which of the following illnesses causes a degeneration of the central nervous system
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HESI Maternal Newborn

1. Which of the following illnesses causes degeneration of the central nervous system?

Correct answer: A

Rationale: Tay-Sachs disease is a genetic disorder that causes a progressive degeneration of the central nervous system, particularly in infants. Choice B, Cystic fibrosis, is a genetic disorder that primarily affects the lungs and digestive system, not the central nervous system. Choices C and D, Turner syndrome and Klinefelter syndrome, are chromosomal disorders that do not directly involve degeneration of the central nervous system.

2. A newborn with a respiratory rate of 40 breaths per minute at one minute after birth is demonstrating cyanosis of the hands and feet. What action should a nurse take?

Correct answer: B

Rationale: Cyanosis of the hands and feet, known as acrocyanosis, is common in newborns shortly after birth and usually resolves on its own. It is not indicative of a need for immediate intervention. Therefore, the appropriate action is to continue monitoring the newborn's condition. Assessing bowel sounds (Choice A) is not relevant to the presenting issue of cyanosis and respiratory rate. Assisting with intubation (Choice C) is an invasive procedure that is not warranted based on the information provided. Rubbing the infant's back (Choice D) is not necessary for acrocyanosis and could potentially disturb the newborn.

3. Why is a client with gestational diabetes being scheduled for an amniocentesis when the fetus has an estimated weight of eight pounds (3629 grams) at 36 weeks gestation? What information is the amniocentesis seeking to obtain?

Correct answer: D

Rationale: An amniocentesis in this scenario is most likely being performed to assess fetal lung maturity. This is necessary when considering early delivery due to macrosomia (large fetal size), which is a common concern in gestational diabetes. Evaluating fetal lung maturity is crucial to determine if the fetus's lungs are developed enough to support breathing independently outside the womb. The presence of a neural tube defect and chromosomal abnormalities are not typically assessed through amniocentesis in this situation, and determining the gender of the fetus is not the primary purpose of the procedure here.

4. A primigravida at 36 weeks gestation who is RH-negative experienced abdominal trauma in a motor vehicle collision. Which assessment finding is most important for the nurse to report to the healthcare provider?

Correct answer: D

Rationale: The correct answer is 'Positive fetal hemoglobin testing' (D). Positive fetal hemoglobin testing (Kleihauer-Betke test) indicates fetal-maternal hemorrhage, which is critical in an RH-negative mother due to the risk of isoimmunization. This condition can lead to sensitization of the mother's immune system against fetal blood cells, potentially causing hemolytic disease of the newborn in subsequent pregnancies. Reporting this finding promptly is crucial for appropriate management and interventions. Choices A, B, and C are not as critical in this scenario. While monitoring fetal heart rate and contractions is important, the detection of fetal-maternal hemorrhage takes precedence due to the serious implications it poses for the current and future pregnancies of an RH-negative mother.

5. Which of the following statements is a symptom of cystic fibrosis in children?

Correct answer: B

Rationale: The correct answer is B. Cystic fibrosis is a genetic disorder that causes the body to produce thick, sticky mucus. This mucus can clog the airways in the lungs and obstruct the pancreas, leading to severe respiratory and digestive problems. Choice A is incorrect because uncontrollable muscle movements and personality changes are not typical symptoms of cystic fibrosis. Choice C is incorrect because cystic fibrosis does not directly cause red blood cells to clump together and obstruct small blood vessels. Choice D is incorrect because cystic fibrosis primarily affects the respiratory and digestive systems, not the central nervous system.

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