Glutaric Aciduria Type I (GA-I) is a rare inherited metabolic disorder of amino acid catabolism with accumulation of neurotoxic metabolites in the brain. Control of dietary intake of certain amino acids improves clinical outcomes. This is important during brain development in early childhood.
Patient selection: glutaric aciduria type I less than 6 years of age
Goals:
(1) reduced intake of lysine (most relevant amino acid precursor of neurotoxic metabolites)
(2) sufficient intake of essential nutrients
(3) sufficient intake of energy substrates
Diet:
(1) control of protein intake, with inclusion of proteins low in lysine
(2) amino acid mixture (AAM) that is free of lysine and that has reduced tryptophan
(3) L-carnitine supplementation, sufficient to maintain normal plasma concentration of free L-carnitine
If the type and amount of protein is known, then the daily intake of lysine can be estimated. For example, the lysine content of cereals is 2-4% (2-4 grams in 100 grams).
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