Montedonico Sandra, Nakazawa Nana, Puri Prem
The Children's Research Centre, Our Lady's Children's Hospital, Dublin 12, Ireland.
Pediatr Surg Int. 2008 Jul;24(7):755-61. doi: 10.1007/s00383-008-2140-x. Epub 2008 Apr 10.
Congenital diaphragmatic hernia (CDH) is a major life-threatening cause of respiratory failure in the newborn. Recent data reveal the role of a retinoid-signaling pathway disruption in the pathogenesis of CDH. We describe the epidemiology and pathophysiology of human CDH, the metabolism of retinoids and the implications of retinoids in the development of the diaphragm and lung. Finally, we describe the existing evidence of a disruption of the retinoid-signaling pathway in CDH.
先天性膈疝（CDH）是新生儿呼吸衰竭的主要致命原因。最新数据揭示了视黄酸信号通路破坏在CDH发病机制中的作用。我们描述了人类CDH的流行病学和病理生理学、视黄酸的代谢以及视黄酸在膈肌和肺发育中的意义。最后，我们阐述了CDH中视黄酸信号通路破坏的现有证据。
