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the composition, so administered may be regarded either as a food additive, a substance that is generally regarded as safe (i.e., a GRAS substance), or a drug within the meaning of Title 21 of the Code of Federal Regulations (CFR)

If diagnosed early the disorder can be treated successfully with a combined metabolic treatment course that includes early catabolic emergency treatment and long-term maintenance nutrition therapy

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doi: 10.1016/j.bbr.2019.111902 144 BruntVELaRoccaTJBazzoniAESapinsleyZJMiyamoto-DitmonJGioscia-RyanRAet al