Immune, inflammatory and infectious consequences of estrogen in women with cystic fibrosis.

Cystic fibrosis (CF), a multi-system genetic disease with predominantly pulmonary manifestations, occurs due to defective chloride secretion at epithelial surfaces. The consequential sodium hyper-absorption leads to mucus hypersecretion, compromised mucociliary clearance and pathogenic colonization by, for instance, Staphylococcus aureus and/or Pseudomonas aeruginosa. This sequence of events leads to recurrent infection and persistent chronic neutrophilic inflammation causing airway damage, irreversible bronchiectasis, respiratory failure and death.