cystic fibrosis Flashcards
what sort of genetic condition is cystic fibrosis?
autosomal recessive
what does the CFTR (cystic fibrosis conductive receptor) do?
pumps chloride ions out of the cells lining the respiratory tract to regulate the liquid volume on epithelial cells
in CF what happens to the mucous in the respiratory tract?
it becomes thicker as it contains less water
what is the consequence of the thickening of the mucous in top of the respiratory epithelial cells?
it causes cilia collapse and excessive inflammation
what are the common presentations of CF in infants ad young children?
recurrent chest infections
failure to thrive
is CF screened for?
yes, 5 days after birth (blood spot test then sweat test if blood test is positive)
what is the benefit of screening for CF?
it allows appropriate nutritional support enzyme therapy to allow better development
what are the two cardinal features of CF?
pancreatic insufficiency
recurrent bronchopulmonary infection
describe the abnormal stools produced by someone with CF
pale and orange
very offensive
greasy or oily
why do patients with CF produce abnormal stools?
their pancreases are abnormal and don’t produce the appropriate digestive enzyme, especially lipid digesting enzymes
what are the treatments for pancreatic insufficiency?
enteric coated enzyme pellets
high energy diet
fat-soluble vitamin and mineral supplements
H2 antagonist and proton pump inhibitors (to help get vitamins through stomach to small intestine.
what are the 2 signs of pancreatic insufficiency?
abnormal stools
failure to thrive
what are the outcomes of the recurrent bronchopulmonary infection in CF?
pneumonitis
bronchiectasis
scarring
abscesses
what is the management of CF on early years (pre-infection)?
annual influenza vaccination prophylactic antibiotics segregation/cohorting to prevent cross infection airway clearance and adjunct mucolytics
what are the common pathogens that CF patients get infected by?
S. aureus
H. influenzae
P. aeruginosa
