Dietetic Management of Adults with Phenylketonuria (PKU) in the UK: A Care Consensus Document

Nutrients

Melanie Hill¹  Anita MacDonald²  Charlotte Ellerton³  Louise Robertson⁴  Gemma Randles⁵  Carla Young⁶  Sarah Adam⁶  Suzanne Ford⁷  Alison Woodall⁸ 

[1] Adult Inherited Metabolic Disorders Service at Sheffield Teaching Hospitals NHS Foundation Trust, Herries Road, Sheffield S5 7AU, UK;Birmingham Women’s and Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK;Charles Dent Metabolic Unit, University College London Hospitals NHS Foundation Trust, National Hospital for Neurology & Neurosurgery, Queen Square, London WC1N 3BG, UK;Department of Inherited Metabolic Disorders, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham B15 2TH, UK;Guys and St Thomas NHS Foundation Trust, London SE1 7EU, UK;NHS Greater Glasgow and Clyde, Royal Hospital for Children, Glasgow G51 4TF, UK;North Bristol NHS Trust, Southmead Road, Bristol BS10 5NB, UK;Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Stott Lane, Salford M6 8HD, UK;

关键词: phenylketonuria;    adult phenylketonuria;    standard operating procedure;    inherited metabolic disorders;    dietary management;    phenylalanine;   

DOI  :  10.3390/nu14030576

来源: DOAJ

【 摘 要 】

There is an increasing number of adults and elderly patients with phenylketonuria (PKU) who are either early, late treated, or untreated. The principal treatment is a phenylalanine-restricted diet. There is no established UK training for dietitians who work with adults within the specialty of Inherited Metabolic Disorders (IMDs), including PKU. To address this, a group of experienced dietitians specializing in IMDs created a standard operating procedure (SOP) on the dietetic management of adults with PKU to promote equity of care in IMD dietetic services and to support service provision across the UK. The group met virtually over a period of 12 months until they reached 100% consensus on the SOP content. Areas of limited evidence included optimal blood phenylalanine reporting times to patients, protein requirements in older adults, management of weight and obesity, and management of disordered eating and eating disorders. The SOP does not include guidance on maternal PKU management. The SOP can be used as a tool for training dietitians new to the specialty and to raise the standard of education and care for patients with PKU in the UK.

【 授权许可】

Unknown