ATI RN
Pediatric Endocrine System Questions
Question 1 of 5
In von Gierke disease; treatment is designed to maintain normal blood glucose levels which is achieved by all the following EXCEPT
Correct Answer: C
Rationale: Von Gierke treatment avoids fructose (A) and galactose (B), uses NG glucose (D) and cornstarch (E) to maintain glucose. Table sugar (sucrose, C) worsens the condition.
Question 2 of 5
Hunter disease is characterized by all the following features EXCEPT
Correct Answer: B
Rationale: Hunter disease (MPS II) features short stature (A), dysostosis (C), intellectual disability (D), and coarse facies (E), but lacks corneal clouding (B), unlike MPS I.
Question 3 of 5
Which of the following mucopolysaccharidoses is susceptible to life-threatening atlantoaxial instability and dislocation?
Correct Answer: D
Rationale: Morquio disease (MPS IV) has severe skeletal dysplasia, including atlantoaxial instability, risking spinal cord compression, unlike others (A, B, C, E).
Question 4 of 5
The least common presentation of acute intermittent porphyria is
Correct Answer: A
Rationale: Sensory loss (A) is less common in AIP than mental symptoms (B), weakness (C), abdominal pain (D), or extremity pain (E), which dominate acute attacks.
Question 5 of 5
The following are counter regulatory hormones which are increased as blood glucose falls EXCEPT
Correct Answer: D
Rationale: Cortisol (A), glucagon (B), epinephrine (C), and growth hormone (E) counter hypoglycemia. Thyroid hormone (D) does not acutely respond to glucose drops.