57 - Lung Diseases Associated With Disruption of Pulmonary Surfactant Homeostasis

Lawrence M. Nogee, Bruce C. Trapnell

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Pulmonary surfactant is a complex mixture of lipids and specific proteins that stabilizes alveoli at the end of expiration and has an important role in innate immune defense. Genetic or autoimmune mechanisms that disrupt the normal production, function, or catabolism of surfactant can result in diffuse lung disease that is variable in its onset, ranging from the newborn period to adulthood, as well as severity. While rare, these disorders are associated with significant morbidity and mortality and provide insights into normal lung cell metabolism. This chapter will review the epidemiology, pathophysiology, and clinical and laboratory aspects of these disorders and discuss the approach to diagnosis and treatment.

Original languageEnglish (US)
Title of host publicationKendig's Disorders of the Respiratory Tract in Children
PublisherElsevier Inc.
Pages836-849.e5
ISBN (Electronic)9780323555951
ISBN (Print)9780323448871
DOIs
StatePublished - Jan 1 2019

Keywords

  • Childhood interstitial lung disease
  • Genetic basis of disease
  • Pulmonary alveolar proteinosis
  • Respiratory distress syndrome

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Nogee, L. M., & Trapnell, B. C. (2019). 57 - Lung Diseases Associated With Disruption of Pulmonary Surfactant Homeostasis. In Kendig's Disorders of the Respiratory Tract in Children (pp. 836-849.e5). Elsevier Inc.. https://doi.org/10.1016/B978-0-323-44887-1.00057-2