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Detection of Pseudomonas aeruginosa in cystic fibrosis after initiation of CFTR modulators: a systematic review. CFTR调节剂启动后囊性纤维化中铜绿假单胞菌的检测:一项系统综述。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2026-01-28 DOI: 10.1016/j.prrv.2026.01.004
Vito Terlizzi, Edoardo Timitilli, Maria Sole Fiumara, Daniela Dolce, Silvia Campana, Elena Chiappini

Pseudomonas aeruginosa remains a major cause of morbidity in people with cystic fibrosis (pwCF). The advent of elexacaftor/tezacaftor/ivacaftor (ETI) has transformed clinical outcomes, but its impact on airway microbiology and diagnostic surveillance remains uncertain. We conducted a systematic review of PubMed and Embase (2019-2025) according to PRISMA guidelines, including studies comparing culture with molecular methods for P. aeruginosa detection in pwCF treated with ETI. Fourteen observational studies (n=2525 subjects) were identified. Chronic P. aeruginosa detection declined from 51.4% at baseline to 24.8% after ETI, with nearly half of colonized subjects achieving apparent clearance. Several studies applying molecular techniques reported persistent P. aeruginosa detection despite negative cultures. These findings indicate that ETI markedly reduces bacterial burden and sputum availability, which may limit the sensitivity of culture-based surveillance. However, the clinical and inflammatory significance of PCR+/culture- results remains uncertain. Combining molecular and culture-based surveillance may help guide infection monitoring in the modulator era.

铜绿假单胞菌仍然是囊性纤维化(pwCF)患者发病的主要原因。elexaftor /tezacaftor/ivacaftor (ETI)的出现改变了临床结果,但其对气道微生物学和诊断监测的影响仍不确定。我们根据PRISMA指南对PubMed和Embase(2019-2025)进行了系统综述,包括比较培养方法和分子方法在ETI治疗的pwCF中检测P. aeruginosa的研究。14项观察性研究(n=2525名受试者)被确定。慢性铜绿假单胞菌检出率从基线时的51.4%下降到ETI后的24.8%,近一半的定植受试者获得了明显的清除。几项应用分子技术的研究报告了铜绿假单胞菌的持续检测,尽管培养阴性。这些发现表明,ETI显著降低了细菌负担和痰的可用性,这可能限制了基于培养的监测的敏感性。然而,PCR+/culture-结果的临床和炎症意义仍不确定。结合分子和基于培养的监测可能有助于指导调节剂时代的感染监测。
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引用次数: 0
Reframing and clarifying melatonin use in paediatric sleep care. 重新定义和澄清褪黑激素在儿科睡眠护理中的使用。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2026-01-21 DOI: 10.1016/j.prrv.2026.01.003
Mon Ohn, Dominic A Fitzgerald

Behavioural sleep problems are common in children. The challenges with establishing and maintaining good sleep hygiene may prove challenging to overcome based on environmental issues such as the liberal availability of electronic media from a young age, permissive parenting, difficult family dynamics and limited parental/carer understanding of a child's sleep needs as they progress through childhood and adolescence. Melatonin is inexpensive, easily accessible, in high demand from the community and, at its simplest, may be perceived as a harmless "silver bullet" for "sleep problems". Clinicians are encountering children already using melatonin, sometimes with minimal behavioural support and misconceptions about its safety. This article provides frontline clinicians with an evidence-based overview of melatonin's role in paediatric care, emphasising its approved indications, the dangers of unregulated products and the risks of unsupervised use.

行为性睡眠问题在儿童中很常见。建立和保持良好睡眠卫生的挑战可能是难以克服的,这是基于环境问题的,比如从小就自由使用电子媒体,纵容的父母,困难的家庭动态以及父母/照顾者对孩子在童年和青少年时期的睡眠需求的有限理解。褪黑素价格低廉,容易获得,社会需求旺盛,简单来说,可能被视为治疗“睡眠问题”的无害“银弹”。临床医生遇到了一些已经在使用褪黑激素的儿童,有时只有很少的行为支持,而且对其安全性存在误解。本文为一线临床医生提供了褪黑素在儿科护理中的作用的基于证据的概述,强调其批准的适应症,不受监管产品的危险和无监管使用的风险。
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引用次数: 0
Update on paediatric lung transplantation: an overview of a challenging therapeutic. 儿科肺移植的最新进展:一项具有挑战性的治疗概述。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2026-01-11 DOI: 10.1016/j.prrv.2026.01.002
Charlotte Roy

