重度支气管肺发育不良的表型特征和治疗策略

doi:10.12372/jcp.2022.22e0605

摘要/Abstract

摘要：

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。

关键词: 支气管肺发育不良, 肺动脉高压, 大气道疾病, 机械通气, 气管造口术

Abstract:

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.

Key words: bronchopulmonary dysplasia, pulmonary hypertension, large airway disease, mechanical ventilation, tracheostomy

夏红萍, 张拥军. 重度支气管肺发育不良的表型特征和治疗策略[J]. 临床儿科杂志, 2022, 40(6): 401-406.

XIA Hongping, ZHANG Yongjun. Phenotypic characteristics and treatment strategies of severe bronchopulmonary dysplasia[J]. Journal of Clinical Pediatrics, 2022, 40(6): 401-406.

参考文献 27

[1]	Jobe AH. Mechanisms of Lung Injury and Bronchopulmonary Dysplasia[J]. Am J Perinatol, 2016, 33(11): 1076-1078. doi: 10.1055/s-0036-1586107
[2]	Higgins RD, Jobe AH, Koso-Thomas M, et al. Bronchopulmonary Dysplasia: Executive Summary of a Workshop[J]. J Pediatr, 2018, 197: 300-308. doi: 10.1016/j.jpeds.2018.01.043
[3]	Ehrenkranz RA, Walsh MC, Vohr BR, et al. Validation of the National Institutes of Health consensus definition of bronchopulmonary dysplasia[J]. Pediatrics, 2005, 116(6): 1353-1360. pmid: 16322158
[4]	Cao Y, Jiang S, Sun J, et al. Assessment of Neonatal Intensive Care Unit Practices, Morbidity, and Mortality Among Very Preterm Infants in China[J]. JAMA Netw Open, 2021, 4(8): e2118904. doi: 10.1001/jamanetworkopen.2021.18904
[5]	Martin RJ, Di Fiore JM, Macfarlane PM, et al. Physiologic basis for intermittent hypoxic episodes in preterm infants[J]. Adv Exp Med Biol, 2012, 758: 351-358. doi: 10.1007/978-94-007-4584-1_47 pmid: 23080182
[6]	van Mastrigt E, Logie K, Ciet P, et al. Lung CT imaging in patients with bronchopulmonary dysplasia: A systematic review[J]. Pediatr Pulmonol, 2016, 51(9): 975-986. doi: 10.1002/ppul.23446
[7]	Ochiai M, Hikino S, Yabuuchi H, et al. A new scoring system for computed tomography of the chest for assessing the clinical status of bronchopulmonary dysplasia[J]. J Pediatr, 2008, 152(1):90-95. doi: 10.1016/j.jpeds.2007.05.043
[8]	Semple T, Akhtar MR, Owens CM. Imaging Bronchopulmonary Dysplasia-A Multimodality Update[J]. Front Med (Lausanne), 2017, 4: 88.
[9]	Allen J, Zwerdling R, Ehrenkranz R, et al. Statement on the care of the child with chronic lung disease of infancy and childhood[J]. Am J Respir Crit Care Med, 2003, 168(3): 356-396. doi: 10.1164/rccm.168.3.356
[10]	Hysinger E, Friedman N, Jensen E, et al. Bronchoscopy in neonates with severe bronchopulmonary dysplasia in the NICU[J]. J Perinatol, 2019, 39(2): 263-268. doi: 10.1038/s41372-018-0280-y pmid: 30518799
[11]	Wu KY, Jensen EA, White AM, et al. Characterization of Disease Phenotype in Very Preterm Infants with Severe Bronchopulmonary Dysplasia[J]. Am J Respir Crit Care Med, 2020, 201(11): 1398-1406. doi: 10.1164/rccm.201907-1342OC
[12]	Stoll BJ, Hansen NI, Bell EF, et al. Neonatal outcomes of extremely preterm infants from the NICHD Neonatal Research Network[J]. Pediatrics, 2010, 126(3): 443-456. doi: 10.1542/peds.2009-2959
