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. Long-term outcome in GA-I is difficult to predict and the impact of controlled diet is unknown.
Patient selection: glutaric aciduria type I
While many patients present during infancy or early childhood, late onset can occur during adolescence or early adulthood. It is uncertain how diet can affect these patients.
Dietary recommendations:
(1) sufficient but controlled protein intake using natural protein low in lysine
(2) avoiding lysine rich food
Regular dietary assessments should be conducted in order to adjust diet based on current needs.
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