ATI RN
Endocrine System Pediatrics Questions
Question 1 of 5
All the following are skeletal manifestations of Morquio disease EXCEPT
Correct Answer: D
Rationale: Morquio disease (MPS IV) causes kyphosis (A), genua valga (B), waddling gait (C), and short trunk/neck (D). Bullet-shaped phalanges (E) are typical of Hurler, not Morquio.
Question 2 of 5
The least well-defined long-term sequelae of severe prolonged hypoglycemia is
Correct Answer: B
Rationale: Personality affection (B) is less well-defined as a hypoglycemia sequela compared to cerebral palsy (A), cognitive impairment (C), seizures (D), or autonomic issues (E), which are better documented.
Question 3 of 5
In X-linked adrenoleukodystrophy (ALD), MRI shows characteristic cerebral white matter lesions mainly in
Correct Answer: D
Rationale: ALD’s white matter lesions are characteristically parietooccipital on MRI, reflecting disease progression, distinct from other regions (A, B, C, E).
Question 4 of 5
The MOST common presentation of primary carnitine deficiency is
Correct Answer: B
Rationale: Primary carnitine deficiency most commonly presents with cardiomyopathy from impaired fatty acid transport, more frequent than liver (A), skeletal (C), fibroelastosis (D), or hypoglycemia (E).
Question 5 of 5
The MOST common initial manifestation of X-linked adrenoleukodystrophy (ALD) is
Correct Answer: B
Rationale: Hyperactivity is the most common initial sign of X-linked ALD, often preceding neurological decline, unlike seizures (A), vision (C), pressure (D), or hearing (E).