Paediatric lung transplantation is a rare but increasingly successful therapeutic option for children with end-stage respiratory failure. Over the past decade, its epidemiology has shifted, with cystic fibrosis becoming an uncommon indication and a growing proportion of candidates presenting with interstitial lung disease or pulmonary arterial hypertension. In parallel, advances in donor allocation, the development of size-reduction surgical techniques and the reconditioning of marginal lungs have expanded the donor pool and reduced waiting-list mortality, including in small children. Peri-operative improvements-including broader use of extracorporeal life support as a bridge to transplantation-have further strengthened early and long-term outcomes. At the same time, progress in infectious disease prophylaxis and antiviral therapy, coupled with a more refined understanding of rejection mechanisms, is reshaping post-transplant care. Together, these developments underscore the importance of early referral to specialised paediatric transplant centres to ensure timely assessment and optimal access to advanced supportive strategies.

小儿肺移植是一种罕见但越来越成功的治疗选择终末期呼吸衰竭的儿童。在过去的十年中,其流行病学发生了变化,囊性纤维化成为一种不常见的适应症,越来越多的候选人以间质性肺疾病或肺动脉高压为表现。与此同时,供体分配的进步、缩小手术技术的发展和边缘肺的修复扩大了供体池,降低了等待名单上的死亡率,包括儿童。围手术期的改善——包括更广泛地使用体外生命支持作为移植的桥梁——进一步加强了早期和长期的结果。与此同时,传染病预防和抗病毒治疗的进展,加上对排斥机制的更精确理解,正在重塑移植后的护理。总之,这些发展强调了早期转诊到专门的儿科移植中心的重要性,以确保及时评估和最佳地获得先进的支持策略。
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引用次数: 0
Diagnostic applications of neonatologist-performed lung ultrasound in the delivery room in late preterm and term neonates: A systematic review of literature. 新生儿肺超声在产房晚期早产儿和足月新生儿诊断中的应用:文献系统综述。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2026-01-10 DOI: 10.1016/j.prrv.2026.01.001
Melina Winkler, Nariae Baik-Schneditz, Viktoria Gruber, Gerhard Pichler, Bernhard Schwaberger

Lung ultrasound (LUS) is increasingly used in the delivery room to assess respiratory transition and guide early respiratory management. The objective of this systematic review was to evaluate current evidence on the diagnostic applications of neonatologist-performed LUS during neonatal resuscitation and immediate postnatal stabilization in late preterm and term neonates. Twelve studies were included in this qualitative systematic review, addressing physiological LUS patterns after birth, the use of LUS scoring systems to predict the requirement of respiratory support, and applications of LUS during neonatal resuscitation and immediate postnatal stabilization. Normal postnatal adaptation was characterized by rapid progression from fluid-rich to aerated lung patterns, whereas persistent high-risk LUS patterns were associated with respiratory morbidity. Several LUS scoring systems demonstrated good discriminative ability for predicting the need for respiratory support shortly after birth. In addition, characteristic ultrasound patterns supported early identification of late preterm and term neonates at risk for surfactant requirement and mechanical ventilation. Despite substantial heterogeneity in study design, timing of imaging, and analytic methods, the evidence supports the feasibility and clinical value of LUS in the delivery room. Neonatologist-performed LUS represents a promising bedside tool that may complement clinical assessment during early postnatal transition in late preterm and term neonates.

肺超声(LUS)越来越多地用于产房评估呼吸过渡和指导早期呼吸管理。本系统综述的目的是评估目前在新生儿复苏和晚期早产儿和足月新生儿即刻产后稳定中由新生儿内科医生执行的LUS诊断应用的证据。本定性系统综述纳入了12项研究,涉及出生后生理LUS模式,使用LUS评分系统预测呼吸支持需求,以及LUS在新生儿复苏和产后立即稳定中的应用。正常的出生后适应的特点是从富液肺迅速发展为通气肺,而持续的高风险LUS模式与呼吸系统疾病有关。几个LUS评分系统在预测出生后不久是否需要呼吸支持方面表现出良好的判别能力。此外,特征性超声模式支持早期识别晚期早产儿和足月新生儿在表面活性剂需求和机械通气方面的风险。尽管在研究设计、成像时间和分析方法上存在很大的异质性,但证据支持LUS在产房的可行性和临床价值。新生儿学家执行的LUS代表了一个有前途的床边工具,可以补充后期早产儿和足月新生儿早期产后过渡的临床评估。
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引用次数: 0
Bubble CPAP in neonatal care: mechanisms, evidence, and pathways to optimization. 气泡CPAP在新生儿护理:机制,证据和途径优化。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2025-12-27 DOI: 10.1016/j.prrv.2025.12.004
Amanda Gross, Emily R Campbell, Lidiaine Oliveira, Guilherme Sant'Anna, Wissam Shalish

