a 6 year old child with sickle cell anemia presents to the emergency department with severe pain in the legs and abdomen the child is crying and state
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Nursing Elites

HESI RN

HESI Pediatric Practice Exam

1. A 6-year-old child with sickle cell anemia presents to the emergency department with severe pain in the legs and abdomen. The child is crying and states that the pain is unbearable. What is the nurse’s priority action?

Correct answer: B

Rationale: In a sickle cell crisis, pain management is a priority to alleviate the child's suffering. Administering the prescribed pain medication is crucial to address the severe pain experienced by the child. Warm compresses, encouraging fluid intake, and monitoring oxygen saturation are important interventions but should follow the priority of pain management in this situation.

2. After observing a mother giving her 11-month-old ferrous sulfate followed by two ounces of orange juice, what should the nurse do next?

Correct answer: D

Rationale: Providing positive feedback to the mother for correctly administering the iron supplements is essential as it reinforces proper medication administration practices. This encouragement can help build the mother's confidence and ensure that she continues to administer the supplements correctly in the future, promoting the infant's health and well-being. Choices A, B, and C are incorrect because there is no need to suggest altering the administration method, changing the liquid used, or restricting feeding immediately after administering the iron supplement. Giving positive feedback is the most appropriate action in this scenario to acknowledge the mother's correct administration technique.

3. During a routine assessment of a 3-year-old at a community health center, the healthcare professional should be alert for signs of autism spectrum disorder. Which behavior by the child should prompt further evaluation for a possible autistic spectrum disorder?

Correct answer: A

Rationale: Engaging in odd repetitive behaviors is a hallmark sign of autism spectrum disorder in children. These behaviors can include repetitive movements, insistence on sameness, or specific routines. Recognizing and addressing these behaviors early can help in providing appropriate interventions and support for the child.

4. 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.

5. 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.

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