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.
ISSN: 17501172
Editorial
BIOMED CENTRAL LTD, 236 GRAYS INN RD, FLOOR 6, LONDON WC1X 8HL, ENGLAND, Reino Unido
Tipo de documento: Review
Volumen: 15 Número: 1
Páginas: 171-171
WOS Id: 000546816600002
ID de PubMed: 32605583
imagen Green Submitted, Green Published, Green Accepted, gold

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