ATI RN
Pediatric Endocrine Nursing Questions Questions
Question 1 of 5
A 28-day-old boy presented with prolonged neonatal jaundice, lethargy, poor feeding, and failure to regain birth weight. On examination there are hepatomegaly and cataract. Of the following, the MOST likely injured organs in this disease are
Correct Answer: A
Rationale: Galactosemia injures eyes (cataracts), liver (jaundice, hepatomegaly), and brain (lethargy) from galactose-1-phosphate toxicity, not kidney (B, C, D) or pancreas (E) prominently.
Question 2 of 5
A 5-year-old boy presented with chronic diarrhea. On examination he had hepatosplenomegaly, coarse facial features, joint stiffness, clear cornea, and short stature. Of the following, the MOST likely diagnosis is
Correct Answer: B
Rationale: Hunter disease (MPS II) matches with hepatosplenomegaly, coarse facies, clear cornea, and short stature, unlike Hurler (A, cloudy cornea), Scheie (C, milder), Morquio (D, skeletal), or Sanfilippo (E, CNS).
Question 3 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 4 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 5 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).