Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

The mainstay of phenylketonuria treatment is a low protein diet, supplemented with phenylalanine (Phe)-free protein substitutes and micronutrients. Adhering to this diet is challenging, and even patients with good metabolic control who follow the dietary prescriptions in everyday life ignore the rec...

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Autores principales:	Iris Scala, Daniela Concolino, Anna Nastasi, Giulia Esposito, Daniela Crisci, Simona Sestito, Stefania Ferraro, Lucia Albano, Margherita Ruoppolo, Giancarlo Parenti, Pietro Strisciuglio
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	phenylketonuria large neutral amino acids phenylalanine tyrosine PKU LNAA Nutrition. Foods and food supply TX341-641
Acceso en línea:	https://doaj.org/article/aa982d4c67f24c4ca624e5e7b6b14a3c
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spelling	oai:doaj.org-article:aa982d4c67f24c4ca624e5e7b6b14a3c2021-11-25T18:36:06ZBeneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria10.3390/nu131140122072-6643https://doaj.org/article/aa982d4c67f24c4ca624e5e7b6b14a3c2021-11-01T00:00:00Zhttps://www.mdpi.com/2072-6643/13/11/4012https://doaj.org/toc/2072-6643The mainstay of phenylketonuria treatment is a low protein diet, supplemented with phenylalanine (Phe)-free protein substitutes and micronutrients. Adhering to this diet is challenging, and even patients with good metabolic control who follow the dietary prescriptions in everyday life ignore the recommendations occasionally. The present study explores the ability of slow-release large neutral amino acids (srLNAAs) to prevent Phe increase following a Phe dietary load. Fourteen phenylketonuric patients aged ≥13 years were enrolled in a 6-week protocol. Oral acute Phe loads of 250 and 500 mg were added to the evening meal together with srLNAAs (0.5 gr/kg). Phe and tyrosine were dosed before dinner, 2h-after dinner, and after the overnight fast. After oral Phe loads, mean plasma Phe remained stable and below 600 µmol/L. No Phe peaks were registered. Tyrosine levels significantly increased, and Phe/Tyrosine ratio decreased. No adverse events were registered. In conclusion, a single oral administration of srLNAAs at the dose of 0.5 gr/kg is effective in maintaining stable plasma Phe during acute oral loads with Phe-containing food and may be added to the dietetic scheme in situations in which patients with generally good adherence to diet foresee a higher than prescribed Phe intake due to their commitments.Iris ScalaDaniela ConcolinoAnna NastasiGiulia EspositoDaniela CrisciSimona SestitoStefania FerraroLucia AlbanoMargherita RuoppoloGiancarlo ParentiPietro StrisciuglioMDPI AGarticlephenylketonurialarge neutral amino acidsphenylalaninetyrosinePKULNAANutrition. Foods and food supplyTX341-641ENNutrients, Vol 13, Iss 4012, p 4012 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	phenylketonuria large neutral amino acids phenylalanine tyrosine PKU LNAA Nutrition. Foods and food supply TX341-641
spellingShingle	phenylketonuria large neutral amino acids phenylalanine tyrosine PKU LNAA Nutrition. Foods and food supply TX341-641 Iris Scala Daniela Concolino Anna Nastasi Giulia Esposito Daniela Crisci Simona Sestito Stefania Ferraro Lucia Albano Margherita Ruoppolo Giancarlo Parenti Pietro Strisciuglio Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria
description	The mainstay of phenylketonuria treatment is a low protein diet, supplemented with phenylalanine (Phe)-free protein substitutes and micronutrients. Adhering to this diet is challenging, and even patients with good metabolic control who follow the dietary prescriptions in everyday life ignore the recommendations occasionally. The present study explores the ability of slow-release large neutral amino acids (srLNAAs) to prevent Phe increase following a Phe dietary load. Fourteen phenylketonuric patients aged ≥13 years were enrolled in a 6-week protocol. Oral acute Phe loads of 250 and 500 mg were added to the evening meal together with srLNAAs (0.5 gr/kg). Phe and tyrosine were dosed before dinner, 2h-after dinner, and after the overnight fast. After oral Phe loads, mean plasma Phe remained stable and below 600 µmol/L. No Phe peaks were registered. Tyrosine levels significantly increased, and Phe/Tyrosine ratio decreased. No adverse events were registered. In conclusion, a single oral administration of srLNAAs at the dose of 0.5 gr/kg is effective in maintaining stable plasma Phe during acute oral loads with Phe-containing food and may be added to the dietetic scheme in situations in which patients with generally good adherence to diet foresee a higher than prescribed Phe intake due to their commitments.
format	article
author	Iris Scala Daniela Concolino Anna Nastasi Giulia Esposito Daniela Crisci Simona Sestito Stefania Ferraro Lucia Albano Margherita Ruoppolo Giancarlo Parenti Pietro Strisciuglio
author_facet	Iris Scala Daniela Concolino Anna Nastasi Giulia Esposito Daniela Crisci Simona Sestito Stefania Ferraro Lucia Albano Margherita Ruoppolo Giancarlo Parenti Pietro Strisciuglio
author_sort	Iris Scala
title	Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria
title_short	Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria
title_full	Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria
title_fullStr	Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria
title_full_unstemmed	Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria
title_sort	beneficial effects of slow-release large neutral amino acids after a phenylalanine oral load in patients with phenylketonuria
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/aa982d4c67f24c4ca624e5e7b6b14a3c
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Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

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