Phenylketonuria patients and recent approaches

Document Type : Review

Authors

1 Research Department of Food Technology Research, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical Sciences, Tehran, Iran

2 Department NDU Infant Cereal Quality Specialist Société des Produits Nestlé S.A. Nestlé Research Rte de Chavornay 31350 Orbe, Switzerland

3 Halal Research Center of IRI, Iran Food and Drug Administration, Ministry of Health and Medical Education, Tehran, Iran

Abstract

Abstract
Background and objective: Infant formula is a food that mimics human milk and is intended for use by infants under 1 year old. Phenylketonuria (PKU) is a congenital defect in the synthesis of phenylalanine (Phe) and is caused by the failure of phenylalanine hydroxylase converting Phe to tyrosine. In this review the current treatments for PKU are discussed.
Results and conclusion:  Patients with PKU should still be treated with dietary therapy, but in the long term the introduction of a wide array of new treatment approaches such as more palatable foods. Treatment, which includes a low Phe diet supplemented with amino acid formulas, commences soon after diagnosis within the first weeks of life.  Other potential issues associated with dietary therapy by micronutrients. Advances in dietary therapy such as the use of neutral amino acids and glycomacropeptides (GMP) have yielded more promising data in the recent years. In conclusion, GMP medical foods and micro/macronutrient supplementation will be useful in providing the evidence allowing for standardization of management and will alternatively provide in a cost-effective way an individualized management plan for PKU patients.

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Volume 3, Issue 2
December 2022
Pages 37-41
  • Receive Date: 18 December 2022
  • Revise Date: 20 December 2022
  • Accept Date: 20 December 2022
  • First Publish Date: 20 December 2022