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Prenatal assessment and prediction in CDH: for what purpose? CDH的产前评估和预测:目的是什么?
IF 2.8 3区 医学 Q1 PEDIATRICS Pub Date : 2025-10-01 DOI: 10.1016/j.siny.2025.101654
Maëlig Abgral , Francesca M. Russo , Jan Deprest , Alexandra Benachi
Congenital diaphragmatic hernia is most often diagnosed prenatally, and accurate assessment is crucial for prognosis parental counselling and therapeutic planning. The assessment of pulmonary hypoplasia is currently the most relevant prognostic indicator. Ultrasound and MRI parameters such as the observed-to-expected lung-to-head ratio and total fetal lung volume serve as the primary predictors of neonatal outcome. However, these markers are not predictive of associated complications like persistent pulmonary hypertension of neonates and cardiac dysfunction, which also significantly influence survival and morbidity. Although new imaging parameters have been proposed, their clinical utility is limited by insufficient validation and inter-center variability. This review critically evaluates current prenatal imaging methods for congenital diaphragmatic hernia, highlighting their strengths and limitations. A better understanding of the underlying pathophysiology, alongside efforts to develop standardized, reproducible and eventually predictive markers, is essential to refine prognosis, optimize fetal therapy selection, eventually improving outcomes for affected neonates.
先天性膈疝最常在产前诊断,准确的评估对预后、父母咨询和治疗计划至关重要。肺发育不全的评估是目前最相关的预后指标。超声和MRI参数,如观察到的与预期的肺头比和胎儿总肺容量是新生儿结局的主要预测因素。然而,这些指标并不能预测新生儿持续性肺动脉高压和心功能障碍等相关并发症,这些并发症也会显著影响生存率和发病率。虽然已经提出了新的影像学参数,但它们的临床应用受到验证不足和中心间变异性的限制。这篇综述批判性地评价了目前先天性膈疝的产前成像方法,突出了它们的优势和局限性。更好地了解潜在的病理生理学,同时努力开发标准化、可重复和最终可预测的标志物,对于改善预后、优化胎儿治疗选择、最终改善受影响新生儿的预后至关重要。
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引用次数: 0
Management of Congenital Diaphragmatic Hernia (CDH) care: Addressing what matters to patients and their families? 先天性膈疝(CDH)护理的管理:解决什么对患者及其家属重要?
IF 2.8 3区 医学 Q1 PEDIATRICS Pub Date : 2025-10-01 DOI: 10.1016/j.siny.2025.101651
K. Saint Denny , J. Lawer , S. Mur , T.M. Prentice
Despite advances in care, Congenital Diaphragmatic Hernia remains a complex and challenging condition to manage and is often associated with long term morbidity. Families are required to navigate considerable uncertainty, challenges in prognostication and communication, and must adapt to an evolving clinical trajectory. This review addresses considerations and practical tips for healthcare professionals to improve the family and patient experience from diagnosis to adult care, from the perspective of clinicians and CDH families. This paper emphasizes the importance of including those with lived experience with CDH in defining topics of priority in research and in clinical and support interventions.
尽管在护理方面取得了进步,先天性膈疝仍然是一个复杂而具有挑战性的疾病,通常与长期发病率有关。家庭需要应对相当大的不确定性,在预测和沟通方面的挑战,并且必须适应不断发展的临床轨迹。本综述从临床医生和CDH家庭的角度,阐述了医疗保健专业人员从诊断到成人护理改善家庭和患者体验的注意事项和实用提示。本文强调了在确定研究和临床及支持干预的优先主题时,包括那些有CDH生活经验的人的重要性。
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引用次数: 0
Developmental pathophysiology and genetic contributions in CDH CDH的发育病理生理和遗传贡献。
IF 2.8 3区 医学 Q1 PEDIATRICS Pub Date : 2025-10-01 DOI: 10.1016/j.siny.2025.101652
Gabriel Guevara , K. Taylor Wild , Richard Keijzer , David J. McCulley
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引用次数: 0
“Gentle” cardio-respiratory management in congenital diaphragmatic hernia: Time for a precision-medicine approach? 先天性膈疝的“温和”心肺管理:是时候采用精准医学方法了?
