ATI RN
Pediatric Endocrine Exam Questions Questions
Question 1 of 5
Irreversible complication of untreated galactosemia is
Correct Answer: D
Rationale: Untreated galactosemia causes irreversible intellectual disability from brain damage, more permanent than cataracts (B), sepsis (A), or organ injuries (C, D), which may be treatable.
Question 2 of 5
In mucopolysaccharidosis, disproportionate severe CNS involvement with mild physical features is unique to
Correct Answer: D
Rationale: Sanfilippo disease (MPS III) uniquely features severe CNS involvement (dementia) with milder somatic findings, unlike others (A-D).
Question 3 of 5
All the following drugs are unsafe in acute intermittent porphyria EXCEPT
Correct Answer: A
Rationale: Nifedipine is safe in AIP, unlike diclofenac (B), cimetidine (C), valproic acid (D), and carbamazepine (E), which induce heme synthesis and exacerbate attacks.
Question 4 of 5
In older infants and children, hypoglycemia is defined as whole blood glucose concentration less than
Correct Answer: D
Rationale: Hypoglycemia in children is defined as blood glucose <60 mg/dL, a standard threshold, unlike lower neonatal values (A-C) or higher normal ranges (E).
Question 5 of 5
The MOST specific and important laboratory finding in X-linked adrenoleukodystrophy (ALD) is elevated level of
Correct Answer: D
Rationale: Elevated very long chain fatty acids (VLCFAs) are the specific diagnostic marker for X-linked ALD, reflecting peroxisomal dysfunction, unlike hormones (A, B, C, E).