Adrenal function in cystic fibrosis

Maria Socorro Rayas, Andrea Kelly, Kara S. Hughan, Tanicia Daley, David Zangen

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

Cystic fibrosis (CF) is not known to directly affect the adrenal gland, but commonly used CF therapies do impact the function of the hypothalamic-pituitary-adrenal (HPA) axis. By binding to the glucocorticoid receptor, medications such as inhaled and oral corticosteroids can enhance the systemic effects of cortisol and result in iatrogenic Cushing syndrome. Prolonged use suppresses the body's ability to make cortisol, resulting in iatrogenic adrenal insufficiency upon medication discontinuation. Chronic use of inhaled and oral corticosteroids can negatively affect bone health, growth, and glucose metabolism. This chapter provides practical guidelines regarding the screening, diagnosis, and treatment of iatrogenic adrenal insufficiency. As the guidelines are mainly derived from the asthma literature, this chapter also highlights the need for studies to evaluate the impact of CF therapies on adrenal function and other CF-endocrinopathies.

Original languageEnglish (US)
Pages (from-to)S74-S81
JournalJournal of Cystic Fibrosis
Volume18
DOIs
StatePublished - Oct 2019

Keywords

  • ACTH stimulation test
  • Corticosteroids
  • Cortisol
  • Cystic fibrosis
  • Iatrogenic adrenal insufficiency
  • Iatrogenic Cushing syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

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