A 25 year old patient with cystic fibrosis wants to know about the disease. Which of the following is majorly associated with chronic airway infection in cystic fibrosis?
**Core Concept**
Cystic fibrosis is a genetic disorder that affects the respiratory, digestive, and reproductive systems. The disease is characterized by the production of thick, sticky mucus that clogs the airways and traps bacteria, leading to recurrent infections and inflammation. The underlying mechanism involves abnormal ion transport across epithelial cell membranes, resulting in impaired clearance of mucus.
**Why the Correct Answer is Right**
The major association with chronic airway infection in cystic fibrosis is the defective function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is a chloride channel that regulates the movement of chloride ions across epithelial cell membranes. In individuals with cystic fibrosis, mutations in the CFTR gene lead to a dysfunctional protein, causing an imbalance in the ionic composition of the airway surface liquid. This results in the production of thick, dehydrated mucus that is more susceptible to colonization by bacteria, such as Pseudomonas aeruginosa.
**Why Each Wrong Option is Incorrect**
**Option A:** Although Staphylococcus aureus is a common pathogen in cystic fibrosis, it is not the primary cause of chronic airway infection in this disease.
**Option B:** Haemophilus influenzae is a Gram-negative bacterium that can cause respiratory tract infections, but it is not specifically associated with cystic fibrosis.
**Option C:** Aspergillus fumigatus is a fungus that can cause pulmonary aspergillosis in individuals with cystic fibrosis, but it is not the primary cause of chronic airway infection.
**Clinical Pearl / High-Yield Fact**
It's essential to recognize that the defective CFTR protein in cystic fibrosis leads to an imbalance in the ionic composition of the airway surface liquid, resulting in thick, dehydrated mucus that is more susceptible to bacterial colonization. This understanding is crucial for developing effective treatment strategies, such as CFTR modulator therapy, to improve airway clearance and reduce the risk of chronic infections.
**Correct Answer:** C.