ATI RN
Pediatric Endocrine Nursing Questions Questions
Question 1 of 5
Matching: 3-Methylglutaconic aciduria type II
Correct Answer: D
Rationale: Type II (Barth syndrome) is X-linked with cardiomyopathy.
Question 2 of 5
Matching: Peroxisomal disorder
Correct Answer: A
Rationale: All listed conditions (e.g., Zellweger) are peroxisomal disorders.
Question 3 of 5
A 7-year-old male previously 'A' student demonstrates progressive hyperactivity is diagnosed with attention deficit hyperactivity disorder. Despite therapy, his school performance deteriorates. He has difficulty understanding people when spoken to on the telephone. Seizures developed 6 months later. Physical examination reveals slight diffuse hyperpigmentation, spasticity, and reduced deep tendon reflexes. The most likely diagnosis is
Correct Answer: D
Rationale: X-linked adrenoleukodystrophy (ALD) causes progressive neurologic decline, seizures, and adrenal insufficiency (pigmentation).
Question 4 of 5
Matching: Lipidosis - Sandhoff
Correct Answer: C
Rationale: Sandhoff disease has both cherry red spot and hepatosplenomegaly.
Question 5 of 5
Treatment of the patient in the previous two questions is best achieved with
Correct Answer: B
Rationale: Enzyme replacement with β-glucosidase (e.g., imiglucerase) treats Gaucher disease.