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无苯丙氨酸婴儿配方奶粉在苯丙酮尿症饮食管理中的应用。

Phenylalanine free infant formula in the dietary management of phenylketonuria.

机构信息

Birmingham Women's and Children's Hospital, Birmingham, B4 6NH, UK.

Department of Nutrition and Dietetics, Ankara Yildirim Beyazit University, 06760, Ankara, Turkey.

出版信息

Orphanet J Rare Dis. 2023 Jan 25;18(1):16. doi: 10.1186/s13023-023-02621-9.

Abstract

BACKGROUND

Phenylalanine-free infant formula is an essential source of safe protein in a phenylalanine restricted diet, but its efficacy is rarely studied. We report a multicentre, open, longitudinal, prospective intervention study on a phenylalanine-free infant formula (PKU Start: Vitaflo International Ltd.).

RESULTS

This was a 2-part study: part I (28 days short term evaluation) and part II (12 months extension). Data was collected on infant blood phenylalanine concentrations, dietary intake, growth, and gastrointestinal tolerance. Ten infants (n = 8 males, 80%), with a median age of 14 weeks (range 4-36 weeks) were recruited from 3 treatment centres in the UK. Nine of ten infants completed the 28-day follow-up (one caregiver preferred the usual phenylalanine-free formula and discontinued the study formula after day 14) and 7/9 participated in study part II. The phenylalanine-free infant formula contributed a median of 57% (IQR 50-62%) energy and 53% (IQR 33-66%) of total protein intake from baseline to the end of the part II extension study. During the 12-month follow-up, infants maintained normal growth and satisfactory blood phenylalanine control. Any early gastrointestinal symptoms (constipation, colic, vomiting and poor feeding) improved with time.

CONCLUSION

The study formula was well tolerated, helped maintain good metabolic control, and normal growth in infants with PKU. The long-term efficacy of phenylalanine-free infant formula should continue to be observed and monitored.

摘要

背景

无苯丙氨酸配方奶粉是限制苯丙氨酸饮食中安全蛋白质的重要来源,但对其疗效的研究甚少。我们报告了一项多中心、开放、纵向、前瞻性干预研究,研究对象为一种无苯丙氨酸配方奶粉(PKU Start:Vitaflo International Ltd.)。

结果

这是一项两部分的研究:第 I 部分(28 天短期评估)和第 II 部分(12 个月扩展)。收集了婴儿血苯丙氨酸浓度、饮食摄入、生长和胃肠道耐受情况的数据。10 名婴儿(n=8 名男性,80%)来自英国 3 个治疗中心,中位年龄为 14 周(范围 4-36 周)。10 名婴儿中有 9 名完成了 28 天的随访(1 名照顾者更喜欢常用的无苯丙氨酸配方奶粉,并在第 14 天停止使用研究配方),其中 7/9 名参与了研究的第 II 部分。无苯丙氨酸配方奶粉从基线到第 II 部分扩展研究结束时提供了 57%(IQR 50-62%)的能量和 53%(IQR 33-66%)的总蛋白质摄入。在 12 个月的随访期间,婴儿保持正常生长和令人满意的血苯丙氨酸控制。任何早期胃肠道症状(便秘、绞痛、呕吐和喂养不良)随着时间的推移而改善。

结论

研究配方奶粉耐受性良好,有助于维持苯丙酮尿症婴儿良好的代谢控制和正常生长。无苯丙氨酸配方奶粉的长期疗效应继续观察和监测。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5cec/9878783/086ca0e926a1/13023_2023_2621_Fig1_HTML.jpg

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