Cystic Fibrosis bullSHIT

Question 1

Delayed passage of meconium

Answer 1

meconium ileus is a distal small bowel obstruction due to abnormally dehydrated meconium in a pt w CF; persistent, tx-resistent infectious pneumonias, bronchectasis and cor pulmonale account for most deaths due to CF

Question 2

19 pt dies, autospy shows dilation of bronchial tree and small airways filled w mucin plugs

Answer 2

typically have chronic cough due to impaired clearance, buildup leads to mucus plugging and bacterial colonization

Question 3

Chronic rhinosinusitis w nasal polyposis in children should prompt what?

Answer 3

a CF evaluation; especially when signs of pancreatic inefficiency (ex: loose stools, failure to thrive, weight loss) are present

Question 4

Sweat ducts vs Respiratory mucosa

Answer 4

electrolytic differences in the body fluids of pts w CF develop due to tissue specific variation in the functioning of the CFTR gene; in sweat glands mut CFTR prevents salt reabsorption, producing sweat that has higher levels of NA+ and Cl-; in respiratory epithelium mut CFTR causes thick viscid secretions that are low in both Na+ and Cl-