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.
Obstructive Lung Disease
Restrictive, Obstructive Lung Disease Linked to Dementia Risk
Associations were seen for both Alzheimer’s disease-related dementia and cerebrovascular etiologies.
Bronchiectasis Severity Associated With Pulmonary Vascular Pruning
Mild bronchiectasis in smokers is associated with distal pulmonary vascular pruning.
Hypertonic Saline Inhalation Safety Tested in Infants With Cystic Fibrosis
In infants with cystic fibrosis, preventive inhalation with hypertonic saline during the first months of life is safe and well tolerated and results in clinical improvements, according to a new study.
Multiple Breath Washout Test Inaccuracies Revealed Via Over-Reader Assessment
Including invalid data decreases the validity of multiple breath washout measurements in clinical trials involving adults with bronchiectasis.
VX-445-Tezacaftor-Ivacaftor May Improve CFTR in Cystic Fibrosis
VX-445-tezacaftor-ivacaftor led to noticeable improvements in Phe508del CFTR protein function.
Cystic Fibrosis Lung Function Improved With Triple Therapy
Adding VX-659 to tezacaftor and ivacaftor therapy improved lung function in patients with cystic fibrosis.
CFTR Dysfunction Likely Associated With Worsened Airway Disease in Smokers
Cystic fibrosis transmembrane conductance regulator dysfunction is associated with worsened airway disease in patients with COPD.
Novel Inhaled Treatment for Cystic Fibrosis Granted Orphan Drug Designation
The FDA has granted Orphan Drug Designation to a novel inhaled treatment that could potentially improve lung function in patients with cystic fibrosis.
CF Severity Predicted by Forskolin-Induced Swelling in Intestinal Organoids
Forskolin-induced swelling in intestinal organoids is a clinically relevant biomarker of disease severity in infants diagnosed with cystic fibrosis.
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