the parents of a 3 yo boy who has duchenne muscular dystrophy dmd ask how can our son have this disease we are wondering if we should have any more ch
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Nursing Elites

HESI RN

HESI Practice Test Pediatrics

1. What information should the nurse provide the parents of a 3-year-old boy with Duchenne muscular dystrophy (DMD) who are concerned about having more children?

Correct answer: A

Rationale: The correct answer is A. Duchenne muscular dystrophy is an inherited X-linked recessive disorder that primarily affects male children in the family. Since it is X-linked, sons inherit the mutation from their mothers who are carriers of the abnormal gene. Therefore, the nurse should explain to the parents that any future sons they have would have a 50% chance of inheriting the mutation and having DMD, while daughters would have a 50% chance of being carriers like the mother.

2. The heart rate for a 3-year-old with a congenital heart defect has steadily decreased over the last few hours; it is now 76 bpm, whereas the previous reading 4 hours ago was 110 bpm. Which additional finding should be reported immediately to a healthcare provider?

Correct answer: D

Rationale: A significant drop in heart rate and blood pressure should be reported immediately as it may indicate worsening of the congenital heart defect. A decrease in blood pressure to 70/40 is a critical finding that suggests potential cardiovascular compromise and requires urgent attention to prevent further deterioration. Oxygen saturation, respiratory rate, and urine output are important parameters, but in this scenario, the alarming decrease in blood pressure is a more critical finding that necessitates immediate reporting to the healthcare provider.

3. What intervention should the nurse implement first for a male toddler brought to the emergency center approximately three hours after swallowing tablets from his grandmother's bottle of digoxin (Lanoxin)?

Correct answer: A

Rationale: Administering activated charcoal is the priority intervention as it binds with digoxin, preventing further absorption in the gastrointestinal tract. This helps reduce the amount of digoxin available for systemic circulation and minimizes its toxic effects. Gastric lavage is no longer recommended due to potential complications and lack of evidence of efficacy. Obtaining an electrocardiogram may help assess the effects of digoxin toxicity, but it is not the initial priority. IV digoxin immune fab (Digibind) is used in severe cases of digoxin toxicity but is not the first-line intervention.

4. The nurse is conducting an admission assessment of an 11-month-old infant with CHF who is scheduled for repair of restenosis of coarctation of the aorta that was repaired 4 days after birth. Findings include blood pressure higher in the arms than the lower extremities, pounding brachial pulses, and slightly palpable femoral pulses. What pathophysiologic mechanisms support these findings?

Correct answer: B

Rationale: The findings are consistent with coarctation of the aorta, where narrowing of the aorta leads to decreased blood flow to the lower extremities. This results in higher blood pressure in the arms compared to the lower extremities, pounding brachial pulses, and slightly palpable femoral pulses. Choices A, C, and D are incorrect because they do not align with the pathophysiological mechanisms of coarctation of the aorta, which specifically involves narrowing of the aortic lumen reducing blood flow to the lower extremities.

5. The nurse is conducting an admission assessment of an 11-month-old infant with CHF who is scheduled for repair of restenosis of coarction of the aorta that was repaired 4 days after birth. Findings include blood pressure higher in the arms than the lower extremities, pounding brachial pulses, and slightly palpable femoral pulses. What pathophysiologic mechanisms support these findings?

Correct answer: B

Rationale: The correct answer is B. Coarctation of the aorta causes narrowing of the aorta, reducing blood flow to the lower extremities. This narrowing results in higher blood pressure in the arms compared to the lower extremities, along with stronger brachial pulses and slightly palpable femoral pulses. Choices A, C, and D are incorrect because they do not align with the pathophysiological mechanism of coarctation of the aorta, which specifically leads to reduced blood flow to the lower extremities.

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