title: Diet in children with phenylketonuria and risk of cardiovascular disease: A narrative overview.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
authors.affiliation: 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.
authors.forename: E
authors.initials: E
authors.lastname: Verduci
authors.affiliation: 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.
authors.forename: G
authors.initials: G
authors.lastname: Banderali
authors.affiliation: 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.
authors.forename: F
authors.initials: F
authors.lastname: Moretti
authors.affiliation: 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.
authors.forename: C
authors.initials: C
authors.lastname: Lassandro
authors.affiliation: 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.
authors.forename: G
authors.initials: G
authors.lastname: Cefalo
authors.affiliation: 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.
authors.forename: G
authors.initials: G
authors.lastname: Radaelli
authors.affiliation: 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.
authors.forename: E
authors.initials: E
authors.lastname: Salvatici
authors.affiliation: 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.
authors.forename: M
authors.initials: M
authors.lastname: Giovannini
mesh_terms: D052242:Adiponectin / Q000097:blood; D001120:Arginine / Q000031:analogs & derivatives / Q000097:blood; D001794:Blood Pressure; D002318:Cardiovascular Diseases / Q000097:blood / Q000517:prevention & control*; D002648:Child; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D004032:Diet; D006710:Homocysteine / Q000097:blood; D006801:Humans; D018384:Oxidative Stress; D063766:Pediatric Obesity / Q000097:blood / Q000517:prevention & control; D010649:Phenylalanine / Q000008:administration & dosage; D010661:Phenylketonurias / Q000097:blood / Q000150:complications / Q000178:diet therapy*; D012307:Risk Factors
chemical_list: D052242:Adiponectin; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D006710:Homocysteine; D010649:Phenylalanine; C018524:N,N-dimethylarginine; D001120:Arginine
keywords: Cardiovascular risk; Lipid profile; Nutrition; Obesity; PKU diet; Phenylketonuria