11. The nurse is admitting a child with Werdnig-Hoffmann disease (spinal muscular atrophy type 1). Which signs and symptoms are associated with this disease?

a. Spinal muscular atrophy

b. Neural atrophy of muscles

c. Progressive weakness and wasting of skeletal muscle

d. Pseudohypertrophy of certain muscle groups

12. Which clinical manifestations in an infant would be suggestive of spinal muscular atrophy (Werdnig-Hoffmann disease)?

a. Hyperactive deep tendon reflexes

b. Hypertonicity

c. Lying in the frog position

d. Motor deficits on one side of body

13. A 4-year-old child has just been diagnosed with pseudohypertrophic (Duchenne) muscular dystrophy. The management plan should include which action?

a. Recommend genetic counseling.

b. Explain that the disease is easily treated.

c. Suggest ways to limit use of muscles.

d. Assist family in finding a nursing facility to provide his care.

14. Therapeutic management of a child with tetanus includes the administration of:

a. nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation.

b. muscle stimulants to counteract muscle weakness.

c. bronchodilators to prevent respiratory complications.

d. tetanus immunoglobulin therapy.

15. The nurse is conducting reflex testing on infants at a well-child clinic. Which reflex finding should be reported as abnormal and considered as a possible sign of cerebral palsy?

a. Tonic neck reflex at 5 months of age

b. Absent Moro reflex at 8 months of age

c. Moro reflex at 3 months of age

d. Extensor reflex at 7 months of age

16. The nurse is caring for an infant with myelomeningocele scheduled for surgical closure in the morning. Which interventions should the nurse plan for the care of the myelomeningocele sac?

a. Open to air

b. Covered with a sterile moist nonadherent dressing

c. Reinforcement of the original dressing if drainage noted

d. A diaper secured over the dressing

17. The nurse is admitting a school-age child with suspected Guillain-Barré syndrome (GBS). Which is a priority in the care for this child?

a. Monitoring intake and output

b. Assessing respiratory efforts

c. Placing on a telemetry monitor

d. Obtaining laboratory studies

18. The nurse is caring for an intubated infant with botulism in the pediatric intensive care unit. Which health care provider prescriptions should the nurse clarify with the health care provider before implementing?

a. Administer 250 mg botulism immune globulin intravenously (BIG-IV) one time.

b. Provide total parenteral nutrition (TPN) at 25 ml/hr intravenously.

c. Titrate oxygen to keep pulse oximetry saturations greater than 92.

d. Administer gentamicin sulfate (Garamycin) 10 mg per intravenous piggyback every 12 hours.

19. A home care nurse is caring for an adolescent with a T1 spinal cord injury. The adolescent suddenly becomes flushed, hypertensive, and diaphoretic. Which intervention should the nurse perform first?

a. Place the adolescent in a flat right side-lying position.

b. Place a cool washcloth on the adolescent’s forehead and continue to monitor the blood pressure.

c. Implement a standing prescription to empty the bladder with a sterile in and out Foley catheter.

d. Take a full set of vital signs and notify the health care provider.

MULTIPLE RESPONSE

1. A 14-year-old girl is in the intensive care unit after a spinal cord injury 2 days ago. Nursing care for this child includes which action(s)? (Select all that apply.)

a. Monitoring and maintaining systemic blood pressure

b. Administering corticosteroids

c. Minimizing environmental stimuli

d. Discussing long-term care issues with the family

e. Monitoring for respiratory complications

2. Which assessment findings should the nurse note in a school-age child with Duchenne muscular dystrophy (DMD)? (Select all that apply.)

a. Lordosis

b. Gower sign

c. Kyphosis

d. Scoliosis

e. Waddling gait

3. The nurse is conducting discharge teaching to parents of a preschool child with myelomeningocele, repaired at birth, being discharged from the hospital after a urinary tract infection (UTI). Which should the nurse include in the discharge instructions related to management of the child’s genitourinary function? (Select all that apply.)

a. Continue to perform the clean intermittent catheterizations (CIC) at home.

b. Administer the oxybutynin chloride (Ditropan) as prescribed.

c. Reduce fluid intake in the afternoon and evening hours.

d. Monitor for signs of a recurrent urinary tract infection.

e. Administer furosemide (Lasix) as prescribed.

4. Which should the nurse expect to find in the cerebral spinal fluid (CSF) results of a child with Guillain-Barré syndrome (GBS)? (Select all that apply.)

a. Decreased protein concentration

b. Normal glucose

c. Fewer than 10 white blood cells (WBCs/mm3)

d. Elevated red blood cell (RBC) count

5. The nurse in the neonatal intensive care unit is caring for an infant with myelomeningocele scheduled for surgical repair in the morning. Which early signs of infection should the nurse monitor on this infant? (Select all that apply.)

a. Temperature instability

b. Irritability

c. Lethargy

d. Bradycardia

e. Hypertension

1. A toddler is admitted to the hospital with a possible diagnosis of tetanus. The health care provider has prescribed lorazepam (Ativan) intravenously 0.05 mg/kg/dose every 6 hours prn as a muscle relaxant. The child weighs 22 lb. How many milligrams of Ativan should the nurse administer per dose? (Record your answer using one decimal place.)