Next Article in Journal
Dietary Protein Intake and Determinants in Māori and Non-Māori Octogenarians. Te Puāwaitanga o Ngā Tapuwae Kia Ora Tonu: Life and Living in Advanced Age: A Cohort Study in New Zealand
Previous Article in Journal
Comment on: “Indirect Assessment of Skeletal Muscle Glycogen Content in Professional Soccer Players Before and After a Match Through a Non-Invasive Ultrasound Technology Nutrients 2020, 12(4), 971”
Open AccessArticle

The Impact of a Slow-Release Large Neutral Amino Acids Supplement on Treatment Adherence in Adult Patients with Phenylketonuria

1
Neurological Unit, St. Bassiano Hospital, Via dei Lotti 40, 36061 Bassano del Grappa, Italy
2
Division of Inherited Metabolic Diseases, Department of Women and Children’s Health, University Hospital of Padova, Via Giustiniani 3, 35128 Padova, Italy
*
Author to whom correspondence should be addressed.
Nutrients 2020, 12(7), 2078; https://doi.org/10.3390/nu12072078
Received: 28 May 2020 / Revised: 10 July 2020 / Accepted: 10 July 2020 / Published: 14 July 2020
(This article belongs to the Section Nutrition and Metabolism)
The gold standard treatment for phenylketonuria (PKU) is a lifelong low-phenylalanine (Phe) diet supplemented with Phe-free protein substitutes. Adherence to therapy becomes difficult after childhood. Supplementing with large neutral amino acids (LNAAs) has been proposed as an alternative medication to Phe-free protein substitutes (i.e., amino acid mixtures). The aim of this study was to evaluate adherence to therapy and quality of life (QoL) in a cohort of sub-optimally controlled adult PKU patients treated with a new LNAA formulation. Twelve patients were enrolled in a 12-month-trial of slow-release LNAAs (1g/kg/day) plus a Phe-restricted diet. Medication adherence was measured with the Morisky Green Levine Medication Adherence Scale; the QoL was measured using the phenylketonuria-quality of life (PKU-QoL) questionnaire. Phe, tyrosine (Tyr) levels, and Phe/Tyr ratios were measured fortnightly. Before treatment, 3/12 patients self-reported a ‘medium’ adherence to medication and 9/12 reported a low adherence; 60% of patients reported a full adherence over the past four weeks. After 12 months of LNAA treatment, all patients self-reported a high adherence to medication, with 96% reporting a full adherence. Phe levels remained unchanged, while Tyr levels increased in most patients. The Phy/Tyr ratio decreased. All patients had a significant improvement in the QoL. LNAAs may give patients a further opportunity to improve medication adherence and, consequently, their QoL. View Full-Text
Keywords: phenylketonuria; large neutral amino acids; health-related quality of life; adherence; adult patients; PKU-QoL phenylketonuria; large neutral amino acids; health-related quality of life; adherence; adult patients; PKU-QoL
Show Figures

Figure 1

MDPI and ACS Style

Burlina, A.P.; Cazzorla, C.; Massa, P.; Loro, C.; Gueraldi, D.; Burlina, A.B. The Impact of a Slow-Release Large Neutral Amino Acids Supplement on Treatment Adherence in Adult Patients with Phenylketonuria. Nutrients 2020, 12, 2078.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Search more from Scilit
 
Search
Back to TopTop