Costs and outcomes over 36 years of patients with phenylketonuria who do and do not remain on a phenylalanine-restricted diet

Journal of Intellectual Disability Research : JIDR
J F GuestR H Lachmann

Abstract

To quantify the costs and consequences of managing phenylketonuria (PKU) in the UK and to estimate the potential implications to the UK's National Health Service (NHS) of keeping patients on a phenylalanine-restricted diet for life. A computer-based model was constructed depicting the management of PKU patients over the first 36 years of their life, derived from patients suffering from this metabolic disorder in The Health Improvement Network database (a nationally representative database of patients registered with general practitioners in the UK). The model was used to estimate the incidence of co-morbidities and the levels of healthcare resource use and corresponding costs over the 36 years. Patients who remained on a phenylalanine-restricted diet accounted for 38% of the cohort. Forty-seven per cent of patients discontinued their phenylalanine-restricted diet between 15 and 25 years of age. Of these, 73% remained off diet and 27% restarted a restricted diet at a mean 30 years of age. Fifteen per cent of the cohort had untreated PKU. Eleven per cent of patients who remained on a phenylalanine-restricted diet for 36 years received the optimum amount of prescribed amino acid supplements. Patients had a mean 12 general practiti...Continue Reading

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Citations

Sep 21, 2013·Molecular Genetics and Metabolism·A WiedemannF Feillet
Dec 22, 2017·NPJ Genomic Medicine·Brett DobleJohn S Mattick
Nov 2, 2019·Journal of Inherited Metabolic Disease·Emma R L C VardyDenise L Hofman
Feb 20, 2019·Clinical Drug Investigation·Josep Darbà, Meritxell Ascanio
Jan 7, 2014·Journal of Human Nutrition and Dietetics : the Official Journal of the British Dietetic Association·B CochraneK Gerasimidis
Jul 22, 2016·Nursing·Janice SampsonDenise M Wall Parilo
May 27, 2021·Molecular Genetics and Metabolism Reports·Friedrich TrefzFrank Rutsch

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