The nurse is admitting a child with Werdnig-Hoffmann disease ...

[formula1]

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

Question 2

The nurse is caring for a neonate born with a myelomeningocele. Surgery to repair the defect is scheduled the next day. The most appropriate way to position and feed this neonate is which position?
 
  a. Prone and tube-fed
  b. Prone, head turned to side, and nipple-fed
  c. Supine in an infant carrier and nipple-fed
  d. Supine, with defect supported with rolled blankets, and nipple-fed

[Viet Thy]

Answer to Question 1

ANS: C
Werdnig-Hoffmann disease (spinal muscular atrophy type 1) is the most common paralytic form of floppy infant syndrome (congenital hypotonia). It is characterized by progressive weakness and wasting of skeletal muscle caused by degeneration of anterior horn cells. Kugelberg-Welander disease is a juvenile spinal muscular atrophy with a later onset. Charcot-Marie-Tooth disease is a form of progressive neural atrophy of muscles supplied by the peroneal nerves. Progressive weakness is found of the distal muscles of the arms and feet. Duchenne muscular dystrophy is characterized by muscles, especially in the calves, thighs, and upper arms, which become enlarged from fatty infiltration and feel unusually firm or woody on palpation. The term pseudohypertrophy is derived from this muscular enlargement.

Answer to Question 2

ANS: B
In the prone position, feeding is a problem. The infant's head is turned to one side for feeding. If the child is able to nipple-feed, tube feeding is not needed. Before surgery, the infant is kept in the prone position to minimize tension on the sac and risk of trauma.

[formula1]

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[bbburns21]

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