Cystic Fibrosis Exam Questions and Correct Answers Latest Update (100% Pass)

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 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* -Increased sputum production -Obstructive pulmonary disease -DOE and SOB -Hemoptysis -Decreased O2 sats -Pulmonary HTN/Cor Pulmonale -Progressive decrease in lung function -Exacerbations=disease progression and *lung damage* What is the Gold Standard method of assessing/monitoring lung function in CF? - Answers FEV1 FEV1 Normal lung function: - Answers or = to 90% FEV1 Mild: - Answers 70-89% FEV1 Moderate: - Answers 40-69% FEV1 Severe: - Answers 40% What CF pathogens are most common in young children and infants? - Answers -Staphylococcus aureus -Hemophilus influenzae What CF pathogens are most common in adolescents and adults? - Answers -Pseudomonas aeruginosa What other CF pathogens are common? - Answers -MRSA -Burkholderia cepacia -Aspergillus fumigatus -Stenotrophomonas maltophilia True or False: S. aureus is the most common CF pathogen. - Answers False, P. aeruginosa is the most common CF pathogen.