Ivacaftor Approved for Cystic Fibrosis in Children as Young as 6 Months of Age
The FDA has expanded the indication for Kalydeco to include treatment of cystic fibrosis in patients 6 months of age and older.
Obstructive Lung Disease
Cystic Fibrosis May Progress Faster Due to Cytomegalovirus
Faster progression to end-stage lung disease observed in patients with cytomegalovirus immunoglobulin G positivity.
Effects of Azithromycin on Pulmonary Exacerbations in Pediatric Cystic Fibrosis
Approximately 70% azithromycin users with cystic fibrosis returned to ≥90% of baseline FEV1 compared with 61% of azithromycin nonusers.
Small-Molecule Ion Channels Restore Host Defenses in CF Airway Epithelia
Unselective small-molecule ion channels can restore host defenses in the airway epithelia of patients with cystic fibrosis.
Antifibrinolytic Therapy Reduces Hemoptysis Admissions in Cystic Fibrosis
Treating hemoptysis with antifibrinolytic therapy involving an inpatient and outpatient approach reduced hospital admissions in patients with cystic fibrosis.
Antifibrinolytic Therapy Reduces Hemoptysis in Adults With Cystic Fibrosis
Systemic antifibrinolytic therapy in adult patients with cystic fibrosis has been associated with a reduced rate of hospital admissions for hemoptysis.
Biomarkers of Pancreatic Function in CF Improved With Lumacaftor/Ivacaftor
Improvements in pancreatic function biomarkers, including increases in fecal elastase-1 and decreases in serum immunoreactive trypsinogen, were seen in patients treated with lumacaftor and ivacaftor.
Buying Time to First Exacerbation in Non-Cystic Fibrosis Bronchiectasis
Inhaled liposomal ciprofloxacin is associated with a longer median time to first pulmonary exacerbation vs placebo in patients with non-cystic fibrosis bronchiectasis and chronic P aeruginosa lung infection requiring antibiotic therapy.
Cystic Fibrosis May Be a Risk Factor for Hemiplegic Migraine and Stroke
Due to brain excitability and hypoxia from coughing, patients with cystic fibrosis have a propensity for cortical spreading depression and hemiplegic migraine.
Primary Ciliary Dyskinesia: Practical Pearls for Diagnosis
Recent guidelines set forth by an interdisciplinary panel assembled by the American Thoracic Society sought to demystify primary ciliary dyskinesia and address 4 main diagnostic questions.
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