IF 2.8 3区 医学 Q1 PEDIATRICS Pub Date : 2025-10-01 DOI: 10.1016/j.siny.2025.101660
Anna Foth , David Tingay , Florian Kipfmueller
Congenital diaphragmatic hernia (CDH) remains one of the most challenging conditions to manage in neonatal intensive care, with outcomes determined by the complex interplay between pulmonary hypoplasia, pulmonary vascular remodeling, and ventricular dysfunction. Traditional treatment strategies, including high-frequency ventilation, inhaled nitric oxide, and vasopressor use, fail to account for the dynamic heterogeneity of CDH physiology and may contribute to persistently high mortality. Emerging evidence delineates three principal hemodynamic phenotypes: (1) preserved biventricular function with mild/no pulmonary hypertension (PH), (2) pre-capillary PH with or without right ventricular dysfunction, and (3) post-capillary PH with primary left ventricular (LV) dysfunction. Each phenotype demands distinct ventilatory and pharmacologic strategies, ranging from cautious respiratory support to targeted pulmonary vasodilators, inodilators, or LV-directed therapies. Ventilation in CDH is further complicated by profound lung inhomogeneity, the “baby lung” phenomenon, and susceptibility to ventilator-induced lung injury. Advances in lung-protective strategies, including low driving pressure, permissive hypercapnia, synchrony-enhancing modes, and real-time functional monitoring with echocardiography, lung ultrasound, electrical impedance tomography, and use of circulating biomarkers enable tailored interventions. A precision medicine approach, grounded in multimodal monitoring and gentle cardiopulmonary support, holds promise to optimize hemodynamic balance, reduce iatrogenic injury, and improve survival and long-term outcomes in infants with CDH.
先天性膈疝(CDH)仍然是新生儿重症监护中最具挑战性的疾病之一,其预后由肺发育不全、肺血管重构和心室功能障碍之间复杂的相互作用决定。传统的治疗策略,包括高频通气、吸入一氧化氮和血管加压剂的使用,未能解释CDH生理的动态异质性,并可能导致持续的高死亡率。新出现的证据描述了三种主要的血流动力学表型:(1)保留双心室功能,轻度/无肺动脉高压(PH);(2)毛细血管前PH伴或不伴右室功能障碍;(3)毛细血管后PH伴原发性左室功能障碍。每种表型都需要不同的通气和药理学策略,从谨慎的呼吸支持到靶向肺血管扩张剂、抑制剂或lv导向治疗。CDH的通气因肺部严重不均匀性、“婴儿肺”现象和对呼吸机所致肺损伤的易感性而进一步复杂化。肺保护策略的进步,包括低驱动压、允许性高碳酸血症、同步增强模式、超声心动图实时功能监测、肺超声、电阻抗断层扫描和循环生物标志物的使用,使量身定制的干预成为可能。基于多模式监测和温和心肺支持的精准医学方法有望优化血液动力学平衡,减少医源性损伤,提高CDH婴儿的生存率和长期预后。
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引用次数: 0
Use of human milk and fortification in the neonatal intensive care unit 在新生儿重症监护病房使用母乳和强化。
IF 2.9 3区 医学 Q1 PEDIATRICS Pub Date : 2025-05-13 DOI: 10.1016/j.siny.2025.101632
David H. Adamkin
Human milk is the gold standard for the nutrition of very-low-birthweight (VLBW) infants. Pasteurized donor human milk (DM) should be provided for VLBW infants when own mother's milk (OMM) is not available. Prevention of Necrotizing enterocolitis (NEC) is an important short-term benefit associated with human milk feeding. Human milk alone does not meet the nutritional requirements for VLBW infants leading to nutritional inadequacy, postnatal growthfaltering,and risk of poor neurodevelopmental outcome. Human milk fortification with multicomponent fortifiers increases calories and provides additional protein and minerals and should minimize nutritional deficits. Human milk derived fortifiers (HMDF) provide an exclusive human milk diet but recent evidence to recommend their use over cow milk derived fortifiers is lacking. Individualized fortification using human milk analyzers in real time is an emerging practice that allows for a more personalized provision of nutrient needs.
