Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study.

Noncompliance is widespread in adults with PKU and is associated with adverse metabolic, nutritional and cognitive abnormalities. Returning to the PKU diet is important for this at-risk population, yet for many this is challenging to achieve. Strategies that ease the return to the PKU diet, while offering nutritional and cognitive advantages, are needed. Twelve PKU adults (33.7 ± 2.6 years), who had been noncompliant for 4.5 years (range: 1 to 11 years), took 33 g of a low-volume, nutrient-enriched, protein substitute daily for 28 days. Outcomes of eating behaviour, nutrient intake and mood were assessed at entry (baseline, days 1-3) and after the intervention period (days 29-31). At baseline, intakes of natural protein and estimated phenylalanine were high (66.4 g and 3318.5 mg, respectively) and intakes of calcium, magnesium, iron, zinc, iodine and vitamin D were below country-specific recommendations. With use of the experimental protein substitute, natural protein and estimated phenylalanine intake declined (

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Employees of Nutricia Ltd.: B.G., G.H. and R.S. Have received educational grants from Nutricia Ltd. for conference attendance: S.A. (Sarah Adam), C.N., S.A. (Sandra Adams), C.D. Have received honorarium from Nutricia Ltd. for conference presentation: S.A. (Sandra Adams) and L.R. Have provided scientific advice and expertise for Nutricia Metabolic Health Professional Advisory Board: Y.R., S.A. (Sarah Adam), L.R., C.D. S.F., F.J., C.D., and A.C. declare no competing interests.