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Self-Assessment Questions

Pulmonary Disease

Cystic Fibrosis: Review Questions

Subramanyam Chittivelu, MD, FCCP

Dr. Chittivelu is Assistant Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, and Clerkship Director in Internal Medicine, Texas Tech University Health Sciences Center, Amarillo, TX

Choose the single best answer for each question.

1. Each of the following statements regarding the genetics of cystic fibrosis (CF) is correct EXCEPT:
  1. The CF gene codes for a protein called cystic fibrosis transmembrane regulator (CFTR)
  2. CFTR is largely expressed in epithelial cells of the airways, the pancreas, the biliary system, the genitourinary system, and the sweat glands
  3. Although there are a few hundred mutations that exist for CF, the most prevalent mutation among northern Europeans is W1282X
  4. Severity of lung disease and liver disease cannot be predicted by genotype
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2. Which of the following exocrine glandular ducts are NOT obstructed in cystic fibrosis?

  1. Pancreas
  2. Lungs
  3. Sweat glands
  4. Glands of the uterine cervix
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3. Which of the following is NOT a pulmonary complication of CF?

  1. Massive hemoptysis and pneumothorax
  2. Atelectasis and acute respiratory failures
  3. Allergic bronchopulmonary aspergillosis and hypertrophic pulmonary osteoarthropathy
  4. Lymphangiomyomatosis and chylothorax
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  4. Which of following statements regarding the mechanism of action of recombinant human deoxyribonuclease (rhDNase) in CF is correct?
  1. rhDNase cleaves the extracellular DNA from the neutrophils in sputum to make it less viscous
  2. rhDNase’s potent antibacterial action prevents the growth of pseudomonads and staphylococci
  3. rhDNase has antibacterial, bronchodilator, and mucolytic actions
  4. rhDNase corrects the defect in the CF gene on chromosome 7
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5. Each of the following statements regarding pregnancy in women with CF is correct EXCEPT:

  1. Impairment of pulmonary function is the major predictor of both maternal and fetal outcome
  2. CF patients have an increased risk for premature delivery
  3. The maternal and fetal outcome is good for most cystic fibrosis patients
  4. The pregnancy need not be a planned one
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