title: Weight Management in Phenylketonuria: What Should Be Monitored.
abstract: BACKGROUND
  Severe intellectual disability and growth impairment have been overcome by the success of early and continuous treatment of patients with phenylketonuria (PKU). However, there are some reports of obesity, particularly in women, suggesting that this may be an important comorbidity in PKU. It is becoming evident that in addition to acceptable blood phenylalanine control, metabolic dieticians should regard weight management as part of routine clinical practice.
  
  
  CONCLUSIONS
  It is important for practitioners to differentiate the 3 levels for overweight interpretation: anthropometry, body composition and frequency and severity of associated metabolic comorbidities. The main objectives of this review are to suggest proposals for the minimal standard and gold standard for the assessment of weight management in PKU. While the former aims to underline the importance of nutritional status evaluation in every specialized clinic, the second objective is important in establishing an understanding of the breadth of overweight and obesity in PKU in Europe.
  
  
  CONCLUSIONS
  In PKU, the importance of adopting a European nutritional management strategy on weight management is highlighted in order to optimize long-term health outcomes in patients with PKU.
authors.affiliation: Centro de Genx00E9;tica Mx00E9;dica Doutor Jacinto de Magalhx00E3;es, CHP EPE, Universidade Fernando Pessoa, Portugal.
authors.forename: Julio César
authors.initials: JC
authors.lastname: Rocha
authors.forename: Margreet
authors.initials: M
authors.lastname: van Rijn
authors.forename: Esther
authors.initials: E
authors.lastname: van Dam
authors.forename: Kirsten
authors.initials: K
authors.lastname: Ahring
authors.forename: Amaya
authors.initials: A
authors.lastname: Bélanger-Quintana
authors.forename: Katharina
authors.initials: K
authors.lastname: Dokoupil
authors.forename: Hulya
authors.initials: H
authors.lastname: Gokmen Ozel
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Lammardo
authors.forename: Martine
authors.initials: M
authors.lastname: Robert
authors.forename: Carina
authors.initials: C
authors.lastname: Heidenborg
authors.forename: Anita
authors.initials: A
authors.lastname: MacDonald
mesh_terms: D000886:Anthropometry; D001823:Body Composition; D015992:Body Mass Index; D000068959:Body Weight Maintenance; D015897:Comorbidity; D005060:Europe / Q000453:epidemiology; D006801:Humans; D008019:Life Style; D009752:Nutritional Status; D009765:Obesity / Q000097:blood / Q000628:therapy*; D050177:Overweight / Q000097:blood / Q000628:therapy*; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000097:blood / Q000628:therapy*
chemical_list: D010649:Phenylalanine