a 6 month old infant is diagnosed with cystic fibrosis what explanation should the nurse provide to the parents about this condition
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HESI Pediatrics Quizlet

1. What explanation should the nurse provide to the parents of a 6-month-old infant diagnosed with cystic fibrosis?

Correct answer: A

Rationale: The correct answer is A. Cystic fibrosis is a genetic disorder that primarily affects the respiratory and digestive systems. It results in the production of thick, sticky mucus that can clog the lungs and obstruct the pancreas. This explanation is crucial for parents to understand the impact of the condition on their child's health. Choice B is incorrect because cystic fibrosis is not an autoimmune disorder. Choice C is partially correct in that cystic fibrosis is a genetic disorder, but it requires a comprehensive management approach beyond just medication. Choice D is incorrect as cystic fibrosis is not caused by prenatal exposure to toxins but rather by inheriting specific genetic mutations.

2. A child with a fever is prescribed acetaminophen. What should the caregiver teach the parents about administering this medication?

Correct answer: C

Rationale: The correct answer is to measure the dose with a proper measuring device. Using a household spoon can lead to inaccurate dosing, which can be dangerous. Administering the medication with food or only when the child has a high fever are not the essential instructions related to the safe and effective administration of acetaminophen.

3. A child with a diagnosis of leukemia is admitted to the hospital with a fever. What is the priority nursing intervention?

Correct answer: D

Rationale: The priority nursing intervention for a child with leukemia admitted to the hospital with a fever is to monitor for signs of infection. Children with leukemia are immunocompromised, making them more susceptible to infections. Monitoring for signs of infection helps in early detection and timely intervention, which is crucial in preventing complications. Administering antibiotics (choice A) may be necessary if an infection is suspected or confirmed, but the priority is to assess for signs of infection first. Administering antipyretics (choice B) may help reduce fever, but it does not address the underlying cause, which could be an infection. Providing nutritional support (choice C) is important for overall health but is not the priority when a child with leukemia presents with a fever, as infection needs to be ruled out or managed first.

4. A child with a diagnosis of hemophilia is admitted to the hospital with a bleeding episode. What is the priority nursing intervention?

Correct answer: C

Rationale: The priority nursing intervention for a child with hemophilia experiencing a bleeding episode is administering factor VIII. Hemophilia is a genetic disorder characterized by a deficiency in clotting factors, such as factor VIII. Administering factor VIII replacement therapy is crucial to stop or control bleeding in individuals with hemophilia. Options A, B, and D are important aspects of patient care but do not take precedence over addressing the underlying cause of the bleeding in a child with hemophilia, which is the deficiency of factor VIII.

5. 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.

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