Pediatricians may receive three credit hours in Category 1 for the American Medical Association's Physician's Recognition Award by reading the material in this issue and successfully answering the quiz below. Follow these simple rules:
1.Read each of the articles carefully. Do not neglect the tables and other illustrative material, as they have been selected to enhance your knowledge and understanding.
2. Following are questions on patient management that have been designed to provide a useful link between the articles in this issue and your everyday practice. Read each question, choose the correct answer, and record your answer on the CME registration form printed at the end of the quiz.
3. Type or print your full name and address (including zip code) and your Social Security number in the spaces provided.
4. Send check or money order ($10) made out to PEDIATRIC ANNALS CME Quiz, with your answer form, to:
501 Madison Avenue
New York, N.Y 10022
5. After your answers have been graded, you will be advised of your score and the answers to any incorrect or missed questions. Review those parts of the article dealing with any questions you answer incorrectly, and read the supplemental materials on this aspect of the subject that are listed in the references in this issue. Unanswered questions will be considered incorrect and so scored. A minimum score of 70 percent must be obtained in order for credits to be awarded.
6. Credits can be awarded only if your answers are received before the deadline indicated on the CME registration form, since after that date correct answers to the quiz will be printed in the magazine.
CME QUIZ: Common Hereditary Blood Disorders
Record your answers on page 48.
Type A Questions. Choose one letter after each of the numbers listed below, and transfer to the coupon. Only one answer is correct.
1. A six-year-old black girl with a diagnosis of sickle-cell disease complains of a severe, continuous pain in the chest The hemoglobin is 5 mg./100 ml., the temperature 39.5°C. It is most likely that the pain
A. Is due to small areas of infarction in the bone or bone marrow.
B. Will last for three or four months.
C. Is a consequence of chelation therapy.
D. Is a psychosomatic complaint not connected with sickle-cell anemia.
2. There is about a _____ percent possibility that this girl will have some type of centralnervous-system manifestation within the next 10 years.
3. Two years before this episode, the girl had dyspnea and a temperature of 38.5°C. She also had pains in the right chest.
A. The most likely diagnosis at that time would have been a pulmonary infection.
B. The most likely diagnosis at that time would have been a pulmonary infarction.
C. The most likely diagnosis at that time would have been a pulmonary embolism.
D. The most likely diagnosis cannot be determined from this limited history.
4. The father of this patient has hemoglobin sickle-cell (Hb SC) disease. From this it can be suspected that
A. The daughter's anemia at the age of 20 will be milder than her father's.
B. The girl is less likely than the father to have sickle retinopathy.
C. The father probably will have a nonpalpable spleen.
D. The father will be more at risk for overwhelming infection than the daughter.
5. Heart complications characterize sickle-cell disease, including
A. Frequent findings of angina pectoris before the age of 20.
B. Myocardial infarction in adolescents.
C. Systolic ejection murmurs in most sicklecell patients.
D. Gross left-atrial enlargement.
6. Leg ulcers are a common complication of which of the following hereditary blood disorders?
A. Willebrand's disease.
B. Hereditary spherocytosis.
C. Thalassemia syndromes.
D. Sickle-cell disease.
7. Sptenectomy is the definitive therapy for
A. Mentzer's syndrome.
B. Hereditary spherocytosis.
C. Sickle-cell disease.
D. Vaso-occlusive crises.
8. Thalassemia, sideroblastic anemia, and irondeficiency anemia are all hypochromic microcytic anemias that affect children. Thalassemia syndromes differ from the other two in that
A. There is a defect in heme production.
B. There is a defect in heme synthesis.
C. There is a defect in globin-chain production.
D. Red cells in peripheral blood are 10 to 12 microns in diameter.
9. The liver is one of the first organs affected in beta-thalassemia, and patients have significant endocrine abnormalities. The majority of deaths reported for thalassemia victims are due to
B. Cardiac dysfunction.
C. Endocrine failure.
D. Uncontrolled gastric hemorrhage.
10. A two-year-old child has had the "definitive" surgical treatment for hereditary spherocytosis mentioned in this issue. This treatment, however will not
A. Alleviate aplastic crises.
B. Lessen the child's susceptibility to infections.
C. Reduce the frequency of cholelithiasis.
D. Reduce the jaundice.
11. A 13-year-old boy is jaundiced and anemic. Both parents are hematologically normal. His mean cell hemoglobin concentration is 36 gm./100 ml., while the mean cell volume is slightly decreased. On the basis of - the above, you suspect strongly that the patient has
A. Thalassemia major.
B, Sickle-cell trait.
C. Sickle-cell disease.
D. Hereditary spherocytosis.
Type B Questions. In the following questions, one or more answers may be correct. Answer:
If, 1, 2, and 3
If 1 and 3
If 2 and 4
If only 4
12. A five-year-old black boy is admitted to the hospital because of a 390C. temperature and a severe pain in his right knee. A diagnosis of sickle-cell disease is made in the emergency room. Both parents are black and asymptomatic. If the above diagnosis is correct, one would expect that
1. The child's condition will be more severe in Denver than in New York.
2. The child will become worse as he gets older and sickling crises continue periodically.
3. The child will be unusually susceptible to overwhelming infection.
4. The child will gain unusual weight during adolescence.
13. Surgery poses several problems for sicklecell disease patients, but
1. Bone-marrow transplantation is often advisable.
2. Cholecystectomy may be advisable if the patient has had at least one episode of acute cholecystitis.
3. Splenectomy should be helpful in improving the rate of hemolysts.
4. Attendant risks may be reduced by maintaining adequate oxygénation and avoiding use of tourniquets.
14. A three-month-old infant is anemic, and you suspect from her background that she might be at risk for beta-thalassemia. If your suspicion is correct,
1 . Red cells in the peripheral blood will be abnormally small.
2. Red cells in the peripheral blood wil be abnormally large.
3. There will be abnormally low hemoglobin content.
4. Red cells in the peripheral blood will have a sickle shape.
15. While working at a clinic, you notice a fiveyear-old boy with maxillary hyperplasia and a significant dental abnormality. He is anemic and small for his age.
1 . These are signs of hereditary spherocytosis.
2. These are signs of prolonged sickle-cell crisis.
3. You could expect from these signs that his mean cell hemoglobin concentration will be above normal.
4. You can expect that his liver and spleen may be massively enlarged.
QUIZ ANSWERS WILL APPEAR IN A SUBSEQUENT ISSUE ANSWERS TO THE CME QUIZ IN THE MAY ISSUE
Computed Tomography and Ultrasound