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. What information should be reinforced with the parents of a school-aged child about Reye's syndrome?

Correct answer: C

Rationale: The correct answer is C: 'Avoid giving any medication containing aspirin during a viral illness.' It is crucial to advise parents to avoid giving any medication containing aspirin during a viral illness to prevent Reye's syndrome. Reye's syndrome is a rare but serious condition linked to the use of aspirin during viral illnesses in children and teenagers. Choices A, B, and D are incorrect because vaccinating against Reye's syndrome is not applicable as there is no specific vaccine for it, keeping the child at home for 2 days after symptoms appear is not a preventive measure for Reye's syndrome, and avoiding citrus juices is not directly related to the prevention of Reye's syndrome.

3. Which nursing diagnosis is a priority for a 4-year-old child diagnosed with nephrotic syndrome?

Correct answer: C

Rationale: In a child with nephrotic syndrome, fluid volume excess is a priority nursing diagnosis due to the risk of edema and related complications. This patient may experience significant fluid retention, leading to edema, hypertension, and potential respiratory distress. Monitoring and managing fluid volume excess are crucial in preventing further complications and supporting the child's health during nephrotic syndrome. The other options are not the priority in this case. Impaired urinary elimination is not typically a primary concern in nephrotic syndrome. While infection is a risk due to compromised immunity, fluid volume excess poses a more immediate threat to the child's health. Risk for impaired skin integrity may be a concern secondary to edema, but addressing fluid volume excess takes precedence.

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. Which nursing intervention is most important to assist in detecting hypopituitarism and hyperpituitarism in children?

Correct answer: A

Rationale: Recording the height and weight of children is crucial in detecting growth abnormalities like hypopituitarism and hyperpituitarism. Inappropriate growth patterns, such as disproportionate weight gain or stunted height, can be indicative of these conditions. Regular monitoring of height and weight is a fundamental nursing intervention that can aid in the early identification and management of pituitary-related disorders in children.

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