Abstract:
In children, pulmonary fibrosis (PF) is an extremely unusual entity that can be observed in some types of interstitial lung disease (ILD). Defining whether ILD is accompanied by PF is important for targeted therapy. Algorithm for the diagnosis of PF in children is not clearly established. Besides, the clinical, radiological, and histological definitions commonly used to diagnose particularly the cases of idiopathic PF in adult patients, is not applicable to pediatric cases. However, a few studies conducted in children offer good exemplary diagnostic approach to fibrosing ILD. Thorax high resonance computed tomography and/or lung biopsy scanning can provide valuable information about PF. Another issue that has not been clearly established is when to start antifibrotic treatment in pediatric patients with PF. The objective of this current review is to provide a comprehensive overview of pediatric PF by drawing upon adult research, particularly focusing on the areas of uncertainty.
摘要:
在儿童中,肺纤维化(PF)是一种非常不寻常的实体,可以在某些类型的间质性肺病(ILD)中观察到。定义ILD是否伴有PF对于靶向治疗很重要。儿童PF的诊断算法尚未明确建立。此外,临床,放射学,和组织学定义通常用于诊断,特别是成人患者的特发性PF病例,不适用于儿科病例。然而,在儿童中进行的一些研究为纤维化ILD提供了良好的示范性诊断方法.胸部高共振计算机断层扫描和/或肺活检扫描可以提供有关PF的有价值的信息。尚未明确确定的另一个问题是何时开始对患有PF的儿科患者进行抗纤维化治疗。本综述的目的是通过利用成人研究提供小儿PF的全面概述。特别是关注不确定的领域。