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. During a follow-up clinical visit, a mother tells the nurse that her 5-month-old son, who had surgical correction for tetralogy of Fallot, has rapid breathing, often takes a long time to eat, and requires frequent rest periods. The infant is not crying while being held, and his growth is in the expected range. Which intervention should the nurse implement?

Correct answer: B

Rationale: Auscultating the heart and lungs while the infant is held can provide important diagnostic information in assessing the cardiac and respiratory status of the infant who had surgical correction for tetralogy of Fallot. This intervention can help the nurse identify any abnormal heart or lung sounds, which may indicate complications or issues that need further evaluation or intervention.

3. The healthcare provider is preparing to administer a vaccine to a 5-year-old child. The child is visibly anxious and asks if the shot will hurt. What is the healthcare provider’s best response?

Correct answer: A

Rationale: When a child expresses fear or anxiety about receiving a vaccination, it is essential for the healthcare provider to provide honest and reassuring information. Choice A acknowledges the potential for some discomfort but also reassures the child that it will be over quickly. This response validates the child's feelings while also preparing them for the procedure. Choices B, C, and D either provide false reassurance, dismiss the child's feelings, or suggest avoidance, which are not appropriate responses in this situation.

4. During a well-baby exam, a nurse finds that a 2-month-old's right testicle is not descended into the scrotum, but the left one is palpable. What should the nurse do?

Correct answer: A

Rationale: The correct answer is to ask if the right testis has been seen in the scrotum before. The initial step in managing an undescended testicle is to determine if it has been previously observed in the scrotum or if this is a new finding. This information is crucial in deciding the next course of action. Choice B is incorrect because addressing future fertility concerns comes after confirming the status of the testicle. Choice C is unnecessary at this stage as the first step is to gather more history. Choice D is unrelated to the issue described and is not indicated in this scenario.

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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