Chronic therapy for the patient in the previous qu

ATI RN

ATI RN Test Bank

Chronic therapy for the patient in the previous question is best characterized by

A. avoiding fasts for longer than 10-12 hours B. high-fat diet C. low-carnitine diet D. galactose-free diet

Correct Answer: A

Rationale: Avoiding prolonged fasting prevents hypoglycemia in MCAD deficiency by maintaining glucose levels.

Matching: Lipidosis - Tay-Sachs

A. Cherry red spot B. Hepatosplenomegaly C. Both A and B D. Neither A nor B

Correct Answer: A

Rationale: Tay-Sachs features a cherry red spot on the retina.

The best diagnostic test for the patient in the previous question is

A. bone marrow B. assay acid Î²-glucosidase in leukocytes C. ophthalmoscopy for cherry red spot D. urine long-chain amino acids

Correct Answer: B

Rationale: Î²-glucosidase assay in leukocytes confirms Gaucher disease.

Matching: Glycogen storage diseases - Fanconi-Bickel

A. Muscle cramps, exercise intolerance B. Severe hypoglycemia, hepatomegaly C. Cardiomegaly, hypotonia D. Renal tubular dysfunction, rickets

Correct Answer: D

Rationale: Fanconi-Bickel features renal tubular dysfunction and rickets.

What percent of newly diagnosed diabetics present with symptoms of diabetic ketoacidosis (DKA)?

A. 10% B. 20% C. 30% D. 40%

Correct Answer: C

Rationale: Approximately 30% of newly diagnosed type 1 diabetics present with DKA, per pediatric endocrinology data.

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