Girosi Donata, Bellodi Simona, Sabatini Federica, Rossi Giovanni A
Pulmonary and Allergy Units, G. Gaslini University Hospital, Genoa, Italy.
Paediatr Respir Rev. 2006;7 Suppl 1:S235-9. doi: 10.1016/j.prrv.2006.04.192. Epub 2006 Jun 6.
Because of the common embryologic origin, alteration of the many factors modulating the development of the alimentary and the respiratory tract often results in structural abnormalities involving the two systems. Indeed, some of the most common embryologic disorders of the airways are frequently associated with anomalies of the gastrointestinal tract. Lung growth occurs as a series of tightly regulated events, depending on a number of factors, including developmental, genetic and environment ones. Abnormalities of any of these factors may causes developmental alterations of the lung leading to a group of disorders termed "bronchopulmonary-foregut malformations". These are usually sporadic, solitary cystic hamartomas, involving conducting airways, arteries, venous drainage, and lung parenchyma, which are now often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the foetus or newborn, many will be asymptomatic at birth, raising controversy about management: simple observation or surgery and, if so, at what age? Over the past two decades molecular studies have started to shed light on the complex series of events that control proper formation of the lung, with the hope that a better understanding of the molecular basis of pulmonary maturation will allow the design of new therapeutic strategies.
由于共同的胚胎学起源，许多调节消化道和呼吸道发育的因素发生改变，常常导致涉及这两个系统的结构异常。事实上，一些最常见的气道胚胎学疾病常与胃肠道异常相关。肺的生长是一系列受到严格调控的过程，取决于许多因素，包括发育、遗传和环境因素。这些因素中的任何一个异常都可能导致肺发育改变，进而引发一组称为“支气管肺前肠畸形”的疾病。这些畸形通常为散发性、孤立性囊性错构瘤，累及传导气道、动脉、静脉引流和肺实质，现在常在常规产前超声检查中被发现。虽然一些病变可能很大，并在胎儿或新生儿期引起严重并发症，但许多病变在出生时是无症状的，这引发了关于治疗的争议：是单纯观察还是手术治疗，如果手术，在什么年龄进行？在过去二十年中，分子研究已开始揭示控制肺正常形成的一系列复杂事件，希望对肺成熟的分子基础有更深入的了解将有助于设计新的治疗策略。
