TY - CHAP M1 - Book, Section TI - Pulmonary Surfactant and Disorders of Surfactant Homeostasis A1 - Whitsett, Jeffrey A. A1 - Weaver, Timothy E. A2 - Grippi, Michael A. A2 - Elias, Jack A. A2 - Fishman, Jay A. A2 - Kotloff, Robert M. A2 - Pack, Allan I. A2 - Senior, Robert M. A2 - Siegel, Mark D. PY - 2015 T2 - Fishman's Pulmonary Diseases and Disorders, 5e AB - Pulmonary surfactant is a complex mixture of phospholipids and proteins that creates a unique interface separating alveolar gas and liquids at the alveolar cell surface, reducing surface tension, and maintaining lung volumes at end expiration. Reduction of the surface tension at the air–liquid interface is a requirement for respiratory function following birth. Deficiency of pulmonary surfactant causes respiratory failure in premature infants, or infantile respiratory distress syndrome (IRDS). The adequacy of pulmonary surfactant is maintained by unique and highly regulated systems mediating the synthesis, secretion, reutilization, and catabolism of surfactant. Loss or inactivation of pulmonary surfactant later in life occurs in the adult respiratory distress syndrome (ARDS), a significant cause of morbidity and mortality following infection, shock, or trauma. Mutations in genes regulating surfactant homeostasis, including SFTPA, SFTPB, SFTPC, ABCA3, TITF1, and CSF2RA cause acute and/or chronic lung disease in newborn infants, children, and adults. Disorders of GM-CSF signaling inhibit surfactant lipid and protein catabolism by alveolar macrophage causing pulmonary alveolar proteinosis (PAP). This chapter reviews the biology of the surfactant system and its implications for the pathogenesis, diagnosis, and treatment of respiratory disease in premature infants and adults. Suggested reviews of these topics are provided in the References section.1–5 SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1122354467 ER -