what should the nurse include in the teaching plan for parents of an infant diagnosed with phenylketonuria pku
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HESI LPN

Pediatric HESI 2024

1. What should be included in the teaching plan for parents of an infant diagnosed with phenylketonuria (PKU)?

Correct answer: A

Rationale: The correct answer is A: 'Mental retardation occurs if PKU is untreated.' Phenylketonuria (PKU) is a metabolic disorder that, if left untreated with dietary management, can lead to severe mental retardation due to the accumulation of phenylalanine. It is crucial for parents to understand the potential consequences of untreated PKU to emphasize the importance of early and consistent treatment. Choice B is incorrect because testing for PKU is typically done through newborn screening shortly after birth, not immediately. Choice C is incorrect as treatment for PKU primarily involves strict dietary management that restricts phenylalanine intake, not lifelong medications. Choice D is incorrect as PKU is inherited in an autosomal recessive pattern, meaning that both parents must pass on a mutated gene for the disorder to manifest.

2. A healthcare professional is reviewing the laboratory report of a child with tetralogy of Fallot that indicates an elevated RBC count. What does the professional identify as the cause of the polycythemia?

Correct answer: B

Rationale: The correct answer is B: Tissue oxygen needs. Polycythemia occurs as the body's response to chronic hypoxia by increasing RBC production to enhance oxygen delivery. In tetralogy of Fallot, a congenital heart defect, the heart's structure causes reduced oxygen levels in the blood. This chronic hypoxia stimulates the bone marrow to produce more red blood cells, leading to an elevated RBC count. Choice A is incorrect as low blood pressure is not directly related to polycythemia in this context. Choice C, diminished iron level, is not the cause of polycythemia in tetralogy of Fallot. Choice D, hypertrophic cardiac muscle, is not the primary cause of the elevated RBC count in this case.

3. What is one of the most important factors that a healthcare professional must consider when parents of a toddler request to be present at a procedure occurring on the hospital unit?

Correct answer: B

Rationale: When parents of a toddler request to be present during a procedure, it is crucial to conduct an individual assessment of the parents. This assessment helps healthcare professionals understand the parents' ability to cope with the procedure, provide support to their child, and ensure a safe and comfortable environment for all involved. Choice A, the type of procedure to be performed, while important, is not the most critical factor in this scenario. Choice C, whether the toddler wants the parents present, is significant but secondary to assessing the parents themselves. Choice D, probable reaction to the toddler’s response to pain, is important but focuses more on the child's reaction rather than the parents' capacity to provide appropriate support.

4. At 0345, you receive a call for a woman in labor. Upon arriving at the scene, you are greeted by a very anxious man who tells you that his wife is having her baby 'now.' This man escorts you into the living room where a 25-year-old woman is lying on the couch in obvious pain. Which of the following statements regarding crowning is true?

Correct answer: D

Rationale: During crowning, it is important to apply gentle pressure to the baby's head. This helps to prevent rapid delivery, which can lead to tearing and other complications for both the mother and the baby. Applying pressure also helps to control the delivery process, ensuring a safer and more controlled birth. Choices A, B, and C are incorrect because crowning does not signify the end of the second stage of labor, does not always occur immediately after the amniotic sac ruptures, and it is not safe to transport the patient during crowning, especially if the hospital is nearby, as rapid delivery can occur.

5. When planning the discharge of a child who had surgery for a congenital heart defect, what is an important aspect of the discharge teaching?

Correct answer: D

Rationale: Explaining the use of prescribed medications is crucial because it helps ensure proper management of the child’s condition after discharge. While teaching the parents about signs of infection and providing instructions on wound care are important aspects of postoperative care, they are not as critical as ensuring the correct understanding and administration of prescribed medications. Scheduling follow-up appointments is also important but does not directly impact the immediate post-discharge care and medication adherence.

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