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A new amino acid mixture permits new approaches to the treatment of phenylketonuria |
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| Authors: | NRM Buist AP Prince KL Huntington JM Tuerck DD Waggoner |
| Affiliation: | Department of Pediatrics, Oregon Health Sciences University, Portland, OR, USA;Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland, OR, USA;Department of Nutrition, Oregon Health Sciences University, Portland, OR, USA;Department of Crippled Children's Division, Oregon Health Sciences University, Portland, OR, USA;Department of Neurology, Oregon Health Sciences University, Portland, OR, USA |
| Abstract: | 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. |
| Keywords: | Amino acids dietary compliance elemental medical foods phenylketonuria |