儿童塑型性支气管炎的常见病因及致病机制

Common etiology and pathogenesis of plastic bronchitis in children

塑型性支气管炎(PB)是一种少见的呼吸道疾病,其特征性改变为支气管内管型形成及由此造成的支气管阻塞,导致呼吸困难乃至呼吸衰竭。儿童PB的常见病因为下呼吸道感染、发绀型先天性心脏病、支气管哮喘及呼吸道变应性疾病,也可见于镰刀型细胞贫血病、珠蛋白生成障碍性贫血、囊性纤维化等。PB的形成有3个主要机制:气道炎症造成气道内黏液分泌大量增多;气道炎症导致呼吸道上皮坏死脱落、黏膜水肿,呼吸道清理能力下降;气道内淋巴液渗漏。但本病病因多样、发病机制复杂,仍需更多的研究来阐明其发病机制。

Plastic bronchitis(PB)is an uncommon respiratory disease characterized by formation of casts in tracheobronchial tree.It can lead to airway obstruction,difficulty of breathing and even respiratory failure.PB in children is commonly associated with lower airway infection,cyanotic congenital heart disease and asthma or atopic diseases.It can also be found in children with sickle cell anemia,thalassemia and cystic fibrosis and so no.There are three main mechanisms for the formation of casts:airway inflammation results in mucus hypersecretion;inflammatory insults lead to necrosis and abscission of the airway epithelium,mucosal edema,and finally cause airway clearance impairment;leakage of chyle from lung lymphatic circulation into airway.But the etiology of this disease is various,pathogenesis is complex.Further research is required to elucidate the pathogenesis.