Question
Which of the following is the most appropriate management for phenylketonuria?
| A. |
a gluten-free diet from age 6 months to 12 years
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| B. |
supplemental insulin
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| C. |
enteral feeding
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| D. |
tyrosine supplements
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Show Answer
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Correct Answer � D
Explanation
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|
Ans. (D)
In phenylketonuria, a low phenylalanine diet with relentless attention to details of diet is required for a good outcome.
The diet should be started by 3 weeks of age.
Children of mothers with phenylketonuria can be affected if exposed to phenylalanine in utero.
Therefore, women with the disorder should stay on a restricted diet until they complete childbearing.
In phenylalanine hydroxylase deficiency, tyrosine becomes an essential amino acid and dietary supplements must be provided.
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