Cystic fibrosis (CF) is an autosomal recessive genetic mutation (CFTR gene) that most prominently affects the pulmonary and pancreatic systems.
The GI, endocrine, and reproductive sys...
Suspect with failure to thrive, steatorrhea, and recurrent respiratory problems.
Chronic/recurrent respiratory symptoms, including airway obstruction and infections
Persistent infiltr...
CF Foundation Guidelines call for yearly evaluation:
Four office visits, four respiratory cultures, PFTs q6mo, and at least 1 evaluation by a multidisciplinary team includin...
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. [View Abstract on OvidMedline]
Mall MA, B...
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...
Sign up for a 10-day FREE Trial now and receive full access to all content.