During acute metabolic decompensation, all of the

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ATI RN Test Bank

During acute metabolic decompensation, all of the following are appropriate therapy EXCEPT

A. 10% glucose infusion B. albumin infusion C. sodium benzoate D. sodium phenylacetate

Correct Answer: B

Rationale: Albumin infusion is not standard for urea cycle defect crises; others help remove ammonia or provide energy.

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

A. serum amino acids B. nerve conduction studies C. serum very-long-chain fatty acids D. MRI angiography

Correct Answer: C

Rationale: Elevated VLCFAs confirm X-linked adrenoleukodystrophy.

Matching: Lipidosis - Niemann-Pick

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

Correct Answer: B

Rationale: Niemann-Pick primarily causes hepatosplenomegaly.

Metachromatic leukodystrophy, an autosomal recessive white matter disease, is characterized by all of the following EXCEPT

A. presentation between 12 and 18 months of age B. genu recurvatum C. cherry red spot on retina D. myoclonic seizures

Correct Answer: C

Rationale: Cherry red spot is not a feature of metachromatic leukodystrophy; itâ€™s seen in Tay-Sachs.

Disorder of carbohydrate-deficient glycoprotein structure are characterized by all of the following EXCEPT

A. autosomal recessive inheritance B. thrombocytopenia C. facial dysmorphism D. hypotonia

Correct Answer: D

Rationale: Stroke-like episodes are not typical of CDG syndromes; others are common features.

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