Question

Short essay question (must be 6-8 sentences)

Explain the connection between cystic fibrosis and bacterial infection.

Answer 1

Cystic fibrosis (CF) is an sutosomal recessive disorder due to gene that encode Cystic fibrosis transmembrane conductance regulator (CFTR) protein. Persons suffering from this disease have extremely salty sweat. It is due to decreased Na+ and Cl- reabsorption in the ducts. Due to a defective glycoprotein, thick mucus develops in lungs and pancreas resulting the formation of fibrous cyst. Amidst this, the cystic fibrosis shares an unique connection with bacterial infections. Patients with CF have a deadly effect with respect to bacterial infections as compared to a healthy individual. Initially, the colonizing bacteria is Staphylococcus aureus which affects half of the lungs of patients during infancy. At the middle stage, Pseudomonas aeruginosa becomes prominent that affects 80% of the lungs with chronic infection. Other bacteria includes Haemophilus influenza, Burkholderia cenocepacia which impose serious threat and are harder to detect and treat. These bacteria interfere with the cell survival process by producing pathogen associated molecular patterns (PAMPs) that are not detected by pattern recognition receptors (PRRs) of the cells immune system. Consequently, it progresses with propagation of inflammation and chronic destruction of lung tissue, or acute infection with sepsis.