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Published on: November 4, 2015
Aberrant Pulmonary Vascular Growth and Remodeling in Bronchopulmonary Dysplasia
1Department of Pediatrics, Division of Critical Care Medicine, Stanford University School of Medicine , Stanford, CA , USA.
Insights
Bronchopulmonary dysplasia (BPD) is a chronic lung disease in premature infants caused by arrested alveolarization. Impaired pulmonary angiogenesis and vascular remodeling contribute to BPD pathogenesis and pulmonary hypertension.
Area of Science:
- Neonatal Medicine
- Pulmonary Biology
- Developmental Biology
Background:
- Lung development, particularly alveolarization, continues postnatally, making the lung vulnerable to injury.
- Premature birth increases susceptibility to lung injury, often leading to bronchopulmonary dysplasia (BPD).
- BPD is characterized by arrested alveolarization and significant pulmonary vascular abnormalities.
Purpose of the Study:
- To review normal lung development and the pathological features of arrested development in BPD.
- To emphasize pulmonary vascular abnormalities in BPD pathogenesis.
- To summarize key pathways regulating pulmonary vascular development and their alterations in BPD.
Main Methods:
- Review of existing literature on lung development and BPD.
- Analysis of experimental and clinical evidence regarding pulmonary vascular abnormalities.
- Focus on key signaling pathways involved in vascular development.
Main Results:
- Postnatal alveolarization is crucial for lung development and susceptible to disruption.
- Impaired pulmonary angiogenesis is a key contributor to BPD pathogenesis.
- BPD involves vascular remodeling and tone alterations, increasing risks of hypoxemia and pulmonary hypertension.
Conclusions:
- Understanding normal lung vascular development is critical for elucidating BPD.
- Pulmonary vascular abnormalities are central to BPD pathology.
- Targeting key vascular pathways may offer therapeutic strategies for BPD.
Abstract:
In contrast to many other organs, a significant portion of lung development occurs after birth during alveolarization, thus rendering the lung highly susceptible to injuries that may disrupt this developmental process. Premature birth heightens this susceptibility, with many premature infants developing the chronic lung disease, bronchopulmonary dysplasia (BPD), a disease characterized by arrested alveolarization. Over the past decade, tremendous progress has been made in the elucidation of mechanisms that promote postnatal lung development, including extensive data suggesting that impaired pulmonary angiogenesis contributes to the pathogenesis of BPD. Moreover, in addition to impaired vascular growth, patients with BPD also frequently demonstrate alterations in pulmonary vascular remodeling and tone, increasing the risk for persistent hypoxemia and the development of pulmonary hypertension. In this review, an overview of normal lung development will be presented, and the pathologic features of arrested development observed in BPD will be described, with a specific emphasis on the pulmonary vascular abnormalities. Key pathways that promote normal pulmonary vascular development will be reviewed, and the experimental and clinical evidence demonstrating alterations of these essential pathways in BPD summarized.
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