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Inconsistencies in the Nutrition Management of Glutaric Aciduria Type 1: An International Survey
Glutaric aciduria type 1 (GA-1) is a cerebral organic aciduria characterized by striatal injury and progressive movement disorder. Nutrition management shifted from a general restriction of intact protein to targeted restriction of lysine and tryptophan. Recent guidelines advocate for a low-lysine diet using lysine-free, tryptophan-reduced medical foods. GA-1 guideline recommendations for dietary management of patients over the age of six are unclear, ranging from avoiding excessive intake ofdoi:10.3390/nu12103162 pmid:33081139 fatcat:7ggnu6ozojc3jiyxvh67htigmi