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Nutrition and Dietetics for Ammonia Production Management

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Nutrition and Dietetics for Ammonia Production Management

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Questions and Answers

What is the purpose of L-arginine supplementation in UCD patients?

To stimulate appetite

To increase ammonia production

To prevent protein catabolism

To prevent arginine deficiency and assist in waste nitrogen excretion (correct)

What is the maximum percentage of total kcal that CHO should not exceed in a low-protein diet for UCD patients?

50% (correct)

40%

60%

30%

What is the frequency of monitoring plasma ammonia levels in UCD patients initially?

Weekly

Monthly

Quarterly

Daily (correct)

What is the purpose of sodium benzoate in UCD patients?

To assist in waste nitrogen excretion Signup and view all the answers

What is the recommended dietary intake of protein for UCD patients?

Less than RDA Signup and view all the answers

What is the purpose of measuring plasma albumin and globulin levels in UCD patients?

To evaluate nutritional adequacy Signup and view all the answers

What is the goal of prescribing sodium benzoate, sodium phenylacetate, or sodium phenylbutyrate in the management of UCD?

To decrease accumulated toxic precursors Signup and view all the answers

What is the role of L-arginine or L-citrulline supplementation in UCD management?

To add a deficient cyclic product Signup and view all the answers

What is the target range for plasma ammonia levels in UCD management?

< 50 µmol/l Signup and view all the answers

Why is protein restriction based on serum ammonia levels in UCD management?

To adjust protein intake according to individual needs Signup and view all the answers

What is the purpose of using an EAA mixture in UCD nutrition therapy?

To supply 50% of the prescribed protein Signup and view all the answers

What is the diagnostic significance of measuring plasma ammonia levels in UCD?

To detect elevated levels indicating UCD Signup and view all the answers

What is the primary function of the urea cycle in the body?

To remove nitrogen from the blood and convert it to urea Signup and view all the answers

What is the consequence of a deficiency in N-acetylglutamate synthetase (NAGS) in the urea cycle?

Decreased plasma citrulline and arginine Signup and view all the answers

What is the primary mode of inheritance for urea cycle disorders (UCDs)?

Autosomal recessive Signup and view all the answers

What is the main clinical presentation of urea cycle disorders (UCDs)?

All of the above Signup and view all the answers

Which of the following is NOT a factor that influences the degree of hyperammonemia in urea cycle disorders (UCDs)?

Age of the individual Signup and view all the answers

What is the typical location where the urea cycle operates?

Primarily in the liver Signup and view all the answers