PKU dietary handbook to accompany PKU guidelines
Por:
MacDonald A, van Wegberg AMJ, Ahring K, Beblo S, Bélanger-Quintana A, Burlina A, Campistol-Plana J, Coskun T, Feillet F, Gizewska M, Huijbregts SC, Leuzzi V, Maillot F, Muntau AC, Rocha JC, Romani C, Trefz F and van Spronsen FJ
Publicada:
30 jun 2020
Ahead of Print:
30 jun 2020
Resumen:
Background Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine. Main body In 2017 the first European PKU Guidelines were published. These guidelines contained evidence based and/or expert opinion recommendations regarding diagnosis, treatment and care for patients with PKU of all ages. This manuscript is a supplement containing the practical application of the dietary treatment. Conclusion This handbook can support dietitians, nutritionists and physicians in starting, adjusting and maintaining dietary treatment.
Filiaciones:
MacDonald A:
Dietetic Department, Birmingham Children's Hospital, Birmingham, UK
van Wegberg AMJ:
Division of Metabolic Diseases, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700, RB, Groningen, The Netherlands
Ahring K:
Department of PKU, Kennedy Centre, Glostrup, Denmark
Beblo S:
Department of Women and Child Health, Center for Pediatric Research Leipzig, Hospital for Children and Adolescents, University Hospitals, Leipzig, Germany
Bélanger-Quintana A:
Metabolic Diseases Unit, Department of Paediatrics, Hospital Ramon y Cajal Madrid, Madrid, Spain
Burlina A:
Division of Inherited Metabolic Diseases, Department of Paediatrics, University Hospital of Padova, Padova, Italy
Campistol-Plana J:
Neuropaediatrics Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain
Coskun T:
Hacettepe University Faculty of Medicine, Ankara, Turkey
Feillet F:
Department of Paediatrics, Hôpital d'Enfants Brabois, CHU Nancy, Vandoeuvre les Nancy, France
Gizewska M:
Department of Paediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age, Pomeranian Medical University, Szczecin, Poland
Huijbregts SC:
Department of Clinical Child and Adolescent Studies-Neurodevelopmental Disorders, Faculty of Social Sciences, Leiden University, Leiden, The Netherlands
Leuzzi V:
Department of Paediatrics, Child Neurology and Psychiatry, Sapienza University of Rome, Via dei Sabelli 108, 00185, Rome, Italy
Maillot F:
CHRU de Tours, Université François Rabelais, INSERM U1069, Tours, France
Muntau AC:
University Children's Hospital, University Medical Centre Hamburg-Eppendorf, 20246, Hamburg, Germany
Rocha JC:
Nutrition & Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal. Centre for Health Technology and Services Research (CINTESIS), Porto, Portugal
Romani C:
School of Life and Health Sciences, Aston University, Birmingham, UK
Trefz F:
Department of Paediatrics, University of Heidelberg, Heidelberg, Germany
van Spronsen FJ:
Division of Metabolic Diseases, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700, RB, Groningen, The Netherlands.
Green Submitted, Green Published, Green Accepted, gold
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