Bubble continuous positive airway pressure (CPAP) is a widely used non-invasive respiratory support strategy for neonates and is commonly employed in the management of preterm infants with respiratory distress. Bubble CPAP generates continuous distending pressure by submerging the distal expiratory limb in water, producing low-amplitude, high-frequency pressure oscillations as airflow escapes and bubbles into the water canister. These oscillations, which are transmitted back to the neonate, vary dynamically with airway and lung mechanics and may contribute to the physiological benefits observed with bubble CPAP. This review summarizes the historical context, physiological rationale, clinical evidence supporting bubble CPAP, and areas for future optimization.

气泡持续气道正压通气(Bubble continuous positive airway pressure, CPAP)是一种广泛应用于新生儿的无创呼吸支持策略,常用于早产儿呼吸窘迫的治疗。气泡CPAP通过将呼气远端肢体浸入水中产生持续的扩张压力,当气流逸出并形成气泡进入水罐时,产生低振幅的高频压力振荡。这些振荡随着气道和肺力学的变化而动态变化,并可能有助于气泡CPAP观察到的生理益处。本文综述了气泡CPAP的历史背景、生理原理、支持气泡CPAP的临床证据以及未来优化的领域。
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引用次数: 0
Risk factors for obstructive sleep apnoea in obese children: a systematic review and meta-analysis. 肥胖儿童阻塞性睡眠呼吸暂停的危险因素:系统回顾和荟萃分析。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2025-12-26 DOI: 10.1016/j.prrv.2025.12.003
Catharina Tao, Mary Fortune, Emma A Webb, Theofilos Polychronakis

Background: Obstructive sleep apnoea (OSA) is prevalent among children with obesity and is associated with long-term morbidity.

Methods: We conducted a systematic review and meta-analysis of risk factors for OSA among children (0-18) who are overweight/obese. Risk factors reviewed included overall adiposity, regional fat distribution, adenotonsillar hypertrophy, sex, ethnicity, and airway neuromuscular tone. PubMed and Embase were searched in July 2025. Random-effects models were applied.

Results: Forty-seven studies were included. Children with OSA had higher BMI z-scores (mean difference (MD) = 0.30), neck circumference (MD = 2.19 cm), and waist circumference (MD = 5.99 cm) compared to obese children without OSA. Adenotonsillar hypertrophy and male sex associated with higher OSA odds (adenoid odds ratio (OR) = 3.28, tonsil OR = 4.16, Male OR = 1.51). Studies also suggested airway neuromuscular tone and non-Caucasian ethnicity as risk factors, but further research is needed.

Conclusion: OSA in obese children is likely to be multifactorial. Assessment accounting for multiple factors may improve screening accuracy and guide targeted interventions in this high-risk population.

背景:阻塞性睡眠呼吸暂停(OSA)在肥胖儿童中普遍存在,并与长期发病率相关。方法:我们对超重/肥胖儿童(0-18岁)的OSA危险因素进行了系统回顾和荟萃分析。危险因素包括整体肥胖、区域脂肪分布、腺扁桃体肥大、性别、种族和气道神经肌肉张力。PubMed和Embase于2025年7月被检索。采用随机效应模型。结果:纳入47项研究。与无OSA的肥胖儿童相比,OSA患儿的BMI z-score(平均差值(MD) = 0.30)、颈围(MD = 2.19 cm)和腰围(MD = 5.99 cm)均较高。腺扁桃体肥大和男性与较高的OSA风险相关(腺样体风险比(OR) = 3.28,扁桃体风险比= 4.16,男性风险比= 1.51)。研究还表明气道神经肌肉张力和非白种人种族是危险因素,但需要进一步的研究。结论:肥胖儿童的阻塞性睡眠呼吸暂停可能是多因素的。考虑多种因素的评估可以提高筛查的准确性,并指导对这一高危人群进行有针对性的干预。
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引用次数: 0
Lung trajectories into adulthood of preterm born survivors and the influence of sex. 早产幸存者进入成年期的肺部轨迹及性别的影响。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2025-12-24 DOI: 10.1016/j.prrv.2025.12.001
Dinh Son Bui, Cassidy Du Berry, Nur Sabrina Idrose, Jeanie L Y Cheong, Shyamali Dharmage

