Use of an amino acid mixture in treatment of phenylketonuria.

Bentovim, A., Clayton, B. E., Francis, D. E. M., Shepherd, J., and Wolff, 0. H. (1970). Archives of Disease in Childhood, 45, 640. Use of an amino acid mixture in the treatment of phenylketonuria. Twelve children with phenylketonuria diagnosed and treated from the first few weeks of life were grouped into pairs. Before the trial all of them were receiving a commercial preparation containing a protein hydrolysate low in phenylalanine (Cymogran, Allen and Hanburys Ltd.) as a substitute for natural protein. One ofeach pair was given an amino acid mixture instead of Cymogran for about 6 months. Use of the mixture involved considerable modification of the diet, and in particular the inclusion of greater amounts of phenylalaninefree foods. All six accepted the new mixture without difficulty, food problems were greatly reduced, parents welcomed the new preparation, and the quality of family life improved. Normal growth was maintained and with a mixture of L amino acids the plasma and urinary amino acid levels were normal. Further studies are needed before the mixture can be recommended for children under 20 months of age.