Abstract
Cystic fibrosis is the most common genetically inherited disease in the Caucasian population, with approximately 30,000 patients in the USA and more than 50,000 patients worldwide. The primary defect in the cystic fibrosis transmembrane regulator gene affects the production and/or function of the cystic fibrosis transmembrane regulator protein. Depending on the severity of the genetic defect, patients may have minimal disease expression (e.g., male infertility) or multisystem involvement, including recurrent respiratory infection progressing to respiratory failure, hepatobiliary disease, exocrine pancreatic insufficiency, diabetes mellitus and gastrointestinal tract motility problems. Pseudomonas aeruginosa is commonly isolated from the lower respiratory tract in early childhood. Chronic infection is associated with increased morbidity and mortality. P. aeruginosa infection may be acquired from the environment or by person-to-person contact. Clinicians should adopt a proactive protocol to prevent chronic infection. The cornerstones of such a policy are microbiological surveillance, infection control and antibiotic-based eradication regimens.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
No writing assistance was utilized in the production of this manuscript.