ATI RN
Endocrine System in Pediatrics Questions
Question 1 of 5
All the following mucopolysaccharidoses are inherited as autosomal recessive EXCEPT
Correct Answer: C
Rationale: Hunter disease (MPS II) is X-linked, unlike autosomal recessive Hurler (A), Scheie (B), Morquio (D), and Sanfilippo (E).
Question 2 of 5
The MOST common electrolyte abnormality in acute intermittent porphyria is
Correct Answer: B
Rationale: Hyponatremia is common in AIP from SIADH or GI losses, more frequent than other imbalances (A, C, D, E).
Question 3 of 5
A 3-year-old boy presented to emergency unit at 9:00 AM with history of difficult arousal from sleep, flue like illness, and low grade fever; the mother stated that he had slept without having his supper. Fasting blood sugar was 42 mg/dL. The following metabolic changes are seen in this condition EXCEPT
Correct Answer: C
Rationale: Ketotic hypoglycemia from fasting and illness shows ketonuria (A), low insulin (B), and elevated cortisol (D) and GH (E). High alanine (C) is not typical, as it’s consumed for gluconeogenesis.
Question 4 of 5
All the following drugs are secondary causes of hypertriglyceridemia EXCEPT
Correct Answer: A
Rationale: Estrogen (B), thiazides (C), β-blockers (D), and steroids (E) increase triglycerides. Tegretol (carbamazepine, A) does not, primarily affecting neurology.
Question 5 of 5
All the following are features of neonatal adrenoleukodystrophy (ALD) EXCEPT
Correct Answer: D
Rationale: Neonatal ALD includes hepatomegaly (A), liver dysfunction (B), hearing loss (C), and retinal changes (E). Chondrodysplasia punctata (D) is more typical of other peroxisomal disorders.