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

作者信息

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

机构信息

Department of Pediatrics, Oregon Health Sciences University, Portland 97207-0574, USA.

出版信息

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

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

摘要

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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.
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引用本文的文献

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Nutrients. 2023 Aug 17;15(16):3598. doi: 10.3390/nu15163598.
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Protein substitute for children and adults with phenylketonuria.用于苯丙酮尿症儿童和成人的蛋白质替代品。
Cochrane Database Syst Rev. 2015 Feb 27;2015(2):CD004731. doi: 10.1002/14651858.CD004731.pub4.
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The reality of dietary compliance in the management of phenylketonuria.
苯丙酮尿症管理中饮食依从性的现实情况。
J Inherit Metab Dis. 2010 Dec;33(6):665-70. doi: 10.1007/s10545-010-9073-y. Epub 2010 Apr 7.
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Acceptability of a new modular protein substitute for the dietary treatment of phenylketonuria.一种新型模块化蛋白质替代品用于苯丙酮尿症饮食治疗的可接受性。
J Inherit Metab Dis. 2001 Nov;24(6):623-30. doi: 10.1023/a:1012754724708.
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A different approach to treatment of phenylketonuria: phenylalanine degradation with recombinant phenylalanine ammonia lyase.苯丙酮尿症治疗的一种不同方法:用重组苯丙氨酸解氨酶降解苯丙氨酸。
Proc Natl Acad Sci U S A. 1999 Mar 2;96(5):2339-44. doi: 10.1073/pnas.96.5.2339.