Metabolomic Insights into the Nutritional Status of Adults and Adolescents with Phenylketonuria Consuming a Low-Phenylalanine Diet in Combination with Amino Acid and Glycomacropeptide Medical Foods

Overview

Journal J Nutr Metab

Publisher Wiley

Date 2018 Feb 22

PMID 29464117

Citations 11

Authors

Bridget M Stroup

Denise M Ney

Sangita G Murali

Frances Rohr

Sally T Gleason

Sandra C van Calcar

Harvey L Levy

Affiliations

Soon will be listed here.

Abstract

Background: Nutrient status in phenylketonuria (PKU) requires surveillance due to the restrictive low-Phe diet in combination with amino acid medical foods (AA-MF) or glycomacropeptide medical foods (GMP-MF). Micronutrient profiles of medical foods are diverse, and optimal micronutrient supplementation in PKU has not been established.

Methods: In a crossover design, 30 participants with PKU were randomized to consume AA-MF and Glytactin™ GMP-MF in combination with a low-Phe diet for 3 weeks each. Fasting venipunctures, medical food logs, and 3-day food records were obtained. Metabolomic analyses were completed in plasma and urine by Metabolon, Inc.

Results: The low-Phe diets in combination with AA-MF and GMP-MF were generally adequate based on Dietary Reference Intakes, clinical measures, and metabolomics. Without micronutrient supplementation of medical foods, >70% of participants would have inadequate intakes for 11 micronutrients. Despite micronutrient supplementation of medical foods, inadequate intakes of potassium in 93% of participants and choline in >40% and excessive intakes of sodium in >63% of participants and folic acid in >27% were observed. Sugar intake was excessive and provided 27% of energy.

Conclusions: Nutrient status was similar with AA-MF and Glytactin GMP-MF. More research related to micronutrient supplementation of medical foods for the management of PKU is needed.

Citing Articles

Impact of Phenylketonuria on the Serum Metabolome and Plasma Lipidome: A Study in Early-Treated Patients.

Weerd J, van Wegberg A, Boer T, Engelke U, Coene K, Wevers R Metabolites. 2024; 14(9).

PMID: 39330486 PMC: 11434371. DOI: 10.3390/metabo14090479.

Micronutrient Deficiency in Inherited Metabolic Disorders Requiring Diet Regimen: A Brief Critical Review.

Tummolo A, Carella R, De Giovanni D, Paterno G, Simonetti S, Tolomeo M Int J Mol Sci. 2023; 24(23).

PMID: 38069347 PMC: 10707160. DOI: 10.3390/ijms242317024.

Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria.

Rocha J, Ahring K, Bausell H, Bilder D, Harding C, Inwood A Nutrients. 2023; 15(18).

PMID: 37764724 PMC: 10536918. DOI: 10.3390/nu15183940.

Characterisation and differential diagnosis of neurological complications in adults with phenylketonuria: literature review and expert opinion.

Merkel M, Berg D, Bruggemann N, Classen J, Mainka T, Zittel S J Neurol. 2023; 270(8):3675-3687.

PMID: 37081197 PMC: 10345006. DOI: 10.1007/s00415-023-11703-4.

Characterization of Choline Nutriture among Adults and Children with Phenylketonuria.

Schoen M, Ramakrishnan U, Alvarez J, Ziegler T, Cui X, Singh R Nutrients. 2022; 14(19).

PMID: 36235708 PMC: 9572308. DOI: 10.3390/nu14194056.

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