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. The practical nurse is caring for a child with suspected appendicitis. Which assessment finding should be reported to the healthcare provider immediately?

Correct answer: B

Rationale: Sudden relief of pain in a child with suspected appendicitis should be reported immediately as it may indicate a rupture of the appendix, which is a medical emergency. Sudden relief of pain is concerning because it can be a sign of a perforated appendix, leading to peritonitis and sepsis.

3. 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.

4. A 7-year-old child with leukemia is receiving chemotherapy. The mother asks the practical nurse (PN) how to manage the child's nausea at home. What advice should the PN provide?

Correct answer: A

Rationale: During chemotherapy, children may experience nausea. Providing small, frequent meals can help manage nausea as they are easier to tolerate, reducing the likelihood of vomiting. It is important to offer bland, non-spicy foods to avoid exacerbating nausea. Encouraging large meals less frequently or allowing the child to eat whatever they want may overwhelm the digestive system and worsen nausea. Therefore, the correct advice is to provide small, frequent meals to help the child manage nausea effectively.

5. A 6-year-old child with sickle cell anemia presents to the emergency department with severe pain in the legs and abdomen. The child is crying and states that the pain is unbearable. What is the nurse’s priority action?

Correct answer: B

Rationale: In a sickle cell crisis, pain management is a priority to alleviate the child's suffering. Administering the prescribed pain medication is crucial to address the severe pain experienced by the child. Warm compresses, encouraging fluid intake, and monitoring oxygen saturation are important interventions but should follow the priority of pain management in this situation.

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