Boni Alessandra, Cristiani Luca, Majo Fabio, Ullmann Nicola, Esposito Marianna, Supino Maria Chiara, Tomà Paolo, Villani Alberto, Musolino Anna Maria, Cutrera Renato
Pneumology and Cystic Fibrosis Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
Academic Department of Pediatrics, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
Children (Basel). 2024 Jul 30;11(8):917. doi: 10.3390/children11080917.
Cystic fibrosis (CF) is a multisystem disorder characterized by progressive respiratory deterioration, significantly impacting both quality of life and survival. Over the years, lung ultrasound (LUS) has emerged as a promising tool in pediatric respiratory due to its safety profile and ease at the bedside. In the era of highly effective CF modulator therapies and improved life expectancy, the use of non-ionizing radiation techniques could become an integral part of CF management, particularly in the pediatric population. The present review explores the potential role of LUS in CF management based on available data, analyzing all publications from January 2015 to January 2024, focusing on two key areas: LUS in CF pulmonary exacerbation and its utility in routine clinical management. Nonetheless, LUS exhibits a robust correlation with computed tomography (CT) scans and serves as an additional, user-friendly imaging modality in CF management, demonstrating high specificity and sensitivity in identification, especially in consolidations and atelectasis in the CF population. Due to its ability, LUS could be an instrument to monitor exacerbations with consolidations and to establish therapy duration and monitor atelectasis over time or their evolution after therapeutic bronchoalveolar lavage. On the basis of our analysis, sufficient data emerged showing a good correlation between LUS score and respiratory function tests. Good sensitivity and specificity of the methodology have been found in rare CF pulmonary complications such as effusion and pneumothorax. Regarding its use in follow-up management, the literature reports a moderate correlation between LUS scores and the type, extent, and CT severity score of bronchiectasis. A future validation of ultrasound scores specifically in CF patients could improve the use of LUS to identify pulmonary exacerbations and monitor disease progression. However, further research is needed to comprehensively establish the role of LUS in the CF population, particularly in elucidating its broader utility and long-term impact on patient care.
囊性纤维化(CF)是一种多系统疾病,其特征为进行性呼吸功能恶化,对生活质量和生存率均有显著影响。多年来,肺部超声(LUS)因其安全性及床旁操作的便捷性,已成为儿科呼吸领域一种很有前景的工具。在高效CF调节剂疗法和预期寿命延长的时代,使用非电离辐射技术可能会成为CF管理的一个重要组成部分,尤其是在儿科人群中。本综述基于现有数据探讨LUS在CF管理中的潜在作用,分析了2015年1月至2024年1月期间的所有出版物,重点关注两个关键领域:LUS在CF肺部加重期的应用及其在常规临床管理中的效用。尽管如此,LUS与计算机断层扫描(CT)扫描显示出很强的相关性,并且在CF管理中作为一种额外的、用户友好的成像方式,在识别方面表现出高特异性和敏感性,尤其是在CF人群的实变和肺不张方面。由于其功能,LUS可以作为一种监测合并实变的加重期、确定治疗持续时间以及随时间监测肺不张或治疗性支气管肺泡灌洗后其演变的工具。基于我们的分析,出现了足够的数据表明LUS评分与呼吸功能测试之间具有良好的相关性。在罕见的CF肺部并发症如胸腔积液和气胸方面,该方法具有良好的敏感性和特异性。关于其在随访管理中的应用,文献报道LUS评分与支气管扩张的类型、范围和CT严重程度评分之间存在中度相关性。未来专门针对CF患者的超声评分验证可能会改善LUS在识别肺部加重期和监测疾病进展方面的应用。然而,需要进一步研究以全面确立LUS在CF人群中的作用,特别是阐明其更广泛的效用及其对患者护理的长期影响。