title: PKU patients on a relaxed diet may be at risk for micronutrient deficiencies.
abstract: OBJECTIVE
  To investigate micronutrient supply in phenylketonuria (PKU) patients on a relaxed diet.
  
  
  METHODS
  Sixty-seven patients (6-45 years) with a phenylalanine tolerance ≥ 600 mg/day were included in the study. From a 3-day diet record, protein supply as well as consumption of essential amino acids and several micronutrients were assessed and compared with the current recommendations and data for the healthy population.
  
  
  RESULTS
  Protein supply and consumption of all essential amino acids were sufficient in all patients. Supply of micronutrients depended on dietary regime. Patients with a total protein supply of 120% or more of the recommended amount and at least 0.5 g protein per kg body weight from amino-acid mixture (AAM) were sufficiently supplied with all investigated micronutrients. All patients without AAM supplement showed severe micronutrient deficiencies in their diet records.
  
  
  CONCLUSIONS
  PKU patients under a relaxed diet are at risk of an insufficient nutrient supply, if they have first no substitution with AAM, second a protein supply less than 0.5 g per kg body weight from AAM or third a total protein supply less than 120% of the recommendations. Therefore, close monitoring, specific dietary counseling and potential supplementation is mandatory to prevent micronutrient deficiencies in PKU patients.
authors.affiliation: Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Rohde
authors.affiliation: Department of Pediatrics, University Hospital, University of Münster, Münster, Germany.
authors.forename: A
authors.initials: A
authors.lastname: von Teeffelen-Heithoff
authors.affiliation: Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: A G
authors.initials: AG
authors.lastname: Thiele
authors.affiliation: Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Arelin
authors.affiliation: Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: U
authors.initials: U
authors.lastname: Mütze
authors.affiliation: Nutricia GmbH, Heilbronn, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Kiener
authors.affiliation: Nutricia GmbH, Heilbronn, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Gerloff
authors.affiliation: Department of Internal Medicine, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Baerwald
authors.affiliation: Center for Obstetrics and Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg-Eppendorf, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Schultz
authors.affiliation: Department of Paediatrics and Adolescent Medicine, University Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Heller
authors.affiliation: Institute of Agricultural and Nutritional Sciences, Martin-Luther-University, Halle-Wittenberg, Germany.
authors.forename: A S
authors.initials: AS
authors.lastname: Müller
authors.affiliation: Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: W
authors.initials: W
authors.lastname: Kiess
authors.affiliation: Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Beblo
mesh_terms: D000293:Adolescent; D000328:Adult; D000596:Amino Acids / Q000008:administration & dosage; D001835:Body Weight; D002648:Child; D003430:Cross-Sectional Studies; D004032:Diet; D015930:Diet Records; D004044:Dietary Proteins / Q000008:administration & dosage; D019587:Dietary Supplements; D006801:Humans; D018977:Micronutrients / Q000008:administration & dosage* / Q000172:deficiency*; D008875:Middle Aged; D010649:Phenylalanine / Q000008:administration & dosage*; D010661:Phenylketonurias / Q000178:diet therapy*; D055815:Young Adult
chemical_list: D000596:Amino Acids; D004044:Dietary Proteins; D018977:Micronutrients; D010649:Phenylalanine