Chapter 16: Selected Agents of Pulmonary Value

Chapter 16: Selected Agents of Pulmonary Value

Complete Chapter Questions With Answers

Sample Questions Are Posted Below

MULTIPLE CHOICE

1. A drug intended for therapy of congenital a₁-antitrypsin deficiency is

ANS:  C

a₁-proteinase inhibitor (API) is indicated for long-term replacement therapy in individuals with congenital deficiency of API, with clinically demonstrable panacinar emphysema. At the present time, three agents are available: Aralast, Prolastin, and Zemaira. Aralast and Zemaira are indicated only for patients who have established a₁-proteinase deficiency. Pulmozyme (dornase alfa) is a mucolytic, Pneumovax is a vaccine, and prednisone is a steroid. Although these drugs may be helpful at some point in the disease process, they do not specifically treat a₁-antitrypsin deficiency.

REF:   p. 275

2. The physician suspects that your patient may have a₁-antitrypsin (a-AT) deficiency. Which disease process would help to confirm this diagnosis?

ANS:  A

a-AT deficiency is a genetic defect that can lead to the development of severe panacinar emphysema. In about 50% of cases of emphysema that result from a₁-proteinase inhibitor (API) deficiency, there is accompanying chronic bronchitis with mucus hypersecretion, perhaps as a result of secretory cell metaplasia caused by unchecked proteases in the epithelial lining fluid.

REF:   p. 275

3. Emphysema is a process of

ANS:  D

The pathogenesis of emphysema is described as a process of alveolar wall destruction caused by insufficient protection from the protease neutrophil elastase, an enzyme that can cleave all forms of connective tissue and degrade elastic fiber in the lungs by solubilizing elastin. With inadequate a₁-proteinase inhibitor (API) levels in the lung to balance the protease activity, emphysema results at a significantly earlier age than is normally seen.

REF:   p. 275

4. The major limitation of Prolastin therapy is

ANS:  B

Prolastin is extremely expensive, with therapy costing $25,000 to $40,000 per year. A cost-effectiveness analysis of Prolastin concluded that a₁-antitrypsin (a₁-AT) replacement therapy is cost-effective in individuals who have severe a₁-AT deficiency and severe chronic obstructive pulmonary disease (COPD). The American Thoracic Society stated that a₁-proteinase inhibitor (API) augmentation therapy should be used for patients with a serum concentration of API less than 11 µmol/L, or 80 mg/dl.

REF:   p. 276

5. The recommended dosage of Prolastin is

ANS:  D

The recommended dosage of a₁-proteinase inhibitor (API) is 60 mg/kg body weight, given once weekly. The dose is given intravenously at a rate of 0.08 ml/kg/min or greater, depending on patient comfort, and usually takes about 15 to 30 minutes for total infusion.

REF:   p. 276