Cystic Fibrosis (CF)

Etiology

  • Genetic disorder affecting multiple organs due to production of thickened, ineffective mucous

Risk Factors

  • Family hx

Symptoms

  • Can present from infant to young adult, based on degree of dysfxn
  • Sinuses→ obstructive sx, recurrent infection
  • Lung→ recurrent infections, w/progressive sob, doe, wheezing, progressive lung destruction
  • Pancreatic insufficiency→ wt loss, small stature, and diarrhea
  • Absent vas deferens (men)→ infertility
  • Impaired cervical mucous→ infertility (women)
  • Tendency to dehydrate easily w/activity

Physical Exam Findings

  • Wheezing
  • Barrel chested
  • Nasal polyps
  • Clubbing
  • Short/low weight
  • Absent vas deferens in males