫血,努南综合征,和唐氏综合症。我们强调,流式细胞术可能在某些种系易感性综合征的诊断(GATA2,唐氏综合征)和筛查(CEBPA)中发挥作用。尽管在其他方面似乎显示出非特异性发现(DDX41,RUNX1)。在其他许多人中,如ANKRD26,范可尼贫血,和努南综合征,需要进一步的研究来更好地了解是否观察到特定的流式细胞术模式.最终,我们得出的结论是,在大多数种系设置中,需要进一步的研究,例如大型病例系列和有组织的数据管道,以更好地了解这些肿瘤的流式细胞术免疫表型.
    Hematologic neoplasms with germline predisposition have been increasingly recognized as a distinct category of tumors over the last few years. As such, this category was added to the World Health Organization (WHO) 4th edition as well as maintained in the WHO 5th edition and International Consensus Classification (ICC) 2022 classification systems. In practice, these tumors require a high index of suspicion and confirmation by molecular testing. Flow cytometry is a cost-effective diagnostic tool that is routinely performed on peripheral blood and bone marrow samples. In this review, we sought to summarize the current body of research correlating flow cytometric immunophenotype to assess its utility in diagnosis of and clinical decision making in germline hematologic neoplasms. We also illustrate these findings using cases mostly from our own institution. We review some of the more commonly mutated genes, including CEBPA, DDX41, RUNX1, ANKRD26, GATA2, Fanconi anemia, Noonan syndrome, and Down syndrome. We highlight that flow cytometry may have a role in the diagnosis (GATA2, Down syndrome) and screening (CEBPA) of some germline predisposition syndromes, although appears to show nonspecific findings in others (DDX41, RUNX1). In many of the others, such as ANKRD26, Fanconi anemia, and Noonan syndrome, further studies are needed to better understand whether specific flow cytometric patterns are observed. Ultimately, we conclude that further studies such as large case series and organized data pipelines are needed in most germline settings to better understand the flow cytometric immunophenotype of these neoplasms.
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  • 文章类型: Journal Article
    小儿肺栓塞(PE)罕见且可能危及生命。尽管成人PE越来越多地使用溶栓和血栓切除术,儿科治疗和结局的趋势仍未完全描述.
    本研究的目的是描述PE的发生率,单用抗凝治疗的病例比例,全身溶栓,和定向治疗(局部溶栓和血栓切除术),临床结果,和总成本。
    使用儿科健康信息系统数据库中的管理数据进行了一项多中心观察性研究,以研究2015年至2021年在美国儿科医院治疗的PE。使用多变量广义线性混合效应模型评估治疗结果。
    在3,136个科目中,70%的人至少12岁,46%为男性。62%的人至少有1种合并症,任何类型的先天性心脏病都是最普遍的(20%)。88%的受试者单独接受抗凝治疗,7%接受全身溶栓,5%接受定向治疗。总体住院死亡率为7.5%。治疗方法没有随时间变化(P=0.98)。在针对患者特征进行调整后,定向治疗与较低的死亡风险相关(调整后的百分比-3%,[95%CI:-5%至0%])比单独抗凝。全身溶栓与住院总费用增加相关(增加113,043美元[95%CI:62,866美元,163,219美元])。住院时间没有因治疗而异。
    患有PE的儿科患者具有较高的潜在慢性疾病发病率。单独的抗凝治疗仍然是主要的治疗手段,很少使用溶栓和血栓切除术。鉴于小儿PE的相对罕见性,需要创新研究设计的额外研究是至关重要的。
    UNASSIGNED: Pediatric pulmonary embolism (PE) is rare and potentially life-threatening. Though thrombolysis and thrombectomy are increasingly used in adult PE, trends in pediatric treatment and outcomes remain incompletely described.
    UNASSIGNED: The purpose of this study was to describe the incidence of PE, proportion of cases treated with anticoagulation alone, systemic thrombolysis, and directed therapy (local thrombolysis and thrombectomy), clinical outcomes, and total costs.
    UNASSIGNED: A multicenter observational study was performed using administrative data from the Pediatric Health Information System database to study PE treated at U.S. pediatric hospitals from 2015 to 2021. Outcomes by treatment were evaluated using multivariable generalized linear mixed effects models.
    UNASSIGNED: Of 3,136 subjects, 70% were at least 12 years of age, and 46% were male. Sixty-two percent had at least 1 comorbidity, and congenital heart disease of any kind was the most prevalent (20%). Eighty-eight percent of subjects received anticoagulation alone, 7% received systemic thrombolysis, and 5% received directed therapy. Overall in-hospital mortality was 7.5%. Treatment approach did not change over time (P = 0.98). After adjusting for patient characteristics, directed therapy was associated with a lower risk of mortality (adjusted percentage -3%, [95% CI: -5% to 0%]) than anticoagulation alone. Systemic thrombolysis was associated with a greater total cost of hospitalization ($113,043 greater [95% CI: $62,866, $163,219]). Length of hospital stay did not differ by treatment.
    UNASSIGNED: Pediatric patients with PE have a high incidence of underlying chronic disease. Anticoagulation alone remains the mainstay of treatment, with thrombolysis and thrombectomy rarely being used. Given the relative rarity of pediatric PE, additional research requiring innovative study designs is paramount.
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