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

HESI RN

Pediatric HESI

1. What information should the nurse provide to parents of a 3-year-old boy with Duchenne muscular dystrophy who inquire about the disease and future children?

Correct answer: A

Rationale: Duchenne muscular dystrophy is an X-linked recessive disorder caused by mutations in the DMD gene on the X chromosome. This disorder primarily affects males because they have one X chromosome, inherited from their mothers, who may be carriers of the mutated gene. Females have two X chromosomes, providing a protective effect as the normal gene on one X chromosome can compensate for the mutated gene on the other. Therefore, the nurse should explain to the parents that Duchenne muscular dystrophy is an inherited X-linked recessive disorder, which is why their son has the disease and why there is a risk of passing it on to future sons. Choice B is incorrect as it inaccurately implies that the lack of dystrophin in mothers impacts their sons' muscle groups. Choice C is incorrect as it suggests a viral infection caused the muscle damage, which is not the case with Duchenne muscular dystrophy. Choice D is incorrect as it attributes the muscle weakness to birth trauma instead of the genetic nature of the disorder.

2. A 10-year-old girl who has had type 1 diabetes mellitus (DM) for the past two years tells the nurse that she would like to use a pump instead of insulin injections to manage her diabetes. Which assessment of the girl is most important for the nurse to obtain?

Correct answer: A

Rationale: In a patient transitioning to an insulin pump, understanding the quality control process to troubleshoot the pump is crucial for ensuring proper and safe management of diabetes. This knowledge enables the individual to identify and address any issues that may arise with the pump, helping maintain optimal glycemic control and prevent complications. Choices B, C, and D, although important in diabetes management, are not as critical as ensuring the correct understanding of troubleshooting the pump, which directly impacts the girl's ability to effectively use the pump for insulin delivery.

3. The mother of a 14-year old who had a below-the-knee amputation for osteosarcoma tells the nurse that her child is angry and blaming her for allowing the amputation to occur. Which response is best for the nurse to provide?

Correct answer: D

Rationale: Acknowledging the child's anger as part of the coping process helps the mother understand her child's emotional response.

4. A child who is admitted to the hospital with anemia is anxious, fearful, and hyperventilating. The nurse anticipates the child developing which acid-base imbalance?

Correct answer: C

Rationale: In this scenario, the child is hyperventilating, which leads to excessive loss of carbon dioxide. This loss of carbon dioxide causes respiratory alkalosis due to a decrease in the partial pressure of carbon dioxide in the blood. Therefore, the correct answer is respiratory alkalosis. Choices A, B, and D are incorrect. Metabolic acidosis is characterized by a decrease in pH and bicarbonate levels due to conditions like kidney disease. Respiratory acidosis is caused by retention of carbon dioxide, leading to an increase in the partial pressure of carbon dioxide. Metabolic alkalosis results from a loss of acid or an increase in bicarbonate levels.

5. 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.

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