Thébaud B, de Lagausie P, Forgues D, Mercier J C
Service de réanimation pédiatrique polyvalente, hôpital Robert-Debré, Paris, France.
Arch Pediatr. 1998 Sep;5(9):1009-19. doi: 10.1016/S0929-693X(98)80015-8.
Described for the first time in 1848 by Bochdalek, congenital diaphragmatic hernia is still a hot topic. How can it be that a simple defect of the diaphragm still has a mortality rate reaching 50% in 1997, and this despite continuous progress in neonatal intensive care? If some problems remain unsolved, experimental studies over the past 30 years have raised some questions concerning the pathogenesis, and have shed some light into the pathophysiology of congenital diaphragmatic hernia. This article reviews the recent knowledge about the aetiology, pathogenesis and pathophysiology of this complex malformation.
先天性膈疝于1848年由博赫达勒克首次描述,至今仍是一个热门话题。尽管新生儿重症监护不断取得进展,但为何一个简单的膈肌缺陷在1997年的死亡率仍高达50%?如果一些问题仍未解决,那么过去30年的实验研究提出了一些关于发病机制的问题,并对先天性膈疝的病理生理学有了一些了解。本文综述了关于这种复杂畸形的病因、发病机制和病理生理学的最新知识。