Abstract
There is a lack of evidence on the impact on body composition of high protein intake and types of protein substitutes in PKU patients—particularly in adolescents, who are more inclined to dietary transgressions. In this observational, cross-sectional study, PKU patients were observed during prepubertal age (p) or after the pubertal spurt (P), assessing body composition and bone quality and correlating these parameters with dietary compliance and types of protein substitutes. Anthropometric and dietary data were evaluated together with bioelectrical impedance analysis (BIA), quantitative ultrasound (QUS) and branched-chain amino acids (BCAAs). A total of 36 patients (16 males, 17 prepubertal and 19 post-pubertal; mean ± SD age 11.4 ± 3.9 years) were included. A higher BMI was observed in adolescents (p-value: 0.018). The BIA revealed a significant increase in total body water (TBW) and muscle mass (MM) in P subjects either compliant (p-value: 0.001) or non-compliant with the diet (p-value: 0.001). MM content correlated with increased Phe intake (r = 0.63; p < 0.001). In the subgroup of five patients taking L-AAs and glycomacropeptides (GMPs), BCAA values tended to be lower than those taking only L-AA mixtures, with a significant trend for valine. Maintenance of body composition parameters within the normal range—for both fat and muscle mass—and levels of BCAAs can be helpful in reducing the risk of becoming overweight in adulthood. Further studies are needed to confirm these findings.
Subject
Pediatrics, Perinatology and Child Health
Reference64 articles.
1. The complete European guidelines on phenylketonuria: Diagnosis and treatment;Macdonald;Orphanet J. Rare Dis,2017
2. Rodrigues, C., Pinto, A., Faria, A., Teixeira, D., van Wegberg, A.M.J., Ahring, K., Feillet, F., Calhau, C., MacDonald, A., and Moreira-Rosário, A. (2021). Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis. Nutrients, 13.
3. Inherited Metabolic Disorders: Aspects of Chronic Nutrition Management;Boyer;Nutr. Clin. Pract.,2015
4. Dietary treatment of inborn errors of metabolism-a balancing act between indulgence and therapy;Schiergens;Bundesgesundheitsblatt Gesundheitsforschung,2020
5. Nutritional treatment for inborn errors of metabolism: Indications, regulations, and availability of medical foods and dietary supplements using phenylketonuria as an example;Camp;Mol. Genet. Metab.,2012
Cited by
1 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献