E Verduci1, G Banderali2, F Moretti3, C Lassandro4, G Cefalo5, G Radaelli6, E Salvatici7, M Giovannini8. 1. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: elvira.verduci@unimi.it. 2. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: giuseppe.banderali@unimi.it. 3. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: francesca.moretti@unimi.it. 4. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: carlotta.lassandro@unimi.it. 5. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: graziella.cefalo@unimi.it. 6. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: giovanni.radaelli@unimi.it. 7. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: elisabetta.salvatici@tin.it. 8. Department of Pediatrics, San Paolo Hospital, Department of Health Science, University of Milan, Via A. Di Rudinì 8, I-20142 Milan, Italy. Electronic address: marcello.giovannini@unimi.it.
Abstract
AIMS: The aim of this paper is to review the possible relationship of restricted phenylalanine (Phe) diet, a diet primarily comprising low-protein foods and Phe-free protein substitutes, with major cardiovascular risk factors (overweight/obesity, blood lipid profile, plasma levels of homocysteine, adiponectin and free asymmetric dimethylarginine (ADMA), oxidative stress and blood pressure) in PKU children. DATA SYNTHESIS: In PKU children compliant with diet, blood total cholesterol, low-density lipoprotein cholesterol (LDL-C), plasma ADMA levels and diastolic pressure were reported to be lower and plasma adiponectin levels to be higher compared to healthy controls. No difference was observed in overweight prevalence and in high-density lipoprotein cholesterol (HDL-C) levels. Inconsistent results were found for plasma homocysteine levels and antioxidant status. CONCLUSIONS: PKU children compliant with diet seem to display non-different cardiovascular risks compared with the healthy population. Well-designed longitudinal studies are required to clarify the potential underlying mechanisms associated with PKU and cardiovascular risk factors.
AIMS: The aim of this paper is to review the possible relationship of restricted phenylalanine (Phe) diet, a diet primarily comprising low-protein foods and Phe-free protein substitutes, with major cardiovascular risk factors (overweight/obesity, blood lipid profile, plasma levels of homocysteine, adiponectin and free asymmetric dimethylarginine (ADMA), oxidative stress and blood pressure) in PKUchildren. DATA SYNTHESIS: In PKUchildren compliant with diet, blood total cholesterol, low-density lipoprotein cholesterol (LDL-C), plasma ADMA levels and diastolic pressure were reported to be lower and plasma adiponectin levels to be higher compared to healthy controls. No difference was observed in overweight prevalence and in high-density lipoprotein cholesterol (HDL-C) levels. Inconsistent results were found for plasma homocysteine levels and antioxidant status. CONCLUSIONS:PKUchildren compliant with diet seem to display non-different cardiovascular risks compared with the healthy population. Well-designed longitudinal studies are required to clarify the potential underlying mechanisms associated with PKU and cardiovascular risk factors.
Authors: Fernando Andrade; Olalla López-Suárez; Marta Llarena; María L Couce; Luis Aldámiz-Echevarría Journal: Medicine (Baltimore) Date: 2017-07 Impact factor: 1.889
Authors: María L Couce; Paula Sánchez-Pintos; Isidro Vitoria; María-José De Castro; Luís Aldámiz-Echevarría; Patricia Correcher; Ana Fernández-Marmiesse; Iria Roca; Alvaro Hermida; Miguel Martínez-Olmos; Rosaura Leis Journal: Orphanet J Rare Dis Date: 2018-06-27 Impact factor: 4.123