ATI RN
Endocrine System Pediatric Questions
Question 1 of 5
A 6-year-old girl that looks tall and thin with light skin. On examination she has a peculiar malar flush, subluxation of the ocular lens, developmental delay, and severe hypertension. Of the following, the MOST likely cause of hypertension is
Correct Answer: C
Rationale: Homocystinuria’s hypertension often stems from thromboembolism due to homocysteine’s vascular effects, fitting this presentation, unlike atherosclerosis (A), hyperthyroidism (B), coarctation (D), or renal stenosis (E).
Question 2 of 5
A 2-year-old girl presented with rapid deep breathing, hypoglycemia, recurrent epistaxis, easy bruising, failure to thrive, fat cheek, and hepatomegaly. Of the following, the MOST likely cause of bleeding tendency is
Correct Answer: C
Rationale: In von Gierke disease, impaired hepatic function from glycogen accumulation reduces clotting factor synthesis, causing bleeding, more than vascular issues (A), platelet count (D), or function (E).
Question 3 of 5
A 7-month-old girl presented with recurrent chest infection, chocking during feeding, large protruded tongue, persistent head lag, and hepatomegaly. Chest X-ray showed cardiomegaly. All the following are beneficial in the treatment of this patient EXCEPT
Correct Answer: A
Rationale: Pompe disease benefits from ERT (E), ventilatory support (D), and transplantation (C). Exercise therapy (A) is ineffective due to muscle glycogen accumulation; high-protein diet (B) may help marginally.
Question 4 of 5
All the following should be restricted in hereditary fructose intolerance EXCEPT
Correct Answer: A
Rationale: HFI requires restricting fructose sources (sorbitol, juice, sugar, cereal, B-E). Milk (A), primarily lactose, is safe and not restricted.
Question 5 of 5
Which of the following mucopolysaccharidoses have normal intelligence?
Correct Answer: D
Rationale: Morquio disease (MPS IV) typically preserves intelligence, unlike Sly (A), Hurler (B), Hunter (C), and Sanfilippo (E), which involve cognitive decline.