ATI RN
Pediatric Endocrine Exam Questions Questions
Question 1 of 5
A 3-year-old girl presented with growth retardation, recurrent attacks of otitis media, diarrhea, and hypoglycemic seizures. On examination, there is hepatomegaly and her absolute neutrophil count (ANC) is 600/μL. Of the following, the MOST likely diagnosis is
Correct Answer: D
Rationale: GSD Ib causes hypoglycemia, hepatomegaly, and neutropenia (ANC 600) from impaired glucose-6-phosphate transport, unlike other neutropenia causes (A, B, C, E).
Question 2 of 5
Enzyme replacement is the main therapy of Hurler disease, it improve all the following EXCEPT
Correct Answer: D
Rationale: ERT in Hurler syndrome (MPS I) improves growth (A), mobility (B), organomegaly (C), and apnea (E), but cognitive impairment (D) persists due to CNS GAG accumulation.
Question 3 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 4 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 5 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).