Are tablets a practical source of protein substitute in phenylketonuria?

• Published in: Archives of disease in childhood Vol. 88; no. 4; pp. 327 - 329
• Main Authors: MacDonald, A, Ferguson, C, Rylance, G, Morris, A A M, Asplin, D, Hall, S K, Booth, I W
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 01.04.2003; BMJ; BMJ Publishing Group Ltd; BMJ Publishing Group LTD
• Subjects: Adolescent; Adult; amino acid tablets; Amino acids; Amino Acids - administration & dosage; Amino Acids - adverse effects; Amino Acids - blood; Aminoacid disorders; Beverages; Biological and medical sciences; Blood; Body Composition; Carbohydrates; Care and treatment; Child; Child health; Children; Children & youth; Compliance; Cross-Over Studies; Diet therapy; Dietary minerals; Dietary supplements; Dietary Supplements - adverse effects; Diseases; Dosage and administration; Errors of metabolism; Female; Food; General and cellular metabolism. Vitamins; Health aspects; Hospitals; Humans; Male; Measurement Techniques; Medical sciences; Metabolic diseases; Original; Patient Compliance; Patients; Pediatric diseases; Pharmacology. Drug treatments; Phenylalanine; Phenylalanine - blood; Phenylketonuria; Phenylketonurias - blood; Phenylketonurias - therapy; Physiological aspects; Plasma; plasma phenylalanine; Product development; Protein preparations; protein substitute; Proteins; Statistical analysis; Tablets; tyrosine; Tyrosine - blood; Young adults; United Kingdom; Human; Nervous system diseases; Phenylketonuria; Replacement therapy; Phenylalanine; Metabolic diseases; Enzymopathy; Protein; Genetic disease; Blood plasma; Drug compliance; Randomization; Palatability; Child; Aminoacid disorder

Background: A phenylalanine-free amino acid based protein substitute is necessary to provide the major source of protein in phenylketonuria (PKU). Protein substitutes in PKU are usually given as drinks. These are unpalatable and compliance is often poor. Tablets containing a suitable mixture of phen...