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- W1551139062 abstract "Amino acids are used to support anabolism throughout the day, but especially following meal ingestion (foods containing carbohydrate, fat and protein). Most patients with amino acid disorders require severe restriction of natural protein intake, and the provision of a suitable precursor-free supplement of L-amino acids is essential to both prevent protein deficiency and optimise metabolic control. The amount of phenylalanine tolerated will vary depending on the severity of the patient's phenotype, age, use of sapropterin, dosage of protein substitute and non-protein energy ratio. It is important that age-related energy requirements are met for optimal dietary protein utilisation. A low protein diet forms an essential part of the management of organic acidaemias, e.g. methylmalonic acidaemia, isovaleric acidaemia, glutaric aciduria type 1 and the urea cycle disorders, e.g. citrullinaemia, ornithine carbamoyl transferase deficiency. This chapter describes the provision of a low protein diet and acute management, common to these disorders." @default.
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- W1551139062 date "2020-06-26" @default.
- W1551139062 modified "2023-10-17" @default.
- W1551139062 title "Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Disorders" @default.
- W1551139062 doi "https://doi.org/10.1002/9781119467205.ch28" @default.
- W1551139062 hasPublicationYear "2020" @default.
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