What are the pathophysiologic mechanisms of cystic fibrosis?

CF is caused by a mutation in the CF transmembrane conductance regulator (CFTR) gene. The CFTR protein produced by this gene regulates the movement of chloride and sodium ions across epithelial cell membranes.

How does cystic fibrosis affect the respiratory system?

Cystic fibrosis (CF) is an inherited disease that causes thickened mucus to form in the lungs, pancreas and other organs. In the lungs, this mucus blocks the airways, creating lung damage and making it hard to breathe.

What is the pathophysiologic mechanism of cystic fibrosis leading to obstructive lung disease?

Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense mechanisms. The link between abnormal ion transport and disease initiation and progression is not fully understood, but airway mucus dehydration seems paramount in the initiation of CF lung disease.

What triggers cystic fibrosis?

Cystic Fibrosis Causes Cystic fibrosis is caused by a change, or mutation, in a gene called CFTR (cystic fibrosis transmembrane conductance regulator). This gene controls the flow of salt and fluids in and out of your cells. If the CFTR gene doesn’t work the way it should, a sticky mucus builds up in your body.

How do you diagnose cystic fibrosis?

If you show symptoms of cystic fibrosis or your baby has a positive newborn screen for CF, a sweat test at a CF Foundation-accredited care center can help provide a CF diagnosis by measuring the concentration of salt in your or your baby’s sweat. The test is painless and is the most reliable way to diagnose CF.

Is asthma upper or lower respiratory?

Asthma is associated with inflammation of the lower airways inside your lungs called the bronchial tubes. Colds result from infection with a virus. Cold viruses mainly affect your nose and throat. These are the upper airways.

How Does asthma affect the respiratory system?

If you have asthma, the inside walls of the airways in your lungs can become inflamed and swollen. In addition, membranes in your airway linings may secrete excess mucus. The result is an asthma attack. During an asthma attack, your narrowed airways make it harder to breathe, and you may cough and wheeze.

Can cystic fibrosis cause pneumonia?

Chronic infections. Thick mucus in the lungs and sinuses provides an ideal breeding ground for bacteria and fungi. People with cystic fibrosis may often have sinus infections, bronchitis or pneumonia.

What are the first signs of cystic fibrosis in adults?

Respiratory signs and symptoms

  • A persistent cough that produces thick mucus (sputum)
  • Wheezing.
  • Exercise intolerance.
  • Repeated lung infections.
  • Inflamed nasal passages or a stuffy nose.
  • Recurrent sinusitis.