title: Adherence to diet and quality of life in patients with phenylketonuria.
abstract: OBJECTIVE
  To investigate adherence to dietary treatment and quality of life (QoL) in patients with phenylketonuria (PKU).
  
  
  METHODS
  In the setting of a tertiary paediatric hospital, 41 early-treated patients affected by PKU aged more than 3 years old were enrolled in a cross-sectional study. Three-days dietary assessment, QoL questionnaires for patients<18 years old (Child Health Questionnaire) and Short Form for adults were completed.
  
  
  RESULTS
  Of 41 patients, 23 (56.1%) were considered adherent to the dietary prescriptions as their phenylalanine intake was less than prescribed. Phenylalanine intake was significantly in excess of prescribed if mothers had a lower level of education. Adherence was not correlated with age. Metabolic control was obtained in 41.5-51.2% of the patients depending on the target. QoL was reduced in children and adolescents. There was no significant correlation between adherence and QoL, except for the domains of Global Health and Family Activities (ρ=0.42 and 0.46, respectively). The overall agreement between adherence and metabolic control varied according to different targets of metabolic control (51.2-65.9%).
  
  
  CONCLUSIONS
  It is necessary to improve the adherence to diet and the QoL in children and adolescents affected by PKU.
authors.affiliation: Division of Metabolic Diseases, Bambino Gesù Children's Hospital, Rome, Italy. giovanna.cotugno@opbg.net
authors.forename: G
authors.initials: G
authors.lastname: Cotugno
authors.forename: R
authors.initials: R
authors.lastname: Nicolò
authors.forename: S
authors.initials: S
authors.lastname: Cappelletti
authors.forename: B M
authors.initials: BM
authors.lastname: Goffredo
authors.forename: C
authors.initials: C
authors.lastname: Dionisi Vici
authors.forename: V
authors.initials: V
authors.lastname: Di Ciommo
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010349:Patient Compliance; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000097:blood / Q000178:diet therapy*; D011788:Quality of Life; D055815:Young Adult
chemical_list: D010649:Phenylalanine