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Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach.
Aguiar, A; Ahring, K; Almeida, M F; Assoun, M; Belanger Quintana, A; Bigot, S; Bihet, G; Blom Malmberg, K; Burlina, A; Bushueva, T; Caris, A; Chan, H; Clark, A; Clark, S; Cochrane, B; Corthouts, K; Dalmau, J; Dassy, M; De Meyer, A; Didycz, B; Diels, M; Dokupil, K; Dubois, S; Eftring, K; Ekengren, J; Ellerton, C; Evans, S; Faria, A; Fischer, A; Ford, S; Freisinger, P; Gizewska, M; Gokmen-Ozel, H; Gribben, J; Gunden, F; Heddrich-Ellerbrok, M; Heiber, S; Heidenborg, C; Jankowski, C; Janssen-Regelink, R; Jones, I; Jonkers, C; Joerg-Streller, M; Kaalund-Hansen, K; Kiss, E; Lammardo, A M; Lang, K; Lier, D; Lilje, R; Lowry, S.
Afiliação
  • Aguiar A; Hospital de Santo Espirito da Ilha Terceira, Portugal.
  • Ahring K; Kennedy Centre, Department of Clinical Genetics, Rigshospitalet, University of Copenhagen, Glostrup, Denmark.
  • Almeida MF; Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Porto, Portugal; Multidisciplinary Unit for Biomedical Research, UMIB-FCT, Porto, Portugal.
  • Assoun M; Service des Maladies Héréditaires du Métabolisme, Hospital Necker Enfants Malades, Paris, France.
  • Belanger Quintana A; Hospital Ramon y Cajal Madrid, Spain.
  • Bigot S; Centre Hospitalier Universitaire de Rennes, France.
  • Bihet G; Centre Hospitalier Chrétien, Centre Pinocchio Liège, Belgium.
  • Blom Malmberg K; Akademiska University Hospital (Adult Centre), Sweden.
  • Burlina A; Division of Inherited Metabolic Diseases, Department of Pediatrics, University Hospital of Padova, Italy.
  • Bushueva T; Scientific Center of Children's Health, Moscow, Russian Federation.
  • Caris A; Centre Wallon de Génétique Humaine, Maladies Métaboliques, CHU de Liège Sart-Tilman, Belgium.
  • Chan H; Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Clark A; National Centre for Inherited Metabolic Disorders, Dublin, Ireland.
  • Clark S; Addenbrooke's Hospital, Cambridge, UK.
  • Cochrane B; Royal Hospital for Sick Children, Glasgow, Scotland, UK.
  • Corthouts K; University Hospitals Leuven, Center of Metabolic Diseases, Leuven, Belgium.
  • Dalmau J; Hospital La Fe, Valencia, Spain.
  • Dassy M; Cliniques Universitaires St Luc, Brussels, Belgium.
  • De Meyer A; Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
  • Didycz B; University Children's Hospital, Cracow, Poland.
  • Diels M; University Hospitals Leuven, Center of Metabolic Diseases, ZOL, Genk, Belgium.
  • Dokupil K; Dr. von Hauner Children's Hospital, Munich, Germany.
  • Dubois S; Service des Maladies Héréditaires du Métabolisme, Hospital Necker Enfants Malades, Paris, France.
  • Eftring K; Queen Silvia's Children Hospital, Gothenburg, Sweden.
  • Ekengren J; Queen Silvia's Children Hospital, Gothenburg, Sweden.
  • Ellerton C; University College London Hospitals, UK.
  • Evans S; Birmingham Children's Hospital, Birmingham, UK.
  • Faria A; Hospital Pediatrico, Centro Hospitalar e Universitário de Coimbra, EPE, Portugal.
  • Fischer A; Klinikum am Steinenberg, Klinik für Kinder- und Jugendmedizin Reutlingen, Germany.
  • Ford S; North Bristol NHS Trust Southmead and Frenchay, UK.
  • Freisinger P; Klinikum am Steinenberg, Klinik für Kinder- und Jugendmedizin Reutlingen, Germany.
  • Gizewska M; Pomeranian Medical University, Szczecin, Poland.
  • Gokmen-Ozel H; Haccettepe University Children's Hospital, Ankara, Turkey.
  • Gribben J; Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Gunden F; Uludag University Medical Faculty, Bursa, Turkey.
  • Heddrich-Ellerbrok M; Universitätsklinikum Hamburg-Eppendorf, Germany.
  • Heiber S; University Hospital, Basel, Switzerland.
  • Heidenborg C; Karolinska University Hospital, Stockholm, Sweden.
  • Jankowski C; University Hospitals Bristol NHS Foundation Trust, UK.
  • Janssen-Regelink R; Radboud University Nijmegen Medical Centre, Netherlands.
  • Jones I; Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
  • Jonkers C; Academic Medical Hospital, Amsterdam, Netherlands.
  • Joerg-Streller M; Medical University of Innsbruck, Clinic for Pediatrics, Inherited Metabolic Disorders, Austria.
  • Kaalund-Hansen K; University College London Hospitals, UK.
  • Kiss E; Semmelweis University, Hungary.
  • Lammardo AM; San Paolo Hospital, University of Milan, Italy.
  • Lang K; Ninewells Hospital, Dundee, Scotland, UK.
  • Lier D; Klinikum am Steinenberg, Klinik für Kinder- und Jugendmedizin Reutlingen, Germany.
  • Lilje R; Oslo University Hospital Rikshospitalet, Norway.
  • Lowry S; Sheffield Children's NHS Foundation Trust, Sheffield, UK.
Mol Genet Metab ; 115(1): 17-22, 2015 May.
Article em En | MEDLINE | ID: mdl-25862610
ABSTRACT

BACKGROUND:

There appears little consensus concerning protein requirements in phenylketonuria (PKU).

METHODS:

A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics.

RESULTS:

The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day).

CONCLUSIONS:

The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Fragmentos de Peptídeos / Fenilcetonúrias / Proteínas Alimentares / Caseínas / Suplementos Nutricionais / Aminoácidos Limite: Adult / Child / Child, preschool / Female / Humans / Infant / Male / Newborn País como assunto: Asia / Europa Idioma: En Ano de publicação: 2015 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Fragmentos de Peptídeos / Fenilcetonúrias / Proteínas Alimentares / Caseínas / Suplementos Nutricionais / Aminoácidos Limite: Adult / Child / Child, preschool / Female / Humans / Infant / Male / Newborn País como assunto: Asia / Europa Idioma: En Ano de publicação: 2015 Tipo de documento: Article