Department of Lung Development and Remodelling, Max Planck Institute for Heart and Lung Research, Bad Nauheim, Germany; Pulmonology, Department of Internal Medicine, University of Giessen and Marburg Lung Center, Giessen, Germany.
Front Med (Lausanne). 2015 Dec 23;2:91. doi: 10.3389/fmed.2015.00091. eCollection 2015.
Bronchopulmonary dysplasia (BPD) is a common complication of preterm birth that contributes significantly to morbidity and mortality in neonatal intensive care units. BPD results from life-saving interventions, such as mechanical ventilation and oxygen supplementation used to manage preterm infants with acute respiratory failure, which may be complicated by pulmonary infection. The pathogenic pathways driving BPD are not well-delineated but include disturbances to the coordinated action of gene expression, cell-cell communication, physical forces, and cell interactions with the extracellular matrix (ECM), which together guide normal lung development. Efforts to further delineate these pathways have been assisted by the use of animal models of BPD, which rely on infection, injurious mechanical ventilation, or oxygen supplementation, where histopathological features of BPD can be mimicked. Notable among these are perturbations to ECM structures, namely, the organization of the elastin and collagen networks in the developing lung. Dysregulated collagen deposition and disturbed elastin fiber organization are pathological hallmarks of clinical and experimental BPD. Strides have been made in understanding the disturbances to ECM production in the developing lung, but much still remains to be discovered about how ECM maturation and turnover are dysregulated in aberrantly developing lungs. This review aims to inform the reader about the state-of-the-art concerning the ECM in BPD, to highlight the gaps in our knowledge and current controversies, and to suggest directions for future work in this exciting and complex area of lung development (patho)biology.
支气管肺发育不良（BPD）是早产儿的常见并发症，它极大地导致了新生儿重症监护病房的发病率和死亡率。BPD 是由挽救生命的干预措施引起的，例如机械通气和氧补充，用于治疗患有急性呼吸衰竭的早产儿，这可能会因肺部感染而复杂化。导致 BPD 的发病途径尚未明确，但包括基因表达、细胞间通讯、物理力以及细胞与细胞外基质（ECM）相互作用的协调作用的干扰，这些共同指导正常的肺发育。使用 BPD 的动物模型进一步阐明这些途径的努力得到了协助，这些模型依赖于感染、损伤性机械通气或氧补充，其中 BPD 的组织病理学特征可以被模拟。其中值得注意的是 ECM 结构的扰动，即发育中肺的弹性蛋白和胶原网络的组织。胶原沉积失调和弹性纤维组织紊乱是临床和实验性 BPD 的病理特征。在理解发育中肺 ECM 产生的干扰方面已经取得了进展，但对于 ECM 成熟和周转如何在异常发育的肺中失调，仍有许多需要发现。这篇综述旨在向读者介绍有关 BPD 中 ECM 的最新知识，突出我们知识的差距和当前的争议，并为这一令人兴奋和复杂的肺发育（病理）生物学领域的未来工作提出方向。