母乳是极低出生体重(VLBW)婴儿营养的黄金标准。当无法获得母乳(OMM)时,应为VLBW婴儿提供巴氏消毒供体母乳(DM)。预防坏死性小肠结肠炎(NEC)是与母乳喂养相关的一个重要的短期益处。母乳本身不能满足VLBW婴儿的营养需求,导致营养不足、出生后生长迟缓和神经发育不良的风险。用多组分强化剂强化人乳可增加卡路里,提供额外的蛋白质和矿物质,并应尽量减少营养不足。人乳衍生强化剂(HMDF)提供了一种专门的人乳饮食,但最近缺乏证据建议将其用于牛奶衍生强化剂。使用实时人乳分析仪进行个性化强化是一种新兴的做法,它允许更个性化地提供营养需求。
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引用次数: 0
Post-discharge nutrition to optimize preterm infant short- and long-term outcomes 出院后营养优化早产儿短期和长期结局。
IF 2.9 3区 医学 Q1 PEDIATRICS Pub Date : 2025-04-09 DOI: 10.1016/j.siny.2025.101637
Sarah N. Taylor, Catherine O. Buck
The preterm infant misses fetal nutrition, and the nutritional deficit is proportional to the degree of preterm birth with very preterm infants demonstrating the greatest need for supplemental nutrition to overcome deficiencies. Preterm infants’ growth patterns are linked to both their risk for neurodevelopmental difficulties and to the development of obesity. Therefore, establishing healthy growth patterns by providing the best nutrition both in the hospital and post-hospital discharge is critical for long-term health. Despite numerous clinical trials, uncertainty persists as to the duration of nutritional supplement, the degree of benefit of maternal milk feedings, and the best short-term measures of growth and body composition to predict long-term outcomes. As more is learned about optimal post-discharge nutrition, it is very likely that best practice in post-discharge nutrition will include use of a standardized approach to deliver individualized care.
早产儿缺少胎儿营养,营养缺乏与早产的程度成正比,早产儿表现出最需要补充营养来克服不足。早产儿的生长模式与他们神经发育困难的风险和肥胖的发展有关。因此,通过在医院和出院后提供最好的营养来建立健康的生长模式对长期健康至关重要。尽管有大量的临床试验,关于营养补充的持续时间,母乳喂养的益处程度,以及预测长期结果的生长和身体成分的最佳短期指标,仍然存在不确定性。随着对最佳出院后营养的了解越来越多,出院后营养的最佳实践很可能包括使用标准化方法来提供个性化护理。
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引用次数: 0
Gastrointestinal (GI)-lung-brain axis 胃肠道(GI)-肺-脑轴。
IF 2.9 3区 医学 Q1 PEDIATRICS Pub Date : 2025-04-09 DOI: 10.1016/j.siny.2025.101639
Catalina Bazacliu, Juan Carlos Roig, Josef Neu
The GI tract-lung-brain axis refers to the communication network linking the gastrointestinal tract, central nervous system, and respiratory system. This axis is particularly significant in preterm neonates because their immune and nervous systems are undergoing rapid development and thus are susceptible to various conditions influenced by GI tract and lung microbiota that are key mediators in this axis. This communication network is connected via neural, hormonal, and immunological regulatory pathways, all of which are pivotal in disease pathogenesis and health. Here we provide a brief introduction to this axis along with interactive mechanisms and perturbations that can affect this system and the roles they play in health and disease.