Preterm birth is increasingly recognised as a major determinant of lifelong respiratory health. Longitudinal studies consistently demonstrate that individuals born preterm have persistently worse lung function from childhood to adulthood compared with their term born peers. These adverse effects vary across gestational categories including extremely preterm, very preterm and moderate-late preterm birth, and are further exacerbated by bronchopulmonary dysplasia (BPD). Pooled analyses show that children or adults born preterm without BPD had 7.2% [95 confidence interval (CI): 5.6-8.7%] lower percent predicted FEV1 than term-born individuals. The %FEV1 deficit was greater in those with BPD: 18.9% [16.7-21.1%] lower for the BPD36 group when compared with the term born group. Lung function deficits in pre-term born survivors persist into adult life. Preterm birth is also associated with increased risk of COPD in later life. There is evidence that male sex confers additional vulnerability in pre-term survivors, with recent literature showing poorer lung function in males compared with females. Understanding these relationships is central to developing future phenotyping frameworks, enabling early identification of those at greatest risk, and informing surveillance strategies aimed at preserving lung function and preventing early onset of chronic obstructive pulmonary disease.

早产越来越被认为是影响终生呼吸系统健康的一个主要因素。纵向研究一致表明,与足月出生的同龄人相比,早产儿从童年到成年的肺功能一直较差。这些不良反应因妊娠类别而异,包括极早产儿、极早产儿和中晚期早产儿,并因支气管肺发育不良(BPD)而进一步加剧。汇总分析显示,与足月出生的个体相比,无BPD的早产儿或成人的FEV1预测值低7.2%[95可信区间(CI): 5.6-8.7%]。与足月出生组相比,BPD36组FEV1的%缺陷更大:比足月出生组低18.9%[16.7-21.1%]。早产儿幸存者的肺功能缺陷会持续到成年。早产也与晚年患慢性阻塞性肺病的风险增加有关。有证据表明,男性会给早产幸存者带来额外的脆弱性,最近的文献显示,男性的肺功能比女性差。了解这些关系对于制定未来的表型框架至关重要,能够早期识别风险最大的人群,并为旨在保护肺功能和预防慢性阻塞性肺疾病早期发病的监测策略提供信息。
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引用次数: 0
Home intravenous antibiotics for cystic fibrosis - setting up a hospital @home service. 家庭静脉注射抗生素治疗囊性纤维化——建立医院@家庭服务。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2025-12-18 DOI: 10.1016/j.prrv.2025.12.002
Nicola Collins, Caroline Devon, Siân Bentley, Emma Dixon, Delyth Jones, Suzie Kenny, Sukeshi Makhecha, Sarah Moledina, Nicola Murray, Michèle Puckey, Chloe Worger, Ian M Balfour-Lynn

This paper reviews the use of home intravenous antibiotics (IVABs) in children with cystic fibrosis (CF). We outline a program we developed during the COVID-19 pandemic for enhancing the experience for children and families by involving full multidisciplinary follow up via video for the duration of the antibiotic course. We did find though, that the majority of families were unsuitable for home IVABs. We hope that this information will be useful for other CF units considering setting up a hospital at home service.

本文综述了家庭静脉注射抗生素(IVABs)在儿童囊性纤维化(CF)中的应用。我们概述了我们在2019冠状病毒病大流行期间制定的一项计划,该计划通过在抗生素疗程期间通过视频进行全面的多学科随访,以增强儿童和家庭的体验。但我们确实发现,大多数家庭不适合进行家庭ivab。我们希望这一信息将对其他CF单位考虑建立家庭医院服务有所帮助。
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引用次数: 0
Cardiovascular complications in children with Down syndrome and sleep disordered breathing. 唐氏综合征儿童心血管并发症与睡眠呼吸障碍
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2025-11-28 DOI: 10.1016/j.prrv.2025.11.008
Wiktoria Gocal, Katarina Zeder, Bradley A Maron, Amal Isaiah

Study objectives: Children with Down syndrome (DS) often present with craniofacial and neuromuscular features that increase the risk of sleep-disordered breathing (SDB), which may lead to cardiovascular morbidity. We conducted a scoping review to profile the current evidence base describing cardiovascular complications in children with DS and SDB. Findings from this work are expected to identify knowledge gaps that could inform future research and clinical care.

Methods: We performed a systematic scoping review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR). Comprehensive searches of Embase, Scopus, and MEDLINE were conducted, and eligible studies included children aged 1-18 with DS and SDB reporting cardiovascular outcomes.

