ATI RN
Pediatric Endocrine Nursing Questions Questions
Question 1 of 5
The earliest radiographic sign in Hurler syndrome is
Correct Answer: C
Rationale: Enlarged J-shaped sella is an early radiographic sign in Hurler syndrome from glycosaminoglycan (GAG) accumulation, preceding other skeletal changes (A, B, D, E).
Question 2 of 5
Acute intermittent porphyria (AIP) could be exacerbated by all the following EXCEPT
Correct Answer: D
Rationale: AIP is worsened by surgery (A), hormonal changes (B, C, D), and fasting (opposite of E). Increased carbohydrate intake prevents attacks, making E the exception.
Question 3 of 5
The MOST important screening test of acute intermittent porphyria is
Correct Answer: D
Rationale: Urinary porphobilinogen (PBG) is the key screening test for AIP, elevated during attacks, more specific than other porphyrin tests (A-D).
Question 4 of 5
A 5-year-old child with recurrent attacks of ketotic hypoglycemia. Of the following, the BEST advice to the parents during any intercurrent illness is to
Correct Answer: C
Rationale: High-carbohydrate diet prevents ketotic hypoglycemia during illness by maintaining glucose, more practical than protein (A), ketone testing (B), monitoring (D), or IV glucose (E).
Question 5 of 5
All the following are recognized laboratory findings of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency EXCEPT
Correct Answer: D
Rationale: MCAD deficiency causes hypoglycemia (A), hypoketonuria (B), hypoketonemia (C), and liver enzyme elevation (E) from fasting stress. Metabolic acidosis (D) is less prominent, unlike organic acidemias.