胃肠道-肺-脑轴是连接胃肠道、中枢神经系统和呼吸系统的通讯网络。这条轴在早产儿中尤为重要,因为他们的免疫和神经系统正在快速发育,因此容易受到胃肠道和肺部微生物群的影响,而胃肠道和肺部微生物群是这条轴的关键介质。这种通讯网络通过神经、激素和免疫调节途径连接起来,所有这些途径在疾病发病和健康中都是关键的。在这里,我们简要介绍了这个轴,以及影响这个系统的相互作用机制和扰动,以及它们在健康和疾病中所起的作用。
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引用次数: 0
Human milk for preterm infants 早产儿的母乳。
IF 2.9 3区 医学 Q1 PEDIATRICS Pub Date : 2025-04-09 DOI: 10.1016/j.siny.2025.101634
Neena Modi
The term “human milk” conceals important differences between that from an infant's own mother and that obtained from a person or persons who have donated or sold their breast milk. These include differences in nutritional content, and a wide range of non-nutritional components that promote immune, metabolic, and brain development and have evolved over the course of time to transmit biological information from mother to infant. Human milk feeding to preterm babies also encompasses elements such as processing and storage, differences between feeding expressed breast milk versus suckling at the breast, and societal and economic considerations. Current evidence of clinical effectiveness of donated or commercial human milk, and whether macro and micronutrient supplementation are required indicates considerable uncertainty and the possibility of harm. Preterm nutrition is an emotive subject, but important evidence gaps need to be recognised, acknowledged, and addressed if the care of very preterm babies is to improve through a strong evidence-base.
“母乳”一词掩盖了婴儿自己母亲的母乳与捐赠或出售母乳的人的母乳之间的重要区别。这些差异包括营养成分的差异,以及一系列促进免疫、代谢和大脑发育的非营养成分,这些非营养成分随着时间的推移而进化,从而将生物信息从母亲传递给婴儿。母乳喂养早产儿还包括加工和储存、母乳喂养与母乳喂养之间的差异以及社会和经济考虑等因素。目前关于捐赠母乳或商业母乳的临床有效性的证据,以及是否需要补充宏量营养素和微量营养素的证据表明,存在相当大的不确定性和危害的可能性。早产儿营养是一个易受影响的话题,但如果要通过强有力的证据基础改善对极早产儿的护理,就需要认识、承认和解决重要的证据差距。
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引用次数: 0
Donor human milk pasteurization methods and the effect on milk components as they relate to Necrotizing enterocolitis 供体母乳巴氏灭菌方法及其对坏死性小肠结肠炎乳成分的影响。
IF 2.9 3区 医学 Q1 PEDIATRICS Pub Date : 2025-04-09 DOI: 10.1016/j.siny.2025.101638
Laiken Price , Kelly A. Orgel , Misty Good
Necrotizing enterocolitis (NEC) is an intestinal disease that predominantly occurs in preterm infants. While there are no definitive treatment options for NEC, the administration of human milk is protective against the development of NEC in preterm infants. However, human milk composition is highly dynamic, containing numerous bioactive components that can be affected by both maternal and perinatal factors. Furthermore, when maternal milk is unavailable, donor human milk, which goes through a rigorous preparation process including pooling and pasteurization, is used. The different pasteurization methods can have implications for the bioactive components of human milk. In this review, we explore the current literature surrounding the benefits of human milk in the prevention of NEC. We further review the bioactive components and the microbiome of human milk and the many factors that affect the diversity of milk content between human milk samples. Finally, we review the different methods of pasteurization and their effects on the components of human milk.
