Physiology and pathophysiology of lung development

Abstract

Summary Over the past few years, there has been a considerable improvement in our understanding of the normal development of the fetal lung and its regulation. These advances have occurred mostly through increased knowledge of molecular biological mechanisms of growth and differentiation. These advances have also resulted in an improvement in our comprehension of the pathological basis of various pulmonary diseases. As a result of this new and improved knowledge, new and innovative therapeutic modalities are being introduced into clinical practice. The introduction of surfactant therapy into the clinical setting was one such milestone in neonatal respiratory management. Pulmonary surfactant is responsible for stabilising alveoli during normal respiration, thereby preventing atelectasis or alveolar flooding. Disease processes which result in an insufficiency in surfactant, such as respiratory distress syndrome (RDS) or congenital diaphragmatic hernia (CDH), generally carry a very high mortality rate. Exogenous surfactant administration reduces both the mortality and morbidity associated with RDS and its sequelae, and its efficacy in the treatment of CDH is now being evaluated clinically. Moreover, laboratory studies suggest that surfactant therapy may be used in combination with other treatments, such as tracheal occlusion to promote lung growth in CDH, in order to achieve a maximal effect in these complex, multifactorial lung diseases.