Abstract: Described herein are nutritional compositions, such as ketogenic compositions, leucine-enriched amino acid mixtures, etc., useful, for example, for dietary management of intractable epilepsy. In some embodiments, the disclosure relates to methods of inhibiting epileptic seizures in a subject comprising administering to the subject a leucine-enriched amino acid mixture.

Abstract: A composition for preventing or treating a metabolic disease, in particular phenylketonuria, comprising sulfur amino acids and minerals is provided, wherein said sulfur amino acids comprise a combination of methionine and cysteine in a methionine/cysteine ratio of 2.2-2.7:1 and said minerals comprise sodium, potassium, magnesium, calcium, chlorine, phosphorus. The composition of the invention is formulated to provide a negative PRAL value, preferably with ?35<PRAL<?20 values mEq/day on 100 g of equivalent proteins.

Abstract: Described herein are nutritional compositions, such as ketogenic compositions, leucine-enriched amino acid mixtures, etc., useful, for example, for dietary management of intractable epilepsy. In some embodiments, the disclosure relates to methods of inhibiting epileptic seizures in a subject comprising administering to the subject a leucine-enriched amino acid mixture.

Abstract: Liquid metabolic formulas for dietary management of tyrosinemia, including nutritional formulas and hydration beverages (sport drinks).

Abstract: Described herein are liquid nutritional formulas, such as liquid ketogenic formulas, useful, for example, for management of intractable epilepsy. A ketogenic diet is a high-fat, adequate-protein, low-carbohydrate diet whose consumption shifts metabolism from glycolysis (the metabolism of carbohydrates) and favors ketosis, the metabolism of fatty-acid derived ketone bodies. A ketogenic diet is useful as an alternative therapeutic option for patients with intractable epilepsy and is often the sole source of nutrition, particularly for infants and children.

Abstract: Liquid metabolic formulas for dietary management of tyrosinemia, including nutritional formulas and hydration beverages (sport drinks).

Abstract: In some aspects, the disclosure relates to compositions useful for the dietary management of inborn errors of metabolism (e.g., PKU, MSUD, HCU, IVA, and MMA/PA). In some embodiments, the disclosure provides a composition comprising a protein source, a fat source, a carbohydrate source, at least one vitamin, and at least one mineral, wherein the protein source comprises at least six free amino acids. In some aspects the disclosure relates to compositions that support bone health.

Abstract: Described herein is a nutrition formula, which is a powder that comprises components described herein and can be combined with a liquid, such as water, to produce a milk-like beverage for the dietary management of phenylketonuria (PKU). The powder and resulting beverage have a balanced amino acid profile suitable for dietary management of individuals who have PKU and are lower in calories than powder and milk-like beverages that comprise whey protein, such as cGMP, presently available for use in dietary management of PKU.

Abstract: Liquid metabolic formulas for dietary management of tyrosinemia, including nutritional formulas and hydration beverages (sport drinks).