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首页> 外文期刊>Pulmonary Circulation >The Robyn Barst Memorial Lecture: Differences between the Fetal, Newborn, and Adult Pulmonary Circulations: Relevance for Age-Specific Therapies (2013 Grover Conference Series):
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The Robyn Barst Memorial Lecture: Differences between the Fetal, Newborn, and Adult Pulmonary Circulations: Relevance for Age-Specific Therapies (2013 Grover Conference Series):

机译：罗宾·巴斯特（Robyn Barst）纪念讲座：胎儿，新生儿和成人肺循环之间的差异：与特定年龄疗法的相关性（2013年格罗弗会议系列）：

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Pulmonary arterial hypertension (PAH) contributes to poor outcomes in diverse diseases in newborns, infants, and children. Many aspects of pediatric PAH parallel the pathophysiology and disease courses observed in adult patients; however, critical maturational differences exist that contribute to distinct outcomes and therapeutic responses in children. In comparison with adult PAH, disruption of lung vascular growth and development, or angiogenesis, plays an especially prominent role in the pathobiology of pediatric PAH. In children, abnormalities of lung vascular development have consequences well beyond the adverse hemodynamic effects of PAH alone. The developing endothelium also plays critical roles in development of the distal airspace, establishing lung surface area for gas exchange and maintenance of lung structure throughout postnatal life through angiocrine signaling. Impaired functional and structural adaptations of the pulmonary circulation during the transition from fetal to postnatal life contribute significantly to poor outcomes in such disorders as persistent pulmonary hypertension of the newborn, congenital diaphragmatic hernia, bronchopulmonary dysplasia, Down syndrome, and forms of congenital heart disease. In addition, several studies support the hypothesis that early perinatal events that alter lung vascular growth or function may set the stage for increased susceptibility to PAH in adult patients (“fetal programming”). Thus, insights into basic mechanisms underlying unique features of the developing pulmonary circulation, especially as related to preservation of endothelial survival and function, may provide unique therapeutic windows and distinct strategies to improve short- and long-term outcomes of children with PAH.
机译：肺动脉高压（PAH）导致新生儿，婴儿和儿童各种疾病的不良结局。儿科PAH的许多方面与成年患者观察到的病理生理和疾病进程相似。然而，存在严重的成熟差异，这些差异导致儿童的不同结局和治疗反应。与成人PAH相比，肺血管生长和发育或血管生成的破坏在儿科PAH的病理生物学中起着特别重要的作用。对于儿童，肺血管发育异常所带来的后果远远超出了单独使用PAH的不良血液动力学影响。发育中的内皮在远端空域的发展中也起着关键作用，通过血管分泌信号传递，为整个产后生命建立气体交换和维持肺结构的肺表面积。从胎儿到产后过渡期间肺循环的功能和结构适应性受损，会严重影响新生儿持续性肺动脉高压，先天性diaphragm肌疝，支气管肺发育不良，唐氏综合症和先天性心脏病等疾病的预后。此外，一些研究支持以下假说，即围产期早期事件改变了肺血管的生长或功能，可能为成年患者增加对PAH的敏感性奠定了基础（“胎儿程序设计”）。因此，对发展中的肺循环独特特征的基本机制的见解，尤其是与内皮生存和功能的保存有关的基础机制，可能会提供独特的治疗窗口和独特的策略，以改善PAH儿童的短期和长期结果。

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