CYSTIC FIBROSIS 2025 FINAL EXAM
QUESTIONS WITH 100% CORRECT
ANSWERS!!
What is cystic fibrosis (CF)?
incurable, hereditary disease
caused by mutation in the gene for
the protein cystic fibrosis transmembrane conductance regulator (CFTR)
How does a mutation in CFTR affect the protein to cause CF?
abnormal transport of
Cl⁻, HCO₃⁻ and Na⁺ ions
across the epithelium,
→ thick viscous secretions
The thick mucus in patients with CF affects what organs?
lungs
pancreas
liver
intestines
With thick mucus production, patients with CF will experience what
symptoms/complications?
difficulty breathing
lung infections
digestive complications
What are the classic symptoms of CF?
classic sx:
salty tasting skin
poor growth
, poor weight gain
thick and salty mucus production
frequent lung infections
coughing and SOB
Patients with CF, that experience obstruction of pancreatic ducts, will experience what
symptom in addition to the classic symptoms?
steatorrhea (fatty stools)
poor absorptions of nutrients
(including fat-soluble vitamins)
What can result if CF is not treated?
malnutrition
By what age are more than 75% of patients with CF diagnosed?
by 2 years of age
What test is performed to confirm diagnosis of CF?
"sweat test"
sweat chloride test
What is the primary pathogen of concern for patients with CF?
Pseudomonas aeruginosa
What is critical for administering inhaled medications to maximize absorption and effect in
CF?
the correct order is critical
What therapies are given or administered prior to inhaled abx?
bronchodilators
hypertonic saline
dornase alfa
QUESTIONS WITH 100% CORRECT
ANSWERS!!
What is cystic fibrosis (CF)?
incurable, hereditary disease
caused by mutation in the gene for
the protein cystic fibrosis transmembrane conductance regulator (CFTR)
How does a mutation in CFTR affect the protein to cause CF?
abnormal transport of
Cl⁻, HCO₃⁻ and Na⁺ ions
across the epithelium,
→ thick viscous secretions
The thick mucus in patients with CF affects what organs?
lungs
pancreas
liver
intestines
With thick mucus production, patients with CF will experience what
symptoms/complications?
difficulty breathing
lung infections
digestive complications
What are the classic symptoms of CF?
classic sx:
salty tasting skin
poor growth
, poor weight gain
thick and salty mucus production
frequent lung infections
coughing and SOB
Patients with CF, that experience obstruction of pancreatic ducts, will experience what
symptom in addition to the classic symptoms?
steatorrhea (fatty stools)
poor absorptions of nutrients
(including fat-soluble vitamins)
What can result if CF is not treated?
malnutrition
By what age are more than 75% of patients with CF diagnosed?
by 2 years of age
What test is performed to confirm diagnosis of CF?
"sweat test"
sweat chloride test
What is the primary pathogen of concern for patients with CF?
Pseudomonas aeruginosa
What is critical for administering inhaled medications to maximize absorption and effect in
CF?
the correct order is critical
What therapies are given or administered prior to inhaled abx?
bronchodilators
hypertonic saline
dornase alfa
