先天性呼吸系统疾病诊治研究进展

Advancement of diagnosis and therapy of congenital lung malfomartion

先天性呼吸系统疾病是一组罕见的累及肺、气管支气管树和/或肺血管的疾病，可在任何年龄阶段出现症状，在临床表现、严重性、预后等方面有很大异质性，容易被误诊、漏诊，且目前治疗方案的选择仍存在较大争议。现就其发病机制、病理、影像学、临床表现、鉴别诊断、治疗及预后等方面研究进展进行综述，并重点阐述成人期发病者的特点。

Congenital lung malformations are a group of anomalies involving the lungs and tracheobronchial tree,vascular abnormalities,or both entities.They can manifest at any age and vary widely in clinical manifestation,severity and prognosis.Misdiagnosis is common and the management of these lesions remains controversial.This article reviews the pathogenesis,pathology,clinical and radiological manifestation,differential diagnosis,management and prognosis of these disorders,especially in adults.