[13]	Mourani PM, Sontag MK, Younoszai A, et al. Early pulmonary vascular disease in preterm infants at risk for bronchopulmonary dysplasia[J]. Am J Respir Crit Care Med, 2015, 191(1): 87-95. doi: 10.1164/rccm.201409-1594OC
[14]	Khemani E, Mcelhinney DB, Rhein L, et al. Pulmonary artery hypertension in formerly premature infants with bronchopulmonary dysplasia: clinical features and outcomes in the surfactant era[J]. Pediatrics, 2007, 120(6): 1260-1269. doi: 10.1542/peds.2007-0971 pmid: 18055675
[15]	Krishnan U, Feinstein JA, Adatia I, et al. Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia[J]. J Pediatr, 2017, 188: 24-34. doi: 10.1016/j.jpeds.2017.05.029
[16]	中华医学会儿科学分会新生儿学组, 中华儿科杂志编辑委员会. 早产儿支气管肺发育不良临床管理专家共识[J]. 中华儿科杂志, 2020, 58(5): 358-365.
[17]	Heching HJ, Turner M, Farkouh-Karoleski C, et al. Pulmonary vein stenosis and necrotising enterocolitis: is there a possible link with necrotising enterocolitis?[J]. Arch Dis Child Fetal Neonatal Ed, 2014, 99(4): F282-F285.
[18]	Zhou L, Xiang X, Wang L, et al. N-terminal pro-B-type natriuretic peptide as a biomarker of bronchopulmonary dysplasia or death in preterm infants: a retrospective cohort analysis[J]. Front Pediatr, 2019, 7: 166. doi: 10.3389/fped.2019.00166 pmid: 31134166
[19]	Abman SH, Collaco JM, Shepherd EG, et al. Interdisciplinary care of children with severe bronchopulmonary dysplasia[J]. J Pediatr, 2017, 181: 12-28. doi: 10.1016/j.jpeds.2016.10.082
[20]	Sun Y, Zhang H. Ventilation strategies in transition from neonatal respiratory distress to chronic lung disease[J]. Semin Fetal Neonatal Med, 2019, 24(5): 101035. doi: 10.1016/j.siny.2019.101035
[21]	Demauro SB, D'Agostino JA, Bann C, et al. Developmental outcomes of very preterm infants with tracheostomies[J]. J Pediatr, 2014, 164(6): 1303-1310. PMID: 24472229. doi: 10.1016/j.jpeds.2013.12.014
[22]	Upadhyay K, Vallarino DA, Talati AJ. Outcomes of neonates with tracheostomy secondary to bronchopulmonary dysplasia[J]. BMC Pediatr, 2020, 20(1): 414. doi: 10.1186/s12887-020-02324-1
[23]	Morrow CB, Mcgrath-Morrow SA, Collaco JM. Predictors of length of stay for initial hospitalization in infants with bronchopulmonary dysplasia[J]. J Perinatol, 2018, 38(9): 1258-1265. doi: 10.1038/s41372-018-0142-7
[24]	Overman AE, Liu M, Kurachek SC, et al. Tracheostomy for infants requiring prolonged mechanical ventilation: 10 years' experience[J]. Pediatrics, 2013, 131(5): e1491-e1496.
[25]	Baker CD, Abman SH, Mourani PM. Pulmonary Hypertension in Preterm Infants with Bronchopulmonary Dysplasia[J]. Pediatr Allergy Immunol Pulmonol, 2014, 27(1): 8-16. doi: 10.1089/ped.2013.0323
[26]	Kwon HW, Kim HS, An HS, et al. Long-Term Outcomes of Pulmonary Hypertension in Preterm Infants with Bronchopulmonary Dysplasia[J]. Neonatology, 2016, 110(3): 181-189. doi: 10.1159/000445476 pmid: 27172918
[27]	Shepherd EG, Knupp AM, Welty SE, et al. An interdisciplinary bronchopulmonary dysplasia program is associated with improved neurodevelopmental outcomes and fewer rehospitalizations[J]. J Perinatol, 2012, 32(1): 33-38. doi: 10.1038/jp.2011.45 pmid: 21546943