ATI RN
Pediatric Endocrine Exam Questions Questions
Question 1 of 5
To continue the insulin infusion in treatment of DKA without causing hypoglycemia, glucose must be added to the infusion. We typically recommend that glucose be added as a 10% solution when the serum glucose has decreased
Correct Answer: D
Rationale: Glucose is added when serum glucose falls below 300 mg/dL (E) to prevent hypoglycemia.
Question 2 of 5
Donohue syndrome is characterized by the following EXCEPT
Correct Answer: B
Rationale: Donohue syndrome (leprechaunism) features fasting hyperglycemia (not hypoglycemia, B) due to severe insulin resistance.
Question 3 of 5
All the following are widely used modalities for treatment of inborn errors of metabolism (IEM) EXCEPT
Correct Answer: B
Rationale: Special diets (A), dialysis (C), transplantation (D), and metabolite replacement (E) are established IEM treatments. Gene therapy (B) is experimental and not yet widely used.
Question 4 of 5
In the classic phenylketonurea, the affected infant is normal at birth, intellectual disability develops gradually if the infant remains untreated, and cognitive delay may not be evident for the first few months. All the following are true about neonatal screening program of this condition EXCEPT
Correct Answer: B
Rationale: The Guthrie test (A) was first, levels rise early (C), modern methods reduce false positives (D), and confirmation is needed (E). However, the optimal Guthrie test time is 24-48 hours, not 7-14 days, to ensure feeding elevates phenylalanine.
Question 5 of 5
A 10-day-old boy presented with lethargy, poor feeding, repeated vomiting, and recurrent hypoglycemic fits not responding to IV glucose. On examination, he was hypertonic with severe opisthotonos alternating with flaccidity. Of the following, the MOST likely diagnosis is
Correct Answer: D
Rationale: Maple syrup urine disease (MSUD) presents neonatally with lethargy, vomiting, hypoglycemia, and neurological signs (opisthotonos, tone changes) from branched-chain amino acid buildup, unlike others (A-D).