Recent progress in understanding pediatric pulmonary hypertension.
Recent progress in understanding pediatric pulmonary hypertension.
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了解小儿肺动脉高压的最新进展。
DOI：
10.1097/mop.0b013e3283464a52
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发表时间：
2011-06
影响因子：
3.6
通讯作者：
Ivy DD
中科院分区：
文献类型：
--
作者：
Abman SH;Ivy DD
肺动脉高压（PAH）在儿童心脏、肺、血液等多种疾病的发病率和死亡率中起着重要的作用。在过去的几十年里，肺血管生物学的进步显著扩展了治疗策略；然而，关于我们对儿童多环芳烃的理解，仍然存在许多独特的问题。最近关于儿童多环芳烃的研究突出了我们对儿童多环芳烃相关疾病与成人多环芳烃相关疾病的理解差距，强调了对儿童多环芳烃发病机制和治疗的独特方面进行专门研究的强烈需求。登记处已经开始提供新的数据，显示成人和儿童形式的多环芳烃在生理、病程和遗传学上的差异。不幸的是，儿童肺动脉高压的治疗策略往往局限于儿童的小型观察性研究，并且依赖于大型成人研究的结果。此外，婴儿和儿童研究和护理的临床终点仍然不明确。尽管取得了许多进展，但多环芳烃儿童的长期预后仍不确定，实质性的挑战仍然存在，特别是在了解严重多环芳烃的机制和方法方面。未来的研究需要开发新的生物标志物、临床终点和干预措施，用于不同病因的幼儿PAH。
Pulmonary artery hypertension (PAH) in children contributes significantly to morbidity and mortality in diverse pediatric cardiac, lung, hematologic and other diseases. Advances in pulmonary vascular biology over the past few decades have significantly expanded therapeutic strategies; however, many unique issues persist regarding our understanding of pediatric PAH. Recent studies of pediatric PAH include those that highlight gaps in our understanding of pediatric diseases associated with PAH from those of adult onset, emphasizing the strong need for specific studies regarding unique aspects of the pathogenesis and treatment of children with PAH. Registries have begun to provide new data showing differences in physiology, course, and genetics between adult and pediatric forms of PAH. Unfortunately, therapeutic strategies in pediatric pulmonary hypertension are often limited to small observational studies in children and are dependent on results from larger adult studies. In addition, clinical endpoints for studies and care remain poorly defined in infants and children. Despite many advances, long-term outcomes for children with PAH remain guarded and substantial challenges persist, especially with regard to understanding mechanisms and approach to severe PAH. Future studies are needed to develop novel biomarkers, clinical endpoints and interventions for young children with diverse causes of PAH.