Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive genetic mutation (CFTR gene) that most prominently affects the pulmonary and pancreatic systems. The gastrointestinal (GI), endocrine, ...

At least one of the following:

• One or more typical phenotypic features of CR:

  • Chronic pulmonary disease

  • Chronic sinusitis

  • Characteristic gastroin...

Cystic Fibrosis Foundation guidelines call for yearly evaluation:

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

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

Routine clinic visits every 3 months, with airway cultures and pulmona...

Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev.  2014;(3):CD002010.

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 virtually pathognomonic for CF.

When sweat test is equivocal, CFTR genetic testing is diagnostic.

CF must be considered in any child with chronic diarrhea, especially i...