Lung Transplantation for Inherited Disorders of Surfactant Metabolism

Abstract

Inherited disorders of surfactant metabolism are rare diseases that provide unique opportunities for understanding the mechanisms of surfactant function and metabolism. Presenting both as acute respiratory dysfunction in the neonatal period with a predictably lethal outcome as well as chronic respiratory insufficiency of variable onset and unpredictable natural histories, these disorders present unique challenges for clinicians and families because the treatment options are limited to lung transplantation and compassionate care. We briefly review the known inherited surfactant disorders, the challenges associated with offering lung transplantation for these disorders, and the outcomes.