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
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Nursing Elites

HESI RN

Pediatric HESI

1. What information should the nurse provide to parents of a 3-year-old boy with Duchenne muscular dystrophy who inquire about the disease and future children?

Correct answer: A

Rationale: Duchenne muscular dystrophy is an X-linked recessive disorder caused by mutations in the DMD gene on the X chromosome. This disorder primarily affects males because they have one X chromosome, inherited from their mothers, who may be carriers of the mutated gene. Females have two X chromosomes, providing a protective effect as the normal gene on one X chromosome can compensate for the mutated gene on the other. Therefore, the nurse should explain to the parents that Duchenne muscular dystrophy is an inherited X-linked recessive disorder, which is why their son has the disease and why there is a risk of passing it on to future sons. Choice B is incorrect as it inaccurately implies that the lack of dystrophin in mothers impacts their sons' muscle groups. Choice C is incorrect as it suggests a viral infection caused the muscle damage, which is not the case with Duchenne muscular dystrophy. Choice D is incorrect as it attributes the muscle weakness to birth trauma instead of the genetic nature of the disorder.

2. The healthcare provider is assessing an infant with pyloric stenosis. Which pathophysiological mechanism is the most likely consequence of this infant's clinical picture?

Correct answer: A

Rationale: Pyloric stenosis leads to obstruction at the outlet of the stomach, causing frequent vomiting and loss of stomach acids. This results in a loss of hydrochloric acid and hydrogen ions, leading to metabolic alkalosis due to an increase in serum bicarbonate levels. Therefore, the correct answer is metabolic alkalosis. Choice B, respiratory acidosis, is incorrect as it is not typically associated with pyloric stenosis. Choice C, metabolic acidosis, is incorrect because the loss of stomach acids in pyloric stenosis leads to metabolic alkalosis, not acidosis. Choice D, respiratory alkalosis, is also incorrect as it is not the usual consequence of pyloric stenosis.

3. The nurse is assessing a 4-month-old infant who has just received routine immunizations. The mother reports that the baby has been fussy and has a low-grade fever since the immunizations. What is the best response by the nurse?

Correct answer: A

Rationale: The correct response by the nurse is to reassure the mother that fussiness and low-grade fever are common side effects of immunizations in infants and should resolve within a few days. It is essential to educate the mother about these expected reactions to alleviate her concerns. Choice B is incorrect because allergic reactions to immunizations usually present with more severe symptoms such as difficulty breathing or swelling. Choice C is not warranted unless there are concerning symptoms present. Choice D is inappropriate as aspirin is contraindicated in infants due to the risk of Reye's syndrome.

4. Which statement by a school-aged client going to summer camp indicates the best understanding of the mode of transmission of Lyme disease?

Correct answer: D

Rationale: The correct answer is D. Wearing long sleeves and pants is an effective measure to prevent tick bites, which can transmit Lyme disease. Ticks are commonly found in wooded areas and tall grass, so covering exposed skin helps reduce the risk of tick bites and subsequently lowers the risk of contracting Lyme disease. Choices A, B, and C do not directly address the mode of transmission of Lyme disease. Option A pertains to respiratory protection against dust, Option B focuses on wound care, and Option C relates to water safety, none of which are directly related to preventing Lyme disease transmission.

5. A 9-year-old child is brought to the clinic with a fever, rash, and swollen joints. The nurse notes that the child had a sore throat two weeks ago that was not treated. What condition should the nurse suspect?

Correct answer: B

Rationale: In this scenario, the child's symptoms of fever, rash, and swollen joints following an untreated sore throat two weeks ago are indicative of rheumatic fever. Rheumatic fever can develop as a complication of untreated streptococcal infections, leading to systemic inflammation and affecting various organs, including the joints. This condition manifests with symptoms such as fever, rash, and swollen joints, aligning with the child's presentation in this case. Scarlet fever typically presents with a sandpapery rash and strawberry tongue but does not involve joint inflammation. Kawasaki disease presents with fever, rash, and mucous membrane changes but does not typically involve joint swelling. Juvenile rheumatoid arthritis can cause joint swelling but is not directly linked to a recent untreated sore throat.

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