Chapter 9 - The Development of the Pulmonary Surfactant System

Abstract

The pulmonary surfactant system is uniquely positioned to meet the physical and environmental challenges associated with the transition to air breathing at birth. Its maturation is under complex multifactorial control that ensures the system is mature in late gestation in readiness for birth. Surfactant dysfunction through preterm birth or genetic causes may lead to respiratory distress syndrome (RDS). The use of antenatal glucocorticoids and neonatal administration of exogenous surfactant has been remarkably successful in the treatment of RDS. However, resistance to therapy has uncovered a genetic basis to many cases of acute neonatal RDS. Identification of gene defects relating to surfactant has provided a specific cause to many previously classified idiopathic interstitial lung diseases during neonatal life, early childhood, or adulthood. These discoveries have led to the search for genetic markers of RDS risk and new therapeutic targets of interstitial lung disease, including the development of gene therapy strategies.