坏死性小肠结肠炎(NEC)是一种主要发生在早产儿的肠道疾病。虽然对NEC没有明确的治疗方案,但母乳对早产儿NEC的发展有保护作用。然而,人乳成分是高度动态的,含有许多生物活性成分,可能受到母体和围产期因素的影响。此外,在没有母乳的情况下,会使用经过池化和巴氏消毒等严格准备过程的捐赠母乳。不同的巴氏灭菌方法可能对人乳的生物活性成分产生影响。在这篇综述中,我们探讨了目前关于母乳在预防NEC中的益处的文献。我们进一步回顾了人乳的生物活性成分和微生物组,以及影响人乳样品之间乳含量多样性的许多因素。最后,我们回顾了不同的巴氏灭菌方法及其对母乳成分的影响。
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引用次数: 0
Short- and longer-term growth and development of fat mass in preterm infants 早产儿脂肪量的短期和长期生长和发展。
IF 2.9 3区 医学 Q1 PEDIATRICS Pub Date : 2025-04-09 DOI: 10.1016/j.siny.2025.101636
Shipra Jain , Belal N. Alshaikh , Seham Elmrayed , Tanis R. Fenton
Preterm infants typically experience faster growth rates than term-born infants, often doubling their weight in six to eight weeks. However, many face challenges leading to growth faltering and suboptimal neurodevelopment. To achieve optimal growth, these infants often require fortified breastmilk or high-nutrient formula. While meeting nutrition and growth targets are essential, concerns arise about rapid postnatal growth during their catch-up phase, particularly regarding increased body fat at term-corrected age, possibly increasing their risk for obesity and chronic health conditions later. However, evidence suggests that although preterm infants may have higher body fat at term-corrected age, this difference diminishes by three months corrected age, aligning more closely with term-born infants. Systematic reviews of more than 20,000 individuals observed that small for gestational age preterm infants do not have higher adiposity in childhood and adulthood; rather, they exhibit lower body mass indexes, waist circumferences, similar body and visceral fat and blood pressure compared to their appropriate for gestational age preterm-born peers. Therefore, it is reassuring that promoting early growth in preterm infants does not necessitate a trade-off when it comes to supporting long-term metabolic outcomes versus neurodevelopment. Healthcare providers should encourage a responsive feeding approach, even in preterm infants, guided by infants' physiological needs, hunger and satiety once they exhibit feeding cues. This approach respects the child's developmental needs and encourages healthy eating habits, fostering positive parent-child feeding relationships, and ultimately allowing the child to grow and develop to their full potential without compromising their long-term health outcomes.
早产儿的生长速度通常比足月出生的婴儿快,通常在六到八周内体重会增加一倍。然而,许多人面临着导致生长迟缓和神经发育不佳的挑战。为了达到最佳生长,这些婴儿通常需要强化母乳或高营养配方奶粉。虽然达到营养和生长目标至关重要,但人们对婴儿在追赶阶段的产后快速生长感到担忧,特别是在足月矫正年龄时体脂增加,这可能增加他们以后患肥胖症和慢性疾病的风险。然而,有证据表明,尽管早产儿在足月矫正年龄时可能有较高的体脂,但这种差异在3个月矫正年龄时就会减弱,与足月婴儿更接近。对2万多人的系统回顾发现,小于胎龄的早产儿在儿童期和成年期没有更高的肥胖;相反,他们表现出较低的身体质量指数、腰围、相似的身体和内脏脂肪和血压,与同龄的胎龄早产儿相比。因此,当涉及到支持长期代谢结果和神经发育时,促进早产儿的早期生长并不一定需要权衡,这是令人放心的。医疗保健提供者应鼓励反应性喂养方法,即使是早产儿,根据婴儿的生理需求,饥饿和饱腹感,一旦他们表现出喂养线索的指导。这种方法尊重儿童的发展需要,鼓励健康的饮食习惯,培养积极的亲子喂养关系,最终使儿童在不损害其长期健康结果的情况下成长和发展,充分发挥其潜力。
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引用次数: 0
期刊
Seminars in Fetal & Neonatal Medicine
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