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What is ONE gene mutation typically associated with Marfan’s syndrome?
- A. FBN1
- B. ACTA2
- C. SIX2
- D. TGFBR2
Correct Answer: A
Rationale: The FBN1 mutation is the most commonly identified genetic mutation associated with Marfan's syndrome.
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Maltese crosses' are identified in the urinary sediments in
- A. Niemann Pick disease
- B. Gaucher disease
- C. Krabbe's disease
- D. Fabry disease
Correct Answer: D
Rationale: Fabry disease is characterized by the presence of 'Maltese crosses' in urinary sediments due to glycosphingolipid accumulation.
A nurse assesses a client who is recovering from a myocardial infarction. The client’s pulmonary artery pressure reading is 25/12 mm Hg. Which action should the nurse take first?
- A. Compare the results with previous pulmonary artery pressure readings.
- B. Increase the intravenous fluid rate because these readings are low.
- C. Immediately notify the health care provider of the elevated pressures.
- D. Document the finding in the client’s chart as the only action.
Correct Answer: A
Rationale: Comparing the current pulmonary artery pressure readings with previous ones helps determine if the values are stable or changing, which guides further intervention.
Which defect results in increased pulmonary blood flow?
- A. Pulmonic stenosis
- B. Tricuspid atresia
- C. Atrial septal defect
- D. Transposition of the great arteries
Correct Answer: C
Rationale: Atrial septal defect results in increased pulmonary blood flow. Blood flows from the left atrium (higher pressure) into the right atrium (lower pressure) and then to the lungs via the pulmonary artery. Pulmonic stenosis is an obstruction to blood flowing from the ventricles. Tricuspid atresia results in decreased pulmonary blood flow. Transposition of the great arteries results in mixed blood flow.
The differential diagnosis of the patient includes all of the following EXCEPT
- A. rheumatic fever
- B. SLE
- C. chronic atrial fibrillation
- D. postmeningococcal immune complexes
Correct Answer: C
Rationale: Chronic atrial fibrillation is unlikely in an 8-year-old with acute onset symptoms.
When planning care for a 8-year-old boy with Down syndrome, the nurse should:
- A. Plan interventions according to the developmental level of a 7-year-old child because that’s the child’s age
- B. Plan interventions according to the developmental levels of a 5-year-old because the child will have developmental delays
- C. Assess the child’s current developmental level and plan care accordingly
- D. Direct all teaching to the parents because the child can’t understand
Correct Answer: C
Rationale: Care should be individualized based on the child’s current developmental level, not just their chronological age or assumptions about delays.