Pharmacy & Therapeutics - April 2008 - (Page 245) CONTINUING EDUCATION CREDIT Continuing Education Questions for Physicians and Pharmacists TOPIC: Pharmacological Management of Sickle Cell Disease ACPE Program # 079-000-08-016-H01-P CE Evaluation: Select the one best answer to each of the following questions, and record your response on the examination answer sheet. Complete the additional requested information. Forward the answer sheet, with appropriate payment, to the Department of Health Policy, Thomas Jefferson University Hospital, at the address indicated. A certificate of completion will be mailed within six to eight weeks of receipt of your exam/payment. (A minimum test score of 70% is required.) Multiple Choice Select the one correct answer. 1. Sickle cell disease is an inherited blood disorder that: a. affects one of every 1,000 to 1,400 African-American births. b. involves episodes of pain, chronic hemolytic anemia, and severe infections. c. is not a commonly inherited blood disorder in the U.S. d. causes complications only in adulthood. The pathogenesis of sickle cell disease does not include: a. sickling of red blood cells when deoxygenated. b. premature hemolysis of red blood cells. c. obstruction of microvasculature resulting from sickling. d. a change in the alpha-globin polypeptide of the hemoglobin molecule. Bone marrow transplantation is the only cure for sickle cell disease, but it is not a common procedure because of: a. mortality rates exceeding 30%. b. a lack of suitable donors. c. graft failure rates below 50%. d. disease-free survival rates below 30%. Management of sickle cell disease with hydroxyurea: a. may result in reversible bone marrow suppression. b. may be indicated for patients who have had at least three pain crises in the previous three months. c. may prevent complications of sickle cell disease by decreasing polymerization time. d. does not require monitoring complete blood counts. According to the authors, which of the following should be avoided in patients with pain and impaired renal function? a. morphine b. codeine c. hydromorphone d. meperidine 6. According to authors, the following statements about infection in patients with sickle cell disease are true except: a. Prophylaxis of infection may be discontinued after five years of age. b. Prophylaxis with penicillin is a problem. c. Erythromycin ethyl succinate is not adequate for prophylaxis. d. Functional asplenia can occur as early as six months of age. Iron overload is a complication of the regularly needed transfusions for the treatment of anemia in patients with sickle cell disease. Which of the following medications was found to be safe and effective compared with deferoxamine in the treatment of iron overload? a. folic acid b. vitamin B12 c. deferasirox d. none of the above A deficiency of glutamine, a consequence of sickle cell anemia, may result in the possibility of: a. skeletal muscle wasting. b. immunosuppression. c. improved wound healing. d. all of the above Folic acid is well supported in the primary literature for use in sickle cell disease. a. True b. False 7. 2. 3. 8. 4. 9. 5. 10. In the treatment of dehydration in sickle cell disease, oral magnesium showed considerable: a. decreases in sickle erythrocyte magnesium. b. increases in sickle erythrocyte potassium content. c. increases in the number of dense sickle erythrocytes. d. none of the above Vol. 33 No. 4 • April 2008 • P&T® 245
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