The following syndromes are caused by chromosomal abnormalities:
- A. Cri-du-chat syndrome
- B. Kearns-Sayre syndrome
- C. Marfan's syndrome
- D. Turner's syndrome
Correct Answer: D
Rationale: Turner's syndrome: Turner's syndrome is caused by a monosomy of the X chromosome, characterized by short stature, infertility, and heart defects.
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A 5-month-old previously well infant is found to have a loud holosystolic murmur (4/6) at the left sternal border. The first and second heart sounds are normal; there is no tachycardia, rumble, or gallop; and hepatomegaly is not noted. The child feeds well and has grown adequately. You suspect
- A. spontaneous closure is more common in muscular vs. membranous defects
- B. closure usually occurs in the first 2 years of life
- C. there is no risk of endocarditis
- D. pulmonary pressures are normal
Correct Answer: C
Rationale: Small VSDs still carry a risk of endocarditis despite being asymptomatic.
Routine hearing tests in the community are usually based on:
- A. Distraction testing
- B. Auditory brainstem responses
- C. Evoked potentials
- D. Object discrimination
Correct Answer: A
Rationale: Distraction testing is a common method used in community settings to assess hearing in young children.
Hypertrophic obstructive cardiomyopathy
- A. Causes LV outflow tract obstruction
- B. Occurs after adenoviral infection
- C. Commonly occurs in infancy
- D. Double apical impulse
Correct Answer: A
Rationale: Hypertrophic obstructive cardiomyopathy primarily causes left ventricular outflow tract obstruction.
Indomethacin is being given to an infant with a patent ductus arteriosus in an attempt to promote closure of the PDA. The nurse caring for this infant becomes concerned about adverse side effects when noticing:
- A. decreased urine output, decreased platelets, and abdominal distention.
- B. increased blood pressure, tachycardia, and decreased oxygen requirements.
- C. increased urine output, increased white blood cell count, and increased reticulocyte count.
- D. Jaundice, pallor, and a petechial rash
Correct Answer: A
Rationale: Indomethacin can cause renal side effects, including decreased urine output, as well as hematologic effects such as decreased platelets, and gastrointestinal effects like abdominal distention, which are concerning adverse effects in an infant.
Hemodynamic definition of 'Isolated post-capillary Pulmonary Hypertension' is
- A. Pulmonary artery wedge pressure > 15 mm Hg
- B. Right ventricular systolic pressure > 36 mm Hg
- C. Diastolic pressure gradient < 7 mm Hg
- D. Pulmonary vascular resistance > 3 Wood units
Correct Answer: A
Rationale: Isolated post-capillary pulmonary hypertension is defined by a pulmonary artery wedge pressure greater than 15 mm Hg.