Morphofunctional Assessment beyond Malnutrition: Fat Mass Assessment in Adult Patients with Phenylketonuria—Systematic Review

• Published in: Nutrients Vol. 16; no. 12; p. 1833
• Main Authors: Luengo-Pérez, Luis M, Fernández-Bueso, Mercedes, Guzmán-Carmona, Carlos, López-Navia, Ana, García-Lobato, Claudia
• Format: Journal Article
• Language: English
• Published: Basel MDPI AG 01.06.2024; MDPI
• Subjects: Body composition; body fat; Body mass index; cardiovascular risk; Clinical trials; Complications and side effects; Cross-sectional studies; Disease; Females; Health aspects; Longitudinal studies; Malnutrition; Medical screening; metabolic diseases; Metabolic disorders; morphofunctional assessment; Nutritional status; Obesity; Pediatrics; Phenylketonuria; Physiological aspects; Risk factors; Systematic Review; Spain; United States > US
• ISSN: 2072-6643; 2072-6643
• DOI: 10.3390/nu16121833

Morphofunctional assessment was developed to evaluate disease-related malnutrition. However, it can also be used to assess cardiometabolic risk, as excess adiposity increases this risk. Phenylketonuria (PKU) is the most prevalent inherited metabolic disease among adults, and obesity in PKU has recently gained interest, although fat mass correlates better with cardiometabolic risk than body mass index. In this systematic review, the objective was to assess whether adult patients with PKU have higher fat mass than healthy controls. Studies of adult PKU patients undergoing dietary treatment in a metabolic clinic reporting fat mass were included. The PubMed and EMBASE databases were searched. Relevance of articles, data collection, and risk of bias were evaluated by two independent reviewers. Ten articles were evaluated, six with a control group, including 310 subjects with PKU, 62 with mild hyperphenylalaninemia, and 157 controls. One study reported a significant and four a tendency towards an increased fat mass in all patients or only females with PKU. Limitations included not having a healthy control group, not reporting sex-specific results and using different techniques to assess fat mass. Evaluation of fat mass should be included in the morphofunctional assessment of cardiometabolic risk in adult patients with PKU.