Results: Seven studies involving 1,437 participants were included. Across various study designs, children with DS and SDB showed blunted autonomic responses to apneic events, impaired nocturnal dipping of heart rate and blood pressure, increased hypoxic burden even with mild OSA severity, and early signs of left ventricular diastolic dysfunction and bi-ventricular remodeling.

Conclusions: SDB and DS are linked to a constellation of clinical signs consistent with cardiopulmonary end-organ dysfunction and elevated clinical risk. Ongoing cardiac monitoring and use of physiologic measures beyond the apnea-hypopnea index are necessary. Addressing persistent upper airway obstruction with multimodal treatments, including surgery, positive airway pressure, and new therapies like hypoglossal nerve stimulation, may be crucial to lowering long-term cardiovascular risk in this vulnerable group.

研究目的:唐氏综合征(DS)患儿通常表现为颅面和神经肌肉特征,这些特征增加了睡眠呼吸障碍(SDB)的风险,这可能导致心血管疾病。我们进行了一项范围综述,概述了目前描述DS和SDB儿童心血管并发症的证据基础。这项工作的发现有望确定知识差距,为未来的研究和临床护理提供信息。方法:我们按照系统评价的首选报告项目和范围评价的元分析扩展(PRISMA-ScR)进行了系统的范围评价。对Embase、Scopus和MEDLINE进行了综合检索,符合条件的研究包括1-18岁患有DS和SDB的报告心血管结局的儿童。结果:纳入了7项研究,涉及1437名参与者。在各种研究设计中,患有DS和SDB的儿童对呼吸暂停事件的自主神经反应减弱,夜间心率和血压下降受损,即使轻度OSA严重程度也会增加缺氧负担,并且出现左室舒张功能障碍和双室重构的早期迹象。结论:SDB和DS与一系列与心肺终末器官功能障碍和临床风险升高相一致的临床体征有关。持续的心脏监测和使用超出呼吸暂停低通气指数的生理测量是必要的。通过多模式治疗解决持续性上气道阻塞,包括手术、气道正压通气和舌下神经刺激等新疗法,可能对降低这一弱势群体的长期心血管风险至关重要。
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引用次数: 0
Asthma in children and young people with sickle cell disease: a diagnostic dilemma. 患有镰状细胞病的儿童和年轻人的哮喘:诊断困境。
IF 4 3区 医学 Q1 PEDIATRICS Pub Date : 2025-11-25 DOI: 10.1016/j.prrv.2025.11.001
Zoe Bothamley, Michele Arigliani, Latika Gupta, Katharine Harman, Anne Greenough, Atul Gupta

Asthma is a common complication in children and young people with sickle cell disease (CYP-SCD), contributing to increased morbidity and mortality. However, diagnosis is challenging due to overlapping SCD-related symptoms and pulmonary complications. This narrative review evaluates the utility of objective measures (spirometry, fractional exhaled nitric oxide -FeNO-, and blood eosinophil count -BEC-) in asthma diagnosis in CYP-SCD. Systematic search identified ten studies comparing asthmatic and non-asthmatic CYP-SCD and data were extracted and critically appraised. Spirometry trended towards lower values and increased obstructive patterns in asthmatic CYP-SCD. Although data interpretation was limited due to inhaled corticosteroid use, likely influencing the inability of FeNO and BEC to differentiate asthma status, alongside SCD-specific pathophysiology. Standardised diagnostic criteria, consistent use of reference ranges and further research into alternative lung function tests and asthma phenotyping are essential.

哮喘是患有镰状细胞病(CYP-SCD)的儿童和年轻人的常见并发症,导致发病率和死亡率增加。然而,由于重叠的scd相关症状和肺部并发症,诊断具有挑战性。这篇叙述性综述评价了客观测量(肺活量测定、呼气一氧化氮分数- feno -和血嗜酸性粒细胞计数- bec -)在慢性阻塞性肺病哮喘诊断中的应用。系统检索确定了10项比较哮喘性和非哮喘性CYP-SCD的研究,并提取数据并进行严格评价。哮喘型cypscd的肺量测定趋向于较低的数值和增加的阻塞性模式。虽然由于吸入皮质类固醇的使用,数据解释受到限制,但可能影响了FeNO和BEC无法区分哮喘状态,以及scd特异性病理生理。标准化的诊断标准、参考范围的一致使用以及对替代肺功能测试和哮喘表型的进一步研究至关重要。
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引用次数: 0
期刊
Paediatric Respiratory Reviews
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