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 mother brings her 3-week-old infant to the clinic because the baby vomits after eating and always seems hungry. Further assessment indicates that the infant's vomiting is projectile, and the child seems listless. Which additional assessment finding indicates the possibility of a life-threatening complication?

Correct answer: D

Rationale: Crying without tears is a sign of severe dehydration, which is a potentially life-threatening complication in infants with projectile vomiting. Dehydration can rapidly progress in infants, leading to serious consequences if not promptly addressed. The combination of projectile vomiting, listlessness, and absence of tears when crying should raise concerns about severe dehydration and the need for urgent intervention to prevent further complications.

3. A 3-year-old with a congenital heart defect has had a steady decrease in heart rate, now at 76 bpm from 110 bpm four hours ago. 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. The blood pressure of 70/40 is dangerously low and requires immediate attention to prevent further complications. Oxygen saturation of 94% is within an acceptable range and does not indicate immediate danger. A respiratory rate of 25 breaths/minute is slightly elevated but not a critical finding. Urine output of 20 mL/hr is low but may not be the most concerning finding in this scenario compared to the drop in blood pressure.

4. The caregiver discovers a 6-month-old infant unresponsive and calls for help. After opening the airway and finding the infant is still not breathing, what action should the caregiver take?

Correct answer: C

Rationale: Providing two breaths that make the chest rise is the correct action in this situation. This helps to deliver oxygen to the infant's lungs and body, which is crucial in a situation where the infant is not breathing. Chest rise indicates successful ventilation, and it is an essential step in pediatric resuscitation, especially for infants. Choices A, B, and D are incorrect because palpating the femoral pulse, delivering chest compressions, and feeling the carotid pulse are not the initial actions to take when an infant is not breathing. The priority is to provide effective breaths to establish ventilation.

5. When observing a distraught mother scolding her 3-year-old son for wetting his pants in the hallway of a pediatric unit, what initial action should the nurse take?

Correct answer: B

Rationale: In this situation, the nurse's initial action should be to provide disposable training pants to manage the immediate issue of wetting while also calming the mother. This approach addresses the current distressing situation and offers a practical solution to alleviate the mother's concerns.

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