The rearch for the factors that affect fetal lung maturation, and pathogenesis of fetal lung hypoplasia

背景自合成表面活性剂问世以来，呼吸窘迫综合征的预后得到了显著改善。然而，新生儿肺发育不全有很高的死亡率，尽管在医疗管理的进步，肺发育不全的治疗仍然是困难的新生儿医学。在妊娠中期，特别是在存活阈值附近，胎儿肺结构的分析从以下角度来看是重要的：1）管理肺发育不良婴儿的策略，以及2）改善小于23周妊娠出生的超早产儿的预后。本研究于2000年4月至2003年3月期间对以下主题进行了调查：1.宫内暴露于除草醚后肺发育不良治疗的基础研究。2.先天性气管发育不全病例的肺成熟。3.妊娠中期，特别是妊娠21周的人胎肺成熟。4.从出生体重的角度来看的生存阈值。5.人胎表面活性蛋白D的宫内表达。结论1.气管内注射全氟碳化物可改善除草醚所致肺发育不良的呼吸功能.妊娠中期气管发育不全的胎儿肺成熟较对照组提前.妊娠21周时，终末气道成熟的表面活性蛋白B的产生较少，这与胞浆中蛋白前体加工机制不成熟有关.我们报告了一个超早产儿，出生体重289克，出生于1999年6月。人胎肺中表面活性蛋白D的产生始于胎龄约21周的细支气管和终末气道。

BACKGROUNDThe prognosis of respiratory distress syndrome has been dramatically improved since synthetic surfactant was available. However, neonates with pulmonary hypoplasia have a high mortality despite advances in medical management, and the treatment of pulmonary hypoplasia remains difficult in neonatal medicine. The analysis of fetal lung structure during mid-gestation,especially around the threshold of viability, is important from the point of view of 1) the strategy to manage the babies with pulmonary hypoplasia, and 2) improvement of the prognosis of ultra-premature babies born less than 23 weeks gestation. We investigated the following subjects between April, 2000 and March, 2003:1.the basic research on treatment of lung hypoplasia following intrauterine exposure to nitrofen.2.lung maturation in a case of congenital tracheal agenesis.3.lung maturation of human fetuses in mid-gastation, especially 21 weeks gestation.4.threshold of viability from the point of view of birth weight.5.intrauterine expression of surfactant protein D in human fetuses.RESULTS1. Respiratory status of lung hypoplasia induced by nitrofen was improved by intratrachal administration of perflurocarbon.2. Lung maturation of fetal tracheal agenesis in mid-gestaion appeared to be more advanced than control group.3. The production of mature surfactant protein B in terminal airways is scarce at 21 weeks gestation, which is associated with the immature mechanism of proprotein processing in the cytoplasm.4. We reported an ultra-premature baby of 289 gram birth weight, who is born June 1999.5. The production of surfactant protein D in human fetal lungs begins in bronchiolar and terminal airways from about 21 weeks gestation.