ATI RN
Endocrine System Pediatrics Questions
Question 1 of 4
All the following are clinical features of acute intermittent porphyria EXCEPT
Correct Answer: D
Rationale: AIP features neuropathy (A), tachycardia (B), abdominal pain (C), and bladder issues (D). Photosensitivity (E) is absent, typical of cutaneous porphyrias.
Question 2 of 4
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 3 of 4
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 4 of 4
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).