Urea Cycle Flashcards
1
Q
What is the treatment for type I hyperammonemia (CPSI deficiency)?
A
Arginine, which increases N-acetylglutamate synthesis.
2
Q
What is the treatment for type 2 hyperammonemia (ornithine transcarbomoylase)?
A
A low protein diet and treatment with phenylacetate and sodium benzoate.
3
Q
What is the treatment for Citrullinemia type I (Argininosuccinate synthase deficiency)?
A
Arginine administration to enhance citrulline excretion and sodium benzoate.
4
Q
What is the treatment for Argininosuccinate lyase deficiency (Argininosuccinic aciduria)?
A
Arginine and sodium benzoate (with an emphasis on sodium benzoate).
