Urea Cycle Defects Flashcards

1
Q

Plasma AA levels and Urine Orotic acid levels for carbamoyl phosphate synthetase 1

A

Inc: Glutamine and alanine

Dec: Citruline

OA: absent/normal

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2
Q

Plasma AA levels and Urine Orotic acid levels for ornithine transcarbamoylase

A

Inc: Glutamine and Alanine

Dec: Citruline

OA: Very High

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3
Q

Plasma AA levels and Urine Orotic acid levels for argininosuccinate synthetase

A

Inc: Very High Citruline

OA: High

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4
Q

Plasma AA levels and Urine Orotic acid levels for argininosuccinate lyase

A

Inc: Argininosuccinate (urine)

Dec: Arginine

OA: High

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5
Q

Plasma AA levels and Urine Orotic acid levels for arginase

A

Very High Arginine

Mildly high Argininosuccinate(urine)

OA: high

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6
Q

Plasma AA levels and Urine Orotic acid levels for NAG synthetase

A

Inc: Glutamine and alanine

Dec: Citruline

OA: absent/normal

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