Cystic Fibrosis Exam Questions and Correct Answers Latest Update (100% Pass)
What ethnicity is CF most common in? - Answers Caucasian
True or False: CF is the most common genetically linked life-shortening disease in Caucasians. - Answers True.
What is CFTR responsible for? - Answers CFTR is res...
What ethnicity is CF most common in? - Answers Caucasian
True or False: CF is the most common genetically linked life-shortening disease in Caucasians. - Answers
True.
What is CFTR responsible for? - Answers CFTR is responsible for transportation of fluid and electrolytes.
-Improper balance of fluid and electrolytes leads to dehydrated mucus membranes particularly in the
lungs and GIT
-Dehydrated secretions result in lung and GIT problems
True or False: The mutation responsible for CF is an autosomal dominant genetic mutation. - Answers
False, it is an autosomal recessive genetic mutation.
-Both parents MUST be carriers.
87% of CF patients have at least one copy of __________. - Answers ∆F508.
What does CFTR stand for? - Answers Cystic Fibrosis Transmembrane Regulator protein
-It regulates a chloride channel that transports electrolytes and water into lumens of exocrine glands.
Where is the CFTR protein found? - Answers -Sweat glands*
-Pancreas*
-Male genital ducts*
-GIT*
-Lungs
-Salivary ducts
Mutated CFTR protein and it's role in the:
-Lungs
-GIT
-Liver
-Pancreas
-Reproductive organs - Answers -Decreased Cl- transport to outside of cell
-Increased Na+ transport to inside of cell
, -Increased H2O inside of cell due to osmotic gradient
-Surface of epithelial cells therefore decreased in Cl-, Na+, and H2O
-Decreased H2O within lumens/epithelial surfaces
Mutated CFTR protein and it's role in the sweat gland: - Answers -Decreased Cl- absorption causing
increased Cl- concentration skin surface
A sweat Cl- of ________ is positive for CF. - Answers >60 mEq/L
Clinical presentation of CF: - Answers -Meconium ileus at birth
-Respiratory illness/symptoms
-Failure to thrive/malnutrition
-Steatorrhea/abnormal stools
-Presence of typical CF pathogens (pseudomonas)
-Nasal polyps
-Endobronchial disease (cough/sputum)
-Digital clubbing
-CF Triad
What is the "CF Triad?" - Answers -Pulmonary Disease
-GI Disease
-Increased chloride in sweat
*Not necessary to have all 3
How are the lungs affected? - Answers -Thick/dense layer
-Decreased ciliary movement
-Poor clearance of mucus
-Perfect environment for infection
-Reduced ability to fight off infections
-Decreased gas exchange
Clinical presentation of the lungs: - Answers -Chronic cough*
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