PKU dietary handbook to accompany PKU guidelines
Anita MacDonald, Annemiek M. J. van Wegberg, Kirsten Ahring, Skadi Beblo, Amaya Bélanger-Quintana, Alberto Burlina, Josep M. Campistol, Turgay Coşkun, François Feillet, Maria Giżewska, Stephan C. J. Huijbregts, Vincenzo Leuzzi, F. Maillot, Ania C. Muntau, Júlio César Rocha, Cristina Romani, Friedrich K. Trefz, Francjan J. van Spronsen
Abstract
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.