A newborn appears with multiple seizures within the first few hours of life. Multiple anticonvulsant medications have failed to control seizure activities. Most likely enzyme deficiency in this patient is:

Questions 56

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Pediatric Gi Disorders Practice Questions Questions

Question 1 of 9

A newborn appears with multiple seizures within the first few hours of life. Multiple anticonvulsant medications have failed to control seizure activities. Most likely enzyme deficiency in this patient is:

Correct Answer: B

Rationale: Glutamic acid decarboxylase deficiency causes pyridoxine-dependent seizures, resistant to standard anticonvulsants (NORD). A, C-E cause other metabolic issues.

Question 2 of 9

The nurse teaches the new mother about closure of the fontanels. Which of the following statements are correct and should be taught:

Correct Answer: B

Rationale: Anterior fontanel closes between 12-18 months B, per document 9.

Question 3 of 9

A 6-month-old infant presents with hypotonia and tongue fasciculations. EMG shows denervation. Most likely diagnosis is:

Correct Answer: B

Rationale: Spinal muscular atrophy features hypotonia, fasciculations, and denervation, per NORD. A, C-E have distinct findings.

Question 4 of 9

Nurses can prevent evaporative heat loss in the newborn by

Correct Answer: C

Rationale: Drying and skin-to-skin contact C prevent evaporative heat loss, per document 9.

Question 5 of 9

What is the most likely diagnosis for this infant?

Correct Answer: D

Rationale: A 5-day-old with severe heart failure, poor pulses, gallop rhythm, click, and ejection systolic murmur suggests critical aortic stenosis (Choice D). This congenital narrowing obstructs left ventricular outflow, causing heart failure and diminished pulses early in life. Arteriovenous malformation (Choice A) doesn’t cause this cardiac picture. Atrioventricular septal defect (Choice B) presents later with shunts. Coarctation (Choice C) affects lower pulses more. Critical aortic stenosis fits the acute onset and findings.

Question 6 of 9

The most important nursing intervention for sickle cell crisis is

Correct Answer: D

Rationale: Pain relief D is critical in sickle cell crisis, per document p15, 3.

Question 7 of 9

The priority nursing action for a child with a head injury is

Correct Answer: D

Rationale: Stabilizing neck *E* prevents spinal injury, per document p30, 2.

Question 8 of 9

The primary purpose of a mist tent for a child with cystic fibrosis is to

Correct Answer: B

Rationale: Liquefying secretions B aids breathing, per document p23, 9.

Question 9 of 9

Following acute bronchiolitis, which virus is associated with the long-term complication of bronchiolitis obliterans?

Correct Answer: A

Rationale: Bronchiolitis obliterans is a rare, chronic obstructive lung disease following severe lower respiratory infection. Adenovirus (Choice A) is the virus most commonly associated with this complication, particularly after severe bronchiolitis in young children, due to its propensity for persistent airway injury. Human metapneumovirus (Choice B), influenza (Choice C), and parainfluenza (Choice D) can cause bronchiolitis but are less frequently linked to bronchiolitis obliterans. Adenovirus’s strong association makes it the correct answer.

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