ATI RN
Endocrine System Pediatric Questions
Question 1 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 2 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.
Question 3 of 5
A definitive diagnosis of mucopolysaccharidoses disorder is established by
Correct Answer: B
Rationale: Enzyme assay confirms MPS by detecting specific enzyme deficiencies, more definitive than biopsy (A), imaging (C), marrow (D), or GAG screening (E).
Question 4 of 5
All the following are features of congenital erythropoietic porphyria (CEP) EXCEPT
Correct Answer: A
Rationale: CEP, a cutaneous porphyria, causes reddish urine (B), erythrodontia (C), photosensitivity (D), and hyperbilirubinemia (E). Neuropathy (A) is typical of acute porphyrias like AIP.
Question 5 of 5
At 10:00 AM Lylaa's mother brought her 3-year-old daughter to emergency department with difficult arousal from sleep, low grade fever, and cough for the last 2 days. Fasting blood sugar was 36 mg/dL with ketonuria. Of the following, the MOST likely diagnosis is
Correct Answer: C
Rationale: Ketotic hypoglycemia, common in young children, presents with fasting hypoglycemia and ketonuria after illness, unlike metabolic disorders (A, B, D, E) with different profiles.