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About Cystic Fibrosis (CF) Genetic Disorder

Cystic fibrosis is a disorder that affects multiple body systems1 and can progress with age, requiring multidisciplinary care and age-specific expertise to treat patients appropriately.2

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Cystic fibrosis hub

Clinical manifestations of cystic fibrosis2

 

Clinical manifestations of cystic fibrosis can change with age of the patient. Please click on the age range below (age in years) to see when the clinical manifestations of cystic fibrosis begin.

Morbidity and mortality

 

Cystic fibrosis morbidity and mortality are mainly caused by bronchiectasis, small airways obstruction, and progressive respiratory impairment. Important comorbidities caused by epithelial cell dysfunction occur in the pancreas (malabsorption), liver (biliary cirrhosis), sweat glands (heat shock), and vas deferens (infertility).2

 

Learn about cystic fibrosis pathology & mechanism of disease >

References

  1.  Farrell PM, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017;181S:S4-S15.
  2.  Elborn JS, Cystic fibrosis. Lancet. 2016;388(10059):2519-2531.

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