Cystic fibrosis (CF) is an autosomal recessive genetic mutation (CFTR gene) that most prominently affects the pulmonary and pancreatic systems.
The gastrointestinal (GI), endocrine, a...
Routine prenatal ultrasonography indicates hyperechogenic bowel.
The risk is highest if there is evidence of meconium peritonitis (scattered calcifications are seen throughout the feta...
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. [View Abstract on OvidMedline]
Mall MA, Bo...
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...
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