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评估一种用于治疗苯丙酮尿症的无苯丙氨酸产品。

Evaluation of a phenylalanine-free product for treatment of phenylketonuria.

作者信息

McCabe E R, Nord A M, Ernest A, McCabe L

机构信息

Department of Pediatrics, University of Colorado, School of Medicine, Denver.

出版信息

Am J Dis Child. 1987 Dec;141(12):1327-9. doi: 10.1001/archpedi.1987.04460120093043.

DOI:10.1001/archpedi.1987.04460120093043
PMID:3318396
Abstract

Ten children with classic phenylketonuria (PKU) participated in a controlled study of a phenylalanine-free formula recently released in the United States (PKU-2). Control data were obtained in the clinic while the children were receiving their baseline formula. The children were given the study formula and returned to the clinic for follow-up after they had been receiving the new formula for 4, 8, and 12 months. Serum phenylalanine and tyrosine concentrations, other hematologic measurements, urine analysis, growth, electroencephalogram, and physical findings remained similar to baseline values throughout the study. Nutrient intakes were comparable with the exception of a decrease in several micronutrients relative to baseline levels. We found that PKU-2 is appropriate for children with PKU who are over 3 years of age and may prove beneficial for the overweight child with PKU.

摘要

十名患有典型苯丙酮尿症(PKU)的儿童参与了一项对美国最近推出的一种无苯丙氨酸配方奶粉(PKU-2)的对照研究。对照数据是在这些儿童接受其基础配方奶粉时在诊所获取的。给这些儿童喂食研究配方奶粉,在他们接受新配方奶粉4个月、8个月和12个月后返回诊所进行随访。在整个研究过程中,血清苯丙氨酸和酪氨酸浓度、其他血液学指标、尿液分析、生长情况、脑电图和身体检查结果均与基线值相似。营养摄入量相当,但有几种微量营养素相对于基线水平有所下降。我们发现PKU-2适用于3岁以上的PKU儿童,可能对超重的PKU儿童有益。

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