Nathan Nadia, Sileo Chiara, Thouvenin Guillaume, Berdah Laura, Delestrain Céline, Manali Effrosyne, Papiris Spyros, Léger Pierre-Louis, Pointe Hubert Ducou le, l'Hermine Aurore Coulomb, Clement Annick
Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris, France.
Inserm UMR_S933, Sorbonne Université, 75012 Paris, France.
J Clin Med. 2019 Aug 26;8(9):1312. doi: 10.3390/jcm8091312.
Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in specific forms of interstitial lung disease. None of the clinical, radiological, or histological descriptions used for PF diagnosis in adult patients, especially in situations of idiopathic PF, can apply to pediatric situations. This observation supports the view that PF expression may differ with age and, most likely, may cover distinct entities. The present review aims at summarizing the current understanding of PF pathophysiology in children and identifying suitable diagnostic criteria.
肺纤维化（PF）在儿童中是一种非常罕见的病症，可在特定形式的间质性肺疾病中观察到。用于成人患者PF诊断的临床、放射学或组织学描述，尤其是在特发性PF的情况下，均不适用于儿科情况。这一观察结果支持了PF的表现可能随年龄而异，并且很可能涵盖不同实体的观点。本综述旨在总结目前对儿童PF病理生理学的认识并确定合适的诊断标准。
