支气管肺发育不良（BPD）是早产儿最常见、最严重的并发症之一。重度BPD（sBPD）指早产儿吸入氧气治疗至少28天,在矫正胎龄36周时需吸入氧浓度>30%或应用正压通气。sBPD可引起患儿后期死亡、心肺功能不全、生长不良、认知落后和运动发育迟缓等。随着我国极早产儿救治成活率的提高,sBPD的发病率逐渐上升。由于不同患儿病理生理机制不一,引起疾病的表型存在较大差异,给临床诊治带来相当大的挑战。近年国内外诊疗共识均提出,以多学科团队合作对sBPD患儿进行综合管理。本文主要介绍中-重度肺实质性病变、大气道疾病和肺动脉高压三种表型的发生机制、临床表现,以及呼吸支持策略、气管造口术和肺动脉高压的管理。新生儿科医师加强对sBPD的关键病理生理和表型特征的认识,建立多学科团队会诊机制,采取有针对性的治疗策略,有望进一步提高sBPD患儿的存活率,改善其预后。

Bronchopulmonary dysplasia (BPD) is one of the commonest and most serious complications in premature infants. Severe BPD (sBPD) is defined as premature infants receiving oxygen inhalation for at least 28 days and requiring oxygen concentration >30% or positive pressure ventilation at 36 weeks of corrected gestational age. Infants with sBPD may cause longterm death, cardiopulmonary dysfunction, failure to thrive, impaired cognitive development and motor retardation. With the improvement of survival rate of extremely premature infants in China, the incidence of sBPD has increased gradually. Due to the different pathophysiological mechanisms of different children, the phenotypes of diseases are greatly different, which brings considerable challenges to clinical diagnosis and treatment. In recent years, the diagnosis and treatment consensus at home and abroad has put forward the comprehensive management of the infants with sBPD through multidisciplinary team cooperation. In this review, the mechanism and clinical features of three disease components were introduced, including moderate-severe parenchymal disease, large airway disease and pulmonary hypertension. Respiratory support strategy, tracheostomy and drug treatment of pulmonary hypertension were also described. Neonatologists are expected to further improve the survival rate and prognosis of sBPD patients by strengthening the understanding of key pathophysiological and phenotypic characteristics of sBPD, establishing multidisciplinary team consultation mechanism and adopting targeted treatment strategies.