1 - Biomedical Sciences Department, University of Extremadura
2 - Clinical Nutrition and Dietetics Unit, Badajoz University Hospital,
3 - Reference Centre of Inherited Metabolic Diseases, Centro Hospitalar Universitário de Lisboa Central, Rua
4 - CHRC—Comprehensive Health Research Centre, Nutrition Group, NOVA Medical School, Universidade Nova de Lisboa,
5 - NOVA Medical School (NMS), Faculdade de Ciências Médicas (FCM), Universidade NOVA de Lisboa,
6 - CINTESIS@RISE, Nutrition and Metabolism, NOVA Medical School (NMS), Faculdade de Ciências Médicas,NMS, FCM, Universidade NOVA de Lisboa
7 - Birmingham Children’s Hospital,
- Artigo Cientifico: Nutrients. 2023 Dec 18;15(24):5133. doi: 10.3390/nu15245133.
Cardiovascular diseases are the main cause of mortality worldwide. Patients with phenylketonuria (PKU) may be at increased cardiovascular risk. This review provides an overview of clinical and metabolic cardiovascular risk factors, explores the connections between body composition (including fat mass and ectopic fat) and cardiovascular risk, and examines various methods for evaluating body composition. It particularly focuses on nutritional ultrasound, given its emerging availability and practical utility in clinical settings. Possible causes of increased cardiometabolic risk in PKU are also explored, including an increased intake of carbohydrates, chronic exposure to amino acids, and characteristics of microbiota. It is important to evaluate cardiovascular risk factors and body composition in patients with PKU.We suggest systematic monitoring of body composition to develop nutritional management and hydration strategies to optimize performance within the limits of nutritional therapy.
Palavras Chave: phenylketonuria; body composition; bioelectrical impedance; anthropometry; microbioma;