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先天性膈疝相关肺发育不全的控制与调节

Control and regulation of pulmonary hypoplasia associated with congenital diaphragmatic hernia.

作者信息

Schnitzer Jay J

机构信息

Pediatric Surgical Research Laboratory, Massachusetts General Hospital, and Department of Surgery, Harvard Medical School, Boston, MA 02114, USA.

出版信息

Semin Pediatr Surg. 2004 Feb;13(1):37-43. doi: 10.1053/j.sempedsurg.2003.09.006.

Abstract

Control of fetal lung growth and development is exquisitely orchestrated and regulated. Branching morphogenesis is carefully choreographed with cell growth, proliferation, differentiation, and apoptosis in a spatially and temporally dependent manner. Some of the signals and pathways mediating these events have recently been uncovered, but much remains unknown. The precise etiologic derangements that give rise to pulmonary hypoplasia in congenital diaphragmatic hernia remain elusive. Some clues have been discovered in developmental and signaling pathways that include receptor tyrosine kinase growth factors, homeobox genes, transcription factors, airway distension, retinoid signaling, and oxidation-reduction.

摘要

胎儿肺生长和发育的控制是精心编排和调节的。分支形态发生与细胞生长、增殖、分化和凋亡以空间和时间依赖性方式进行精心编排。最近发现了一些介导这些事件的信号和途径,但仍有许多未知之处。导致先天性膈疝肺发育不全的确切病因紊乱仍不清楚。在发育和信号通路中发现了一些线索,包括受体酪氨酸激酶生长因子、同源框基因、转录因子、气道扩张、视黄酸信号和氧化还原。

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