A new amino acid mixture permits new approaches to the treatment of phenylketonuria

Acta Paediatr Suppl. 1994 Dec:407:75-7. doi: 10.1111/j.1651-2227.1994.tb13458.x.


A new amino acid mixture for incorporation into medical foods for the treatment of hyperphenylalaninemia has been tested in a regular clinic. The mix is designed to be as unobtrusive as possible, consistent with good nutrition. After more than 1 year of trial as a beverage, we have shown that it is safe and well tolerated but that plasma phenylalanine is no better controlled than with some other products. The mix can be incorporated into a large number of different foods without affecting the taste. Occult monitoring of the quantity of medical foods purchased compared with the amounts reported to be consumed in diet histories provides an excellent way to monitor dietary compliance.

Publication types

  • Clinical Trial
  • Randomized Controlled Trial
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acids / administration & dosage*
  • Amino Acids / blood
  • Beverages*
  • Child
  • Child Nutritional Physiological Phenomena
  • Child, Preschool
  • Food, Formulated*
  • Humans
  • Nutrition Assessment
  • Patient Compliance
  • Phenylalanine / blood
  • Phenylketonurias / blood
  • Phenylketonurias / diet therapy*


  • Amino Acids
  • Phenylalanine