The question asked was: “What is the difference between CF and PCD?”
Script:
PCD was once considered a milder form of CF as the two present the same. However, the pathophysiologies differ.
Now in cystic fibrosis, we have a defective CFTR gene. This CFTR gene is responsible of movement of chloride ions out of the cells and inhibition of the epithelial sodium channel or ENaC. Remember wherever sodium travels water follows. Now this usually ensures that the lumen is lubricated and hydrated, but if this fails then the lumen becomes dehydrated and mucus thickens, bacteria builds up, and patients develop complications
The Gold standard for CF diagnosis is a sweat test
In primary ciliary dyskinesia or PCD the pathophysiology involves the cilia. Now normally the cilia move in a synchronised rhythmic motion allowing for clearance of mucus, bacteria, foreign particles, etc. The is coordinated by specific proteins within the cilia. In PCD there is disruption or an abnormality of these proteins this results in either loss of synchronised motion or complete failure of cilia. There is no specific investigation for PCD but measuring nasal nitric oxide production is very sensitive to a PCD diagnosis.
Management of both involved chest physiotherapy, and airway treatment such as salbutamol and inhaled corticosteroids, with use of prophylaxis antibiotics.
Remember other organs can be involved which require additional treatment.
