The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis

• Type: Article in International Scientific Journal
• Date: 2018

Authors

Pena, MJ (Author) Other; Pinto, A (Author) Other; Daly, A (Author) Other; MacDonald, A (Author) Other; Azevedo L (Author) FMUP; rocha, jc (Author) Other; Borges, Nuno (Author) FCNAUP

Journal

Title: Nutrients

Vol. 10 No. 11

Final page: 1794

ISSN: 2072-6643

Publisher: MDPI

Indexing

ISI Web of Knowledge - 29 Citations

Scopus - 30 Citations

Scientific classification

CORDIS: Health sciences

FOS: Medical and Health sciences

Other information

Authenticus ID: P-00P-X51

DOI: 10.3390/nu10111794

Abstract (EN): In phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through supplementation with rate-limiting amino acids (GMP-AAs), although it still contains residual Phe. This review aims to systematically evaluate published intervention studies on the use of GMP-AAs in PKU by considering its impact on blood Phe control (primary aim) and changes in tyrosine control, nutritional biomarkers, and patient acceptability or palatability (secondary aims). Four electronic databases were searched for articles published from 2007 to June 2018. Of the 274 studies identified, only eight were included. Bias risk was assessed and a quality appraisal of the body of evidence was completed. A meta-analysis was performed with two studies with adequate comparable methodology which showed no differences between GMP-AAs and AAs for any of the interventions analysed. This work underlines the scarcity and nature of studies with GMP-AAs interventions. All were short-term with small sample sizes. There is a need for better-designed studies to provide the best evidence-based recommendations.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 15