Inconsistencies in the Nutrition Management of Glutaric Aciduria Type 1: An International Survey.

ABSTRACT: 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 of intact protein to counting milligrams of lysine intake. A 22-question survey on the nutrition management of GA-1 was developed with the goal of understanding approaches to diet management for patients identified by newborn screening under age six years compared to management after diet liberalization,

SUBMITTER: Bernstein L 

PROVIDER: S-EPMC7602866 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature