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 child admitted with diabetic ketoacidosis is demonstrating Kussmaul respirations. The nurse determines that the increased respiratory rate is a compensatory mechanism for which acid-base alteration?

Correct answer: D

Rationale: Kussmaul respirations are deep, rapid breathing patterns observed in metabolic acidosis, such as diabetic ketoacidosis. In this condition, the body tries to compensate for the acidic environment by increasing the respiratory rate to eliminate excess carbon dioxide (CO2) and decrease the acid levels, thereby helping to correct the acid-base imbalance. Therefore, the correct answer is metabolic acidosis.

3. What is the priority action for a 2-year-old child with croup presenting with a barking cough and stridor?

Correct answer: C

Rationale: The priority action for a 2-year-old child with croup and stridor is to administer nebulized epinephrine. Nebulized epinephrine helps reduce airway swelling, alleviate symptoms, and improve breathing by causing vasoconstriction and reducing upper airway edema. Administering a corticosteroid may be done but is not the priority in this scenario. Obtaining a throat culture is not necessary for the immediate management of croup. Placing the child in an upright position can aid in breathing but is not the priority action when the child is presenting with stridor.

4. When reviewing developmental changes with the parents of a 6-month-old infant, what information should the practical nurse reinforce?

Correct answer: C

Rationale: The correct answer is C because providing a developmentally safe environment for a 6-month-old infant is crucial as they begin to explore their surroundings more actively. This includes ensuring that the environment is free of hazards and that the infant is supervised to prevent accidents. Choice A is incorrect because self-feeding finger foods may not be developmentally appropriate for a 6-month-old infant. Choice B is incorrect as most infants are able to sit up with support around 6 months of age without the need for specific teaching strategies. Choice D is also incorrect as while appetite changes can occur, explaining a specific increase in appetite over the next 6 months is not a primary focus when discussing developmental changes with parents of a 6-month-old.

5. An infant with tetralogy of Fallot becomes acutely cyanotic and hyperpneic. Which action should the nurse implement first?

Correct answer: C

Rationale: In a situation where an infant with tetralogy of Fallot is acutely cyanotic and hyperpneic, the priority action should be to place the infant in a knee-chest position. This position helps increase systemic vascular resistance, improving pulmonary blood flow and subsequently ameliorating the cyanosis and hyperpnea. It is a non-invasive and effective intervention that can be promptly implemented by the nurse to address the immediate respiratory distress. Administering morphine sulfate (Choice A) is not the priority in this case as it may cause further respiratory depression. Starting IV fluids (Choice B) may not address the immediate cyanosis and hyperpnea. Providing 100% oxygen by face mask (Choice D) can help with oxygenation but may not be as effective as placing the infant in a knee-chest position to improve blood flow dynamics.

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