the parents of a newborn with phenylketonuria pku need help and support in adhering to specific dietary restrictions they ask the nurse how long will
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HESI LPN

Pediatric HESI Practice Questions

1. The parents of a newborn with phenylketonuria (PKU) need help and support in adhering to specific dietary restrictions. They ask the nurse, “How long will our child have to be on this diet?” How should the nurse respond?

Correct answer: D

Rationale: The correct answer is D: “This is a lifelong problem, and it is recommended that dietary restrictions must be continued.” Phenylketonuria (PKU) is a metabolic disorder where the body cannot process phenylalanine properly. The diet for PKU must be continued lifelong to prevent cognitive and developmental issues, as phenylalanine buildup can cause irreversible damage. Choice A is incorrect because the nurse should provide information about the lifelong nature of the dietary restrictions for PKU. Choice B is incorrect as it suggests reintroducing protein-containing foods, which is not recommended for individuals with PKU. Choice C is incorrect as it underestimates the duration of the necessary dietary restrictions for PKU.

2. After a discussion with the healthcare provider, the parents of an infant with patent ductus arteriosus (PDA) ask the nurse to explain once again what PDA is. How should the nurse respond?

Correct answer: D

Rationale: Patent ductus arteriosus (PDA) is an abnormal connection between the pulmonary artery and the aorta. In a fetus, the ductus arteriosus is a normal connection between these two vessels, allowing blood to bypass the lungs. However, it should close shortly after birth. When it remains open (patent), it leads to PDA. Choices A, B, and C do not accurately describe PDA. Choice A describes an enlarged aorta, choice B describes a ventricular septal defect, and choice C describes pulmonary stenosis, which are different cardiac conditions.

3. The nurse is assessing an infant and notes that the infant's urine has a mousy or musty odor. What would the nurse suspect?

Correct answer: C

Rationale: The correct answer is C: Phenylketonuria (PKU). PKU is suggested by a mousy or musty odor of the urine, which is caused by the inability to metabolize phenylalanine. Choice A, Maple syrup urine disease, is characterized by a sweet-smelling urine. Choice B, Tyrosinemia, typically presents with cabbage-like odor in the urine. Choice D, Trimethylaminuria, is associated with a fishy odor in the urine, breath, and sweat.

4. A healthcare provider is assessing a child with suspected rheumatic fever. What clinical manifestation is the provider likely to observe?

Correct answer: D

Rationale: Severe joint pain is a classic symptom of rheumatic fever, resulting from inflammation of the joints. Rheumatic fever primarily affects the joints, heart, skin, and the central nervous system. Jaundice (Choice A) is not typically associated with rheumatic fever. Peeling skin on the hands and feet (Choice B) is more characteristic of conditions like Kawasaki disease. While high fever (Choice C) can be present in rheumatic fever, it is not as specific or characteristic as severe joint pain.

5. A healthcare professional is teaching a class of new parents about how to position their infants during the first few weeks of life. Which position is safest?

Correct answer: A

Rationale: The correct answer is 'On the back, lying flat.' Placing infants on their back to sleep is recommended to reduce the risk of sudden infant death syndrome (SIDS). This position promotes safe sleep practices and helps prevent accidental suffocation. Choices B, C, and D are incorrect as placing infants on their side or with the head slightly elevated may increase the risk of breathing difficulties or other hazards during sleep.

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