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The Capital Region of Denmark - a part of Copenhagen University Hospital

Large neutral amino acids in daily practice

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  1. The complete European guidelines on phenylketonuria: diagnosis and treatment

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  2. Issues with European guidelines for phenylketonuria - Authors' reply

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  3. Key European guidelines for the diagnosis and management of patients with phenylketonuria

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At the Kennedy Centre for Phenylketonuria, Denmark, large neutral amino acids (LNAAs) are being used to treat adult and adolescent patients who are nonadherent to dietary treatment for phenylketonuria (PKU). At the start of treatment, a patient must undergo dietary analysis and regular blood sampling to measure plasma amino acid (AA) concentrations. The aim of this analysis and treatment is that the patient receives 25-30% of the daily protein requirement from LNAA supplementation and the remaining 70-75% from natural, low-phenylalanine proteins (although some patients have difficulties in maintaining this level of protein intake). Patients are therefore able to follow a more "normal" diet than those adhering to a PKU diet with AA supplementation (in which only 20% of the daily protein requirement is provided from the diet and 80% from AA supplementation). LNAAs have also been used to treat older patients with untreated/late-diagnosed PKU who show profound intellectual, psychological, and behavioral impairments. Treatment with LNAAs has been shown to improve measures of concentration and awareness of external stimuli in some of these patients and thus enhance their socialization, emotionality, frustration tolerance, and mood.

Original languageEnglish
JournalJournal of Inherited Metabolic Disease
Volume33 Suppl 3
Pages (from-to)S187-90
Publication statusPublished - Dec 2010

    Research areas

  • Administration, Oral, Adult, Aged, Aged, 80 and over, Amino Acids, Neutral, Biological Markers, Combined Modality Therapy, Denmark, Diet, Protein-Restricted, Female, Humans, Male, Middle Aged, Phenylalanine, Phenylketonurias, Powders, Tablets, Treatment Outcome, Young Adult

ID: 45088150