Question 1

A young boy has just been diagnosed with pseudohypertrophic (Duchenne)muscular dystrophy.What should the management plan include?&#10;A) Recommend genetic counselling.&#10;B) Explain that the disease is easily treated.&#10;C) Suggest ways to limit the use of his muscles.&#10;D) Assist the family in finding a nursing facility to provide his care.

Accepted Answer

Pseudohypertrophic (Duchenne's)muscular dystrophy is inherited as an X-linked recessive gene.Genetic counselling is recommended for parents,female siblings,maternal aunts,and their female offspring.No effective treatment exists at this time for childhood muscular dystrophy.Maintaining optimal function of all muscles for as long as possible is the primary goal.It has been found that children who remain as active as they possibly can are able to avoid wheelchair confinement for a longer time.Assisting the family in finding a nursing facility for the boy is inappropriate at the time of diagnosis.When the child becomes increasingly incapacitated,the family may consider home-based care,a skilled nursing facility,or respite care to provide the necessary care.

Question 2

What is the most common problem for children born with a myelomeningocele?&#10;A) Neurogenic bladder&#10;B) Cognitive impairment&#10;C) Respiratory compromise&#10;D) Cranioschisis

Accepted Answer

Myelomeningocele is one of the most common causes of neuropathic (neurogenic)bladder dysfunction among children.Risk of cognitive impairment is minimized through early intervention and management of hydrocephalus.Respiratory compromise is not a common problem in myelomeningocele.Cranioschisis is a skull defect through which various tissues protrude and is not associated with myelomeningocele.

Question 3

The parents of a child with cerebral palsy ask the nurse if any drugs can decrease their child's spasticity.What knowledge is the basis of the nurses' response?&#10;A) Anticonvulsant medications are sometimes useful for controlling spasticity.&#10;B) Medications that would be useful in reducing spasticity are too toxic for use in children.&#10;C) Many different medications can be highly effective in controlling spasticity.&#10;D) A pump can be implanted that delivers medication into the intrathecal space to decrease spasticity.

Accepted Answer

Baclofen given intrathecally is best suited for children with severe spasticity that interferes with activities of daily living and ambulation.Anticonvulsant medications are used when seizures occur in children with cerebral palsy.The intrathecal route decreases the adverse effects of the drugs that reduce spasticity.Few medications are presently available for the control of spasticity.

Question 4

What term is used for a hernial protrusion of a saclike cyst of meninges,spinal fluid,and a portion of the spinal cord with its nerves through a defect in the vertebral column?&#10;A) Rachischisis&#10;B) Encephalocele&#10;C) Meningocele&#10;D) Myelomeningocele

Accepted Answer

A myelomeningocele is a visible defect with an external,saclike protrusion containing meninges,spinal fluid,and nerves.Rachischisis is a fissure in the spinal column that leaves the meninges and spinal cord exposed.Encephalocele is a herniation of the brain and meninges through a defect in the skull,producing a fluid-filled sac.Meningocele is a hernial protrusion of a saclike cyst of meninges with spinal fluid but no neural elements.

Question 5

A 14-year-old girl is in the critical care unit after a spinal cord injury 2 days ago.Which should be included in nursing care for this child?&#10;A) Avoiding the use of diazepam.&#10;B) Administering methylprednisone.&#10;C) Minimizing environmental stimuli.&#10;D) Discussing long-term care issues with the family.

Accepted Answer

Corticosteroids are administered to minimize the inflammation present at the injury site.Patients with spinal cord injury are physiologically labile,so close monitoring is required.Diazepam is the medication of choice for seizure control and muscle relaxation;therefore,it should not be avoided.Minimizing environmental stimuli and discussing long-term care issues with the family actions do not apply to providing care for this patient.

Question 6

Which supplement is recommended to prevent neural tube defects?&#10;A) Vitamin A throughout pregnancy&#10;B) Multivitamin preparations as soon as pregnancy is suspected&#10;C) Folic acid for all women of child-bearing age&#10;D) Folic acid during the first and second trimesters of pregnancy

Accepted Answer

The widespread use of folic acid among women of childbearing age is expected to decrease the incidence of spina bifida significantly.Vitamin A does not have a relation to the prevention of spina bifida.Folic acid supplementation is recommended for the preconception period and during the pregnancy,but only 42% of women actually follow these guidelines.

