Literature DB >> 7766965

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

N R Buist1, A P Prince, K L Huntington, J M Tuerck, D D Waggoner.   

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.

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Year:  1994        PMID: 7766965     DOI: 10.1111/j.1651-2227.1994.tb13458.x

Source DB:  PubMed          Journal:  Acta Paediatr Suppl        ISSN: 0803-5326


  3 in total

Review 1.  The reality of dietary compliance in the management of phenylketonuria.

Authors:  Anita MacDonald; Hulya Gokmen-Ozel; Margreet van Rijn; Peter Burgard
Journal:  J Inherit Metab Dis       Date:  2010-04-07       Impact factor: 4.982

2.  A different approach to treatment of phenylketonuria: phenylalanine degradation with recombinant phenylalanine ammonia lyase.

Authors:  C N Sarkissian; Z Shao; F Blain; R Peevers; H Su; R Heft; T M Chang; C R Scriver
Journal:  Proc Natl Acad Sci U S A       Date:  1999-03-02       Impact factor: 11.205

3.  Acceptability of a new modular protein substitute for the dietary treatment of phenylketonuria.

Authors:  F J Rohr; A W Munier; H L Levy
Journal:  J Inherit Metab Dis       Date:  2001-11       Impact factor: 4.982

  3 in total

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