Question

) Which is a common manifestation of cystic fibrosis (CF)?

Red eyes
Trunk rash
Chronic cough
Bad breath
2) In newborns, what laboratory test is used to diagnose CF when the test result remains elevated between 7 and 14 days of life?

Plasma blood glucose (mg/dl)
Low-density lipoprotein (LDL)
Serum potassium (K)
Immunoreactive trypsinogen (IRT)
3) Which of the following is a clinical presentation of pulmonary exacerbations?

Increased respiratory symptoms, coughing, sputum production
Wheezing and walking 500 feet before resting
Fatigue and the ability to pass the mannitol tolerance test
Hospitalization, but the quality of life is not affected
4) What is one recommendation for long-term maintenance of optimal lung health in patients with CF?

Clearance of airway secretions
Give tetracycline to children from ages 1 to 7
Increase sputum viscosity
Give lumacaftor/ivacaftor with fat-free meals
5) Which of the following bacteria has been isolated in chronic infections that occur in patients with CF?

Achromonius streptus
Pseudomonas aeruginosa
Pseudomonas aureus
Achrostreptus aeruginosa
6) Cystic fibrosis mainly affects which system of the body?

Nervous system
Skeletal system
Respiratory system
Urinary system
7) Which symptoms in a baby or young child might help identify CF?

Pale stools and ear infections
Skin that appears jaundiced and forced vital capacity (FVC) ≥90%
Frequent wheezing and no mucus
Chronic cough and thick phlegm
8) Which choice below is a patient counseling opportunity?

Advise that dornase alfa can damage electrical equipment
Exercise as tolerated, wash hands, and avoid smoke
Ask about the history of kidney problems with hypertonic saline
Inspect capsules but not ampules for possible leaks
9) Cystic fibrosis patients with _____________ should not fly on a plane, perform spirometry, or lift weights for the 2-week period after the pneumothorax has resolved.

hemoptysis
chronic rhinosinusitis
bacterial infection
pneumothorax
10) Which option below does not reflect genetic or molecular issues associated with CF?

Deficiency in POMC, PCSk1, or LEPR
Misfolded CFTR seen with F508del
No production of CFTR protein
CFTR therapy addresses modular defects

Answer 1

Here are the correct answers with brief notes:

1) Chronic cough — common respiratory manifestation of CF.
2) Immunoreactive trypsinogen (IRT) — newborn screening marker for CF.
3) Increased respiratory symptoms, coughing, sputum production — typical of pulmonary exacerbations.
4) Clearance of airway secretions — key long-term maintenance strategy for lung health in CF.
5) Pseudomonas aeruginosa — a common chronic pathogen in CF airways.
6) Respiratory system — CF primarily affects the lungs (though it is multisystem).
7) Pale stools and ear infections — pale/greasy stools (steatorrhea) in infants suggest pancreatic insufficiency from CF.
8) Exercise as tolerated, wash hands, and avoid smoke — practical counseling to help reduce infections and maintain lung health.
9) Pneumothorax — after a pneumothorax patients should avoid flying, spirometry, heavy lifting for ~2 weeks after resolution.
10) Deficiency in POMC, PCSk1, or LEPR — these are not CF-related genetic defects (they relate to other metabolic/weight-regulation disorders).