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List THREE indications for intervention for aortopathy in Marfan’s syndrome in an older teenager.
- A. Aortic root or ascending aorta dilation >44-50mm
- B. Symptomatic (pain consistent with rupture)
- C. Growth of >0.5cm/y
- D. All of the above
Correct Answer: D
Rationale: These are significant indicators that require surgical intervention to prevent further complications, such as rupture or dissection.
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Recognised features of ABO incompatibility include:
- A. Normal haemoglobin on day 1
- B. Worsening with subsequent pregnancies
- C. Conjugated hyperbilirubinaemia
- D. Negative Coombs test
Correct Answer: A
Rationale: In ABO incompatibility, haemoglobin levels are typically normal on day 1. The condition does not worsen with subsequent pregnancies and is associated with unconjugated hyperbilirubinaemia.
Which of the following features favor a diagnosis of restrictive cardiomyopathy over constrictive pericarditis?
- A. Elevated pulmonary artery pressures
- B. Normal BNP levels
- C. Annulus reversus
- D. Ventricular interdependence
Correct Answer: A
Rationale: Restrictive cardiomyopathy is associated with elevated pulmonary artery pressures
A previously well 3½-month-old presents with poor feeding, diaphoresis during feeding, and poor growth. Vital signs reveal respirations of 70, pulse of 175, and blood pressure of 90/65 mm Hg in the upper and lower extremities. The cardiac examination reveals a palpable parasternal lift and a systolic thrill. A grade 4 holosystolic murmur and a mid-diastolic rumble are noted. The chest radiograph reveals cardiomegaly. The most likely diagnosis is
- A. cardiomyopathy
- B. myocarditis
- C. VSD
- D. coarctation of the aorta
Correct Answer: C
Rationale: The presentation suggests a large ventricular septal defect (VSD) causing significant left-to-right shunting and heart failure.
The nurse explained how to position an infant with tetralogy of Fallot if the infant suddenly becomes cyanotic. Which statement by the father leads the nurse to determine he understood the instructions?
- A. If the baby turns blue, I will hold him against my shoulder with his knees bent up toward his chest.
- B. If the baby turns blue, I will lay him down on a firm surface with his head lower than the rest of his body.
- C. If the baby turns blue, I will immediately put the baby upright in an infant seat.
- D. If the baby turns blue, I will put the baby in supine position with his head elevated.
Correct Answer: A
Rationale: In the event of a paroxysmal hypercyanotic or tet spell, the infant should be placed in a knee-chest position.
Features that would increase suspicion of cerebral palsy at age 9 months include:
- A. Presence of Moro reflex
- B. Abductor spasm
- C. Hand dominance
- D. Grasp reflex
Correct Answer: C
Rationale: Hand dominance at 9 months is a red flag for cerebral palsy. The persistence of primitive reflexes like the Moro and grasp reflexes can also be indicative.