Quiz 5: Multifactorial congenital disorders

Please choose the one, most correct answer to each question or statement.

1. What is a neural tube defect?
   • An abnormality of the peripheral nerves.
   • A malformation of the neural tube.
   • A narrowing or absence of the neural tube.
   • A form of nerve deafness.
2. Which of the following is an example of a neural tube defect?
   • Meningomyelocoele.
   • Omphalocoele.
   • Microcephaly.
   • All of the above.
3. With anencephaly:
   • The spine is open in the lumbar and sacral region of the back.
   • Only the membranes bulge out of a defect in the vertebral column.
   • Both the membranes and spinal cord bulge out of the defect in the vertebral column.
   • The top of the skull is open and the malformed brain is exposed.
4. Infants with anencephaly usually:
   • Die soon after birth.
   • Are operated on after delivery.
   • Are only referred for surgery when they are able to walk.
   • Survive with blindness and deafness.
5. Which of the following is an open spinal defect?
   • Spina bifida occulta.
   • Encephalocoele.
   • Meningomyelocoele.
   • Hydrocephalus.
6. The birth prevalence of neural tube defects in South Africa is:
   • 1–4/ 100.
   • 1–4/ 1000.
   • 1/ 10 000.
   • 4/ 100 000.
7. In South Africa, neural tube defects are commoner in:
   • Urban areas.
   • Rural areas.
   • Older mothers.
   • Teenage mothers.
8. Most neural tube defects are due to:
   • Autosomal dominant inheritance.
   • Autosomal recessive inheritance.
   • X-linked recessive inheritance.
   • Multifactorial inheritance.
9. Neural tube defects may occasionally be caused by:
   • High doses of folic acid.
   • Smoking cigarettes.
   • Some anticonvulsants.
   • Taking iron supplements during pregnancy.
10. Common complications of encephalocoele include:
    • Intellectual disability.
    • Paralysed legs and bladder incontinence.
    • Meningitis.
    • Kyphosis or scoliosis.
11. Meningomyelocoele commonly results in:
    • Deafness.
    • Club feet.
    • Anencephaly.
    • Blindness.
12. Meningocoele usually presents with:
    • Paralysed legs.
    • A bulge over the midline of the spine.
    • Incontinence of bladder and bowel.
    • Bed sores.
13. A spina bifida occulta may be recognised by:
    • A bulge over the lower spine.
    • A large head at birth.
    • A patch of hair in the midline of the lower back.
    • Club feet.
14. Meningocoele can be corrected with:
    • Surgery.
    • Antibiotics.
    • Neurodevelopmental therapy.
    • Anticonvulsants.
15. To reduce the risk of neural tube defects, supplementation should be given:
    • Three months before and three months after conception.
    • As soon as pregnancy is diagnosed.
    • Only after the first trimester.
    • Throughout the second half of pregnancy.
16. What dietary fortification can reduce the risk of neural tube defects?
    • Vitamin A.
    • Vitamin C.
    • Folic acid.
    • Zinc.
17. What are the risks of a mother having a second child with a neural tube defect?
    • 1%.
    • 5%.
    • 25%.
    • 50%.
18. Which maternal serum screening test helps to identify pregnant women at increased risk of having a child with neural tube defects?
    • Alkaline phosphatase.
    • Glucose 6 phosphate dehydrogenase (G6PD).
    • Alpha-fetoprotein.
    • Ferritin.
19. Ultrasound screening for neural tube defects is best done at:
    • 10 weeks.
    • 18 weeks.
    • 26 weeks.
    • 36 weeks.
20. What is the management of a woman who has a fetus with a neural tube defect?
    • Nothing can be done.
    • Amniocentesis.
    • Induction of labour at 36 weeks for early surgery.
    • Genetic counselling.