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European Journal of Clinical Nutrition (2011) 65, 275278
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ORIGINAL ARTICLE
Blood phenylalanine control in phenylketonuria: a survey of 10 European centres
K Ahring1, A Blanger-Quintana2, K Dokoupil3, H Gokmen-Ozel4, AM Lammardo5, A MacDonald6, K Motzfeldt7, M Nowacka8, M Robert9 and M van Rijn10
1Department of PKU, Kennedy Centre, Glostrup, Denmark; 2Unidad Enfermendades Metabolices Servicio de Pediatria Hospital Ramon y Cajal, Madrid, Spain; 3Department of Metabolism and Nutrition, Dr. von Hauner Childrens Hospital, University of Munich, Munich, Germany; 4Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey; 5Department of Pediatrics,San Paolo Hospital University of Milan, Milan, Italy; 6The Childrens Hospital, Birmingham, UK; 7Department of Paediatric Research and Division of Pediatrics, Oslo University Hospital-Rikshospitalet, Oslo, Norway; 8Department of Pediatrics, National Research Institute of Mother and Child, Warsaw, Poland; 9Nutrition and Metabolism Unit, Hpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium and 10Section of Metabolic Diseases, Beatrix Childrens Hospital, University Medical Center Groningen, Groningen, The Netherlands
Background: Only limited data are available on the blood phenylalanine (Phe) concentrations achieved in European patients with phenylketonuria (PKU) on a low-Phe diet.
Objective: A survey was conducted to compare blood Phe control achieved in diet-treated patients with PKU of different age groups in 10 European centres.
Methods: Centres experienced in the management of PKU from Belgium, Denmark, Germany, Italy, The Netherlands, Norway, Poland, Spain, Turkey and the United Kingdom provided retrospective audit data of all patients with PKU treated by diet over a 1-year period. Standard questions were used to collect median data on blood Phe concentrations, percentage of blood Phe concentrations below upper target reference ranges and frequency of blood Phe sampling.
Results: Data from 1921 patients on dietary management were included. Blood Phe concentrations were well controlled and comparable across centres in the early years of life. The percentages of blood Phe concentrations meeting each centres local and national target ranges were 88% in children aged up to 1 year, 74% for 110 years, 89% for 1116 years and 65% for adults (416 years). The frequency of home blood sampling, compared with local and national recommendations for monitoring Phe concentrations, appeared to decline with age (from approximately 100% in infancy to 83% in teenagers and 55%...