Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive mutation that primarily affects the pulmonary and pancreatic systems, but may involve any organ system (1)

Due to improvements in medic...

At least one of the following:

• One or more typical phenotypic features of CF:

  • Chronic pulmonary disease

  • Chronic sinusitis

  • Characteristic GI and...

Cystic Fibrosis Foundation guidelines call for:

• Four office visits, four respiratory cultures, PFTs q6mo, and at least one evaluation by a multidisciplinary team, including di...

Upon discharge for a pulmonary exacerbation, follow-up with CF specialist within 2 to 4 weeks

Routine clinic visits every 3 months with airway cultures and PFTs as...

E84.9 Cystic fibrosis, unspecified

E84.11 Meconium ileus in cystic fibrosis

E84.0 Cystic fibrosis with pulmonary manifestations

E84.19 Cystic fibrosis with other intestinal manifestations

E84.8...

Meconium ileus is pathognomonic for CF

When sweat test is equivocal, CFTR genetic testing is diagnostic

Consider CF in any child with chronic diarrhea, especially with poor growth or fai...