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苯丙酮尿症和轻度高苯丙氨酸血症患者的肌酸水平：一项初步研究。

Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study.

作者信息

Verduci Elvira, Carbone Maria Teresa, Fiori Laura, Gualdi Claudia, Banderali Giuseppe, Carducci Claudia, Leuzzi Vincenzo, Biasucci Giacomo, Zuccotti Gian Vincenzo

机构信息

Department of Pediatrics, Vittore Buzzi Children's Hospital-University of Milan, Via Lodovico Castelvetro, 32, 20154 Milan, Italy.

Department of Health Science, University of Milan, Via di Rudinì 8, 20142 Milan, Italy.

出版信息

Life (Basel). 2021 May 6;11(5):425. doi: 10.3390/life11050425.

DOI:10.3390/life11050425
PMID:34066566
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8148514/
Abstract

BACKGROUND

Creatine (Cr) levels are strongly dependent on diets, including animal-derived proteins. Cr is an important metabolite as it represents a source of stored energy to support physical performance and potentially sustain positive effects such as improving memory or intelligence. This study was planned to assess Cr levels in PKU children adhering to a diet low in phenylalanine (Phe) content and compared with those of children with mild hyperphenylalaninemia (MHP) on a free diet.

METHODS

This retrospective pilot study analyzed Cr levels from Guthrie cards in 25 PKU and 35 MHP subjects. Anthropomorphic and nutritional data of the study populations were assessed, compared and correlated.

RESULTS

Cr levels of PKU subjects were significantly lower than those of MHP subjects and correlated to the low intake of animal proteins. Although no deficiencies in PKU subjects were identified, PKU subjects were found to have a 26-fold higher risk of displaying Cr levels <25° percentile than MHP counterparts.

CONCLUSIONS

This pilot study suggests that Cr levels might be concerningly low in PKU children adhering to a low-Phe diet. Confirmatory studies are needed in PKU patients of different age groups to assess Cr levels and the potential benefits on physical and intellectual performance of Cr supplementation.

摘要

背景

肌酸（Cr）水平很大程度上取决于饮食，包括动物源性蛋白质。肌酸是一种重要的代谢产物，因为它是储存能量的来源，有助于支持身体机能，并可能维持诸如改善记忆力或智力等积极作用。本研究旨在评估坚持低苯丙氨酸（Phe）饮食的苯丙酮尿症（PKU）儿童的肌酸水平，并与自由饮食的轻度高苯丙氨酸血症（MHP）儿童的肌酸水平进行比较。

方法

这项回顾性试点研究分析了25名PKU受试者和35名MHP受试者Guthrie卡片中的肌酸水平。对研究人群的人体测量和营养数据进行了评估、比较和关联分析。

结果

PKU受试者的肌酸水平显著低于MHP受试者，且与动物蛋白摄入量低相关。虽然未发现PKU受试者存在缺乏情况，但发现PKU受试者肌酸水平低于第25百分位数的风险是MHP受试者的26倍。

结论

这项试点研究表明，坚持低苯丙氨酸饮食的PKU儿童的肌酸水平可能低得令人担忧。需要对不同年龄组的PKU患者进行验证性研究，以评估肌酸水平以及补充肌酸对身体和智力表现的潜在益处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/f8873c40e121/life-11-00425-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/b2da95fe1d51/life-11-00425-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/2d434fab0c6d/life-11-00425-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/f8873c40e121/life-11-00425-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/b2da95fe1d51/life-11-00425-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/2d434fab0c6d/life-11-00425-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5775/8148514/f8873c40e121/life-11-00425-g003.jpg

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本文引用的文献

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Creatine Supplementation and Brain Health.肌酸补充与大脑健康。
Nutrients. 2021 Feb 10;13(2):586. doi: 10.3390/nu13020586.
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Creatine in Health and Disease.肌酸在健康与疾病中的作用
Nutrients. 2021 Jan 29;13(2):447. doi: 10.3390/nu13020447.
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The Evolving Applications of Creatine Supplementation: Could Creatine Improve Vascular Health?肌酸补充剂的应用进展：肌酸能改善血管健康吗？
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Creatine as a Neuroprotector: an Actor that Can Play Many Parts.肌酸作为神经保护剂：多才多艺的演员。
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Beyond sports: Efficacy and safety of creatine supplementation in pathological or paraphysiological conditions of brain and muscle.超越运动：肌酸补充在脑和肌肉病理或生理状况下的疗效和安全性。
Med Res Rev. 2019 Nov;39(6):2427-2459. doi: 10.1002/med.21590. Epub 2019 Apr 23.
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Chemistry of Human Breast Milk-A Comprehensive Review of the Composition and Role of Milk Metabolites in Child Development.人乳化学——乳代谢物在儿童发育中的组成和作用的综合评述。
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