Question 7

Which set of symptoms are characteristic of spastic cerebral palsy?&#10;A) Hypertonicity and poor control of posture,balance,and coordinated motion&#10;B) Athetosis and dystonic movements&#10;C) Wide-based gait and poor performance of rapid,repetitive movements&#10;D) Tremors and lack of active movement

Accepted Answer

Hypertonicity,poor control of posture,balance,and coordinated motion are part of the classification of spastic cerebral palsy.Athetosis and dystonic movements are part of the classification of dyskinetic/athetoid cerebral palsy.Wide-based gait and poor performance of rapid,repetitive movements are part of the classification of ataxic cerebral palsy.Tremors and lack of active movement may indicate other neurological disorders.

Question 8

When assessing a child for botulism,which are clinical manifestations? Select all that apply.Express answer in small letters followed by a comma and a space-e.g. ,a,b,c&#10;A) Scotoma&#10;B) Weakness&#10;C) Headache&#10;D) Vomiting&#10;E) Diarrhea&#10;F) Exaggerated gag reflex

Accepted Answer

Clinical manifestations of botulism include weakness,headache,diplopia,and vomiting.With infants,they also experience constipation and a reduced gag reflex.

Question 9

How much folic acid is recommended as a daily dose for women of child-bearing age?&#10;A) 0.1 mg&#10;B) 0.4 mg&#10;C) 1.5 mg&#10;D) 2 mg

Accepted Answer

It has been estimated that a daily intake of 0.4 mg of folic acid in women of child-bearing age will prevent 50 to 70% of cases of neural tube defects.A 0.1 mg dose is too low,and 1.5 and 2 mg are not recommended dosages of folic acid.

Question 10

What clinical manifestation is suggestive of spinal muscular atrophy (Werdnig-Hoffmann disease)in an infant?&#10;A) Hyperactive deep tendon reflexes&#10;B) Hypertonicity&#10;C) Lying in the frog position&#10;D) Motor deficits on one side of body

Accepted Answer

The infant lies in the frog position with the legs externally rotated,abducted,and flexed at knees.Deep tendon reflexes are absent.The child has hypotonia and inactivity as the most prominent features.Motor deficits are bilateral.

Question 11

The nurse is talking to a parent with a child who has a latex allergy.Which statement by the parent indicates she correctly understands the teaching?&#10;A) &#34;My child will have an allergic reaction if he comes in contact with yeast products.&#34;&#10;B) &#34;My child may have an upset stomach if he eats foods made with wheat or barley.&#34;&#10;C) &#34;My child will probably develop an allergy to peanuts.&#34;&#10;D) &#34;My child should not eat bananas or kiwis.&#34;

Accepted Answer

The answer of The nurse is talking to a parent...

Question 12

A latex allergy is suspected in a child with spina bifida.Which is an appropriate nursing intervention?&#10;A) Avoid using any latex product.&#10;B) Use only nonallergenic latex products.&#10;C) Administer medication for long-term desensitization.&#10;D) Teach the family about long-term management of asthma.

Accepted Answer

The answer of A latex allergy is suspected in a...

Question 13

The nurse is caring for an infant born with a myelomeningocele.Surgery to repair the defect is scheduled for the next day.What is the most appropriate way to position and feed this newborn?&#10;A) Left lateral and tube feed&#10;B) Prone,turn head to side,and nipple feed&#10;C) Supine in infant carrier and nipple feed&#10;D) Supine,with defect supported with rolled blankets,and nipple feed

Accepted Answer

The answer of The nurse is caring for an infant...

Question 14

Which problem is most often associated with a myelomeningocele?&#10;A) Hydrocephalus&#10;B) Craniosynostosis&#10;C) Biliary atresia&#10;D) Esophageal atresia

Accepted Answer

The answer of Which problem is most often associated with...

Deck 54: Neuromuscular or Muscular Dysfunction