Diet Therapy and Nutritional Management of Phenylketonuria

• Other Authors: MacDonald, Anita (Editor)
• Format: Electronic Book Chapter
• Language: English
• Published: MDPI - Multidisciplinary Digital Publishing Institute 2022
• Subjects: Research & information: general; Biology, life sciences; Food & society; phenylketonuria; special low protein foods; nutritional composition; UK; macronutrients; phenylketonuria (PKU); consensus; Delphi method; food labelling; phenylalanine; Phe; protein; exchanges; PKU; glycomacropeptide; amino acid; absorption; diet therapy; protein substitute; gastrointestinal symptoms; prolonged release; satiety; macronutrient intake; protein content; free from; gluten free; aspartame; sugar tax; phenylalanine hydroxylase deficiency; hyperphenylalaninemia; medical formula; amino acid mixture; tetrahydrobiopterin; sapropterin; BH4; body composition; bone mineral density; bone turnover markers; osteoporosis; blood biochemistry; casein glycomacropeptide; amino acid protein substitute; low-protein diet; newborn screening; metabolic control; guidelines; nitrogen balance; amino acid catabolism; blood urea nitrogen; body mass index; obesity; overweight; phenylalanine restriction; phenylalanine-restricted diet; food; school; IHCP; parent/caregiver experiences; England; prescribing patterns; costs; adult phenylketonuria; standard operating procedure; inherited metabolic disorders; dietary management; eating out; low protein food; restaurants; amino acids; adherence; epigenetics; health; preconception; women; dietary patterns; food frequency questionnaire; validation; reproducibility
• Online Access: DOAB: download the publication; DOAB: description of the publication

Phenylketonuria (PKU) is an established inherited amino acid disorder with a very traditional dietary therapy, but there is still more to learn and verify about its nutritional composition, application and overall effectiveness. Although in the 1950s, the first patient successfully treated with diet...