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What are the pathophysiologic mechanisms of cystic fibrosis?

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?

How does CF affect the respiratory system? The abnormal electrolyte transport system in CF causes the cells in the respiratory system, especially the lungs, to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move.

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.

Can cystic fibrosis be mistaken for asthma?

Cystic fibrosis is another chronic lung disease. Its symptoms may mimic asthma symptoms. Sometimes it occurs alongside asthma.

How old is the oldest cystic fibrosis patient?

The oldest person diagnosed with CF for the first time in the U.S. was 82, in Ireland was 76, and in the United Kingdom was 79. Those diagnosed after age 50 tend to have a long history of frequent bouts of colds, sinus infections, pneumonia, stomach pains, acid reflux, and trouble gaining or keeping on weight.

How can you tell the difference between asthma and cystic fibrosis?

The diagnosis of asthma in a patient with CF is predominantly based on the patient’s history. The presence of cough is irrelevant, but recurrent wheezing is a cardinal symptom. There may be bronchial hyperreactivity and there may be bronchodilator responsiveness, but both are common in patients with CF.

What is often misdiagnosed as asthma?

Asthma mimickers can be extrathoracic or intrathoracic. Other more common mimickers of asthma include pulmonary eosinophilic disorders, sarcoidosis, hypersensitivity pneumonitis, CF and CHF.

Has anyone survived cystic fibrosis?

While there is no cure yet for cystic fibrosis (CF), people with CF are living longer, healthier lives than ever before. In fact, babies born with CF today are expected to live into their mid-40s and beyond. Life expectancy has improved so dramatically that there are now more adults with cystic fibrosis than children.