Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach.

Autor: 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., Giżewska 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., Luyten K; Center of Metabolic Diseases, University Hospital, Antwerp, Belgium., MacDonald A; Birmingham Children's Hospital, Birmingham, UK. Electronic address: anita.macdonald@bch.nhs.uk., Meyer U; Clinic of Paediatric Kidney, Liver and Metabolic Diseases Medical School Hannover, Germany., Moor D; Kinderspital Zürich, Switzerland., Pal A; Akademiska University Hospital (Children's Centre), Sweden., Robert M; Hôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium., Robertson L; Northern General Hospital, Sheffield, UK., Rocha JC; Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Porto, Portugal; Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Porto, Portugal; Center for Health Technology and Services Research (CINTESIS), Portugal., Rohde C; Hospital for Children and Adolescents, University Hospitals, University of Leipzig, Germany., Ross K; Royal Aberdeen Children's Hospital, Scotland, UK., Saruhan S; Haccettepe University Children's Hospital, Ankara, Turkey., Sjöqvist E; Children's Hospital, University Hospital Skåne, Sweden., Skeath R; Great Ormond Street Hospital for Children NHS Trust, London, UK., Stoelen L; Oslo University Hospital Rikshospitalet, Norway., Ter Horst NM; Emmakinderziekenhuis AMC, Netherlands., Terry A; Alderhey Children's Hospital, Liverpool, UK., Timmer C; UMC, Utrecht, Netherlands., Tuncer N; Dokuz Eylül University Nevvar-Salih İşgören Children Hospital, Turkey., Vande Kerckhove K; University Hospitals Leuven, Center of Metabolic Diseases, Leuven, Belgium., van der Ploeg L; University Hospital Maastricht, Netherlands., van Rijn M; University of Groningen, University Medical Center, Groningen, Netherlands., van Spronsen FJ; University of Groningen, University Medical Center, Groningen, Netherlands., van Teeffelen-Heithoff A; Univ.-Kinderklinik, Pädiatrische Diätetik, Munster, Germany., van Wegberg A; Radboud University Nijmegen Medical Centre, Netherlands., van Wyk K; Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK., Vasconcelos C; Centro Hospitalar São João - Unidade de Doenças Metabólicas, Porto, Portugal., Vitoria I; Hospital La Fe, Valencia, Spain., Wildgoose J; Bradford Teaching Hospital NHS Trust, UK., Webster D; University Hospitals Bristol NHS Foundation Trust, UK., White FJ; Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK., Zweers H; Radboud University Nijmegen Medical Centre, Netherlands.
Jazyk: angličtina
Zdroj: Molecular genetics and metabolism [Mol Genet Metab] 2015 May; Vol. 115 (1), pp. 17-22. Date of Electronic Publication: 2015 Mar 26.
DOI: 10.1016/j.ymgme.2015.03.006
Abstrakt: 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.
(Copyright © 2015 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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