the parents of a 3 yo boy who has duchenne muscular dystrophy dmd ask how can our son have this disease we are wondering if we should have any more ch
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Nursing Elites

HESI RN

HESI Practice Test Pediatrics

1. What information should the nurse provide the parents of a 3-year-old boy with Duchenne muscular dystrophy (DMD) who are concerned about having more children?

Correct answer: A

Rationale: The correct answer is A. Duchenne muscular dystrophy is an inherited X-linked recessive disorder that primarily affects male children in the family. Since it is X-linked, sons inherit the mutation from their mothers who are carriers of the abnormal gene. Therefore, the nurse should explain to the parents that any future sons they have would have a 50% chance of inheriting the mutation and having DMD, while daughters would have a 50% chance of being carriers like the mother.

2. 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 children.' What information should the nurse provide to parents?

Correct answer: A

Rationale: The correct answer is A. Duchenne muscular dystrophy is an X-linked recessive disorder that primarily affects males. It is crucial for the nurse to explain to the parents that this condition follows an inheritance pattern where the gene mutation responsible for the disorder is located on the X chromosome. Males have only one X chromosome, so if they inherit the mutated gene, they will develop the disease. Females, on the other hand, have two X chromosomes, providing a backup copy that can compensate for the mutation. Understanding this inheritance pattern is essential for family planning decisions, as the risk of passing on the disorder to future children can be explained based on this genetic inheritance. Choices B, C, and D are incorrect because they do not address the genetic basis of Duchenne muscular dystrophy or its inheritance pattern, which is crucial information for the parents in this scenario.

3. A 14-year-old adolescent with a history of substance abuse is brought to the emergency department by the parents, who suspect that the adolescent has relapsed. What is the nurse’s priority action?

Correct answer: A

Rationale: In this scenario, the nurse's priority action should be to obtain a urine sample for toxicology screening. This step is crucial in identifying the substances involved in the relapse, which will guide appropriate treatment strategies. By knowing the specific substances, healthcare providers can tailor treatment plans effectively. Establishing a therapeutic relationship and discussing treatment options come after obtaining necessary diagnostic information to ensure a comprehensive care plan. Administering a medication to reverse substance effects is not the initial priority; identification of the substances through toxicology screening should precede any treatment intervention.

4. The healthcare professional working on the pediatric unit takes two 8-year-old girls to the playroom. Which activity is best for the healthcare professional to plan for these girls?

Correct answer: A

Rationale: Selecting a board game is the best activity for the healthcare professional to plan for these girls as it is age-appropriate and promotes social interaction. Board games can enhance cognitive skills, encourage teamwork, and provide an enjoyable and interactive experience for the girls.

5. A 9-week-old infant is scheduled for cleft lip repair. Which information is most important for the nurse to convey to the surgeon before transporting the infant to the surgical suite?

Correct answer: A

Rationale: The correct answer is A because a low red blood cell count may indicate anemia, which could pose risks during surgery. Anemia can affect oxygen delivery to tissues, impacting wound healing and overall surgical outcomes. The other options, such as white blood cell count, weight gain, and urine specific gravity, are less critical for immediate surgical considerations. White blood cell count is more related to infection risk postoperatively rather than immediate surgical risk. Weight gain reflects good overall growth but does not impact the immediate surgical situation. Urine specific gravity is more indicative of hydration status rather than immediate surgical risk.

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