Matching: Peroxisomal disorder

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Pediatric Endocrine Nursing Questions Questions

Question 1 of 5

Matching: Peroxisomal disorder

Correct Answer: A

Rationale: All listed conditions (e.g., Zellweger) are peroxisomal disorders.

Question 2 of 5

Matching: Lipidosis - Sandhoff

Correct Answer: C

Rationale: Sandhoff disease has both cherry red spot and hepatosplenomegaly.

Question 3 of 5

Treatment of the patient in the previous two questions is best achieved with

Correct Answer: B

Rationale: Enzyme replacement with β-glucosidase (e.g., imiglucerase) treats Gaucher disease.

Question 4 of 5

Matching: Glycogen storage diseases - Anderson

Correct Answer: D

Rationale: Anderson (type IV) causes cirrhosis and failure to thrive.

Question 5 of 5

Typical insulin dosage requirement in prepubertal children after the 'honeymoon' period is

Correct Answer: D

Rationale: Post-honeymoon, prepubertal children with type 1 DM typically require 0.7-1 U/kg/day; 0.7 (D) is the most common average.

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