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ATI RN · Obstetrics and Pediatrics · Endocrine System Pediatric

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Showing all 5 practice questions with expert explanatory rationales.

Question 1 of 5

Which of the following mucopolysaccharidoses have normal intelligence?

A. Sly disease
B. Hurler disease
C. Hunter disease
D. Morquio disease

Expert Explanatory Rationale

Correct Answer: Morquio disease

Question 2 of 5

Inborn errors of metabolism (IEM) are hereditary biochemical disorders caused by single-gene mutations that result in alteration of

A. chromosomal number or structure
B. mitochondrial synthesis or function
C. primary fat structure or the amount of fat synthesized
D. primary protein structure or the amount of protein synthesized

Expert Explanatory Rationale

Correct Answer: primary protein structure or the amount of protein synthesized

Question 3 of 5

Hypoglycemia may be a manifestation of child abuse or Munchausen syndrome by proxy induced by exogenous administration of insulin (i.e., factitious hyperinsulinemia). Of the following, the MOST reliable test that confirms the diagnosis of factitious hyperinsulinemia is

A. very low concentration of C-peptide
B. very low level of blood glucose
C. presence of ketones in urine
D. presence of ketones in serum

Expert Explanatory Rationale

Correct Answer: very low concentration of C-peptide

Question 4 of 5

All the following should be restricted in hereditary fructose intolerance EXCEPT

A. milk
B. sorbitol
C. fruit juice
D. table sugar

Expert Explanatory Rationale

Correct Answer: milk

Question 5 of 5

A 1-week-old infant presents with poor feeding, vomiting, lethargy, and opisthotonus posturing. The critical impression is meningitis, but all cultures are negative. Seizures occur and hypoglycemia is documented; however, the seizures do not stop after the hypoglycemia is corrected with intravenous glucose. The arterial blood gas is 7.10 PCO2 23, PO2 90, with an anion gap of 35. A CT scan demonstrates cerebral edema. The most likely etiology is

A. kernicterus
B. Reye syndrome
C. nesidioblastosis
D. organic acidemia

Expert Explanatory Rationale

Correct Answer: organic acidemia

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