Defect of the cystic fibrosis transmembrane conductance regulator (CFTR)
CFTR functions as an ATP-regulated chloride channel that regulates the activity of chloride and sodium channel...
Failure to thrive
Recurrent respiratory tract infections
Anasarca in infancy
Salty taste of skin
Head, ears, eyes, nose, and throat (HEENT):
Nasal polyps; severe headach...
Transcutaneous pacing for unstable type II 2nd- or 3rd-degree block
Avoid with type II 2nd-degree block because it may precipitate 3rd-degree block.
Pulmonary exacerbation with significant deterioration from baseline, hypoxemia, resistant bacteria, failure of outpatient therapy
With CF patients in respiratory distress, always consider pneumothorax: Obtain CXR.
For CF patients with abdominal pain/vomiting, always consider DIOS and intussusception
Hoffman LR, Ramsey BW. Cystic fibrosis therapeutics: The road ahead. Chest. 2013;143:207–213.
Ryan G, Jahnke N, Remmington T. Inhaled antibiotics for pulmonary exacerbatio...
277.00 Cystic fibrosis without mention of meconium ileus
277.02 Cystic fibrosis with pulmonary manifestations
277.03 Cystic fibrosis with gastrointestinal manifestations
277.01 Cystic fibrosis ...
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