Nursing Elites

1. The parents of a 3-year-old boy with Duchenne muscular dystrophy (DMD) ask, 'How can our son have this disease? We are wondering if we should have any more children.' What information should the nurse provide these parents?

• A. This is an inherited X-linked recessive disorder, which primarily affects male children in the family.
• B. The male infant had a viral infection that went unnoticed and untreated, leading to muscle damage.
• C. The lack of the protein dystrophin in the mother can impact the XXX muscle groups of males.
• D. Spinal cord damage due to birth trauma during a breech vaginal birth weakens the muscles.

Correct answer: A

Rationale: The correct answer is A. Duchenne muscular dystrophy is an X-linked recessive disorder, meaning the gene mutation causing DMD is located on the X chromosome. Males have one X chromosome and one Y chromosome, so if the X chromosome they inherit from their mother carries the mutated gene, they will develop DMD. Females have two X chromosomes, so they are carriers of the gene but are usually not affected by the disease. Therefore, the nurse should explain to the parents that DMD is an inherited X-linked recessive disorder that primarily affects male children in the family.

2. A 5-year-old child is admitted to the pediatric unit with fever and pain secondary to a sickle cell crisis. Which intervention should the nurse implement first?

• A. Obtain a culture of any sputum or wound drainage
• B. Initiate normal saline IV at 50 ml/hr
• C. Administer a loading dose of penicillin IM
• D. Administer the initial dose of folic acid PO

Correct answer: B

Rationale: In a child with a sickle cell crisis, the priority intervention is to initiate normal saline IV at 50 ml/hr to manage dehydration and help alleviate pain. This intervention helps improve hydration status and supports the circulation of sickled red blood cells, reducing the risk of vaso-occlusive episodes and associated pain. Obtaining a culture of any sputum or wound drainage (Choice A) may be necessary but is not the initial priority. Administering a loading dose of penicillin IM (Choice C) is important but not the first intervention. Administering the initial dose of folic acid PO (Choice D) is beneficial but does not address the immediate need for hydration in a sickle cell crisis.

3. At 40-weeks gestation, a client presents to the obstetrical floor with spontaneous rupture of amniotic membranes at home, in active labor, and feeling the urge to push. What information should the nurse prioritize obtaining?

• A. Color and consistency of fluid.
• B. Estimated amount of fluid.
• C. Any odor noted at the rupture of membranes.
• D. Time of membrane rupture.

Correct answer: A

Rationale: Assessing the color and consistency of amniotic fluid is crucial as it can indicate the presence of meconium, which suggests potential fetal distress. This information guides the need for further assessments and interventions to ensure the well-being of the mother and fetus. Estimating the amount of fluid is not as critical as determining the color and consistency to identify fetal distress. While noting any odor is important, it is secondary to assessing the fluid itself. Knowing the time of membrane rupture is helpful but not as crucial as evaluating the characteristics of the amniotic fluid.

4. In which chromosome pattern is Duchenne disease inherited?

• A. Autosomal dominant
• B. Autosomal recessive
• C. X-linked recessive
• D. Mitochondrial

Correct answer: C

Rationale: Duchenne disease is caused by a mutation in the DMD gene located on the X chromosome, leading to an X-linked recessive inheritance pattern. Males are typically affected by this disorder as they have only one X chromosome, while females are carriers with one normal and one affected X chromosome.

5. A 6-week-old infant diagnosed with pyloric stenosis has recently developed projectile vomiting. Which assessment finding indicates to the nurse that the infant is becoming dehydrated?

• A. Weak cry without tears.
• B. Bulging fontanel.
• C. Visible peristaltic wave.
• D. Palpable mass in the right upper quadrant.

Correct answer: A

Rationale: In infants, a weak cry without tears is a classic sign of dehydration. Tears are produced by the lacrimal glands, and reduced tear production is a result of dehydration. This assessment finding should alert the nurse to the infant's dehydration status, requiring prompt intervention to prevent further complications.

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